NOT Giving Up

Okay. So, I've had a rough week month year decade?  But the last few months have been especially hard due to digestive problems.  But my digestive problems are finally easing up a bit!!!  I'm finally eating 1500 calories every day!!!  Yes. This is huge. My pain level is better when I get enough calories. My energy level is better when I get enough calories. My migraine frequency is definitely reduced when I get enough calories. Plus, my mood is so much better when I get enough calories. Who'd have thought? Starvation is bad for you! I feel so much sympathy for my friends with severe Gastroparesis, IBD, or food intolerances that cannot get enough calories in a day.

Basically, I got myself into trouble with elimination diets and trying to identify my food triggers. I kept eliminating problem foods until I had almost nothing left. I tried to follow a low FODMAP diet for IBS. I was also following a low fiber, low fat, low residue diet for Gastroparesis. I also added the low histamine diet for suspected mast cell reactions. But when you don't eat enough food, Gastroparesis actually gets worse. Your stomach shrinks and slows down when it isn't constantly forced to handle more food.

So, I started a new plan of taking in calories once an hour throughout my 12-hour day. I alternated between liquids and solids, with pretty small portions. I added Naked Fruit Juice for extra calories. I found a meal replacement drink called Orgain that is pretty healthy, and full of liquid calories without a bunch of sugar and artificial chemicals. (You can buy it at Whole Foods or on Amazon. It's pretty tasty-but quite expensive.) And I've started baking more breads, muffins, and cookies.  This has really helped my digestion!

Finally, I'm starting to incorporate some fruits and vegetables. Applesauce is still the easiest and safest. But I've successfully eaten a few pears and small servings of tiny chopped broccoli. I've even been able to switch back to whole grains, little by little. It is not a great diet, but it's a start.

I almost forgot the strangest part about this! My weight has not changed. When I dropped down to 600 calories a day, I weighed 116 lbs. Now that I'm back to 1500-1600 calories a day, I weigh 116 lbs. Maybe this is why I didn't notice sooner how undernourished I was! My weight didn't drop at all. My clothes still fit. I don't know what to think of this. Calories in and calories out certainly do not determine my weight though. Just thought I'd throw this in there for others trying elimination diets. Just because you are maintaining your weight does NOT mean you are getting enough calories! Add them up and be sure you are getting enough to keep your body running. I use My Fitness Pal, an iPad app. Based on my age, weight, gender, activity level, and weight loss goals (maintenance), it determined I need about 1560 calories a day. If I burn calories with exercise, I need to make those up. It has really helped me.

I still eat mostly a soft foods and liquid diet. And I need to avoid high histamine foods, since I have such clear systemic reactions to those. But I usually can get my calories without the meal replacement drink. It takes a lot of effort and careful planning. I have to count my calories to be sure I hit my goal. But I can do it. YAY!!!

Okay...before we get carried away here, I have a lot of symptoms going on every day. My dysautonomia is still bad. I still have spells of reduced consciousness every day, and I still lose consciousness at times. The cerebral hypoperfusion is still such a huge problem for me. And my allergy/mast cell issues are still seeming to progress.

I am considering making an appointment at Rush in Chicago with their Allergy/Immunology department to see if they can evaluate and treat mast cell disease. But, due to my trauma related to doctors/hospitals/etc., I'm still putting it off. Testing could include a bone marrow biopsy, skin biopsies, and an endoscopy with biopsies. I would refuse those tests at this time.

Next week, I'm starting with a local allergist. I do not believe he will know anything about Mastocytosis or Mast Cell Activation Disorder. But, he should be able to test me for true IgE allergies, especially to dogs, since this is my biggest concern right now. Unfortunately, you're supposed to go seven days without antihistamines before the testing, and I have no idea how I will tolerate that. I'm currently taking Allegra, Zyrtec, Astepro, Nasalcrom, Benadryl, and sometimes Atarax every day. Yes, every day. More when I'm around Charlie. I have no idea how I will survive without them for even one day.

If I can survive until the testing, and I test positive, maybe I could start allergy shots (immunotherapy) to try to reduce my reactions to dogs. This would improve my quality of life so much, because I could live with my little Charlie again!!! Allergy shots would be a logistical nightmare, since you have to go in three times a week for six months...then once a week for three to five years. I usually cannot drive, and my hubby works, so this would be quite a challenge to figure out. Beyond that, the allergy shots themselves could trigger a mast cell reaction, in which case I wouldn't be able to tolerate them at all. But it's worth a try. Anything for Charlie.

On a totally different note, I haven't been able to start working yet. But, I have joined a book club! It meets once a month in the evening, so it's a very small commitment. I've only been once, but it is nice to get out and be around people.

I'll update after my appointment with the allergist next week. Thanks for caring enough to keep reading.

Giving Up

I can't handle this much longer. Everything is worse. My body is not working. I am mostly unable to digest food, with symptoms that seem to be from Gastroparesis.  I have constant nausea. When I actually do eat, I quickly have the sensation of being full and still hungry at the same time.

Apparently, I was only eating 600-800 calories a day in October, while trying to identify food sensitivities. This was causing my daily migraines. So, with much effort, I am now up to 1100-1200 calories a day (soft foods and liquids). The migraines are actually improved. Apparently, my body doesn't like to be starved. My goal would be to get about 1600 calories a day, but just getting to 1200 is so hard with such intense nausea and fullness.

Everything else is quite horrible. I won't be having any follow-up for this last surgery. After 2 months of being given the run-around, I've learned that a Skype appointment is not an option for some stupid reason. And believe me, I'm not up for traveling by any means. I can barely take care of myself at home.

I still struggle to remain conscious most days.

I am so traumatized by doctors, hospitals, procedures, tests, and surgeries that I honestly refuse to see a gastroenterologist for these newer issues. I truly believe there is nothing that any doctor in the world can do to help me with any of my problems, and that I just need to manage this on my own.

My system is just so sensitive and easily overwhelmed by everything. A lot of this seems like mast cell symptoms. But that's something else I will just manage on my own. I don't have access to mast cell stabilizers, but I can sure self-medicate with antihistamines as needed.

I am just done. I'm done looking for elusive answers and treatments that end up making things worse. I just can't tolerate anything. Pain meds, migraine meds...they all cause mast cell reactions. My mast cell reactions are just getting stronger and stronger. A single whiff of fragrance can take me out. My sensitivity to high-histamine foods is just getting worse and worse. It feels very progressive to me.

I just have to survive as best I can, and hope that my body heals itself. There are no magical answers out there. There is no surgery that can help me. There is no test or procedure I'm willing to go through at this time. I am not up for any type of travel, so most of the expert doctors are out of reach. Perhaps one day, I'll go back to Dr. Francomano in Baltimore, since she clearly understands the GI motility issues and mast cell problems associated with EDS.

I am getting worse in many ways. I don't know how to handle it. But I'm done fighting it. I'm going back to bed now. Hoping for some type of escape. The suffering is just so intense. The constant migraine is fighting to come back...I have to figure out what the heck I can eat. I wish I could be done.

Just Keep Swimming

I know I haven't posted in a couple of months. I also haven't been very communicative in general. I guess it's just that I know people like to hear good news. It's been a challenging time - some good times, but a whole lot of bad times. I'm dealing with quite a few issues every day, and coping as best as I can.

I'm having a lot of migraines. Almost half of my days are migraine days right now. I've already flunked out of a headache clinic (we tried every treatment available), so I have only Maxalt (a triptan) to treat my migraines. The Maxalt generally does help. I certainly can't skip the Maxalt too often, because the migraine blows up and becomes unmanageable if I don't take the Maxalt as early as possible. Unfortunately, it makes me feel very drugged. It also makes me incredibly itchy (possibly a mast cell reaction). So, I have to take 2 Benadryl to be able to tolerate the Maxalt. So, migraine days are generally lost days. Additionally, if I have more than three migraine days in a row, I just have to tough it out to avoid rebound migraines from the Maxalt use. (Today is a day three migraine, so I'm really hoping for a break tomorrow!)

Another issue that's been challenging is my allergy (suspected mast cell) issues. The main symptoms are itching and swelling. But sometimes, the itching is so bad that it keeps me from sleeping at night, and the lack of restorative sleep triggers my dysautonomia severely. I'm also reacting more strongly to all of my allergic triggers. Unfortunately, this seems to include my sweet puppy, Charlie. Plenty of other symptoms could be related to a mast cell activation disease, but for now, I'm just calling these allergies. Benadryl is becoming my best friend. I'm trying to avoid foods that are naturally high in histamine to lower my overall histamine load, but I can't tell if it's helping. (This includes fermented foods, strawberries, leftover meats, and a bunch of other foods.)

Challenge #3: My digestive tract is a mess. My main symptom is severe bloating and pain. My chronic constipation is improved, and I'm no longer dependent on Miralax. But the bloating and associated pain are still so severe. I've already tried things like fiber supplements, digestive enzymes, and probiotics, but they don't seem to do anything. So, I started a food and symptom diary to try to figure out what is making me sick. Unfortunately, it seems to be a lot of foods. So far, rice, almond milk, potatoes, and chicken seem to be my safest foods. I initially tried a diet I've tried in the past, the Eating for IBS diet, which involves eliminating foods that are high in insoluble fiber, egg yolks, dairy, red meat, and fatty foods. This hasn't been enough. Next, I have eliminated foods that are high in FODMAPs. If you don't know about FODMAPs, there is lots of information out there. It is a scientifically proven approach to treating IBS. FODMAPs include foods like: apples, onion, garlic, honey, agave, wheat, dairy, legumes, pears, cruciferous vegetables, artificial sweeteners, sugar alcohols, and a lot of other foods. They are foods that contain high levels of short-chain carbohydrates, like lactose, fructose, fructans, galactans, and polyols. I had a lot of hope for this diet, and gave it a really solid try. Unfortunately for me, FODMAPs elimination has not been enough to eliminate my symptoms. I really cannot tolerate any fruits or vegetables right now. The pain is too severe. I can sometimes handle half a banana or some cooked spinach (ew!). My diet is one of constant experimentation. I don't do well with smoothies, which is really disappointing. But I'm considering trying juicing. It isn't as healthy as smoothies, but all of the hard-to-digest fiber is removed from the food, and you still get the other vitamins and nutrients. I have no idea if this will work. Some days, it really doesn't matter what I eat. Even when I eat my safest diet, I can still have symptoms. I wish I could just stop eating. On the other hand, it's hard not to think: if even this bland, boring, generally not-too-healthy food is making me sick, why don't I just eat whatever I want?? But then, I'm reminded of past bowel obstructions, and I know I'm doing better than I would be if I were eating dairy, salads, and red meat. IBS symptoms can be caused by EDS. Malabsorption and motility issues are common with EDS or dysautonomia. But my motility seems to be okay if I avoid the worst dietary offenders.

I have a lot of other symptoms to manage on a daily basis. But these are the biggest ones right now. I'm also just trying to cope emotionally with my body's limitations. Many days are really so miserable that I just have to cope with my physical state. I often have widespread pain, nausea, shortness of breath, and a really intense feeling like having the flu (hard to describe). During these times, I just can't get comfortable, so I curl up in bed, put on some Netflix, and just wait for it to ease up. I missed out on my hubby's 30th birthday festivities due to this sickness. I hate it. I hate how much I have to miss out on. I miss my puppy. I miss my independence. I miss earning my own money. I just hate it. I hate being so self-involved due to this illness, and I wish I could be a better spouse, family member, and friend. I think we all thought I would be doing better by now.

The good news: many days, the sickness eases up in the evening, and I am able to enjoy myself a bit. I exercise every night, recumbent bike-riding and light toning exercises. I think it helps my POTS, but I still get out of breath just walking to the bathroom most mornings. I do believe that I have a successful bony fusion. I don't feel like I need to wear my collar, and I never need traction. My neck still gets tired and my muscles seize up with tension every day. I haven't had my post-op scans yet. Communication with TCI has been pretty bad. But I will find a way to get them done this calendar year. Like I said, I do believe that I am finally fully fused this time. My range of motion is actually reduced this time! I still use my bone stimulator every day, just to be sure.

Clearly, the fusion doesn't resolve everything. But I still expect that recovery from the trauma of surgery and the anesthesia will continue up to two years after surgery. I know that sounds crazy, but my body really does take a long time to recover.

The question that often comes up: what do you do all day? I have a lot of trouble answering this one. Basically, I just survive, and hope for better. I keep working toward a better life, even if it seems like I'm not getting anywhere.

I will be happy when I can live closer to my family. My life will be more meaningful if I can spend more time with my loved ones, the ones that support me through all of these years of illness. I am so very lucky to have these people, along with my amazingly supportive husband, in my life. In the meantime, I'll be here...just trying to do what's best for my body, and maintaining the hope that it may still get better.

Feeling Hopeless

I've had a few really great days over the summer.  They make me hopeful that the future could be brighter.

But right now, I feel completely hopeless.  I have been having frequent spells of low blood pressure that do not get better when I lie down.  My go-to medication for hypotension is Midodrine.  The last couple times, Midodrine actually made me worse.  It caused my heart rate to drop dangerously low, in addition to low blood pressure, which causes chest pains.  Now, I have nothing to go to when my blood pressure drops.  The spells usually come on after exercise or some type of activity.  They're usually later in the day, which is unusual for my POTS.  They aren't necessarily POTS-related.  I have lost consciousness quite a few times in the last week.  And it's not like you lose consciousness and then wake up dazed and confused a few seconds later.  These are prolonged episodes of unconciousness or near-unconsciousness.

If my sleep is not restful enough or my body is fighting any kind of infection, I can be in a stupor the entire day.

For this kind of low blood pressure, Florinef is commonly prescribed.  Unfortunately, it raises intracranial pressure and causes me massive headaches.

The only thing that can help me is Thermotabs and fluids.  All day, every day, I chug water.  For every 10 ounces of water, I take another Thermotab (buffered salt tablet).  This is about 12 a day right now.  Yes, I drink at least 120 ounces of water a day.  And over 2000 mg of sodium from pills.  It is all I have right now.

I contacted a nurse at Dr. Grubb's office.  She basically told me to not worry about it.  She reminded me that I have a chronic condition (Thanks!).  She said that IV fluids are not a good long term solution, especially in someone with EDS, cause your veins will all get blown.  She said to only go to the ER if I fall and hurt myself.  I don't, it comes on gradually. So, we just have to accept the spells of unconsciousness.

I cannot take anything else for my depression/anxiety, because I have had serotonin syndrome.  That's a dangerous, potentially fatal condition, so you really can't mess with it.

I am supposed to be raising a puppy and starting to teach piano lessons again.  I don't know when I'll be able to do that, because my condition is so unpredictable.  I don't want to turn away potential students that are inquiring about lessons, but I don't know what else to do.  We clearly need the money, but I cannot commit to anything in my current state.

I know that the overall lesson here is patience.  I know that my condition has highs and lows, and 5 months out of surgery, it should still be mostly lows for someone like me.  I just don't see the light at the end of the tunnel right now.  I have no idea how much better I will get, or how long it will take to get better.  This could be it.  I want to participate in my life, not just endure it.  And I want to be a better wife, family member, and friend.  I just have nothing to give right now.  The only thing I know that could help me get to that better state of health is exercise, but exercise makes me pass out.  I just can't stand being stuck like this.  I can't stand feeling so sick and with such a low level of functioning and a low level of consciousness.

It's really hard when your husband has to check and see if you're still breathing, because you look dead and are completely non-responsive.  And it's hard that no one can help me.  When I'm barely conscious, there's just nothing to do anymore.

These low blood pressure spells could potentially be a mast cell activation reaction, but I'm seriously getting nowhere with that diagnosis.

I still don't have a local doctor due to an insurance problem.  Not like some random PCP would have any idea what to do with me.  There is no help.  Just deal with it.

Sorry for writing when I'm so upset.  I know I'll get past this feeling.  I just needed to reach out a bit.  I know someone reading will relate to these feelings...and others will probably think I'm just a depressed nutcase that should get some therapy.  But in reality, these are just the ups and downs of living with chronic health problems.

5 months Post-op

I had my 2nd total cranio-cervical fusion reconstruction exactly 5 months ago today.  It is a very difficult surgery.  Since I have dysautonomia and suspected mast cell problems, recovery is very prolonged.  It's not fair to judge the success of a surgery based on symptoms at 5 months.  It's better to check at 12 months, maybe even later.  But I'll try to look objectively and see what may be better.

The last month has been challenging.  I was sick with an upper respiratory virus, causing a sinus infection and bronchitis.  For those two weeks, my POTS symptoms were out of control: high tachycardia, lots of shortness of breath, some spells of hypotension, lowered level of consciousness.  Then, I got one of my 4 day migraines.  Luckily, the virus and migraine were gone by August 2, the day we picked up our new puppy!  His name is Charlie.  He is a sweet little Cockapoo.  I'm not allergic to him!  I completely adore this little guy.  Unfortunately, after 4 nights of poor sleep due to the puppy's cries, my whole system shut down.  I couldn't stay conscious, my digestion went crazy, spells of very low blood pressure, tachycardia, shortness of breath, couldn't take care of myself, much less my beloved little Charlie.  So, after 5 days with us, Charlie moved in with my mom.  She and my stepdad are willing to take care of him until he grows up a bit and until I am a bit recovered.  But I really miss him!  I tear up just thinking about him.

The thing is, with all of these complications, my progress has definitely had a setback.  So, I'm rebuilding again...starting with sleeping as much as I can at night, resting as needed during the day, and exercising every single day, as much as I safely can.  My well-being depends on a very delicate balance of rest, exercise, diet, fluids, medications, stress, and most importantly, sleep.  98% of my focus is currently devoted to getting all of these things right, so that I can devote the other 2% to actually living.  Hopefully, this proportion will improve, and I'll be able to participate more in life and work again in time.

The symptoms I'm dealing with the most this week are:
-migraines
-tension headaches
-fatigue
-lowered consciousness
-loss of consciousness/passing out
-tachycardia
-spells of hypotension (not orthostatic)
-shortness of breath
-temperature dysregulation
-exercise intolerance
-body pain
-digestion (usually C, now more D--(friends with IBS will understand)

When things are so hard, it's difficult to see the improvements.  But they are there!  I'll do my best to think of some, as it is important to see the big picture in these difficult times.
-As of today, I do not need to wear the collar at all times.  Admittedly, I've been weaning for a few weeks.  I'll still be wearing the rigid collar in the car and a soft collar as needed for comfort.
-Excluding the muscle tension/tension headaches, I feel capable of going without the collar for reasonable periods of time.  This is a good sign that I have a solid fusion.
-My swallowing has actually gotten better.  I still have good days and bad days with swallowing, but I'm not getting extremely bloated nearly as often!
-I can sit up for longer periods of time.  For less pain, I generally like to sit on the couch so I can rest my head.  But it's still upright, which is better than before surgery!!!
-Before my setback, I think my overall level of functioning was improved from pre-surgery.  I just have to rebuild to get back to that.  Before the setback, I was able to: get dressed most days, leave the house for short trips most days, socialize for about an hour at a time, and do a fair amount of walking (I had reached 1.25 miles.)

My POTS is not better yet.  I'm not to the point where I was before surgery yet.  That will probably take the rest of this year, or more.  This means that I use the wheelchair at times, and I can't stand for long periods of time.  My aerobic endurance is still very low, but I'm working on it every day.

My headaches are not really better.  I either have a tension headache or a migraine (or both!) every day. Either one can be debilitating, although the migraine is most always debilitating.

I am very lucky to have a supportive family.  I also feel very grateful today for a wonderful primary care doctor back in Illinois, who is helping me get what I need through this transition to living in a new area.

Thanks for checking in with me!  Little by little, I'm coming back.  Sorry this post was a bit scattered.  I'm feeling a bit scattered today.  The migraine doesn't help either.

I heard a really great quote yesterday, so I'll post it here:
"Life is mostly froth and bubble; two things stand like stone: Kindness in another's trouble, courage in your own."
--Adam Lindsay Gordon

*I almost forgot: photos of Charlie!  Charlie is my reason to get better.  My motivation to exercise every day.  I want to be healthy enough to bring him home!

Sick and complaining

First, our new insurance plan started on July 1, but we haven't received the new cards yet, so I can't get any prescriptions or see any doctors right now.

So, on Thursday, I woke up with pink eye in my right eye.  Luckily, I had leftover and not too expired drops from my last case of pink eye, so I started using those, and sanitized everything I could find in the house.

Then, Friday, I woke up with a really sore throat...which is always the first sign of me getting sick.  I came down with a cold, but it wasn't too bad yet.

Friday night, I couldn't sleep too well, due to the sore throat.  Lots of salt-water gargling, lozenges, and Tylenol to get me through the night.

By Sunday, after more sleepless nights, I had a deep chest cough and mostly lost my voice.

Last night, I couldn't sleep again, from the coughing and the painful throat.

I slept a lot today, just trying to recuperate.  Feeling the same, so far.

Of course, since my body is busy fighting off this bug, everything else is worse.  My POTS symptoms are flaring, as well as potential mast cell symptoms. 

Then, of course, I have a sinus headache.  This is, of course, in addition to my regular tension headache.  And, just for fun, I've been fighting off a migraine for 8 days.  Plus, my massage therapist had to cancel, so I've gone a few weeks without a massage, making the headaches all worse.

Now, my hubby contracted my pink eye.  This is all overwhelming.  So sick.  Hoping for some relief soon.  And hoping that my hubby doesn't get my bronchitis/laryngitis thing.  It's hard living out in the middle of nowhere, far from family.  Especially when things are hard like this.

Hopefully, I'll be recovered soon, cause we're getting our new puppy in less than two weeks!

But, every illness is a setback, so I will try not to expect too much too soon from my delicate body.  This was a pretty big hit, so there will be some rebuilding to do.

Avoidance Gets You Nowhere

I have so much to write about.  I keep thinking of new things I need to blog about, but I never get it done.

So, I guess I'll start with a subject I've been trying to avoid for at least a year now.  It's only now that it seems I can't completely avoid it anymore.  I may have another condition.  No, I'm not diagnosis-hunting.  It's just that there is another condition that is now being linked to the whole EDS/POTS/Chiari/CCI group.  And I absolutely would not care for a second unless I were actually having symptoms right now, which unfortunately, I am.  I also would not care unless there was a chance something could be done about this that would improve my level of functioning and quality of life.

That being said, the name of the condition is "Mast Cell Activation Disorder" or MCAD.  I will add some informational links here soon.  In the meantime, if you are looking for information, the disease functions very much like Mastocytosis.  There is not much information on MCAD (also called MCAS for "Syndrome".)  But it is still being treated.  The current understanding is that MCAD is caused by over-reactivity of the mast cells to many benign triggers.  If you've known me for a while, you know that I've had quite a few odd and even dangerous reactions to medications over the years.  You may also know that my throat closes up whenever I'm around cigarette smoke.  These could potentially be mast cell reactions.  You know how I have had a variety of food intolerances over the years?  These could potentially be mast cell reactions.

A seemingly insignificant part of my medical history is my allergies.  As a child, I tested positive for every major allergen.  During college, my allergy symptoms worsened, so I was retested, but found to have no true allergies.  This would be an important part of my mast cell history.

If it were just these things, I still probably wouldn't look into this diagnosis, because who needs another diagnosis?  But, mast cell reactions can also include: persistent fatigue, brain fog, headaches, tachycardia, sudden drops in blood pressure, fainting, cognitive impairment, vision problems, nausea, problems with anesthesia, decreased bone density, shortness of breath, temperature sensitivity, gastrointestinal pain, and bloating, If these symptoms are being caused by inappropriate degranulation of mast cells, treating these overactive mast cells could be an important part of my treatment.

There are a few easy treatments that doctors often try as a way to see if MCAD should be considered.  If you respond positively to these treatments, you probably have some form of mast cell disorder.

The first treatment is with OTC Histamine blockers.  H1 blockers include typical allergy medications, like Zyrtec, Claritin, and Allegra.  H2 blockers also block histamine, but in the stomach.  These include typical antacid medications, like Zantac and Pepcid.  So, to treat MCAD, you start with daily use of H1 and H2 blockers.

The thing is, I've been taking these for a while (since I've had an inkling that this might be part of my medical puzzle), and I was still seeing an increase in symptoms.

So, I often have to take Benadryl, an anti-histamine, to resolve a reaction I am having to a known or unknown trigger.  Since this is happening more often, I decided that I need to start working on figuring out my specific triggers, and avoiding them when possible.

Every mast cell patient has different triggers, so it's important to try to find your individual triggers.  Sometimes, it's obvious what has triggered me, like medication reactions.  So, I need to avoid medications that have caused reactions.  I have known reactions to medications like Morphine, Compazine, and a few others.

A huge mast cell trigger is fragrance.  I have known of my sensitivity to strong fragrances, like air fresheners, perfumes, and hair sprays, for a long time.  But I need to take these more seriously now, and avoid fragrances whenever I can.  So, we're trying to switch over to fragrance free products.

An easy trigger for me to avoid is alcohol, since I've always known I've reacted negatively to alcohol, so I never drink.

The trickier triggers to figure out are food triggers.  The most common triggers seem to be foods that are high in histamine, high in salicylates, or high in tyramine.  This includes a lot of foods.  I only need to identify which ones are my personal triggers, not actually eliminate every possible food.  It's still a bit tricky.  A strict elimination diet would be the most effective way to identify my triggers.  I already eat a very regular and simple diet, so I should be able to figure out some of my triggers.  One obvious group of foods is fermented foods.  So, I have to give up my Greek yogurt.  A couple other very high histamine foods are tomatoes, strawberries, and shellfish.  I can avoid those pretty easily.  The rest of it will be trial and error, which I'm working on now.  Most trigger foods cause me to get an itchy nose and throat, so I am keeping a food diary and taking note of symptoms.  I recently had a pretty dangerous reaction to a protein bar, with my throat getting all itchy and swollen, my airway constricting.  Had to take Benadryl for that one.

Hopefully, this will be all it takes to reduce the frequency of mast cell degranulation, which actually increases your ability to tolerate small exposures.

If these measures don't work, there are plenty of other things to try.  I'm am using NasalCrom, an OTC mast cell stabilizing nose spray, and Ketotifen opthalmic, an OTC mast cell stabilizing eye drop.  If I need further help, I will have to see one of the few doctors willing to treat this disorder.  Other potential treatments include: Singulair, GastroCrom, and Doxepin.  A commonly used medication that is most effective at stabilizing mast cells in the whole body is Ketotifen oral, but it is not yet available in the US.

Okay, I know that's a ton of information.  I've got a terrible headache.  So, that's all for today.  I know this is a ton of information.  This is what I'm working on right now.  I'll let you know how it works for me.

3 Months Post-op

I know: I'm late.  Surgery was March 14, so it's a little more than three months post-op.  This past week, my husband and I moved to a new apartment.  It's a little smaller, so we've been sorting through everything and donating what we don't need.  It already feels pretty homey here, but it is hard being even further from our families.

So, I've missed a lot of updates along the way.  The biggest was our trip to Toledo to see Dr. Grubb's APNP, Beverly Karabin (specialist in dysautonomia).  It was a 6 hour drive each way, but it was worth it.  She validated my post-surgical experiences being most likely related to my dysautonomia.  The surgery, the medications, the bed-rest, the lack of sleep, and the lack of medication management in the hospital all contributed to a horrible crash of my autonomic nervous system.  She said to expect recovery to take nine to twelve months.  Until then, I need to be patient with my body, recognize that it is healing very slowly, and gradually increase activity as I am able.  To help with that, we are experimenting with some new meds.  I'm taking a very low dose of a beta blocker (Propranolol 5 mg) at bedtime.  This is to reduce the heart rate spikes that happen in my sleep, which should improve the restorative quality of sleep.  I think I am sleeping better on it, but I can only handle a very low dose before it starts affecting my blood pressure.  The other change is that I am taking Nuvigil to help with my daytime alertness.  It actually really does help!

I've also been able to discontinue Midodrine and Florinef.  Which means that my POTS is getting more under control.

I'm still having serious problems with muscle spasm in my neck and right shoulder.  Daily trigger point massage is the only thing keeping the tension-induced migraines away.  Unfortunately, we moved away from my physical therapist that was doing dry needling therapy on my trigger points, so we're managing on our own for now (meaning my husband is providing daily massages on all of the rocks in my neck).

I definitely have ups and downs during every day.  If I exert myself, I know I will have to spend some time resting, likely with reduced consciousness.  So, I just have to plan my life around that.  I am trying really hard to keep exercising, which is a challenge since I don't have a gym anymore.  But I do have my recumbent bike, which is the very best cardio exercise for me.  I'm also doing lunges and squats to build up my leg muscles when possible (although this past week, the work of moving has been exercise enough!).  So, every day still has its own challenges.

But, the improvements really are apparent at times.  On Tuesday, the weather was perfect, so Gustavo and I went to explore downtown Madison.  We took my wheelchair, but I didn't have to sit in it the whole time.  We went from the capitol to campus and back.  And we stopped for dinner along the way.  Then, we bought some groceries.  When we got home, I fully expected to crash, but I was actually able to do a little more organizing around the apartment that evening!

So, my endurance really does seem to be improving.  I still have migraine days.  I have times where I'm barely conscious.  But I have plenty of time to keep recovering (although financially, the sooner I can start working, the better).

EDIT: I forgot, I also got my butt kicked by an infection a couple weeks ago.  For a few days, I just couldn't wake up, my bp was really low and non-responsive to meds.  I could barely stand without blacking out.  Thank goodness, we figured out what was wrong (UTI) and it resolved pretty quickly with antibiotics.  Thank goodness we had COBRA insurance this month.  I really needed help to get through it.

Finally, we have put down a deposit on a new puppy, which we're expecting to get by the end of the summer.  I'm so excited!  We're getting a cockapoo.  Training and exercising a new puppy will be a good next step in my recovery, to get me moving again.  After a couple months of that, I hope to be well enough to take on some new piano students.  But I know I need to be patient with my body.  Little by little, I expect to continue to improve.  Long term prognosis is still unknown.  So, I don't know how well I will get.  I don't think I'll ever be very functional in the mornings.  I don't think I'll ever work a full time job.  But I am still getting everything I can out of life.  I am so grateful for these new moments of feeling more alert, more alive, and more capable.  I cherish the good times, and cope with the bad.  My family has been so supportive of me through yet another surgery.  I am so lucky to have them in my life.

Thanks for reading today.  Sorry for the lack of updates.

Great article on living well with chronic illness

http://www.psychologytoday.com/blog/turning-straw-gold/201305/12-tips-12-years-sick

What is Ehlers-Danlos Syndrome

From the Ehlers Danlos National Foundation
Written by Mark C. Martino

More than 1.5 million people around the world have Ehlers-Danlos syndrome (EDS). Someone with EDS is born at least every half hour, and many won’t know what’s wrong with them until it's too late for them to be helped. EDS occurs in between one in 2,500 and one in 5,000 men and women, of every race and ethnicity. EDS was named less than 100 years ago, and it remains largely unknown. Recent evidence indicates it may be one of the most under-diagnosed disorders, because there is no cure and many doctors consider EDS too rare for anyone to have. They fail to see that diagnosis is important, not just in accurately assessing how many have EDS, but in getting help to their patients for the many problems known to be caused by EDS.

Ehlers-Danlos syndrome is a group of disorders. Each type of EDS is defined as a distinct genetic problem in making or using one of the types of collagen. Collagen is used by the body to provide strength and elasticity to tissue; normal collagen is a strong protein that allows tissue to be stretched, but not past its limit, and then is safely returned to normal. WIth badly built or processed collagen, some of the soft tissue can be pulled further than is safe, causing damage. Collagen is the body's most abundant protein and can be found almost anywhere, in skin, muscles, tendons and ligaments, blood vessels, organs, gums, eyes, and so on.

Vascular Type EDS is particularly destructive because of potential arterial or organ rupture; life expectancy is shortened with a majority of individuals living only into their forties. Arterial or intestinal rupture commonly presents as acute abdominal or flank pain that can be diffuse or localized. Spontaneous arterial rupture is most likely to occur in a person’s twenties or thirties, but can occur at any point in life. Cerebral arterial rupture may present with altered mental status and be mistaken for drug overdose. Mid-size arteries are commonly involved. Arterial, intestinal, or uterine fragility or rupture usually arise in EDS Vascular type, but should be investigated for any EDS type.

There is a very wide range of symptoms because of how widespread in the body collagen is, and it is still unclear why one collagen defect can be expressed so differently in people, even in the same family. EDS can cause systemic weakness and instability throughout the body, resulting in many and unexpected effects. Some can be seen, but not all; EDS is largely invisible. Pain, for instance, can be widespread—collagen makes up the fascia, the tissue sheet that holds everything together and wraps around your entire body—but the pain will not show up in MRIs or X-rays, and will be probably be out-of-proportion to any findings. While EDS is not curable, many symptoms can be prevented or treated as they arise: joint hypermobility (loose or unstable joints that slip or dislocate); early osteoarthritis; tissue fragility, skin that tears and bruises easily, blood vessels and internal organs such as intestines that can rupture easily; eye problems and gum disease; spine problems that may also involve the cerebellum and cerebrospinal fluid pressure.

Collagen is found throughout the body. Ehlers-Danlos syndromes come down to a structural problem. An analogy. If you build a house with bad materials, say weak wood or cheap nails, you know you'll have problems. Some problems are more likely to show up than others, but because the bad materials were used everywhere and aren't necessarily visible, you can be surprised by a problem. So the problems resulting from being built out of a protein that doesn't behave the way it should can be widespread, in a wide range of severities, and maybe even show up in places you wouldn't think are related until it occurs to you that collagen is used there, too.

Each doctor you see is going to look at things differently. Endocrinologists see hormones as the problem, or CFS. Rheumatologists in the US think fibromyalgia's the problem. Surgeons see weaknesses that need surgery. And if none of them are remembering that EDS is the root cause, and maybe their solution won't work in that environment of your body, they may be inappropriately treating you. There are some medical specialists, and more with each generation of doctors, but the best suggestion for the longterm health of someone with EDS is to learn everything you can about EDS and what/how it affects you, so you can educate those doctors and nurses who are willing to admit they don't know everything and are willing to learn.

More than a quarter century of experience, free information and resources from Ehlers-Danlos National Foundation can be found at www.ednf.org or reaching the foundation on Facebook. "So You Think You May Have EDS?" is an introduction to EDS written by EDNF members at http://bit.ly/Olw4L, where you can also discover many links to professional and patient publications and web pages through the "EDS Guides" and "EDS Knowledge" menus on the left side, throughout the web site. EDNF holds a national conference, usually each year.

Recovery and Setbacks

Please consider donating to my medical expense fund.  Surgery is expensive.  So is recovery!  http://www.youcaring.com/medical-fundraiser/carolyn-s-surgery-fund/36820   Thank you so much to everyone that has donated!  This wouldn't be possible without your help!!! 

Every recovery has its setbacks, and this one is unfortunately no different.  After a few weeks of relative peace, with my condition gradually improving, this week has been a setback.

I've had a change in my POTS symptoms.  My blood pressure is no longer low.  I don't need Florinef or Midodrine to keep it in the normal range.  This is great news!  Unfortunately, I'm not feeling any better, because I've developed constant tachycardia.  Not just orthostatic tachycardia.  Constant tachycardia.  Perhaps the related autonomic condition called Inappropriate Sinus Tachycardia.  This makes me feel very short of breath all the time.  It really feels like I'm running, even when I'm laying down or sitting.

I don't want this condition to prevent me from working hard on my recovery.  I still need to exercise on the recumbent bike twice a day.  Unfortunately, I'm not able to walk around much right now, because my heart gets out of control.

So, we've booked an appointment with Dr. Grubb's PA, Beverly.  We'll be driving out to Toledo for an appointment on May 20th.  I've just got to survive until then.  This is so freaking exhausting.  It's one thing after the next with this recovery, as usual.

Six Weeks Post-Op

Please consider donating to my medical expense fund.  Surgery is expensive.  So is recovery!  http://www.youcaring.com/medical-fundraiser/carolyn-s-surgery-fund/36820   Thank you so much to everyone that has donated!  This wouldn't be possible without your help!!!

Things are continuing to improve, just very gradually.

I've had two unexpected improvements from surgery:
1. My digestion and food sensitivities have improved a lot!  I seem to tolerate gluten okay, at least small portions.  I can also tolerate small amounts of dairy without a problem!
2. My hormone regulation has improved.  (I formerly had amenorrhea for 1 1/2 years, but that has resolved.)

I don't know how to explain either of these things really, but I appreciate being a little more normal.

Post-surgical issues are still improving gradually.  This includes my POTS symptoms, my muscle tightness, my surgical pain, my muscle weakness, my endurance, etc.

The POTS symptoms are the most challenging, functionally.  These include:
-unstable blood pressure
-unstable heart rate
-lightheadedness
-palpitations
-shortness of breath
-tightness in chest
-extreme fatigue
-exercise intolerance

These symptoms affect my life a lot.  The mornings and early afternoons are still when my POTS symptoms are worse.  Also, if I overdo my exercise or activity level, it may cause an episode later that day, or not until the following day.  I don't get any immediate feedback from my body that I am overdoing it.  So I have to carefully measure and track the amount of activity I have each day, and the amount of time spent upright.

Surgical pain and muscle tightness are still daily (and nightly) issues, and will probably remain issues for some time...especially as I try to become gradually more active.  The pain is worse when I sit up and when I move.  I also have trouble with bumpy rides in the car.  I don't get out much yet.  So I also have to find a balance between increasing activity, and pushing my body too hard.  Sometimes, I overdo it, and the pain becomes too much.

I finally got my bone growth stimulator today!  It is an electromagnetic device that I wear over my collar.  I am to wear it for four hours a day, for several months.  I will continue to wear it until I have x-ray confirmation that there is a successful bony fusion.

Thanks for still caring, after all this time. 

I have Chiari; why should I be concerned over EDS or Tethered Cord?

Compiled by Robin Armstrong Griffin

When I hear a member in the support groups is diagnosed with Chiari the next question they often ask is why then should I concern over Ehlers-Danlos Syndrome (EDS) or Tethered Cord (TC)? Isn't chiari just caused by a small skull so the brain is forced down?

Chiari is a hind brain herniation of the cerebellar tonsils/lower brain down out of the skull and into the neck.  It is caused by a malformation of bones within or near the skull or the pulling from below by Tethered Cord Syndrome, (explained below). In other words, chiari is a symptom caused by a malformation/underlying condition of something else. And a small skull is not the only condition that can be present to cause it, which many doctors feel is the case. So knowing the true underlying condition is key to the proper treatment of your Chiari.

Ehlers-Danlos Syndrome (EDS) is a connective tissue disorder. Connective tissue acts like "glue" in the human body, holding everything together. Collagen, a protein that adds strength to the connective tissues, is not produced correctly in an individual with EDS. The resulting weak connective tissues are too weak to hold the organs, ligaments, joints, etc.of the body in place. Ehlers-Danlos Syndrome is currently separated into 6 major types. Hypermobility, Classic, Vascular, Kyphoscoliosis, Arthrochalasis and Dermatosparaxis. More and more individuals with Chiari are being found to also have EDS and/or Tethered Cord (TC). (The EDS and/or TC being the CAUSE of the hind brain herniation/chiari.) Although at risk for any type, Hypermobility or Classic are seen the most, some the more rare Vascular type.

Individuals with Ehlers-Danlos Syndrome (EDS) are at risk for cranio-spinal instability (CSI), which often results in cranial settling. In cranial settling the tissues in the neck are so weak (due to EDS) that the cervical bones are not held in place. Slumping or collapsing onto itself occurs. These usually leads to abnormal angles/malformations of various bones and this can lead to both anterior and posterior brain stem compression. Many chiari surgeries (Decompression) fail because only the posterior brainstem compression was addressed. And since most have an MRI laying down the true picture isn't seen because the patient wasn't upright for gravity to reflect to true image. Hence the importance of upright MRI's. These malformations force the cerebellar tonsils out of the skull into the spinal column through the small hole at the base of the skull called the foramen magnum, a phenomena called cerebellar herniation, AKA Chiari. When the cerebellar tonsils are out of place, they take up space normally allotted for the cerebral spinal fluid (CSF), the brainstem, and all the arteries and veins leading through this area to the brain. Blockage can be both posterior and anterior. The cerebellar tonsils are the lowest part of the cerebellum, which controls the sensory perception and motor control systems of the body. The brainstem controls the autonomic nervous system, or everything your body does without you having to think about it, heart rate, respiration, blood pressure control, etc. Damage to these systems is very serious. CSI may also allow the Odontoid bone at the front of the neck to angle into the brainstem (Retroflexed Odontoid), causing even more concerns, such as Basilar Invagination or Basilar Impression. (Brain Stem Compression).

Postural Orthostatic Tachycardia Syndrome (POTS) is a type of dysautonomia. It is also commonly seen with individuals who have EDS and EDS with Chiari as a result of brainstem concerns. In addition to this, an individual with EDS and instability is at risk of subluxation (partial dislocation of bones) of the neck or abnormal sliding of the skull in flex/extension, further compromising the brainstem, tonsils and vertebral arteries. Making this life threatening. When you flex and extend your head, your skull should shift up to 1-2mm, with instability your skull can exceed that, (Stephens' is 8mm) further endangering the individual and for some is even life threatening.

As shared from Dr. Clair Francomano at Harvey Institute of Genetics. "The term "Chiari Malformation" is used to describe the situation where the cerebellum, a structure at the base of the brain, is extending through the opening at the base of the skull, which is called the foramen magnum. The extension through the foramen magnum is called "herniation." This can result from cranial settling, which "pushes" the cerebellum through the opening, or tethered cord, which pulls the cerebellum from below. The conventional definition of a Chiari Malformation states the cerebellum tonsils must protrude at least 5mm below the foramen magnum. However, patients with less extreme herniations can also experience significant neurological symptoms involving the brain stem and cerebellar functions.

A wide variety of neurologic symptoms include headaches, neck pain, tinnitus, swallowing difficulties, visual disturbances, a sensation of pressure behind the eyes, autonomic nervous system dysfunction (POTS, neurally mediated hypotension), sleep disturbances, brain fog and memory issues may result from Chiari Malformations.

Another complication of hereditary connective tissue disorders is called occult tethered cord. In this condition, a band of connective tissue called the filum is wrapped around the base of the spinal cord and exerting pressure on the nerves that go to the legs, pelvis, bladder and bowel. This condition may cause numbness in the legs and pelvis area as well as severe incontinence as well. Because of the hyperextensibility of the connective tissue in patients with this disorder, the filum may become stretched out over the years and is not visible on an MRI. This is why it is called "occult", it cannot be seen using current imaging techniques. Diagnosis is usually made based on clinical findings and also the consequences of tethering which may be seen on a cervical and brain MRI, affecting the angles between specific landmarks in the spine and skull (Milhorat TM et al. Association of Chiari Malformation type 1 and tethered cord syndrome: preliminary results of sectioning filum terminale. Surgical Neurology 72: 20-25, 2009). At this time, it is not known why this condition occurs more frequently in patients with hereditary disorders of connective tissue."

The problem is if you have EDS and it isn't known and you have decompression, ESP with laminectomy, it adds instability. This can greatly effect the success of the decompression because you've added more instability and only addressed the posterior issues and not the anterior issues often seen with cranial settling. Often times those with EDS & Chiari need fusion done at the same time or known to be needed at a later time or tethered cord release done first or soon after. Many also chose to wear cervical collars to help with the instability symptoms and pain. The decompression alone often treats only a part of the issue, not all and even if CSF flow is restored posterior the pain and neuropathy issues still remain due to cranio-instability and continued brainstem compression in other areas or tethered cord still pulling the tonsils from below. Thank you for reading!

Resources: Dr. Clair Francomano, Harvey Institute of Genetics and www.MedicalZebras.com

1 Month Post-Op

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This has been a really interesting time.  Last Monday, we had to let go of our sweet dog, Jango.  He lived a very happy life, but was in a lot of pain at the end.  I am glad that we could at least relieve his pain.  He was a wonderful spirit, full of joy, and we miss him dearly.

As part of the healing process from this loss, we decided to get a new puppy.  He is a delight, and helps to fill the emptiness that we are feeling.  His name is Dexter.  He is an 8-week old Coton, and he loves to cuddle!

I got my stitches out on Thursday.  What a relief!  The stitches were very embedded after a month in my head, so it took quite a while to work them out, but it feels so much better now.

My dysautonomia is my biggest problem at this point.  My body is still very unstable and unpredictable.  My heart rate jumps up, my blood pressure drops, or I become suddenly short of breath or have heart palpitations.  I also experience reduced consciousness at times...particularly in the early hours of the afternoon.  I am on several medications for this, but they don't always help.

I am exercising daily to try to improve the symptoms of dysautonomia.  I have to exercise sitting down, so the recumbent bike is perfect.  But I have to be very careful.  If I overdo it one day, the next day may be a very sick day with low blood pressure and reduced consciousness.  So, I have to be very careful about exercising the same amount every day, and barely ever increasing.  At this point, I can do 11 minutes, twice a day.  This will probably take months to improve, with a lot of hard work and persistence.

The pain is still being managed with Tylenol.  I'm taking 1500 mg of acetaminophen a day, only 3 tablets (when 8 is the daily maximum).  This keeps most pain manageable.  I've still had a few migraines that require separate migraine medicine.

The muscle spasms are still the biggest cause of pain.  I still cannot tolerate any muscle relaxants.  So, I'm using heating pads, topical creams (China-gel), and massage to relieve the pain.  The pain still feels very similar to migraine pain, but in a slightly different location than a normal migraine.  I can usually tell them apart now.

My eyes were injured during my 16 hours under anesthesia.  I got large corneal abrasions across each eye.  The intense pain only lasted a few days.  But I still get burning, dry eyes many times a day.  I'm not ready to wear my contact lenses yet.  I believe they are healing, but not healed yet.

I also developed large areas of numbness on each thigh from surgical positioning.  The numbness was complete at first--no sensation for temperature, sharp/dull, or pressure, besides very deep pressure.  Gradually, the feeling returned, but these areas became hyper-sensitive to touch and quite painful.  These areas are still healing.  The pain is decreasing.

Ready for the good news?  My digestion seems to be improved since surgery.  I am having less food sensitivities and less digestive disturbance and pain.  This is an unexpected improvement!

My swallowing is still improved, but no longer perfect.  I still have aerophagia at times with large amounts of water.  It is usually mild, currently.

So, given the complexities of this recovery, here is what daily life is like for me right now:

I need to sleep about 11 hours each night.  That's about 11:00 pm to 10:00 am.  I wake up once each night.  As long as I can get back to sleep, I'm fine.  If something disturbs my sleep, my dysautonomia symptoms will flare the whole day.

My mornings are a bit better recently.  If I take my morning meds right away, including my salt and extra fluids, my blood pressure is usually pretty good, and my heart rate is mildly elevated.  I need to spend the first few hours of the day mostly horizontally.

After lunch, I seem to have a flare of autonomic symptoms.  My heart starts racing, my blood pressure drops, I become short of breath, and I experience reduced consciousness, or even temporary loss of consciousness.  We manage this as well as we can with medication, salt, and elevating my legs above my heart.

On a good day, these symptoms will improve by dinner time.  I can sometimes eat dinner at the table, something I could never do before surgery.  Sitting up in a chair has always worsened my symptoms.  So, I think this is a really good sign for my long-term recovery.  I also get my exercise on the recumbent bike at this time of day.  I still sometimes get shortness of breath or an elevated heart rate, but I usually stay fully conscious.  I can spend some time upright during this window.  I still have to rest frequently.

I still get frustrated by my symptoms, especially on bad days, where I feel like I'm not getting better at all, or when I spend a lot of time really out of it.  But overall, I am satisfied with my progress so far.  I just have to keep reminding myself that recovery takes a very long time, and this is just the beginning.  I know there are good times ahead for me.  A new puppy helps!  I really look forward to being able to move back in with my husband, even though my mom and step-dad are taking such good care of me!

Thanks to everyone for caring and supporting me.

Pics from surgery

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Here's some photos of hospital and home recovery.  As always, I can't seem to control the order of the photos.  So it's more of a collage.  Recovery is slow, but I do see some tiny improvements overall.  I'll update more tomorrow, after my stitches finally come out!!!

3 weeks post-op

Please consider donating to my medical expense fund.  Surgery is expensive.  So is recovery!  http://www.youcaring.com/medical-fundraiser/carolyn-s-surgery-fund/36820   Thank you so much to everyone that has donated!  This wouldn't be possible without your help!!!

______________________________________________________

Today marks 3 weeks since my operation.  Yesterday, I got out of the house for the first time--went to Portillo's for dinner with my mom and stepdad.  There's a lot of sensory overload in the outside world, but it was really good to get out.  This would have been inconceivable just one week earlier, when I was barely conscious all day, wondering if I would ever be well enough to get out of the hospital and get back home.

Today, my mom washed my hair--carefully, with the bandage covering up the incision.  The stitches are still in for one more week.  Can't wait to get them out!

I've been gradually building up the amount of active time each day.  I have to be so careful, because if I overdo it, especially in the early hours of the day, I pay for it later with reduced consciousness.  But I have started getting some things for myself (some of my food and water refills).  I've also gradually built up my time on the recumbent bike.  Today I rode for 11 minutes, two times.

My tachycardia is improving very gradually.  But my blood pressure has been stable since discontinuing all muscle relaxants on Tuesday.  That means I've been conscious all day for three full days!  :)  I'm not saying I'm 100% alert all day, or that I would make a great conversationalist at noon.  But, I'm awake, aware of my surroundings, and able to respond when called all day.  (This is with the help of Midodrine and Florinef, along with salt pills, to keep my blood pressure up.)

The muscle tightness in my neck and shoulders is still problematic, and quite painful.  I haven't had a full-blown migraine since Sunday.  Just migraine-like pains from the muscle contractures.  I'm trying to arrange to have dry needling sessions in May to alleviate this.  Until then, it's heat packs, icy hot, and massages.

I've been noticing that I sometimes have started swallowing air with my water again.  I'm trying to ignore this fact for now.

The most difficult thing has been that my beloved dog, Jango, has become very sick, suddenly.  He has some neurological symptoms, and he frequently cries out in pain.  I have cried most of the day today, because it is so hard to watch my happy little buddy become so miserable.  We know there isn't much time left for him.  Watching Jango in pain gives me even more appreciation for my caregivers.  Watching someone you love suffer is so painful.  And my caregivers are amazing!  I am so lucky to have such wonderful support.

I am also grateful every single day that I survived this whole ordeal.  This hospital stay did some damage to me, physically and psychologically.  But healing is possible.  I will get stronger.  The psychological wounds may take a bit more time to heal...

Sorry to end on a down note.  I am an emotional wreck.  But I am so happy to see little improvements in myself each day.  Recovery is slow, as expected, but improvements are still noticeable.

Unpredictable Recovery

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It's not like this is my first major surgery.  But it seems like it, since my body is reacting so differently this time.  So many unexpected complications and problems.  The primary unexpected problem has been adverse reactions to medications that I tolerated only 3 years ago.  I have developed severe episodes of hypotension/loss of consciousness after taking several medications.  The medications that have caused this problem in the last 3 weeks are: Morphine, Oxycontin, Flexeril, and Zanaflex.  Basically, the good pain meds and the muscle relaxants are all out.  That makes recovery very challenging!!!  But these episodes are so scary, for me and for my family, that I am scared to take anything for pain and muscle spasms.

It is really hard to face this recovery without pain meds or muscle relaxants.  The muscle contractures and spasms are so severe that they are triggering migraine-like pain and actual migraines.  So, I'm in a lot of pain most of the time.  I use heat on my neck and shoulders, along with topical creams and massages.  If anybody wants to give me a massage, any day of the week, I welcome it!  I wish I could afford daily massage therapy.  It helps reduce the pain dramatically.  But, of course, insurance doesn't cover my massage therapy.  Also, when I am well enough to go back to Milwaukee, I have a physical therapist that does dry needling therapy on my muscle contractures.  I'd really like to get back to her as soon as possible.

The last complication to deal with is the POTS symptoms.  I wake up with my heart going 105 bpm.  It peaks around 140 standing, 120 sitting.  It's worst in the mornings, then gradually decreases throughout the day.  By the evening, my heart rate is at a happy 72 bpm, and I can finally catch my breath.  I'm taking Florinef and Midodrine for my POTS.  And I'm using the recumbent bike to try to improve my conditioning.  I also take salt and high fluid intake throughout the day...all just to get my cardiovascular system to calm down.  The POTS will take months to improve, but I've gotten better before, and I can do it again.

I haven't ever had to recover from this major surgery without pain meds and muscle relaxants before.  So, that is my new challenge.  It is a long, long road.  So, I'll just keep going.  My family has been amazing with their support.  I am so incredibly lucky to have them to help me through this.  Thanks to them, my attitude has remained quite positive most of the time.  As long as I can remain conscious throughout the day, I feel like I can get through anything.  Although I really hope to not get another migraine that reaches a 10 on the pain scale.  That would bring anyone down.

Home from Surgery

I have never been so glad to be home!

My Cranio Cervical Fusion Revision on March 14 did not go smoothly.  It was the most harrowing experience of my life.

There were several complications.  It started off with a difficult intubation.  Since my neck is already fused, I have to have a conscious fiber-optic intubation.  I remember the difficult process clearly.  They spray your throat to numb it, first.  Then, they try to put the breathing tube in while you're still awake.  It apparently did not go well.  My throat was really torn up in the process, causing a lot of inflammation.  Due to this inflammation, they had to keep me under anesthesia for 7 additional hours, after a 9 hour operation.  Being under for 16 hours was a really bad start.

I woke up at 4 AM the next day during the extubation, which was horrible.  But as much as my head and neck hurt from surgery, and as sore as my throat was from being intubated, my number one complaint the first day was my burning eyes.  By the end of that day, I had an explanation, which was that during the long operation, I developed corneal abrasions all the way across both eyes.  The pain was unbearable.  My eyes are still red and healing now, 12 days after surgery.

My POTS (Postural Orthostatic Tachycardia Syndrome- a form of autonomic dysfunction) got a lot worse after my long surgery.  There were multiple triggers for my POTS, including 16 hours of anesthesia, changes in medication, fighting off a virus, and a lot of sedentary time.  So, my heart rate and blood pressure became very unstable, with episodes of very high tachycardia and quite low blood pressure.  My heart is also very reactive to every movement I make.

The hardest part came days 5-7 in the hospital.  After making some progress in terms of moving around and starting to heal, I spent Tuesday through Thursday fighting for consciousness.  I never got a solid explanation for what was wrong.  Could have been severe POTS or a reaction to the pain medication (even though I only took pediatric doses of anything, and I've tolerated these meds fine in the past).  I felt like I was under a heavy blanket of fog that I couldn't get through.  These days were so long, and the darkest days of my life.  I felt my heart straining, with a constant feeling of palpitations.  There was a fear at this time that I would never fully regain consciousness.  The doctors really never explained what went wrong.  I kept telling my family that I didn't want to be intubated again, and I didn't want a feeding tube.  "No Tubes!"

Thankfully, on Thursday, late afternoon, I started to come out of it.  A substantial part of waking up was my husband deciding to give me my usual dosage of salt for my POTS.  They didn't give me this at the hospital.  I became a bit more alert and aware of my surroundings.  I did my PT.  I got the last of 4 drains removed that night.  I was released on Friday afternoon-9 days in the hospital.  Due to these complications, my family had to extend their travel plans.  We had expected to fly home by Thursday, but couldn't leave until Saturday.  So, flights had to be changed (which was incredibly expensive) and hotel and car reservations had to be extended.  I was just so relieved to be getting out, after thinking that this hospital stay may have lasted for weeks.

Our travel day was difficult.  Our flight was delayed, so we didn't get home until 3 AM (which felt like 4 AM to us).  The pain was intense.

Ever since Tuesday (4 days after surgery), I had to switch to taking only Tylenol for pain.  Any other medication could have potentially contributed to my reduced consciousness. The pain is intense.  The Tylenol brings it to a just tolerable level, most of the time.  The worst pain is where my old rods were removed (using all sorts of fun tools like bone saws and chisels).  There was a lot of destruction in those areas, and a lot of healing is needed.  I have a constant ache with frequent sharp pains in these areas.  The other major pain is from muscle spasms in the neck and shoulders.  This will take months to loosen up, with massage therapy and dry needling.  The muscle spasms cause migraine-like headaches.

Currently, my days are really unpredictable.  I'm still having trouble with: skull pain, muscle spasm, tachycardia, episodes of reduced consciousness, burning eyes, numbness in various areas of my body, and regaining bladder control.

My POTS is so severe that I require salt pills and water spread throughout the day (basically like receiving IV saline all day).  If I don't take the salt, my tachycardia gets worse, I get short of breath, my lungs start burning, and I tend to lose consciousness.  Thermotabs are the only thing keeping me going at this point.  Hopefully, my dependence will be reduced over the next few months of recovery.

With so many complications, it will take some time to see improvements to be gained from this terrible ordeal.  So far:
-Swallowing seems to be improved--I can swallow water without swallowing excess air, and I can swallow pills without contorting into strange positions.
-Pupil reflexes are sometimes improved.
-POTS is worse, for now.
-Migraines are the same--I've had 2 migraines since surgery--The first was day 5 in the hospital.  The second was our travel day and the following day.
-Pain and muscle spasm are worse, for now.
-Balance may be improved.
-Digestion seems to be improved--I'm still experimenting, so I'll update more on this once I know more.

Current plans:
Staple removal is this Thursday (just 2 staples where my last drain hole was).
Stitch removal is 2 weeks from Thursday.  (They want them in for 4 full weeks.)
No lifting of anything over 5 lbs. for 6 months!  It makes me feel completely helpless, but I'm not willing to risk the perfect positioning of this fusion.

I have been traumatized by this horrifying surgery, more than any past surgery.  I get a wave of nausea thinking about hospitals and doctors.  And I have an intense fear of losing consciousness again, feeling that I'll never be able to wake up.

So, I really hope this fusion last, because I'm not going back.  I refuse.  I'm too scared.  I spent my 20's having surgery, needing surgery, recovering from surgery.  I'm hoping to spend my 30's focused more on living.

Wonderful things about being home:
-I get to take my normal daily medications that they did not give me in the hospital, including my anti-depressant and my anti-anxiety meds.  I will never understand why they don't administer these meds in the ICU.  It's the most stressful time of your life, and your emotions get completely out of control without your daily meds that help you to stay stable.
 -I don't get woken up every hour of the night for: blood pressure checks, Heparin shots in my stomach, finger pricks for blood glucose checks, bright lights in my eyes to check my pupils, and medication that I definitely don't need to take in the middle of the night.  I get to sleep as much as I want, which makes my POTS more manageable.
-I get to eat real food.  Seriously, the first night out of the hospital, it was like a flavor explosion in my mouth!
-My dogs are here :)

Thanks to Dr. B, surgery has been approved, and we can go forward with our original plans.  Fly to NY on Tuesday, pre-surgical testing at the hospital on Wednesday, surgery on Thursday.

Surgery Coverage Denied!

Very bad news today.  Insurance called me and informed me that surgery is not authorized.  I will spend the next 2 days making phone calls, but it is very likely that surgery will need to be reschedule after approval is attained.  I cannot believe this is happening.  Everything is already set in place.  Flights and hotels are booked.  Family has taken off of work.  I've discontinued piano lessons with my students and packed all of my stuff to move back in with my mom.  Everything is planned around this surgery date.  I am officially freaking out, and I feel completely helpless at this time.  It will be up to my surgeon's office to convince my insurance company that surgery is a medical necessity.  I don't know how to handle this one.

The Countdown is On

17 days until surgery!!!  Yikes.

Here's a fun list of our expected medical expenses for this year:

Surgery with an out-of-network provider at an out-of network hospital
Flights to and from New York for surgery
Physical Therapy before and after surgery
Lodging for 10 days in New York
Rental car for 10 days in New York
New Aspen Collar
Massage Therapy after surgery
Dry Needling after surgery
DEXA scan before surgery

Also, we found out that our insurance premiums, deductibles, and out-of-pocket maximums have all been doubled this year.  In addition, our out-of-network coverage has been lowered to 70%.
















If you are able to help in any way with these costs, it would mean so much to us.  My medical fundraiser page can be found here:

http://www.youcaring.com/medical-fundraiser/Carolyn-s-Surgery-Fund/36820

We are so grateful for your support!

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Updates:

Last week was torture.  I had a migraine with pain level between 8 and 10 for five days straight.  It killed me, and made me really scared for the upcoming surgical pain.  But also hopeful that days like these may be less once my skull is stabilized.

Today, I had my DEXA scan.  A simple test that consists of a few x-rays that is used to determine your bone density.  In a week, I'll get the results to confirm that my bones are still strong enough for surgery.

This week, I have to say goodbye to my piano students, which breaks my heart.  I won't be living in Milwaukee ever again!  I'll spend recovery in Illinois, then move to Madison, with my husband.  So I have to leave my students behind, which is really heart-breaking.

Next week, I have a doctor's appointment for surgical clearance.  This is basically a very thorough physical with a complete blood work-up.

We leave for New York on March 12.  Pre-surgical testing at the hospital in NY is the 13th.  Surgery starts at 7:30 AM on the 14th.

I'll update again soon, as my thoughts are more and more consumed with anxieties, fears, maybe even a few hopes?

Hope things are going well, and thanks so much for checking in!

A Change is Coming

My surgical fundraiser page:
http://www.youcaring.com/medical-fundraiser/Carolyn-s-Surgery-Fund/36820

"Any change, even a change for the better, is always accompanied by drawbacks and discomforts." —Arnold Bennett

So, emotionally, I’ve been dealing with the quickly approaching major life changes.  In March, I will move out of my home with my husband, and back in with my mom and stepdad.  I will have to leave behind my small piano studio and my students.  When my surgical recovery is complete, my husband will have moved to a new home (location yet to be determined).  So I will not be able to return to our home or to my piano studio.  When I leave in early March, I have to plan to live in a new home for at least 6 months, away from my husband.

In the middle of March, I will travel to New York for 10 days for my surgery.  As always, this surgery brings a lot of anxiety.  There is, of course, the expected surgical pain and post-surgical complications.  I have a fear of the moment when you wake up from surgery to your new reality; the pain and new sensations that you feel, and will continue to feel through the healing process.  Along with this fear comes the hope of improvement with healing.  After four major neuro-surgeries over the past 11 years, I know not to set my hopes too high.  There is a potential for dramatic improvement, and a potential for great disappointment.

So, what is reasonable to hope for?

Not a relief of all chronic pain.  This has multiple causes, including EDS-related body pain and chronic hereditary migraines unrelated to cranio-cervical instability.

Not a relief of all POTS symptoms.  POTS is also associated with EDS.  It may improve, and it may stay the same.

Fatigue will probably linger, as well as food sensitivities, IBS, back pain, and various other symptoms.

What can improve?

Any symptoms related to cranio-cervical instability have the potential to improve.

The biggest ones I am hoping to see improvement of are the periods of stupor, reliance on the collar and cervical traction, and increased endurance for upright activity.

Other symptoms that could potentially improve are:

-episodes of nausea

-enlarged pupils

-swallowing problems, including choking and aerophagia (with associated bloating)

-nighttime one-sided weakness and paralysis

-double vision, difficulty focusing eyes

-jaw pain and function

 -dizziness and balance

Okay, I’ll stop there.  I don’t want to get carried away. 

The surgery will take many things from me.  I will miss out on at least 6 months of my life for this.  I will undergo more traumatic times in the hospital and pain and discomfort after surgery.  I will not be able to live with my husband for at least the first 6 months.  Also, depending on where he moves, I may have to endure much of the recovery without seeing him.  But l know that it will be worth it when I think through the things that should get better, and the many, many things that could get better.

If I have improvement in just a few major symptoms, like my upright endurance, my periods of stupor, and my dependence on traction, this surgery will be worth it.  With those improvements, I could live a much more functional life.  I could have more than one hour upright every day.  I could probably teach more piano students and spend more time with friends and family.  That would make it all worth it.

So, I’ll do everything I can to make that happen.  I have to try.

Currently, my focus is on scheduling pre-surgical testing and getting insurance issues worked out.  The cost of the surgery is so daunting, since so much of insurance coverage is unknown.  I’m also just trying to enjoy the month I have left of my regular life.