HYPERMOBILITY SYNDROME/EDS III

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HYPERMOBILITY SYNDROME/EDS III

• LORRAINE FRIEL
• EXTENDED SCOPE PRACTITIONER
• CENTRE FOR RHEUMATIC DISEASES
• GLASGOW ROYAL INFIRMARY

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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)

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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.

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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

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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

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Non articular presentation

• Fatigue
• Deconditioning
• Autonomic dysfunction
• Pelvic organ prolapse
• Urinary incontinence
• Psychological
• POTS

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Examination

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

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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

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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

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Poor postural alignment

• Faulty relationship produces stress and strain on
  supporting structures
• Less efficient balance

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Active movement

• Look well
• Move well
• Subjective and objective often at odds
• Check normal range for that patient

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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

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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

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Functional movement testing

• One leg stand
• Standing knee bend
• Walking
• Heel raise
• Sit to stand

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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

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Prioritise treatment

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

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Treatments

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

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Correct movement dysfunction

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

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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

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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

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Education

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

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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

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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

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Contacts/resources

• www.hypermobility.org
• www.ehlers-danlos.org
• www.arthritisresearchuk.org