With a celebrity guitarist as their new front man, doctors at the Johns Hopkins Myositis Center are hopeful they will be able to raise awareness about and research funding for a rare and misunderstood condition that causes muscle weakness.
Peter Frampton told Rolling Stone he has inclusion body myositis, a muscle-wasting condition that causes weakness in the legs, forearms and fingers. Known as IBM, the disease will eventually rob his fingers of the dexterity needed to play guitar, so Frampton is planning a farewell tour while he can still shred.
As with other IBM patients, there is no way to know how quickly or in what manner Frampton’s condition will progress.
“That’s one of the most frustrating things for a physician is our lack of precision. We are embarking on an era of precision medicine, and every patient wants to know, ‘What will I look like in 20 years or two months or 10 days?’” said Dr. Lisa Christopher-Stine, director of the Johns Hopkins Myositis Center and Frampton’s physician. “And it is so variable.”
Christopher-Stine said she hopes Frampton’s name will draw awareness to the little-known disease and help doctors answer those questions by generating more funding for research on its origin and possible treatments.
“It is absolutely the kick-start that we often need to bring awareness as well as philanthropic funding,” Christopher-Stine said. “He and I spoke years ago about whether he would one day disclose his illness and potentially become a voice for this illness.”
To date, doctors do not know what causes IBM, which is one of several forms of myositis, a category of illnesses characterized by muscle inflammation and damage. Other myositis conditions include dermamyositis, juvenile myositis and polymyositis.
Unlike its sister conditions, IBM exhibits qualities of both autoimmune and degenerative diseases. The “inclusion bodies” present in IBM patients are small abnormal particles that appear in cells.
“In IBM there is a combination of really intense inflammation as well as all of these degenerative features like inclusion,” said Dr. Tom Lloyd, co-director of the Hopkins myositis center. “And so one of the real questions in the field is, is IBM an autoimmune muscle disease, where the actual immune system is attacking the muscle — which is what we see in other forms of myositis — or is it really primarily a degenerative disease, which is thought to be the case in ALS and Alzheimer’s disease?”
The rare disease affects an estimated 20,000 people in the United States, and it’s twice as common in men than women. IBM is most common in people over age 50, so its symptoms are often mistaken for signs of aging, which can delay diagnoses.
“Many times patients tell us, ‘I just thought I was getting older,’ ” Christopher-Stine said. “And while certainly aging can contribute to some loss of function and some modest decrease, when something doesn’t feel right to you, it probably isn’t. And IBM is in that sphere.”
Frampton told Rolling Stone he became worried after several falls on stage, and he sought medical advice when he noticed difficulty lifting items onto airplane overhead compartments. Christopher-Stine said falls, loss of finger strength and trouble rising from chairs are the most common symptoms that cause patients to seek care.
“What is most dramatic — and it seems so unfair — is that this particular inflammation affects not only the quadriceps, the legs ... it affects the finger flexors,” Christopher-Stine said. “Most people don’t use their finger flexors in the way a professional guitarist would.”
IBM’s rarity makes it difficult to generate funding. But Christopher-Stine hopes Frampton’s outspokenness will change that. He launched the Peter Frampton Myositis Research Fund, a pool of money at Hopkins to which he will donate $1 for every ticket sold on the farewell tour.
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“For rare diseases you often do not have enough early patient data to further study,” Christopher-Stine said. “Grassroots fundraising efforts are invaluable.”
There is no known treatment for IBM; steroids and other anti-inflammatory drugs do not ease its symptoms. But physical activity, including exercise, physical therapy and occupational therapy, can slow its progression.
The Johns Hopkins Myositis Center is participating in two clinical trials for possible IBM treatments. One international study is examining the effects of arimoclomol, an oral drug that is not yet approved by the U.S. Food and Drug Administration. And a second, smaller trial is looking at the benefits of pioglitazone, which is already on the market.