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Expert Care Improves Rare Myositis

Gene Fox

November 05, 2019

Gene Fox thought he was just out of shape. The 62-year-old hospice chaplain admits he was overweight and rarely exercised. But when he tried to wash his hair on a May 2018 morning, he couldn't raise his arms high enough to do the job.

Gene and his wife, Rhonda, of Olathe, Kansas, were busy with 8 young grandchildren and were building a retirement home in Leavenworth County, Kansas. Chronic illness wasn't on their radar, and Gene initially dismissed the occurrence as an annoying, isolated event.

"But when it happened again, I knew something wasn't right," he recalls.

A downward spiral and search for answers

As Gene began to pay closer attention, he realized his limbs were becoming weaker and weaker. During the next few weeks, he began having difficulty standing from a seated position, getting in and out of his car, and even rolling over in bed.

"I just had no strength, to the point that I had to use the nightstand to slowly push myself into a sitting position to get out of bed in the morning," he says. Rhonda struggled to help him and eventually bought a mechanical lift to assist.

Working with medical professionals in his hospice chaplaincy, Gene mentioned his condition to a nurse colleague. Alarmed, she advised Gene to see his doctor right away. A call to his primary care physician and a description of his symptoms earned Gene a near-immediate appointment.

"I was so weak by then, I couldn't even walk on my own," Gene says. "Still, I just kept thinking it would get better by itself. At that point, I wasn't sure what to believe."

Gene's doctor advised him to stop taking his cholesterol-lowering statin medication. Although well tolerated by most people, statins can cause muscle pain and weakness in some patients, which generally improves by stopping the medication. But when this measure didn't reverse Gene's symptoms, his doctor referred him to a neurologist for additional assessment and testing.

"They ran every test under the sun," Gene says of his initial neurology appointments. The results did not reveal any common neurological disorders. Having ruled out the more obvious diagnoses, Gene's neurologist suspected myositis, a rare disease affecting only about 100,000 people in the United States.

"Then he pulled out his phone and said he was calling the man who could fix me," Gene says.

Expertise promotes hope

That man was Mazen Dimachkie, MD, a neuromuscular disease specialist with The University of Kansas Health System. An internationally recognized expert, he recently completed his 2nd term as a member of The Myositis Association's medical advisory board. Dedicated not only to treating but eventually curing neuromuscular disorders, Dr. Dimachkie serves as vice chairman for research programs for the University of Kansas Medical Center, a role he holds in tandem with his patient care focus at The University of Kansas Health System.

"We are unique in having a strong team of neuromuscular experts here at the health system," he says. "We've written numerous scientific articles about neuromuscular conditions, including myositis. This expertise helps us rapidly confirm diagnosis of rare conditions, such as in Gene's case."

Myositis is an autoimmune condition marked by severe muscle inflammation. The disease can take various forms, but common features are weakness of the proximal muscles, which are those situated near the torso, such as hips and shoulders. Patients who have myositis also often develop difficulty swallowing.

"Dr. Dimachkie examined me and said he agreed with my neurologist and suspected myositis. Then he said, 'I'm going to make you better.' I broke down and cried," Gene recalls.

Additional blood testing to identify a specific muscle enzyme that is elevated in people with myositis, and electromyography, a test that shows evidence of muscle disease, helped confirm the diagnosis.

"We also performed a muscle biopsy," Dr. Dimachkie says. "We have our own muscle pathology lab to process tissue samples, and I'm certified to perform these tests and read the results. It's a high caliber of service we can provide to our patients. By putting all these pieces together, we can make the proper diagnosis."

Intensive treatment for a rare disease

While Gene was thrilled to know he would get better, he had a long road ahead. Myositis doesn't resolve overnight, especially when it's a severe case like Gene's. The typical treatment is steroids, but "we treat patients with 2-3 different medications if they are profoundly weak," Dr. Dimachkie notes. For Gene, this meant about a week in the hospital receiving intravenous immunoglobulin (IVIG) in addition to the oral steroid prednisone.

IVIG is used for a number of neurological and autoimmune disorders. Immunoglobulin is present in blood plasma and contains antibodies that help strengthen the immune system. Because myositis is an autoimmune disease in which the body's immune system begins attacking muscle, IVIG can be helpful.

"I needed help with everything that first week in the hospital," Gene recalls. "I was really bummed out. I knew my situation was serious. But everyone was very professional, kind and respectful."

Gene's next stop was The University of Kansas Hospital Acute Rehabilitation Care, where he began physical and occupational therapy.

"The therapists were very attentive, even pushy, but in a good way," Gene says with a chuckle. After a couple of weeks, Gene moved to a rehab center near his home in Olathe, where he remained for 2 more months. "The goal was for me to be able to walk up and down stairs before I went home. And I was so ready to get out of there when they released me!"

Once home, Gene continued therapy with home health services. He follows up with Dr. Dimachkie every 3 months and is slowly regaining his strength. His last IVIG treatment was near the end of 2018, and he's down to just 10 milligrams of prednisone daily.

"I'm able to do stairs and take care of myself again," he says. "I don't have much stamina, but I'm improving. I'm back to work, and I'm very fortunate to have my faith and family to support me."

Dr. Dimachkie notes that while about 5% of myositis patients recover completely, most need continued treatment.

"We usually see significant improvement within a few months and continued recovery for months or years," he says. "We are pleased that our team here in the heartland has the expertise to help people locally and those who come to us from across the country. It's gratifying to see people become functional again and enjoy their lives."

Patient testimonial: As with all treatments, individual patient results vary. It is important to discuss your treatment options with your healthcare provider.

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