Tuesday, August 8, 2017

Another ordeal

The last few weeks, I feel like I've been through another endless ordeal, where one thing after another goes wrong without a break.

It started July 20, when I woke up with what felt like a corneal abrasion. I have experience with this, because after my 2013 surgery, I woke up with 2 corneal abrasions. They were very severe, much more pain than the surgery had caused, pain that was untouched by my post-op opiates. This was clearly not that severe, but I would consider it a moderate corneal abrasion. Pain was a level 9 initially. I had severe burning, stinging, tearing, and nasal dripping. I was unable to keep the eye open for three days. The pain and the sinus leakage triggered a mast cell reaction and a migraine. It was horrible.

On July 21, I woke up with a milder corneal abrasion in my left eye with a pain level 7. So, I had severe burning and tearing in both eyes. It hurt to open my eyes or look around. Reading was impossible. So was watching TV and playing games on my iPad. My eyelids were swollen and red. The facial pain on the right side became worse with the barometric pressure dropping and storms outside, so I had a consistent pain level 8.

On July 22, there were more storms and low pressure, so the facial pain continued at the same level. I also still had some photophobia and difficulty opening my eyes.

On July 23, the pain was lessening, although my eyes were still burning. Unfortunately, I was exposed to perfume in a restaurant (we risked going out to celebrate our wedding anniversary). I developed left facial pain that started gradually and worsened overnight.

On July 24, the left facial pain was worsening (from the perfume exposure). I then was also exposed to hospital scents for a doctor's appointment that day. The left facial pain was an 8 by the time we got home. The pain persisted and worsened to a 9 by bedtime. I also had burning in my chest and face (with a temperature of 99.0-which is elevated from my baseline by a couple of degrees).

Over the next few days, there were no new exposures, but my whole body was in pain and exhausted from all of the reactions. And the facial pain persisted at a level 6-7. The eye burning continued, as well. I now believe I have developed chronic dry eyes. I have been using gel and drops many times each day and night.

On July 28, I had widespread body pain worse than usual. I also had nausea, facial burning, and lethargy. Then, in the afternoon, I developed a spell of extreme weakness and reduced consciousness.

On July 29, I decided to try my compounded Zantac (cornstarch in a gelatin capsule) in the afternoon. It was  rough day for various reasons. I had another spell of reduced consciousness/weakness in the afternoon. Late night, I developed left facial pain and a headache. Very late at night, I was extremely emotional with hysterical sobbing until 3:00 AM (probably unrelated to the Zantac trial).

On July 30, I slept for four hours, then awoke with a pounding heart and was unable to sleep more. I had lingering facial pain.

On July 31, I slept okay, but developed a burning face and diarrhea. For some stupid reason, I decided to try another compounded Zantac, to try to confirm which symptoms were related to the Zantac.

On August 1, I only got 5 hours of sleep before waking with low blood pressure (88/55) and a pounding heart which lasted for hours, unable to sleep anymore. I then developed abdominal cramping, abdominal pain, bloating, and diarrhea. I also developed new hives on my chest and face.

On August 2, the Zantac reaction persisted. I had more abdominal cramping and diarrhea, plus burning face. I also had burning, stinging eyes that day, along with lack of balance. Then, I risked cheating on my diet and ate half a taco. It was just chicken in a tortilla, but I developed burning lips and chest, itching throat and ears. I had to take a Benadryl. The extra anti-histamine dried out my eyes too much, and I ended up with another eye injury.

On August 3, it was a stormy day, so I had head and facial pain and nausea from the storm. Unknowingly, I also started a new formulation of Omeprazole that day. (This has been a daily medication for a long time with no known side effects for me. But my pharmacy mailed a different manufacturer with a totally different list of ingredients, 15 new ingredients that were not in the previous formulation.) That day, I developed abdominal cramping and gas, and diarrhea again.  It was a severe cramping sensation in my upper middle stomach area that lasted all day and night. There were also storms that day, so I had a throbbing temple going on as well.

On August 4, I was still unaware of the change in Omeprazole, so I took it again. I had continued abdominal pain all day and night. My stomach became very tender to the touch, unable to tolerate a bra or a waistband. My diet had become mostly saltines and rice.

On August 5, I finally realized that the Omeprazole was probably the new culprit. I had continued abdominal pain all day, but no diarrhea. Without Omeprazole, I developed severe heartburn, for which I took baking soda in water many times to reduce the acid. I also experienced severe bloating.

On August 6, the abdominal pain was less, but still persistent. I went back to my normal diet and requiring Miralax. I had a lot of bloating and was still very uncomfortable. I tried OTC Prilosec for the GERD, until my pharmacy can sort out getting me a new prescription for the previous manufacturer of Omeprazole.

On August 7, the upper middle abdominal pain has still persisted. The bloating and cramping have, too. The pharmacy is arranging for the safe Omeprazole to be ready tomorrow. I really hope everything will calm down after that.

My thoughts:
Diarrhea is a really unusual symptom for me, since I have chronic constipation (since age 18) requiring daily doses of Miralax. So, when I have diarrhea without Miralax, my system is really upset and irritated. I really hope my digestion can calm down soon. I had gotten to the point where I was pretty regular and digesting my food without too much trouble. But now, I'm back to major GI symptoms throughout the day. I would love to load up on probiotics, but I don't have one I can tolerate right now (although I just ordered a new one to try, when I'm up for trying something new).

After 3 weeks of almost constant reactions and symptoms much above my baseline, I am really ready for a break. I desperately want to avoid all triggers and give my body a rest. I won't be trying anything new this week.

This means I still have a few medicine trials to get through before my next immunology appointment. I still need to try the Zantac again, as well as the Ketotifen, but without the capsule. There is a good chance I am reacting to the gelatin capsule. The difficult thing about that is that you can react to some gelatin but not others. The same is true for cellulose as an ingredient in medicine. Some is from wood and some is from cotton. It's possible to tolerate some cellulose and not others. But none of it will be labeled, so it's almost impossible to sort out.

I am still very scared to try anything new, and I desperately hope I can get back to a comfortable baseline before I try it.

I haven't been able to wear my contacts in three weeks due to burning, stinging, sometimes tearing dry eyes. Unfortunately, my glasses give me a headache, so I really hate wearing them all day every day, and I am totally blind without them. I should probably try to see an ophthalmologist, but getting to appointments is difficult, they are always scented, and who knows if I would even tolerate the treatment. I will try to do this when things calm down (I have a long list of things to get done if things ever calm down!).

I have identified a few new triggers recently.

*First, I realized I was getting burning in my chest from drinking cold water or eating hot food. Everything needs to be room/body temperature, which is a real pain and generally less enjoyable.

*Then, I realized I was reacting to blueberries. After eating about 1/3 cup of blueberries, I would develop a spell of reduced consciousness/extreme weakness for at least an hour. These have been happening for a long time, but are still very scary spells for anyone around.

*Next, I replaced my blueberries, which were my only fruit, with grapes. For a while, they seemed to be pretty safe. Maybe, I got too bold in my portion size. But I started developing the same reaction to grapes. Now that I have removed all fruit from my diet, I am not having these reactions anymore. Which is a relief, but really sad. I love fruit! I am hoping finding the right medicine for my mast cells will help broaden my diet.

*Then, I developed a contact reaction to potatoes. Wilma has potatoes as a main component of her diet. Unfortunately, the process of peeling and chopping potatoes now triggers itchy hands and face.

*I know for certain that I am reacting to one of three ingredients in my compounded Zantac (Ranitidine, cornstarch, and gelatin) with a prolonged, multi-stage reaction. Trying it twice was painful, but did make it clear which symptoms it was causing.

*I now know I cannot tolerate a specific generic Omeprazole (Kremers Pharmaceuticals). I now think of my previous brand as the only safe brand, since it's impossible to sort out which ingredient is triggering me in a pill that contains 20+ ingredients. I have to find a way to make sure that my pharmacy only ever fills this manufacturer.

It's hard to see these new reactions develop, because I have to acknowledge that my mast cell disease is continuing to progress. Hopefully, some of the new compounded medications without the capsule will end up turning things around for me. Because it feels like nothing can stop this progression.

It was also really sad to be so symptomatic during my husband's vacation week (although it was really only a few days, since he was at a conference). It was rough on us to try to find something to do for our anniversary, and have that cause me so much pain in the end, too. We did make it to a beach. It was a handicap-accessible beach, so I was able to go in my wheelchair. I even went in the water a bit, although I lost circulation to my fingers, since the water was so cold. We didn't stay long, but at least we can say we got out.

I hope everyone is doing well. I'm still here. Still plugging along. I've had some really depressed days, but otherwise I'm managing. Just exhausted from the constant fight.

Saturday, July 15, 2017

July 2017

I have a bunch of posts in mind to write, but there is enough going on that a standard update is needed first.

I had some major reactions in June.

On June 19, I got a bug bite. I didn't even notice the bite itself at first. Just my body's reaction to it. It was the day I developed a fever of 101.5, severe joint and muscle pain, head/facial pain, abdominal cramps, nausea, absent appetite, tachycardia/pounding heart, low blood pressure, reduced consciousness with intermittent loss of consciousness, and marked weakness. At the time, I thought it was a reaction to going to a doctor's office. I now believe this was mainly a reaction to a spider's venom, followed by an infection, complicated by a mast cell reaction.

When I saw a GP one week later, I still had a fever, and the bite was rather grotesque looking. For fear of triggering another reaction, I delayed taking the prescribed antibiotic for a day. Very luckily, my fever went away, and the bite started quickly fading. Since then, the fever has not returned (thank goodness). I know many people will suggest this, but I'm told this didn't really follow the pattern of a tick bite/Lyme infection. I will make sure to get re-screened for it, but I truly believe I would be feeling worse by now, when really, I'm back to my baseline.

I know. It got pretty terrible looking. It never hurt or itched too much, but it was very hot. The doctor reassured me that it sounds like a spider bite.

On the last day of fever (June 27), I had another bad reaction. I believe the triggers were multiple nights of poor sleep, as well as a formulation change for my probiotic (Culturelle has additional fillers now). There could have been other triggers, but it is sometimes really hard to sort it all out. I was struck with a sudden, intense headache, followed by a hot flash. In the following 6 hours, my vitals tanked. I spent a long time with a reduced state of consciousness (basically a stupor), with some intermittent loss of consciousness. Even after medication to raise my blood pressure and extra salt and fluid intake, my blood pressure was 80s/50s the rest of the day, along with a low heart rate around 50 (not typical of a POTS reaction). I had shortness of breath with movement and upright posture, burning chest, heart pounding, and developed a throbbing migraine at bedtime. I still don't know the true trigger, although often MCAS reactions happen when you stack triggers. Individually, they may not trigger very noticeable symptoms. But when stacked together, they can trigger a major reaction. There is also a bucket analogy to explain this. Each trigger adds to the bucket, and you don't get symptoms until the bucket overflows. (I still love this article to explain the phenomenon of cumulative triggers and why you can tolerate something one day but not another day: http://www.mastattack.org/2016/04/the-devils-arithmetic/)

Anyway, I had a follow-up with my allergist on July 7. The doctor wasn't as proactive as I'd hoped regarding treatment. She only wanted me to take more of my current antihistamines. Luckily, I got up the nerve to ask about a few other treatments, and she was amenable to trying them both, although she was not optimistic. She told me she only has three other Mast Cell patients, so she isn't sure what else to try. But, she agreed to prescribe two medicines to be compounded. One is Ranitidine, which an OTC H2 blocker, used to stabilize mast cells. I have to discuss with a pharmacist the need to use a minimal number of fillers. (This was not included on the prescription.) The other one is Ketotifen, which is commonly used in other countries, but not FDA approved in oral form. It is supposed to be an H1 blocker as well as a mast cell stabilizer. Again, I need to find a compounding pharmacist to help me figure out the least and safest fillers we can use to compound it. Different compounders have access to different medications, and different willingness to compound in new ways. Also, prices can vary wildly, because the pharmacy sets the cost and doesn't take insurance. So, I haven't started the process yet. Once I do, I'll have to trial one medication at a time, and desperately hope that I can tolerate them, and that they have some kind of positive effect.

I've been dealing with a few other triggers this summer. One is that I can no longer tolerate iced or cold water. It adds to the burning sensation in my chest. I'm getting used to drinking room temperature water (about a gallon a day is needed).

A huge problem in summer is outdoor smoke. Grilling, bonfires, and meat smokers. All of it kills me, and makes me glad I now have an EpiPen. It's hard, because I can't take Wilma outside if it's in the air. And we have to avoid even opening doors to outside. I get immediate pain in my head and coughing/wheezing from even a single breath of smoke.

Also in the summer, I have to run the A/C a lot of the time. We have a wall unit. Unfortunately, it doesn't filter out all smoke. That puts me in a really scary situation, because there is nowhere to escape. So, I feel on edge whenever the A/C is running, because I don't want to let anything dangerous into my home. Even a person walking past with a cigarette while the A/C is running can ruin my entire day. But, I have to run the A/C. You don't want to see me when the indoor temperature is higher than 76 degrees (I develop presyncope with reduced consciousness.) So, I have to risk it a lot of the time.

Finally, I've started having trouble just sitting in the family room at mom's house. The room is connected to the attached garage. I will randomly start smelling faint gas fumes, and a reaction slowly develops, getting very severe some days. Having to avoid the family room just keeps me even more isolated. We even stopped using the door to the garage, but I can still smell the fumes.

I'm also reacting to fragrance free sunscreen now. I wish I could find a sunscreen with truly no scent, but then it probably wouldn't actually work. I don't personally wear any. I just cover up with clothes (which does not help my heat tolerance). But people around me who spend a longer time outside obviously need to use it.

It drives me crazy to constantly have the world attacking me, and to see my sensitivities continue to progress.

Anyway, that's what's new. I am still so grateful for my safe havens. Luckily, my bedrooms in each home are both still safe spaces, which I have to protect carefully. I am so lucky that the people I live with help me to keep it that way.

I'm also so lucky to have my little companion, Wilma. She is the most low-maintenance dog I've ever known, and we are very attached, spending nearly 24/7 together. I am allergic to most dogs, but I don't believe I react to her.

I'll post again soon, hopefully. Thank you for still reading and still caring about my lonely saga.

Tuesday, June 20, 2017

Another diagnosis...finally.

Well, I have some news. I got in with the new allergist 2 weeks ago. She completely believed me about all of my symptoms, not blaming any of them on anxiety. She didn't waste time and immediately ordered comprehensive testing for MCAS (Mast Cell Activation Syndrome). She ordered several blood tests and a 24-hour urine collection. I don't have all of the results yet. But, the doctor called me herself on Friday afternoon when the blood work came in. She found multiple elevated levels of mast cell mediators in my blood. Clearcut evidence that I actually have this disease that I have suspected for four years. She said it was so conclusive that I don't even need to bother with the urine collection.

The best thing about this diagnosis is the validation that I am not imagining my symptoms or my progression, and that someone who knows what they're talking about has confirmed this. It is still a new diagnosis, so plenty of doctors are not yet even aware of it, much less able to recognize, test for, diagnose, or treat it. But it is such a relief after so many doctors have told me that my symptoms must be anxiety, or some sort of conditioned response (Thanks to my condescending allergist for that one). It's hard not to believe they're right after you've heard it multiple times. But I finally know that I am not imagining or catastrophizing any of my symptoms. When I am exposed to any type of trigger (environmental, medication, food, temperature, weather changes, vibration...anything my body decides is a trigger), my body goes through a cascade of symptoms due to the release of specific chemicals from my mast cells. My body believes it is being attacked, and responds as such. This condition is often progressive, as in my case. It is also notoriously difficult to treat, because so many potential treatments end up becoming triggers themselves.

So, that is the hard thing about the diagnosis. Everything that I have suspected is happening in my body is really happening. I knew I was suffering severely, and I have been mostly housebound for 6 months, and have had to avoid interactions with most people for quite a bit longer. This certainly explains why it is happening to me. The hard thing is figuring out how to stop the progression. I am lucky, because I do not experience anaphylaxis. It is now pretty confirmed that my reactions to cigarette smoke and running in high school and college were in fact anaphylactic reactions. Never asthma. Not "Vocal Cord Dysfunction." Which explains why the treatments never helped. But the EMTs giving me a shot of Epi always resolved the issue. I now have a prescription for an Epipen, because in my state, I could escalate to anaphylaxis at any time.

But let me tell you, even without throat closing anaphylaxis, my symptoms are becoming insane. Each reaction seems more severe than the last. I have a couple of recent examples of reactions that really demonstrate this:

About a month ago, I decided to try taking Pepcid. It is an H2 blocker, so it is helpful in treating MCAS. I suspected that I wasn't tolerating Zantac, so I discontinued that and tried the Pepcid. One 12-hour pill. My reaction included:
-Insomnia for 3 days
-Sensation of a fever, alternating between hot and cold, with no measurable fever
-Pounding heart for 3 days
-Full-body itching (pruritus)
-Diarrhea for 2 days (only able to eat white rice)
-Swollen face (edema)
-Burning face
-Burning throughout my torso
-Headache/facial pain

A week and a half ago, I tried a new H1 antihistamine my new allergist prescribed. It is a prescription called Cyproheptadine, and generally very well tolerated. I was still cautious, so I tried only 1/4 of the lowest dose. My reaction was almost identical to the Pepcid reaction, although the diarrhea and insomnia were more severe. I slept less than 30 minutes, despite heavy sedatives.

Then came yesterday. A hellish day I won't soon forget. I had to leave the house. I was overdue for a psychiatrist appointment. It was this office that made me miss the holidays last winter. But I had to go, because he prescribes my sleep medications. They are controlled, so no one can prescribe them without regular appointments. So, we had to leave for the office at 12:45, which is waaaaay too early for me to be leaving the house (sounds ridiculous, but true). There were also storms around, which really agitates my mast cells. I wore my respirator the entire time. Yes, a full respirator. Not just a mask. A ridiculous respirator. I did not smell anything through the respirator. Unfortunately, the reaction was insane. On the way home, my pain levels became very high, especially in my head and my sacrum. We got home, and all I could do was curl up in fetal position and moan. I crawled into bed and kept moaning. My mom came to check on me and noticed that I was hot. Really hot. I developed a fever that reached 101.5 by evening. (I have normally low body temperature, and normally do not get a fever with infection, so this was very notable for me.) So besides the fever, I experienced:
-Severe, widespread muscle and joint pain (especially sacrum) - Pain level 9
-Skin sensitivity
-Headache/Facial pain - Pain level 8
-Abdominal cramping
-Complete lack of appetite
-Tachycardia/Pounding heart (heart rate 100-120 for over 12 hours)
-Low blood pressure (90/60)
-Intermittent loss of consciousness
-Bladder pain
-Marked weakness (unable to lift my purse or my 7 lb. dog)
-Insomnia, because who could sleep through all of that?
Today, I woke up and still had a bit of fever, but it eased off as the day went on. We were pretty sure I was getting really sick, having contracted a flu or virus or maybe a urinary/kidney infection. But, if it were any of those things, I would still be sick tonight. And I'm mostly just back to my usual, crappy state. So, it was obviously a major mast cell reaction.

I feel completely traumatized by these reactions. I honestly don't know how I will make myself try another medicine or enter another building ever again. I am terrified at the amount of suffering I go through from such small things, and at the progression of my illness. The pain and fever are so difficult, because I cannot tolerate anything that might ease them. Anything!

A brief list of my medication intolerances:
Sulfa antibiotics
Wellbutrin/Cymbalta--seemingly all SSRIs and SNRIs are out
Nsaids--everything from Advil to Aleve to Toradol to Indomethacin
Triptans--every migraine medicine
Vitamin B2
Most probiotics
Cromolyn Sodium--at least the generic

So, in general, with MCAS, it is thought that when someone is reacting to most medications, they are not reacting to the medicine itself, but to one or more of the fillers/excipients/inactive ingredients. Most likely it is a commonly used ingredient. (Most medications contain between 10 and 20 of these extra ingredients...some even more.) Most doctors don't believe it is possible to react to such tiny traces of ingredients. But MCAS specialists see it all the time. And it certainly seems to be the case for me, since I can have the same exact set of symptoms from so many different medications. So, the next logical step seems to be to try to order compounded medication. It is special ordered through a pharmacy to contain no fillers, or only 1 safe filler when necessary. There are a few problems. One is that insurance companies don't cover compounded medication, even when medically necessary. The other is that most doctors won't even consider prescribing these medications. My new MCAS-savvy allergist doesn't seem to prescribe them. But I may have to convince her to try for me.

The other problem is that I could be reacting to one or more of my current medications, and that could be keeping me in a reactive state. It feels almost impossible to sort out which one is problematic though.

Another issue is that the only mast cell stabilizing medication approved by the FDA is Cromolyn Sodium. I have tried the generic. I did not benefit from it, and once I reached therapeutic doses, I seemed to be doing worse. (Although it's hard to say, because my condition has been progressing all this time.) I have heard of people that do poorly on the generic version, but the brand name version is very helpful. So that still may be worth a try. Again, my doctor would have to agree it's worth a try.

So far, I know that my blood contained elevated levels of Prostaglandins and Chromogranin A. Just these two chemicals explain so many of my symptoms: burning, increased pain perception, sleep dysfunction, joint and muscle pain, nerve pain, changes in heart rate and blood pressure, bronchoconstriction, etc. And mast cells release any combination of over 200 chemicals in any given reaction. Most of them cannot be tested for in a commercial lab yet. So, the array of symptoms this can cause is staggering. I will explain much more about this in my next blog post. (Hopefully, I'll get to it soon!) I plan on the next post being explanatory about MCAS, especially information I've learned in Dr. Afrin's book Never Bet Against Occam. I am still on a 2+ year wait list to see Dr. Afrin. He is leaving the University of Minnesota to start a mast cell clinic, so there will be a bit more of a delay than expected. But I am so relieved I have a local doctor to help me in the meantime. I am so lucky to belong to so many different FB support and awareness groups that have helped me to locate this doctor and learn that I am not a freak. I am one of them. I am extremely isolated, but I am not the only one going through this insanity. In fact, Dr. Afrin believes it is an epidemic. I learned that with EDS and POTS, along with my progressing chemical reactivity, MCAS was the natural explanation. They are genetically linked. I hope to get the explanatory post up soon. In the mean time, for anyone curious, the blog mastattack.org is an excellent resource written by a medical researcher.

In particular, this post explains the connection between EDS, POTS, and MCAS really well:

Also, this post is amazing:

For a simplified version, I really like this blog:

Especially this post:

I am incredibly lucky to have a core support group that are helping me to maintain my bubble-like existence, protecting me from triggers whenever possible. These are the people that are forced to live with me: my husband some of the time, and my mom and stepdad the rest of the time. I would absolutely not be here without them and their support. They have believed in me long before this diagnosis. And I can never thank them enough for it, because I know it sounds crazy that I have another rarely heard of diagnosis. It is crazy. But it's probably not that crazy, because it's possible that the mast cell activation has been responsible for every symptom and diagnosis in my lifetime. Nonetheless, I could not survive this without their help and endless accommodations. And....I'm crying now, out of gratitude with a hint of despair.

Thanks for reading. I'm hopefully going to go sleep now, since I barely slept through my fever last night.

Wednesday, April 26, 2017

Spring 2017

Well, I fired the last doctor. After our last appointment, it became clear that she would not be able to help me. I don't think I have the emotional energy to go through all of the reasons why. But mostly, I wasn't being heard. She planned to put me through her usual "immune dysfunction" treatment protocol, despite my intolerance of every treatment she tried. She wanted the key components of my treatment to be Vitamin B12 and D supplementation, despite the fact that my levels were what she admitted were optimal, and my severe reaction to various supplements (which she totally ignored). We were definitely not seeing eye to eye on anything. Especially the potential diagnosis of MCAS, and the fact that mast cell issues are definitely possible with normal tryptase levels. In fact, Dr. Afrin (the international specialist in MCAS) states that most MCAS patients have normal tryptase levels. This is new information for most doctors, and not taught in medical school yet.

I honestly got so despondent, that I started to question the utility of even pursuing a diagnosis. But, then I started reading Dr. Afrin's book (Never Bet Against Occam: Mast Cell Activation Disease and the Modern Epidemics of Chronic Illness and Medical Complexity). As I read through his extensive case histories and thorough explanations of mast cell symptomatology and heterogeneity, I have become more certain that this is a route I need to pursue for myself (rather than just give up all hope on ever feeling better or stopping the progression). So, I am on Dr. Afrin's wait list, and will have an appointment in approximately 2 years.

In the meantime, I have a list of things I can try, but I will need a local doctor to help me with some of them. So, after more searching, I got a recommendation for an immunologist in Evanston that apparently works with several of Dr. Afrin's local patients. (Dr. Vivian Chou) She reportedly accepts MCAS as a diagnosis and will work with Dr. Afrin on treatment plans. My appointment will be May 17. I know better than to get too hopeful, but as I've said before, I have just enough hope to try, which is really all you need. You don't have to have any level of certainty or confidence. You have to have just enough to be willing to try the next thing. (I don't buy into the whole power of positive thinking thing. It only causes me heartache from disappointment. I like to proceed with caution, realism, and my eyes wide open to possible outcomes. So, I am aware this appointment may be another bust. But it may get me headed in the right direction, so I'm willing to put myself through it. What more can you really ask of me?)

Anyway, the various things I haven't tried yet to try to calm my mast cells include:

  • Continued trials of Cromolyn Sodium
  • Switch to brand name Cromolyn Sodium (Gastrocrom), as it may be better tolerated (but more expensive)
  • Try the compounded or imported medication Ketotifen, which is a mast cell stabilizer in addition to an antihistamine
  • Try switching all of my medications to compounded forms without fillers (another expensive option...and a huge hassle)
  • Try adding an H2 blocker back into my regimen to see if I can tolerate it (may also need to be compounded, as medication fillers can be huge mast cell triggers)
  • Try the OTC supplement called Quercetin, which has been studied and shown to stabilize mast cells--I would need to try the purest form possible.
  • Try a pure non-citrus, non-acidic supplemental vitamin C, which in higher doses, can stabilize mast cells
  • Try filtering all my water (drinking and bath water)
  • Low dose Doxepin is sometimes helpful (but I have a history of not tolerating serotonergic medications or tricyclics, so I would dread trying it.)
  • Low dose Aspirin therapy helps some people, but I currently don't tolerate any NSAIDs, so I would be hesitant to try this, too.
Those are about all of the things I expect to be able to try before seeing Dr. Afrin. Medication trials take a long time in mast cell disease, because you can only try to change one thing at a time, various forms have to be tried, and doses have to be titrated up very gradually. Also, if you are having a major reaction, you can't try anything new until you get back to baseline. So each of these is a long, drawn-out process. With frequent reactions, it can take a long time to figure out what you are reacting to. Is it one of your current medications or foods that you no longer tolerate? Is it something environmental? Is it a change of season or weather causing a flare? Or is it actually the new change you are testing? And treatment outcomes are so variable and individual, so finding the right regimen for an individual with noticeable improvement often takes 2+ years of constant experimentation. Dr. Afrin says that about 80% of his patients have significant improvement after 2 years of treatment.

Once I get to Dr. Afrin, if these treatments do not get me to a tolerable place, there are more intense treatments that are often tried. These are mostly milder chemotherapy agents, especially Gleevec or Hydroxyurea. I don't know a lot about them, except that they have a higher risk profile than other medications, and many MCAS patients get significant improvement in their quality of life on them. Tolerances can be drastically improved. And symptoms you have accepted as part of your normal can just disappear.


That's the mast cell update.

My EDS body has also been failing me. I've been trying a PT protocol for EDS for 4 months now, and I've pretty much had to give up on it. Trying to stabilize my sacrum and pelvis with gentle exercises has caused massive, disabling pain in the area. I've had to use my cane to get around at times. I can't seem to even handle simple bridges without a massive pain flare up.

This has happened with so many exercise routines over the years. I was once in quite good shape, working out with a trainer that took a special interest in me and gave me free sessions. I was the strongest I've ever been. Then, I had my lumbar surgery, and I couldn't return to that type of exercise. I switched to a routine with a PT in Milwaukee who had dealt with hypermobility a lot, and she helped me with small exercises to stabilize my troublesome joints and my whole core. She helped me come up with alternate exercises when things got too painful. Then, I had CCF surgery, and moved to Madison, so I lost all progress again.

At some point, I was using walking as my main form of exercise, but my feet became so painful that I could barely stand any weight on them. My custom orthotics got me able to walk again, but only for as few steps as possible a day. Any extra walking, and the foot pain becomes unbearable. So I have to limit my time on my feet to the bare minimum.

After my last surgery, my POTS got really bad, so I started recumbent biking as my main exercise. This was so awesome for me. I started really gradually and got to the point of decent cardio workouts while also building strength in my legs. This worked so well for a few years. Until it didn't anymore. I developed severe, disabling pain in my hips. I tried a new recumbent bike with slightly different positioning, and that worked for another 6 months. But the disabling hip pain came back. That was last fall, and I haven't been able to work up a sweat since then. The timing certainly did correlate with my most recent deterioration, but causation is questionable. It could certainly be that the worsening mast cell activation increased the joint pain and weakened my connective tissue. Or it could be that the lack of exercise caused the mast cell activation to worsen. (Dr. Afrin suspects that the connective tissue weakness in my form of EDS may be caused by mast cell activation itself.)

Either way, I started this new PT protocol in January, working on my own, since I can't actually go to a PT office. I've been really dedicated to it. The exercises are all pretty familiar to me, since I've had literally years of physical therapy. But for whatever reason, it just doesn't seem to be working out. For now, the less I use my body, the less pain I am in, making it easier to get through my activities of daily living. And I need to be able to keep caring for myself. No one can help me get all of my meals and bring me everything I need to get through the day, much less feed Wilma and take her outside for me. Those are the few things I'm responsible for in life, so I have to continuing managing them myself. Also, losing my mobility would really make my POTS go crazy. It's bad enough that I can't do cardio. But being bedbound brings the POTS to a whole new level.

So, for now, I've given up on any specific exercise and just try not to stay idle for too long at a time. My eating schedule and Wilma's eating and outside schedule help with this. The only thing I can think of trying sometime is an exercise machine called a NuStep. It's used in PT gyms, especially for people with severe arthritis and multiple joint replacements. It is a seated stepper with minimal range of motion. But, even used, it costs about $5000. So, it probably isn't in my future. There's no way to know how long my body would tolerate it anyway. I would love to go back to PT, especially aquatic PT, but I'm pretty sure I wouldn't tolerate the chlorine anymore.

My diet has been pretty much the same. I can't be around the smell of cooking or plenty of foods anymore (especially coffee and hot sauce--which may be my husband's favorite things). We figured out that the smell of coffee is what was triggering me in our apartment. So, my husband has sacrificed his favorite fresh-brewed coffee and switched to the much less enjoyable cold brew. And I can live at home again (at least, until the weather gets too hot. We don't have central A/C, so I can't tolerate the hot months here). Anyway, my diet is much the same. I'm eating blueberries now (frozen so they are fresh). They make my nose run and sometimes my face a little itchy, but since they are my only fruit, I'm sticking with them for now. I still splurge and try something outside my diet about once a month (usually some form of chicken and broccoli...I don't eat meat on a daily basis. I'm actually eating mostly vegan at this point, out of necessity.)

April has been a bad month for headaches. The weather changes always torture my head. I've had migraines with visual aura, plus the usual pressure/tension headaches and facial pain more days than not this month. The pressure is dropping really low tonight, and I sure feel it.

Sleep is inconsistent. But if I haven't had any recent triggers, and the weather is okay, and the stars align just right, I can sleep adequately multiple nights in a row. (For me, that usually means two bouts of sleep, about 3 1/2 hours each.) Other nights, I only get 4 hours of broken up sleep. Environmental and food triggers, as well as weather all contribute to this. But I am so grateful that I am not as sleep deprived as I once was.

Unfortunately, fatigue, malaise, and brain fog are still severe daily symptoms for me. I spend most of my time between meals staring at a wall or staring at a screen. In a daze. Not capable of much conversation or clear thinking. I'm still not tolerating any stimulants or exercise, so there's nothing to do for it.

I still have daily pain averaging a 6, usually worse in the evenings, and some days much worse than that. Pain includes headaches and body pain. The body pain is pretty widespread. My hips, knees, and feet hurt every day. My knees, especially on the outside of the knee by my unstable fibula, require constant bracing, and have chronic pain that definitely limits my mobility. My feet are also major hurdles of my mobility. My spine hurts often, My neck and shoulders hurt, too. My shoulders are easily pulled out of place just by leaning wrong or laying on my side. My SI joint comes out of place just by sitting wrong for a minute, causing widespread pain. I have muscle aches, especially in my legs, every day. This can often be micro-tears, as well as overuse injury from trying to compensate for my unstable joints. I also have bone pain, presumably from the mast cells, in my leg bones, every day. And currently, I cannot tolerate a single pain or migraine medication or supplement. Just nothing for pain. It is an often excruciating life.

Depression has been a persistent issue, given the limitations of my lifestyle. I have risked several social gatherings this spring, but I have suffered a flare after each one, with at least several days of worsened symptoms.  Usually head/facial pain and burning in my chest, as well as insomnia and sometimes agitation. So, the isolation still largely persists, and so does the depression.

My anxiety is dependent on triggers. If I get exposed to anything (most recently the smell of hot sauce 4 days ago), I will feel immediate anxiety, a direct effect of mast cell activation on the brain. But, I can go many days with absolutely no anxiety, which is such a relief. We identified my Xyzal antihistamine as a major trigger of chest burning and anxiety. It just took 3 months to sort it out. Now, I'm back to only having these symptoms from a trigger, although they last for days at a time.

Okay...I've used up enough of your time, and gotten everything major out of my head. Hope you all are doing well! Thanks for reading. I'll write again after my next appointment.

Saturday, March 11, 2017

Autoimmune Disease?

After being rejected by the MCAS specialist at Rush, I decided to ask my support groups again for recommendations of doctors that might be able to help with diagnosis and treatment. I made an appointment (with a strong push from my brother) with a local allergy/immunology doctor that apparently does not necessarily diagnose MCAS, but does treat a few patients for it.
She was very local, and I had no problem getting an appointment with her. So, I saw her Thursday this week.

In order to make sure I presented my case well, my brother and my husband helped me put together a medical and allergy history for the appointment. This is something I probably wouldn't have had trouble doing in the past, but thinking clearly is not my strong suit anymore.

So, this is the history I put together. I keep thinking of things I missed, so it's not totally comprehensive, but I can keep adding to it, as needed.

Medical and Sensitivity Chronology

Summary and Current State

Significant and progressively worsening symptoms throughout my life
  • Diet is restricted to about 15 foods.
  • Unable to tolerate any scented products, cooking smells, and most medications.
  • Frequent symptoms of a reaction include: burning sensation throughout my torso, sudden and intense migraine, sinus pressure, excessive mucous, trigeminal pain, pounding heart, agitation and restlessness, insomnia, increased heart rate and/or blood pressure, sometimes hypotension/bradycardia, vertigo, increased GI motility, bladder pain, facial burning. Most reactions last 3-5 days.
  • In between reactive episodes, I have low blood pressure, low heart rate, low energy, almost non-existent anxiety, constipation, and 10 hours of deep sleep a night. During episodes, I have either low or high BP and HR, excess energy, sometimes seeming manic, increased motility, and 5 hours of restless sleep.
  • Nearly every week I develop a new sensitivity. I'm running out of foods and medicines that I can tolerate. I currently can't tolerate my own apartment, due to old carpeting, brewing coffee, and cooking smells. I stay at my mom's house, and often have to retreat to the bedroom to avoid a trigger. We are unable to cook in the house. I am very isolated, and haven't seen my friends or much of my family in almost a year.
  • Progression has been accelerating recently, and symptoms are often constant at this point, making it hard to identify new triggers.
  • My overall condition has deteriorated, including hair loss and weight loss (BMI 17.8)


Allergy to breast milk and dairy formula—projectile vomiting, bright red face, excessive mucous


Allergies (all skin test positive) to cats, dogs, grass, trees, dust, mold, mildew—hayfever, runny/stuffy nose, itchy eyes/nose/ears/throat


Severe allergy to cigarette smoke and other smoke—deep cough, difficult breathing, tight throat, sometimes passing out from lack of air (inhalers didn't help, epi shot did). This was never officially diagnosed as anaphylaxis.

Excessive fatigue, nausea, headaches, narcolepsy, body aches, GERD, IBS


Age 18
Tonsillectomy—reaction to Compazine—dystonic reaction: dislocated jaw, stroke-like symptoms; resolved with Benadryl

Chiari surgery—temporary improvement, but then developed POTS—hypotension, tachycardia, passing out, “reduced consciousness spells”

Age 20: Cranio-Cervical Fusion surgery—Condition worsened after, developed symptoms of EDS

Early 20s
Allergic symptoms became more bothersome, repeat allergy skin testing, but came up negative to everything.

Age 24: Spinal cord detethering surgery and cholecystectomy

Age 26: Fusion Reconstruction; Official diagnosis of hypermobile EDS

Age 29 (2013): Repeat Fusion Reconstruction (2013)
This surgery had multiple immediate complications: bilateral corneal abrasions, difficult intubation with significant inflammation and prolonged sedation, extended periods of unconsciousness, intolerance to most post-op medications.
  • Developed severe insomnia, after years of hypersomnia
  • Developed Inappropriate Sinus Tachycardia, POTS worsened- which was later switched to a diagnosis of Hyperadrenergic POTS
  • Lifelong IBS worsened: constipation, severe bloating, abdominal pain, occasional episodes of diarrhea
  • Developed chronic migraines—failed many treatments over the next 3 years.

Allergy symptoms got progressively worse
  • Started noticing worsened chemical sensitivities
  • Developed a reaction to Dawn dish detergents and spray air fresheners: causing widespread itching, runny nose, itchy skin.
  • Allergic response to dogs became more severe, although allergy testing was negative.

Allergy testing results
  • Rast blood testing showed no allergies.

Age 30 (2014)
Allergy symptoms
  • Major reaction to hardwood floor varnish and excessive heat in new apartment that led to eventually having to move. Symptoms included: full body itching and flushing, tachycardia, palpitations, wheezing, repeated syncopal episodes, intense facial pain, deep cough and feeling of throat closing, blue lips, feeling of doom, and urgent diarrhea. I was eventually carried out of the building, unconscious.
  • Developed severe, intractable facial pain after the varnish episode—triggered by fragrances, smoke, weather
  • Full body pruritus with significant agitation and insomnia became severe.
  • H1 and H2 Blockers plus Singulair helped with the severe itching and hayfever symptoms.

Allergy tests
  • Late 2014, saw Dr. Kentor (allergist) for Mcas testing: Mcas was suggested by my geneticist and my POTS specialist. Tests that were run at the time: Serum tryptase (3.1 ng/mL) and urine 5-HIAA (3.6 mg/24h)

Age 31 (2015):
Allergy symptoms
  • After developing severe itching, bladder pain, runny nose, and agitation after certain meals, a low histamine diet also became essential to control these symptoms.
  • All other sensitivities progressed.

Age 32-33 (2016-Present):

  • In June 2016, I was hospitalized for status migrainosus. Treatments (steroids, Toradol, Zofran, DHE) triggered a major reaction: hypertension, tachycardia, severe internal burning, twitching, agitation, panic. Severe headache/facial pain persisted for 6 weeks, and has returned often since then, almost always triggered by a chemical exposure or a weather change.

  • Started Nasalcrom and Gastrocrom in early 2016, but chemical sensitivities continued to progress.
  • Currently taking: Zantac 2-3x/day, Zyrtec, Xyzal, Hydroxyzine, and Gastrocrom for mast cells. Midodrine/Clonidine as needed for dysautonomia. Klonopin and Ambien for insomnia.

Symptoms - Current State
  • Ever since the 2016 hospital stay, I've developed the internal and/or facial burning, agitation, pounding heart reaction to more and more medications: Cymbalta, Nsaids, Prevacid, Nexium, triptans, Tylenol, Sudafed, Vitamin B2, probiotics, and magnesium. I have had to discontinue all of them. I suspect that I am reacting to some of my current medications, as well.
  • After certain foods or smells, I get the reaction of hypotension, bradycardia, white lips, reduced consciousness.
  • A sudden fragrance can cause me to pass out.
  • A carbon filter mask used to reduce reactions to scents, but doesn't help anymore.
  • December 2016, after cold turkey Cymbalta withdrawal, I became so sensitive to everything that I had to remain housebound. I also had to quit teaching piano lessons.
  • Reactions have been increasing in frequency and severity to a larger number of triggers and smaller exposures.
  • In February, 2017, I started developing severe reactions to cooking smells, coffee brewing, and any scent. Even reheating many dishes in the microwave causes: sudden, intense migraine, sinus pressure, and trigeminal pain, pounding heart, agitation, insomnia, increased heart rate and blood pressure, vertigo, increased GI motility, facial burning, internal burning sensation (mostly in my torso- but not related to acid). This reaction lasts 3-5 days.
  • A single mosquito bite leads to hives, widespread itching, and restlessness/insomnia for a week.
  • Exposure to bleach leads to itchy nose, chin, throat, excessive phlegm, wheezing cough, facial pain, hives.
  • Any type of tea causes a hive outbreak.
  • My current medication regimen reduces itching and hayfever reactions, prevents hives, reduces swelling of eyes and lips.
  • My head, body and especially facial pain reaches a 6 most days, and some days reaches an 8 or 9, but I am unable to tolerate a single medication to relieve pain or migraine anymore.

  • Chiari Malformation--Repaired surgically
  • Cranio-Cervical Instability/Cranial Settling/Retroflexed odontoid/Basilar Invagination--Repaired surgically
  • Occult Tethered Spinal Cord--Repaired surgically
  • Gallbladder disease--Repaired surgically
  • Ehlers Danlos Syndrome - Hypermobility type
  • Dysautonomia
  • POTS with Hyperadrenergic episodes
  • Osteopenia
  • History of serotonin toxicity
  • Irritable Bowel Syndrome
  • Gastroesophageal Reflux Disease
  • Raynaud’s Phenomenon
  • Chronic Migraines
  • Chemical Sensitivities
  • Chronic Dehydration
  • Chronic trigeminal pain/atypical facial pain


So, armed with this 3 page document, summarizing my miseries, I went to meet Dr. Sullivan. (I was also armed with my respirator. Even though I was reassured that this was a fragrance-free office, and that the staff would be unscented, I correctly assumed that would not be true.)

We were in the office for 4 hours. I was in immense pain from fragrances. I collapsed and nearly passed out after a standing pulmonary function test. I did not agree to have skin testing done for allergies, because I have already had negative skin testing done in the past, and I don't believe IgE allergies are my problem.

This led to a lot of waiting time. But, we explained much of my story to a nurse, and gave her my medical history document. She passed it along to the PA, who came in and went through more details of my history. The PA discussed my case with the doctor. The doctor read over the document. Then finally, the doctor came in.

She didn't have a lot of questions left for me. She mostly had a prepared speech to give me. But the first thing she said was, "You've come to the right place."

That was obviously very reassuring to hear. But having been through my fair share of overly-confident doctors, I made sure to keep my guard up. (I have to protect myself from getting crushed!)

Next, she basically went through a presentation for me on autoimmune diseases. She didn't actually use the phrase "autoimmune disease," but described a state of immune dysfunction. She certainly believes this is responsible for most of my problems.

She does not like my long list of syndromes. She'd rather I say that I have headaches than migraines (although I certainly believe that I have both!) She'd rather say I have joint problems than EDS (which seems to be a major lack of understanding of connective tissue disorders). She doesn't love POTS as a diagnosis either.

The reason for her distaste for all of these separate labels seems to be her belief that an immune dysfunction is underlying the vast majority of these syndromes.

She also really doesn't want me to get hung up on the "mast cell activation syndrome" label. Although, she does agree that mast cell degranulation may be responsible for many of my symptoms. She thinks the degranulation is a result of the immune dysfunction.

I believe she is trying a more holistic approach, rather than just focusing on mast cells.

So, she first went through this pamphlet with me, listing all of the triggers and irritants that could be upsetting my immune system. This list included:
  • Allergens--She doesn't think this is a major component for me.
  • Chemical irritants--I am already avoiding everything on this list, out of necessity.
  • Changing weather conditions--Unavoidable, but she suggests that I move south and to the coast.
  • Infections--Not a frequent issue for me, although there could be some chronic, low-grade infection
  • Strenuous exercise--I am already avoiding this.
  • Reflux disease--I am already treating this, as well as following diet/lifestyle recommendations.
  • Medications--She would like me to avoid NSAIDs and beta blockers (already doing).
  • Food--Mainly, she recommends avoiding MSG, sulfites, and preservatives. With my diet though, she hopes we'll be able to expand rather than restrict.
  • Emotional anxiety--I have no work or social life to worry about, so all I can do is manage my anxiety about my health.
  • Hormones--She doesn't believe this is a factor for me anymore, since I have been on continuous birth control to keep my levels steady for 5+ years.
So, for the most part, I'm already following the lifestyle recommendations. Unless I feel like moving to the beach in southern Florida, Arizona, or California, I can't change much. (I told her I can't tolerate the heat, but she reassured me that I will be able to in the future.)

But she does seem to have a plan of action. I will be seeing her every two weeks, and making small changes at each appointment. She has very high hopes for me, and believes that I will be feeling significantly better in just a few months. I am, as always, hesitant to get too hopeful about any treatment, but I'm willing to follow the plan, as long as it doesn't make my life significantly more miserable.

She did a ton of bloodwork, but didn't tell me specifically what was being tested, so I'll have to report back on this one.

She wants me to get back on Vitamin D, which I discontinued along with all of my other supplements after things flared up. She wants 4000 IU daily, in tablet form (not gelcaps)

She wants me to continue Singulair, and says that we may double the dose at the next appointment. This is a Leukotriene blocker, and she says it can reduce inflammation throughout the body, although it is marketed as an asthma medicine. I've been on it for 2 years already.

She wants me to drastically reduce my anti-histamine usage. She believes some of my daily symptoms, including random bouts of anxiety, are side effects of too much anti-histamines. This one is difficult, because I am soooo itchy without them, but I'm making my best effort.

Next, she wanted me to add Culturelle Health & Wellness. She was very impressed that I was aware that this one is a better choice, because it doesn't contain inulin, which is a prebiotic. I have been on probiotics for several years, and I have tried this one before. But I had actually been planning on trying it again. I even bought a box of it just a week ago, so I was glad to see we agreed on this one.

Next, she would like me to decrease my dosage of Prilosec. This is unfortunate, because after my body could no longer tolerate Prevacid or Nexium, I finally found that I could tolerate the prescription strength Prilosec. I also went 3 months (November-February) without any drug in this class. The heartburn was absolutely miserable. And I didn't get to tell her this, but my grandma died of esophageal cancer, so heartburn is not something to take lightly in my family! Also, both of my parents and one of my brothers need a PPI to control the GERD. I've tried going without before, but it seems to always come back. I understand that it is not good to use long-term. But, it feels unavoidable. I am glad that she is trying to ween me down, and I will try my best to cut my dose in half, and see if it is tolerable. I just know that lifestyle measures may not be enough with such weak connective tissue in my gut. Maybe if I switched to an all liquid diet? But if I'm eating solids, I'm not sure it will be possible, since her plan is to get me off of them completely again.

Finally, she recommended Vanicream products for skin care. I already use their shampoo, but I will try switching for my face to see if it can clear up the rash I always have on my cheeks.

She was running short on time, and talking very quickly with few pauses. So, I couldn't add much or ask many questions. I hope this will be better next time I see her.

I don't know if she really understands that my EDS makes me a medical zebra. That I may not react as expected to changes. And that simply putting me on the autoimmune protocol she uses with her patients may not be practical. But I really appreciate that she wants to change things gradually, since my body can't handle big changes.

It sounds like she has a lot more in mind to change at upcoming appointments. And of course, we'll have to see if anything shows up on the blood work. I'm so used to blood tests coming up perfectly normal that I never expect much from them. But who knows what she tested for? Maybe something that hasn't been checked before. Maybe, this whole time, I've had some autoimmune disease driving my symptoms. I'm certain I've been tested before for most things, but not everything.

Believe it or not, I actually liked her. I really did. I am very careful to not get my hopes up when someone says they can change my life. Because even as things have evolved over the years, this has always been my life. Sickness and downward progression. I don't love the over-confidence. But it is actually reassuring to hear a little bit of it. And it's really nice that someone wants to manage things, when most doctors seem to just shrug and turn me away at this point.

But, I am absolutely dreading going back into the office. My facial pain is so severe since the appointment. How can you make yourself walk into an office with a level 3 pain, knowing you'll come out with a level 8? I did it once, but every two weeks? I don't know how to force myself to do it. It is just so much suffering. I assume my next appointments will be shorter, but I don't know what can be done to expose me to less fragrance. I guess I wear the respirator the entire time, even when I don't smell a strong scent. But the respirator itself irritates my facial pain. I can call again, but they've already reassured me that they do all they can to reduce fragrances. I guess since my throat doesn't close up, my reaction isn't significant. But it is to me. I still feel like I was run over by a bus since the appointment. And my face is so sore and tender.

Somehow, I'll have to make myself, because she is the only person trying to help me right now. I don't know if I'm hoping for an underlying autoimmune disease or not. I'm not sure how treatable most of them are. But it would least open up a few untried treatment options.

In the meantime, I'm back to living the unsettled lifestyle. Weekdays at mom's house, weekends at home. I think it spreads the misery around a bit. My husband can brew coffee and work late without feeling guilty during the week. My mom can do some cooking and baking during the weekends. It seems to be the best way to manage for now. I can't seem to tolerate staying in our apartment for more than a few days, for whatever reason (I do best when the window can be left open, if the outdoor air is clean.) The back and forth is pretty exhausting. The amount of stuff I have to bring with me is ridiculous! My food and Wilma's food. Mattress toppers and pillows. A big suitcase. Lots of medicine. Exercise equipment. Wilma, too! Wilma, by the way, is doing awesome. Almost 12 (estimated) years old. And as long as she has her prescription dog food plus some potatoes to keep her bowels regular, she's a healthy girl. She is still pretty miserable around my husband, due to her fear. That hasn't really improved at all. But she loves staying at mom's house with her cousins (Dexter and Lexie!).

Also, for two months now, I've been working through the physical therapy protocol in the EDS handbook. I've been progressing pretty slowly, which is recommended with EDS and widespread joint instability/weakness. So, the exercises don't look like much. They are working to stabilize my pelvis and lumbar spine. I believe they are helping, as I have less pain in my sacrum, and I can sometimes sit in a regular chair without a coccyx cushion. I just look forward to when I can work more on leg strength, knee stabilization, and shoulder stabilization. And of course, when I can reincorporate some cardio. But I'm following the program, and this is the recommended order of joint stabilization.

Apparently, I wrote a term paper today. I seem to be a bit manic, so you got the long-winded version. Sorry for taking up so much of your time. Thank you so much to those that support me and those that are following my progress.

Tuesday, February 21, 2017

Another Progression

I need to update more regularly, because my foggy brain can't keep track of even small developments. But I'll do my best to remember.

I developed a new sensitivity in late January. Basically, the smell of almost anything cooking brings on devastating, torturous symptoms. In particular, I develop that severe burning sensation throughout my torso. It starts in my chest, but then fills my torso. It feels like my insides are on fire. It is very intense, and lasts at least a whole day. It's accompanied by intense agitation and insomnia. I've come to understand that this is, of course, a symptom of mast cell activation. Some patients refer to it as masto-burn. It's like the common symptom of facial flushing and burning, but internal. Of course, I have another invisible, but extreme and intolerable, symptom.

I originally got the burning last June, after a week of steroids, while in the hospital for migraine, and was treated with strong IV medications. Then, I continued to get this symptom when I was on the Indomethacin for my facial pain. Then, I developed it again during the suspected serotonin syndrome episode in November. Maybe, it was mast cells all along.

I have also developed other sensitivities, including NSAIDS, Tylenol, Relpax, Prevacid, Nexium, and Gas-X. Basically, all of my as needed medications, I can no longer tolerate. I have had to go without treating most problems that come up in a day. The burning torso is just too intense. I have to avoid it at all costs. And the cost is very high when I can no longer treat so much pain.

I also discontinued my vitamins and supplements. Because I'm just so desperate to make sure I'm avoiding anything contributing to the burning.

Finally, I've recently discovered that I no longer tolerate eggs. Severe GI bloating and cramping seem to be caused by eating a single egg. I don't get much protein at all, and my diet is so restricted right now, so this is another big loss.

The progression of all of these sensitivities has been really discouraging. Especially since I'm on the medication that should be helping prevent this. It's just not enough, I guess.

So, I became aware of an allergy/immunology doctor at Rush that treats MCAS. Unfortunately, when I finally worked up the courage to contact her office, it was a big let down. She greatly restricts the number of MCAS patients she is willing to treat. And she won't see me at this time. I can try to get together documentation and get further testing done, but it will be a huge project. Why do I have so many conditions that doctors are unwilling to treat? It's nuts. My awesome POTS doctor is currently not taking new POTS patients. I'm very lucky I got in with him when I did. There is a huge list of geneticists/rheumatologists that have blacklisted EDS patients. And now MCAS specialists that are unwilling to take on new MCAS patients. It's just so hard to be so unsupported and so alone in managing these conditions. I guess I have to keep seeing my current allergist, because at least he is willing to try things with me (although we haven't managed to make it through an appointment without him infantalizing and condescending to me yet--I always leave crying.) But I should try to get him to order new testing, since the last testing was totally mismanaged.

I did see Dr. Barboi, who I'll now be seeing only yearly, due to his schedule. He basically wanted me to hold everything for now, and wait to see if things calm down. He is worried that getting off of the Cymbalta has destabilized my autonomic nervous system. We are hoping that things will calm down in a month or two. But I'm not sure he's acknowledging how much the mast cells are interfering, and the fact that my reaction to Cymbalta could have been caused by my mast cells, meaning I really can't go back on it.

Being off of my vitamins and supplements has impacted my frequency of headaches and migraines. I really believe the vitamin B2 and Butterbur were helping a lot. But I just can't take anything extra right now. So headaches seem to be daily again. That's getting really exhausting. If I get back to a point where I feel stable, I will try the vitamin B2 again, first, to try to reduce the headaches.

My daily life has certainly been impacted by all of this. I've been able to move back home! I think the biggest difference is the weekly vacuuming my husband has really committed to. With very old carpeting, I just can't handle the dustiness. It upsets my whole system. My diet is even more restricted. And the inability to cook in the house is so limiting. I love to let in fresh air from outside, but we have to be so careful that no one smokes near our window, and to close the place up whenever there is grilling smoke in the air. Because that smoky air that comes with fair weather just about kills me. I get the same bronchospasm and throat closing that I did in high school that was called "asthma," but we now know is from mast cells.

My diet is down to this:

Joe's O's with almond milk
Whole grain graham crackers with peanut butter
Quinoa pasta (made at my mom's house-I can't even be around boiling anymore.)
Carrots and Broccoli, steamed in the microwave
Asparagus or green beans, steamed in the microwave
Potato or corn chips
Rice Krispie treats

I may try cooking sweet potatoes in the microwave, to mix things up. I miss my daily egg! (Although microwaved egg was pretty gross.)

I still occasionally get some type of chicken from a restaurant, but I've had some questionable reactions, so I'm not sure how well I tolerate it. I believe it has to do with the freshness of the chicken, which determines the histamine level in the chicken. 

So, I spend my days alone with my little Wilma. I try to care for myself and for her. I get both of us food. I take her outside several times a day. I keep myself entertained with television, my ipad, books on my kindle, and my phone. I haven't really been coloring. I have very little physical or mental energy. And most of the time, I suffer. I know it sounds like boredom would be a huge problem for me. But it's not. Loneliness? Of course. I have almost no contact with the outside world, outside of Facebook. We haven't really worked out visitors yet, with my progressing sensitivities. But boredom is just not something I contend with. Most of the time, I am dealing with severe symptoms, and I am suffering. I am desperately trying to figure out what is causing my current misery. But I'm not bored. When it's not one symptom, it's definitely another. If I get a break from the itching and burning and insomnia, I am plagued with reduced consciousness, severe facial pain, or migraines. There is no break. When symptoms are on the mild side, I honestly just try to let my body relax and recover from the constant assaults. So, I'm sure my daily routine would bore most people, but not me. 

I am still so relieved that I can basically care for myself. Home health care would be expensive, and add an intruder into my safe space. And I just can't handle that. It's hard enough to keep my home scent-free as it is.

I need to arrange a trip to the dentist and a trip to the optometrist soon. The dentist, because my EDS gums are receding, and my teeth hurt often. (Although actually getting dental work done with no pain killers available would be horrible.) The optometrist, because glasses give me headaches, so I need a renewed contact prescription.  I don't know how either one will happen, when I can't go anywhere without triggering a major reaction.

Emotionally, I'm struggling. The depression is hard to live with, as usual. I have deteriorated to the point that most days are a fight to maintain my will to live. It feels like all I have to give at times. At least I'm still here. I'm still going through my days. I have not abandoned my loved ones. That feels like all I have to offer. But I sure wish I had more to offer. I wish I had more to share.

I found this Quality of Life Scale by the American Chronic Pain Association:
Quality Of Life Scale 
A Measure Of Function For People With Pain 

0    Stay in bed all day 
      Feel hopeless and helpless about life 
1    Stay in bed at least half the day 
      Have no contact with outside world 
2    Get out of bed but don’t get dressed 
      Stay at home all day 
3    Get dressed in the morning 
      Minimal activities at home 
      Contact with friends via phone, email 
4    Do simple chores around the house 
      Minimal activities outside of home two days a week 
5    Struggle but fulfill daily home responsibilities 
      No outside activity 
      Not able to work/volunteer 
6    Work/volunteer limited hours 
      Take part in limited social activities on weekends 
7    Work/volunteer for a few hours daily
      Can be active at least five hours a day
      Can make plans to do simple activities on weekends 
8    Work/volunteer for at least six hours daily 
      Have energy to make plans for one evening social activity during the week 
      Active on weekends 
9    Work/volunteer/be active eight hours daily 
      Take part in family life 
      Outside social activities limited 
10  Go to work/volunteer each day 
      Normal daily activities each day 
      Have a social life outside of work 
      Take an active part in family life

According to this scale, my current quality of life is 1-2 on average. I'm not sure what can be done to improve this. I'm doing what I can.

There are still more mast cell medications to try, but not a lot. A supplement called Quercetin. An out-of-the-country prescription called Ketotifen. Some risky injections called Xolair. And eventually, chemotherapy. But I don't have a mast cell specialist to help me try these. And I'm so afraid I won't tolerate anything new.

Finally, I would not be surviving all of this if it weren't for my mom and stepdad making their home a safe space for me, as well as my husband accommodating my needs at home. I am no fun to live with with all of my sensitivities. Light, sound, smells, cooking, extra pads and cushions on every seat. I need a million accommodations just to be less miserable. And I am very lucky that I have two safe households to choose from, in case anything goes wrong in one of them. I am so grateful to these three people for allowing me to limit their lives so much. And I'm also grateful for my little Wilma for being my constant companion, no matter which home I'm in.