HIT v MCAD

I often get asked on my blog what the difference is between Histamine Intolerance and Mast Cell Activation Syndrome, whether there is any link between the two and how to tell if you have one or the other (or like me, both!).   The answer is we know very little about Histamine Intolerance, and not a whole lot more about MCAS, and virtually zero about the two conditions in tandem.

I have no medical qualifications and am not an expert in either disease, so I can only write about my own experience and what I was told by the Consultant who diagnosed me (arguably the UK’s leading expert on both conditions).  Knowledge is an ever changing thing and what I know now may obviously change in the future as more is discovered about my little understood diseases.

I’m as certain as I can be that I was born with Mast Cell Disease.  I have photos of myself as a very young child where I am flushed bright red and my Mum tells me I was dermographic from a tiny baby.  She didn’t think much of it, however, as she was dermographic and got wheals and hives from tight clothing so she thought it was normal, just as she thought my “double jointedness” was normal and my velvety elastic skin was normal because she too was hypermobile and had stretchy skin.  As far as I’m concerned my MCAD is hereditary and genetic.

Flushing as a 3 year old child.

As most of you know, people with Ehlers-Danlos Syndrome are massively more at risk of MCAD than the healthy population but quite why is still a mystery.  There are several theories but they are just that – theories.  The truth is no-one knows.  We also don’t know if MCAD causes EDS, if EDS causes MCAD or if neither causes either and there is another mechanism at play altogether.

My mast cells are activated by all manner of things which the mast cells in a healthy person would rightfully ignore.  Heat, hot water ( very itchy!), cold, cold water (ouch and double ouch!), stress, hormones, some pollens, alcohol, some animals, emotions (particularly excitement), strong winds, exercise, just about all drugs and a host of other situations cause my mast cells to lose the plot, leading from mild problems like nausea or feeling fluey to full blown anaphylaxis and everything inbetween.  But until the age of 44 I could eat whatever I liked and I loved my food.  For the decade I was bedridden with severe M.E., and could do virtually nothing but stare at the ceiling all day, food was my biggest pleasure and I looked forward to mealtimes like an oasis in a burning desert.

Everything changed one day when I was 44 and suddenly started passing out every time I ate.  This was the start of what I discovered was Histamine Intolerance and for me it felt totally separate to my MCAD.  Very little is know about HIT but it’s thought to be caused by a lack of, or badly functioning, enzymes called Diamine Oxidase (DAO) and/or Histamine N-methyltransferase (HNMT).  My HIT was brought under control in about 6 months purely from changing to a low histamine diet, but it’s not ‘cured’.  I still have to maintain a low histamine diet otherwise my symptoms start to creep back and very soon I’m reacting again to everything I put in my mouth (histamine is part of the digestive process for everyone but my poorly enzymes can’t effectively deal with it, so it’s vital I keep my ingested histamine to a minimum).

So, for me, there are some vital differences between my MCAD and my HIT:

• HIT is only related to what I eat or drink.  MCAD is related to my wider environment, both external (eg pollen) and internal (eg emotions).
• HIT developed in middle age.  MCAD has been there since birth.
• HIT causes anaphylaxis-type symptoms only (eg drop in blood pressure, palpitations, nausea, vomiting, fainting, flushing).  MCAD too causes anaphylaxis but also other symptoms (eg itching, dermographia, hives, swelling, sneezing, migraine, pain).
• The symptoms from my HIT subside within 2 hours of eating.  The symptoms from a mast cell flare can last days, weeks even months – mast cell activation perpetuating mast cell activation and causing a neverending vicious circle.

As far as I know no link has been established between HIT and MCAD.  None of the major papers written by world experts on mast cell disease mention HIT as part of the puzzle which, if HIT were a major cause or perpetuating factor of mast cell degranulation, you would think they would.  I’m not saying the two aren’t connected because I have no clue, but for me they feel like very separate entities and my Consultant diagnoses them as two distinct and separate diseases.  That they both involve histamine is intriguing (though the mechanisms are vastly different), but we have no idea if people with MCAD have a higher incidence of HIT than the general population because no data on this exists.  It may just be coincidence if you have both diseases or it may not.  It may be that mast cells degranulate as part of HIT or it may not (I suspect not but that’s just based on my own experience).

When I’m having a mast cell flare, my HIT does appear to worsen.  However, I personally don’t think this proves a causal link.  What I suspect is happening is that my mast cells are producing excess histamine which my body is already struggling to cope with, so when I eat and even more histamine is produced from the act of digestion it causes overload and I get symptoms after eating that I wouldn’t get if my mast cells were behaving themselves.  So food/digestion isn’t causing mast cell degranulation, which is already happening from other causes, it’s just adding to my body’s histamine excess.  I may, however, be totally wrong.  No-one knows what’s happening yet and it’s not an area anyone is researching as far as I’m aware.

There are sites online which appear to contain authoritative and convincing information linking HIT and MCAD but I can find no research, and therefore facts, on the two conditions and none of the articles I’ve read contain links to research papers.  So as far as I’m concerned information linking the two diseases is based on nothing but speculation and guesswork and you all know how I feel about that 😉

So, that’s my take on HIT and MCAD but of course my experience may be different to yours.  According to my Consultant, you can have MCAD without HIT, you can have HIT without MCAD but you can also, of course, have both.

 

 

Advertisements

New Test for Histamine Intolerance

A friend pointed me towards a link they’d seen in their Facebook news feed about a quick test being developed in Germany which anyone could use to measure the histamine content of their food. Can you imagine how absolutely brilliant that would be??!  To know that the food you are eating is actually low histamine, as against guessing its histamine content with nothing but your gut instinct to go on which is the situation at the moment.  It would be HUGE and totally revolutionize my, and thousands of other HIT sufferer’s, lives.

The FB link had come via Genny Masterman’s Histamine Intolerance website. I really like Genny’s site because we have views about HIT in common, for example this post about not believing all the crap online about histamine in foods and low histamine food lists, our distaste at the profiteering which goes on in the histamine world and the money that is being made from people’s suffering, and also that receiving a proper diagnosis (difficult as that may be) is crucial – you cannot diagnose yourself off the internet for heaven’s sake, not least because you could have some other disease which needs totally different treatment. Maybe it’s a  British thing but Genny just calls a spade a spade and there’s no psycho-babble airy-fairy bullshit to wade through.

Back to the article on the new histamine test, the link to which can be found here in German, but which I’ve Google translated below.  Bear in mind though that HIT and Mast Cell Disease are two totally different issues as outlined in my post here.  If you’re expecting a low histamine diet to significantly help your mast cell disease you’re going to be sorely disappointed, though it may play a role in helping some symptoms.  For anyone who is confused about the role of a low histamine diet in MCAD please read my post on the issue here.  For anyone with HIT though (and I have both MCAD and HIT) this test could potentially be fantastic and I’ll be keeping a very close eye on developments.

____________________________________________

Testing instead of forgoing – Quick help with histamine intolerance

Article published 27/09/2017

In Germany, more than two million people suffer from a histamine intolerance. Heart palpitations, stomach pain or rash are the consequences. Tübingen researchers have developed a rapid test that determines the histamine content of food.

A glass of red wine, a long-ripened cheese, a few slices of Parma ham – these are culinary delights that do not pose a problem for most. However, people with histamine intolerance may respond to such foods with symptoms such as cardiac arrhythmia, gastrointestinal discomfort or skin rash. By means of a new rapid test, those affected can determine the histamine content of individual products before consumption. For them, this means a higher quality of life.

The danger can be almost everywhere. Almost all foods contain histamine in a lower or higher concentration. Products which are produced by long ripening or fermentation processes such as wine, fish, cheese or sauerkraut are particularly stressed. The histamine content varies greatly depending on the type and storage. Even one and the same cheese variety can have different histamine values. Histamine is involved as a messenger in the human body involved in the control of various processes such as sleep-wake rhythm, allergic reactions or inflammation. It is produced not only by the body itself, but also by many foods. The substance is usually degraded by enzymes in the intestine. In case of intolerance this degradation is disturbed, so that too much histamine accumulates in the body.

Very limited quality of life

Histamine intolerance can manifest itself in a variety of allergy-like symptoms. These include migraine, anxiety, swollen eyelids, eczema and gastrointestinal discomfort. A clear diagnosis is therefore difficult. The symptoms usually occur two hours after the meal and usually last for half a day. If they want to be safe, they must adhere to a strict diet. “The quality of life is severely limited and can lead to shortages, so it is important to help,” says Christoph Pfefferle, who is currently preparing the founding of ELEFA Bioscience GmbH, which has developed the rapid test for the determination of the histamine content of food ,

So far, it is only in the laboratory how much histamine contains a certain foodstuff. With the new rapid test, people with histamine intolerance can quickly and easily check a product on the spot. The test has the size of a ballpoint pen. With a punching device at the tip, the user can take small samples from the cheese. A special liquid dissolves the histamine from the sample. This is then given to an integrated test strip, which indicates whether the food contains no, little or much histamine. “The principle is similar to a pregnancy test. The result is within five minutes, “says Pfefferle.

However, the test can not give a binding recommendation as the tolerable histamine content is individual in each human being. “Those affected need to assess what they can and can not do on the basis of their experiences. And also take into account what else they have taken, “says Pfefferle.

Further application potentials

In higher doses, histamine leads to poisoning symptoms in all humans, so it can trigger a form of fish poisoning. An EU regulation therefore requires that traders are not allowed to sell fish products with an excessively high histamine content. With the existing technologies, a test can hardly be carried out on the spot, but the quick test could still inform the shopkeeper whether the fish is safe. The test could also be used in wine production. Wines with a high histamine content are considered to be inferior in quality and could thus be sorted out during the manufacturing process.

The rapid test was developed at the Institute of Natural Sciences and Medicine at the University of Tübingen. The idea was born at the “Innovationsakademie Biotechnologie” in 2010. The Federal Ministry for Education and Research ( BMBF ) invited funded researchers, experienced economists and creative cross-thinkers to develop new product and business ideas for this two-day creative event. The path from the idea to the finished product was then thought to be longer than initially at the Histamine rapid test. Without the EUR 650,000 funding from the BMBF for the necessary research and development work, there would be no prototype today. Now the new company ELEFA Bioscience is to lead the histamine test to market maturity. In the course of the coming year it is to be available in the trade. Then people with histamine intolerance could test their foodstuffs and then eat with a corresponding test result also thought-free.

 

Weekly roundup

It’s been a strange week with one thing and another.  My Step-Dad’s Mum, who is 103, has been in a brilliant care home for the past 4 years.  A couple of weeks ago she had a heart attack, but being as though she has a DNR (do not resuscitate) order she wasn’t hospitalized and was cared for in the home.  Ten days ago it looked like she was passing away but the nurse inserted a drip and gave her antibiotics for a chest infection and she rallied.  I do wish the nurse hadn’t intervened because all it achieved was to drag her dying out.  Five days ago the Doctor went to see her and took the drip and meds away.  My Dad has sat faithfully by her bedside ever since and she finally passed away last night.  She’s never liked either me or my Mum for reasons unknown and I’ll go as far as to say she’s been a right bitch to both of us so it’s not like I’m sad about her death, however my Dad will be devastated.  No matter how old or how ill, our Mums are still our Mums.

We had a couple of dry(ish) days at the start of the week so I managed to get a few much needed jobs done in the garden.  It’s rained here in Cumbria almost non-stop for about 6 weeks now and was so cold last night I’ve woken to condensation on my bedroom windows.  FFS, does it not know it’s the middle of bloody summer??!

Thursday I took a friend from Camera  Club to photograph red squirrels at a secret location I don’t shout about.  They’re so cute I could watch them all day.  By mid afternoon I’d started with tummy pain and was a bit flummoxed as to what could be causing it.  It got worse as the day progressed, which is all I needed as I was having 5 other camera club members round to my house in the evening for a meeting about next season’s programme.  The meeting went ahead but I was feeling pretty ropey and couldn’t wait for them all to leave so’s I could get into bed with a hot water bottle.  My period pain always starts initially with stomach pain, but I thought I was only on about day 9 or 10 of my cycle so it couldn’t be that.  Er, wrong!  Woke the next morning to Aunt Flo and when I checked my diary I was actually on day 24 of my cycle – doesn’t seem like two bloody minutes since the last one!  Consequently I was awake for most of Friday night with awful endo-related period pain and a migraine which hung around through most of Saturday.

My lovely furry child Bertie turned 9 this week 🙂  I adopted him when he was 2½ and have no clue where those six years have gone!  I also have no idea what I did with my time or money before I got him but wouldn’t swap him for the world.  It’s because of him I got into photography and for that I will always be grateful.  He is my loyal and faithful companion and as I type this, sitting up in my bed, he is asleep between my knees with his head resting on my thigh.  I love him to the moon and back even though he pinches food the second my back is turned, eats sheep shit then licks my face and barks so much he’s in danger of perforating my eardrums 😉

Today is forecast to be dry and, dare I say it, even a little bit sunny so I’m palming the dog off with my Mum and taking my photography hide down to the river.  In the past 12 months I’ve managed to get good pictures of a red squirrel and a heron, both of which have been on my bucket list for the past 4 years but I still haven’t managed a picture of a Kingfisher.  I know where one is currently perching every day at noon, so I’m hoping if I keep going to where I know he hangs out I’ll eventually manage a shot.  Wish me luck!

No time to cook

It’s been some considerable time since I wrote anything diet/food related on my blog, so I thought I should explain why.  When my MCAD finally exploded back in 2012 I was having anaphylaxis every time I ate anything and sometimes after just drinking water.  I have never been so terrified in all my life and honestly thought I would die, especially as the Doctors I saw just looked at me like I was nuts and told me it wasn’t possible.  I was given diagnoses like gastritis and IBS, which I knew were absolute bullshit but I was told “it’s not cancer” and I should be grateful, then just left to get on with it.  Thank God for the internet and eventually receiving my MCAD and HIT diagnoses from Dr Seneviratne.

For the first two years following my diagnoses I was naturally obsessed with food.  I spent months wading my way through the information online on low histamine diets, adapting recipes to be low in histamine, experimenting and finally finding a plan I could stick to and which (on the whole) controls my symptoms.  I am so grateful to no longer pass out after I have a meal I can’t even tell you, though I’m not always symptom free.  That’s because my mast cells react to a whole host of things other than food, so it depends on any one day how full my histamine bucket is as to whether the mere act of digestion (which produces histamine!) sets off a reaction or not regardless of which food I’m eating.  But the symptoms aren’t anywhere near as extreme as they used to be and, although still not pleasant, are liveable with.

After my condition stabilized I really began investigating low histamine foods and made the shocking discovery that hardly any foods have been tested for their histamine content and every diet online is based, for the most part, on guesswork.  It rocked my world really because my life depends on keeping histamine at bay, yet the information on which I was basing my diet couldn’t be trusted.  I could still be eating ‘safe’ foods which are nothing of the sort and may have been cutting out foods, like egg whites, for absolutely no good reason!  What a bloody nightmare.  So I began experimenting again and discovered I don’t react to baker’s yeast, small amounts of lemon juice, vinegar, stone fruits or fresh berries though chocolate is still off the menu *sob*.

By now it’s 2016, I’m in peri-menopause and am having to contend with extra symptoms on top of the dozens I already have.  My Mum has been diagnosed with severe COPD and Emphysemia, is in heart and kidney failure and has become an alcoholic, while my Dad is in the early stages of Dementia.  They both need help with daily living and the job has fallen to me (quite why it hasn’t fallen to my 3 healthy brothers, their wives or adult children, three of whom ironically work for Home Care Agencies, is another story).  It’s hard enough keeping my own life and home going and I’m finding keeping two homes and three lives going tough.  I’m so busy I meet myself coming back, then am so exhausted from all the extra work and my rampant hormones I literally can’t think straight.

These days I no longer have the time or energy to spend hours in my kitchen thinking up and experimenting with new recipes.  In fact, I spend one day a week cooking then shove what I’ve made in the freezer as I don’t have the time or energy to cook myself dinner every day, let alone anything extra.  In any event, I’ve become quite disillusioned with ‘low histamine’ diets because neither I, nor anyone else, have any idea of the histamine content of individual food items and I now use all sorts of ingredients in my dinners which would cause outrage if I were to put them online.  For example, this week I happened to be in Sainsburys and bought 2 jars of pre-made sauces – don’t fall off your chair in shock 😉  One contained concentrated lemons and the other contained additives!  I know for a fact I won’t react to either, yet if I put them on my low histamine shopping list I’d be crucified, because according to the lists online they contain things I shouldn’t be eating.  Only of course no-one knows if I should be eating them or not because they haven’t been tested for histamine – try telling the low histamine zealots that though!  Obviously my ‘low histamine’ diet has helped my symptoms enormously so obviously some foods are higher in histamine than others, but whether I needed to have cut out all the foods I have is anyone’s guess.  My reduction in symptoms might literally be down to cutting out spinach, cheddar cheese and fish and I’ve been unnecessarily cutting out 30 other foods for no good reason.  Or it might be that I absolutely have to cut out 30 foods in order for my diet to be low in histamine.  I have no idea and neither does anyone else.

According to people like the Low Histamine Chef and others I shouldn’t be eating low histamine anyway, but I should be eating anti-inflammatory.  Only there’s about as much robust evidence for anti-inflammatory diets as there is for low histamine diets.  Point me to the research on people, not rats, that measures inflammation after ingesting a particular food and I might change my mind.  Only of course it doesn’t exist.  It’s as impossible to measure inflammation in our bodies after we’ve eaten a specific food as it is to measure histamine in our bodies after we’ve eaten a specific food.  It’s all such bullshit yet is talked about by these self-proclaimed ‘experts’ as fact.  And just because they include research references at the end of their articles doesn’t make it fact either.  Does anyone actually read the research?  Is it a properly conducted, double blind, randomized, controlled trial which has been peer reviewed and published in a nutritional journal?  Because if it’s not it’s not worth the paper it’s written on.  When I was investigating low histamine foods, I discovered that the only reason egg whites were included in low histamine food lists is because of a tiddly trial done on mice in the 1950s, which was never reviewed or published.  The way food was made and stored in the 1950s bears no resemblance to the way food is made and stored in 2017, so quite why this pseudo half-experiment is quoted as fact in the low histamine world astounds me.  All this stuff plays on the absolute desperation of very sick people and it makes me furious.

It extends beyond the world of histamine though.  For years saturated fats were ‘bad’ for us as they raised cholesterol and gave us heart attacks, until it was discovered that actually heart disease is a much more complex issue, trans fats were much more unhealthy than saturated fats and the jury is still out on how big a role saturates play in plaque formation.  Then salt was bad for us, until it was discovered that eating too little might be as bad as eating too much, though again the debate rages on.  Now it’s sugar that’s the demon, until in 30 years time it will be discovered that without sugar our energy levels are half what they used to be or some other such nonsense.  If we’re honest, we know very little about digestion, diet and the impact what we eat has on our health, and I suspect genes and how we as individuals process food will turn out to be the determining factor for health, rather than the foods we eat per se.

I admit I’m no expert on food and the information here on my blog is simply based on my own thoughts and experience.  And my experience is that I am currently managing my symptoms OK with the diet I choose to follow and, due to changing circumstances in my life, I no longer have the time or energy to experiment with new recipes particularly when I’m not even convinced they are low histamine as I have no evidence to back that theory up.  So apologies to anyone reading my blog and hoping for loads of inspiring recipes and foodie facts.  Having said all that, I hope the information and recipes listed in the menus at the top of my site are useful in your own journey through the histamine maize and at least it’s all free and I’m not making money off the back of other people’s suffering.

A novel experience: part 5

By the time I developed M.E. I’d had a couple of regular allergic reactions to drugs.  The first to anaesthetic and the second to an anti-sickness injection.  These were well recognized reactions, if rare, and very different to the weird reactions I’d go on to develop as part of my mast cell disease.

Most Doctors only recognise full on anaphylactic shock, where your throat swells and you have problems breathing, but anaphylaxis can be graded from 1 to 5 (or I to IV depending on the Grading system used) and as my mast cell disease progressed I started having grade 3 anaphylaxis to just about every drug or supplement I put in my mouth.

My very first mast cell reaction back in 1994 was to alcohol, though I didn’t know it was an anaphylactic reaction at the time.  My second reaction was to a supplement and this time I was fully aware my body was in meltdown though of course didn’t know why.  My Mum, who we are now almost certain has both EDS and MCAD, had quite often reacted badly to drugs so to me it was fairly normal and I really didn’t think much of it.

This exert from my book describes this first serious mast cell reaction.  I’d had ME for about 12 months at this stage and was having awful problems sleeping.  My GP wouldn’t prescribe anything as she said sleeping tablets were addictive, so I thought I’d try something natural.  After all, what harm could it do?

“I swallow the little white pill I’d bought at the Health shop, wincing as it makes its way past my swollen tonsils, the result of yet another throat infection.  The tablet contains something called GABA, which is apparently made naturally by the brain and I’d read in a magazine can cure insomnia.  I’d eat rat poison if I thought it would help and as I pull my sleep mask over my eyes and push in my earplugs, shutting out all external stimulation, I offer up a silent prayer for seven hours of heavenly uninterrupted kip.

Twenty minutes later far from being in the land of nod I’m starting to feel a bit peculiar.  My whole body is tingling and I feel hot, ridiculously hot, so with an irritated sigh I sit up and turn on the bedside lamp, crawling forward on the bed so I can see myself in the dressing table mirror.  I’m shocked to find an ashen face with flaming cheeks staring back at me and I can see my chest inside my pyjama top is mottled scarlet red.  Hmmmm.    My sleepy brain is just starting to register that something’s not quite right when every nerve in my body explodes with pain, pricking my skin with a thousand razor sharp needles.

Owwwa!  What the hell?!  I’m wide awake now and more annoyed than worried, realizing my chances of some shut eye are diminishing by the second, but as I try to exhale my frustration I’m kicked in the chest by an imaginary horse, the thump of its hooves a physical blow which steals every ounce of air in my lungs.   The sensation is so real I slump over, my heart suddenly pounding ferocious beats which thud in my ears and all the while I get hotter and hotter until fire reaches my brain, a raging inferno which melts everything in its path.

Conscious thought is lost to the blaze and I hug my head protectively with my pyjama clad arms, rocking back and forwards to soothe the onslaught.  I’m obviously having a bad reaction to the GABA but it’s nothing like the allergic reactions I’ve had to anti-sickness drugs, which made my eyes roll in my head, or the reaction I had after my spinal surgery as a kid which caused my muscles to spasm so violently my back arched inches off the bed.  No, this is something new and I have no idea what’s happening.

I’m weirdly calm yet antsy with a sudden unstoppable urge to move, despite the fact my body is heavy with fatigue, so I start pacing the length of my bedroom, my heart battering against my ribcage, pain tunnelling along every nerve with each thudding step.  I’m not liking this God I tell my invisible Mentor, not liking it one little bit.  In fact, I’m freaking out so if you feel like a bit of Divine Intervention now would be a really good time.  God’s answer is to make me abruptly nauseous and I only just reach the bathroom before vomiting so violently I’m worried my stomach will rupture.

Three hours later I’m still pacing, a rhythmic voyage round the bed and back keeping my adrenalin fuelled body occupied while my heart does the equivalent of a sprint hurdle, my trudge broken only by the need to retch stringy bile into the toilet bowl.  Please make it go away God I beg.  I’m so tired, so very very tired and my tummy hurts.  My knees buckle and I stumble but despite overwhelming exhaustion I keep moving because when I stop the fire in my veins is so intense I don’t know if I can bear it.  It doesn’t cross my mind to dial 999 because it’s the middle of the night and anyway I hate hospitals and would only want to go to one if I were dying.  Which I’m not.  At least, I don’t think I am.  Corpses have to lie down and be dead and I’m far too wired to be that still.

I shiver uncontrollably, fat goose bumps lifting the hairs on my arms, but when I catch a glimpse of myself in the mirror my face is still alight with burning heat.  I wrap my arms round my ribcage, hugging myself for both warmth and comfort, then try some of the breathing the hypnotist taught me, in through my nose and out through my mouth, in a vain attempt to quieten my thudding heart.  It doesn’t work and I become so out of breath I see stars.

‘For crying out fucking loud!’ I stamp my foot hard on the laminate floor, angry that everything I try only seems to make things worse, then instantly regret my strop as searing pains shoot up my leg, sending shock waves rippling up my spine.  ‘And you can piss off as well!’ I tell the pain petulantly.

Indeterminable hours of pacing and puking later, as the streetlights are replaced by weak winter sunshine, the fire in my brain finally dies to glowing embers and my heart, after one final thumping finale, settles into its usual quiet, steady rhythm like nothing has ever happened.  Not quite trusting that the reaction might finally be over I sit tentatively on the edge of the bed waiting for my nervous system to complain, but although every inch of my skin is tingling like it’s plugged into the electric the sensation is just uncomfortable rather than agonizing.  Relief and crushing exhaustion suck the last ounce of strength from my muscles and I crawl gratefully between the sheets, my brain singed, my stomach raw, blissfully unaware I’d just had the first skirmish in a twenty year war with my immune system which would ultimately try its best to kill me.”

 

The Disease Trilogy

Well, it’s actually a disease quadriology but that didn’t sound quite as good 😉  What on earth am I rabbiting on about now you ask yourself?  Good question, and one I ask often lol!  I’m talking about:

MCAD, hEDS, POTS & M.E. 

which we now know, in some patients, have a bad habit of occurring together.

Mast Cell Activation Disorder/Syndrome (MCAD/MCAS) is a fairly rare disease, although not as rare as previously thought.  There are no statistics available, but mast cell disease is estimated to affect roughly 1 in 2000 people.  When the patient has hypermobile Ehlers-Danlos Syndrome, however, this shoots up to an estimated 1 in 10.  There is obviously something about having hEDS which affects our mast cells in a way that isn’t seen in the rest of the population, which is why I can get arsy about non-EDS patients talking about ‘curing’ or treating MCAD like it applies to everyone – their disease pathway is not the same as my disease pathway.  This is important.

We don’t yet know why people with hEDS are massively more at risk of also having MCAD, but Professor Anne Maitland (Immunologist specializing in hEDS +MCAD) has the following theory: mast cells live in connective tissue.  In hEDS the connective tissue is faulty and in some patients this leads to incorrect messages being sent to the mast cells, making them behave inappropriately.  Dr Maitland is brilliant and in 2016 did a free webinar on hEDS and mast cell disease which is well worth a listen.

On the flip side, MCAD can affect our collagen and make it more stretchy.  I’ve had people comment on my blog that treating their MCAD has ‘cured’ their hEDS and, while I’m happy for them, they are mistaken.  hEDS is much more than having flexible joints so if that’s the only symptom you have you didn’t have hEDS in the first place.  You had lax ligaments as a symptom of MCAD, so treating your mast cells will have had a beneficial impact on your over-bendy joints.

To clarify: in patients with MCAD minus hEDS, misbehaving mast cells make otherwise normal collagen faulty.  In patients with MCAD plus hEDS, faulty collagen makes otherwise normal mast cells misbehave.  So in the first instance, the mast cells are driving the disease, whereas in the second instance the collagen is driving the disease.   In those patients without hEDS, if you can get the mast cells under control the collagen should improve.  But in patients with hEDS there is no effective treatment for the underlying collagen disorder, and without that the mast cells are going to remain overactive.  Yes, hEDS patients can still take mast cell stabilizing drugs but although that may help to control the overactive mast cells it won’t ‘cure’ MCAD, because the mast cells are being driven by faulty collagen for which there is as yet no effective treatment.   I hope that makes sense because I’m ridiculously brain fogged today!

There is increasing recognition amongst doctors in the know that POTS (postural orthostatic tachycardia syndrome) can go hand in hand with MCAD and hEDS and in 2015 a small research study was carried out by Dr Vadas on this very subject.  An estimated 80% of hEDS patients will have POTS and an estimated 1 in 10 of those will also have MCAD, so it’s not an insignificant issue.  Quite why some people have all three diseases no-one knows, but it is a subject a small handful of Doctors are thankfully looking into.  I personally don’t have POTS (ie an increased heart rate when I stand) but I do have Orthostatic Intolerance whereby standing and even sitting for any length of time makes me feel ill, faint, weak and very mentally confused.  There is a good article on both POTS and OI here.

What’s being left out of the current research is the link between hEDS, POTS, MCAD and M.E.  “Chronic fatigue” is talked about but chronic fatigue is not M.E. (although I appreciate M.E. is called CFS in the States).  Most people with M.E. have orthostatic intolerance or POTS – it’s a characteristic of the disease.  And, as my readers know, I think M.E. is a type of mast cell disease.  My reasons for thinking this are:

• M.E. follows a viral infection.  Mast cells can be chronically activated by viruses.
• M.E. has many hallmark symptoms of MCAD, in particular fatigue (mast cells affect the mitochondria), brain fog, problems with cognition, sense hyper-reactivity (ie light, sound, touch) and we now know that the sensory part of our brain called the Diancephalon contains mast cells, ‘allergies’ (the more severe the M.E. the more allergic-type symptoms such as reactions to smells, pollens, chemicals, drugs, foods), muscle twitching/dystonia (mast cells affect smooth muscle contraction), IBS-like symptoms (our GI tract is packed with mast cells), pain (mast cells live next to sensory nerves), sleep disturbance (mast cells affect the sleep/wake cycle).
• Worsening symptoms at menstruation and menopause (mast cells react to hormones),
• Improvement during pregnancy.
• Improvement in symptoms have been seen in some M.E. patients when taking high dose Vitamin C or Tricyclic antidepressants (both are used to treat MCAD).
• Recent research has shown the drug Rituximab can help in M.E.  A similar cancer drug has been found to be helpful in treating MCAD.  Both drugs work on b cells.
• Stress worsens M.E. (mast cells live close to nerves, in particular the nerves which release corticotropin-releasing hormone (or CRH). CRH is a hormone which is released in the ‘flight or fight’ reaction when the body is under stress).
• See more about M.E. and mast cells in my Canary post.

There is a recognized subset of M.E. patients who also have hEDS, and nearly all have either OI or POTS.  In addition, there is not a single one of my many moderate/severe M.E. friends who don’t have some kind of allergy, whether it be asthma, gut issues, food intolerance, drug or chemical intolerance and none of us can tolerate alcohol.  This, alongside the symptoms listed above, scream Mast Cell Disease to me and I wish it were something researchers would look into.  Even if they just tested for mast cell mediators in severe M.E. patients it would be a start and I’d be very interested in the results.

The good news is that things have come on a long way in just the 7 years I’ve been diagnosed with hEDS and there is increasing recognition that collagen disorders, dysautonomia, mast cell disease and M.E. are in some way linked, at least in a subset of people.  This hasn’t filtered down to your average Consultant or GP, so please don’t expect your local hospital or doctor’s surgery to know anything it, but for those health professionals specializing in either MCAD or hEDS they are definitely aware of the link (even my local Rheumatologist knew that hEDS patients often present with MCAD, so if the knowledge has made it to the wilds of the lake district there is hope for everyone 😉 ).

 

 

 

Back pain

When I was a kid we used to live in a house with ramshackle outbuildings.  My friends and I built a little Den in the upstairs of an old barn, which we approached by way of a ladder through a hole in the floor.  One day when I was messing about I stepped backwards, fell through the hole and landed on my back on the bonnet of my Dad’s car which was parked below.  Ouch!  Obviously I hurt my back but after several weeks it didn’t get any better – little did I know then it would hurt for the rest of my life.

I had an x-ray and was told all was fine.  Why, then, a whole year after the accident did my back still hurt?

By the age of 14 the pain was starting to affect my school life and my sleep.  I was on all the sports teams (hockey, netball, tennis) but was having to curtail my training because the pain was intrusive. It was in the lower part of my back, was bilateral, ie it affected both sides of  my spine, and went across my butt cheeks but not down my legs.  It was a dull aching pain which only went when I lay down and I also had some incredibly sore spots particularly in the hollows of both butt cheeks.

My GP referred me to the local hospital who did yet more x-rays.  Everything was still ‘fine’ so they referred me on to Physiotherapy, who gave me some heat treatment and various exercises to do.  The treatment made the pain ten times worse, but when I relayed that to the Physio I was told it wasn’t possible and they implied that I was making the pain out to be worse than it was.

I went back and forwards to the Hospital for 5 years, with the pain getting worse and worse, and in the end was told that my symptoms were psychological and that there was nothing physically wrong with me.   “Do you think referral to a psychologist would be helpful?” I was asked.  “No I fucking do not!” was my answer.

At the age of 16 I could barely sit or stand let alone sleep or play sports, so I went to see my GP, bawled my eyes out and refused to move from her office until she’d referred me to another Hospital for a second opinion.  A few months later I was admitted to the brand new Freeman in Newcastle for 3 days, where they carried out all sorts of tests.  They were the first people to tell me I was “super bendy” but it was only mentioned in passing.  MRIs didn’t exist then but I was given a CT scan, a new invention, very expensive and something my local hospital didn’t have.  The 3D scan showed that one of the vertebrae in the bottom of my spine hadn’t grown properly and was trapping all the nerves and blood vessels which ran through it to my bum and legs.  The fall I’d had was irrelevant and just acted as a catalyst for the pain.  I’d actually been born with the condition, which was rare as it usually develops as we age, and my pain finally had a name: Congenital Spinal Stenosis.  I was also told I had a curve in my spine and diagnosed with mild Scoliosis, though why this hadn’t been picked up on all the X-rays I had done in Carlisle is beyond me.

The only cure for my pain was spinal surgery, which I had 3 months later.  In a procedure called a Laminectomy, the ‘roof’ of the deformed vertebrae was removed which freed the nerves and blood vessels though I was told they had been trapped for so long they were permanently damaged.  I thought the surgery would cure my pain, and while it did lessen it never went away.

When I developed severe M.E. in my late twenties my back used to go into the most awful spasms for which I used to take muscle relaxants (this was before my Mast Cell Disease really took off and I was able to tolerate drugs mostly OK).  My spine was still very flexible, though, and I got some relief by lying on my stomach in a yoga pose.  I also took potent painkillers (co-dydromol) every day, plus anti-depressants to help the pain at night and thereby improve my sleep.  One by one, however, over the years I became allergic to all of them – little did I know my Mast Cell Disease was ramping up ready to explode.

Fast forward to the age of 40 and my “flexibility” started to change.  Lying on my stomach, which increased the arch in my back, was impossible and even lying on my back became problematic.  My pain was no longer confined to my lower back either – it had travelled to my upper back, in particular my ribcage and the area between my shoulder blades.  I was also now getting shooting pains in my upper legs and pelvic bones and standing for even a few minutes was agonizing.  In 2010 I had an MRI of my lumbar (ie lower) spine, which showed absolutely nothing other than some mild osteoarthritis.  Where was the goddamn pain coming from then?!

In 2013 I received my MCAD diagnosis and embarked on a ‘low histamine’ diet (I’m deliberately using inverted commas because there really is no such thing!).  Within a few months my back pain was much improved and the muscle spasms I’d had for years had all but gone.  My flexibility didn’t get any better though, and by this stage I was struggling to bend over to put on my socks or tie my shoe laces, but I wasn’t in constant pain.  It doesn’t take a rocket scientist to work out my mast cell mediators (ie the 30 chemicals released by mast cells when they activate) were influencing the muscle spasms in my back as it’s known that some mediators affect smooth muscle contraction.

For the past 3 years or so my back pain has been really quite good – well, as good as it gets for someone with a progressive connective tissue disorder and an already permanently damaged spine.  And then this year it’s all gone to shit and I have no idea why.  Yesterday I spent a few hours sitting on the floor sorting a pile of paperwork ready for filing, and last night my back was so bloody painful I barely slept a wink.  The pain down my legs is increasing, and now I quite often wake in a morning to find both arms are totally dead so there’s obviously something going on with the nerves in the top of my spine.  I have no clue why my back is suddenly so bad, and no idea what’s causing all the pain and numbness.

To be honest, I’m not going to bother investigating my spinal issues.  If the problem requires surgery I can’t have that as it’s considered too risky because of my MCAD.  If it requires physio I can never do the exercises because it makes my M.E. so much worse, and if it requires drugs I can’t take them as I’m so drug allergic.  I really can’t see the point in putting myself through the stressful diagnostic process when in all probability I won’t be able to cure whatever the issue turns out to be, if indeed anyone can tell me what the issue is!  But, OMG, living with back (and leg, and arm) pain is shit and at 3am this morning I was proper tearful – 39 years of pain is enough for anyone, especially when you can’t take drugs to give yourself a break.

Thank God for my TENS machine, and warm baths, without which I’d be suicidal.  And, yes, before anyone mentions it I’ve had osteopathy, chiropracty, acupuncture, reflexology and every other therapy you can think of – every single one of which gave me a mast cell reaction (the chiropracty made me so ill I had to be blue lighted to hospital in an ambulance in the middle of the night then had to live with my parents for 9 months while my body recovered from the anaphylaxis).  I’d rather live with the pain than risk that again.

I usually try to end my blog posts on a positive, or at least with some humour, but today I’m just too sodding fed up.  I’m on day 3 of awful endo-induced period pains, feel nauseous, am in pain from my ankles to my neck, my right arm is numb and tingling, after only 2½ hours sleep I’m exhausted and to top all that off I didn’t win the €69million Euro jackpot last night when I was convinced my numbers were going to be up 😉   I’m hoping tomorrow will be a better day.