Peter Frampton, a name synonymous with iconic rock anthems like “Show Me the Way” and “Baby, I Love Your Way” from his groundbreaking album Frampton Comes Alive!, has been captivating audiences for decades. However, beyond the stage lights and roaring crowds, Frampton has been facing a personal challenge: Inclusion Body Myositis (IBM). For fans asking, What Is Wrong With Peter Frampton?, the answer lies in this progressive muscle disease, and his inspiring response to it.
The Subtle Signs and the Diagnosis Journey
It was a seemingly ordinary hiking trip with his son Julian about ten years ago that first hinted at something being amiss for Frampton. While enjoying a father-son weekend in Big Sur, California, a simple uphill climb revealed an unusual fatigue. “It was a father-son rustic weekend,” Frampton recounted. “We took our guitars, we jammed, I wrote a song, and we went hiking in this beautiful area.” Recalling the moment on the trail, he noticed Julian’s effortless pace compared to his own struggle. “When I got to the top, I looked at him and said, ‘You know, Julian, I’m finding this more difficult than usual.'”
Initially, Frampton, then 60, attributed this fatigue to the natural wear and tear of a long and illustrious career. His journey through music history includes stints with bands like The Herd and Humble Pie, collaborations with legends such as George Harrison and David Bowie, extensive tours with renowned artists, and even a Grammy Award for his instrumental album Fingerprints. However, the subtle signs persisted.
Years after that hike, Frampton noticed difficulty running while tossing a Frisbee backstage. He dismissed these occurrences for two years, until two stage falls within three weeks in 2015 became a stark wake-up call. “I got up and we all laughed, but it was humiliating,” he admitted, emphasizing the sobering impact of these incidents.
These events prompted Frampton to seek a checkup in Nashville. His primary care physician immediately observed a significant 10-pound weight loss since their last meeting, triggering further investigation. Referred to a neurologist, Frampton underwent a series of tests. One test, involving gripping and resisting the doctor’s attempt to pry his fingers open, revealed unexpected weakness. Another test, challenging him to jump on each leg, highlighted a distinct disparity in strength between his right and left legs.
Following lab tests, the neurologist delivered the diagnosis. Initially suspected to be Amyotrophic Lateral Sclerosis (ALS), the diagnosis was ultimately Inclusion Body Myositis (IBM). While sharing similarities with ALS as a progressive neuromuscular disorder, IBM is not fatal. Further tests, including a muscle biopsy, confirmed the diagnosis, setting Frampton on a new path of understanding and managing his condition.
Understanding Inclusion Body Myositis (IBM)
Inclusion Body Myositis, or IBM, is a rare and progressive muscle disease characterized by muscle weakness and atrophy. Like ALS, it falls under the umbrella of neuromuscular disorders, but it progresses more slowly and is not considered life-threatening. Dr. Yadollah Harati, MD, FAAN, a neurology professor at Baylor College of Medicine, explains that IBM typically affects muscles asymmetrically, which explains why Frampton’s neurologist tested for strength imbalances between his legs. The weight loss Frampton experienced was also attributed to muscle atrophy, a hallmark of IBM.
The muscles most commonly affected by IBM include the quadriceps, crucial for knee stability and walking, and finger flexors, essential for hand dexterity. Weakness in quadriceps can lead to falls, often prompting medical consultation. For a guitarist like Frampton, the impact on finger flexors is particularly concerning. Dr. Harati notes that tasks requiring grip strength, such as using tools or even turning a doorknob, can become increasingly difficult.
Swallowing difficulties affect about a third of IBM patients, though Frampton has not experienced this symptom. Currently, there is no cure or specific treatment for IBM, aside from surgery to address swallowing issues if they arise. Dr. Harati emphasizes that IBM is ultimately a debilitating condition.
IBM is considered rare, affecting approximately 30,000 Americans. However, Dr. Harati believes it may be more prevalent than diagnosed, often misattributed to aging or other muscle disorders. Diagnosis delays of five to eight years are common, as people may mistakenly believe muscle weakness is a normal part of aging. Frampton himself emphasizes, “People think becoming significantly weaker with age is normal. It’s not.”
The term “sporadic inclusion body myositis” (sIBM) is often used by neurologists to distinguish it from the rarer familial form (fIBM). The cause of IBM remains unclear, with ongoing debate about its origins. The term “myositis” indicates muscle inflammation, while “inclusion body” refers to abnormal protein clumps found within muscle cells. While inflammation suggests an autoimmune component, typical autoimmune treatments are largely ineffective against IBM. Recent research points towards mitochondrial dysfunction as a potential cause of sIBM, but FDA-approved therapies for mitochondrial disease are lacking.
Frampton’s Resilience: Music, Exercise, and Advocacy
Despite the challenges of IBM, Peter Frampton has adopted a remarkably positive and proactive approach. He likens his diagnosis to other life obstacles he has overcome, stating, “I’ve had ups and downs throughout my life… I knock the dust off and get myself back up.” Frampton, who has embraced sobriety, emphasizes his pragmatic and positive mindset, viewing IBM as “life-altering” rather than “life-ending.”
Remarkably, Frampton’s guitar playing remains largely unaffected. Dr. Lisa Christopher-Stine, director of the Johns Hopkins Myositis Center, initially expected finger flexor weakness in Frampton, but was surprised to find his guitar skills undiminished. She noted, “He was disarming, genuine, charming, and a lovely human being from the first moment. It took about a minute to feel like I was speaking to an old friend.” Clinically, she recognized typical IBM traits in Frampton, but his exceptional finger dexterity challenged assumptions about the disease’s progression.
Frampton maintains a rigorous exercise routine six days a week, incorporating treadmill, stationary bike, lunges, and strength training via Zoom sessions. He believes activity is crucial for maintaining function, a view supported by neurologists. Dr. Conrad C. Weihl, a neurology professor at Washington University School of Medicine, advises exercise for IBM patients, although emphasizing enjoyable activities over prescribed programs. However, moderation is key, as Dr. Harati cautions against overexertion, which could potentially harm muscle tissue.
Facing a progressive disease, Frampton has prioritized performing and recording as much as possible. He embarked on a farewell tour in 2019 and publicly announced his IBM diagnosis, raising significant awareness. He established the Peter Frampton Myositis Research Fund at Johns Hopkins, channeling tour proceeds and donations towards research. Frampton now considers IBM advocacy his “new vocation,” committed to raising funds and awareness. He participated in a clinical trial for the diabetes drug pioglitazone, hoping to contribute to future treatments, regardless of personal benefit.
Frampton’s openness has resonated deeply within the IBM community. At his concerts, he invited audience members with IBM backstage, forging connections and offering support. One man recounted how Frampton’s CBS interview led to his own diagnosis, highlighting the power of Frampton’s public advocacy.
The Future of IBM Research
While current IBM treatments are limited, the field of research is advancing. Dr. Conrad C. Weihl notes, “this is an exciting period in IBM research, and Peter Frampton is increasing awareness just at a time when I believe therapies will emerge.” Research is focusing on areas like cytotoxic T cells, potential drug therapies, and understanding disease progression. Clinical trials are ongoing, exploring drugs like arimoclomol and rapamycin, and investigating new approaches like targeting specific T-cells. Pioglitazone, the drug Frampton is trialing, is being studied for its potential to improve mitochondrial function, a key area of IBM research. Dr. Bruce H. Cohen suggests that insights gained from IBM research could even benefit other conditions like diabetes and Parkinson’s disease.
Peter Frampton’s willingness to share his journey with IBM has been transformative, bringing much-needed attention to this rare disease. His music continues to inspire, and his advocacy offers hope and community to those affected by IBM. While what is wrong with Peter Frampton is a serious condition, his response exemplifies resilience, determination, and a continued passion for life and music.
Research by Thak Ka from the Noun Project
The State of IBM Research
No effective treatment for inclusion body myositis (IBM), a neurologic disorder that rock star Peter Frampton was diagnosed with in 2015, has been found so far, and several drugs have proved disappointing in clinical trials. “But this is an exciting period in IBM research, and Peter Frampton is increasing awareness just at a time when I believe therapies will emerge,” says Conrad C. Weihl, MD, PhD, professor of neurology at Washington University School of Medicine in St. Louis.
In one area of research, neurologists are investigating the role of cytotoxic T cells. Sometimes called killer cells, cytotoxic T cells are responsible for attacking and destroying other cells in the body. Usually they target invaders such as infectious agents, but in autoimmune diseases, cytotoxic T cells can attack healthy tissue.
“One potential approach to treating IBM might be targeting the specific population of T cells involved,” says Steven A. Greenberg, MD, professor of neurology at Harvard Medical School and associate neurologist at Brigham and Women’s Hospital in Boston. As cytotoxic T cells become more evolved and differentiated over time, they also become more potent. “Targeting highly differentiated T cells that have been around in the body for years honing their attacks, getting stronger, and dividing more might be a way to treat IBM,” Dr. Greenberg says. He chairs a medical advisory board for a company that is preparing to begin clinical trials of a drug that binds to select cytotoxic T cells and destroys them without depleting helpful T cells.
Other trials are continuing or have been proposed for drugs that have failed to meet target goals in previous research. These include arimoclomol, a drug that prevents the abnormal clumping or folding of proteins that is thought to cause muscle damage in IBM, and rapamycin, an immunosuppressant given to kidney transplant patients. The drug bimagrumab, which binds to receptors along a molecular pathway shown to have potential muscle-sparing activity, generated early excitement but performed poorly in clinical trials. “Either the drug is ineffective or we don’t have a good grasp of the right end points to study,” says Lisa Christopher-Stine, MD, associate professor of medicine and neurology at Johns Hopkins Medicine and director of the Johns Hopkins Myositis Center in Baltimore.
Before the COVID-19 pandemic, Frampton had enrolled in a trial of pioglitazone, a diabetes medication that increases production of an enzyme linked to improved mitochondrial function, which contributes to energy in cells and that is impaired in people with IBM. “This is a pilot study to see if we can detect beneficial signals in blood tests or muscle biopsies,” says Dr. Christopher-Stine. “The answer might not be this drug but another that’s developed based on successfully targeted pathways we see—such as increasing PGC-1, a key regulator of energy metabolism that may protect skeletal muscle from atrophy.”
Pioglitazone is one of many drugs that increase PGC-1, says Bruce H. Cohen, MD, FAAN, director of the brain, muscle, and mitochondrial research program at the Children’s Hospital Medical Center of Akron, OH. “Exercise is another potent way to increase PGC-1 quickly, but you need to keep exercising,” he adds. Unlocking clues in IBM may unlock clues for treating diabetes, Parkinson’s disease, some dementias, and even cancer, Dr. Cohen says.
IBM’s rarity can challenge researchers recruiting patients for clinical trials. “We need to know more about how patients progress and what they consider meaningful,” Dr. Weihl says. To find current trials, search “inclusion body myositis” at clinicaltrials.gov.
