Until about 12 months ago I’d never heard of Mast Cell Disease, Mastocytosis or Mast Cell Activation Disorder (MCAD), let alone have any inkling that I am suffering from the disease myself. It was only when I was diagnosed with Ehlers-Danlos Syndrome (EDS) in 2010 and started accessing EDS forums online that I came across the symptoms of MCAD and had several light bulb moments.
I’ve had Dermographism (also known as skin writing) for as long as I can remember. Until fairly recently I had no idea other people’s skin didn’t react like mine and come out in wheals when they scratched themselves, pressed their skin or wore tight clothing. My dermographism isn’t particularly bad, and I only tend to get red wheals rather than the more dramatic white marks (caused by oedema or swelling), but the wheals do itch like mad until they settle down. If I scratch myself I look like I’ve been raked with a fork! In order to support my joints due to my HEDS I have to wear various braces, particularly if my joints are in active flair-up. The photo above shows what happened when I used a fairly basic neoprene elbow support (available from any chemist). You can see the red itchy wheals from the pressure of the support on my skin, plus a hive has formed in the shape of the company logo which was printed on the neoprene. Needless to say, despite trying various kinds, I still haven’t found an elbow support that doesn’t cause my dermographism to go bonkers.
As a young child my lips were constantly red and chapped in winter, sometimes in a huge line right down to my chin. During puberty I developed duck lips where, in cold and wet weather, my lips would swell up and look like I had a trout pout (I can’t believe that these days people pay good money to have fillers put in their lips - don’t they know it looks ridiculous?!) My GP thought I was completely nuts (no change there then) and had no idea what was going on. Me neither, and it disappeared 2 years later. I now know I was having oedema urticaria, ie. an allergic reaction to something in the weather (you can be allergic to water, heat or cold – yes really!) whereby histamine was causing my lips to swell. It was another early indication that I had mast cells which needed to be reined in.
All my life, from the moment I was born, my skin has flushed bright red, but only when I was hot, emotional (ie. angry, excited, stressed), over-tired (usually from mental over-concentration rather than physical over-exertion) or, later, drank alcohol. The flushing starts as a blotchy red
patch on my chest, spreads up my neck, and over my cheeks, leaving a white ring around my eyes which looks like I’ve been in a tanning booth wearing goggles. Sexy! When I’ve had a bath I also have the blotchy rash on my stomach, as well as my chest, neck and face, but no-where else. It’s just part of who I am, though it embarrassed the hell out of me as a teenager when I would go out with friends looking like a lobster. Even now I do still get upset by the stares and remarks about my flaming red cheeks – like I can do anything about it! Funnily enough, I don’t get the blotchy rash when out in the sun, a situation I can’t quite figure out. Maybe this has nothing to do with MCAD, despite the fact I flush after alcohol and during my ever increasing drug reactions – my upcoming appointment with the mast cell consultant should hopefully provide some answers.
Another pointer towards mast cells behaving badly is my reaction to insect bites. While walking my dog in woodland last year I was bitten by what turned out to be a mosquito (luckily they are harmless here in the UK and don’t carry disease). The photo on the left is how the bite looked for a good 3 weeks. Not knowing it was a mosquito bite and because I’d been in woodland which is prime tick territory, I was so concerned about the bullseye nature of the bite reaction, and therefore possible Lyme Disease, that I had it checked out at my local health centre. Thankfully all was well, but I’m fairly sure a histamine reaction like this from a bite is fairly untypical, unless it’s me in which case it’s an everyday occurrence and another reason I don’t holiday abroad
All of these issues have been my normality since I was a baby and have never raised any red flags. It was only after developing M.E. in my mid twenties, and consequently began having strange reactions to medications, that I started to wonder if something might be going seriously awry in my body.
I had my first drug reaction when I was 16 following surgery for Congenital Spinal Stenosis (part of Ehlers-Danlos Syndrome, though of course we didn’t know that at the time). For 3 days after the surgery I had awful muscle spasms particularly over the site of my wound which was incredibly painful, and I had to be injected with a muscle relaxant. The nursing staff were quite nasty with me, saying I was “getting worked up” and needed to simply relax. I later found out that muscle spasms are a known reaction to some types of general anaesthesia.
When I was 17 I picked up a severe tummy bug, and was given an injection of the anti-emetic Stemetil, the active ingredient of which is prochlorperazine. Prochlorperazine belongs to a group of drugs called phenothiazines which act on D2 dopamine receptors in the brain. Dystonic allergic reactions, whereby the muscles jerk and move incontrollably, are a well known side-effect of phenothiazines and I had my first truly allergic reaction in the form of an Oculogyric Crisis.
Apart from phenothiazines I could tolerate any number of medications, and regularly took travel sickness tablets, pain killers such as paracetomol and ibuprofen and the contraceptive pill. In 1993, however, I had a bizarre episode concerning alcohol. I’ve never been a big drinker, always getting to the throwing-up stage before I ever reached the drunk and giggly stage, but did like the odd glass of wine or cider with my evening meal. One Valentines Day, my then boyfriend took me out of for a meal with which we had a lovely bottle of white wine. But within about 15 minutes of my first glass I started to feel really, really hot and when I mentioned this to my boyfriend he said my eyes had gone all red and puffy round the edges. The heat started to spread until I felt like I was going to internally combust, so I made my excuses and went outside into the freezing, wet, February night and lay on the rain-covered pavement to cool off – it was either that, or just pass straight out. I’ve never been able to touch alcohol since and this seemed to be a sign of crappier things to come.
When I developed M.E. in 1994 I had, as part of the illness, severe insomnia. I initially tried supplements from the health shop, one of which was an amino acid called GABA which is supposed to have a sedating effect on the brain. This was the beginning of my extremely unconventional drug reactions, which include tachycardia (ie. increased heart rate), high blood pressure, facial flushing, muscle spasms, intensive all-over nerve pain, severe headaches/pain, oesophageal spasms inducing retching/vomiting, extreme anxiety/agitation, muscle weakness and sometimes dystonic-like reactions including my head tilting backwards uncontrollably, developing a stammer which can take several days to subside, severe muscle weakness, and partial paralysis. I found a video on Youtube of a woman who was having a mast cell reaction – I’ve had reactions with symptoms just like this, especially the speech impediment, but it never crossed my mind to call the doctor or go to A&E as I’d been told by several consultants I was just having a panic attack (see later notes).
As the years have gone by I’ve developed reactions to more and more drugs, many of which like Paracetomol (known as Tylenol in the States) I’ve taken my whole life without incident. I’m now at the stage where I’ve reacted to pain killers of all descriptions and potencies; anti-emetics; sedatives including valium; hypnotics & all brands of sleeping tablets; anti-depressants taken for pain and insomnia including both tricyclics and SSRIs; inhaled steroids taken for bronchitis; muscle relaxants; beta blockers and even some innocuous herbs like camomile and hops which were part of a herbal tea drink. I can currently take one type of antibiotic (Oxytetracyclene) and that’s about my lot! Suffering from a chronic pain disorder and not being able to take pain killers of any description has been particularly trying and there are days I’d give my left arm for some Co-dydramol!
I’ve discussed these drug reactions with various doctors and consultants over the years, and the consensus has been that it is impossible to react in the same way to all these varying classes of drugs. Ergo, it is behavioural, my own fault entirely due to mental weakness and I’m simply having a panic attack. There have been times I’ve cried tears over this, and other times I’ve wanted to batter the doctor with a hammer in anger and frustration. I knew they were wrong, but didn’t know how to prove it.
In 2004 I started to cough. This was no ordinary cough, but a chronic, dry, unproductive, irritated cough which went on, and on, and on and……..well, on. My record is 5 months and I coughed so much I honestly thought I would die from oxygen deprivation. Chest X-rays were normal. No signs of viral infection. My peak-flow was superb, I wasn’t wheezy or gasping for breath, so asthma was ruled out. No-one had a clue what the cough was all about and I just continued to suffer with chest tightness, air hunger, coughing and the feeling that an elephant had taken up residence on my breastbone. This is one of the few symptoms that has really gotten me down and, to be honest, at times scared the absolute crap out of me.
In 2007 I developed hives on my behind. It would start with an itch and a tiny raised, red blister. These would spread over the course of a few days, until they were larger, had raised red edges and white-ish centres, and sometimes spread into each other to form larger, itchier, spots. They would last for weeks on end, I would treat them with Tea Tree cream from the health shop, and eventually they’d just disappear. They’ve been coming and going for absolutely no reason ever since, but are always thankfully confined to my butt for no reason I can fathom.
In March 2011 I went through a particularly terrifying phase of my illness. I started having a mild version of my drug reactions after eating. About 20-30 minutes after food I would feel faint, have tachycardia, my blood pressure would plummet, I’d feel quite anxious, nauseous, have muscle weakness which meant I had to lie down, and an awful pain in my head like my brain was being squeezed in a vice. These symptoms resolved after about an hour, but were extremely scary and I had no idea what was causing them or what to do. By the time each set of symptoms had subsided, and I was virtually back to normal, it was time for my next meal and the whole thing would start all over again. My biggest fear was that the reaction would get so bad that I’d literally be unable to eat and, unlike drugs which you can stop taking, food is a fairly vital component to life!
My GP referred me to my local hospital Gastroenterologist, who arranged for an endoscopy and a belly MRI scan (he actually wanted a CT Scan using contrast dye but, knowing my drug reactions and after discussing it with the scanner operator, it was deemed too risky). The MRI scan came back perfectly fine, which surprised the hell out of me as I’d had lower right abdominal pain for years and I was convinced at least something would show up on the scan to explain it. The endoscopy showed I had several stomach polyps, which were biopsied and found to be benign and therefore not the cause of my eating problems. I was also tested for ulcers, H.Pylori and Coeliac Disease, all of which came back negative. The conclusion was chronic gastritis, probably due to poor gut motility as part of my HEDS, and I was prescribed H2 blockers which I am too terrified to try. I was also diagnosed with GERD (Gastro-oesophageal Reflux Disorder, or heartburn to you and me), despite having no symptoms of heartburn to my knowledge. To treat this I did risk taking some liquid Gaviscon Advance which I seem to tolerate fairly well and now use 3 times per day after meals. Treating the GERD has had 2 major benefits: firstly the “muscle pain” between my shoulder blades, that I’d had for donkeys years, has virtually disappeared. I now think the acid was burning the back of my oesophagus (and therefore causing back pain), rather than the front (causing pain between the breastbone) which is more usual. The second very welcome benefit is that my cough has disappeared. The conclusion can only be that I was inhaling acid from my stomach into my lungs, which caused chronic inflammation in my airways, at times bordering on walking pneumonia.
Thankfully, it was around this time that I joined some EDS forums online and saw several posts about Mast Cell Activation Disorder. Ding, ding, ding ladies and gentlemen, we have another winner! This would explain the GERD, drug reactions, hives, dermographism, flushing, reactions to insect bites and my puberty-related trout pout. I’ve since asked my GP for a referral to an Immunologist who specializes in Mast Cell Disease and my first appointment is scheduled for 19th June.