Hanging on to My Inner Human

I’m in hospital. It’s an infection, but probably nothing major, so I’ll be out in a day or two. 

Despite that, it took me two attempts to get to the hospital. I contacted one of my nurse specialists, who said I should head to A&E. I checked my Hospital Bag, put on some Strong Lipstick* and headed for the hospital. I made it as far as the bus stop before freaking out at the thought of another hospital admission. All the bad experiences of years of admissions have accumulated, and I just couldn’t face another admission. So I went home. 

This morning I remembered a favourite quote by Mary Ann Radmacher: Courage doesn’t always roar; sometimes courage is a small voice at the end of the day saying, “I will try again tomorrow”

And I got up and tried again. 

I’ve been actively looking for moments of gratitude; moments squeezed in between the hustle and bustle of the hospital, the procedures, the paperwork. I’ve been so grateful for the nurses who’ve greeted me with hugs, the lovely roomie, the doctor who remembered me and asked after my husband and the cat, everyone who has fought my corner today. One theme that runs through my gratitude list is humanity. All those little reminders that I have a real life outside the hospital and that I matter; that there’s a person inside the patient. 

As time has passed, I’ve found it more and more difficult to be what I consider to be the real me. At home as well, but especially in hospital. I find myself tongue tied, mutely acquiescing to things I don’t want, saying “thank you” and “sorry” and “thank you” again. I feel as though I’m slowly being swallowed up by this condition, gradually being submerged; drowning by degrees. 

Every time someone acknowledges the human within the patient, they are metaphorically reaching out a hand to pull me back to Me. The condition is still there, of course, but I would like to be Me for a bit longer. For all those people who manage to see me drowning even in the midst of all the hospital processes and rush, and who reach out and acknowledge me, I am profoundly grateful. 

*Strong Lipstick: Any lipstick that makes one feel better, stronger, more confident. In this case, Ruby Woo by MAC


Another Day, Another Infection

This time, it’s an infection around my feeding tube. These infections build up behind the tube site, like an abscess, and it’s the pressure from this build-up that causes the pain that lets me know it’s time for some more antibiotics.

At times like this, I’m so grateful for a wonderful GP, who squeezed me into her busy clinic within an hour of my call. Actually, not my call, but a call from the nutrition nurse (M) who was visiting me, and insisted that I shouldn’t wait three weeks to mention it to the practice nurse at my next appointment. We both agreed that the site was infected, so M called my GP surgery and they saw me pretty much straight away. As usual, they took swabs of the pus oozing from the tube site (think that’s too much information? You should just be grateful that you couldn’t smell it!) and prescribed IV antibiotics and special dressings to cover the tube site.

More gratitude, this time for my local pharmacy. They deal with all sorts of weird medications on a pretty punishing schedule without batting an eyelid. They even keep a small stock of certain IV medications so they can give me some straight away while we wait for the rest to be delivered. For a busy community pharmacy, they do a pretty impressive job of providing a personal service – every time I go in there (which is a lot) they are offering advice, ordering medications and chatting to people. It took a while for them to get to know me when we first moved to this area, and there were quite a few teething problems with my usual (unusual) medications (almost all of them have to be ordered, and quite a few are compounded just for me, though most of those are delivered to my home). Now, though, I feel totally confident in the pharmacists, and don’t know what I’d do without them – being able to get IV antibiotics the same day from a community pharmacy is pretty impressive!

As usual, having an infection has knocked everything else out of balance. More pain, weakness, fatigue, etc.

I’d love to find out what my ‘baseline’ would be like without lurching from infection to infection, but for now, this is my baseline. Infection after infection after infection… The antibiotic routine is pretty punishing as well – every 6 hours, which doesn’t fit in terribly well with my other IV medications, most of which are given every 8 hours. It just makes everything very busy. So I sleep and medicate, and sleep some more. In between times, I’ve been watching White Collar on Netflix, which is a lot of fun. I’m approaching the end of the current series, though, so I need recommendations for my next box set… Nothing too taxing – brain fluff is just right!


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?


Self-Censorship (or, the lost art of telling it like it is)

I like to make my loved ones feel happy. I am almost compulsively nurturing, and slip very naturally into ‘Mummy-Jo’ mode. I love to spend quality time with people, and am at my happiest when I can cook for people, give handmade gifts, write personal letters and cards, and ‘be there’ for my loved ones. I wish that I could give back to my friends and family as much as I receive from them. I know that’s unlikely (verging on impossible), so the only way to make things equal is for me to take less. I don’t want people to feel obliged to look after me, and I certainly don’t want to ‘pollute’ the precious few social events I can attend with my medical misery.

That’s ok, though, because I started a blog, didn’t I? A blog that would be a safe place where I could complain at length, and then send it out into the ether to be read (or not read) by friends and family. It should have been the perfect solution.

It could still be the perfect solution. All I have to do is write.

So why haven’t I written anything for so long?

One reason is that I don’t want to be responsible for making people feel sad, whether those people are close friends, casual acquaintances, or someone who’s stumbled on my blog while trying to find out more about Ehlers-Danlos syndrome (EDS). There’s quite enough awfulness in the world without me playing the Melancholic Minstrel, singing ‘pity me’ songs on loop.

A few months ago, someone very close to me asked for a detailed medical update, but stopped me after a minute or two because they were finding it too upsetting. As far as I was concerned, I’d barely scratched the surface. I had hoped, at the very least, for a listening ear and a hug. As it was, I ended up being the one doing the comforting, and left the conversation feeling guilty for having upset someone that I love.

At other times, when I have tried to talk about medical stuff, and especially about some of the future plans that I’ve been putting in place, people have told me that I shouldn’t be so negative, and that I should focus on my blessings, and on the here and now, instead of seeking out troubles for tomorrow. I agree with that in principle; there are lots of very good things in my life, for which I am extremely grateful. There are also some Terrible, Horrible, No Good, Very Bad things, and feeling that I can’t/shouldn’t talk about them is very isolating. I’m trapped inside this body that is crumbling around me, cut off from the outside world; grieving for lost abilities, lost freedom, lost dreams. I’m afraid for the future and the new tortures that my body has in store for me (or even just more of the old, familiar tortures), but I’m even more afraid of not having enough future left ahead of me, and having to face the thought that I may shuffle off into the sunset, leaving no legacy; nothing to say that I was here.

My reaction to all of this probably should have been to continue to write the blog, giving you the choice of whether to read every word of it, read some of it, or not read it at all. But it wasn’t. I didn’t want to be responsible for making you feel sad. I didn’t want to admit that I can’t cope; that I feel as though I’m barely existing, and certainly not living life to the full. I didn’t want to see, in harsh black and white, how small my world has become.

Something changed for me recently, though, which is why I’m writing now. I saw a friend for the first time in ages, and heard about some of the dreadful things that have been happening in her world – I felt so sad that I hadn’t known. Not that I could have done anything, but I could have been there, on the end of the telephone, or sending hugs and chocolate via Royal Mail. I hope that you don’t feel the same way when I do finally admit how bad things have been.

I’m sorry for not giving you the opportunity to decide how much you want to know. I’m sorry for not letting you be there for me and my husband. I’m sorry for not trusting you to handle my raw emotions gently.

So, let’s try this again.

I will write.

The rest is up to you.


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?


Hospital Glam


I loathe hospital gowns and the uniform anonymity that comes with them. All patients look alike, stripped of all identifying features, and devoid of clues as to their background, interests, likes and dislikes, and professions. I think this can be extremely damaging, as it encourages the mindset in clinicians that patients are somehow different – it’s them and us.

The comedian Richard Herring writes about the distinction between the disabled and able-bodied. Except that he refers to these two groups as ‘the disabled’ and ‘the not yet disabled’. Any one of us, at any time, could be touched by the cold fingers of severe illness, or severe accident, either of which could have long-term ramifications. Disability does not respect status, education, age or profession. The line between these two groups is narrow, and easily crossed (in one direction, at least).

Hard as it is, I think it’s important to recognise the humanity behind the suffering of hospital patients (and those outside the hospital who are sick or disabled, or both).

So I put up photographs of my life outside the hospital, I get dressed, and I put on make-up. It takes effort, of course, but I think it goes at least some way towards bridging the gap; reminding clinicians that, in many ways, I’m just like them.


I’m in hospital again

So, ironically enough, I am in hospital with an infection. So much for being an expert in the theory of infection prevention!

I was admitted yesterday, through A&E, which, thankfully, wasn’t too busy. I was moved to ‘Majors’, where everything was routine and normal.

As a quick aside, A&E in the UK is usually split into three areas: Resus, for the sickest patients; Majors for people who are quite unwell and will almost certainly be admitted to the hospital; and Minors, where the less life-threatening cases are seen.

Back to me: I was seen by one of the A&E doctors, who was very kind and very efficient (a rare combination!) and got the ball rolling for all the tests anc treatments I would need. One of the Emergency Department technicians came and put a cannula into a vein in my arm. She got a gold star for getting a vein on her first try. Once the technician had found a vein, she took lots of blood for various tests, and then left me alone to read my book.

About 3 minutes later, the technician returned with a slip of paper containing some blood results. She looked worried. The doctor read the results and looked worried. My nurse read the results and looked worried. I read the results and suddenly had a mind swirling with unprintable words.

After that, everything happened very quickly. Lots more tests, lots of medications, lots of concerned faces.

The magic numbers that caused all the worry, for those who are medically knowledgeable or like to google things:

Potassium: 2.6 mmol/L (normal 3.5 – 4.5 mmol/L)
Lactate: 4.5 mmol/L (normal range 0.5 – 1.0 mmol/L, or 0.5 – 2.0 in critically ill patients)
pH: 7.31 (normal 7.35 – 7.45)
Blood sugar: 8.9 mmol/L (normal about 4 – 6 mmol/L)

Since then, I have been on a medical admissions ward, where I’m perfectly content. The plan is to take out my Hickman line, which has been infected since January, with the same bacteria (staph aureus) despite 5 courses of intravenous antibiotics.

One of the wonderful nurses from the IV team came to see me, and has promised that he will squeeze me onto the list tomorrow for a PICC line (a less permanent central line, which ends in the same place as my current line, just above the heart, but is inserted in the arm and not tunnelled under the skin). This will give me a reliable way to get medications and fluids until they’re happy that the infection has gone, and can insert a new Hickman line.

I will be in hospital for a while…