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…


Preventing Infections

I was lucky enough to be invited to the annual Biopatch Infection Control Symposium again this year, as a patient representative. I was even paid to be there, which was quite a bonus. I haven’t, however, been asked to write about the experience. All views here are my own.

The symposium really couldn’t have come at a better time for me. I’ve been finding it increasingly difficult to find the motivation for anything, especially medical things. I’m just about coping with the most essential daily tasks, but not much more, and I’m certainly not being a good advocate for myself or others. Spending the day yesterday with a room full of people who want to improve IV line care and patient experience has given me a top-up of motivation and interest, and gave me a glimpse of ‘old Jo’ – it’s been a long time since I last felt knowledgeable and connected and worthwhile.

The symposium is organised and sponsored by Ethicon (part of Johnson & Johnson), who make Biopatch. Biopatch is a small circular sponge with a hole in the middle that is designed to fit around IV lines. The sponge is impregnated with freeze-dried chlorhexidine gluconate (CHG), which is basically an antibacterial disinfectant. It’s policy at my local hospital that all central IV lines (those that end up in or near the heart, regardless of where on the body they’re inserted) have a Biopatch dressing with a clear dressing on top, so I’ve been using these little sponges for three years now.

The research is pretty impressive (HUGE randomised controlled trials and other high-quality, well-designed studies, published in peer-reviewed journals), showing that Biopatch has a significant impact on infection rates.

You can find the data and recommendations if you’re interested (start with the CDC and SHEA guidelines in the US and Epic3 guidelines in the UK – they link to lots of the studies).

That’s not really what I’m writing about.

The biggest take-home message for me was that we know lots of very good ways to reduce infection risk for people with central lines. Best practice guidelines around the world recommend ‘bundles’ of interventions, at the time the line is placed, and for long-term maintenance. None of these interventions alone is enough to keep people safe – it’s only when they’re all implemented, all the time, that the marginal benefits of each one combine to give impressive results.

Standardisation and checklists are popular at the moment, with good reason. There will always be people who have poor techniques, mavericks who think the guidelines don’t apply, and people who are good, but distracted, exhausted or overwhelmed. Checklists help to make sure that every patient receives the best possible care from every clinician, at every hospital, every time. One of the reasons, I think, that I have had such good experiences with my central line, is that I don’t take shortcuts. I was taught well, and I still use the techniques that I was taught every time. It isn’t at the front of my mind, but I’m fully aware that people die from central line infections (septicaemia), so although I am extremely grateful for my Hickman line and the medical stability it has allowed me, I try to keep a healthy fear of infection. Every time I have any contact with my line I am taking my life in my hands. I know that sounds really melodramatic, but I don’t think it’s an exaggeration.

It’s so important that patients are given the opportunity to take ownership of their line (and their treatment, more generally). I think this should be included in the best practice ‘bundles’ – the patient is the one who suffers when mistakes are made, so we really have a strong interest in ensuring that things are done correctly every time. It can be hard to criticise consultants or other senior clinicians (the balance of power is very much in their favour, after all), but I try to think of it as a teaching opportunity rather than a complaint!

I say to myself over and over, NO SHORTCUTS! It doesn’t matter how tired I am, how late I’m running, how much I want to be doing something else. An extra minute or two in order to do things properly could save me from a tedious hospital admission, or worse.

And with that, it’s ‘life in my hands’ time again.

Until next time…



As if I didn’t have enough to update, I’ve just had a couple of days away at the Costa del NHS.

Nothing major, but I had a high temperature on Monday, which is one of those issues that means, ‘Go straight to hospital. Do not pass Go. Do not collect £200.’ You know the score, I’m sure.

It turned out to be an infection of the skin around my Hickman line, plus a UTI (bladder infection). The team looking after me could not have been better – they performed a really thorough ‘sepsis screen’ which involves checking for the most likely cause of infection. My Hickman line and j-tube sites were both swabbed, and blood was taken from both sides of my line (it has one lumen reserved for nutrition and one for medication) as well as peripherally (from a vein in my arm). A urine sample was checked, and I had a chest x-ray to rule out a chest infection and to check the length of the Hickman line for infection. The chest x-ray was perfectly clear apart from a dislocated shoulder, which was no surprise to anyone.

My team liaised with the microbiology team and prescribed some heavy duty antibiotics (Co-Amoxiclav and Vancomycin). Once the results started to come in, I was started on Flucloxacillin – the Hickman line swab grew staphylococcus (staph.) aureus. This is the same bug as in MRSA, but mine isn’t resistant to any antibiotics, thankfully.

I’ve been home for a couple of days now, continuing with the IV antibiotics. Fluclox is my least favourite antibiotic because it’s given every 6 hours, and almost all my other medications are given every 8 hours, so it messes up my system. That’s not a complaint – well, it is, but anything that allows me to be at home with Husband and Feline is a Good Thing.

I’ve been feeling pretty wiped out, from the hospital stay (security guards helping to convince my roomie not to use violence when interacting with the doctors and nurses, at 3 am), as well as the infection and the medications to treat the infection. Every day has been a little better, though, so I’m sure I’ll be back to normal in no time.

Today is a lovely sunny day, so I’ll be hanging out with my loved ones. I’ll make sure I find time to take the pics I promised, of sketches and sewing.

Thanks for being patient with me!


I’m Still Here


Remember me?!

Sorry it’s been so long. Several reasons, but the main one is that I have been miserable. I tend to pull away from people when things are not going so well, so I’ve been ignoring phonecalls and text messages, email, and most other forms of communication. I currently have 15 voicemails, 4143 unread emails, and 12 other (Facebook/WhatsApp/Viber/text) messages.

I really hope that hasn’t caused any of you to feel as though I don’t care about you. I really do, but I am just finding it hard to make connections at the moment.

Part of the problem is that I am still processing some recent medical stuff. It’s hard to respond to the, “How are you?” questions (honestly) when I don’t know the answer. I also feel a certain amount of responsibility and guilt for sharing miserable and negative stuff with people, making them feel sad. I like to work out how to put a positive spin on things before I share them with others.

Another problem is that I haven’t been well. No one thing in particular, but lots of little things in combination have meant that I have been spending the majority of my time in bed. There have been some wonderful exceptions to that – film marathons with friends (this counts, even though I was technically still in bed – sofabed in the sitting room), a few trips to the park, to the local gelateria, and even a trip up north to see Husband’s family at Easter. These exceptions make me feel more human, and remind me of who I am, once the veneer of medical-badness is stripped away, and I cling to the memories of recent adventures and plans for future adventures; little lights in a tunnel that is sometimes very dark and very unpleasant.

I haven’t been able to bake or knit much recently (I haven’t even picked up knitting needles for almost 2 months), which hasn’t helped my mood. Those two things made me feel as though I could contribute something to the world – I love to give things to people, as well as enjoying the process of making them. It’s been hard not to feel like a waste of space, and a colossal drain on NHS resources, when I’m not able to make anything tangible. I get to the end of the day and feel as though I’ve accomplished nothing. Watching box sets and reading Dickens doesn’t really count as a worthwhile contribution to society, does it?

I’ve been trying to find other interests and other ways to feel productive within these new limitations. I have been drawing and painting a little bit, and trying out several other craft techniques. As part of my New Year’s resolution this year, I have been keeping a creative journal, in which I write, draw, design, plan, collage, doodle, etc. I suppose it’s a Smashbook of sorts.

One of my long-term goals, when I left hospital in November 2013 (after a 3-week inpatient rehabilitation programme), was to sign up for a course to learn new skills or improve old ones. That hasn’t been as easy as it sounds. Early mornings are out, for reasons that I explained in my previous post; evenings are difficult because I’ve usually run out of energy and run in to pain; at the best of times, I struggle to sit for more than a few hours, so each session/class would need to be relatively short and/or close to home. Mondays are generally a write-off after the weekend, when I use all my energy in order to spend quality time with Husband; likewise, I try to rest on Fridays, to conserve energy for the weekend. All these factors limit my choice of courses quite considerably, and that’s without even thinking about whether the course interests me, or if it would be physically possible. With all those limitations, it’s taken a while, but I’ve found a course that could have been made especially for me: Experimental Pattern Cutting and Design (intermediate) at our local adult education centre, which is a 15 minute ‘walk’ (in the electric wheelchair), or 5 minute drive, from home: 2 hours on a Tuesday afternoon, for 8 weeks.

Frustratingly, the course was cancelled at the last minute due to lack of interest, but I met with the tutor, who went through some of my sketches, photos of previous projects and patterns I’ve drafted, and gave me lots of helpful advice and criticism. One of her suggestions was to put together a more formal portfolio and apply for a course at the London College of Fashion, Camberwell College of Arts, or Central St Martin’s. It hadn’t even crossed my mind that I might be good enough for anything like that (I’ve spent my whole life being nudged towards the sciences, and firmly put in my place – Not an Artist – by art teachers), so I felt really encouraged, if somewhat shocked! In addition to giving so much of her time to meet with me, the tutor emailed me with lots more information and guides for further reading/projects to try, and offered to meet me again to talk about the projects, and any stumbling blocks I encountered.

I don’t think my body would handle the travel to and from any of the above colleges (except perhaps Camberwell, which is pretty close to home), let alone the physical demands of even short courses, but it has certainly given me food for thought. I’ve signed up for Craftsy courses (online video-based) on couture sewing techniques and drawing, and have been working my way through those, as well as a couple of books and lots of blogs.

This has all come at just the right time, and has given me such a boost. I have something exciting to occupy my mind, and so many hands-on projects for when my body is feeling cooperative. I’m currently sewing a dress and a jacket for myself, making adjustments to the patterns, and using the couture techniques I’ve been learning. If they go well, I’ll be looking for volunteers for my next project. I’d really like to sew/design things for other people, but I’m not terribly confident yet, and I’m quite slow – a combination of actually being slow (tactfully, I’m a meticulous detail person) and spending a lot of time in bed. I’m very happy, but am trying not to let myself get too excited about these fun projects – it seems as though the more emotionally invested I am in something, the more likely it is that my body will find some way of sabotaging it!

I’ll post photos of some of my recent creations soon. I know I still owe you posts about my bladder operation, a medical update (sigh) and photos of the garden (Husband has been doing wonderful things!) and the cat, who is ridiculous and hilarious, and is still a ray of (sharp and spiky) sunshine in my days, but those posts will have to wait for another day – my sewing machine is calling to me.

Until next time…


** Love life; Dream big; Be positive **
Rina Goldberg