Before you begin to read this column, a warning — some of it will be most upsetting. Please be aware my intention is not to disturb, but to inform.
Today, Feb. 28, marks an observation I never knew existed until a couple of months ago. International Rare Disease Day began in Europe several years ago as a way for people dealing with ailments not well known in the medical community to bring their illnesses to the public eye. On Dec. 9 last year, our extended family became instantly familiar with what this means.
That day, after a harrowing experience, my niece gave birth to her first child — a little boy with enormous blue eyes — whom she and her husband named Shea.
The first inkling that all was not well was when the attending physician discovered the baby did not have any skin on his right leg.
Baby Shea was airlifted to Vancouver Children’s Hospital where it was determined he had an extremely rare disease called epidermolysis bullosa, or EB as it’s known.
Shea is missing the protein that binds the first and second layers of skin together. The disease manifests itself in painful blisters resulting from friction. The baby’s skin is so fragile that the slightest contact with clothing or another person results in new blisters. The severity of the EB ranges from mild to fatal.
Shea has the worst form of the disease. Already blisters have formed in his throat, esophagus and nose that make feeding very difficult. To combat the pain the little guy endures on a daily basis, he receives morphine and other stronger medications. Doctors have told his parents it’s doubtful the baby will survive infancy.
At two and a half months, he’s already cut his first teeth, he stares intently at all the people who care for him, and he has definite likes and dislikes. His grandpa tells me that Shea tries to spit out the iron drops, but smacks his lips over another nutrient flavoured with sugar.
The family gives credit to Canuck Place, the children’s hospice in Vancouver. There, for the first time since Shea was born, they were able to take their baby for a walk and show him off like any proud parents would. The mansion where the facility is located is large and airy, run by professionals and staffed by an army of volunteers. The folks there treat all these hurting people like royalty, according to my brother.
Once the baby’s care is in place, the little family will be going home to Prince George. Care requires changing of bandages without touching other parts of Shea; any contact leaves fresh blisters in its wake. EB patients are called butterfly children because of the extreme sensitivity of their skin. The blisters are likened to severe burns. Shea is unable to wear any clothing; contact with a cotton sheet resulted in the skin on his head being rubbed off. He remains swaddled in soft sheepskin. Understandably Shea rarely sleeps, and neither do his parents. They work 24/7 to make their son as comfortable as possible.
All of this means that neither of them, young professionals, are able to return to their jobs, so in addition to a very sick baby they have financial worries.
If you are interested in helping this amazing family or would like to read more about this devastating disease, go to www.loveforshea.com.
I sincerely hope your family never has to mark this day.
