Some early or mild CRPS cases recover on their own, but treatment is most effective when started early. It’s important to understand that treatment is multi-focal and there is no single treatment that can cure the disease.
Here are several primary therapies that are widely used.
Rehabilitation and physical therapy
This is probably the single most important treatment for CRPS. Keeping the painful limb or body part moving improves blood flow and lessens circulatory symptoms, as well as maintains flexibility, strength, and function.
Rehabilitating the affected limb helps prevent or reverse secondary spinal cord and brain changes associated with disuse and chronic pain. Occupational therapy can help people learn new ways to become active and return to work and daily tasks.
Therapists need to be trained and experienced in CRPS treatment options. The “no pain, no gain” concept does not apply to CRPS. Pushing a patient too hard can cause extreme pain well beyond the therapy sessions, so experienced therapists will adapt therapies to the individual patient.
Psychotherapy
People with severe CRPS often develop secondary psychological problems including depression, situational anxiety, and sometimes post-traumatic stress disorder. These heighten pain perception, further reduce activity and brain function, and make it hard for patients to seek medical care and engage in rehabilitation and recovery.
Psychological treatment helps people with CRPS to feel better and better recover from CRPS.
Graded motor imagery/mirror therapy
Individuals are taught mental exercises including how to identify left and right painful body parts while looking into a mirror and visualizing moving those painful body parts without actually moving them. This is thought to provide non-painful sensory signals to the brain that helps reverse brain changes that are prolonging CRPS.
Medications
Several classes of medication have been reported as effective for CRPS, particularly when given early in the disease. However, none are approved by the U.S. Food and Drug Administration (FDA) to be marketed specifically for CRPS, and no single drug or combination is guaranteed to be effective for everyone.
Since their use is off-label, reimbursement from insurance plans may be more difficult. Drug regimens can be complex and need to be tailored to the patient and closely monitored for adverse reactions.
Drugs often used to treat CRPS include:
- Acetaminophen to reduce pain associated with inflammation and bone and joint involvement.
- Non-steroidal anti-inflammatory drugs (NSAIDs) to treat moderate pain and inflammation, including over-the-counter aspirin, ibuprofen, and naproxen in sufficient doses.
- Drugs proven effective for other neuropathic pain conditions, such as nortriptyline, gabapentin, pregabalin (Lyrica), and duloxetine. Lyrica is very similar chemically to gabapentin but has been reported as more effective with fewer side effects. When these drug regimens are established, It is important not to suddenly stop the medications as seizures or severe transient psychological episodes may occur.
- Antioxidants such as Vitamin C and alpha-lipoic acid have value in reducing free-radical damage to nerves and tissue. Vitamin C has been demonstrated to reduce the risk of CRPS onset in surgeries with an increased risk of CRPS such as carpel tunnel of the wrist and total knee arthroplasty. There is an OTC alpha-lipoic acid supplement called Nervive now available to promote nerve health and healing. The use of these antioxidants in existing CRPS patients helps promote the repair of nerve fibers.
- Topical local anesthetic ointments, sprays, or creams such as lidocaine and patches such as fentanyl. These can reduce allodynia, and skin coverage by patches can provide additional protection.
- Bisphosphonates typically used in osteoporosis, such as high dose alendronate or intravenous pamidronate, may reduce bone changes that can trigger pain.
- Corticosteroids that treat inflammation/swelling and edema, such as prednisolone and methylprednisolone. Steroids may be especially useful if the patient is diagnosed in the early stages of the disease, but prolonged use of the drug requires careful monitoring.
- Botulinum toxin injections can help in severe cases, particularly for relaxing contracted muscles and restoring normal hand or foot positions.
- Opioids such as oxycodone, morphine, hydrocodone, fentanyl, or methadone may be required for individuals with the most severe pain. However, opioids can convey heightened pain sensitivity and run the risk of dependence and respiratory depression. Patients must be trained to not exceed prescribed doses and monitored periodically for compliance and adverse effects.
- As an alternative to opioids, the opioid-antagonist naltrexone may be used if the patient has not been using opioids recently. Low-dose naltrexone (LDN) as a nasal spray can be made at a compounding pharmacy and has demonstrated pain reduction in some CRPS patients. LDN reduces glial cell activation in the brain and stimulates endorphin release.
- N-methyl-D-aspartate (NMDA) receptor antagonists such as dextromethorphan, amantadine, and especially ketamine are somewhat controversial but potentially useful treatments.
- Recently, a new class of drugs known as CGRP antagonists (e.g. Aimovig, Ajovy, etc) has been approved to treat migraine headaches. These drugs work by blocking glial cell activation in the brain. Glial cell activation also plays a role in CRPS, so the efficacy of these compounds in the treatment of CRPS needs to be evaluated. Note: TJ’s Foundation is looking to explore the possibility of a grant with NIH to fund a pilot clinical trial for the off-label use of these compounds for CRPS.
Spinal cord stimulation
Stimulating electrodes are threaded through a needle into the spine outside the spinal cord. They create tingling sensations in the painful area that help block pain sensations and normalize signaling into the spinal cord and brain.
Electrodes can be placed temporarily for a few days to assess if stimulation is likely to be helpful. Minor surgery is required to implant the stimulator, battery, and electrodes under the skin on the torso.
Once implanted, stimulators can be turned on and off and adjusted with an external controller. Only about 50% of patients show significant improvement and there is a risk of electrode migration and infection.
Other types of neural stimulation
Implanted neurostimulation can be delivered at other locations including near injured nerves (peripheral nerve stimulators), under the skull (motor cortex stimulation with electrodes), and within brain pain centers (deep brain stimulation).
Recent noninvasive commercially available treatments include nerve stimulation at the peroneal nerve at the knee. Another is repetitive Transcranial Magnetic Stimulation or rTMS, a noninvasive form of brain stimulation that uses a magnetic field to change electrical signaling in the brain.
Similar at-home use of small transcranial direct electrical stimulators is also being investigated. These stimulation methods have the advantage of being non-invasive; however, repeated treatment sessions are needed to maintain benefit, so they require time.
Spinal-fluid drug pumps
These implanted devices deliver pain-relieving medications directly into the fluid that bathes the nerve roots and spinal cord. Typically, these are mixtures of opioids and local anesthetic agents such as lidocaine, clonidine, and baclofen. The advantage is that very low doses can be used that do not spread beyond the spinal canal to affect other body systems. This decreases side effects and increases drug effectiveness. Drug pumps carry an elevated risk of infection and need to be monitored.
Alternative and holistic therapies
Based on studies from other painful conditions, some individuals are investigating accessible treatments such as medical marijuana, behavior modification, acupuncture, relaxation techniques (such as biofeedback, progressive muscle relaxation, and guided motion therapy), and chiropractic treatment.
These do not benefit the primary cause of CRPS, but some people find them useful. They are generally accessible and not dangerous to try. TJ found medical cannabis therapy helpful in controlling his pain and promoting sleep.
Limited-use therapy for the most severe or non-resolving pain that has not responded to conventional treatment
The most promising drug in this category is ketamine. Some investigators report benefits from low doses of ketamine—a strong anesthetic—given intravenously for several days in a strictly monitored clinical setting.
Ketamine has been shown to be useful in treating pain that does not respond well to other treatments. However, it can cause side effects such as temporary delusions. Periodic booster doses are required.
Also of interest, low-dose ketamine has been approved for the treatment of depression that is resistant to other drugs. The possibility exists that this delivery system may prove useful for CRPS and needs to be evaluated.
Important note: Any diagnosed or suspected CRPS undergoing surgery should ALWAYS have ketamine incorporated into the anesthesia protocol to prevent the spread of the condition.
Treatments to avoid
These former treatments now rarely used should be avoided if possible:
Sympathetic nerve block
Previously, sympathetic nerve blocks—in which an anesthetic is injected next to the spine to directly block the activity of sympathetic nerves and improve blood flow—were used. More recent studies demonstrate no long-lasting benefit after the injected anesthetic wears off and there is the risk of injury from needle injections, so this approach has fallen from favor.
Surgical sympathectomy
This destroys some of the nerves that carry pain signals. Use is controversial; some experts think it is unwarranted and makes CRPS worse, while others report occasional favorable outcomes. Sympathectomy should be used only in individuals whose pain is temporarily dramatically relieved by sympathetic nerve blocks.
Cutting injured nerves or nerve roots
People with CRPS often ask if cutting the damaged nerve above the site of injury would end the pain. In fact, this causes a larger nerve lesion that will affect a larger area of the limb.
Also, the spinal cord and brain react badly to being deprived of stimulation which can result in central pain syndromes. Other than in exceptional circumstances such as palliative care, this should not be performed.
Amputating the painful lower limb
This is an even more drastic and disabling form of nerve cutting, and the consequences are irreversible, whereas CRPS usually improves over time, albeit sometimes slowly.
Amputation is thus not appropriate for pain control alone, but it is rarely required to manage bone infection or to permit the use of a prosthesis for long-affected non-recovering individuals.
This last resort should not be performed without input from several specialists along with psychological counseling. There is no guarantee that amputation will relieve the pain as some amputees develop phantom pain where the limb was amputated. However, lessons learned by the military in severely wounded Iraq and Afghan combat veterans have improved the success rate of the procedure.