HYPERMOBILITY HYPERMOBILITY SYNDROME/EDS III SYNDROME/EDS III

LORRAINE FRIELLORRAINE FRIELEXTENDED SCOPE PRACTITIONEREXTENDED SCOPE PRACTITIONER

CENTRE FOR RHEUMATIC CENTRE FOR RHEUMATIC DISEASESDISEASES

GLASGOW ROYAL INFIRMARYGLASGOW ROYAL INFIRMARY

HYPERMOBILITY & HYPERMOBILITY HYPERMOBILITY & HYPERMOBILITY SYNDROMESYNDROME

Range of movement Range of movement in excess of the in excess of the accepted normal accepted normal range of motion at range of motion at a joint, taking into a joint, taking into account the age, account the age, gender and ethnic gender and ethnic background of the background of the individual individual (Grahame 2010)(Grahame 2010)

Musculoskeletal Musculoskeletal symptoms in the symptoms in the presence of presence of generalised joint generalised joint hypermobility but hypermobility but in the absence of in the absence of other defined other defined rheumatic diseases rheumatic diseases (Kirk et al 1967)(Kirk et al 1967)

What is joint hypermobility syndrome?What is joint hypermobility syndrome?

Pereception of JHS as a mild or trivial Pereception of JHS as a mild or trivial condition with lax joints, pain, joint condition with lax joints, pain, joint dislocation/subluxation, possible OA in later dislocation/subluxation, possible OA in later life.life.

This has changed…..This has changed…..

Now considered an inherited, genetically Now considered an inherited, genetically determined multisystemic disorder of determined multisystemic disorder of connective tissues rendering them more connective tissues rendering them more vulnerable to injury and mechanical failure.vulnerable to injury and mechanical failure.

WHAT IS HMS?WHAT IS HMS?

• A family of related genetically based A family of related genetically based conditions. The protein affected conditions. The protein affected varies and the degree of difference varies and the degree of difference variesvaries

• Marfans SyndromeMarfans Syndrome

• Ehlers-danlosEhlers-danlos

• Benign Joint Hypermobility syndromeBenign Joint Hypermobility syndrome

PresentationPresentation

• Chronic pain and kinesiophobiaChronic pain and kinesiophobia

• Joint laxity,subluxations/dislocationsJoint laxity,subluxations/dislocations

• Vulnerability to injuryVulnerability to injury

• Rest at EOR/”lock” joints and poor posture Rest at EOR/”lock” joints and poor posture habitshabits

• Dysfunctional movement patternsDysfunctional movement patterns

• Poor healing and slower recoveryPoor healing and slower recovery

• Easy bruising and tendency towards Easy bruising and tendency towards bleedingbleeding

Non articular presentationNon articular presentation

• FatigueFatigue

• DeconditioningDeconditioning

• Autonomic dysfunctionAutonomic dysfunction

• Pelvic organ prolapsePelvic organ prolapse

• Urinary incontinenceUrinary incontinence

• PsychologicalPsychological

• POTSPOTS

ExaminationExamination

• Observation – skin, postural alignmentObservation – skin, postural alignment

• Range of movementRange of movement

• Functional activitiesFunctional activities

• Muscle testingMuscle testing

• Neurological testingNeurological testing

• Passive movementPassive movement

• Ligament integrityLigament integrity

• Balance/proprioceptionBalance/proprioception

Good postural alignmentGood postural alignment

• Muscular and skeletal Muscular and skeletal balance which protects the balance which protects the supporting structures supporting structures against injury and against injury and progressive deformityprogressive deformity

• Muscles function most Muscles function most efficientlyefficiently

• Optimum positions for Optimum positions for thoracic and abdominal thoracic and abdominal organsorgans

Habitual posturesHabitual postures

• Frequently rest at EOR Frequently rest at EOR and poor postural and poor postural alignmentalignment

• Stress and strain in Stress and strain in HM collagenous HM collagenous tissuestissues

• Decreased muscle use Decreased muscle use leading to stiffness, leading to stiffness, weakness, weakness, deconditioning, deconditioning, fatiguefatigue

Poor postural alignmentPoor postural alignment

• Faulty relationship Faulty relationship produces stress produces stress and strain on and strain on supporting supporting structuresstructures

• Less efficient Less efficient balancebalance

Active movementActive movement

Look wellLook well

Move wellMove well

Subjective and Subjective and objective often at objective often at oddsodds

Check ‘normal’ range Check ‘normal’ range for that patientfor that patient

Assess muscle functionAssess muscle function

• BreathingBreathing• Transversus abdominusTransversus abdominus• Deep multifidusDeep multifidus• Pelvis floorPelvis floor• Timing, atrophy, loss of tonic function, Timing, atrophy, loss of tonic function,

loss of co-ordination, asymmetry, lengthloss of co-ordination, asymmetry, length• Overactivity in globa, muscles – quads, Overactivity in globa, muscles – quads,

latissimus, pects, obliques, erector latissimus, pects, obliques, erector spinaespinae

Muscle strategyMuscle strategy

• High load strategy for low load taskHigh load strategy for low load task

• Produces excessive compression, Produces excessive compression, loss of mobility, loss of shock loss of mobility, loss of shock absorbtionabsorbtion

• Tendency to rely on ‘ankle strategy’ Tendency to rely on ‘ankle strategy’ to maintain balanceto maintain balance

Functional movement Functional movement testingtesting

• One leg standOne leg stand

• Standing knee bendStanding knee bend

• WalkingWalking

• Heel raiseHeel raise

• Sit to standSit to stand

ManagementManagement

• Time – listen to story, answer questions, Time – listen to story, answer questions, identify needs/expectations, address identify needs/expectations, address fears/barriersfears/barriers

• Communication – greater benefit and cost Communication – greater benefit and cost effectiveness when patients who effectiveness when patients who expressed apreference received their expressed apreference received their preferred treatmentpreferred treatment

• Reassurance – finally have diagnosis, not Reassurance – finally have diagnosis, not life threatening, can be proactivelife threatening, can be proactive

Prioritise treatmentPrioritise treatment

• Try to avoid chasing the painTry to avoid chasing the pain

• Patients expectationsPatients expectations

• Short and long term goalsShort and long term goals

• Achievable Achievable

• EnjoyableEnjoyable

Treatments Treatments

• SupportsSupports

• TapeTape

• Pre-exercising readiness – breathing, Pre-exercising readiness – breathing, relaxation, pain relieving modalities, relaxation, pain relieving modalities, manual therapy, posture re manual therapy, posture re education education

Correct movement Correct movement dysfunctiondysfunction

• Start in non weight bearing, pain free Start in non weight bearing, pain free positionspositions

• Closed chainClosed chain

• Improve joint positioning and Improve joint positioning and awarenessawareness

Joint stability and controlJoint stability and control

Challenge stabilityChallenge stability

Improve balance and coordinationImprove balance and coordination

• Incorporate into weightbearing and Incorporate into weightbearing and functional positionsfunctional positions

• Introduce unpredictability using Introduce unpredictability using balance boards, wobble cushions, gym balance boards, wobble cushions, gym ballball

StretchingStretching

• Often advised not to stretch –danger of Often advised not to stretch –danger of overstretching/damageoverstretching/damage

Reassure and educate – good to stretchReassure and educate – good to stretch

• Maintain muscle length, joint range, Maintain muscle length, joint range, stretch out old injuries and muscle stretch out old injuries and muscle spasmspasm

• No stretching beyond their No stretching beyond their hypermobile rangehypermobile range

EducationEducation

• Be positiveBe positive

• Joint care – avoidance of unhelpful Joint care – avoidance of unhelpful postures and activitiespostures and activities

• PacingPacing

• Discuss lifestyle modifications – Discuss lifestyle modifications – occupation, family life, sport, occupation, family life, sport, pregnancy and other health issuespregnancy and other health issues

General fitnessGeneral fitness

• Encourage lifelong commitment to Encourage lifelong commitment to exercise and maintenance of good exercise and maintenance of good general fitnessgeneral fitness

• Encourage normal activities and Encourage normal activities and return to sportreturn to sport

• Pilates, yoga, exercise in water, Pilates, yoga, exercise in water, walkingwalking

Main aim of treatmentMain aim of treatment

• Increase functionIncrease function• Decrease disabilityDecrease disability• Self managementSelf management

Treatment often takes longer(many Treatment often takes longer(many affected areas, longer healing time, affected areas, longer healing time, mismanaged in past)mismanaged in past)

Complete resolution rarely occursComplete resolution rarely occurs

Contacts/resourcesContacts/resources

• www.hypermobility.orgwww.hypermobility.org

• www.ehlers-danlos.orgwww.ehlers-danlos.org

• www.arthritisresearchuk.orgwww.arthritisresearchuk.org