Arthritis: Not just for grown-ups
When people hear arthritis, older people immediately come to mind. But juvenile rheumatoid arthritis (JRA), an auto-immune disease, affects one out of every 500 children, making it among the most prevalent chronic childhood disease in the United States.
JRA's cause is not fully understood. "Children can be genetically susceptible or develop inflammation in their joints when they encounter certain infections or environmental triggers," says Robert Sundel, MD, director of Children's Hospital Boston's Rheumatology Program, which sees more than 5,000 JRA patients annually, ranging in age from infancy to adults in their mid-30s.
"Pediatricians should refer patients to a rheumatologist as soon as JRA is suspected," Dr. Sundel says. "Within six months of onset, damage to the joints can begin, so it's better for the patients to be seen by a specialist immediately. Through increased early recognition and aggressive treatment, children are doing much better than in years past."
One such child is 8-year-old Ezra Altabet, who was diagnosed with JRA at age 4. "He was a very healthy child," says his mother, Lisa. "His knees hurt, and he had a fever, but there were no symptoms of arthritis." After multiple tests, there was no explanation for Ezra's constant crying. "Two and a half months later, he was walking awkwardly and couldn't move his neck, jaw or wrists," Lisa explains. "I had learned about JRA during all of his tests, and I thought that might be the problem."
Musculoskeletal complaints, which are the reason behind 20 percent of visits to pediatricians, are one of the first signs of JRA. "Pain is not usually indicative of JRA," Dr. Sundel says. "Having morning stiffness for at least 30 minutes and/or moving like an old person are symptoms of JRA and reasons for referral."
Ezra was put on the steroid prednisone for two and a half years, which improved his walking and increased the mobility of his hands. Unfortunately, it also caused intolerable side effects, including tremendous weight gain and bad behavior. When he came to Children's at age 6, he began treatment with infliximab and thalidomide.
"You have to be more afraid of the disease than the medication," says Lisa. "So we're working with Dr. Sundel to treat Ezra's JRA aggressively." Once beginning this more aggressive, yet experimental approach, Dr. Sundel was able to eliminate prednisone from Ezra's treatment. He currently takes eight pills a day, comes to Children's every five weeks for an IV infusion of infliximab and attends clinic as needed, usually every three to four months.
"His quality of life has been significantly improved," says Dr. Sundel. "And while 40 to 50 percent of children with JRA eventually develop chronic damage to their joints, Ezra has avoided the long-term effects of both the JRA and the medications so far."
"JRA is a part of Ezra, but it's not who he is," Lisa says. "It affects our family's life, but it's our version of Žnormal'."
Twenty percent of children outgrow JRA, but most will have to be followed by a specialist indefinitely. "Progress in pediatric rheumatology is moving faster than ever before," Dr. Sundel says.
The hospital currently participates in three national studies of new medications for JRA: thalidomide, anakinra and a new medication based on anti-inflammatory proteins made by living cells. A dozen Children's JRA patients are prescribed at least one of the medications in these studies.
"Medications like the ones we're testing at Children's are making wheelchairs for children with arthritis a thing of the past," says Dr. Sundell. "They're hopefully paving the way for actually curing this formerly devastating disease."