BJHS and the heart

So, recently I had a heart scare. It started with palpitations that went on for hours. Then nine hours in the ER. Then weeks of follow ups with echocardiograms and something called a “nuke test” and wearing a little portable heart monitor for a day.

It’s all fun and games, kids, until someone loses a heart!

I tell you, nothing has ever scared me on the level that this did.

Turns out, I had a mild virus that caused the sac around my heart to swell, and I will be fine. But given the propensity of all BJHS sufferers to have problems with their hearts or their aortas, may I suggest a visit to your internist or a cardiologist, just to be sure?

Better safe than sorry.

In other news, my specialist (the only one in the US) is retiring. So I think I’m going to bug the University of Washington Rheumatology teaching staff to find me a hotshot (not hot, just hotshot) young doc who wants to learn a lot about a “rare” condition. I bet I could line up a few patients in the Pacific Northwest, just from this blog! Who’s with me? <battle cry ensues>

Just for Fun

20130218-222011.jpg

My fortune at dinner. I don’t think it’s true.

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.

Great.

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.

 

Life without High Heels

I have bad feet. It’s just part of this disease. Let me catalog the badness of my feet for you:

  • Both my big toes have joints that are so painful it feels like someone is stabbing an ice pick into them when I flex them. I can’t have shoes that hit across the top of these joints, or that point my feet too much, or that put too much pressure on the balls of my feet.
  • I get numbness and tingling in my toes so severe it keeps me awake at night through my sleep meds (my feet are the number one reason I use painkillers at all).
  • I have plantar fasciitis, which means that the bottoms of my feet swell and hurt, particularly in the morning.
  • I have overly-flexible ankle joints that roll spontaneously, leading to lots of twisted ankles and ungainly trips to the concrete.
  • My toes sometimes spontaneously dislocate and then pop back in, leading to giant purple bruises.

So as you might imagine, I can’t wear high heels. This was not always so. In fact, as a young woman I loved heels, and wore them right up until I was in my late-thirties. Just four years ago, I owned, and routinely wore, five inch heels. But no more.

What’s a girl to do? The media would have you think that it’s impossible to be sexy without high heels. Jessica Simpson wore them until she gave birth, tottering around like a toddler in her mother’s dress-up shoes. Woman spend thousands of dollars a year on heels, nearly worshipping them.

Eh, whatever. I’m over it.

What I decided was that if I couldn’t wear heels, I would wear interesting shoes. So over the past few weeks, I’ve taken photos of nearly every pair of shoes I own, so that I could show you all what it means to have built a collection of colorful, fun shoes, rather than high-heeled ones. The only shoes I own with heels now are my Dansko sandals, which if you look closely, keep your feet nearly flat anyway, and my Ariat cowboy boots, which are nearly flat on the inside as well.

Not pictured here are a couple pairs of flip-flops, my winter Ariat black clogs, and my run-down snow boots. Otherwise, this is my “collection.” Enjoy!

  

Left to Right: Ariat Cowboy Boots from Renton Western Wear, Via Spiga Cap-Toe Oxfords from Nordstrom’s Rack, Sperry Top-Siders from Ross.

  

Left to right: Pink Converse One Stars from Target, Yellow Polka-Dot Converse One Stars from Target, Foil Rainbow Star Converse from Fred Meyer

  

Left to right: Merrill Floral Flats from REI, Orange Espadrilles from Ross, Ralph Lauren Pink Plaid Tennis Shoes from Ross.

  

(old) Ecco Walking Shoes from the Walking Co., (new) Ecco Walking Shoes from 6pm, Butterfly Twists Folding Ballet Flats.

  

Left to right: Dansko Embroidered Sandals, Miz Moots Mary Janes, and Dansko Cut-Out Sandals, all from Nordstrom’s Rack.

As you can see, I have a certain “type” of shoe I prefer: cheap. I generally buy my shoes from Nordstrom’s discount shop, The Rack; or from Ross. I rarely pay full price for shoes. This is partially because I’m generally fond of a bargain, and partially because my feet are so picky that shoes have to be minor investments, in case I find I really can’t wear them after all. I’ll often try things on in the store, only to find that after an hour or two, they are unwearable. Ah well. Still, I like all my crazy, fun shoes. It’s fun to hunt them down, and I receive compliments on them all the time. I think I look adorable. So there you go: no heels necessary.

Update: May 14th

Well, it’s been a while. I’ve been sort of in a holding pattern for the last few months: very busy with my new business start-up while still finishing up at my old job. I’ve also had really awful colds this year. They seem to be hitting everyone hard, though, so while they were terrible, it’s always comforting to know it isn’t just me.

My doc tried me on a new sleep med, Trazadone, but I only made it through one night. It left me groggy and miserable at the lowest dosage. Oh well. Mostly I’m sleeping okay now.

Except for last night, when my feet were absolutely killing me. I have been neglecting the chiropractor for the last few weeks, and have cut down on massages. Boy, am I feeling it! I have an almost pathological need to walk all the time, like restless leg but when I’m awake, but then when I do… oh my aching feet!

That said, I’m sort of hard-core about walking through the pain. Perhaps this is my body getting ready for the trips I’ll be leading starting in June.

Must see the chiropractor tomorrow :).

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 indestructible, 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.

Update: December 28th

So many small changes… after months of dragging around, but feeling increasingly like I’d acquired some sort of evening ADHD, I ended up back at my doc’s office. Not because I wanted to be there, but because it was the only way to get my prescription refilled. I suppose this is why they do that whole requirement.

Anyway, we discussed my depression/anxiety/hyperactivity and inability to sleep and he suggested the opposite of what I thought he would suggest.

First off, he said I should be off the Ambien! That surprised me, as Ambien has been the only way for me to sleep soundly through the snoring, thrashing and general huffing of my bedfellow. Instead, my doc upped my level of Lorazepam, and cut the Ambien dosage in half, with the goal of reducing it completely.

So I did as instructed and I do admit, on half the Ambien and 1.5 times the Lorazepam, I do feel better. I’m sleeping soundly, and though I’m still not getting to bed on time, I don’t feel as anxious. My moods are improved as well. Tonight I’m going to try 2mg of Lorazepam and NO Ambien. We’ll see how that goes.

My naturopath also put me on 50mg of 5-HTP at bedtime, which is probably helping too. She gave me some B6, multivitamins and fish oil, but I keep forgetting to take everything but the B6.

The days are getting lighter, which should also make a difference, and I’ve had two weeks off, which has improved my mood in myriad ways.  So all in all, everything feels a bit more hopeful, though it still hurts to type this.

Update: December 16th

The funny thing about any chronic illness is that I’m never sure what should be blamed on mine, and what shouldn’t. Assumedly, for instance, other people find themselves suffering from severe insomnia and a wee bit o’depression this time of year. Since my disease is rare, they can’t all be closet BJHS patients.

So do I blame my complete inability to not only go to sleep, but to actually go to bed on the disease? You see, not only do I find it very hard to fall asleep (even on my meds), but I also find it hard to get into bed. This has gotten progressively worse over the last few years. It’s as if my nervous system wakes up the more tired I get, and by the time I’m exhausted, my brain is as chattering and restless as a two year-old on speed. I’ve always had this problem, to some degree, but right now it’s downright debilitating. If I go to bed too early, my mind screeches at me for hours. If I go to bed when my mind has finally worn itself out, then I don’t get enough sleep. But my mind’s relentless chit chat bears no relation to how tired I actually feel, so even as I sit there, exhausted to the point of nausea, I’m still awake.

Ambien is, obviously, designed to combat this, but I’ve been on it too long and need to switch meds. This requires a trip to the sleep doctor, which I’m just going to have to make. Bummer. I’m soooo tired of doctors, even though my sleep specialist is excellent and knows my BJHS doc very well.

Then there’s the deep blue funk I’m feeling. Some of it is no doubt a product of the season, and obviously the lack of sleep doesn’t help, either. Then I suspect that part of it is from the Lorazepam, which is, after all, a sedative. One of the side-effects of the drug is depression. So I’m thinking it may be time to talk to my doc about that, especially as generally this drug is counter-indicated for long-term use (though I’m on a very low dose). That said, I’m always funky in the winter, and I didn’t feel this way earlier this year, when we had sunshine.

So what to do? I don’t really like taking any medications. I also suspect that things will improve as I get to sleep more over the break, and then as the seasons change. I hate going to the doctor. But I’m also miserably tired and fussy right now.

Being sick stinks, I must say.

Update: December 7th

I feel like I ought to title this: a day that will live in infamy, except it totally isn’t, in terms of my illness.

Back in September, I fell and tore a muscle in my back. I was shocked at how long the pain lingered. Only in the last week and a half have I felt pretty much recovered. So I’m back to “normal.” But what does that mean?

Most of the time, I’m low on the pain scale. On that smiley-face-to-frowny-face thing they use in the doctor’s office, I’m sort of neutral. I realized the other day that I don’t really ever end up on the smiley-face end of the scale any more. Chronic pain really stinks.

Last week I was manic as heck: I was working like crazy, doing all sorts of projects, and unable to sleep until 1 or 2 am. I’ve never identified these phases in the illness as “manic” before, but that’s clearly what it was. I think it’s really the absence of severe pain that does it: suddenly my body has all this excess nervous energy to burn off. This week, it’s clearly gone and I’m feeling relatively calm again.

My fingers hurt when typing, and my toes and lower back are tingly when I sit for any length of time, but otherwise, I’m good. We’ll see how long it lasts!

New Site and Update

Hi folks,

After a long debate with myself, I’ve decided to put all the information I have about my disease here on this blog. Before, it lived as a single page on my bike blog, rideblog. While that worked for a bit, I realized at some point that I needed to be able to put up posts about my illness, and I couldn’t do that to a static page so that people could get those updates. So BJHSblog was born.

For now, I’m going to leave all the info up at rideblog for a while. It’s a fun site, so check it out it if you have time. Otherwise, this is the spot to hear all about how I’m doing, and what I’m learning, about my illness: Benign Joint Hypermobility Syndrome.

For those of you who are new to this blog, the history of my diagnosis and an understanding of the disease itself are on the BJHS Basics page. Only new updates are here.

Thanks so much for visiting, and be sure to let me know if you have questions! I’ll post an update about how I’m doing soon.

Follow

Get every new post delivered to your Inbox.