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THE INTERNATIONAL PHYSIOTHERAPY GUIDELINES FOR EHLERS DANLOS SYNDROME

THE INTERNATIONAL PHYSIOTHERAPY GUIDELINES FOR EHLERS DANLOS SYNDROME. Nicoleta Woinarosky , BSocSc (Hon), MHK (Master in Human Kinetics ), Canada Raoul Engelbert, PhD, Professor in Physiotherapy, Netherlands TORONTO, 4-5 November 2017. Plan. Process of collaboration

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THE INTERNATIONAL PHYSIOTHERAPY GUIDELINES FOR EHLERS DANLOS SYNDROME

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  1. THE INTERNATIONAL PHYSIOTHERAPY GUIDELINES FOR EHLERS DANLOS SYNDROME NicoletaWoinarosky, BSocSc (Hon), MHK (Master in Human Kinetics), Canada Raoul Engelbert, PhD, Professor in Physiotherapy, Netherlands TORONTO, 4-5 November 2017

  2. Plan • Process of collaboration • Conceptual framework and approach to development of the guidelines • Children, adolescents and adults • Evidence for management • Controversies • Application of the guidelines with cases • Future directions for research and practice

  3. Need for International Guidelines Joint Hypermobility Syndrome / hypermobile Ehlers Danlos Syndrome (JHS/ hEDS) is common in musculoskeletal physical therapy settings (30% - 50% of new patients) (Adib et al., 2005; Clark & Simmonds 2011; Connelly et al., 2015) Physical therapy plays a central role in the management (Murray, 2006; Simmonds & Keer 2007; Grahame 2008; Scheperet al., 2015;16) Yet ……understanding of the clinical presentation, diagnostic criteria, presentation and management amongst physical therapists is limited (Billings et al, 2015; Rombaut et al 2015; Terry et al., 2015; Russek et al., 2016; Lyell et al., 2016) British Society of Paediatric and Adolescent Rheumatology 2013 Guidelines for Management of Joint Hypermobility Syndrome in Children and Young People Cincinnati Children’s Hospital Guideline 2014 Evidence based care guidelines for management if Pediatric Joint Hypermobility

  4. PATIENT JOURNEY Patient Values and Beliefs Optimal Decision Research Evidence Clinical Assessment

  5. Fatigue Pain Dysautonomia Neuromusculoskeletal Cardiovascular Urogenital Gastrointestinal Psychological Symptom Profile Ninis, de Wandele, Simmonds 2015

  6. Fatigue Pain Dysautonomia Neuromusculoskeletal Cardiovascular Urogenital Gastrointestinal Psychological Symptom Profile Ninis, de Wandele, Simmonds 2015

  7. International Classification of Functioning Disability and Health (WHO, 2015) Recognition and Management of Hypermobility Syndrome Biomedical Biopsychosocial Model

  8. Pacey, 2014 PhD thesis

  9. CHILDREN BODY FUNCTIONS AND STRUCTURES / IMPAIRMENTS • Pain(Adib et al., 2005; Pacey et al., 2015) • Weight bearing joints, especially the knee joints • Chronic widespread • Fatigue(Pacey et al., 2015) • Joint instability, dislocations and subluxations(Pacey et al., 2015) • Muscletone, strength and endurance (Mitz-Itzen et al., 2009; Celetti et al., 2012; Pacey et al., 2015) • Proprioception, balance, coordination and gait (Ferrell et al., 2004,7; Kirby et al., 2007; Hanewinkel et al., 2009; Fatoye et al., 2009;11; Celetti et al., 2012; Schubert – Hajlmarsson et al., 2012 ) • Bone density (Engelbert et al, 2003)

  10. CHILDREN BODY FUNCTIONS AND STRUCTURES / IMPAIRMENTS • Cardiovascular fitness (Engelbert et al., 2006) • Gastrointestinal dysmotility(Abonia et al., 2013) • Bladder dysfunction (Pacey et al., 2015) • Psychological – mood, self esteem and body image, sleep disturbance (Pacey et al., 2013)

  11. CHILDREN ACTIVITY AND PARTICIPATION • Mobility (Adibet al., 2005) • Mobility aids • School attendance and performance (Jansonn et al., 2004; Birt et al. 2014) • Handwriting • Physical education and sport participation (Jansonn et al., 2004; Birt et al., 2014) • Increased sedentary activities (Schubert – Hajlmarsson et al., 2012) • Impact on domestic life (Schubert – Hajlmarsson et al., 2012) • Quality of life (Pacey et al., 2015) • Pain, fatigue and stress incontinence can have the biggest impact on quality of life

  12. ADULTS BODY FUNCTIONS AND STRUCTURES / IMPAIRMENTS • Pain(Remvig et al., 2011; Rombaut et al., 2011; Connelly et al., 2015; Rombaut et al., 2015) • Multiple joints, localized or widespread, neuropathic • Fatigue; most disabling, mild-severe (Voermans et al., 2011; de Paepe et al., 2012) • Muscle strength; reduced muscle strength and function may be due to muscle dysfunction rather then reduced muscle mass (Ferrell et al., 2014) • Joint instability (Clark & Simmonds 2011) • Reduced proprioception, balance and coordination, 95% fell past year; gait velocity, step length and stride lengths smaller than control group • Reduced bone health (Nijs et al., 2000)

  13. ADULTS BODY FUNCTIONS AND STRUCTURES / IMPAIRMENTS • Dysautonomiaautonomic nervous system does not work correctly cardiovascular dysfunction (Gazit et al., 2013; deWandele et al., 2014) • Postural Orthostatic Tachycardia Syndrome (PoTS) • Temperature dysregulation, syncope/ pre syncope, tachycardia, chest pain, brain fog • Bladder and sexual dysfunction UTI (in women) (Mastooudes et al., 2013) • Gastrointestinal dysmotility(Zarate et al., 2010; Fikree et al., 2014) • Psychological distress– depression, anxiety and panic, sleep disturbance (Smith et al., 2015)

  14. ADULTS ACTIVITY AND PARTICIPATION Significant disability (Rombaut et al., 2011) • Pain, fatigue and psychological distress: anxiety, panic and depression (Scheper et al., 2016) • Difficulties with walking, running and stair climbing • Difficulties with activities of daily living: self care • Treatment received: physiotherapy, medications, surgery • Reduced sports participation Reduced quality of Life

  15. MANAGEMENT APPROACH • Holistic, empowering, evidence based approach • Important to consider and rule out other related disorders • Osteogenesis Imperfecta, Marfan Syndrome, Loeys – Dietz syndrome, EDS, neurological conditions (myopathies), Ehlers Danlos Syndrome, Lupus and other rheumatological conditions • Specialist referral - multi-systemic or associated conditions • Provide patients with education and reassurance • Direct patients to support groups and information • Clinically reasoned, goal directed functional restoration programme • May/may not be alongside multidisciplinary team • Exercise interventions carefully implemented based on American College of Sports Medicine Guidelines (ACPSM) recommendations and motor control theory (Faigenbaum 2009;10; Garber et al., 2011; Smidt, 2013)

  16. DIAGNOSTICS CHILDREN (Scheper 2017)

  17. DIAGNOSTICS CHILDREN (Scheper 2017)

  18. MANAGEMENT - CHILDREN • 6 week graduated exercise intervention • Improvements in pain – child and parent perspectives • Parental global assessment reported better outcomes with a targeted motion control approach

  19. MANAGEMENT - CHILDREN 8 week graduated exercise intervention * Improvements in knee strength and pain in both groups * Parent reported - psychological health, self esteem , mental health and behaviour was significantly different in favour of exercising into the hypermobile range

  20. MANAGEMENT - CHILDREN Orthotics and footwear? * Improved gait efficiency Evans & Rome 2011 Cochrane Review of evidence for non surgical intervention for flexible flat feet. Eur J Phys Rehab Med. 47 (1): 69 - 89 * Judicious use of orthotics or sensible footwear

  21. MANAGEMENT - CHILDREN Splinting? Frolichet al., 2011 Physical & Occupational Therapy in Paediatrics32(3):243–255 * Splints not effective for hand pain or writing speed Expert opinion - Judicioususe

  22. MANAGEMENT - ADULTS 8 week graduated proprioception, balance and plyometric training * Reduced knee pain and improved proprioception

  23. MANAGEMENT - ADULTS CaseStudies and Cohort Studies • Strength, core stability and pain education(Bathen et al., 2013) • Resistance training (Moller et al., 2014) • Pain management education (Rahman et al., 2014)

  24. EMERGING LITERATURE CaseStudies and Cohort Studies • Strength, core stability and pain education(Bathen et al., 2013) • Resistance training (Moller et al., 2014) • Pain management education (Rahman et al., 2014) Significant pain reduction with physical therapy and cognitive approaches

  25. MANAGEMENT of PoTS Dysautonomia– Postural OrthostaticTachycardiaSyndrome (PoTS) • Patients need reassurance • Advice: • fluids, electrolyte, compression tights • positioning, anti syncope manoeuvres • Syncope/fainting: temporary drop in blood flow to the brain caused by sudden decrease in blood pressure, heart rate, blood volume/dehydration • Monitoring of medications when prescribed (Midodrine, Fludrocortizone, Beta blockade) • Respiratory physiotherapy – hyperventilation • Anxiety management – psychological support • Graded cardiovascular exercise and resistance training –focus on lower limbs • Recumbent to upright * Incorporating exercise to manage joint instability Mathias et al., 2011 Fu et al., 2011 Jarjour 2013 Clinical expert opinion

  26. Case Examples

  27. Meet Jim5 years I want to play just like all the other kids PARTICIPATION IMPAIRMENTS ACTIVITY Joint hypermobility 9/9 + hips & shoulders Everted ankles/over pronated, flat feet Coordination problems (gross and fine motor) Poor balance Low muscle tone Muscle weakness Generalised leg pain Tired Struggling at nursery Parents nervous about Jim starting school Not keeping up with peers Unable to walk for > 10 mins Struggling to run Unable to hop Struggling to throw and catch Unable to ride a bike/ tricycle Struggling with dressing Struggling with pencil skills ENVIRONMENT PERSONAL FACTORS Male Low confidence/ self esteem Supportive family Very sport father and sister

  28. Meet Helen17 years I want to be a professional performer PARTICIPATION IMPAIRMENTS ACTIVITY Widespread hypermobility ++ Recurrent shoulder subluxations Widespread persistent pain and fatigue Dizzy, fainting (started 1 year ago) Chest pain Anxious Low mood Early satiety when eating/ bloating and slow transit constipation Struggling with dance Struggling with eating Unable to travel on public transport Reduced attendance and performance at school Reduced social activity with friends (on social media) ENVIRONMENT PERSONAL FACTORS Female Very keen dancer and actor High achiever – A student Low confidence/ self esteem Protective family Single child Mother not well

  29. Meet Bridget40 years I need to work and look after my family PARTICIPATION IMPAIRMENTS ACTIVITY Struggling with full time work as a designer Struggling to care for family Difficulty walking for > 20 mins Unsteady when walking Struggling to carry shopping Unable to drive due to dizziness Widespread hypermobility ++ + Neck, shoulders, hips (dysplasia) & ankles Widespread muscle weakness Poor balance Everted ankles/over pronated, flat feet Persistent pain and fatigue Dizzy/ faint Headaches/ migraines Incontinent (recurrent UTI) Low bone density PERSONAL FACTORS ENVIRONMENT Supportive husband 1 child with autism and severe EDS/HT 1 child mildly affected Female Stoic Resourceful Wealthy

  30. What Next? • Try to understand and help in the patient journey • Rigorously designed randomised controlled trials, internationally • Symptom profiling • Further validation of outcome measures aligned to the ICF • Education and training for patient groups and therapists

  31. THANK YOU Physiotherapy rehabilitation committee

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