Ehlers-Danlos Syndrome, or EDS, is a rare inherited connective tissue disorder. It’s a multi-system disorder caused by defective collagen. I have Type Three, or Hypermobility Type (there are six types). According to the Ehlers-Danlos Society, all types are characterized by “joint laxity, soft skin, easy bruising, and some systemic manifestations. Each type is thought to involve a unique defect in connective tissue, although not all of the genes responsible for causing EDS have been found.” Basically, collagen keeps skin, organs and joints firm, supported and in the right place. Because mine is abnormal, things are stretchy, which causes all sorts of problems, including debilitating chronic pain and fatigue, joint hypermobility or “double jointedness,” dysautonomia (autonomic nervous system dysfunction), gastrointestinal problems including dysmotility, skin problems, and many other symptoms.
According to Dr. Forest Tennant, a leading pain specialist, “At this point in time, I put EDS in the category of being in the top three or four most severe pain problems. A lot of people for example think that cancer pain is the worst pain, but let me assure you that many EDS patients have pain far beyond any cancer patient I’ve ever seen. And so it’s one of the pain problems that is severe, has been very troublesome, many physicians are afraid of the disease and of the kind of pain that EDS patients have.” Dr. Forest Tennant presents “Managing Intractable Pain in Ehlers-Danlos” Published on April 8, 2015
So how has EDS affected my life?
- Problems Started at Birth – When I was a baby, I was sick a lot. I had to wear special shoes to correct the way my feet turned in. My GI problems began in infancy as well – alternating diarrhea and constipation, and GI pain.
- Unexplained Pain – As a kid, I had frequent pain in my legs and sometimes arms that kept me up at night crying. My mom or dad would have to rub my legs and sing to get me to sleep. My doctors dismissed this as “growing pains,” but I still have them to this day.
- Frequent Injuries with Little Provocation – I got hurt a lot – jamming and spraining fingers and toes, other random injuries where I would go get an x-ray but they would never find anything, so I stopped getting x-rays. My parents teased me gently and called me “Ace” because I frequently used Ace bandages when my joints hurt.
- Hypermobile Joints – I used to freak my friends out by showing them my super flexible joints. When I was a kid I asked my doctor about my “double” joints, specifically my thumbs, elbows and knees, and was told it wasn’t unusual and was no cause for alarm. I’ve also always been really good at gymnastics and yoga, but managed to hurt myself a lot.
- Knee Surgery at a Young Age – I had to have knee surgery at age 13 to repair a torn meniscus in my right knee. While recovering, I developed tendinitis and problems in my other knee because I was relying on it so much, and in both hips. I didn’t feel I fully recovered for a few years.
- Mouth and Jaw Problems – When I was a teenager, I had to have periodontal (gum) surgery because my gums were growing down over my teeth. I’ve also had TMJ since I can remember, and started getting cavities in my 20s despite excellent dental hygiene.
- Dysautonomia – Beginning when I was a teenager I had dysautonomia symptoms (although we didn’t know that’s what they were) – trouble regulating body temperature, extreme thirst, near syncope, orthostatic intolerance, low blood pressure – that were written off as medication side effects or just “we don’t know.” I was tested for diabetes and thyroid problems several times. I was frequently sick – I had pneumonia, frequent bronchitis, viruses and infections.
- Allergy-Like Symptoms and Respiratory Issues – I always assumed I had allergies – this turned out to be Mast Cell Activation Syndrome, a condition that is sometimes comorbid with EDS.
- Fractures and Injuries in Adulthood – In 2010 I fractured my elbow and fractured and sprained my wrist in a bike accident. When I was 25, I started running cross country. Almost immediately I sprained my ankle badly. After a little over a year of running I managed to injure my IT band and left knee – my kneecap wasn’t tracking right and there was evidence of damage to my meniscus. After months of physical therapy I was able to walk and hike normally but was never able to run more than half a mile again.
- Gastrointestinal Problems – I have had GI problems my whole life – GI pain, alternating constipation and diarrhea, cramps, gas, nausea, frequent “stomach flu,”and “attacks” of intense pain and explosive diarrhea where I would be in the bathroom for hours and almost faint from the pain. In 2012, for some reason, my GI symptoms went into overdrive, I became so sick I was unable to eat at all, and was diagnosed with gastroparesis. Two years later I had to have a feeding tube surgically implanted because I had lost 30 pounds and was frequently admitted to the hospital with severe malnutrition. I will probably have my tube for the rest of my life as I am still unable to eat more than a few mouthfuls of only a couple different foods.
- All Symptoms Magnified in 2012 – Up until my GI system went crazy, I was managing. I had a career. But suddenly every symptom I had been dealing with seemed to be magnified 100 fold, and I became completely debilitated. Even after my feeding tube corrected the malnutrition and helped me gain most of the weight back, I remained in chronic pain and with severe autonomic dysfunction
So, as you can see, EDS has been with me my entire life. Each symptom was treated as it came up, and usually dismissed, and even if I got tested it was usually negative, so the parts were never added to the whole. As I got older I felt like there was something wrong but it was never severe enough to pursue except when I was injured.
The other factor is that my mother has almost the same symptoms, so I grew up thinking that the things I was experiencing were normal. When I had constipation, nausea and attacks of cramps and diarrhea, I didn’t think much of it because my mom was told in her 20s that she has IBS, so I thought that’s what I had. I figured I had allergies because she did. I thought extreme menstrual pain was normal because she and my aunt had suffered with it. My mom always saw stars when she stood up too fast, so it didn’t seem unusual. Of course, now we know that she has EDS and so do I, so we’re actually both VERY unusual.
My mom is a very active, positive person, and an incredibly strong woman (she likes to tell me that we’re descended from a line a strong, independent women – my grandmother was a Navy WAVE in WWII) so she’s my model in how to deal with all of this, which might be another reason why I tried no to allow my health challenges interfere with my life – up until the point when it grew beyond my control. I’ve been doing my best for the past few years to regain control, and I’m hopeful that although there’s no cure for my condition, I will be able to improve my quality of life.