The story of Dina
My son Adam was like any other healthy 4 year old boy. He loved Chuck E. Cheese, his bike, his friends, and adored his 2 year old little sister, Emily. He had the kind of smile that warmed hearts and gave the biggest bear hugs. We were lucky to be blessed with a son who was gentle, kind, compassionate, and had a seemingly infinite capacity for love. We were a happy young family of 4 and we were so grateful for the life we had built.
On August 9, 2014, our whole world turned upside down. Adam woke up with a fever and headache that day. Over the next few days, he continued to complain about neck pain and headaches that started to worsen. A few trips to the pediatrician and eventually the ER gave us little answers. Finally, a CT scan was done and a small fluid collection was found in his brain, which Drs believed was from a possible past head injury. We were sent home, told it would resolve on its own. When it didn’t, we ended up back in the ER of a different Childrens hospital and were finally admitted. After a spinal tap and MRI were performed, it was believed we were dealing with a form of viral meningoencephalitis. Something didn’t feel right and our worst fears were realized a week later, when Adam suffered his first of 15 strokes. He was diagnosed with CNS Vasculitis after a brain biopsy and started on steroids & chemotherapy. He continued to have strokes despite aggressive treatment and we watched our lively, beautiful boy slowly lose function of different body parts. In November, we transferred to CHOP for a second opinion, as we were no longer convinced we were dealing with vasculitis. It was there that the doctors finally diagnosed him with Systemic Degos Disease, an incredibly rare vasculopathy that affects the lining of the small and medium blood vessels. He was immediately started on the only known treatment, but unfortunately, did not respond and continued to have strokes. Despite all his suffering, our sweet Adam continued to fight bravely. He rolled with the punches, never got angry or upset. He would make his daddy bring in munchkins so he could give them out to all the nurses and doctors, and made goody bags of medical supplies he’d sweet talk out of the nurses for all his friends back home. He gave us the strength the face each day.
On Christmas Eve, we received the devastating news that Adam had suffered more strokes and would likely no longer be able to breathe without the assistance of a breathing tube. We spent our last Christmas together as a family of four and then our little boy got his wings on December 28, 2014 at 2pm. It is still infathomable to us how our preciously healthy child who had rarely even had an ear infection or cold could suddenly just suffer from a disease that has only approximately 200 documented cases in the world. We had certainly never heard of Degos Disease before, and even most doctors we speak to ask us what it is. There is such a feeling of isolation in having a child with such a rare disease. There is such little information, such a scarce amount of resources for support. Our hope and goal is to spread awareness, not only to help other families like ours but to honor our sweet Adam’s memory.