Recent research has indicated that more people in the United States are suffering from Reflex Sympathetic Dystrophy (RSD) than Breast Cancer, HIV and Multiple Sclerosis combined, yet there is so little attention paid to this devastating disease, and so little is know about it. Currently it is estimated that, in the United States, approximately 4,237,500 people suffer with RSD, but only a minimal amount of research is being done.
RSD, also known as Complex Regional Pain Syndrome (CRPS), is a chronic and often times crippling neurological disorder that can strike anyone, at any age, in any physical condition. The National Institute of Neurological Disorders and Stroke (NINDS), describes RSD as "a chronic pain condition that is believed to be the result of dysfunction in the central or peripheral nervous systems.” There is relatively little known about this elusive disease, and it appears that just about everyone is vulnerable to it; there is no apparent predisposition nor are there any genetic indicators signifying a likelihood of contracting the disease. In clinical terms, it is an irritation and abnormal excitation of the nervous tissue leading to abnormal impulses along the nerves that affect blood vessels and skin. In simpler terms, the nerves begin to malfunction and misfire, sending rouge pain signals. The body receives and reacts to the signals, but fails to shut them off. The result is that the patient experiences significant sensations of pain in any number of places in their body. The pain is constant and relentless, varying in degree and intensity, and tends to worsen over time.
The onset of the disease can be sudden or gradual, and the types of pain have been described in a number of ways, such as aching, burning, crushing, electric shocks, feeling as if your on fire, sharp, stabbing, throbbing, tingling, and any combination thereof. The pain can start in one area of the body (i.e. feet and legs) but spread to other areas (i.e. upper extremities, neck, back) as the disease progresses. Aside from the constant pain, other common symptoms include:
- Pathological changes in bone and skin, including bruising, lesions and rashes.
- Excessive sweating, even though the victim may feel cold, due to an increased inability of the body to regulate its own temperature
- Tissue swelling and edema in and around joints, arms, legs, face and other parts of the body, as an internal response to the sensation of pain
- extreme sensitivity of the skin to touch
- Exhaustion, from the body being under constant physical stress
- Inability to sleep
- Weakening of the bones
- Muscle spasm
- Muscle atrophy
- Visual changes or disturbances (i.e. blurred vision, spots or halos)
- Auditory changes or disturbances (i.e. ringing in the ears, throbbing sensation in the ears)
- Severe headaches and/or migraines
- Memory problems (especially short term)
- Changes in speech patterns
- Changes in vocal tone due to atrophy of the vocal chords
- Mood swings and irritability
- Depression and/or suicidal thoughts
- Skin color changes (blotchy, purple, pale, red)
- Skin texture changes (shiny, thin, sweaty)
- Changes in nail and hair growth patterns Decreased ability to move affected extremities.
Some research suggests that there are three basic stages to RSD: Acute, Dystrophic, and Atrophic. During the first stage (Acute), said to last from 1 to 3 months, there is severe, burning pain, usually isolated to a particular area of the body. The pain is usually accompanied by muscle spasm and joint stiffness. There can also be noticeable increase in hair growth and apparent skin color and temperature changes. In the second stage (Dystrophic), which generally lasts from 3 to 6 months, the pain becomes more intense and may spread to other areas of the body. Swelling of the joints and affected areas becomes more pronounced. Muscle tone weakens and hair growth decreases. The bones become softer and the nails may become cracked and brittle, having a grooved or spotty appearance. In the third stage (Atrophic), the affects of the disease on the skin and bones become irreversible. The pain is severe and continuous and the muscle atrophy is greatly increased. Mobility is significantly limited and the muscles and tendons may become subject to extreme contractions, causing the limbs to take on a twisted appearance. Note, though, that the theory that RSD proceeds in stages is still open to some debate, and the stages as described may vary widely from patient to patient.
WHAT CAUSES RSD?
The exact cause of RSD is yet unclear, although many “trigger” events have been documented as preceding the onset of RSD. The most common trigger for the disease appears to be a physical injury or trauma of some sort, often relatively minor in comparison to the severity of the disease ( i.e. a needle stick, a sprained ankle, a cut or burn). Another trigger could be the existence of an underlying disorder (diabetes, heart disease, cancer, brain disorder). It is estimated that about one third of patients with RSD cannot identify a “trigger” event for their symptoms. As far as explaining the actually physical response of the body to those trigger events, some studies suggest that after a tissue or nerve injury, a chemical called norepinephrine, normally released from sympathetic nerves, develops the capacity to activate pain pathways thus causing the varying sensations of pain. Another theory suggests that the injury or trauma sets off an immune response within the body, causing the often painful symptoms associated with it. Generally, the pain begins in a limited area (i.e. the feet or legs) but, as the disease progresses, the pain can spread to other parts of the body.
HOW IS RSD TREATED?
Some research has shown that RSD is most effectively treated within the first three months of its onset. However, as of today, there is not a definitive quantitative test to determine if a patient has RSD. Generally, the diagnosis is made by observing the patient’s clinical symptoms and ruling out other causes. This is troublesome in and of itself since it can cause a delay in diagnosis and treatment of the disease during those crucial early months.
There is no known cure for RSD and the goal of treatment is to minimize the symptoms enough to allow the patient to tolerate daily life. A plethora of medications have been used in the attempted treatment of RSD symptoms, with very limited success. They include the following:
- Aspirin and acetaminophen (also known as Tylenol)
- Anti-convulsants such as Carbamazepine (Tegretol), Gabapentin (Neurontin), Phenytoin (Dilantin)
- Antidepressants such as Amitriptyline, Nortriptyiline
- Corticosteroids to reduce inflammation and swelling ( i.e. Calcitonin spray)
- Muscle relaxants such as Baclofen (Lioresal) and Klonopin
- Non-steroidal anti-inflammatory drugs (N-SAIDS)
- Narcotic analgesics
- Opioids (used when the pain becomes widespread)
- Intrathecal drug delivery (drugs delivered into the spinal fluid through the spinal cord or delivered through a pain pump- pain pump drugs include morphine and Baclofen)
Unfortunately, medication alone often will not control the symptoms. Other available treatments have been developed as follows, but have also met with only a very limited degree of success for long term pain management, especially in advanced cases of RSD. They include the following:
Topical pain patches, such as Lidoderm, Lidocaine, Fontanel and Clonidine.
Radiofrequency procedure (risks include new nerve injuries, bleeding, allergic reactions to the medications being used, seizures and the stress and fear about the procedure).
Sympathetic nerve block
Hyperbaric Medicine, which advances wound healing, increases the delivery of oxygen to injured tissues, encourages greater blood vessel formation, preserves damaged tissues.
Tens Unit (nerve stimulator), which provides small amounts of electrical nerve stimulation, attempting to overcome the sensation of pain
Acupuncture (risks include worsening inflammation, intensification of pain, nerve irritation and or injury, infections, poor wound healing, and skin irritations).
Physical Therapy and Pain Management Therapy
Sympathectomy: an invasive surgical procedure whereby the sympathetic ganglion nerve bundle is removed. This procedure is extremely high risk because, if it does not eliminate the symptoms, it may exacerbate them considerably, and is often considered in certain specialized circumstances and even then only as a last resort.
Spinal Cord Stimulator: Another invasive procedure in which a stimulator is attached to the spine interrupting the pain signals being sent to the brain. Again, this is not a cure, but may result in a reduction of pain to a more manageable level for an extended period of time.
The latest treatment that has showed promise in treating RSD, and even sending it into complete remission involved the use of the drug Ketamine.
There are currently three types of Ketamine treatments (or protocols), namely, Outpatient, Inpatient and Coma. All three protocols have opponents and proponents in the medical field, but for many RSD patients this option has become a glimmer of hope and possibility of returning us to a life of normalcy. According to NIDA Research Report Series entitled Hallucinogens and Dissociative Drugs (2001), Ketamine is an odorless and tasteless drug that is found in liquid, pill, and powder form. It is classified as a type of dissociative drug in that it alters the actions of the neurotransmitter Glutamate throughout the brain. Glutamate is involved in perception of pain, responses to the environment, and memory.
Outpatient Protocol (sometimes called “boosters”): Outpatient treatment is usually done over a 5-10 day period. Ketamine is infused intravenously over an approximate four hour period. Studies have shown that this treatment generally results in a reduction of pain, although the relief is generally temporary, and the pain returns after a relatively short period of time.
Inpatient Protocol: This treatment is typically done over 5 continuous days of IV infusion with a combination of Ketamine and Clonidine while the patient is in the hospital. The patient will typically undergo outpatient booster treatments after the inpatient protocol is complete to extend any relief obtained from the inpatient procedure. The success rate with this procedure is higher than with outpatient treatment alone, but results still vary from patient to patient.
Coma Protocol: Patients are placed into an induced coma with a high dose of Ketamine run intravenously for 5 to 7 days. Two, even three, such treatments may be required before any lasting effect is achieved, but currently, this protocol seems to hold the most promise in the treatment of advanced RSD. Unfortunately, this procedure is not FDA approved in the United States so patients currently must travel to Monterey, Mexico with a team of American doctors to obtain this treatment. It has been extremely difficult to get approval for the procedure in the United States because the FDA is requiring a “Double Blind Study” (one in which patients are placed into a drug-induced coma and some are given the drug in question while others are given a placebo). Due to the risks of placing people into a drug-induced coma state only to administer a placebo, such a study will be nearly impossible to obtain. Still, there are a number of RSD patients who have gone into remission with this treatment, and it remains the glimmer of hope for many patients, although it is financially prohibitive to most.
There is still too much medical science does not know about RSD, and research into this devastating disease has been sporadic at best. Still, millions of people in the United States alone are crippled by it every year—worldwide the numbers are even greater. It is time that the victims of RSD and their families had a voice; it is time to give those who suffer from this disease some hope for their future.
If you are reading this, you can be that voice; you can be that hope.