Update: BJHS or…

Recently, I’ve been pretty quiet on this blog. That’s mostly because, to be honest, I’ve been very busy with my own life and also relatively pain-free. Little things have reminded me that I have something going on (a hour riding on a horse, for instance, which was about the physical equivalent to being stretched on the rack), but I’ve been managing.

Until recently. The stress of my everyday life has increased a great deal, but the activity level I enjoyed over the summer has decreased. The weather has turned. And just generally, everything hurts more.

My fingers tingle all the time. The pain sometimes radiates up through my wrists. The other morning, I woke up to this:

The pain on the left wrist, with the bracelet and those magnificent veins, was severe and there was a bit of swelling. The other side? Just fine, thanks very much. What did I do to cause this? I have no idea. It went away in a day. I get finger pains that come from nowhere and then disappear.

My feet are agonizing. Socks hurt. My toes feel like someone is shoving knives into the joints.

My back hurts. My neck hurts. Sometimes the pain in my hands radiates up to my elbows and shoulders. Sometimes my hips ache. My sciatica is outstanding!

Overall, I’m in what I call a “pain jag.” Things just hurt. I have headaches and body aches and I’m crabby as hell.

Add into all of this the idea that maybe it’s all not caused by BJHS at all. Maybe the hypermobility is just making something else, something more sinister, worse.

Enter Chiari, or hypoplastic posterior fossa.

My specialist has an interest in this strange disorder and in a doctor in North Carolina who treats it. Operations to correct Chiari in adults (don’t worry, I’m getting to the explanation) have helped patients with Chronic Fatigue and with Fibromyalgia, which he treats. He suspected I might be suffering from this disorder, and asked if he could send my MRIs over to North Carolina so the specialist there could take a look. I consented, and off they went. The results were inconclusive (more tests were indicated), but the specialist thought there was a pretty good chance I had the problem.


So what is Chiari? Basically, it means that the skull is too small at the base and presses on the spinal cord. This compression, depending on what gets compressed, can cause pretty much all the symptoms (pain-wise) that I experience. It wouldn’t explain everything, but it would explain the pain. And there is a surgical “cure” for Chiari, involving, from what I understand, a bit of shaving off of the skull to allow the brain stem and spinal column more room. This relieves the pressure and the symptoms.

In his exam of my MRIs, the specialist in North Carolina found evidence of compression of one of my veins, right at the base of my skull. Two-thirds compression, in fact, which isn’t encouraging. My neck is also clearly hypermobile, which is probably not helping anything. But to know for sure, I would have to head out to North Carolina.

As I do not have the funds to do this right now, I can’t see that happening anytime soon. Which is frustrating in and of itself, because even if I declined what is pretty scary surgery, it would be nice to know what’s up with my brain, you know?

For now, I live in relative ignorance, and more pain.



Blanket Supports

So for folks with BJHS, or any disease that causes nerve pain in the feet, a blanket support is one of the paramount ways to relieve pain. As my disease worsened, I began to find the weight of my blankets on my toes nearly unbearable. First, there’s the stabbing pain in my big toe joints. Then, there’s the dreaded parasthesia, which is a tingling/numbness/pain in the extremities. Imagine you’ve sat on your foot strangely and caused it to go numb. You know the feeling you get as it wakes up? I have that feeling in my toes, all the time.

For a while, I just wore heavy socks to bed and kicked my feet out, but frankly, the pressure of the socks wasn’t pleasant for that many hours. Finally, I discovered blanket supports, or as we call them: bed tents.

These are essentially u-shaped supports that slide under the mattress and hold up the blankets off my toes. They are wonderful in theory, but so far, I’m on my third one in as two years, because in practice they are often poorly made or badly designed.

I started with the one that I’m sure most folks buy first, an adjustable “clamp” style support like this one:

There are thumb clamps on the bottom so you can raise and lower the bar. Unfortunately, they quickly started lowering on their own due to the weight of our blankets. The support wasn’t very high off the mattress to begin with, which didn’t make the lowering thing any better. The supports were flimsy as well, and leaned over once the blankets were on. Eventually, even with small clamps from my boyfriend’s workshop on the bars, it broke for good.

Next I moved on to a more substantial, PVC piping made support. This support is made by a small mom-and-pop company, so I’m not going to give them bad press by name. I’m simply going to say: there’s only one place selling these, so it should be easy to avoid them. These are composed of several lengths of PVC piping joined with plumber’s joints and drilled so that the height can be adjusted with screws inserted at various “stops.” The folks who sent it lovingly wrap each one in a pretty home-sewn damask bag and are obviously committed to making a useful product. The only problem was that I don’t sleep by myself: I sleep with a blanket twister.

I am someone who lies in exactly the same position all night long. I never thrash, even when I roll over. I get up in the morning, twitch the covers back into place, and I’m good to go. My boyfriend, on the other hand, is the king of the thrash. The first thing he does upon getting into bed is to kick all the covers off his feet, and roll the rest of his body into the blankets, which he jerks around and twitches all over the place. By the time I get into bed, I have to readjust them, sometimes prying them off of him in order to have covers for myself! All this twisting about was the death of the second blanket support. It failed right at the joints for the parts that slipped under the mattress. First one side went, and I duct-taped it back together. Then the other side failed spectacularly. Too much torque.

So I had to order a new one. I was annoyed by this, as these supports run between $35-40 with shipping.

I have been waiting over a week, due to the holidays, with stacks of pillows under the blanket providing a bit of relief. Though this works in a pinch, or a hotel room, I don’t love the solution as I can never get the pillows very high and so I have to sleep all night with my ankles rolled to one side. If I roll over on my side, I often find the pressure on whichever big toe is unlucky enough to be touching anything too much to take. I was very happy to see the new blanket support arrive.

This one is from Amazon.com, by a company called TFI. It’s made of steel, so I’m hoping it will last longer. It uses two bent pipe loops connected by two pipes drilled with holes. The bent pipes have push buttons on them. Even on the smallest setting (when the two bent pipes meet inside the drilled pipes, and annoyingly the buttons can’t quite pop out at this point, but it doesn’t really matter), it’s very high. Probably more high than I would like. I am not, however, going to cut the pipes down, though someone else probably could with some simple tools.

I’m really hoping this one holds up. It seems virtually┬áindestructible, but then I would have said that about the PVC one. Only time will tell how well it survives The Blanket Twister!

For the moment, my feet are already happy to see it.