Disorder puts patients in cycle of pain
BY RANDY GRIFFITH
It is a pain that has been compared to undergoing an amputation without anesthesia.
Characterized by chronic pain without apparent cause or specific treatment, reflex sympathetic dystrophy syndrome, or RSD, can strike at any age.
“For a while, I thought I was going to die,” said Marci Carlson of Southmont.
“There is a limit on how much pain your body can take, right?”
In five days, RSD took 11-year-old Lacey Berardi from having a limp to needing crutches, then a wheelchair, to being bedridden, said Vicki Walker, her mother.
“People who don’t have it can’t say they understand because they don’t,” Lacey, now 13, said at their Dormer Street home in Parkhill.
“It’s the worst thing you can imagine.”
Also called Complex Regional Pain Syndrome, or CRPS, the condition is considered the most severe of all chronic pain disorders. It also is one of the more difficult to diagnose, said Dr. David Carlson, Marci Carlson’s husband and chief medical officer for Conemaugh Health System.
“That’s one of the typical problems with CRPS,” he said. “It often gets diagnosed later, rather than sooner.”
Despite his years of experience in family medicine and hospital administration, David Carlson was frustrated in early efforts to help his wife identify and find help for her pain.
The disease strikes the sympathetic nervous system, also known as the fight or flight nervous system, which controls the body’s response to pain and emergency. It often follows an injury, but the pain is out of proportion to the damage, Carlson said.
“Injury sets up painful stimuli, but in RSD, the stimuli become abnormal,” Carlson said. “It sets up a cycle where it keeps getting worse and worse and worse. Normal things won’t make it go away.”
Marci Carlson’s pain started after her second elbow surgery.
“I knew something was very wrong,” she said.
“It kept getting worse and worse and worse.”
Doctors tried pain medicine and other tests, to no avail.
“Finally, I went to a neurologist,” Marci Carlson said. “He said, ‘I know exactly what’s wrong: You have RSD.’ ”
In some patients, skin becomes discolored or warm; in others, skin may become more sensitive, swell, or have changes in texture. But the key symptom is continuous, intense pain, according to the National Institute of Neurological Disorders and Stroke of the National Institutes of Health.
The syndrome originally was identified among Civil War soldiers recovering from horrific wounds on the battlefields of Gettysburg, David Carlson said – back then, it was called causalgia.
Lacey’s pain developed in her back and later was linked to repetitive stress injury from cheerleading. Doctors treated her for a bruised kidney, giving her strong doses of ibuprofen, blood-thinning pain medicine that actually caused her some kidney problems, her mother said.
“We went to the emergency room three times due to pain,” Walker said. “Every time, she left in the same condition she went in.”
In December 2005, Lacey entered Children’s Hospital of Pittsburgh. A battery of tests confirmed RSD.
“In medicine, we are used to making diagnoses with lab work or X-rays,” said Dr. Paul Rosen, clinical director of rheumatology at Children’s.
“There is no lab test or X-ray for RSD. It is a diagnosis of excluding all the other possibilities.”
Treatment is even more complicated and less precise. What works for one patient will not be effective for another, David Carlson said. Both Marci Carlson and Lacey were put on series of painkillers and nerve blockers.
“The theory is that if you stop this crazy loop of pain for a period of months, you can make it better,” Marci Carlson said. “There is remission.”
Other treatments include anti-seizure medicine and antidepressants, David Carlson said. An experimental treatment in Europe involves putting patients into comas for several days and hoping they wake up pain free.
“You are trying anything you can to interrupt the pain cycle,” he said.
Marci Carlson has found relief in technology that disrupts the nerve signals that create the pain. Surgeons inserted a spinal-cord stimulator, essentially an implanted electrode that targets the area of the spine connected to the source of the pain.
Lacey’s remission came after several weeks of intense exercise therapy at The Children’s Institute in Pittsburgh. The daily, drug-free regimen is so intense, parents aren’t allowed to watch.
“At first, I wanted to quit. It hurt so bad,” Lacey said. “It was really, really worth it. I’ve been pain free for a long time.”
Conemaugh and its John P. Murtha Neuroscience and Pain Institute have several programs for RSD in adults, David Carlson said. Interventional pain management specialists work closely with patients and other specialists to diagnose and treat the condition.
Because the condition affects so many areas, Carlson said the local hospital does not have the resources for serious RSD cases in children.
Rosen’s work is part of Children’s extensive pain management program, which is gaining recognition and patients from throughout the Eastern U.S., he said.
Lacey urges patients to stick with the treatment: “Don’t give up. It’s like my teacher says: You can’t say you are going on a diet and have someone else eat right and exercise for you. You have to do it yourself.”
When Lacey Berardi of Parkhill said her back hurt, her cheerleading coach thought she was trying to get out of practice.
“Every time I complained about it, I had to do 20 pushups,” the 13-year-old reflex sympathetic dystrophy patient said.
The lack of understanding is not limited to coaches. Many physicians miss the painful disorder, said Dr. David Carlson, chief medical officer at Conemaugh Health System.
“Early recognition remains problematic,” Carlson said at his Memorial Medical Center office in Johnstown. “It can be very hard.”
As the husband of a reflex sympathetic dystrophy patient, Carlson understands the need to raise awareness among physicians. Although the disorder is characterized by intense, burning pain, specific symptoms vary from patient to patient and even from month to month for a patient.
“It is not necessarily at the top of the mind,” Carlson said.
“There is not one single hallmark of the disease.”
Lacey and her mother, Vicki Walker, have seen the problem. It took several months and three emergency room visits before Lacey found understanding from doctors at Children’s Hospital of Pittsburgh.
Walker’s advice to doctors: “If you don’t know, refer.”
Both Lacey and Carlson’s wife, Marci, stressed the need for more research into the condition. Lacey said she’d like to organize a support group for RSD patients and help raise public awareness.
The pain affects every aspect of the patient’s life, extending into the entire family, Lacey said.
“I got my first ‘C’ and my second ‘B,’ ” she said, adding that her friendships also suffered.
“Your mind begins to turn,” Lacey said. “You become an angry person.”
Her school, Conemaugh Valley High School, allowed her to wear shorts beyond the November deadline because fabric aggravated her skin pain. She was given extra time between classes and a student helper when she was using crutches. But she warns friends, family and school officials that the help can go too far.
“I don’t like them making me feel different and left out,” Lacey said.
“I can deal with it. I don’t like when people say you are different.”