Last week, David E. Bierer began an intense regimen that includes five hours of physical therapy every weekday, his best hope for a cure from a baffling illness.

He has often suffered severe pain during the past five years -- sometimes in his arms, sometimes in his joints or other parts of his body.

Steve Mellon/Post-Gazette
Ryan Whelan, 10, practices cross country at Hartwood Acres last weekend. Therapy helped the Hampton youngster overcome the pain from Reflex Neurovascular Dystrophy.

"It's been almost crippling," the 17-year-old from Parks Township in Armstrong County said.

He has had extensive medical tests to determine a cause and even tried acupuncture, which has provided some relief. But the pain has always come back, and doctors could link it to no physical cause.

Finally, in May, Dr. Paul Rosen, clinical director of rheumatology at Children's Hospital, determined that he had Reflex Neurovascular Dystrophy, a condition that causes children and adults to suffer severe, often disabling pain for reasons that are not entirely clear.

He prescribed an intensive therapy program at the Children's Institute, a pediatric rehabilitation facility in Squirrel Hill. He should complete the inpatient portion of the therapy this week, then receive follow-up outpatient care.

The inpatient program is among only a few in the nation designed to treat the illness.

About two to four children per 1,000 have the disorder, which also is known as Pain Amplification Syndrome, Complex Regional Pain Syndrome, Amplified Musculoskeletal Pain Syndrome and Reflex Sympathetic Dystrophy, said Dr. David Sherry, clinical director of rheumatology at Children's Hospital of Philadelphia.

Dr. Sherry developed the therapy regimen used at the Children's Institute and several other centers around the nation, building on the work of two other physicians, Virgil Hanson and Bram Bernstein.

Injury, stress may be causes

The pain caused by the disorder is real. Usually, it begins after an injury, and psychological stress often appears to play a role, Dr. Sherry said. Sometimes, affected areas can turn blue or red, suggesting that nerve pathways may be disrupted and may cause interference in blood flow, factors that could exacerbate the pain.

But a clear-cut cause has not been determined, he said, and the diagnosis is made from a patient's symptoms and by ruling out other causes.

Since the disorder is not well known, even among doctors, many young people, like David, spend years going from doctor to doctor and undergoing expensive medical tests, only to find that the pain fails to improve or even worsens.

"We never learned about this in medical school," said Dr. Rosen. "It's pain without a clear explanation."

Parents, too, sometimes have trouble grasping that the pain can exist even though medical tests are normal.

"How can everything be normal if my kid is dropping out of school, or dropping out of football, is socially isolated and unable to function?" is a typical reaction, Dr. Rosen said.

Dr. Sherry said he isn't sure why intensive physical therapy, continued over a period of weeks, seems to result in dramatic improvement in many children. He suggested that vigorous exercise and rubbing the affected areas over and over may desensitize them, much as massaging a scar often makes it less painful.

The therapy regimen, which lasts one to seven weeks, also calls for discontinuing pain medication and medical testing.

Adults' therapy harder

Dr. Sherry said he knew of no similar therapy program for adults, though some have used the techniques and have been helped by them. Dr. Rosen said adults with chronic pain generally respond less well to physical therapy alone and often need medication or other treatment.

Some children have the therapy as outpatients. After beginning treatment at Children's Hospital, Ryan J. Whelan, of Richland, continued therapy for months at home and at school.

Ryan, now 10 and in fourth grade at St. Ursula Catholic School in Hampton, has had a recovery so complete that he is now pain-free and able to compete on his school's cross-country team.

His pain began two years ago after he had a minor injury to his right knee at Hance Elementary School in the Pine-Richland School District. At the time, he was being bullied by two children, said his mother, Sharon A. Whelan.

The pain quickly became so great that he was taken to the emergency room, where doctors could find nothing wrong. He injured the knee again the following week and went back to the emergency room, where doctors referred the case to Dr. Rosen.

		More info A video on Reflex Neurovascular Dystrophy in children is available through the Childhood RND Educational Foundation, www.childhoodrnd.org. For more information, contact the Reflex Sympathetic Dystrophy Syndrome Association, at 1-877-662-7737 or visit www.rsds.org

At the time, even touching the knee would cause him to scream with pain, his mother recalled. The skin from the knee down to the ankle would change from bright red to blue, then back to normal color.

"Even if the wind would blow on Ryan's knee, he would cry," she said, noting that at that point, her son was using a wheelchair.

The tears continued during the therapy, which was three times a day for about seven months. His mother would pour dry rice or popcorn over his knee or rub sponges or brushes across the skin. Sometimes, his sister Reagan, now 8, would help him with his exercises. Over time, the pain subsided.

Though he ended up repeating third grade last year because he missed so much school, his mother said she was grateful that only weeks lapsed before the condition was diagnosed.

Dr. Sherry said about nine in 10 children treated with the therapy remain pain-free after five years, a better success rate than less intensive therapy regimens, medication or surgery.

The Children's Institute began its program in April 2004 and has cared for about 30 patients.

Expanding treatment

The Children's Institute has only one child in therapy at a time, and there is a waiting list. The institute plans to open an outpatient therapy program next year, said Dr. Jamie Calabrese, the facility's medical director.

One of those on the waiting list is Christina M. Brown, 11, of Morgantown, W.Va.

A sixth-grader, Christina used to swim and run on a cross-country team, but gave up those activities because of the pain.

"If anyone touches me, it hurts," she said -- so much, she even avoids embraces from her parents, Christopher R. and Mary O. Brown. "They know it will hurt me, so they just don't hug me at all."

"Once she gets better, the first thing she'll get is a big hug," her mother said.

Her problem began in April 2003, when she sprained her left ankle, her mother said. This year, she also began having pain in her neck, shoulders and back. She has had more than 60 physical therapy visits, yet the pain has often left her in tears.

A pediatrician referred Christina to Dr. Rosen, who first saw her and diagnosed the disorder this summer.

Unlike Christina, David Bierer never gave up some activities he enjoyed, including karate and playing his guitar. But he's had to scale back.

"I used to do karate three or four nights a week, two or three hours at a time," he said. "Now, I can't even stand for the whole class."

For David, a senior at Kiski Area High School, the pain began in October 2000. While waiting for the school bus, he complained of pain in two fingers on his left hand. Later that morning, he developed severe pain in his left arm, which was cold to the touch and blue from his fingertips to above his elbow.

After a battery of tests, emergency room doctors at Children's Hospital thought he might have a blood clot, said his mother, Patricia A. Bierer. Before long, he complained of pain in other parts of his body.

Sometimes, the pain was so great that he needed to rest. But that didn't help, said his father, John Bierer. The pain "would come back in a different area -- a knee, then an ankle, then a shoulder, ricocheting like a pinball machine."

Doctors appeared baffled by the condition, Mrs. Bierer said. After he developed wrist pain early this year, he went to several doctors, one of whom referred him to Children's Hospital. There, he saw Dr. Rosen, who diagnosed Reflex Neurovascular Dystrophy.

"After five years," she said, "we were grateful to have a diagnosis."

David hopes he will recover completely, like so many other children who have had the therapy.

"I'd like to get back to where I was."