THE INTERNATIONAL PHYSIOTHERAPY GUIDELINES FOR - PowerPoint Presentation

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THE INTERNATIONAL PHYSIOTHERAPY GUIDELINES FOR EHLERS DANLOS SYNDROME

Nicoleta Woinarosky, BSocSc (Hon), MHK (Master in Human Kinetics),CanadaRaoul Engelbert, PhD, Professor in Physiotherapy, NetherlandsTORONTO, 4-5 November 2017

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PlanProcess of collaboration

Conceptual framework and approach to development of the guidelinesChildren, adolescents and adultsEvidence for managementControversiesApplication of the guidelines with casesFuture directions for research and practice

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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; Scheper

et 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

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Research Evidence

Clinical Assessment

Patient Values and BeliefsOptimal Decision

PATIENT JOURNEY

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Gastrointestinal

DysautonomiaFatigue

Psychological

Pain

Urogenital

Cardiovascular

Neuromusculoskeletal

Symptom Profile

Ninis, de Wandele, Simmonds 2015

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Gastrointestinal

DysautonomiaFatigue

Psychological

Pain

Urogenital

Cardiovascular

Neuromusculoskeletal

Symptom Profile

Ninis, de Wandele, Simmonds 2015

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International Classification of

Functioning Disability and Health (WHO, 2015)

Biomedical

Biopsychosocial Model

Recognition and Management of Hypermobility Syndrome

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Pacey, 2014 PhD thesis

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CHILDREN

Pain (Adib et al., 2005; Pacey et al., 2015)Weight bearing joints, especially the knee joints Chronic widespread Exacerbated by activityIncreased fatigue (Pacey et al., 2015)Joint instability, dislocations and subluxations (Pacey et al., 2015)Decreased muscle

tone, strength and endurance-activity limitation (Mitz-Itzen et al., 2009; Celetti et al., 2012; Pacey et al., 2015)

Decreased 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 )

Decreased bone

density

(Engelbert et al, 2003)

BODY FUNCTIONS AND STRUCTURES / IMPAIRMENTS

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CHILDREN

BODY FUNCTIONS AND STRUCTURES / IMPAIRMENTSCardiovascular fitness: low blood pressure(Engelbert et al., 2006)Gastrointestinal dysmotility: reflux, pain, constipation or diarrhea (Abonia

et al., 2013)Bladder dysfunction: stress incontinece (Pacey et al., 2015)Psychological distress: low

mood, self esteem and body image, sleep disturbance

(

Pacey et al.,

2013)

Resulting in lower quality of life

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CHILDRENMobility

(Adib et al., 2005)Mobility aids School attendance and performance (Jansonn et al., 2004; Birt et al. 2014)HandwritingPhysical 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

ACTIVITY AND PARTICIPATION

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ADULTS

Pain (Remvig et al., 2011; Rombaut et al., 2011; Connelly et al., 2015; Rombaut et al., 2015)Multiple joints, localized or widespread, neuropathicFatigue; 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)

BODY FUNCTIONS AND STRUCTURES / IMPAIRMENTS

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ADULTS

Dysautonomia autonomic 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 fogBladder 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

)

BODY FUNCTIONS AND STRUCTURES / IMPAIRMENTS

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ADULTS

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 careTreatment received: physiotherapy, medications, surgeryReduced sports participation Reduced quality of Life

ACTIVITY AND PARTICIPATION

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MANAGEMENT APPROACH

Holistic, empowering, evidence based approachImportant to consider and rule out other related disordersOsteogenesis Imperfecta, Marfan Syndrome, Loeys – Dietz syndrome, EDS, neurological conditions (myopathies), Ehlers Danlos Syndrome, Lupus and other rheumatological conditionsSpecialist referral - multi-systemic or associated conditionsProvide patients with education and reassurance

Direct patients to support groups and informationClinically reasoned, goal directed functional restoration programmeMay/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

)

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DIAGNOSTICS CHILDREN (Scheper 2017)

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DIAGNOSTICS CHILDREN (Scheper 2017)

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MANAGEMENT - CHILDREN

6 week graduated exercise interventionImprovements in pain – child and parent perspectivesParental global assessment reported better outcomes with a targeted motion control approach

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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

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MANAGEMENT - CHILDREN

Orthotics and footwear?

Evans & Rome 2011 Cochrane Review of evidence for non surgical intervention for flexible flat feet.

Eur

J

Phys

Rehab Med. 47 (1): 69 - 89

*

Improved gait efficiency

*

Judicious use of orthotics or sensible footwear

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MANAGEMENT - CHILDREN

F

rolich

et

al., 2011

Physical & Occupational Therapy

in

Paediatrics

32(3):243–

255

* Splints not effective for hand pain or writing speed

Expert opinion - Judicious

use

Splinting?

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MANAGEMENT - ADULTS

8 week graduated proprioception, balance and plyometric training * Reduced knee pain and improved proprioception

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Case Studies and Cohort StudiesStrength, core stability and pain education

(Bathen et al., 2013)Resistance training (Moller et al., 2014)Pain management education (Rahman et al., 2014)MANAGEMENT - ADULTS

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Case Studies and Cohort StudiesStrength, 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 approachesEMERGING LITERATURE

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MANAGEMENT of PoTS

Dysautonomia – Postural OrthostaticTachycardia Syndrome (PoTS)Patients need reassurance Advice:fluids, electrolyte, compression tightspositioning, anti syncope manoeuvres Syncope/fainting: temporary drop in blood flow to the brain caused by sudden decrease in blood pressure, heart rate, blood volume/dehydrationMonitoring of medications when prescribed

(Midodrine, Fludrocortizone, Beta blockade)Respiratory physiotherapy:

hyperventilation

Psychological support: anxiety

management

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

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Case Examples

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Supportive family

Very sport

father and sister

IMPAIRMENTS

PARTICIPATION

PERSONAL FACTORS

ENVIRONMENT

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

Not

keeping up with peers

Unable to walk for > 10

mins

Struggling to run

Unable to hop

Struggling to throw and catchUnable to ride a bike/ tricycleStruggling with dressingStruggling with pencil skillsStruggling at nursery Parents nervous about Jim starting schoolMeet Jim5 yearsMaleLow confidence/ self esteem

I want to play just like all the other kids

ACTIVITY

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IMPAIRMENTS

ACTIVITYPARTICIPATIONPERSONAL FACTORSENVIRONMENT

Meet Helen17 years

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

)

Protective family

Single child

Mother not well

Female

Very

keen dancer and actor

High achiever – A student

Low confidence/ self esteem

Widespread

hypermobility ++

Recurrent shoulder subluxations Widespread persistent pain and fatigueDizzy, fainting (started 1 year ago)Chest painAnxiousLow moodEarly satiety when eating/ bloating and slow transit constipationI want to be a professional performer

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IMPAIRMENTS

PARTICIPATIONPERSONAL FACTORSENVIRONMENT

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

Struggling with

full time

work as

a

designerStruggling to care for family

FemaleStoicResourcefulWealthySupportive husband1 child with autism and severe EDS/HT1 child mildly affectedI need to work and look after my familyACTIVITYDifficulty walking for > 20 minsUnsteady when walkingStruggling to carry shoppingUnable to drive due to dizzinessMeet Bridget40 years

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What Next?Try to understand and help in the patient journey

Rigorously designed randomised controlled trials, internationallySymptom profiling Further validation of outcome measures aligned to the ICFEducation and training for patient groups and therapists

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THANK

YOU

Physiotherapy rehabilitation committee