Special Report: Managing RSD
Do you groan when you get a reflex sympathetic dystrophy case?
Here’s help demystifying the syndrome’s diagnosis and treatment
A 22-year-old woman admitted to Bethesda Naval Hospital in Maryland with a bleeding disorder had contracted reflex sympathetic dystrophy syndrome or complex regional pain syndrome (RSD/CRPS) at age 17, after undergoing routine surgery. The pain and frustration led to a depression that caused her to attempt suicide. And the issues raised by her RSD left nurses and physicians at Bethesda Naval scrambling for advice on how to treat her bleeding disorder.
She’s not alone. The Reflex Sympathetic Dystrophy Syndrome Association (RSDSA) in Haddonfield, NJ, estimates that as many as 5% of all injuries or surgeries result in RSD, a disorder marked by burning pain, swelling, and motor and sensory disturbances, associated with sympathetic nervous system dysfunction. "It’s a hidden disability with very powerful psychiatric overtones. There are times when you want to take your life," confides Wilson H. Hulley, a member of the executive staff of the President’s Committee on Employment of People with Disabilities in Washington, DC, and a special assistant to both the Bush and Clinton administrations on disability issues. Hulley also lives with RSD. He was contacted by a patient advocate at Bethesda Naval when the young woman was admitted to the hospital. "It was an emergency call. The woman was clotting at the same time she was having a very painful RSD episode. I’ve been working with her, but she’s in the later stages of Stage III RSD and it’s making her life miserable," he says.
"When I was first diagnosed, I met with a neurologist at Johns Hopkins in Baltimore who told me he could help me for two years and then I would probably commit suicide like the majority of his other RSD patients," says Hulley. "I never went back to him again."
Intense emotional strain
It shouldn’t surprise case managers that RSD patients experience emotional and psychological disturbances, notes Anthony F. Kirkpatrick, MD, PhD, associate professor of medicine at the University of South Florida in Tampa and editor of the RSD/CRPS clinical practice guidelines. "It’s not only the excruciating intensity but also the bizarre nature of the pain caused by RSD that leads to these disturbances," he says. "Some RSD patients describe their pain as feeling as if bugs are crawling all over their bodies, and as the pain spreads from one region to another, patients comment that it’s as if a demon’ is moving around inside them."
Kirkpatrick emphasizes that the unique nature of RSD pain and its psychosocial components are well-described in the literature. "The pain of RSD is the most excruciating form of pain. It has been studied and compared to the pain of cancer, arthritis, and childbirth and it always comes out as one of the most intense types of pain imaginable."
He adds that studies designed to determine whether RSD patients had a pre-existing psychiatric condition prior to developing RSD or a predisposition for psychiatric disturbances have been negative. "These studies simply haven’t panned out. It’s simply the nature of this pain, not underlying psychiatric issues, that causes emotional and psychiatric problems in RSD patients."
The psychological impact of the pain and disability caused by RSD must be addressed adequately throughout treatment before the primary diagnosis shifts from RSD to a psychiatric diagnosis, cautions Janet M. Frontera, RN, BSN, CCM, A-CCC, medical consultant for the Pittsburgh claim office of CIGNA IntegratedCare. "Too often, the treating physician keeps the patient on such high doses of pain medication that the patient simply sits at home watching television and moving very little. Patients become so frustrated and depressed that, if nothing is done to address the psychiatric component of this diagnosis, the depression moves from a comorbid condition to becoming the primary disability claim."
Clinicians who specialize in pain management often fight case managers to provide adequate psychiatric treatment for their patients. "We commonly run into this issue with case managers," says Srinivasa N. Raja, MD, with the department of anesthesiology, division of pain medicine at Johns Hopkins Hospital in Baltimore. "This condition often affects young people who have been very active. RSD impacts their lifestyle and hits them hard financially; therefore, we observe a high percentage of depression in these patients."
Raja urges case managers to seek psychotherapy for RSD patients designed to help them cope with their disability and motivate them to facilitate functional improvement. "It’s common that RSD patients start out very optimistic," he notes. "As their condition fails to resolve, the physical problems are less important and the psychological aspects of this condition worsen. The degree of anger, frustration, and depression must be adequately addressed as part of the overall management of the syndrome."
Diagnosing RSD requires a careful and thorough medical history. A diagnosis of RSD/CRPS requires a history of trauma to the area associated with pain that is disproportionate to the inciting event, plus one or more of the following symptoms:
• abnormal function of the sympathetic nervous system;
• movement disorder;
• changes in tissue growth, either dystrophy or atrophy.
A survey of more than 1,300 RSD sufferers found that 33.5% of respondents experienced RSD following surgery. Another 19.8% experienced the first symptoms of RSD following a fall.
Other triggering events reported by respondents include:
• Nearly 19% of respondents experienced their first symptoms following a fracture.
• Roughly 15% of respondents experienced their first symptoms following a sprain.
• Roughly 14% of respondents experienced their first RSD symptoms following a crush injury.
• Just over 12% experienced their first RSD symptoms following a contusion.
A diagnosis of RSD/CRPS is often made when there is an absence of all other possible pathology that might explain the pain and symptoms of the patient. "RSD is an end-of-the-spectrum diagnosis," says Frontera. "By the time a diagnosis of RSD is made, the treating physician has usually looked at and treated many other conditions. This is often a when all else fails’ diagnosis."
When case managers see a diagnosis of RSD, or CRPS, as it is becoming more commonly known, they should review the patient’s medical record for evidence that all other possible causes of the pain have been ruled out. "Nerve conduction studies and selective diagnostic nerve blocks are very useful as diagnostic tools to help pinpoint the source and cause of a patient’s pain," notes Raja, adding that a significant number of patients who are referred to him with a diagnosis of RSD have been misdiagnosed. (Other useful diagnostic tools are described in the recently published RSD/CRPS guidelines available on the RSDSA Web site at www.rsds.org.)
"If the patient has not undergone a thorough neurological examination, order one immediately," he urges. "A few years ago, I had a patient referred to me with a diagnosis of RSD who had pain that had spread from the hand to the shoulder region. We subsequently worked up the patient’s cervical spine and found a protruding disc that was pressing on the spinal cord and causing the pain."
Why not the best?
Kirkpatrick urges that case managers refer RSD patients to a university medical center for initial diagnosis and treatment. "When care is initiated at a major teaching center, it gives the patient a jump start," he notes. "University-affiliated hospitals have clinicians who are current on research and development issues and are more likely to be able to properly do a sympathetic block of the lower extremities. An inexperienced clinician may do an epidural block instead of a selective nerve block and the results would not be useful for determining the cause of the pain — it’s not cost-effective."
In addition to being a good diagnostic tool, nerve blocks are also useful for promoting rehabilitation, agree Raja and Kirkpatrick. "Early intervention with physical therapy is essential for maintaining or restoring functionality in RSD patients," says Raja. "Many patients discontinue physical therapy due to the pain caused when the affected limb is moved. Physical therapy scheduled immediately following a selective nerve block can greatly increase a patient’s range of motion by increasing the patient’s ability to tolerate pain," he notes, adding that physical therapy should be limited to improving range of movement and increasing muscle strength in RSD patients.
Patients with RSD may experience some or all of the following six clinical features of RSD/CRPS described in the clinical practice guidelines, which were developed by a panel of 13 RSD specialists and published by RSDSA.
1. Pain. Patients with RSD/CRPS suffer from pain and mobility problems out of proportion to those expected from the initial injury. The first and primary complaint occurring in one or more extremities is described as severe, constant, burning, and/or deep aching pain. All tactile stimulation of the skin, even the wearing of clothes, may be perceived as painful.
"We had a woman contact us because an airline refused to let her fly because she couldn’t tolerate a shoe on her foot," says Jim Broatch, MSW, executive director of RSDSA. "Eventually, the airline did allow her to fly rather than face a lawsuit."
Other RSDSA members have been unable to return to work because of workplace dress codes, he adds. "You can’t go back to work if wearing clothes is too painful. Employers have to be very flexible about everything, including dress codes, for an employee with RSD to successfully return to work. A case manager could help by explaining the situation to the employer and discussing options such as the possibility of the employee wearing shorts to work."
In addition, Frontera and Raja both note that it’s important to negotiate part-time work for RSD patients. "Too many patients try to return to work full-time and end up regressing," says Raja. "Few patients with RSD can return to their original workload without backsliding."
Frontera always encourages patients to first try part-time work before jumping back to their original workload, especially when they have psychological issues. "The stress of returning to work with the added issue of dealing with pain often causes patients who have been out on disability to relapse. It’s much better for patients to return part-time and gradually increase their hours, as they feel able."
2. Skin changes. The skin over the affected area may appear shiny, dry, or scaly. Hair may initially grow coarse and then thin. Nails in the affected extremity may be more brittle, grow faster, and then slower. The patient may perceive sensations of warmth or coolness in the affected limb without even touching it. The skin may show increased sweating or increased chilling.
Changes in skin color range from a white mottled appearance to a red or blue appearance. Hulley experienced his first symptoms of RSD after orthopedic surgery to repair a fractured bone in his foot. "My foot grew swollen and my entire leg turned the colors of the rainbow," he recalls. "The surgeon was positive I had acquired RSD, but it was another four months from the original swelling and discoloration that the diagnosis was confirmed."
3. Swelling. Pitting or hard edema is usually diffuse and localized to the painful and tender region. If the edema is sharply demarcated on the surface of the skin along a line, it is almost proof that the patient has RSD/CRPS.
4. Movement disorder. Patients with RSD/ CRPS have difficulty moving because they hurt when they move. In addition, there seems to be a direct inhibitory effect of RSD/CRPS on muscle contraction. Decreased mobilization of extremities can lead to wasting of muscles.
"There isn’t an RN or PT who won’t tell you that atrophy can do more damage than anything else psychologically or physically," says Hulley.
"It’s essential to initiate activity in the affected extremity and not allow it to just hang there and do nothing," says Frontera. "Physical therapy can keep the affected extremity as functional as possible and prevent or reduce atrophy. Therapy also can keep the patient from developing a disability mindset."
5. Spreading symptoms. Initially, RSD/CRPS symptoms are localized to the site of the original, triggering injury. With time, the pain and symptoms tend to become more diffuse. The RSDSA survey found that more than 71% of RSD sufferers cannot identify a reason for the spread of their symptoms. In addition, survey respondents indicated that the average time from initial symptoms to spread to other regions of the body was 12.8 months.
The disorder typically starts in an extremity. Three patterns of spreading symptoms are described in the literature:
• continuity type spread where the symptoms spread upward from the initial site, for example from the hand to the shoulder;
• mirror-image type spread where the symptoms spread to the opposite limb;
• independent type spread where the symptoms spread to a separate, distant region of the body.
6. Bone changes. X-rays may show wasting of bone, or patchy osteoporosis. A bone scan may show increased or decreased uptake of radioactive substance (technecium 99m) in bones after intravenous injection.
Of course, once a diagnosis of RSD has been confirmed, it takes a team approach to effectively coordinate the necessary care. "At a bare minimum, following an initial evaluation and treatment at a university center, a primary care physician must work with a mental health provider — either a psychologist or a psychiatrist, as necessary," says Kirkpatrick. "In an ideal situation, the patient lives close enough to a major medical center that an RSD specialist could be added to the patient’s team."
The four treatment goals outlined in the RSDSA clinical practice guidelines are:
• educate about therapeutic goals;
• encourage normal use of the limb with physical therapy;
• minimize pain;
• determine the contribution of the sympathetic nervous system to the patient’s pain.
Case managers must play an active role on the treatment team of the RSD patient, says Raja. "Case managers and physicians working together can help the patient set realistic treatment goals. Too often the patient has unrealistic expectations and those complicate the entire situation. We are not talking about a cure’ when we work with RSD patients but rather improvements in function and quality of life. It helps if the physician and the case manager can sit down together and talk — too often valuable time is lost in the communication process."
Kirkpatrick stresses that treatment flows more smoothly when all interested parties are kept fully informed about the patient’s treatment protocol and progress. "Many of these patients are involved in litigation. There are attorneys and case managers involved as well as physicians and therapists," he notes. He suggests that a patient update report be shared with the patient and include the following five areas:
• procedures performed;
• medications prescribed;
• physical and occupational therapy notes;
• psychosocial issues;
• new laboratory tests or consults.
Hulley agrees that the patient should play an important role in the communication process. "Every time I see one of my physicians, I ask for a copy of the report and fax it to each of my other treating physicians," he notes, adding that currently his own treatment team includes a pain management specialist, a psychiatrist, a physical therapist, and an orthopedic surgeon. "My dentist is also an RSD specialist who understands my special pain management needs."
He urges case managers to advocate for RSD patients and support all areas of their lives. "RSD can tear families apart. It puts tremendous strain on marriages," he says. "Case managers can be a godsend to a patient."
Who gets reflex sympathetic dystrophy?
A survey of more than 1,300 individuals with reflex sympathetic dystrophy (RSD) conducted by the Reflex Sympathetic Dystrophy Association of America in Haddonfield, NJ, reveals the following about the demographics of this disabling syndrome:
• 77.9% of respondents were women.
• 94.4% of respondents were white.
• The average age of respondents at the onset of RSD was 36.8 years.
• 92.8% of respondents had no family history of RSD.
• The average time span between the onset of symptoms and diagnosis of RSD was .87 years.