Complex Regional Pain Syndrome
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ARTICLE BY The Princess in the Tower | princessinthetower.org Complex Regional Pain Syndrome (CRPS), is an excruciating chronic and complex disorder of the autonomic nervous system (dysautonomia), leading to constant pain that is recorded as being as severe as finger amputation without anaesthetic. It is devastating for patients as is often diagnosed too late for full remission, though it is vital to never give up hope, people can and do go into remissions but sadly [at the moment] it is only a small percentage. However, with more research being done into this complex condition, our knowledge of CRPS is evolving and hopefully, we too as patients may benefit from that research. Currently, CRPS is considered to be a condition that includes interactions between the immune system, the ANS and the Central Nervous System (CNS). There are a huge number of coexisting symptoms, many of which are incredibly hard to deal with simply as they are so contrary to both how we appear (often looking healthy) and how at odds they are to ‘normal’ healthy human behaviour. Pain being caused by sound is one example. Stating that someone needs to step back, because the allodynic pain of them standing too close is making your nerves go crazy, or asking someone to speak more quietly or stop rustling a plastic bag all sound very strange yet these things can cause our already excruciating pain to crescendo and magnifies our symptoms and discomfort. CRPS is a severely disabling condition characterised by burning pain, increased sensitivity to all stimuli, and sensations of pain in response to normally non-‐painful stimuli including: light touch, a breeze, sound, vibration, bright lights and more. In addition, CRPS is characterized by motor disturbances such as weakness, tremor and muscle spasms and sympathetic dysfunction, such as changes in vascular tone, temperature changes and increased sweating. Neurological effects of CRPS, including long term cognitive and mood changes may be incorrectly treated as existing separately from the condition, however new research will hopefully help raise awareness. It has been shown that neuropsychological deficits are present in 65% of CRPS patients, including deficits in the executive functions, for example planning, organising, self-‐awareness, self-‐regulation and initiation of action, word recall, lexical memory and conscious memory of events. Sleep is massively affected, though this is unsurprising given the CNS and ANS changes and of course, constant severe pain. CRPS is currently viewed as involving interactions between the immune system, the ANS and the Central Nervous System. In most cases an upper or lower limb is affected but spreading can and does occur to other body parts, or many different body parts at the same time (as with full body or systemic CRPS) can be involved including internal organs. CRPS may be understood as a bio psychosocial disorder, whereby psychological, behavioural and pathophysiological factors interact in a highly complex manner. Most studies of CRPS have focused on the peripheral and spinal mechanisms responsible for the origin and development of the syndrome. However, the emerging view is that the peripheral (autonomic and somatosensory) changes in CRPS must be viewed as a manifestation of changes in the brain. Complex Regional Pain Syndrome is a multifactorial disorder with clinical features of neurogenic inflamma-‐ tion, nociceptive sensitisation (which causes extreme sensitivity or Allodynia), vasomotor dysfunction, and maladaptive neuroplasticity, generated by an aberrant response to tissue injury. [[email protected]
CRPS is ranked as the highest form of chronic pain that exists in medical science today. Reading 42 on the McGill Pain Scale (RSD/CRPS is referred to as Causalgia, which is Latin for “burning pain”). CRPS is a debilitating disease if not treated promptly and properly. The onset of CRPS usually follows a trauma, injury or surgery and increasing evidence suggests that psychological trauma can cause CRPS or increase the chance of its development after an injury by an estimated eight times. Without going into a full-‐blown description of the disease let us initially concentrate on the four main symptoms: Constant chronic burning pain – also throbbing, aching stabbing, sharp, tingling, and/or crushing in the affected area or areas. Allodynia is a huge problem with RSD/CRPS (extreme pain response from innocuous stimuli); even a light breeze can cause pain, let alone the noise, lights, crowds and vibrations, all having a debilitating and life-‐limiting effect. In CRPS normal inputs such as touch, stroking and movement are misinterpreted as painful. This on-‐going “painful” interpretation is a big part of the problem. Inflammation – is not always present. It can take various forms, the skin may appear mottled, become easily bruised, have a shiny, dry, red, and tight look to it. An increase in sweating usually occurs as well. Spasms in blood vessels and muscles of the extremities – this results in a feeling of coldness in the affected extremity, which feels like ice between the bones or fire burning the affected areas. Because of an inability to regulate our inner thermostats, touching something cool can be excruciating or cause freezing or burning pains. Insomnia/Emotional Disturbance – CRPS affects the limbic system of the brain. This causes many problems that might not initially be linked to a disease like CRPS, among them are depression, insomnia, extreme difficulty concentrating, and short-‐term memory problems. Cognitive difficulties similar to fibro-‐fog are prevalent, simply due to the sensory overload of constant severe pain.
Early Treatment The main goal of treatment for CRPS is reversal of the course, amelioration of suffering, return to work if at all possible, avoiding surgical procedures such as amputation, and improvement in/some quality of life. The key to success is early diagnosis and early assertive treatment. Devastatingly, lack of proper understanding and proper diagnosis leads to improper treatment with poor outcome. There is a desperate need for future research in the treatment of CRPS. Delay in diagnosis is a factor in therapeutic failure. Early diagnosis (up to 2 years) is essential for achieving the goal of successful treatment results. Simple monotherapy with only nerve block, only Gabapentin, or otherwise, is not sufficient for management of Complex Regional Pain Syndrome. Treatment should be multidisciplinary and simultaneous: effective analgesia, proper antidepressants to reduce pain and insomnia; physiotherapy, nerve blocks, proper diet, when indicated channel blockers, and anticonvulsant therapy should be applied early and simultaneously. Administration of minimal treatments is apt to fail leading to lifelong disability and such severe pain that work is often seldom ever returned to.
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Trauma Soft-‐tissue injuries Fractures Heart disease (caused by inadequate blood supply) Spinal cord disorders Cerebral lesions Infections Surgery Repetitive motion disorder Recent research suggests that severe emotional trauma prior to a physical trauma, massively increases the likelihood of CRPS developing
It is not known why these factors cause CRPS but there are many hypotheses that are the subject of research. Severe emotional trauma such as rape or abuse has been the suggested to increase the chances of CRPS developing, with so many patients being trauma survivors. Another interesting link made by a doctor at Bath Hospital was the correlation of eating disorders in the patient histories of those who have developed CRPS. However, just as each human is unique, each case is unique. CRPS type two (causalgia) is defined by burning pain, allodynia (innocuous stimuli causing severe pain/an increase in symptoms), and onset usually occurs after nerve injury but it may be delayed. The burning pain is constant and exacerbated by: • light • touch • vibration • stress • sounds • temperature • movement of the limb (though remaining still is also painful, it can be a strange art and balance) • emotional disturbance • someone standing to close or being very animated • barometric changes Abnormalities in skin temperature and blood flow may occur as well as sudomotor dysfunction. Dystrophic changes may occur in the skin, hair or nails. CRPS vertigo and a whole host of ANS-‐dysfunction symptoms may be experienced. The pain of CRPS is constant and characterized, at least initially by burning. Not in a descriptive sense but as if your limb or limbs are actually in a fire. This unrelenting pain is enhanced with every movement or stimulus. Allodynia is involved (innocuous stimuli causing severe pain), making socialising even more complicated and painful. Even a light breeze is enough to make the pain rocket so having hectic people around massively increases the pain.
Inflammation Swelling is sometimes localized, but often relentless and progressive. Swelling intensifies the pain and promotes stiffness, which can be the beginning of atrophy and deformity. Keeping the movement going is crucial! When tissue is injured or inflamed, excess fluid enters the tissues from damaged blood vessels within these injured tissues. If the veins cannot remove all of this fluid, the part swells (oedema). However this swelling is usually only temporary, because the tissues heal and the blood vessels no longer leak excessively. Swelling is one of the symptoms of CRPS. Early in the course of the disease, this inflammatory process causes oedema. The swelling in CRPS may exist far longer than it would take normal tissue to heal because CRPS: • Prevents healing • Causes constant inflammation • May cause dilation of the arteries which will cause more fluid to leak, and may cause the veins to contract, which also prevent the normal removal of non-‐protein fluid from tissues. • The oedema of RSD may last for long periods of time • STIFFNESS, like swelling, is progressive resulting in less motion of the joints, which again, results in increased swelling and pain. This in turn, can produce further deformity and joint changes. • DISCOLOURATION indicates circulatory changes that diminish the nutrition of the tissues of the skin, ligaments, bones and tendons. The result is thin, shiny skin, pencil-‐like fingers and changes in ligaments. This further contributes to stiffness and pain. CRPS in the upper extremities had been classified in the following five ways, based on the location and intensity of symptoms. Traditionally, complex regional pain syndrome was seen as a three-‐stage disease; however, these stages are increasingly being seen as theoretical as all the features may not be present and the speed of progression varies hugely between patients. Some patients never actually progress to stage III, while others get to stage III and lose some of the symptoms of the earlier stages. Early recognition of the disease, correct diagnosis, and proper treatment, are all essential in keeping RSD from becoming a chronic, life-‐long condition. Treatment must begin within months of onset, ideally within three months. Here are some of the many symptoms of CRPS: • Skin temperature, skin colour changes • Pain caused by innocuous stimuli, for instance sound, vibration, light touch, even someone in the room can increase pain. Allodynia is pain from thing that you would never expect to cause pain, while hyperalgesia is an exaggerated pain response, so far more pain than should result from a stimulus. • Tremors, shakes, spasms and muscle (dystonia) • Temperature changes and inability to regulate temperature • Changes in hair/nails/skin • Sweating changes, sometimes sweating varies from one side of the body to the other • Fluid build-‐up causing swelling (oedema) • Lower bone density as they become more porous (osteoporosis) • Avoiding using the part of the body which hurts, which then causes additional problems like the muscles starting to waste away through lack of use (atrophy) • Central Nervous System (CNS) dysfunction and hyperactivity. The CNS is made up of the brain and spinal cord which use the information from the wider spread ANS to control and co-‐ordinate what we do. The CNS can do strange things when it’s dependent on a faulty ANS for it’s information. As the condition develops the pain becomes continuous, it is truly relentless. There are brief periods where it may not be as severe, but considering that the pain experienced is off the scale of previous personal experience for most patients, the lesser pain moments are still excruciating by ‘normal’ standards. No wonder the natural response is to stop using whichever part of our body is affected. [[email protected]
Complex Regional Pain Syndrome is frequently dismissed by health professionals for many reasons including: • They don’t understand the diagnosis and/or they are not familiar with the disorder. • They understand the diagnosis but lack experience in how to treat it properly. • Many think that the client is pretending to be ill or exaggerating their pain. • CRPS is thought to be hopeless and there is no cure. • CRPS is purely psychological and is not a medical condition • Many people who work within the health care system dread accepting a client with CRPS because they know that effective treatment requires an on-‐going, almost daily assessment of the condition to develop the proper regimen. It is far too time consuming for most clinics to adequately care for the patient. Generally, doctors like to cure not manage chronic illness. • Due to the nature of CRPS, the condition can quickly change for better or worse for reasons that are not fully understood. Therefore it is necessary to schedule evenly spaced treatment sessions in order to benefit, which is often not possible for many patients who now are unable to work. • The health care provider must address the plan of care very carefully once the diagnosis is made and must thoroughly customise therapy for each Complex Regional Pain Syndrome patient. Many patients suffer needlessly through a lack of understanding from their GP/doctor, resulting in insufficient pain management that causes additional stress on an already over-‐taxed body. All individual characteristics (psychological, social, physiological) must be taken into account during therapy. Communication between the family members, health professionals, and the patient must be clear, on-‐going and well established. It is common for the patient to have failed in a previous program if a positive, creative, caring relationship was not in place. If either the patient or the therapist senses a communication problem, it is far better to acknowledge that another clinician may be of greater benefit to the patient’s progress.
How is Complex Regional Pain Syndrome treated? Everyone with CRPS needs good medical support and treatment. While this is not always possible, especially if you live in a rural or remote area, princessinthetower.org can help guide you in things that help improve your pain and function. Because pain involves the whole person, to get the best outcomes treatments and management usually require a combination of some of the following approaches: • Pacing • Relaxation techniques; yoga; breathing; Tai Chi; Chi Kung etc. • Mindfulness based relaxation • Gentle movement and mobilisation techniques • Coping and Managing your pain • Use of pain medicines and medical procedures • Complimentary medicine The primary task is to eliminate or treat all possible causes. Successful treatment of CRPS is dependent on: • Early diagnosis. If diagnosed early, the prognosis is very good. • Begin treatment of the underlying cause, if there is one. If not, then focus on the treatment of the CRPS process. • The key approach is to provide adequate pain relief in order to undertake rehabilitation with the primary aim of restoring function as early as possible. Also it is vital to control pain so that pain pathways in the brain do not become maladaptively rewired, making recovery even more difficult. [[email protected]
What Does CRPS/RSD Feel Like?
CRPS pain can be anywhere in the body where there are nerves. Here Keith Orsini answers the question: what does it feel like? “Well, imagine your hand was doused in gasoline, lit on fire, and then kept that way 24 hours a day, 7 days a week, and you knew it was never going to be put out. Now imagine it both hands, arms, legs, feet; well, you get the picture. I sometimes sit there and am amazed that no one else can see the flames shooting off of my body. The second component to CRPS is Allodynia. “Allodynia is an extreme sensitivity to touch, sound, and/or vibration. Imagine that same hand now has the skin all burned off and is completely raw. Next, rub some salt on top of it and then rub some sandpaper on top of that! THAT is allodynia! Picture getting pretty vivid? Now, because of the allodynia, any normal touch will cause pain; your clothing, the gentle touch of a loved one, a sheet, rain, shower, razor, hairbrush, shoe, someone brushing by you in a crowded hallway, etc. “In addition, sounds, especially loud or deep sounds and vibrations, will also cause pain; thunder, loud music, crowds, singing, yelling, traffic, kids screaming, loud wind, even the sound in a typical cinema. This is what allodynia is all about. Imagine going through your daily life where everything that you touch, or that touches you, where most every noise around you from a passing car to children playing, causes you pain. In addition to the enormous pain you are already experiencing from the CRPS itself. Imagine living with that pain and allodynia 24 hours a day, every day.” ~ KEITH ORSINI
Fibromyalgia and CRPS Although many CRPS patients are later diagnosed with Fibromyalgia, the two are frequently confused but remain vastly different in terms of day-‐to-‐day living and severity of symptoms. Some fibromyalgia patients are completely debilitated but even they can have moments of reduced pain. CRPS is constantly at a severe level and personally, I feel the difference of character between the two pains also confirms the differences between the two pain conditions. There may be a continuum, with dysautonomia and central sensitization being present in both but there are clearly distinct symptoms that are present in CRPS that are absent in fibromyalgia.
When it All Gets Too Much
The suicide rate among RSD/CRPS sufferers is extremely high due to the intensity of the never-‐ending pain, sleep deprivation, frustration, social isolation, misunderstanding, and lack of support from medical professionals, family and friends. If you are a patient suffering from depression and contemplating suicide, please, please get help – Suicide Prevention / Depression Support: • Pain Association Scotland: 0800 783 6059 • Pain Concern UK: 01620 822 572 • Painline: 0845 603 1593 • Samaritans (National and local): 08457 90 90 90 Feeling suicidal is not a character defect, and it doesn’t mean that you are crazy, or weak, or flawed. It only means that you have more pain than you can cope with right now. No matter how you feel in this moment, and no matter how impossible it may seem, things can and do improve. Life with severe, constant pain is hard, very hard. There will be times when what is already excruciating flares up to being beyond comprehension in terms of pain, and it is these times where you must take extra special care of yourself and mind. Know that you’re not alone; the human mind can sometimes be your own worst enemy, especially when bottling up how you’re feeling both physically and mentally. Consider joining a community who knows how you’re feeling and speak with members who have first-‐hand experience with depression stemming from Complex Regional Pain Syndrome.