A potentially crippling disease in which the slightest touch of the skin can cause intense pain is often misdiagnosed in children, needlessly leaving some young patients in wheelchairs or on crutches, pediatric experts said.
More is known about Complex Regional Pain Syndrome in adults but an apparent increase in the number of young patients is putting pressure on doctors to find ways to better identify and treat the rare disease, said Dr. Elliot Krane, a professor of pediatrics at Stanford University.
Primary care pediatricians often know little or nothing about the condition and may miss it because they deal with such a wide range of illnesses in young patients, experts told a conference on the excruciating condition.
Krane said the gathering marked the first time pediatric experts on the disease — formerly know as Reflex Sympathetic Dystrophy Syndrome — were meeting to pool their knowledge.
“It is one of these diseases where you have experts in pain management in kids across the country who all see a smattering of cases but none of us get a large enough experience to see what is going to work and what won’t,” said Krane, who is also the director of the pain management program at Lucile Packard Children’s Hospital at Stanford.
“The frustration is that the knowledge base in children is not evidence based — it is extremely anecdotal.”
The disease likely affects more than 200,000 Americans, including an unknown number of children, said Jim Broatch, the executive director of the Reflex Sympathetic Dystrophy Syndrome Association of America.
He added most research has focused on adults.
Krane said the condition often follows an injury such as a sprained ankle when nerves start firing pain messages to the brain. At some point, however, the nerve activity essentially takes on a life of its own and pain messages keep getting sent.
“The pain is intense, unbearable and crippling,” Krane said at the two-day conference ending on Thursday. “People cannot function with that kind of pain.”
Kids in wheelchairs
A key to treating the condition is intense physical therapy, and doctors often administrator nerve blocks to diminish pain so patients can work to reactivate the nervous system and use of a limb.
The pain often goes away with proper treatment but if left alone the disease can leave children in wheelchairs and with skin so hypersensitive that the simple task of putting on sock or shoe can trigger intense pain, Krane added.
Jon Levine, a professor of medicine at the University of California San Francisco, said doctors tend to misdiagnose the disease more in children in part because of the way the U.S. health-care system works.
While adults often head straight to a specialist more likely to be familiar with the condition, children rely on primary pediatricians who often misdiagnose a disease they know little about, Levine said.
This makes it critical to begin developing guidelines for standard of care treatment pediatricians can look to, Levine said. It is also important to educate doctors that this is a real problem, not a psychological one as many diagnose, he added.
“It is a real disease and these kids are in a lot of pain,” he said.
