Someday...

I kind of count down the days of my life...just biding time until I can have surgery. And now I can say that I made it through another weekend. It was mostly sick and rough and crabby, but ended on a more positive note, when I was able to cook dinner for Gus and myself. That hasn't happened in months! But he's swamped with work for grad school, of course. And next weekend, he'll be out of town for a conference, so it will be a while until I can see him again. This would be so much easier if we could just live together full-time already. But circumstances like his work and my health don't allow.

So, I'm wondering if my workout Friday contributed to my particularly sick day Saturday. The other option is that whatever was causing my fever was the culprit for my misery. But, it doesn't matter much anymore, because my fever seemed better today, and the abdominal pain was much less than it has been. So perhaps Dr. Marshal was right in his guess that I had torn some scar tissue and adherences by twisting the wrong way. It seems unlikely that this would have caused so much pain, but I'll take it, if that means the pain will go away on its own. I would just need to maintain some amount of core twisting to not allow the scar to adhere to the surrounding tissue so that doesn't happen again. We'll wait and see on this one.

I can't believe it snowed this weekend, and it's already Monday. That makes my mood much darker and discouraged. I sometimes feel so hopeful about my bone density improving and the surgery helping. And other times, I feel like this vitamin D can't possibly be enough to harden my bones, and who knows if the surgery will work? The doctors are always so confident that the surgery will make me better. But better in what way? Normal? Yeah right; I am so far past normal, I could never get back. Less pain? Less fatigue? Less nausea? No pain? No fatigue? No nausea? Who knows? No one can really tell me what to expect or what state I can hope to be in. Because some people have this surgery, and it fails...they just don't get any better. Due to the effectiveness of manual cervical traction, I believe that I will feel better. I just don't know how much better.

I wonder what I'll be able to do, once I spend a year (yes, a full year...maybe more) recovering from surgery. Will I become a perfect housewife? Will I become a volunteer at a hospital or animal shelter? Will I finally learn how to speak Spanish (Yo pienso que no, nunca, es imposible)? Or will I be satisfied with my current existence? Will I want to find more work?

I know I'll want to walk around more (ditch the wheelchair)...take my dogs for walks outside...dance for no reason...be a better supporter for those I love...develop more friendships...do something adventurous...be able to do things that other people do...become the person I was meant to be, but haven't had the chance!!!

I've been getting gradually sicker since childhood...moreso during adolescence, but certainly during my most important formative years. If my health were to drastically change, I could change, too. Maybe I would like myself more.

I file all of these things under the category of "someday".

And while I'm on the subject, I might as well mention the subject that never really leaves my mind...the possibility of becoming a mother in my lifetime. I believe that it is part of the reason I am on this earth. With such good role models and examples all around me, I know I would have so much to pass on to another person. My life wouldn't be complete without it. There are, of course, many practical dilemmas with this idea. First of all, when I talk about this, I'm truly speaking of a time many years from now. However, with my personality, I need to be allowed to think about and discuss the possibilities now, so I don't feel that I am doomed for life.

One major restriction on my future motherhood is my health and its effect on my ability to raise a child. I hope that in time, and with the right treatment (this next surgery), my health will not be such a great restriction. In addition, if I reach a state of improved health, could I really risk losing it by putting my body through the trauma of pregnancy and childbirth? I might never come back from that.

The other problem may be the moral dilemma of my lifetime. Chiari, POTS, and my form of EDS are still genetic mysteries. However, when you talk to families in the chiari world, you see how common it is for a mother to pass on one or all of her conditions to all of her children. With chiari alone, it seems less likely. But with the "trifecta", we're seeing whole families that are extremely sick and requiring a lifetime of medical treatment. Would I be able to live with myself if I knowingly gave this sickness and pain to someone I loved? Is it right to create a person just to put them through a life of torture...just so I can feel that my life is more complete? How could I take my child, as many are having to do, to have the same surgeries that I have suffered through? It doesn't seem right. We don't know the statistics, but with each study that is done, the chances of inheritance seem to get higher, as more and more chiari families are found and studied.

So, without too much more rambling, other options may need to be considered, such as surrogacy and adoption. This is a challenge for another day, but as people say, "my biological clock is ticking..." I do see pregnant women and women with infants around me and envy them for having something I may never have.

Those are all of my hypotheticals for the day. I'm a girl with a lot on my mind.

Still sick

Day 8 of my mystery fever. It's quite unusual for me to have a fever at all, as my body temperature runs so low. But my eyes and face have been burning. Abdominal pain was not too bad today. But lethargy hit me hard about the time my Provigil wore off. At 5:00, I fell into a deep sleep. Even when I woke up, I didn't move until 9:00; not an inch. My whole body felt so heavy that I just stayed in the same position. Now, I'm not sure how my nighttime sleep will be.

I did PT yesterday, and even though we did all lying down exercises, just the straining put the pressure in my head through the roof. I thought I was decompressed! My headache has still been killing me all day today.

All in all, a completely unproductive day. I got nothing done, except study my Spanish verb conjugations a bit. I learned the preterite tense of regular verbs today. But that's it. Gus has been great. He's so good at taking care of me and being understanding. I love him so much and his patience amazes me. But it's still hard for me not to feel guilty that our relationship is sometimes so limited and one-sided: he takes care of me. It's hard to be romantic and loving when you're so very sick...all the time!!!

A frustrating and fruitless day

Today was so hard. A lot of that was due to inept and/or rude medical staff. Another part was some extreme emotional sensitivity on my part which is probably attributable to hormonal issues. I need to remember that due to my PMDD, I need to increase my dose of Klonopin during certain times.

The main thing that got done today was the appointment and testing regarding my recurring gallbladder pain. My fever was higher today, to the point that my eyes were burning and tearing up. So some type of infection is likely in progress. The doctor originally did not take me seriously, suggesting that I had possibly pulled a muscle in the area. But, after the research I did last night, I knew what to focus on to be taken seriously. I explained the localization of the pain and the fact that I had had hundreds of gallstones. He actually took me seriously enough to order an abdominal and pelvic CT scan with oral and IV contrast. He also did a blood test to check my liver enzymes.

The results of the CT came back tonight, after a pile of bureaucratic frustration. But the doctor came back saying (what else?) that nothing was seen on the CT scan that could be causing my pain, fever, and nausea. The only abnormality noted, which has also been noted in a previous abdominal CT is an excess of "waste product" in the colon. Based on my diagnosis of POTS, this is likely due to a dysfunction of the autonomic nervous system. My brain probably doesn't tell my large intestine to contract. This is a very common problem with POTS, and I am lucky it doesn't effect more of my system. However, this can only explain some mild "back-up" and bloating, no other symptoms.

I still have no explanation for the epigastric and right upper quadrant pain. And it is actually the dominant pain at times, which is astounding, based on the level of the rest of the pain in my body. I also have a level of constant pain in this region, which is just adding to my list of constant pains.

The fever is on day 5, and it's quite uncomfortable, because my eyes are burning and tearing up all of the time. This fever must be caused by something. I never get fevers!

I wish someone could just give me an answer.

If this pain persists, I will have to see a specialist. Hopefully, my former gastroenterologist will be willing to see me about this problem, and be able to diagnose and treat it. A cholangiogram would be useful, since it is a nuclear medicine test designed to image the functioning of the biliary system. An endoscopy would have to be the next step, to look inside and see if there's anything they can see. But that's only if a doctor is willing to see me. And then the doctor would have to take me seriously.

Unfortunately, based on my recent experiences, I'm skeptical that any of this will go smoothly, or that a diagnosis will actually be found...much less a treatment that resolves the symptom.

This is just not my main concern right now, and I'd really like to get it out of the way, so I can deal with preparing my body for a much more major operation, and treat some much more serious symptoms.

But my body seems to have other plans sometimes. Nothing ever goes as planned with this body. As a fellow chiarian has said, "When you hear hoofbeats, think zebras, not horses." http://www.medicalzebras.com/index.htm. If my doctors could just learn that I really am a zebra, and if it can happen to a tiny percentage of people, it's much more likely to happen to me than others, then I would get much faster and more effective treatment.

Actually, my wonderful gynecologist said something along those lines today. He said that I'll always surprise people and always challenge my doctors. He said that I've been surprising him as long as he's known me (age 16).

So, I'm exhausted and very frustrated, and the pain in my right upper quadrant is quite bad right now. But so is my headache and nausea...seriously.

Time to spend the night getting this barium out of my system...oh how pleasant.

No more...

This headache will not go away. I can't sleep much, and I'm needing a lot of pain meds just to get by.

But in addition, I'm going to the doctor tomorrow for this recurrence of my gallbladder-type pain. Apparently, a lot of people develop problems after gallbladder removal. One reason is that a stone could have been left behind or a new stone could have formed within the bile duct. Another problem has to do with the drainage of bile causing esophagitis and gastritis. A less likely problem is that one of the sphincters in the area is spasming, causing extra pressure in the ducts. Whatever it is, it seems that this resurgence of gallbladder pain, in addition to the low grade fever, nausea, and increased acid reflux, could all be related to some problem in this region. Hopefully, the doctor I see tomorrow will be willing to order the necessary tests and figure out what is wrong quickly...and then be able to fix it! I need to be taken seriously, since this problem seems to be getting worse, and the pain is competing with my headache at this point.

So, that just feels like one too many things to handle right now, while my instability symptoms are so bad. I just would like this to be resolved easily, for once. Unfortunately, I always tend to be a "special" case, so we'll just see what happens.

There's only so much pain a body can handle at once. My arms don't hurt. That's all I can think of right now. Everything else does hurt. I just can't handle much more.

Headaches and more

Last night, I developed an intense headache. It lasted all night, no matter which position I lied in. The headache focused over my right eye, through the right side of my face. It has continued through the day today. Tonight, it has settled into more of a classic instability headache (the sunken halo) with nausea that has limited my food intake (only a couple graham crackers with peanut butter). I did go to work, and that was almost impossible, but made everything worse, including joint pain. My knees hurt a lot, and there are two areas in my spine causing me pain. One is right above my lumbar incision, where there is swelling, and what feels like some bone protruding. I'm not sure who to talk to about this. Also, the joints below my cervical fusion are sore, which is to be expected due to the extra strain on this region.

In addition, for four days in a row, I've had a low-grade fever most of the time. If you know me, you know I never have a fever...I usually am quite a bit colder than the average human being.

So basically, I am completely miserable. I'd love a break from this. I just want all of this to end. I'm so very tired. How much longer must I endure? It's just so exhausting.

My Story: An Overview

Here is an overview of the conditions I have:

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. The main things affected in me are my ligaments and veins. 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.

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.

Cranio-Cervical Instability/Cranial Settling: When EDS and Chiari join together, the skull-spine joint often becomes unstable, due to loose ligaments in the neck. This causes trouble swallowing, nausea, heartrate abnormalities, and other autonomic dysfunctions.

Vitamin D deficiency causing Osteopenia: The theory is that a severe vitamin D deficiency has lead to my bones becoming brittle at a very young age. This osteopenia would lead quickly to osteoporosis without treatment. It also makes the fusion of bones more challenging.
__________________________________________________________________

Symptoms:

Headaches
constant pressure headache (pushing out around the skull)
the "fallen halo" headache (pain in a ring around the head over the eyes and temples)
sharp pains above and behind the eyes
facial pain through cheekbones
jaw pain--tires easily, making it difficult to chew
eye pressure/pain
Usually worsened by light and low sounds
migraines

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.

Back pain--I have pain throughout my spine. This is caused by the tethered spinal cord and the loose ligaments holding my spine together.

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. Basically, I am a slave to my body. If it wants more sleep, it will get it. I have to spend most of the day in bed most days.

Tachycardia/Low blood pressure/fainting--After being upright for too long, my heart races and my blood pressure drops. I get out of breath very quickly, and sometimes faint. Sometimes for no reason at all, my blood pressure gets very low, and I can fall into a state of reduced consciousness.

Heavy head--Due to the instability, I have difficulty holding my head up. I'm a living Bobble-head. This is very tiring and leads to severe muscle spasms in my neck from trying to support my head, since the ligaments aren't doing their job.

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 and hyperparathyroidism.

Difficulty swallowing--I am more and more frequently having choking spells, when I inhale a food or liquid, and my airway closes. I'm also having trouble swallowing pills. I swallow pills past my throat by arching my back and bending my chin to my chest, but then they sit in my esophagus. The muscles don't constrict well enough to help completely swallow. This is caused by worsening cranio-cervical instability.

Tingling in hands, face, and feet

Temperature dysregulation--I am very sensitive to changes in temperature. I get the chills or overheat easily. I also get confused temperature states, when I feel hot and cold at the same time, which is really uncomfortable and unsettling. I also have cold feet all the time!

Gastro-esophageal reflux--My acid reflux doesn't seem to react to medication anymore.

__________________________________________________________________

Treatment

Medication

Midodrine- to constrict my veins, to help improve blood flow
Salt pills- to force my body to retain more fluid, which raises blood pressure
Provigil- to fight fatigue.
Diamox- to decrease the production of cerebro-spinal fluid, decreasing pressure in my head
Vitamin D (50,000 units/week)- for vitamin D deficiency and osteopenia
Citracal- calcium supplement, for osteopenia
I take other medications, but these are the major ones.

I've already had three 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.

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

After that last surgery, I worked very hard to recover. When symptoms didn't improve, the doctors said that nothing else could be done. Then, I began to search online for more answers. 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 have an occult tethered spinal cord. It was also found that I have an inadequate fusion.

In June 2008, I had a section of the filum terminale operation to de-tether my spinal cord. This released the tension in my spinal cord. My bladder function has become more regular. This surgery also prevented a worsening of lower body symptoms that could have occurred over time or after further extraction of my cervical spine.

I will require a revision to my current cranio-cervical fusion. This will reset the position of my head, allowing for greater stability. I'll get my next bone scan in early May, and then I will be waiting for the approval for surgery from Dr. Bolognese. If my bones are strong enough, I will have surgery done at the beginning of this summer.

A Good Weekend

It was so nice to have a normal weekend with Gus. It was just so great to be back to my baseline. I had headaches and nausea and fatigue, but I was able to function. I didn't lay in bed all day. For a 3-day weekend, I sat up, got work done on my computer (transcribing songs), and went out to eat twice. It was great not to have excessive shortness of breath or severe nausea. I just had baseline amounts of pain and fatigue. It may not sound like much, but it was amazing.

The only thing that happened is that I had a fever Saturday and Sunday, which is unusual for me. I also had some moderate aching in my upper central region of my abdomen...the same area I felt pain associated with my gallstones. But since I don't have a gallbladder anymore, I tried to ignore it. It didn't worsen or become severe...but it stayed pretty uncomfortable and constant. I'll have to wait and see what happens with the fever and abdomen. If anyone knows why one would have biliary pain once the gallbladder is removed, I'd be interested.

Otherwise, I'm just grateful for a break from the severe symptoms.

Eating!

Starting yesterday, I've been able to eat food again! Yay! It feels so much better to be eating. I still have strong nausea spells when I've been upright too long, but I eat in between spells.

Just wanted to put something positive in here.

Email to June at TCI

Here's my recent correspondence with June at TCI:

June,

I have faxed over my DEXA scan and my blood work from January and February. I hope you have received them.

I'm still having good and bad days, but overall my symptoms are still terrible. My instability symptoms persist, with instability headaches waking me up at night. The nausea has worsened significantly this week. Some days, I can barely get anything down. Sometimes, I can only swallow food when my body is actually horizontal, or I will gag it back up. Hopefully, this symptoms will pass or reduce soon, because I'm not getting adequate nourishment right now. I will have to start supplementing with Ensure.

I have been blacking out more often, and the shortness of breath was so severe that my local cardiologist and my POTS specialist sent me to the E.R. for testing. Of course, my heart and lungs were functioning fine. They said I was just suffering from sinus tachycardia, where even my resting heart rate wouldn't go below 95 bpm, and this was causing the shortness of breath.

But each day has different challenges. Some days the nausea is the worst, sometimes the headaches, and sometimes the shortness of breath and passing out. Some days, it seems like everything is wrong, like this weekend, when I've been completely restricted to bed except for assisted bathroom trips.

My next scheduled doctor's appointment is April 26th with Dr. Camacho, my endocrinologist. I will ask her to order my next DEXA scan at that point. I plan on getting it done as soon as possible after that appointment. Then, I will send the results along to you.

Should I consider scheduling an appointment at TCI for sometime in May? If so, what kind of appointment would I request? I just don't want to have to deal with too much of a wait for an appointment. What would be the best thing to do?

Thanks so much for your ongoing help.

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

Hi Carolyn,
I got your January DEXA scan. I compared it to the one done on 3/2008: there was a slight worsening. I don't remember when you started treatment. So let's see what the next DEXA scan in April will say. Hopefully if it is improve, you can go ahead with the surgery.

I guess you can come for a visit in May. You usually see Dr. Kula, correct? You may need to book now because sometimes he is booked up fast. If the DEXA scan is normal and if Dr. B says OK, you might want to book for surgery too. You can't book for surgery until the DEXA is normal and/or Dr. B says OK.

I don't know, do you want to wait until the result is back or make the appoiment with Dr. Kula for May?

Please let me know,
June

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

June,

Thank you so much for responding. Dr. Camacho ordered the January DEXA scan as a preliminary scan. It was performed only 3 weeks into my treatment, so for her, it is considered the "before treatment" test. My next scan will be done at the end of April or the beginning of May, and hopefully that one will show improvement.

The reason the osteopenia got worse from 3/2008 to 1/2009 is that I was untreated during this time. I was not able to find a doctor willing to treat my osteopenia for 8 months, which gave it time to worsen.

I don't feel that I need another appointment with Dr. Kula at this time. I think the next time I come in, it will be to see Dr. B for surgery. So for now I will wait on scheduling any appointment.

Thanks again.
Carolyn

Bedbound weekend

I had a good week. I got a lot done, and visited with family, too.

This weekend, my instability symptoms became unbearable. Friday night, I developed the worst instability headache. It kept me awake in agony the whole night, with very little sleep. All day Saturday, every time I sat up, I got sickening waves of nausea. I couldn't eat anything except a bit of pudding all day. I tried eating other foods, but I can only eat while lying flat, and I have to be able to digest that food while horizontal...always a challenge. Also, I have to find food that seems palatable when I am so nauseated. So for Saturday, it was only pudding.

Overnight last night, the headache was not as invasive, but I still had several hours in the middle of the night where I was wide awake and incredibly uncomfortable.

Today, I made it over to the couch to watch a movie with Gus. I ate my cereal for breakfast, but the waves of nausea are still strong when I sit up for more than a minute.

I really don't know how long this severity of symptoms will last, but as long as it does, I won't be able to drive, work, or even eat and take care of myself very well. But I lived for so long with these symptoms, that I fear that they could last until surgery, which still has no set time.

The only dates I have to look forward to are April 26th, when I will see Dr. Camacho, who will order my next DEXA scan. If this scan can confirm that my bone density is improving, then I will make an appointment with The Chiari Institute in New York. They know about my current condition, and they understand that surgery will be needed a.s.a.p. Maybe I could try to make the appointment ahead of time, to be sure there isn't a long waiting period. I just don't know the exact dates I would want for an appointment...especially if the appointment and surgery are all in one trip.

I just so desperately need things to get better soon, somehow, someway. It's so hard to live like this.

Cardiology and drop attack

I saw the cardiologist this morning. He couldn't come up with much to say. He said I'm suffering from bouts of sinus tachycardia, that seem to last all day some days. But that there is no structural reason in the heart responsible for this. He said that either the tachycardia is causing the shortness of breath, or the shortness of breath is causing the tachycardia. To find out if my lungs are functioning well, I am to schedule pulmonary function testing. I'd rather have the stress test he mentioned, since my symptoms are worsened by exertion. But for now, just the lung function testing that we'll set up sometime. After the appointment, I sat in the waiting room while my mom checked out. And I started zoning out, and when I stood up, I had a drop attack right there in the office. This is not a strange occurrence for me, because I have drop attacks every few days. But it was ideal for it to happen with nurses there to check my stats. They found that my blood pressure had dropped by about 20-30 points from my bp during the appointment. (It ranged from 120/60 to 110/72 in the appointment, and after the drop attack, it was 90/--) My heart rate was actually lower, probably because I was lying down. But my O2 stats were still great--98/99%. It's good to have these on record for right after a fall. I recovered quickly after drinking several glasses of water. I had been complaining about my thirst, so I was probably a bit dehydrated, which always makes fainting more likely.

I was stuck in bed all day yesterday with severe nausea and fatigue. I barely moved all day. Less fatigue today, but still severe nausea and reflux. Obviously, I still have to be careful about my blood pressure, too.

I wish someone at Dr. Grubb's office could help me out with all of these POTS symptoms right now. But I kind of doubt there's much he could do. My problem is a stuctural problem in my brain...not much can be done about that...just SURGERY. Just gotta keep waiting. The rest of this is just filler. I hate being patient.

Takes my breath away

So, on Friday, I heard back from all of my cardiologists and went to physical therapy. Every single doctor told me that I needed to go to the emergency room to find out what was going on with my breathlessness.

So, we spent the evening in the e.r. They did every standard heart and lung test they could come up with. But really, as soon as we got there, they gave me oxygen, and I got to lay down in a bed, so I felt significantly better from that. My mom said that my face and lips had more color than they'd had in weeks. My lips are normally light pink and even a little blue. With oxygen, some of that paleness went away and my lips turned pink. The e.r. doctor said that this was meaningless, because the oxygen saturation level on my finger was 97% or higher. So, she said there would be no reason to consider a portable oxygen unit. However, I'll be seeing my local cardiologist, Dr. Jobski, on Friday, and we'll see what he has to say.

I'm also considering a local POTS specialist, Dr. Janice Gilden, since Dr. Grubb's office says there's nothing they can do for me two states away.

There's a new pain management doctor that's been recommended by a new friend, so I'll keep that in mind as well.

Until then, I'm still out of breath whenever I'm not lying down most days.

However, I had a nice weekend. On Saturday, I took my provigil (which is supposed to keep me awake), but still felt so fatigued that I fell asleep anyway. After a nap, I felt pretty good. I still had breathlessness when standing. Overnight Saturday to Sunday, I had a horrible headache (an 8), which included sharp pain above the eyes, pain with movement, and constant nose dripping (probably a leaking of excess spinal fluid). That headache lasted throughout Sunday and Monday, despite treatment with Darvocet and Vicodin. I also took provigil, since I'm trying to take it daily, as prescribed. So, that gave me a fair amount of "artificial energy" to enjoy Medieval Times, where we went for my brother, Rob's 21st birthday. By the end of the day, I was exhausted, and I have been ever since.

I feel that the Provigil makes me "overdo it", using up all of that fake energy. It is followed by pure exhaustion and pain all over my body.

I don't know what to do about it, but Dr. Grubb's office says to give it a full month to work...I'm trying, but it just might kill me (presumed exaggeration). But I would like to point out that the shortness of breath began several weeks before I started taking the Provigil, so the Provigil is not the cause of the breathlessness, but it could be contributing to palpitations and tachycardia.