Shannon lost her battle at age 30 to what was thought to be vascular type EDS in March 2017. She was diagnosed in 2005 after her second surgery for Chiari malformation. The neurosurgeons at had started noticing a subset of patients with Chiari malformation also had an Ehlers-Danlos syndrome. They told me she was one. Her mitral valve had already stretched/given way after the first surgery.
We went home from NY to Texas after they fused the base of her skull through to C-4, and we began a search for more information on this other condition that nobody seemed to know about. I read and read until my eyes hurt. All of it was true. She had had signs earlier that we didn’t recognize because she had an unremarkable early childhood. The Chiari, her heart, the bruising, the joints that we starting to subluxate were all because of EDS. Then came the kicker: her type would most likely would shorten her life. I cried for two weeks straight then presented the facts to Shannon, my husband Jay, and her little brother James. Nobody took it well but Shannon dealt with the news better than the rest of us. Friends and even family questioned the diagnosis. Some criticized the use of opiates to treat pain, some were hurt because she couldn’t come to a holiday or some gathering, some thought she was malingering and crazy. I never in my wildest dreams thought that my own people wouldn’t believe us. That was the most painful part of the journey.
For the next decade we watched EDS rob our girl of so many of life’s joys. The possibility of a mitral valve replacement talk came, but nobody was eager to try it. Even Shannon said she didn’t want another surgery for fear she wouldn’t make it out alive or worse, with more pain than she already lived in. I was sure any more pain would have have been the end of her as well. We went with palliative care over anything invasive. We hired doctors, fired a few, but in the end we had the best team we could ask for.
However, in the darkness came a bright light in the form of a young man. I was leery of Jon at first but she fell head over heels for him. I took him aside and told him, you can stay but know that this won’t be a normal relationship, and chances are she will have to leave us someday. She won’t be able to give you children or have supper on the table every night. He looked at me and said. I’m OK with that because I love her and I’ll take whatever time I can get with her. They married in 2008 and moved into the house next door so we could all be near her. She would be mad if I didn’t mention her constant cat companion Tummy Le Chat Noir. She wouldn’t go anywhere without her baby.
On March 16, 2016 she died suddenly while she and Jon were watching television; Tummy cat was on her belly, and she was in mid-text to me—we liked to text at the end of the day even though I was 20 feet away next door. I could hear Jon yell for me. I flew those 20 feet, but she was gone. I started CPR, 911 was called, everything was tried but to no avail. In minutes we were down the street to the emergency room. I was asked if I wanted to be in the trauma room at the hospital while they worked; after being in the medical profession for over 20 years I knew it was going to be hard to watch, but I hadn’t left her side in over 12 years and I wasn’t going to check out now.
The team that worked frantically asked about EDS. I explained about the comorbidities and complication in EDS as they intubated her, pumped amps of epi, and blasted her heart with shocks. The EKG strip was starting to pool on the floor and except for the paddle waves, it showed a flat line. The lead asked me if they should keep going. I whispered no…no more. I thanked the team for all their efforts. One said to me, “I never heard of EDS before, but I’ll remember it now.” EDS came to live with us a decade ago and had won, but not before I taught one more person about its rotten existence.
I came out to my brave men and told them. I hated that moment. I hugged Jon who was crying and told him, “I’m sorry, I should have run you off to save you this pain.∏ He whispered, “It was totally worth it.”
People said to me, “I never knew she was that sick.” They were shocked, and upset that we hadn’t told them about her chances of dying—we had, multiple times, but nobody wanted to believe it.
I believe that this is the biggest challenge to those with EDS or other rare/invisible conditions: to make our doctors, families, and friends aware of the physical and emotional pain these patients face daily. There is no cure, but that doesn’t mean we can’t help.
I go on with my days now. I work in a private internal medicine practice. I have shared EDS Awareness Month (May) with my co-workers and providers joining in. I gave a talk about EDS and raised money for the EDS Society. We are educating our little corner of the world, for Shannon and the others who are still trying to navigate the world with EDS.
Rest in peace, my sweet baby girl. Your husband and family misses you, and your cat is still living like the spoiled rotten princess, as you wished.