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Chronic Fatigue in EDS

EDNF Learning Conference July 22-23, 2011. Chronic Fatigue in EDS. Peter C. Rowe, MD Sunshine Natural Wellbeing Foundation Professor of Chronic Fatigue and Related Disorders Department of Pediatrics Johns Hopkins University School of Medicine. Chronic Fatigue in EDS.

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Chronic Fatigue in EDS

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  1. EDNF Learning Conference July 22-23, 2011 Chronic Fatigue in EDS Peter C. Rowe, MD Sunshine Natural Wellbeing Foundation Professor of Chronic Fatigue and Related Disorders Department of Pediatrics Johns Hopkins University School of Medicine

  2. Chronic Fatigue in EDS • Chronic fatigue and CFS definitions • Lessons from CFS • CF and CFS in EDS • Insights of treating chronic fatigue • Treating orthostatic intolerance • Non IgE-mediated food protein allergies • The paradox of movement restrictions in EDS • Ovarian vein varices/pelvic congestion

  3. Fatigue An overwhelming sustained sense of exhaustion and decreased capacity for physical and mental work. Piper BF. 1989

  4. Fatigue Definitions • Prolonged fatigue: fatigue lasting 1 – 6 mo. • Chronic fatigue: fatigue lasting > 6 mo. • Chronic fatigue syndrome: new onset fatigue, lasting > 6 mo., unrelieved by rest and 4/8 somatic symptoms From MJA 2002; 176:S17-S55

  5. Symptom Criteria For CFS4 of 8 needed for diagnosisFukuda et al. Ann Int Med 1994;121:953-9 • unrefreshing sleep • postexertional malaise lasting > 24 hours • self reported impairment in short-term memory or concentration • sore throat • tender cervical or axillary glands • muscle pain • multijoint pain without swelling • headaches of a new type, pattern, severity

  6. CFS Clinical EvaluationFukuda et al. Ann Int Med 1994;121:953-9 • History, physical, mental status exam • Screening labs: • CBC, ESR/CRP, Chemistries, TSH • Urinalysis • Iron studies, vitamin B12, celiac screening, and, in endemic areas, labs for Lyme and tick-borne infections • Other labs as clinically indicated

  7. Chronic Fatigue in EDS • Chronic fatigue and CFS definitions • Lessons from CFS • CF and CFS in EDS • Insights of treating chronic fatigue • Treating orthostatic intolerance • Non IgE-mediated food protein allergies • The paradox of movement restrictions in EDS • Ovarian vein varices/pelvic congestion

  8. CFS Epidemiology General Affects previously active individuals Heterogeneous precipitating & perpetuating factors Shift in perception of CFS: No longer considered a single disease More likely a convergence of co- morbid pathophysiologic influences

  9. CFS Epidemiology Prevalence 4/1,000 adults; 1/1,000 adolescents Age Uncommon under 10 years Peak prevalence 40-49 years Gender 2-4 F : 1 M SES Affects all groups GeneticsTwice as common in MZ as DZ twins Associated with EDS Associated with joint hypermobility

  10. Research Findings • Acute illness appears to precipitate symptoms in up to 2/3, but evidence of active infection not detected in chronic state (enteroviral infection, Lyme may be exceptions) • Severity of acute infection, not psychological factors, is key determinant of who develops CFS after acute illness • XMRV not an etiologic agent • Immune abnormalities inconsistent & mild • Post-exercise increases in cytokines and genes involved with adrenergic function and pain

  11. Light AR et al. J Pain 2009;10:1099

  12. Research Findings • Orthostatic stress and exercise consistently provoke CFS symptoms • All pediatric and most adult studies confirm higher prevalence of orthostatic intolerance • Open treatment of OI leads to improvement in function • CBT and graded exercise provide modest improvement in function but not cure • Low rates of spontaneous improvement for those with > 3 yrs of symptoms

  13. Orthostatic Intolerance The term “orthostatic intolerance” refers to a group of clinical conditions in which symptoms worsen with quiet upright posture and are ameliorated (although not necessarily abolished) by recumbency. Modified from: Low PA, Sandroni P, Joyner M, Shen WK. Postural tachycardia syndrome (POTS). J Cardiovasc Electrophysiol 2009;20:352-8.

  14. Low PA

  15. Rowell LB Human Cardiovascular Control, 1993

  16. Lightheadedness Dyspnea Syncope Chest Discomfort Diminished concentration Palpitations Headache Tremulousness Blurred vision Anxiety Fatigue Nausea Exercise intolerance Nocturia Symptoms Of Orthostatic Intolerance

  17. ↑pooling, ↓vasoconstriction ↓ intra-vascular volume Orthostatic stress ↑ sympatho-adrenal response NE/Epi NE/Epi NMH POTS

  18. Response To Upright Tilt: CFS Abnormal Normal Stage of tilt 1 2 3 CFS 16 3 3 1 CONTROL 0 1 3 10 OR for abnormal tilt in those with CFS: 55 (95% CI, 5.4 - 557) Bou-Holaigah, Rowe, Kan, Calkins. JAMA 1995;274:961-7.

  19. Response to open treatment of orthostatic intolerance • JAMA 1995;274:961-7.

  20. CFS And Psychiatry • Many CFS patients have anxiety or depression, but prevalence estimates vary widely depending on the case definition used • Severity usually mild, anhedonia uncommon • Post-exertional malaise more common in CFS • Treating depression and anxiety can improve these symptoms, but usually does not cure CFS

  21. 12 wks 24 wks 52 wks White PD et al. PACE trial. Lancet 2011

  22. Chronic Fatigue in EDS • Chronic fatigue and CFS definitions • Lessons from CFS • CF and CFS in EDS • Insights of treating chronic fatigue • Treating orthostatic intolerance • Non IgE-mediated food protein allergies • The paradox of movement restrictions in EDS • Ovarian vein varices/pelvic congestion

  23. Classical type EDS: “Fatigue is a frequent complaint.” Beighton P, De Paepe A, Steinmann B, Tsipouras P, Wenstrup R. Ehlers Danlos Syndromes: Revised nosology, Villefranche, 1997

  24. Orthostatic Intolerance CFS EDS/ Joint hypermobility

  25. Orthostatic Intolerance And Chronic Fatigue Syndrome Associated With EDS Among approximately 100 adolescents seen in the CFS clinic at JHH over a 1 year period, we identified 12 subjects with EDS 6 classical-type, 6 hypermobile-type EDS 11 female; median age 15 yrs (9-21) NMH in 9/12, POTS in 10/12 Rowe PC, Barron DF, Calkins H, Maumanee IH, Tong PY, Geraghty MT. J Pediatr 1999;135:494-9

  26. Joint Hypermobility In Children With CFS Study question: do children with CFS have a higher prevalence of joint hypermobility? Beighton scores obtained in 58 new & 58 established CFS patients, and in 58 controls Median Beighton scores higher in CFS (4 vs. 1) Beighton score > 4 higher in CFS (60% vs. 24%) Barron DF, Cohen BA, Geraghty MT, Violand R, Rowe PC. J Pediatr 2002;141:421-5

  27. Beighton Joint Hypermobility Scores in 58Adolescents With CFS And 58 Healthy Controls # Barron, Geraghty, Cohen, Violand, Rowe. J Pediatr 2002;141:421-5 Beighton scores

  28. How might joint hypermobility be associated with OI and CFS? Working hypothesis: Connective tissue laxity in blood vessels allows increased vascular compliance, promotes excessive pooling during upright posture, leading to diminished blood return to the heart, and thus to OI symptoms Rowe PC, et al. J Pediatr 1999;135:494-9

  29. Fatigue is a frequent and clinically relevant problem in EDS (Voermans NC, et al. Semin Arth Rheum 2010; 40:267-74) • 273 patients with EDS • 77% severe fatigue • 57% reported fatigue as 1 of their 3 most important symptoms • Severe fatigue was more common in hypermobile than classical EDS (84% vs. 69%; P=.032) • Fatigue had a greater impact on daily function than did pain

  30. Fatigue is a frequent and clinically relevant problem in EDS (Voermans NC, et al. Semin Arth Rheum 2010; 40:267-74) On the basis of their results, the authors speculate about a potential treatment: “A cognitive behavioral intervention focusing on pain, sleep disturbances, the reaction of others to the symptoms, and self-efficacy concerning fatigue could help reduce fatigue and fatigue-related disabilities.”

  31. Chronic Fatigue in EDS • Chronic fatigue and CFS definitions • Lessons from CFS • CF and CFS in EDS • Insights of treating chronic fatigue • Treating orthostatic intolerance • Non IgE-mediated food protein allergies • The paradox of movement restrictions in EDS • Ovarian vein varices/pelvic congestion

  32. Infection Movement restrictions Inhalant allergies/asthma Migraines Food allergies Chiari type I or c-spine stenosis Orthostatic intolerance Anxiety EDS/JHS Depression Pelvic vein incompetence Chronic fatigue syndrome

  33. Treating chronic fatigue • Careful history and physical exam, supplemented by questionnaires, to develop working hypotheses about the dominant influences on fatigue • Begin working on graded increases in activity, physical therapy if needed • Begin treating the dominant influences on symptoms • Reassess and repeat steps 1-3

  34. 16 year old with fatigue: visit 1 Gastroesophageal reflux and colic in 1st year of life Onset of fatigue and daily lightheadedness at age 13 Develops syncope X 3; Migraines GI: early satiety, reflux, abdo pain, aphthous ulcers O/E: Beighton score 7/9, blue sclerae, easy eyelid eversion, pes planus, papyraceous scar of L knee. Limitations on physical therapy ROM despite joint hypermobility Beck Depression Inventory: dysthymia

  35. Visit 1 hypothesis formulation Imp: EDS OI (already on Florinef) Milk protein intolerance Migraines Movement restrictions Dsythymia Plan: Milk-free diet instituted Low dose cyproheptadine

  36. Infection Movement restrictions Inhalant allergies/asthma Migraines Food allergies Chiari type I or c-spine stenosis Orthostatic intolerance Anxiety EDS/JHS Depression Pelvic vein incompetence Chronic fatigue syndrome

  37. 16 year old with fatigue Visit 2: GI symptoms resolved unless he gets inadvertent milk re-exposure; mood more of the problem Plan: Low dose Lexapro for mood Visit 3: Mood improved, but still has some orthostatic exacerbation of migraines; still tight on PT exam Plan: Add midodrine for OI; begin PT

  38. 16 year old with fatigue Visit 4: Migraine resolved; better ROM Trial off Lexapro: mood & HA worse, but able to drop to 2.5 mg daily “The more I do, the more I can do” Plan: Continue PT Visit 5: Good year, on HS soccer and tennis teams No syncope; migraines only if he does not maintain good hydration Plan: No changes.

  39. Non-IgE mediated food allergy :3 cardinal features • Recurrent vomiting or GER • Recurrent epigastric or abdominal pain • Food refusal, picky eating, early satiety Other: aphthous ulcers, unexplained fevers, diarrhea or constipation, headache, myalgias, fatigue, asthma Kelly KJ et al. Gastroenterology 1995;109:1503-12

  40. Non-IgE mediated food allergy • Reaction to suspected food usually delayed 2-6 hrs • IgE level, prick skin tests, RAST tests often neg. • Eosinophilic esophagitis only the tip of the iceberg • Treated with strict avoidance of offending food proteins (milk > soy > egg > wheat); amino acid formulas occasionally needed in infants • Diagnosis supported by clinical response to diet, recurrence of symptoms 2-6 hours after inadvertent dietary challenge, confirmed by DBPCOFC

  41. Improvements in esophageal eosinophils after amino acid formula dietKelly KJ et al. Gastroenterology 1995;109:1503-12

  42. Chronic Fatigue in EDS • Chronic fatigue and CFS definitions • Lessons from CFS • CF and CFS in EDS • Insights of treating chronic fatigue • Treating orthostatic intolerance • Non IgE-mediated food protein allergies • The paradox of movement restrictions in EDS • Ovarian vein varices/pelvic congestion

  43. Paradox of movement restrictions in EDS • Increased prevalence of postural abnormalities and movement restrictions among those with CFS • CFS symptoms can be reproduced by selectively placing tension on the neural tissues • Focal movement restrictions are common even in those with generalized joint hypermobility/EDS • Improvement in ROM, orthostatic tolerance, and exercise tolerance can follow manual therapy

  44. Abnormal postures

  45. Restricted Straight Leg Raise Healthy CFS

  46. Symptom Changes with SLR over 12 minutes in Adolescent with CFS Severity Degrees of SLR

  47. How Might Movement Restrictions Be Associated With CFS? • Pathophysiology of symptoms with neural elongation strain awaits clarification, but we hypothesize that it contributes to central sensitivity • Informally, improvement in symptoms, ROM, orthostatic tolerance, and exercise tolerance appears to follow manual therapy designed to reduce adverse neural tension and improve movement restrictions

  48. Manual Therapy Principles • Use of the hands to restore full, symptom-free mobility within the neuromuscular and articular systems • Goal of treatment is the same as that of exercise-based PT, but manual practitioners treat movement restrictions first before advancing the patient to strenuous activity

  49. Manual Techniques • Slow non-thrust manipulations • Sustained stretching • Passive oscillatory movements (neural mobs) • Muscle energy techniques • Gentle indirect techniques • Myofascial release • Strain and counter-strain • Cranio-sacral therapy

  50. Chronic Fatigue in EDS • Chronic fatigue and CFS definitions • Lessons from CFS • CF and CFS in EDS • Insights of treating chronic fatigue • Treating orthostatic intolerance • Non IgE-mediated food protein allergies • The paradox of movement restrictions in EDS • Ovarian vein varices/pelvic congestion

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