Botulinum Toxin Therapy for the Upper Limb CP Network May 2013 Susan Horsburgh

Outline of Session How BTX-A works Evidence to support use History of Service Case Studies

How BTX-A Works BTX-A blocks the release of acetylcholine at the neuromuscular junction Produces a short-term, and reversible, paralysis of the treated muscle Effects are at their maximum after 10 to 12 days Best evidence supports therapy after injection to maximise outcomes

BTX-A Therapy BTX-A is protein produced by the bacterium Clostridium Botulinum – Botox® Allergan – Dysport Given by intramuscular injection as near to the motor end plate as possible Protein is reconstituted in 0.9% saline solution

Best Practice Good pre- and post-treatment assessment with appropriate outcome measures linked to the ICF and child’s GMFCS level Identification of the muscle using – EMG stimulation – US guidance Number of Units – 4-6 units/kg body wt. within a range 1-20U/kg – Maximum 100U for large muscles, 50U for small Volume of Dilution – 100U per 1-2ml solution

Factors Influencing Treatment Site of Injection – Muscles of children with cerebral palsy are not where expect to be – Need to use US to detect upper limb muscles – Slows process increasing stress Dilution – Too much solution can spread too far, takes longer to inject – Too little and effect is inadequate

Factors Influencing Treatment Clinic Process – Younger children have oral sedation prior to injection Midazolam – Older children can use Entonox – Local anaesthesia Ethyl Chloride spray LMX/Emla cream - lidocaine

Question Does botulinum toxin improve hand function in children with cerebral palsy and upper limb spasticity?

Evidence Pubmed Search Terms – botulinum toxin; cerebral palsy; upper limb spasticity 41 articles – 18 reviews 5 relevant, peer reviewed, English Language, several authors – Reeuwijk et al (2006) Clinical Rehab – Boyd et al (2001) European Journal of Neurology – Hoare et al (2010) Cochrane Database – Lukban et al (2009) Journal of Neural Transmission – Delgado et al (2010) American Academy of Neurology

Evidence Younger children respond better First treatment produces the largest response BTX-A should be combined with OT input for maximum benefit Careful selection of muscles required

Muscle Selection 6 articles – Search Terms: Upper limb spasticity; muscle selection; botulinum toxin; cerebral palsy – 4 – 1 case report, 2 clinical reviews, 1 RCT (effect on nerve endings) – Children can have unusual muscle action therefore muscle palpation is very inaccurate – Pronator teres may be first muscle to contribute to ↓ ROM – Small doses, serial treatment, and multi-level for function – large dose, multi-level for cosmetic/ease of handling – Thumb significant in grasp – muscle selection unclear

Lanarkshire/Yorkhill Service Pre- and post-treatment assessment takes place in Lanarkshire Children are seen in Yorkhill Hospital with Consultant Neurologist and Community Consultant Paediatrician Treatment of upper limb since April 2012 Recently purchased US machine Supply of low dose vials

Aims of Treatment Cosmetic Ease of handling Reduce pain Maintain/Improve range of movement – Splinting Improve function

Personal Experiences of Treatment for Upper Limb Cosmetic – Teenager with hemiplegia Ease of handling – GMFCS level 5 dressing upper garments Improve tolerance of splint Function

Cosmetic 15 year old boy with hemiplegia GMFCS level 1; teased at school because of associated reactions causing arm to flex on effort Pre-Assessment – None Treatment – 50U each to brachialis and brachioradialis Post-Assessment – Subjectively ROM increased but forearm very pronated; less marked associated reactions on walking – Teenager happy with outcome Future – Visual analogue scale – ROM

Ease of Handling 7 year old boy with dystonic athetosis GMFCS level 5; post hip surgery with gross asymmetry Pre-Assessment – CPUP for lower limbs – Subjective upper limbs Treatment – 50U bilateral pectorals Post-Assessment – Ease of all areas of ADL – Improved alignment Now use Care and Comfort Questionnaire

Maintain Range of Movement 14 year old boy with spastic quadriplegia GMFCS 5; wants to maintain range of movement at his wrists for accessing computer and wheelchair Pre-Assessment – Comprehensive ROM upper limb Treatment – 25U very specific treatment using US guidance Post-Assessment – All joints improved ROM – Improved access to playstation

Improve Function 12 year old girl with athetoid hemiplegia dystonia GMFCS level 1; had treatment with no assessment with excellent outcomes Pre-Assessment – Comprehensive ROM Treatment – Brachialis due to -4° elbow extension 25U – Pronator Teres due to ¾ range 25U – FDP 2 sites due to flexion fingers 30U – FDS 2 sites 30U – Thenar eminence due to thumb adduction 10U

BUT Post-Assessment – Full ROM all joints – Unable to use hand functionally Fixing hair Closing car door – Lost natural swing of arm for walking and running

Summary BTX-A is a useful adjunct to therapy – Can ease management for families of more severely impaired children – Can reduce pain (calf muscles, back, splints) – Can improve function BUT Needs to be linked to OT assessment and treatment