Complex Regional Pain Syndrome: updates on treatment Reflex Sympathetic Dystrophy Pradeep Chopra, MD Assistant Professor (Clinical) Brown Medical School Director, Pain Management Center, RI

Copyright © 2013 by Pradeep Chopra. No part of this presentation may be reproduced or transmitted in any form or by any means without written permission of the author

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Disclosure and disclaimer • I have no actual or potential conflict of interest in relation to this presentation or program • This presentation will discuss “off-label” uses of medications • No financial interest in any pharmaceutical company or otherwise

Copyright © 2013 by Pradeep Chopra. No part of this presentation may be reproduced or transmitted in any form or 2 by any means without written permission of the author

Introduction • Pain Medicine specialist • Training and Fellowship, Harvard Medical school • Assistant Professor – Brown Medical School, Rhode Island • Assistant Professor, Boston University Medical Center 3 Pradeep Chopra, MD

What is CRPS / RSD • Complex Regional Pain Syndrome formerly Reflex Sympathetic Dystrophy • Syndrome characterized by a continuing pain that is disproportionate to the usual course of any trauma or lesion. • Usually starts after a trauma, immobilization. Maybe spontaneous or after a stroke.

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How common is RSD? • Not sure – because there are a lot of cases that are undiagnosed and misdiagnosed. • • USA: no great studies but estimated to be 50,000 new cases per year

1 Andreas Kopf, Patel N, IASP 2010 2 DeMos M, de Bruijn AG, et al 2006 3 Van Den Eeden, Tanner, et al 2003 Deussing, Jankosky 2012 Marinus J, Moseley GL et al 2011

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Signs and Symptoms of CRPS 1 • Pain starts in one limb but can present in the trunk (spine, abdomen, perineum) • Constant pain, even at rest with intermittent exacerbations. Unexplained and diffuse • Severe pain - burning, tearing, shooting • Temperature, color change. • Edema • Area of pain larger than the primary injury • Limited range of motion 6 Pradeep Chopra, MD

Signs and Symptoms of CRPS

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• Cannot be explained by any other medical condition • Allodynia - pain on light touch • Hyperalgesia - increased pain to mildly painful stimulus • Trophic changes - nail growth changes (faster, distorted), hair growth changes (coarser, darker, rapid growth, hair falling), skin changes (atrophy of skin), skin lesions 7 Pradeep Chopra, MD

Color, temperature and swelling 94 °

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Swelling

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Skin lesions

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Bilateral CRPS

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Best Diagnostic tool • A good history and physical examination • A repeat examination should be done to come to a diagnosis because of the fleeting nature of some of the symptoms (color change, temperature asymmetry

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Tests that are not helpful for diagnosing RSD • Imaging techniques – x-ray, MRI, fMRI, Three phase bone scan, bone density • Blood tests • Skin biopsy • Sympathetic nerve tests – sweat test, sympathetic skin response, • Nerve tests – EMG, nerve conduction, • The tests MAYBE used if another diagnosis is suspected. Atkins RM, Tindale W, Bickerstaff D, Kanis JA. Quantitative bone scintigraphy in reflex sympathetic dystrophy. Br J Rheumatol 1993;32(1):41-5. Todorovic-Tirnanic M, Obradovic V, Han R, Goldner B, Stankovic D, Sekulic D, et al. Diagnostic approach to reflex sympathetic dystrophy after fracture: radiography or bone scintigraphy? Eur J Nucl Med

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CENTRAL SENSITIZATION Key concept to understanding all chronic pain

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Glia and nerves under normal conditions

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Activated Glia

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Chemicals released by activated Glia

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Nerve inflammation

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NMDA Receptors • A barrage of strong and persistent pain signals to the CNS results in activation of NMDA receptors • Activated glia that release proinflammatory cytokines increase neuronal excitability and strength by increasing the number of NMDA receptors 24 Pradeep Chopra, MD

Receptors • Most drugs in the human body work as a lock and key mechanism. • Specific drugs (keys) fit in specific receptors (locks) • When the correct key fits into the correct lock then the body reacts • Sometimes we use dummy keys that block receptors so as to prevent the body from reacting 25 Pradeep Chopra, MD

NMDA receptors • In CRPS, the NMDA receptors increase in number and sensitivity • There are several drugs that block NMDA receptors. • Ketamine blocks the effect of NMDA receptors thus decreasing pain

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Ketamine for CRPS

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N-methyl-D-aspartate (NMDA) receptor blockers • Ketamine, dextromethorphan, memantine • Dextromethorphan (cough syrup) – effective in DPN but not CRPS or PHN. Weak NMDA blocking property.

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Ketamine • • • • • •

Strong NMDA Receptor blocker One of the safest anesthetic drugs Powerful analgesic CRPS - activation of NMDA receptors Poor absorption when administered orally. Effective as IV or sublingual (Troche)

Correll GE, Maleki J, Gracely EJ, Muir JJ, Harbut RE. Subanesthestic ketamine infusion therapy: a retrospective analysis of a novel therapeutic approach to complex regional pain syndrome. Pain Medicine 2004;5(3):263-75.

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Ketamine in RSD

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• Administered in sub-anesthetic doses – blocks NMDA receptors without causing too many side effects • In RSD it decreases Central Sensitization • Administration: IV, oral, sublingual, nasal • Rough estimates – 85% show improvement in daily activities, reduction in their medications and improved lifestyles • It is not a cure. It is to be done along with other therapies Correll GE, Maleki J, Gracely EJ, Muir JJ, Harbut RE. Subanesthestic ketamine infusion therapy: a retrospective analysis of a novel therapeutic approach to complex regional pain syndrome. Pain Medicine 2004;5(3):263-75.

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Ketamine – out patient • • • • • • •

Increasing dose of ketamine over 10 days Start at a low dose, increase everyday Usually start to see some relief by day 4 or 5 If no relief by day 5, stop Infusion done over 4 to 5 hours Full standard monitoring Qualified personnel must be present at all times

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Ketamine protocols in RSD

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• Low dose protocol: • Loading dose: a low dose IV ketamine administered over a few hours (usually 4 hours). It is increased everyday over the next 10 days based on the response. • Booster dose: Low dose IV ketamine is repeated for 1 or 2 days after 2 weeks and then again at 4 weeks to 6 weeks tailored to patient’s response 32 Pradeep Chopra, MD

Continuous infusion protocol • Done in an Intensive Care Unit • A continuous 24 hour infusion done over five to six days • Usually used only for the loading dose. • Follow up booster infusions after discharge may be done as an outpatient procedure

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IV Ketamine - boosters • Very important part of the treatment protocol • As the effect of the initial ketamine wears, the glial cells begin to get activated again. • Boosters may be done after 2 weeks for 2 days • Then, for one day every 4 to 8 weeks depending on the severity, chronicity and response • Sometimes, it may be necessary to do a 2 day booster.

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Ketamine infusions • Must be done under strict ASA (American Society of Anesthesiologists) standard monitoring • Continuous oxygen levels, heart rate, EKG • Intermittent (every 15 minutes) blood pressure, conscious level • No noise - Very quiet room • No bright lights - Dark room 35 Pradeep Chopra, MD

Ketamine infusions • Avoid talking, television, music, etc • Avoid any visual and sound stimulation • Dreams, hallucinations – to a mild degree imply effective dose. • Triggered by loud sounds and light • Rest of the day – avoid loud traffic, spend the day at home in a quiet, dark room • Take a benzodiazepine pill after infusion 36 Pradeep Chopra, MD

Ketamine side effects • Most of the side effects are temporary and short lived and reversible. • We do not know of any long term side effects of ketamine infusions. • Nausea, vomiting, colorful dreams, hallucinations, headache

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Ketamine sublingual • Only for acute flare up. Not for regular use. • Take 10mg in your cheek or under tongue every 1 hour till relief or for a total of 50mg 10mg ___1 hour___10 mg___1 hour____10mg _____1 hour_____10mg

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Which Protocol is best ? How much ketamine is enough?

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Factors that are important in getting the best out of a ketamine infusion

• How long does the ketamine stay in the body i.e. how long are the receptors blocked • How much is needed to keep the ketamine in the body / keep the receptors blocked • Minimize trauma while delivering the infusion • Ketamine infusions are good only if done in conjunction with other therapies, especially exercise 40 Pradeep Chopra, MD

Severe RSD with skin lesions

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IV ketamine and LDN

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Low Dose Naltrexone LDN

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Low Dose Naltrexone (LDN) 1 • Dummy key that blocks opioid receptors • Suppresses glial cell activation, hence reducing nerve inflammation •

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Low Dose Naltrexone (LDN) 2 • Reduction of pro-inflammatory cytokines can be achieved with ultra low doses of naltrexone (Liu et al, Greeneltch et al, Tsai et al)

• Effect not mediated by opioid receptor activity • Potentially mediated by activity on Toll Like Receptors 4 (TLR4) • LDN increases production of endorphins and density of opiate receptors (Zagnon) 45 Pradeep Chopra, MD

Low Dose Naltrexone (LDN)

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• There are several theories as to how LDN may work. 1. Transiently blocks opioid receptor leading to positive feedback production of endorphins (Zagnon) 2. LDN increases production of OGF (opioid growth factor) as well as number of and density of OGF receptors by intermittently blocking the opiate receptor. Increased in OGF repairs tissue and healing. 3. Effect not mediated by opioid receptor activity. Potentially mediated by activity on Toll Like Receptors 4 (TLR4)

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Low Dose Naltrexone (LDN) 3 • Dose can vary anywhere between 1.0 mg to 4.5mg • May cause insomnia, mild headaches initially. • Patients report increased physical activity, flare ups not as acute, better tolerance to pain. • To avoid all opioids or tramadol.

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Case of RSD treated with LDN RSD with dystonia before LDN

RSD after LDN

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FREE RADICAL SCAVENGERS

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Free Radicals – what are they? • When molecules break up, some electrons are left free to float around. • These unbalanced molecules are called free radicals • These unbalanced molecules become very unstable and attack another molecule or electron to grab onto for stability. • In our body, when these unstable electrons attack other molecules to achieve stability they damage human cells – nerves, muscles 50 Pradeep Chopra, MD

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Free Radicals attack and rob energy from other cells to satisfy themselves

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Free Radicals – what are they? • • • •

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Human body is made up of cells Cells are made up of molecules Molecules are made up of atoms Atoms are made up of electrons and protons (1:1)

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Free Radical scavengers (Antioxidants) • Alpha Lipoic Acid • Vitamin C • DMSO (Dimethyl sulphoxide) • N-Acetyl Cysteine (NAC) 55 Pradeep Chopra, MD

DMSO 50% - Dimethyl Sulphoxide • Topical use only.

• Particularly helpful for ‘warm’ CRPS

Geertzen JH, Bruijn H de, Bruijn-Kofman AT, Arendzen JH. Reflex sympathetic dystrophy: early treatment and psychological aspects. Arch Phys Med Rehabil 1994;75(4):442-6. Zuurmond WW, Langendijk PN, Bezemer PD, Brink HE, Lange JJ de, Loenen AC van. Treatment of acute reflex sympathetic dystrophy with DMSO 50% in a fatty cream. Acta Anaesthesiol Scand 1996;40(3):364-7.

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N- Acetyl Cysteine (NAC) • Useful for cold allodynia • N-Acetylcysteine 600mg three times a day for three months • Gastric irritant

Perez RS, Zuurmond WW, Bezemer PD, Kuik DJ, Loenen AC van, Lange JJ de, et al. The treatment of complex regional pain syndrome type I with 57 free radical scavengers: a randomized controlled study. Pain 2003;102(3):297-307

Vitamin C • Natural antioxidant • Recommended daily allowance of Vitamin C is 60mg (National Research Council, USA). • Vitamin C 500 mg for 45 days to 50 days was shown to prevent development of CRPS • ? Any value to using it in established CRPS, certainly helpful in prevention Zollinger Paul, Tuinebereijer, Keir R, Breederveld, 1999, Lancet

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Alpha Lipoic acid (ALA)

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• Free Radical scavenger • Promising results in diabetic neuropathy and other polyneuropathies • No trials in CRPS • Has been approved in Germany for treating neuropathic pain Kapoor S, Foot Ankle Spec, 2012 Aug;5(4); 228-9 Snedecor SJ, Sudarshan L, Cappelleru JC etc al. 2013 Pain Pract, Mar 28

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Alpha Lipoic acid (ALA)

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• Its also helps with autonomic neuropathy (common in CRPS) POTS, Dysautonomia • Effective when taken as IV (Intravenous) • May be taken orally • Naturally produced in the body • Spinach, red meat, potatoes, broccoli, yams, carrots, beets, yeast

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Clonidine • Used for treating high blood pressure • Patch more effective than oral • Effective for hyperalgesia and allodynia (Davis et al)

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Muscle symptoms in CRPS

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Muscle symptoms in CRPS • • • •

Muscle spasms Dystonia Tremors Myoclonus

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Muscle spasms • • • • •

Magnesium Diazepam (Valium®), Clonazepam Flexeril Tizanidine Baclofen

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Muscle Relaxants • Cyclobenzaprine, tizanidine • Not very helpful for muscle symptoms of RSD • Baclofen and diazepam or clonazepam may have some benefit • Intrathecal (spinal pump) baclofen is not helpful Schwartzman RJ, Kerrigan J. The movement disorder of reflex sympathetic dystrophy. Neurology 1990;40(1):57-61. Bhatia KP, Bhatt MH, Marsden CD. The causalgia-dystonia syndrome. Brain 1993;116 (Pt 4):843-51. Hilten JJ van, Beek WJ van de, Vein AA, Dijk JG van, Middelkoop HA. Clinical aspects of multifocal or generalized tonic dystonia in reflex sympathetic dystrophy. Neurology 2001;56(12):1762-5.

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Magnesium • IV magnesium: small study with 8 patients, administered IV magnesium over 5 days, for 4 hours each day. • Significant decrease in pain • Improvement in quality of life

Collins et al., Pain Medicine, 2009, 10:930-940 66

Magnesium • Magnesium down regulates NMDA receptor (responsible for CRPS) • Study done with IV magnesium was very positive for helping CRPS • Magnesium has been used to treat migraines • Blood tests for Magnesium levels are not accurate • Best to use Chelated Magnesium

Collins, S, Zuurmond WW et al. Intravenous Magnsium for Complex Regional Pain Syndrome Type I (CRPS I) patients: A Pilot study. 67 Pain Medicine Vol 10, Number 5. 2009

Gabapentin and pregabalin • May help some patients with CRPS • Gabapentin –Slow acting drug. • Pregabalin – no evidence that it helps CRPS but a trial course may be tried • If there is no difference in 8 weeks, taper it off. Serpell MG. Gabapentin in neuropathic pain syndromes: a randomised, double-blind, placebo-controlled trial. Pain 2002;99(3):55766. Vusse AC van de, Stomp-van den Berg SG, Kessels AH, Weber WE. Randomised controlled trial of gabapentin in Complex Regional Pain Syndrome type I [ISRCTN84121379]. BMC Neurol 2004;4(1):13.

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Antidepressants • Tricyclic antidepressants (TCA) well studied in neuropathic pain, not CRPS • Reuptake blockers of serotonin and noradrenaline (Amitrityline, nortriptyline) – work well • Selective noradrenaline blockers (desipramine) • SSRI (Prozac®, Zoloft®)– do not work well Watson CP, Chipman M, Reed K, Evans RJ, Birkett N. Amitriptyline versus maprotiline in postherpetic neuralgia: a randomized, double-blind, crossover trial. Pain 1992;48(1):29-36. Raja SN, Haythornthwaite JA, Pappagallo M, Clark MR, Travison TG, Sabeen S, et al. Opioids versus antidepressants in postherpetic neuralgia: a randomized, placebo-controlled trial. Neurology 2002;59(7):1015-21.

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Antidepressants ( SNRI’s ) • Milnacipran (Savella®) – approved for fibromyalgia • No studies for CRPS • A trial of Milnacipran may be considered

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Bones, joints in CRPS

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Osteopenia / Osteoporosis • • • • • • •

Thinning of the bone is a feature of CRPS Prevention is the best way to treat it Use the limb as much as you can Do weight loading exercises Legs – do weight bearing exercises Arms – lift weights, push against a wall Clordronate and alendronate 72 Pradeep Chopra, MD

Bisphosphonates • • • •

Group of drugs to treat osteoporosis Clodronate IV Alendronate IV Helpful for treating CRPS

Forouzanfar T, Koke AJ, Kleef M van, Weber WE. Treatment of complex regional pain syndrome type I. Eur J Pain 2002;6(2):105-22. Adami S, Fossaluzza V, Gatti D, Fracassi E, Braga V. Bisphosphonate therapy of reflex sympathetic dystrophy syndrome. Ann Rheum Dis 1997;56(3):2014.

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Calcium regulating drugs – in refractory cases • Clodronate (300mg) daily IV – pain, swelling, movement range in acute CRPS • Alendronate (7.5mg) daily IV - pain, swelling, movement range in acute CRPS • Use in refractory cases

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Exercise and Physical Therapy

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Effect of RSD on function • Pain decreases mobility of the limbs. They experience extreme pain with the slightest activity • Not using the limb causes the muscles to atrophy and the joints to become stiff • Immobilizing a limb increases RSD pain 76 Pradeep Chopra, MD

Physical Therapy - two types

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• Pain Focused: Patients who have recently developed RSD – PT should focus more on pain • Time based: Patients who have had RSD for a while (Chronic) – PT should be more time based

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MCAD Mast Cell Activation Disorder

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Mast cells • Found in blood. • Release histamine (chemical causes the redness, itchyness in allergy) • Mast cells release histamine in inflammation

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Mast Cell Activation Syndrome (MCAD) • • • • • •

Common in CRPS Affects most symptoms Chronic fatigue, feeling cold (common), feeling hot Sweats – unexplained Weight gain

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MCAD and CRPS • Itchy – comes and goes, • Rash – unpredictable, unprovoked, • Inflammatory chemicals released by mast cells cause nerves to become inflamed.

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Mast Cell Activation Syndrome (MCAD) • Sores, poor wound healing, • Eyes – gritty, increased water, difficulty focusing • Mouth – burning mouth • Dizziness, palpitations, Pre-syncope (‘almost dizzy’) • Stomach – intestinal pain • Bladder pain 82 Pradeep Chopra, MD

Mast Cell Activation Syndrome (MCAD) • • • •

Repeat examination and history by physician. Testing done during flare ups Serum tryptase level Bone marrow biopsy

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Mast Cell Activation Syndrome (MCAD) • • • •

Anti-histamine (cold medicines) Acid reflux medicines (Ranitidine) Cromolyn sodium Avoid triggers

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Lotions and creams • Not very helpful for the pain of CRPS • Remember, the cause of the pain is now in the central nervous cells and not in the nerves in the arms or legs • May be helpful for associated joint pains • Ketamine cream helpful CRPS skin lesions • Active Max® or DMSO 85 Pradeep Chopra, MD

Tadalafil (Cialis) • Treatment of cold CRPS resulted in significant reduction of temperature difference between affected and unaffected limbs • Long term effect unknown

Groeneweg et al., BMC Musckoskeletal Disorders, 2008, 9:143 86

Sleep disorder in CRPS

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Sleep and CRPS

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• Reasons why patients with CRPS have poor sleep: 1. Pain 2. Increased adrenaline

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Sleep and CRPS

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• Sympathetic over activity in CRPS causes increased levels of adrenaline • In a constant state of ‘flight or fight’ mode • Not rested even after a full night’s sleep. ‘Non-restorative sleep’ • Frequent sleep arousals on brain wave activity

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Sleep and CRPS

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• Sleep study to understand the nature of the problem • Home sleep monitors to measure number of arousals during sleep • Sleep hygiene 1. Sleep at same time every night 2. Bed for sleeping only – no TV, computer 3. No caffeine, alcohol 90 Pradeep Chopra, MD

Sleep and CRPS

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• Adrenaline blocking drugs such as beta blockers (propranolol) • Diazepam (valium®) group of drugs may work partially • If pain is the main factor then an appropriate pain medicine but with caution

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Vaccination and CRPS • Vaccines are administered to prevent inflammatory diseases • There are no studies to show any adverse effect of vaccinations in CRPS • Not getting a vaccination puts a patient at far greater risk of contracting the disease and making the CRPS worse • From a CRPS standpoint its better to be protected by vaccination 92 Pradeep Chopra, MD

Neuropathic pain medications •Gabapentin (Neurontin®) •Pregabalin (Lyrica®)

•Duloxetine (Cymbalta®) •Milnacipran (Savella®)

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Gabapentin and pregabalin • Promising results have been shown. • • Gabapentin – go up to 900mg, then 1800mg. Slow acting drug. • Pregabalin – 150mg to 300mg. Faster acting drug. • The difference may not be obvious at first but when they come off it, they notice an increase in pain • Chance of increased dizziness in POTS - usually stops after being on the drug for sometime. • Start low, go slow 94

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Needle stick trauma • Avoid needle stick injuries as far as possible – combine a blood test from different physicians into one procedure • Ask that the thinnest needle possible be used. • Let them know that the veins are ‘difficult’. CRPS patients have thin and friable veins • For those undergoing regular infusions (IV fluid rehydration or IV Ketamine) should consider a chest port • PICC line is not a good option 95 Pradeep Chopra, MD

Therapies of questionable value

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Opioids

• Counterproductive for chronic pain • Activate glia • Opioids hypersensitize patients to pain

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Opioids • No long term studies • Counterproductive for CRPS • Activate glia through a receptor that is distinct from classical opioid receptors called Toll-like receptors (TLR4) • Opioid induced activation of glia induces them to release neuroexcitatory pro-inflammatory cytokines, suppressing opioid analgesia Watkins, L, Hutchinson, Rice KC, Maier, 2009 Harke H, Gretenkort P, Ladleif HU, Rahman S, Harke O. The response of neuropathic pain and pain in complex regional pain syndrome I to carbamazepine and sustained-release morphine in patients pretreated with spinal cord stimulation: a double-blinded randomized study. Anesth Analg 2001;92(2):488-95.

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Hyperbaric Oxygen • No good evidence that it helps in the long term • Anecdotal reports (mostly from hyperbaric centers)

Kiralp MZ, Yildiz S, Vural D, Keskin I, Ay H, Dursun H. J Int Med Res. 2004 May-Jun;32(3):258-62. Effectiveness of hyperbaric oxygen therapy in the treatment of complex regional pain syndrome 99

Sympathetic Nerve blocks • Stellate ganglion blocks for upper extremity • Lumbar sympathetic blocks for lower extremity • No good data on long term efficacy of these blocks • No diagnostic or therapeutic value • Temporary at best Price DD, Long S, Wilsey B, Rafii A. Analysis of peak magnitude and duration of analgesia produced by local anesthetics injected into sympathetic ganglia of complex regional pain syndrome patients. Clin J Pain 1998;14(3):216-26. Azad SC, Beyer A, Romer AW, Galle-Rod A, Peter K, Schops P. Continuous axillary brachial plexus analgesia with low dose morphine in 100 patients with complex regional pain syndromes. Eur J Anaesthesiol 2000;17(3):185-8.

Spinal Cord Stimulator (SCS)

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• An electrode is inserted surgically into the epidural space and connected to an implanted generator • The electrode produces an electrical current is felt as a tingling sensation and suppresses pain. • Mechanism of action unknown • Painful and expensive Kemler MA, Barendse GA, Kleef M van, Vet HC de, Rijks CP, Furnee CA, et al. Spinal cord stimulation in patients with chronic reflex sympathetic dystrophy. N Engl J Med 2000;343(9):618-24. Bennett DS, Alo KM, Oakley J, Feler CA. Spinal cord stimulation for complex regional pain syndrome I (RSD): a retrospective multicenter experience from 1995 to 1998 of 101 patients. Neuromodulation 1999;2:202-10.

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Spinal Cord Stimulator (SCS)

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• 25% to 50% of patients develop complications requiring further surgery. • Done in a very select group of patients, improves quality of life but not function • In a huge study SCS reduced pain and improved quality of life but did not improve function for up to 2 years after implantation. • From 3 years after implantation there was no difference between those who had it implanted and those who did not Kemler MA, Barendse GA, Kleef M van, Wildenberg FA van den, Weber WE. Electrical spinal cord stimulation in reflex sympathetic dystrophy: retrospective analysis of 23 patients. J Neurosurg 1999;90(1 suppl):79-83. Calvillo O, Racz G, Didie J, Smith K. Neuroaugmentation in the treatment of complex regional pain syndrome of the upper extremity. Acta Orthop Belg 1998;64(1):57-63. Kemler MA, Vet HC de, Barendse GA, Wildenberg FA van den, Kleef M van. The effect of spinal cord stimulation in patients with chronic reflex sympathetic dystrophy: two years’ follow-up of the randomized controlled trial. Ann Neurol 2004;55(1):13-8.

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Dizziness and Palpitations • POTS (Postural Orthostatic Tachycardia Syndrome) • Dizziness and racing heart – usually with standing up • Increase in heart rate by 30 beats/ min or increase to 120 beats/ minute • Increase salt intake, fluids, compression stockings • Medications 103 Pradeep Chopra, MD

CRPS in children

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Children and RSD

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• Children develop the same symptoms • 58% to 93% of cases of RSD in children will resolve with proper treatment • Relapses following apparent healing are often observed (10% to 48%) • More common in girls 105 Pradeep Chopra, MD

Children and RSD 2 • It is often labeled as a behavioral disorder, conversion disorder and parents are labeled as having Munchausen’s syndrome • To make any of the above diagnosis is very challenging. • Usually takes years by a Psychologist in conjunction with other treating physicians. • Imperative that all other medical conditions have been ruled out • Cannot be made by physicians with little or no mental health training. • Very important that parents pay close attention to the child’s complaints 106 Pradeep Chopra, MD

Children and RSD

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• The incidence of these disorders (Munchausen's, Factitious disorder) is less than 1% far more less than the incidence of CRPS • There are no studies to show that it exists. Only 2 cases have been reported • Parents should consult a physician familiar with CRPS because being labeled as one of the above is far more devastating and closes the doors to any further treatment for RSD 107 Pradeep Chopra, MD

Children and RSD

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• Often associated with other conditions such as – Ehler’s Danlos Syndrome (EDS) – Mitochondrial disorder – Nerve entrapment

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Ehlers Danlos Syndrome • Defect in tissue (connective tissue) that provides support to many body parts • Extremely loose joints (Double jointed) • Dislocate or subluxate joints easily • Hyperelastic skin that bruises easily • Inherited • Symptoms of CRPS may be either because of repetitive trauma or nerve damage 109 Pradeep Chopra, MD

Ehlers Danlos Syndrome

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•EDS is a group of inherited disorders •Affects connective tissue (‘connects’) •Connective tissue is found in skin, joints and blood vessels •Very flexible, unstable joints (‘Double jointed’), stretchy

skin and many other symptoms •Orthostatic intolerance / POTS - excessive distention of

veins in upright posture

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RSDS.ORG – research library

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Information on RSD

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Acknowledgements • Nancy Cotterman, CRPS Partners in Pain www.crpspartnersinpain.com • Board Members, CRPS Partners in Pain • Patients and their caregivers • Drexel University • RSDSA (www.rsdsa.org)

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Thank you Pradeep Chopra, MD, MHCM [email protected] Phone 401 729 4985 115 Pradeep Chopra, MD