UPDATE: I decided to put all the info about Benign Joint Hypermobility Syndrome up on it’s own blog. I’m going to leave this stuff here for now, but please go here to read updates and see more information on the disease, as I learn it. Thanks!!!!
Don’t worry, this isn’t about to become diseaseblog. As someone with a rare, chronic illness that does affect my cycling life (as well as all other areas), I figured it would be a good idea to post something about it here. That way I can both explain myself with a simple hyperlink, and help others who are looking for information on life with what I have.
Disclaimer: I am not a doctor, nor do I play one on TV. I’m only explaining things as I understand them, and to the best of my ability. I can’t give advice, beyond recommending stuff I already do or have tried. I may be wrong about some of this (it happens). This is just my experience.
Benign Joint Hypermobility Syndrome is the technical name of what I have.
Parsed: Benign meaning “it will not kill me,” not that it’s all fluffy-kittens-and-sunshine. Joint Hypermobility meaning my joints move too much in the wrong ways (yes, “double jointed,” though of course I have only a single set of each joint). Syndrome meaning that this problem creates a cascade of other issues throughout my body.
The Basics: essentially, BJHS is a connective tissue-based disorder. Somewhere along the line, my genetics have made it so that I don’t either produce enough of, or don’t process completely, the protein collagen. Collagen is like the scaffolding for every connective tissue in your body. Connective tissues include: skin, muscles, ligaments, tendons, hair, bones, eyes, blood vessels, spinal discs, cartilage, the intestines, the heart, the liver… basically, everywhere. In fact, I can’t actually name a part of the body that doesn’t contain it.
Loose muscles/tendons/etc. leads to loose joints, which leads to pain. But there’s so much more to this disease than that.
Part 1 – Loose Joints: Loose connective tissues mean joints that move too easily, which means pinched nerves and pain. Think about how it feels when you throw your back out, or get carpal tunnel syndrome. Now imagine this feeling all over your body.
The biggest problems for me lie in my spine. A loose spine means several things:
- My spinal cord is stressed or compressed, especially when I sleep. This compression triggers my autonomic nervous system to create a “fight/flight” response. This, in turn, creates anxiety, sleeplessness, and an amplified response to pain. Do not underestimate the sleeplessness part of this. It’s HUGE.
- My nerves get pinched, causing several types of pain. I get back pain, obviously. I get numbness and mild paralysis in my thighs and hands. I get weakness in my legs and arms, and sciatica/restless leg sensations if I sit too long. Worst of all, I get parasthesia in my fingers and toes (that’s the tingling, painful sensation you get when you sit funny and your foot goes numb, then starts to wake up). I have this sensation, in varying degrees, all the time.
- My immune system is hyper-active as well, mounting elaborate, full-scale mucus or lymph node or other responses to mild colds and viruses. I’m sick a lot longer than normal people, and a lot more seriously, yet it’s not due to a poor immune system, but rather one that’s too strong.
That’s not all, though! Those loose connective tissues aren’t just affecting my spine.
I have painful big toe joints, so no high heels for me. Walking long distances can be a disaster, so good shoes are very important. I suffer from plantar fasciitis, which means the bottoms of my feet hurt, especially in the morning. Depending on where my disease is in its cycles (I have flair ups, and flair downs, much like many diseases. These seem to be hormonal), I also randomly dislocate my fingers and toes. They pop forward, then back, leaving them bruised and painful for several days. This isn’t technically a true dislocation, as they pop right back, but I don’t know what else to call it.
Part 2 –10 Other Bad Things:
- Insomnia: did I mention the hyper-wired, jazzed-up, over-talkative, never-shuts-down nature of my brain? That I get more hyper the more exhausted I become? That I used to sleep so lightly that my roommate in college could wake me up by writing in her journal? Did I mention that, without the right meds, I probably get one or two nights of truly deep, restful sleep… a year?
- Liver metabolism: I have serious problems processing medications, as I have a liver that is slow to metabolize chemicals. I cannot take a very long list of drugs, including most pain killers. This also includes naturopathic treatments… even my own hormones! I get side effects (or no effects) with everything I take. As my naturopath likes to say: “You just walk by a chemical, and it affects you.”
- Complex PTSD and anxiety: like the stuff combat veterans get, only not quite. Caused by excessive exposure to stressful situations that one cannot escape, like, say… a bully at work (not saying that was my situation. Just as an example…). Though I received great treatment, people with BJHS are prone to stuff like this: fight or flight, remember?
- Heart palpitations: yucky, but harmless
- Irritable bowel syndrome
- Sinus problems and severe allergies (go immune system, go!)
- GERD (bad acid reflux)
- Poor balance and an inability to fully understand where my body is in relation to everything else.
- An inability to regulate body temperature, including both freezing extremities and excessive sweating, sometimes at the same time
- Hair that breaks easily and nails that are bent and curl off the tips of my fingers, as well as fingers that wrinkle like I’ve been in the tub for days
Everyone who has this disease suffers differently. Many people are hypermobile (my doc says 20% of the general population), but very few suffer from BJHS. Why is that? I’m not all that bendy. Remember the Presidential Physical Fitness Test back in the 80’s? I always flunked the flexibility part (my thigh muscles are short and tight: this turns out to be a blessing, as it keeps my hips in line!). So why did I get all the nasty side effects? No one really knows, but there’s clearly a genetic component, as well as other issues like scoliosis, that affect the progression of the disease as well.
Part 3 – Three Good Things
- Hypermobile people have fast neural connections (fight or flight, my brother, fight or flight). They think quickly. Very quickly. And easily. Bang-bang-bang go the neurons and understanding is born!
- Hypermobile people are good at yoga, until they hurt themselves by stretching too far. Before that moment, though, they are really impressive.
- Hypermobility appears to keep me from breaking my bones. I have had some very serious falls on my very hypermobile wrists that resulted in… nothing. I think I just bent instead of breaking. I can’t confirm that this is true (there were no double-blind tests done here).
Heredity: There’s some controversy over whether or not BJHS is hereditary. People who are heavily invested in studying BJHS’s more exotic cousin, Ehlers-Danlos Hypermobility Type, like to think that BJHS is just a crock made up by docs who want to profit off whiny folks whose joints hurt for no discernable reason. The few docs who treat BJHS believe fervently that there is a genetic marker for this disease, but science just hasn’t found it (or them) yet. I’m sure there is. My mother suffered from many, if not all, of the same complaints I have when she was my age. She was referred to a rheumatologist, who is the doctor who would deal with these types of diseases, but she never received a definitive diagnosis. Since she died of breast cancer over ten years ago, I can’t ask her about it now, but I’m sure it was the same disease. A set of diagnostic criteria for this disease was around in her day, but few rheumatologists were trained to spot it. Today, the criteria are more stringent, and pretty much all rheumatologists are trained to look for BJHS.
Diagnosis: The best way to get diagnosed, in terms of this disease, is to visit a qualified rheumatologist, but there’s just a word of caution here: there are almost no official specialists in this disease in the United States. Like, none, really. So while lots of folks can spot it, almost none can effectively treat it. I see a specialist in my area, but I got lucky: I had to badger literally every rheumatology department/group in the entire Northwest before I found my doctor, and he’s not technically taking new patients. Even the Mayo Clinic has no specialists.
In London, there are a few specialists and an actual Hypermobility Clinic. But it’s the British medical system, so take that information as you will, in terms of their easy accessibility to folks outside (or even inside) the UK.
Anyway, the standard for diagnosis is called the Brighton Score, and tests for both hypermobility in the joints and pain.
Treatment: Good BJHS treatment involves treating sleep disorders and rehabilitating the spine. So far, though I haven’t been in treatment for long, this seems to help me a bit, especially in terms of my over-active immune system. Getting enough deep, restful sleep is a critical component of staying well.
Sleep: I take a combination of Ambien and a low dose of an anti-anxiety medication called Lorazepam. In “normal” folks, this combo would actually reduce the amount of time spent in REM sleep, but things are different for us hypermobile types. Combined, these medications help me get into a deeper state of REM. I still have behavioral issues with sleep, though, as I find it very hard to go to bed (fight or flight). I am hoping that some Cognitive Behavioral Therapy will help with that. The good news is, having completed an extensive sleep study, I have confirmed that on this combination of medications, I sleep like a normal person! This means I’ve been sick much less since I started this treatment.
Pain: Pain meds are dicey treatments for any disease, so I use them sparingly. I take ibuprofen when things hurt, and never take opiates, even after surgery, if I can help it. I just don’t find they work well for me. I haven’t found the big guns pain meds work much on my daily aches and pains anyway. The most effective pain management treatment I have found is targeted massage therapy and chiropractic care. Combined, these help to keep me with a very low, manageable level of pain.
Update as of 7/8/11: My WONDERFUL massage therapist has been doing a new treatment for me, and after four treatments (1 month), I’m really feeling like this is helping tremendously with much of my pain. He is doing something involving pressure points and ligaments. Basically, he puts pressure on certain points and then has me move my body in counter to that. This helps the ligaments release, and the result is immediately noticeable. For example, when he works on my feet, the one he finishes first feels instantly looser and less painful than the other one. Not just a little, but a lot! He then stretches that area, and the difference in mobility between the two limbs (one worked, one not yet worked) is striking. Though my hands are still an issue, this treatment seems to be doing wonders for my feet and back. In fact, this week I asked him to do nothing but this treatment, skipping relaxation massage altogether! I think for my hands, we just haven’t found the sweet spot yet, but it’s coming. My pain is noticeably lower, and has remained that way longer than normal.
Update 8/14/11: The treatment still works better than anything else I’ve tried. We’ve found ways to reduce the pain in my hands as well as my feet, though the relief is not as thorough. My trip to Ireland was largely pain-free, which is a big change compared to the week I spent traveling in London just last year. The therapy is a form of myofascial release, focusing on ligaments. Training for this specific form of massage is available to most therapists. Give it a go!
Update 10/16/11: Remember that for anything to work, you have to take care of yourself! So I went to Ireland, and messed up my sleep schedule. Then I came back and spent three weeks knocked out with killer bronchitis. Then I fell, hard, onto my hands and knees and didn’t go see the doctor. Then I let my sleep problems get worse without revisiting my sleep doc. And I’ve been missing most of my massages due to scheduling issues. So here I am, a few weeks later, with severe pain in my hands, wrists and forearms that comes and goes, but that is pretty debilitating. I’m trying out voice recognition software next week, seeing a new primary care doc (to satisfy the needs of my new health plan), and heading back to the sleep doc once I have a new referral. I’ve been (mostly) going to my massages. But in the end: it’s a chronic illness. It will get worse if you slack off on what matters. Lesson learned (ouch).
Here’s an article on a study Group Health did (and my therapist participated in, I found out when I mentioned it today) on pain relief and massage. It really is the most effective treatment I’ve found. There are no side effects. Because of that, it’s cheaper than most medications (though not all health plans seem to have caught on to this yet) since pain meds often cause so many problems that more meds are required to counter the effects of the pain meds! I don’t understand why it isn’t the primary go-to treatment for chronic pain, at least before people are put on possibly addictive medications. If your health insurance covers any massage at all, the article suggests it’s well worth the effort to go and get a good work-over by a qualified therapist.
All the other symptoms of this disease I either tolerate, or treat symptomatically if I need to. I’d love to figure out a way to really get my digestive system fixed! Probiotics help, but nothing has completely stablized it yet. My asthma has disappeared with proper sleep (no kidding, it really has!), but my allergies are worse, probably because my immune system is no long hyped-up.
Final Word: If you have BJHS, or think you do, there are many resources out there to help your doctor navigate treatment. The work of Dr. Grahame in England is critical. In the end, this isn’t a fun disease to have, but it isn’t the end of the world. Riding a bike works well for me as therapy. Something else may work best for you. Good luck!
My favorite JAOA article explaining what this stuff is.