Why Having Ehlers-Danlos Syndrome Feels Like Being a Bird on a Wire

Seeing a bird perched perilously upon a high wire seems reminiscent of living with chronic illness, which is a human health condition or disease that is persistent or otherwise long-lasting in its effects. I’ve thought about what life can be like for the chronic illness community. Some days, chronic illness can seem to take up the entirety of a room, and accomplishing anything outside of the difficulties of the illness may feel impossible. Other days, you may be able to hold chronic illness in your hand, place it in your pocket, and carry on as you try to create enough good memories to get you through to the next inevitable hard day.

• What is Ehlers-Danlos Syndrome?
• What Are Common Ehlers-Danlos Syndrome Symptoms?

Ehlers-Danlos syndrome (EDS) is a rarely diagnosed condition that affects every system in the body. It mismanages the production of collagen, the protein “glue” of the body, and it weakens every part that is involved. Skin, joints, ligaments, and hollow organs are directly affected — which leaves the rest of the body with a cascade of struggles.

There are 13 different types of EDS, and more are added as they are discovered. Some are more severe in their presentation than others, and they affect each patient differently. This makes it difficult to have a universal treatment plan for all of the symptoms that may occur. Currently, there is no cure for Ehlers-Danlos syndrome, so mediating a body’s response to the collagen defects incrementally is typically the best way to have the highest quality of life. Also, responding to each patient’s individual difficulties in their own way can help them feel validated and respond better to treatments that can truly help.

One major hurdle many of us with EDS often have to deal with is not being heard and treated properly by our doctors. Generally, doctors are not trained to deal with conditions that can affect every part of the body. Medicine is usually more of a “reactive” profession — treating what is obvious and emergent — than a proactive trade in which symptoms are treated before they get out of hand.

This system can be difficult to navigate as a chronically ill patient with multiple needs. We are sometimes pushed through appointments without having the time to dig to the root of each symptom completely. The areas in which we live, the insurance that we have (or don’t have), and the amount of money we are able to spend on treatments and out-of-pocket costs are big factors in the amount of care we are able to obtain. This process can leave us feeling lost, embittered, and alone when we are not able to stay on top of our chronic illnesses. It can also make us feel ecstatic, hopeful, and accepted when we are simply heard and a doctor is willing to try the treatment plans we have researched so diligently.

There are often no easy answers for this, and no amount of being more assertive about what our needs are can necessarily help. It’s a delicate balance in a system that is not built for treating chronic illness. I’ve had multiple doctors placate me to not look for further diagnoses, stating that “One ‘zebra’ found is enough” or “We like to keep everything in one basket,” though doctors don’t often realize that with EDS, all diagnoses are the same “zebra” and in the same basket. The more identifiable traits that can be addressed and the more manageable our entire syndrome can be, the higher the quality of life and the greater longevity we’ll tend to have.

Another major hurdle we often deal with is having bodies that have “minds of their own” and will often not cooperate when we want them to be active. Many types of EDS are multi-systemic conditions that have many comorbidities, which are other illnesses or conditions that are caused by Ehlers-Danlos syndrome. EDS is also a progressive illness, which means it can continually get more severe as we age. When we have flares that exacerbate the symptoms in one body system, it can have a ripple effect to other systems and be so overwhelming, painful, and untreatable that we cannot leave our homes. These ripples can last for days, months, or even years without proper treatments, guidelines, and cures.

Living with EDS can be a never-ending cycle when there are so many factors that lead to delicate imbalances in such a difficult-to-manage system. We must often consider everything we consume, how we sleep or spend our time, where the bathrooms are at every location, whether we’ve been keeping up with a hygiene routine, how accessible the areas we wish to visit are, and the effects we may have to deal with after a social excursion of any kind. At this time last year, I was barely able to leave my home for appointments — and I couldn’t do much else. My health had gotten to the point of being bigger than I could handle, and I couldn’t stabilize it. For the sake of learning, growing, and teaching, I’ll share some details in this latest physical hurdle.

1. I feel exhausted to the point of often not being able to drive myself.

Even walking the dogs in a zombie-like state was a chore I could hardly endure. Running an errand? Never. I do have blood sugar symptoms too, which contribute to exhaustion, but fortunately, there are current studies regarding EDS and diabetes. Many rare diseases are on the verge of breakthroughs of varying degrees all over the nation, but for now, I struggle with being tired.

2. Everything I eat goes through me.

I can no longer determine which foods I was sensitive to even though I don’t have many foods I can eat. CT scans revealed stool retention even after a 24 hour cleanse, so I was “backed up.” Everything getting through is called “overflow,” which generally consists of collected water. I was incontinent because of this — sleeping on pads and towels, only leaving the house when necessary, and using pads, crossing my fingers, and hoping for the best. This is a rarely talked-about part of health decline and can keep a person at home all on its own. I knew the next steps could be dire, and I wasn’t looking forward to those possibilities.

3. My thyroid wasn’t always producing as much of its hormone as it should be.

This is no surprise since I only have half of my thyroid. Just getting it within “healthy range” was not enough for me — I had to fight to get it down to a lower number based on symptom improvement. This took several months and appeals to the doctor, and it made a positive difference, but did not clear up all of my health struggles. I’m used to not having all health symptoms worked out, but the more symptoms resolved, the better.

4. My muscle fatigue and weakness has increased.

This is probably because of all of my exacerbated symptoms. My legs are the weakest, but I do have generalized muscle weakness due to neurological issues resulting from hypermobile Ehlers-Danlos syndrome and tethered cord . The earlier EDS and its comorbidities are diagnosed and treated, the better.

5. I’m always in pain everywhere.

I believe the increase in my pain is due to exacerbated symptoms and other conditions, like lipedema and Sjogren’s syndrome, that I’m still trying to get diagnosed. This is yet another reason why diagnoses and treatments are so important.

6. My skin and liver are struggling right now.

I’ve always had skin that flares after a shower. Lotion helps, and treating mast cell symptoms does so even more, but splotchy, red, dotty, tiny, itchy patches everywhere? This is new. Also, we found a spot on my liver, and we have to keep an eye on it. I had one “good” organ left! Ha!

7. I visit many doctors.

I even finally have a new internist, but they just seem to be a cog in the wheel of never-ending tests, procedures, and difficulties. I have recently received some help in much better ways than I have before, and now, I’m starting to catch up on treating a few of my chronic symptoms. I feel blessed that I still have some life left in me and am not completely relegated to my house at all times, but I still have to be vigilant about “overdoing it.” I sometimes feel like I have a cloud of worry hanging over my head as I wonder when my life-changing symptoms will surprise me next, even as I try to live my life to its fullest and stay grateful for what I have. When you see me smiling, it is genuine. My smile may mask my difficulties, but I’ve truly found happiness in focusing on the positives. Having a silly husband who naturally evokes laughter helps too.

Here are some treatments that are currently helping and making a positive change for the better:

1. I get saline IV infusions.

Navigating the medical system to receive this much-needed care was difficult, but the effort was worth it. I now access my own port weekly and administer my own fluids. I have dysautonomia (My autonomic nervous system does not regulate properly), I have hypovolemia (I have low amounts of bodily fluids), and I have a lot of neurological damage that keeps my gastrointestinal (GI) system from working correctly. This treatment has brought some of my body back to life.

2. I take supplements.

Some of my supplements help me rebalance electrolytes, while others keep my joints and tracts in working order. Potassium, magnesium, salt, vitamin D3, B12, multivitamin, even cranberry and glucosamine chondroitin all play roles in helping all of my systems work as well as they can.

3. I wear braces.

It’s best for me to stabilize my joints from the ground up. I have shoe orthotics to keep my arches in place and ankle, knee, and joint braces to keep everything as stable as possible — which helps lower my pain levels and increase my leg stamina.

4. I use adaptive devices.

I use a wheeled walker for any distance outside of my home and a power wheelchair for longer distances that I can’t navigate on my own. My legs gradually lose power as they are used. This is common in EDS, but society can be difficult to navigate when your ability level isn’t constant. Most days I don’t notice reactions, other days I can’t help but notice. Hopefully, someday, tolerance and objectivity will rule.

5. I eat to meet my body’s needs.

I don’t eat meat or gluten, and I watch my histamine intake too. With so many medical conditions constantly vying for my attention, I try to keep all of my systems as happy and compliant as I can. I cannot tolerate statins, and after my stroke, eliminating meat was the best solution for keeping the right cholesterol levels and a healthy heart rate and blood pressure. I keep my protein levels up with plant proteins. My body doesn’t respond well to gluten, so it’s not an option. Everything in my body works more easily when my GI system is engaged and functioning as best as it can.

When the medical community and our physical bodies are determined to hinder our best efforts, I am thankful that the internet can provide some answers and relief. It helps me find other doctors in my area and locate insurance providers through the help of online support groups. The experiences and connections that others with chronic illness share can be so helpful in finding any small measure of positive reinforcement. A friend in one group stated that people with chronic illness often get so used to being in “survival mode” that we may tend to have a different quality of life baseline than most other people have. That sure hit home. We also may “mask” well to try to fit in, but masking our symptoms can be invisible to others and may twist our perceptions of ourselves.

If you’ve been surprised by anything I’ve mentioned thus far and wonder why you don’t know these things are happening, keep in mind that “survival mode” is a place in life that may make it difficult to reach out or talk about what we may be going through. Chronic illness survival calls for extra grace, mercy, and leniency in not taking cancellations or long pauses personally. In fact, reaching out can help us have support systems that can help us move forward. It can also feel daunting to breach the chasm that forms while we’re busily trying to keep our bodies going or are figuring out how to navigate our conditions in public. Consider that some of us with chronic illness may feel like a “burden,” which can take a lot of work to overcome. In fact, it took me two weeks to write this article. It is daunting to put myself out there and be vulnerable about such life-altering symptoms, but hopefully, the help it can potentially bring others will be worth it.

Trying to receive the basic care needed to enhance wellbeing with chronic illness can feel like a two-step shuffle. The possibilities of something going “wrong” can seem endless, but I urge and encourage anyone struggling with lack of proper care and diagnoses to strive to move forward regardless of the hurdles. While it can feel lonely and daunting along the path of living with chronic illness, you are not alone. Together, just like a herd of zebras, we are a dazzle, and the more we all learn from our conditions independently, the more we can share and look forward to as a group. We can be a flock of birds on a wire, perched perilously but proudly.