What is Complex Regional Pain Syndrome?

Distracted in a lecture, I watched as a fellow classmate rubbed his hand over his left wrist consistently throughout the class. It wasn’t long before he caught me staring and quickly whispered something about breaking his hand a few years ago. Embarrassed at having stared for too long, I nodded, smiled and looked away, but curiosity still had the better of me; why, if this person had broken their hand so long ago, were they still acting as if it hurt? Going back to my lecture, I forgot about this incident, until months later when I sprained my own ankle, and for months after it had supposedly healed, I still felt it ache on a daily basis. Suddenly remembering my classmate and his old aching injury, I set off to the internet, which lead me to discover term Chronic Regional Pain Syndrome. I, as it turned out, did not have this condition, but curiously enough, it is estimated that 90 percent of people who experienced injuries and traumas develop the syndrome.

Have you heard of Chronic Regional Pain Syndrome? Unless you have it or you are involved in the medical world, chances are high that you probably haven’t. At least I hadn’t heard of it before then, and even though it is a rather common affliction, it still eludes many people’s understanding. As with a variety of other medical conditions, much about this ailment is hard to comprehend, especially at first. The main symptom of this syndrome is pain that lasts longer than what it is assumed to take for the initial injury to heal, and that can range from mildly uncomfortable to incredibly painful. The pain may stay in the immediate area of the injury, or it can spread as far as to the opposite body limb, or in the case of my classmate, spread from the hand up to the wrist. I may not know with certainty that this classmate had CRPS, but the idea is similar, it is condition where pain from an injury remains and sometimes worsens even after the initial trauma has healed. This can occur at any time and can happen to anyone, though it is something that manifests after an injury has taken place, such as a fracture, a bruise, burn or cut, or sometimes even after a medical procedure like surgery. Sometimes there are physical symptoms such as changes to the color of the skin on or around the site of affliction, there can be stiffness, strange positioning of the limb, spasms and more, although sometimes there are no visible changes to the immediate area at all.

How is it that condition develops? Though much is still unknown on how this condition occurs and why, some researchers believe that it is caused by nerve damage experienced in the initial injury that later contributes to sending pain signals from the afflicted area. It is estimated that of those who experienced peripheral nerve injury have a 1% chance of developing CRPS. The condition is one in which many factors contribute to its manifestation, and commonly observed features are neurogenic inflammation, which is a swelling within the central nervous system, and extreme sensitivity in the affected area called nociceptive sensation. CRPS also manifests with vasomotor dysfunction, where in blood vessels fail to regulate their diameter correctly, causing swelling of the affected area, reducing the flow of oxygen to the muscles underneath, which then can cause painful spasms, tightening and discoloration. Overall there are many factors that contribute to the manifestation of CRPS, and because of this complexity the pathophysiology of the condition has yet to be defined, also leading some researchers to believe that there could be not one, but multiple pathophysiologies for CRPS.

Diagnosis of this condition involves categorizing the CRPS into either type I or type II, which are only different on the basis of initial injury, type one involving an injury that could have been traumatic in nature and type II occurring after a nerve injury itself. Though there is no single or specific test for diagnosing this condition, observation of the physical changes and symptoms to the affected area are a starting point from which doctors go off on. Further tests include thermography, x-rays, electrodiagnostics and administration of sympathetic blocks in order for doctors to obtain an understanding of the patient’s condition. The complex manner in which the symptoms appear and the uncertainty of how they may occur in individual patients along with the duration and severity of the condition makes diagnosis of this condition difficult to establish, and may sometimes leave patients suffering while the conclusions are drawn.

Adding to the complicated nature of diagnosis, the condition can be incredibly debilitating, leaving some sufferers with life-long complications that can interfere with their day-to-day activities, putting some of them out of work and sometimes homebound. Despite these possibly life changing outcomes, CRPS and its symptoms and consequences, is still widely unknown amongst the medical community, and even less so amongst the patient population. Because of this lack of awareness, there are several organizations dedicated to drawing more attention and understanding towards this condition, such as Color the World Orange. This organization, which was founded in 2014, organizes an annual event to rally and raise awareness of CRPS to the general public. In their events, the organization aims to inform the public about this condition and to help them better understand how the condition effects those afflicted and how it can be managed. The motto of this organization is “let’s show the world that while we are in pain, we are strong,” and they state that one of their main goals is to bring to attention the mental, emotional and psychological tolls that CRPS can have on those afflicted and the family members or caretakers involved. No one likes to be in pain, and even less so when it is constant with no clearly identifiable cause, and sufferers are always in a state of pain that may prevent them from working and living independently, leading them to experience anxiety, states of depression and sometime even post-traumatic stress disorder, all of which can lead to a heightened perception of pain. Because of these debilitating consequences and the little awareness surrounding the condition, Color The World Orange holds a rallying event every first Monday in November in order to raise awareness for doctors, patients and the general public.

So far, treatments for Chronic Regional Pain Syndrome include physical therapy, psychotherapy and medications, all which can range from nerve blocks, pain killers and topical creams. Physical therapy regimens that are used in treating CRPS include mirror-box therapy, a technique used in the treatment of amputation patients who experience phantom limb pain, pain associated or felt in a limb that is no longer physically there. This technique is one in where an illusion using a mirror is created in order to trick the patient’s brain into thinking that the limb in the mirror is the affected limb. This visual feedback makes the patient believe that the limb in the mirror is the affected limb and watching it move without pain or struggle is thought to relieve the amount of pain experienced. Another popular forms of therapy includes contrast baths, the use of hot and cold baths to help stimulate the tissues of the affected limb by increasing blood flow and decreasing swelling, relieving the pain in the affected area. Along with the physical therapy, CRPS patients can be given certain neuropathic pain medications such as gabapentin, a medication that is usually used for the treatment of seizures but can be helpful in relieving nerve pain. Nonsteroidal anti-inflammatory drugs, NSAIDs, are also typically prescribed for CRPS patients to reduce inflammation of the affected area. Medicinal treatments can also include ketamine or lidocaine infusions, where the medication is delivered intravenously to alleviate pain by blocking the receptors in the brain and spinal cord, though those require special supervision and are conducted under EKG monitoring. There are also sympathetic nerve block treatments that target specific regions affected, such as Stellate ganglion blocks for upper extremities affected with CRPS and lumbar sympathetic blocks, which target lower extremities affected by the condition. Both of these treatments work on the same basis where a mixture of local anesthetics, steroids and opioids is injected into or around the sympathetic nerve in the area where pain is originating.

If the condition of the patient is chronic and the treatments are not having significant effects, there is the more invasive method of treatment which is that of spinal cord stimulation, or SCS. This technique is one in which a small device is surgically implanted under the skin that has wires leading from it to the patient’s spinal cord. This device can then send mild currents of electricity to the spinal cord which disrupts the currents of pain being sent to the affected area, relieving the pain felt. This method of treatment has undergone various studies to test its effectiveness and other possibly long-term effects, and in one particular study conducted, the results of a year-long treatment of SCS indicated a 35% reduction in pain felt in the affected area. Though this device does not cure nor completely eliminate the sources of the patient’s pain, it helps manage and relieve the level of pain that is felt, helping in improving the patient’s mental state and quality of life. Like the manifestation of the symptoms, treatment for this condition can be dependent on the patient’s individual case, and the results seen through use of SCS can also vary depending on the patient’s particular status, initial injury, and level of nerve damage and pain. Despite the many treatments available for sufferers of this condition, much research still needs to be done to find what it is that causes such prolonged pain. It is easy to think that once an injury has healed, it will no longer hurt, but that can be seen as untrue due to CRPS, and there are many people who suffer from this, therefore it is important to be aware, for both patients and doctors, of this disorder.

 
References:

  1. http://www.ninds.nih.gov/disorders/reflex_sympathetic_dystrophy/detail_reflex_sympathetic_dystrophy.htm

  2. http://www.colortheworldorange.com/news/#press-releases

  3. http://www.medscape.com/viewarticle/430537_5

  4. http://healthcare.utah.edu/paincenter/treatments-conditions/lidocaine-infusion.php

  5. http://www.hopkinsmedicine.org/healthlibrary/test_procedures/orthopaedic/sympathetic_nerve_blocks_for_pain_135,54/

  6. Van Eijs F., Geurts J.W., Van Zundert J., Faber CG., Kessels A.G.H., Joosten E.A.J., van Kleef M. 2011. Spinal Cord Stimulation in Complex Regional Pain Syndrom Type I of Less Than 12-Month Duration. Neuromodulation 2012; 15: 144-150

Susan ParragaComment