POEMS Syndrome Patient Goes from Immobility to Recovery

POEMS syndrome patient Chukwuma Anyanwu and his physician, Claudia Paba-Prada, MD, and his nurse, Kathleen Finn, RN.

The vow Chukwuma Anyanwu made to himself shortly after the birth of his third child in June 2015 speaks volumes, not only about the dire state of his health at the time but also about the mettle with which he is made.

“My goal was to be able to walk before my son did,” the 42-year-old Hyde Park resident says, seated in a meeting room at Dana-Farber. The cane by his side – the successor to the wheelchair, walker, and crutches he once needed – is testimony to how far he has come toward that goal.

A month after his son’s birth, Anyanwu showed the first symptoms of a disease that would soon leave him bedridden, unable to move his legs because of muscle weakness and in unremitting pain in his arms and hands. The condition would eventually be identified as POEMS syndrome, an extremely rare disorder affecting multiple bodily systems. The name of the disease is an acronym of those symptoms: Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal gammopathy, and Skin abnormalities – or progressive weakness in the arms and legs, an enlarged liver and/or spleen, abnormalities of the endocrine glands, and darkening or thickening of the skin.

Anyanwu’s recovery has been so dramatic that he now works out at a gym every day, drives his car, and hopes to return to his job at a bank in a few weeks. He is quick to cite the treatment he underwent and the care he received at Dana-Farber/Brigham and Women’s Cancer Center (DF/BWCC) over the last year and a half. His physician at DF/BWCC, Claudia Paba-Prada, MD, marvels at his determination – the optimism with which he undertook his rehabilitation, and the sedulousness with which he follows his doctors’ advice.

“I’ve never seen a patient with such debilitating neuropathy,” says Paba-Prada, a specialist in blood diseases known as plasma disorders. Neuropathy, one of the hallmarks of POEMS syndrome, is a nerve disorder that can produce extreme muscle weakness, pain, numbness, and lack of coordination. “When we first saw Chuk, he was almost immobile; he couldn’t move his legs in order to turn in bed. He was in such severe pain that he required narcotics every two hours. But even then, I could tell how strong he was. He always thought he would get better, even when I couldn’t promise that he would.”

A Fall, Then Pain

The disorder that would radically constrict Anyanwu’s life for more than a year announced itself abruptly. Anyanwu recalls a day in August 2015 when he went to clean out a garage across the street from his home. “I noticed that I couldn’t move my legs the way I used to,” he remarks. “I couldn’t run across the road.”

He had been experiencing some minor leg pains, numbness, and tingling in his hands and legs, but nothing severe enough to hamper his movement. Later that day, he fell twice while mowing the lawn. Thinking the problem was due to exhaustion, he went home, showered, and drove to a hospital emergency room. There, and in later tests run by his primary care physician, no underlying health problems were discovered.

“He always thought he would get better, even when I couldn’t promise that he would.” -Anyanwu’s physician, Claudia Paba-Prada, MD.

Two weeks later, Anyanwu found himself unable to move his right leg. Referred to several different specialists, including a neurologist, he was diagnosed with Guillain-Barré syndrome, a rare disorder in which the body’s immune system attacks the nerves. He received an infusion of gamma globulin (antibodies) with no improvement in his symptoms. He was unable to stand. A visiting nurse checked him every day, but nothing relieved the burning, shooting pains.

By mid-October, the pain had become unbearable, and the medication to treat it made him light-headed. He was admitted to Brigham and Women’s Hospital, where a team of specialists including Paba-Prada ran a new series of tests, including spinal tap, magnetic resonance imaging, extensive blood work, and removal of a small piece of abdominal fat for examination.  He then went to a rehabilitation hospital for help in managing pain and to receive therapy.

The imaging tests showed sclerotic lesions – areas of abnormal bone – in Anyanwu’s pelvis, spine, and ribs. Laboratory tests showed a monoclonal protein and very high levels of the protein VGEF. A biopsy of the diseased bone marrow in his pelvis found evidence of a plasma cell neoplasm, in which the body overproduces a set of B cells called plasma cells, which are white blood cells that make antibodies against disease. Together, these results pointed to POEMS syndrome with demyelinating polyneuropathy – the erosion of the protective fatty sheath around the nerves and loss of nerve fibers. Doctors and scientists don’t know why the oversupply of certain B cells produces the range of symptoms associated with POEMS syndrome – particularly the nerve-related pain and weakness that Anyanwu experienced.

A Plan for Recovery

Shortly after Thanksgiving 2015, Anyanwu had 10 radiation therapy session to treat the lesions in his bones. He vividly recalls his condition at the time: “I was virtually paralyzed from my legs to my neck, and in severe pain.” The debility made everyday activities – including many central to his identity as a father and provider – all but impossible. He went on medical leave from his job as an operations analyst at a bank; his family moved from a third-floor residence to a ground-floor one, which had been modified for accessibility; he needed help eating and bathing; he moved about his home in a motorized wheelchair.

After receiving the chemotherapy drug Revlimid, Anyanwu slowly began to regain his strength.
After receiving the chemotherapy drug Revlimid, Anyanwu slowly began to regain his strength.

Following radiation treatment, he began receiving the chemotherapy drug Revlimid in combination with steroids. It would mark a turning point in his recovery.

He slowly began to regain his strength and his pain started to subside. By the end of January he was discharged from the rehabilitation facility and by June was able to walk using a walker. His response to the drug was encouraging, but its duration was anyone’s guess. “Dr. Paba-Prada explained that the best way to prevent a relapse is a stem cell transplant using my own cells,” Anyanwu relates.

The transplant took place in July, and the past year has been a time of steady gains in his strength and mobility, with one, very telling, exception. Three months after his transplant, Anyanwu was so excited to have recovered his ability to stand that when he tried to go from a chair to his bed, he fell and broke his ankle, a setback that required him to return to a wheelchair for a few weeks. “He was so apologetic about fracturing his leg, because he felt it would slow down his recovery,” Paba-Prada recalls.

Today, Anyanwu still shows some of the effects of the disorder – the need for a cane in walking, braces to help straighten some of his fingers, which became misshapen during his illness – but his progress is evident at each of his monthly checkups at DF/BWCC. He resumed driving in May, helps his wife, Benedicta, a Dana-Farber staff member, with their three children and does chores around the house. He is now preparing to return to his job at the bank, which held the position open for the two years he was incapacitated.

“He follows every instruction we give him,” says Paba-Prada. “He’s always excited to show us his progress. Every time he comes in now, he asks when he can return to work.”

While most patients with POEMS syndrome respond to treatment, the extent of their recoveries varies widely, Paba-Prada notes. Some recover fully, others don’t. Some have residual neuropathy. Anyanwu’s neuropathy continues, gradually, to recede.

Anyanwu remembers the lift given to his spirits when, after his diagnosis, Paba-Prada discussed the treatment.  “I asked if it would be curative. She couldn’t guarantee that, but she said it’s treatable and gave me hope that I’ll be fine. I thought of the saying, “Humans treat, but God cures.’”

He continues on physical and occupational therapy and tracks his progress meticulously. At the gym where he works out daily, he went from being able to lift 15 pounds to being able to lift 70 pounds – and more on some days. He’s gone from walking 300 steps at his six-month post-transplant checkup, to walking nearly 1,500 at his most recent one.

Of the persistence with which he’s worked toward recovery, he refers to the strength he’s gained from the ordeal, rather than the strength needed to endure it. “I’m stronger now than before this happened,” he remarks. “I’ve tried to be an example to others: Whatever situation you’re in, don’t give up. Don’t be angry – it doesn’t help.  Try to be resilient, have faith.”

Learn more about treatment for blood disorders at Dana-Farber.