Publications
APS Bulletin • Volume 8, Number 6, 1998
Patient Advocacy Groups
Myra Glajchen, MSSW, Department Editor
Reflex Sympathetic Dystrophy Syndrome
Idamarie Scimeca Duffy
EDITOR'S NOTE: In 1984 a steel door crushed the toes of the author's right foot. Six years and two surgeries later she was diagnosed with reflex sympathetic dystrophy (RSD). In January 1992 the RSD spread after she had a sympathectomy. Since then, she has been totally disabled and is now confined to a motorized wheelchair. The RSD is in her pelvic and groin areas, right hand, and left leg. RSD has affected the muscles of her eyes, causing vision problems. It has also affected her esophagus and bladder. Three days a week she goes to a swimming pool. Her range of motion increases and the intensity of the pain decreases while she is in the warm water.
Insofar as more than 6 million people have reflex sympathetic dystrophy (RSD) syndrome, one questions why it takes so long to get an accurate diagnosis. Could it be that medical professionals are not familiar with the many faces of RSD? Or could it be that in its early stage, RSD can look like many other injuries? In fact, RSD may not actually present itself until days or weeks after an injury. Initially the area of trauma looks and feels the way it should when it has been traumatized. The distinct difference, in the beginning, may be ongoing pain that is out of the ordinary for the particular type of trauma. It is not surprising that many with RSD are sent through the mill of nerve blocks, surgical procedures, spinal cord stimulators, morphine pumps, opioid trials, and referrals from one rehabilitation facility to another.
To add to the confusion, RSD has been given more than 13 different names. In 1994, the International Association for the Study of Pain introduced new terminology for RSD—complex regional pain syndromes, type I (RSD) and type II (causalgia). These terms will be phased in over the next 8 years. Some medical professionals have embraced the name change and others have not. Not many people with RSD are comfortable with the name change, however. The reason is simple: It has taken a long time to get recognition in the International Classification of Diseases Code Book (9th ed.). The codes for RSD began to appear in the code book only in October 1993. The name change is already having an adverse effect on patients with RSD who are applying for Social Security disability benefits.
History of RSD
Civil War surgeons Mitchell, Morehouse, and Kean first described this phenomenon in 1864. They noted a particular pain syndrome that developed in the hands and feet of wounded soldiers, who would fill their boots with water and wrap their affected limbs in wet rags to "extinguish the fire." In 1867, Dr. Mitchell named this condition causalgia, from the Greek kausos (heat) and algos (pain) (Levin, 1991). More than a century later, RSD is still poorly understood and little recognized. Early detection, correct diagnosis, and immediate intervention are thought to produce better outcomes. Research is ongoing but to date, there is no known cure.
Etiology of RSD
RSD is most often initiated by trauma and may follow central nervous system damage such as stroke, tumor, or syrinx. Its six major components are pain, edema, autonomic dysfunction, movement disorder, trophic changes, and autoimmune phenomena. The process can spread to contiguous and contralateral areas of the body and can become generalized. In general, pain, swelling, and autonomic dysfunction are most prominent early in the illness, whereas movement disorder, trophic changes, and autoimmune abnormalities may dominate the later stages (Schwartzman, 1992). Its devastating ability to spread is one of the most distressing characteristics of RSD.
No segment of the population has been ruled out as potential victims of RSD. RSD knows no age limits—it can affect children as well as adults. RSD can be caused by a variety of precipitating events, including trauma to either a minor or major body part. These events include some surgical procedures (e.g., the surgical procedures for breast biopsy and bypass surgery); cuts; injury to any area rich in nerve endings; fractures, sprains, and dislocations; infections; multiple traumas to a particular body part; soft-tissue injuries; broken bones; casting of an extremity after fracture; myocardial infarction; crush injuries; repetitive motion disorders (e.g., carpal tunnel syndrome, the surgical procedure for it, or both); and some invasive procedures.
The pain of RSD
In patients with RSD, there is thought to be a short circuit at the point of partial nerve interruption. The pathway remains open due to the continual release and increase of sympathetic activity and results in an increased production of the brain chemical norepinephrine, which causes patients to become extremely sensitive to touch, pressure, and pain. The sympathetic nervous system never calms down; instead, it is locked in a vicious cycle in which pain produces nerve impulses that, in turn, produce additional pain (Bonica, 1990).
Descriptions of the pain of RSD include "severe," "burning," "knifelike," "piercing," "stabbing," "throbbing," "tearing," "lacerating," and "aching" (sometimes felt deep in the affected part). At times, unrelieved by rest, RSD is also subject to exacerbation by the slightest emotional or physical stimulation.
RSD pain is unpredictable. Environmental temperature changes have varying effects on patients. In some patients, cold aggravates the pain and warmth relieves it. Some patients have an opposite response, whereas others are either aggravated or relieved by both cold and warmth. Still others are unaffected by changes in temperature.
Almost without exception, the pain is aggravated by use of the affected body part, by passive movement, or by touching or tapping the part. In some patients, the skin is so hypersensitive that the slight friction from clothing, bedclothes, or blowing produces excruciating pain. Consequently, these patients learn not to move the part and go through seemingly absurd extremes to protect a painful limb from touch or any other physical stimuli.
Visual and auditory stimuli, such as unexpected noise or bright light, rattling of a newspaper, noisy conversation, motion of others, bumps in the road, a gentle kiss, a hug, a breeze, whistling, music, high-pitched sounds, an airplane, or a banging door, can cause a sudden, marked aggravation of the pain.
Emotional disturbances, such as anger, fear, excitement, and mental distress, invariably aggravate the pain. The initiating factor may be an argument, hearing an exciting story, or watching a dramatic motion picture or television program. The pain can also flare up for no apparent reason.
Patients with RSD have greatly varying symptoms. Some may have moderate pain that then flares to severe pain. Others may not have constant severe pain but experience periods of extreme exacerbation. Pain is the most disabling and the most difficult aspect of the illness for medical professionals to treat (Schwartzman, 1992).
Misunderstanding of patients with RSD
Because many healthcare professionals are unfamiliar with RSD, patients are told there is nothing wrong with them—the pain is all in their heads. Many mistakenly believe RSD is more of a psychological condition than a physical one. They believe that patients with RSD were not as stable as the general population before developing their illnesses.
The physical, emotional, and mental effects of RSD are very real, strong, and difficult to manage. In addition to producing extreme pain, RSD wreaks havoc on the lives of the patient and family, friends, and coworkers. Associated problems include prolonged family disruption, patient disability and unemployment, misdiagnosis, improper treatments, multiple surgeries, unsuccessful surgery, diminished quality of life, increased healthcare costs, and the inexperience of healthcare professionals in treating RSD.
The pain of RSD strikes so quickly and severely that patients do not have time to adjust. They become panicky, search desperately for answers, and do things that appear absurd to protect their limbs from more pain. The suicide rate for people with RSD is very high, partly because of the intensity of never-ending pain, sleep deprivation, frustration, and lack of support from medical professionals, family, friends, and coworkers.
The Reflex Sympathetic Dystrophy Syndrome Association
The RSDSA of New Jersey and Delaware, a not-for-profit organization, was founded by patients with RSD to establish RSD Awareness Month in the states of New Jersey and Delaware. The association offers the following programs:
- an annual conference highlighting research and treatment updates
- medical, social, and therapeutic information on RSD
- support groups for patients, family, friends, and concerned healthcare professionals
- a speakers' bureau
- mutual aid and support via telephone, e-mail, fax, literature, information, and research articles
- self-help for people with RSD to share experiences, techniques, coping skills, information, and resources
The goals of the association are to promote public awareness of RSD, provide support to people with RSD, educate healthcare providers about RSD, and promote and support funding for RSD research. We are very grateful to the American Pain Society for giving us this opportunity to further the awareness of RSD.
References
Bonica, J.J. (1990). Causalgia and other reflex sympathetic dystrophies. In J.J. Bonica (Ed.), The management of pain (pp. 220-243). London: Lea & Febiger.
Levin, D.Z. (1991). Burning pain in an extremity. Reflex Sympathetic Dystrophy, 90(2), 175-185.
Schwartzman, R.J. (1992). Reflex sympathetic dystrophy. In H.L. Frankel (Ed.), Handbook of clinical neurology (pp. 121-136). New York: C. Elsevier Science Publishers B.V.
Idamarie Scimeca Duffy is founder and executive director of RSDA of New Jersey and Delaware.