Pain, Pain, Go Away
People with reflex sympathetic dystrophy sometimes experience level-10 agony daily
By Emily Wengert

March 2002 changed Kelly's world forever.

An LPN since 1994 and an RN since 2000, Kelly was a hard-working nurse who loved her job. But after coming out of a patient's room one day, Kelly caught her foot on a wheelchair and twisted her knee.

Accustomed to walking off minor injuries, Kelly reported the incident but didn't think she needed to have it checked out. It was just a sprain, after all. When it still hurt the next day, she had it examined. She was given acetaminophen and a knee sleeve.

When an abnormal level of pain remained 2 days, 2 weeks and then 2 months later, Kelly knew this was more than just a sprain. Thus began her search for a diagnosis, a search that would end more than a year later with a syndrome Kelly had never heard of before: reflex sympathetic dystrophy (RSD).

She Has What?

If you haven't heard of CRPS, also called complex regional pain syndrome type I (CRPS), you're not alone. In fact, you're in the company of the numerous doctors who examined Kelly before someone finally recognized her condition (and even then the doctor didn't know how serious it was).

In the majority of cases, CRPS begins with a precipitating injury, after which the patient experiences more pain than would be typical for the inciting event. The pain is neuropathic, shooting and sharp, but also can be described as burning, aching or cold.

CRPS can cause abnormal skin color changes, hair or nail growth, or sweating. (See Table for other common symptoms.) These symptoms result from the neurological damage believed to have occurred.

Terry Paylor, RN, CCRC, is nurse coordinator for the Drexel University College of Medicine Department of Neurology Pain Center, Philadelphia. Paylor works with one of the pre-eminent physicians researching CRPS: Robert J. Schwartzman. She said Dr. Schwartzman describes the condition as an electrical box gone wrong.

"If an electrical box is wired correctly, you can only initiate power through a switch," Paylor said. "If there are wires hanging live, that can send out an electrical signal at any time. That's what CRPS is. It continues to fire even when the switch isn't thrown."

Physicians and pain specialists say CRPS can be treated most effectively in the first 3-6 months following the inciting event. In Kelly's case, the delay in her diagnosis meant she went through needless and even damaging treatments.

Mystery Syndrome

Initially, the examining physicians believed she had suffered a meniscus tear. They hoped it would heal on its own with the help of aggressive physical therapy, but when it didn't, they scheduled her for knee surgery. Kelly described the pain as a 7 or 8 on a 10-point pain scale before the surgery. Afterward, it shot up to a 10 and has remained nearly that high ever since, as if an already throbbing limb had been hit with a sledgehammer.

"There are some days when the pain is so excruciating," Kelly said. "Just a light breeze outside creates pain. The slightest touch sends me through the ceiling."

The surgery took place August 2002, and Kelly went back to her nursing job in November. She could not tolerate it and her employer put her in a desk job in December. However, because of severe knee pain, she had difficulty with that position as well. After developing pain in both arms, which at first was diagnosed as carpal tunnel syndrome, she was finally diagnosed with CRPS in the upper extremities with the left side worse than the right. Kelly stopped working by doctor's orders in February 2003.

Untreated CRPS often spreads to other limbs of the body. The pain that started in Kelly's left leg has now engulfed her whole left side, as well as her right arm. She gets violent headaches, feels pain at the base of her skull and has numbness in her hands and feet.

Despite the multiple medicines she takes, including non-narcotic pain meds and a muscle relaxant, she has trouble sleeping at night, which only exacerbates her agony. Kelly's situation is not rare. CRPS typically is misdiagnosed several times.

"The average person has been to seven physicians before they come to see me," said Mary Stegman, MD, who treats CRPS patients at her Cypress Pain Management Clinic, Fort Myers, FL.

She believes less than half of the people in the country with CRPS have been properly diagnosed. Only 5 percent of her patients come to her in the early stages of the disease, she added. The Reflex Sympathetic Dystrophy Syndrome Association (RSDSA) estimates between 1.5 million and 6 million Americans have this condition. So why don't doctors know about it?

One of the primary reasons may be prejudice. For years, healthcare workers have stereotyped pain patients as drug seekers, and it's been hard for CRPS advocates to shake that suspicion.

"Pain patients on narcotics are not addicts. They have a dependence on medications just like people who take blood pressure medications or wear glasses have a dependence on something," Dr. Stegman said.

Pain patients are seen as demanding troublemakers, and doctors seem to want to steer clear of them, she said. Another drawback to recognizing CRPS is that it doesn't fall under any particular specialty, so patients get shuffled around, trying to find someone with expertise on their problem. Dr. Stegman said only recently have fellowships been available in pain management.

Presentation

To recognize an CRPS patient, Paylor looks for a body stiffened and tensed in pain. The patient might be forward flexed and favoring one side more than the other. Some patients experience allodynia; livedo reticularis, which is a lace pattern on the skin; and edema. Extremities, like the hands, ears, face and feet, may redden and swell.

Dr. Stegman has noticed her CRPS patients often react during their first visit with something she has dubbed "the fear sign."

"The patient immediately goes to the farthest corner [of the room] because they're afraid you're going to touch them," she said. "So, I always tell them exactly what my hands are going to do."

Because so little is known about CRPS, treatment methods vary. The pain is so severe that a treatment like physical therapy can be too taxing for many patients.

"Automatically, people think to send patients into physical therapy," Paylor said. "But you can't do PT if your pain scale is at a 9."

To keep the pain in check, Kelly goes to an occupational therapist 3 times a week, attends pool therapy twice a week and has doctor's appointments regularly in Philadelphia, about an hour away from her home. She goes to hypnosis every 3 weeks and also sees a psychologist.

Trying to dull the pain has become a full-time job for her — and her husband, who must drive her to the Philadelphia appointments because she is no longer safe behind the wheel for long distances.

Research

Most CRPS treatment begins with sympathetic nerve block, which means injecting medicine around the spinal column to numb the nerves. A TENS (transcutaneous electrical nerve stimulator) unit is another common way to treat CRPS.

Five days of lidocaine IV infusion has been effective for some of Paylor's patients. She has seen a 40 percent improvement in quality of life for 90 percent of her patients. Some patients have dropped from a 9 pain level to a 1.

She also works closely with Dr. Schwartzman on off-label medication research using ketamine, which is infamous as the street drug Special K. The study follows a 5-day, low-dose ketamine infusion regimen for healing, and Paylor said the results so far are promising.

Researchers in Germany use ketamine to induce a 5- to 7-day coma in CRPS patients. Rarely does an CRPS patient's pain completely disappear, but Paylor said that of the 20 patients from her clinic who have returned from the ketamine trials in Germany, all reported less pain and approximately 25 percent remained pain-free afterward.

Paylor soon will begin working with Dr. Schwartzman on a newly approved study involving 48 hours of continuous ketamine delivered subanesthetically.

Dr. Stegman, who was a nurse for 10 years before becoming a doctor 20 years ago, has tried both lidocaine and ketamine infusions for her patients and hasn't had as much luck. She prefers a combination of narcotics, psychosocial therapy and other adjuvant therapies to help manage her patients' pain.

Social Effects

Treating someone with chronic pain involves treating more than just a medical problem. For chronic pain diseases, depression tends to follow and, with that, suicide. To help bring that issue to light, Dr. Stegman asks her patients if they've ever considered ending their own lives, and their response is almost overwhelmingly yes.

She's heard detailed plans of how patients dream of putting an end to the pain. But the pain isn't the only cause of stress. CRPS patients miss events in their children's or spouse's lives because of their condition.

Kelly regrets that she cannot walk around the mall with her teenage daughter. She described her family as supportive, but admitted that her condition has added considerable strain to all relationships.

Because it hurts to be touched and to touch others, hugs become a moment of agony instead of affection. Dr. Stegman said pain patients often lose the possibility of a sex life as well. She said incorporating the family into a care plan can be critical for the patient to receive the understanding and support they need.

Kelly has added stress because of a workers' compensation court case she has been involved in for more than a year. Her employer's doctors will not recognize she has CRPS. (Because of the litigation, Kelly requested that ADVANCE not use her full name.)

Lack of information about the disease does not help its sufferers explain to family and friends what they're going through. Kelly described being embarrassed that she has to use a cane. She feels others look critically on her condition because they have never heard of it before.

Groups like the RSDSA work hard to get the word out about CRPS. In addition, three states have made efforts to mandate more education. In September 2003, the Delaware state legislature passed a bill that promotes education of healthcare professionals and consumers about CRPS. Pennsylvania legislators followed suit 3 months later. New York lawmakers started the trend by passing a similar bill in 2002, according to an Associated Press report.

Kelly encourages other nurses to take reports of pain seriously and hopes CRPS will someday be included in nursing and medical school curriculums.

"Listen to the patients and do not accuse them of blowing their pain out of proportion," she pleaded.

Emily Wengert is a freelance writer in New York and former assistant editor at ADVANCE.

Case Study: Childhood CRPS

At the age of 9, Rachel Charlesworth experienced her first of three bouts with reflex sympathetic dystrophy (RSD). Her mother, Daria Charlesworth, MS, BSN, RN, tells her story.

"My daughter, Rachel, was in third grade, a happy, well-adjusted kid. She tripped in school and basically sprained her wrist. We didn't really think too much about it that afternoon, but the pain became more and more intense, from fingertips to shoulder. With the very lightest touch on her skin, she would jump, and she had trouble sleeping.

"I called my pediatrician who gave us a referral for an orthopedic specialist. The orthopedic specialist, thank goodness, recognized CRPS right away. By this time, she's holding her arm near her chest. She doesn't want anyone to touch it, doesn't even want to socialize with her friends. She was depressed within a week. It was extreme.

"It was interesting to go to professionals who were looking for the psychogenic rationale. They started asking about our marital status. You start thinking, 'Maybe there is something wrong with me,'" Charlesworth said. "It was really confusing. She went from being a typical well-adjusted kid to a kid who was school-avoidant. Her friends didn't understand what was going on."

Through a course of intense physical therapy, pain medication and antidepressants, her daughter's pain began to subside. But after 6 pain-free months, CRPS recurred in Rachel's other arm, her left, after she received a DPT booster shot. She faced another 9 months of physical therapy (she spent a lot of time working in a heated pool) and medication before her CRPS disappeared again.

After 4-6 months in the clear, Rachel once again showed signs of CRPS, this time in her right leg after a day of strenuous bicycle riding, and thus faced rigorous physical therapy for a third time in her life.

A cure is a rarity in the CRPS world. Rachel's three bouts with the syndrome could have ended in debilitating, lifelong pain, but her mother thankfully reports her daughter has been pain-free for 6 years. She attributes her recovery to the orthopedic specialist's early diagnosis 8 years ago.

Charlesworth and her husband have allowed Rachel to resume many of her old activities, like dancing, and try new ones, including cheerleading, surfing and waterskiing. However, she still forbids her now 17-year-old daughter to rollerblade or play basketball and soccer because of the high injury rate with those activities.

Charlesworth's husband, Paul, is currently president of the Reflex Sympathetic Dystrophy Syndrome Association. Through that connection, they know other parents whose children still battle with CRPS and sometimes resent Rachel's success. "Lots of kids don't get better. They get worse," Charlesworth explained. Rachel is one lucky girl.

Used with permission from the Reflex Sympathetic Dystrophy Syndrome Association (www.rsds.org).

Advance for Nurses. 6(13):45.