Ehlers-Danlos Naonal Foundaon August 2013 Conference Pain management in Ehlers Danlos Syndrome Pradeep Chopra, MD, MHCM Director, Pain Management Center, Assistant Professor, Brown Medical School, Rhode Island Assistant Professor (Adjunct), Boston University Medical Center [email protected] [email protected] Pradeep Chopra, MD 1 Disclosure and disclaimer • I have no actual or poten.al conflict of interest in relaon to this presentaon or program • This presentaon will discuss “off-label” uses of medicaons • Discussions in this presentaon are for a general informaon purposes only. Please discuss with your physician your own par.cular treatment. This presentaon or discussion is NOT meant to take the place of your doctor. Pradeep Chopra, MD 2 All rights reserved. 1 Ehlers-Danlos Naonal Foundaon August 2013 Conference Introduc.on • Training and Fellowship, Harvard Medical school • Pain Medicine specialist • Assistant Professor – Brown Medical School, Rhode Island Pradeep Chopra, MD 3 Pain in EDS by body parts • Head and neck • Shoulders • Jaws • Chest • Abdomen • Hips • Lower back • Legs • Complex Regional Pain Syndrome – CRPS or RSD Pradeep Chopra, MD 4 All rights reserved. 2 Ehlers-Danlos Naonal Foundaon August 2013 Conference Pain in EDS • From nerves – neuropathic • From muscles – Myofascial • From Joints – nocicep.ve pain • Headaches Pradeep Chopra, MD 5 Muscle pain Myofascial pain Pradeep Chopra, MD 6 All rights reserved. 3 Ehlers-Danlos Naonal Foundaon August 2013 Conference Muscle Pain • Muscles are held together by fascia – ‘saran wrap’ which is made of collagen • Muscle spasms or muscle knots develop to compensate for unbalanced forces from the joints Pradeep Chopra, MD 7 Muscle pain 1 • Most chronic pain condi.ons are associated with muscle spasms • Oben more painful than the original pain • Muscles may .ghten reflexively, guarding of a painful area, nerve irritaon or generalized tension Pradeep Chopra, MD 8 All rights reserved. 4 Ehlers-Danlos Naonal Foundaon August 2013 Conference Muscle pain 2 • Myofascial pain syndrome is a chronic form of muscle pain. • Myofascial pain syndrome centers around sensi.ve points in your muscles called trigger points Pradeep Chopra, MD 9 Treatment of muscle pain • Treat the cause – joint pain, repe..ve use • Trigger point injec.ons • Stretching • Relaxaon techniques Pradeep Chopra, MD 10 All rights reserved. 5 Ehlers-Danlos Naonal Foundaon August 2013 Conference Trigger Points • When muscles are in chronic spasm they stay taut – ‘knots’. • They develop trigger points which trigger the muscle in to a constant state of spasm • The trigger points can be painful when touched. • The pain can spread throughout the affected muscle and other parts Pradeep Chopra, MD 11 Trigger Points 2 Pradeep Chopra, MD 12 All rights reserved. 6 Ehlers-Danlos Naonal Foundaon August 2013 Conference Pradeep Chopra, MD 13 Pradeep Chopra, MD 14 All rights reserved. 7 Ehlers-Danlos Naonal Foundaon August 2013 Conference Trigger Point injec.ons Pradeep Chopra, MD 15 Trigger Point injec.on Pradeep Chopra, MD 16 All rights reserved. 8 Ehlers-Danlos Naonal Foundaon August 2013 Conference Dry Needling Pradeep Chopra, MD 17 Manual Techniques • Myofascial release: Gentle techniques to improve the mobility of the muscles by mobilizing the .ssue around the muscle • Muscle energy technique: U.lizes posi.oning of the body and using ac.ve muscle contrac.on to relax muscles and align joints Pradeep Chopra, MD 18 All rights reserved. 9 Ehlers-Danlos Naonal Foundaon August 2013 Conference Manual Therapy Techniques • Strain-Counterstrain: A posi.onal technique to help relax .ght muscles • Craniosacral Therapy: A gentle technique to help balance the nervous system Pradeep Chopra, MD 19 Muscle Relaxants • Cyclobenzaprine (Flexeril) • Carisoprodol (Soma) • Tizanidine (Zanaflex) • Baclofen • Benzodiazipine (Valium) Pradeep Chopra, MD 20 All rights reserved. 10 Ehlers-Danlos Naonal Foundaon August 2013 Conference Muscle relaxants • Not true muscle relaxants • Decrease tone • Lethargy – good for bed.me dosing to sleep • Approved for long term use: Baclofen, .zanidine Pradeep Chopra, MD 21 Risk Factors • Muscle injury. Stress on your muscles can cause trigger points to form.. Repe..ve stress also may increase your risk. • Inac.vity. If you've been unable to use a muscle, such as aer surgery or aer a stroke, you may experience trigger points in your muscle as you start to move it during your recovery. Pradeep Chopra, MD 22 All rights reserved. 11 Ehlers-Danlos Naonal Foundaon August 2013 Conference Nerve pain Neuropathic pain Pradeep Chopra, MD 23 Nerve pain in Connec.ve .ssue disorder / EDS • Nerve connec.ve .ssue is more fragile • Fragile nerves get over stretched when crossing or associated with hypermobile joints • Small Fiber neuropathy • Neuropathic pain, Complex Regional Pain Syndrome (CRPS) or Reflex Sympathe.c Dystrophy (RSD) Pradeep Chopra, MD 24 All rights reserved. 12 Ehlers-Danlos Naonal Foundaon August 2013 Conference Nerve pain in EDS - Mitochondria • Is it Mitochondrial disorder ? • Mitochondria found in cells of the human body • They produce energy • Suspect, if the pain is widespread and involves nerves • Exercise intolerance, muscle weakness, seizures, developmental delays etc. Pradeep Chopra, MD 25 Neuropathic pain medicaons • Gabapen.n (Neuron.n®) • Pregabalin (Lyrica®) • Duloxe.ne (Cymbalta®) • Milnacipran (Savella®) Pradeep Chopra, MD 26 All rights reserved. 13 Ehlers-Danlos Naonal Foundaon August 2013 Conference Gabapen.n and pregabalin • Promising results have been shown. • Gabapen.n – go up to 900mg, then 1800mg. Slow ac.ng drug. • Pregabalin – 150mg to 300mg. Faster ac.ng drug. • The difference may not be obvious at first but when they come off it, they no.ce an increase in pain • Chance of increased dizziness in POTS - usually stops aer being on the drug for someme. • Start low, go slow Pradeep Chopra, MD 27 SNRI (selec.ve nor-epinephrine release inhibitors) • Duloxe.ne (Cymbalta®) • Milnacipran (Savella®) Pradeep Chopra, MD 28 All rights reserved. 14 Ehlers-Danlos Naonal Foundaon August 2013 Conference SNRI (selec.ve nor-epinephrine release inhibitors) • Increase nor - epinephrine levels in the central nervous system • Increased nor-epinephrine levels help in modulang pain signals • Milnacipran increases nor-epinephrine by 3 .mes as compared to duloxetne • May increase blood pressure and heart rate Pradeep Chopra, MD 29 An.depressants • Tricyclic an.depressants (TCA) well studied in neuropathic pain • Reuptake blockers of serotonin and noradrenaline (Amitrityline, nortriptyline) – work well • SSRI (Prozac, Zolob) – do not work well for pain Pradeep Chopra, MD 30 All rights reserved. 15 Ehlers-Danlos Naonal Foundaon August 2013 Conference Reflex Sympathe.c Dystrophy (RSD) Complex Regional Pain Syndrome (CRPS) Pradeep Chopra, MD 31 Common clinical features of RSD • Con.nuous burning pain • Pain dispropor.onate in intensity to the inci.ng event • Pain to touch – Allodynia • Pain not in any specific nerve distribu.on or even to the site of injury • Swelling • Increased / decreased sweang Pradeep Chopra, MD 32 All rights reserved. 16 Ehlers-Danlos Naonal Foundaon August 2013 Conference Symptoms of CRPS / RSD • Pain – severe, constant • Temperature difference • Hypersensi.vity • Tremor, Involuntary movements, muscle spasms, atrophy (weakening of the muscle) • Increased sweang • Color difference • Bone thinning • Swelling • Hair and nail changes Pradeep Chopra, MD 33 Pradeep Chopra, MD 34 All rights reserved. 17 Ehlers-Danlos Naonal Foundaon August 2013 Conference Headaches and Neck pain Pradeep Chopra, MD 35 Headaches • Mostly unknown cause • Cerebrovascular reac.vity • Prone to migraines and Tension Type Headaches Pradeep Chopra, MD 36 All rights reserved. 18 Ehlers-Danlos Naonal Foundaon August 2013 Conference Headaches and neck pain • Headaches may be caused by neck pain • Headaches may be from – inside the head (Migraines) or – outside (chronic daily headaches or tension type headaches) • Treatment for both is different Pradeep Chopra, MD 37 Headaches and Neck pain • C1-C2 instability • Kyphosis (Bent backwards) of neck spine • Degeneraon of neck disks (especially C4-C5, C5-C6) • Cranio-cervical instability (junc.on between head and neck not stable) • Chiari malformaon Pradeep Chopra, MD 38 All rights reserved. 19 Ehlers-Danlos Naonal Foundaon August 2013 Conference Symptoms of neck (C1-C2) instability • Neck pain • Headaches in the back of the head (occipital headaches) • Fain.ng sensaon with rotang head to the side • Choking sensaon • Treatment – surgical fusion, strengthening of neck muscles, try bracing for flare ups Pradeep Chopra, MD 39 Migraines • Treatment depends on the frequency – if they are occasional, do not treat • Aborves for infrequent migraines • Prophylac.c for frequent migraines • Triptans (Rizatriptan - Maxalt®, Sumatriptan - Imitrex®, etc – very effec.ve as abor.ve, not prophylac.c) • Triptans not approved for children under 12 years • Prophylac.c in children – Cyprohetadine - Periac.n® Improves appe.te also • Other prophylac.cs – amitriptyline, nortriptyline, topiramate Pradeep Chopra, MD 40 All rights reserved. 20 Ehlers-Danlos Naonal Foundaon August 2013 Conference Red Flags • Acute (sudden) abdominal or flank pain may be due to a ruptured uterine, intes.ne or twisted intes.ne • Bleeding in the head may present with sharp pain in head, change in mental status or seizure • Emergency Pradeep Chopra, MD 41 TMJ Pain Temporo Mandibular Joint Dysfuncon Pradeep Chopra, MD 42 All rights reserved. 21 Ehlers-Danlos