HYPERMOBILITY SYNDROME/EDS III LORRAINE FRIEL EXTENDED SCOPE PRACTITIONER CENTRE FOR RHEUMATIC DISEASES GLASGOW ROYAL INFIRMARY

HYPERMOBILITY & HYPERMOBILITY SYNDROME Range of movement in excess of the accepted normal range of motion at a joint, taking into account the age, gender and ethnic background of the individual (Grahame 2010) Musculoskeletal symptoms in the presence of generalised joint hypermobility but in the absence of other defined rheumatic diseases (Kirk et al 1967)

What is joint hypermobility syndrome? Pereception of JHS as a mild or trivial condition with lax joints, pain, joint dislocation/subluxation, possible OA in later life. This has changed….. Now considered an inherited, genetically determined multisystemic disorder of connective tissues rendering them more vulnerable to injury and mechanical failure. E effects of injury

WHAT IS HMS? A family of related genetically based conditions. The protein affected varies and the degree of difference varies Marfans Syndrome Ehlers-danlos Benign Joint Hypermobility syndrome

Presentation Chronic pain and kinesiophobia Joint laxity,subluxations/dislocations Vulnerability to injury Rest at EOR/”lock” joints and poor posture habits Dysfunctional movement patterns Poor healing and slower recovery Easy bruising and tendency towards bleeding

Non articular presentation Fatigue Deconditioning Autonomic dysfunction Pelvic organ prolapse Urinary incontinence Psychological POTS

Examination Observation – skin, postural alignment Range of movement Functional activities Muscle testing Neurological testing Passive movement Ligament integrity Balance/proprioception

Good postural alignment Muscular and skeletal balance which protects the supporting structures against injury and progressive deformity Muscles function most efficiently Optimum positions for thoracic and abdominal organs

Habitual postures Frequently rest at EOR and poor postural alignment Stress and strain in HM collagenous tissues Decreased muscle use leading to stiffness, weakness, deconditioning, fatigue

Poor postural alignment Faulty relationship produces stress and strain on supporting structures Less efficient balance

Active movement Look well Move well Subjective and objective often at odds Check ‘normal’ range for that patient

Assess muscle function Breathing Transversus abdominus Deep multifidus Pelvis floor Timing, atrophy, loss of tonic function, loss of co-ordination, asymmetry, length Overactivity in globa, muscles – quads, latissimus, pects, obliques, erector spinae

Muscle strategy High load strategy for low load task Produces excessive compression, loss of mobility, loss of shock absorbtion Tendency to rely on ‘ankle strategy’ to maintain balance

Functional movement testing One leg stand Standing knee bend Walking Heel raise Sit to stand

Management Time – listen to story, answer questions, identify needs/expectations, address fears/barriers Communication – greater benefit and cost effectiveness when patients who expressed apreference received their preferred treatment Reassurance – finally have diagnosis, not life threatening, can be proactive

Prioritise treatment Try to avoid chasing the pain Patients expectations Short and long term goals Achievable Enjoyable

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

Correct movement dysfunction Start in non weight bearing, pain free positions Closed chain Improve joint positioning and awareness

Joint stability and control Challenge stability Improve balance and coordination Incorporate into weightbearing and functional positions Introduce unpredictability using balance boards, wobble cushions, gym ball

Stretching Often advised not to stretch –danger of overstretching/damage Reassure and educate – good to stretch Maintain muscle length, joint range, stretch out old injuries and muscle spasm No stretching beyond their hypermobile range

Education Be positive Joint care – avoidance of unhelpful postures and activities Pacing Discuss lifestyle modifications – occupation, family life, sport, pregnancy and other health issues

General fitness Encourage lifelong commitment to exercise and maintenance of good general fitness Encourage normal activities and return to sport Pilates, yoga, exercise in water, walking

Main aim of treatment Increase function Decrease disability Self management Treatment often takes longer(many affected areas, longer healing time, mismanaged in past) Complete resolution rarely occurs

Contacts/resources www.hypermobility.org www.ehlers-danlos.org www.arthritisresearchuk.org