Uncategorized

Infections and Ehlers-Danlos Syndrome

People with EDS seem to get more infections than people without EDS, and these infections often have similarities. I’m currently in hospital with yet another skin/wound infection, and want to share some of the things that my team and I have learned over the years, about the type of infections, how to diagnose them, treat them, and how to prevent them from happening in the first place. 

Within the last decade or so, there has been significant progress in understanding the truly multi-system nature of the syndrome. In London, Professor Rodney Grahame, who has dedicated the majority of his career to treating people with EDS, has brought together a number of different specialists to try to understand some of the symptoms commonly experienced by his EDS patients. The group started small, but has grown rapidly with the realisation that virtually every organ or body system can be affected by EDS.

I have been lucky enough to have been treated by several of the new specialists with an interest in EDS. Not only have they helped to diagnose some of the conditions that I have as a result of EDS, they’ve also, because of their contact with EDS specialists in other fields, been able to understand some of my ‘random’ symptoms, and have referred me to some of the other EDS specialists for investigation/treatment. These super-specialists tend to work in large tertiary referral centres, and despite the very niche areas of interest (e.g. Urogynaecology specialising in bladder dysfunction in women with EDS), their clinics are bulging at the seams with patients.

One of the newer observations is that many people with EDS get lots of infections, most commonly ear/sinus/chest, bladder/kidney, and skin/wound infections. I am no exception.

Wounds tend to take a long time to heal, and scar badly. These wounds often get infected, delaying healing even further. I have a feeding tube and a Hickman line, both of which go through the skin. I’ve ‘lost’ two feeding tubes and one Hickman line due to skin infections that just wouldn’t go away, despite heavy-duty treatments, 

People with EDS often have a lowered immune system. Part of this is probably related to being generally unwell for a long time, but there also seems to be a specific deficiency in IgG-3, which is one of the antibodies that fights infection. This can result in a vulnerability to infection, especially those mentioned above, and especially caused by viruses and certain (gram negative) bacteria. The gastrointestinal problems often associated with EDS can mean that oral antibiotics aren’t absorbed very well, making it even more difficult to treat these frequent infections.

The basic problem in EDS is stretchy collagen (connective tissue), but it’s not just stretchy – it’s also fragile. This means that skin can split open or tear easily. Having a heavy feeding tube pulling against the skin can cause the skin to tear, giving infection a way in. This is especially problematic when the other end of the tube is in the gastrointestinal tract, which is full of bacteria.

One of the things we’ve tried that has made a difference is using smaller tubes. My current feeding tube is just over half the size of a standard tube, and has been so much less troublesome than my previous two tubes. 

The other thing that has made a difference is using dressings/tape that don’t tear my skin (or cause an allergic reaction). It can be a long process of trial and error to find the right dressing/tape, but it’s so worth it. Having to stick another harsh dressing over already broken skin is not nice, and broken skin is beautifully ready for infection, especially if it’s kept warm and moist under a dressing!

Finally, arranging my tube and taping it carefully to my skin takes some of the weight off the wound, allowing it to heal, and protecting it from infections.

I’ll be trying all those things, and more (suggestions are always gratefully received, and I’d love to hear of your experiences). I hope to get my new Hickman line this week, after having to have it removed last week, after 5 months of grumbling infection. I hope to keep this new line for at least five years. Third line lucky, right?

Standard
Uncategorized

When Experience Counts for Nothing

Many of us with long-term health conditions, whether common or rare, come to know an awful lot about our conditions and our bodies.

In many ways it’s a little like the experience of a car that my grandparents used to drive. She was known as Little Yellow, named for her external colour; her replacement, many years later, was known as Little Vomit, also a reflection of the external colour, or at least my grandmother’s opinion of it. Anyway, Little Yellow was a teeny little thing with slung canvas seats, perennially filled with grandchildren and sand. One thing that Little Yellow didn’t always like was starting. It took a great deal of experience with the 2-stage choke (perhaps I’m showing my age, or at least Yellow’s age) and knowledge of just how much coaxing on the accelerator pedal was enough and how much would just flood the tiny engine, at which point it was best to go for another swim, or to get another ice cream, rather than continue to try to start the car.

My body, similarly, does not always like to start. I used to describe myself as a ‘morning person’ – now something that gives rise to a slightly cynical chuckle. My blood pressure tends to be very low in the mornings, making me feel quite wobbly. My joints are also sore in the mornings (oh, hellsticks, what am I talking about? My joints hurt all the time). My joints hurt in the mornings because they slide in and out of socket, or can ‘hang’ out of socket for extended periods while I am asleep and all my muscles are too relaxed to hold my joints where they should be held (in the absence of competent ligaments, which do this for you, ‘normal’ people). Trying to get up too quickly is basically the same as saying, “I feel really optimistic today, so I need to collapse onto the floor to reconsider my attitude.” It’s the Ehlers-Danlos equivalent of time-out on the naughty step.

At the best of times, I take a lot of medication. I try not to count how many I take at the worst of times. Currently, I take somewhere in the region of 25 different medications on a regular basis. In the mornings, with low blood pressure that clouds my thoughts and keeps me lying flat for fear of enforced lying down (fainting), and joints that are weak and painful, it can take two hours just to unhook IV fluids and tube feed, hook up to daytime IV fluids (same fluids, just in a separate bag; no idea why), prepare and administer various IV, injected (with a needle), stuck-on, rubbed in, liquid (via j tube), dissolved in the mouth, or inhaled medications.

If I need to have a shower, it can double the length of the getting up process. Low blood pressure plus hot water is not a good combo (hot water makes the veins dilate, so they allow more blood away from the brain and heart), and I no longer feel safe showering when I’m alone in the house. It’s one thing to be scooped off my own kitchen floor at 3 am by paramedics because I got dizzy, fell over and dislocated my shoulder and wrist; it’s quite another thing to have that same scenario repeated when I’m half-clean (or half not, depending on your particular way of seeing the world), covered in soap, wet and naked.

For reasons that I hope are now obvious, I try not to schedule morning appointments. I’m due to have an operation in a few weeks, and I have to be ready to leave via patient transport (up, showered/dressed, and appropriately medicated) by 5 am. That means I need to get up at 2 am, optimistically. If I didn’t go to bed so early, I’d say it was hardly worth it!

I didn’t mean to write any of this extra detail (sorry). My original point, in case you’ve forgotten, was that people get to know their own particular idiosyncrasies, and learn to modify their environment accordingly. Needing several espressos before anyone can talk to you (or you’re capable of responding) is a common ‘normal’ world example, with coffee-related paraphernalia in the house (or choosing a flat above St*rb*cks) a related environmental adaptation.

Hospitals are often designed to meet the needs of as many people as possible: staff, patients and visitors, and sometimes end up suiting nobody perfectly. In this system, it can be really hard as a patient (often viewed as the person who knows least about their condition) to be assertive about their individual needs.

Many of the nurses on night shifts like to try to get tasks done in order to lighten the load for the day staff who replace them. The intention is admirable, the reality sometimes less so. In reality, ‘getting things done’ can involve putting all the lights on at 5 am and waking patients in order to check their blood pressure and stick pins in them to see if their blood sugar is ok. Quite apart from the fact that many people have low blood pressure at that time of the morning (resulting in lots of scared nurses when they check my blood pressure at that time), it’s not very helpful for people recovering from illness to have their precious sleep interrupted. Additionally, shaking me awake (I sleep with headphones in or my non-deaf ear in the pillow when in hospital) can dislocate or otherwise injure my already-sore joints.

I’d love to see a health system in which patient experience – human experience – is truly valued. I would love patients to be able to choose what time they want to sleep, eat and drink. I would love options for those with dietary restrictions to be more widely available. I would love it if there were some way of making it easier for patients and their friends and families to make the hospital environment more amenable to their own needs, whatever those happen to be.

I’m delighted that so many hospitals allow me to display (LOTS of) photographs and cards, delighted that they sometimes allow me to use my own pillowcase, and bring my own herbal teas and proper-ish coffee. I’m thrilled to report that in the 3 decades that I’ve been an NHS ‘frequent flier’, many hospitals have made great strides in improving patient experience. I remember having an operation when I was quite young (not my first surgery, but I was still under 3 years old) and remember that my parents were not allowed to stay with me outside visiting hours (which used to be pretty much just that – an hour or two once or twice a day), and I remember being intimidated by the long ward and high, narrow ‘big girl’ bed. Things have come a very, very long way since then!

Now, I’m often allowed to administer my own medication, sometimes even intravenous medication, without a huge fight (that seemed like an impossibility back in the ’90s). Not only that, but many of the nurses react with pride that I’ve been taught to look after my own care so well, and encourage me; sometimes they even ask me to talk them through the techniques that I use (I use a sterile technique of Both Hands Clean, as opposed to the more commonly used Clean Hand/Dirty Hand – more on that in another post) or ask to watch me prepare and administer my medications, to learn, rather than supervise.

Every time something like this happens, I’m so grateful to those nurses (and sometimes doctors and pharmacists) who are willing to look beyond the hospital stereotypes and see the things that I do well, or the things that I know about myself, or even the things that make me who I am.

You may have read about Dr Kate Granger’s remarkable positive campaign in the NHS, Hellomynameis – it’s a simple way to remind busy medical staff that they usually know a lot more about the patient than the patient knows about them. By offering their name at the start of any interaction, staff can make patients feel more confident, more human and less alone. This campaign was launched in 90 hospitals and NHS trusts across the UK yesterday, and I’m so proud of Dr Kate and all those who have supported and encouraged her.

I spent yesterday in my local A&E department (another other post), and almost everyone greeted me with a smile and offered me their name, from domestic staff to consultants, technicians and porters, junior doctors, nurses and admin staff. It makes such a difference. One doctor even asked how I’d met my husband (I try to throw out little reminders that I’m not just a rare disease in a bed, so she was responding to that). It pretty much made my day, along with the fact that she let me look at my own blood test results and talked to me like an educated human. She gave me the tiniest of ‘looks’ when her more senior colleague (who has met me numerous times and should have known better) tried to explain to me what blood pressure is. Having studied both medicine and physics, I know a bit about blood, pressure and even both together. I could have hugged this doctor, who didn’t know about the education, but had picked up that her colleague had pitched his ‘little talk’ at the wrong level. I don’t know if it’s that my expectations are too low from a whole life spent in close contact with the NHS, or if those tiny little changes really can change our perceptions so drastically.

Maybe this calls for a wider social experiment: smiling and being kind and aware of our fellow humans in order to change the world. I don’t know. Maybe I’ll try it. For now, though, I’ll continue to be a cheerleader of #Hellomynameis (the Twitter stream from this hashtag is inspiring and heartwarming, if you’re interested, as is @grangerkate herself), and continue to try to pluck up the courage to advocate for myself when necessary.

More soon, I promise!

Things have come a long way since I was this age (this is about the age I was at the surgery mentioned above)

2015/02/img_3633.jpg

Standard
Gastroparesis

What Not to Eat

It’s 4 am, and I am sitting on the floor in the bathroom, alternately cradling the basin and a hot water bottle. I have been sitting here for a little over three hours, and I need a distraction from the misery of throwing up, as an attempt to stave off the impending Pity Party, so hello WordPress!

Why am I vomiting?

The complicated answer (and a quick recap of previous blog posts) is that I have gastroparesis and pan-enteric dysmotility, secondary to Ehlers-Danlos Syndrome. Which means, as you may recall, that my entire digestive tract is too stretchy, and hence inefficient.

The simple answer is that I ate too much on Thursday, and food has to leave the body somehow. The usual route is closed due to strike action today, so my stomach has decided to try things in reverse. It always amazes me that my stomach is all weak and pathetic in the right direction, but seems to work beautifully in reverse.

So far today, the retching and vomiting has been forceful enough to dislocate my jaw, my shoulder (twice) and at least one rib. Hence the Pity Party.

I thought I’d put my experience to good use, and recommend a few foods that are really not very nice on the way back up, as well as a few that are not so bad.

People with gastroparesis are usually advised to eat small meals every couple of hours, in liquid form if solid food is hard to tolerate. The meals should be low fat and low fibre. That makes for quite a limited, and not terribly healthy, diet (more details here if you’re a glutton for punishment interested).

When I was diagnosed with gastroparesis, I took all these diet recommendations to heart. I memorised the tables of foods to avoid, trawled the internet for recipes and meal ideas, and even drank supplement drinks with minimal fuss, and yet I was still sick. For months and months I forced myself to eat and drink all the ‘right’ things, knowing that doing so would cause pain, bloating, nausea and vomiting. Like Pavlov’s dog, I became conditioned to gag and retch at the very thought of Fortisip. I felt so guilty for not wanting to ‘comply’ with my doctor’s instructions, and so miserable that I couldn’t eat the foods that I craved, or take pleasure in the social gatherings that revolved around food.

In 2012, I had surgery to place a feeding tube, which completely bypasses my unhelpful stomach. I get most of my nutrition and some medications through the tube, so anything I eat ‘normally’ is purely for pleasure. Although eating is still associated with unpleasant symptoms, it’s no longer linked to feelings of guilt, judgement and shame, and I am gradually starting to mend my relationship with food.

The decision about whether something is worth eating takes some trial and error, but here are a few suggestions of foods to avoid, and a few that aren’t as bad as you might imagine. Don’t forget that whatever you choose to eat today, which may be ok when tasted in reverse, will mix with the food still in your stomach from earlier (maybe even yesterday or the day before). Strawberry jelly might be fine in isolation, but is a different flavour entirely when mixed with yesterday’s tuna mayo. A different kettle of fish, so to speak.

Not so bad:
– coffee
– bread
– vanilla ice cream
– mashed potato
– pancetta/lardons

Not recommended:
– cottage cheese
– beansprouts
– coleslaw
– sauerkraut
– orange juice
– red, red wine

Would you add anything to either of those lists?

Standard
Update

Allergic

I had an allergic reaction to something this afternoon.

My tongue and throat started to feel a bit scratchy, and then my eyes started to get red and itchy, and to swell up, with the muscles around my eyes spasming them closed.

I took some antihistamines.

The scratchy feeling in my throat became clusters of blisters over the roof of my mouth, over my tongue and down my throat.

I took a dose of another antihistamine that blocks a different type of histamine receptor.

I found my ‘allergy bag’ which has medications, including injectable adrenaline (everyone wanted to be my friend on sports day). I started to make my way downstairs, in case I had to do the dash to hospital in an ambulance, keeping a tight grip on the allergy bag, epi-pen and mobile phone.

My throat, mouth, tongue and lips continued to swell and my breathing started to feel more difficult. Inhalers and nebulisers, monitoring my heart rate and oxygen % saturations.

It’s been a long time since my asthma has been severe enough for a hospital admission. More than 2 1/2 years, in fact. Part of me started to panic at the memories of terrified clock-watching in the intensive care unit, dragging oxygen into my overfilled lungs; forcing the air out; muscles shaking and burning with the effort of every breath. Worse, the memories of being surrounded by doctors, nurses, anaesthetists. They gather round, bend over my bed, close in on me, then the medications, ventilator and blessed rest.

IMG_0132.JPG

In 2011, I spent five months in hospital, in total. I got out of hospital almost exactly 48 hours before my wedding, and was back in hospital just a few days after the honeymoon.

I try not to think of the stable asthma that has taken so much careful monitoring and treatment. I try not to think of the events that I missed because of unstable life-threatening asthma; the plans that we didn’t/couldn’t make for the future; the 30th birthday ‘celebrations’ in intensive care; having to sign release forms to leave the hospital against medical advice for my dad’s funeral.

Today’s afternoon turned into evening, and things steadfastly refused to settle. I took the maximum ‘rescue’ dose of steroids, another dose of both types of antihistamine, plus a third antihistamine, plus inhalers. Inhalers and nebulisers again and again and again.

Have you been back to the beginning of this blog post to see if you missed a crucial bit of the story? Are you wondering why this story doesn’t feature an allergen?

Yes, that worried me too.

The only obviously new thing, on a day with low pollen and good air quality, is the laundry, hanging in the next room to dry. The smell of the new fabric softener immediately caught in my throat, but there’s a world of difference between an unpleasantly overpowering fragrance and something that causes my mouth, tongue, throat and lips to swell and close, and my airways to tighten, isn’t there?

I wrote about unstable mast cells recently, and I think they might be able to take credit for this seemingly-unprovoked reaction.

My husband moved the laundry downstairs when he got home, and things have been stable-ish since then. I’m no longer clutching my phone, 999 ready on the screen, epi-pen hovering.

IMG_2891.JPG

Feeling a bit better now. My heart rate is the top number (normal = 60-100 beats per minute), oxygen % saturation is the lower number (normal > 94%). Not good numbers, but not getting worse. My throat and tongue are less swollen, and the crazy itching has subsided.

I’m still scared to close my eyes; to let sleep take me. I’ll have one last nebuliser treatment, and set the oxygen monitor to beep if my saturations (sats) drop below 88% and hope that things will be easier when I wake.

Deep breath…

Standard
Uncategorized

The Internal Filter

The internet is full of articles, lists and rants about things that one shouldn’t say to a person with a life-limiting illness or disability. I think that many of the things on those lists are a matter of personal experience. I quite enjoy, for example, being told that I look well, but a person who doesn’t yet have a diagnosis might hear that phrase, however kindly meant, as a way of questioning, or invalidating, their suffering.

Most people have an internal filter of some kind. It is used to edit the available thoughts into those that are right for this place; this time; this audience. With varying degrees of awareness, we select or reject the stories, anecdotes, debates, queries and words that run through our minds, allowing some to be spoken, some to be put aside for another time, and some to be censored entirely.

I want to be able to have a conversation with you. I want you to feel able to ask questions, to offer help, to talk about the state of my crumbling and decrepit body, or maybe just about the weather, current affairs, or the latest book you’ve read. I don’t want these conversations to be lexical assault courses, leaving you exhausted from hurdling handicapped, sprinting past spastic, ducking, diving and dodging diagnosis, deterioration, death, demise.

There are so many words, from the dictionary or slang; words borrowed from other languages. There are so many cultural variations, euphemisms and idioms, and so many different people, with different experiences, background, education, and understanding of language and disability. I hate the thought that someone might not talk to me because they’re scared by this minefield of words, and don’t want to cause offence or hurt my feelings.

I hate the idea that I might be ignored and isolated because some internal filter blocks all avenues of conversation, and as a general rule, I would rather people talked to me than not.

There are exceptions to this, of course, and many of you will have heard this story, but I’ll tell it again, for posterity.

It happened one evening, as I was on my way home from work, navigating the underground sea of commuters. I was wearing a suit, stilettos, a wheelchair, and the blank stare of a long day. I paused, considering whether to go out for a drink with friends, or to heed the siren-call of my bed. A man in a suit walked towards me, and I smiled, as most Londoners do when approached by complete strangers.

“I hope you don’t mind,” he began, “but I wanted to let you know that I was having an awfully bad day today, and then I saw you, and realised that in comparison to yours, my life isn’t so bad, after all, so thank you.”

Years later, I still haven’t thought of a response, except the silent wish that his internal filter had killed and buried that thought before he could share it with me.

IMG_0028.JPG

IMG_0029.JPG

Standard
Uncategorized

The effects of EDS on the entire body

In my first post I wrote a little bit about Ehlers-Danlos syndrome (EDS).

I’d like to give you a quick overview (hahaha – quick!) of the different ways in which my body is affected, so here’s a little reminder of the basics before I start:

Collagen is a protein that helps to provide strength and stability to many of the tissues within the body. By ’tissues’ I mean a group or layer of cells that perform specific functionsBy this definition, bone is a type of tissue, as is skin, and the coating around nerves and blood vessels. Organs may be composed of several different types of tissue.

EDS collagen is not strong or rigid – it is weak and stretchy.

Because collagen is found throughout the body, the signs of EDS can be found in just about every organ and body system. I’ll write more about the medical specialists and their developing understanding of the complexities of EDS in another post. Here, I just want to tell you about how this condition affects me.

Joints

My joints are unstable, and dislocate very easily because the ligaments that should hold them in place are stretchy and weak. There is a capsule around each joint, which provides a ‘safety net’ and can hold the joint in place to some extent if the ligaments are weak. My joint capsules are stretchy and don’t provide any sort of safety net. Some of my joints dislocate or sublux (partially dislocate) numerous times a day; others less frequently. Every time a joint dislocates, the tissues around it stretch or tear, and there may be damage to the other structures nearby – blood vessels, nerves, etc. It is possible to surgically ‘fix’ some of this damage, and to stabilise some joints so that they don’t dislocate (or at least dislocate less often). Unfortunately, surgery that involves the soft tissues is unlikely to last, and surgery that involves the bones is complicated, risky and very painful, and may involve lengthy rehabilitation.

As an example, I have had numerous operations on both wrists. I have had operations to tighten the capsule around the joint to hold the joint in place. I have had operations to shorten the tendons so that they are tight and hold the joint in place and prevent dislocations. Most of these operations were performed before I was diagnosed with EDS. With the benefit of hindsight, these operations were always going to fail. The joint capsule stretched again within a matter of weeks, and my wrist started to dislocate again. The surgically-shortened tendons stretched back to their full length (and more) within days, and my wrist started to dislocate again. I can’t tell you how disappointed I was to have gone through so many surgeries, each with long periods in a plaster cast (or similar), only to be back in the same position in less time than I’d spent in plaster. The only way to stop my joints dislocating is to fuse the bones together, locking them securely in one position with metalwork and bone grafts. This is fine for joints that had relatively limited range of motion to start with (I’ve had both my thumbs fused with great success), but to have a joint like a knee fused in one position (bent, straight, or somewhere in between) would make life rather difficult.

I have certain bony deformities because of the stretchy connective tissue between the bones, and/or structural damage as a result of dislocations. One of these is a cervical kyphosis (my neck curves outwards instead of inwards). It is painful, and puts extra stress on some of my muscles, tendons and ligaments because of the abnormal position of the bones. I have glenohumeral dysplasia (my shoulder socket is too shallow), hip dysplasia (my hip sockets are too shallow) and patellofemoral dysplasia: there is no groove for my kneecap, which should be formed by lumps on my femur and tibia, and a pointy bit on my kneecaps to sit in the groove – I don’t have any of those things – a flat kneecap and flat femur and tibia.

Pain

Most of the pain is as a result of joint dislocations, but it is currently believed that people with EDS have abnormally sensitive nerves, causing far more pain than would be felt by ‘normal’ people. Rather frustratingly, people with EDS are also resistant, or relatively resistant, to local anaesthetics, sedatives and painkillers. When a relative of mine had terminal cancer, he was given a patch to stick on his skin, which would deliver a constant dose of a strong painkiller. This helped a lot with his pain, and made him quite drowsy. I remember being quite shocked that I have no noticeable sedation and inadequate pain relief from a patch EIGHT times as strong.

Chronic pain is frustrating because if the pain is ignored, the body responds by amplifying the signals from the pain nerves. This is fair enough if the pain is a warning about severe damage, and if it is a signal to me to take my hand out of the fire, for example, but when there is nothing I can do about the damage to the joint, it would be nice if the pain would give up and go away!

Despite being extremely resistant to some medications for pain, I am strangely sensitive to others, and have had to increase the dose of an atypical painkiller (originally developed from a medication for epilepsy, but found to have a good effect on chronic pain) very slowly. I have been taking this medication for over three years now, and I am still gradually increasing the dose – I have a long way to go before I get to the maximum dose. It took me over 6 months just to get to a therapeutic dose (one that would be expected to do anything at all). I am adjusting to the side effects now. They are annoying for a week of two and then manageable. I’m really proud of myself for not giving up on this medication, especially now that I’ve reached a dose that helps to control my pain.

Bladder

I’m going to get this section out of the way quickly because I find it extremely embarrassing and upsetting, and find it difficult to tell people about something that should be so private.

My bladder is stretchy. Most people would be crossing their legs and hopping from one leg to the other if their bladder contained 500ml of urine. Mine will quite routinely hold 1100ml. I had some tests done at the end of last year, which showed that my bladder muscle doesn’t really work at all, though it will sometimes spasm, which causes leakage of urine. I’ve been self-catheterising for over a decade (I insert a very small catheter into my bladder to drain it into the toilet, then remove the catheter and throw it away), but because my bladder gets so full before I have any sensation from it, I have to catheterise on a timed schedule. I’m on a waiting list for a surgical procedure to inject botox into my bladder, which will totally paralyse the muscle and prevent the spasms that I get, which should stop the intermittent incontinence.

If the bladder doesn’t empty properly, bacteria in the urine that is left behind in the bladder can grow out of control, causing symptoms of infection. This infection can be just in the bladder (which is pretty unpleasant) or it can spread to the kidneys, where it can really wreak havoc. When my bladder spasms, the urine tends to come out by the usual route, but it can also be pushed back into the kidney, which can cause all sorts of damage, especially if there is an infection at the same time. Infections in any part of the urinary tract (kidneys, bladder, the tubes between the kidneys and the bladder – ureters – or the tube from the bladder to the outside world – urethra) are known by the collective term, Urinary Tract Infection, or UTI.

Digestive System

When you eat food, you chew it and then swallow it – the food goes down the oesophagus and into your stomach. It may sit in your stomach for a while, being churned and mixed with acids and enzymes, before being pushed, a little at a time, into your small intestine (duodenum, then jejunum, then ileum, then cecum), where it spends time being absorbed, stripped of anything useful, and on into the large intestine, where more water is removed, and finally, once your body has finished with it, it is ejected, and you think no more of it.

All of this travelling, from mouth to anus, is powered by muscular contractions within the walls of the digestive tract. These timed contractions are called peristalsis and they propel the food through each stage of its journey.

My digestive tract is stretchy. Are you bored of the word ‘stretchy’ yet? Good thing you’re not playing drinking games while reading this…

I had a gastric emptying study a couple of years ago, which involved me eating some radioactive mashed potato, and then sitting under a scanner, walking around for a bit, then back to the scanner, etc. For four hours. After four hours, most people will have less than 10% of their meal left in the stomach, unless it was an unusually large or fatty meal. I had almost 100% of the mashed potato left in my stomach after four hours. I even had radioactive mashed potato left in my oesophagus after four hours. It hadn’t even made it to my stomach! I had an appointment recently to test the function of my small intestine. It failed because they couldn’t get the probe (which was pushed down my nose and into my stomach) out of my stomach and into my small intestines.

My stretchy stomach doesn’t churn food very well. It also doesn’t allow food to move through into my intestines. I had botox injected into the exit valve (pylorus) of my stomach, in the hope that it would allow food to just trickle through. This didn’t work terribly well, so they did it again a week later. When it still didn’t work, they put a feeding tube from my nose into my small intestine, totally bypassing my stomach. I vomited this tube up three times, and each time it was replaced. Eventually, I had a tube placed through the skin on my abdomen into my jejunum – a jejunostomy tube, through which I have received most of my medications and nutrition ever since.

My problems don’t end with the stomach – there was a lot of emphasis about trying to bypass my stomach, or even place a type of pacemaker into my stomach to make it empty into the small intestine. After a while, we all realised that it wasn’t just my stomach, but my entire digestive tract. It can take up to 14 days for food to make its way through my entire digestive system. You know the ‘experiment’ that you do when you eat sweetcorn? That.

The current debate is whether I should be fed into a vein near my heart (via the Hickman line that I already have), which is very risky because the feeding solution is so sugary, and bacteria loooove sugar. It’s also damaging to the liver in the long term, and can have other serious effects on the body. On the plus-side, I have felt brilliant when I’ve had it in hospital, for a couple of weeks at a time. I have more energy, my skin and hair look good, and my joints are slightly more stable. I saw my consultant on Friday, and her feeling is that she wants to wait until I am seriously underweight, as the risks of IV feeding are so great.

Immune system

EDS has a couple of different effects, which are seemingly opposite. There is a deficiency of one particular type of antibody, which means that the immune system is underreactive and can’t fight off certain types of bacteria. Almost all of the many infections I’ve had in the last few years have been caused by gram-negative bacteria, of various types. On the other hand, my immune system is overreactive, because I have a mast cell activation disorder.

Mast cells are like little grenades, circulating around the body. They are filled with histamine and other inflammatory chemicals. When the body encounters an allergen, it triggers a particular antibody to be released, which can ‘pull the pin’ on the grenade-like mast cells, releasing the histamine and inflammatory chemicals. The histamine causes swelling in the airways, tongue and throat (and some other places too), hives all over the body, and a drop in blood pressure. This is anaphylaxis, which is a severe type of allergic reaction.

Mast cell activation disorders occur when the mast cells are unstable. Using the grenade analogy again, they have a loose pin. Instead of waiting for the antibodies to tell them that an allergen is present, they decide all by themselves, and release the chemicals that lead to anaphylactic shock. This reaction is usually in response to particular foods or medications that are known to have an effect on mast cells that are a bit unstable. There’s a helpful list on the Allergy UK website that lists foods that are known to destabilise mast cells. I am sensitive to almost half of the foods on that list. Some people with EDS also have a deficiency in diamine oxidase, which is responsible for getting rid of the histamine that is present in some foods, and for clearing up the histamine that is released by mast cells after an allergic reaction. This makes them very sensitive to foods that contain histamine, which are also listed on the Allergy UK website on the above link.

Endocrine system

The endocrine system is responsible for the hormones in the body. Endocrinologists may look after people with conditions as diverse as diabetes and testosterone deficiency.

People with EDS sometimes have problems with a chemical in their digestive system that causes reactive hypoglycaemia. I had a test for this condition, which involved drinking a sugar solution then having my blood tested every 15 minutes for four hours. Yes, they took a vial of my blood every 15 minutes for four hours. And then I didn’t even have the condition they were looking for. In fact, as a result of that test, they diagnosed me with diabetes. Ugh! I had the test done in January and saw the doctor in clinic to receive the results in July. Yes, it took six months, and a LOT of telephone calls and chasing from me to get the results of the test. I was then referred to the diabetes clinic at King’s as an urgent case. As a result, I’ll be seeing them in October to talk about possibly starting insulin treatment.

The heart

Some people with EDS have a problem with one of the valves in their hearts (mitral valve prolapse). Thankfully, I don’t have that problem. I do, however, have problems with my autonomic nervous system. This is either treated by cardiologists or neurologists or, in my case, both. The autonomic nervous system is responsible for keeping a constant internal environment within the body, even when there are changes in the external environment. One example of this might be controlling body temperature. If you walk out of the house on a hot day, you might start to sweat, which is your body working to reduce the effect of the high outside temperature and keep the temperature within your body the same.

In lots of ways, the autonomic nervous syndrome is like the Chuckle brothers. It has two parts – one of which causes the ‘fight or flight’ response, while the other promotes ‘rest and digest’ functions. They can both work at the same time, and often work a bit at a time as they adjust things like heart rate, blood pressure, body temperature, etc. on a moment by moment basis – to me, to you, to me, to you…

I have severe autonomic dysfunction. The part of the autonomic nervous system that promotes ‘rest and digest’ functions and turns off the ‘fight or flight’ response (the parasympathetic nervous system) doesn’t really work in my body. My heart rate is almost always high (more than 100 beats per minute) and gets even higher when I stand up or move around. I’ve stopped checking it most of the time because it’s scary to see my heart rate go over 180 bpm just by going downstairs. Recently, I’ve had a lot of problems with my oxygen levels being too low, which makes me feel a bit woozy. Combined with low blood pressure and fast heart rate, abnormal blood sugars and an inability to control my body temperature, that makes me feel pretty icky a lot of the time.

Another problem with my heart is that the electrical conduction is slightly skewed. My heart takes longer to get through one part of the electrical cycle than it should, which allows time for abnormal electrical signals to come in during the gap and cause an abnormal beat, which could cause my heart to beat erratically, or to stop altogether. It’s called long-QT syndrome, and can sometimes cause sudden death in young people. I have to avoid lots of medications as a result of this condition.

In addition to all these things, I have a heart murmur. It’s not a structural problem, but is a sign that my heart is under stress from all the different things that are wrong with it, and with the other parts of my body, which have an impact on my heart and circulation. It’s important for me to have infections treated, and to avoid situations (like standing up) that cause my heart rate to increase beyond a certain level.

Hearing

It seems almost trivial after everything else, but I’m totally deaf in one ear. This could be related to EDS and/or long-QT syndrome, or it could be totally unrelated. I have a bone-anchored hearing aid (BAHA) on the left side, which picks up sound from my left side, translates it into vibrations, which travel through the bone of my skull and are picked up by the intact nerve in my right ear. I still can’t locate sounds, and don’t have any sense of ‘stereo sound’, but despite that, the BAHA has been one of the best medical interventions I’ve ever had (along with my thumb fusions and Hickman line placement).

Pending diagnoses:

Tethered cord syndrome: It’s relatively common for people with EDS to have a problem with their spinal cords, so that they are stretched abnormally, which can cause spinal nerve damage. I had a scan a couple of months ago to see if this is affecting my spine.

Mitochondrial myopathy: mitochondria are the power generators inside each cell of the body. If there is a problem with their function, organs/systems that require a lot of energy, e.g. heart, digestive system, immune system, tend not to work so well. One common problem is an inability to process lactate properly. When I’ve been admitted to hospital, it’s often been noted that my lactate levels are very high, which can cause my body to be too acidic, leading to some functions not working properly. I’ve recently realised, with my doctors, that I’m also very sensitive to Hartmann’s solution, which is a type of IV fluid, and which contains latate. It causes me a lot of muscle pain, which would make sense if I’m not able to process lactate properly and get rid of it from my body. Mitochondrial diseases are not yet well understood, but it is believed that they may be linked to EDS in some cases. There is no cure, but treatments involve avoiding infections and any other physical stressors, aggressive treatments of physical symptoms to prevent deterioration, and supplementation of the vitamins and minerals that may support mitochondrial function.

So…

There we are.

I was planning to add some pictures to break up all those words, but I don’t think I can bear to go back and reread it. I can write about all these things happening to my body relatively dispassionately, but there are times when I can’t dissociate myself from my body any longer, and each word is like another crack in my ability to deal with this condition that is breaking my body apart, one piece at a time. So I’m not even going to go back and spell-check. Sorry, but you’ll have to work out the typos on your own.

Standard
Uncategorized

Ten things I’ve learned about coping with the heat

This heat wave has been pretty miserable, but the consistently hot weather has given me the opportunity to experiment and see how my body reacts to the various things I’ve tried.

My body doesn’t do a very good job of adjusting to hot or cold weather. When it’s hot I often overheat, with a reaction that’s not unlike sunstroke. In cold weather, I often get hypothermia. This inability to maintain a normal body temperature under different environmental conditions, or to respond appropriately to changes in the ambient temperature, is called poikilothermia. In my case, poikilothermia is a result of severe autonomic dysfunction, which is due to Ehlers-Danlos syndrome (EDS). Frogs and many other reptiles are poikilotherms – cold blooded. The only poikilotherm mammal (apart from me and a few other defective humans) is the naked mole rat. Yep. Naked mole rat.

Still with me after all the long words?

Great!

There is some general advice that is often given to people with EDS about how to cope with hot weather:

1. Stay out of the sun
2. Drink lots of water
3. Wear light layers, a hat, and LOTS of high-factor sunscreen when out in the sun
4. Eat lots of salt

 

This is helpful stuff, but it’s a bit too generic. When this heat wave started, I did all the things listed above, and my body continued to make a fuss, with tachycardia (very fast heart rate), hypotension (low blood pressure), and what I call ‘autonomic storms’ – waves of goosebumps, intense shivering/shaking, gasping for breath, with profuse sweating from my face, head and neck. During the autonomic storms, my heart rate and blood pressure swing wildly between much too low and much too high. My heart takes on a slight ‘skip’ too, and the mild heart murmur that I have becomes much more pronounced.

This would be unpleasant enough on its own, but remember that my muscles are weak, and my joints are very unstable. Intense shivering/shaking involves muscle contractions, leading to all-over body pain and muscle fatigue. Muscle contractions can be strong enough to dislocate joints. Painful. Miserable. Exhausting.

These autonomic storms can be triggered by all sorts of things if my body is in the right mood. Just a gentle stroke on the arm could trigger an hour of storms, leaving me crying big, fat, helpless tears of pain and exhaustion.

So…what helps?

1. Lying FLAT: not sitting, not just resting, but proper lying down. For some reason, people with autonomic dysfunction have real problems being upright. This is known as orthostatic intolerance. It’s taken me a long time to work it out, but lying flat helps a lot, and I’m able to bring some of the autonomic symptoms under control almost immediately by lying down and staying there.

2. Ice packs – I surround myself with ice packs when I’m up in my wheelchair, which allows me to wear clothes with long sleeves/trousers (in order to protect my very thin and very fair skin from the sun) without fainting/melting into a puddle of misery. I know a number of people with EDS who wouldn’t be without their cooling vests (which use phase change packs to maintain a cool, but not cold, effect). I don’t have a cooling vest yet, so I can’t comment, but after my success with ice packs, I’m looking forward to getting one for next summer. I usually carry an instant ice pack with me, originally in case of injuries, but it gives me great peace of mind to know that it’s there. Like a spare can of fuel in the car – the ice pack might be just enough to keep me conscious and calm – just enough to get me home.

3. Don’t get cold. Yes, I did just write that. No, it’s not a typo. Don’t get cold. My body seems to react very strongly to sudden changes in environmental temperature. I might be wearing light clothes, surrounded by ice packs, reclining in my wheelchair as far as is practical, and feeling comfortable. Take a quick trip down the refrigerated aisle in the Co-op, which is colder than the other aisles, and everything will suddenly be wrong: shivering, shaking, goose-bumps, and turning a dusky grey colour around the edges. This reaction would be understandable if I had ventured into the Arctic Circle, but in my local Co-op in south-east London in the middle of a heat wave, it is just really, really unhelpful. All my body’s attempts to get warm continue long after I’ve left the supermarket, causing me to get hotter and hotter, until my body realises that it is far, far too hot, and turns all the internal dials to the ‘get cold quick’ setting. Once my body gets going, it is possible to alternate between the two extreme temperature reactions every few minutes for hours, or even days, which, I’m sure you can imagine, is not much fun. I usually carry a light scarf and a thin blanket so that I can cover myself up before my body realises there’s a cold wind blowing through the cheeses.

4. Have a place of refuge; a room, or even just part of a room, that is as close to your ideal temperature as possible. You should be able to lie down there, and sleep comfortably, even if it isn’t your bedroom. How you achieve this will depend on where you live, and what the weather is like, as well as the range of temperatures that you can tolerate. This summer I have kept the curtains closed in our north-facing bedroom, and the windows open. I have a choice of bed coverings, can have my feet and/or head raised and, during the height of the heatwave, I had a large bowl of cold water and a couple of flannels next to the bed, so that I could soak my feet, and lay the damp flannels across my neck, wrists and forehead. I have found that fans tend to make my body think it’s cold, triggering more of the miserable alternating storms, so some kind of gentle air conditioning unit (one that doesn’t blow cold draughts around the room) might make its way on to my wish list at some point.

5. Don’t let yourself get dehydrated: this is true for everyone, but for people with EDS, it’s even more important. Dehydration can reduce muscle strength, increase nausea and vomiting, and can worsen tachycardia (fast heart rate) and hypotension (low blood pressure). In other words, dehydration can make everything worse. In addition, dehydration can increase the risk of bladder/urinary tract infections (UTIs). Infections can make everything Even More Haywire, and lead to significant physical setbacks that can take a lot of time and effort to overcome. Why don’t you have a glass of water now, just to humour me?

It’s been suggested that people with EDS may need more fluids than those without, but because there are relatively few people with EDS, and because EDS affects people in different ways, it’s hard to give detailed guidelines. I had been aiming for 2 litres of fluid intake per day, but recently noticed, by keeping a fluid balance diary for a couple of weeks, that my symptoms reduced and my blood pressure and heart rate were more stable when I increased my fluid intake to 3 litres per day. Don’t assume that a ‘normal’ fluid intake is right for you – if you can bear it, keep a fluid diary for a week or two (I’m happy to share the blank record charts that I use, if you’re interested), and see if you can spot any patterns.

6. Eat salt. I’ve always loved salt, and eat a diet that is heavily salted. My husband thought for a while that I might have made up my diagnosis, as an excuse for all  the ‘unhealthy’ things that I like to do: eat lots of salt, sleep a lot, wear high heels…! Salt increases water retention. Not a good thing if your blood pressure is high, but extremely useful if you want to increase it. Why go to all the effort of drinking 3 litres of water per day if you can’t hang on to any of it?

7. Eat little and often – hot weather can worsen autonomic symptoms, which can include delayed stomach emptying (gastroparesis) and slowed intestinal transit. If this is a problem for you, try eating a low fat diet, and reducing the fibre in your diet. This goes against most healthy eating recommendations, so talk to your doctor/dietician about it first. If you can’t bear to eat at all when it’s so hot, try supplement drinks, soups, ice cream. Something is usually better than nothing, even if it’s not the healthiest of calorie sources. Consider taking a multivitamin/mineral (as before, check with your doctor/dietician/pharmacist). Even if you can only tolerate it every other day, it’s still better than nothing.

8. Don’t be afraid to ask for help. Contact your specialists (or their secretaries, nurse specialists, or PAs). They may not be able to offer any help, but you won’t know that unless you ask. They might be able to offer increased doses of medications (this might include Fludrocortisone, which many people with autonomic dysfunction take, increases salt and water retention; Midodrine increases blood pressure; Botox can be used to reduce areas of profuse sweating; Octreotide can help with many of the symptoms of autonomic dysfunction, for reasons that are not yet understood), or to bring your next appointment forward, or arrange for a telephone appointment. Ask them to check the list of medications that you take – a huge number of medications have some effect on the autonomic nervous system. This might be ok when you’re well, but when your body is under great stress from the heat, you might need to reduce the stress from other areas, such as medications. Even if your doctor/nurse specialist can’t do anything, they may be able to reassure you that you’re not alone.

Don’t forget about the expert patients out there. The internet is a wonderful resource for people with rare conditions, because distance doesn’t matter. There might only be 250 people in the UK who have nutrition via a drip at home, but some of them will be right at your online doorstep. If that isn’t enough, there are approximately 12,000 more of those people in the USA. Ask questions, listen to stories, learn what other people have found helpful or unhelpful, what their doctors recommend, and how their condition has been explained to them.

Many people with EDS seem to get more benefit from IV fluids than oral fluids. Your doctor might be able to arrange for you to have IV fluids at a local hospital, clinic or GP surgery, or for the district nurses to administer IV fluids for you at home. You might only need this once or twice, or you might arrange to have IV fluids on a regular basis. If this becomes a long-term thing, I would strongly recommend that you think about long-term IV access (I have a Hickman line, which I wrote about in this blog post).

9. Be honest with yourself about the symptoms that you’re experiencing. This is not weakness, you’re not being neurotic, and you can’t push through it with mental strength alone. We are so conditioned to believe that we can overcome anything if we just *want it enough* – this isn’t always the case, and beating yourself up for being ill will not help anyone, least of all you. Denial is one of my very favourite ‘coping strategies’ but no-one will be able to help you if you don’t acknowledge that there is a problem. Noting the symptoms, when they are worse and when they improve, can be a powerful thing to do, and might set you on the right track towards finding solutions and treatments. Being honest with yourself naturally extends to being honest with those close to you. They love you because you’re you, not because you are healthy and glowing, and will be happy to socialise in a low-key way, and to help you with things that seem impossible from a flat-on-the-bed viewpoint!

10. Be CAREFUL! If you faint a lot, try to learn the warning signs so that you can lie or sit down before you fall down. This can save you a lot of injuries, and a lot of mess/expense replacing things that you break, and cleaning curtains/carpets/furniture to get rid of the red wine that goes everywhere when you pass out. If you don’t have reliable warning signs, or you have trained yourself to ignore them, you may have to avoid dangerous situations (drink that red wine out of a plastic sippy cup with a tight-fitting lid) until you can learn to accept the warning signs. You can go up and down stairs on your bottom or on your hands and knees, carry hot drinks in a flask with a lid, don’t wear stilettos or flip-flops on the stairs. Don’t carry precious things: children, crystal glasses, iPad.

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

Standard