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…

Update, As Promised

Hello, nice person.

Thank you for waiting so patiently for this latest update.

In the last update (here), Husband was in Mexico, and I was struggling without a carer, fighting with the complicated ordering systems for my medical supplies, and trying to fight off a persistent infection.

Things have changed a little, but not as much as I might have liked.

The good news is that I’ve managed to do lots of fun things – Thanksgiving was great fun (more on that another time), and I’m looking forward to Christmas. I’ve been out a couple of times, shopping in our local area, enjoying the festive sights, sounds and smells, and taking advantage of the dry weather by walking in our local park (really just an excuse to drink hot chocolate in the cafe), and have had a few visitors at home, as well as a few Skype ‘visitors’. There has been payback (there is ALWAYS payback), so I’ve spent the rest of my time lying down, reading, working on my crochet blankets, Christmas crafts, and snuggling with the adorable fluffball.

The infection around my jejunostomy tube (the feeding tube that goes straight into my small intestine) is still determinedly present, despite three courses of intravenous (IV) antibiotics. The most recent swab didn’t grow anything, possibly because the antibiotics reduced the number of bacteria enough that they couldn’t be measured in the lab. My GP considers this ‘case closed’ despite the fact that my tube site is still hot, red, swollen, painful and oozing large amounts of foul-smelling pus. The smell bothers me more than anything else. The pus is ruining my clothes, leaving them wet, stained and sticky; combined with the smell, this makes me feel extremely self conscious, and reluctant to be near other people, even my husband. Feeling so unattractive and dirty is making me even more isolated than usual. I have had contact with a few close friends over the last few weeks, but I can’t help worrying that they are repulsed by the smell. I certainly am!

I was scheduled to have bladder surgery next week, but have cancelled it for now. It’s not just that it’s too close to Christmas; rather, it’s too much medical stuff, regardless of the time of year.

There are times when this medical roller coaster ride feels too much; too fast; too painful; too difficult. I am overwhelmed by the desperate urge to get away from all of it.

“Let me off! I need to get off!”

I feel like ripping off all the patches, dressings, splints, tubes and monitors. I want to throw away all the medications and supplies and just forget about everything health-related. I’m so fed up of having to have all this stuff in my thoughts All The Time, and of the constant reminders that I’m not ‘normal’. The boxes and boxes of supplies and medications, the equipment that clogs up all my plug sockets, the tubes and lines and scars that mark my body might as well be neon signs, flashing negativity into my mind. I have to shut it out, and run and run and run, away from it all.

I know that all this medical gumph is what keeps me alive and medically stable (ish), and most of the time I’m extremely grateful for that, but the feeling of panic doesn’t come from a place of logic and reason, and the more I try to rationalise it, the louder the volume of the panic-beats. So I’ve cancelled the surgery. I’ve also stopped taking the least important of my medications and stopped answering the telephone. Silly, I know, but it’s the only way I feel able to reclaim some control. I’ve promised myself that I’ll get back on top of it all after the holidays, but for now I am being kind to myself, and not piling on the guilt about being a bad patient and not taking care of my body properly.

The problems with the carers (I’ll refer to them as PAs – personal assistants – from now on) are ongoing, and I’m sure that’s not helping with my feelings of being out of control.

Together with my care agency, I’ve been trying to recruit a couple of PAs to work 32 hours per week between them – during the day, Monday to Friday, with flexible hours. Perhaps naively, I thought that there would be lots of people keen to work for a few hours a week, within school hours, especially as there are so many young families in this area, but that certainly hasn’t been the case so far. We have been advertising for almost 18 months, and in that time, we have received almost 100 applications.

Of these initial applications, about half were rejected immediately – mainly because they gave the impression that they were applying for every job listed on the site, in every employment category. The remainder were contacted for interview, but we were only able to get hold of 14 of them, and four of those didn’t turn up for interview. Of those that did turn up, two were not suitable for the job.

And then there were eight.

The eight successful candidates were formally offered the job, and were booked onto the 5-day specialist training course.

One candidate accepted another job before starting her training, one went AWOL part way through the training (we later found out that she had returned to her home country), and two failed the training.

And then there were four.

One completed and passed the training, but then stated that she hadn’t realised what the job involved, despite having had two interviews, a meeting with me (during which she was shown around my home and given a detailed run-down of some of the regular tasks), and approximately 35 hours of training.

And then there were three.

One worked with me full-time for a week, and did a great job. I felt very positive about her work ethic, liked her personally, and felt that she was picking up the individual training from me very well. But she resigned without notice at the end of her first week, stating that the agency was too disorganised.

And then there were two.

One worked with me for nine months and then moved on to another client within the same agency.

And then there was one.

The last candidate worked for me for four months, but called in sick with less than an hour’s notice on 20% of the days that she was scheduled to work, and then went off-radar and no-one ever heard from her again.

And then there were none.