Evan Lau works out with Kristin Fortner, an occupational therapist, to improve his strength to battle Reflex Neurovascular Dystrophy at the Children's Institute in Squirrel Hill.
Morgan Hamby, 16, of Jeannette, has Reflex Neurovascular Dystrophy, which affects her right foot. With therapy she was able to return to dancing and practice for an appearance at Disney World with the Susan Yadamec Dance Company All-Stars.
By Kate Luce Angell
Whether it's called RND, RSD or CRPS, the syndrome is a parent's nightmare.
A few weeks after a minor injury, just when he or she should be all but healed, your child suddenly reports feeling terrible pain at the injury site.
The family doctor is baffled. X-rays and MRIs show no bone breaks, no reason for the pain. Pain relievers don't work. Your child can't attend school, can't stand clothing to touch the affected part, perhaps can't even walk and each day says the pain grows worse.
Jordan Mauck, 17, an RND sufferer from Woodbridge, Va., who underwent treatment for the syndrome at the Children's Institute of Pittsburgh, described what her RND was like:
"It was the worst pain I ever felt. I would beg my parents to do something about the pain, anything."
Reflex Neurovascular Dystrophy is becoming more common among young people, especially young women, and as a result physicians are growing more familiar with it. Children as young as 3 have been diagnosed with RND, although the syndrome is more likely to appear in teens and young adults. Girls are three times more likely to suffer it than boys.
But RND remains a puzzling syndrome that is imperfectly understood. Even its name is at issue: While RND is sometimes the diagnosis given to pediatric patients, those under 18 can also be diagnosed with Reflex Sympathetic Dystrophy or Complex Regional Pain Syndrome. Both diagnoses are also used for adults.
Regardless of the name, the symptoms in both children and adults are similar. A few weeks after a soft tissue or nerve injury, pain increases instead of disappearing. The affected part, usually an extremity like the foot, may swell, change color and exhibit altered hair or nail growth. The pain is often described as burning, yet the affected part feels cool to the touch.
If diagnosis is delayed or treatment is not effective, muscles around the affected part may atrophy, bone loss may occur, the skin may thin and the pain may extend to other parts of the body. After some time, these changes can become permanent.
Although doctors continue to research the syndrome, it is thought to involve an overreaction by the sympathetic nervous system to an injury. Blood vessels constrict, reducing blood flow to the affected part and increasing sensitivity to pain. After a few months, the pain mostly originates in the brain, not the nerves of the affected part, and becomes far more difficult to treat.
The good news for parents and children is that because their nervous systems are still growing, young people's chances of breaking the cycle and recovering from RND are higher than an adult's would be from RSD or CRPS.
Inpatient treatment here
The treatment for young people is different than for adults, including a large amount of physical therapy. The Children's Institute is one of only a few hospitals in the United States to offer an inpatient program for those with RND, treating patients as young as 6 and as old as 23 with methods that include five daily hours of physical and occupational therapy. An outpatient program for RND has also recently been introduced.
Beth Heuer, certified pediatric nurse practitioner and coordinator of the Institute's RND program, said the intensive physical therapy accomplished several goals.
"We want to interrupt the pain cycle, improve blood flow to the affected area," she said. "But our program is also very individualized. We tell dancers, 'Bring your shoes.' We tell sports players, 'Bring your soccer ball.' We look at what's important to them and try to get them back to that."
Ms. Heuer noted that RND had a significant psychological component that the program also addresses.
"People don't believe them, they think it's in their heads because they can't see the problem," she said. "And the nature of pain is that it is isolating and can cause anxiety and depression. So we teach coping skills."
One of the goals of the Children's Institute RND program is to reduce or eliminate the child's use of drugs to manage pain. Some pain management methods used by the program are biofeedback, often through video games, as well as breathing exercises and yoga.
According to Jordan and her parents, Dixie and David Mauck, this approach has allowed her to greatly reduce her dependence on medication.
In March 2010, Jordan injured her foot in a high school soccer game. Instead of healing, the pain spread to her knee and side.
Three months later, she was getting cortisone shots in her foot several times a week; by July, she was in a wheelchair. Her medication made it difficult for her to concentrate in school, so she stopped going.
At different times, Jordan said, her doctors had her on "Tylenol 3, Percocet, Vicodin, Nucynta, Dilantin, Lyrica, Cymbalta, and Neurontin." She even underwent a three-day course of anesthetic ketamine infusions, a treatment considered only in extreme cases and not covered by the Maucks' insurance.
But after three weeks at the Children's Institute, Jordan was out of her wheelchair and planning to return to soccer, although she added that her ordeal had distanced her from her peers.
"I've lost a lot of friends over this," she said.
The mother of another Children's Institute patient, Linda Lau of Gettysburg, noted that although her son Evan, 10, also arrived in a wheelchair, the therapists didn't let him stay in it, urging him into the pool for water exercises right away.
"They had him walking the second day," she said. "I couldn't believe it."
Evan's RND had progressed unusually quickly, from an injured thumb in a basketball game to his elbow and both feet just weeks later.
Although she said the family's experience has been difficult, Evan was lucky in that it was such a short time between the onset of his severe pain and the correct diagnosis and treatment.
"It wasn't long, but when your child is in pain, each second is a second too long," she said.
Other treatment programs
Physical therapy doesn't always bring relief to RND patients. Jack Kabazie, director of the Institute for Pain Medicine at West Penn Hospital, said that he sees many young patients with RND for whom conventional treatment has not brought results.
"The first thing is to make the diagnosis early, and aggressive physical therapy, if tolerated, is the mainstay," he said. "But if they've already done significant physical therapy, then we have other options we can offer."
Those options include nerve blocks and spinal cord stimulators. Both help prevent pain signals from reaching the brain. Dr. Kabazie cited a current patient, Morgan Hamby, 16, of Jeannette, who recently underwent a lumbar sympathetic block to help deal with pain she has suffered since injuring her right foot in a skating accident March 5.
A dancer since age 5, Morgan continued to practice even while recovering from her ankle sprain. But she noticed something odd about her injury, even as her pain increased.
"I couldn't control my foot -- It would twitch. And it would change colors. We called it my Technicolor dream foot," she said.
Both Morgan and her parents, Renee and Michael Hamby, wondered if the pain could be in her mind. But after an MRI report suggested RND, Morgan said she was relieved to find out her condition had a name.
"It really bugs me that no one knows about this, that even doctors aren't always familiar with it," she said.
When physical therapy and medication failed to stop the pain, she and her family met with Dr. Kabazie in June to consider their next step.
"It was good that it was only four months since the injury," said Dr. Kabazie. "We wanted to nip this in the bud."
He explained that the nerve block would interrupt the pain cycle, hopefully not only putting Morgan into "remission," but also giving physical therapy a better chance to work in the absence of constant pain.
On June 28, Morgan underwent the block, in which a patient has a powerful local anesthetic and anti-inflammatory injected through a needle into spinal nerves. Using a contrast dye and fluoroscopic equipment, the needle is guided to just the right area for relief.
Usually a series of injections is required. Morgan said she felt relief after the block, but that she still has bad days and "can't do what I used to do." She is scheduled for another block this month.
Mrs. Hamby added that while RND was not a diagnosis any parent would want for their child, she was glad they had learned what was causing Morgan's pain.
"When we got confirmation of her diagnosis, the relief on my daughter's face when someone understood what she was going through," she said. "Just to have someone say, 'I know you're not making this up' means a lot."
For more information on the Children's Institute program for RND, call 412-420-2400 or go to www.amazingkids.org. For more on the Institute for Pain Medicine at West Penn Hospital, call 877-284-2000 or go to www.wpahs.org.
For more information on RND, RSD and CRPS, go to the Reflex Sympathetic Dystrophy Syndrome Association at www.rsds.org or the American Chronic Pain Association at www.theacpa.org.
Kate Luce Angell is a Pittsburgh-based freelance writer.