HYPERMOBILITY SYNDROME

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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.
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
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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
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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
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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
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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
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