Mast Cell Madness.

I’m officially terrified by my mast cells because Christmas heralded another sick, sick few days. Almost as sick as Thanksgiving, so I’d have to say the 2nd sickest night of my life. However, this time, it all started with my tongue swelling up, which gave me more insight into the mechanism behind it.

I sometimes wonder if all my health issues stem from mast cell activation syndrome. I can tell the difference between ME symptoms and mast cell reactions, but, still, there’s this little seed in my brain that says, what if they’re at the root of EVERYTHING and I should be spending my time finding a doctor with MCAD expertise on this side of the country (it doesn’t seem to exist in Seattle)? I don’t do this because I am generally stable. On a day-to-day basis, I’m not having reactions — unless, of course, many of my chronic symptoms have mast cell degranulation at their core and I just don’t realise it.

Screenshot_2014-11-15-08-21-33-1-1

My health issues started with full-blown anaphylaxis, out of the blue, 9 days after my 28th birthday. Doctors were hopeless and gave no advice back then, not even daily preventative antihistamines. The common denominator was alcohol (but not every time I drank, so it was confusing), so, after the last trip to the emergency room in Dublin, where I almost died, I finally quit drinking and haven’t touched a drop in 13 years.

Before that, I had swelling in my eyes and hands and a severe edema episode once or twice that I didn’t really think much about. I linked it to Asian food, so stopped eating that and MSG and didn’t look any further into it. This was eventually diagnosed as autoimmune urticaria and angioedema and I was told to take Zyrtec, but didn’t want to medicate daily for an intermittent condition.

I’ve always had trouble with my periods — crippling dysmenorrhea — but they got progressively worse until I collapsed with syncope and shock 13 days after my 32nd birthday and was taken off in the ambulance. For 6 years, no doctor gave me any advice until, finally, an OBGYN told me to dump salt on my tongue. This doesn’t stop the collapses, but it certainly helps. These episodes continue to happen randomly to this day, always on the first day of my period and are, without a doubt, mast cell mediated, presumably low-grade anaphylaxis (very low blood pressure and pulse, bowel problems, syncope, shortness of breath).

anaphylaxis scale

I have a spot in my throat that has itched for years. It was actually the thing that lead to diagnosis of my toxic thyroid goiters and Grave’s Disease because I mentioned the itch to some random doctor who palpated my throat. I’ve now realised it signals reactivity in my body at a very low level. It’s almost always there, but, when it’s not or when it’s very bad, I pay attention.

I was flushing badly for years, thinking I had developed bizarre self-consciousness, but the self-consciousness was actually a result from flushing and having people point it out! When I was diagnosed with Grave’s, I thought it was a symptom of that, but it never went away after ablation.

Of course, in retrospect, there have always been issues I have dealt with, which may or may not originate with mast cells: thyroid problems and Raynaud’s can be a result of mast cell disorders. Also, constipation, headaches, low blood pressure, and temperature sensitivity (all of which got much worse in recent years). Finally, many of my ME symptoms could also be from MCAS: fatigue, joint and tissue pain, eye pain, vision problems, vertigo, episodes of low body temperature, scent/odour/chemical sensitivity, sinus problems, cognitive impairment, hair loss, decreased bone density (I have osteopenia, on the cusp of osteoporosis), shortness of breath, medication reactions, malabsorption, and tinnitus. See a list of signs and symptoms here.

It would be wonderful to be able to manage and control any of these issues, but none of them scares me like the nights I’ve had recently, not even full-blown anaphylaxis. I’ve tried so hard to figure out my triggers, but they are moving targets. Tongue swelling and angioedema are obvious, as is the very specific breathing difficulty you get with anaphylaxis (it is nothing like asthma or wheezing from an infection). I don’t get daily hives and itching like many people. My reactions now are all about the histamine bucket and completely dependent on where I am in my cycle and what is happening in my life. I may be able to eat anything one week and then suspect that those same foods are giving me sinus trouble, insomnia and a jaw ache a different week. My chronic daily headaches, tinnitus, brain fog and exhaustion could be from food choices, but I’ve never been able to pin down any causation. My diet is very low-histamine compared to normal people and how it used to be, but I still allow myself chocolate, coconut, store-bought chips, beef, almost all fruit, including dried and many things that others avoid. Could these things be contributing to my problems? Yes, but, without a definite correlation, I don’t want to eliminate foods. Once you’ve experienced anaphylaxis, “reactions” like a runny nose, constipation or aching hands are quite ignorable. The only thing that consistently caused a reaction was alcohol and my periods. And, now I can say with certainty, holidays and events, no matter how careful I am.

—————————–

I prepared for Christmas over the course of a month and a half, slowly bought presents and wrapped them, slowly wrote some Christmas cards, slowly got the spare room ready for my sister, slowly did laundry — over the course of weeks! Didn’t overexert myself at all. There was no excitement, no activities. My sister and her small dog came over, we watched tv and opened presents. I had rested multiple times throughout the day and the only not normal thing I ate was half a tiny piece of fresh King salmon, which had been brought in off the docks that same morning and, I was told, caught the day before.

My tongue started to swell up after dinner. By the late evening, I had gotten upset for really no good reason (which has historically happened with my mast cell reactions) and was flushing. I had a bad reaction to about 15mg of Benadryl a week or two prior, so I was scared to take a decent dose on this night. I bit a dye-free capsule and put a drop on my swollen tongue and went to bed. At 2am, I awoke with the same evil that I experienced on Thanksgiving and the night after starting Cromolyn (before going to the AirBnb rentals back in September — it was a few days before my period that time, too). I was shaking so badly, I couldn’t lift the water glass, I was drenched in sweat and had weird runaway chills coursing through my body. I crawled on my hands and knees to the bathroom, which scared the shit out of me because, through all the worst of ME, that’s only happened once before. I fell into harrowing nightmares and woke up gasping for breath over and over, feeling poisoned and infected. I dreamt that I was sick and dying and my husband wasn’t paying attention or taking it seriously. I dreamt that I was sick and dying and my mother laughed at me (this isn’t remotely based in truth, this is my terrified mind not knowing how or where to get help). I dreamt that my dog’s neck was broken and I was carrying him to get help, but I was sick and dying and couldn’t do it. And, finally, I dreamt that I was lying on the floor begging my husband over and over: “Please kill me. Please kill me. Please kill me.” I woke up sobbing and so wrung out.

That morning, my period came 5 days early. You better believe, if I had known my period was going to arrive Christmas Day, I might have cancelled Christmas. Or at least postponed present opening for a day. And definitely not eaten even the freshest salmon.

In the past, my anaphylaxis episodes went like this:
My friend A’s birthday party.
My friend C’s birthday party.
Oktoberfest.
Easter party.
C used to joke that I was allergic to fun. I can’t believe he was right. I collapsed and had the paramedics called twice while my mother was staying with us and, also, when my best friend was here from Ireland — both were “events”. I started to get paranoid that, psychologically, I was somehow causing my system to crash when there were visitors. But, every single one of these times, I had my period. There were only a few anaphylactic episodes that I can remember when it wasn’t the first day of my menstrual cycle. EVENT + MENSTRUATION = MAST CELL MELTDOWN. But I think I only really and truly started to believe this 100% on Christmas.

So, Christmas day is a total haze. I crawled downstairs a few times to eat and try to put on a good face, but I don’t remember much and dozed most of the day. Like Thanksgiving and September, however, I bounced back quicker than I could have ever anticipated. That night I kept marveling, “How am I speaking? How am I sitting up? How am I alive?” When it’s bad, you honestly want to die. When it ebbs, the human spirit kicks back in shockingly quickly and you just get on with it, until the next time when you are surprised anew at just how bad the bad is. I didn’t even really modify my diet. I continued to eat my almond butter, coconut ice cream and drink bone broth and tea (all high-ish histamine). If anything, I felt more, Oh fuck it, how much worse could it be? At this stage, I’m much more scared of menstruation and engaging in any sort of event — even one in my house, in my pajamas, with only a single guest.

IMG_20150105_132432-1

I am currently putting together an informational kit (in a bag that was donated to me by a member of one of my groups), so my husband has something to grab in the event of an emergency. My dilemma is that I’ve managed to avoid drugs all this time (never had to use my EpiPen), so I have no way to premedicate for things like plane flights, dental work or necessary procedures like a CT scan or colonoscopy (which my doctor has wanted me to get for years, but I refuse because I’m worried about reactions). I have no safe protocol. 13 years ago, I got IV diphenhydromine for anaphylaxis, now I react to 15mg of Benadryl! 5 years ago, I had IV morphine for dysmennorhea, now my breathing shuts down with a crumb of hydrocodone or codeine. What would happen in a real emergency? If I need surgery? Knock on wood, toba toba, ptooey, ptooey. Once I have everything compiled, I will post it here.

Having said all that, I’m really in quite a good place, feeling happy and hopeful about the new year. Maybe because I realise that these reactions are mast cell degranulations and not ME relapses and that takes some of the fear away. Somehow dying from anaphylaxis is less scary than becoming permanently bedbound with ME. Perhaps only people with both illnesses will understand that. So, here’s what I did New Year’s Eve:

NCM_0042_20141231005400879

Screenshot_2015-01-05-14-33-55-1

As well as resilience, forgiveness, positivity and optimism, I’d also like to request that 2015 doles out truckloads of health, wealth and happiness to all of us. That’s all. That’s not too much to ask, right? 🙂

Advertisements

May 12: My 20 years with Myalgic Encephalomyelitis

Tomorrow is International M.E. Awareness Day. Everyone should read this post by Mary Schweitzer on her blog, Slightly Alive. It is informative and moving and should light a fire in everyone’s souls to raise awareness and find justice for so many patients and their family members who suffer from this disease.

Sunday, May 11, 2014

May 12: My 20 years with Myalgic Encephalomyelitis

I have had Myalgic Encephalomyelitis, or M.E, for 20 years.  The CDC does not recognize this.  They insist that I have a condition called “Chronic Fatigue Syndrome,” or CFS.  I have M.E.

At the age of 44 I led a charmed life.  I had been married to the love of my life for 20 years, and we had two lovely children.  We were both college professors – a deliberate choice that allowed us to do what we enjoyed – researching and teaching subjects that deeply interested us – while having the income to live comfortably (because we both worked) and plenty of time to spend with the children (because of the nature of academic life).  I had tenure at a good university, although my sights were set higher than that.  I had a working relationship as an associate fellow with a research institute at an Ivy League school, which enabled me the luxury of being around the best and the brightest in my field.  We traveled all around the country going to each other’s conferences, often taking one of the kids along.  We also went to four Olympics, two final fours (NCAA basketball championships) and countless playoff games, several World Series, and, eventually, twenty years of baseball AllStar games.  We skied in the winter and went to the beach in the summer.  It was a charmed life.

On October 24, 1994, I went to my office to grade exams and suffered a blackout.  When I came to, I could not understand one word in the Bluebooks in my lap – they might as well have been written in Cyrillic alphabet.  It took time – and concentration – to be able to stand.  I had fallen down the rabbit hole; my life would never be the same.

Over the next four years I suffered from severe pain in the back of my neck and behind my eyes, 24/7. My muscles ached, and I had migraine-level headaches.  I had ataxia, dyslexia, sensitivity to light and sound (to the point I had to wear sunglasses all the time), tinnitus, partial paralysis, memory loss, disorientation, expressive dysphasia, and massive confusion.  My family took care of me.  Obviously, I could not drive, and by 1996 I was using a wheelchair when I left the house (which someone else had to push).

I was lucky to have a family to take care of me, because I could not take care of myself.  I also soon discovered an Internet discussion list of fellow sufferers, and was referred to a very good specialist in Washington, Marsha Wallace (who unfortunately hasn’t practiced since 2000).  Dr. Wallace taught me to live within my energy envelope and helped with sleep disruption and NMH/POTS, but I continued to deteriorate.

In the fall of 1998, Dr. Wallace introduced me to Dharam Ablashi, a researcher who had just retired from the National Cancer Institute at NIH.  Dr. Ablashi had been the co-discoverer of HHV-6 and it’s two variants, A and B, while working with AIDS.  I had the version the AIDS patients did – Variant A – and my viral load was ten times the amount used to diagnose an active infection.

I would also test positive for active EBV or mono (which I had more than once – most notably in 1990, four years before my collapse, during an outbreak on my college campus), CMV (cytomegalovirus), HHV-7, and three strains of Coxsackie B.

My immune system was severely compromised: My natural killer cell function was less than 3%, I had the defective 37kDa Rnase-L, and I had an abnormal cytokine pattern.  But no one knows how all this happened.  All we know is that this disease can occur in cluster outbreaks, and it can pop up in individuals.  No one in my family got it from me, but I believe the outbreak of EBV in 1990 marked the beginning of my illness – the beginning of the cycle of immune defect-virus-damage that characterizes this disease for many of us.  I had to continue to teach through my infection with EBV, including an hour’s commute and back, and while I recovered from mono at the end of the fall semester, my health began to deteriorate in seemingly disparate ways, until the ultimate collapse in 1994.

Years later I would have a spinal tap that revealed both HHV-6 and Cytomegalovirus were active in my spinal fluid.  No wonder I had the symptoms of encephalitis, and with the stiff neck, meningitis.  Along with the muscle pain, that meant literally that I had Myalgic Encephalomyelitis, or M.E., a disease that had been diagnosed in the UK since the mid-1950s.  In the United States, however, all I was given was a diagnosis of “chronic fatigue syndrome,” a name chosen by committee and adopted by CDC in 1988 to replace the name given a number of cluster outbreaks occurring in the USA at the time, Chronic EBV.  They did not mention M.E. – though there were specialists at the meeting who insisted that was the correct diagnosis for these outbreaks.  They did not ask anyone in the disease community what they thought of this name.  They simply adopted it, and having done so, consigned the disease to the backwaters of medicine where neither research nor treatment could be found.

There could not have been a worse choice of a name for this disease if CDC had hired a focus group,  Chronic (as in chronic whiner) Fatigue (as in “yeah, I’ve been feeling tired lately myself”) Syndrome (as in syndrome of the month) – applied to upper middle class white women “trying to have it all” (as the late Bill Reeves of CDC once phrased it) – how inconsequential, silly even.  Twenty-five years later, 85% of patients – over one million Americans – have no idea what is wrong with them, because, according to both CDC and private demographic evidence, only 15% have a diagnosis.  25 years later only 15% have a diagnosis.  That is a mighty admission of failure.

The infectious disease specialists in northern Delaware dismissed my illness as minor.  “You’ll be back to normal in two years,” they assured me. Oh good, I responded – I won’t have to miss more than two seasons before I can go back to skiing.  “Oh no,” was the response.  “You’ll never ski again.”  How was that “normal?” I asked.  They got angry at that.  That’s when I was referred to Dr. Wallace and, thankfully, only had to deal with these people once more, when I was on the antiviral Vistide for my cytomegalovirus infection.  Dan Peterson, my new specialist, had asked them to let me get the infusions at their center, and they had agreed.  But when I showed up at their office, one of the doctors took me aside and said that they could not let me have Vistide because my medical records showed I “only had CFS – nothing serious, like AIDS or cancer.”  They said they could not justify using the drug on someone with a diagnosis of CFS – even though it was an FDA-approved drug for the virus CMV, which was active in both my blood serum and my spinal fluid.

Let me repeat that:  once given the label Chronic Fatigue Syndrome, I would meet disrespect from many doctors and people at NIH and CDC. None of my extensive testing mattered.

Although the progressive version of M.E. that I suffered from was unusually severe, I turned out to be lucky.  I was given the opportunity to go on the experimental Phase III drug Ampligen, in what is called a cost-recovery (I pay cash), compassionate care (I am allowed to do this because I was so very sick), open label (I know I am on the drug so FDA ignores me) study.  I have to get Ampligen at the study site by IV infusion twice a week.  And FDA can take the drug away from me whenever they want.

I have been on Ampligen for eleven of the past fifteen years.  Again, I am unusual in that my illness erupts again within a year of going off the drug (which I did once voluntarily, and once because FDA did take the drug away).  FDA has admitted, in writing, that the drug is not toxic.  But they are not “convinced” it is effective.  My experiences do not count because I was not in a placebo trial; I knew I was on the drug.  There is no other drug in the FDA pipeline for either CFS or M.E. (Although there are immune boosters and antivirals available for patients, and an anti-cancer drug called Rituximab is showing some promise).  This is the only one expressly targeted to M.E. or CFS.  Over one million Americans suffer from my disease.  FDA, CDC, NIH – none of them cares – though in fairness, there are individuals within those agencies who do.  It is those who make decisions who do not care.

[Side note about the obsession with placebo trials – If just knowing you are on a drug can make your immune markers return to normal, your active viruses return to a dormant stage, and change tests such as SPECT scans and CPET scores, we should all be cured of anything by happy thoughts.  Does FDA really believe this?]

So here I am today.  I would not have written this were I not on Ampligen.  On Ampligen, I can drive, take care of myself (mostly), read a book, work on my own writing, spend time with my children and grandchildren.  Off Ampligen I am an invalid in bed in severe pain, curled up in the dark because light is too painful, listening to a favorite movie over and over again.

So twice a week I leave my house at 8:15 and commute by train 100 miles north to Dr. Derek Enlander’s office in New York City, the closest site where I can get Ampligen.  I usually get home around 7 pm.  It is grueling, but at least I am getting the drug that keeps me from being a bedridden invalid.

Myalgic Encephalomyelitis is a serious disease.

CDC betrayed us by giving it a silly-sounding name in 1988 – CFS.  NIH allocates less than $5 per patient per year to study this disease – a pathetic amount.  We came back with private research initiatives, funded by cash-strapped patients and their families, and more good biomedical research is being published than ever before.  The whole concept of what “CFS” is, silly sounding name and all, is undergoing a transformation. And for the first time in my memory, clinicians and researchers have agreed on a definition – the Canadian Consensus Criteria, updated with current research.

So how is our government responding?  Suddenly there are three different initiatives within the U.S. department of Health and Human Services (HHS) to redefine the disease and rename it – done behind closed doors.  At CDC there is the Multi-site clinical assessment – which brought in respected clinics, but is now being polluted with research from a poorly conceived and run study by CDC in Georgia that used a different definition entirely.  HHS has once again turned to the IOM – Institute of Open Medicine – with a committee of whom the majority are not experts in either CFS or M.E.  IOM already weighed in with the opinion that both CFS and Gulf War Syndrome be renamed “Multi-Symptom Disorder,” provoking anger within the larger veteran community.  And NIH as a whole has given the “problem” of the name and definition to their  “pathways to prevention” program, or P2P.  In this case a committee was explicitly created consisting of individuals with NO experience -either medical of personal – with the disease, “like the jury system,” a spokesman explained cheerfully.  “Stakeholders” with different viewpoints testify to the committee, and then this committee of amateurs will recess and vote on the choice of what to do next.  Precisely when did the jury system replace scientific method in determining medical policy?

They are going against the expressed wishes of 60 specialists who signed a letter asking that the U.S. adopt the Canadian Consensus Criteria (CCC), and the public members of the CFS Advisory Committee to HHS asking that the government adopt the CCC, and hold an open workshop of specialists to update it (it is ten years old) with current research results.  Why are those of us within the world of M.E. ignored?  Why is 60 years of biomedical research into M.E. ignored internationally?

Perhaps more important, why don’t people outside our community – people in the media, in government, our doctors, our neighbors, our employers – why don’t they know that there is a growing epidemic of a severe, life-altering and in some cases life-taking disease that CDC and NIH are keeping under wraps?  I have friends who were teenagers when they got sick, and are now in their 40s. They did not get to marry their soulmate like I did.  They did not go to college or have a career.  They did not have children or grandchildren (I have two grandchildren now).  I was lucky compared to them.

They can barely afford to live from day to day.  They cannot afford the testing I have had, and they most certainly cannot afford the treatment I am on.

I have lost friends to this disease; we have lost young people to this disease.  The viruses can get into your heart muscle; they can get into your liver.  Patients die of rare cancers as well.  And then there are the suicides.

There has been a new series of outbreaks in the past five years.  Look at those you love, and if you care for them – whether or not you care about us – do something.  Because they could be the next victims.

Thank you for reading.

April Memorial

Here’s what I want to memorialize today: My head is heavy and cloudy, but I don’t have a headache. My neck is stiff, but not sore. My muscles are weak, but they don’t hurt. My throat – this throat that has felt as if I have strep every day for a year, maybe two – is not sore and has not bothered me in a while. My mood is miraculously light. I may grimace, I may be grumpy and curse this wretched illness, but I haven’t felt sad or despairing in a long time. My period this month came as a quiet, rolly-polly visitor. It shifted and moved around some, as if trying to get comfortable, but didn’t bother me too much.

I worked on the computer today for a few hours, gathering info on doctors, clinics and tests, readying myself for the eventual disability application. I then stood in the kitchen for a while, washing and chopping vegetables and preparing some food. I was dizzy and slurry and weak, but, after lying down to meditate for a while, I was able to go the cemetery on my mobility scooter with the dogs and hubby.

Don’t get me wrong, my vision is still blurry, tinnitus is deafening, hair is falling out, voice is weak, energy is preternaturally low, and nighttimes are torturous battles with my ever-present sleep spectre… But. I’m getting stronger.

I waited a week to post this to see if I jinxed myself and the chronic illness gods would strike me down… I have taken a downturn in the last few days, but I still feel like a different person than I was over Christmas, so I’m posting it. Publicly proclaiming to all and sundry: there might, after all, be life after lifelessness. Universe, please don’t let this slip away.

IMG_20140413_174623