Linda* and her husband, who live in Florida, are “trying to figure out a new name for retired,” she laughs. “I do a lot of volunteer work, so I’m what you’d call semiretired.”
For 30 years, Linda worked for Bell Atlantic (now Verizon). “I had a lot of management skills; I’d worked my way up,” she says. But everything changed when she manifested polymyositis, an autoimmune disease characterized by chronic inflammation of the muscles. “At age 51, this autoimmune disease struck without any warning,” she says. “I went from being a busy, active person to a quadriplegic.”
The first sign of polymyositis varies widely among those who get it. Linda’s first symptom was something seemingly innocuous, which quickly became a problem. “I started with hiccups, of all things,” she says. “Just uncontrollable hiccups all day long.”
Soon, she was unable to lift her morning cup of coffee, blow-dry her hair or lift herself out of a chair. “I truly was a quadriplegic. I could move [only] my fingers and my toes,” she says. “It was pretty awful.”
Polymyositis’ inflammation causes pain, weakness and fatigue. “All of my muscles got very weak,” Linda says. “It took the doctors not nearly as long [to diagnose me] as it does for many people. The disease usually hits people over 50.”
After a neurologist confirmed her diagnosis, Linda started taking “massive amounts” of Prednisone, a corticosteroid that works as an immunosuppressant. The drug reduced the inflammation for a while. “And then, what we call a ‘flare,’ or a relapse, occurred,” she says. “What happened was I started getting weaker. [The inflammation] started coming back.”
Linda’s MedPro home infusion nurse: “Potentially, she saved my life”
Linda’s doctor recommended an immunosuppressant known as Imuran and a drug called methotrexate. She also began IVIg therapy. “Up until about a month ago, I was on four medications,” she says. At first, she got her IVIg treatment through a nearby hospital. “It was a very cold environment,” she says. “You’re just a number … they didn’t do a great job.” At one point, two of her nurses disagreed completely about the proper administration of her treatment, so Linda had to correct one of them.
After she “got out of that,” Linda began receiving her IVIg infusions at her doctor’s office. All was well, until the office said it simply couldn’t handle the paperwork anymore. She looked into home infusion despite her doctor’s opposition, and found one company to try—but discovered that her costs went up enormously.
By this point, Linda had begun attending the Myositis Association of America’s annual patient conference. “When I first got this disease, I was your typical Type A, independent, assertive, aggressive businessperson,” she says. “‘Nothing gets me down; I can handle anything.’ I went to my first myositis conference probably a good seven years after I was first diagnosed.”
At that conference, Linda met a MedPro Rx representative and shared her home-infusion billing issues. “She said, ‘Let me see what I can do for you,’” Linda says. “They bent over backward to take over billing. The nurse who they sent to the house was so outstanding.” After receiving infusions twice monthly for years, Linda’s veins were a little tired—but her new nurse handled the problem with ease.
“They bent over backward to take over billing. The nurse who they sent to the house was so outstanding.”
What’s more, Linda had been sitting at home throughout her entire eight-hour infusion, two times per month. Her MedPro nurse helped her make the treatments portable, so the two could venture outside. “We would go to the beach; we would go shopping; I would go to meetings; we would go to lunch,” Linda says. “She was superb, but not only that, she was treating the whole patient.”
Linda’s nurse took her vitals every hour, and soon became concerned about Linda’s high blood pressure. “Finally, I went to my doctor, and he said, ‘This nurse is your friend,’” Linda says. “‘It’s time for you to go on medication.’ Potentially, she saved my life.”
When IVIg therapy became less effective for Linda, and it came time to stop infusing, she nearly grieved her nurse’s bimonthly visits. “But I still get together with her for lunch,” she says.
Journey to Johns Hopkins and a new medication
Linda’s doctor wasn’t sure why the IVIg therapy was becoming less effective against her inflammation. IVIg is a well-studied treatment for polymyositis. “My doctor, who had been treating me for about eight years, was stumped,” Linda says.
She turned to the Johns Hopkins Myositis Center in her home state of Maryland, traveling there three times in 2014 for evaluation and treatment. “Each time, it was for kind of a different aspect of what we were doing,” she says. “They said, ‘Something’s really wrong here.’ Johns Hopkins determined that I have a really rare version of polymyositis. It’s considered to be practically untreatable, but they said, ‘There are things we should try.’”
At Johns Hopkins, Linda’s doctors took her off methotrexate and increased her Imuran dose. Then they took her off Imuran, because among its side effects is an increased risk of nonmelanoma skin cancer—cancer for which Linda is now being treated. “And then, they said, ‘It’s time to take you off IVIg, because we don’t know if it’s helping you,’” Linda says.
Linda is currently infusing Rituxan, an intravenous medication often used to treat non-Hodgkin’s lymphoma and rheumatoid arthritis. “It’s a really weird dosing [schedule],” she says. “It was about a four-hour infusion. It has to be done in a medical facility with doctors present.”
Two weeks after their first Rituxan infusion, patients receive an identical dose. Then, the medication is allowed six months to work. Linda saw no improvement by her four-month checkup. “At about the five-month point, I noticed I was getting stronger,” she says. She’s regained some of her strength, and is able to go about her daily life.
Linda is now taking Rituxan and Prednisone for polymyositis. “The other medications that I take are both for my lungs, because you don’t think of your breathing as being a muscular action, but it is,” she explains. “I started coughing about 10 years ago, and I have coughed every day of my life since then.” She uses an “airway clearance” vibrating vest to help rid her lungs of blockages twice daily.
Finding personal support for myositis
Linda’s first myositis conference yielded not just a connection to MedPro, but a new focus and passion. “The speaker said, ‘We really believe in the power of support groups,’” she remembers. When Linda learned that her area of Florida lacked a “Keep In Touch” group, or KIT, “Somehow or another, my hand was in the air, saying, ‘I’ll start one!’” she says.
She and another woman with myositis founded their myositis support group in 2007 with three members. “We are now the largest in the country, with over 170 people along about a 140-mile stretch through Florida,” she says. Linda has also taken on an executive role on the board of the Myositis Association, helping organize the group’s quarterly gatherings.
“We are now the largest in the country, with over 170 people along about a 140-mile stretch through Florida,”
Four times a year, myositis patients—and their partners—come together in person to learn about the latest myositis research, share their experiences and catch up with each other. Those who can’t attend in person still keep in touch by phone or email. “That’s what has kept me going,” Linda says. “People with diseases like this need to know they’re not alone. They can choose to live alone, and not go to all these meetings, but support groups have such important value.”
Patients with myositis can find and join the support group nearest them, or start their own chapter, on the Myositis Association website.