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Physiotherapy Management of Joint Hypermobility Syndrome BSPAR: Guidelines for Management of Joint Hypermobility Syndrome in Children and Young People Louise Bailey Senior Paediatric Physiotehrapist in Rheumatology Our Lady’s Children’s Hospital Crumlin [email protected] Background Common in general population (7% UK, 12% USA, 14% Dutch, 53% India) Many people do not experience symptoms Many use it to their advantage: Dancers, Athletes Some people experience difficulties and pain related to joint hypermobility Referred to as Joint hypermobility syndrome Ehlers-Danlos syndrome Type III (Hypermobile EDS) Can be unhelpful for a young person to have the label of EDS III because the significant risks associated with the other forms of EDS can mistakenly be assumed to also apply to this group. The preferred term to use is Joint Hypermobility Syndrome (JHS) Signs and Symptoms Vary considerably The main features are: Muscle pain Joint pain (+/- swelling) /Arthralgia Fatigue Easy bruising (common symptom & should not cause concern) Clicking of joints (common symptom & should not cause concern) Less commonly Abdominal pain +/- some levels of bladder and bowel dysfunction Even Less common: postural orthostatic tachycardia syndrome (POTS) Hernia Uterine or rectal prolapse Joint dislocation (though subluxation is slightly more common) Not life threatening Proactive & Positive self-management Full participation in all activities “Connective Tissue Advantage” Not a disability Treatment Aims Improve symptoms Avoid future complications Widespread and longstanding pain can result in a downward spiral of general physical deconditioning, pain and fatigue. Improve patient understanding of JHS and the skills and knowledge to self-manage the condition Full participation in all activities Management of JHS MDT Physiotherapy Occupational Therapy Psychology Clinical Nurse Specialist Consultant Subjective Assessment Presenting Complaint Past Medical History Medication History Social and Family History Mental health and well being Benefits Participation in ADLs including selfcare, leisure and sleep Impact on learning and participation in school activities Career planning / development Information given to date? Level of understanding of problems Occupational / Physiotherapy / Other Professionals Presenting Complaint: Joint and/or muscle aches and pains Usually after exercise or increased activity During the night May occur for a few days after increased activity More often lower limb than upper Fatigue Often associated with reduced walking distance and reduced exercise tolerance School absenteeism due to tiredness. Usually linked to level of deconditioning of the child. Poor sleep will also impact upon fatigue levels. Fidgetiness, difficulty sitting still and poor organisation of movement etc Headaches This is often related to muscle spasm of the Trapezius muscles. Poor sleep Easy bruising This is benign and not of concern. Clicking joints Joints can click spontaneously or be clicked deliberately, both are fine Reduced co-ordination and balance Poor proprioception leading to clumsiness and reduced balance. Poor core stability leading to difficulties such as hand writing challenges and other issues with fine motor control. Reduced fine motor control Abdominal pain This is common in childhood generally and the incidence may be slightly higher in children who are hypermobile. Constipation is a common cause of abdominal pain. Remember that many of these symptoms have normal variants in the general population and care must be taken to ensure that there is a balance between medical investigation and effective symptom management Additional but rare Symptomatology: Gastrointestinal and urinary tract symptoms Cardiovascular autonomic dysfunction Orthostatic hypotension (Low BP on standing) Orthostatic intolerance (Symptoms after standing for long periods) Postural orthostatic tachycardia syndrome (POTS) (rise in HR on standing, AX and diagnosis not validated in children) Objective Assessment: Baseline measurements: Height Weight Blood pressure Pain (VAS) Fatigue (VAS) General Wellbeing (VAS) Joint Assessment Joint Range of Motion (Which joints are hypermobile; Swelling; Pain; Restrictions) pGALS & Goniometry Beighton Scale Goniometry recommended pGALS Assessment http://www.rch.org.au/ uploadedFiles/Main/Content/ MedEd/fracp/pGALS%20.pdf Beighton Scale for JHS Objective Assessment Muscle length tests Be aware of muscles that move over 2 joints Hamstring Tightness common Popliteal angle Gastrocnemius WB Lunge test NWB Ankle DF Objective Assessment Muscle Strength A basic assessment of muscle strength is vital Kendal Scale (0-10) Oxford Scale Specific muscles should be assessed including: Inner Range Quads (Straight Leg Raise without a quadriceps lag) Hip abductors – specifically Gluteus Medius (Clam) Hip Extensors – specifically Gluteus Maximus (Prone, knee flexed to 90) Plantar Flexors (10 single leg calf raises through full range) Core Stability Posture and Gait Both should be assessed especially with the view to prescribe orthotics. Stamina 6 min walk test Function CHAQ- childhood health assessment questionnaire (Not yet formally validated for JHS) Assessment of self-care, leisure activities, school activities, dressing etc. Physiotherapy Treatment Physiotherapy Treatment Restore and maintain full muscle strength and function throughout the full range of movement. Restore effective and efficient movement patterns. Improve general fitness. Restore normal range of movement, including into hypermobile range. Provide education, reassurance, advice, pain management and develop problem solving. Strengthening exercise Specific exercise programme Progressive resisted exercise programme Targeting specific muscles that are weak and that are required to control the joints into their hypermobile range. Open chained exercises Non-weight-bearing High repetitions Low weights Activity and Sport Physical activity should be actively encouraged The child must be fit enough to engage in the sport they wish to do. May need to gradually build up their strength and stamina before engaging in the new activity. Physiotherapy programme can be used to ensure that every muscle is strong enough With the correct preparation any sport can be considered. Posture Protects supporting structures against injury Enables muscles to function most efficiently Core Strengthening Improve stability, balance and coordination Incorporate functional and everyday activities. Balance boards, wobble cushions and gymnastic balls as well as specific exercises. Stretching maintain muscle length, joint range and to stretch out old injuries or muscle spasms Stretching should not be to increase an already hypermobile range Joint Subluxations Repeated self-subluxation of joints should be avoided Goal setting ‘Realistic’ goal setting (both short and long term) Help promote progression Proprioception training Propriocetion exercises are helpful in JHS Use of balance boards, mirrors, bio feedback Sport specific assessment enable child/young person to participate fully. Education Important to identify any catastrophising and avoidance behaviours fear cycle Parents Strateiges to help them understand their child’s pain Promote positive rather than unhelpful patterns of behaviour Realistic expectations Ensure rest is not promoted for prolonged periods Fatigue Not more rest Improved fitness Pacing Sleep hygiene Education - Self management Non life threatening condition Fully involved in the treatment plan Benefit from maintaining a healthy everyday life: A well balanced diet Regular exercise Full participation in school and activities Quality sleep Encouragement is needed to include appropriate exercise into their daily routines Participate in all activities Inactivity greatly exacerbates symptoms Understanding of pacing avoiding the ‘boom and bust’ cycle As children and adolescents grow up, gaining an understanding themselves about JHS will help them develop the motivation needed to manage their condition and develop a healthy lifestyle incorporating regular exercise Education - Pain Management Recognition and management of chronic pain is important Pain Usually warning about damage to the body Withdraw or avoid activity JHS pain is an indication that the body is not strong enough to do the task It requires strengthening Keep engaging in everyday activities and exercise Can affect concentration, memory, mood and sleeping Fear of the unknown can be a source of anxiety Referral to psychology (if possible!) Pain medication is often ineffective Aim is to increase quality of life Develop patient goals Management: Pain Tool Kit http://www.paintoolkit.org/images/uploads/downloads/ Pain_Toolkit_patient_booklet_copy_Short_Versions.pdf Comfort Tool Box Warm baths Music Relaxation Wax Hot/cold packs Favourite cartoons/ Distraction Relaxation: Sleepy Starfish ...Lie down on your back with your legs and arms apart. Stay very still and imagine you are a sleepy starfish resting at the bottom of the ocean. Breathe in and as you breathe out relax you arms. Breathe in and as you breathe out relax your legs, breathe in and as you breathe out relax your head. See how still you can be at the bottom of the ocean, resting like a sleepy starfish. Repeat to yourself I am silent, I am silent... Ref: www.relaxkids.com Trampolining Special consideration needs to be taken with trampolining and bouncy castles These activities put significant stress on the joints They often involve several children at a time which can increase the risk of injury. Weight Management Normal/ optimal weight is important Extra weight can put significant strain on the joints This can increase the symptoms of pain and fatigue Advice may be provided about healthy diets Equally important not to become underweight Can promote muscle weakness May not be safe to engage in an intensive exercise until the young person is at a healthy weight Mood and Motivation Symptoms of JHS can impact mood Reducing capacity to participate in normal ADL’S and recommended plan Symptoms can be further exacerbated Low mood and anxiety are commonly experienced by people stuck in such a negative cycle and they may start to avoid activities such as a result of a fear of causing more pain and /or fatigue. MDT assessment: may benefit from further support in managing emotional distress associated with chronic symptoms. Equipment Special equipment is not required NEVER provide a wheelchair Crutches are equally unhelpful and should also be avoided in the management of JHS. Case Study Subjective Assessment Patient X aged 7 PC: Bilateral non-specific leg pain. HPC: Pain following activity. Affecting function. Unable to attend school for a number of days following high levels of physical activity. Pain generally nonspecific. Affecting the lower limbs. Also complains of wrist and hand pain but less frequently Past Med Hx: Attended orthopaedics for review of genu valgus from age of 4-7 years (Normal variant). Referred to rheumatology for review due to leg pain. Investigations: MRI with contrast: Left ankle- No synovitis X-Rays: Knees, wrists, ankles- Normal (No effusions. No Erosive joint disease) Subjective Assessment Meds: Calpol & Neurofen (Minimal affect) Social Hx: Not participating in any extracurricular activities Mum and Dad separated. Mum main carer. Dad involved. Fam Hx: Maternal Aunt diagnosed with Behcets Maternal Grandmother terminal Ca. Mum a carrier of BRCA gene and awaiting surgery Objective Assessment pGALS: No evidence of joint swelling No joint restriction No heat, No redness No rash Pain: Forward flexion, bilateral wrist pain at EROM, left hip pain on flexion, left ankle range of movement Posture Genu valgus Internal rotation of lower limbs Mild anterior pelvic tilt Gait: In-toeing gait pattern. No orthopaedic concerns. Normal variance. Can correct on cueing Beighton 6/9 Objective Assessment Muscle Length: Hamstrings very tight+++ Muscle Strength Tests: Global weakness. Glute Med, Glute Max, IRQs, Quadriceps, gastrocnemius, reduced core strength, reduced shoulder stability Increased BMI Management In-patient week 1 Education Pain tool kit Exercise Programme: Stretching and strengthening Advice about participation in sport/extracurricular activities Pacing Out-Patient Appointments every 2-3 weeks Poor compliance with HEP School absenteeism Poor understanding of pacing RX: Education Management: Education Education About Symptomatic Hypermobility http://apcp.csp.org.uk/documents/ parent-leaflet-symptomatichypermobility-2012 Management: Exercise Pain Tool Kit Tool 8 - Stretching & Exercise Many people with pain fear exercise in case it causes more problems. However this is not true. Regular stretching and exercising actually decreases pain and discomfort. It prepares the body for other activities. It can strengthen weak muscles and you will also feel better for it. Remember to start slowly and build up or increase your stretching and exercising. It is not as hard as you think. If you are in pain, remember that unfit and under used muscles feel more pain than toned ones……. Management: Exercise Strengthening Hip strengthening: SLR, side-lying Hip ABD, Prone Hip Ext, Clam, Quads Strengthening: Squats Core Strengthening: Superman, Abdominal curl up Upper limb: Wall press-ups, Down Dog Stretching: Hamstring stretches: Down Dog Quad Stretch: Side lying Gastroc Stretch: Standing At 6-months Still having a lot of difficulties with compliance Secondary to pain Poor evidence of Pacing Attending psychology Commenced group (7-9yrs) 4-week programme PT & OT Mat work, stretching, Handwriting, group games HEP to be completed at home Parent education x 1-hour at week 2 Engaging well at week 3. Started athletics in a graded manner. Pain greatly improved. Completed a full week of school. Case 2 12 year old boy PC: Leg pain mainly at the front of the thighs and the back of the knees worse after activity. Agg: Running Back Pain Agg: carrying heavy items. Hx of wakening at night. Wrist & hand pain Agg: Writing in school Eases: Heat, hot bath/shower HPC: Referred to orthopaedics who referred to rheumatology consultant for joint pain. No concerns of inflammatory disease. Diagnosed with Symptomatic JHS. Past Med Hx: Right Clavical fracture 9/12 ago Investigations: Nil Meds: Neurofen Soc Hx: Boxing. Stopped soccer and gaelic. Also stopped going out with friends School: Difficulty with hand writing. Takes a long time to do homework. Teacher not very understanding. Fam Hx: Older sister PAS Concerns: Concerned about serious pathology. Gets upset and feels down. Feels under pressure at school about his handwriting Objective Assessment pGALS: No evidence of joint swelling No joint restriction No heat, No redness No rash Pain: Forward flexion in back of knees bilateral wrist pain at EROM Posture Slumped posture in sitting Gait: Nil of note Beighton 6/9 (Elbows +18 R, +16 L, Knees+10 bilaterally, Little finger ext >90 B/L) Objective Assessment Muscle Length: Hamstrings: Popliteal angle Left -45 Right -35 Hip flexors: positive Thomas test Bilaterally R>L Rec Femoris: Tight bilaterally Muscle Strength Tests: Reduced core strength, reduced shoulder stability Treatment Seen every 3-weeks for 2-months before Christmas Stretching exercises Pain education Posture education Relaxation techniques Strengthening exercises for Hands Pacing education Information letter to school Advice re sport participation Treatment Apt in Dec (4th) Apt 1 after x-mas: No pain Reviewed stretches Hams, Quads, Hip Flexors length WNL Educated regarding exercise, relaxation and heat Started MMA and athletics Posture education Having warm baths daily after activity Educated regarding return to sport Doing HEP daily Apt 2 after xmas: Relapsed over christmas Mild traps pain Increased pain when returned to school. Quite down. HEP daily in school at 8:30 Hadn’t been doing HEP Hams, Quads, Hip Flexors length WNL Activities finished for approx 1-month Returned to Boxing, MMA, Athletics Take home Message JHS should be regarded as a “connective tissue advantage” providing the child is strong and fit, difficulties occur mainly when the body has become weak and deconditioned. Self management of the symptoms is vital for the long-term effective management of JHS in children and young people. This may include a specific exercise regime and active participation in nonpharmacological pain management techniques to ensure full participation in all activities. Pain management and education is an important role of any Allied Health Professional.
