Carolyn's Chiari

Emotional Breakdown Days

2011-12-07T22:38:00.003-06:00

So, some days, I just have to break down. I feel it building over time. I know the breakdown is coming. But it's still so overwhelming when it finally hits.

Today, there were several factors contributing. I've just started rebuilding a home piano studio from nothing, after having to stop teaching 2 years ago (and once before that). So, after advertising for months, I finally have some new students. It seems like it should be easy: one student a day, four days a week. But I've been completely disabled for 2 full years (and a while before that). And I've been working so hard on rebuilding my health since my cranio-cervical fusion in August 2010. I know from my first fusion in 2003 that it takes about 2 years to fully rebuild. But I also know it doesn't always work, since my first fusion failed. I've been devoting all my energy to getting healthy (and planning my wedding, which was last summer, then marketing my new piano studio in a new city). But still, most of my energy since surgery has gone toward getting healthy. I eat healthy. I allow plenty of time for sleep (but can't actually sleep lately). I don't push myself too hard very often. But I do push myself enough to get the minimum of exercise needed each day to keep my POTS under control. But in reality, I spend a lot of time resting and taking care of myself.

But it just ISN'T ENOUGH. I am still SO sick. A GOOD day for me is when I get myself breakfast, take my pills, rest in the recliner for 2 hours, get my lunch, take my pills, rest 2-3 more hours...then maybe am able to get dressed by 3:00. 3:00 is a great day! This only happens after I've had tons of salt and water and high doses of Midodrine PLUS caffeine pills to get me going. I can have a couple of "active hours"-meaning not in bed or in the recliner. Most days, I get dressed just before dinner to be honest. And I still can't shower as often as I'd like. About twice a week.

Supposedly, my head has been fused in the optimal position this time....and so many people just like me are completely relieved of symptoms after this surgery....blah blah blah. My head hurts!!! My constant head pain is lower than before...around a 2 at it's lowest. But about half the days, I get a migraine, cluster headache, or a pressure headache that blasts the pain to a 7-10. And these headaches are all day headaches. And that's just my head.

Today, I had a baseline headache of about 4, but I had severe nausea all day. No vomiting. I know, I'm lucky there's never vomiting. But nausea and zero appetite are pretty freaking miserable too. Of course, sometimes I can eat, but my GI tract doesn't know how to digest food, and I get very sick from that. Anyway, today I was super-nauseated with a mid-range headache. I got through breakfast and lunch. And then, I crashed, which is a possibility every day. Something in my careful daily formula didn't work right, and I just never got going. I could barely move. When my husband got home around 3:30, I couldn't react to him. I could barely see through my slits of eyes. He rushed and got me a salt pill and water. That didn't work so we got a caffeine pill. I chugged 20 ounces of water. About 20 minutes later, I was finally a bit awake. But not enough to get up...just to open my eyes and maintain a conversation.

I had to teach a piano lesson at 5:45. And, like magic, I pulled it off. It's amazing how much you can fake. It took everything I had.

Then, the realization set in. I have a new piano student tomorrow. I have another new student on Monday and another new one on Tuesday. What in the world am I thinking??? How do I expect to build a business with my health like this? I didn't make it to the music store, so I don't have all the materials for the new student tomorrow. Plus, tomorrow is a 3:00 lesson!!! Why did I ever agree to teach at 3:00! And what am I thinking taking on new students when I can barely survive a day?

That's when it all just broke down. I just had to cry and cry at my mess of a life...at how hard it all is...at how pointless all of this hard work seems. I rebuild a business for what? Just to get overworked and end up having to quit again??? And disability won't even pay me since my marriage. So I can't even help that way.

But it's not just that. After we got married, I realized that I could get dressed most days, and my husband was gone a lot of the time. And I wanted something to do with my time. Something useful. Something to help out. Especially since we are barely getting by right now...or actually not really getting by.

Accepting less from myself is something I've done so many times. There were very high hopes for my life. I was supposed to do something great. I had straight A's, was talented, excelled at everything I did. I could do anything. Then, in high school, I had to start accepting less. At first, I just took one less AP class. Then, I went to a small school, cause it was close to home. In college, I had to take a year off for my first cranio-cervical fusion. I had such trouble picking a major, because I wanted to do more, but had to accept less. I finally settled on an interdepartmental major...just so I could graduate. And chose a career that I had already been doing for a few years, but didn't really enjoy. I went on disability at 22, and was in an electric wheelchair. I rebuilt, but got knocked back down and had surgery again at 26. I was down for a while. Now I'm rebuilding again. But it feels like a pointless cycle. Rebuild your life. Become more. Do something with yourself. Get knocked down so hard you have no choice but to do nothing. Accept less. Just survive. Start to rebuild again...

I don't see the point right now. It's all so hard. But for what? Should I even be doing this? Or should I accept that I can't do this? That my body is just not getting better and this cycle is pointless. That no matter how hard I try, my body will fail me. That the other shoe will always drop. This EDS body is not getting stronger. It's getting harder to hold together. My joints will continue to hurt worse my whole life. These symptoms are not going away. Headache is not leaving. Extreme fatigue is going nowhere. It's all just learning to cope. Learning new ways to manage. But it's not really about getting better anymore, is it? I'm past that point. I will have "good" and bad days. But I won't get better. This is the body I'm stuck with for life. And I should know from experience, that acceptance is the real key to emotional coping. If I can just accept that this is the state of my body, then I will be able to deal with it.

There's no more fight to "recover." It's just an ultra-marathon now...the kind with frequent obstacles, and uphill 90% of the time. It's all about endurance. And the only way to endure is to stay strong. Accept the course of your life. Accept this is the crappy body you're stuck with for life. There will be some better days. But most of them will be the same miserable fight you've been dealing with for 10 years.

I can't imagine why anyone would read this. But I had to write it today. It just got to be too much today. Some days are brighter. I know this intellectually. But it's a stormy life. And I've got to hold on tight, cause it's not getting any easier. I know that I wouldn't face this life without my husband, the best friend a girl could ever have, and my mom, who always listens and knows what to say, even when there's nothing to say. I would not deal with all of this without the love and support of my family. I would just give up.

I'm done with metaphors for tonight. Sorry. It's a rough one.

Happy Thanksgiving

2011-11-22T23:43:00.002-06:00

I'm sorry if my last post painted a sad picture-my mood is definitely affected by the long days alone in my apartment. However, it's not all so bleak. My dear husband, Gmex, is the best partner I could ask for. He is constantly supportive of me. He takes care of me on days I need it, but also encourages me to do as much as I can comfortably do. He is always thinking of what might make my life easier and more comfortable. He's very patient when my body gives out or my mood plummets (or both happen at the same time!). All while working toward his PhD way more than full-time.

I couldn't have asked for or expected to find such an ideal life partner for myself. I know I'm a very lucky one, as chronic illness destroys so many relationships. We just make the most out of the time we can spend together. I obviously try to be as supportive and helpful as possible, but he deserves most of the credit for making it work. I know there are more good men out there. But I have the only one I'll ever need. I hope that everyone in my life finds this kind of satisfaction, recognizes it, and appreciates it.

In other news, I'm dealing with migraines that may be cluster headaches. Unfortunately, insurance only covers 8 Relpax a month, and I have the torture headache more than 8 consecutive days when they come. It's gone for now, but I am so scared for when it will start again. I have an appointment at a headache clinic in January.

I had an ob-gyn scare, but the biopsy came back clear. Ultrasound will be tomorrow.

Finally, there is a fascinating new theory on the cause of the EDS/POTS/Cranio-cervical Instability conundrum. Dr. Diana Driscoll has these conditions, as do her children, and she's been conducting clinical trials to support her theory. You can find it at http://www.prettyill.com.

Hope everyone is doing well. Happy Thanksgiving!

Married Life

2011-10-05T13:01:00.002-05:00

I've been married for 3 months and living in Wisconsin with my husband. This means I don't live near family right now. So I spend a lot of time alone, while my husband is at work...and I mean, a lot of time.

I've been seeking work, but it's quite difficult getting a new piano studio going in a new area where you don't know everyone.

My health is still up and down, but reaching a better point, I believe. It's hard to write that at a time when I don't feel good at all, but overall, I know it's true.

I sleep about 10 hours now, down from 12, which has changed things quite a bit. Getting off of Topamax was difficult (insomnia, migraines...), but it was really decreasing my energy, so I'm glad to be off of it.

A Day in The Life
I tend to wake up around 10:00. I eat breakfast and rest until noon. On a good day, I try to get dressed and ready for the day from 12:00-1:00. I eat lunch at 1:00. Then, by 2:00, I'm ready to start the day. This only happens on good days, but it's a big improvement over the 6:00pm ready time I used to have.

This means that I really could work a few days a week pretty successfully. I just have only 1 student right now.

It's a bit of a difficult adjustment, getting used to being a "housewife." I spend the days doing laundry, cleaning, cooking, etc. I don't really mind. But it is lonely. I am proud when I have really productive days. But so many days are not too productive, and that's when I tend to get down. Finding piano students is mostly a waiting game. I put my information out there, but then just wait for someone to call.

So, I'm off to make today a more productive day. Gotta start somewhere. Why not laundry and a shower? Hopefully I'll be able to pull it off today.

Massage Miracle

2011-08-12T19:46:00.002-05:00

Okay. I would never do this. But when something works, it works.

I think you can tell how desperate my situation has been. Bedridden.

June recommended trying massage therapy, heat, and muscle relaxants for 2 weeks, before we do any scans, in case I strained a muscle and had spasms going on from overuse.

June was right.

But more unbelievable than the fact that muscle injury could have been causing my vast array of symptoms is the fact that I found a brand new massage therapist in a new city, and he gave me the most effective massage of my life. In the past, I've never had a massage with noticeable lasting results before. But this was a new kind of massage. He called it neuromuscular. It didn't hurt. It was very slow motion. I didn't know it could possibly help so much. When he told me the effects would be more noticeable and improving over the following 24, 48, and 72 hours, I smiled, but didn't really believe him. But I wanted to.

But then, he was right. It's only been 48 hours actually, but the pain in my upper back is drastically improved. And I believe that my crazy EDS muscles were pulling on my spine and irritating my nerves. Because other symptoms have improved too, especially the nausea.

I do have a migraine today. As soon as I see him again, I will ask if he can work more on my neck to try to help the migraine area more. But he has already given me such a huge gift.

So, I'd like to share his website with anyone who may be in the Milwaukee area...even for a day. Just go see him. I'd even travel from surrounding areas to see him if I didn't live here. His name is Stuart Blystone. From my experience, he knows what he's doing, and he can really help.

Here's Stuart's page:
http://www.milwaukee.massagetherapy.com/

I just wish that all of my Chiari/EDS/fusion friends could find a massage therapist like him.

Maybe one day I'll find a physical therapist that can be helpful like some of my friends have found.

But I'm certainly grateful for the help I've found. I'll let you know how it goes in the future.

(I am simply sharing my own experience, and this should not be seen as a substitute for medical advice. I'm also not getting paid anything to share this information with you. :))

Dear June

2011-08-09T14:17:00.003-05:00

I sent a message to June last night. I think it summarizes my current concerns:

Hi June,

This is Carolyn Richardson. I'm writing with a problem. I'm not sure who to go to for help, but it seems that your and Dr. B's opinion would be the most helpful place to start in trying to figure out my current situation. I got married sixteen days ago. The wedding day was fabulous. I was able to stand a lot of the day and even dance for a couple hours in the evening, though we did sit during the ceremony. Not surprisingly, the first week after the wedding, I rested a lot to recover from the exhausting day.

One week after the wedding, my husband and I moved into a new apartment. This day, there was a lot of cleaning, packing, lifting, and moving into the new place. It was a very strenuous day. I rested a lot, and I didn't carry anything too heavy, but I tried to do my part in any way I could. Since moving day, I have become sicker and sicker, and I have now been completely bedbound for 5 days. I'm having troubled describing the symptoms to family, because they are not exactly like my usual symptoms. They are a little different. If I am not laying flat, I now get a headache and nausea again. But my worst problem is a severe, burning pain in my upper thoracic spine that is very difficult to alleviate without very specific positioning. It also comes with severe nausea. And I've lost my appetite almost completely. I have a sense that my spine is being compressed intensely, and that it makes me choke and gag, along with the pain in my thoracic spine, and later, the headache.

I don't want to get stuck in this whole bedbound thing again, as I had just been getting healthier and I've been exercising regularly. I had even just begun looking for work again. I am on the verge of beginning my new life, in a new state, with my new marriage, healthier than ever. But the move has crushed me.

What should I try at this point? The pain in my back is so intense, I can barely find a tolerable position to rest in. And I can't sit up for long, because the nausea overtakes me. I'm also just transitioning to a new insurance, so I haven't seen anyone locally yet, but I should be able to very soon.

Are there any scans I can have done, to check on my fusion, or on the spine (area between the shoulder blades)? I'm sure I could get my GP to rewrite them.

Do you have any thoughts on what these symptoms could be?

Thank you so much for your help.

The Long Road of Recovery, 10 months post-op

2011-06-14T13:53:00.001-05:00

I know. After four months, you can't really call yourself a blogger. I have excuses and explanations. Instead, I'll try to remember, with my limited memory, what's been going on, or at least where I'm at now, 10 months after my cranio-cervical fusion revision.

The fun stuff: I've been planning my wedding. It's less than 6 weeks away. Am I prepared? Of course not. I'm not magically healed and functioning like a normal person. I've done what I've been able to do and planned what I can, and the rest will get done well enough. I don't have too much anxiety about it though. I'm actually just looking forward to a very happy day.

I am concerned about my stamina on such a long day like a wedding. It's difficult to be "on" all day, when your body feels so "off." It actually makes me quite emotional to have to fake it for too long at a time. But I'm doing all I can do to prepare, like plenty of rest and enough exercise, trying to build up my endurance. I'll also just have to get lucky and have a "good day" that day, plus schedule a lot of breaks during the day, which is not quite typical for a bride, but what I'll need to do.

Onto the next:

SYMPTOMS

Of course, I look back, and I'm so happy for all progress I've made. But I still tend to disappoint myself with my limitations and my remaining symptoms.

Headache--Migraines occur an average of 2-3 days per week. The tryptans help, but only at the higher dose, which makes me drowsy.

My constant headache is still present. It worsens throughout the day. I believe it may be a tension headache due to muscle atrophy and spasm in my neck and shoulders. I try to make it to the massage therapist. I also exercise at the gym now and am really trying to strengthen everything. My head feels heavy, but after wearing a collar for so long, I'm sure those muscles are very weak. It's a slow rebuilding process to try to decrease the tension headaches. (I've never found a physical therapist that was capable of treating me in any useful way, due to the complications related to all of my conditions, so I just manage as best as I can.)

Fatigue--sucks. But I've been working really hard to fight it. Exercise helps (I joined the gym again.) Just getting dressed most days, doing activities around the house, and trying to get out of the house helps build up your stamina, when you don't overdo it. That line is so easy to cross, it's scary. I'm also taking Provigil. It's crazy expensive, so I only take half the prescribed dose, and I divide that dose during the day, because it seems to wear off. I may need to raise my dose though. I have zero caffeine intake, for multiple reasons. I'm working on building up my energy. I get enough sleep, so I should be able to make it through a day. I'm just experimenting with increasing my salt and fluid intake even more, which seems to be helping with POTS/fatigue. Often, other symptoms also limit me, like...

Body pain--joints and muscles. My muscles sometimes ache for no reason. My joints always, always, always hurt. Whichever joints are being used will start to hurt. If I lean on my wrists, they will be so sore. If I walk or stand, my knees will hurt the most, but my spine will hurt, too. I've had just about any body part randomly become almost unusable for stupid reasons, like driving. Random muscles or tendons seem to seize up when I use them (just like my neck does--always). I have shin splints now, so those hurt whenever I walk around or stand still. My feet hurt like heck after I am on them for about an hour, no matter what type of footwear I have. So after a longer period of time, they hurt a lot worse. I also began having jaw pain when I eat anything too crunchy (like raw carrots). These symptoms are ALL Ehlers Danlos symptoms.

POTS symptoms--improved since surgery, but still difficult. I basically never pass out anymore. I also refuse to use a wheelchair anymore, but will on occasion use a "cane seat," which is just a little portable seat, so you don't have to stand in one place. A recent heat wave gave me a POTS setback. I can't be anywhere near heat, or I become almost comatose. And when I say heat, I mean when the house temperature is set at 79 degrees instead of 73 degrees. I don't dare to go outside when it's too hot. Unfortunately, I am that heat sensitive. It makes the difference between a day sprawled out on the couch chugging water and salt, trying to raise my bp, while being basically incoherent, and a day where I can walk all around the house and do laundry, get dressed, shower, etc., with very little rest.

I have plenty of other fascinating symptoms to discuss, but that seems like the major ones for now.

So, my favorite feature of my blog has become:

A Day in the Life

So I'll bring it back now, to show where I'm at.

People see me for a snapshot of time. Time when I'm dressed, perhaps bathed, might even have makeup to cover an outbreak of acne. It's also usually a time when I smile, and pretend I don't feel how I'm feeling. I put things in a positive light, because I've been told a million times to focus on the positive. Apparently positive "energy" is magic. I do recognize my progress, which I couldn't be more grateful for, after how long I've waited for it (first surgery 2002). But people don't ask what I really go through from day to day too often, because that's usually not what they want to hear when they ask "How's it going?"

But here, I'll try to explain an "average" day. Which is quite difficult, if you think about the difference between a good day and a bad day. But I'll just talk about the past week, to make it easy.
Basically, I wake up around 10:30.
I prepare and eat breakfast and take pills.

I prepare and eat lunch around 1:30 and take pills.
I prepare and eat dinner around 5:30 and take pills.
Around 7:00, I either get dressed, or shower and get dressed.
Then, I can begin my day.
I can go to the store, meet up with a friend, go to the gym, clean up around the house, do laundry, etc.
Too bad I have to get ready for bed by 11:00, even though I'm not tired.

I know this begs the question: what do I do all day? Unfortunately, this question always feels like an accusation of laziness. But to satisfy curiosity, I'll try to answer. Due to jaw pain and dysphagia, my meals take a lot of time. (Seriously, an hour each.) I also have to keep my body reclined for much of the early part of the day, due to POTS. I'm still working on finding energy earlier in the day, whether it comes from salt, Provigil, or something else. But lethargy is another reason my day takes so long to kick into gear. Perhaps part of it is a lack of motivation, because I don't have too many things on the schedule right now, but I do usually have a to-do list, with things that I really want to get done.

This isn't to say that I don't ever schedule activities in the afternoon (although I don't in the mornings). And I need to get used to this schedule, because it's the only time I'll be able to work. But when I do go out in the afternoon, I have a hard time getting through anything. Plus, my body pays for it later.

Another part of it seems to be that I seem to zone out a lot during the day. I blame it on a lack of oxygen to the brain. It's like the world moves in slow motion for me. The little on-off switch in my brain is flipped off all day. And on a good day, it gets flipped back on by 7:00 PM.

FUTURE
So, my life is in a transitional stage this summer, and I'm definitely thinking about everything that is going to change. For one thing, on the day of my marriage, I will no longer qualify for disability. I will also be switching to Wisconsin state employee insurance. So, there's a lot to think about and plan for.

The biggest thing left for me to figure out is that I need to start working again. First, I need to replace the disability income with my own income. Then, I need to make additional income to help in my new household. I have to help support myself, and with my medical needs, that costs a lot. But I can't take on too much, or I will overwhelm my body. But I'll be in a new town, so finding new piano students, enough to make the needed money, but not more than my body can handle, will be challenging. And it's always difficult starting a new business from scratch.

I'll try to post again soon...

Progress

2011-02-08T20:19:00.003-06:00

It seems that I've let quite a bit of time get away from me. However, I'm glad to say that a lot of progress has been made in this time. This month is my sixth month after surgery.

I had another week of severe bradycardia, but this time, there was a clear cause: we had just raised my dose of Lithium. So, it is now clear that Lithium has been causing my bradycardia all along, including in the time right after surgery. I quickly weaned off of Lithium after this discovery, because bradycardia is very dangerous and uncomfortable. (I should have gone to the E.R., but chose to avoid it; a risky choice.) My heart rate has been much more regular since then, and never ever lower than 70 bpm. This gives me much more "conscious" time.

Just this past week, I ran out of Topamax and wasn't able to get it refilled for 7 entire days. I had a migraine every one of those days. Sunday was the worst of it. I attended part of my brother's college graduation, but at high cost. For a few hours, I labeled my pain at a 15. I couldn't help but scream. On Monday, I was able to obtain two bottles of Topamax. But over the weekend, the answering service could do nothing for me. I just barely avoided the E.R., only because I hate it so much.

Onto the good news.......

I haven't used my wheelchair or my walker in 2011. I'm gradually driving more. I'm taking a Spanish class at community college, which I attend independently. I'm trying to fit in adequate exercise. Overall, my pain has decreased. I'm able to think clearly more of the time. I can sit up and walk around more of the time, with increased stamina.

I'm still fighting fatigue, but Provigil helps with this. I have good and bad POTS days, but there are more good ones, and the good ones are much better than they ever used to be. I usually have a bad POTS day when I've overdone it the day before. I never sleep through the night. Despite my one reliable nightly wakeup, I seem to require less sleep lately, often waking at 10 a.m. (about ten hours of sleep).

I am to the point that I believe I will look for part-time work in the fall. I still don't know how well I be six months from now or one year from now. But I fully expect to continue to improve. I am wondering what career path will be right for me. It's hard to make plans, when you don't know what to expect. But to even to be able to consider new plans is more than I ever expected. I am considering grad school. I'll be meeting with a career counselor at my alma mater in a couple weeks. It may be too soon, but I'm ready to get the ideas flowing!

Also, looking forward to my wedding in July. Plans are going well.

Thank you to everyone for your support!

Please keep my Chiari friends in your thoughts, as not everyone has reached a hopeful stage in their journey yet.

Pace yourself

2010-12-27T22:46:00.002-06:00

I had a chance to live a more normal life, so what did I do?
-I showered, instead of bathing.
-I baked cookies.
-I ran errands.
-I drove.
-I socialized.
-I lived a life.
-I made the most of it.

-I overdid it.

It's hard to pace yourself when your body suddenly isn't telling you to stop.

But now, I'm at the bottom again, and it's a long, slow climb back up. I hope to deal with it better next time I get out. I hope I've learned. I also hope it's a shorter climb and a longer peak next time.

It's so hard being back down at the bottom, unable to get off the couch. Barely able to walk. Trouble catching my breath. Pain, extreme fatigue, overwhelming feeling of sickness. Such a long way to fall, and so suddenly. How will I ever climb back out of this hole again?

Getting Carried Away...

2010-12-22T01:49:00.005-06:00

Things that are better, at least some days:
-waking up at 11:00, naturally, instead of noon
-able to wake up at 10:00 to an alarm one day, with no consequences, no nap
-able to take a shower instead of a bath
-able to shower more frequently
-able to have more active and upright time, on some days

The thing is, good days and bad days are a part of Chiari. They are a part of POTS. Good days are not unheard of. This doesn't necessarily mean anything. So I have to be careful not to get carried away, right? Keeping that in mind...here I go anyway:

I'm feeling like there are possibilities for my future. I'm considering looking into a new career. Am I crazy? I have no idea how plausible this is. I seriously would need to get SOOOO much better. I have no idea how much better it's possible for me to get, or if it will last. But maybe...

Resilience

Hope is a scary thing.
I wish I could stop myself.
Cause I can never handle the let downs
Not another let down
Not this time.
I've done everything.
I've given up everything.
I lost everything and rebuilt.
Then I did it again.
And again.
I've waited.
I've endured.
I continued waiting.
I've been shut down so many times.
I've gone through more pain than I ever imagined I could.
But I did not give up.
I kept going.
I came very close.
I hit rock bottom.
Then I hit even lower.
I waited and endured and withstood even more pain.
This time should be my time.
If there is any way for me, this is it.
There's nothing else to try.
This has to be it.
It must be.
I don't have much left in me.
I don't know how to give much more.
It hurts too much.
I just don't think I could give any more.
But that's never really true, is it?
As much as it hurts, I always find a way.
I persist.
I push forward with might.
I always find new ways to forge ahead.
So it's never really over.
There's no last chance.
As long as I can always find a way to keep trying.
------------------------------------------------------------------

Sorry for any typos and the unorganized nature. This is just stream-of-consciousness. I don't really know anything about poetry. Just wanted to get some thoughts out.

I dedicate this to all of my Chiari friends that also persist in a daily battle. It takes strength and resilience. I don't know how some of them do it with a smile on their faces! I don't necessarily stay positive. I just don't give up. And that will still get you from one day to the next, which is all you can do sometimes.

In reality, I know that many people have this surgery and get better. Other people have this surgery and don't get better. And others have this surgery, get better, and then get worse again. There's still not enough research. It's still so early. There's no explanation for the outcomes. The doctors are doing their very best to help us with limited information. And I am very grateful that there are doctors out there willing to try.

At the very least, I have recovered from the side effects of the surgery at this point (no more crazy autonomic instability--back to normal POTS). Surgical pain is mostly gone--still some sharp pains in my head at times. My skull has healed in a strange way--it's got a pretty large protrusion that is very sore and sensitive. My scalp still has very strange sensation (hypersensitive and partially numb at the same time). My hair is growing back at the usual rate.

But I also feel that the number of good days is increasing. It's nothing dramatic. If I'm improving, it's at a very slow rate. I would call it incremental improvements. But that's good enough for me. I wish I could see into the future to know how much better I will get and how long it will take. Then I could plan better. I could decide if it makes sense to choose a new career and go get the necessary training. Will I ever be able to work a real job? How many hours a day? Would I be healthy enough to work reliable hours? I'd like to work. I'd like to contribute financially to the family, and feel like I have a purpose.

I will save the more long term question of having a family for another day...

For now, I will just keep going. Living every day as best as I can. Trying to be upright and active without overdoing it. Keeping track of any improvements without expecting too much of myself and my body. Being grateful for wherever I'm at in my long recovery process, and trying to be patient to find out where it will end up. Trying not to get carried away and be accepting of wherever I may land.

Never giving up. I must remember that recovery is not a linear process. There are always steps forwards and backwards. After the busy holidays, I should expect some bad days to follow. But I must remember not to be discouraged by the bad days, even if they last for a few weeks. The good days will return, and ultimately, recovery will progress forward.

Of course, I must also keep in mind that we have no idea how far this recovery will progress (thus, not getting carried away).

Okay, I've said way too much, which tends to happen this late at night. I will probably not be waking at 11:00 tomorrow, as it is 3:00 here. My body is used to 11 hours. A bit less tonight I guess. Just got carried away again.

Finally, A Response.

2010-11-10T22:10:00.005-06:00

Xray of my new fusion

MRI showing the suspicious fluid which glows bright white

So, I finally heard from my doctor, and I did get some surprising news.

He looked at the scans. He said that the fluid in the picture is not spinal fluid! I don't have a leak. It is actually chemicals that were put in during the fusion. And my fusion looks exactly how it should look at this point in my recovery! So, everything is healing well. That part is a relief.

As far as my return of symptoms, my surgeon believes that they are being caused by my cardiovascular problems. He believes that I will need a pacemaker to stabilize my heart rate and blood pressure. He believes this would alleviate many of my symptoms.

I also talked to my local cardiologist today, since my 30 day event monitor is complete. He is referring me to an electrophysiologist. He also suggested that I may benefit from a new type of pacemaker.

Coincidentally, my POTS doctor, Dr. Grubb, installed the first in this new generation of pacemakers this past June. It is particularly advanced, and especially able to help stabilize both heart rate and blood pressure. [Correction: This is a false statement, which will be amended in the next blog entry.]

Here's a brief article describing this pacemaker: http://utnews.utoledo.edu/index.php/06_03_2010/patient-travels-from-dc-to-receive-new-generation-pacemaker-from-utmc-surgeon

My next step will be to contact a local electrophysiologist and to schedule a consultation with Dr. Grubb. I understand, of course, that there will be another waiting period.

Wow, I sure get to practice patience a lot.

In the meantime, I'm going to try to keep a good record of my heart rate and blood pressure. I find my current situation to be quite difficult to describe. Also, it's complicated by the multiple medications that are helping to raise my blood pressure, which stabilizes my heart rate, to some extent. It's hard to know how severe my situation would be without these medications.

That's all for now. It's a relief to know it's not a leak. But I guess the saga is never really over, is it? I don't want a pacemaker. I don't want heart surgery. But I would do pretty much anything to feel better at this point, obviously.

2010-10-25T13:05:00.002-05:00

Here's an email I sent to my surgeon that summarizes my post-operative struggles:

I had the MRI and xrays done this week that Dr. B requested based on my post-surgical cardiological problems. I wanted to make sure that Dr. B has a full picture of my post-op symptomatology to consider, along with the images, when they arrive.

My cranio-cervical fusion revision was August 19. My time in the hospital went smoothly. Immediately after surgery, we noticed a complete improvement in my pupillary reflexes as well as my swallowing. I flew home on August 27. I experienced dramatic symptom relief in the following days. I experienced relief of all of my POTS symptoms, my headache, and my fatigue. My balance was very steady, and my "brain fog" was gone. I woke up every day feeling energized and light. I built up my pace and endurance on daily walks very quickly.

September 7 was the first day I noticed a change. When I went from sitting to standing, my vision would black out. This caused me to check my blood pressure. It was 90/70, which was quite a bit lower than it had been when I was feeling good (around 120/80). I also felt a headache this day. In the following days, I had low blood pressure (as low as 80/40), vision blackouts, legs giving out, palpitations, nausea, chills, and fatigue return.

On October 5th, I went for a walk with my heart rate monitor on, and noticed my pulse get very low (HR=38). This happened several times in the following days, in conjunction with reduced consciousness. During a reduced consciousness time, which can last for several hours, or even much of the day, I experience limpness, lethargy, reduced awareness, reduced alertness; and trouble focusing, understanding, and producing words.

In addition, my dysphagia, which had gone away, has started to return, which choking on water and pills.

My pupils only work sometimes.

My baseline headache has gone and returned.

My fatigue and flu-like feeling have gone and returned.

My autonomic dysfunction has gone and returned, however, it used to be strictly POTS. Now, my blood pressure can be too high or too low; my heart rate can be too high or too low. So, my POTS doctor doesn't know how to treat me, and my cardiologist is considering a pacemaker, pending the results of my 30-day event monitor. I have marked heart rate lability and blood pressure lability. In addition, I experience shortness of breath, and my lips are often blue.

I expect this recovery to be a long one, so I don't mean to complain. But I did want to alert you to the seemingly complete elimination of my symptoms in my early recovery, followed by the return and even worsening of many symptoms right now. It's hard to understand and hard to explain what has happened.

I cannot remember anything happening to my head or neck that could have caused any damage.

I am continuing with my Forteo shots at this time, and to the touch, it feels that my skull has filled in with bone.

Thank you for considering my situation.

Bradycardia, Reduced Consciousness

2010-10-11T14:22:00.004-05:00

I know. I'm a delinquent blogger. It's just hard to know what to say when things are going wrong and not looking up. So, I'll simply report the facts.

I've been having a new symptom, and some more worsening symptoms. I've had some spells of prolonged bradycardia, as low as 38 beats per minute. I haven't lost consciousness. But I have had many different levels of reduced consciousness, and it feels like I'm never fully awake anymore. I am often in this state at least 6 hours a day: reduced awareness, reduced alertness, unblinking, limp, lethargic, difficult to rouse. I've reported this symptoms to my POTS specialist and my neurosurgeon. I'm now on a 30-day holter monitor to try to catch the bradycardia episodes (of course, the lowest I've recorded is 46, but we're getting closer). My heart rate is also hyperreactive to anything I do, but the monitor is really able to catch that. The most it can record is 2 minutes and 15 seconds a day. I would need more of a 24-hour holter for that type of problem.

The POTS specialist says that this is not related to POTS. Tachycardia is typical of POTS. Bradycardia is not. So this is a new and unexplained symptom.

My surgeon also ordered scans of my cervical spine that we're arranging to have set up. I guess he's worried about the bradycardia episodes being related to the fusion.

I also need to have my Lithium levels tested, although my dosage has been constant for a long time.

I still haven't actually had an appointment with a doctor about this new problem.

During the bradycardia episodes, and actually most of the time, my state of consciousness has been reduced. I've been really out of it. Staring. Limp. Weak. Tired. Short of breath. Not very reactive. Can't think much (Reduced mental status).

Besides all of this, essentially every symptom I've ever had has returned or gotten worse.

Sunday 9/26
Fatigue/Lethargy
Pain: 3, Achy
Walk: 30 minutes, up and around the hill

Monday 9/27
Fatigue/Lethargy
Aches/Pain after walk
Chills at night
Exercise: 27 minute walk, 20 minute bike

Tuesday 9/28
grocery trip: bad, caused a spell of reduced consciousness
Fatigue/Lethargy
Aches
Chills
Headache: brain, especially at the back--definitely Migraine
30 minute walk w/ ankle weights

Wednesday 9/29
Fatigue/Lethargy
No appetite
31 minute walk, 25 minute bike

Thursday 9/30
Fatigue
Achy
No appetite
Migraine
Exercise video: 40 minutes, Heart rate: 170

Friday 10/1
saw a movie in the theater
GI distress

Saturday 10/2
Collapsed when climbing stairs
Unable to move all day: restricted to bed

Sunday 10/3
bike 15 minutes

Monday 10/4
bike 20 minutes

Tuesday 10/5
Migraine
Nausea
Fatigue
Muscle aches
Bruised feeling all over
Joint pain
Reduced appetite
22 minute walk

Reduced consciousness spell: weak, tired, out of it, short of breath, palpitations
120/76-HR 48
126/81-HR 47
134/88-HR 44
134/83-HR 44
138/90-HR 39
136/89-HR 42
134/81-HR 42
Lasted at least 3 hours

Wednesday 10/6
Recumbent cross trainer-10 minutes
Reduced consciousness spell
135/92-HR 39
145/94-HR 40

Thursday 10/7
after breakfast, resting
feeling "normal"
102/67-HR 81
102/64-HR 88

Later, out of breath just talking
108/72-HR 53

Friday 10/8
Begin 30-day Event Monitor

Rest after exercise: HR 52

Saturday 10/9
Decreased consciousness most of the day, HR low was 46

Sunday 10/10
Decreased awareness, decreased alertness, weak, tired, HR low was 48

Monday 10/11
Zoning out all day. Low so far around 52. Will probably get lower later, as it usually does. It seems to trade off between low blood pressure and low pulse, so I'm always symptomatic, but always just barely conscious.

It seems like I should see a doctor, but the cardiologist just gave me this monitor. Don't know who I should see.

Some bad days, Days in Bed, A Plateau

2010-09-26T13:50:00.002-05:00

Friday 9/17
Switch to hypomanic
Awoke with heart pounding
Sharp pains in right rod
Nausea
Chills
Spell: decreased conscioussness

Saturday 9/18
Nausea
Hot/Cold
Body aches
Sensitivity
Fatigue
Heart pounding
Lethargy
Chest pains
Couldn't get out of bed all day
Walk: 10 minutes- excruciating

Sunday 9/19
Fatigue early
Better energy
Less pain
Reduced consciousness spell
Heart feeling better

Monday 9/20
Lethargy improved
Heart improved
Reduced consciousness spell
Low blood pressure all day
Nausea
Abdominal pain
Chills
Low pain level: 2-3
Bike: 20 minutes HR 120's

Tuesday 9/21
Flu-like symptoms
Hot/cold
Lethargy
Nausea
No appetite
Migraine kept me up at night
Exercise video: 20 minutes- HR 120's

Wednesday 9/22
No appetite
Flu-like symptoms
Walk: 20 minutes

Thursday 9/23
Nausea
Hot/cold
Headache: Top of head/forehead

Friday 9/24
Migraine woke me from sleep
Flu-like symptoms
Walk: 34 minutes- HR 130's/140's

Saturday 9/25
Sick
Pain
Headache
Back left upper skull pain/throbbing- 8
Bed day
Walk: 5 minutes- excruciating

Four Weeks Post-op

2010-09-14T16:24:00.009-05:00

My recovery is progressing and improving, but not in a linear fashion, of course. Recovery is always "2 steps forward, 1 step back." It's very frustrating, but overall, I can tell that even with multiple setbacks and plenty of symptomatic days, I'm still getting better overall.

Friday 9/10
Pupils functioning fully.
POTS symptoms much better.
Exercise tolerance improved.
Intractable headache
Walk: 2 laps
Walk: 2 blocks

Saturday 9/11
Walk around golf course: 23 minutes
Active day
Headache

Sunday 9/12
Walk: 2 blocks
Active all day
Headache
Pain, neck and head: 6

Monday 9/13
No activity all day
Bike: 8 minutes
Headache
Lethargy
Nausea

Tuesday 9/14
Headache
Walk: 18 minutes
Headache worsened: 7
Bike: 6 minutes

Wednesday 9/15
Physical therapy
Bike: 12 minutes

Thursday 9/16
Grocery trip
Heart palpitations/pounding
Nausea
Walk: 30 minutes

Three Weeks Post-Op

2010-09-09T20:52:00.003-05:00

For some reason, by the time we got home from the hospital, it looked like my recovery was going to be one of those rare quick and dramatic recoveries; a sharp contrast to the long and drawn out recoveries that I'm used to.

For ten days, my body surprised me. Besides the surgical pain and the post-surgical "quirks," I was symptom-free. Really. No headache (again, besides localized surgical pain). No nausea, no facial pain, no POTS symptoms. I was amazed.

I certainly wasn't expecting this when it happened. It seemed too good to be true. I tried not to get my hopes up. But it really seemed the the surgery had very quickly solved all of my major complaints.

Then, Tuesday happened. Blackouts, collapsing, low blood pressure, pupils not working, tachycardia, palpitations, arrhythmias, the old headache, body aches. They all came back, and have persisted.

Now, I never expected to be symptom free, at three weeks out, or ever, really. But for ten days, I was. It's hard not to wonder why I was doing so well.

Especially something like pupillary constriction. That was immediate after surgery. It was perfect. And now, it's sluggish and incomplete. What changed to make this happen, along with other symptoms coming back so abruptly? My blood pressure hadn't been noticeably low since surgery. Now, it seems to be constantly low.

However, I know to expect a long recovery. I expected not to see symptom relief for a long time. Twelve months is a good guess for recovery time from symptoms for this operation. POTS recovery can take longer than that, and it is not expected to be complete.

But for those ten days...I had such a feeling of well-being. It felt like I had a new body. I just wonder what that was about...

------------------------------------------------------------------------------------

I had my first physical therapy appointment on Wednesday.
The script from Dr. B only had one instruction: "Optimize neck ROM" (range of motion).
So, when I went to my appointment, that's what we started doing. Stretching. I left with quite a headache. I wrote to my nurse, to find out if this was okay. She said that no stretching is to be done. Just massage, warm compress, and later, ultrasound, and TENS. Quite a contradiction to the script. Hopefully, we'll get this straightened out.
I also hope I can find a physical therapist that can give a good massage. He seemed to be scared to touch me. I need to get to a massage therapist, and fast!

------------------------------------------------------------------------------------

I really wish those ten days hadn't happened. They got my hopes up and left me disappointed with my current condition. I'm actually having a good recovery. I'm still in pain (between 4 and 7). I have a lot of trouble finding a way to rest my head on a pillow at night. But I'm getting out for daily walks. I'm able to get in and out of bed easily. I still have a lot of fluid. Once that is absorbed, the pain should decrease. It's still much better than I expected. I know it's good. I'm working on readjusting my expectations. I knew it was too good to be true. That's why I didn't want to admit just how well I was doing. I didn't want to admit that my headache was gone. But I was really ecstatic. Now, I've actually been crying for three days straight. It doesn't make sense, because I know how lucky I am, still. I guess I just really got my hopes up that things were really going to stay so drastically better so early on. I wish I could stop crying. I know it's ridiculous. Oh well, I will adjust my expectations again, in time. I've done that enough times in my life; I'm sure I can do it again.

19 days after surgery

2010-09-07T19:31:00.006-05:00

Day 14
Day 19

I've had some strange "side effects" from surgery that I expect to wear off after some time. Some have already begun to wear off.

First, my voice was very weak, strained, and hoarse after surgery. Luckily, this one has already improved dramatically.

Also, my jaw cracks on the right side when on open it. This is also happening less.

My right ear has been behaving very strangely. At first, it would vibrate and buzz at certain sounds. Now, it just echoes my voice and amplifies sound sometimes. Getting better.

There is a strange clicking sound and feeling in the back left side of my head with the impact of walking. I wasn't sure if this would improve, but during today's walk, for the first time, the clicking decreased in frequency.

The back of my head is still full of fluid. It is one large pocket of fluid. I have trouble resting my head at all, because of the pressure of the fluid. Still waiting for the fluid to be absorbed and the pressure to go down.

In the time from pre-surgical testing to the time I got my stitches removed, I lost 10 pounds. That wouldn't be hard to gain back, but I'm hoping to gain back more muscle than fat, so it could take some time.

On Saturday, September 4th, I took my first shower. Washing my hair was near impossible. My head and scalp are so sensitive to the touch. Just moving my hair around is painful at this point. Unfortunately, as I did wash my hair, I noticed it falling out in chunks. It was distressing. Luckily, I wasn't left with nothing. This was apparently an effect of the eight hours of anesthesia. Hopefully, I won't lose the rest of it! It seems to come out at the lightest touch.

Sunday and Monday, I began having new, sudden, sharp pains in my head. I'm sure that's just part of the healing process.

Today, I had a bit of a rough time. My vision has blacked out three times. I collapsed once. My blood pressure has been low today, around 90/70. I've also had palpitations, tachycardia, and the feeling of a very reactive heart.

I'm also feeling very achy today and my joints hurt more than they have been.

I've been feeling emotional today, too. Knowing that the POTS recovery will take a long time, and certainly wasn't "cured" by the surgery. Everything in its own time.

Recovering

2010-08-31T17:25:00.005-05:00

Well, it's been 13 days since surgery. I would say that this fusion surgery didn't hit me nearly as bad as the first fusion did. I prepared for the worst, again, and this isn't it. That being said, I'm still in quite a lot of pain. The new rods feel terrible, but that's normal. It's quite a weird feeling, having my old rods gone.

There's still a fair amount of swelling. I actually have what feels like pockets of fluid on either side of the back of my head. It's very strange to touch. So, I'm still icing that area a few times a day to try to reduce the swelling.

Also, my muscles are in extreme spasm. The muscle relaxants are helpful, but some intensive massage therapy will be necessary for these muscles to really let go. I use a heating pad on my neck and shoulders to help relax the muscles. Massage therapy and physical therapy will start next week.

Tomorrow, I get my stitches removed. Yay! On Saturday, I'm allowed to wash my hair! That's 16 days after surgery that I will finally be allowed to wash what's left of my hair. Luckily, my incision is closing up really well.

While on my current pain regimen, my base level of pain is about a 6. It's tolerable. Nothing like I expected based on my first fusion.

The improvements I've seen so far:
*Swallowing
*Pupil constriction
*I can now stand with my eyes closed without falling over!

The single most important factor that has gotten me this far in my recovery is the amount of support from family and friends during this challenging time. I have never felt alone. I know how lucky I am to have so many supporters to help me through.

Making the Switch

2010-08-15T01:10:00.002-05:00

I'll be switching over to Caring Bridge for the coming weeks, for post-surgical updates.

http://www.caringbridge.org/visit/carolynrichardson

How are you feeling about your upcoming surgery?

2010-08-12T18:05:00.003-05:00

Am I nervous? No. Not really. I know what to expect. The only thing to be nervous about is that my bones fuse the way they are supposed to this time. What I feel is much more a feeling of dread. I dread waking up after surgery. I dread the time in the hospital and all that comes with a major operation. Mostly, I dread month after month, alone in my bed, wondering if I'm going to feel that horrible forever...seeing no signs of improvement, wondering if I'll ever get any better. I dread that darkness returning, that hopelessness that accompanies unending pain and sickness, when things are supposed to be finally turning around. I dread the dark days.

"At least you know, one week from now, it will all be over with." No. One week from now, it begins again.

The end of life as we know it

2010-08-11T13:32:00.004-05:00

So, in four days, we're leaving for our trip. If all goes according to plan (meaning, I get approved for surgery based on my traction results), I will go through some very traumatic times in New York. I am really worried about pain management after surgery. In the past, this has been a major concern, because of my allergic reactions to everything. I'm also worried about the usual issue of getting my GI system working again after surgery. It's an even bigger concern this time, because this will be the longest surgery I've ever had (8+ hours).

And, being a female, with very thin hair, I will be sad to lose most of it for this operation. My hair grows thickest in the area where it will be shaved. The area that I keep is actually quite sparse, so I really won't be left with much. I wouldn't care too much, except that I am planning a wedding, and I know it won't really have time to grow back.

Anyway, back to things that actually matter. On Tuesday, I will have Invasive Cervical Traction. This is a surgical procedure. I will be awakened in the operating room, so I can be involved in providing subjective information about the feel of the positioning of the skull. They will also be taking images in the operating room, so they can take measurements to determine precisely the right location for my skull.

If all goes well, I will be approved for a revision of my cranio-cervical fusion. I will be having my existing hardware removed. The bolts will have to be removed from my skull, and the screws will have to be removed from my spine. The titanium rods will come out. Next, they will begin to reconstruct a new fusion, in a new location, with all new materials. The titanium is a stronger alloy than was previously used. They will also include cadaver fibula bone and bone powder. This will encourage my bones to grow around the new materials to form a bony fusion. They will hold it all together with a wire. The goal is for a more solid fusion to be formed and for that fusion to be in an optimal location.

I am mostly just dreading recovery at this point. It's all I can really think about. With POTS (dysautonomia), anesthesia is a trigger that can worsen your symptoms, and it's really hard to come back from. So even though they say there is an expected 2 month recovery, that's not really how it works with POTS. I would say that I still haven't recovered from my tethered cord surgery 2 years ago, in terms of my autonomic dysfunction.

Hopefully, this surgery will be able to alleviate some autonomic symptoms, making the POTS recovery easier than it has been in the past. Even so, it wouldn't be strange to spend the next year of my life working on regaining my ability to be upright.

The thing is, I'm not starting out at the top. I'm actually quite ill going in to surgery, so the time of rebuilding may be very long. One to two years would not be unusual for my autonomic system to require to adjust and allow me to recover fully.

With POTS recovery, you have to walk a very fine line of working enough to start rebuilding your cardiovascular system and working too much. Even working a few minutes too many may cause you to spend days unable to get out of bed. I know because I've already been through all of this before. I've already gotten myself from a mostly bedridden state, similar to this, to a place where I could work out at a gym and drive and work part time. The problem was, I was still restricted to bed much of the time. That's because I was limited by my sub-optimal fusion in my head. This time, there should be no restrictions on my recovery.

However, I still no better than to set really high expectations for myself. I don't expect to live pain free. But I hope that my head pain level is significantly decreased. I don't know if my fatigue will improve. If it doesn't, I can always try treating it again with Narcolepsy meds. I really hope my POTS will be improved, with a combination of surgery, physical therapy, and hard work. I believe my nausea and swallowing problems are the most likely to go away. I don't know if my overall body aches could be improved. I assume joint pain is related to EDS and won't be improved. So, I'm not sure what these means for quality of life. I there was a quality of life scale at TCI. Right now I'm around 45/100. They say you can hope to move up by 20-30 after surgery. My quality of life could improve to 75/100. Is that too much to hope for? I'm so scared to be let down again by another surgery. I'm even more scared to give up another year of my life just to be let down again by another failed surgery. But I must believe that there's a chance, or I wouldn't be doing this. I'd have to be crazy to try this again if I didn't believe that it was going to help me.

But it will all start at the bottom. First the pain. Pure survival. Next, the physical therapy and massage therapy to begin moving again. Then, all the will-power I've got to fight the exhaustion and get my body working again.

These really are my last few days of the status quo. Everything will change.

I will be fighting every day to endure the immense pain caused by the surgery. No one can say how severe it will be, how hard it will be to manage, or how long it will last.

I'm as ready as I'll ever be.

Surgical updates will be posted at my caring bridge page, not here on my blog. That will allow family and friends to receive e-mail updates to keep informed.

http://www.caringbridge.org/visit/carolynrichardson

Big News

2010-07-08T22:20:00.003-05:00

I have a surgery date. I am booked for Invasive Cervical Traction 8/17/10. If the traction test is positive for instability, they will go through with Cranio-Cervical Fusion Revision on 8/19/10.
My pre-surgical testing is 8/16/10. So, we will be flying into New York on 8/15/10. It is uncertain how long I will stay in New York after surgery at this time.

That's the schedule.

There's a lot of pre-operative planning to be done. All of the paperwork is done online now, so I can gradually chip away at that over time. I need to get a pre-operative physical with my general practitioner and I need cardiac approval with an echo-cardiogram with my cardiologist. I'm also supposed to have at least a few weeks of physical therapy. We'll see how much there is time for.

Plenty of preparation.

In terms of my feelings, I completely understand that this is good news. The best news I've gotten in a long, long, long, long time. My stagnant life will finally move forward. Something will change. In the big picture, this is a good thing, because my quality of life is likely to improve...eventually. When I first got the news, I was ecstatic. However, my enthusiasm was short-lived.

Please don't accuse me of being pessimistic here, because we all know that would be the worst thing in the world. I'm just human. I'm facing the scariest surgery I've had so far. I've been through a similar operation in the past, so I know approximately what to expect...torture, a living nightmare. Remember, I'm allergic to all of those wonderful pain meds that ease the process for most. And the allergy meds don't seem to help me. So I feel the full reality of what they do to my head unarmed. It's a battle.

So I know I should be the happiest girl in the world right now. But I'm scared and anxious. It's making me irritable about the whole thing. I don't like to think about surgery or hear about it or talk about it or plan for it. But I understand that I have no choice. I just don't want to be happy about it right now. I'm so glad that the time has come to get it over with. But am I really happy about giving up my mobility for the next year of my life? About facing pain levels above an 8 possibly for months on end, like last time? About being restricted to bed for several months, maybe more? After the fusion surgery, the never-ending pain pushed me to suicidality after 5 months. It's hard to be happy when facing this again.

Now, my life has been so restricted for so long. With many symptoms and much pain. And I'm about to tell the doctors to go ahead and make it worse?

By the way, if a patient does not have POTS, the recovery from this surgery is expected to be about 2 months. Unfortunately, I have POTS, which is a chronic illness. It may become "easier to manage" with this surgery, but no one expects it to go away. Any surgery causes a POTS relapse: the more invasive the surgery, the worse the relapse. This is an 8+ hour reconstructive cranio-spinal surgery. They will remove my current hardware and replace it with all new hardware and bone, completely rebuilding my cranio-cervical junction. This essentially "resets" the nervous system. That's as invasive as it gets for POTS.

No risk, no reward. Right?

So, I completely understand that this is wonderful news. I just don't feel it. The long-awaited surgery has just become very real.

Trip to New York June 18

2010-06-25T12:09:00.002-05:00

June 18 was the big day. It was officially 2 years after my tethered cord surgery and my previous invasive cervical traction test.

Gustavo and I went on this trip. We stayed at the Variety House.

In the early morning of the 18th, I went to get new imaging done. 2 MRIs, 2 CTs, and several x-rays. Of note, we did a flexion and extension x-ray. I was surprised to find that during the extension x-ray, I was able to extend my head back all the way until it was touching my upper back, despite having an intact skull to C-4 cranio-cervical fusion in place. That's EDS for you. Super flexible!

Anyway, my appointment at The Chiari Institute was scheduled for 3:00, so of course, we went in at 6:00. We talked with Andrea, a very nice nurse practitioner for quite a while. We discussed all of my symptoms and my current condition. After that, we waited a bit more. Then, we finally went in to see Dr. B. It was his last appointment on a Friday evening, so he was very relaxed and making jokes. We got to meet with him in his main office instead of an exam room. During the visit, I did not feel pressure to prove myself to him at any time. Instead, he just explained what the next steps would be for me, in detail.

1. Need a good DEXA scan. Good means borderline normal, or a T-score at or above -1.0.

2. Need to continue treatment for bones now and after surgery.

3. Invasive Cervical Traction needs to test positive for instability.

4. Cranio-Cervical Fusion Revision will be performed.

Dr. B said that no bony fusion formed between the skull and C2, so the area has collapsed, due to osteopenia. That is the reason why the symptoms never got better, and have even gotten worse.

This is why it is so important to have strong, growing bones at the time of fusion.

Instead of one solid mass of bone and titanium, I have just naked rod connected to my bones. The area never filled in with bone.

The newest revision technique has been very successful at preventing this problem. It involves replacing 90% of my hardware. It also involves using a cadaver fibula in the shape of a V along with ground up cadaver bone, all tied together with titanium wire. This all encourages a bony fusion to form.

This surgery has been successful for many people and formed solid fusions for them.

It felt like a very successful visit, and I feel like there are not many roadblocks left on my way to surgery.

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Next, I went to my general practitioner this week. He listened to the update, and he immediately prescribed Forteo for me. Awesome. I started it on Monday! So I'm back in the bone-building game.

He also ordered a new DEXA scan to be done, which I had done yesterday. We got the results today. Unfortunately, the results were not what we were hoping for (-1.5 and -1.7). I believe very strongly that the difference has to do with slight differences between machines. My former endocrinologist told me that every single DEXA machine reads differently from every other machine, so you should try to stick with the same machine. Also, on the DEXA results, it says that changes of <5% are not statistically significant on repeat scans.

So, my current plan is to continue DEXA treatment for 2-3 more months, and then have a repeat DEXA on one of the other two machines that I have been using for treatment, whichever one I can get in to use. I truly hope that that makes the difference in the results that I need. When we're looking for such small changes, it seems like anything could make the difference. Hopefully, it really is just the change in machines.

The hard part will be convincing insurance to cover another DEXA this year. But it's so important, I would do whatever it takes to get another one this year. I can't put surgery off forever, not when I know how good it could be. This is just one more frustrating delay.

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My roommate at the Variety House was recovering from this exact surgery. And she was doing remarkably well. She felt so much better. She looked so good. She sat up by choice. Her post-surgical pain seemed minimal. She went into the city to see a show! Her success is my new inspiration to keep going until I have this surgery.

30 Things About My Invisible Illness You May Not Know

2010-05-15T22:41:00.002-05:00

1. The illness I live with is: Chiari I Malformation, Postural Orthostatic Tachycardia Syndrome (Autonomic Dysfunction), Ehlers Danlos Syndrome, Cranio-cervical instability/Functional cranial settling

2. I was diagnosed with it in the year: Chiari in 2002, the rest in 2003

3. But I had symptoms since: 1998-ish

4. The biggest adjustment I’ve had to make is: giving up on the idea of having a career; getting used to a drastic reduction in ALL activity

5. Most people assume: that if I look like everyone else, I must feel like everyone else. That if I put a smile on my face, then I’m doing just fine.

6. The hardest part about mornings is: Facing another day. Knowing it will feel like 10,000 days. Feeling like I weigh 10,000 pounds. Feeling worse than I did the night before. Feeling more tired than I did the night before. Feeling like my life currently holds such little meaning—why should I force my body out of bed just to watch TV all day?

7. My favorite medical TV show is: House

8. A gadget I couldn’t live without is: My laptop…connection to civilization

9. The hardest part about nights are: Being all alone with my pain and my thoughts, and unable to sleep, over and over and over again.

10. Each day I take __34__ pills & vitamins (Plus fish oil and any as needed meds, like pain meds). (No comments, please)

11. Regarding alternative treatments I: generally stick to what a doctor recommends, with a couple recent exceptions. I believe in empirically-supported medicine. I love massage therapy!!!

12. If I had to choose between an invisible illness or visible I would choose: I think I’d prefer a visible illness, because then people would understand immediately that my smile does not come easily. It is a gift for them.

13. Regarding working and career: When I thought I was getting better, around 2007, I had a piano studio of 24 students. But it was really a ruse. I was living off of stimulants and narcotics. I couldn’t keep it up. My body started protesting with narcoleptic episodes. As soon as I slowed down on the meds, my ability to work slowed down, too. I’m now down to about 3 half-hour lessons/week. And even that is a challenge. I don’t even know if this is the career I want to stay with. It’s just the only career I thought I could pull off as I was going through college. I have no idea if or when I’ll be able to work more. And I have no idea what job I would have chosen without limitations, or if I'll ever find a different one.

14. People would be surprised to know: that I never have a moment that is symptom free or pain free. I cannot remember what it feels like to live without pain. Also, the severity of everyday fatigue.

15. The hardest thing to accept about my new reality has been: My lack of independence and loss of social life. They do go together.

16. Something I never thought I could do with my illness that I did was: Graduate from a 4-year college. At one point, I also got into really good physical shape. I hope to do that again when my body allows.

17. The commercials about my illness: n/a

18. Something I really miss doing since I was diagnosed is: Gymnastics, Exercising, Singing, Performing on stage in some way.

19. It was really hard to have to give up: Driving…I need to drive again for my sanity. I feel so confined.

20. A new hobby I have taken up since my diagnosis is: I started a support group, but then became physically incapable of running it. I read a lot of books, but that’s not new. I watch endless hours of tv, but I’m not proud of it. I guess I’ve learned to relax. I always used to be so busy and stressed in high school and younger.

21. If I could have one day of feeling normal again I would: spend it doing all of the things my fiancé has always wanted to do with his partner, but can’t with me. He deserves that day of normalcy even more than I do. So it would be his choice. I think it would involve skipping and jumping and running (for me).

22. My illness has taught me: how to relax. How to listen to my body. How to revise my expectations for myself in life. How to accept myself as I am now.

23. Want to know a secret? One thing people say that gets under my skin is: if you just stay positive 24/7, you’ll be better off. That’s not necessarily true! Okay? We need to live in the real world, and we need to accept our circumstances as they are in order to cope with them. Not just pretend that everything is fine and put on a happy face. No one should have to pretend to be optimistic 100% of the time!

24. But I love it when people: Care, Listen, Learn about my messed up brain. Especially doctors.

25. My favorite motto, scripture, quote that gets me through tough times is: “A journey of a thousand miles begins with a single step.” –Lao Tzu. Because what can we really do, other than persevere.

26. When someone is diagnosed I’d like to tell them: Allow yourself to grieve, time and time again, over everything you lose to this illness. It’s the only way you will learn to accept your new self.

27. Something that has surprised me about living with an illness is: How ignorant the entire medical community is about all of these illnesses. The number of times and ways they have let me down.

28. The nicest thing someone did for me when I wasn’t feeling well was: Lay next to me, without touching me, when my skin was too sensitive to be touched. Just because my skin hurts, doesn’t mean I don’t want to be held, just that I can’t be.

29. I’m involved with Invisible Illness Week because: n/a

30. The fact that you read this list makes me feel: Heard.

A Day in the Life (with Chiari/POTS/EDS/Cranial Settling/Cranio-Cervical Instability)

2010-05-14T23:08:00.003-05:00

I wake up around noon (please don’t express jealously--that's just mean). I immediately feel the aches and pains of the morning and the headache of the day, but it’s usually too soon to know how bad the day will be. At this point, I can only tell if it will be an easy or a difficult wake-up. Some mornings, I wake up feeling like I’ve actually slept. Most mornings, though, I wake up feeling the peak of lethargy. Either way, I drag my heavy body out of bed and head downstairs.

First, I eat a bowl of Grape Nuts on the couch. This is followed by my morning pills. I may or may not have a dramatic and prolonged choking spell on a pill, or even my water, for that matter. At this point, I am unable to move until lunch time. I force myself to eat lunch exactly two hours after I finish eating breakfast. The routine is helpful in remembering to eat, because hunger often never comes. Quite often, before lunch comes, I have a spell of reduced consciousness. My body goes limp, and I am unable to respond to much. If anyone is around and realizes that I am not just asleep, they may come and force a salt pill and water in my mouth. That will resolve the spell within ten minutes, most days. If no one is around or notices, I may remain semi-conscious for an extended period of time, unable to do anything. For lunch, my mom or my fiancé helps me to get apple slices and Triscuits. Then, I take my lunch pills. At this point, I am still unable to get off the couch for several more hours.

On a good day, around 5:00, I will be able to go get washed up and dressed. I don't have the energy for a shower every day, or to even get dressed every day. But most days, I do get to eat dinner and take more pills. Since I can’t drive, there is nothing to do after dinner. I don’t have the energy anyway. I’d like to go for a walk around the block for my Physical Therapy, but currently, I’m not allowed to, because I’m so likely to have a drop attack. (I would enjoy getting out of the house, but even a walk around the block causes a considerable increase in pain, even on a good day. But this isn't allowed right now.) So, I generally just sit around and watch t.v. until 9:00, because that’s the only time of day that I may have company. At 9:00, I may try a few exercises (planks, toe raises, etc.). I’m always too tired, but I’ll force myself somedays. It is pure torture to exercise. If you see me exercise, you'll wonder what is wrong with me. But that's just the look of pain on my face from the exercise.

Miraculously, the best time of day for me, and many POTS patients, is late at night, which is why I’m typing this at 2:00 a.m. Unfortunately, this is another lonely time of night. Lately, the loneliness leads to a lot of tears, but that's not too unusual. It also ends up being a very unproductive time. But it’s hard to go to sleep too early, when it’s the best you’ve felt all day.

I've skipped over exciting things, like drop attacks, where I collapse straight to the floor in the middle of whatever I'm doing, or days of horrifying pain that prevent me from leaving my darkened bedroom. We'll skip any emotional peaks or particularly strange symptoms. We'll stick with average days.

So, that’s life. And I think it explains why I find it essentially impossible to work, nearly impossible to work out, and why I haven’t been able to maintain any friendships in years. How do you maintain a social life with a life like this (other than a virtual one)? Virtual friends are great, but they may or may not be counter-productive to real life relations, or so I’ve heard.
And so, I keep on living. Thank goodness for compassionate caretakers and little canine companions.

Disclaimer: I don't mean to say that this is what a day in the life of anyone with these conditions is like...just that this is what an average day at this point in my journey is like.

Nothing New

2010-04-30T23:35:00.002-05:00

No new developments in my medical life.

This Monday, May 3, the Fox show, "House," will be focusing on diagnosing a patient with Chiari Malformation. The episode is called "The Choice." Based on the promo, we know three things so far: 1. The patient is male. 2. He is accused of faking his symptoms. 3. He begins lactating. The second fact is very common of Chiari patients. And the lactating seems to be a sign of hormonal imbalance, probably caused by flattening of the pituitary gland, which is also common in Chiari patients. They just had to find a shocking presentation of the disease for the show. (At least this is my analysis.)

I'm pretty excited to see it. I hope a lot of people see it. We'll see if it can bring some awareness to the condition.

I found a compilation of symptoms of autonomic dysfunction that is really comprehensive. I'm including the link here. I'm also going to include a few that I found to be particularly specific to me that I hadn't seen before.

http://heavenleigh412-ivil.tripod.com/id23.html

-"Irritability due to decreased blood flow to the brain"

-"Inability to tolerate changes in temperature"

-"Abnormal deep tendon reflexes"

-"Intelligence is normal when receiving adequate cerebral perfusion."

-"Disruption of sleep/wake cycle usually consisting of increased energy late in the evening and lowest energy level in the morning regardless of amount or quality of sleep."

-"Need to sleep 12-14 hours in order to complete simple activities of daily living."

-"Distorted depth perception resulting in a feeling of unsteadiness. Often appears to be "clumsy" or excessively cautious when climbing stairs or reaching for an object."

-"Decreased awareness of what is in the peripheral visual field. This often causes the patient to startle because he/she did not perceive anyone or anything next to them.

-"Frequent "bumping into things." Attributed to a combination of visual and depth perception deficits."

-"Noise sensitivity. Loud or beating sounds can cause pre-syncopal episodes. Difficulty filtering out sounds. Easily distracted by sounds."

-"Sensitivity to odors. May cause extreme nausea, retching, vomiting, dizziness, and headache."

-"Sensitivity to touch. Mild pat on the arm or squeeze of the hand can cause excruciating pain, especially right after an episode or if the patient has not had enough sleep."

-"Taste and appetite changes. Fruits and other acidic foods may taste extremely acidic. Foods may taste differently if patient is tired, stressed, or post-syncopal episode."

-"Speech disturbances: Inability to finish a thought, loss of train of thought, especially if up and about for 2 hours or more without lying flat and resting."

-"Comprehension difficulties. Inability to follow a conversation. May hear words but be unable to understand their context in a sentence. Cannot focus on more than one activity at a time. May not realize they are being addressed. Looses conversation focus when topic is changed. Is easily distracted from the conversation focus by any environmental stimuli.

-"Memory recall deficits in long and short term memory. Improves with lying down and resting."

-"Abdominal migraines. Severe stomach pain triggered by large meal or sitting or standing too long. Usually resolves if patient lies quietly."

-"Drifting to the right or left when walking."

I just found these to be interesting, as they are all quite specific symptoms that may not have been noted elsewhere. I found them all at the website listed above.

More Doctors

2010-04-19T22:47:00.002-05:00

Appointment with Endocrinologist 4/5/2010

I don't quite know how to explain what happened at this appointment. It didn't go well. It felt like an interrogation, actually. First, she asked if my surgeons were satisfied with my recent DEXA scans. I told her that I wasn't sure, but I thought that they wanted me to continue on the medication, since I am still in the range of Osteopenia. She said that this was ridiculous, and with any other patient, we would be celebrating right now. She said that she would not renew my prescription for Forteo any longer, because my bones are strong enough as they are, and no reputable surgeon would require them to be any denser. The interrogation began. She started asking me questions about the reasoning for each of my surgeries and what improvements I saw with each one. Then she questioned why I was having my current surgery. She told me she thought I should consider if I really needed the surgery, and getting a different surgeon. At the very least, she thought I should see a neurosurgeon that she recommends for a second opinion...a neurosurgeon that doesn't even specialize in Chiari, much less cranio-cervical instability and fusions. Before I could even ask her the rest of my questions, she walked out of the appointment, not to return. It was completely condescending and rude. I felt like I'd been slapped across the face. At the same time, I wasn't surprised. I've been through all of this before. It's just been a while.

I did learn one positive thing at this appointment: I asked her if my salt intake was affecting my bones, considering I take salt tablets regularly, so I get more than the average recommended amount. She said that all of my tests showed normal amounts of sodium in 24-hour urine samples, and I don't need to worry about my salt intake at all. YAY!!! My body simply metabolizes much more salt in a day than a normal person, so the salt doesn't leach calcium from my bones. So, as long as I don't take more than I feel I need, I'm fine. I know I was taking 5-6 tablets a day when I had those tests done. So that should be a safe amount for me.

I am not certain if I will return to this endocrinologist. I may need to find a new one to prescribe Forteo, which I know won't be easy, from experience. But I will wait for now.

Appointment with Dr. Grubb 4/14/2010

My appointment with Dr. Grubb was excellent.

He is unhappy for me that I need another surgery, but he is in support of the surgery, and expects that I will see some improvement in POTS symptoms from the surgery. It seems we have come to the understanding that my POTS symptoms will never be cured. But he says that if the surgery helps, then the symptoms will be easier to manage with medications.

Surgery recovery is expected to cause another large setback in POTS symptoms. There is simply nothing to do about that. But if I am struggling a lot, I should consider contacting him. Perhaps to consider IV fluids to get me through the rough time.

Exercise is still Dr. Grubb's number one concern. He suggested a recumbent bike for ongoing cardio exercise. We are looking into buying one used. They are quite expensive. But if we can find a good deal, it may be a possibility. I'm also supposed to walk around the house wearing ankle weights to build up leg strength. We'll see how that goes.

He's changing my dose of Midodrine a bit. We're going to try for higher doses with breakfast and lunch to see if I can be active earlier in the day. Usually, I cannot walk around until after dinner pills.

Finally, he wants me to add a couple of supplements. He wants me to be taking D3 in liquid form instead of solid form. Also, I'm supposed to look for a flavored liquid fish oil supplement. That sounds absolutely disgusting to me, but I guess I could try it. (I'm particularly picky, so I'm really not sure if it will work out...)

Upcoming Appointment with Dr. Bolognese 6/18/2010

I have an appointment set to see Dr. Bolognese in New York. Here is what I know about it:

The surgical committee did NOT deny my ICT (Invasive Cervical Traction) because of bone density. They did not believe there was enough evidence showing I was a good candidate for ICT.

So, protocol states that to qualify for ICT, I need to have a consultation at TCI (The Chiari Institute) with new imaging and new testing showing that I have symptoms of cranio-cervical instability/cranial settling.

After the appointment, the surgical committee will vote on ICT again.

If they determine me to be a candidate, I will have ICT.

If the ICT is positive, the surgical committee will vote on surgery.

If they determine me to be a candidate, I will have the cranio-cervical fusion revision.

It's a TON of red tape, but it's the only way that I will have this surgery. And it's the only way that they can be absolutely certain that I need this surgery, and that this surgery will help me.

So, this is the way it needs to be done.

It's a long, long, long road since that first surgery in 2002.

This time, I'm hoping to recover from surgery in time to have my wedding in the summer of 2011, whether or not I have hair!

Exercise and Doctor's visit

2010-04-12T22:26:00.004-05:00

First, I have been exercising regularly (but not quite daily) since March 1st. It has been pure torture, to be honest. I wish I had a gym available to me, but I certainly can't drive to one right now, and I don't belong to a gym, much less one that's open at 10:00 at night, and I don't have a personal valet to drive me to one, anyway. So I'll just have to deal with that. That means that I don't have a recumbent bicycle for cardio (or the seated elliptical trainer from PT). So, I have two options, depending on the day: take a brisk walk around the neighborhood or climb up and down the stairs in my house. I hate the stairs, but it feels like good exercise. My best is up and down 40 flights in about 20 minutes. It hurts my joints and I have to monitor my heart rate carefully. Every time my heart rate surpasses 170 bpm, I sit down. It happens pretty frequently. I break out a good sweat. I can't describe how horrible it feels, but it's what I'm trying right now.

That's cardio. For strength, I do weight lifting with a 5 and a 10 lb. weight, and I do various core exercises. They feel terrible, as well, but what are you gonna do?

Easter week, I was feeling quite a bit better. But I think it was just a fluke. I'm definitely back to where I was, if not worse. I can barely move most of the time. It seems like I save up all of my energy for exercise, and then I suffer for it the rest of the time.

There's a study taking place right now by a research team in Texas on POTS and exercise. It has been accepted for publication in the American Journal of Cardiology. I will link to the findings when they become available. From what I've learned so far, I may be going about this exercise all wrong. This team advises only sitting exercise...no standing, or you will "overtax your system and setback your recovery." Maybe that's what I'm doing right now. They advise the use of a rowing machine or a recumbent bicycle for cardio every other day. No walks! Certainly no stairs. That does sound more manageable. I will have to consider this more. I look forward to the research being published. For more information, read here: http://www.potsrecovery.com/

I have an appointment Wednesday in Toledo with Dr. Grubb to discuss my POTS treatment. I plan to ask him for his advice on exercise, although I know he generally advises leg strengthening the most. I don't have a leg press machine at home, so stairs are probably the best thing I've got, along with ankle weights. (I wish I could still go to the gym!) I don't have any other specific questions, so I will just see if there is anything else the good doctor has to offer at this time.

I have more on which to update, but it will have to wait until after this trip to Ohio.

Surgical Review Board Says....

2010-03-30T17:23:00.002-05:00

No to Invasive Cervical Traction at this time.

I'm not surprised, nor am I disappointed, as I did not expect anything at this time. It's too soon, and I know it.

Oh, the wonders of having very low expectations and very little hope (for now).

Dr B, however, wrote to me directly, telling me that I should schedule a follow-up visit with him, now. I really don't understand why I would need to see him right now. He's the neuro-surgeon. I don't qualify for invasive traction or re-fusion at this time. So, why would he want me to fly to New York to meet with him right now? Maybe he's changing my surgical plan? I'm just not sure why I need this appointment right now. This is the waiting time. Waiting for the bones. Then, when I have a better DEXA scan, wouldn't that be a logical time to have a follow-up visit? When my body is ready for surgery?

I'm going to ask my nurse about the details of the visit, to see if she has any answers. But I suppose, if THE man wants to see me, then I probably want to see him, too. I don't have much else to do right now, to be honest. Really quite bored.

Maybe...

2010-03-16T00:07:00.002-05:00

I heard from my nurse at TCI, and she said that Dr. B is willing to see me for a follow-up appointment now, meaning that he thinks my bone density has shown enough improvement. However, we need to wait until the surgical review board votes to determine if I am approved for Invasive Cervical Traction. So, I was given the option of going to see Dr. B right away, or waiting until a decision is made on the traction. I decided that for financial reasons, we will wait until a decision is made on the traction, and hopefully do both things on the same trip to New York. Of course if it isn't approved, then the whole trip will be delayed anyway.

That's the update...not much of one. I'll write again when I have actual news, from the surgical committee, since they seem to decide everything now anyway.

Reassessing

2010-03-10T13:50:00.003-06:00

As I discussed in the last post, I've needed to reassess my situation, based on the fact that my surgery will most likely not be this spring. (I haven't yet heard anything final from my nurse at TCI or Dr. B...I'll let you know when I do hear something final.) I am determined to not let this discouraging news paralyze me.

So today, I would like to write about what my new plan for the next months of my life will be. Without a plan, it's easy to lose hope. But with it all written out, I can know that I am on track for a determined future.

First, I need to hear from TCI, of course. I need to find out what bone density numbers I should be aiming for, so I can know how soon I should plan on having my next DEXA scan. Also, I'd like to ask them for a prescription for a new CTO (cervical-thoracic-orthosis). I haven't been able to wear mine, ever, because it doesn't fit right. I'd be interested in getting either an Aspen CTO or a custom CTO. This may allow me more upright time during the day until I can have surgery.

Next, I made an appointment to see my endocrinologist. I would like to discuss several things with her. First, I'd like to make sure that she is willing to extend my Forteo treatment until surgery (and for at least 3 months after surgery, to allow adequate bone growth after surgery). I'd also like to find out if she has other recommendations to raise my bone density. I'd love her advice on specific exercises I can do or any dietary changes I can make. I am also curious if there are any other supplements she can recommend or if she would recommend a limit on the amount of salt I take in each day.

Also, I have made an appointment to see my POTS specialist. I need to see him yearly. I would be interested in his ideas on how to manage exercise with the extreme heart rates endured by my body.

I also need to get a new local cardiologist to monitor my bicuspid aortic valve and valve leakage problems. I need a new echocardiogram as well.

Dr. F recommended that I try Myofasical Release. However, I have yet to find any doctor that practices this within the confines of evidence-based medicine, so it is not covered by insurance, and I am not really interested in it at this point in time. I know that physical therapists have other therapies to offer that may be of benefit (like laser therapy). Unfortunately, only myofascial release was recommended at this time.

I have gotten orthotics for my shoes. They are extremely uncomfortable, but supposedly, that means they're working! If they were all squishy and gel-based, they wouldn't be doing anything for my sagging arches. Mine are made of cork and leather, and they are serious business. I think I am gradually getting used to them.

Since I got my bone results at the beginning of March, I have started two things: I've begun nightly exercise, no matter how terrible I feel. I can tell that I am stronger already. I'm following my own exercise plan, based on the equipment I have available, and the ridiculous number of exercises I've learned in my year of private training and multiple stints of physical therapy. But I really have to base the plan on my body's abilities for that day.

I've also reduced my salt intake by 66%. I'm now taking only 2 salt pills a day (instead of 6). It's a challenge. I've added Gatorade, and I think I'll need to add more. I just may chug Gatorade all day. I'm wondering if I can cut the salt pills in half, so I don't get such a megadose of salt. Just a little kick to keep me going. Then, maybe I could take them more frequently and not zone out so much of the day.

Besides that, I also plan to take a Spanish class at the local community college this summer. They offer a blended course (half classroom, half online). That way, you spend half as much time in the classroom. And classes are offered in the evenings. So, it would be a real possibility for me. It might be overdoing it, but I'd really like to do this.

So, that's the plan. I just need to hear from TCI for confirmation.

Dealing with Disappointment

2010-03-08T15:55:00.002-06:00

I've been forced to learn a lot about dealing with disappointment in my years of being sick. The most constant disappointment has always been the failings of my body. I had to learn in high school that for some reason, my body couldn't do the things that it should be able to do. I missed out on opportunities that I could have enjoyed. Every day was more challenging than it should be. But I learned to deal. I had no choice but to get through high school, excel in my courses, and move on to college. So that's what I did.

My next major disappointment came with the first surgery, in July 2002. It was an initial success. I felt better than I'd ever felt. So, when I began a quick decline after six weeks, having to eventually drop out of college, that was a major disappointment. But I learned to deal. I needed to remain a student to keep my health insurance. So I enrolled in community college and adjusted my expectations for myself. I also made an appointment at The Chiari Institute (TCI). I had to wait 9 months to go there. In that time, I became quite underweight, and lost most of my ability to function. But I kept pushing on, with a new goal in mind.

At TCI, we made plans for another surgery, a cranio-cervical fusion. I went through hard times. I missed out on a lot of life. But I believed that if I got through this, I would have my life back. So, I kept pushing.

My next major disappointment came after this surgery. Despite intense physical therapy, massage therapy, and a lot of patience, I wasn't getting any better. There was nothing more that could be done for me at the time. And my body was more broken than it had ever been. I was very lost for a while. In the end, I had no choice but to form a new plan and start moving forward again. So, I kept working and found a way to return to my college, in an electric wheelchair, 9 months after surgery. It was a long road, but I worked hard, with a goal in mind, and graduated school as planned. I even began working, part time, after graduation. I started my own piano studio, teaching 10 hours of lessons a week.

Unfortunately, the next disappointment was never far behind. As hard as I tried to pretend that I was fine, it was clear that I was not. My symptoms were unbearable. I hadn't contacted TCI in a while. When I did, they ordered some tests. They decided it was time for my next surgery. Life would need to be put on hold again. In June 2008, I had my spinal cord untethered. During the same trip, I had a life-changing experience: I had invasive cervical traction repeated. This test came back positive. This means that even though I already have my head fused to my spine, I still need more surgery to correct a problem of functional cranial settling in the area. So, I had a new reason to hope. The surgery on my spinal cord didn't seem to help much. I have actually been on a decline since then. I would have begged for them to perform the other surgery that day, but they wouldn't. They won't perform the surgery until my bone density improves.

That has been a new source of disappointment for me: my frustrating bone density. When I was first tested, my bones were practically average; just a little soft. But not strong enough to qualify for surgery. Unfortunately, the first endocrinologist I saw refused to treat me. There were two reasons: he refused to treat a pre-menopausal woman for osteopenia. And, he refused to treat osteopenia that was so mild. When I brought him a note from my neurosurgeon, he refused to see me anymore. He did refer me to another doctor.

The next doctor had similar concerns. The medications used to treat osteoporosis and osteopenia are untested in women my age. So you need to have really bad bones to get approved for them. It was decided that we would start with non-drug treatment. Unfortunately, my next DEXA scan, of course, came back worse than the last. So surgery was looking further and further away. Then, in October 2009, we finally started with a drug treatment (Forteo). Since it is such a strong drug, there was hope that it would show significant improvement after 4 months of treatment, which is when I had my next DEXA scan. Unfortunately, this showed very little improvement. So, we are back to waiting. This may mean postponing my wedding this time. We had actually planned the date based on having surgery sometime around this April. So, we may have to re-think that.

If I've learned anything from all this, it's that there are ways of coping with major life disappointments. For me, I need to stop and grieve over the loss. Then, I need to re-assess the situation. Then, I need to set new goals and time-frames. Then, I will be able to accept my situation once more, and keep moving forward. Of course, I know that disappointment comes in cycles, and acceptance doesn't last. So I don't expect to stay in this part of the cycle.

Misinformed

2010-03-01T19:09:00.003-06:00

I received my DEXA results in the mail today. Apparently, the woman I talked to on the phone misread the results. I actually made very little improvement. I haven't heard from The Chiari Institute yet, but I am very doubtful that these results will qualify me for anything. But, here they are:

DEXA scan performed 1/14/09:
Lumbar Spine:
The average bone density of the L2-L4 region = 0.989 GM/CM2
T-score = -1.8
Findings consistent with Osteopenia.

Femoral Necks
The average bone density of femoral necks = 0.855 GM/CM2
T-score = -1.3
Findings consistent with Osteopenia.

DEXA scan performed 2/22/10:
Lumbar Spine:
The average bone density of the L2-L4 region = 1. 029 GM/CM2 (an increase of 0.04)
T score = -1.4
While the value has increased, the findings are still consistent with Osteopenia.

Femoral Necks
The average bone density of the femoral necks = 0.830 GM.CM2 (a decrease of 0.025)
T-score = -1.2
The value has decreased, but the T-score has not significantly changed, and the findings are still consistent with Osteopenia.

Basically, this means that I was misinformed. I was told that these T-scores were positive values, but they are actually negative values. That makes a huge difference. If the numbers were positive, I could reasonably be approved for surgery this spring. But with negative numbers and bones still osteopenic, I will have much longer to wait before surgery. There's no way to predict how much longer the wait will be at this time. But, just for fun, I would guess the earliest I could be tested again would be 6 months from now, in September. However, if I wait until November, it would be a full year of treatment. Either one of those is a possibility. Insurance may require that I wait until January. So even that is a possibility. At this point, my doctor has only prescribed 6 months of Forteo. Clearly, I will need more than 6 months of treatment to get my bones dense enough for surgery.

My plan right now is to see my endocrinologist as soon as possible to ensure that I continue Forteo. It would also be good to hear from TCI what numbers they are looking for me to achieve, so that I can convince my endocrinologist to continue with the treatment.

Either way, this is a considerable step back in my progress.

I thought that this leg of my journey, the waiting part, was almost over. I thought I'd be planning two trips to New York in the near future.

Instead, I'm facing many more months of the same. Waiting. Missing out on work. Waiting. Missing out on life. Waiting. Possibly delaying my wedding. More waiting. I just may be the most patient freaking girl in the world.

My Appointment with Dr. Francomano

2010-02-25T16:58:00.003-06:00

I've been meaning to update on this for a while. I just haven't been able to get it done.

Dr. Francomano has very thorough appointments, and she even sends you home with a summary of everything discussed during your visit. So, I just have to go through my 8-page "Initial Consultation Summary" and decide what information is the most important. I'll do my best below. Afterward, I'll include any thoughts on the appointment.

First, there was a very complete medical history taken. I'll skip over that, since that information is all available at my website, which is now archived (http://zippergirl28.tripod.com).

There is then a list of diagnoses from my history:
*Chiari 1 Malformation, decompressed 2002
*Dysautonomia, POTS, diagnosed 2003
*Cranio-cervical instability/cranial settling, status/post fusion 12/2003
*Occult tethered cord, status/post untethering 6/2008
*GERD
*Paipilledema
*Osteopenia, diagnosed 3/2008
*Hyperparathyroidism
*History of Vitamin D deficiency
*IBS
*Scoliosis
*Restless Leg Syndrome/Periodic Limb Movement Disorder
*Hypovolemia
*Bicuspid Aortic valve
*Aortic Regurgitation

Surgical History
*Tonsillectomy, 3/2002
*Posterior fossa decompression, craniectomy, C-1 laminectomy, 7/2002
*Invasive cervical traction, 11/2003
*Cranio-cervical extraction fusion, skull to C-4, 12/2003
*Cholecystectomy, 4/2008
*Section of Filum Terminale, 6/2008
*Repeat invasive cervical traction, 6/2008

Hospitalizations
*1/16/03-1/25/03: severe drop attacks and blackouts, diagnosed with POTS

Known Allergies
*Compazine: dystonic reaction
*Wellbutrin: seizure
*Sulfa drugs: severe gastrointestinal distress
*Opiates: rash, severe pruritis
*Adhesives: skin deterioration
*Sensitivity to many medications, especially antibiotics and narcotics

Review of Systems
Frequent fevers
Difficulty falling asleep
Difficulty staying asleep
Chronic fatigue
Nearsightedness
Ringing in the ears
Swallowing difficulties
Shortness of breath
Ches pain
Racing heart beat
Light-headedness
Fainting
Heart murmur
Exercise intolerance
Nausea
Abdominal pain
Digestive problems
Urinary urgency
Urinary frequency
Easy bruising
Temperature instability
Migraine headaches
Non-migraine headaches
Tremors
Numbness
Frequent falls
Brain fog
Loose joints
Joint pain
Joint subluxations
Muscle pain
Scoliosis
Vertigo
Palpitations
Myoclonic jerking

Next was the review of my family history. I list a couple of the key points here:

A three-generational family history was obtained. Pertinent findings include:
*Joint pain and history of two shoulder dislocations in one family member
*Knee subluxations and joint pain in one family member
*Joint laxity in one family member
*Joint laxity and history of dislocations in one family member
*Knee subluxations in one family member
*Intra-cranial hypotension, spinal cord leak, hip subluxations, and joint laxity in one family member
*Degenerative disc disease in one family member
*Osteoporosis, scoliosis, arthritis, and loose knees in one family member
*Migraines, early-onset osteo-arthritis, weak ankles in one family member
*loose ankles, runner's knee, and tendonitis in wrist in one family member
*Atrial septal defect, arthritis, and degenerative disc disease in one family member

Pertinent Findings from the Physical Examination
Height: 64" (I shrunk again!)
Facial Features:
Grey sclerae (The whites of my eyes are not white when you shine a light in them; they're grey, because the connective tissue is very thin.)
History of dental crowding
Palate: High, not narrow on examination, but she is status post palatal expansion
Uvula: asymmetric
Other: widely dilated pupils, symmetric, reactive to light (must have been a good day)

Neuromuscular
Abnormal: absent proprioception, marked dysmetria on finger to nose testing, difficulty with rapid alternating movements. Reflexes are 2-3+ and symmetric. Downbeat nystagmus noted. No clonus, no Babinski. (Basically, this means that I have a number of strange neurological responses.)

Musculoskeletal
Downsloping ribs present (My ribs go down lower than they should, so there's a short distance between my rib bones and my hip bones.)

Beighton Score
Passive dorsiflexion of 5th MCP >90 deg. R and L (My pinkie finger can be bent back beyond 90 degrees, barely. That gets me 2 points.)
Thumb can touch forearm R and L (I can do this on both sides, getting me points 3 and 4.)
Elbow hyperextension beyond 10 deg. R and L (She actually measured. Every other doctor just "eyeballed" it. Both elbows did bend backward more than 10 degrees. Points 5 and 6)
Knee hyperextension beyond 10 deg. R and L (Both were quite bendy. Points 7 and 8)
Can touch palms on ground with knees extended. No (I always could do this one, but haven't been able to since my detethering, for some reason. So, my Beighton Score is officially on 8 out of 9. Much higher than other doctors have measured it.) Measurements to follow.

More hypermobility
Able to touch tongue to tip of nose

Shoulder Hypermobility: Hyperextension on both sides

Elbow Hypermobility:
Left: 15 degrees hyperextension
Right: 11 degrees hyperextension

Hip Hypermobility: Hyperextension on both sides

Knee Hypermobility:
Left: 20 degrees hyperextension
Right: 25 degrees hyperextension

Small Joint Hypermobility
CMC joint hyperextension
MCP joint hyperextension
DIP joint hyperextension
(My hands really are not very flexible. You want to see crazy flexible hands, you should see Gustavo's. He doesn't have EDS. Just stretchy hands.)

Pes Planus present (I have flat feet.)
Piezogenic papules present (These little tiny bumps that appear on the inside of my heel when I stand.)
Scoliosis present
Straightening of the cervical lordosis present
Straightening of the thoracic kyphosis present
Lower thoracic lordosis present

Skin
Soft texture present.
Hyperextensibility of the skin present (I never thought I had this. But she stretched the skin on my hand and around my neck and I guess it stretched more than normal.)
Translucent skin present (We all knew this one...everyone whose ever tried to do my makeup!)
Scarring present: atrophic scars on both knees. Well healed surgical scars, cervical and lumbar.
Evidence of bruising

Other Findings
She is wearing a Vista collar. There is a II/VI systolic ejection murmur heard best at the left upper sternal border.

Impression
Based on the above, it is our impression that Ms. Richardson has joint hypermobility, soft, translucent, stretchy skin, and craniofacial features consistent with classical Ehlers Danlos Syndrome. Her bicuspid aortic valve and the atrial septal defect in her [family member] are of interest and may suggest a specific sub-type of heritable connective tissue that has been described recently.

The family history is remarkable for connective tissue findings on both sides of the family and it is possible that she has inherited one allele from her mother and another from her father.

Other notes
"EDS affects collagen. Collagen is a protein, which acts as a 'glue' in the body, adding strength and elasticity to connective tissue.

It is important to note that the range and severity of symptoms may range from person to person, even within the same family. Most types of EDS are inherited in an autosomal dominant manner. This means that if a parent has EDS, each of his or her children have a 50% chance of also having EDS.

(We also discussed the genetics of the trifecta. She agreed that it is much more unpredictable, and while research is being done, we may never be able to predict or prevent passing on the trifecta. She couldn't say much more about advising me in my decision, because we just don't know. She was aware of "trifecta families", in which both Chiari and EDS seem to be passed down at a much higher rate than they would on their own.)

Individuals with joint hypermobility often experience chronic muscle and joint pain. The age of onset may vary from adolescence to older adulthood. The pain can vary in the number of sites of the body that it affects, in duration, severity, and response to therapy. The severity of pain is generally greater than what is expected based on physical and radiologic examination. Sleep disturbances and chronic fatigue are often present among individuals with chronic pain. Affected individuals are often diagnosed with chronic fatigue syndrome, fibromyalgia, depression, and hypochondriasis prior to receiving a diagnosis of joint hypermobility. The cause of the pain is not entirely understood. However, it is thought that the pain experienced by individuals with joint hypermobility is related to myofascial and neuropathic pain. Myofascial pain is usually located around or between the joints and is often described as aching, throbbing, or stiff and may be caused by muscle spasms. Chronic joint instability may cause the myofascial pain, as the muscles attempt to respond to the joint instability. Neuropathic pain may be caused by nerve impingement (caused by subluxed vertebrae, herniated discs, osteoarthritis, or other subluxed joints) and is often described as electrical, burning, shooting, numb, tingling, or hot or cold discomfort." All quoted from Dr. Francomano

Recommendations
Aquatic physical therapy for muscle strengthening (however, she said to hold off for now, because my body is not ready at this point)
Myofascial trigger point workbook to help with myofascial pain
Lidoderm patches for pain
Actipatch for pain
Orthotics for flat feet (I wonder if I just get these at the store, or go to the orthotist?)
Diclofenac topical for joint pain
Epsom salt foot baths
Follow up with TCI as planned

And that was it. I really like her. I would say the only thing that disappointed me a bit were the treatment options. I didn't realize there was so little to do for EDS pain. I'm not sure where to find a myofascial release practitioner, and she prefers that it is a physiatrist, and I prefer that it is one covered by insurance. Either way, I'm not sure that right now is the best time to start a new therapy regimen.

Also, I had 8 questions I prepared for her, but she didn't really have any answers for me.

She had no ideas on recovering from surgery.
She had no ideas on finding a physical therapist or a myofascial release specialist.
She had no ideas on my episodes of reduced consciousness.
She had no ideas on maintaining bone density after discontinuing Forteo.
She had no suggestions on IBS other than fiber.

So, I guess I thought she would have more to recommend on those ideas, but I definitely should have focused more on EDS, but those really are related to EDS, mostly.

I'm still glad I went. I have a definitive diagnosis. I have another specialist on my side. And I believe I'll be able to count on her if a problem arises.

A Very Big Day

2010-02-23T20:00:00.003-06:00

After being diagnosed with osteopenia in March 2008, then continuing cranial settling in June 2008, I knew exactly what I needed to do. I needed to have my cranio-cervical fusion revised. I knew that if I could have my skull raised and re-fused into the sweet spot, I would have less pain, more energy, and a higher quality of life. Dr. Milhorat believed that was in my future, and I do, too. Before I could have that surgery, I would need to have my osteopenia corrected. The medication Forteo was recommended for me by my New York doctors.

Unfortunately, this would be a much bigger challenge than I anticipated. My first endocrinologist refused to treat me, because I was too young, and my osteopenia was not severe enough to require treatment. So, I went to a new endocrinologist. She insisted on trying a year of conservative treatment first. I had no choice. She was willing to work with me and my doctors in New York, so she was the best choice I had. The conservative treatment involved calcium supplementation and mega-doses of vitamin D. I ended up with too much vitamin D in my system, but I still wasn't absorbing enough calcium, based on the blood and urine tests. So, on October 26, 2009, she finally prescribed Forteo, which is one of the only medications known to actually rebuild bone.

I used my Forteo as prescribed, in daily injections. The time until my DEXA bone scan passed so slowly. But when the day finally came, the question was: had it been enough time? Had the bone had enough time to grow?

I had my bone scan yesterday. I got the results over the phone today.

This is how to understand a DEXA scan results:
A T-score compares your bone density to the optimal bone density for your gender by standard deviation.
Here's the scale for T-scores:

Osteoporosis: T<-2.5 Osteopenia: T= -2.5 to -1.0 Normal Bone Density: T= -1.0 to 1.0 Increased Bone Density: T> 1.-0

Here are the results of my DEXA scan performed 1/14/2009:
Bone density at the lumbar site: T= -1.8
Bone density at the femoral site: T= -1.3

Here are the results of my DEXA scan performed 2/22/2010:
Bone density at the lumbar site: T= 1.4
Bone density at the femoral site: T= 1.2

This shows a significant improvement after only four months of treatment with Forteo!!!!!

So, I emailed my nurse in New York immediately. I told her my results. I told her to watch the mail for her copy of the results. And I told her that I'm ready any time for the consultation and invasive cervical traction. So, I expect to hear back from her shortly. And I'll keep you up to date.

UPDATE: I LATER FOUND THAT THESE RESULTS WERE INCORRECT AND I WAS MISINFORMED. THE POST-TREATMENT T-VALUES ARE NEGATIVE SCORES.

An Eventful Day

2010-02-07T14:57:00.002-06:00

Friday was quite an eventful day, which is pretty impressive, considering my many limitations.

It started off with a morning in which I couldn't sleep too much. This has continued since then, and I know I'm leaning toward the hypomanic side, so I blame it on this. Due to this, I started reading my book around 8:00, and I got out of bed around 9:15. I ate breakfast. Then, went back upstairs and sorted out some of my stuff (in the a.m. hours). I took a Provigil at this point, because I knew I would need it for the day. (I'm not taking Provigil with any regularity anymore, because it makes me do more than my body is capable of doing.) Next, at about 11:00, I got in the bath, which is actually quite an arduous task, for me. I packed up the rest of my stuff for my weekend in Wisconsin. My mom and I left the house at 1:15 (This is a very active start to the day).

We went to a 1:50 appointment with the eye doctor. I had two scans done there. Since I have very large pupils that are completely non-reactive to light, I didn't need to be dilated for either scan (just another sign of worsening autonomic dysfunction due to brainstem compression). One scan was a 3D optic nerve scan. This was just a couple really bright flashes in each eye. Really bright lights. It took a 3D picture of my optic nerves. Next, I had an optical coherence topography scan done. This one is pretty cool. It shows the depth of the optic nerve. With these scans, they were able to determine that my vision is essentially "safe." Even though the discs are bulging, underneath it, there are thousands of healthy nerve fibers. So, my vision is not at risk, over time, or with surgery. She also explained the bulging as an anatomical anomaly. Whereas most people have optic nerves that are "innies" (think belly buttons), I have "outies." What a perfect explanation, right? So, that's why my optic nerves always look so funny, and seem to perplex so many doctors. I may not have even ever had papilledema in the first place. But my weird optic nerves did catch the needed attention to get a brain mri ordered in the first place. Just another weird anomaly of my very interesting anatomy. I really do have an interesting list of birth defects. What a weird little girl my parents never knew they had!

After the eye doctor, we were hoping to catch a 3:37 train to Milwaukee. Unfortunately, there wasn't nearly enough time to get to the station. So, we had to stall. The next train was at 5:32. It seemed like a lot of time, but the station was a ways away, in rush hour traffic, in snowy weather. So, we decided to stop at Costco to sort out a membership issue. Then, we stopped at Subway to buy my dinner that I would eat on the train. I got to the station by 5:00. I had to sit around a bit, but it's better than missing my train.

Okay, are you ready for another train story?

Well, I was ready for my train this time. I had my bags. I asked for help with only getting my walker up the stairs. Sounds easy enough. I was the last one onto the car, as always, because I'm handicapped. So, I stood there alone, trying to fold up my walker while holding 3 bags. It was actually really hard to do. You need to get the walker at just the right angle to fold the sides in. By the time I had it folded up, I saw that there was absolutely no where to put it. There's only one place on each car to put walkers and wheelchairs, but the area isn't actually reserved for them. It was completely full of suitcases. I decided I would just lean my walker sideways against the suitcases and get to a seat as soon as possible, because I know how hard it can be to find a seat. Unfortunately, I was too late. The train started moving. My walker was already folded up. There was nothing on the walls to hold on to. I fell flat on my butt, and with the movement of the train, rolled backwards onto my back. Everyone in the train car was staring at me, but no one moved to help me. I really didn't know what to do.

Luckily, on my train each weekend, a guy named Marty sells concessions. He is one of the friendliest guys you'll ever meet. While I was on my back, he came running down the aisle. He immediately moved the baggage out of the way for my walker. One lady whined/complained ("Hey! That's my bag!"), but he didn't care at all. Then, he told the person sitting in the front seat she would need to move. She looked shocked and appalled, but she did it immediately. Because Marty meant business. Keep in mind, the train attendant didn't do any of this himself, even though it's his job. Marty helped me up and even brought me a free bottle of water. It's amazing. Everyone will stare at you. But not a single person other than Marty offered to help. They even looked annoyed at having to do anything that might disrupt their day.

So, I should have been set for the rest of my ride, right? No. Because I'm me. But really this part of the story isn't about me, so I will do my best not to make it about me. After about 10 minutes of our ride north, our train came to a sudden stop. The emergency brake and been pulled. I was facing backward (bad idea), so the pressure on my neck of the inertia of the train was intense. The lights flickered off and on. It was a little scary. A very vague announcement was made about an "incident" and a "delay." So, we sat. For quite a while, we were under the impression that something was wrong with the train. Eventually, during one of my phone calls with Gustavo, he read online that pedestrians had been hit by a train in Lake Forest. I knew immediately that it was our train. We learned later that two teenagers were hit, and at least one of them was killed by our train. We stayed where we were for 2 1/2 hours. No passengers were allowed off the train. Marty wasn't his same cheerful self, but he did sell out pretty much all his merchandise, especially all of his alcohol. But we, the passengers, were never officially informed of the nature of the "incident." We figured it out on our own.

My back was in serious pain by the end of the night. The seats recline, but just barely. And my head pain spread throughout my skull. I felt very faint during much of our delay. But again, this night wasn't about me. Some passengers didn't realize that this delay wasn't about them. For example, as we were waiting, one woman was making very loud phone calls during most of the ride. On every phone call, she would say, "I'm having the worst day ever. You wouldn't believe what's happening to me." I understand that it was an uncomfortable inconvenience. But we had a bathroom and concessions that didn't quite run out. So, I'd say, we didn't have it so bad. The families of the victims had a very bad day. The train engineer that left the train with a pale, blank face after having seen everything happen: he had a very bad day. But for the passengers, it couldn't possibly be the worst day.

But, I'm very glad it's over, and I'm very sorry that it happened. It's just a rumor, but I heard that they were just walking across the tracks, and didn't see the lights. There was another death at this same station just a couple months ago.

Moving on, because it's hard to think about this too much.

For me, personally, I need to figure out how to work out the train ride a little bit better. I certainly don't have a manageable system figured out yet. I could go back to the wheelchair, because then I won't need to stand up on the train, but I will have to deal with the lifts and the fact that I don't get a real seat; I have to stay in my wheelchair. That causes the bumps of the ride to be especially rough. So I don't like that option. So, I think what I need to do is the following:
*Get in line with my walker, but collapse the walker on the platform, so I don't have to do it on the train, as it takes up valuable time.
*Ask the attendant to stow the walker for me AND assist me to a seat, so I don't fall down.
*Bring less baggage.
*Wait until the train has definitely stopped to stand up, or I will just fall down again when it lurches at the end.
Maybe, if I do these things, I won't have so many little disasters on the train. I'm still feeling the falls. I really cannot stand up whenever the train is moving. It just multiplies my vertigo, making it impossible to stay upright without strong assistance.

Medication update:
The side effects from the Topamax are wearing off (the tingling), but I don't think my headaches are any better. I will try to increase the dose this week. The Mobic, which is supposed to be like Celebrex, but better, isn't working well at all. I think I'd be better off with Advil, at this point. So, I'm going to test out Celebrex soon.

Coming out of hibernation

2010-02-03T19:26:00.005-06:00

So, I haven't written in quite a while, because my life has been stuck at such a standstill. Lying around in bed all day doesn't provide much inspiration for writing. I think I've finally collected enough little stories to add up to one post.

I've seen Dr. W, my pain management doctor, a few times. I've been taking Topamax for my chronic daily migraine. Unfortunately, I can't get up to a very high dose, because of my main side effect. The problem I have is with tingling in my hands, feet, and face. So, I'm not sure how much that medication will be able to help, unless the side effect wears off. Also, I've been put on Mobic. It is for arthritis pain. I've been on it for two days, so it's early, but I think it helps a little. I took my mom's Celebrex one day, out of desperation, and it seemed to help much more. But I'll give my medication a fair chance, before I ask for a switch.

I'm having severe hayfever symptoms, even though my last allergy tests were negative. My medications are not helping, and the symptoms are quite severe at times. The sneezing is definitely making my headache worse. Same with the sinus problems. I'll be seeing the allergist tomorrow.

I'm seeing the eye doctor for a 3D optic nerve scan Friday.

I also started seeing a new psychiatrist. Over the holidays, it became clear that my psychological state was evolving. I didn't feel comfortable going to my current doctor with this issue. So, I arranged to see a new psychiatrist in January. I really like her a lot, and I really trust my treatment to her. It's hard to say whether my psychological condition is organic (occurring naturally) or secondary to a neurological or pharmacological problem. Nonetheless, it needs to be treated and managed. I have developed symptoms of hypomania that are very troublesome at times. This is a symptom of a condition called Bipolar II. That is the "lesser" form of Bipolar, because the episodes of mania are less severe. However, the episodes of depression are no less severe. This is not necessarily part of the Bipolar II. As long as I stay on top of my meds, I don't have much trouble with it, for now, thank goodness. This is actually a manageable condition, unlike my others. I should mention, though, for some people, it is much less manageable. I've just been very lucky, so far. In addition, I have a condition involving rage episodes. This is a very difficult thing to deal with, and can be quite severe at times.

We removed the car insurance from my car, because I haven't driven it in so long. That's perfectly logical. It just makes it very clear that I will not be well enough to drive for a very long time. It's an overwhelming thought: that I will be trapped in this house most of the time for so long. And that I really have lost my independence. I can't go anywhere without asking someone else first!!!! This is a tough one to get used to. But it's made less hard by the fact that it's been a very gradual transition. I actually haven't driven my car in at least two months, so this isn't actually something new. It's just the possibility that is gone.

Luckily, with the money we're saving on car insurance, we were able to buy a 10-ride ticket on the Amtrak for me to go to Milwaukee. So I'll be able to take the train into Milwaukee to be with Gustavo most weekends in the near future. It's quite an expensive ride, and the ride to the station is a little out of the way on both ends, but it's worth it to continue my weekend visits.

So far, I've taken the train twice. The first time, I used my wheelchair, and bought a handicapped ticket (which gets you 15% off, with an unpublished discount). When you want to ride the train in a wheelchair, you have to call ahead, and arrive early. Then, you have to ride out to the train, with all your stuff in your lap. Then, you roll onto a lift. They crank the lift up. Next, you have to ride off the lift, onto the train. You can tell that they don't do it very often, because they're not very good at getting the levels even, or lined up with the train. It seems like a pretty perilous transfer. Luckily, I made it okay onto the train. There's only one place you can sit. It's next to the garbage cans in the front of the car. You slide into place facing sideways; then lock into place. You don't get to move the entire ride. You hold your baggage on your lap the entire ride. I was incredibly uncomfortable. Also, while the other passengers are casually swaying side to side, you are in your own miniature amusement park ride, getting miniature cases of whiplash every time the train "sways" from side to side. Since you are facing sideways, this sway is a front-to-back jerking to you. Overall, the ride was incredibly uncomfortable, and I wouldn't recommend sideways train riding to anyone with neck damage. Also, the luggage in my lap made it impossible to readjust my position or stretch my legs. So my legs and back were all knotted up by the end of the ride. I couldn't believe the passengers so near to me were yawning, listening to iPods, casually relaxing, or even sleeping through this nightmare!

Then, on the ride home, my fellow train-riders and I were in for a surprise, as we waited an hour and a half for our train to be ready to board. When the announcement was finally made, I wasn't too surprised to hear that the train was not in working order, and we would all be taking buses to Chicago. This was all new to me, but I figured if I could survive the ride in on the train, I could certainly survive the ride out on a bus. But that was not the plan. Since I was their only "special" passenger, I was the only one that would not be taking the buses, which were not accessible. Instead, I would be taking a cab all the way to Chicago. It was already 10:00 at night. If I were given the choice, I probably would have chosen to stay in Milwaukee one more day, but this was already planned without me. So, I waited another 25 minutes, until after the buses had already departed. My "cab" came. It was actually just a guy in a car. Not even a specially marked car. Luckily, he was legitimate. But my wheelchair had to be completely broken down to fit in his car. This was definitely not any more accessible than the bus would have been. Plus, we ran out of gas, and he didn't have money. Plus, he didn't use any heat, in January. So it was freeeeeezing. And I had such a headache after the long night, so the loud music didn't help.

So, after taking the train on a wheelchair, you'd think I wouldn't be anxious to do it again. But when you run out of transportation options, public transportation starts to look pretty good. So, the next time, I decided that maybe the train would work better if I took my walker. At least I wouldn't need the lift, right? So, we bought the 10-ride ticket, which gives you a 25% discount. You can't get a discount for being disabled, in addition. So this discount is better, anyway. When my train arrived, I was zoning out. I didn't even hear it called. So the guy working at the station came to help me, saying he didn't want me to miss my train, which was really nice. He offered to help me with my stuff, which was also really nice. I had with me: my walker, a messenger bag, a backpack, and a purse. He took: the walker and the messenger bag. Yes. The walker. He pushed the walker all the way out to the platform from inside the station. Luckily, I can walk without the walker. I use it for security, for balance, and for sitting. But this was one of the strangest kinds of help I've ever received. At this point, a worker on the train took the walker from him and loaded it on the train. I had to walk all by myself from the station to the platform and onto the train. My messenger bag was dropped onto the train for me. Unfortunately, it had my laptop in it, when it was dropped. This seemed like a very strange way of helping me. So, I was very tired at this point. I was out of breath and my legs were starting to give out on me. I had to find a seat, and none were readily available. I walked the entire length of the car (about 30 rows), carrying my 3 bags, holding onto seats as I walked. Whenever I saw an empty seat, it was a window seat, and the person in the aisle seat, blocking it, was either asleep, or pretending to sleep. I reached the other end of the car, and still had no seat. By this point, I was very weak, tired, and woozy.

Plus, the train was moving by now, so my vertigo was worse than usual. I could barely stay upright, and probably appeared drunk. What choice did I have but to turn around, looking even more pathetic, and this time, actually start begging for a seat. Luckily, the first person I asked actually took pity on me and let me sit. Probably because I looked quite sickly at this point. Once I sat down, I could finally relax. Other than my problem with germs in public places, I found myself to be pretty comfortable. My seat reclined (heaven). I didn't even need something to do. The crazy "turbulence" didn't bother me too much. Of course, I still wore my neckbrace, which I always wear. But, the side-to-side swaying didn't seem so rough. When I reached my stop, I took charge of my own possessions, meaning I carried my three bags and my walker down the steps (very difficult), but it was worth it. My ride back to Chicago was actually uneventful. No one stole my walker, even for a short time. I had temporary help with my bags. And I got a seat in the front row. So easy.

My other news is that I have an appointment with Dr. F, geneticist extraordinaire, scheduled! I'll be seeing her February 17th. I've been trying to figure out what to expect from this appointment, and what to ask her. I always compile a complete list of concerns and questions before an important doctor's appointment. I'm just not sure which concerns should be directed toward her. I know that she specializes in EDS. But she also helps people with POTS. And she is the one to talk to with genetic counseling type questions. So here's what I've got so far:

1. a. What can help with the especially slow recovery after cranio-cervical fusion (and revision)? My recovery took at least two years last time, with overwhelming and lasting fatigue, paralyzing muscle spasms, and relentless pain.
b. What kind of improvement is reasonable to expect (long-term) from a cranio-cervical fusion?

2. a. How to make a physical therapist understand EDS and POTS and establish a helpful and safe exercise routine?
b. What kinds of exercise are helpful and safe?
c. What movements/activities should be avoided to keep my joints strong as long as possible?
3. a. Is it okay to be on medication like Mobic/Celebrex/Vicodin forever?
4. a. What are my chances of passing this on (Chiari/EDS/POTS)--even a reasonable guess would be appreciated; any input would be appreciated?
5. a. Are there any vitamins/supplements that I should be taking regularly?
b. Is there a way to test to find out my levels of important vitamins to check for deficiencies?
6. a. I take 2000+ mg NaCl/day to maintain consciousness, but I'm worried about my bones. Any ideas on what else I can do about this?
b. I am on Forteo, to strengthen my bones before surgery, but I know my endocrinologist won't let me stay on it post-surgically, because she doesn't believe in off-label use. What should I do after that to keep my bone density up?

I have a million other questions, but I don't know if they're really her "field" or not. I can't just ask her every question that I don't know who else to ask. But I am also wondering about the following things:

*Heart palpitations-strong and resonating (intermittent symptom)
*hypersensitivity in skin (intermittent)
*severe PMDD, hormonal problems--------maybe this one
*IBS/bloating/severe abdominal pain (intermittent)--------maybe this one
*severe allergies with negative allergy testing (intermittent)
*Glucose level high in every blood test, but not diabetic--------maybe

rambling thoughts

2009-12-23T16:39:00.004-06:00

Too sick to write for real. Just thoughts...

Still restricted to bed. Can't be touched, cause it hurts. palpitations resonating through my upper body every 10 seconds. hot and cold flashes are painful and frequent.

trying to avoid e.r. they won't help. so what will? cardiologist will only deal with palpitations

how to get a doctor to take me seriously. dr. g is known to make me cry by saying that i just need to exercise. but he's the only pots doctor i have.

paroxysmal rage and crying spells are making me scary and useless. maybe i'm just mentally unstable

Dr. W--look into sleep meds for worsening insomnia

Custom CTO? How to get one. If it would help.

Dr. K--help with emotional pain

how to keep living this life? when you just don't want to anymore. nothing anyone does can actually help.

poor buttons, my puppy with gallbladder disease, she hurts all the time. she has no choice but to wait, too.

consider a port for frequent fluid infusions. symptoms seem to lessen with increased salt and fluids

Frustrating Heart Symptom

2009-12-18T01:20:00.002-06:00

I have had this symptom occasionally for a long time, but every doctor has ignored it. I just want to know what it is, especially since it is getting more and more frequent.

So, originally, I labeled this symptom as a palpitation. But it seems to be more than that.

I will do my best to describe.

It feels like my heart forgets to beat, and then starts pounding and trying to make up for what it missed. It feels a bit like being startled. But I don't just feel it in my chest. I feel the pounding in my hands and the front of my face, too.

It usually happens spontaneously. But when it's really bad, it will happen repeatedly, especially when I try to talk or even just exhale.

Another way to describe it is the feeling you get when you hold your breath for too long, so your heart starts pounding. But I'm not holding my breath.

I would really like to know what is happening, and why. I also wonder if an anti-arrhythmia drug might help.

Too bad none of my cardiologists have ever taken me seriously. But it feels like a symptom that should be taken seriously. It disrupts whatever I'm doing, often for an entire day. The only relief comes from laying flat and breathing steadily (not too fast or slow).

Yet another reason to never again leave my bed.

The Inevitable Reality Check

2009-12-18T00:12:00.003-06:00

Whenever I write a painfully real blog, I later feel guilty for sharing this dark side with the world. I don't think I'll delete the last post. But I will say that rage attacks are horrific for anyone involved. And I'm currently suffering from Pre-Menstrual Psychosis. I believe this is caused by compression of the pituitary gland. So, I will officially be skipping my period for the foreseeable future. There is no good reason to go through that kind of torture. I do not like being possessed and out of control.

However, my hormones simply made my everyday struggles seem even worse. My feelings were still valid. Just a bit augmented.

Thanks for caring.

The Emotional Toll

2009-12-17T21:12:00.002-06:00

Today was truly unbearable. My symptoms have been so persistent. My functioning has been non-existent. The misery is so all-encompassing. It reached that point again...where I just can't take it anymore. The hysteria lasted longer than it ever has. The rage and despair just took over. I was a person possessed. I know it put such a strain on my family. I wanted to stop. I just couldn't. For hours. I finally put myself in a cold shower, after 3 Klonopins failed to calm me. I feel so terrible for my family. My loved ones are the only reason I haven't left this world much sooner. There's nothing within me that would stop me from giving up, completely and utterly. How else would I go through 7 years of intense distress...10+ years of pain? I have no choice. I can't even choose to cut myself anymore. I have no idea what I would do without my support. They are the only reason I hold on.

It's just that sometimes, it feels like a tug of war. My body is constantly telling me that something is horribly wrong. But there is nothing to do about it. Absolutely nothing. And it's so frustrating for those around me to not be able to do anything to help. But the tug of war is in me. I want to give up. I want to succumb. I don't know how to face another day (which I've said 1,000 times before). But I can't succumb, because I could never hurt my family that way, after all they've done for me.

But when will it be enough? When will I finally have gone through enough? How bad does it have to get before they'd let me leave?

I know the answer to this question. I don't get to leave. It will never be an option, no matter how unbearable my illness becomes.

I guess, on days like today, when I am so completely out of control, I wish we could have just called 911, had an ambulance come over and tranquilize me. That is what I needed. The medicine didn't work. After several hours, I put myself in a cold shower and screamed and cried. I had to treat my body like a body possessed. It was completely out of my control. Believe me. I'm great at meditation and progressive muscle relaxation. I can lower my blood pressure within a couple minutes (which is very unsafe). But none of that was an option today. With this level of depression and rage, it truly is beyond your control. So, sometimes it's best to just isolate yourself, so your loved ones don't have to go through all of the pain with you.

The shower worked this time. I truly hope that next time, we will have something stronger than Klonopin. I've been dealing with these hysterical rage attacks for quite a while.

I'm just sure I shouldn't be sharing all of this information with the whole internet. However, I know that other people in my situation read this page sometimes. And I'd rather not put up a front, in the one place I'm supposed to be able to express myself...to describe what it is really like to go through torture for 7 years straight.

I understand that this is a down time. Today is a day when I need to learn, again, how to come to terms with my situation. I will reach a point of acceptance again. And the cycle will continue. As it has for so long.

Do you know how long 7 1/2 years is? Think of how much has happened in 7 1/2 years. And all that time, I've been feeling just like this. All these same symptoms. Constant, unending pain. Body failing. Since I was 18 freaking years old.

I follow the people that used to be my friends on Facebook. I watch, I read, I follow their lives. I see all of the things they do, all of the places they travel to, all they achieve and accomplish. I lost my shot at achieving anything. I had to give up on my dreams.

And honestly, most of the time, I can accept that. I really can. I just wish that each day didn't have to be so hard. If I just had to lie in bed all day and accept the loss of everything I should be doing, I could accept that. Seriously. Believe me, I've been accepting that for quite a while. Since high school, my symptoms have made me miss out on things that I should be doing. If I could just rest during the day, in semi-comfort, I would be able to handle this better.

But that's not my life. I don't just lie in bed all day, comfortably, watching the minutes drag by. Nope. I hurt. I hurt so much. The pain is so omnipresent. My body twitches uncontrollably. My heart cannot keep a steady rhythm, so it races and skips and pounds. I feel like I'm being electrocuted. I can't get comfortable no matter how hard I try.

Is it time to seek medical marijuana? Ketamine? Is it time to be hospitalized and tranquilized full-time? All of those sound preferable to my present.

But I wouldn't know where to start. And I don't think my family could accept that. It's hard for them to accept that I should not be getting out of bed for anything, most of the time.

I'm just glad I'm not alone. I need to try to stop venting to the same people. It's too much of a burden for them, because they love me so much. That's why I put myself in a cold shower today. I didn't want to drag my mom through the storm with me.

I wish I could stop crying, so I could stop torturing my loved ones.

Loneliness as time crawls by

2009-12-15T23:36:00.002-06:00

Nothing new here. Just spending more days isolated in my room, confined to bed except for bathroom trips (thank goodness for that).

I don't so much mind my time being pointless (for now). I do mind that time moves so slowly. That I am so often alone. And that I can't have more "comfortable" time.

So much of the time, I'm having twitches throughout my body, especially in my legs. The muscles are fatigued and achy because they twitch all the time.

I'm also having trouble chewing, due to jaw pain.

It hurts to move even in tiny ways so often. Like right now, I get a wave of pain throughout my body every time I move. It's so frustrating.

Then, of course, there's the ice pick in the head type headache that lasts 2-5 days at a time.

Then, there's the baseline headache of pressure everywhere and the "sunken halo" headache.

I've been having palpitations more recently, as well. It feels like my heart misses a beat or two, then tries frantically to make up for lost time. I feel my heart pounding through my chest, neck, and shoulders when this happens.

The knee pain is very sharp with weight bearing, or even sitting or laying in the wrong position.

The body ache is quite severe tonight, as well.

Oh well. That's all that is happening here. I wish this time could go faster. I wish I could skip ahead to better days. I know there will be better days. It's just getting there that is so hard.

Denied again. The Plan.

2009-12-10T00:55:00.004-06:00

"we cannot book a surgery until:

*
the bone density has significantly improved
*
a follow-up visit at TCI is done
*
the CCF failure is again confirmed with an ICT
*
the TCI surgical committee ratifies the CCF revision

PB"

Blunt, as always. Initially, this news was absolutely heart-breaking. I spent about an hour in hysterical, inconsolable, angry tears. However, I now have a bit of a more concrete plan.

#1 Survive the next 2 months, doing whatever is necessary. This includes: spend less time alone, don't feel obliged to work the 1-2 hours/week scheduled, focus on the goal.

#2 Go in and get the DEXA scan on February 22. Pray for "significant improvement".

#3 Schedule a follow-up and new Invasive Cervical Traction surgery date a.s.a.p. (hoping early March?)

#4 Schedule my LAST surgery, a Cranio-Cervical Fusion Revision a.s.a.p. (hoping in April?)

#5 Recover and move on with LIFE!!!!

That all sounds great. I'm glad I wrote it out. I don't feel quite that confident yet. Intense sadness makes you feel a bit fragile. However, I still believe that recovery is a possibility for me.

Over the years of illness, however, the term "Recovery" has had dynamic meaning to me. Initially, after my first brain surgery, at age eighteen (2002), recovery meant complete recovery. It meant going on to live a "normal" and active life with no pain, no sickness, and no restrictions. With the extended length of the illness, going into the eighth year since decompression surgery with NO lasting relief, I now have a new meaning for recovery.

I understand that my life will never be without restrictions. I will always need plenty of rest. I will never work 40 hours/week.
It will never be without sickness (significantly more than average).
And it will certainly not be without pain.

I now have modified my hope for the future, to be more realistic for someone with this amount of nerve damage (from postponed surgeries) and the bones and joints of someone fifty years older than me.

My hope is to have pain that only interferes with my functioning some of the time. My hope is to resume working 10-20 hours/week. My hope is to have enough health and energy to have a (small) family. My hope is to make my life about more than just me. To contribute more to the lives of loved ones. To be a shoulder to cry on, instead of the one crying. To payback all of those that have helped me get to this point. To be the supporter and joy that my lifelong partner deserves. To bring happiness instead of stealing it. To stop surviving my life and start living it!

I need to find a way to accept that I have lost much of my teens and 20's to this illness. That doesn't mean there's nothing left. Look ahead, and stop looking back. Don't hold onto worthless resentments of everything that was lost. Understand that what really matters is what I haven't lost...what I still have left.

I know these things to be true. It's really living that way that is the challenge. It's finding a way to feel that way all of the time. Not just know it, but feel it.

Especially when I have to cope with being weighed down by seemingly limitless bouts of depression. For the majority of the last year, I've had a lingering numbness about my health. That's one kind of depression. Today, I do not feel numb. The floodgates are open. The tears haven't really stopped all day. Today, I feel incredible, deep, desperate agony.

But I do understand that this is just today. If I would just go to sleep, it will be a new day. A new day! To keep developing that most-hated virtue named patience. And it's challenging partner: perseverance.

So I will keep living. Because I have many people that love me. That's what makes me so lucky. I may still be crying ('cause I can't seem to stop), I will certainly have pain, and I'll want to give up many more times. But giving up is never an option. How could I let down all of the people that have loved me and cared for me through these very dark times? That would be ungrateful, unforgivable.

I apologize for the intense mood swings. It's probably best that I spent most of the day alone. This is quite unbearable. But somehow, someway, there is still hope. Because there is still a chance, a possibility for a better life; for a change. I'm not out of chances yet, so I'll keep fighting, even when the pain is this complete.

Thank you to my family for loving me through this...literally, pushing me through this with love.

Desperate Message to New York

2009-12-09T15:53:00.002-06:00

June,

This is Carolyn. In October, my endocrinologist, Dr. Camacho, finally agreed to start me on Forteo. (20 mcg injected daily) I'm supposed to wait until the end of February for my next bone scan. She is certain that by then, the DEXA will reflect a higher bone density. However, it doesn't feel like I can wait even that long.

I previously taught piano lessons to 25 students. Gradually, I've had to stop working completely. I am now restricted to bed 99% of the time. I'm missing out on my life. I've been quite patient, but I am now in a very desperate situation. I've been seeing a neurologist at a pain clinic, but it doesn't seem to matter anymore. Nothing can help with the severe headaches, arrhythmias, tachycardia, blackouts, choking spells, and nausea. Most of the time, I am so hypersensitive that I can't be touched.

The only thing that temporarily relieves symptoms is traction. That is the reason I am so certain that the revision of my cranio-cervical fusion is the only thing that can help. My fusion was done in 2003, when I was 19.. In 2008, it was found to be "inadequate" during invasive cervical traction. I'm 26 this month.

I understand that I am supposed to wait until I have been on Forteo for 6-12 months. However, it took me 15 months just to convince my doctor to start the Forteo after finding out my BMD was low. My condition has been on a steady decline during that time.

I am wondering how soon it would be possible to schedule surgery. Please. I understand that I am supposed to continue waiting. It just doesn't seem like a possibility anymore. I know how invasive the surgery is, and how long the recovery takes. But the longer I wait to have surgery, the worse state I'll be in at the time of surgery. Is there anything that can be done?

Thank you for your help.

Carolyn

A wedding.......but still in bed the rest of the time

2009-12-08T23:49:00.005-06:00

The wedding and related festivities were wonderful. Luckily, my body cooperated when it had to. Of course, we knew not to expect very much of it. We even had a pillow so I could lie on the floor at the restaurant.

Since then, my body has continued on low. I often wear the same pajamas for 5 days straight. I only leave the house about once a week (and even that seems like way too much). I spend basically all of my time in bed or on the couch. My body is really failing. I'm just grateful for days that the pain stays at a 7 or below. But many days, it goes higher.

It's hard to really comprehend how long I've been sick. Luckily, I'm too sick and tired to really think about all of the lost time. Still, looking back, it seems that my functioning has decreased a lot this year, meaning I was more functional before. It's hard to even remember that time. Time goes by so very slowly. That's why the wait for surgery (hopefully this spring) is so difficult.

I would write more, but there hasn't been much to say. My only medical update is: STILL WAITING!

I still have the constant headache and constant joint pain, both of which get worse when I try to get out of bed. The fatigue varies in intensity from day to day, but is still ever-present. I also have extra headaches, that feel like pressure in the head or sometimes ice-picks digging into my skull.

A frequent symptom bothering me lately is severe hypersensitivity. This is when my skin is so sensitive that my clothes hurt. My body feels too hot and too cold all at once. And I can't get comfortable no matter what I do. I also feel that my spine is being compressed like an accordion. When I feel like this, the only option is sedation. Any other existence is unbearable.

My mom was away today, so my grandparents came to spend time with me, which was really nice, even though, as always, I provide no entertainment. This bed-bound life is very boring. Especially when even talking takes too much energy.

The only reason I have the energy to type right now is that it's late at night. Computer usage is now limited to high-energy times. That's how low it's gotten.

Okay, headache is an 8.5 tonight. OUCH. I haven't been sleeping well, but I guess I'll try. If nothing else, the room will be dark and silent...just the way I like it.

Holiday Weekend

2009-11-27T16:26:00.003-06:00

Yesterday was Thanksgiving, which was a lot of fun, but also quite a strain to get through. Tomorrow is my mom's wedding, which will be a wonderful day, but also a huge physical challenge. It is so important that tomorrow is NOT about me. That means: no passing out, no semi-conscious spells, no severe pain episodes that disrupt this important day. That's quite a challenge. Currently, my body is punishing me for the very active day yesterday (two different holiday dinners in different towns, and a game of pool). Today is payday. Every time I do more than I should, I pay for it later. There is no escape!!! Nothing comes free. So, today, I spent about an hour unconscious. Since then, I've been trying to rest with overwhelming head pain (about an 8, I guess...). It also hurts to move my body at all. Even typing. So I will keep resting and spend the day in bed. Hopefully, I can take a bath tonight, so I'm fresh and clean for the big day, tomorrow.

The other big news: It seems that I may be able to make a trip out to Maryland to see Dr. Francomano in the new year. Nothing is certain yet. But it is at least a possibility. It would be wonderful to see her, because she fully understands patients just like me, with Chiari, Cranial settling/instability, EDS, and POTS. If I were her patient, she would be able to help me get through the challenges of recovering from my upcoming surgery, which will hopefully be in the spring. I'll post more once I actually have some concrete information, not just an idea.

High pressure

2009-11-13T16:49:00.003-06:00

Not blood pressure. That's very low, of course. The pressure inside my head seems to be at an all time high lately. My temples are so very swollen. Like my brain no longer fits in my head. It hurts so much. I wish I could have a spinal tap to empty out some CSF and decrease the pressure.

I've been trying to sort through tons of old junk in my room, but it just looks neater. It's actually a disorganized disaster. But it takes so much out of me to get anything done.

I barely move anymore. I'm limp and lifeless almost all of the time. I can't take it.

I want to go work out. I want to be in shape and not hate the way I look. I want to improve my body fat percentage. I want to feel well enough to do something that requires wearing make-up. I have such a meaningless existence, during the wait. I can't believe I'm still waiting. Watching more and more days pass by. Wasting time. So many years wasted. When can I finally live again? My joints will continue deteriorating for the rest of my life. I just want to get my head fixed while I've still got some life left to live.

What a waste! I wish this didn't all take so long. I wish I could have had all of my traumatic surgeries very close together. Then, spent a couple years recovering {which is how long it takes with POTS}. Instead of wasting so many years between surgeries. I want a life.

Sorry for complaining. I know it could be so much worse. But the headache pain, all-over pain, nausea, weakness, fatigue, and general sickness make me pretty crabby and miserable.

Lost time...

2009-11-01T15:10:00.006-06:00

The Forteo shots are going well. The needle is the tiniest thing I've ever seen. And it comes pre-dosed in a pen, so it's really easy to use. The medicine doesn't seem to effect me at all, either. So that's good. This medicine is the very best medicine for increasing bone mass, which is what I need. Just wishing every moment that I'd been able to start this medication when I first went to an endocrinologist for Osteopenia in August 2008. Those 15, yes FIFTEEN months of my life have been wasting time (at least medically). And more than that, I'm still waiting on the surgery to revise the surgery I had in 2003. Yes. 2003, as in the year after high school. Now, I expect the surgery in 2010. Seven, yes SEVEN years after my cranio-cervical fusion was done "inadequately." After seven years, I expect to finally have my revision done, and done right. Maybe even feel a little better and get back to LIVING my life, instead of watching life pass me by.

In the meantime, I have a new massage therapist who is great. As always, I need a massage on a daily basis (if not more), but I'll have to settle for every other week for now.

Also, I found out that my vitamin D levels are in the range of Toxicity. That means I'm taking too much vitamin D. I guess this proves the existence of "too much of a good thing." Vitamin D Toxicity causes the following symptoms:

-Nausea
-Poor appetite
-Constipation
-Weakness
-Confusion
-Heart rate abnormalities

Obviously, I wouldn't have noticed those symptoms. I have those all the time anyway.

So, we're decreasing, but not stopping my dose of vitamin D. I'll stop taking the extra daily vitamin D, however I will still get it in my Citracal every day. I will decrease my megadose of vitamin D to once every 2 weeks, instead of once a week. So, my level should gradually decrease. But I am not to decrease my calcium, or discontinue anything. I guess this happens when you only have bloodwork done every 6 months.

My pain management neurologist is trying me on some new meds. Axert and Topomax. He also did some nerve blocks over my eyes. Didn't help much. I'll write if the meds help. He's decided that I have Chronic Daily Migraine...basically I wake up with a migraine every morning. I have a migraine of varying degrees all day every day. I go to sleep with a migraine every night. And my migraine is there all night long, too! Constant Migraine. That's a nice name for it. Sounds like my life.

Whatever. I hate all of this patience. It makes me very angry to have to be so patient. I've had to go to the psychiatrist recently, because the amount of anger inside just swells up and becomes unbearable at times. I don't think patience actually exists. It's just being willing to hide your impatience. More than that, you are considered patient if you are in a situation that forces you to wait. When you are forced to wait, you seem patient. It's kind of like courage, I guess. If you are forced to endure difficult things, you seem courageous. But really, you're not patient or courageous. You are just in a difficult situation that drags on endlessly.

So many people simply do not understand invisible chronic illness and invisible chronic pain. (Chronic=Constant) When you try to seem happy and cheerful, but you are actually in constant pain and frustration, you have to explode every now and then. Being sick makes me seem like a crappy person. I seem to be careless toward others, a bad friend, and really, really lazy. When actually I'm doing the very best I can to be a good person and make others happy. I'm just really, really bad at it! I seem lazy, because I can't get my own meals; I can't wash myself daily; and I can't earn an income. Even though it sometimes appears that I would be capable of doing these things.

Whatever. My head and neck hurt. I'm gonna lie down and forget about the world. Don't care if it seems selfish. That's how much it hurts.

FORTEO. FINALLY!!!

2009-10-26T22:43:00.003-05:00

Because it is not the FDA approved use of the medicine, I cannot say which doctor I saw today. He/she would like his/her identity protected. I was told this was an exception that was made.

Anyway, the doctor I saw today said that my urine Calcium done last May was low, so I will up my Calcium dose. However, since there may be something else causing this (like EDS-related absorption problems), the doctor finally agreed to prescribe Forteo!!!!! Forteo is a daily injection that you do yourself. It is the only medication proven to regrow bone. I have been prescribed a 6 month course. (The usual is 2 years.) This doctor has agreed that after 4 months of treatment, my bones should be showing improvement. So my DEXA has been pushed back to February 22, 2010.

Is this a good thing? In general, YES. I've been waiting for this medication for so long. And now I have it. Problem: Why did I have to wait so freaking long to get it? Another problem: This means that surgery is at least postponed another couple of months. I can't expect to have surgery before March at this point.

The medication itself seems pretty easy. It's made into a Pen. The medication has to be refrigerated. The pen measure the dose for you. You just insert the needle in an area that has at least 1 inch of fat (which is, evidently, my entire body!), and inject the medicine. The needle is so tiny, you really don't feel it going in! I mean it! I was a little disappointed. You know how much I love needles and blood! (Seriously, I love needles. Not sarcasm.) I took my first dose this afternoon, and fell into a very deep sleep. So I will be taking it at bedtime from now on. Another common side effect is dizziness, but I haven't noticed that. Other symptoms to look out for are muscle cramps and growing pains in the bones. So far, I think it's a great sedative for nighttime. No real complaints as of yet.

I just really wish I could have gotten this prescribed sooner, so I could be scheduling surgery for immediately after the holiday season. At least now, I can be sure that my DEXA test results will definitely improve next time, so I will be approved for surgery in the spring.

My main concern: I was expecting surgery in early January, so I didn't feel so bad stopping work 2 months before then. However, if I have 5 months to wait until surgery, I cannot be completely without income during that time. But how do you tell your piano students that you would like to try teaching for a few more months? I don't want them to think I'm messing around. I just need to make my life as useful as I can.

This doctor finally agreed to prescribe this medication, because my condition is finally pathetic enough to warrant drastic treatment (haha, like this medication is so drastic!) The thing is, my condition has been steadily worsening for a long time now. All information told me that this would happen. Why do the doctors have to WAIT until you hit rock bottom? Why won't they take you seriously until your condition is truly desperate? And why does New York insist on me becoming essentially bedridden before they'll perform surgery? We know I need the surgery. I am prepared for the challenges of recovery. But they're making me wait and wait and wait before they will do it.

Perhaps, I will write to my nurse at The Chiari Institute, and inform her that I have started Forteo. I can also inform her of how desperate my situation is. Maybe there could be some kind of lenience.

For tonight, I have a migraine that is reaching an 8, 8.5, so I will try to go to sleep. Of course, I wish I could exercise so I wouldn't have such a high body fat percentage. I hate being too sick to exercise. It drives me crazy. I'm one of those weirdos that actually enjoys going to the gym. It makes me feel good. (Not during, but after...I do have POTS, after all=exercise intolerance.) But I do miss getting a good workout and being proud to be able to keep my body healthy that way. I burn very few calories laying around all day. But my body doesn't want to allow me to be upright at all. Ahh...approaching a 9 on the pain scale. I guess I'll try to sleep.

Nervous...

2009-10-25T23:42:00.003-05:00

Lots of pain. Very few coherent thoughts. So tired.

I hope to schedule the bone scan for some day this week, to keep things moving. I expect that the bone scan will reflect the improved blood numbers, by showing much denser bones. This will get me approved for surgery a.s.a.p. I'm feeling as optimistic as I can about this. I'll post as soon as I get results.

The Higher You Fly, the Harder You Fall

2009-10-24T22:26:00.003-05:00

If your body is telling you that you should stay in bed and do nothing, and you choose not to listen...If you take stimulants to make you seem awake and opioids to mask the pain...you will always, always pay. I've used stimulants and opioids to try to defy my body for too long. And the costs are too great. There is always a downfall. Always some level of withdrawal. And your body keeps track of what you stole from it. You will always pay for every minute of borrowed time. You will pay with pain, fatigue, and distress. I wonder if I will ever catch up, if I will ever make up for the time I stole. For so long, I've tricked my body into doing things it wasn't able to do. And now it has shut down. And it tortures me with pain and sickness. I don't know how I'll survive sometimes.

Get me to a freaking neurosurgeon. Cut me open. Rip out my titanium. Give me new rods. Make me new. I don't care what it takes. I don't care how much it hurts to get there. I can't live like this anymore. Just make this end. How will it ever end? I don't know how to go on like this.

2009-10-20T22:23:00.002-05:00

Drop attack tonight. Fell on my left side on the hardwood. So far, I'm very comfortable in my decision to stop working. I would have called in sick every day this week.

Vertigo has been very bad. My temples feel quite swollen, as well. Full of pressure. I wonder if my pressure is up.

Post from last night

2009-10-20T20:22:00.003-05:00

I wrote this last night, when I didn't have access to the internet:

I’ve been having quite a difficult time lately. However, I consider myself the luckiest girl in the world. This weekend, my boyfriend of 3 1/2 years and friend of 7 years became my fiance. Gustavo actually wants to be with me the rest of my life, in sickness and in health. Many of you know the kind of commitment that is with someone like me. I have been very blessed to be in love with my best friend. It makes all of this pain and sickness seem a bit more bearable, when you know you won’t have to do it all alone.
Of course, now that I have had to discontinue all of my work, I do spend a lot of time alone. Today, I officially quit teaching piano lessons, at least for a while. I have several very loyal families that are willing to wait for my health to improve. In the meantime, I am left spending much of the day alone in the house, because most people work all day. It’s amazing how much time there is to deal with when you can’t work at all. Most of that time is spent being tortured. My own body tortures me all day. My pain level has been very high. I’ve been very symptomatic, with my autonomic symptoms, nausea, and vertigo all worsening and happening more frequently. I’ve had a lot of pain in my head, including severe facial pain. I’ve also had pain in my spine, feeling like it’s being crushed like an accordion. I also have a constant feeling of a lump in my throat, causing frequent gagging. It's impossible to sit up at times. The only escape is laying flat almost all of the day.
I will be seeing my endocrinologist, on Monday, October 26th. I expect her to order a new DEXA bone scan to determine the effectiveness of my first year of osteopenia treatment. Hopefully, my bones will have improved sufficiently (with only Vitamin D, Calcium, and Magnesium treatment). I will send the results to Dr. Bolognese, my neurosurgeon in New York. I will also certainly be sending him a pleading letter, describing the worsening of my symptoms. The hope is that he will be willing to schedule a surgical revision to my cranio-cervical fusion in the very near future. I will probably have to wait a couple months, however, because my mom will be getting married at the end of November! And I don’t want to interfere with the wedding and honeymoon plans. My health (or lack thereof) tends to interfere with everything. But I’ll do my best to keep being patient. The situation is just becoming rather desperate. I sometimes wonder just how many more days I can stand this. It’s just been so long. I just don’t want to lose more years of my life to this. I need help, now.
I am frustrated that I can’t post this entry while typing it, because my current laptop has difficulty with wireless internet reception. So it doesn’t seem to work on the top level of the house, where my bedroom is. Being connected to the internet during the day would probably help me to feel less isolated alone on my bed 24 hours a day. I wish someone could help me (a lot of good wishing does).

Symptom Categories

2009-10-17T00:11:00.002-05:00

The ice-pick in the eye pain went away. YAY!!! So I haven't needed too much pain medicine today. However, my body was still very sick today.

I'm going to write a description of the different types of complaints I have:

If I say that I have pain:
-Pain in the head/eyes/temples/jaw
-Skin hurts to the touch
-Joint pain all over
-Muscle aches
-Aches in torso

If I say that I am sick:
-Like having the flu
-Feverish
-Tired
-Weak
-Achy all over
-Lethargic

Low BP/Heart/Breathing/Oxygen
-I have low blood pressure, making me feel tired and weak
-Zoning out/brain fog
-Dizziness
-Pounding heart
-Shortness of breath (oxygen is not being transported efficiently)

Torture headache
-Beyond my usual headache
-Pain level at 8-10
-Usually the feeling of ice picks over one or both eyes

Just so you know what I'm talking about.

Learning to accept even less from myself

2009-10-14T19:51:00.004-05:00

Well, it's been difficult. (This won't be very articulate, because I'm in a brain fog, but need to write.)

I'm back to being almost completely bedbound. I just reached that point...the point where I just know I can't hold on any longer. Something needed to change just to ensure my survival. I had reached the point where I would smile for others, and then scream and sob as soon as I got to my car. I have reached the point where the time between lessons is no longer enough to get me through the lessons themselves. I'm very uncertain how much longer I will be able to work at all. I planned on going two more months, but I need to bow out early. I've canceled all piano lessons at the church. So no more daily drive to work. But even with lessons at the house, I don't think I'll be able to do this. It's simply a matter of acceptance. But it's also a challenge for those closest to me. To accept that I am falling back down. That I'm back to that place where I need to be taken care of. That's a challenge for anyone to accept, certainly a 25-year old woman that has had glimpses of independence and adult life.

So, the following symptoms are the reason that I am again restricted to bed about 23 hours a day:
Nausea, worsened to the point that I'm eating very little
Vertigo has returned, especially when I don't wear a collar or traction
Orthostatic intolerance: tachycardia and dizziness
Weakness: each limb seems to weigh an extra 20 lbs., more at times
Lethargy
Intense head pain: the ice picks over my eyes are back! And my temples seem to be permanently swollen
Cognitive deficits:
-brain fog (feeling really out of it)
-concentration/focus is a challenge
-difficulty talking or writing with any other sensory stimulation

These symptoms are only relieved by laying flat or with traction. Unfortunately, my traction options both have some pretty major flaws. (one blocks the hole in the back of my skull, squishes my ears, and hurts my jaw; the other pushes so hard into my rods that it creates a whole new kind of headache) Even the padding in my collar under the chin is thinned out so much that it's becoming uncomfortable. I use Kleenex for padding, but I think I need something more.

Anyway. As soon as I think I'm at a healthy level of acceptance of my limitations and my lifestyle, it gets worse. Then I have to learn to accept even less from myself.

Some people think that this is just one of the many ups and downs of my condition. That's partially true. But my good times are getting shorter and less positive. And my bad times are now unbearable, and lasting longer. So while I am still a study in oscillation, it is clear that my condition is still steadily deteriorating, overall.

I'm trying to accept this. Then, I'll be able to help others accept it.

The Walk-still recovering

2009-10-01T20:46:00.002-05:00

I am grateful to everyone that helped me in any way with the recent Conquer Chiari fundraising walk. At the 30 nation-wide sites, 3500 walkers participated, raising over $250,000. More information about funds raised is to come. Currently, my team has raised a confirmed $1445. However, I know that some donations haven't yet been accounted for. Whatever the final number ends up to be, I am so thankful to everyone that walked and donated.

Since the walk last Saturday, my body has had a flare of all symptoms, particularly those directly related to settling/dysautonomia. It's like every step I took in the walk made my head sink a little lower on my spine (at least that's how it feels). I've had a migraine for several days in a row in addition to a difficult instability headache. I've also had worsened nausea/pounding heart/breathing problems, all associated with cranial settling. The only thing that helps is traction. I've been using my Aspen Vista collar for cervical traction. It's not perfect, but it works as well as any home traction. Except for if I lay on the floor, and someone pulls my head away from my body...that is the ultimate symptom reliever. It's a great simulation of my hopes for a surgical outcome: relief of the headache, the overall sick feeling, and better breathing/heart functioning. I can't WAIT until surgery.

More than I really embody the adjective "courageous," I have been forced to practice the virtue of patience. Because I always seem to be waiting to feel better. Waiting for the doctors to think of a new treatment, to carry out that treatment. I wish I didn't have to be so darn patient!!!

Work is hard. My workdays are quite oppressive. I am definitely working as much as my body will allow. My days are as long as I can possibly stand them to be, often longer. I smile for those around me, but when I get in my car, I often scream just to release the pain and frustration. Then I go home and remain horizontal until the next time I have to work. Living this life is really like putting on a show. It's all an act! I guess I'm getting pretty good at it. I just wish I didn't have so much pain and sickness to hide.

I've been working hard to change two things about myself:
#1 I've finally switched to Skim (lactaid) milk, after a very long and gradual transition. It may not seem like a big deal, but it is to me.
#2 I've changed my sleep schedule. I took advantage of the calendar alarms in my phone to gradually wake up earlier and earlier, which makes me get tired earlier and earlier. It was a challenge, because tiring myself too much makes life impossible. But I'm proud to say that I usually fall asleep (without a bunch of sedatives) in the p.m. hours, and I wake up around 10 a.m. with no alarm. With an alarm, I can wake around 9:30. Any earlier, and it just doesn't seem to be worth it. But I'm very proud that I'm no longer up all hours of the night. I only need 50mg of Provigil, plus 30mg of caffeine, to get through the day. The extra stimulants were causing my insomnia (along with my episodes of hypomania).

In fact, it's almost 9:30 p.m., and I'm getting very sleepy. I may not need any sedatives or tranquilizers tonight. And I should have no problem waking up in the a.m. hours if I go to bed by 10 p.m.

That's all for today. Thank you to everyone for your ongoing support. I know that it's hard to remain interested and supportive when my challenges have been going on for so long. I also know that my problems are not the worst problems to have. I know that I am lucky to have so many people encouraging me. I just still have to find a way to force myself through each day. And that will can only come from inside.

Recovering from the Walk

2009-09-28T23:37:00.006-05:00

I would love to write all about the walk, which was amazing! My team was amazing! It will take some time to get the final numbers. I'll post when I know.

I chose to walk for about 3K out of the 5K. After the walk, we went home. At 3:00 that afternoon, I fell uncontrollably into a deep sleep. Poor Gustavo tried to wake me up for dinner, but I was way out. I woke around 6:30 PM. I still went to sleep at 11:00 that night. The pain has been much increased since the Walk. Same with my POTS symptoms. Feverish at times. And I've just been feeling weak and tired overall.

Gustavo was correct in what he said that afternoon. He said that it's almost a shame that everyone sees me only when I'm at my best, or at least pretending to be. At the walk, I had a lot of adrenaline, and I wanted to make sure everyone enjoyed themselves. So I put on a pretty good show. Unfortunately, this means that people saw the best version of me; not really an accurate representation of living with Chiari. That came later.

Very few people see the pain and sickness I go through every day. The most they might see is a semi-conscious spell or a sleep attack. I really hide any other symptom from people. It's just the best way to maintain positive relationships, from my experience. If you don't hide your symptoms, then you are seen as a "downer," or even a negative person. You have to put that smile on your face.

The aftermath of an active morning:

Overwhelming fatigue. Exploding head. Pounding heart. Dry heaves of nausea. Overall pain. Can't make it up the stairs without resting. Intense, severe sweating spells throughout the night; then awakening with chills. Digestion at a halt. Light-headed. Cannot go without my collar for a moment. Did I mention my head is exploding?

This is how my body is reacting to over-doing it on Saturday morning. Nothing unexpected.

Bonus: my legs are not too sore. I think I've been walking a lot more lately. And the MBT shoes must really be helping to strengthen my legs. Otherwise, I'd be really sore by now.

Plus: I didn't faint at the walk. I'm sorry I had to walk so quickly, but I knew that if I walked at a slower pace, I would collapse or even pass out. I had to just keep moving.

Bedtime now. At least I'm having no trouble getting to sleep...except I need an ice pack on my head. It helps distract my nerves from the enormous amount of pain coming from my exploding brain.

Thank you to all.

There are more thank you's and more details to come, once I'm feeling stronger.

Chiari Walk Tomorrow!!!

2009-09-25T11:38:00.003-05:00

I had another mystery virus this week. The fever was terrible, because it made all of my other symptoms unbearable. Thankfully, I seem to be getting better! :)

Just in time for the 2nd annual national Chiari fundraiser: Conquer Chiari's Walk Across America.

This will be my last shameless plug. I promise!

It is too late to register online for the walk. However, they will be accepting registrations at the walk sites, prior to the walk.

In Illinois, that means show up at the walk location in Busse Woods (Grove 5) at 8:30 AM to check-in or register tomorrow morning!

If you can't do that, it would mean so much to me if you could sponsor me. Every little bit counts! http://www.conquerchiari.org/ccwaa09/CarolynRichardson

I am so excited to have a team of 24 this year! It means so much to me. Our team name is Courageous Carolyn.

The definition for Courage is as follows: the quality of mind or spirit that enables a person to face difficulty, danger, or pain, without fear.

I think that is really nice. However, I've changed it a little, to more accurately define how I see it:

Courage is being strong enough to act like it doesn’t hurt, smile through your struggles, pretend you’re not afraid, and keep living as much as you can, despite EVERYTHING!

I used to think that smiling when you’re in pain was being fake. But now I understand that it helps everyone cope with your illness better if you put on a brave face, when you are able. And it does take strength and courage to do so.

Now, I can embrace my team’s name, Courageous Carolyn.

See you all tomorrow at the walk!

TIRED BUT WIRED: The Energy Crisis

2009-09-15T00:48:00.005-05:00

I've been saving up all of my ideas for posts for some time now, so look below to read all of the latest updates.

For today, I would just say that my constant state is that of being tired but wired. I have symptoms of narcolepsy a lot of the time. So I take medication for narcolepsy. It doesn't always work, so sometimes I take extra stimulants. Sometimes it works too well, so I need a depressant. But in general, I still feel tired, but wired. Not able to sleep (I've developed persistent insomnia). Not able to relax (I have frequent episodes of hypomania, with a need to get things done). But not really awake enough to be useful. I'm still so tired so much of the time. It may not seem like it, because that's how the medication works. If I'm talking, my brain switches on. If I'm listening, my brain switches off. So if I'm the one talking, I feel wide awake. But if I stop talking for some time, like when I drive, my mind tends to turn off, and I am prone to sudden sleep spells or constant yawning attacks.

Obviously, energy management is quite a science. I don't have it perfected yet, as I have chronic fatigue, and yet I seem to be awake until 3 a.m. every night, with the night hours being my most active, usually.

I've also developed an immunity to most sleep medications. Anything with Benedryl does nothing. Ambien does not make me sleep, however it does make me do strange things and then forget about them in the morning. But it doesn't help me sleep. On the other hand, I'm very sensitive to caffeine. I take a half of an excedrine (about 30 mg of caffeine), and it helps me stay awake for most of the day, when combined with 50 mg of Provigil. (Thankfully, Beverly from Dr. Grubb's office was able to help me get my Provigil re-approved, so I won't have to go without it!)

My whole personality is really affected by these drugs. I talk a lot...seriously, a lot. I zone out. I'm crabby. I'm giddy. I don't know if I'm asleep or awake. I feel that I am constantly almost asleep, except for the short bursts of energy I sometimes get.

But I'm still a really great actress. I'm sure most people I encounter on a daily basis are easily fooled by my peppy exterior.

In addition, being more active brings on significantly more pain. I'm to the point where I'm masking severe pain most of the day. So much so, that as soon as I am alone (like when I get in my car), I scream and yell to let out all of the pain that I've been holding inside. I sometimes need to yell or cry for several minutes, because of the amount of physical pain I've been hiding.

Then, there's the end of the day. At the end of the day, whoever is around to see me, usually my mom, is the unfortunate witness to the crash. I am in a horrible mood when I get home from work. I am always convinced that it has been the worst, most painful, most difficult day to get through, ever. So I always dread and fear the valid possibility that the next day will be even more painful and difficult. I'm in a pretty awful mood at the end of each day.

On the weekends, Gustavo is usually the one to witness the crash. I often sleep almost the entire weekend in Milwaukee. He has work to do, but I sleep. The laundry and dishes and cooking need to be done, but I sleep. I sleep, rest, and cry. I'm all used up by then. It's the end of the week. I've worked constantly, and hidden my pain all week. I've fought through the exhaustion all week. So, when I finally get to see Gustavo, when I should be able to enjoy myself, I usually crash. I just have nothing left to give at this point.

It's unfortunate, because I'm always in such a bad mood for the people I care about the most. It's just a side effect of "faking it" all day long, every day. The pain and fatigue and discouragement has to come out sometime. I'm so tired of living this way. And I wish I didn't have to be this way for my loved ones.

But no one can fake it all the time. There is always a crash. The downside always has to come out, eventually. And the more I do during the week, the happier I pretend to be, the harder the downfall.

So, no matter what medication combination is discovered to manage my energy just right, it is still very clear that this surgery is essential and urgent for my long-term well being. You can't fake your way through life. The body eventually gives out. I need this surgery in order to have a life. Until then, I will try to keep faking it. But, there is a point when you can't fake it anymore. I'll do my best to wait until surgery for that. You never know when it's coming. At some point, your body, and your will, just give out. I expect it to happen, and I understand that it will happen. When the time comes, I will know. I've been through it before, when I had to drop out of college. My body will make it clear that I need to stop working for a while and give my body all of the rest it needs. My current schedule can't continue forever. It's like running a marathon. It's an amazing act of strength and will. But eventually, you have nothing left to give. I'll hold out as long as I can. That's all I have to offer.

But if you see me, remember, I'm nearing the end of a very long marathon. I'm using everything I have just to keep going and to put a smile on my face for you.

I really hope this is close to the end. It would be nice to have the finish line in sight. I'm hoping that finish line is surgery in January. But then, we all know that this kind of surgery is really just the beginning of a new marathon, one of recovery! Maybe my time is getting nearer. The day when I won't have to force myself to smile. The day when I won't break down into tears over the severe physical pain I'm hiding. The day when it won't be so hard.

Courage is just knowing how to act like it doesn't hurt, smile through your sickest times, and pretend that you're not scared.

I can't wait for the day that I don't have to be so brave. But I will keep waiting, because apparently, patience is one of those virtues I'm supposed to really learn well.

Swallowing pills

2009-09-15T00:42:00.003-05:00

I've been having more and more trouble swallowing pills. It requires me to recline back, arch my back, and drop my chin down and head back. Even then, I often fail. The pill slides back and forth between my throat and my esophagus. This is called dysphagia.

I found something that is very helpful, especially for larger pills with a rough surface. It's called Spray 'N Swallow. It's a pleasant tasting spray oil that you coat your pills with, which helps them go down easier. (I recommend the Wintergreen.) It may not be a miracle. I still have trouble sometimes. But less often, and less trouble. I still need to recline to get a good angle, but I don't need to arch my back so much. I still sometimes have the pill get stuck, but I've found that an extra spray right into my throat can help.

Just a thought, if anyone else is having trouble with dysphagia. I know after my next fusion, the swallowing is only expected to get worse.

Body image problems

2009-09-15T00:20:00.003-05:00

So, I'm having some serious problems with my body image lately. Most of it has to do with being "skinny fat", as they call it these days. I'm still a slender person, but I have low muscle mass, and therefore, high body fat percentage. This makes me self-conscious about little bulges I never used to have. Also, having a high body fat percentage (as well as borderline cholesterol) is not healthy. I know I appear healthy. But by now, we must realize that looks can be deceiving when it comes to health.

I don't eat very much or very often, so simply reducing calories won't help me. I'm adding more fruits and vegetables to my diet. I'm also continuing to eliminate hydrogenated oils. (Gross)

It also doesn't help that I take very high doses of salt, which causes the body to retain fluid...which makes me "squishier" than I would be otherwise.

I also often bloat after meals due to poor digestion, exacerbating the problem further.

So, my pants don't always fit, and it makes me hate my body even more.

So, in addition to diet, I've tried to add some exercise to my routine, although it is quite difficult to do this with my busy teaching and traveling schedule, especially if I don't want to cause a setback.

Walking in the MBT's definitely adds muscle and helps me burn more calories when I do walk. Unfortunately, I don't walk all that much.

The other thing I do is some general core exercises to strengthen all the way around my core. I do things like planks, side plank raises, leg raises, flutter kicks, and bicycles. (These exercises can all be found here: http://www.ab-core-and-stomach-exercises.com/ab_exercises.html) I also try to do push-ups, but they are beginning to hurt my wrists. I ordered those push-up handles that rotate to help you do the "perfect push-up".

So, maybe all of this will make my pants fit a bit better. I hope so, because having such a hatred for my body and an increasing obsession with my imperfections is not healthy. I know- I shouldn't care about this. I should just worry about my health. But my health sucks. And even if it shouldn't matter to me, it does matter. I have very high expectations for myself, and it's very frustrating to be able to do so little to improve myself. But I know I'm doing my best, so that's all I can do.

MBT's, my new shoes

2009-09-15T00:13:00.003-05:00

So, on the advice of a blogger friend, I bought myself of a pair of MBT's. MBT's are the anti-shoe. They produce an unstable walking surface, which strengthens muscles, challenges balance, improves posture, and absorbs impact. Basically, they do what they're supposed to do. You have to make sure to walk heel to toe to get the most out of them.

The best thing about them is that they activate the leg muscles, even when standing still, making me less likely to pass out, since my muscles are squeezing blood back up to my brain. Awesome!

They're quite pricey, so I recommend trying them on for fit at a local store, then buying them online at a clearance rate. I was able to purchase a discontinued style. It doesn't seem to bother me that my shoes are "so last season".

The MBT's should also be good for EDS, since they strengthen the small stabilizing muscles around the joints. I still can't spend a day on my feet without my futuro knee braces though.

The only thing is, these shoes are supposed to be the most comfortable shoes you'll ever wear. And maybe, if I could have bought the style I tried on, they would be. But I could only afford a discontinued style. And man, my feet really hurt at the end of the day. Maybe it's the style, or maybe it's just that I'm doing more standing and walking than I normally would, so my feet need to get used to it. Either way, foot pain is now a daily complaint for me. (By the way, the shoes feel great for the first hour I wear them. So it is likely just my feet adjusting to the extra use. The shoes are really not the problem.)

If you can't find MBT's, Skechers has come out with a line of shoes called "shape-ups" that are supposed to be very similar. They should be more widely available. But I haven't actually tried them to vouch for them.

New allergist

2009-09-15T00:05:00.002-05:00

I saw a new allergist this week. My allergies have been contributing to my misery. My skin gets itchy and looks like it is covered in bug bites. Plus, my eyes and knows get so itchy that I just may rub them off one day. All that, and I'm already on Zyrtec, Optivar eyedrops, Astelin nosespray, and Benedryl as needed. I also use saline nose spray and a Neti Pot at times. Nothing's working...

So, I went back to the allergist, even though the last time I was there, they tested me for everything, and the only allergy I tested positive for was milk. And I am clearly allergic to dogs, cats, grass, trees, and other outdoor things. I saw a new allergist, and he's having me try some new things.

Dr. Handoyo is really great. I switched from Zyrted to Xyzal. I added a nasal steroid called Veramyst. And when things get really bad, I can try Atarax instead of Benedryl (since I'm immune to Benedryl these days). So, at least it's a plan.

He also ordered a blood test, to be sure there isn't any type of immune process going on. I just have to figure out when I can give up some of my blood, considering it makes me weak and faint to lose even one vial.

More doctor's appointments coming this week, including the pain doctor, the ladies' doctor, and the dermatologist. Fun, fun, fun!

New Pain Management Doctor/Neurologist

2009-09-14T02:16:00.002-05:00

I've been meaning to post about this for quite a while. I've been seeing a new pain doctor for about 2 months now. I try not to a doctor's name if I have something negative to say, but I only have positive things to say about Dr. Wilkin.

I've had a strong aversion to neurologists ever since a bad experience at Children's Memorial (no need to mention a name here). But Dr. Wilkin is awesome. He is so well-informed and up to date. He actually understands all about Chiari, EDS, and POTS. I didn't know that was possible outside of the Long Island and Baltimore.

He's be trying to help me manage my pain and my fatigue. It's actually quite a saga, so I won't describe every step of the medication experiments. Just the basics:

Tried replacing Provigil with Nuvigil. It was too strong and caused me to have a very scary episode of clinical mania. I'm back to Provigil, and at lower doses.

However, Provigil alone seems to only keep my brain awake...not actually give my body the energy to do anything about it. So, I've added a low dose of caffeine (about 30 mg) to my daily regimen. I'm very sensitive to caffeine, so this actually does a lot for me. When I see Dr. Wilkin next, I will ask him if he wants me to continue on caffeine, or switch to a different stimulant. I think if the caffeine is helping, I should just stick with this. Everyone else is addicted to caffeine. Why not me? And any stimulant will make your heart race. I've already learned that.

The only problem with all of the stimulants is that I am staying up later and later at night. It's getting out of control, with my average bedtime being around 3 a.m. and wake-up at 1 p.m. I have developed an immunity to Ambien, so I'll have to see what Dr. Wilkin thinks I should to about my new insomnia.

Unfortunately for me, my insurance company no longer wants to prescribe me Provigil, because, while I have the clinical symptoms of Narcolepsy, it has not been confirmed by a sleep study. So, we'll have to keep battling to keep getting Provigil. I don't think I could function anymore without it. At least I'm taking a low dose now, so the pills I have should last me a while...hopefully until the insurance company is willing to help me out.

For pain, we first tried Percocet. Unfortunately, I had a bit of a stimulant effect from it. So it also made me manic, without providing enough pain relief.

Next, we tried Ultram. Ultram (generic: Tramadol) is a non-addictive, non-narcotic pain reliever for moderate to severe pain. It has NO side effects (for me), and it truly does help me overall. The only thing it doesn't help with is when my pain level is above an 8. That type of pain truly does need an opioid (narcotic). Maybe Norco, since it is just Vicodin with less Acetaminophen.

I also plan on asking Dr. Wilkin about Trigger Point Injections and Nerve Blocks for my head and neck pain. We'll see if he is up for some needles!

I'll be seeing him again on Wednesday. I'll try not to wait so long to post this time. I've been so manic, that I've suddenly had a lot to get done (bipolar joke...some of you know just what I mean).

IBS pain/bloating

2009-09-11T03:07:00.003-05:00

So, getting my gallbladder removed truly made a huge difference in my life. The biggest one is that my chronic constipation is gone. The next is that my list of forbidden foods is significantly shorter.

Unfortunately, with POTS and EDS messing up my digestion, I still do have abdominal pain and bloating about half the days. My theory is that due to my dysphagia, I probably swallow a lot more air than I need to when I eat and drink. It makes sense, because I often inhale my food or water. This is another flaw of the same mechanism. I also strongly believe, after a year of food journaling, that for the most part, it doesn't matter what I eat! I already avoid foods high in fat. And I still follow some of my old rules about how to eat insoluble fiber safely.

If I liked tea, I know that fenell tea would help me greatly. Since I can't stand it, I do take fennel capsules (from a vitamin store) when the bloating is severe.

In addition, I always know that a heating pad can really help reduce the pain of intestinal spasms or bloating. And my gas pains can be quite severe.

So, I'm writing to say that I've found a couple additional methods of relieving abdominal bloating pain. They work for me, but I make no promises that they will work for you.

#1 Abdominal massage. You can google this to learn more about it. But really, it is what is sounds like. When you have trapped gas, it often feels like a very hard and tender spot in your abdomen. It really helps to relax your abdominal muscles and massage these spots. But there are specific ways to do it. There's a good demonstration video on YouTube:
http://www.youtube.com/watch?v=DBdWbYakqGo

#2 Yoga. Certain yoga positions are really, truly beneficial for bloating. They can be done right after meals, if you've eaten too much, or you feel the bloating coming on. They can also be done whenever else you feel bloating pain. The main poses that are helpful include bends, twists, and inversions. Here are a couple of links about yoga for digestion:
http://www.helpforibs.com/yoga/sequences.asp
http://www.self.com/fitness/workouts/2007/06/pms-relief-yoga-moves-slideshow
I really think these methods are helpful. They are just so logical. If you keep your abdomen sedentary, it will take a lot longer for the gas to dissipate. If you move your core around in different positions, you are bound to help things get moving in there. Even if you can't get rid of all of the gas or pain, there should be a noticeable improvement. If not, at least it was worth a try.

Hope this is helpful to someone. I know it has become of useful part of my daily routine. With medications, you often can't tell if they are helping or not. But these methods seem to show drastic improvements, almost immediately. These can also help you avoid needing to take extra medications for stomach upset (although sometimes, nothing beats MiraLax!)

*I am not a doctor. This is not a substitute for actual medical advice. Just some extra things you can try to make your life a bit easier. If they don't help, make sure to talk to your doctor!

Labor Day Weekend...ugh

2009-09-11T03:01:00.002-05:00

The week before Labor Day was great. My energy was up, so I took advantage of it. In other words: I lived life. I didn't overdo it. Nonetheless, during the weekend, I paid dearly for my living. I was sick straight through til Wednesday, missing work on Tuesday.

My narcolepsy symptoms were the worst they've ever been...falling asleep mid-sentence while on multiple stimulants to keep me awake. It really does make driving a challenge. (Clearly, I do not drive unless my medications are working quite well. So don't worry about that part!)

I also had every other typical cranial settling symptom: arrhythmias, pounding heart, shortness of breath, migraines, fatigue, nausea, achiness, overall sick feeling, etc. This made getting out of bed impossible most of the time. Luckily, I was able to rest much of this time, and I'm getting back to my baseline levels today.

As a side note, my massage therapist is moving to Texas (Understandably, she can't stand the Chicago winters). So, I only have one massage left with her...then I have to see who they find to replace her. Ugh. A good massage therapist is so hard to find...especially with all of these rods and crazy tight muscle knots.

The first of tons of new posts: Chiari Walk

2009-09-11T02:46:00.002-05:00

I have been building up a list of blog post topics for some time now. It's just taking me more time than usual to get them written and posted. So, I'll do my best to be efficient. Don't be surprised to see 5 or 10 posts all in one day.

The first thing I must post about is the chiari walk. The date of September 26th is readily approaching. I cannot express how humble and grateful I am that so many people have donated and even signed up to walk! What a wonderful surprise!

The important information is a change of time and location.

The walk is now taking place at Busse Woods (Grove 5). The entrance is on Higgins Rd., east of 53 and west of Arlington Heights Rd. The time has been pushed back to 9:30 (yay!), with an 8:30 registration time. All information about the walk, including the updated flyer and a map of Busse Woods is available here: http://conquerchiari.org/walk%2009/Walk%2009%20List%20of%20Sites.htm

Other than that, it would be great to have even more sponsors and walkers. If you are interested, go to the link above. At the top of the page, you can click on "Register to Walk" or "Sponsor a Walker".

If you'd like my personal donation page, go to: https://www.conquerchiari.org/ccwaa09/CarolynRichardson.

I am truly blessed to have so many people interested and participating in my fight to conquer Chiari, both personally, and in general.

If you have any questions at all, please contact me via email.

Thanks!

Overview

2009-08-29T19:29:00.001-05:00

Here is the latest edition of my medical overview:

Diagnoses:

Chiari Malformation: A skull malformation. My skull was formed too small, causing pressure in the back of my head, and a blockage of fluids that should flow freely in and out of the brain.

Ehlers Danlos Syndrome (EDS): A connective tissue disorder. My ligaments are looser than they should be, causing certain joints to easily dislocate. My veins and arteries are also lax, making it difficult for blood to be pumped the way it should. I also have associated heart valve defects.

POTS: An autonomic dysfunction. My autonomic nervous system malfunctions, causing problems with all involuntary functions of the body. This causes difficulty standing, due to my heart rate and blood pressure not reacting properly to the effects of gravity.

Tethered Cord Syndrome (TCS): A spinal cord malformation. My spinal cord is anchored in place, not allowing for it to move freely within the spinal column.

Cranial Settling: When EDS and Chiari join together, the skull-spine joint often becomes unstable. The skull begins to settle on the spine. This causes trouble swallowing, nausea, heartrate abnormalities, and other autonomic dysfunctions, including POTS.

Vitamin D Deficiency --> Hyperparathyroidism --> Osteopenia: Lack of vitamin D absorption causing excess PTH, reducing the amount of calcium in the bones, which results in low bone density.
__________________________________________________________________

Major Symptoms:

Headaches--I have a constant pressure headache. This pressure sometimes reaches a debilitating level. I also get migraines and more acute, sharp headaches. These are from Chiari and Cranial Settling.

Nausea--Nausea can be caused when there is increased pressure on a certain part of the brain. It is often worse when I am upright, and relieved when I lie down, but it never goes away. It is a symptom of Chiari and Settling

Neck and back pain--I have pain throughout my spine. This is caused by loose ligaments in the spine, allowing the vertebrae excessive movement, causing arthritis of the spinal joints.

Fatigue--I require a great deal of sleep. If I don't get enough sleep, my symptoms will worsen, leading me to return to bed and get more sleep. I also sometimes fall asleep at inappropriate times or have insomnia at night.

Tachycardia/Low blood pressure/fainting--After standing for too long, my heart races and my blood pressure drops. Sometimes for no reason at all, my blood pressure gets very low, and I can fall into a state of reduced consciousness. I get short of breath very easily. These are the main symptoms of POTS.

Heavy head--Due to the settling, I have difficulty holding my head up. This is very tiring and leads to severe muscle spasms in my neck from trying to support my head. All of my symptoms get worse if I don’t wear a collar to keep my head from settling too much.
Joint pain--I have pain in all my joints, especially my knees and hips. This is sometimes severe enough to prevent me from walking. It is caused by EDS. It is gradually causing early osteo-arthritis in all of my joints.

Flu-like symptoms--I have have low-grade fevers, cold sweats, and overall body aching, very similar to the flu.

Vertigo and Balance problems
__________________________________________________________________

Treatment:

I take medication to constrict my veins, in an attempt to help my blood flow improve and raise my blood pressure. I also take salt pills regularly to force my body to retain more fluid, which can help keep my blood pressure up. Salt pills are also the only thing that helps when my level of consciousness lowers. I drink a LOT of water.

I have medications that help me stay awake during the day. They usually work, but they sometimes make me a little too talkative and hyper.

I am very cautious about taking pain medications. I treat pain with ice packs, heating pads, and rest, when possible. Any pain medications I take a carefully monitored by my pain management doctor, whom I see regularly.

I've already had several major operations, but obviously, I'm not better yet.

In July 2002, at the age of 18, I had a posterior fossa decompression surgery. This involved removing part of the base of my skull and the back of my C-2 vertebra. I felt much better at first, but started to get worse again after a couple months.
When my symptoms continued to worsen, I began to search online for more answers. I sent my MRI’s to the doctors at The Chiari Institute in New York. They found that I had cranial settling, caused by cranio-cervical instability.

In December 2003, the day after my 20th birthday, I had a cranio-cervical fusion in New York. Titanium rods were bolted to my skull and screwed into my top 4 vertebrae. This fused my skull in place atop my spine.

After that surgery, I worked very hard to recover. I struggled to keep living my life, but my symptoms persisted. This led me to have another set of MRI's. I sent these to my doctors at The Chiari Institute in New York. It was confirmed that I had an occult tethered spinal cord. This is a hidden form of Spina Bifida that isn’t found until later in life. It was also found that I have an inadequate fusion.

In June 2008, I had my filum terminale clipped to de-tether my spinal cord. This released the tension in my spinal cord. Mild lower-body symptoms, such as leg pain, low back pain, and bladder control improved. These symptoms probably would have progressed without this operation.

I still require a revision to my current cranio-cervical fusion. This will reset the position of my head, allowing for greater stability. This surgery cannot be scheduled until my bone density has improved. My bone density scan will be done in November 2009. If my bones are stronger, I will be approved for surgery, to be done early in 2010.
______________________________________________________________________

Lifestyle:

Currently, I sleep about 12 hours a night. I have medication that helps me stay awake the rest of the time, usually. This is a great improvement over a few years ago.

I still need to spend most of the rest of my time resting, either in bed or on the couch. About 4 hours a day, I am able to be somewhat active, most days.

I use a wheelchair or a walker for assistance at times. I also wear a rigid cervical collar at all times. These things make it easier to get by.

I am in pain all of the time, but I have learned to live with it. I am expecting my next (and last) surgery to decrease my overall pain and increase my level of functioning, so that my life is slightly more livable.

More thoughts on today

2009-08-13T00:04:00.004-05:00

I think the reason I'm so upset today is that I am certainly having a sick day, I'm noticing the downward pattern in my health, I am worrying about my ability to keep pushing through...and I don't feel that I'm allowed to be feeling this way!

The ones that love me do have other things going right now. But I don't know where and when is appropriate to discuss all of these feelings, and more importantly, have these feelings validated.

I have serious fears about my ability to continue working, to continue commuting back and forth to Milwaukee, and to handle the severity of my symptoms.

I have been sicker than this before, but then, I was bedridden. There was no pressure on me to do anything. This time, there's tons of pressure. I need to keep doing everything.

And the constant struggle to do what is needed, despite horrible pain and serious worries, is turning me into a witch. I'm not the person I want to be. I don't know how to keep being nice when I'm struggling just to exist. Even just laying down is a struggle. The guilt is immense. Guilt that I'm not doing what's expected of me. Guilt that I am making other people's lives so much harder. And guilt for being so mean and irritable.

I also have serious concerns about if my bones will be ready in time for my December DEXA scan. I haven't had any actual medication for it, just supplementation. And people with EDS do NOT absorb the way we should. But my doctor wouldn't prescribe an actual medication to help rebuild bone. How do you think my bones got like this in the first place? I don't absorb properly!!! Adding more won't make me any better at absorbing! So it's quite likely that this year long wait will be pointless. The bones have to get better, or I can't have this surgery. And I've already waited so long. It's unfathomable that I may have to wait even longer.

I am overwhelmed by all of this. I can't stand this existence. I just can't handle all of this.

So that is what is upsetting me. I suck at this life. I need it to change. But I don't think anything can change for a while. Who knows how long? It's an endless wait.

I know, I just have to do my best. And not every day will suck this much. But for today, it just seems like more than I can handle.

I just wish it could be different. I wish I could be different. I wish it didn't have to be this way. I want to be better for everyone I love. I really hope that one day I will be.

Deterioration

2009-08-12T21:23:00.003-05:00

I had a new post written. But it was very raw and dark. The point of it is that my body is deteriorating, and the failings of my body are depressing. Sorry if these posts are ever too much. These are simply the truth behind the smiles. But I've re-written the post to make it a little easier to handle.

I have a new pain doctor. Dr. Wilkin is great, but I'm having a hard time with the insurance company approving medications. Ugh. Hope this gets worked out soon. I'm in so much pain. I have another appointment next week, and I hope to try something different and get the new medication approved.

I need help. I need to find a way to get through until surgery, which is at least 5 months. I have many new and worsening symptoms. But most importantly, I need adequate medication for my energy and my pain to help me get through until then. But my determination that I need this surgery has never been stronger. I just hope that once my bone density levels are improved, there is no further delay. I hope that things are settled at The Chiari Institute and I can get surgery scheduled early in the new year.

I need to stop burdening those around me...to be the person I should be and live the life I should live...to be a positive force in the world. I want to have this surgery so that one day, it will be worth all the trouble. Worth the trouble we've already been through, and all of the trouble that is ahead. It is such a huge burden to ask someone to help me bear. How can I ask someone to take care of me, to spend time with me, to watch so much more suffering? Only if they too have the hope that one day, it really will be worth their while. This is the only hope I have to offer, to hold on to. That after recovery from this surgery, I'll feel well enough for it to be worth everyone's trouble.

ASAP Conference 2009

2009-07-22T22:30:00.006-05:00

The ASAP Syringomyelia and Chiari Conference took place in Madison, WI this year. I only went to one day of the conference, so I can only report on that part. I've been recovering from it since Friday night, and I've been trying to find the strength to type up all of my notes. (I took excellent notes. It was a lot like being in college. And I was a really good note-taker in college!) I will include a comprehensive overview here. (My thoughts may be inserted at times, but I will separate them from the facts presented.)

I attended the conference on Friday, July 17. We were very lucky to be able to go, even for one day, this year. My mom, Gus, and our friend, Susan, all went together. We were only able to go due to the generosity of my Uncle Greg and Aunt Corinne, who have a home near Madison. It was so nice to be able to visit with them on Thursday and stay at their place that night. We are so blessed to have such wonderful family!

So, we had an early start on Friday, because the conference day started at 8:00. Thanks to Provigil, I was able to stay awake all day.

The first speaker was Dr. Bermans Iskandar, from the University of Wisconsin-Madison. He was the host of the conference and did an excellent job. He had a dry sense of humor, and seemed very friendly and smart. He introduced the topic of Module 3: Complex and Controversial Problems.

-The older the patient, the smaller the tonsillar herniation. Children will have exaggerated hernations, but that decreases with age. So older patients may have much less obvious herniations.

-Chiari malformations come from many different causes. Is it really one disease? Is there really one treatment that treats all? A thorough understanding of the pathophysiology is needed for proper treatment. A Chiari caused by a volumetrically small posterior fossa will be improved with decompression surgery. A Chiari caused by cranio-cervical instability will be improved with a cranio-cervical extraction fusion. Treatment is not "one-size fits all".

-Sometimes increased intra-cranial pressure causes Chiari; sometimes Chiari seems to cause increased intra-cranial pressure.

-Imaging: Simple MRI's give a very limited picture. Even CINE MRI's, which are the gold-standard for imaging Chiari's, provide inconsistent radiology findings.

>Thoughts: Throughout his speech, Dr. Iskandar inserted advertisements for tourist spots in and around Madison. He did it all with a straight face, too. Quite charming!

The next subject was Occult Spinal Dysraphism and the Tethered Cord Syndrome, presented by Dr. Jerry Oakes. He presented a quite strong stance against the current trend in treating Occult Tight Filum Terminale. I will present the information as fact, just as he presented it. This doesn't make it so.

-Occult Dysraphism is another name for a primary tethered cord.

-Anatomy: In vitro, the spine grows faster than the cord. Therefore, the cord ascends. The cord normally ends at the L1 vertebra or between L1 and L2. The nerves connected to S1-S5 are responsible for bowel, bladder, and sexual function. Therefore, all symptoms of tethered cord are related to bowel bladder, and sexual function. Because of normal variations in anatomy, 3.8% of normal patients will have termination of the cord at L2-3.

-Signs of primary tethered cord in children (when it's usually identified):
---Skin abnormalities at the location of the tethering. 60%
---Bladder disturbances. 20%
---Weakness of legs. 10%
---Deformities of bones in feet, hips, or spine. 5%
---Pain. 5%

-Adults frequently have pain.

-To diagnose tethered cord, an MRI of the lumbar spine is done. The following findings require surgery:
---If the conus (base of spinal cord) is below the L1-2 space and there are symptoms, the filum should be sectioned.
---If the conus is below the L1-2 space and there are accompanying changes, such as a fatty filum, bony deformities, skin abnormalities, or a terminal syrinx, the filum should be sectioned.
---If the conus is above the L1-2 space and there are symptoms AND accompanying changes, the filum should be sectioned.

*Since a section of the filum terminale surgery carries very little risk, the temptation is to abuse and overuse the operation. (This statement strongly implies that recent developments in treating tethered cord are unnecessary and irresponsible. He was ready for a fight.)

>So, that speech was a lot to hear. The room was quite silent. Dr. Oakes had spoken quite assertively.

Next, Dr. Arnold Menezes, from the University of Iowa, spoke about "Complex Chiari: To fuse or not to fuse".
>So exciting! I hope he wants to fuse me!!!

-Cranio-cervical junction fusions are only recommended for a small percentage of Chiari patients. Fusions leave the patient with a limited range of motion, and they carry a high risk for complications and ongoing problems.

-Cranio-cervical fusions are only indicated in 4 cases:
---A reducible bony abnormality, such as a retroflexed odontoid leading to basilar invagination. 25%
---A previous decompression surgery done from the front. 44%
---Occipital-Cervical instability. 26%
-----This is when there is a significant difference in the size of the tonsillar hernation between flexion and extension.
---Musculo-skeletal abnormality, such as EDS or Marfan's syndrome. 15%

-Symptoms of cranio-cervical instability are postural:
---Occipital headache
---No headache in the morning
---Worsened while upright and with exertion
---Dizziness and nausea when upright
---Feeling of impending crisis with exertion
---Relieved with head support and horizontal rest

-Treatment

---A properly-fitted brace is both diagnostic and therapeutic. If the brace relieves symptoms, this is diagnostic of instability.

---Types of fusions: Instrumented (Can be either rigid, with plates, screws and rods, or semi-rigid, with loops and cables.) Non-instrumented (Use of patients own bone to fuse.)

*Every fusion surgery requires bone placement for a successful bony fusion. Bone must fuse with bone ultimately. A brace needs to be worn for 3 months post-surgically to ensure a solid bone-to-bone fusion. Bone to metal fusions are not adequate. Either the bone will give out or the metal will.

---A good fusion should use limited instrumentation to preserve as much range of motion as possible. Usually, the skull needs to be fused to C2.

*Reiteration: There must be bone placement into the fusion for a successful osseous fusion.

>It was great to hear Dr. Menezes speak so assuredly about fusions. I even got to talk to him during the break, and he was so friendly! He looked at my nametag. Maybe he'll remember my name and decide to take my case. I would feel confident with my head in his hands.

Next, Dr. James McCarthy from U of W-Madison spoke about Scoliosis and how it relates to Syringomyelia. I'll include some abbreviated notes on this section, as it wasn't one of the most important sections.

-When Scoliosis is present with Syringomyelia, which includes 60% of SM patients, the syringomyelia should be treated first, as it often resolves the scoliosis.

-An exception is when the Scoliosis is larger than a 70 degree curve. This needs to be treated with a full spinal fusion.

Next came a question and answer session.
>Each of these sessions were quite painful to get through, since so many people presented their personal problems to the panel, which was explicitly not allowed.

The panel included the previous speakers and Dr. Kula, the neurologist at The Chiari Institute.
>There was a very definite clash between Dr. Menezes and Dr. Kula. It was quite tense at times.

-The first question (my question that was submitted online before the conference) was: What can be done to prevent the need for a fusion revision due to a sub-optimal cranio-cervical fusion?

Menezes: For a fusion to be optimal, bone should be used in the fusion, not just metal. It also should only be performed when indicated.

Kula: Fusions often fail due to a lack of adequate bony material in the area of C1 and C2. Osteoporosis and Vitamin D deficiency should be treated to ensure a bony fusion

-Question: Why are so many neurologists unaware of Chiari malformations?

Kula: There are often minimal neurological findings in patients with Chiari. Besides a downbeat nystagmus, which may or may not be present, the neurological exam is often perfectly normal. For this reason, neurologists don't see the need for treatment unless they are very well-informed.

Next, there was a break. After the break, we began module 4: pain.
>This section was not very helpful to me. I will only mention the few points that were made.

The first speaker was Dr. Miroslav Backonja, from the U of W-Madison pain clinic.

-In Syringomyelia, the pain is often neuropathic.

-Neuropathic pain is caused by diseases affecting the somato-sensory system (injury or lesions of the nerves).

-Hyperalgesia=an increased pain response to normal stimulus.

-Hyperpathia=an increased pain response to repeated stimuli.

-Most people have descending modulation, which makes it possible to ignore pain. People with chronic pain tend to have a loss of pain inhibition.

-A multi-modal and multi-disciplinary pain management clinic will approach both problems: Medications will be prescribed to decrease hyperexcitability of the nervous system, including hyperalgesia and hyperpathia. Cognitive-behavioral therapy will be used to help with pain modulation and develop coping skills.

>I'm not even going to include information from the next speaker, Dr. Angela Mailis-Gagnon, because I found her to be so unhelpful.

Next was the question and answer session on pain. The first question was about chronic opioid use.

-First, Dr. Backonja clarified that opioids are legally prescribed medications, and narcotics are illegally sold and taken drugs.

-Moderate use of opioids is often necessary in treating moderate to severe chronic pain. As long as the use is controlled and in moderate doses, it's generally safe.

-Unfortunately, some people are not helped with narcotics. Some people experience severe side effects. These people won't be able to tolerate narcotics.

Question: How to deal with persisting headache after decompression.

-The Alexander techniques for cervical alignment can alleviate headaches. Improving posture through physical therapy and lifestyle adaptations can help.

-Being in pain heightens the sensitivity of pain. It lowers the pain threshold and heightens pain awareness.

Next was the lunch break.

>I was very fortunate to be able to eat lunch with Laurie, a fellow Chiarian and blogger. It was great to meet her. I'll include a picture when I get around to loading it onto my computer.

Next was module 5: Clinical Research.

First, Dr. Paolo Bolognese presented on Intra-operative Ultrasound Techniques.

-Color Doppler Ultrasound is used intra-operatively in every operation done at The Chiari Institute, but nowhere else.

-Most decompression operations are done with only a B scan real time ultrasound. Color Doppler Ultrasound has three parts: B scan real time ultrasound, color coding, and pulse waved doppler. The pulse waved doppler measures the velocity and the flow of fluid.

-This technique was actually invented by Dr. Bolognese, and described its use in a paper published in 2003. Pulse waved doppler was originally used in cardio-vascular surgery, to measure the flow of blood through veins and arteries. Dr. B decided to try this to get a more accurate view of the flow of cerebro-spinal fluid flow intra-operatively.

>He showed pictures for comparison throughout the presentation. The differences were quite impressive.

-Color Doppler Ultrasound is used in every operation at The Chiari Institute, but nowhere else. This is because it is very, very expensive, difficult to use, and operator dependent. It requires a particular expertise.

*Using a B scan, you can only make assumptions about the flow. CDU is the only way to actually measure the flow.

>Here, Dr. B mentioned what a funny word "assume" is in the English language. hehe

-A B scan can show brisk movement of the structures, even if the flow is not really going anywhere, just back and forth. This kind of flow is called "stump flow".

-With CDU, you can measure the CSF flow before decompression, then make sure that the flow is improved after decompression by tailoring the operative technique to the individual.

-Even a CINE MRI, which is the gold standard for measuring CSF flow, is often inaccurate. Measurements of flow with CINE are affected by cardiac and respiratory variations. Everything that is not in sync with the heartbeat is lost.

-The only other doctor that has mastered the use of CDU and uses it regularly is Dr. Lu, in China.

-At TCI, they have been able to establish that in cases of Occult Tight Filum Terminale, the tension in the cord creates stagnant flow within the filum. This has been demonstrated using CDU in each operation.

>This was great for me to hear. It really validates the existence of OTFT, and the need to detether in these patients, despite what other doctors may say.

-So, CDU allows for much more insight into CSF dynamics. However, it is difficult to master and expensive to use. It provides anatomical and functional feedback throughout the operation, allowing for a personally tailored decompressive operation based on individual anatomy.

>We all know that each Chiari malformation is unique. This demonstrates how TCI is able to tailor the operation for the individual. They use more aggressive tonsillar shrinking often, because it makes more pathways for CSF flow. They determine whether or not to open the dura during this operation.

>In the last couple minutes, Dr. B honored Dr. Milhorat. He said that he was an incredible mentor and friend, and he was very sad to be operating without him. He looked so sad. It was very sweet. I was in tears. They were clearly very close. It's a shame that Dr. Milhorat couldn't be at the conference, because of recent controversy.

>I think Dr. B did a great job presenting on the topic. He was also quite the comedian. It was good to see him keeping his sense of humor. Unfortunately, he came in soon before his presentation and left soon after. I heard it was because his kids were alone in the hotel room at the time.

Next, Dr. Nicholas Wetjen spoke about outcomes after decompression for Syringomyelia. I don't really have anything to report on this section.

Dr. Frim, from the University of Chicago Hospital, spoke next. He discussed neuropsychological issues in Chiari and Syringomyelia patients.

-The most common neuropsychological problems in childhood are brain fog, school problems, and ADHD.

-Most Chiari 1 patients are broadly average both cognitively and emotionally. However, they tend to have specific deficits. these deficits include memory retrieval deficiency, low processing speed, reduced executive functioning, and mild cognitive impairment (memory type). The lowest scores overall were in verbal memory.

-This classifies most Chiari patients as learning desabled.

-Further study should be done to find out if decompression surgery helps with cognitive functioning. Dr. Frim is performing a study like this right now, testing neuropsychological functioning both pre-operatively and post-operatively.

-Cognitive rehabilitation could be done to help with Chiari-specific cognitive weaknesses.

Next, Dr. Brandon Rocque spoke about trends in surgeries. This was a pretty basic presentation discussing the differences in decompression surgeries.

The next speaker was Dr. Allison Ashley-Koch. She works at Duke, studying the genetics of Chiari. Her work is a continuation of the work of Dr. Marcy Speers, who passed away in 2007.

-There are multiple causes of Chiari, that may all have different genetic causes:

---Push down: Hydrocephalus
---Suck down: CSF leak causing hypotension
---Squeeze down: Reduced volume of the posterior fossa
---Shake down: Trauma causing acquired Chiari
---Pull down: Tethered cord

>The rest of her speech just said that there are some possible leads, but they haven't really figured anything out yet.

The next speech was on canine Chiari and Syringomyelia. Small breeds, especially but not exclusively the Cavalier King Charles Spaniel, are most susceptible.

Lastly, there was the final question and answer session.

-The only valid question was about the long-term prognosis for Chiari patients post-decompression.

Frim: We don't know, because it's still a fairly new surgery. We can only compare to similar surgeries that have more long-term evidence. For example, patients with tethered cord that are successfully de-tethered have early deterioration of spinal cord function. And patients with shunts have early cognitive problems that are usually associated with old-age. All of these patients have a more "condensed" life-span. We don't know if Chiari patients will have similar experiences.

>And we'll end with a joke. One panelist was explaining that there are many roads to Chiari. Dr. Frim mumbled, "There's construction on all of them."

Sorry it took me so long to post this. I've had a bit of a rough time since then, which I'll post about soon.

Knee problems

2009-07-15T13:58:00.005-05:00

This pictures shows the anatomy of the knee. By looking at it, I can guess at what's wrong with my knees. The LCL's must be very loose, causing the fibulas to dislocate...all the time. I'm sure that they are so loose that they're never really in the socket. The fibulas just slide around all the time. I wonder what kind of damage this is causing. It sure hurts a lot!!! It hurts all the time...not just when I walk, not just when I stand, not just when I move my legs around. It hurts when I drive, when I sit, when I rest, even when I lie down!!! That's how I know I need to do something about it. I hope it is something that can be helped. I've been wearing Ace bandages on my knees, and it seems to help a bit, but not enough. What if it's too late and the damage has already been done?

My shoulders and my hips also have this kind of hypermobility. It just doesn't cause too much pain, yet. I better watch out, because eventually, they will all be in trouble.

I also have a weird sensation in my calf. It feels like a bug is crawling around on my leg. Really annoying.

My other issue is having patches of skin sensitivity. Right now, it's an area on my left forearm. It doesn't look like anything is wrong, but if you touch it, at all, it hurts a lot!!!

Stupid body is falling apart. I'm so tired of being so sick. It's so hard get get through even the easiest days. I can't wait for surgery. I wonder when I'll hear from Dr. Menezes. I wonder what he'll say. I guess I'll keep busy with other doctors until then.

2009-07-14T15:42:00.002-05:00

The shortness of breath and knee pain have been worse lately. I need to figure out what kind of doctor to see for the pain in my knees. They hurt even when I am at rest. I'm wearing knee braces. They hurt all the time. I may see a rheumatologist. Who knows?

I'm still choking on pills pretty often. I really need to lie down to swallow anything.

I have a pretty bad spinal pain, too: pain around C-4 to C-5 (under my fusion), pain in the middle of my T-spine, and pain at L-4 (where I had my detethering).

I'm not sure if the chiropractic treatments are helping, but I'm still going to every treatment (3 days a week), even though it is a strain on my body to fit it into my schedule.

We're leaving on Thursday for Madison to attend the ASAP Conference on Friday. We chose to go to only one day, but we'll be seeing quite a few important doctors, including Dr. Batzdorf, Dr. Menezes, Dr. Bolognese, and Dr. Grim. Very cool. I'm just worried that it's so early in the morning. (It starts at 8:30 am.) Yikes! I've been sleeping 'til noon lately. And I never get dressed until the afternoon. We'll see how it goes.

I'm exhausted and hoping to be able to make it to work today.

Been a while

2009-07-07T15:43:00.002-05:00

Gus has nagged me for not writing in my blog lately. I know it's been a while. I've been distracted by my word game that I'm addicted to.

I've seen Dr. Don, the chiropractor, a few times. Financially, he's been extremely generous. He'll be getting very little pay for my course of treatment. He's recommended 24 treatments in 12 weeks. He did some x-rays. They showed that my scoliosis in my thoracic spine, which has always been considered mild, has progressed to "significant". It looks really bad on the x-ray, although I don't know the degree of the curve. I just know that it looks horrible. It curves to the right, quite a bit, and I seem to lean to the left, perhaps to compensate, in each picture. Shoot. Let's hope that it doesn't continue to progress. Although, with Ehlers-Danlos, most things are progressive, since the ligaments wear out more and more over time.

The chiropractor is doing two kinds of treatment: electro-stimulation of acupuncture points in the ear. It hurts a bit when he does it. He also uses acupressure on neuro-reflex points on my head. Nothing jarring. Nothing cracking. Nothing forceful. And it seems to decrease my head pain, at least a little bit. I really like Dr. Don, so I'm going to go through with the whole treatment.

Another example of the EDS worsening is my knees. They've become very painful. I need to seek treatment for this soon. Otherwise I won't be walking too much longer.

I haven't sent my package to Dr. Menezes yet, but it really is together and ready to go now, so I'll get it out tomorrow. I'm also sending out a request for the surgical report that was missing in the package that was sent to me.

My mom was out of town for the 4th of July weekend. I wasn't well much of the time at all. I participated in celebrations on the 3rd of July. That night, my body crashed. I couldn't get out of bed for the next few days.

One week ago, I passed out while driving to work. I just barely was able to pull over onto a small shoulder to wait to be rescued. Today, I'm feeling really tired and out of it, so I'm a bit worried that it will happen again tonight. I'm doing what I can to prevent it. Lots of Provigil. We'll see if it helps.

I'm so very tired and my knees are killing me.

That's all for now.

Bad Day

2009-06-29T19:42:00.005-05:00

Well, it's been a week since I've last written. Is that a good sign or a bad sign? Neither, I guess. I might have been a little busier than normal. We had our Chiari meeting, but it was a very small (but friendly) group. My Uncle Jim is in town, which is very exciting. I met with a chiropractor last week. He's giving me a free initial evaluation and formulating a treatment plan. Don't worry, the plan involves absolutely NO adjustments of the spine. Not smart with Chiari, EDS, or Osteoporosis. He does want to try some energy healing. I don't believe in it too much, but he feels that he can reduce my body's pain reception, so I don't feel as much of this pain. Who am I to turn down the offer?

I'm also eagerly waiting for my appointment with Dr. Wilkin, the neurologist and pain specialist, on July 20...that was the earliest appointment there was.

I've been using a Thera Cane for trigger-point therapy. I like it. I'm glad I have the option of giving myself a massage, essentially.

I received the long-awaited packet of surgical reports from the hospital in New York. Unfortunately, they sent tons of papers, but none from my cranio-cervical fusion, even though I explicitly requested it. I'll have to start the long process of requesting those particular pages again.

I don't think I'll wait anymore to send the package to Dr. Menezes. I can send him the surgical report later. I just don't want to wait anymore, so I'll be getting the package together this week.

Today was a day off, because work is very slow this summer. But I would've had to miss work anyway, because it's been a VERY sick day. I haven't been able to get off the couch at all, except for bathroom trips. Otherwise, I'm totally stuck here. Even lying down, it's hard to get comfortable after being here all day. I've been nauseated, tired, out-of-it, crabby, and in lots of pain. I had a low BP spell where I was barely conscious and not able to move. My joints hurt, just laying around on the couch...my knees and hips hurt. And I have an ice pick over my right eye, in addition to all over head pain. It's hopefully peaking right now, at an 8 or 9. I haven't been able to eat much, and I have the feeling of a lump in my throat that makes me want to gag constantly. That also makes it feel like I can't breathe.

I want to buy a cane that turns into a seat, since my walker doesn't always fit in places, like restaurants. I've just got to decide which one to get, and wait a while, since I'm not allowed to buy anything else this month. There's a few on amazon.com for around $20.

In my free, bored time, I've found something really fun to do. I signed up for a free 7-day membership of lumosity.com. It has some really fun games to train your brain! I love it! I don't know what I'll do when my membership runs out. It's so addictive! I love all the different games.

Okay, back to dealing with overwhelming pain and sickness.

Heat

2009-06-22T21:32:00.002-05:00

The heat was killing me today. Going into my car is complete torture. Wearing the collar in the car makes it even harder to stop sweating. I just get overheated so easily. And then I went inside a building, then back out to the car several times. Those changes in temperature really cause complete exhaustion. I barely made it home. The drive home was a little rough, cause I was really losing alertness. Since then, my headache has just been building and building. And my body hasn't been able to reach a comfortable temperature since then.

This weekend was fun, but exhausting. On Saturday, we went to a Hawaiian restaurant and show to celebrate my grandparents' 55th wedding anniversary. On Sunday, we went to dinner for Father's Day. It was a nice weekend. I'm just all out of energy to type about it. The words are not coming to me today.

Home alone

2009-06-19T20:18:00.007-05:00

I'm home alone on a Friday night, and I'm actually fine with it. I think that's a good sign for my self esteem. Although it might just mean that I'm becoming agoraphobic, due to my dislike of crowds, my fatigue, and my need to be horizontal most of the time. Nonetheless, I'm sure in a less secure time in my life, or when I'm feeling sad about my life, I wouldn't be happy to have a night to myself.

It was a rough, fatigued day, because it was hot and humid, and our air conditioning didn't seem to be cooling the house very well. It sounded like it was running, but nothing was actually coming out of the big vents in the hall.

I'm officially not allowed to spend any more money for a long time. The only big thing I've bought this month is my gps device, the delivery of which I'm anxiously awaiting. I think it was a good purchase, because it comes with traffic updates with no ongoing fees. That should really help me in planning my route up to Milwaukee each week starting in August! I know that traffic will be key to making the drive short enough to survive. So, it wasn't a bad purchase. But add that to a few medications, new contacts, and a few "non-essential" purchases, and you get one scary credit-card bill!

Well, I missed dinner tonight, so I'm going to go eat a baby spinach salad. I'm not sure what else I can eat right now, only because I'm pretty nauseated.

I would love a massage tonight. I ordered the thera cane, so soon, I'll be able to see if I'm able to give myself some trigger point therapy. With the crazy rock-hard muscle spasms in my neck and shoulders and lower back, it seems like they must be contributing to this monstrous headache. My head is seriously killing me tonight...as always. But my spine is really painful tonight, too, in quite a few places. My knees are also killing me. I stood up at a store for about 20 minutes, which was a pretty stupid idea. My body is hurting all over now. But it's nice to be home and out of the rain.

I have some news: I have actually agreed to visit my cousin's chiropractor. But before you freak out, please know that I am completely against having anything in my spine cracked. No adjustments! I agreed to go with him, because he actually provides other treatments, including trigger point therapy, electric muscle stimulation, and physical rehab. However, I'm only go to meet him and see what treatment he would offer and how much it would cost. It's important to note that none of this treatment would be covered by insurance, so I probably won't be able to do anything.

Maybe the best idea would be to get a prescription for trigger point massage, which my massage therapist can't do without a specific prescription. But if she does it, it WILL be covered by insurance, completely.

Okay, need to eat. I also hope to go to sleep early tonight so I can get my schedule back on track.

The next Very Important Person: my cousin Laura

2009-06-18T16:10:00.006-05:00

I've gotten very behind on my tributes to the very important people in my life. There are still so many to mention, but the person of the day is my cousin Laura.

My dad and Laura's mom are siblings. She is my only in-state, female cousin. And lucky for me, she's just a year and a half older than me, and we grew up just a couple towns away from each other. As kids, we would have sleep-overs pretty often. She played with Barbie's, and I played with American Girl dolls. She liked cats, and I liked dogs. (I remember there being debates along the lines of: "Cats rule! Dogs Drool!") And she liked to sleep in, while I was crazy enough to wake up at 6:00 in the morning. (Which is funny, because now she wakes up every day for a real job, while I stay in my pajamas until 3 in the afternoon.) But we made it through those drastic differences (hyperbole). In high school, we spent more time together. She even introduced me to my high school boyfriend of 2 1/2 years, Bobby.

I believe it was the summer after my junior year of high school that Laura developed a spontaneous spinal cord leak, causing intracranial hypotension and a temporary acquired chiari malformation. She was in the hospital for weeks. The doctors tested her for everything, and they never figured out what was wrong. They did attempt a spinal tap multiple times, only to find that she had zero pressure in her spinal cord. She suffered from low-pressure symptoms for quite a while. For Laura, going to a chiropractor actually helped her symptoms to resolve, thank God.

So, when I received my diagnosis a year later, Laura was the first person I called. When I had my first spinal tap, Laura was the first person I called. Even though she suffered during an acute period of illness, I know that she truly understands what it feels like to be this miserable. This year, she will be walking in the Chiari Walk, and she has been amazing at helping to get the word out.

Laura is the closest thing to a sister that I have in this life (although I have two amazing brothers and a sister-in-law that will have to be mentioned in a later post). I know that we will be close for our whole lives. I am so grateful for her constant support and compassion. And considering that I had to steal this picture from her facebook page, we definitely haven't spent enough time together lately. I hope I don't take her for granted. And yes, this could be seen as my passive-aggressive way of apologizing for dismissing the chiropractor recommendation so quickly. I know you just want what's best for me, and I think you are amazing. Love you cuz!

Symptom Watch and Alternative Treatments

2009-06-17T22:02:00.006-05:00

My head is killing me. It gets blurry at the top of the pain scale, but I'll call this headache an 8.
My heart and breathing have been bad on and off.
I actually don't have a stomach ache today (which leads me to be more confused about whether or not I should see a GI doctor, since my symptoms are intermittent).
My knees have been really sore. The fibulas have been popping out often, which is probably the cause of the pain.
My muscles are insanely tight. I have large "marbles" of muscle throughout my neck, shoulders, and lower back.
The fatigue has been keeping me in bed for 12 hours nightly, and then on the couch for at least another 6 hours EVERY DAY!
For the hours that I am upright, I have to wear my neck collar all the time. It is the only thing that keeps the symptoms from becoming unbearable, most of the time. If I had a CTO that fit or a traction device that was made for my size, it might also help with symptom alleviation.
I have difficulty sleeping at night. I have trouble falling asleep. Then I wake up after about 4 hours, and I'm wide awake. I cannot fall back asleep by any method other than medication. My whole life, I was successful using progressive muscle relaxation to fall asleep. But that doesn't work for this kind of awake. I feel unnaturally wide awake...especially knowing how much sleep my body requires.
And my head pain is horrible! Did I mention that? So much pain. It hurts through the back of my head, my temples, the top of my head, my forehead, behind my eyes, my cheeks, and my jaw. So, pretty much everywhere.

I wanted to share a few articles about chiropractic care and Chiari. I will also include articles about cranio-sacral therapy and Chiari. Chiari is a contraindication for both of these types of treatment. Both treatments can actually make the symptoms of Chiari worse. I believe that most practitioners mean well when they use these therapies to try to help Chiari patients. They simply are not adequately trained or equipped to improve the situation. And the treatments they have to offer are not safe for those with fragile cranio-vertebral junctions and CSF pressure sensitivity.

http://www.chirobase.org/17QA/chiari.html

http://www.pressenter.com/~chip/chiro-nono.htm

http://jnnp.bmj.com/cgi/content/extract/70/6/816

http://www.pressenter.com/~wacma/chiro.htm

http://www.quackwatch.com/01QuackeryRelatedTopics/chirostroke.html

http://www.quackwatch.com/01QuackeryRelatedTopics/cranial.html

http://www.massagetoday.com/mpacms/mt/article.php?id=13387

However, massage therapy can be helpful. I do get massages, and they do provide some amount of relief. It is just very temporary relief. I would probably experience less pain if I had daily massages, but obviously, that isn't a feasible option. I did read about something that has helped a fellow Chiarian to relieve the terrible knots of muscle. It is called a Thera Cane. It is a tool that maximizes leverage to allow for beneficial self-massage of "hard, knotted trigger-points". I'm considering buying it, although I just feel that I can't spend any money right now. (I recently bought a GPS for my car, some more salt pills, and new daily contacts. My bank account is a bit depleted.)

Also, the neurologist that I am going to see next month is part of a group called "Integrative Pain Medicine". They believe in the use of both medical and holistic treatments for the management of chronic pain conditions. I hope they can come up with something to help me survive until surgery.

I'm also concerned (although this is all based on hearsay). I've read about multiple patients now that were scheduled for Cranio-Cervical Fusion surgery, and upon completing osteoporosis treatment, it was found that surgery is no longer an option, due to some amount of bony fusion between C-1 and the skull having formed. The problem: whatever kind of fusion is forming in these patients, it is not fusing them in the needed extracted position. Only surgery could do that. Something seems to have gone awry at TCI. (Again, keep in mind that I don't have any real evidence of this.) Dr. B doesn't seem like himself. He is no longer promoting surgeries for which he used to advocate so strongly. I truly hope this doesn't happen to me. If anything, maybe my conservative treatment for osteopenia will prevent any improper bony fusion.

I just have my whole future wrapped up in this surgery. I can't move into the next stage in my life until I have this surgery. I just can't wait. I don't know how to wait any longer. Not when my body is torturing me daily.

I've been watching "Mystery Diagnosis" daily since it became summer rerun season. I relate so much with the suffering patients; but when they are diagnosed and treated, I get jealous. These people are very unfortunate in the medical world, because they've gone so long without diagnosis. But on this show, most patients return to their normal life after treatment. It just seems so unfair that with my conditions, people suffer endlessly. So many are enduring so much pain right now. Please keep my Chiari friends in mind, including Kristen, Hannah, and Hannah!

Symptoms continue...

2009-06-16T21:48:00.002-05:00

So, I haven't slept well for a while. I'm not sure what to do about it.

I'm also a little concerned about seeing a new doctor. I have to figure out what kind of information to bring to him about my multiple conditions and symptoms. He'll have to get a complete symptom list, to say the least.

Tonight, I'm afraid that I won't be able to fall asleep again. And even when I do, I know I won't stay asleep.

My muscles are so tense. There are marbles throughout my neck, shoulders, and back. I could use some mega deep-tissue massage. I'm trying to stretch and do some self-massage, but it is so freaking tight!

And my headache is terrible. Really so painful.

Symptom Watch

2009-06-16T16:32:00.003-05:00

Sunday: We had visitors to our apartment during the day. It was really fun! I saw my friend Heather. We had a great time catching up, and she brought me books to borrow! Then we had Julie and Claire over for dinner. Gus cooked. I actually stayed awake all day.

I've been paying for it since then. Sunday night, I did not sleep well at all. Monday, during the day, I slept most of the time. I accomplished essentially nothing.

Monday night, I had a lot of trouble sleeping again during the overnight hours. So, I couldn't get out of bed until noon today. And I'm still tired now, and I haven't been able to get up. But I'm off to the bath to get ready for work, no matter how hard it is.

I did get an appointment scheduled with Dr. Wilkin, a neurologist in a group called "Integrative Pain Medicine". Hopefully he'll have something new to try.

I also contacted the hospital in New York, and they said that they are in the process of collecting my surgical reports.

Lastly, I still need to consider a GI doctor, for my confusing digestive problems.

Off to the bath.

Enough

2009-06-13T13:13:00.003-05:00

Okay. My pain has been at a consistently high level for too long. I worry most about how it is affecting my loved ones and our relationships. I'm not the person I want to be. And I know how tiring it can be to see me sick and in pain all the time.

So, even though I've seen multiple pain doctors in the past, and they have all run out of ideas for me, I'm going to try again. I have had a couple of recommendations. One is a neurologist in Schaumburg that has experience with Chiari patients, and is apparently not afraid of pain medication. (Dr. Lawrence Wilkin) The other is a pain management specialist in Morton Grove that deals with pain related to spinal fusions quite aggressively. (Dr. Howard Konowitz)

I'm not sure how to decide which doctor to try first.

I also need to arrange an appointment with a gastroenterologist, even though my symptoms are very intermittent and unpredictable, so probably hard to diagnose.

I just want to be a better person. And maybe I could feel even a tiny bit better while I'm at it.

Irritable

2009-06-12T12:17:00.004-05:00

So, unfortunately, yesterday was another bad day. Most of the day, I was just so low on energy that I was a bit like an angry zombie. It was another bad mood day, and again, I was really irritable toward my poor mom and boyfriend. I wish I could be in a good mood all the time, or at least more of the time. But I just don’t seem to be handling my symptoms very well lately. It’s so overwhelming. And it can make me mean. I know it is wearing on them, as even the most patient people have their limits. And they are just as frustrated with my worsening symptoms as I am.

I’m incredibly frustrated by the postponement of my surgery for another 6+ months. I’m even more concerned about my friend Hannah. She had her fusion revision scheduled for next week. Drs. Milhorat and Bolognese agreed that she needed the surgery. But apparently, the newly-appointed director of The Chiari Institute informed her that her surgery was cancelled, because it wouldn’t help her, so it was unnecessary. WHAT? I have no idea who this guy is, where he came from, or if he knows what he’s talking about. But how does he have the power to overrule a decision made by the most experienced doctors there? I don’t have any more information about this situation, but I will post if I learn more.

This certainly makes me wonder if having my fusion revision at The Chiari Institute will even remain a possibility for me. If her surgery is now considered "unnecessary", how do we know that my surgery won't also be deemed "unnecessary"? I hope that Dr. Bolognese will somehow be able to rectify the situation. Really, he needs to be promoted to the director position. He has earned that position with his experience. He needs a new partner with Chiari experience to help him out with the overwhelming number of surgeries that need to be done, but he should be in charge. I don’t see how any doctor coming in from the outside would be able to fill Dr. M’s position. Dr. B is the only one with enough experience to be making the major decisions.

In any case, I am relieved that I am in the process of contacting Dr. Menezes. I have everything together except for the surgical reports that I haven’t yet received from the hospital in New York. On Monday, I’m going to have to call the hospital and see if it is in process. Hopefully, that will be able to be sent out soon. I’d really like to know if I will have Dr. Menezes as an option for this surgery. It would be reassuring at this point, even though he’s never seen the inside of my head, which makes me uncomfortable.

Last night was another night when my body made it very clear how much I need this surgery. On the way home from work, I developed terrible stomach cramps. The pain was so intense that I couldn’t breathe normally. I went ahead and ate a bit of my Subway sandwich, after taking a peppermint oil capsule and a digestive enzyme. That pain actually eased up, for once. Unfortunately, it was immediately replaced with an “exploding head”. I had the headache all day, but it really worsened in the evening. The pain was unbearable for several hours, during which I turned into an irritable, cranky, PMS-ing witch.

Manual traction helped (lifting my head with my hands), so I tried real traction. First, I tried the Pronex. Unfortunately, it not only squeezes my ears uncomfortably; it also seems to press right against the rods in my skull. It just hurt too much for me to feel any relief. Then, I tried the inflatable traction. Neither of these fit me quite right, because my neck is smaller than the recommended sizes for each device. When I was sitting up, the inflatable traction caused too much jaw pain. (Does this sound a little like Goldilocks to you?) Then, I tried it laying flat. Unfortunately, in that position, the back of the device presses into the hole in my skull, which blocks off the flow of cerebro-spinal fluid, causing a pressure build-up. This is due to the fact that my craniectomy area is rather large, and only covered with skin. A titanium mesh plate would normally be inserted to cover the area, but there wasn’t room for it. So it was determined that it would be safe to leave it uncovered. It’s mostly okay, but at times, there are problems with having an area of your brain exposed. For example, if you just tap the area with your finger, it sounds like you are tapping a microphone inside my skull. Weird...

Eventually, I went ahead and took a Vicodin and an Ambien last night. The Vicodin doesn't actually relieve the pain, but it does somehow make it more bearable. It's hard to explain, but it's the truth. And the Ambien failed. I was awake for at least two hours before I fell asleep. Wonderful.

Today, my headache is not as bad as last night, but not much better, either. I'd say last night I reached a 9, and this morning I'm around a 7. Thankfully, I don't have anything that needs to get done today. So, I'll just lie here, watching the time pass me by. I do wish I could make more out of this life. But at this point, all I can do is try to endure it, which has been my approach to much of my life. Learn to cope. I've been learning since I first developed symptoms in junior high. So many days have been lost to pain. So many opportunities have been missed to sickness. This hasn't just been one sick year of my life. After all this time, it IS my life. Every day that I lie in bed, I feel that I am wasting precious years of my life. I've learned that each day is something to survive. Every day presents a new battle to fight. But there is no point in grieving over the lost years. Only acceptance is meaningful. I look forward to a time when I'll be able to live more fully.

So, here’s to hoping for a better weekend, for my mom’s and boyfriend’s sake. I also hope that my Chiari friends have a better weekend; especially Hannah, whose surgery has been postponed indefinitely (I am so saddened for you, Hannah.), and Kristen, who’s symptoms of cranial settling are worsening, just like mine (I'm sorry you have to feel this way, too!).

Profound fatigue

2009-06-08T14:34:00.003-05:00

Yesterday, I had debilitating fatigue all day. I was forced to sleep or rest the entire day (even on 200 mg Provigil). It is so frustrating. But I am very grateful that the headache intensity has dialed back down to the usual level. I slept for 12 hours last night, and the fatigue is back today. I have to find a way to take a bath and go to teach a lesson. Searching for any ounce of energy in my weighed-down body.

I am so honored by the number of people interested in the Chiari Walk this year. The walk will be September 26th at 31 sites across the country, including one in Palatine, Illinois. I'll include the information here for anyone else interested:

The national walk information page: http://www.conquerchiari.org/walk%2009/Walk%2009%20General%20Info.htm

List of walk sites: http://www.conquerchiari.org/walk%2009/Walk%2009%20List%20of%20Sites.htm

Printable flyer for the Palatine, Illinois walk: http://www.conquerchiari.org/walk%2009/flyers%2009/PALATINE-IL%20FLYER.pdf

Walker registration form: https://www.conquerchiari.org/ccwaa09/ccwaa_register.asp
*If you are registering for the Palatine walk, you can choose to join my team, which is called "Courageous Carolyn".

Thank you so much to my friend Jennifer for creating a team in my honor.

Torture headache...and late-night thoughts

2009-06-07T00:26:00.003-05:00

Hasn't let up all day. Medication barely touches it, but I have to try something. The inflatable traction helps a bit, but only while my head is lifted pretty severely, to the point that my jaw is tightly clenched. I had Gus do manual traction, and all of my pain went away. But that's very temporary, too. And as soon as he lets go, I feel like I am being crushed. At least I know that re-fusing me in the right place will take away all of this pain. I just have to find a way to survive. Today was hell. So, so much pain. I doubt that I will be able to sleep much. Maybe a sleeping pill combined with a pain pill will give me some tiny break from this agony.

The manual traction really shows me how much pain I am living with. There is a constant heavy weight on top of my head, just crushing me. There's a feeling of sickness inside my core that goes away with my head in the right position. There's pain through my face and a feeling of pressure throughout my skull that goes away. I can't imagine what my life would be like if all of these symptoms that are such a constant part of my life could be relieved. I have an idea what it would feel like. Manual traction gives me a glimpse of having a totally different body. It feels like anything would be possible if I could just get fused in that perfect spot. Of course, there is always the fear that it won't work...the fear that I will be just another example of a failed fusion surgery. I know that surgery failing is a possibility, and that I may have this pain for a lifetime. But I have to believe that there is a chance that it will work...a chance that I will be different. Without that hope, without that light at the end of the tunnel, there's no reason for me to persist. I just don't know how to go on living like this. How to continue working??? Functioning? Getting out of bed, ever?

How to not cry every moment of my life, knowing how much I've missed out on, how much I will miss out on. Knowing that I'll never know what my life could have been; who I could have become. Knowing that I will not be able to have my own child...to pass on a part of myself and create a new person. That's a very big deal to me, considering how much I am like my mom...how important she is in my life. Knowing that the possibility of having a child of my own doesn't exist. My broken body doesn't have enough to offer. It wouldn't be able to survive pregnancy. And even if it could, my diseased genes should not be passed on. Who knows if I will ever have what it takes to care for any child, even if it is not biologically mine? How empty would my life be? I love my dogs more than most people, but that isn't the kind of fulfillment I need from my life. I need a bigger meaning for my life. Someone to teach the things that I'm learning. But it isn't a dream of mine to teach my own child how to deal with these horrible illnesses. I won't pass this evil on. This disease must be stopped. And the best way I can think of is to not pass on my genes.

I do have hope that the next surgery will do it. I just have to figure out how to get there...how to survive that long, how to make my future happen. How do I face a single more day like this? I don't want to wake up. I want to go to sleep...the kind of sleep where you can get far enough away from your pain...where you get a break. But I don't want to wake up anymore. Too much pain. Too much straining of my body, watching it deteriorate and waste away. It gets harder and harder to do anything, as my body gets more and more out of shape, my muscles waste away, and my veins get worse and worse at pumping my blood back to my brain. I don't know how to keep trying. And yet, I really have no alternative. It's not courage. It's that I wasn't given a choice in this matter. I used to think death was a reasonable alternative, but I now understand that it's not an option. It doesn't even feel fair at times. I am trapped in this life...in this damaged body. Shouldn't I be allowed to choose death? But no, I have too many people to live for. I can't abandon them. It feels like an obligation at times...like I'm being forced to keep living this life. But I am not suicidal anymore. I promise you that. I'm simply stating that I do not have any option but to continue living, to keep trying, no matter how terribly hard it gets. But that is not bravery on my part. It's simply having no way out of my situation.

I am incredibly grateful to not be alone in this constant, daily battle. Without the unfailing support of my loved ones, I would have given up many times over. In that way, I am the luckiest girl in the world. I am truly blessed. I hope to continue to share about my closest supporters in future posts. I am also grateful to have a place to release my negative, depressing thoughts. It helps to keep me sane. I am humbled that anyone reads this, and even more humbled that people are not scared away by my true feelings.

Crazy headache

2009-06-06T16:26:00.003-05:00

I woke up with this headache, and it is insane. I've been incapacitated all day. I have trouble choosing pain numbers, but I feel like this is about an 8. And I've had it since I woke up this morning. If I had this headache all the time, I would never leave the house. I'm very grateful that it isn't always this intense. Please fix me already!!! Whatever you have to do. I'll take it. Just fix me now!

Here's a link to a bio on Dr. Menezes, showing how well-respected and prolific he is in the medical world:
http://www.neurosurgeon.org/history/honoredGuests/bio.asp?ID=54

Bad symptoms

2009-06-03T16:39:00.002-05:00

My head is exploding. It hurts all over. Even through my face. The pain is astoundingly intense. It really does feel like pressure pressing out around my skull: top of head, temples, and cheekbones are the worst. I also have pain throughout my body, too. The fatigue is very strong today, and the nausea is ever-present, as well. But, it is time for work. Luckily, only 2 lessons. I saw the psychiatrist today, which was altogether banal.

If only I could be having my head cut open and re-set today. Please????? I'll beg.

That's when you know you're ready for surgery: when you know what type of hell the surgery brings, and you'll do anything to get it. Then, you know it will be worth it to you. If your life is already so miserable that this outrageous surgery sounds better, then surgery is the right choice.

Depression continues, due to continuing symptoms and surgery rejection.

Good news: Gus and I put down a deposit on a new apartment, in Milwaukee. I haven't seen it yet, but I'm sure he chose the right place. It's tiny, but it has an underground garage for parking, an elevator, and central air!

Life goes on, whether or not my body is ready for it.

Symptom watch

2009-06-02T23:50:00.002-05:00

Post nasal drip still making me cough. The coughing is driving me crazy. If my throat would just clear, I'd be fine. Until then, my chest hurts from the deep coughing. And the pressure in my head just can't take any more of it.

I have a low grade fever (99, because my "normal" is around 97). I also have my regular body aches back, but in addition, I feel bruised. My upper arms, my outer thighs, around my ribcage, and neck/throat are affected. I'm just really tender to the touch. I don't know if this is part of the mystery virus or just a random symptom. I'm done with all of my prescriptions, so I'm trying to just wait it out. Unfortunately, my body is not great at healing.

I've been in a bad mood today. I'm upset about my surgery, definitely. But today was just a bad mood day. Those days just happen. I just acknowledged the mood, and tried not to take it out on anyone.

People have trouble understanding my need for the walker, but it is simply an assistive device to help with vertigo and sudden loss of balance. No, my legs are not too weak to walk, and I do not plan on making them any weaker. I just want to stop falling down everywhere and clinging to walls.

Why the delay?

2009-06-02T13:56:00.004-05:00

Post nasal drip is giving me the feeling that I need to cough constantly. So my head is still killing me from the excessive coughing. Ugh.

In March 2008, I was diagnosed with mild osteopenia. I was told it would take about 12 months of treatment to prepare my bones for revision surgery. So, I went to an endocrinologist, seeking treatment for my weakening bones. Unfortunately, this endocrinologist thought that I was crazy, asking for strong medications for having bones that were simply on the low side of normal density. After one appointment, this doctor refused to see me ever again or treat me.

In June 2008, I had my de-tethering surgery, which just felt like a stupid stop along the way to recovery. So, I made it through that surgery, and as soon as physically possible, I was out seeking treatment for my bones again. I was even more convinced of the necessity of the surgery after my Invasive Cervical Traction. In this procedure, they lifted my skull off my spine, and showed that many of my symptoms will be instantly relieved. Unfortunately, it wasn't until December of 2008 that I found a doctor willing to treat my osteopenia.

Even then, my doctor is using "conservative" methods to treat my bones. So, it's not certain whether or not my bones will be ready in time for my next DEXA scan, in 2010.

So, that is the reason for the delay in my revision surgery, which was supposed to take place this summer (like, right now). Instead, it's much more likely that I will have to wait a whole extra year until surgery. I'll aim for spring 2010, if possible. It's just hard to imagine being sick so much longer than I need to. It's also hard to imagine that I could be getting this surgery over with right now, but instead, I will have to anxiously await surgery for quite a bit longer.

Answer to the question: Blame the endocrinologist.

Bronchitis and Lots of News

2009-06-01T21:29:00.005-05:00

So, I'm still working things out. Today is the first day that I've had no fever. But I have a nagging cough that is very deep in my chest. So, it seems that it has turned into bronchitis now. I don't have any more prescriptions to deal with it, so I'm sticking with maximum strength Mucinex and maximum strength Robitussin. They don't do much. I just have to hope that it gets better soon.

As far as my other symptoms go, the coughing is still making my headache worse. The pressure is pretty bad. I could use some traction to relieve the head pain. I also need a massage, since I missed it last week. I've also been very tired all day. Very, very tired. I slept 14 hours last night, and I feel like I could do it again right now. I used my walker for the first time today, and it really helped me deal with the balance problems. So, I'm a big fan of the walker.

I'm a bit concerned about summer work, because my schedule is not going to be regular, and I won't have regular income.

After sending a second email, I did hear back from Dr. B, through June. Unfortunately, Dr. B says that my bone quality is not yet ready for a revision surgery. He blames this on my endocrinologist avoiding the use of Forteo, which is the most effective bone-builder, according to some. He says that waiting until 2010 for my next DEXA scan should be fine. Probably, the longer I wait, the better the chance of having dense enough bones show up in the scan. So this means, NO SURGERY THIS YEAR. I'm just going to have to get used to that, I guess. Have to adjust to staying sick a lot longer than I'd hoped.

There's a really interesting article I found on POTS. It's pretty good at describing the lifestyle:
http://www.bendbulletin.com/apps/pbcs.dll/article?AID=%2F20090531%2FNEWS0107%2F905310408%2F1041&nav_category

Here's an interesting article on the reasons why Chiari surgeries fail:
http://www.conquerchiari.org/subs%20only/Volume%205/Issue%205(4)/Symposium%20Failed%20Surgery%205(4).asp

The most exciting news: There is a Conquer Chiari Walk Across America site in Palatine again this year. The walk will be on Saturday, September 26th. All the information can be found here: http://www.conquerchiari.org/walk%2009/Walk%2009%20General%20Info.htm

Yay for Prednisone

2009-05-30T18:16:00.002-05:00

I never thought I'd say it, but the steroid seems to be helping, at least a little bit. And I haven't had any of the weird mood side effects I used to get. I'm still glad it's only a three-day course.

The Zithromax normally works quickly, and it was having no effect, so it probably isn't a bacteria, but a virus...not an influenza virus, but a mystery virus.

I still have a fever today, and my cough got significantly worse again last night and this morning. I've also had complete nasal congestion, with no air going through my nostrils. Luckily, Afrin nasal spray helps clear that up and get rid of the sinus headache. But you can only use it for three days, so hopefully I'll only have one more day of sinus headaches.

My body just doesn't react well to being sick. But I feel like I am just slightly getting better, like I'm on the upswing. Hopefully, I'm right. I'm still stuck in bed today, but the body aches are much less. That's the best part. I've slept a little less today, but still needed rest. My cough is still horrible. It sounds like it's deeper now, but at least it's more productive. I've gone through so much water today, so my body must be trying to flush my system. It helps that my throat isn't so sore today.

Thanks for checking in. I hope to be ready to go back to work on Monday. So much for a week off.

Getting worse, not better

2009-05-29T19:35:00.002-05:00

The flu swab came back negative, but since I have been continuing to get worse, the doctor prescribed a 3-day course of Prednisone. I was hesitant to take it, so I didn't start it yesterday. But when I woke up with an even higher fever today, and every day getting worse, I decided to start the Prednisone. It's known to have bad side effects, particularly for me, but I'm desperate.

I slept almost all day today. I thought I was feeling a bit better after my last nap, but my fever is back along with most of my symptoms. The only thing that is a little better is the body aches, but the fever is worse than ever. Ugh. And it's Friday night, so I have to start feeling better. I won't be able to see a doctor all weekend. It's definitely time to start getting better.

FLU

2009-05-28T13:11:00.004-05:00

I am very sick. It was just a cough until Monday afternoon, when the fever and all-over body aches came on. It became painful to move or be touched in any way. By Tuesday night, I could barely breathe, I was coughing so much. The cough is a very deep, "croupy" cough. Wednesday, I went to see my doctor. He agreed that I have the flu. He prescribed an antibiotic and Tessalon pearls for cough. He also did a nose swab to test for Influenza A. If it comes back positive, he will prescribe Relenza to treat the flu.

Dr. Shah said that he has seen a lot of swine (H1N1) flu cases recently, and that it seems likely that I have it. However, the only way to confirm H1N1 flu is to be hospitalized, and wait in isolation while the test is sent to the CDC. This option was not offered to me, as it is unnecessary. H1N1 is a strain of Influenza A, and it can be treated with Tamiflu or Relenza. However, Dr. Shah uses Relenza, because it also treats B strains of the virus.

So, for today, I am waiting for my flu test result, to find out if I need Relenza. Apparently, it is an inhaler, so it won't cause digestive distress (yay!). Many people will never know if they had the so-called swine flu, because it isn't tested for most of the time. It's also only treated if it is a severe case, or if the person is at-risk for any reason. That is why I would need treatment if I the test comes back positive. My system is weak and not good at fighting off viruses. I'm continuing to get worse at this point.

My worst symptoms today are fever, cough, fatigue, body aches, and weakness. The cough has caused deep chest pain and a very sore throat. Dr. Shah said that my throat is really irritated (and that was after only one day of coughing). I also have a few sores in my mouth and throat. I'm not sure if these are related or not. All of this coughing has made my headache a lot worse, too. The assault on my body is also making my autonomic system act up: tachycardia, loss of balance, and vertigo. I'm stuck in bed, and no one is allowed to touch me, as it hurts so much to move.

I'm pretty miserable, but I understand that this will pass soon enough.

Support Group/Updates

2009-05-26T16:28:00.002-05:00

There is a flu that has been going around my family. First my younger brother, then my boyfriend, now me. So, I've got a sore throat, annoying cough, and some extra body aches. The cough is the worst, because it increases my headache a lot.

Also, I've decided to have another support group meeting in June. The exciting news? I've found a few new forms of publicity for the group. One is a website called Meetup, which I paid to join for one month only, in order to contact the six people listed that are interested in Chiari Support. Next, I contacted Conquer Chiari so that our group can be added to their new Local Support Page. Last, I was contacted by the CEO of ASAP (American Syringomyelia and Chiari Alliance Project)! Really! He asked if he could list the group, and if there was anything he could do to help. He even said he would like to see me the next time he's in the Chicago area.

So, that's all good news. Hopefully, our little group will grow.

Next, I am planning on going to the American Syringomyelia and Chiari Alliance Project National Conference in July this year, which will be held in Madison, WI. I haven't bought tickets yet, but the plan is in the works.

Last, I finally ordered a walker. I ordered an Invacare Rollite Rolling Walker. It should be delivered sometime this week. I think that will help with my ability to move around, especially since it has a built-in seat to rest on. I think it should be really helpful. It will give me an alternative to my wheelchair.

Medications

2009-05-26T12:58:00.002-05:00

The following are the medicines I take daily:
*Midodrine--this medicine is a vasocontrictor, it helps your veins to constrict, thus raising dangerously low blood pressure
*Seasonique--low dose birth control pill, 13 week cycle, reduces the frequency of migraines
*Nexium--to treat Gastric-Esophageal Reflux Disease
*Zyrtec--to help with severe allergies to everything
*Thermotabs--salt tablets that increase fluid absorption, raising blood pressure
*Provigil--to prevent daytime sleeping due to Narcolepsy. If I don't take this medicine, I am not allowed to drive.
*Lithium--a kind of salt. I was frequently suicidal for 10 years until I began this medicine. I haven't been suicidal since.

Supplements:
*Calcium
*Vitamin D
*Magnesium
*Vitamin C

Please notice that none of these daily medications treat pain.

I use the following methods to treat pain:
*Sleep at least 10 hours every night.
*Rest in bed 90% of the time.
*Ice packs for certain headaches.
*Heating pads for muscle relaxation or intestinal cramps.
*Peppermint oil capsules for intestinal cramps.

Notice that this involves dramatic lifestyle changes. I am unable to live a normal life. I work 2 hours a day, 4 days a week, when possible. I very rarely socialize.

If none of these work for my pain, I then resort to a medication.
*Advil
*Vicodin--I take half of the lowest adult dose.
*Darvocet--I take half of the lowest adult dose.

I used to take daily meds, but a doctor was concerned that I was having rebound headaches. So I discontinued my daily Advil and Tylenol. Nothing changed.

So, I know that it seems scary to take a lot of medication. But I am being closely monitored by my doctors and myself. It is the way that I cope with multiple chronic illnesses and endless pain. Please don't fault me for needing to resort to prescription pain medication at times. I am doing my best to survive. However, don't be concerned that I have any kind of addiction. I average taking one dose of a narcotic per week. I also have my liver enzymes checked by my primary care doctor, to be sure that I am not causing any damage.

2009-05-25T14:58:00.002-05:00

So sick today. Can't get off the couch. Need help just to get to the bathroom. But everyone's out and enjoying the day. I'm just stuck here. Yes, I'm sad and in pain and almost completely incapacitated. No, I don't need sympathy or pity. I'm having enough of a pity party on my own, for today. I need surgery so badly. My body is failing. I really need to be fixed. This is not fair.

And please don't tell me to cheer up. That doesn't actually help anything. Sometimes you can just let someone recognize the unfairness of their situation and feel justifiably sad and angry about it. Sometimes, that's the best thing a real friend can do.

New/Worsening Symptoms

2009-05-24T11:51:00.003-05:00

I'd like to apologize in advance. I am extraordinarily tired, but I will try my best to write coherently.

Yesterday, Gus and I went to his cousin's wedding, an hour and a half away. It was quite a challenging day for me, and really showed the new symptoms I'm developing. I used my wheelchair and my Aspen collar the whole day to try to help me last as long as possible.

First, I took my pills in the morning, as I always do. Unfortunately, as has been happening lately, I had a lot of trouble getting them down. Already, I have to arch my back, pull my head back, and lower my chin to my chest to get big pills down. (I don't take any huge horse pills.) Even while doing all of these things, I have been having trouble swallowing pills. Sometimes, they come back up, sometimes they kind of go back and forth, and now, sometimes I actually gag and vomit several pills back up. I drink copious amounts of water in between pills. It seems that now, I have to add a little bit of food in between to encourage the pills to go down, in addition to the large amounts of water I drink.

Next, at the wedding ceremony itself, I had one of my new "sleeping" spells. I became extraordinarily tired, and basically fell asleep. The only difference, I could still hear what people were saying. I just didn't have the ability to react. Gus discovered a neat little trick. He guessed that I could hear them talking about me, and asked me to move a finger if he was correct. I didn't know if I could, but he was right. I could move a finger! So, we've developed a new way of dealing with this, which is choosing one finger to mean yes and another to mean no. This way, I can still communicate a little while I'm "sleeping". The only question is, how to deal with these spells. Salt pills don't seem to be the answer anymore. Yesterday, I think the Provigil helped, followed by a lot of water to get the Provigil working sooner.

After the Provigil kicked in, I started trying to wake myself up by squeezing my hands and feet, trying to pump the blood back to my brain. The best thing was when Gus squeezed my legs, which were not circulating much blood, based on how cold they were. He just kept squeezing them, pretty forcefully. I had decreased sensation in my legs. He thought he was hurting me, but I could barely even feel it. After a couple minutes, this really seemed to help send the blood back to my brain. I know that there are support hose for this, but in the heat, I promise that overheating causes just as much stress.

After the ceremony, we were the last ones to leave. We made it out to the car, where I was able to rest in the back seat for a bit. Then, we went with Gus' sisters and nephew to Subway, where I was able to eat and take more meds. I was feeling pretty well at this point, compared to the rest of the day.

So we went to the reception, expecting to be able to stay an hour or two. Unfortunately, there was another problem. The band, which included multiple horns, were all using microphones, amplifying the sound to a completely intolerable level. Of course, I did my very best to be polite and ignore it. Unfortunately, my brain just couldn't take it. I started having a very interesting reaction. The upper half of my body starting convulsing. After I couldn't control it anymore, I begged Gus to get me out of there. We found a couch in the hall, where I let the convulsions go for a couple minutes, without trying to stop them. I've had this reaction a few times before, but never this severe. I certainly never deal well with too much noise or lights or activity. I have the same reaction to being startled, too. I'm not sure the name for it, but I know that it is an autonomic reaction to the brain being overwhelmed. It's not a real seizure, but similar. After I stopped convulsing, I was completed exhausted and hypersensitive. Even the flash of a camera felt like an attack. My whole body felt like it was being attacked. My nerves were on edge. At that point, we gave up on the rest of the night, because we knew the music would last throughout the evening.

We drove home, a little frustrated with my failing body. I was completely exhausted and zoned out for the rest of the night. I slept fine last night, but I'm still exhausted now.

I'm glad we were able to go to most of the day, but as always, I feel guilty for causing problems.

I find that these symptoms are really hard for people to understand. When the symptom is a headache or nausea, everyone can relate, because they've all experienced it at some point. When the symptom is falling partially asleep or going into convulsions, but not having a real seizure, it's a lot harder to explain. The easiest thing is to say that the "sleeping" spells are similar to narcolepsy, and the convulsions are similar to a seizure.

Today, I will be resting until it's time for the piano recital. I will have to find a way to be awake/alert for that time. Then, it's a week off!!! I'm so glad I gave myself this week off. My body is truly exhausted. The real problem is, after a week off, how will I ever convince myself to return to work?

I have gotten started on getting all of the necessary pieces together for Dr. Menezes. I'm still very torn about whether or not I could let another doctor operate on me. But, I'm at least going to get his opinion, if he's willing to see me.

Ice picks

2009-05-22T13:00:00.002-05:00

So, last night, I was easily exhausted, and fell asleep quickly (that's not usually my problem). I awoke at 3:30, which wasn't unexpected, as this happens quite often these days (4 nights in a row now). Of course, my Ambien CR is still in the process of being approved. But I'm scared to take it every night and become dependent. Anyway, I woke up with another ice pick headache. It feels just how I imagine a trans-orbital lobotomy would feel (you know, the kind where they stuck an ice pick right over someone's eye and shifted it from side to side). Anyway, this time it was my right eye. Even when I got into a position with traction that lessened the pain, I was unable to fall back to sleep, as my mind was racing. It now takes two .5 doses of Klonopin to let me fall back to sleep (another example of building up a tolerance). Last night, it took the addition of a Vicodin to allow me to sleep. Hopefully the Ambien will help me solve this problem (although it only seems to allow me to sleep from 10:30 PM until 5:30 AM, which just isn't close to enough sleep for me).

So, even though I still have the ice pick headache, I've got some things to get done. My friend, Juan, has inspired me to gather the necessary paperwork/medical information needed to send to Dr. Menezes. I don't have any idea if I would actually have surgery done by a different doctor, but I hope to see him so I have every possibility available to me. It will also be nice to get a new perspective on my situation.

So, my job is to get:
*Copies of X-rays and MRI's done last year
*Copy of surgical reports from NY
*Referal from Dr. Shah to see Dr. Menezes
*Written history of symtpoms/surgeries

Lots of work to do. We'll see what I can get done before the holiday weekend.

still painfully tired

2009-05-19T22:43:00.003-05:00

So, I added a B Complex vitamin to my list of pills, but it definitely doesn't affect my energy level. I was in a state of sleep paralysis most of the day. I describe it this way, because I am technically aware of my surroundings and my brain is awake, but my body is so tired that I am essentially asleep. I certainly appear asleep. I only wish I could be asleep. But I suppose the Provigil keeps me from actually sleeping. I'm definitely raising the Provigil dose tomorrow. If I take more with my breakfast pills, maybe I will be able to actually function normally during the day. I went to work today, during the small window of time when I had the most Provigil in my system. Without it, I would be completely bedridden again. I've tried skipping Provigil to get some actual sleep, and I truly do remain bedridden for the entire day.

So very tired. Time to sleep again. Haha. Like it does any good. I'll wake up just as tired as I am right now.

I still have all my other symptoms. My head is killing me. But the lethargy is the most limiting symptom right now.

I'm not a fun person to be around right now. It's kinda like being with a person who is sleeping. When a person is sleeping, he/she is not very happy to be bothered in any way. Any touch or sound just annoys the sleeping person. That's how my body is all day long, since I am essentially sleeping. It actually hurts to move, hurts to be touched, and hurts to be talked to. So clearly, I am not the best version of myself right now. Everything seems to bother me. I'm sorry to those that have to interact with me. I'm trying my best.

Still have no idea when I'll be eligible for surgery. I know it's not going to be a quick fix, but I'm ready to get started. This is so frustrating. The libelous journalism about my doctors continues. I'm just ignoring it now. I assume I will still have surgery with The Chiari Institute, when the time comes. Boy, this patience thing really is a challenge.

Still so tired

2009-05-18T13:42:00.002-05:00

So, I made it through the weekend, I guess. The recital yesterday went well, even though I was mostly brain-dead the whole time. The fatigue and brain fog has not left me. On Saturday, Gus and I went to a barbecue to celebrate the end of the school year. After 2 hours of sitting down outside, we went inside, and as soon as there was a couch, my body shut down. I didn't fall asleep exactly. I was still aware of my surroundings. But I was mostly paralyzed and very out of it. I would call these low-blood pressure spells, as I have in the past, but they are a bit different, because salt and water don't seem to help. Instead, I seem to need to just stay "asleep" for some time. Most likely, this is a symptom of low oxygen in the brain. I may benefit from oxygen at times like this, but I don't think I'll ever find out, since my O2 stats measured on the finger don't seem to drop too low. Unfortunately, I know that for people like me, the brain can be deprived of oxygen, even if an O2 stat monitor doesn't recognize it. I would like to try oxygen, to see if it would help. On the other hand, nothing beats lying horizontally and allowing the body to rest at times. It's just that this brain fog and extreme fatigue have been with me for at least a week.

Today is another work day. Hopefully, the Provigil will help me again with some artificial energy. Thank you to medical science for finding ways to keep this sick body going.

Not just tired

2009-05-15T14:17:00.002-05:00

Despite taking extra salt and Provigil, I have suffered from excessive sleepiness all day. I have fallen asleep multiple times, just briefly, while upright. I also have had temporary paralysis. I can't stop yawning and my eyes keep tearing. I am profoundly tired, but not actually asleep. I feel like if I could fall asleep, I may finally feel rested. I may not be getting recuperative sleep during the night. I may be in a semi-conscious spell, but salt hasn't helped. I'm in such a haze. Maybe my next doses of meds will help. Maybe exhaustion is setting in due to overdoing it due to hypomania this past week. Good night.