RARE Daily

A Rare Disease Gets Rock Star Treatment

February 27, 2019

It’s not unusual for rock stars to have farewell tours—I can think of several who have had more than one. In most instances, though, they come without urgency.

That’s not the case for Peter Frampton.

The 68-year-old British rock star, who produced the iconic 1976 Frampton Comes Alive! album, recently announced he was embarking on a farewell tour because he’s been diagnosed with inclusion body myositis (IBM), a degenerative muscle disease. It’s not fatal, but it’s a devastating disease for a skilled guitarist. Among other effects, the condition will slowly rob him of his ability to play.

“When he was referred for IBM, my first thought was ‘Oh my gosh, it’s his fingers,’” Lisa Christopher-Stine, director of the Johns Hopkins Myositis Center, who is Frampton’s doctor. “Any disease is obviously something of a challenge, but I thought this was an unfortunate disease for him. The fact that it preferentially affects the finger flexors seems particularly cruel for a world-famous, phenomenal guitar player, which makes me want to work harder every day.”

Christopher-Stine, who is also a rheumatologist and associate professor of medicine and neurology, said Frampton is already drawing a lot of attention to a poorly understood and largely invisible rare disease because of his willingness to speak to the media about his condition.

In fact, the musician is using his fame to raise money for research. As part of his farewell tour that will extend to more than 40 cities in the United States and Canada and run from June to October, he is donating $1 from every ticket sold to The Peter Frampton Myositis Research Fund at Johns Hopkins. Fans, friends, and others have already begun donating to the fund.

IBM leads to slow, progressive weakness and wasting of the quadriceps and forearm muscles. People with the condition experience difficulty swallowing, have weakness in their fingers and wrist, and their forearms and thigh muscles will atrophy.

People often seek medical treatment because they experience difficulty getting out of a chair, problems walking, or recurrent falls. It is an autoimmune condition where a patient’s immune system attacks the muscles. Patients with IBM usually develop symptoms after age 50, although some patients develop symptoms as early as their thirties.

Because people suffering from the condition often attribute their symptoms to aging, it’s unclear what the actual prevalence of the condition. It can also be misdiagnosed as other degenerative muscle diseases. About 25,000 people in the United States are believed to have the condition.

While IBM is an autoimmune disease, it generally doesn’t respond to the use of steroids and other drugs that are used to dampen the immune system in other autoimmune disease or other forms of myositis, such as polymyositis or dermatomyositis. Right now, there is no available treatment. Christopher-Stine said exercise is the most under-recognized management form for IBM and other forms of myositis.

The funding from The Peter Frampton Myositis Research Fund will help boost the work being done at the Johns Hopkins Myositis Center. The center has a 2,000-patient myositis cohort that it is using for a longitudinal study. It has biosamples from these patients and is working to understand the aberrant immune response in these patients, as well as understanding the clinical features of the disease, and identify biomarkers.

She believes Frampton’s wiliness to go public about his condition will be a great help toward advancing research and awareness about IBM.

“I can’t begin to tell you how extraordinary it is and how generous it is on his part to reveal this,” she said. “With rare diagnoses, we are all struggling to gain recognition for them in the public sphere. His disclosure brings potential funding, awareness and support. Not every disease has a celebrity spokesperson for it, but when it does, it accelerates progress exponentially.”

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