While most of us that are familiar with EDS wish it was as entertaining as the book Fifty Shades of Grey, one thing we can all agree on is that it can be just as controversial with all of the real shades of grey we find ourselves wading through. With proper diagnosis often taking years, and even longer to find experienced physicians to help properly manage our care, many in the EDS community sadly share frustrations and even horror stories of things that happened to them because their condition was not recognized or completely understood. This past week, ABC’s 20/20 did a special report on EDS and featured the Ross family whose struggle is an extreme, but sadly not rare, example of one of the most serious shades of grey that EDS families can face – accusations of child abuse. Their youngest child has sustained several fractures triggering an investigation. While the safety of the child must always be first and foremost, since EDS is involved, the following grey shades can make this case even more difficult for all involved parties to sort out:
- There is not a genetic test for all types of EDS.
- There are additional subtypes of EDS that are thought to be discovered, but lack money for studies to complete confirmation. In fact, there have been several studies noting a possible EDS/OI (Osteogenesis Imperfecta) overlap. (For the record, TCAPP has several EDS kids in our support group who have had several fractures and struggle with bone density issues.)
- EDS is a connective tissue defect. Connective tissue is the building blocks of many body parts, not just joints. Therefore, a defect in the connective tissue can affect anything from the joints to the spinal column, to the gastrointestinal tract, to one’s vessels, and much more. These defects can lead to certain nutritional deficiencies (due to absorption issues through our connective tissue in the gastrointestinal tract) that can lead to lower bone density, or predispose one to “brittle bones.” I often tell people to think of a house that was built on a faulty frame (the body being the house and the connective tissue the frame); there are so many parts of that house that can fall apart and cause a cascade and fixing it piece by piece is just that, the infrastructure is still faulty so there is always the potential for collapse.
- While contortionists may be able to exhibit what EDS can do to the bodies and joints, people tend to focus on the artistry of contortionism. They do not realize that with repeated dislocations, etc., there is internal damage being done and many will suffer from chronic arthritic or nerve pain at a very early age for the rest of their life.
Even when one can manage to sort through the shades of grey above, there are still the other shades of grey those with EDS live with:
- The shades of grey in treatment of EDS: Many hospitals, physicians, and even families judge successful treatment by a cure. There is no cure for EDS and until the standards of success are changed to judge the quality of life of an individual and EDS is looked at as an illness that needs to be under the realm of palliative care, we run the risk that many will continue to be denied treatment (for fear of failure), or constantly be disappointed in outcomes.
- The shades of grey in living with EDS: How does one learn how to live their life to the fullest or allow their child to live their life to the fullest when the slightest motion, movement, such as opening or closing a car door can result in a catastrophic event that medically disables that person or child for months or years at a time?
- The shades of grey in advocating for a child with EDS: How do parents manage to support their family and care for their child afflicted with EDS when the medical community itself doesn’t fully understand the disease or its ramifications? There is no treatment plan or guidelines to follow and yet so many of us seem to follow similar challenges. How do families juggle the added finances of multiple out-of-pocket expenses, such as medical travel just to keep the quality of the life of their child optimal, and, how do they juggle the other everyday issues of raising a child in today’s world?
The “shades of grey” for EDS go on and on. So, in cases like the Ross family that ABC’s 20/20 featured, before one is quick to judge, jump to conclusions, or, consider one guilty BEFORE being proven innocent, perhaps some extra time, due diligence, and digging should be done before a family already condemned to live with a lifetime of shades of grey, has to go through more heartbreak.