Help Jamie's EDS and Craniocervical Instability
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On October 5th, after two previous ER visits in New York, Jamie was rushed to a third hospital for an emergency spinal surgery.
After two weeks of frightening symptoms following a brainstem concussion, a ‘Tethered Spinal Cord release’ was carried out to address the profoundly worsening neurological and spinal complications sometimes seen in the Ehlers-Danlos Syndrome population. The only solution to arrest the nightly full-body seizures, loss of sensation in his lower limbs, permanent nerve damage and potential paralysis below the waist, was to operate.
A year later, after a sharp and rapid decline in symptoms and progressive difficulty walking, we have been told that Jamie's spinal cord, cut and “de-tethered” in the operation, has now re-tethered. Our worst-case scenario has become a reality after an extremely challenging year post-surgery, both neurologically and physiologically. The re-tethering was confirmed on imaging by the world’s leading Neurosurgeon for Tethered Cord – Dr. Petra Klinge at Brown University.
We are again in position where we have to move very fast to ensure that the progressive damage to the brain, spinal cord and nervous system does not become permanent and paralysis is avoided. We need to work ASAP on getting Jamie safely to the east coast (from Los Angeles), schedule the second emergency spinal surgery and allot for all the medical expenses pre-and-post surgery and into rehabilitation. Once again Jamie will be unable to work for another extended period of time and will need to engage in daily extensive and intensive rehab of his spine, brain and nervous system, and potentially entertain serious additional surgeries. It goes without saying that despite various efforts, the past year has been extremely challenging financially. The costs remain extreme and unrelenting.
Please see below for the history of Jamie's case and multiple complex and chronic conditions that have changed life for him and us over the past six years. As ever, we thank you from the bottom of our hearts, and if there are any thoughts or questions about the above, please do write to us and we can fill-in all the blanks.
With love,
The Wright Family.
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Our son and brother Jamie, has spent four-and-a-half years searching for answers for, and solutions to his degenerative, debilitating health struggles. In 2017, Jamie fell ill, and has since been diagnosed with a set of serious conditions, including Mast Cell Activation Syndrome, Lyme disease, POTS and most importantly, a serious, rare, genetic condition called Ehlers Danlos Syndrome (EDS) that has caused Craniocervical Instability (CCI).
Over the years, we have all been doing our best to support Jamie finding ways to have appropriate treatment and achieve less chronic and debilitating musculoskeletal pain. It has been a tremendously challenging and winding path up to the point of needing to create this page.
In order to best convey the current situation, we have done our best to condense a bit of the background as to how we got to this point and what we can do about it moving forwards.
What is Ehlers Danlos Syndrome and how it is affecting Jamie…
EDS is a genetic disease that results in faulty collagen production and a weakening of the body's 'connective tissues' that hold us all together. This results in loose, unstable joints that are easily damaged. In Jamie’s case, this ligament ‘laxity' has caused Craniocervical Instability, whereby the ligaments holding Jamie’s skull onto his spine are too weak. He now has a structural compression of his brainstem, due to his inability to hold his head up, resulting in an insufficient oxygen flow to his brain.
If left untreated, this can be life-threatening, weakening the blood vessels, heart and organs.
Alongside countless widespread autonomic nervous system and cognitive dysfunctions that Jamie experiences as a result of a compressed brainstem, EDS makes it extremely challenging for him to support his own body. Keeping it upright throughout the day is a huge challenge as the muscles exhaust from being the sole provider of structural stability, otherwise provided by the skeletal system.
EDS and its ligament laxity also results in multiple joints rotating out of position very easily, leading to frequent subluxations, dislocations and damage to a number of different parts of the body. In Jamie’s case, he has been managing pronounced deterioration in the strength of his neck, shoulders, spine, feet and hips specifically and wears numerous braces under his clothing in an effort to mitigate excessive movement of a number of key joints.
Physically, his daily life has become immensely challenging. He lacks the structural stability to do everyday tasks without injury and pain. The simplest everyday tasks are extremely demanding for him, and as time progresses, some are becoming impossible to perform. Daily, he circumnavigates repeated joint subluxations, relentless muscular and joint pain, and the inability to sit, lie-down and walk without suffering these symptoms.
When allied with an underlying, complex immunological condition – Mast Cell Activation Syndrome, post-infectious processes due to chronic Lyme disease, and autonomic dysfunction due to POTS, the picture is multi-layered and tremendously difficult to treat. As we have learned through our doctors and various patient advocacy groups, for those that suffer with EDS, the pattern of overlapping circles with these specific conditions, each feeding into, precipitating, and exacerbating the other, is all too common with EDS sufferers.
How you can help…
Over the past four-and-a-half years, Jamie has seen 30+ doctors. Misdiagnosis is extremely common with most of Jamie’s conditions, but through force-of-will and huge expense up to this point, we have largely overcome the challenges in achieving an accurate understanding of the conditions (and their consequences) that he's facing.
After a long waitlist, Jamie is now finally in the care of two specialists who are global leaders in the field: Neurologist Dr Ilene Ruhoy – the Medical Director for Mt Sinai Hospital's Chiari and EDS Centre in New York, and Dr Alissa Zingman – the founder of PRISM Spine & Joint in Silver Springs, Maryland. There is now a plan in place, we just need to act on it.
Jamie has always been a kind and caring son, brother and friend, there for people when they most need him. For his family, business partner and those friends he has confided in, seeing him in such daily pain has been devastating. For years, he has done his utmost not to let these illnesses define him or his relationships, or even overly restrict him from following his professional hopes and dreams.
However inspiring it has been to see, the time has come for us to try and actively intervene with more advanced, and more consistent therapies to help improve Jamie’s quality of life. Having found focused care with an excellent medical team, we know what measures we can take to stabilise his neck and spine specifically. Without acting on these measures, Jamie’s conditions and associated pain will not improve, and will likely deteriorate and degenerate further. We want to do all we can to prevent that from happening, and in so doing, both we and Jamie hope to spread as much awareness about these complex, chronic conditions as possible to help those in the community currently suffering without care or hope of care. As a result a portion of donations from this fundraiser will go towards The Ehlers Danlos Society, and their ongoing research efforts to raise awareness for the disease – https://www.ehlers-danlos.com/what-is-eds/
One interventional measure that could be taken is Occipito-Cervical spinal fusion, to stabilise the cervical spine and correct instability at the junction of the spine and skull. Though Jamie is regrettably a candidate for surgery, due to the severity of his conditions, as a family we have decided to pursue an alternate path, due to the associated risks and costs of such surgery.
As outlined by his specialists, there do exist a number of other options that could advance the goal of healing Jamie's tendons, ligaments and some of his deteriorating muscle tissue. Only via aggressive, long-term, interventional treatment will this be successful, however. This would include targeted prolotherapy and/or PRP injections to attain more functioning and slow the progression of his disease to experience less chronic pain. These injections are intensive, not covered by insurance and would need to be administered in multiple sites around his body, focusing on problem joints and surrounding soft tissues – roughly $1300 per treatment with between 50-75 treatments needed for improvement over time. Jamie has also been advised to begin Muscle Activation Therapy, EDS literate physical therapy, purchase an ALF appliance to help with cranial torsion and the untwisting of his skull (roughly $13,500 over the course of a year with osteopathic monitoring). To those ends, the costs associated with managing even non-surgical approaches are extreme.
Throughout Jamie’s continued efforts to work through his illness, with our financial support over the years, the medical care has resulted in significant debt, as the therapies and treatments that are effective for EDS (including the majority of the options mentioned above) are not covered by insurance, whether in the US or UK. The financial burden of this treatment moving forwards has led to the act of creating this page, where we are seeking financial assistance so this ongoing treatment is a possibility for Jamie.
We are truly grateful for any contribution big or small to ensure that our son and brother has the best possible chance of improving over time. We will of course be happy to answer any and all questions, and will post updates as to Jamie’s progress over the course of the year.
With love.
The Wright family – Stephen, Sophie and Peter
Additional reading:
Craniocervical Instability
https://metro.co.uk/2021/10/08/student-in-danger-of-internal-decapitation-due-to-rare-conditions-15386522/
Ehlers Danlos Syndrome
https://www.theguardian.com/society/2019/nov/05/we-live-in-constant-fear-the-reality-of-life-with-ehlers-danlos-syndromes
Mast Cell Activation
Lyme Disease
Fundraising team: Wright family fundraising team (3)
The Wright Family
Organiser
Los Angeles, CA
Lewis Carpenter
Team member
Sophie Wright
Team member