Zebras Unite!

Zebras? Why are we talking about zebras? I thought this was about your weird health condition, Jae?

I promise it will make sense by the end, keep reading!

This past March I was finally diagnosed with Hypermobile Ehlers-Danlos Syndrome (hEDS, also known as EDS Type III). At once I felt validated but also angry, which then turned into a inner hollowness that was hard to shake. Why had it taken more than two decades to get me to this point? Years of pain and suffering to be told, at last, that something was actually wrong with me, despite all my normal lab tests and “can-do” attitude. My entire life, I have been treated like I’m crazy and lazy by people who were supposed to help me; I’ve been gaslit, dismissed, and condescended to. While misogyny definitely played its role in my missed diagnosis (and misdiagnoses), EDS is not well known by most physicians, despite its growing prevalence. At last I found the right professionals who took me seriously enough to look outside the box.

So what is EDS anyway?

Ehlers-Danlos Syndrome is actually a spectrum disorder, with 13 different subtypes. I happen to have the most common type, Hypermobile EDS or EDS Type III. According to the EDS Society (at ehlers-danlos.com), all of these subtypes are classified as connective tissue disorders “that can be inherited and are varied both in how they affect the body and in their genetic causes. They are generally characterized by joint hypermobility (joints that stretch further than normal), skin hyperextensibility (skin that can be stretched further than normal), and tissue fragility. . . Accurate estimates for the occurrence of hEDS are lacking, but a minimum of 1 in 5,000 people are thought to have EDS, 80–90% of which are cases of hEDS.” And yet, hEDS is the only subtype to not yet have its genetic marker (gene mutation) discovered.

“The best way to describe hEDS is as an autosomal dominant disorder influenced by age and gender with symptoms more common in females.”

– Ehlers-Danlos Society

In Claire Smith’s publication, “Understanding Hypermobile Ehlers-Danlos Syndrome and Hypermobility Spectrum Disorder,” she defines hEDS:

“by the association of generalized joint hypermobility, joint instability complications, widespread musculoskeletal pain, (minor) skin features and/or pelvic/rectal/uterine dysfunction. . .”

She continues to list other signs and symptoms:

• chronic fatigue
• acute and/or chronic pain, which is often widespread, with possibility for neuropathic pain
• poor proprioception (your inherent understanding of where one’s body parts are in space)
• reduced response to local anesthetic
• functional gastrointestinal disorders, often associated with IBS or sluggish bowel
• bladder over-activity and pelvic floor weakness
• asthma-like symptoms
• cardiovascular autonomic dysfunction
• anxiety, phobic states, and depression
• reduced mobility
• earlier than ‘normal’ osteoarthritis
• tethered cord and chiari malformation (brain tissue issues)
• Mast Cell Activation Syndrome
• sprains, subluxations, dislocations, tendon tears, disc prolapse, and skin that can stretch, scar, or bruise more easily are all more prevalent in EDS patients

This is a very truncated list, and it is not exhaustive either. More and more symptoms are being associated with this condition as people have been taking it more seriously in research, and as community members come together to relate to each other.

Essentially, my body does not produce collagen properly, which adversely affects my joints. My muscles are overworked from trying to hold all the pieces together. I haven’t quite researched enough to understand my comorbid conditions yet, but they’re often the source of most of my pain, discomfort, and disability. I experience different forms of pain and fatigue everyday of my life, usually without rhyme or reason to it. If I don’t exercise enough, I suffer. If I exercise too much, I suffer. I never get enough rest. I rarely feel hydrated. I can be fine eating an item one day, and the next it will make me sick. No position that I’m in—sitting, standing, lying down—is ever comfortable and usually brings me different forms of pain depending on the day.

This disease affects every aspect of my life, and has effectively rendered me disabled in one way or another (this will be its own post soon).

Now let’s take a quick look at my hEDS:

• “growing pains” in my legs that didn’t stop when I was 10/11, which forced me to quit dance and contact sports, as well as landed me in a wheelchair for a few months at the end of 6th grade (have since subsided into only periodic episodes)
• widespread musculoskeletal pain (considered to be fibromyalgia); acute and chronic depending on the day; myofascial related
• neuropathic pain (pins and needles, tingling, numbness, electric “zaps” especially through to my hands)
• episodes of muscle weakness, especially in upper body
• chronic fatigue (often debilitating)
• super soft skin when I was a child; at 27 my skin is still soft, but incredibly more stretchy and just hangs on my features, especially on my face; easy bruisability/fragility
• stretch marks without weight gain
• hypermobility in my shoulders and hips; complications with wrists, elbows, knees, and ankles
• Snapping Hip Syndrome
• poor proprioception (I have always been clumsy, run myself into doorframes and other things, constantly spill drinks on myself, the list goes on…)
• pelvic flooring weakness (I have been mildly incontinent since childhood); pain
• hyperflexible, though I’m not a contortionist
• spina bifida (small hole in my spine)
• cervical spine instability
• flat feet
• sleep disturbance (complicated by neurodivergence)
• Irritable Bowel Syndrome; constipation/diarrhea
• acid reflux
• frequent bouts of nausea
• depression/mood issues/anxiety (complicated by neurodivergence)
• astigmatism, convergence insufficiency, and frequently dry eyes
• painful menstruation cycle (pretty sure I have endometriosis but have not been diagnosed yet)
• painful intercourse (not all the time, but enough to notice)
• dysautonomia (poor “fight or flight” and “rest and digest” function)
• poor temperature regulation
• electrolyte abnormalities/dehydration (I literally have to take salt capsules; so far, not that effective)
• headaches/migraines (complicated by neurodivergence)
• complications with my TMJ (jaw joint and muscle): locking, popping, subluxation, pain, teeth grinding, temple headaches; I also clench my jaw like a fiend, but is not strictly hEDS related
• fragile gums
• swallowing difficulties
• episodes of changes in speech
• episodes of difficulty walking
• brain fog (complicated by neurodivergence)
• impaired immune response (until the last 2 years, I have gotten significantly ill with colds and flus for 2 months of the year on average)
• MUSCLE SPASMS EVERYWHERE; ranging from benign and annoying to absolutely debilitating/painful
• Mast Cell Activation Syndrome; incessant post-nasal drip, itchy throat and inner ear, hives, flushing

All this, in addition to athletically induced asthma and hypothyroidism.

Overwhelming much? Tell me about it.

It’s genuinely hard to keep track of all the crap my body and I have gone through over the years. I’m almost positive I’ve missed listing at least two things above, but memory is affected by this too (on top of the effects from trauma and neurodivergence). 🥴

At last! Zebras!

“In medicine, the term ‘zebra’ is used in reference to a rare disease or condition. Doctors are taught to assume that the simplest explanation is usually correct to avoid patients being misdiagnosed with rare illnesses. . .”

– ehlers-danlos.org

In the 1940s, medical researcher and professor Theodore Woodward coined the term “zebra” with the phrase, “When you hear hoofbeats behind you, don’t expect to see a zebra.”

But too often, doctors forget that “zebras” do actually exist. The zebra is the symbol of the EDS community because EDS is considered a rare condition. My symptoms have confused and confounded doctors, family, and myself since early childhood. Then EDS finds its way to me, and suddenly everything makes sense. ALL of my symptoms are related.

Even though there is no cure for this disease, just knowing how I am medically valid in my suffering has helped SO much for coping. Now I have new resources, community, and a curated medical team to rely on in order to figure out how best to manage my symptoms and still have good quality of life.

I am particularly grateful for my fellow zebras. For the first time in my life I feel fully seen and understood when it comes to my pain, daily struggles, and future fears. No two zebras have identical stripes, and no two EDS affected folk are the same. But when we come together online, seeing those shared similarities, being able to commiserate, and learn from each other has drastically improved my relationship with my condition, as well as my mental health (though admittedly sometimes seeing everyone’s issues does have the opposite effect, and I do have to step away for a bit).

A silver lining: I absolutely adore how the word for a group of zebras is “dazzle” because we definitely dazzle together.

Despite the dazzling, living with hEDS isn’t pretty.

I will say, however, that I am grateful for the perspective it’s given me over the years. My condition is a part of what’s taught me to have compassion for not just those who are hurting visibly, but also for those who might have something going on under the surface. After all, my hEDS is an invisible disease for the untrained eye. It’s also helped bring me to understand how pervasive ableism is in nearly all facets of society.

My hEDS does not define me, but it IS a lens through which I see and experience the world. I hold onto that fact throughout the bad days, and celebrate the good days when I can.

Stay tuned for Part 2: What in the Pain?