Reflex sympathetic dystrophy (RSD), also called complex regional pain syndrome (CRPS), is a rare chronic, painful, and progressive pain disorder that affects the skin, muscles, joints, and bones and can have disabling effects in people dealing with the condition. The syndrome usually develops in an injured limb, such as a broken leg, or following surgery. However, many cases of RSD involve only a minor injury, such as a sprain. And in other cases, no trigger event is evident.
RSD/CRPS is characterized by varying levels of painful burning, excessive perspiration, swelling, and sensitivity to touch. Pain may begin in one area or limb and then spread to other limbs. In some cases, symptoms of RSD/CRPS diminish for a period of time and then reveal themselves again with a new injury.
Two types of RSD/CRPS have been defined:
Type 1without nerve injury
Type 2 (formerly called causalgia)with nerve injury
Both types of RSD/CRPS share the same signs and symptoms. The condition currently known as CRPS was described by Silas Weir Mitchell during the American Civil War, who is sometimes credited with inventing the name "causalgia. However, causalgia was actually phrased by Mitchell's friend Robley Dunglison from the Greek words for heat and for pain. Millions of Americans suffer from RSD/CRPS. This chronic pain syndrome affects both men and women, and even occurs in children. It can occur at any age, but usually affects people between the ages of 40 and 60 years. CRPS has been diagnosed in children as young as two years old. It affects both male and female alike; however, CRPS triples in frequency among women compared to men. The number of reported CRPS cases among adolescents and young adults is increasing in recent years.
Investigators estimate that two to five percent of those with peripheral nerve injury, and 13-70 percent of those with hemiplegia (paralysis of one side of the body); will labor with pain from CRPS. It is theorized that certain people might be genetically predisposed to develop symptoms of RSD/CRPS after a significant or seemingly insignificant injury, such as a broken or dislocated bone. These tests are being performed by The Reflex Sympathetic Dystrophy Syndrome Association (RSDSA), American RSD Hope, and Richard G. Boles, M.D. Research began in October 2008, but the results have not yet been released as of this writing.
The National Institute of Neurological Disorders and Strokes (NINDS) reports that two to five percent of patients dealing with peripheral nerve injury and 1221% of patients with paralysis on one side of the body (hemiplegia) develop reflex sympathetic dystrophy as a further complication. The Reflex Sympathetic Dystrophy Syndrome Association of America (RSDSA) has found the condition developing after one to two percent of occurrences of broken bones.
RSD/CRPS may involve a complex exchange of signals between sensory, motor, and autonomic nervous systems, and the immune system. It is theorized that the central nervous systems control over these various processes may have been altered after an injury to the body, such as a blow resulting in deep tissue damage or a bone fracture.
Causes and risk factors associated with triggering the first flare-up of RSD/CRPS include the following:
Heart disease, heart attack
Paralysis on one side of the body (hemiplegia)
Repetitive motion disorders like carpal tunnel, back pain or bad joints
Spinal cord ailments
Shrapnel or a gunshot wound (prevalent in military veterans)
No direct cause for RSD/CRPS can be found in roughly 10-20 percent of cases. Injury that precedes the onset of the condition may or may not be significant at all.
The symptoms of RSD/CRPS often progress in three stages. They are acute, dystrophic, and atrophic stages. The acute stage occurs during the first 13 months and could present in such things as burning pain, puffiness in joints and extremities, oversensitivity reaction to touch, unusual increase in hair and nail growth in the affected area or extremity, joint pain, as well as changes in color and temperature of the area.
The dystrophic stage is characterized as constant pain and swelling. The affected limb may feel cool to the touch and have a bluish appearance. Stiff muscles, muscle atrophy and osteoporosis (early bone mass loss) also may be a feature. This stage usually develops 36 months after onset of the disorder.
During the atrophic stage, the skin becomes cool and has a sheen. Intensified muscle stiffness and weakness may happen, as well as spreading of symptoms to other parts of the body.
Signature symptoms of sympathetic nervous system involvement include the following:
A painful burning sensation
Extreme sensitivity to touch
Red or blue development of skin color
Hot or cold skin temperature
The pain of RSD/CRPS is not necessarily comparable to the degree of an injury, such as broken bone or tissue damage in that area. It can be triggered by lack of use of the arm or leg impacted or by stress. The pain may be a constant or happen sporadically.
Symptoms associated with an immune reaction include:
Swelling in area affected
Signs of motor system dysfunction may be:
Difficulty with movement
Stiffened or increased muscle
Spasms, tremors or weakness
Other symptoms of RSD/CPRS may be:
Skin inflammation, chronic rash or redness
Increase in perspiration
Patients with any chronic illness, including RSD/CRPS, often (but not always) have a diagnosis or depression and anxiety as well. Skin, muscle, and bone atrophy are likely complications. Atrophy may result from lack of use in an affected limb.
RSD/CRPS can, at times, be very difficult to diagnose. Diagnosis often involves a thorough medical history and performing a battery of neurological examinations to exclude other conditions that produce similar symptoms, rather than to pinpoint RSD/CRPS. A doctor may notice mild touch produces severe pain. A physical involves skin color and temperature observation, detection of swelling, reaction of blood flow in the extremities to stimuli or motion, overgrown and grooved nails in the toes and fingers, swollen and stiff joints, weakness and atrophy of muscles.
Differential diagnosis sometimes involves ruling out a pinched nerve. Conditions like diabetes, osteoarthritis, and obesity, as well as repetitive use, may heighten the risk for nerve disorders such as pinched nerves. Nerve compression in the extremities and spine may also be evident, as well as bone spurs, carpal tunnel syndrome and elbow nerve compression.
As for treatment of RSD/CRPS, the goals are to control pain and maintain as much mobilization of the affected limb as possible. Treatment will involve in individualized treatment plan, which often combines medications, physical therapy, nerve blocks, support groups, peer support and counseling. Treatment involves medications to control the pain of the condition so the individual can resume as typical a routine as possible. The type of medication prescribed is determined by the type of pain experienced by the patient.
Physical therapy is also part of the course of action for treating and managing RSD/CRPS, including daily range of motion exercises. Patients are advised to avoid activities that could accelerate osteoporosis or cause joint injury.
Sympathetic nerve block interrupts the transmission of pain signals from a group of nerve cell bodies. This procedure, performed by a doctor, involves inserting a needle into the appropriate location (e.g., alongside the windpipe [trachea], in the lower [lumbar] spine) and injecting a type of anesthesia into a specific group of nerve cells where the pain will most likely respond to treatment.
RSD/CRPS patients who have a good but temporary response to nerve block may be candidates for a surgical procedure called sympathectomy, which involves cutting and sealing (cauterizing) a portion of the sympathetic nerve, which runs along the spinal column. The goal is to suppress sympathetic messages in the affected area. The role of sympathectomy to treat RSD/CRPS, however, is controversial and in some cases, the procedure worsens symptoms.
A transcutaneous electrical nerve stimulation (TENS) unit, which sends impulses to affected nerves, may be used to treat the area in patients who have RSD/CRPS. In some cases, a spinal cord stimulator is permanently implanted. This device supplies low-intensity impulses to a location in the spinal cord to interrupt the pain signals that are being transmitted to the brain.
CALMAREฎ Pain Therapy Treatment, another type of treatment, is a non-invasive method for rapid treatment of high-intensity oncologic, neuropathic, and drug-resistant pain through a biophysical rather than a biochemical manner. The method incorporates electromedical equipment for nerve stimulation, and uses nerve fibers as passive pathways to send messages of normal sensitivity to the central nervous. Its described as a means of scrambling or tricking of nerve center information, enabling the CNS to change reflex response to lessen or still the pain and spasms.
Other treatments in the alternative realm that may help RSD/CRPS include acupuncture, biofeedback, Reiki, massage, water therapy, yoga, meditation. Reaching out to others, particularly of the same age and background (youth peers, disabled veteran peers, etc.) dealing with the disabling disorders are helpful to build a sense of empathy and support outside family and caregivers. Talking with someone who understands the pain and frustration of RSD/CRPS pain can make a tremendous difference.