Another New Normal

I spend most of my time these days lying flat. Usually in bed, but sometimes on the sofa. I’ve been trying to keep track over the last few months, so as not to sound melodramatic by underestimating the amount of time I am able to be vaguely ‘functional’ each day. On average, I spend just over 80% of my time in bed.

There are two main reasons for this:

  1. Pain
  2. Autonomic dysfunction

I’ve talked about autonomic dysfunction before, and the effects that it has on my body, so I won’t go into depth about the basic stuff. As a very quick reminder, the autonomic nervous system (ANS) has a role in regulating pretty much all the ‘unconscious’ bodily functions, from heart rate, blood glucose and blood pressure to sweating, and bladder, bowel and sexual functions. I was diagnosed with severe autonomic dysfunction about 4 years ago, though I’ve been symptomatic for decades. It’s very difficult to diagnose autonomic dysfunction, not only because it’s rare, but also because so many different body systems are involved. Going to the GP with symptoms of heart racing and dizziness as a young woman is likely to earn a diagnosis of anxiety and ‘reassurance’ as the ideal treatment. Even if the symptoms are taken as an indication of a physical, rather than psychological, disorder, it can be a struggle to believe that the cardiac symptoms are related to the bladder dysfunction, and that both of those things are related to the dermatology symptoms of abnormal sweating, gastroenterology symptoms of intestinal dysfunction, and so on.

Anyway, I am ‘lucky’ enough to have a firm diagnosis, with “severe autonomic dysfunction” in black and white, on paper, and signed by a consultant neurologist. I carry this around with me, as there are more than enough doctors in A&E departments who don’t believe in things they can’t see, without written ‘proof’ from a specialist!

The most difficult thing for me at the moment is that being upright, whether sitting or standing, requires a lot of work from the autonomic nervous system, in order to maintain blood pressure and heart rate, mostly. My heart rate tends to run at about 110 beats per minute when I’m lying down. When I sit up, this can jump to 150 bpm, and can be even higher if I try to stand. This is uncomfortable, because I can feel the ‘flutter’ of my heart when it races like that. I also get very dizzy, and often black out, or faint completely, as my heart can’t pump efficiently at these very high rates, so my blood pressure drops. Combined with stretchy blood vessels (thanks, Ehlers-Danlos Syndrome) which allow my blood to pool by gravity in whichever part of my body is lowest, it becomes almost impossible to maintain my blood pressure when I’m upright. Everything else (autonomic function-wise) seems to be more difficult when I’m upright, perhaps because my ANS is so busy trying to deal with my blood pressure/heart rate that it can’t cope with anything else. So I really struggle to control my body temperature, with extreme sweating above shoulder level, wild swings in blood sugar levels, nausea, dizziness, etc. The thing that I find most unpleasant (apart from the sweating, which I find intensely embarrassing) is what I can only describe as ‘autonomic storms’ – intense shivering, combined with waves of goosebumps, followed by hot flushes and sweating, and back to shivering.

The shivering has an additional effect, of stressing my already-weak muscles, and pulling my joints out of socket. Yes, my joints are unstable enough that shivering can cause dislocations. Multiple dislocations.

This leads us nicely on to talk about pain.

I don’t like to talk about pain. I don’t like experiencing it, of course, but there’s something almost worse about sharing it. People can understand pain. Almost everyone has experienced pain of some degree, so it’s easier to imagine the pain than some of my other, less common symptoms.

Pain has become a constant companion. It seems normal to shift position because my hip is dislocated, or to check for dislocations before trying to roll over in bed. There are times when my muscles are too weak to hold my joints in place, even just lying in bed. When that happens, I can feel my joints sliding out of place, and all the pain associated with it, but I don’t have the strength to stop the dislocations, or even to move to be able to get the joints back in socket. At times like that, it takes everything I have to be able to reach the pain medications that I need, and even then, I find myself crying out, or even screaming with the pain. I don’t scream easily. I have stitches without local anaesthetic. I have had surgeries without anaesthetic, or even sedation. I have had IV lines tunneled under my skin and cut out without flinching or crying out. The pain of all my joints dislocating is indescribable; trying to breathe shallowly so as not to irritate the dislocated ribs; wanting to change position to get my joints back in place, but not being able to do that because my muscles are too weak; when I do manage to change position, I dislocate a different joint, or joints. This happens not just once, but over and over and over, accompanied by all the autonomic symptoms, nausea, vomiting, dizziness, constant infections. It’s too much to bear.

My life pretty much revolves around medications in general, and pain medications in particular. I can cope without some of my other medications, but pain meds, not so much. It’s much easier to try to keep pain under control than it is to try to get it under control once things have gone haywire. Additionally, pain seems to ‘stress’ everything else, especially the autonomic stuff, so being late with pain meds can lead to autonomic storms, which leads to muscle contractions and joint dislocations and more pain. I try to plan all my activities around pain medications. I’m so grateful to have medications that allow me enough relief from pain to be able to have a conversation with a friend, hold a friend’s baby, play with the children, or just to be able to have a shower and wash my hair.

Having so little ‘upright’ time (four hours a day, on a relatively good day) makes it really hard to socialise, so once again I’m finding myself incredibly isolated, lonely and frustrated. Those four hours are not just free for doing fun things. Having a shower takes about an hour, and requires at least an hour lying flat afterwards, leaving three ‘upright’ hours. Getting dressed and putting on make-up is another half hour, followed by more lying down, leaving two and a half ‘upright’ hours. Travelling requires extra pain and nausea medications, and reduces the time I can be upright – if it takes half an hour to get somewhere, and half an hour to get back, that’s another ‘upright’ hour gone, plus a reduction in ‘upright’ time of maybe another half an hour, or even an hour, leaving only half an hour of ‘upright’ time. All that effort, planning, getting up early so I have time to get ready and do lots of lying down, planning/preparing/administering extra medications to deal with the extra symptoms associated with being upright and getting ready to go out.

All that effort for half an hour with friends.

And then there’s the payback. Hours, or even days of having to lie flat in bed, needing extra medications, struggling with extra symptoms. Payback days mean that nothing gets done. All the chores get pushed back to another day, when I’m feeling well. For half an hour with friends, there’s all the preparation, there’s the payback, and then the days spent trying to catch up with all the chores that didn’t get done because I wasn’t well enough to do them.

At most, I am able to go out twice a week. That includes medical appointments.

There have been several times this week when I’ve wondered if anyone, other than my husband, would notice if I just disappeared. I feel as though my mark on this world is getting lighter and weaker, and my ability to leave any kind of legacy is getting less and less every day. I still have things that I want to give, things that I want to say, but I’m fighting against a body that just wants to stay still, and seems happy to fade into oblivion.

I’m not happy to fade into oblivion.

Not yet.

I’m trying to use my good hours/minutes to reach out to those that I love. Please understand that I want to be there for you, with you. I want to be involved in your life, to be supportive, to share the good times and the bad times with you. I long to break free of this body, but unfortunately, the only way to do that is to shuffle off this mortal coil, which doesn’t get the results I want. My mind is still active, and I think about all of you so much. I wish that I could show you how much I think about you, how much I care about you, and how much I wish I could be with you.

After months of hoping that this wouldn’t be my new normal, I’m now trying to come to terms with the fact that it is, and trying to find new ways to be in touch with you. I may need to start hosting pyjama parties!

Anyone keen?


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?


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.



















Today is an Exciting Day!

Today started, much like every other day, with no indication of the grey-plastic-covered excitement to come.

The usual medication, followed by medication, with a side-serving of medication, was followed by a rather disheartening phone call from one of the cardiac arrhythmia nurse specialists at King’s. I left her a message yesterday, hoping against hope that she might be able to fix my body and/or stop this overreaction to the hot weather. She couldn’t, of course, but did let me know that she was receiving dozens of desperate messages like mine. That shouldn’t have helped, but it did. I’m not alone in hating my dysfunctional physiology, hating the hot weather, and hating all the ‘normal’ people, basking in the sun.

Medication, medication, medication

Medication, medication, medication

It turns out that I’m doing all the right things. Which didn’t come as a surprise. No arrogance there; just that there are so few things that can be done, and so much time to lie flat on my back, thinking about solutions. If I had been able to sit up, I’d have colour coded my thoughts, sharpened my pencils, and put sticky tabs on every pages of The Textbook, just in case.

In many ways, I’m luckier than others with dysautonomia, as I have a Hickman line (see previous post for more details). I can get IV fluids, lots of IV fluids, without having to go to hospital or to a clinic, and explain to the overworked, overtired and disbelieving junior doctor that there are conditions barely mentioned in Kumar & Clarke that have treatments and medications not even listed in the British National Formulary, and that I need them. Now.

The biggest disadvantage of IV fluids, at least for me, is that they’re not very portable (initially typed ‘potable’, which is also true, but less relevant to the story). In order to run, the bag needs to be higher than my heart, and needs to remain upright with the tubing straight. The rate of IV fluids that I receive depends on my position, my blood pressure, the amount of fluid left in the bag, and whether I sleep under the tubing or on top of it.

Until now!

Because now I have a PUMP! And not just any pump. A pump that is lightweight and portable and battery powered and small.

In case your imagination didn’t fill in the pitch and tempo of that last sentence, it started just above my normal speaking pitch and tempo and got faster and faster, and higher and higher, until all the bats in south east London came to a crashing halt.

All I need to do before starting to use my pump, is read the 83 page manual. Yes, that’s just the bit in English. No-one ever accused the Germans of cutting corners.

More pictures of the pump, and its newly-upright owner to follow (for those not able to witness with their own eyes this weekend). I will be unstoppable!