Diagnosis Roulette: Formally Bestowed the Title of “Bendy”

I’ve written frequently about how I’m a bendy individual, and how having a defective collagen gene or two has presented a multitude of difficulties and challenges. Ever since a physiotherapist first frowned at my feet and muttered under his breath, “Oh, where do we begin?” I’ve been aware that there’s something not quite right about how my body works.

For a long time, I thought there was nothing that could be done. I figured that all I could do was physiotherapy – strengthen the muscles that support my joints, build up my core strength in particular, make sure that I undertook activities that would train my dysfunctional proprioception (feedback from the rest of my body, knowing where I am in space, balancing).

A few years ago, I learned that hypermobility syndrome, or Ehlers-Danlos, depending on which moniker one ended up with, was a multi-systemic condition. This makes perfect sense. Collagen is a family of structural proteins distributed throughout the entire body. Defective collagen doesn’t just make you stretchy in your joints and skin: it affects temperature regulation, energy levels, immune response, gut function, urinary function, and so on. People with hypermobility syndrome can present symptoms from all of these areas or maybe just one or two.

But I still figured there was no point pursuing a formal diagnosis. It sounded expensive and troublesome.

I got talked around by two people, who pointed out that having a diagnosis would help me access help I needed if I ended up doing something truly wacky to my joints, and also pointed out that there were medications that could help with some of those weird extra symptoms.

Plus, I’d finally reached the point where I didn’t know what to do any more or how to manage the situation. I’d discovered that the day after running, I was wrecked. Absolutely useless. Horrifying fatigue. Full body ache. And I was convinced I wouldn’t be able to run any more, that as a bendy person, I’d be doing continued accumulating damage to my delicate tissues. All the online advice I saw strongly discouraged running. I was pretty devastated. I’ve worked really hard on being able to run, for a long time, and it’s one of my most intense personal achievements: I could never run as a kid. Never.

So, I took the specialist name I was given, I requested a referral from my GP, and I made an appointment.

I went in on a Friday morning, and the friendly nurse asked me a slew of questions about my history of fainting, migraine, allergies and various other things. I was fitted with a blood pressure monitor that I was required to wear for 24 hours as I continue my normal activities (yes, I took it to the gym), and a bunch of electrodes distributed across my torso to monitor my heart rate. This, I was required to wear for 36 hours.

I won’t lie, it was a pain in the fricken’ butt. The blood pressure monitor was constantly beeping whenever it was in a position that it didn’t like, the electrodes needed to be coddled whenever I moved too much, and two weeks and several showers later I’m still finding bits of electrode adhesive clinging to my skin.

It was worth it.

I went in on the Monday morning with the requisite hardware no longer attached to my person, and I spent the next hour and a half chatting to a very enthusiastic man about my entire strange history of illness and pain and discomfort and crappy balance and anxiety and so on and so forth.

Here’s the summary of how this went and what it means:

Actual Diagnosis

Under the pre-2017 diagnostic criteria, I would be readily diagnosed with Ehlers-Danlos Syndrome, Hypermobility Type. Under the new criteria (which are quite weird), I wind up with “Benign Joint Hypermobility Syndrome”, which is a stupid name, as it feels profoundly ­un-benign, and it’s really not just about joints (we didn’t get through all the diagnostic stuff before we ran out of time so it is still technically possible I might qualify for EDS, but he thinks it unlikely).

Sidebar: Dentists

I’m just inserting this here, because it has been a bit useful. People who are bendy and have defective collagen often have quite thin and porous enamel on their teeth (I told you: collagen is everywhere). This makes dentist visits a lot more painful for them than for other people! Add in the fact that bendy people are often highly resistant to local anaesthetic and need more shots (I need 2-3 relative to a normal patient’s single injection), and that often those shots contain adrenaline and bendy people are sensitised to adrenaline (I explain this below), and you’ve got a situation just crying out for panic, pain, misery and tears.

Recently I went to my dentist for the six month clean and check-up, and I told her about this diagnosis. “Aha,” she said. She knew exactly what it meant, was familiar with the effects on enamel, adrenaline tolerance and anaesthetic resistance, and exactly how to deal with it. She’s always been good when I just say I have “sensitive teeth”, but this just made it very straightforward. So now I get the numbing gel (lots of it, and no adrenaline), and also get given a spiky massage ball to fiddle with when sometimes a clean does get briefly painful (which was very helpful. Maybe that has something to do with the ADHD, I don’t know, but it really helped!).

But now, onto perhaps the most overwhelming and life changing consequence of defective collagen.

Low Blood Pressure

One of the key and most significant effects of the defective collagen is low blood pressure and orthostatic intolerance (i.e.: you feel lightheaded when you stand up too fast, sometimes your vision blurs or becomes tunnel vision or goes black or grey, your heart rate ramps up, and sometimes you might actually pass out). I’ve always had that “ah crap, I stood up too fast” issue, and I frequently have to sit immediately back down if I don’t pace myself on it. I don’t even have a choice, my legs just fold. I sit down in the shower. When I was a teenager and in my early twenties, I sometimes had to crawl out of the shower and lie down on the bath mat for a bit.

This effect occurs because the collagen in your blood vessels is too stretchy to effectively constrict, and constriction of your blood vessels (vasoconstriction) is how the blood gets back up to your heart and your brain from the lower half of your body. Now, muscular movement helps with that pumping, which is why I can walk basically forever (or until my muscles or bad feet give out), but I can’t stand still for longer than five minutes (and even five minutes makes me feel horrible).

Low blood pressure makes you lightheaded, often nauseated, and exhausted. Constant low blood pressure results in brain fog, fatigue, sometimes weird pain like that tight pain in a “coat hanger” distribution, from your shoulders up to your neck and jaw (I always thought I’d just been tensing my jaw too much when I ran, but noooo!).

Low blood pressure can cause migraines. It can cause you to be extremely sensitive to cold, particularly in your extremities (I just spent a fortune on skiing socks to wear in my house, and also set up a radiant heater under my desk because I am so unbelievably tired of my feet going numb when I’m wearing two pairs of socks, ugg boots and an electric lap blanket, and the household heater is going). This condition is associated with Reynaud’s phenomenon, where blood vessels constrict in the extremities and fingers and toes turn white and blue due to lack of blood flow. Low blood pressure often means you hate the heat, because your body’s natural response to heat is to dilate your blood vessels, and your already low blood pressure plummets, and you get to feel exhausted and sick all the time.

So cold makes you miserable and causes pain, and heat makes you nauseated and exhausted and you fall over. This is why I have such a narrow optimal temperature range!

Important sub-heading: ANXIETY

Low blood pressure also causes anxiety. This is because when your blood pressure drops, your brain thinks you’re dying, and decides to pump up the adrenaline so you can escape the threat – except there’s no immediate physical threat, of course, and here you are with an unnecessary, constant flow of adrenaline, leaving you with the very clear symptoms of an anxiety disorder, complete with panic attacks. Understandably – and unfortunately – it gets misdiagnosed as an anxiety disorder, and then treatment of it is a bit iffy in terms of effectiveness.

I’ve always had a bit of anxiety, and some days it’s dreadful enough that I basically panic anytime anyone comes near me, and I’ve never really known why. Theories abound – I have some baggage – but this makes sense. And now, when my blood pressure tanks, I have noticed that my anxiety gets a lot worse!

(As a side note: this is why a lot of bendy people become sensitised to adrenaline, and why when we are given local anaesthetics containing adrenaline, we freak out. I start shaking and crying like I’ve just been given the most terrible news, right there in the dentist’s chair. It took me a few tries to figure it out, but now I ask if dentists and doctors would please not give me adrenaline if there is any reasonable alternative.)

Back to Low Blood Pressure

Now, the low blood pressure issue is why I was ending up completely benched after running. Intense exercise necessitates muscle recovery and repair; and that process demands blood. And when you’ve got low blood pressure, and the blood is being redirected elsewhere, well, it’s not getting to your brain.

It wasn’t tissue damage. There’s no actual evidence from actual scientific studies to suggest that high impact exercise like running is going to be a problem for me in the long term. As long as I’m careful and try to avoid injury, I should be fine. I probably shouldn’t take up any contact sports where I’m going to fall over a lot, but I can run.

I can run.

Three Treatments for Low Blood Pressure in Bendy People

1) Get Moving

Exercise is encouraged. It helps raise blood pressure overall, and that’s probably why I’ve found it so helpful on a shitty fatigue day to just go for a walk. It probably takes about 5-10 minutes before I stop feeling like death, but then everything picks up and I start to feel human again. The problem is that for a lot of people who have been struggling with this for a long time, all they’ve learned is that exercise wipes them out and they feel dreadful – so they don’t move at all. However, a slow increase in activity – 20% a week is about what’s recommended – can help raise your baseline blood pressure. Just be very careful not to overdo it (obviously, the right exercise can be helpful for joint support as well, so it’s two birds and one stone).

Given my activity levels – which are pretty high – the specialist said that this wasn’t really a problem for me. I was doing the right things there, particularly as regards physiotherapy, clinical pilates and weights.

2) Granny Knickers, Corsets and Girdles

After exercise, we move on to the next solution: compression garments. Blood tends to pool in the large blood vessels in the abdomen, so it needs to be something that compresses that area.

Essentially, we’re talking shapewear. High waisted granny knickers, girdles, or under-bust corsets (waist shapers) fit the bill. They have to be tight, really tight, to raise blood pressure. Gentle compression to smooth outlines under a dress is not going to cut it.

You shouldn’t wear it lying down (blood will pool in your thorax, not desired), but if you’re up and about a lot, it’s worth giving it a go. I’ve bought a couple of items to experiment and yeah, it helps. It really helps. It’s like I can just let out a breath and relax, as though I don’t have to try so hard to be upright. I didn’t even know I was trying that hard!

They’re not always the most comfortable things, and I have Crohn’s Disease and chronic abdominal pain: compressing my abdomen has consequences (basically an increase in pain!). So I have to pace myself on these.

The other issue for me personally is that the very concept of shapewear – or at least, the way it’s marketed, to try and standardise women’s (and men’s) body shapes – kind of triggers a wee bit of feminist frustration. And yet, very functional garments!

3) Drugs, drugs, all the drugs

Then there’s the final step: medication. You can’t take medication to fix your collagen, or repair your autonomic dysfunction, but what you can do is take medication to raise your blood pressure. These generally fall into two categories: the first increases your blood volume by causing you to retain water, and the second encourages more vasoconstriction (given that your collagen is bad at it, but not necessarily non-functional).

I’ve opted for that second category. I instinctively shy away from the notion of retaining water on purpose. I have a uterus, I know what fluid retention feels like, and I want no fucking part of it, thank you very much (not that I have any evidence that it feels the same). I don’t deal with it well. However, that category of drugs does work very well for a lot of people. I just don’t want to add it to my slew of unrelated hormonal issues.

Where am I at?

How’s it working?

It’s working well. Really. It’s much easier for me to stay awake and upright. I can sit up at my  computer in the evening without wanting to just drag my laptop to bed. I don’t crash after intense workouts in the same way.

Combine this with my ADHD meds and… I’m starting to bear a dangerously close resemblance to a functional human being. None of these meds are magical. I still forget things, even though I have my ADHD meds, and get distracted; I still get really sad, even though I’m on Zoloft for depression; and I still get a bit lightheaded sometimes, even though I’m on vasoconstrictors (although I suspect that’s because they’ve worn off – a dose only lasts four hours, supposedly, and I’m pretty sure I don’t even get that long).

There’s so much more I could write about this. It’s an extremely complex condition and it touches on various aspects of my life, many of which I haven’t even listed or described here. Numerous pains and aches and weirdnesses (like that coat hanger pain that is very unpleasant and I always thought I’d just tensed my upper back and jaw too much) now have an explanation.

I no longer feel like a sooky lala for getting too cold or too hot when other people don’t, or for having sensitive teeth at the dentist, or for needing to lie down at short notice. I just take the assistance I need, and move on.

So. Here I am. ADHD, Crohn’s Disease, Hypermobility Syndrome: fully diagnosed and treatment in progress for all of them. Finally. It’s been a long fucking road.

But I think I can see the end of it.

Postscript: as a side note, while it’s not widely diagnosed or well understood, joint hypermobility is actually extremely common in the population, especially in women. If some of these symptoms sound familiar to you – and I’ll write another post with a few others that might pop up – it’s worth getting it checked out. At the bare minimum, you need a lying down vs. standing up blood pressure/heart rate test.

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