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?


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…


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!







Hickman Line – what is it and why do I have one?

Most of you will have seen the transparent dressing on my upper chest, and the white plastic line underneath it. This is my Hickman line.

What is a Hickman Line?
In technical terms, it’s a tunnelled central line.

Central because the tip of the line sits either just in, or just above my heart (that’s pretty central, no?). To get the line to that spot, it was inserted into a vein in my neck and just gently pushed through the vein until it arrived at my heart. All roads lead to Rome; all veins lead to the heart (with one exception, in case my pedantic medic friends are reading this).

If the line had been left like this, it would be just a ‘normal’ central line.

This is a 'normal' central line

This is a ‘normal’ central line

These are used in hospitals all the time (usually in intensive care units). However, because the line comes out of the skin right next to the vein, it provides a direct route for infection to get into the bloodstream. This is a Bad Thing.

To reduce the risk of infection, long-term lines like mine are ‘tunnelled’ under the skin so that the exit point is a long way from the vein. Hickman lines (a brand name) have a cuff around the line that sits in the tunnel, usually an inch or two away from the exit site. This is designed to encourage the skin to heal around the cuff inside the tunnel, providing another barrier to infection, and anchoring the line firmly in place.

Hickman lines can have one, two or three lumens (separate lines with a plastic outer coating). My line has two lumens: one is reserved for IV nutrition and the other one is used for my normal daily medications and fluids.

Why do I have a Hickman line?
If you were paying attention, you might notice that I just answered that, but there’s a more detailed answer. Because my stomach and intestines are too stretchy, they don’t work very efficiently to break up food and move it through the GI tract, absorbing nutrients along the way. We know that I absorb some oral medications extremely erratically, because there are blood tests to check absorption. We also suspect that I don’t absorb other medications, fluids or nutrients from food. Because I have severe autonomic dysfunction (dysautonomia), my body needs about 3 litres of fluids per day, but I can take in less than 1 litre orally, of which some is not absorbed. In this hot weather, I’m running 3 litres of fluids per day through my Hickman line.

In hospital, it is commonplace to use peripheral cannulae (peripheral being the opposite of central), usually in the hands or arms. In the year or two prior to getting my Hickman line, I had literally hundreds of these peripheral cannulae placed, and hundreds more unsuccessful attempts. My veins are very small, and very fragile, like the rest of my skin. If someone is skillful or lucky enough to get a cannula into one of my veins, it’s almost certain to fail within 24 hours (the policy at my local hospital is to change them every five days routinely, or more often if necessary). After trying all the usual places, I ended up with IV lines in places as unusual  as my fingers and feet.

Some of the medications that I need are very irritant to veins, so must be administered into a large vein, i.e. via a central line, to avoid causing local damage. This includes TPN – the IV nutrition that I am likely to need at home at some point. I had an appointment with one of the specialists at the Intestinal Failure Unit at St Mark’s Hospital (one of only two such units in the country), which lasted for over two hours, discussing my medical history and possible treatment options. All my doctors seem to be agreed that I will need TPN at some point, as my GI tract will continue to deteriorate. The Intestinal Failure specialist was in favour of a trial of TPN sooner rather than later, despite the fact that I’ve managed to gain back much of the weight that I lost when things went really pear-shaped back in 2012, but my local nutrition specialist would like to delay TPN as long as possible, as there are so many risks associated with it, and once started, it’s likely that I’ll be on TPN for the rest of my life.

Finally, I need to be able to use this line and care for it at home. There are very long lines (called PICC lines), which are inserted into a vein, usually in the arm, and are then threaded up the vein to the heart, under x-ray guidance. The main problem with PICC lines, from my point of view, is that if the line is in one of my arms, I can’t take care of it myself. PICC lines are also more likely to come out than Hickman lines and don’t have the cuff that protects against infection.

What could go wrong?

There are lots of reasons not to make the decision to have a Hickman line too lightly. There are a number of potentially serious complications:

  • Infections – any ‘foreign’ object within the body is a playground for bacteria, and a playground that leads straight into the bloodstream is a big risk. Having IV nutrition into a Hickman line increases the infection risk, because TPN is sugary, and bacteria just loooove sugar! I’ve had a number of episodes of TPN, always in hospital, and usually for around two weeks, but I’ve only had one infection. Bloodstream infections can be really serious, leading to organ failure or even death. I was lucky that the bacteria that got into my bloodstream was sensitive to the usual antibiotics, and that I got the appropriate treatment quickly enough to prevent serious damage to any of my organs, and that the bacteria was sensitive enough to antibiotics that I was able to keep my line (with certain types of bacteria, the line has to be removed, as the bacteria can form a ‘biofilm’ within or around the line, which allows them to ‘hide’ from antibiotics).
  • Blood clots – foreign objects, again, provide a point where the blood flow slows down, and if the walls of the blood vessel get damaged, e.g. by the line rubbing against them, chemicals can be released that encourage the formation of blood clots. These clots can cling to the walls of the Hickman line, where they can grow and grow, and then float off around the body. The size of the clot will determine where it gets stuck – big clots stick in big blood vessels; little clots stick in little blood vessels. Mostly, large clots cause more problems than small ones, but if the clot lodges in the lungs, heart or brain, they can cause really serious problems. I’ve been lucky not to have any clots so far, but I’m not on TPN, which is a significant risk factor for clots
  • Damage to the line – some lines can migrate out altogether, especially those that don’t have the cuff around them, but even if the line stays put, it can degrade over time. I lost my first Hickman line due to degradation of the plastic of one of the lumens, allowing a large hole to form. I was very glad to have the second lumen, so that I could still receive the medications and fluids that I need at home, in the month or so that I had to wait for the line to be replaced
Oh dear... Look at that hole!

Oh dear… Look at that hole!


Who looks after the line?

The short answer to that is that I do. I get supplies from a number of places, including my local hospital, clinical commissioning group, district nurses and on prescription from my GP. Most people would have district nurses come in to change their dressing, and possibly also to administer medications. The district nurses did come out to watch me change my dressing the first few times when I left hospital, but they couldn’t tell me when they would arrive, beyond ‘Tuesday’, which I found frustrating, and which would have been even more frustrating if I’d needed the nurses to come out to give antibiotics or other medications up to four times daily.

I’m lucky that my line was placed at a hospital where they have an IV team committed to patient safety, and that they were supportive of my wishes to take care of the line myself. It’s not just being obsessive (though it is mostly that), but every person who touches my line is a potential carrier of infection. I can’t check everything, such as the handwashing technique of my local nurses, or whether they have pushed their hair out of their face with sterile gloves. If I take care of the line, I know exactly the standard of care that I receive, every time my line is accessed. If something goes wrong, the blame lies squarely in my court.


I hope that answers your questions, but feel free to ask questions in the comments, or by email. I’m always happy to share…