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Complex regional pain syndrome is a disease characterized by severe pain, swelling, and skin changes, according to David N. Maine, MD, director of the Center for Interventional Pain Medicine at Mercy Medical Center in Baltimore. “It can affect one or multiple extremities [with characteristics including] severe pain and sensitivity, swelling, skin changes [abnormal color] and decreased range of motion,” he says.

Injuries triggering the onset of CRPS can range from seemingly mild, such as a twisted ankle, to major, such as a heart attack or stroke, according to Maine. However, the disorder, which can occur at all ages, also may happen with no clear inciting event. The level of pain often exceeds that expected from the initial injury and can be very debilitating, Maine notes.

Making a Case

In most cases, known as CRPS 1, there is no definable nerve lesion. CRPS 2 refers to cases in which a definable nerve injury does exist. Genetic factors may play a role, although this is not completely clear.

Edward Carden, MD, FRCPC, who specializes in pain management at D.I.S.C. Spine and Sports Center in Marina del Rey, Calif., describes Type 1 (CRPS 1) as a “pain syndrome characterized by an exaggerated response to a painful stimulus. The character of the injury may be severe, as in acute trauma or surgery, low grade as in chronic overuse, or quite insignificant.”

One factor that is key to diagnosing CRPS 1 is pain out of proportion to the noxious stimulus, according to Carden. “This pain not only long outlasts the healing phase of the acute event, but may spread within an extremity or to other extremities, causing significant neurological, functional, and psychological impairment,” says Carden, who also serves as a clinical professor at the University of Southern California, Keck School of Medicine, in Los Angeles.

Although the upper extremity seems to be involved more than the lower extremity, Carden has observed that lower extremity CRPS 1 is becoming more frequently recognized. Women are impacted more than men, with most cases occurring between ages 40 and 60, he says. However, “CRPS 1 has been reported in all age groups, including infants,” he adds.

Associate professor of occupational therapy at Dominican University of California in San Rafael, Bonnie Napier, EdD, OTR/L, notes that CRPS can be caused by repetitive stress to shoulder, elbow, wrist, or fingers, or by a variety of injuries. In addition to injuries, “fractures that include nerve injuries can also be part of the sequalae,” Napier says. “It can also be the result of complications related to cerebrovascular accidents.”

Treating CRPS

The optimal approach tends to be the application of multiple modalities in concert for the best functional outcome, according to Maine. Treatment options include:
• physical therapy
• occupational therapy
• anticonvulsants, such as Gabapentin
• bisphosphonates
• nasal calcitonin
• oral glucocorticoids (in the early stages)
• sympathetic blocks (stellate ganglion or lumbar sympathetic block)
• spinal cord stimulation

In diagnosing and treating the disorder, Napier recommends that the clinician prepare “a lengthy list of pain descriptors from which the person can select those that apply,” such as dull versus sharp, piercing versus stabbing, or quick versus long-lasting. These specifics are particularly helpful in light of pain’s dual biological and cultural aspects, she says. Additionally, she views it as essential to seek information about when the pain occurs: “Is it related to activity? Is it worse in the morning or evening? Is it episodic or continuous?” she asks. Similar questions can assist in determining what functions may aggravate or relieve the pain.

Encouraging the patient to rest is usually not the corrective treatment, because scar tissue can become more problematic with rest and can be shaped by activity, Napier notes. “Patients need to learn the difference between the sensations of stretch versus pain. Stretch is essential to scar tissue modeling, but taking it too far creates pain that decreases healing. This is one reason that range of motion is most effective when taught to the patient to perform on him [or] herself, rather than being done by a therapist who may inadvertently range into the painful range; the difference usually cannot be determined by palpation, even by the best of therapists,” she explains.

OT’s Key Role

Napier views occupational therapists as “uniquely skilled to provide treatment in this area because of the understanding of the biological, cultural, vocational, recreational, and psychological issues that contribute to CRPS.” Based on that depth and breadth of comprehension, OTs can offer the patient mechanical interventions, environmental adaptations, and pain-coping guidance, among other modalities, she says, adding that OTs can guide patients in separating the “biological and psychological contributions to the pain and injury.”

Carden describes OTs as taking an integral role in getting the patient back to work. “They [help] the patient with either an upper or lower extremity problem utilize the affected extremity and become stronger and more adept so they can re-enter the workplace,” she says, comparing these challenges as similar to those required for individuals who have had surgery on a lower or upper extremity.

The difference: CRPS requires a much longer period of time to get better, Carden says. Additionally, patients may still have residual pain, so the therapy will need to be modified so it doesn’t aggravate the pain situation.

Breaking the Pain Cycle

Can a specialist retrain the brain not to feel pain?

“CRPS can sometimes be viewed as a futile cycle. There is some thought that if we are able to put the brakes on this cycle — even if for a short period — you [could] alter the course of disease and even stop it,” Maine says, adding that psychological therapy and counseling can sometimes help the process, as can modalities such as biofeedback and guided imagery. Evidence does exist that retraining can help to diminish or desensitize the person to the perception of pain, Napier says.

“When chronic pain has been endured, learned painfulness can occur. The person needs to re-experience movements without the expectation, and sometimes self-fulfilling prophesy, of pain. Pain is a strong deterrent from continuing movement, and retraining may need to occur to reinforce that movement can occur without the anticipated pain response,” she concludes.

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Joanne Eglash is a medical writer for the Gannett Healthcare Group. To comment, e-mail oteditor@gannetthg.com.

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