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?