I have Ehler's Danlos Syndrome, a degenerative genetic collagen defect disorder that is degenerative. Many people who have it go undiagnosed their whole lives, and many people only after many years of trying to figure out what is wrong with them and their body and often being told they're making a big deal out of nothing, it's all in their head, or all their fault. There isn't a cure, though there are ways to manage symptoms and live a more productive life with fewer negative symptoms. Some people might wonder what the benefit is of actually knowing you have Ehlers Danlos Syndrome. For me, it is to be able to figure out why I'm having specific issues and how to deal with them, instead of basically flailing around directionless and unable to get help.
Ehlers Danlos Syndrome is caused by a problematic gene, the COL gene, which directs the body how to make collagen, a soft tissue that is found in practically every part of your body. People associate collagen with joints and skin, but that is by far not the only thing affected by this defect. Taking collagen supplements doesn't help, because it isn't a lack of collagen in the body, it is just bad collagen.
In honor of International Ehlers Danlos Syndrome Awareness Month, I already talked here about what happened when I went to visit the Ehlers Danlos Syndrome specialist. As a follow up, I wanted to talk about the different ways that knowing that I have Ehlers Danlos Syndrome helped me put together the puzzle of my life. How different seemingly unrelated issues all had the root in the same thing, Ehlers Danlos Syndrome. Until I learned more, I never in my wildest dreams would have connected these things. And once I knew what the cause was, I was able to find ways to deal with those issues.
Some of what I will be writing about I've covered in my previous on my life with Ehlers Danlos Syndrome, but I've since learned so much more and have a lot more to share on the topic. I wanted to also warn you that there will be quite a few things that fall under the TMI category, so if you don't want to read details about certain parts of the female anatomy, you might want to skip this post (or at least the starred sections).
Yes, it gets personal, but the reason I'm sharing these is to hopefully help other people who may be suffering similarly and not know the reason why or what to do.
I was thinking about dividing this up by body part, but I realized that would be too complicated because there is so much overlap, but I'm trying to divide this up by systems.
Joint Hypermobility, Dislocations, Subluxations, and Sprains
Some of what I will be writing about I've covered in my previous on my life with Ehlers Danlos Syndrome, but I've since learned so much more and have a lot more to share on the topic. I wanted to also warn you that there will be quite a few things that fall under the TMI category, so if you don't want to read details about certain parts of the female anatomy, you might want to skip this post (or at least the starred sections).
Yes, it gets personal, but the reason I'm sharing these is to hopefully help other people who may be suffering similarly and not know the reason why or what to do.
I was thinking about dividing this up by body part, but I realized that would be too complicated because there is so much overlap, but I'm trying to divide this up by systems.
Joint Hypermobility, Dislocations, Subluxations, and Sprains
When someone thinks of Ehlers Danlos Syndrome, hypermobile joints are what generally comes to mind, if you're at least somewhat educated on the topic. In fact, most people diagnosed with Ehlers Danlos Syndrome are diagnosed with Hypermobile Ehlers Danlos Syndrome (tentatively) until they get genetic testing to see if they, in fact, have a different type.
This hypermobility means you are likely to frequently dislocate or sublux (partially dislocate, or dislocate and then it pops back into place) your joints, or joints just give way, which can cause you to get frequent sprains (caused by stretched or torn ligaments that attach muscles to joints), are made even more likely by the fact that your ligaments are also made of collagen. When joints are dislocated or subluxed, or the ligaments torn or stretched, the muscles around them can spasm really badly. This spasming, usually caused by subluxed joints in my case, by far is my most frequent symptom and the one that costs me the most money.
From when I was a child, I sprained my ankles probably at least 100 times in my life, often from doing nothing, and sprained my wrist many times as well.
Starting when I was pregnant with my third child, but worsening after an ice skating fall in 2019, my pelvis subluxes regularly (specifically the sacroiliac joint). Anything can cause it to sublux. (The last time it happened was pushing a wagon of groceries.) When this happens my entire butt is in spasm as is my lower and mid back. Putting the joint back into place on its own isn't enough, because if the spasm isn't dealt with, the spasmed muscle will pull the joint back out of place, so I need really painful manual physical therapy to release the spasmed muscles so my body can return back to normal.
This issue with my pelvis and my glutes makes it painful for me to sit for too long; when I sit I need to constantly moving and shifting positions, because staying in one place makes me in a lot of pain. When my pelvis is out of place my hips also end up hurting, as do my knees. On the reverse end of things, when I sprain my ankle, the change in my walk pushes my pelvis out of place and causes my butt and lower back to hurt.
In addition to my pelvis doing this, my spine also does funny things and subluxes, getting incorrect spacing between my vertebrae (the sacroiliac joint being out of place doesn't help this) which causes the rest of my back muscles to spasm as well. On top of that, the muscles between my ribs, the intracostal muscles, also really hurt. Touching most of my torso on my back and side is typically really painful because there is always some muscle or many in spasm.
When my spine is acting particularly egregiously, my nerves in my arm can get really painful and achy, either throbbing along the nerve itself or the skin on my arm feels like it's burning. I went to neurologists many times over the years and no one had any solutions. However, my physical therapist can get that pain to stop when she works on my spine.
I usually have one physical therapy appointment a week, but for the past 3 weeks I've needed 2 or more appointments a week, and this gets really expensive since I pay for this out of pocket since all the physical therapists I've tried through my health insurance injured me more. However I cannot function without physical therapy.
I usually have one physical therapy appointment a week, but for the past 3 weeks I've needed 2 or more appointments a week, and this gets really expensive since I pay for this out of pocket since all the physical therapists I've tried through my health insurance injured me more. However I cannot function without physical therapy.
Sometimes I'm in a lot of pain but I can't figure out why, but my physical therapist has explained to me that with a body as complicated as mine, a cough, a stomach bug, or even stress can cause the muscles to spasm, even without a subluxation.
I've been diagnosed with costochondritis in the past, an inflammation of the cartilage connecting your ribs to your sternum, which makes you feel like you're having heart pain and causes pain breathing. This is also another type of joint issue. Fortunately, I haven't had it recently, since I started going to weekly physical therapy, so I assume that that is why.
**TMI Women's Issues and Women's Body Parts** (this is the section you most likely want to skip if you're squeamish, and I'd prefer men readers skip it even if you aren't)
For the last 2 and a half years or so I've been having peeing issues. Peeing issues are actually quite common among people with Ehlers Danlos Syndrome. (Guess what? Your bladder is made of collagen.) More common issues with Ehlers Danlos Syndrome are incontinence, but my issue is the exact opposite. So often I feel the need to pee, but I sit on the toilet and just can't pee unless I really, really, really have to pee badly. Or I have to wait a good minute or two to be able to pee. This is called voiding dysfunction.
When this issue first started, I went the obvious route and ruled out urinary tract infections. Then I went to a gynecologist to rule out prolapses. I've had small prolapses in the past (they are quite common with EDS, and in fact are one of the criteria for an HEDS diagnosis) and they can put pressure on the urethra and cause peeing issues, but that wasn't the issue here. I went to a urologist who sent me for a bladder ultrasound, but nothing in particular was visible there. Then I went to a urogynecologist who sent me for a urodynamic test, which didn't find anything wrong. By this time I was entirely frustrated having done all these tests and no progress or solution...
For the last 2 and a half years or so I've been having peeing issues. Peeing issues are actually quite common among people with Ehlers Danlos Syndrome. (Guess what? Your bladder is made of collagen.) More common issues with Ehlers Danlos Syndrome are incontinence, but my issue is the exact opposite. So often I feel the need to pee, but I sit on the toilet and just can't pee unless I really, really, really have to pee badly. Or I have to wait a good minute or two to be able to pee. This is called voiding dysfunction.
When this issue first started, I went the obvious route and ruled out urinary tract infections. Then I went to a gynecologist to rule out prolapses. I've had small prolapses in the past (they are quite common with EDS, and in fact are one of the criteria for an HEDS diagnosis) and they can put pressure on the urethra and cause peeing issues, but that wasn't the issue here. I went to a urologist who sent me for a bladder ultrasound, but nothing in particular was visible there. Then I went to a urogynecologist who sent me for a urodynamic test, which didn't find anything wrong. By this time I was entirely frustrated having done all these tests and no progress or solution...
Another issue I was having was frequently having a really itchy vulvar area, and if I scratched it I'd start bleeding, and even if I'd just rub it to try to get some relief I'd often get cut too- moving my skin too quickly, especially if anything got caught on a hair could cause my skin to split in half. I always assumed it was a yeast infection and took many rounds of meds to try to combat it, but it never really helped. Then it got worse and worse to the point of being intolerable and spread down to my underwear line. I'd have a hard time being in public because it would be embarrassing to rub my crotch but to not was hellacious. I booked an appointment with a female skin doctor who came highly recommended, having a really hard time waiting a few weeks until she had an available appointment, and she said that the issue was dry skin, and gave me moisturizer and a medicated feminine wash. All I can say is that it did not help.
I asked on EDS Facebook forums if they had any idea what it could be, assuming that maybe there was a connection, and they said I should look into lichen sclerosis, common among people with EDS, which can cause itchiness and bleeding. But the gynecologist I went to, to check for a prolapse because of my peeing issue, said that I didn't have lichen sclerosus.
Finally, after so much suffering, I decided to bite the bullet and pay privately for a urogynecologist who specifically specializes in vulvovaginal health, who I'd heard about both from my PT (who actually specializes in pelvic floor PT, even if she's terrific with EDS) as well as the EDS specialist. She cost a lot of money, but by this point, I was really suffering, and at least I'd get back $200 of the $575 her visit costs from my insurance.
This urogynecologist checked me out, using a microscope to swab me, and told me that it wasn't an issue of recurrent yeast infections, I was completely free of that. She told me that had I not told her I had EDS, she would have sent me to the EDS specialist as she could tell just from looking down there that I had EDS. She also repeated what the gynecologist had said, that I didn't have lichen sclerosis.
Finally, after so much suffering, I decided to bite the bullet and pay privately for a urogynecologist who specifically specializes in vulvovaginal health, who I'd heard about both from my PT (who actually specializes in pelvic floor PT, even if she's terrific with EDS) as well as the EDS specialist. She cost a lot of money, but by this point, I was really suffering, and at least I'd get back $200 of the $575 her visit costs from my insurance.
This urogynecologist checked me out, using a microscope to swab me, and told me that it wasn't an issue of recurrent yeast infections, I was completely free of that. She told me that had I not told her I had EDS, she would have sent me to the EDS specialist as she could tell just from looking down there that I had EDS. She also repeated what the gynecologist had said, that I didn't have lichen sclerosis.
The doctor confirmed that the incredible itchiness and discomfort I was having was because of EDS, because it gives us fragile sensitive skin that is easily irritated. She told me that it was a cycle of irritation which made me rub it which made it more irritated etc... and it was non stop. She said because of how sensitive my skin is, anything is enough to irritate it, even a tiny bit of sweat. She gave me a steroid cream to stop the itchiness but told me to only use it for a short duration, because steroids make skin more fragile and even more likely to rip. Fortunately, only one use of the steroids was enough to stop the cycle. And another time that it started up, one dosage again stopped it in its tract. These days, I just use what I can to keep the skin as healthy and clean as possible, using a medicated pH lowering intimate wash that the dermatologist I went to recommended, and I've fortunately been in the clear and don't suffer anymore.
While I was at the doctor's appointment, I asked about my peeing issue and she said something that both relieved me and frustrated me because I finally found answers but the solution is far from easy. There wasn't "another thing wrong". The problem was that the muscles in my pelvic floor were in spasm from my sacroiliac joint being out of place, and because they were in spasm, they had a hard time relaxing enough to let me pee. The treatment for it was strengthening the weak pelvic floor muscles so they could hold my joints in place so that the muscles would stop spasming and my body would let me pee. I have noticed since then that this peeing issue gets worse when my pelvis is out of place, and at least a physical therapy appointment is able to bring me relief, just like it does when the nerves in my arm are acting up.
Skin Issues (you can start reading this again if you skipped the previous section)
Skin Issues (you can start reading this again if you skipped the previous section)
We EDS people have stretchy skin, but that isn't a bad thing specifically. In fact, many people with EDS tend to look younger, since we tend not to get wrinkles until a much older age. The issue with EDS and skin is its fragility. (I know, that's weird. How can something both be extra stretchy and extra rippable? Seems like an oxymoron. But it isn't.)
All those lotions they market to pregnant women to prevent stretch marks? Ha. It's down to genetics. EDS makes you very prone to stretch marks. I didn't just have a few when pregnant, my entire stomach was covered with them, top to bottom. My mother also was the same. I first got stretch marks when I was a teenager, suddenly on a walk. Stretch marks are just partially ripped skin, which we fragile-skinned people get easily.
2 years ago I walked next to a brand new plastic wrapped barbecue grill, and just touching the corner was enough to rip a long and deep gash into my calf (it was gross, so only look if you don't mind) which ended up needing quite a few stitches. I'm still amazed at how easily it ripped when I barely remember touching the grill. And now, 2 years later, I have a really ridiculous looking scar on my leg, what is called cigarette paper scarring, typical with EDS, and one of the potential diagnostic criteria for EDS.
All those lotions they market to pregnant women to prevent stretch marks? Ha. It's down to genetics. EDS makes you very prone to stretch marks. I didn't just have a few when pregnant, my entire stomach was covered with them, top to bottom. My mother also was the same. I first got stretch marks when I was a teenager, suddenly on a walk. Stretch marks are just partially ripped skin, which we fragile-skinned people get easily.
2 years ago I walked next to a brand new plastic wrapped barbecue grill, and just touching the corner was enough to rip a long and deep gash into my calf (it was gross, so only look if you don't mind) which ended up needing quite a few stitches. I'm still amazed at how easily it ripped when I barely remember touching the grill. And now, 2 years later, I have a really ridiculous looking scar on my leg, what is called cigarette paper scarring, typical with EDS, and one of the potential diagnostic criteria for EDS.
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| I think the scar looks like a lizard. My daughter thinks it looks like a centipede. |
Another time I slipped on water on my floor and hit my head hard, and in addition to getting a nice concussion, just hitting my head against the (obviously not sharp) floor made my skin split open and I needed about 6 stitches.
Interestingly, I never got stitches my whole life until a few years ago other than after my hernia surgery at 5 year old, but apparently, the skin issues get worse as you age, and therefore it makes sense that my skin issues have become more frequent.
Interestingly, I never got stitches my whole life until a few years ago other than after my hernia surgery at 5 year old, but apparently, the skin issues get worse as you age, and therefore it makes sense that my skin issues have become more frequent.
Blood Vessel Issues
Guess what the walls of your arteries and veins are made of? If you guessed collagen, you're right! The same way faulty collagen makes you have stretchy and easily rippable skin, joints, and ligaments, it also does that to the walls of your blood vessels!
One of the most dangerous types of EDS is vascular EDS, which fortunately is rare, but from its name you can tell that it causes issues with blood vessels, among other things, from early varicose veins to ruptured blood vessels and organs, which can cause early death. Unfortunately, the typical lifespan of someone with vEDS is 48.
I would assume that the varicose veins I got during pregnancy and still have now (fortunately not too bad) have to do with these stretchy blood vessels, but who knows. There isn't a definitive connection.
Even among people without vEDS, there are blood vessel connections. 80% of people with EDS, especially those with hypermobile EDS, have POTS or other similar orthostatic intolerance. POTS stands for postural orthostatic tachycardia syndrome, which means that when you change posture (lying down to standing or sitting, etc...) you get tachycardia, a racing heart, and other orthostatic intolerances can make your blood pressure drop when you stand. This causes dizziness and lightheadedness especially when you change positions.
The connection between this and EDS is that your veins are stretchy... and if your veins are stretchy they can be sluggish at pumping your blood when it's going against gravity, so your heart goes into overdrive to try to get the blood moving.
The typical treatment for this, therefore, is to drink more, and have a lot of salt. We tend to have low blood pressure to begin with and it gets even lower when we stand, so salt is not only not unhealthy, but necessary, as it causes you to retain liquid.
One of the most dangerous types of EDS is vascular EDS, which fortunately is rare, but from its name you can tell that it causes issues with blood vessels, among other things, from early varicose veins to ruptured blood vessels and organs, which can cause early death. Unfortunately, the typical lifespan of someone with vEDS is 48.
I would assume that the varicose veins I got during pregnancy and still have now (fortunately not too bad) have to do with these stretchy blood vessels, but who knows. There isn't a definitive connection.
Even among people without vEDS, there are blood vessel connections. 80% of people with EDS, especially those with hypermobile EDS, have POTS or other similar orthostatic intolerance. POTS stands for postural orthostatic tachycardia syndrome, which means that when you change posture (lying down to standing or sitting, etc...) you get tachycardia, a racing heart, and other orthostatic intolerances can make your blood pressure drop when you stand. This causes dizziness and lightheadedness especially when you change positions.
The connection between this and EDS is that your veins are stretchy... and if your veins are stretchy they can be sluggish at pumping your blood when it's going against gravity, so your heart goes into overdrive to try to get the blood moving.
The typical treatment for this, therefore, is to drink more, and have a lot of salt. We tend to have low blood pressure to begin with and it gets even lower when we stand, so salt is not only not unhealthy, but necessary, as it causes you to retain liquid.
If you imagine your blood vessels as one of those long balloons, partially filled with water... but we want to fill the blood vessels with as much blood as possible, like a very full water balloon. When it is more full, your blood vessels are able to pump more easily and keep the blood pressure proper, and then the heart doesn't need to race to get oxygen where it is needed. This is why I make sure to drink a lot of my salted sports drink every day make sure to drink a lot of my salted sports drink every day, and I just ordered some salt pills from Iherb on top of that. I also give myself a break after lying down before getting up- after my physical therapy appointment that I spend lying down, I usually will give myself a good 10 minutes sitting or more before I go to the car to drive, so I no longer am dizzy.
When I was younger, I thought I was hypoglycemic. It turns out that my issue was actually POTS, and they have similar symptoms. However, with POTS, often the body also has difficulty with blood sugar regulation, so you're told to eat small meals multiple times a day to help with that. As I mentioned in my previous post on the subject, I have a really hard time remembering to eat 3 times a day, let alone 5 or 6, but I think that it's possible that the fact that I'm drinking my sports drink throughout the day, with small amounts of sugar all day long, my body isn't having blood sugar spikes and lows... but that's just my theory.
When I was younger, I thought I was hypoglycemic. It turns out that my issue was actually POTS, and they have similar symptoms. However, with POTS, often the body also has difficulty with blood sugar regulation, so you're told to eat small meals multiple times a day to help with that. As I mentioned in my previous post on the subject, I have a really hard time remembering to eat 3 times a day, let alone 5 or 6, but I think that it's possible that the fact that I'm drinking my sports drink throughout the day, with small amounts of sugar all day long, my body isn't having blood sugar spikes and lows... but that's just my theory.
One of the common effects of POTS is chronic fatigue. I don't think they know exactly what causes this, but there are a few potential causes. Number one is that this blood flow dysregulation puts a strain on the cardiovascular system which makes you more tired. The body's response to POTS mimics mild fight or flight which causes the body to release adrenaline which can give a short burst of energy but leads to exhaustion over time. POTS often involves dysfunction in your autonomic nervous system which leads to an imbalance in energy expenditure and cause sleep quality to worsen. Once I understood this connection, my chronic exhaustion made more sense, and my drinking more sports drink does help somewhat with the exhaustion.
At this point this post is already over 3000 words, and I have only covered 4 sections (though it is 4 of the longer ones) and I have a bunch more... so I think there will have to be a part 2 for the rest of this.
At this point this post is already over 3000 words, and I have only covered 4 sections (though it is 4 of the longer ones) and I have a bunch more... so I think there will have to be a part 2 for the rest of this.
But a quick recap for now.
Knowing I have Ehlers Danlos Syndrome pointed me in the right direction to get help for these many issues. I never would have connected any of these things to each other had I not been lucky enough to have been informed by friends about Ehlers Danlos Syndrome, and I would have kept suffering, not knowing where to turn to, to get help. So I want to say thank you to everyone in my Ehlers Danlos Syndrome circles, for helping me learn all this, so I could be on top of these issues and have a better life.
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