Blog dedicated primarily to randomly selected news items; comments reflecting personal perceptions

Saturday, November 01, 2014

The Butterfly Disease

"The wounds are bigger, deeper, they don't heal and they are everywhere. And they hurt very much. [Pain scale?" In the past week, between 8 and 9, closer to 9."
"I try to invent myself as a dragon and use their powers to heal me or fight through the pain. I used to control the pain with just my imagination then I started Advil and Tylenol. Then Advil and Tylenol at the same time. Then we moved to morphine, and now we're at methadone. Imagine that. It's crazy."
"I don't have any friends. I don't have much time to socialize during lunch [at school] because I haven't been feeling well."
"I kind of have to deal with more pain to be less tired, or more tired to have less pain."
"He [Gibson, a Boston terrier] will never try to jump on me, bite me or scratch me. Let's say we all come in the house, one by one. Even if I was in the middle of the pack, he would be excited to see me, but he'd just lick my hand, and as soon as I got out of the way, he would jump on the next person and the next person."
"My dog has some kind of power; I can feel him and he can feel me. We have a special bond. It's not just that he's my best friend. I think I'm his best friend."
Jonathan Pitre, 14, with Epidermolysis bullosa
 Julie Oliver / Ottawa citizen   Jonathan Pitre, his mother Tina Boileau

Epidermolysis bullosa is a rare genetic disease causing the sufferer's skin to blister, shear and sear, on an ongoing, never-ending journey to ongoing and increasing pain. Jonathan's skin is so sensitive his body must always be wrapped in gauze to create a protective barrier against any kind of friction. Jonathan's disease severity is such that it is painful to do any of the normal things that humans are accustomed to, from eating to walking and bathing.

He is now fourteen, a student at an Ottawa-area high school. In the past four months alone the severity of his symptoms have increased to the extent that his wounds have become larger, deeper and slower to heal. The pain he suffers is so severe that methadone is the pain treatment he must now depend upon for hope of achieving relief. A Grade 9 student at Embrun's Catholic secondary school, he is trying to find a balance between pain relief and ability to focus on concentration during school.

Jonathan is helped into school by his teacher's aid, Nathalie Courillon-Chevalier. He arrives to school early and gets to classes a couple of minutes late to avoid all the traffic in the hallways. His skin is so sensitive, often the slightest of touches in the wrong place can hit a wound and cause excruciating pain. Jonathan suffers from Epidermolysis Bullosa (EB) - a rare disease defined by severe blistering and shearing of the skin that leaves Jonathan in constant pain - the likes of which most would find intolerable.

After school it has become a struggle to remain awake but he must because there is a ritual bath protocol where his body is slowly unwrapped from its gauze protection and he is immersed in a warm water, Javex and salt solution to attack the bacteria that lead to infection. New blisters must be lanced, and then he is re-wrapped up to his neck in gauze. The process consumes between 3-1/2 to four hours. "As soon as it's done, I grab him and put him to bed", his mother Tina Boileau says.

She is the one who does the wrapping. And she must live with the knowledge that those with Dystrophic EB, like her son's, are susceptible to squamous cell carcinoma, an aggressive form of skin cancer, at a rate higher than among the general population. Jonathan discovered, to his great relief, that there are others who suffer as he does, and from the very same affliction. That knowledge and his introduction to others like himself came about through exposure at a Toronto gathering of sufferers sponsored by DEBRA Canada. "Before that, I felt alone", he said.

The disease afflicts about 2,500 young people in Canada. They are often called "butterfly children", in reflection of their fragile state where key structural proteins that connect the two upper layers of skin are missing. Without that "glue" to move in synchronization the skin literally falls apart. Jonathan's best friend, his Boston terrier, seems to sense Jonathan's uniqueness. Gibson stays with Jonathan during his baths, to lick his wounds and pin back his ears when sensing a particularly problematical blister.

When Jonathan weeps with pain, Gibson will attempt to distract him by walking into a wall, or falling off a chair. While Jonathan attends to his homework assignments Gibson, his close companion in his trials through life, will pant and whine while waiting for attention.

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