It's that time of year, when all the referrals from the hospital appointment season come through, spreading like a contagious multi-coloured Outlook rash. This morning was dermatology.
For someone with EDS I have remarkably good skin so I've never needed to see a dermatologist before. I still don't really, except I've got this little 'might be a skin tag, might be a mole' on my back which has been getting bigger for years. It's still teeny tiny in the grand scheme of things though. If it was just an inch to either side I wouldn't need to do anything about it, but it's right on my spine. The way I get up from chairs means it occasionally gets caught, but it gets squished every time I do the kind of spinal curling required to put wayward parts back in their rightful places and it makes me feel sick. I'm aware there is a certain irony in it being the teeny tiny little mole that makes me sick rather than the loudly popping, painful joint relocations, but whatever. It's my mole to be nauseous for.
For a while anyway. The mole is to be removed. It's the kind of job which my GP could do, but because of the potential to bleed lots, and issues with local anaesthesia not working it is wiser to do it in a hospital setting. In, off, out. Simples.
But it never is with EDS. Issues with the local anaesthetic lignocaine not working properly are so commonly seen in EDS that it's something experienced clinicians ask about when diagnosing people. It seems to vary a little; for some people they can be properly numbed with lignocaine it just takes more than usual to do so and doesn't last as long, others find it 'travels' and they'll get numb...but not in the part of the body the injection was intended for, and others just don't respond at all. I'm in the latter category, it was really shocking to me when I was told that local anaesthetics actually make most people fully numb as I'd always assumed they were just the world's worst painkillers. Having four impacted wisdom teeth removed with only a failed local anaesthetic will be forever etched upon my memory, along with everything said by the dentist who seemed most disconcerted by having to hold down a screaming, fighting patient insisting they could feel everything. Sadly that wasn't the only occasion I was held down, screaming in pain, as a procedure was done, whilst medical staff told me to calm down as I couldn't possibly feel a thing, they'd used lots of local anaesthetic.
The dermatology consultant is lovely and charming, and fortunately quite happy to admit he knows very little about Ehlers Danlos Syndrome. It might seem like a bad thing for a consultant not to know anything about the underlying condition a patient has, but it's probably quite common, there are far too many different rare conditions for doctors to know more than the odd fact about them all. The only time I find it a problem is when doctors very clearly don't know anything at all about EDS...but they won't admit that, and insist upon making fools of themselves by getting fact after fact wrong. That only ever goes very badly for both patient and doctor. As it's unrealistic to expect expertise in every condition, I stick to the rule that any doctor confident enough to admit they don't know everything is a doctor I'm happy to let treat me. I like the ones who use google during appointments to see if they can find any relevant research even more.
Google had a few things to say about the inefficacy of local anaesthesia in people with Ehlers Danlos Syndrome but had no opinion on what anaesthetic should be tried instead of lignocaine. I explained to the doctor that given the problems with lignocaine I've had in the past I would prefer them just to remove the mole without any anaesthetic. Usually they laser the mole off, then cauterise any bleeding, which apparently hurts. I have a suspicion it's also a disaster of an idea as it's usually very difficult to close wounds in anyone with EDS, the skin just sort of pulls apart and the wound gets wider. I think actually cutting out the mole then stitching will be the only way to get it to heal properly. As it'll only take a tiny incision and couple of sutures at most I'm not really bothered that there won't be any anaesthetic.
The doctor does seem bothered, in fact almost stressed by the idea and insisted on prescription for EMLA cream. EMLA hasn't worked on any EDS'er I've ever come across, which makes sense as it's a combination of lignocaine and prilocaine, but it does no harm to give it a go. Just not on the morning of the procedure, I'll need to be quite focused mentally to get through the pain without anaesthetic and messing about with people insisting EMLA should work will make that impossible. I'll conduct my own EMLA and pin trial over the next few weeks.
Professor Bird will know if there are any local anaesthetics more suited to people with EDS than lignocaine so the consultant has asked me to contact Leeds and let his secretary know the answer. That expertise of that nature will be impossible for patients and doctors to easily access once Professor Bird retires in September is perhaps the biggest tragedy of Chapel Allerton's continued failure to appoint a consultant with a special interest in EDS to replace him.