Why Ehlers-Danlos Syndrome Is Much More Than Being 'Bendy'
I’ve always had something going “wrong” with my body. Whether it was repeatedly getting injured as a child, being the clumsiest dancer, running on less energy than other kids, ear infections, or tummy aches, I was always experiencing some sort of medical inconvenience. Despite having a laundry list of random symptoms as a child, I was still able to relatively function like the rest of the students my age. Everything changed the second I turned 18.
After starting university with a full-ride scholarship, I started experiencing a dramatic increase in chronic pain, dizziness, fainting, gastrointestinal issues, nausea, depression, severe allergies, and debilitating fatigue. It was as if becoming an adult had unleashed the Kraken of debilitating, declining health problems that would never end, ultimately leading me to drop out of college. At age 20, I was diagnosed with fibromyalgia, chronic fatigue syndrome (also known as myalgic encephalomyelitis or CFS/ME), and irritable bowel syndrome (IBS). For the next seven years, I dealt with doctors unwilling to look beyond those conditions, seven years of no improvements, seven years of continual decline in health, seven years of more and more symptoms added, and seven years of more medical neglect and invalidation endured. By the time I was 23, I wanted to give up, and by the age of 25, I could no longer avoid the progressive nature of my confusing health and needed to fight for answers.
At 27 years old and after over a decade of my health falling apart, I was finally diagnosed with Ehlers-Danlos syndrome, hypermobility type (hEDS) by a geneticist. Because of my hEDS, I experience many complications and comorbidities such as postural orthostatic tachycardia syndrome (POTS), small fiber neuropathy (SFN), GI issues, chronic pain, chronic fatigue, and plenty of other illnesses involving damn near every letter of the alphabet. All these years of not understanding my body, of wondering if all of my random symptoms had anything to do with each other, if this was in my head, etc. were finally answered. Ehlers-Danlos syndrome is the beast I have yet to tame.
Like many zebras before being diagnosed, I knew very little about hEDS. I knew there was more than one type of EDS. I knew that EDS caused soft, stretchy skin and extra bendy joints. I had no idea the number of complications, other parts of the body, and additional symptoms that people with EDS experience. Because EDS is a group of inherited syndromes that are caused by genetic mutations in one’s collagen or collagen-like proteins, many people assume it just affects the skin and joints. That is not only a myth, but a dangerous assumption that can lead to years of neglect and invalidation. Because collagen is found in the vast majority of one’s body such as in our skin, ligaments, bones, organs, blood vessels, gums, and even our eyes, it makes sense that EDS would be a condition that affects one’s entire body.
Although hEDS is the most common type of EDS, that does not mean it is necessarily less serious or less severe. While the vascular type of EDS (vEDS) is the most life-threatening, there are many serious and potentially life-threatening complications that can occur to anyone with any type of EDS. EDS is a spectrum disorder, meaning not only are there over 13 different types, but that the level of severity, combination of symptoms, and lived experiences are going to be different from person to person even if they have the same type. Some people with hEDS are able to walk without mobility aids, are able to work, etc., while others may be unable to get out of bed and struggle with serious and potentially life-altering complications.
I wanted to list some of my daily experiences and symptoms because of how debilitating, dangerous and poorly understood Ehlers-Danlos syndrome can be. We are not just bendy. We are sick. Here is a glimpse of what it’s like living with my genetic disease:
1. I am constantly getting injured.
There is not a single day in which I do not sublux at least one joint. I am also prone to dislocations. These subluxations and dislocations have caused me to have many permanent complications such as bursitis in both hips and arthritis throughout my body. I struggle to use my hands due to my hypermobility, tremors, injuries, and joint damage, and often rely on using voice to text on my phone because of it. I struggle to walk or stand for longer than a few minutes without being in constant pain or risking injury.
2. I am always in pain.
Even if I may not be experiencing pain directly in my bones or specific areas of injury, I am experiencing significant amounts of musculoskeletal pain on a daily basis. Many people with hEDS are often initially diagnosed with fibromyalgia (myself included) because of the prevalence of chronic widespread pain, sensitivity to touch, and pressure points.
3. I have chronic fatigue.
And no, I do not just mean tired from not sleeping well for a few nights. My chronic fatigue causes brain fog, short-term memory issues, cognitive dysfunction, depression, lethargy, limited mobility, poor sleep, and a litany of other manifestations. If I am in pain, I can often push a little bit past it, but when I experience severe fatigue, I cannot function with a brain running on internet as old as AOL dial-up. It’s as if I am being run by a backup generator with limited and selective functioning.
4. I am constantly dizzy.
Because of collagen’s prevalence in our blood vessels, many people with hEDS also have POTS. Because our veins are more stretchy, we are more prone to blood pooling, orthostatic intolerance, and various manifestations of dysautonomia/autonomic dysfunctions, POTS being the most common. Because of this, I am dizzy any time I am not laying down or resting. I am prone to fainting, falling, black spots in my vision, nauseousness, lower blood pressure, tachycardia, vertigo, and even migraine and neck/shoulder pain.
POTS may not be life-threatening, but is by far one of the most debilitating illnesses I experience thanks to my hEDS. I have spent roughly 75% of the last eight years mostly in bed or at home due to the severity of my hEDS and POTS that comes from it. I rely on taking beta-blockers and I have a port surgically implanted in my chest so that I can have IV fluids on almost a daily basis to decrease the severity of my POTS. I also need to use a mobility aid (most often a rollator walker) any time I leave the house due to my chronic pain and debilitating dizziness and fatigue.
5. I am constantly struggling with gastrointestinal complications.
I have IBS that fluctuates dramatically between extreme constipation and extreme diarrhea that is still being investigated by my doctor. I have chronic acid reflux (GERD) that led me to develop a serious complication known as Barrett’s esophagus that causes my esophagus to incorrectly grow intestinal tissue over areas that have been damaged from chronic acid reflux. I will need to get an upper endoscopy every three to five years for the rest of my life to prevent/monitor any potentially cancerous growths. I had a hiatal hernia by age 19. I had my gallbladder removed due to gallstones by age 21. I also currently have a protein deficiency due to malabsorption. I have to take medications daily for the rest of my life and routine maintenance testing to look out for any additional damage over time.
6. I have nerve damage. A lot.
Because of my constant injuries and general joint instability, I have small fiber peripheral neuropathy that has progressed so much that it has already spread to my hips and made my nerve fiber density in my lower legs and feet almost obsolete. I struggle with nerve pain down my spine and numbness and tingling down my entire left arm frequently due to shoulder and neck instability, subluxations, and dislocations.
7. I have horrible headache attacks on a nearly daily basis.
Migraine is overwhelmingly common amongst those with hEDS for many different reasons such as CSF leaks, craniocervical instability, chronic pain and tension, fatigue, etc. I have to get injections on my forehead, scalp, neck, and shoulders every three months, and it does not solve the problem completely.
8. I am allergic to what seems like almost everything.
I am allergic to the vast majority of plants, molds, fungi, dust, etc. native to the area I live in. I am allergic to many perfumes, chemicals, dyes, adhesives, etc. I am allergic to most foods that I eat, and my symptoms abruptly change in type and severity and to what trigger. I am prone to chronic hives, itchiness, congestion, sinus infections, sore throats, diarrhea, nauseousness, and even sometimes swelling, and my reactions are always unpredictable.
9. I heal poorly and slowly.
On top of my hEDS, I have an auto-inflammatory skin condition called hidradenitis suppurativa which causes acne, cysts, and tunnels on areas of my skin close to my sweat glands that are inflamed. Because of my hEDS, my acne and cysts in those areas of my body (such as under my breasts, my inner thigh, buttocks, etc.) heal poorly, leading to atrophic scarring, open wounds exposed for too long, causing me to be a chronic staph carrier that requires me to take antibiotics long term to prevent serious infections such as MRSA. I have atrophic scarring, unexplained stretch marks, slower healing time after surgeries, and weaker, softer scar tissue.
10. I have many reproductive issues.
I struggle with pelvic floor pain, heavy periods, painful cramps, nausea, diarrhea, etc. when menstruating. I’ve had IUDs for the last seven years because I cannot handle the severity of the heaviness and pain from menstruating. I also struggle with bladder issues ranging from phases of incontinence to even bladder retention.
While there are many other symptoms and complications I have from my hEDS such as neck instability, rapid hearing loss, TMJ, etc., these are some of the main ones I deal with on a daily basis. Because of the progressive nature of EDS and its complications, my experience at age 27 will not be the same 10 years from now, and I will have more and more issues over time.
EDS is more than just hypermobility and stretchy skin. Many people with EDS require mobility aids to move around, lifelong medication management, regular physical therapy, and sometimes surgical interventions. We can be stuck at home, in bed, or even hospitalized long-term. Some of us even have feeding tubes due to severe gastrointestinal complications. EDS is not a one-size-fits-all illness. There are so many symptoms, complications, comorbidities, and levels of severity making no case the same.
We cannot let EDS be continuously misunderstood, poorly researched, invalidated, underdiagnosed, or neglected. Whether someone experiences mild or life-threatening EDS, we all deserve to have our stories be validated and treated thoroughly and accurately. We are more than just your flexible party tricks. We have a debilitating, complicated disease that can lead to a low quality of life that we deserve to have improved and cared for. We are zebras who deserve to be heard and seen in a stampede of horses.
Getty image by Miltiadis Louizidis.