"Like a bird on the wire... I have tried in my way to be free."
-Lyrics by Leonard Cohen *photo of a Zebra Finch on barbed wire
written by: Amanda Henderson, May 28, 2022
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. In honor of EDS Awareness month, I thought I would write a bit about what life can be like for the chronic illness community. Some days it takes up the entirety of a room, and accomplishing anything outside of the difficulties of the variables of illness are impossible. Other days you can hold it in your hand and place it in your pocket, and carry on as you try to create enough good memories to get you through to the next inevitable worse day.
Ehlers-Danlos Syndrome is a rarely diagnosed condition that effects every system in a body. It mismanages the synthesis of collagen, the protein 'glue' of the physical being, and weakens every part that is involved- skin, joints, ligaments, and hollow organs are directly effected which sends a cascade of issues to the rest of the body. There are different types of EDS, and seen here is one type, classical EDS- there are twelve subtypes, with more added as they are discovered. Some are more severe in their presentation than others, and they effect each patient differently. This makes it difficult to have a universal treatment plan for all of the symptoms that occur. Currently, there is no cure, so mediating a bodies' response to the collagen defects incrementally is the best way to have the highest quality of life. Also, responding to each patient in their own way with their individual difficulties can help them feel validated and respond better to treatments that can truly help.
One major hurdle we collectively seem to have to deal with is not being heard and treated properly by all doctors. Generally, they are not trained to deal with syndromes that can effect every aspect of the body. Medicine is more of a reactive trade- treat what is obvious and emergent- than a proactive trade- treat symptoms before they are out of hand. This system is difficult to navigate as a chronically ill patient with multiple needs who are pushed through appointments without having the time designated to digging to the root of each issue completely. The very 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. It can leave us feeling lost, embittered and alone when we are not able to stay on top of chronic issues. It can also make us 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 no easy answers for this, and no amount of being more aggressive about what our needs are will always help- it's a delicate balance in a system that is not built for chronic issues. 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 they don't realize that for EDS, all diagnoses ARE the same zebra and in the same basket. The more identifiable traits that can be addressed, the more manageable our entire syndrome can be, the higher quality of life, and the better longevity we'll tend to have.
Another major hurdle we often deal with is having a body that has a mind of its own and will often not cooperate when we want it 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 the syndrome. It is also a progressive illness, which means it can continually get worse as we age. When we have a flare that exacerbates the symptoms from one issue, it can have a ripple effect to other systems and be so overwhelming, painful, or untreatable that we cannot leave our homes. These ripples can last for days, months, even years without proper treatments, guidelines, and cures. It 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 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 due to lack of energy and fatigue, how accessible the area we wish to visit is if we require adaptive equipment, and the effects we will 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 not much else. My health had gotten to the point of being bigger than I could control or handle, and many issues were unable to be stabilized. For the sake of learning, growing, and teaching, I'll share some details in this latest physical hurdle.
• Exhaustion 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 issues, which contribute to exhaustion, and there are current studies regarding EDS and diabetes at Vanderbilt, so I'll be keeping an eye on that. This is one example of living a 'rare-ly diagnosed' life- many of our medical issues are on the verge of breakthroughs of varying degrees all over the nation. For best results, keep an open mind and an avid passion for research.
• Everything I ate went through me- I could no longer determine which foods I was sensitive to even though I have a fairly restrictive diet. (No meat, no gluten, watch the histamine intake.) CT scans revealed stool retention even after a 24 hour cleanse, so I was backed up and everything getting through is called overflow, which generally consists of collected water trying to push everything through. I was incontinent because of this- sleeping on pads and towels, only leaving the house when necessary and then using pads, crossing my fingers, and hoping for the best. This is a rarely talked about part of health decline and will keep a person house-bound all on its own. I knew the next steps could be dire, and I wasn't looking forward to those possibilities.
• An annual checkup revealed my thyroid wasn't producing as much of its hormone as it should, which is no surprise since I only have half of it. Just getting it within 'healthy range' was not enough for me- I had to fight and push to get it down to a lower number- what my body prefers- based on symptom improvement. This took several months and appeals to the doctor, and made a positive difference, but did not clear up all issues- though I'm used to not having all health issues worked out- a larger percentage helped is better, though.
• Muscle fatigue and weakness was worse than usual, probably from all of these exacerbated symptoms. My legs are the worst area, but I do have generalized muscle weakness due to neurological issues resulting from hEDS, CCI, and Tethered Cord (which was treated much too late). Plug here: the earlier EDS and comorbidities diagnoses and treatments occurs, the better! Awareness is key, so spread the word!
• Body pain, turn it up, up, up! I'm always in pain - everywhere- but, I believe more pain than usual is due to exacerbated symptoms overall and some conditions that I am seeking diagnosis and treatment for- such as Lipedema and Sjogren's Syndrome- both of which can be comorbidities of hEDS and are familial, as well. Yet another reason why diagnoses and treatments of everything you can put a name to is so very important. hEDS has no cure, so symptoms need to be explored and treated.
• Urinary incontinence, pain and inflammation, oh my! Standard issues, especially during increased flare-ups.
• My skin and liver has joined the long list of medical problems. I've always had 'go jump in a lake to put the fire out' skin that goes crazy after a shower. Lotion helps, and treating Mast Cell issues does so even more, but splotchy, red dotty, tiny itchy patches everywhere skin? This is new.. Also, a liver spot was seen and will be kept an eye on. I had one good organ left! Ha!
•I have a lot of neurological issues due to Craniocervical Instability (CCI) and the effects of having an occult tethered cord since birth. The toll it takes on the body is vast, but it's starting to effect my vision (eyeball movement when concentrating) and hand coordination and dexterity. Imagine what that's like for an artist..
• More doctor visits of other specialties and, finally, a new Internist to be a cog in the wheel of never ending tests, procedures, and ever-increasing difficulties. Finally, I have recently received some help in much better ways than I had before, and now I'm starting to catch up on a few of my chronic issues. I feel blessed that I still have some living left in me and am not completely relegated to my house at all times, but I still have to be vigilant about easily overdoing it or the consequences of a debilitating disease that continually worsens. I sometimes have a cloud of worry hanging over my head wondering when the life changing symptoms will surprise me next, even as I try to live my life to its fullest and be grateful for what I have. When you see me smiling, it is genuine- it may mask my difficulties, but I've truly found happiness in focusing on the positives. Having a silly husband who naturally evokes laughter can help, too.
•Saline IV infusions three times per week- Navigating the medial system to receive this much needed care was very difficult, but the efforts were worth it. I now access my own port weekly and administer my own fluids. I'm dysautonomic (Autonomic Nervous System does not regulate properly), hypovolemic (low amounts of bodily fluids), and have a lot of neurological damage that keeps my GI system from working correctly, so this has brought that part of my body back to life, so to speak.
•Thyroid medicine switched to a more bioavailable form- from Synthroid to Thyroid NP, which is made from hog thyroid. It contains t3 and t4 hormones instead of just t4, which can be more helpful to certain patients, including myself. My levels of the TSH test (0.5-4 is normal range) were climbing above normal, but it didn't help me only to get them into normal range- I function better when they are within 0.5-1.9.
•Supplements- some help rebalance electrolytes, others keep joints and tracts greased and in working order. Potassium, magnesium, salt, vitamin D3, B12, multivitamin, even cranberry and glucosamine chondroitin all have roles to play in helping my systems work as well as they can.
•Braces- from the ground up it's best to stabilize joints. I have shoe orthotics to keep arches in place, ankle, knee and SI joint braces to keep everything as stable as possible which helps pain levels lower and leg stamina last longer.
•Adaptive devices- I use a wheeled rollator (walker) for any distance outside of my home and a power wheelchair for longer distances that I can't reach on my own. My legs gradually lose power as the are used. This is common in EDS, and and ableist, judgmental society can be difficult to navigate when your ability level isn't constant or something that they don't comprehend. Most days I don't notice reactions, other days I can't help but notice. Hopefully, someday, tolerance and objectivity will rule.
•Diet- I don't eat meat or gluten and I watch my histamine intake. With so many medical conditions constantly vying for my attention as to which to deal with next, I try to keep my systems as happy and compliant as I can. I cannot tolerate statins, and after a stroke, eliminating meat was the best solution for keeping good cholesterol levels, heart rate, and blood pressure. I keep my protein levels up with plant proteins. My body just doesn't respond well to gluten, so it's not an option. Histamine levels play an important role in several conditions, also. Everything works better when the GI system is engaged and functioning as best it can.
•This list is not exhaustive and is ever-changing as needs and treatment options change.
When the medical community and our physical bodies are determined to hinder our best efforts, I am thankful that the internet has provided some answers and relief, including finding other doctors within our area and insurance providers through the help of online support groups. The experiences and connections can be so worth it to find any small measure of positive reinforcement. A friend in one such group stated that we get so used to being in survival mode that we tend to have a different baseline for 'normal' than most people have in terms of what an acceptable quality of life is. That sure hit home. We also mask well to try to fit in- it can be deceiving to others and eventually, to our own 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 leaves little room for expectations from others, and makes it difficult to reach out or talk about. Chronic survival calls for extra grace, mercy and possibly a little leniency in not taking cancellations or long pauses personally. In fact, reaching out despite social norms can greatly enhance a survivalists chances of having a support system that can help them move forward. It can also feel daunting to breach the chasm that is created while we're busily trying to keep our bodies going, or keep adding to the list of differences that make us more difficult to navigate in public. Consider that we often feel like a special needs burden, it's part of this package, and it can take a lot 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 issues, but hopefully the help it can potentially bring others will be worth it.
It can seem like a two-step shuffle to receive the basic care needed to enhance wellbeing for chronic illness. The possibilities the body has of malfunctioning 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 alone and daunting along the path, you are not alone- together we dazzle (as a herd of zebras is called), 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 proud. *photo of zebra finches on a wire
