Everything You Wanted To Know About Managing Hypermobility Syndrome But Didn't Know To Ask
...or, the entry I will simply point people at when people come to me for advice in living with one of the hypermobility disorders!
More and more doctors are starting to become aware of Ehlers-Danlos Syndrome and other forms of hypermobility disorder, and many people who have been having problems for their whole lives are finally finding a diagnosis, thanks in large part to the internet. (I diagnosed myself thanks to some Google work; my doctor, when I found her, was amused by the story.) I've had a double handful of people come to me about EDS and other forms of hypermobility disorder in the past year or two, and this is sort of an attempt to boil down my standard patter of What I've Learned and get the input of some other EDS sufferers (I know there are a few others who read here) to incorporate into it, for people who have just received their diagnosis and are wondering what the fuck comes next, as this is a situation I find myself in quite often...
Standard disclaimer: I am not a doctor (although I play one on the internets from time to time), and this is not medical advice. Also, EDS and the related disorders are so specific and individual that your presentation may be far different than mine or anyone else's. This is what works for me; what works for you is going to be different.
The first thing I will tell you, because you have likely had the same fights with the medical profession that most of us have had, prior to your diagnosis: You are not making this up. This is not in your head. You are not imagining things. If a doctor tells you to suck it up and deal (as so many of my doctors did, over the years), the doctor is wrong, not you. Fire the doctor and find another one.
(It's sad that I have to say that, but every single person I've talked to has gone through a string of doctors who did tell them it was all in their heads until they found one who didn't suck. Mine used to tell me it was growing pains, or that I was looking for attention, or that I was just lazy. Let's just take the doctor rant as a given here, okay?)
Treatment Plans
Your first step should be the Ehlers-Danlos National Foundation. They are a treasure trove of information, including forums, and your local group will be the first place to go to find practitioners who are familiar with EDS and its management. There's also a huge psychological impact to finding other people who have the same problems -- you are not alone, etc. There's a low-traffic LJ community as well: ehlersdanlos.
Finding a doctor familiar with EDS is your first step. In many cases, you'll get your diagnosis from either a geneticist or a rheumatologist; either way, you need a primary care doctor who's familiar with the disorder, since it tends to be comorbid with a lot of other problems. The first thing your doctor should do -- after working out treatment plans, etc -- is to send you for a full hormone panel workup; something like 80% of EDS patients also present with insulin resistance or low-functioning thyroid, and treating thyroid problems can have seriously beneficial effects on chronic joint pain such as that which accompanies EDS. Check your vitamin levels, too. Everybody presents differently, but there's a really good chance that you're low on something (most common candidates: vitamin D and potassium).
Finding a medication treatment plan is going to take some hit-and-miss, and it'll all depend on your individual doctor's suggestions. Me, I'm currently on tramadol daily for pain management, carisoprodol as a muscle relaxant, and oxycodone and lidoderm patches for breakthrough pain. I have sung the glories of Lidoderm before; if your doctor doesn't know about them, ask for them. (They are pretty much responsible for me being able to get out of bed, most days.)
Bodywork
Physical therapy is going to probably be the first thing your doctor prescribes. Be sure to do the extra work to find a PT who's familiar with EDS; if they don't know what they're doing, they will hurt you and not help you. (This is why I am not currently in PT; I can't find a PT I trust enough.)
I get really good results from chiropractic work. Again, you have to find a chiro who knows EDS (and if you're in the Baltimore/DC area, can I just take a second and plug Dr. Eric Horne in Pasadena, MD, who knows exactly what he's doing with EDS and works miracles). Chiropractic work can really contribute to fixing some of the long-term damage; I, for instance, keep dislocating the vertebrae in my neck and upper shoulders, and the long-term results of said dislocations involve lots of scar tissue building up around the cerebrospinal fluid in my neck, which is likely going to lead to paralysis sooner or later. (This is why regular MRIs are a good idea: to track the progress of the scarring.) Chiropractic work can help re-seat the joints and keep them there, if the chiro knows what s/he's doing, and stave off the inevitable.
Likewise, a good massage therapist works wonders. Again, you need someone who knows or is willing to learn how to work with hypermobility. The American Massage Therapist Association has a practitioner locator; don't hesitate to interview the MT ahead of time, and don't expect miracles, but a long-term relationship with a massage therapist can really help you. (Again, MD locals, allow me to plug Kevin Hatcher at the Catonsville Wellness Center, who is incredible.)
In terms of physical activity, swimming is your best bet: the water will prevent you from doing more damage to things, while at the same time giving you a workout and building muscle strength. Particularly avoid body-contact sports or anything that involves high impact. You want to minimize the chance of dislocating things or stressing your joints. (Weightlifting, for instance, is right out.)
Comorbidity
Which is a fancy word for "other shit that this stupid condition causes". EDS is known as a syndrome and not a disease for the fact that it's a whole bunch of fun things rolled into one. You will slowly become familiar with all of these. (In many cases, it's one of these other things that will lead to your diagnosis; I found EDS because of the Novocaine immunity.)
Not everyone who presents with EDS will have these problems, but there's a pretty good chance you'll have some of them. I've already mentioned the insulin resistance and the underactive thyroid problem. Some other fun side effects:
* Novocaine resistance (and resistance to other forms of anaesthetic). You may have already noticed this over time, when getting dental work done; you're probably used to needing eighty jillion shots and still not getting complete anaesthetic. One thing that will help with this: whenever you need novocaine delivered, ask to have it done with intra-ligamentory injection. (Your dentist might not be trained in this method of delivery; if not, get a different dentist.)
* Painkiller resistance. Over time, you'll build up a resistance to painkillers of all kinds, but particularly the opiate-based derivatives. This is one of the reasons why it's critical to avoid using those drugs for regular pain relief, to avoid building up a tolerance. (You'll need them later.) Many patients report that their regular medication will work for a while and then stop working over time; if you're experiencing a downswing in the efficacy of your usual regimen, it might be time to switch daily-dose drugs for a while. (I have to trade off mine every 9-12 months.)
* Stomach problems. Not necessarily because of the EDS, but because a lot of the drugs cause issues with the stomach and digestive tract. I don't really have any advice here, except to keep your doctor fully informed about what's going on. (There are days when the only thing I can eat are granola bars and brown rice.) Many people report that a low-glycemic-index diet helps them a lot, not only in being able to eat but also in having more energy. (It's connected to the insulin resistance.)
* Polycystic ovarian syndrome. Many female EDS patients find that they also have PCOS. It's annoying. The drugs to fix it are even more annoying. Unfortunately, there's not much in the way of treatment that doesn't have horrible side effects (many of which compound with the previous bullet point). If you do decide to treat the PCOS, I'd stick some extra magazines in the bathroom; you'll be in there a lot.
* Skin problems. EDS is associated with lots of skin problems -- hyper-fragile skin, rapid skin growth, bad scarring, etc, etc. One thing you should be familiar with, and keep around the house, are gel-based vitamin E caplets; whenever you have any cuts or incisions, treat them by puncturing a caplet and squeezing the gel onto the scab, rubbing the gel in very lightly, three times a day for up to two weeks after injury. This will help in reducing the likelihood of forming keloid scars. (You'll likely still scar, but not as badly.) If you have the type of EDS that comes with rapid skin growth, you're likely plagued by ingrown/abcessed hairs a lot in areas you shave regularly; make friends with your tweezers, and pick up a tube of ichthammol salve at your pharmacist's. (You might have trouble finding it; if you do, go into a local pharmacy, not a chain pharmacy, and ask the oldest pharmacist there if they carry "black drawing salve".)
Managing your ergonomics
Part of the fun of having a disorder that results in loose joints is the fun of randomly dislocating things. This can be as showy as the typical traumatic dislocation that everyone thinks of when they hear 'dislocated', or the more mild subluxation of joints that after a while doesn't even need a trip to the ER to have it reduced. (I've gotten very good at fixing things on my own, to the point where people barely even notice; this weekend at the stitch & bitch, I popped out my wrist while rooting in the yarn bins, and I bet half the people there didn't even realize I'd done it.)
There are a lot of things you can do to fix up your ergonomics, most of which is outside the scope of this entry, and all of which is going to be very individual based on your particular trouble joints. The key point in all of this is to arrange your world so that you avoid putting stress on joints that tend to dislocate. If you're lucky, you don't yet know which joints those are going to be. (I hope this continues for a long time.) Common targets, however, are elbows, knees, ankles, wrists, and fingers -- joints that are highly hypermobile in EDS patients and joints that are under a lot of daily stress. (My trouble spots include my hip joints, too, which is why I can't walk properly a lot of the time.)
Many, many EDS patients are diagnosed with carpal tunnel syndrome. Whether you actually have it or not is often even odds, since EDS can present as carpal tunnel a lot of the time, but since wrists are a real problem with EDS, you should still take care of them. The Typing Injury FAQ is not just a FAQ, it's a whole host of information about repetitive stress injury, and you should live it, learn it, love it. One thing in particular to keep in mind; if your wrists are at all susceptable, you should get a pair of good, well-fitting wrist braces (the kind with the metal inset to keep your wrists from moving) and sleep in them, even if you can't wear them while typing. You do more damage to your wrists while you're asleep than you do during a full day.
Compression braces or athletic braces are hit-and-miss for a lot of EDS patients; some people they help, some people they injure worse. Don't feel bad about trial-and-error. We have a whole cabinet full of various braces; some days they help, some days they hurt. If all else fails, a good selection of Ace bandages and the willingness to try different methods of wrapping them until you hit on the one that works will usually suffice. (They're also good at keeping the lidoderm patches on; the damn things have a tendency to peel off at the drop of a hat.)
While you're sitting and working at the keyboard (and you have arranged your work area so that your elbows are at precisely a 90* angle -- no higher, no lower -- and the top of your monitor is exactly at eye level when you're holding your head unbent, right?) you should not only take frequent breaks -- 5 minutes every 30 minutes -- but also remember to move around and shift your weight in the chair, sitting in different ways. This contradicts the usual ergo advice given to non-EDS patients, who are told to sit with their feet flat on the floor in front of them, but most EDS patients find that this results in unpleasant hip dislocation (the longer you stay in one position, the more your joints will try to shift out weirdly). I tend to cycle through feet down, legs crossed, legs crossed in half-lotus position, kneeling seiza in my desk chair, sitting on my feet, then back to feet down. My doctor suggests a yoga ball -- one of those things you sit on and have to constantly adjust your positioning -- to keep you cycling through various positions, but I couldn't adjust to it. If you can, though, it might help. (I also tend to pick up my laptop and lie face-down on the floor for five or ten minutes per hour, or at least until the cat comes over to sleep on my keyboard.)
I've had a lot of luck with support gloves lately, since my wrists have gone all wacky and prone to unseat themselves. They keep me from hyper-extending the wrist without really interfering in regular range of motion. You'll probably have to work out your own methods for reminding yourself not to hyper-extend your joints; whatever works for you. The key is to remind yourself that normal joints don't bend that way, and you shouldn't either. Just because you can hyper-extend your elbows doesn't mean that you should; the more you can keep yourself from hyper-extending your joints, the better results you'll get.
Many products that are designed for people with arthritis will be useful for you. Even if you don't think you'll use them, look over a catalog and get some ideas for how you can make things easier on yourself. The more you work with adaptive equipment, the more you'll lessen the impact on your joints. Remember, your fingers (and your toes) are joints, too, and they are also likely hypermobile. The less you strain things, the better.
Resist the urge to crack things just because you can. It'll feel better for a second after you do it, but it'll go right back to hurting, and the more you do it, the more it'll need to be done. (I totally do not follow this point of advice. I regularly crack everything from my neck to my back to my hip to my wrist all the way down, not just my knuckles, and I have to pop my left second toe back into joint at least three times a week.)
I'm sure I'll think of fifty other points of advice as soon as I post this entry, but this is good enough for now, especially since my knock-you-out drugs are starting to do their knock-you-out thing. I hope that other EDS sufferers will chime in with their practical bits of advice in the comments, to add onto this, and I'll come back and edit in things as I think of them.
In the meantime: Good luck. You are not alone. There are others of us out here. This disorder is annoying, painful, and complicated, but it's not impossible to manage, and you are not weak if you make accomodations.
Hang in there.
More and more doctors are starting to become aware of Ehlers-Danlos Syndrome and other forms of hypermobility disorder, and many people who have been having problems for their whole lives are finally finding a diagnosis, thanks in large part to the internet. (I diagnosed myself thanks to some Google work; my doctor, when I found her, was amused by the story.) I've had a double handful of people come to me about EDS and other forms of hypermobility disorder in the past year or two, and this is sort of an attempt to boil down my standard patter of What I've Learned and get the input of some other EDS sufferers (I know there are a few others who read here) to incorporate into it, for people who have just received their diagnosis and are wondering what the fuck comes next, as this is a situation I find myself in quite often...
Standard disclaimer: I am not a doctor (although I play one on the internets from time to time), and this is not medical advice. Also, EDS and the related disorders are so specific and individual that your presentation may be far different than mine or anyone else's. This is what works for me; what works for you is going to be different.
The first thing I will tell you, because you have likely had the same fights with the medical profession that most of us have had, prior to your diagnosis: You are not making this up. This is not in your head. You are not imagining things. If a doctor tells you to suck it up and deal (as so many of my doctors did, over the years), the doctor is wrong, not you. Fire the doctor and find another one.
(It's sad that I have to say that, but every single person I've talked to has gone through a string of doctors who did tell them it was all in their heads until they found one who didn't suck. Mine used to tell me it was growing pains, or that I was looking for attention, or that I was just lazy. Let's just take the doctor rant as a given here, okay?)
Treatment Plans
Your first step should be the Ehlers-Danlos National Foundation. They are a treasure trove of information, including forums, and your local group will be the first place to go to find practitioners who are familiar with EDS and its management. There's also a huge psychological impact to finding other people who have the same problems -- you are not alone, etc. There's a low-traffic LJ community as well: ehlersdanlos.
Finding a doctor familiar with EDS is your first step. In many cases, you'll get your diagnosis from either a geneticist or a rheumatologist; either way, you need a primary care doctor who's familiar with the disorder, since it tends to be comorbid with a lot of other problems. The first thing your doctor should do -- after working out treatment plans, etc -- is to send you for a full hormone panel workup; something like 80% of EDS patients also present with insulin resistance or low-functioning thyroid, and treating thyroid problems can have seriously beneficial effects on chronic joint pain such as that which accompanies EDS. Check your vitamin levels, too. Everybody presents differently, but there's a really good chance that you're low on something (most common candidates: vitamin D and potassium).
Finding a medication treatment plan is going to take some hit-and-miss, and it'll all depend on your individual doctor's suggestions. Me, I'm currently on tramadol daily for pain management, carisoprodol as a muscle relaxant, and oxycodone and lidoderm patches for breakthrough pain. I have sung the glories of Lidoderm before; if your doctor doesn't know about them, ask for them. (They are pretty much responsible for me being able to get out of bed, most days.)
Bodywork
Physical therapy is going to probably be the first thing your doctor prescribes. Be sure to do the extra work to find a PT who's familiar with EDS; if they don't know what they're doing, they will hurt you and not help you. (This is why I am not currently in PT; I can't find a PT I trust enough.)
I get really good results from chiropractic work. Again, you have to find a chiro who knows EDS (and if you're in the Baltimore/DC area, can I just take a second and plug Dr. Eric Horne in Pasadena, MD, who knows exactly what he's doing with EDS and works miracles). Chiropractic work can really contribute to fixing some of the long-term damage; I, for instance, keep dislocating the vertebrae in my neck and upper shoulders, and the long-term results of said dislocations involve lots of scar tissue building up around the cerebrospinal fluid in my neck, which is likely going to lead to paralysis sooner or later. (This is why regular MRIs are a good idea: to track the progress of the scarring.) Chiropractic work can help re-seat the joints and keep them there, if the chiro knows what s/he's doing, and stave off the inevitable.
Likewise, a good massage therapist works wonders. Again, you need someone who knows or is willing to learn how to work with hypermobility. The American Massage Therapist Association has a practitioner locator; don't hesitate to interview the MT ahead of time, and don't expect miracles, but a long-term relationship with a massage therapist can really help you. (Again, MD locals, allow me to plug Kevin Hatcher at the Catonsville Wellness Center, who is incredible.)
In terms of physical activity, swimming is your best bet: the water will prevent you from doing more damage to things, while at the same time giving you a workout and building muscle strength. Particularly avoid body-contact sports or anything that involves high impact. You want to minimize the chance of dislocating things or stressing your joints. (Weightlifting, for instance, is right out.)
Comorbidity
Which is a fancy word for "other shit that this stupid condition causes". EDS is known as a syndrome and not a disease for the fact that it's a whole bunch of fun things rolled into one. You will slowly become familiar with all of these. (In many cases, it's one of these other things that will lead to your diagnosis; I found EDS because of the Novocaine immunity.)
Not everyone who presents with EDS will have these problems, but there's a pretty good chance you'll have some of them. I've already mentioned the insulin resistance and the underactive thyroid problem. Some other fun side effects:
* Novocaine resistance (and resistance to other forms of anaesthetic). You may have already noticed this over time, when getting dental work done; you're probably used to needing eighty jillion shots and still not getting complete anaesthetic. One thing that will help with this: whenever you need novocaine delivered, ask to have it done with intra-ligamentory injection. (Your dentist might not be trained in this method of delivery; if not, get a different dentist.)
* Painkiller resistance. Over time, you'll build up a resistance to painkillers of all kinds, but particularly the opiate-based derivatives. This is one of the reasons why it's critical to avoid using those drugs for regular pain relief, to avoid building up a tolerance. (You'll need them later.) Many patients report that their regular medication will work for a while and then stop working over time; if you're experiencing a downswing in the efficacy of your usual regimen, it might be time to switch daily-dose drugs for a while. (I have to trade off mine every 9-12 months.)
* Stomach problems. Not necessarily because of the EDS, but because a lot of the drugs cause issues with the stomach and digestive tract. I don't really have any advice here, except to keep your doctor fully informed about what's going on. (There are days when the only thing I can eat are granola bars and brown rice.) Many people report that a low-glycemic-index diet helps them a lot, not only in being able to eat but also in having more energy. (It's connected to the insulin resistance.)
* Polycystic ovarian syndrome. Many female EDS patients find that they also have PCOS. It's annoying. The drugs to fix it are even more annoying. Unfortunately, there's not much in the way of treatment that doesn't have horrible side effects (many of which compound with the previous bullet point). If you do decide to treat the PCOS, I'd stick some extra magazines in the bathroom; you'll be in there a lot.
* Skin problems. EDS is associated with lots of skin problems -- hyper-fragile skin, rapid skin growth, bad scarring, etc, etc. One thing you should be familiar with, and keep around the house, are gel-based vitamin E caplets; whenever you have any cuts or incisions, treat them by puncturing a caplet and squeezing the gel onto the scab, rubbing the gel in very lightly, three times a day for up to two weeks after injury. This will help in reducing the likelihood of forming keloid scars. (You'll likely still scar, but not as badly.) If you have the type of EDS that comes with rapid skin growth, you're likely plagued by ingrown/abcessed hairs a lot in areas you shave regularly; make friends with your tweezers, and pick up a tube of ichthammol salve at your pharmacist's. (You might have trouble finding it; if you do, go into a local pharmacy, not a chain pharmacy, and ask the oldest pharmacist there if they carry "black drawing salve".)
Managing your ergonomics
Part of the fun of having a disorder that results in loose joints is the fun of randomly dislocating things. This can be as showy as the typical traumatic dislocation that everyone thinks of when they hear 'dislocated', or the more mild subluxation of joints that after a while doesn't even need a trip to the ER to have it reduced. (I've gotten very good at fixing things on my own, to the point where people barely even notice; this weekend at the stitch & bitch, I popped out my wrist while rooting in the yarn bins, and I bet half the people there didn't even realize I'd done it.)
There are a lot of things you can do to fix up your ergonomics, most of which is outside the scope of this entry, and all of which is going to be very individual based on your particular trouble joints. The key point in all of this is to arrange your world so that you avoid putting stress on joints that tend to dislocate. If you're lucky, you don't yet know which joints those are going to be. (I hope this continues for a long time.) Common targets, however, are elbows, knees, ankles, wrists, and fingers -- joints that are highly hypermobile in EDS patients and joints that are under a lot of daily stress. (My trouble spots include my hip joints, too, which is why I can't walk properly a lot of the time.)
Many, many EDS patients are diagnosed with carpal tunnel syndrome. Whether you actually have it or not is often even odds, since EDS can present as carpal tunnel a lot of the time, but since wrists are a real problem with EDS, you should still take care of them. The Typing Injury FAQ is not just a FAQ, it's a whole host of information about repetitive stress injury, and you should live it, learn it, love it. One thing in particular to keep in mind; if your wrists are at all susceptable, you should get a pair of good, well-fitting wrist braces (the kind with the metal inset to keep your wrists from moving) and sleep in them, even if you can't wear them while typing. You do more damage to your wrists while you're asleep than you do during a full day.
Compression braces or athletic braces are hit-and-miss for a lot of EDS patients; some people they help, some people they injure worse. Don't feel bad about trial-and-error. We have a whole cabinet full of various braces; some days they help, some days they hurt. If all else fails, a good selection of Ace bandages and the willingness to try different methods of wrapping them until you hit on the one that works will usually suffice. (They're also good at keeping the lidoderm patches on; the damn things have a tendency to peel off at the drop of a hat.)
While you're sitting and working at the keyboard (and you have arranged your work area so that your elbows are at precisely a 90* angle -- no higher, no lower -- and the top of your monitor is exactly at eye level when you're holding your head unbent, right?) you should not only take frequent breaks -- 5 minutes every 30 minutes -- but also remember to move around and shift your weight in the chair, sitting in different ways. This contradicts the usual ergo advice given to non-EDS patients, who are told to sit with their feet flat on the floor in front of them, but most EDS patients find that this results in unpleasant hip dislocation (the longer you stay in one position, the more your joints will try to shift out weirdly). I tend to cycle through feet down, legs crossed, legs crossed in half-lotus position, kneeling seiza in my desk chair, sitting on my feet, then back to feet down. My doctor suggests a yoga ball -- one of those things you sit on and have to constantly adjust your positioning -- to keep you cycling through various positions, but I couldn't adjust to it. If you can, though, it might help. (I also tend to pick up my laptop and lie face-down on the floor for five or ten minutes per hour, or at least until the cat comes over to sleep on my keyboard.)
I've had a lot of luck with support gloves lately, since my wrists have gone all wacky and prone to unseat themselves. They keep me from hyper-extending the wrist without really interfering in regular range of motion. You'll probably have to work out your own methods for reminding yourself not to hyper-extend your joints; whatever works for you. The key is to remind yourself that normal joints don't bend that way, and you shouldn't either. Just because you can hyper-extend your elbows doesn't mean that you should; the more you can keep yourself from hyper-extending your joints, the better results you'll get.
Many products that are designed for people with arthritis will be useful for you. Even if you don't think you'll use them, look over a catalog and get some ideas for how you can make things easier on yourself. The more you work with adaptive equipment, the more you'll lessen the impact on your joints. Remember, your fingers (and your toes) are joints, too, and they are also likely hypermobile. The less you strain things, the better.
Resist the urge to crack things just because you can. It'll feel better for a second after you do it, but it'll go right back to hurting, and the more you do it, the more it'll need to be done. (I totally do not follow this point of advice. I regularly crack everything from my neck to my back to my hip to my wrist all the way down, not just my knuckles, and I have to pop my left second toe back into joint at least three times a week.)
I'm sure I'll think of fifty other points of advice as soon as I post this entry, but this is good enough for now, especially since my knock-you-out drugs are starting to do their knock-you-out thing. I hope that other EDS sufferers will chime in with their practical bits of advice in the comments, to add onto this, and I'll come back and edit in things as I think of them.
In the meantime: Good luck. You are not alone. There are others of us out here. This disorder is annoying, painful, and complicated, but it's not impossible to manage, and you are not weak if you make accomodations.
Hang in there.