My son Billy, age 7, loves pepperoni pizza and TV. He has big blue eyes and a naughty laugh. He zips around school in a wheelchair called “Streaking Blue.” When kids ask him why he uses a wheelchair, he says: “I have a hurt hip.”
Billy fractured his right femoral neck (the part of the femur bone that hooks into the hip) over 3 years ago. Likely, it was the jumping from coffee table to couch over and over again as 4 year old boys like to do. That, and the fact that he has a rare bone disease called fibrous dysplasia that makes many of his bones flimsy like marshmallows. There is no real treatment for his weak bones other than surgery. His disease has no cure. Billy is on his second set of titanium rods right now, one rod in each femur for support. Some doctors describe the surgery as “sticking a straw into a glass filled with cottage cheese.”
Kids like Billy face grim statistics. Only one in four adults with disability are employed and less than one in ten own a home. Transportation is complicated. Most vehicles on the road don’t come with a wheelchair lift or space for a wheelchair. When the one ticket out of the house is mom’s wheelchair accessible minivan, it can be hard to hang out with friends and build social connections.
Just a few generations ago, kids with serious medical conditions died before reaching adulthood. Medical advances have improved survival rates and now each year 500,000 boys and girls in the United States with serious medical conditions turn 18 years old. The flipside of this gift of longer life is that these kids often have nowhere to go when they age out of pediatric medicine – when they turn 18 they may be handed a stack of medical records and simply directed to try the hospital across the street.
The move from pediatric doctors to adult doctors is called transition of care. Adult doctors don’t exactly open their arms to kids like Billy. His health problems are rare, complex, and expensive. Providing care to Billy can be emotionally hard for doctors because even the best medicine cannot improve his condition – fibrous dysplasia humbles the most skilled surgeons.
Transition isn’t easy, but it has to happen. Pediatric doctors can’t match adult doctors in ability to address adult needs – sexuality, fertility, adult behaviors (smoking, alcohol, drug abuse), employment, and risks for cancer. Billy, even more so than his healthy peers, must maintain his health and actively prevent further health issues so that he can live his best possible life.
Billy has big plans for his future – racecar driver, policeman, or engineer. He’s not sure yet, but he’s already thinking of adult things. Just the other day he asked: “Are there wheelchair ramps at college?” It was his first question of the day asked as he peeked through a pile of 20 stuffed animals he pretends are ninjas and calls his “lovies.”
He may not realize that maintaining the best possible health will be key for his success as a racecar driver, policeman, or engineer, but he is already building the skills he will need to transition to adult medical care. He signs himself in for medical appointments. When he leaves physical therapy he makes sure that Janet knows he will be back next Wednesday at 4. He takes his morning medicines from a Sunday through Saturday pillbox – and knows to wait one hour before eating his biscuit and drinking his chocolate milk.
Billy hasn’t started asking around for recommendations for good adult doctors, but he does know how to set his Nintendo GameBoy aside and look his current pediatric doctors in the eye to ask and answer questions. His doctors ask his permission before sharing his medical history with their medical students. Billy expects the respect.
He gives his doctors updated copies of his medical summary listing current medications, past surgeries, and medical contacts. Billy is beginning to grasp his medical baselines, including: (1) normal body temperature, (2) normal respiration, (3) normal elimination habits, and (4) normal skin quality. Sometimes he uses this information for good – naming medicines that cause him hives – and sometimes for trick – faking a “temp” with the digital thermometer to get out of school.
Billy lacks a few things for his transition of care, including: (1) insurance card in pocket at all times, (2) complete medical record, (3) written transition plan (by age 14), and (4) sexual health education. But considering his young age, he is on his way to becoming an empowered adult patient.
Don’t expect to find Billy driving a minivan when he takes on the adult world, he recently declared: “I don’t want to drive a minivan when I grow up, a minivan is only so-so cool.” He raised an eyebrow and tilted his hand in seesaw fashion for the “so-so” which he clearly felt was a polite exaggeration of the minivan’s zero coolness. With his angel’s smile and politician’s wiles – will adult medical care be ready for Billy?
By Catherine Calhoun, with many thanks to Al Condeluci, Patti Hackett, Dr. Nadja Peter and my Gotham Writers Workshop friends led by Jonathan Mandell who were extremely helpful to me in putting this essay together.
For more posts celebrating May 1 as a global day of blogging power, see BADD.
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Living with a rare disease is a rare challenge for which none is prepared nor people around know what to do or what to say. So mostly when trying first, people do fail. It is not relevant that they fail, it is the thing that failing means the other gets emotionally concerned. And then mostly none is there to talk about it because rare disease people don´t live in the same house.
The most challenge is, that we have to climb high mountains and when maybe winning, as a teenager or a young adult, which could be getting back to quite normal after many struggles, we have to climb up mountains again although we have been there already!
Sometimes people don´t see when rare disease people show up that they don´t need to be tested again if they´re strong enough for life. It is energy wasting for them to go through all these struggles but this is life. There is no other planet for those who made it as young ones, this kind of winning is not visible.
And this is more than a pitty!
Living myself with a rare disease made me run throu´ all those tests and there was no adequate result for me.
It started with the thing, that I did not do the education I wanted to, because nobody thought that it would be my best and so I ended up in a job which was OK but not the ideal thing, for which I am burning inside. I never really burned for something – all I burned for was getting back on my feet and when I reached this goal, I was kind-a lost afterwards, out there in life.
It is true, to ask oneself if the life out there will be ready for little Billy or any other child with a rare disease. And it is confusing that there are rules on earth which say that we´re not enough to be seen and picked-up to care about.
WHAT?
Yes, true.
I found something interesting in the Maslow´s pyramid: the way he set-up the rules in life is not the exact way it matches to our lives. If it shall work for us, two lines have to be switched and that would mean: turn the world around.
The big question is: Will a little crowd be able to turn the world around?
I hope so – one day.