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.

Sunday, January 8, 2017

How to Go Fragrance Free

......And be able to visit me!!!

Okay, I have a few updates. Then, I'll be describing which products are safe for me, and how someone would need to be unscented enough to visit with me.

The holidays were pretty sad for me. I mostly had to visit with people through Skype. There are so few people with whom I can share a room anymore. I also had laryngitis and bronchitis for a few weeks, so I was sicker than usual, and my mast cells were really irritated, so I was needing extra Benadryl around the clock.

I stayed at my mom's house through all of December, since our apartment was still triggering a major reaction.

Earlier this week, I saw my supposed mast cell doctor (allergist/immunologist). His office is supposed to be fragrance free. But they have started using an air freshener spray in the waiting room and the bathroom. They also have air purifiers in each room, but none of them are turned on. So, his office is no longer safe for me. I had to wait in the car for an hour and a half before my appointment. The appointment was not very productive. He doesn't seem to be treating me for mast cell disease anymore. He didn't seem to remember any of the medications that he is prescribing me, including the mast cell stabilizer. He did recommend I try the supplement CoQ10, but that is more likely to help fatigue and pain than allergies or environmental sensitivities. So, I feel like he's given up on actually helping my mast cell activation. I appreciate that he wants to improve my quality of life, but it seems that I really need more of a mast cell specialist. I'll be looking into getting in with a doctor at Rush next. Although, I'm still afraid of going inside a doctor's office, since they are all so scented.

The same day as the appointment, I finally moved back home. It was a little earlier than I wanted to, but the house cleaner used scented products at my mom's house, so we had to move me out abruptly. Coming home was difficult, because something in the air was still problematic. After having bronchitis, my airway still seems to be very reactive, so I developed a persistent cough at home. (Reactive airways are common in mast cell patients.)

Yesterday, we had the carpet steamers come by to try to reduce the amount of dust in our carpeting and in our newly inherited 30-year old couch. We made sure they didn't use any scented products. Initially, it made things worse, because it pulled all the dirt and dust out and into the air (It also smelled really bad.) So, last night was pretty miserable. But, today, now that the dust has settled, it seems to be easier to breathe in here. It's hard to say for sure, as there are so many factors contributing to my state at any one time, but I believe the air is clearer. We also bought a dust mite encasement for the couch, so any dust inside will stay inside. We also bought a new heavy-duty air purifier, so we have one in each main room of our house. Next on the list will be mattress encasements, but I'm putting it off for now, since I seem more comfortable in the bedroom. (It would be a good idea, since both of our mattresses are 12 years old at this point.)

Okay, onto the list of products I use that do not trigger a reaction! I know you're excited!

I am not guaranteeing that anything on my "safe list" will be safe for anyone else with chemical sensitivities, or that I will not become reactive to these in the future. These are just the ones that work for me today. The disease tends to be progressive, so this list may get shorter with time.

Also, I seek out the most affordable products that I can tolerate, not necessarily the most natural or safest products on the market.

I get asked all the time about cleaning products. Most people know there are free and clear laundry detergents. We usually go with Tide, because it seems effective at getting out smells, but All and Seventh Generation work, too. This one is the Target brand, which is usually the best price:

I don't use any fabric softener, because it is just a major irritant for me.

Here is the dish soap we use, which is the Target brand:
Seventh Generation also makes a free & clear dish soap. This one is okay for me, too. And dish soap also makes a good general all-purpose cleaner around the house. Anywhere you want suds in your cleaning, dish soap is a good solution.

For general cleaning, I use the seventh generation brand. They make an "all purpose natural cleaner," as well as a "glass and surface natural cleaner." These are the only ones I know of that do NOT contain essential oils, which are super problematic for me. I use these for most of my cleaning:

For dusting, I just use a lightly dampened cloth. Apparently, that's safest for those with dust allergies.

For hardcore cleaning, I'm still experimenting on what works best. I'd like to try this product for our shower:

I think that's most of the cleaning products. (Obviously, I don't have the energy to keep a perfectly clean home, but these are the products I use when I can clean!)

Personal products can be really tricky, too.

A good example is unscented deodorant. "Unscented" deodorant is usually NOT fragrance free. Here's two good examples, that I used for a while, before I became too sensitive:

But as you see on the ingredients list, both of these products contain fragrance!

So, I've switched to this Almay brand, which I've only been able to find online:

It says "fragrance free," not "unscented," and so it actually contains no fragrance. This is true for most products. Unscented is not enough! This deodorant literally smells like nothing. If someone else were wearing the Sure deodorant, I probably wouldn't notice, because it contains very little scent. But this may not always be true for me.

Another one I have to order online is hair products. Regular stores just don't sell unscented hair products. I have used several brands. I don't love them as much as I used to love my "traditional" scented shampoos, but at least several brands are acceptable for me.

Right now, I have the ones by Free and Clear. They also sell hair sprays and gels, as well as various other fragrance free personal products.

Some pharmacies will order this brand for you.

But there are other choices. Sometimes, I just look for the best price. Desert Essence makes a decent FF (fragrance free) shampoo and conditioner:

There are others, but these are the ones I use the most. Jason and Earth Science are two other brands that make FF shampoo and conditioner. Available on Amazon or Walgreens online.

I know you can also find FF products at Whole Foods, but I can't shop there anymore, due to the essential oils in the air. (Reminder: essential oils may be more natural, but they are still very dangerous for me!!!)

Body wash is so much easier. The Target brand makes a liquid body wash:

It's generic for Aveeno Active Naturals Skin Relief Body Wash.

I use this soap for hand soap, too. I've even gone crazy and tried it as shampoo, and it's not bad. I know that sounds nuts, but the ingredients are pretty similar to a mild shampoo. And it is much cheaper and easier to find than actual FF shampoos.

There's also Dove sensitive skin beauty bar, which is fragrance free. I've gone a little crazy and tried this for washing my hair, too. It will also work in a pinch.

So, you could literally wash head to toe in either of these, and end up clean and fragrance free. It would be cheap and easily accessible for anyone that can get to Target.

*****If someone is normally heavily scented (from products in their home, at their work, on their clothes, or any exposure to cigarettes), they may need to use an odor-removing wash, which is often used by hunters. This is an example:
This is also an easy all-in-one solution.

If someone's clothes are normally strongly scented, especially if they usually use fabric softener, they may need to use an odor-removing detergent like this one:

BUT, even if a person were to wash their clothes in FF detergent, not use fabric softener, and use only FF personal products on the day they see me, I can still have a major reaction. This seems to be due to residual scents. Our hair, skin, and clothes are so absorbent. They hold on to fragrances. So, if you usually use any type of scented product (natural or artificial!) in your house or in your daily life, it will probably be stuck on you. I also know that vapor from e-cigarettes are a huge problem for me, as well as regular cigarette smoke (which has been a problem for me forever).  ***Another reminder that products with essential oils or diffused essential oils are a major trigger for me!

The best way around this that I can think of is storing clothes in an air-tight bag immediately after washing in FF detergent. Ziploc Big Bags should work for this:

You can get them at Target and Walmart.

Then, you would just need to be freshly showered with FF products, and put on the unscented clothes that have been sealed in the bag before seeing me. And then not be exposed to any new scents/vapors/essential oils/etc. on the way to seeing me.

I am absolutely not asking anyone in my life to go to all of this trouble. This is my problem, not theirs. I would like to try to maintain my current relationships through texts, calls, Skype, etc. Especially Skype, which really helped me to "participate" in holiday gatherings. No, it wasn't the same. But it was better than nothing.

This is just for people in my life that would like to have an in-person relationship, and are willing/able to go to all of this trouble in order to visit with me.

My fear is that even with all of this, I'll still have a reaction, which happens so often. My head will start spinning, I'll get suddenly congested, or I'll start having palpitations. The cascade of symptoms will follow, ending with my days of severe facial pain and exhaustion. And I have a serious problem with speaking up when an exposure has happened. If the scent is really noticeable, I've gotten better at quickly fleeing the situation. But for scents that more gradually seep into my system, I'm terrible at being assertive. Especially when I know that someone has really tried to come unscented. (See: Very British Problems)

And honestly, even though our home is full of safe products, we still have to run two air purifiers on high all day in order for me to be somewhat comfortable. I think it's just an older space with older furnishings. So, I'm not sure how I will ever be able to go anywhere outside my safe spaces, like other people's home or stores. I have had some success eating out. If the people I'm with are unscented, and the restaurant isn't crowded, I can sometimes survive. Most restaurants want you to be able to smell the delicious food, not numb your senses with added scents. But, if something in the kitchen burns, or someone scented sits down near me, I'm out.

***Reminder: essential oils are huge on my list of triggers. I have serious reactions and cannot tolerate being near them, even if it is just residual scent on hair or clothes.

Anyway, I thought I should lay out what it seems like might work to help me to be less isolated. Feel free to ask if you have any questions. Thank you for caring enough to read my updates. I still need to work on staying in touch with people. I just still don't have anything interesting to share with people. Dealing with all of this has been my life for at least a year now. This life is not getting easier, but I'm still here.