And finally…it’s Mast Cell Activation Syndrome

A long and winding road….

Can you get a disease before it actually exists?
Turns out you can!
I first became ill in 2005 and ended up completely in the dark in 2006. For the next eight years I tried all sorts of things in the hope of finding a cure. Sometimes I managed to get well enough to go out for walks at dusk and dawn, but sooner or later I always had another relapse. The histamine intolerance approach I’ve described elsewhere on this blog proved the turning point for me and in 2016 I had improved enough to go back to the dermatology clinic in London which I’d last attended in 2006. There I had tests for a group of conditions known as mast cell disorders, but the tests came back negative, and I didn’t pursue this further.

Although I was much better than I had been, some areas of my skin were still excruciatingly sensitive to light. Also, my skin had become very sensitive in other ways, reacting to friction from many kinds of clothing, and to various forms of repetitive movement and exertion. So I was vulnerable to relapses from accidental exposures, or slight miscalculations regarding the amount of movement I could do.

In summer 2018, following energetic gardening under blazing sun (yes, I know – hindsight is a wonderful thing!), I had a very bad relapse, and was back in the dark for large parts of each day. Previously, I’d been contacted by a lady in California who had listened to my book while she was also living in the dark. After tracking down my website she’d started following the histamine intolerance approach, and had got well enough to find a specialist who had diagnosed and then treated her – for a condition called Mast Cell Activation Syndrome. (Read her story at ‘Another Girl Out Of the Dark’.) I became extremely motivated to research mast cell disorders properly. Here’s what I found out:

Mast cells are a vital part of everybody’s immune system. When they detect an injury or an intruder such as bacteria, they release various specialist types of chemical known as mediators to deal with the problem. One of the most important of these mediators is histamine.  Some mast cell disorders such as mastocytosis involve an abnormal proliferation of mast cells in the body. However Mast Cell Activation Syndrome (MCAS), a new disease identified in 2007, does not involve the presence of an excessive number of mast cells. Rather, sufferers have a normal number of mast cells, but the cells themselves react inappropriately and excessively, in a way that goes well beyond allergy, releasing their chemical mediators in response to normal everyday triggers. These unnecessary and powerful chemicals wreak havoc in the body, causing pain, swelling, burning, rashes and many other problems. This condition can affect all systems of the body, so sufferers exhibit a very wide and diverse range of symptoms. The range of triggers are many and highly individual – and can vary in one person across time. Triggers include – but are not limited to – foods, chemicals, smells, heat, cold, exercise… and light.

I also came across the astonishing sentence: “MCAS usually presents with normal tryptase levels; instead, histamine, mast cell-specific prostaglandins and other mediators all need to be assessed.” In fact, while a negative tryptase test rules out mastocytosis, it does not rule out MCAS, which is much harder to diagnose  There are a range of other tests, but at the time they were not available on the NHS.

I became determined to see a mast cell specialist, and these are rare beasts in the UK. When I finally ended up sitting opposite a clinical immunologist who was an expert in this area, and started hesitantly to describe my impossible, incredible history (I mean who lives in the dark for years and then gets a reaction from grating cheese?), his attitude was blissfully refreshing. Basically, he said, “Look, stop worrying about how weird your symptoms are. I’ve seen MCAS causing all sorts of seriously weird symptoms. Let’s see what comes up in the tests.” So off I went to get tested.

MCAS was added to the International Classification for Diseases in 2017. The ICD specifies four diagnostic criteria:.
          1 – a detailed history of symptoms
          2 – the exclusion of possible alternatives
          3 – evidence of a chemical mediator
          4 – response to treatment.
So the first set of tests, which my GP ordered, were to rule out every other possible cause. I’d had many of these during my years of searching for a cure, but I had them all over again. Nothing showed up apart from vitamin D deficiency (surprise). Then, I had to go privately to The Doctors Laboratory in London, to have other tests looking for evidence of a chemical mediator. This evidence is sought from many different angles, because the chemical mediators which mast cells release spike very quickly and then disappear, while their painful and disabling effects on the body last much longer.

The first batch of tests were negative for MCAS, but positive for histamine intolerance. I have a measurably low level of Diamine Oxidase (DAO), which is an enzyme that degrades histamine. And this low level was measurable even on my low histamine diet – the advice is to come off a low histamine diet and eat normally for two weeks prior to the test, but I explained to my consultant that if I did that, I would most likely be unable to attend the lab at all due to a resurgence of my light sensitivity. So my levels must have been low! At last, the reason for my amazing improvement on a low-histamine diet and histamine-reducing supplements was clear.

Then I had the next batch of tests – and there it was. A prostaglandin F2 alpha to creatinine ratio which was above the normal range.

I later found out that because of the evanescent nature of the chemical mediators in the bloodstream, even with the full range of tests it can be hard to catch them in the act. Some people receive a diagnosis of ‘suspected’ or ‘presumed ‘ MCAS based on the other three criteria being met, because their chemical mediators keep slipping through the testing net.

Since receiving my MCAS diagnosis, on the advice of my consultant, I’ve continued with my low histamine diet and supplements, which are also recommended for people with MCAS. I’ve started taking DAOsin, which directly supplements DAO, and gradually added in Clarityn, an antihistamine which reduces the receptivity of my other cells to the chemical mediators which my mast cells are producing in such exciting quantities. I still have ups and downs, but things are definitely improving.

It’s only the start of a journey. There are other medications to explore and experiment with to help manage the condition, but there is currently no cure. People’s symptoms tend to wax and wane, and alter over time; new symptoms and triggers can develop. Compared to the UK, diagnosis and treatment are much better established in Germany and the USA. However knowledge and understanding of MCAS are now developing in some pockets of the NHS. A charity, Mast Cell Action (www.mastcellaction.org), was set up in 2015 to promote awareness and support sufferers, many of whom have spent decades going from specialist to specialist trying to find out what is wrong.

When I wrote my book, it was an unusual illness memoir because it did not have the standard happy ending. At the point of publication in 2015 I was still suffering extreme, incredible and unexplained symptoms, was mostly housebound and frequently in my blacked-out room. Because there was no way I could do the usual kind of book publicity, I stayed in my house and journalists came to interview me in the living room, with the curtains more or less open depending on how well I was at the time. Inevitably, they all asked the same question: “So, have you considered the possibility that your symptoms have a psychological cause?”

I would sigh inwardly and say something along these lines: “Obviously I have considered every possible route out of the dark. However I’ve got four reasons why I think my symptoms have a physical cause. First, my dermatologist, who’s continued to support me over all these years, has never suggested that my symptoms are psychological. In fact he’s been very explicit that there are many dermatological conditions which are currently not well understood. Second, I’ve tried many different approaches over the years, including mind-body techniques. The mind-body techniques have never helped, whereas certain supplements and dietary approaches definitely have, including beta-carotene, which is prescribed on the NHS for various light sensitivity conditions. Third, here is a scientific paper which describes a case very similar to mine in Sweden (and I could now add: since publishing the book I’ve been contacted by people in similar situations all over the world – see my post Sadly Not Unique). Fourth, medical knowledge is still progressing. Symptoms which are not fully understood in the current state of knowledge may nonetheless be genuine physical symptoms. To say either your symptoms can be explained in the current state of knowledge or they must be psychological is a false dichotomy which misunderstands how medical advances happen.”

Well, it turns out I was absolutely right!

It hasn’t been comfortable, being on the cutting edge. But now, in 2020, finally the world’s caught up with me. I’m so grateful to the doctors and researchers who worked to identify and understand MCAS during all those years that I was in the dark. I’ve got an official diagnosis at last, underpinned by measurable biochemical abnormalities. I’ve got a clear direction for the future, and the hope that I can continue to improve.

More about Food

pearVarious people have been asking about the low-histamine diet. Unfortunately, there’s a lot of contradictory information on the web –  I’ve seen the same foods on a “do not eat” list on one site and a “totally ok” list on others; googling can send you quietly mad.  So I thought I’d share some info, based on my own reading and experience.

NB: This blog is not a substitute for professional medical services. Consult a competent professional on health matters.

What is histamine?
     Histamine has many useful functions in the body.  It is released from mast cells as part of a normal immune system response.  When someone has an allergy, e.g. to pollen, it is released in response to a normally harmless substance, causing unpleasant symptoms – hence ‘anti-histamine’ pills.  Histamine also occurs in foods to varying degrees.  Some foods are naturally high in histamine, such as tomatoes.  Others are high because of the way they have been produced.  Ageing, preserving, smoking, fermenting, pickling and processing all increase the histamine content of food, so for example blue cheese, smoked fish, sauerkraut, alcohol and tinned food are all high.  Some foods, additives and medications aren’t high in themselves, but are “histamine liberators” (encourage the body to release histamine) and some are “enzyme blockers” – i.e. reduce the body’s normal capacity to get rid of histamine.
In most people, histamine gets rapidly broken down by enzymes: DAO (histamine in the gut) and MAO-b and HNMT (intra-cellular histamine).  But people with low activity in these enzymes can end up with an imbalance – too much accumulated histamine, and not enough capacity for metabolising it.
Too much histamine can cause various symptoms including headaches, diarrhoea, asthma, runny nose and arrhythmia, plus a whole range of skin symptoms.  Often these symptoms are attributed to food allergies or intolerances which then prove frustratingly hard to manage.  The really interesting thing in my case was that reducing histamine levels reduced my sensitivity to an entirely separate and specific trigger – i.e. light.  I’ve also become less sensitive to other things that used to make me ill, such as dust and certain foods.

Low-histamine diet
Unlike most diets, this one has two elements:
1 What foods to eat – the best list I found is on the Swiss Community of Interest for Histamine Intolerance site
2 How food should be prepared and stored – the best guide to this aspect is a little book called “What HIT me? Living with histamine intolerance” by Genny Masterman.  It’s really worth investing in.  It contains lots of accessible scientific background; plus key tips for managing the diet, including “Learn to cook” and “Your fridge/freezer is your friend!”  Because yes… when you can’t open a tin of beans, snack on a packet of crisps or get a ready meal out of the freezer, you really do have to get cooking…!
I still have flashbacks to my early months on an extremely strict version of the diet.  They seemed to consist mostly of standing amid perpetual clouds of water vapour in the gloom of a blind-screened kitchen as I boiled frozen sweetcorn and steamed frozen fish.
But it’s all been worth it.  And as I’ve got better, I’ve been able to allow myself more leeway.  I eat chocolate now and again.  I’ve even experimented with low-histamine wine, available from a company in Austria called Eller Finest Selections.  (There seems to be much more awareness of histamine intolerance in Germany, Austria and Switzerland compared to the Anglosphere.)
The wine tasted pretty much like normal wine.  I went pink in the face, waved my arms about and started talking very fast about politics.  So one concludes that the effects are pretty normal too.

 

 

Holiday!

I’ve just been on holiday. In our caravan.  In the part of Hampshire where the South Downs is just beginning to roll.  In a week of crazy totally unseasonal September heat (30° on some of the days). Under glorious blue skies and strong sun.

We saw the majestic pines on Lepe beach, the arboretum and herbaceous borders at Exbury,  strolled round the property-lust-inducing village of Chawton and visited the museum in Jane Austen’s house.  We went on a steam train on the Watercress line from Alton to Alresford, had fish and chips in a pub, watched ducks float serenely on the beautifully clear chalk stream.

I wore a long-sleeved jacket and a below-the-knee skirt and a large-brimmed hat and put my UV-protective but nonetheless excitingly lace-trimmed parasol up when the sun was strong and direct.  But I had sandals on my feet, and no additional layers.

2010 was the last year I went on holiday.  During one of my better periods, we visited the same caravan site, a small square field bordered by tall trees.  I knew the sunrise and sunset times by heart; we had a run of early starts and evening strolls, and for the rest of the time I stayed in the van and Pete went off on his own.

This time we dozed luxuriously through the dawn, watched dusk from the caravan windows and went on day trips like normal people.

At last I’m seeing the world in its true colours.  Someone has taken away my box of subtle pastels and given me a primary school paint set.
Red orange yellow green blue purple
Rowan dahlia sunflower grass sky blackberry
Wham!  Kapow!  I’m still reeling.
Hooray for low-histamine diets, histamine-reducing probiotics, and dna-test-targeted supplements.
Hooray for my wonderful J, who gave me back every last leaf.

The Pain of Incredulity

number-4I am on the phone, talking to one of my telephone friends (this one has fibromyalgia and an autoimmune disorder and she lives in South West London). She is describing how badly she is affected when aircraft fly low over the house; it is getting so bad she may have to move, with all the disruption that entails.

And I feel it starting.  Deep down in the centre of my brain: the embryonic stirring of a question mark, a tiny curl of doubt.

Aircraft? Really? Surely things can’t be that bad….  It’s a natural human reaction.  Then I remember my own experience. I am ashamed, and I immediately suppress the thought.

For so many years, my situation was so rare, extreme and unusual that when I described it to people, often in an attempt to seek help, the response was usually incredulity.  Somebody once said that three of the most powerful words in the language are “I believe you.”  Their implied opposite is no less powerful.  Repeated over and over again, the incredulity became a sort of psychic flaying, a periodic acid bath on top of the agonising burning of my skin.

I learnt all sorts of things during my years in the dark.  I learnt how to locate and identify clothes and talking books by touch; how to find ecstatic joy in being well enough to clean the loo; how to sift, from a day of crushing boredom, a tiny nugget that might make my husband laugh.

The biggest lesson of all has been the importance of listening – really listening – to what people are telling me; keeping my mind open, no matter how what they are saying differs from my own experience of life; resisting the temptation to pull up the shutters of scepticism and think “this cannot be”; remembering that we understand only one small part of this wonderful, terrible world.

I needed this so much.  And I’m just immeasurably, immeasurably grateful that the people closest to me gave it, absolutely, freely and without question.

I know many others are not so lucky.

Back to the light

I was pretty normal until my early thirties. Then my skin gradually became excruciatingly sensitive to light.  The condition grew so extreme that I had to spend most of my time in a totally blacked-out room.

Over the years I’ve tried everything to get out of the dark: acupuncture, meditation, hypnotherapy; spiritual healing, strange diets, internet pills; private doctors who could be persuaded to offer a telephone consultation or paid to come to the house.  Sometimes I did manage periods of improvement when I went for walks, Dracula-fashion, at dawn and dusk.  But these patches of hope were fragile and never sustained.  Lying in the dark with my skin on fire I often planned my suicide.

I survived because of the love of my partner and my family.  Because of talking books – I’ve listened to hundreds – that took me to different worlds.  Because of unexpected telephone friendships with other people living with chronic illness.  And because at my absolute lowest ebb, desperate to find something to do in the dark, I started to write about my experience [Girl in the Dark, published by Bloomsbury 2015]. [In USA, published by Penguin Random House]

Then something amazing happened.

The wife of a friend trained as a nutritional therapist.

She persuaded me to be her client number 2.

She looked at my whole case from first principles.

And she found the answer!  Or as I know now – in 2020 – the first part of the answer. I got well enough, thanks to her recommendations, finally, to track down a specialist in Mast Cell Activation Syndrome (MCAS) – and it turns out this is what I have.  MCAS was only identified in 2007, and recognised in the International Classification of Diseases in 2017 – so I managed, like so many other people with MCAS who’ve spent decades going from doctor to doctor trying to find out what is wrong, to get a disease before it officially existed.

I’m still very far from normal and I still have ups and downs – but my underlying improvement has gone far beyond anything I’d dared to hope.

I’m coming back to light, to life and to the world, after so long alone in the dark.  Each new step is intense and surprising and crazy and beautiful.  That’s what I want to share in this blog.

 

Useful Links

People with various degrees of light sensitivity find their way to this site from all over the world. Here are some links which may be useful, in response to the many questions they have asked.

https://lightaware.org/ We need to talk about light! A growing number of people worldwide experience severe and painful reactions to new forms of lighting like CFLs and LEDs. This charity aims to raise awareness and combat social exclusion.

https://www.lupusuk.org.uk/eclipse/ The Eclipse group is part of Lupus UK, but helps people with severe light sensitivity whatever the underlying cause. They provide an excellent product list.

Thick black wool felt is the most light-protective material. I got mine from www.anglotechnicaltextiles.co.uk. Ask for a sample first – it smells a bit.

This scientific paper describes a case in Sweden, which began with a burning face when using a computer and progressed to full-blown extreme cutaneous light sensitivity all over, even through clothing. Olle Johansson Doc

Sticked