Renee J. Hill, M.S. US Pain Foundation Vice President Director of Research Studies uspainfoundation org

Renee J. Hill, M.S. US Pain Foundation Vice President Director of Research Studies uspainfoundation org www.uspainfoundation.org research@uspainfoundation.org www.awarenessthroughed.com

Precautions U.S. Pain Foundation cannot provide a diagnosis or individual treatment advice via webinar. Please consult your physician about your specific health care needs. This information is intended for educational purposes only. Consult your doctor and health team If you are planning to increase your physical activity or start an exercise program. Consult your doctor if you are planning on discontinuing or starting a new treatment regimen.

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y CRPS I- minor trauma: hairline fracture, immobilization of a limb, cryotherapy, venomous bite, electrical burn, and other minor injuries. 2 y CRPS IIII Injury with know nerve damage: fractures, fractures surgeries, trauma, dislocations, electrical burns, and other serious insults that cause nerve damage. g 2 Acute = Warm Chronic = Cold

1. The presence of an initiating noxious event or a cause of Immobilization 2. Continuing gpain, allodynia, or hyperalgesia in which the pain is disproportionate to any known inciting event 3. Evidence at some time of edema, changes in skin blood flow, or abnormal sudomotor activity in the region of pain 4. This diagnosis is excluded by the existence of other conditions that would otherwise account for the degree of pain and dysfunction Not required for diagnosis; 5 10% of patients will not have this. Many symptoms are not visible to others, and often people with the syndrome don t look sick. However, research has proven that CRPS is a very real condition. Do not confuse with Conversion Disorder, Pain Disorder, Factitious disorder, Munchausen's s disorder in the DSM-IV-TR and ICD-10 www.rsds.org

The Autonomic Nervous System

The Acute Stage can last from one to three months from onset. Some characteristics are warmth, coolness, burning pain, edema, increased sensitivity to touch, increased pain, accelerated hair/nail il growth, tenderness or stiffness in the joint, spasms, limited mobility, some bony changes, abnormal amount of pain for the injury. There is decreased sympathetic activity. The patient may feel as if their limb is on fire and is amazed when it actually feels cool to the touch (lack of blood flow). Early interventions (e.g., anticonvulsants) have shown to delay or prevent stage progression. Diagnostics: Thermography, X-rays, and EMGs The National Institute of Neurological Disorders and Stroke. Retrieved from. http://www.ninds.nih.gov/disorders/reflex_sympathetic_dystrophy/detail_reflex_sympathetic_dy strophy.htm

The Dystrophic Stage can last three moths to one year. Pain is constant and described as throbbing, burning, aching, crushing in nature and is exacerbated by any stimuli. The affected limb may still be edematous, cool, cyanotic (discolored) or mottled (different shades). Nails are brittle and ridged and stiffness persists. Muscle wasting may begin (PT/OT). Patient usually starts experiencing short-term memory problems, as well as increased pain from noises and/or vibrations.

The syndrome progresses to the point where changes in the skin and bone are no longer reversible. Pain becomes unyielding and may involve the entire limb or affected area. There may be marked muscle loss (atrophy), severely limited it mobility, and involuntary contractions ti of the muscles and tendons that flex the joints. Limbs may be contorted. High risks for cancer, Mood Disorders, Sleep Disorders, and Suicide.

Female: Male = 4:1 50,000 new cases dx in the US yearly Prevalence (1990) = 20.57 per 100,000 The mean age at diagnosis is 42 years; although children as young as 3 have been diagnosed (www.rsds.org) Upper limb affected 2x s as commonly as lower limb. Fracture most commonly precipitates CRPS I CRPS I AKA: Reflex Sympathetic Dystrophy (RSD), Sudeck's atrophy, reflex neurovascular dystrophy (RND) or algoneurodystrophy CRPS II: Causalgia burning gpain Bruehl, S. (2010). An Update on the Pathophysiology of Complex Regional Pain Syndrome. Anesthesiology, 113, 713-725.

Occluded the blood flow to one hind paw for 3 hours under general anesthesia. The treated hind paw exhibits an initial phase of hyperemia and edema. This is followed by hyperalgesia, allodynia, and cold-allodynia that lasted for at least 1 month. Electron microscopy shows that t the ischemia-reperfusion i i injury produces a microvascular injury ( slow-flow/no-reflow ) in the capillaries of the hind paw muscle and digital nerves. Slow-flow/no-reflow phenomenon initiates iti t and maintains i deep-tissue ischemia and inflammation, and leading to the activation of muscle nociceptors. The cause of the abnormal pain sensations is related to ischemia i and inflammation due to microvascular pathology in deep tissues, leading to a combination of inflammatory and neuropathic pain processes. Coderre, T. J. and Bennett, G. J. (2010), A Hypothesis for the Cause of Complex Regional Pain Syndrome-Type I (Reflex Sympathetic Dystrophy): Pain Due to Deep-Tissue Microvascular Pathology. Pain Medicine, 11: 1224 1238. doi: 10.1111/j.1526-4637.2010.00911.x

No relationship between CRPS I and depression, anxiety, 1 neuroticism, and anger 1 Increased life events (stress) related to CRPS I 1 CRPS I and II increased somnolence or no difference 1 Depression or anxiety does not predict severity of disease or progression 1 Depression accompanies other Chronic illnesses 1

CRPS I decreased somatization than controls or no difference CRPS I high pain day was predictive of increased anger in one study; increased anger not predictive of increased pain 1 CRPS I decreased d levels l of hostility (chronic pain pts) 1 CRPS l decreased OCD behavior than headache pts 1 CRPS I high h pain day was predictive of increased anxiety 1 Psychological factors do not appear to precipitate the pathogenesis of CRPS, but CRPS pathology can cause depression, anxiety, anger, and sleep disturbances 1.

Symptoms can be confused with malingering, somatization disorder, pain disorder, conversion disorder, factitious disorder, and Munchausen s disorder DSM-IV-TR Chronic pain can exacerbate psychiatric symptoms such as depression, anxiety, and somnolence; assess for AXIS I diagnosis. 1 Implications for the use of SNRI s or anti-seizure medications for comorbid disorders Pts with CRPS are accused of malingering and Munchausen's disorder b/c of the obscure clinical i l presentation (e.g., bruises, discoloration, i and limb contortions). 3 Doctors use the label psychogenic pain when pts don t respond to medical or surgical treatment, or pts display behaviors that doctors find difficult to cope with. 2 CRPS/RSD is becoming more prevalent possibly due to increases in orthopedic surgeries, cryotherapy, lidocaine injections, and immobilization techniques. 2

Trauma associated with endless painful medical procedures (PTSD) Frustration about misdiagnosis and mismanaged symptoms Stigma about utilizing narcotic pain medication Messages that create cognitive dissonance You don t look like you are in that much pain! Messages that create helpless attitudes don t do that, I will get that for you. Insidious unpredictable disability: crutches, wheel chair, loss of function, difficulty with basic self-care Relationship difficulties and dfamily care issues Family confusion, disbelief, and anger Lack of CRPS/RSD awareness in the Healthcare system and public creates isolation nobody understands what I am going though, not event doctors! GRIEF Financial difficulties: difficulties acquiring disability ins, workers comp, medicare/medicaid, and obtaining work

Problem Solving develop effective problem solving strategies, reduce impulsivity, and increase self-efficacy (financial, medical, & care related problems) 14 Relaxation Training reduce anxiety, muscle tension, HR/BP, and increase feelings of wellbeing 12 Guided Imagery Hypnotherapy Progressive Muscle Relaxation Acceptance Therapy Cognitive Relabeling/ CBT 13 Assertion Training Help pt communicate with doctors, family, friends, and loved ones.

A. A history of many physical complaints beginning gbefore age 30 years that occur over a period of several years and result in treatment being sought or significant impairment in social, occupational, or other important areas of functioning. B. Each of the following criteria must have been met, with individual symptoms occurring at any time during the course of the disturbance: (1) four pain symptoms: a history of pain related to at least four different sites or functions (e.g., head, abdomen, back, joints, extremities, chest, rectum, during menstruation, during sexual intercourse, or during urination) (2) two gastrointestinal symptoms: a history of at least two gastrointestinal symptoms other than pain (e.g., nausea, bloating, vomiting other than during pregnancy, diarrhea, or intolerance of several different foods) (3) one sexual symptom: a history of at least one sexual or reproductive symptom other than pain (e.g., sexual indifference, erectile or ejaculatory dysfunction, irregular menses, excessive menstrual bleeding, vomiting throughout pregnancy) (4) one pseudoneurological symptom: a history of at least one symptom or deficit suggesting a neurological condition not limited it to pain (conversion symptoms such as impaired i coordination or balance, paralysis or localized weakness, difficulty swallowing or lump in throat, aphonia, urinary retention, hallucinations, loss of touch or pain sensation, double vision, blindness, deafness, seizures; dissociative symptoms such as amnesia; or loss of consciousness other than fainting) C. Either (1) or (2): (1) after appropriate investigation, each of the symptoms in Criterion B cannot be fully explained by a known general medical condition or the direct effects of a substance (e.g., a drug of abuse, a medication) (2) when there is a related general medical condition, the physical complaints or resulting social or occupational impairment are in excess of what would be expected from the history, physical examination, or laboratory findings D. The symptoms are not intentionally feigned or produced (as in Factitious Disorder or Malingering)

One or more symptoms or deficits are present that affect voluntary motor or sensory function that suggest a neurologic or other general medical condition. Psychologic factors are judged to be associated with the symptom or deficit because conflicts or other stressors precede the initiation or exacerbation of the symptom or deficit. The symptom or deficit is not intentionally yproduced or feigned (as in factitious disorder or malingering). The symptom or deficit, after appropriate investigation, cannot be explained fully by a general medical condition, the direct effects of a substance, or as a culturally sanctioned behavior or experience. The symptom or deficit causes clinically significant distress or impairment in social, occupational, or other important areas of functioning or warrants medical evaluation. The symptom or deficit is not limited to pain or sexual dysfunction, does not occur exclusively during the course of somatization disorder, and is not better accounted for by another mental disorder.

A. Pain in one or more anatomical sites is the predominant focus of the clinical presentation and is of sufficient severity to warrant clinical attention. B. The pain causes clinically significant distress or impairment in social, occupational, or other important areas of functioning. C. Psychological factors are judged to have an important role in the onset, severity, exacerbation, or maintenance of the pain. D. The symptom or deficit is not intentionally produced or feigned (as in Factitious Disorders or Malingering). E. The pain is not better accounted for by a Mood, Anxiety, or Psychotic Disorder and does not meet criteria for Dyspareunia. 307.80 Pain Disorder Associated With Psychological Factors: psychological factors are judged to have the major role in the onset, severity, exacerbation, or maintenance of the pain. (If a general medical condition is present, it does not have a major role in the onset, severity, exacerbation, or maintenance of the pain.) This type of Pain Disorder is not diagnosed if criteria are also met for Somatization Disorder.

Depression 2x s as likely in women then men Prevalence of Major Depression is ~ 17% in general pop Individuals with depression report more physical symptoms, poorer self-appraisal health, and seek medical attention at increased rates Individuals with depression with chronic diseases have poorer medical outcomes Depression can cause increased fatigue, difficulty with motivation, hopelessness, sleep disturbances, poor self- management for health problems, and is related to overeating and obesity. Boyer, B. A. and Paharia, I. M. (2008). Comprehensive Handbook of Clinical Health Psychology. New Jersey: John Wiley & Sons, Inc.

Prevalence of Anxiety Disorders in the major population is ~ 8.5% The prevalence of Panic disorder, PTSD, and GAD is increased with chronic medical conditions; especially if pain is a primary symptom Greater disability and utilization of health care Anxiety causes elevated arousal of the sympathetic nervous system and can interact with the pathophysiology of CRPS via increasing heart rate and BP, vasoconstriciton, and neuroendocrine changes that increase blood glucose Boyer, B. A. and Paharia, I. M. (2008). Comprehensive Handbook of Clinical Health Psychology. New Jersey: John Wiley & Sons, Inc.

137 pts with CRPS were administered tests that assessed: executive control, naming/lexical i l retrieval, & declarative memory 5 2-step cluster analysis classified pts into 3 gps; no control 5 gp1 (n = 48) average range all scores 5 gp2 (n = 58; dysexecutive gp) mild impairment or low avg test performance on working memory/verbal fluency test 5 gp3 (n = 31); global gp low average/borderline range on all tests with reduced scores on naming/declarative memory 5 tests 5. (89/137) impaired working memory/verbal fluency 5 MANCOVA assessed scaled scores; no effect size 5

Clinical observations: pts pay less attention to or fail to care for their affected limb as if it were not part of their body 4 Neurological neglect-like syndrome? 4 27 pts classified by IASP CRPS were interviewed qualitatively to explore feelings and perceptions about their affected body parts 4 Semi structured interviews were analyzed utilizing grounded theory 4

Pts revealed bizarre perceptions about their aff d limb 4 Pts expressed desire to amputate limb; despite probability of further pain and functional loss 4 A mismatch was experienced between the sensation of the limb and how it looked 4 Anatomical parts of the CRPS limb were erased in mental representations of the affected area 4 Pain raised awareness of limb; but less awareness of limb position 4 Interaction between pain, disturbances in body perception, and central mapping 4

Motor and Sensory Homunculus

PAIN / INJURY Pain can cause a person to withdrawal from activities that are necessary for physical rehabilitation PERSISTENT PAIN, ATROPHY, WEAKNESS, AND PERCEPTUAL DISTURBANCES People are more vulnerable to re-injury, flareups, and significant loss of function once deconditioning and atrophy becomes continual MUSCLE ATROPHY, SWELLING, AND STIFFNESS CAN LEAD TO INCREASED PAIN WITH ACTIVITY LONG-TERM INACTIVITY Rest is important for healing, but can become an obstacle when people fail to engage in activities necessary for recovery of function GUARDING AND AVOIDANCE BEHAVIORS Altered movement patterns and guarding against pain can effect cognitive mapping and lead to both motor and sensory dysfunction Russo, Personal Communication, 2011

Body perception disturbance may be more appropriate than neurological neglect-like like syndrome 10 Protective guarding and pain/avoidance posturing can lead to muscle imbalances, decreased function, and atrophy 11 Protective guarding and other avoidance behaviors can amplify to agoraphobic proportions; which causes further disability Desensitization techniques help to normalize sensations of the affected area by utilizing progressive stimulation 11 Progressive Muscle Relaxation (PMR) and other relaxation techniques may be required prior to implementing Desensitization techniques

Treatments that target cortical areas may reduce body 10 perception disturbance and pain 10 Mirror Therapy: pt receives visual feedback in order to train the brain to configure a new body map 10

Thank you for attending Introduction to CRPS Renee Glick (Hill) U.S. Pain Foundation Vice President Director of Research Studies www.uspainfoundation.org research@uspainfoundation.org

References 1. Beerthuizen, A., Spijker, A. V., Huygen, F., Klein, J., & Wit, R. (2009). Is there an association between psychological factors and the Complex Regional Pain Syndrome type 1 (CRPS 1) in adults? A systematic review. Pain. Advanced online publication. doi: 10.1016/j.pain.2009.05.003 2. Bruehl, S. (2010). An Update on the Pathophysiology of Complex Regional Pain Syndrome. Anesthesiology, 113, 713-725. 3. Harden, N. R., Bruehl, S., Stanton-Hicks Hicks, M., & Wilson, P. R. (2007). Proposed New Diagnostic Criteria for Complex Regional Pain Syndrome. Pain Medicine. Advanced online publication. doi: 10.111/j.1526-4637.2006.00169.x 4. Lewis,,J J. S., Kersten, P., McCabe, C. S., McPherson, K. M., & Blake, D. R. (2007). Body perception disturbance: A contribution to pain in complex regional pain syndrome (CRPS). Pain. Advance online publication. doi: 10.1016/j.pain.2007.03.013 5. Libon, D. J., Schwartzman, R. J., Eppig, J., Wambach, D., Brahin, E., Peterline, B. L., Alexander, G., & Kalanuria, A. (2010). Neuropsychological deficits associated with Complex Regional Pain Syndrome. Journal of the International Neuropsychological Society. Advance online publication. doi: 10.1017/S1355617710000214 6. Schwartzman, R. J., Aexander, G. M., Grothusen, J. R., Paylor, T., & Reichenberger, E. (2009). Outpatient intravenous ketamine for the treatment of complex regional pain syndrome: A double-blind placebo controlled study. Pain. Advanced online publication. doi: 10.1016/j.pain.2009.08.015

References: Book 11. Goldfried, M.R. & Davison, G. C. (1976). Clinical Behavior Therapy. In E. W. Craighead (Ed.), Systematic Desensitization (pp. 112-135). New York, NY: John Wiley & Sons, Inc. 12. Goldfried, M.R. & Davison, G. C. (1976). Clinical Behavior Therapy. In E. W. Craighead (Ed.), Relaxation Training (pp. 81-111). New York, NY: John Wiley & Sons, Inc. 13. Goldfried, M.R. & Davison, G. C. (1976). Clinical Behavior Therapy. In E. W. Craighead (Ed.), Cognitive Relabeling (pp. 158-185). New York, NY: John Wiley & Sons, Inc. 14. Goldfried, M.R. & Davison, G. C. (1976). Clinical Behavior Therapy. In E. W. Craighead (Ed.), Problem Solving (pp. 186-207). New York, NY: John Wiley & Sons, Inc.

Online References & Brochures 7. Bruehl, S. (2007). Cognitive Behavioral Therapy for CRPS. Retrieved from http:/www.rsds.org/1/publications/review_archive/bruelcbt.html p 8. Bruehl, S. Co-Morbidity and CRPS: Implications for Treatment [PDF document]. Retrieved from Lecture Notes Online Web site: http://www.rsds.org/3/education/pdf/crps_comorbidity_bruehl.pdf 9. Bruehl, S. (2010). Psychological Interventions. 37-50. Retrieved from http://www. sds.org/3/clinical_guidelines/txguidelines_psychological.pdf l d l dl hl l df 10. Longo, U. G., Maffulli, N., & Denaro, V. ((2009). Mirror Therapy for Chronic Complex Regional Pain Syndrome Type I and Stroke. The New England Journal of Medicine. 361, 6. 11. Treating Complex Regional Pain Syndrome. (2009). A Guide for Therapy. [Brochure]. Swan, M. E.: Author.