BALTIMORE – From the time he was a teen, Kevin Dooley was drawn to the mountains.
"It's been the focus of almost all of my vacations during my life."
But recently, steep climbs became difficult.
At first, Kevin thought it was normal aging. That is until his hands lost strength.
"When I go to pump gas sometimes I would have to use two hands on the lever to squeeze hard enough," he recalled.
When his doctor couldn't tell him what was wrong, this Harvard-educated ophthalmologist did his own research online.
He didn't like what he found. A specialist confirmed he had inclusion-body myositis, or I-B-M.
"No, I'd never heard of it. Even though I'm a doctor it was totally unknown to me."
Dr. Thomas Lloyd is co-director of the Johns Hopkins Myositis Center. With I-B-M, muscles in the thighs and forearms degenerate. About half of all patients have severe difficulty swallowing.
"They either will not be able to eat and take in adequate nutrition, or oftentimes, even aspirate," explained Dr. Lloyd.
Doctors don't know what causes I-B-M.
- The disease strikes mostly men, but women can also be affected
- The onset typically occurs after the age of 50
- The symptoms that occur are weaknesses of the wrists and fingers, muscles in front of the thigh, and muscles that lift the front of the foot
There's no cure, but Dr. Lloyd says researchers are testing promising therapies that target the muscles.
"On the one hand, stimulating regeneration, and on the other hand, drugs designed to actually slow muscle breakdown," said Dr. Lloyd.
Researchers supported by MDA are studying the causes of inflammatory myopathies. One research team studying the mechanisms of muscle destruction in IBM-affected muscle fibers is building on recent observations that two proteins are abnormally elevated in these fibers. One is called myostatin, which limits muscle growth; and the other is called NF kappa B, which is known to play a role in inflammation.
Other MDA researchers are studying inflammatory myopathies in dogs, with the goal of developing new tools for the diagnosis and treatment of these diseases in humans.
Kevin says he's thankful this condition progresses very slowly. For now, he's still able to enjoy the outdoors and hike on even ground.
"It's great cause I can still do something I love."
TREATMENT: There has been no actual treatment for IBM. Early reports recognized the failure of patients to respond to steroids, methotrexate, azathioprine, and cyclophosphamide. Muscle biopsy is performed to diagnose the condition. Severe dysphagia, which is a symptom of having trouble swallowing, can require placement of a gastrostomy tube. Depending on the severity of weakness, physical therapy comes to play. For severe dysphagia, the treatment may have to include botulinum toxin injections (Botox), and cricopharyngeal myotomy (surgical sectioning of the upper esophageal sphincter). There is a debate on whether physical exercise can aggregate inflammation and increase muscle breakdown. Studies have shown that exercises can be instituted safely. (Source: http://emedicine.medscape.com/article/1172746-treatment#d9)
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