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HYPERMOBILITY SYNDROME/EDS III

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

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HYPERMOBILITY SYNDROME/EDS III

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

  2. 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) HYPERMOBILITY & HYPERMOBILITY SYNDROME

  3. 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.

  4. 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

  5. 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

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

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

  8. 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 Good postural alignment

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

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

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

  12. 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

  13. 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

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

  15. 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

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

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

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

  19. 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

  20. 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

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

  22. 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

  23. 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

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

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