Wednesday, December 27, 2017

6 Tips For Newly Diagnosed Ehlers Danlos Hypermobility Patients

"Ewwwwww," says the kid in class ogling at the unnatural angle of my arm. The rude remarks and endless childhood injuries all started to make sense once I was diagnosed with Ehlers Danlos Syndrome Hypermobility Type.

After enduring months of concerning gastrointestinal and cardiac symptoms in 2010, I was sent to the Mayo Clinic in Rochester, Minnesota. My mom's phone rang as I was undergoing a workup for a condition called Dysautonomia/Postural Orthostatic Tachycardia Syndrome (POTS). It was my pediatrician from home. The phone conversation entailed a series of random questions — Is Cheyanne's 'double jointed?' Does she have flat feet? What about a high, narrow palate? Yes, yes, and YES! In the midst of our confusion, the doctor had sworn she had figured it out. She explained that Ehlers Danlos Syndrome is a genetic connective tissue disorder that causes my symptoms and is commonly associated with my already established diagnoses of Gastroparesis, Dysautonomia, and mast cell issues.

We were seeking a condition that had a cure, the pediatrician included. EDS did not quite fit that criteria. It was dismissed to search for a condition that did. Two years later, ignoring the obvious did not prove to be fruitful.

I was sent to yet another specialist who took a thorough family history, assessed joint hypermobility with the Beighton Scale, and went down a symptom checklist. The geneticist diagnosed me with Ehlers Danlos Syndrome Type 3, or the Hypermobility Type (h-EDS). I was barely 14 and rightfully scared. Since then, I have acquired valuable tips to manage the condition.

  • Invest in supportive braces. 
My joints go out more than I do. While that pun is funny enough, it is beneficial to invest in braces with varying levels of support for problematic areas. I have knee braces, elbow braces, ankle braces, finger splits, wrist supports. It can be as simple as an ace bandage purchased at the local drug store, or heavy duty custom made hinged braces that insurance typically covers for the diagnosis.

Out of all of my joints, my neck causes the most disabling issues. My head is like a bobble head, my neck too weak to support its weight. It causes intense headaches at the base of the skull. A cervical collar is imperative to soothe the pain.

  • Deconditioning is real. 
Doctors often go off on deconditioning tangents. They ramble on forever about how it is beneficial to keep moving, why exercise is important, and that anyone who spent their days sick on the couch would suffer symptoms of increase pain and fainting spells. In the midst of misery, their words are kind of...annoying. And it seems less than helpful, like they are trying to place blame rather than improve my quality of life. Regardless, deconditioning is not an empty warning. It is real.


Exercise makes a world of difference. Gaining muscle provides support to faulty EDS connective tissues. With muscle loss, like in the instance of severe GI involvement, my injuries increase tenfold. Muscle is also conducive for the EDS patients with Postural Orthostatic Tachycardia Syndrome. Upon standing, muscles contract around the loose blood vessels that contribute to tachycardia, syncope, and dizziness. Movement keeps the blood from settling in the lower extremities, increasing the chance it gets where it needs too be in the brain.

It is perfectly understandable that intense exercise is impossible. The goal for an h-EDS patient is to increase muscle mass enough for support, not to become the next body builder. Choose exercises that do not take the joints out of normal range of motion. Exercises done laying in bed serve the same purpose.

I saw many physical therapists that did more harm than good. As another example, when I did attend PT, I would do aqua therapy. That exercise style is typically preferred, as it is not strenuous. The physical therapist would have me hold light weights in my hands, moving my arms up and down against the water. Doing so allowed my elbows to go out of normal range of motion. I was frequently injured afterwards. Learn what feels okay for you. It may be different!

Use aids  

If daily activities are causing symptoms, do not be hesitant to use mobility aids or braces. I was embarrassed initially, but I should not have been. They are there for assistance.

Unless stated otherwise by a doctor, be sure to have scheduled time without the braces to prevent certain muscles from weakening. I use them as a guide to learn the normal range of motion. Once comfortable, I exercise without them even if I am dependent on them the remainder of the day.

  • "Party tricks" are entertaining to everyone but you. 
Leave the acrobatics to the circus. Party tricks are fun, but only at the expense of the h-EDS patient. Weird bending of the fingers, popping joints out at will, and other bodily contortions tend to elicit much attention. The "ewwwwww's" and snarky comments I received can attest! However, purposeful party tricks will cause unnecessary pain.

Read This Is What Ehlers Danlos Syndrome Is Like.

Unless it is the Beighton Scale used as part of the diagnostic process, I find it best to avoid "party tricks." Faulty collagen is not designed to stand the test of time. To minimize future problems, it is in my best interest to preserve my joints as long as possible.

  • Get yearly echocardiograms. 
Ehlers Danlos Syndrome Type 4, also known as the vascular type, is infamous for causing aortic dissections and the spontaneous rupturing of organs. Fortunately, the hypermobility type substantially lessens that risk. It is still suggested to get yearly or bi-yearly cardiology workups to measure the aorta and rule out structural abnormalities that all EDS patients are prone to.

  • Have an emergency plan in place. 
Like already mentioned, the hypermobility type of EDS is at a lesser risk for organ rupture, but h-EDS patients are more susceptible to it than the general population. It is important to keep that in mind in an emergency.
I keep the EDNF Wallet Card on hand to inform about the following:
- Joints may be lax and dislocate easily.
- Skin tearing and splitting 
- Mitral Valve Prolapse and hernias  
- Cerebral arterial rupture
- Avoid elective procedures
- Often require vascular surgery, ICU 
- Delayed healing
- Local anesthetics are shorter lasting
- Spinal involvement
All emergency staff should heed those warnings in the case of an emergency or trauma, like a car accident.

In addition to the wallet card, my medical ID bracelets says, "EMRGY INFO W EPIPEN." I have typed emergency information alongside my EpiPens that is specified to my particular presentation. It includes contact information, a list of current medications and dosing, my food and drug allergies, doctors on my team and their specialists, feeding tube and central line details, and an anesthesia protocol.

  • Live despite limitations. 
Every day Ehlers Danlos Syndrome hypermobility type and its comorbid conditions brings surprises, both good and bad. There are weeks to months where I cannot function. Expecting flares is realistic.

Find ways, within reason, to adapt. I wish someone had told me to not hold back when I was first diagnosed. Do not hold back in the activities that you wish to participate in because the memories from healthier times are forever cherished.