Category Archives: Medical & treatments

Drug Shortages

We have a BIG problem here in the UK that no-one is talking about.  It’s extremely serious and I simply can’t work out why it’s not been headline news.

As soon as the referendum took place in 2016, voting Britain out of the EU, we started to run seriously short of medicines.  Really vital things like anti-rejection drugs for transplant patients, tablets to treat psychosis and heart disease, anaesthetics and even cancer drugs.

Every-day patients started to see shortages of drugs such as HRT, corticosteroids, antibiotics and…………most importantly for me and many of you………….. EPIpens and anti-histamines, amongst loads of other medicines.

I have horrendous GERD.  When I don’t take medication I have the equivalent of a cup-full of bleach in my stomach 24 hours of every day, which comes back up through my lax sphincter muscle into my oesophagus and lungs. I then cough so much I develop walking pneumonia and the pain is so bad I’ve genuinely considered topping myself.

For some-one as drug allergic as I am, finding medication I could tolerate to help with the reflux was a year-long journey, but several anaphylactic reactions to various types of drugs later I found I could take Famotidine, an H2 antagonist which controls stomach acid through an anti-histamine action.  Not only did this help my GERD, but it’s also had a knock-on effect on some other MCAD symptoms like hives and muscle pain.

It’s not the H2 drug of choice for most people.  The common H2 antagonist used to treat reflux is ranitidine, brand-name Zantac.  However, last autumn there was a ranitidine recall as some batches had been found to contain high levels of a potential carcinogen so everyone, and his dog, switched from ranitidine to famotidine causing a global shortage.

I started having issues getting hold of famotidine in November 2019 and it’s been an ongoing problem.  I’ve even tried to import famotidine, in the form of Pepcid a/c, from abroad but it’s just not available.  As a consequence, I’ve gone weeks with the equivalent of Domestos burning me from the inside out which causes ulcers taking months to heal.  If anyone reading this is taking famotidine or Pepcid who doesn’t really need it or could use something else like Omeprazole (a PPI) please, I beg you, switch.

In March this year, at the start of the pandemic, the drug situation in the UK got a whole lot worse.  Now, not only can I not get hold of famotidine for my stomach acid I also can’t get hold of the only brand of pain killer I can tolerate for my horrendously painful migraines.  It’s Sainsburys own child’s ibuprofen suspension, which has been out of stock now for over 2 months.  There are other ibuprofen suspensions, such as Calprofen, but they all contain bloody stupid flavourings, colourings and other shite which my immune system objects to – Sainsburys was the only one that I could take and I’m down to my last 1/3 of a bottle.  Having recently had 9 migraines in 12 days which I’ve barely been able to control with pain killers, I can’t imagine my life without 😥.

No-one really seems to know what the drug shortages here in the UK are really about.  There are loads of theories, but because it’s not really been covered by the media the government haven’t been forced to provide an explanation, so the real reasons are anyone’s guess.  And because it’s been swept under the carpet, the government haven’t been forced into doing anything about it which means it’s just going to continue.

There has to be some silver linings to come out of Covid-19, otherwise it’s all been for nothing.  I hope one of the up-sides is that the UK start to manufacture their own drugs.  That way, we wouldn’t have to depend on other countries who could withdraw or withhold at will.  We have the skill and we have the technology – all it would take is a financial commitment from our government and if they can provide pop-up intensive care hospitals across the country in less than 4 weeks they can provide laboratories to make medicines.

Of course, it’s much more complex than that.  Drugs are patented and licensed, which means the originating company can hold the entire world to ransom – a situation so morally bankrupt it’s sickening.  The world needs to come together on this issue and agree a solution.  It’s do-able, the pandemic has shown us that.  If there is a common will and a common goal, such as a vaccine for Covid-19, we are all willing to give medicine away for free.  I don’t see how that differs to drugs for cancer, a killer disease which affects 1 in 2 of us during our lifetime.

The situation has to change and sooner rather than later.  That it’s gone on this long is reprehensible.

 

Close To Home

Yesterday saw the death from Covid-19 of someone I know well and the seriousness of the situation really hit home.  When you live in a rural area you feel a bit immune to stuff that goes on in cities, but this virus cares not for age, location, sex, ethnicity nor anything else.

The person who died was one of the women who treated me badly at my last camera club, so I have mixed feelings about her passing.  She put me through hell last year when I had done absolutely nothing to deserve it and despite her knowing how difficult my life already was, so I’m not going to be a hypocrite and say I’m deeply affected.  On the other hand, her partner also died who was lovely and I genuinely feel for their families and friends as they can’t have normal funerals where they celebrate the lives of their loved ones and receive support from each other.  I simply can’t imagine losing my Mum or Dad and not having a funeral.  It’s a huge part of the grieving process and I think will have repercussions for those left behind.  I know at least one of my lovely readers is also currently in this position and my heart goes out to her.

Having just heard the news yesterday, I totally lost my shit with my neighbour up the drive.  As regular readers will know, I am currently pursuing legal action against him as he has to travel over the private driveway to my home to access his upholstery business and for the past 4 years has let delivery vans, staff and customers park on my drive to load/offload which blocks me in my garage.

On Sunday, our Government here in the UK announced a total lockdown.  You should only be going in to work if you are classed as “essential”, though can work from home if needed.  So what did I find on Tuesday?  My neighbour working as usual and a customer, who had come to pick up furniture, blocking me in my drive.  Livid doesn’t even begin to describe my reaction.

I am classed as being in the vulnerable group as I am chronically ill, and my 80 yr old neighbours who live only 6ft away from the workshop, are also in total lockdown.  The very last thing we need are people tooing and froing up the drive to our homes, especially as my elderly neighbours have no garden or outdoor space so tend hundreds of pot plants in the driveway and were out doing just that all day in the sunshine to pass the time.  So I sent a very stern email to shit-for-brains telling him to fuck off home.  If he wants to put himself at risk by continuing to work and have customers visiting he can do it at his own house, but not ours.  FFS.

I continue to have mixed feelings about the general public’s reaction to the pandemic.  They are all freaking out at how they will manage financially with no money coming in.  Welcome to our world.  When I first became ill with M.E. and had to stop working I lived alone and had a massive mortgage which I could only pay by taking in lodgers even when I was working.  So despite being bedridden and at times critically ill, I still had to share my home with a succession of total strangers for 10 years so I didn’t end up losing my home.

People are already going stir crazy with boredom and it’s only been a week.  Trying lying on your own, sick in bed, with no internet, mobile phone, TV, radio or books for 23 hours a day for the next 6 years then tell me what stir crazy feels like.  Seriously people, get a grip.

I have friends who have been bedridden and lived in almost total isolation for over 2 decades.  It is not headline news.  Welfare benefits for the sick and disabled continue to be disallowed, or reduced to the point where no-one can possibly survive on them.  Social care is down to 15 minutes twice a day – you can’t possibly make a fresh meal in that time.  Yet the healthy population haven’t given a flying fuck and, once this pandemic is over and their lives return to normal, they will continue not giving a flying fuck.

So I’m afraid my sympathy for healthy people living for a couple of months without their usual income and in isolation (though for the most part with family) and with little health or social care isn’t particularly high.  They are getting a taste of what life is like for millions of chronically ill and elderly people, but at least their life will resume by the end of the summer, unlike ours.

 

Coronavirus

I have conflicting emotions surrounding the outbreak of Coronavirus.  On the one hand, I totally understand the seriousness of a novel virus with rapid transmission which can kill people with pre-existing health conditions, and on the other hand I want to scream that isolation, infection risk and vulnerability are situations already encountered by chronically ill people the world over and few have given a shit for a very, very long time.

I was bedridden with M.E. for a decade.  I lived alone and lay in my bed, seeing and speaking to no-one, for 23 hours and 50 minutes of every single day.  There was no internet, Skype, mobile phones or Whats App in those days and I could only speak for a few minutes on the phone before being totally wiped out.  Not that anyone rang me, as my boyfriend and friends all fucked off.  The only people I ever saw were my parents, who both worked full time and popped in with my dinner each night, and my cleaner who I paid to visit for 3 hours each Wednesday (if I hadn’t paid her she wouldn’t have been there).  I maybe saw my GP twice a year if I begged her to call round, but other than that I lived in complete isolation.  Did anyone give a flying fuck?  That would be a big, fat no.

I dreaded going out for any reason.  Not only did it make me feel so ill I prayed to die to escape the torture, but I also risked picking up any number of community infections – anything from norovirus to the common cold.  And, of course, because my immune system was all to pot, if a virus came within 30 feet of me you could guarantee I would succumb to it.  Healthy people suffer for a few days and then are back to normal, but if you have a compromised immune system all hell can break loose and it can have very serious consequences.  I wish more people realized this.  I went to my Camera club on Wednesday night and a woman sat next to me who was absolutely stinking with cold.  She hacked, coughed and sneezed the entire time while I did my best not to breathe in her germs.  Why the HELL was she there, spreading her infection to all in sundry?!  Well, she didn’t have Coronavirus so apparently it’s fine to infect everyone with her lergie!  Jaysus.

If I get her cold it means I then can’t visit my parents, despite the fact I am their main carer.  My Mum, who has severe heart & lung disease, caught a cold mid December and has spent the last 3 months struggling to breathe.  Last week she was on her 4th lot of antibiotics and her 4th lot of steroids, despite also using a daily steroid inhaler for her COPD.  She looks dreadful and has lost loads of weight because the antibiotics make her feel sick to the pit of her stomach and give her the runs.  Healthy people are incredibly selfish to go out into the community when they are obviously infectious and have no thought to the risk they pose to others less fortunate than themselves.  All the advice over Coronavirus isn’t getting this message home if the situation with the woman at my camera club is anything to go by.

As a kid, I contracted Chicken Pox despite having been immunized.  I was also the only girl in a 1300 pupil school to contract Scarlet Fever, and in my teens I also contracted Glandular Fever (Epstein Barr).  I initially developed M.E. following a tummy bug I caught while working on a luxury cruise liner.  I then developed severe M.E. after contracting viral meningitis while in a 5 star holiday resort in Kenya.  My Mum had a heart attack in 2016 and on my first visit to her in hospital I contracted Norovirus.  My immune system clearly doesn’t work as well as other people’s and doesn’t respond as it should when it comes into contact with viruses.  Yet I look completely healthy!

Community born infections kill vulnerable people all the time yet it doesn’t make the headlines.  28,330 died of the flu in 2014/15 in the UK alone, let alone the figures from the rest of the world.  Coronavirus doesn’t seem to affect children much, even if they get it, nor healthy young adults.  You are more vulnerable to serious complications the older you are, or if you have pre-existing health issues, but that applies to any community born infection.

I’m not saying that Coronavirus isn’t serious, simply that any community born infection can be fatal for the vulnerable – it’s nothing new.  And if you have to self isolate for a week or two quit whining and spare a thought for those people who have been isolated by chronic illness for years and who receive none of the attention being given to those affected by the current situation.   STAY AT HOME if you are infectious with anything.

 

Anaphylaxis

For some strange reason, it’s never occurred to me until today that some of my readers might not know whether or not they are having anaphylaxis.  When I first started having anaphylaxis I had no clue that’s what it was, although I knew it was something pretty catastrophic and I genuinely thought I’d die (turns out that wasn’t an irrational fear!).

Grades of Anaphylaxis

All most of us know about anaphylaxis, if we know anything at all, is actually about anaphylactic shock, ie an acute, life-threatening allergic reaction which results in loss of consciousness and potentially death.  However, anaphylaxis is a graded allergic response, which can vary in severity from mild to severe.  Just to complicate matters, there is no universally agreed grading system but most anaphylaxis charts are a variant of the one below:

ana grade chart

My experience of anaphylaxis

We are all different and in typical Jak form my anaphylactic reactions don’t conform to any of the grades in the table!

Symptom 1 is a massive head rush.  It feels a bit like something has exploded in my brain with a big whoosh and my whole head gets severe pins and needles.  These pins and needles then spread throughout my body and explode with pain in my feet and finger ends.  I know, right there and then, that I am in trouble.  My brain then feels like it’s cramping, just like when you get a charley horse in your calf.

Symptom 2 is the feeling of being thumped hard in the chest, followed by a racing, pounding heart beat.  This comes on only seconds after the head rush.  This of course is accompanied by a rise in blood pressure, usually with at least a 30 point increase in both systolic and diastolic pressures.  Note: this does not fit the usual criteria for anaphylaxis, where there is a drop in blood pressure.

Symptom 3 is facial flushing.  This comes on almost simultaneously with the increased heart rate.  I also have a large, blotchy red rash all over my torso.

Symptom 4 is muscle spasms.  My entire body gets the cramps, my back being particularly badly affected – it can be intensely painful.

Symptom 5 is a huge adrenalin rush.  I absolutely cannot sit still, and usually have to pace up and down, up and down, up and down, virtually wearing a hole in the carpet.  Depending on the severity of the reaction, this has been known to go on for several hours.  It is impossible to either sit or lie quietly in my bed, no matter how utterly exhausted or ill I might be.

Symptom 6 is oesophageal spasms, which lead to me retching and retching and retching.  Sometimes I feel intensely nauseous, other times not.  I don’t vomit, just retch to the point where I can hardly breathe.

Other symptoms include either sweating or shivering, facial pallor in addition to the flushing, needing to pass a bowel movement (though normal in consistency and not diarrhoea), needing to pee, muscle weakness and utterly overwhelming fatigue.

My reactions have never included itching (pruritus) or hives, and I’ve only had very occasional swelling (oedema) of my eyes and/or lips.  I do have itching and hives on a chronic level which I’ll talk about in another post, but never as part of an acute reaction.  I’ve never thankfully been rendered unconscious, though I have fainted.

As anaphylaxis is a graded response, not every reaction is severe.  Yesterday, I was at a friend’s house having a brew.  He’d bought some little mini chocolate fondant cakes to eat which I’d never had before, and within 5 minutes I got the head rush and some mild pins and needles.  My heart started to race and my face to flush, but I was able to control my breathing, the reaction didn’t escalate and after a short while the symptoms settled down.  I carried on the conversation with my friend like nothing was happening and he had no idea I was having an allergic reaction to the cake!

Triggers

Without a doubt, the trigger most likely to bring on a severe anaphylactic reaction for me is medication.  Luckily I can avoid drugs most of the time but when I desperately need to take something, such as pain meds or antibiotics, I can sometimes tolerate a child’s dose, or liquids which I cope with much better than tablets or capsules.  Some drugs, though, are totally off the table, including sedatives, anti-emetics (ie drugs for nausea and vomiting), anti-depressants of any variety and general anaesthesia.

Alcohol is to be avoided at all costs and is the only thing which has ever caused me scary facial swelling.

Foods high in histamine, such as cheddar cheese, tinned tomatoes (which are high in GABA, see below!) and tinned or fresh (aka days old) fish, are also off the menu as they cause instant reactions.  I’ll talk about chronic reactions in my next post.

Other foods which have caused me problems seem to be fairly random, such as the iced fondants mentioned above, chamomile tea, the red jam found in cream cakes like doughnuts, paprika and cayenne pepper (but not curry or chilli powder), yoghurt (though I’m fine with other dairy, so it’s either the fermentation process or the live cultures), some chocolate (but not other chocolate).  Whenever I try something knew I never have any idea how it will go and eating out is still a game of Russian Roulette.

I’ve also had anaphylactic reactions to some supplements including magnesium (including epsom salts), GABA (found naturally in tomatoes to which I also react), sublingual B12, glutamine, melatonin, valarian, lemon balm and the herbal laxative Senacot, hypromellose eye drops, massage, osteopathy, chiropracty and acupuncture.

Menstrual hormones have always caused me problems and it was starting peri-menopause in my early 40s which sent my mast cells into melt down.  I am always more reactive in the week leading up to my period, and have more issues with reflux, itchy skin, insomnia, muscle cramps and migraines.  Roll on the menopause is all I can say, which seems to be taking for sodding ever!

Progression

When I was a very small child, I used to get “chapped lips” in winter.  Almost all my winter primary school photos show me with a huge red welt under my mouth which sometimes reached down to my chin.  Then, when I was around 14, my lips started to swell if I went outside when it was cold and wet.  I know that sounds completely bonkers, but the weather caused my first allergic reaction.  My lips swelled to such an extent that even my GP could see the oedema, but simply couldn’t get her head around the fact it was rain that set it off.  It lasted about 2 years then the swelling just stopped.  I do, though, still get tingly chapped lips when it rains.  As I type this we are having a storm outside and I have done nothing but cover my lips in Chapstick since the minute I got up!  I never mention this to anyone……can you imagine their reaction? 😉

I had my very first acute allergic reaction at the age of 16, following spinal surgery.  I suspect it was the general anaesthetic, but it could actually have been any of the other drugs used in the operation or one of the post op drugs given for pain relief.  It wasn’t a full on anaphylactic reaction and was confined to my muscles, which spasmed so badly for 3 days following the op I had to be given muscle relaxant injections.

The next reaction was an ocular-gyric crisis to the drug Stemetil, which I’d been given because of a severe vomiting bug when I was 17.  I was unwittingly given this drug again when I was 24 and had another severe reaction – it was this which led to me developing M.E.!

My next reaction at the age of 25 was to alcohol, which caused my whole face to swell and me to pass out.  I’d always flushed after drinking alcohol but the allergic reaction came out of the blue and I have no idea what set it off.  I haven’t been able to drink from that day to this – I’d kill for half a cider 😉

The next reaction was to melatonin aged 26, and is one of the scariest reactions I’ve ever had.

During the next 10 years I reacted to one thing after another, including various drugs like paracetomol that I’d used without problems since I was an infant, and innocuous things like chamomile tea and laxatives, plus any kind of bodily manipulation such as massage or osteopathy.

By the time I was 40, almost all drugs were out of the question.

At the age of 44 I started having anaphylaxis to food after having no food reactions before in my life.  At the time it seemed random and anything would set the reaction off, though of course I later discovered it was to foods high in histamine.

Avoidance

As my biggest trigger is drugs, on the whole it’s easy to avoid them.  Things, however, become more tricky when I absolutely need to take meds, such as having an infection requiring antibiotics or anaesthetic for dental or other procedures.  In these situations I avoid drugs I’ve reacted to in the past, and just keep my fingers crossed when trying anything new.

Going on a low histamine diet was an absolute game-changer for me.  It took several months for my food reactions to calm down, but now it’s rare I react to something and if I do it tends to be mild and manageable.

I am now very careful not to get bitten by insects, so use a non-chemical insect repellant spray during spring, summer and autumn whenever I’m outdoors, even in the garden.

I know stress and strong emotions (both positive and negative) cause my mast cells to have a hissy fit, so keeping my life calm, avoiding conflict, and having a strict routine where I eat well and get plenty of rest is massively important.

Treatment

Due to past reactions with supplements I have never had the courage to try Quercetin, Vitamin C or any of the other things recommended for MCAD.  I do, however, take a Vitamin D supplement as I’m largely housebound and live in the north of England where sun isn’t exactly at a premium, and iron as my ferritin stores were low.  You’ll notice both are in liquid form – I avoid tablets and capsules like the plague.

There are 2 types of antihistamines used in HIT and MCAD:

  • H1 blockers like cetirizine (Zyrtec), diphenhydramine (Benedryl), Chlorphenamine (Piriton) and loratadine (Claritin) used to treat hayfever-type symptoms, or in the case of Benedryl more acute allergic reactions;
  • and H2 blockers like Ranitidine (Zantac), Cimetidine (Tagamet) & Famotidine (Pepcid) used to treat reflux, which occurs from an abundance of overly-zealous mast cells in the stomach.

I have had reactions to various H1 antihistamines so am very careful with them.  Luckily I have found one liquid brand of H1 antihistamine I can tolerate, but will only take it if I have really troublesome hayfever issues (usually in the spring).

I’ve also had problems with H2 antihistamines.  I frequently react to ranitidine, so am currently on famotidine.  I have reacted to famotidine in the past too, but after a break have started to tolerate it again – weird, but then that’s my life 😉

Many people with MCAD and/or HIT have a big problem with stomach acid/GERD and in addition to H2 antihistamines I also use Gaviscon liquid which creates a barrier to stop the acid from entering my oesophagus which, trust me when I say, is pure torture.

I have chronic hives and neither H1 nor H2 antihistamines have any effect on them whatsoever.  If they become really itchy and bothersome I have to resort to topical steroid cream, which is the only thing that will clear them up.

All the above keep my symptoms at a manageable level and I thankfully have never had to resort to more potent mast cell stabilizing drugs such as sodium cromoglycate, montelukast, ketotifen or similar.  I would be petrified to even try them, having had a reaction to both steroid tablets and inhalers, both of which are used to treat acute allergic reactions :-/

Conclusion

MCAD doesn’t conform to what most doctors are taught about allergic reactions.  We don’t even have ordinary anaphylaxis, which is driven by an IgE immune reaction, and little is currently understood about the mechanisms which lead to symptoms in mast cell activation disorder.

We are all, also, individual and your reactions and triggers will differ to mine, as will your response and reaction to treatments.

I think the best any of us can hope for is to identify, and avoid, triggers and to work out a treatment plan which keeps the illness manageable, which is no easy feat and can take a long time.  I feel lucky that my MCAD has been stable now for a good 5 years which isn’t to say I don’t have symptoms and no longer react to anything, but my reactions are milder and I’ve been able to identify and avoid the things which launch my mast cells into orbit.  Long may that continue!

 

 

 

If it quacks like a duck..

As you know, my Dad has been having symptoms of an unspecified autoimmune disease for over 3 years now.  The facts are:

  • He has confirmed severe sensorimotor polyneuropathy
  • He has confirmed Mild Cognitive Impairment
  • He has confirmed inflammatory markers in his spinal fluid
  • He has a small IgA kappa paraprotein in his blood, which is rare as kappa paraproteins are usually IgG.
  • He is B12 deficient.
  • He has a dry mouth, nose and eyes which wax and wane in severity
  • He is constantly fatigued, ie he sleeps for 10 hours is only up for 3 then nods back off in the chair.  He also nods off every afternoon and sometimes again while watching telly in the evening.

There is clearly something amiss, but after travelling 3,000 miles, to 5 different hospitals and seeing nearly a dozen consultants we are no further forward in finding out the cause of his symptoms.  He doesn’t fit neatly into one illness profile and as each consultant only looks at their own speciality, ignoring anything else, no-one is piecing together the bits of the puzzle.

My best friend has also been struggling for over 2 years now to receive a diagnosis to explain what we suspect are auto-immune symptoms.  One consultant said she had Palindromic Rheumatism, but 2 others have said she doesn’t!   Her fingers have been swelling, turning weird colours, her skin is hard, shiny and flaky and she has pain and stiffness.  She:

  • already has an M.E. diagnosis
  • already has a POTS diagnosis
  • already has a Coeliac diagnosis
  • has seen a marked increase in period pain
  • has back pain which keeps her awake at night
  • is low in iron, despite taking the equivalent of a handful of nails every day as a supplement and being a meat eater
  • has had blood results which aren’t right, but aren’t quite wrong enough for an auto-immune disease
  • is under-weight despite eating relatively well
  • has been having infrequent bouts of severe stomach pain and vomiting

A uterine scan showed no problems such as fibroids or cysts.  A scan of her hands showed the joints were not inflamed and, as her Rheumatoid factor blood work is all negative, she was told she didn’t have Rheumatoid Arthritis which she flippin’ well knew already.   The scan did, however, show swelling in the soft tissues in her hands but the rheumy said that wasn’t his area of expertise – well who’s is it, then, exactly?  Your fingers don’t randomly swell up like a bloody sausage and turn bright purple for no reason.

Both my friend and my Dad are so fed up of the fight for diagnosis they’re giving up.  They can’t face yet another 6 month wait for an appointment to see a consultant who tells them everything they don’t have and not what they do.

Of course, this scenario won’t be news for many of you who, like me, struggled to get diagnosed with hEDS, MCAS or both.

Despite a lifetime history of classic hEDS symptoms, including:

  • joint hypermobility which was commented on by both doctors and physios
  • visible veins
  • blue sclera
  • soft, velvety, stretchy skin with tore easily
  • rare issues like congenital spinal stenosis and scoliosis (both linked to EDS)
  • gut issues since I was a very small child
  • dental overcrowing necessitating tooth extraction
  • easy bruising
  • cigarette paper surgical scar
  • GERD
  • hiatus hernia
  • gastritis
  • lifelong constipation
  • and the fact my Mum is hypermobile, has pectus carinatum (a common finding in all types of EDS & Marfans) and had a collapsed lung for no reason in her early forties (a complication of EDS)

    no-one even mentioned connective tissue.

I was told my chronic pain from childhood was because my parents had an unhappy marriage, I focused too much on my symptoms, was deconditioned as a result of having M.E. and/or was simply “overly sensitive to pain”!   It didn’t help that every x-ray, blood test and scan was ‘normal’, from which the doctors concluded there couldn’t be anything physically wrong with me.  Yet again no-one joined the dots.

Of course, getting Mast Cell Activation Disorder diagnosed was much, much harder!  All the symptoms were there:

  • flushing since birth
  • dermographism since birth
  • 2 years of lip swelling at puberty
  • chronic migraine, which began at puberty
  • pruritus (itching)
  • chronic hives which began at peri-menopause
  • hayfever which also began at peri-menopause
  • going from being allergic to 1 drug as a teenager, to just about all drugs by the time I hit peri-menopause
  • going from no food reactions to passing out every time I ate, at peri-menopause
  • awful gut spasms and pain
  • chronic, unremitting nausea
  • oesophageal spasms
  • difficulty swallowing
  • chronic sinusitis
  • tongue tingling and throat itching
  • chronic cough
  • over a dozen episodes of acute, grade III anaphylaxis
  • and of course a diagnosis of both hEDS and M.E. (common MCAS bedfellows) not to mention endometriosis (another common link)

Yet I was told for years that my allergic reactions were actually panic attacks (not sure how that causes hives, dermographism or your lips to swell?!) because it was physically impossible to react to all drugs or all foods – this little gem, despite no tryptase or histamine testing during a reaction.  My IgE (antibodies to an allergen) was normal y’see and that’s all the testing they needed.  Oh, apart from when they actually did proper skin prick testing and found I was actually allergic to birch pollen, apples and my dog – FFS!

It is absolutely necessary to have diagnostic criteria for diseases.  Having M.E. has taught me that, because I swear when a definitive test is actually developed around 1/3 of those currently diagnosed with ME will be found not to have it.  Having said all that, diseases have common histories and symptoms and in the absence of a firm diagnostic test if it looks like a duck and quacks like a duck, the chances are it’s not going to be a fucking elephant but a small, feathered bird that loves water!

Both my Dad and my friend have obviously got some kind of autoimmune problem.  They don’t fit neatly into a single disease profile but that isn’t a reason not to diagnose them with anything or not to try any form of treatment.  Similarly, if someone clearly has mast cell mediator release it’s important to recognize that, even if specific testing isn’t available, and to treat accordingly.  What’s not acceptable is to say you don’t perfectly fit the criteria for x, y or z so we are discharging you from our care, or even worse that your symptoms must therefore be all in your head.

In an ideal world…

….. what would I actually want and need from a good health-care service?  We whinge a lot about what we don’t receive and don’t talk about what we’d ideally like.

1.   BELIEVE US

A poll was conducted recently on the ME Association website about this very issue, and number 1 on the list of things we’d all like is simply to be believed.  It’s not much to ask is it, doesn’t cost anything, but would make a massive difference to our experience of living with chronic illness.  Yet I know it often doesn’t happen.

My first example of not being believed took place when I was just 14 years old.  I’d had back pain since I was 11, brought on by a fall through the 1st floor of an outbuilding, yet because nothing showed up on x-rays and I complained that physio made me worse not better, I was told I was attention seeking and was obviously just unhappy due to problems at home.  And then when I was 16 CT scans were invented.   I insisted on having one, which showed rare congenital spinal stenosis necessitating urgent decompression surgery.  Obviously it’s not the medical profession’s fault that X-rays don’t show this kind of issue and CTs weren’t available, however to tell a teenager who is sleeping on a board due to severe back pain and can no longer take part in the sports she loves that her pain is “all in her head” is wrong on every level.  This disbelief and blaming of me, the patient, for my own health issues has been a long running theme throughout my life and makes me really, really angry.  That anger transfers as mistrust in the medical profession, which in turn does not lend itself to good relations with my caregivers.  All it would take for this mistrust not to have developed would be for a doctor to have said to the young me “I know you’re in pain, I know there’s a pysical problem, but unfortunately we don’t yet know what that problem is.  While we figure it out we’ll work together on pain relief until we find something which works for you”.

2. COMPASSION

Hot on the heels of being believed is to be treated with empathy and compassion, which you would think is the bedrock of medical care yet has been largely absent in my dealings with doctors.

Following meningitis, I was bedridden with M.E. for a decade during which time I spent 4 months in a semi-coma unable to open my eyes and crawled to the loo and back on my hands and knees.  Yet I vividly remember a visit from a GP who said to me and I quote “my wife is away, I have 2 children and a full-time job – you don’t know what tired is!” and he was deadly serious.  A subsequent visit from a locum GP told my parents I was so critically ill I might not make it through the night and asked whether I wanted to spend my final hours at home or in hospital.

We’re not daft enough to think that doctors have all the answers to our health issues, but to be treated with dignity and respect costs nothing and I do expect that from the people involved in my care.

3. MONITORING

I have been ill now for 26 years and during that time I have had no monitoring from my GP, nor anyone else.  I was bedridden for years, which can cause pressure sores, muscle wasting and issues with continence, yet I only ever saw my GP if I requested it and was never visited at home by a nurse.

As we age, regular monitoring becomes ever more important.  We are more prone to osteoporosis, blood pressure issues, high cholesterol and heart problems due to lack of activity, plus vitamin D deficiency if we are housebound, and things like low thyroid function, B12 deficiency and anaemia are easily missed when one is exhausted all the time.   It’s not too much to ask to have a yearly medical and blood tests.  It would cost peanuts in the scheme of things and take 15 minutes of a nurse’s time (30 mins if they had to come to the house).

4. A SPECIALIST POINT OF CONTACT

Getting on to the nitty gritty of living with chronic and or/complex diseases, I’m sure all of us would love a single point of specialist contact like MS patients have with their MS nurse.  My GP is really nice and very approachable, but getting in touch with her is like pulling teeth and she has 7 minutes in which to speak to me, consequently our conversations are rushed and stressful and I know she can’t wait to get off the phone and on to her next patient.  Nurses often have more time and if one had been trained in my particular illnesses and I could ring her for advice on a particular injury, or worrying symptom, a change of drugs or even simple things like being consitpated it would make the world of difference to me.  I have felt utterly alone in dealing with my diseases over the decades which has definitely impacted my mental health.

5. SKYPE!

We are very lucky to live in the digital age, yet here in the UK we seem to be stuck in the 20th century.  When you have a disease like M.E., and just getting dressed can make you feel like you’re dying, medical professionals need to make more use of the internet and Skype appointments.  We don’t always need to be physically present for a consultation and it would make the world of difference to severely ill patients if they could speak to medical professionals over the net, or even over the phone!  This is particularly important for patients, like myself, who live in rural areas and have to travel huge distances to hospital.

As my regular readers know, my Dad is having health problems and is under the care of neurology at a hospital 90 miles away.  We had an appointment recently with the spinal surgeon.  It had taken us nearly 2 hours to get there, the Clinic was running late so we’d sat in the waiting room for an hour, and we went in to the appointment only to be told “I’m not going to do your spinal surgery until you’ve seen the orthopaeds about your hips, so make another appointment at reception for about 6 months time.”  Really?!  Literally an entire day spent travelling and sitting around a hospital for an 80 year old with crippling back pain for that, when he could have rung us and said the same thing.

6. CO-ORDINATED CARE

This is the Holy Grail for anyone with multiple and/or complex diseases.  At the moment each Consultant deals with their particular speciality and doesn’t talk to the other health-care providers.  My Dad is a case in point.  He needs spinal surgery so is under the spinal team at Newcastle.  He’s also under neurology at Newcastle due to severe neuropathy.  He’s been diagnosed with the auto-immune disease Sjogren’s Syndrome, so will be seen by Rheumatology at our local hospital.  He also has a kappa paraprotein in his blood so is under Haematology at our local hospital.  He needs a hip replacement, so is being seen by Orthopaedics at Hexham hospital.  He is B12 deficient, so has injections at our local health centre from the nurse.  He needs cataract surgery, whichis being performed at Sunderland Eye Infirmary.  And his GP works 2 days a week so we can never get an appointment with her.  There are times I’ve literally pulled my hair out trying to co-ordinate his care and, before his diagnoses, for each Consultant to speak to the other Consultants about his symptoms.

Because each Consultant works independently, care is not continual.  Again, my Dad is a classic case.  The local muscular-skeletal team knew he had spinal stenosis so referred him to the surgeons at Newcastle.  He was then seen as a new patient, so his MRI was unnecessarily repeated and he was then placed at the bottom of an 8 month waiting list.  When we finally saw the surgeon he said my Dad had more pressing issues with his nervous system, so referred him to neurology where he had yet another unnecessary MRI scan, then was seen as a new patient and placed at the bottom of a 6 moth waiting list (it was only my huge efforts for him to be seen urgently which resulted in a faster appointment).  The neurologist ordered tests for Sjogren’s Syndrome which came back positive, so referred him to Rheumatology where he is now being seen as a new patient, will have all his tests unnecessarily repeated and placed at the bottom of an 8 month waiting list.  And so on ad nauseum.  It has taken 1½ years to finally get to the bottom of his multiple issues because for each referral he was treated as a new patient and all the tests and investigations were ordered afresh.  If you are referred by a Consultant to another Consultant you should be treated as an ongoing patient, which would mean much faster appointments.  Test results also need to be transferred with you so they don’t have to be repeated!  It’s not rocket-science, particularly when we have the internet and patient records can be accessed online from any hospital in the country.

7. ACCESS TO SPECIALISTS

There will always be times when you need to seek specialist help, however accessing knowledgeable Consultants is usually the hardest part of having rare and/or misunderstood diseases.

There are various ME/CFS clinics around England – my nearest is a 180 mile round trip away.  There are only 2 specialist EDS Centres in England – both are a 6 hour round train journey away, and in any event don’t see hEDS patients who also have M.E.
My nearest MCAS consultant is also a 6 hour train journey away, and recently refused to see me.
There are no specialist services in Scotland, Wales or Northern Ireland for any of my 3 diseases as far as I’m aware which is a disgrace and I’m surprised patients haven’t banded together and sued the health service for lack of care.

My GP is under the impression that, having been diagnosed, I don’t need to see a Consultant because none of my conditions are curable and she can provide ongoing care.  This is not the case.  I have severe endometriosis and need a hysterectomy.  I was seen at the specialist endo centre, 90 miles away, last year but due to my severe MCAS-related drug allergies they wanted to speak to my Mast Cell consultant before deciding on surgery.  Only, I don’t have one and because there was no-one for the Gynaecologist to consult with over my MCAS she decided blind surgery would be too risky, so I’m now continuing to live with excruciating chronic pain instead.  A few years ago I needed a tooth extraction.   I had to have this done in hospital as the tooth roots were near my sinus cavity, and again the Dentist wanted to speak to my MCAS consultant about how this would impact my mast cell disease.  Only, I don’t have one.  I recently had an endoscopy for my stomach issues and severe GERD and in order to know if my issues were mast cell related I needed a biopsy where the cells were ‘blue stained’.  I requested this, only they wouldn’t do it because the order hadn’t come from a Consultant.  That’s because I don’t have one.  So now I know I have gastritis but not why.  It’s for situations like these that having a specialist referral point is so important, yet they are lacking for the majority of M.E., hEDS and Mast Cell Disease patients.

CONCLUSION

Notice that not one of my wish list includes actually being ‘cured’.  I know that’s not possible, but just because I can’t be ‘cured’ doesn’t mean I shouldn’t be monitored or have access to specialist services should they be needed.  Two of the items on my wish list would cost money, but the majority would either cost nothing or peanuts.  All it would take is a will to offer services for severe, chronic and/or complex patients and it’s the will which is lacking, for which there really is no excuse.

Another M.E. Death

The M.E. community were shocked and saddened to hear of the death of M.E. warrior Jennifer Chittick yesterday (24th May 2019).  Jenni had endured severe M.E. for a decade, was bedridden and reliant on her parents for care.  We don’t know the cause of death, but to lose your life aged just 31 is absolutely tragic and my heart goes out to her family and friends.

Just a day before she died Jenni had been the subject of an excellent article in the Daily Mail newspaper on the abuse and neglect faced by M.E. patients.   Her story echoed mine and her tragedy could so very easily have been mine.

I became severely affected by M.E. aged 26.  I lived alone and was told by my GP “we don’t have enough home care workers for people with cancer so you stand no chance” and I was just left to look after myself, despite the fact I once spent 4 months barely able to open my eyelids.  For 6 years I crawled to the loo on my hands and knees and never cleaned my teeth.  My Mum made a meal for me every day, after working full time, and my Dad drove across town with it in the car on a tray for me – it’s the only nutrition I had other than cereal and the odd smoothie.

At one stage I was so ill I simply couldn’t manage any longer at home alone.  My GP arranged for me to be admitted for respite care to the neurological rehab wing of my local hospital.  There were no doctors there and I was mostly looked after by physios – this was a ‘rehab’ ward after all!  I didn’t know then that I had Ehlers-Danlos syndrome, and I was told it was brilliant I was so flexible!  I had huge problems eating solid foods, not least due to oesophageal spasms (now known to be due to mast cell disease) and gastroparesis (now known to be due to hEDS), so the psychiatrist was sent for and she told me I had anorexia, despite the fact I was starving, wanted to eat but was just too ill to.  I had a seizure in the presence of a nurse one day and she didn’t touch me or send for a doctor …………I’m sure if I looked at my notes it would have some psychological explanation, even though I couldn’t move my entire right side for a whole day afterwards.  I was given no help with bathing, despite the fact it was one of the things I struggled the most with.   In the end, after 3 weeks of zero help and disbelief, and being looked after by a girl who used to bully me at school, I discharged myself and went back to living at home alone.  It was pure luck I survived the next decade, both physically and mentally.

My experience happened back in the late 1990s/early 2000s.  It makes me furious that the situation hasn’t improved in the intervening two decades.  Just because a cause hasn’t yet been found for the disease shouldn’t mean we are treated as subhuman or denied basic care and compassion.  The denial of M.E. as a physical disease, and the neglect and abuse of patients, will eventually be uncovered as one of the biggest scandals of modern times.

Still searching

In 2017 my Dad was walking 6 miles up a mountain every week with his best mate.  He got a bit stiff afterwards but other than that he was in great nick for a man of 77.

In August of 2017 he caught a mild cold.  He passed it to my Mum and by September she was paralysed.  The cold had triggered Guillain Barre Syndrome, an auto-immune disease.  Looking back, however, my Dad was also affected by the cold and started having some leg weakness after his walks, along with chronic fatigue and ever increasing stiffness.

In December 2017 he was concerned enough to see his GP, who did some routine blood work and found a small IgA kappa paraprotein.  Paraproteins can be a precursor to lymphoma, however in the early stages they are classed as MGUS (monoclonal gammopathy of undetermined significance) so he is now regularly monitored.

In January 2018 he first saw the spinal team who ordered an MRI scan of his spine.  They found a narrowing of his spinal cord called spinal stenosis and referred him to the surgeons at the RVI in Newcastle.

We waited 8 months for our first appointment at the RVI, during which time my Dad deteriorated.  In June he had to stop walking the fells with his friend, but could still potter to the local shops and back.  He also started having dizziness & nausea spells, about one every two months.

His GP referred him to the heart unit, who found he had bradycardia (a very slow heart beat) when he slept but apart from that all was fine with his ticker.

His dizziness & nausea episodes increased over the next few months, until he had actual vertigo with prolonged projectile vomiting about every 2-3 weeks.  His hands had also periodically started cramping and going spastic.  His walking ability also continued to decline and by the time we saw the actual Spinal Surgeon in November 2018 I had to take him to the appointment in a wheelchair as he couldn’t walk from the car park to the Clinic.

The Surgeon said the stenosis in his spine was mild and couldn’t possibly be causing the severe weakness in his legs so referred him for an upper MRI scan and some nerve conduction tests.  The MRI was fine but the nerve tests showed severe sensorimotor neuropathy in both legs.

The Surgeon strongly suspected CIDP (chronic inflammatory demylinating polyneuropathy), an inflammatory auto-immune disease which eats away at the myelin sheath around the nerves and can be caused by viruses (like the cold that led to my Mum’s GBS).  He cancelled my Dad’s surgery as he said the nerve issue needed to be sorted first.  However, he didn’t refer us to anyone so we were left floundering alone in the dark.

My Dad was getting worse by the week, so in December I took matters into my own hands.  I found a neurologist at the RVI who specialized in CIDP, pinched his email address from a paper he’d written, and emailed him directly – frowned upon here in the UK!  However, he was very kind and emailed me straight back.  To cut a long story short my Dad was referred to the rapid access neurology unit and seen by a Neurologist on Christmas Eve.

She ordered a lumbar puncture, which showed raised CSF protein.  However, the protein wasn’t indicative of CIDP, so now they had no clue what was causing his severe sensorimotor polyneuropathy.

The Neurologist ordered a CT scan, as raised CSF protein can indicate cancer.  My Dad had this done at the start of February and every 2 weeks since I’ve rung for the results, which have never been available.

It is now April, 7½ weeks since his scan and 18 months since he first started to have problems with his legs.  We are no further forward and were so pissed off with being passed from pillar to post on the NHS and having to waits months and months for every little thing we made an appointment to see a private neurologist next Tuesday.  However, yesterday I decided to have one more go at getting my Dad’s CT results (to take with us to the private appointment) and discovered they were back but hadn’t been seen by the Neurologist yet – fuck knows when that was going to happen.

However, yesterday afternoon the Neurologist’s secretary rang to say that the Neurologist had now seen the CT scan and all was fine.
“So when is our follow up appointment?” I ask.
Secretary:  “Were you expecting a follow up appointment?”
ME:  “Well, being as though 18 months ago my Dad could walk 6 miles up a mountain and is now in a wheelchair, we have no clue what’s wrong with him and he’s receiving no treatment, yes I do think we’d like a follow up appointment!”  FFS!
So she slotted us in today, being as though the Neurologist was about to go on annual leave.  If I hadn’t rung yesterday to chase his CT scan results for the fourth time we’d still have been none the wiser by May!

So today I’ve driven another 180 miles to see the Neurologist, who to be fair is very nice and very thorough.  Here’s where we are:

  • She has no idea how much his spinal stenosis is contributing to his symptoms.  Do we go ahead with surgery nor not?
  • She was concerned my Dad may have arthritis in his hips and/or knees which is contributing to his pain, so we had all of those xrayed while we were there.  No results yet.
  • She has no clue what is causing his severe neuropathy.  30% of people have idiopathic peripheral neuropathy, ie no cause is ever found, however they don’t tend to have quickly progressing PN like my Dad so she thinks there must be an underlying cause.
  • As he also has a dry mouth and dry eyes she’s wondering about Sjogren’s Syndrome (another auto-immune disease which can cause neuropathy), so is referring him to a Rheumatologist.
  • In the meantime, she’s getting a second 2nd opinion from the CIDP specialist, just to check again.  She’s going to ask if it’s worthwhile doing a nerve biopsy to check if inflammation is present once and for all.  If it is, it might still be CIDP (it fits his symptoms more than any other diagnosis). If it isn’t we’re up the shittiest creek in Shitsville without a proverbial paddle.

So it’s yet more waiting, and testing, and waiting for the results – if we don’t all lose the will to live in the meantime!

 

Another lump

It’s been over a week now since I saw my GP about my armpit lump and swelling.  As my doctor suspects something sinister I’m supposed to be being seen under the 2 week cancer rule, but so far I’ve not heard a word about my scan :-/

To rub salt into the wounds, the same day I saw my GP I took a friend through to the city to have a consultation about his bad back.  I heard today that he’s already been for an MRI scan, despite nothing sinister being suspected.  It’s fucking outrageous.

Today I discovered another lump.  I have no idea how I haven’t noticed it before because once you know it’s there it stands out a mile!  It’s inside my elbow (on the right as you look at the picture) and on the same arm as the armpit lumps:

Click on the picture to see a larger image

There are lymph nodes exactly where the swelling inside my elbow is, so there’s clearly some kind of lymph issue going on.  In addition, my armpit is now hurting when it hasn’t before, and my whole arm is feeling heavy and keeps going dead on me.

Until now I’ve just brushed the whole armpit lump thing off, but I have to admit having found this new lump and feeling sooooooo exhausted since Christmas (and actually, well before) I’m more worried now and just want the bloody scan so that I know what’s happening one way or the other.

It could be something

Neither of my paternal grandparents ever had cancer.  Neither of my maternal grandparents ever had cancer.  My biological Dad had stomach cancer, but not until he reached the ripe old age of 82 and his 85 year old sister is still alive and cancer free.  Two of my Mum’s siblings died of cancer, but they were heavy smokers and it was lung cancer, so it wasn’t exactly a shock.  My Mum smoked like a chimney for nearly 60 years but despite having a tumour on her lung it was benign.  Two of her siblings are still alive, both in their seventies, and have never had cancer.  So, to be honest, even though 1 in 2 of us will get cancer I’ve never really given it a second’s thought because it doesn’t look like it runs in my family and I have none of the lifestyle factors which increase my risk, such as drinking or smoking.

I thought a little bit more about the disease when my maternal cousin got breast cancer aged 60, though she isn’t classed as a close relative and I’ve tested negative for the BRCA gene and when I discovered that MCAS significantly increases the prevalence of certain cancers, including breast cancer, and having endometriosis is also associated with a risk of certain cancers, such as ovary, uterine or cervical.  But you never think it will actually happen to you, do you?

The reason I’m prattling on about the Big C is that I had my appointment with the GP today about my armpit lump.  I genuinely thought she’d take one look and say “everyone’s armpits are different and it’s nothing to worry about” but she didn’t.  She said there is most definitely a lump and it is not a cyst, nor swollen lymph nodes due to infection.  In addition, there is also swelling around a second lymph node and some puckering of the skin.  She said she couldn’t tell if the lump was an enlarged lymph node or a thickening of the tissue, but that neither should be occurring and it is possible it may be cancer related.  I waited for the “or it could be nothing” but it never came, and when she looked at me in sympathy and gently rubbed my back as she guided me out the door my brain screamed very loudly FUCK! and double FUCK!

There are two common types of cancer which could originate in this way: breast cancer, although I have no obvious signs of breast cancer, and lymphoma which I might possibly have signs of.  A persistent cough can be a symptom of lymphoma and, as you all know, I’ve been coughing for nearly 3 months.  Tummy pain and feeling full are also symptoms and I have been having both in spades, plus fatigue is common.

Soooo, I’ve been referred for an ultrasound and will get an appointment through within the next two weeks.  I’m not a big worrier and I’m sure it will turn out to be absolutely nothing, and even if it turns out to be absolutely something worrying won’t cure it, nor will stressing out or getting worked up.  I’ll just forget about it until I know for sure what’s happening.