Debilitating Affliction Wreaks Pain and Wrecks Lives

Mary Anne Nelson went through dozens of doctors, surgeries and other painful procedures trying to discover--and stop--whatever began ravaging her legs after a skateboard ride left her with a sore foot when she was 14.

Most were baffled, some of their treatments made things worse, and many tried to persuade her parents that she was imagining the whole thing.

Today, at 37, Nelson is mostly paralyzed from the waist down by a debilitating affliction called Complex Regional Pain Syndrome.

Often triggered by a minor nerve injury, and sometimes with no obvious source, the syndrome causes horrible pain, swelling, skin sensitivity, sweating, and discoloration and skin breakdown as circulation deteriorates.

Nelson, who lives in Montville, recalls one awful day in 1984 when, with her left leg a swollen purple blob, she said to her doctor: “Why can’t you just amputate it?”

The doctor wouldn’t do it, but William Swanson’s did 3 1/2 years ago after he sprained his right ankle in a fall at work and his ordeal with the syndrome began.

At Swanson’s insistence, a surgeon cut off the leg just below the knee after it turned purple. Pain in the amputated leg gradually faded, but after an 18-month respite during which Swanson was finally able to walk with a prosthetic, severe pain returned in the stump and it turned purple again.

“So I’m right back where I was five years ago, still in a wheelchair,” said Swanson, who lives in Barnegat.

He has endured repeated medical complications caused by heavy medications for pain and for depression brought on when his wife left him because of his health problems.

While Nelson and Swanson pray for minor improvement, doctors and therapists who treat patients with CRPS--previously called Reflex Sympathetic Dystrophy--say 95% of those diagnosed quickly are promptly cured or made better.

“Up to three to four years ago, it was grossly, grossly under-diagnosed,” said Dr. Wen-Hsien Wu, director of the Pain Management Center at the University of Medicine and Dentistry of New Jersey Medical School in Newark.

Physicians have written about patients with such symptoms dating at least as far back as the Civil War, said Dr. James N. Campbell, professor of neurosurgery at Johns Hopkins University School of Medicine in Baltimore.

Wu’s clinic, one of the nation’s first to specialize in the syndrome, sees patients with advanced and complicated cases. The center focuses on “reducing enough of the pain so they can go on living.”

Treatments include painkillers and muscle relaxers, physical therapy, injections of nerve blockers, morphine pumps implanted in or near the spinal column, and electrodes threaded near the spine to block the pain.

Wu describes the syndrome as “a vicious cycle” that begins with a perhaps unnoticed “tiny nerve injury” amplified when the spinal cord, as if on autopilot, rekindles the pain signal in the limb’s sympathetic nerves so that nearby areas are oversensitized.

The sympathetic nervous system controls involuntary activities of the glands and organs, including sweating, blood flow, heart rate, bowel and bladder functions, and skin reactions such as goose bumps.

Over time, hypersensitivity at the injury site can spread via the spinal cord to other body parts. Hands and feet are most often affected, with patients often unable to wear long pants or shirts, socks or gloves.

Many build “tents” with their bedding to keep sheets off affected limbs so sensitive to the touch that even a gentle breeze can cause excruciating pain, said Nancy Just, director of psychological services at Wu’s center.

Just is directing a study in which some patients will be injected with a drug to stop muscle spasms by blocking nerve signals. That should allow enough pain relief for patients to participate in physical therapy, she said.

Campbell isn’t convinced.

“It may be that it helps some people in ways that are useful, [but] I don’t see this as a big breakthrough,” he said.

Despite the discouraging prognosis facing them, Nelson and Swanson hope for brighter days.

Swanson, happy with a new girlfriend, said his medications allow him to do household chores and some volunteer work.

Nelson regained some use of her legs through rehabilitation. She’s waiting for new leg braces that may enable her to walk with crutches.