Living with an 'orphan' disease: Is there hope for Hannah?
Seven years ago, an assignment editor at my former employer WJZ-TV in Baltimore handed me a news release from Best Buy. It announced that it was giving a young girl living with a disability a customized pink computer. The story had undertones of a publicity stunt, and I was skeptical. But by the end of the day, I understood why workers at that store went the extra mile for a marvelous young girl.
Hannah Brammer’s laugh is completely infectious. When she spoke, workers at that Best Buy lit up with smiles. When she graciously thanked them for a computer in her favorite color, they fought back tears.
I had never before encountered someone living with osteogenesis imperfecta, more commonly known as brittle bone disease. It’s a genetic disorder, where bones fracture for little or no apparent reason. During her short life, Hannah has broken more than 126 bones.
Reuniting with Hannah for a segment airing Monday, I still imagined her as that little girl. But now she’s much like any other 13-year-old. Her bedroom is plastered with posters of celebrities. She loves to talk about shopping and doing her nails. She's constantly texting friends. Hannah confessed that she has dreams about boys, something her mother did not know until our interview.
But there was an uncomfortable moment during our latest chat. We were talking about her hopes for the future, and Hannah choked up. Now that Hannah’s a teenager, she may be coming to terms with certain things for the first time, her mother later explained.
Osteogenesis imperfecta, OI for short, does not have a cure. People living with the disease rely on drugs used to treat other diseases such as osteoporosis or bone cancer, which prevent or control symptoms. People with severe OI risk premature death, primarily from respiratory failure or accidental trauma.
It’s what doctors call an orphan disease. It affects too few people to make investing in a cure or treatments potentially profitable for drug makers. Who is going to make money on a pill or injection that only 25,000 to 50,000 Americans would buy?
Fortunately, OI research funding has grown greatly in the last several years – jumping from around $1 million a year to $12 million a year, according to the OI Foundation. But that amount of money pales in comparison to many other diseases, such as $24 million for headaches, and $450 million for alcoholism.
On top of funding research, the OI Foundation helps people affected by brittle bones pay for expenses that aren't covered by insurance. For example, Hannah’s mother Valerie recently needed a new van to transport Hannah's wheelchair. The OI Foundation stepped in to help.
Families affected by this disease face unimaginable expenses. Valerie told me she had to quit her job as a real estate agent after the state of Maryland stopped providing an in-home nurse for Hannah. The mother and daughter barely survive financially: Valerie has pawned her jewelry and dried up her personal savings account. They've nearly been evicted multiple times. She's fundraising online in hopes of buying Hannah a new manual wheelchair.
Valerie's dream is to see her daughter walk. People living with OI hold on to hopes that the rapid advancements we’re seeing in genetics will lead to a cure, since surgeries and medicines alone are not enough. Hopefully researchers make this discovery in time to help Hannah. I would love for that to be my next report on her.
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