Cerebral Palsies Pathway, Camden Community Child Health Team

Transcription

1 Cerebral Palsies Pathway, Camden Community Child Health Team Contents Pages Content 1. 1 Contents 2. 2 Flow chart: overall pathway process Pathway mapping by age group: 4. 3 Initial assessment and diagnosis (any age) 5. 3 Birth 3 months months months months months years years years years years years Appendix 1: Physiotherapy Input according to GMFCS level Appendix 2: Occupational Therapy Packages of Care Appendix 3: Standardised and Non-standardised assessments administered by Occupational Therapists Appendix 4:Gross Motor Function Classification System (GMFCS) Appendix 5: Manual Ability Classification System (MACS) Appendix 6: Assisted Hand Assessment (AHA) Appendix 7: Audit of Need Scores Appendix 8: Goal Attainment Scaling (GAS) scores Appendix 9: Communication Function Classification System (CFCS) Appendix 10: Guide to levels of risk of negative health consequences from dysphagia Appendix 11: Assessment of the child with suspected Cerebral Palsy Appendix 12: Cerebral palsy growth charts Appendix 13: Guidelines for Hip Surveillance in Children with Cerebral Palsy Appendix 14: Guidelines for the management of osteopaenia Appendix 15: Management of gastro-oesophageal reflux Appendix 16: Management of constipation Appendix 17: Management of drooling Appendix 18: Medical management of spasticity Appendix 19: Referral to Camden Dental Services Appendix 20: End of life plans References Contributors & Thanks 1

2 Flowchart to demonstrate overall pathway, Camden Child Development Services: GP / HV Referral of child with suspected CP or evolving motor disorder High risk groups, including perinatal network Therapy Services Referral Secondary Care Services Out of borough Private healthcare Single point of referral meeting (Community Child Health team) Hospital Clinic: Paediatrician +/- PT +/- OT Child Development Team Clinic: Paediatrician +/- PT +/- OT Mosaic Clinic: Paediatrician, PT & OT Initial Assessment Normal Development Other developmental disorder Suspected CP or evolving motor disorder Discharge Multi-disciplinary / specialist referrals Follow-up appointment: with or without child present Intervention Pre-School Age Children Regular assessment Intervention according to age and audit of need score Goal-directed therapy evaluated using GAS score Support from education professionals Transition to School Services School Age Children Regular assessment Intervention according to age and audit of need score Goal-directed therapy evaluated using GAS score Support from education professionals 2 Assessment & Intervention: Family Support Development, motor skills & play Language & communication Feeding Education Physical Health Transition to Adult Services

3 Initial Assessment & Diagnosis (any age) Physical Health (see pathway for diagnostic assessment for children with suspected CP) Initial Appointment: multi-disciplinary assessment to consider Diagnosis Further investigations as indicated Onward referrals as appropriate: consider cognitive delay, hearing, vision, speech & language, feeding, nutrition and growth Discussion with family re: diagnosis, prognosis, other avenues of support and arrangement for follow-up Follow-up Appointment: multi-disciplinary review to consider Results of investigations Diagnosis and prognosis GMFCS & MACS classification Need for hip surveillance Blood test screening for osteopaenia Plans for engagement with PT & OT teams Agree named professional for child and family Offer referral to CAMHS team if appropriate With consent, copy all correspondence to the SEN Section at Crowndale Family Support Offer support via Children s Centre Services (referral via CAF or families can access services independently) Offer referral to Disabled Children s Team Information on local and national support groups Paediatrician OT PT Paediatrician OT PT HV Play and Development Plan engagement with PT & OT teams PT & OT Language, Communication & Feeding Referral to SLT if indicated Paediatrician New referral to SLT checked with Mosaic SLT Education Special Educational Needs section notified via notification form SEN Birth 3 months: predominantly for those infants identified as high risk in immediate neonatal period (Note diagnosis is unlikely to have been made at this stage) Family Support Visit at earliest opportunity when home by HV or SHV to: Observe and advise about feeding and development Monitor growth Supply inserts for PCHR (red book) Obtain parental consent for referrals Offer support via Children s Centre Services (see above) Consider child care requirements with family Ensure that the following professionals/teams are notified as appropriate: o community paediatric physiotherapy (PT) o community paediatric occupational therapy (OT) o speech and language therapy (SLT) o community paediatrician Referral to KIDS (home based learning Portage services) Information offered: Early Support Information for parents, Cerebral Palsy 3 HV / SHV SHV

4 Development, Motor Skills & Play Consider multi-disciplinary assessment with PT Baby visited in Neonatal Unit or Home Visit to carry out Prechtl Assessment and Brazelton Neonatal Behavioural Assessment Scale and offer Anticipatory Guidance to parents OT input according to level I, II or III package of care Evaluate intervention using GAS scores Consider multi-disciplinary assessment with OT Assessment of tone (severity & distribution), posture and pattern of movement Assessment of righting and equilibrium reactions, and protective extension Assessment of range of movement (passive & active) PT input according to audit of need score Evaluate intervention using GAS scores Demonstration of activities for parents to implement with their child and provision of home activity programme Advise parents on appropriate positions for carrying, dressing and feeding Consider picture programmes for advice on activities and posturing Language & Communication Information from support organisations provided e.g. Bliss guide for feeding premature babies Awareness of newborn hearing results Feeding MDT liaison re: feeding with acute and community services Home based support provided as needed OT PT OT & PT SLT SLT Education Special Educational Needs section notified via notification form. SEN Support child care facilities in delivering developmentally appropriate programme EYIT 3 months: predominantly for those infants identified as high risk in immediate neonatal period (Note diagnosis is unlikely to have been made at this stage) Family Support Information on local and national support groups provided if not previously accessed Referral to Disabled Children s Team (DCT) should be offered Consider to children s centre Offer counselling services if required Provide advice about benefits including DLA Physical Health Growth & nutrition (weight, length, BMI) Review immunisation status Assessment of hips and spine Consider management of spasticity as appropriate Review cardiac and respiratory function Gastrointestinal review including reflux, constipation and drooling Consider need for Ophthalmology and Audiology services Developmental status: including learning ability Review behaviour & emotional well being Screen for sleep disorder Consider end of life plans with family if appropriate 4 HV Paediatrician

5 Offer referral to CAMHS team if appropriate With consent, copy all correspondence to the SEN Section at Crowndale Development, Motor Skills & Play General advice given to promote all areas of development. Ensure Early Support materials provided where appropriate. Consider multi-disciplinary assessment with PT Offer 3 month Bayley Scales of Infant and Toddler Development assessment and offer Anticipatory Guidance. Score Bayley and write report OT input according to level I, II or III package of care Evaluate intervention using GAS scores Consider multi-disciplinary assessment with OT Assessment of tone (severity & distribution), posture and pattern of movement Assessment of righting and equilibrium reactions, and protective extension Assessment of range of movement (passive & active) PT input according to audit of need score Evaluate intervention using GAS scores Demonstration of activities for parents to implement with their child and provision of home activity programme Advise parents on appropriate positions for carrying, dressing and feeding Consider picture programmes for advice on activities and posturing HV OT PT OT & PT Language & Communication Advice for promoting early language/communication skills SLT Feeding Ongoing support for feeding needs if appropriate SLT Education Special Educational Needs section notified via notification form. SEN Support child care facilities in delivering developmentally appropriate programme EYIT 6 months Family Support Ongoing support for family and ensure referrals to relevant services (as listed above) have been made, depending on needs of the family Physical Health Growth & nutrition (weight, length, BMI) Review immunisation status Assessment of hips and spine Consider blood test screening for osteopaenia Consider management of spasticity as appropriate Review cardiac and respiratory function Gastrointestinal review including reflux, constipation and drooling Consider need for Ophthalmology and Audiology services Developmental status: including learning ability Review behaviour & emotional well being Screen for sleep disorder Consider end of life plans with family if appropriate HV Paediatrician 5

6 Offer referral to CAMHS team if appropriate With consent, copy all correspondence to the SEN Section at Crowndale Development, Motor Skills & Play Offer 6 month Bayley Scales of Infant and Toddler Development assessment and offer Anticipatory Guidance. Provide therapy input as part of multi-disciplinary team OT input according to level I, II or III package of care Evaluate intervention using GAS scores Assessment of tone (severity & distribution), posture and pattern of movement Assessment of righting and equilibrium reactions, and protective extension Assessment of range of movement (passive & active) PT input according to audit of need score Evaluate intervention using GAS scores Home Visit if required to look at equipment needs Demonstration of activities for parents to implement with their child and provision of home activity programme Advise parents on appropriate positions for carrying, dressing and feeding Consider picture programmes for advice on activities and posturing OT PT OT & PT Language & Communication Review of SLT needs as part of developmental review SLT Sessions/ programme to promote early language/communication skills If complex medical needs to be jointly managed by ISDC SLT ISDC SLT Education Special Educational Needs section notified via notification form. SEN Support child care facilities in delivering developmentally appropriate programme EYIT 12 months Family Support Ongoing support for family and ensure referrals to relevant services (as listed above) have been made, depending on needs of the family Physical Health Consider GMFCS classification Growth & nutrition (weight, length, BMI): plot on appropriate growth chart Review immunisation status Assessment of hips and spine: initial hip surveillance with X-ray at months of age Consider blood test screening for osteopaenia Consider management of spasticity as appropriate Review cardiac and respiratory function Gastrointestinal review including reflux, constipation and drooling Consider need for Ophthalmology and Audiology services Developmental status: including learning ability Review behaviour & emotional well being Screen for sleep disorder Consider referral for dental review HV Paediatrician 6

7 Consider end of life plans with family if appropriate Offer referral to CAMHS team if appropriate With consent, copy all correspondence to the SEN Section at Crowndale Development, Motor Skills & Play Offer 12 month Bayley Scales of Infant and Toddler Development assessment and Anticipatory Guidance. OT input according to level I, II or III package of care Evaluate intervention using GAS scores and review appropriate package of care Refer to Upper Limb Clinic if appropriate Request Assistive Technology assessment if appropriate. Refer to seating clinic if appropriate. Refer to Social Care OT for housing adaptations / equipment needs if appropriate Assessment of tone (severity & distribution), posture and pattern of movement Assessment of righting and equilibrium reactions, and protective extension Assessment of range of movement (passive & active) Evaluate intervention using GAS scores Classification of GMFCS MACS equivalent score Do Risk assessment and develop Manual handling plan considering GMFCS level. Home Visit if required to look at equipment needs. Refer to Wheelchair clinic for buggy/ wheelchair if needed. Demonstration of activities for parents to implement with their child and provision of home activity programme Advise parents on appropriate positions for carrying, dressing and feeding Consider picture programmes for advice on activities and posturing (GMFCS III, IV & V) OT & SLT OT PT OT & PT Language & Communication Planned assessment/ review/ therapy sessions according to need. SLT Feeding Ongoing feeding support if needed Close liaison with MDT/ including dietetics re: hydration and nutrition Management of oral and non-oral feeding needs considered dependent on child s needs Developmental feeding needs closely monitored throughout e.g. transition to solids, independent feeding skills Consider assessing dysphagia score to evaluate risk of associated negative health consequences SLT Education Special Educational Needs section notified via notification form. SEN Support child care facilities in delivering developmentally appropriate programme EYIT 7

8 18 months Family Support Ongoing support for family and ensure referrals to relevant services (as listed above) have been made, depending on needs of the family Parent and toddler groups / playgroups discussed Physical Health Reconsider GMFCS classification Growth & nutrition (weight, length, BMI): plot on appropriate growth chart Review immunisation status Assessment of hips and spine: hip surveillance X-ray may be required Consider blood test screening for osteopaenia Consider management of spasticity as appropriate Review cardiac and respiratory function Gastrointestinal review including reflux, constipation and drooling Consider need for Ophthalmology and Audiology services Developmental status: including learning ability Review behaviour & emotional well being Screen for sleep disorder Consider referral for dental review Consider end of life plans with family if appropriate Offer referral to CAMHS team if appropriate With consent, copy all correspondence to the SEN Section at Crowndale Development, Motor Skills & Play OT input according to level I, II or III package of care based on assessment. Evaluate intervention using GAS scores and review appropriate package of care. Home visit if required to look at equipment needs Refer to Upper Limb Clinic if appropriate Request Assistive Technology assessment if appropriate. Refer to Seating clinic if appropriate Refer to Social Care OT for housing adaptations / equipment needs if appropriate Hemiplegics: Assisting Hand Assessment Assessment of tone (severity & distribution), posture and pattern of movement Assessment of righting and equilibrium reactions, and protective extension Assessment of range of movement (passive & active) PT input according to audit of need score Evaluate intervention using GAS scores Classification of GMFCS MACS equivalent score Refer to Wheelchair clinic for buggy/ wheelchair if needed. Demonstration of activities for parents to implement with their child and provision of home activity programme Advise parents on appropriate positions for carrying, dressing and feeding Consider picture programmes for advice on activities and posturing (GMFCS III, IV & V) HV Paediatrician OT PT OT & PT 8

9 Language & Communication Communication based sessions focused on verbal and non-verbal systems as appropriate Feeding Ongoing feeding support if needed Consider assessing dysphagia score to evaluate risk of associated negative health consequences SLT SLT Education Special Educational Needs section notified via notification form. SEN Support child care facilities in delivering developmentally appropriate programme EYIT 2 years Family Support Children s Centre Services including: o Parent and toddler groups/playgroups. o Early Years Intervention Services Local and national support groups. Referral to Disabled Children s Team (DCT) should be offered EP based in EYIP (Early Years Intervention Project) to give advice re: intervention strategies to children s centre services as needed Continence advice and support Physical Health Reconsider GMFCS classification Growth & nutrition (weight, height/knee height, BMI): plot on appropriate growth chart Review immunisation status Assessment of hips and spine: hip surveillance X-ray may be required Consider blood test screening for osteopaenia Consider management of spasticity as appropriate Review cardiac and respiratory function Gastrointestinal review including reflux, constipation and drooling Consider need for Ophthalmology and Audiology services Developmental status: including learning ability Review behaviour & emotional well being Screen for sleep disorder Consider referral for dental review Consider end of life plans with family if appropriate Offer referral to CAMHS team if appropriate With consent, copy all correspondence to the SEN Section at Crowndale Development, Motor Skills & Play Offer 2 year Bayley Scales of Infant and Toddler Development Assessment and offer Anticipatory Guidance. OT input according to level I, II or III package of care based on assessment. Evaluate intervention using GAS scores and review appropriate package of care Nursery visit to provide OT Programme and advise on equipment needs Home Visit if required to look at equipment needs 9 HV Paediatrician OT

10 Refer to Upper Limb Clinic if appropriate Request Assistive Technology assessment if appropriate. Refer to seating clinic if appropriate. Refer to Social Care OT for housing adaptations / equipment needs if appropriate Assessment of tone (severity, distribution & dynamic tone using PT Tardieu scale), posture and pattern of movement Assessment of righting and equilibrium reactions, and protective extension Assessment of range of movement (passive & active) Functional assessment of strength through activities such as sit to stand and ability on stairs Assessment of physical endurance in terms of distance a child is able to walk either outdoors or within nursery PT input according to audit of need score Evaluate intervention using GAS scores Provide therapy input as part of multi-disciplinary team OT & PT Classification of GMFCS MACS equivalent score Review Risk assessment and Manual handling plan. Contribute to Statutory Assessment and submit Risk Assessment with SA report. Highlight to SEN any child with GMFCS III-V in terms of manual handling needs. Demonstration of activities for parents to implement with their child and provision of home activity programme Advice provided to nursery/school on activities to be incorporated into play / physical exercise sessions to maximise gains of therapy Refer to wheelchair clinic for buggy/wheelchair if needed Language & Communication Communication based sessions focused on both verbal and nonverbal systems as needed. This would involve consideration of type of CP/ bulbar involvement and impact on saliva control, speech production e.g. dysarthria/dyspraxia Consider CFCS (or equivalent) for augmented and alternative communication (AAC) Feeding Close liaison with MDT/ including dietetics re: hydration and nutrition Management of oral and non-oral feeding needs considered dependent on child s needs Developmental feeding needs closely monitored throughout e.g. transition to solids, independent feeding skills Consider assessing dysphagia score to evaluate risk of associated negative health consequences SLT SLT Education Discuss nursery and education options and needs with parents Advise family to apply for nursery place of choice Referral to EYIT from setting if not already done Information to parents about statementing process Referral to education professionals (SEN section and/or EPS) if not already done Consider initiation of statementing process Ongoing facilitation of support in nursery as necessary EYIT Any professional (Children s Centre services may be able to give an overview if required) 10

11 3 years Family Support Referral to Disabled Children s Team (DCT) should be offered On-going support for family and ensure referrals to relevant services have been made, depending on needs of the family Continence support and referral for supply of nappies All children become eligible for funded nursery education placement between the age of 3-4 years: explore this with the family if not previously done Physical Health Reconsider GMFCS classification Growth & nutrition (weight, height/knee height, BMI): plot on appropriate growth chart Review immunisation status Assessment of hips and spine: hip surveillance X-ray may be required Consider blood test screening for osteopaenia Consider management of spasticity as appropriate Review cardiac and respiratory function Gastrointestinal review including reflux, constipation and drooling Consider need for Ophthalmology and Audiology services Developmental status: including learning ability Review behaviour & emotional well being Screen for sleep disorder Consider referral for dental review Consider end of life plans with family if appropriate Offer referral to CAMHS team if appropriate With consent, copy all correspondence to the SEN Section at Crowndale Development, Motor Skills & Play If appropriate offer 3 year Bayley Scales of Infant and Toddler Development assessment and offer Anticipatory Guidance. OT input according to level I, II or III package of care based on assessment. Evaluate intervention using GAS scores and review appropriate package of care. Home Visit if required to look at equipment Needs Nursery visit to provide OT Programme and advise on equipment needs Refer to Upper Limb Clinic if appropriate Request Assistive Technology assessment if appropriate. Refer to seating clinic if appropriate. Refer to Social Care OT for housing adaptations / equipment needs if appropriate. Assessment of tone (severity, distribution & dynamic tone using Tardieu scale), posture and pattern of movement Assessment of righting and equilibrium reactions, and protective extension Assessment of range of movement (passive & active) Functional assessment of strength through activities such as sit to stand and ability on stairs Assessment of physical endurance in terms of distance a child is able to walk either outdoors or within nursery PT input according to audit of need score Evaluate intervention using GAS scores 11 HV Paediatrician OT PT

12 Review Risk assessment and Manual handling plan. MACS score Classification of GMFCS Demonstration of activities for parents to implement with their child and provision of home activity programme Refer to Wheelchair clinic for buggy/ wheelchair if needed. OT Language & Communication SLT to work closely with nursery to provide IEPs/staff training and support, and educational placement and environment SLT intervention determined by communication skills/mode (verbal/non-verbal). Consider more focused MDT blocks of intervention Consider CFCS (or equivalent) for augmented and alternative communication (AAC) Feeding Ongoing feeding support if needed Consider assessing dysphagia score to evaluate risk of associated negative health consequences SLT SLT Education Contribute to IEP +/- statutory assessment as needed SLT, PT, OT Contribute to Statutory Assessment and submit Risk Assessment with SA report. Highlight to SEN any child with III-V GMFCS classification in terms of manual handling needs. Provide training for nursery staff if needed. OT & PT Advice provided to nursery/school on activities to be incorporated into play / physical exercise sessions to maximise gains of therapy Share information from EYFS (Early Years Foundation Stage) Nursery assessment with other education and health professionals keyworker Consider referral to Educational Psychologist SENCO SEN section consider request for statutory assessment if appropriate SEN section 4 years Family Support Referral to Disabled Children s Team (DCT) should be offered On-going support for family and ensure referrals to relevant services have been made, depending on needs of the family Physical Health Reconsider GMFCS classification Growth & nutrition (weight, height/knee height, BMI): plot on appropriate growth chart Review immunisation status Assessment of hips and spine: hip surveillance X-ray may be required Consider blood test screening for osteopaenia Consider management of spasticity as appropriate Review cardiac and respiratory function Gastrointestinal review including reflux, constipation and drooling Consider need for Ophthalmology and Audiology services Developmental status: including learning ability Review behaviour & emotional well being Screen for sleep disorder HV Paediatrician 12

13 Consider referral for dental review Consider end of life plans with family if appropriate Offer referral to CAMHS team if appropriate Consider handover to school health service With consent, copy all correspondence to the SEN Section at Crowndale Development, Motor Skills & Play Consider formal assessment using standardised ax e g VMI, Mvt ABC, PEDI Complete MACS classification OT input according to level I, II or III package of care (appendix 2) based on assessment. Evaluate intervention using GAS scores and review appropriate package of care. Advise re home and school equipment if necessary Review Equipment needs at home Refer to Upper Limb Clinic if appropriate Request Assistive Technology assessment if appropriate. Refer to seating clinic if appropriate. Refer to Social Care OT for housing adaptations / equipment needs if appropriate. Annual assessment Hypertonicity Assessment Tool (HAT) Modified Ashworth Scale (MAS) Tardieu Scale PROM/AROM LLs & ULs Consider spasticity vs. contracture Gait analysis Functional LL strength & balance: functional assessment of strength through activities such as sit to stand and ability on stairs; assessment of physical endurance in terms of distance a child is able to walk either outdoors or within nursery Core stability Chailey levels Assess transfers GMFCS PT input according to audit of need score Evaluate intervention using GAS scores Demonstration of activities for parents to implement with their child and provision of home activity programme Joint mobility assessment (home & school) Joint assessment for suitable chair provision if needed Refer to Wheelchair clinic for buggy/ wheelchair if needed. Review Risk assessment and Manual handling plan. Language & Communication Planned programme for school to include training for staff Regular input to IEP and statement Support with transition to school Transfer to school based SLT Consider CFCS for augmented and alternative communication (AAC) OT PT PT & OT SLT Education Parents apply for school reception place via admissions section Parents & SEN 13

14 (if child has statement) Transition protocol to be instituted Nursery keyworker Ensure multi-agency planning at entry for IEP and at review SENCO Input to IEP and advice for SEN, TAs & PE as appropriate: advice provided to nursery/school on activities to be incorporated into play / physical exercise sessions to maximise gains of therapy advice on equipment needs training for staff if needed OT & PT 5-6 years Family Support Referral to Disabled Children s Team (DCT) should be offered On-going support for family and ensure referrals to relevant services have been made, depending on needs of the family Transfer to school nurse Physical Health Annual review: Reconsider GMFCS classification Growth & nutrition (weight, height/knee height, BMI): plot on appropriate growth chart Review immunisation status Assessment of hips and spine: 6-12 monthly hip surveillance X-ray may be required Consider annual blood test screening for osteopaenia Consider management of spasticity as appropriate Review cardiac and respiratory function Gastrointestinal review including reflux, constipation and drooling Consider need for Ophthalmology and Audiology services Developmental status: including learning ability Review behaviour & emotional well being Screen for sleep disorder Consider referral for dental review Consider end of life plans with family if appropriate When appropriate, child should be given the opportunity to discuss their condition: diagnosis, explanation, prognosis and any other questions Offer referral to CAMHS team if appropriate With consent, correspondence to be copied to SEN section at Crowndale and SENCO Development, Motor Skills & Play OT input according to level I, II or III package of care Administer any standardised assessments as appropriate Evaluate intervention using GAS scores and review appropriate package of care. Review Equipment needs at home. Refer to Upper Limb Clinic if appropriate Request Assistive Technology assessment if appropriate. Refer to seating clinic if appropriate. Refer to Social Care OT for housing adaptations / equipment needs if appropriate. Annual assessment Hypertonicity Assessment Tool (HAT) 14 HV Paediatrician / School Paediatrician OT PT

15 Modified Ashworth Scale (MAS) Tardieu Scale PROM/AROM LLs & ULs Consider spasticity vs. contracture Gait analysis Functional LL strength & balance: functional assessment of strength through activities such as sit to stand and ability on stairs; assessment of physical endurance in terms of distance a child is able to walk either outdoors or within nursery Core stability Chailey levels Assess transfers GMFCS PT input according to audit of need score Evaluate intervention using GAS scores Demonstration of activities for parents to implement with their child and provision of home activity programme Joint mobility assessment (home & school) Joint assessment for suitable chair provision if needed Refer to Wheelchair clinic for buggy/ wheelchair if needed. Review Risk assessment and Manual handling plan. PT & OT Language & Communication School based SLT input (in line with child s statement) regardless of placement Consider CFCS (or equivalent) for augmented and alternative communication (AAC) Feeding Ongoing feeding support if needed Consider assessing dysphagia score to evaluate risk of associated negative health consequences Education If statemented, annual review to monitor progress: paediatrician, school nurse, GP & therapists involved to be invited to attend EP to offer ongoing advice, support and training: including advice on managing behaviour problems and sharing best practice Ensure continued multi-agency planning involving parents Input to IEP and advice for SEN, TA s & PE as appropriate: advice provided to nursery/school on activities to be incorporated into play / physical exercise sessions to maximise gains of therapy advise on equipment needs consider manual handling needs at school and advise appropriately. provide training for school staff if needed Discuss with child, family & school re: realistic activity levels, possible modifications and pacing (GMFCS III) SLT SLT Education professionals OT & PT PT 15

16 7-11 years Family Support Referral to Disabled Children s Team (DCT) should be offered Ongoing support for family and ensure referrals to relevant services have been made, depending on needs of the family Physical Health Annual review: Reconsider GMFCS classification Growth & nutrition (weight, height/knee height, BMI): plot on appropriate growth chart Review immunisation status Assessment of hips and spine: 12 monthly hip surveillance X-ray may be required Consider annual blood test screening for osteopaenia Consider management of spasticity as appropriate Review cardiac and respiratory function Gastrointestinal review including reflux, constipation and drooling Consider need for Ophthalmology and Audiology services Developmental status: including learning ability Review behaviour & emotional well being Screen for sleep disorder Consider referral for dental review (see referral form: Camden and Islington Primary Care Trusts Community Dental Service) Consider end of life plans with family if appropriate When appropriate, child should be given the opportunity to discuss their condition: diagnosis, explanation, prognosis and any other questions Offer referral to CAMHS team if appropriate With consent, correspondence to be copied to SEN section at Crowndale and SENCO Development, Motor Skills & School Work OT input according to level I, II or III package of care (Annual assessment at IEP / Annual Review will influence package of care) Administer any standardised assessments as appropriate Evaluate intervention using GAS scores and review appropriate package of care. Review Equipment needs at home. Refer to Lycra Clinic if appropriate Refer to Upper Limb Clinic if appropriate Request Assistive Technology assessment if appropriate. Refer to seating clinic if appropriate. Refer to Social Care OT for housing adaptations / equipment needs if appropriate. Annual assessment Hypertonicity Assessment Tool (HAT) Modified Ashworth Scale (MAS) Tardieu Scale PROM/AROM LLs & ULs Consider spasticity vs. contracture Gait analysis Functional LL strength & balance: including mobility on different terrains and walking endurance (where appropriate) Core stability?chailey levels Assess transfers GMFCS 16?Named professional Paediatrician / School Paediatrician OT PT

17 Consider implications of growth on ROM, strength & control PT input according to audit of need score Evaluate intervention using GAS scores Joint mobility assessment (home & school) Joint assessment for suitable chair provision if needed Refer to Wheelchair clinic for buggy/ wheelchair if needed Review Risk assessment and Manual handling plan PT & OT Language & Communication School based SLT input (in line with child s statement) regardless of placement Consider CFCS (or equivalent) for augmented and alternative communication (AAC) Feeding Ongoing feeding support if needed Consider assessing dysphagia score to evaluate risk of associated negative health consequences Education If statemented, annual review to monitor progress: paediatrician, school nurse, GP & therapists involved to be invited to attend EP to offer ongoing advice, support and training: including advice on managing behaviour problems and sharing best practice Ensure continued multi-agency planning involving parents Advise on equipment needs at school. Consider manual handling needs at school and advise appropriately Provide training for school staff if needed. Input to IEP and advice for SEN, TA s & PE as appropriate: discuss with school staff re: mobility around school and changes between class rooms (consider transporting books and equipment) Determine participation in extra-curricular physical activities: encourage active lifestyle and promote PE & clubs where able gradual move from school based therapy to after school activities including peers with similar capabilities (PT groups) SLT SLT Education Professionals OT & PT years Family Support Referral to Disabled Children s Team (DCT) should be offered Ongoing support for family and ensure referrals to relevant services have been made, depending on needs of the family Transfer to secondary school nurse Physical Health Annual review: Reconsider GMFCS classification Growth & nutrition (weight, height/knee height, BMI): plot on appropriate growth chart Review immunisation status Assessment of hips and spine Consider annual blood test screening for osteopaenia Consider management of spasticity as appropriate Review cardiac and respiratory function Gastrointestinal review including reflux, constipation and drooling Consider need for Ophthalmology and Audiology services Developmental status: including learning ability Review behaviour & emotional well being 17 Named professional Paediatrician / School Paediatrician

18 Screen for sleep disorder Consider referral for dental review Consider end of life plans with family if appropriate When appropriate, child should be given the opportunity to discuss their condition: diagnosis, explanation, prognosis and any other questions Offer referral to CAMHS team if appropriate With consent, correspondence to be copied to SEN section at Crowndale and SENCO Development, Motor Skills and School Work OT input according to level I, II or III package of care (Annual assessment at IEP / Annual Review will influence package of care) Administer any standardised assessments as appropriate Evaluate intervention using GAS scores and review appropriate package of care. Review Equipment needs at home. Refer to Lycra Clinic if appropriate Refer to Upper Limb Clinic if appropriate Request Assistive Technology assessment if appropriate. Refer to seating clinic if appropriate. Refer to Social Care OT for housing adaptations / equipment needs if appropriate. Annual assessment Hypertonicity Assessment Tool (HAT) Modified Ashworth Scale (MAS) Tardieu Scale PROM/AROM LLs & ULs Consider spasticity vs. contracture Gait analysis Functional LL strength & balance: including mobility on different terrains and walking endurance (where appropriate) Core stability Chailey levels Assess transfers GMFCS Consider implications of growth on ROM, strength & control Determine participation in extra-curricular physical activities: encourage active lifestyle and promote PE & clubs where able PT input according to audit of need score Evaluate intervention using GAS scores Joint mobility assessment (home & school) Joint assessment for suitable chair provision if needed Refer to Wheelchair clinic for buggy/ wheelchair if needed. Language & Communication School based SLT input (in line with child s statement) regardless of placement Consider CFCS (or equivalent) for augmented and alternative communication (AAC) Feeding Ongoing feeding support if needed Consider assessing dysphagia score to evaluate risk of associated negative health consequences OT PT PT & OT SLT SLT 18

19 Education If statemented, annual review to monitor progress: paediatrician, school nurse, GP & therapists involved to be invited to attend Year 9: transition-focussed annual review to take place EP to offer ongoing advice, support and training: including advice on managing behaviour problems and sharing best practice Ensure continued multi-agency planning involving parents Advise on equipment needs at school. Consider manual handling needs at school and advise appropriately. Provide training for school staff if needed. Facilitate smooth transfer from primary school to secondary school Input to IEP and advice for SEN, TA s & PE as appropriate: discuss with school staff re: mobility around school and changes between class rooms (consider transporting books and equipment) Support PE department to include children in mainstream exercise Goal directed exercise programmes (with education) Education Professionals OT & PT >18 years Transition to adult services see transition pathways 19

20 Appendix 1: Physiotherapy Input according to GMFCS level Age GMFCS I GMFCS II GMFCS III GMFCS IV & V Birth 2 years Family centred approach with SMART goals set in liaison with parents Neurodevelopmental therapy Aquatic therapy 2-4 years Neurodevelopmental therapy Strengthening through functional activities Aquatic therapy 4-6 years Orthotics Lycra Serial casting Botox Strength training Groups Trampolining 7-11 years Orthotics Botox Serial casting Lycra Groups Trampolining years Orthotics Botox Serial casting Lycra Family centred approach with SMART goals set in liaison with parents Neurodevelopmental therapy Aquatic therapy Neurodevelopmental therapy Strengthening through functional activities Aquatic therapy Mobility aids Orthotics Night time splinting Lycra Botox Gait aids Orthotics Lycra Serial casting Botox Strength training Groups Trampolining Orthotics Botox Serial casting Lycra Groups Trampolining Gait aids Orthotics Botox Serial casting Lycra Gait aids 20 Family centred approach with SMART goals set in liaison with parents 24 hour postural management Neurodevelopmental therapy Aquatic therapy Neurodevelopmental therapy Postural management Orthotics Lycra Aquatic therapy Botox Mobility aids Neurodevelopmental therapy Orthotics Lycra Aquatic therapy Botox Strength training Groups Serial casting Rebound therapy Mobility aids Orthotics Lycra Strength training Groups Botox Serial casting Rebound therapy Activity modification Walking aids & mobility devices Orthotics Lycra Botox Serial casting Activity modification Mobility devices Family centred approach with SMART goals set in liaison with parents 24 hour postural management Neurodevelopmental therapy Aquatic therapy Neurodevelopmental therapy Postural management Orthotics Lycra Aquatic therapy Botox Mobility aids Standing frame & seating Neurodevelopmental therapy Orthotics Lycra Aquatic therapy Botox Mobility aids Gaitors Postural management Wheelchair assessment Manual handling advice Strengthening programme Strengthening Orthotics Equipment monitoring Strengthening Equipment Orthotics

21 Aquatic Therapy Aims& Summary of Evidence Evidence suggest that may help with development of increased freedom of movement (Kelly& Darrah, 2005) and increased functional mobility (MacManus & Kotelchuck, 2007) Group sessions provide a medium for parents to interact with other families May have positive impact on quality of life Botox (Botulinum Toxin) Summary of Evidence Evidence supports long term benefits and convenience for families (Flett et al, 1999 & Houltram et al, 2001) Aims (APCP, 2008): Improve functional walking Improve appearance of walking Improve gross motor skills including standing, transfers and sitting ability Reduced short term risk of musculoskeletal deformity and delay surgical intervention Improve tolerance of casting or orthoses Reduce painful spasms Improve sleep pattern May improve longitudinal growth Recommendations by GMFCS GMFCS I & II: indicated for the treatment of focal spasticity and correction of dynamic equinus gait GMFCS IV & V: consider for hip adductor spasm, to improve pain and reduce chance of hip subluxation Comments Need to consider appropriate window for treatment, including considering growth periods and physiotherapy provision post treatment Botox should be used in conjunction with other treatment modalities including physiotherapy, orthotics and orthopaedic surgery (Cosgrove, 1997) Intensive physiotherapy is required post botox (Mulligan, 2006) Gait & Mobility aids Recommendations by GMFCS GMFCS II: consider most appropriate type and use for both indoor and outdoor use, e.g. tripod sticks, anterior vs. posterior walker (Logan et al, 1990); older children may require a wheelchair for outdoor use GMFCS III: provision of hand-held mobility device; educate on used and monitor usage and condition; discussion with wheelchair clinic / OT / borough / charities regarding provision of appropriate wheelchair for outdoor use GMFCS IV: provision of walking aids for use at home or short distances within school; liaison with OT for referral to wheelchair services for provision of wheelchair GMFCS V: provision of gaitors for maintaining hamstring length in standing; liaison with OT for referral to wheelchair services for provision of wheelchair Groups: Summary of Evidence Evidence supports short term functional gain and longer term improvement in motivation and participation in training programmes (Crompton et al, 2007) Intensive programmes using goal-directed activity-focused methods may improve motor abilities and self-care skills (Sorsdahl et al, 2010) Indications Post botox for functional strengthening Motivation for exercise compliance Prepare for or as an adjunct to PE classes Aims to improve: Motor planning Coordination Strength Balance Stamina & endurance Motivation 21 Function Comments Move to community activities from age 6 years

22 Lycra: Summary of Evidence Evidence supports functional improvements (particularly as a result of improved proximal stability) but discomfort (including toileting difficulties) may be a barrier to sustained use (Rennie, 2000, Nicholson, 2001, Knox, 2003, Coghill & Simkiss, 2010) Recommendations by GMFCS GMFCS I & II: children with poor core stability,?dystonia > spasticity on HAT GMFCS III, IV & V: children with poor proximal and / or poor distal stability in the lower extremities Neurodevelopmental therapy Summary of Evidence Results in gains in motor skills and self-care (Knox & Lloyd-Evans, 2002) Evidence suggests that benefits are increased with higher intensity of therapy (Tsorlakis et al, 2004) Majority of studies are on children over the age of 2 years Orthotics: Recommendations by GMFCS GMFCS I & II: Decision based on gait analysis (especially shank angle in stance phase), gastrocnemius length, spasticity and lower limb range of movement (Owen, 2005) GMFCS III: decision based on gait analysis and degree of hip and knee flexion contracture contractures of 15 degrees limit the efficacy of ankle foot orthosis (AFO) to control knee extension in midstance and are therefore a contraindication to the prescription of this orthosis (Rogozinski Benjamin et al, 2009); hinged AFOs can be used in pre-ambulatory children with spastic diplegia and uncontrolled dynamic equinus to reduce time taken for transitions, e.g. sit to stand (Wilson et al, 1997) GMFCS III, IV & V: use of orthoses for improved alignment or limb position (including night-time splitting) may prevent undesirable structural changes and delay the need for surgery (Baumann & Zumstein, 1985; Evans et al, 1994) Comments May be less need for orthotics as growing slows or ceases Postural Management Recommendations by age & GMFCS Introduction of 24 hour postural management at an age appropriate to normal development Night-time: symmetrical lying position Standing: in standing frame at 1 year of age (for functional and social benefits, and to maintain bone density) For children in nursery: standing frame and seat will be required with demonstration to staff of positioning and transfer in and out of equipment Seating: to be provided in liaison with Occupational Therapy GMFCS V: children will need to be hoisted in and out of equipment, and manual handling assessment and advice should be provided to parents and nursery staff Rebound Therapy Summary of evidence Claims are as yet anecdotal. Aims Thought to improve cardio-respiratory fitness, reduce hypertonia, improve posture, improve balance reactions and improve kinaesthetic awareness. Serial Casting: Summary of evidence Inconsistent evidence that casting leads to a sustained improvement in muscle length, gait or function (Blackmore et al, 2006, Gough, 2007 & NcNee, 2007) There is no evidence that combining casting and botox is superior to either intervention alone (Blackmore et al, 2006) One study suggests that serial casting is preferable to botox for the treatment of fixed equinus contractures in children with cerebral palsy (Kay et al, 2004) Aims (GMFCS I) Increasing range of movement at the ankle Encourage heel strike & maintain hamstring length 22 Improve tolerance of orthotics

23 Strength Training: Summary of evidence Evidence that training can improve functional strength in children and young adults with CP (Dodd, 2002 & Blundell, 2003) There is some evidence that strengthening programmes are effective in improving gait function (Lee, 2008) No evidence as to whether or not participation in normal societal roles is improved by this Strength training is now an accepted therapeutic approach in these patients (Darrah et al, 1997 & Haney, 1998) No evidence that increased spasticity is an associated risk (Dodd, 2002) Trampolining: Summary of Evidence No published evidence of benefits for children with mild physical needs Indications Post botox Intensive active stretching Aims Exercises for: o Strength & power o Balance o Motor planning o Control & coordination Comments Move to trampolining in the community (Talacre) from age 6 years, if desired 23

24 Appendix 2: Occupational Therapy Packages of Care To determine the appropriate package of care, the following factors are considered in conjunction with the outcome from standardised and non-standardised assessments: The functional need of the child based on assessment scores Medical and social issues The child s needs / views / perceptions Parental needs and views Needs identified by School and other professionals. OT needs on a statement are statutory requirements. Timing of intervention Age of the child and previous input All pupils on the OT caseload are offered as a minimum, Level 1 Occupational Therapy package of care. Mosaic (preschool) Level 1 Package Enhanced Level 1 Package Level 2 Package: Level 3 Package: 1. Initial OT assessment as part This package is offered to children who of the multi-disciplinary need individually tailored early assessment, to include intervention to be delivered on a regular functional assessment in the basis. This involves demonstrating following areas: handling techniques to parents and Motor abilities both fine and providing advice. gross motor skills ADL (activities of daily living) as appropriate for developmental level Play Sensory assessments 2. Contribute to CPM report following assessment 3. Attend pre-cpm and CPM to liaise with rest of multidisciplinary team 4. Joint target setting with MDT / teacher / SENCO for child s IEP (Individual Education Plan) 5. Contribute to Statutory Assessment 6. Ongoing review of targets 7. Provision of equipment at Level 1 package plus: Monthly sessions with the OT to review progress, update programmes and provide support and advice to carers/ nursery staff. Individually tailored OT programme to be delivered by carers / nursery staff and carried out on a daily basis at home / nursery. Individualised training to be provided for carers / nursery staff in order to deliver the programme. Liaison with other professionals. This package is offered to children who need more intensive individually tailored early intervention. Level 1 package plus: Twice monthly individual therapy delivered by the occupational therapist in the clinic, home or nursery setting. Individual therapy sessions will normally last around minutes, depending on the child s level of tolerance. Individually tailored OT programme to be delivered by carers / nursery staff and carried out on a daily basis at home / nursery. It is envisaged that this package will be delivered in the following way: Therapist provides intervention Therapist provide intervention with carer / staff member observing Therapist and carer / staff 24

25 home / school as needed. 8. Risk Assessment and Manual Handling Plan 9. Annual written review and contribution to AR (Annual Review) once child has a statement. (Report from above assessment may be used as appropriate) 10. Attendance at AR if appropriate 11. Liaise with carers 12. Liaise with other professionals 13. Provision of OT nursery programme if appropriate 14. Provision of OT home programme if appropriate 15. Written administration and note-keeping 16. Nursery training programme member provide intervention together Therapist supervises member of class team to provide intervention Liaison with other professionals Mainstream Schools OT assessment to include functional assessment in the following areas: Gross and Fine motor and handwriting ADL (activities of daily living) to include dressing, undressing for PE, toileting and cutlery for feeding Sensory assessments Written report following assessment with recommendations and where appropriate and no further intervention needed the child will be discharged 1. Joint target setting with teacher / SENCO for pupil s IEP (Individual Education Plan) as appropriate. 2. Ongoing review of targets as needed. 3. Annual written review and contribution to AR (Annual Review). (Report from above assessment may be used if appropriate) 4. Attendance at AR if appropriate 5. Liaison time made available for carers 6. Liaison time made available for professionals 7. Provision of OT school programme if appropriate 8. Provision of OT home programme if appropriate 25 This package is offered to pupils who need individually tailored intervention in order to develop the skills required in order to access learning opportunities within the classroom. Enhanced Level 1 package plus: Individually tailored OT programme to be delivered by TA OT programme to include activities to be carried out on a daily basis within the classroom setting. OT programme to be reviewed on a termly basis and updated as necessary or pupil moved to level 1 package. Individualised training to be provided for the class team in order to deliver the programme. Liaison / training time made available for class team. This package is offered to pupils who need individually tailored intervention in order to develop the skills required to access learning opportunities within the classroom. Enhanced Level 1 package and usually Level 2 package, plus: 1-2 half-termly blocks of once weekly individual therapy delivered by the occupational therapist in the classroom or school setting. Individual therapy sessions will normally last around minutes. Half-termly blocks usually are comprised of up to 6 x 1 weekly sessions. It is envisaged that this package will be delivered in the following way:

26 9. Written administration and note-keeping 10. School training programme When numbers 1-10 above are no longer needed the pupil will be discharged. Therapist provides intervention Therapist provides intervention with member of staff observing Therapist and member of class team provide intervention together Therapist supervises member of class team to provide intervention When the above intensity is no longer needed pupil may be moved to Level 1 or 2 package or discharged. Resource Base Kentish Town & Beckford Schools As for mainstream schools plus: 1. Initial OT assessment to include need for specialist equipment such as seating/hoisting/toilet equipment/switches etc) 2. Written report following assessment 3. Ordering of any specialist equipment necessary 4. Monitoring and review of special equipment 5. Home visit if needed 6. Joint target setting with teacher / SENCO for pupil s IEP (Individual Education Plan) as appropriate. 7. Ongoing review of targets as needed. 8. Annual written review and contribution to AR (Annual Review). (Report from above assessment may be used if appropriate) 9. Attendance at AR if appropriate 10. Liaison time made available for carers 11. Liaison time made available for 26 As for mainstream schools As for mainstream schools

27 professionals 12. Provision of OT school programme if appropriate 13. Provision of OT home programme if appropriate 14. Written administration and note-keeping 15. School training programme If the above OT interventions are no longer needed the pupil will be discharged. 27

28 Appendix 3: Standardised and Non-Standardised Assessments administered by Occupational Therapists General Movements - Prechtl Brazelton Bayley Scales of Infant and Toddler development, 3 rd Edition Assisting Hand Assessment School AMPS Bruininks-Oseretsky Test of Motor Proficiency (Robert H. Bruininks) Movement Assessment Battery for Children Second Edition (Henderson and Sugden) Peabody Developmental Motor Scales Erhardt Developmental Prehension Test Clinical Observations (J.Ayres) Sensory Integration and Praxis Tests Miller Function and Participation Scales (M-FUN) Motor-Free Visual Perception Test Third Edition (MVPT-3) (Colarusso and Hammill) Test of Visual-Perceptual Skills-Revised (Gardner) Test of Auditory-Perceptual Skills-Third Edition (Martin & Brownell) Developmental Test of Visual Motor Integration (Beery and Buktenica) Detailed Assessment of Speed of Handwriting (DASH) Evaluation Tool of Children s Handwriting (ETCH) Draw-a-Person Test (J.Naglieri) Goodenough Draw a Person Informal handwriting and cutting observations Sensory Profile Infant and Toddler (W. Dunn) Sensory Profile Sensory Profile School Companion Short Sensory Profile Adolescent / adult Sensory Profile Sensory Processing Measure (Parham) Pediatric Evaluation of Disability Inventory (PEDI) School Function Assessment Perceived Efficacy and Goal Setting System (PEGS) Clinical Observation of Motor and Postural Skills (COMPS) Ravens Matrices Connors Third Edition Parental Interview and Questionnaire Teacher Questionnaire 28

29 Appendix 4: Gross Motor Function Classification System (GMFCS) (adapted from Palisano et al, 2007) The Gross Motor Function Classification System (GMFCS) for cerebral palsy is a five-level classification system, where distinctions between levels are based on functional limitations (Palisano et al, 1997). GMFCS has been shown to have implications in terms of risk of orthopaedic complications developing (Soo et al, 2006) as well as prognosis for gross motor function (Rosenbaum et al, 2002). Classification of motor function is dependent on age, especially during infancy and early childhood, therefore, separate descriptions exist for children in several age bands. LEVEL I - Walks without Limitations LEVEL II - Walks with Limitations LEVEL III - Walks Using a Hand-Held Mobility Device LEVEL IV - Self-Mobility with Limitations; May Use Powered Mobility LEVEL V - Transported in a Manual Wheelchair Before 2nd Birthday LEVEL I: Infants move in and out of sitting and floor sit with both hands free to manipulate objects. Infants crawl on hands and knees, pull to stand and take steps holding on to furniture. Infants walk between 18 months and 2 years of age without the need for any assistive mobility device. LEVEL II: Infants maintain floor sitting but may need to use their hands for support to maintain balance. Infants creep on their stomach or crawl on hands and knees. Infants may pull to stand and take steps holding on to furniture. LEVEL III: Infants maintain floor sitting when the low back is supported. Infants roll and creep forward on their stomachs. LEVEL IV: Infants have head control but trunk support is required for floor sitting. Infants can roll to supine and may roll to prone. LEVEL V: Physical impairments limit voluntary control of movement. Infants are unable to maintain antigravity head and trunk postures in prone and sitting. Infants require adult assistance to roll. Between 2 nd and 4 th Birthday LEVEL I: Children floor sit with both hands free to manipulate objects. Movements in and out of floor sitting and standing are performed without adult assistance. Children walk as the preferred method of mobility without the need for any assistive mobility device. LEVEL II: Children floor sit but may have difficulty with balance when both hands are free to manipulate objects. Movements in and out of sitting are performed without adult assistance. Children pull to stand on a stable surface. Children crawl on hands and knees with a reciprocal pattern, cruise holding onto furniture and walk using an assistive mobility device as preferred methods of mobility. LEVEL III: Children maintain floor sitting often by "W-sitting" (sitting between flexed and internally rotated hips and knees) and may require adult assistance to assume sitting. Children creep on their stomach or crawl on hands and knees (often without reciprocal leg movements) as their primary methods of self-mobility. Children may pull to stand on a stable surface and cruise short distances. Children may walk short distances indoors using a hand-held mobility device (walker) and adult assistance for steering and turning. LEVEL IV: Children floor sit when placed, but are unable to maintain alignment and balance without use of their hands for support. Children frequently require adaptive equipment for sitting and standing. Self-mobility for short distances (within a room) is achieved through rolling, creeping on stomach, or crawling on hands and knees without reciprocal leg movement. LEVEL V: Physical impairments restrict voluntary control of movement and the ability to maintain antigravity head and trunk postures. All areas of motor function are limited. Functional limitations in sitting and standing are not fully compensated for through the use of adaptive equipment and assistive technology. At Level V, children have no means of independent movement and are transported. Some children achieve self-mobility using a powered wheelchair with extensive adaptations. 29

30 Between 4 th and 6 th Birthday LEVEL I: Children get into and out of, and sit in, a chair without the need for hand support. Children move from the floor and from chair sitting to standing without the need for objects for support. Children walk indoors and outdoors, and climb stairs. Emerging ability to run and jump. LEVEL II: Children sit in a chair with both hands free to manipulate objects. Children move from the floor to standing and from chair sitting to standing but often require a stable surface to push or pull up on with their arms. Children walk without the need for a handheld mobility device indoors and for short distances on level surfaces outdoors. Children climb stairs holding onto a railing but are unable to run or jump. LEVEL III: Children sit on a regular chair but may require pelvic or trunk support to maximize hand function. Children move in and out of chair sitting using a stable surface to push on or pull up with their arms. Children walk with a hand-held mobility device on level surfaces and climb stairs with assistance from an adult. Children frequently are transported when traveling for long distances or outdoors on uneven terrain. LEVEL IV: Children sit on a chair but need adaptive seating for trunk control and to maximize hand function. Children move in and out of chair sitting with assistance from an adult or a stable surface to push or pull up on with their arms. Children may at best walk short distances with a walker and adult supervision but have difficulty turning and maintaining balance on uneven surfaces. Children are transported in the community. Children may achieve self-mobility using a powered wheelchair. LEVEL V: Physical impairments restrict voluntary control of movement and the ability to maintain antigravity head and trunk postures. All areas of motor function are limited. Functional limitations in sitting and standing are not fully compensated for through the use of adaptive equipment and assistive technology. At Level V, children have no means of independent movement and are transported. Some children achieve self-mobility using a powered wheelchair with extensive adaptations. 30

31 Appendix 5: Manual Ability Classification System (MACS) Fig 5.1: Supplementary MACS Level Identification Chart (from 31

32 Appendix 6: Assisted Hand Assessment (AHA) (Holmefur et al, 2010) Children with a low 18 month AHA score (raw score range 22-40) rarely perform bimanual play tasks independently at the age of 18 months. This group reaches 90% of their average limit level at about age 7 years. At this age they usually engage in self-initiated holding of objects, although the grip is not always stable. Children with a high 18 month AHA score (raw scores >40) are already performing bimanual tasks at 18 months, although with some difficulty. They typically use their well-functioning hand to pick up objects, which they thereafter grasp with the hemi hand. Children in this group reach 90% of their average limit at about age 3 years. At this age they usually manage bimanual tasks more effectively and use the hemi hand as a fairly useful assisting hand. Note: this pattern is opposite to that described for GMFCS children at level I reach their limit later than children at levels III to V. 32

33 Appendix 7: Audit of Need Scores Score 0-5 for each category (5 is the highest level of need) Only score if therapy intervention can influence outcome 1. Potential for deformity 2. Potential for development (score may depend on factors other than therapy e.g. home/school support) 3. Potential for deterioration (will depend on underlying diagnosis & stage of disease) 4. Expectation for change (e.g. post intervention such as botox) 5. Threat to life Appendix 8: Goal Attainment Scaling (GAS) scores GAS is an individualised measure of change after an intervention has been made GAS involves setting specific goals for a patient and then specifying a corresponding range of outcomes Figure 8.1, GAS 5 point rating scale (McDougall & King, 2007) GAS Score Predicted Attainment -2 Much less than expected outcome -1 Less than expected outcome 0 Expected outcome after intervention +1 Greater than expected outcome +2 Much greater than expected outcome 33

34 Appendix 9: Communication Function Classification System (CFCS) Level Identification Chart (From 34

35 Appendix 10 (from Guide to levels of risk of negative health consequences from dysphagia This guide identifies the factors that increase the risk of negative health consequences arising from a person s dysphagia. The negative health consequences are asphyxiation and/or choking episode, aspiration incidents, dehydration and poor nutritional status. These factors are not related to the severity of the dysphagia itself but to other intrinsic and extrinsic factors which may exacerbate dysphagia risks. These factors affect the predictability of the person s presentation and interact with the dysphagia. High risk and low risk are easier to manage than fluctuating risk i.e. the person s risk varies for any intrinsic or extrinsic factor from meal to meal or day to day. Each factor can increase the risk of all the negative health consequences outlined above. Intrinsic factors Indicators associated with low risk Indicators associated with increasing risk Level of learning disability/cognitive function Alertness and cooperation Person able to understand risks associated with their dysphagia. Person able to understand and implement their management strategies. Person maintains alertness during eating and drinking. Person alert throughout the day. Person shows anticipation of the food and drink presented. Person opens and closes mouth appropriately. Person reliant on others to implement dysphagia management strategies. Person is cognitively dependent on others to eat and drink. Person has reduced ability to focus. Person less alert at different times of day (e.g. early morning and after bathing). Person has reduced response to helper or food and drink. 35 Indicators associated with high risk Person unable to understand risks associated with his/her dysphagia. Person unable to recognise the health and safety aspects of eating and drinking (e.g. volume, temperature, rate of intake and presence of inedibles). Person is sleepy or drowsy and hence less alert. Person is unable to maintain sustained periods of alertness. Person has no anticipatory response to helper or food and drink. Person does not anticipate arrival of bolus.

36 Intrinsic factors Indicators associated with low risk Indicators associated with increasing risk Person does not experience things which may affect level of alertness or cooperation. Person does not consistently open and close their mouth in response to food and drink. Person experiencing things that may affect levels of alertness or cooperation (e.g. medication and ill health). Indicators associated with high risk Person is experiencing many things which affect levels of alertness or cooperation (e.g. medication cocktail and serious ill health). Distractibility Person focusing on helper and/or food and drink. Fatigue Person does not get fatigued during meals and drinks. Person has a regular sleep pattern and is refreshed after sleeping. Person sleeps unaided by medication and/or is not on medications that affects sleep. Person s safe posture is not compromised as they become fatigued. Rapid decline in function (decompensation) due to ill health Person has good general health. Person appears physically unaffected by everyday infections. Person appears cognitively unaffected by everyday infections. Person is occasionally distractible during mealtimes. Person may become fatigued during meals and drinks (e.g. because they tire after physical activity, have sleep apnoea or require postural adjustments during the night which disturbs sleep). Person sleeping during the day. Person requires medication to attain regular sleep pattern. Person s safe posture is compromised as they fatigue. Person has a suppressed immune system. Person has underlying medical problems that may interact with new infections. Person decompensates when experiencing any kind of infection e.g. UTI. Person has reduced ability to 36 Person is highly distracted by environmental sounds or activity. Person visibly and/or rapidly fatigues during course of meal or drink showing signs of inco-ordination which affect eating and drinking. Person falls asleep during oral intake. Person takes medications which increase fatigability and reduce energy levels. Person s safe posture cannot be maintained when they fatigue. Person has seriously compromised immune system. Person decompensates rapidly when experiencing any kind of infection e.g. UTI. Person has extremely reduced or no ability to make decisions (e.g. volume, temperature, rate of intake or presence of inedibles).

37 Intrinsic factors Indicators associated with low risk Indicators associated with increasing risk Seizure activity Oral health problems Underlying respiratory problem Person does not have seizures. Person s seizures are well controlled by medication or very infrequent and easy to recognise. Person has no oral health problems. Person has minor oral health problems which do not impact on eating and drinking. Person has minor oral health problems which are managed well. Person has no underlying respiratory problems. Person s respiratory function is effectively monitored and managed and does not affect eating, drinking or swallowing. Person s underlying respiratory problems is well controlled by medication. Person s respiratory function is make decisions because of decompensation (e.g. volume or rate intake). Person s seizure activity is less controlled or predictable. Person experiences some changes in skills pre or post seizure. Person s seizure activity is affected by other things (e.g. increased temperature or fatigue). Person has occasional and/or low level oral health problems which impact on eating and drinking (e.g. occasional mouth ulcers or mild case of oral thrush). Person has mild or occasional tooth decay, gum disease or toothache which may impact on eating and drinking. Person prone to infections that affects respiratory function. Person finds it difficult to adapt eating and drinking style to compensate for respiratory problems. Person finds it difficult to implement medication regime e.g. inhaler. Person is unable to participate in 37 Indicators associated with high risk Person s seizure activity leads to increased arousal pre or post seizure. Person s seizure activity leads to loss of alertness and inco-ordination. Person s seizure activity is difficult to recognise or is atypical. Person has severe oral health problems which impact on eating and drinking (e.g. many mouth ulcers or severe case of oral thrush). Person has serious and/or frequent tooth decay, gum disease or toothache which does impact on eating and drinking. Person has severely impaired respiratory function (e.g. COPD, rapid respiratory rate, reduced functional reserve or reflux with ascending aspiration). Person unable to adapt eating and drinking style to compensate for respiratory problems (e.g. inspires post swallow due to rapid respiratory rate, swallows during inhalation or incoordination between respiration and deglutition).

38 Intrinsic factors Indicators associated with low risk Indicators associated with increasing risk Postural control Behavioural difficulties being improved via, for example, exercises and postural management. Person has active and effective cough reflex. Person has no postural difficulties. Person can be assisted to achieve and maintain a stable position during and after oral intake. Person can achieve and maintain a stable position during and after oral intake. activities to improve respiratory function. Person has delayed cough reflex and/or less effective cough. Person s postural stability deteriorates during and after eating and drinking (e.g. head tilting forward or backwards or changes to hand to mouth coordination as a result of loss of postural stability). Person needs to reposition self and this is judged to be safe or the person is repositioned and this is judged to be safe. Person requires equipment to achieve and maintain postural stability. Person experiences. Indicators associated with high risk Person s respiratory problems are not improved by medication. Person has respiratory problems which will not or are unlikely to respond to intervention. Person has severely delayed, weak or absent cough reflex. Person unable to achieve and maintain a stable posture aided or unaided during and after eating and drinking. Person requires frequent repositioning, aided or unaided and this affects functioning (e.g. disrupts concentration, increases fatigue or makes respiratory demands). Person has no behavioural Person s behaviour affects the Person s behaviour is incompatible with safe problems. efficiency and safety of their eating and drinking. Person is able to eat and drink eating and drinking (e.g. Person s management strategies are safely with appropriate support. increasing level of agitation or incompatible with safe eating and drinking. wanting to move when eating and drinking). Person s management strategies impact on the safety of eating and drinking. Person does not have any Person is likely to or does Person is likely to or does experience 38

39 Intrinsic factors Indicators associated with low risk Indicators associated with increasing risk Unmanaged pain Mental health problems unmanaged pain. Person s unmanaged pain does not impact on eating and drinking. Person is able to communicate about any pain and this can be managed (e.g. using medication). Person has no mental health problems that impact upon safe eating, drinking and swallowing. Person s mental health problems are well controlled by medication and/or therapy. experience unmanaged pain which may distract them during eating and drinking. Person has limited ability to communicate about pain experienced. Person has mental health problems that are less controlled or predictable which may impact upon safe eating, drinking and swallowing. Person has mental health problems that are exacerbated by other things (e.g. changes to daily routine increasing anxiety, exposure to stressful situations or noise). Indicators associated with high risk unmanaged pain which distracts them during eating and drinking. Person cannot communicate pain experienced. Person has severe and enduring mental health problems which impact upon safe eating, drinking and swallowing. Person has fluctuating mental health problems which can impact upon safe eating, drinking and swallowing (e.g. response to offered support, behaviour around eating and drinking and capacity to make decisions are all likely to be more variable). Medication Person is not on medication. Person is on medications that have no or minimal impact upon their physical, sensory or cognitive functions. Person is on medications with no or minimal side effects that impact on their physical, sensory or cognitive functions. Person s dysphagia medication sensitivities are considered when Person is on medications that may impact on physical, sensory and cognitive functioning (e.g. some anticonvulsants and neuroleptics can cause dyskinesia and some antipsychotics can cause loss of concentration). Person is on medications with side effects which may affect physical, sensory and cognitive 39 Person is on medication the primary action and/or side effects of which cause dysphagia (e.g. dantrolene sodium (muscle relaxant effect)). Person is on medication the primary action and/or side effects of which can suppress the cough or gag (e.g. haloperidol). Person has a history of sensitivity to medications. Person is prescribed medication in an unsafe form (e.g. gelatine capsule).

40 Intrinsic factors Indicators associated with low risk Indicators associated with increasing risk Physical environment selecting the form and administering medications. Person needs no environmental adaptations for safe eating and drinking. Person s environment is appropriate and adapted to their management needs. functioning (e.g. some antipsychotics, antispasmodics and diuretics can cause xerostomia (dry mouth)). Person is taking a number of medication some of which may be the same type (e.g. polypharmacy). Person s environment is temporary (e.g. respite or hospital) and hence not adapted to their specific management needs. Indicators associated with high risk Person s environment is not temporary and is inappropriately or insufficiently adapted to their specific management needs. Social environment Person needs no environmental adaptations for safe eating and drinking. Person s safe eating and drinking is not adversely affected by others in the environment. Person s support needs do not conflict with others in the environment. Person s safe eating and drinking may be adversely affected by others in the environment. Person s support needs and those of others in the environment compete for caregiver time. Person s safety during eating and drinking is seriously compromised by others in the environment. Person has incompatible support needs with others in the environment during meals and drinks. Extrinsic factors Indicators associated with low risk Indicators associated with increasing risk Access to eating and Person needs no specialised equipment. Person s access to equipment is dependent upon carers. Indicators associated with high risk Person can only be safe eating and drinking with specified 40

41 Extrinsic factors Indicators associated with low risk Indicators associated with increasing risk drinking equipment Person has a ready supply of appropriate working equipment for safe eating and drinking. Person can access and recognise his/her own equipment needs. Person can adapt eating and drinking to non-personal adapted equipment (e.g. different sized spoon). Person does not require staff support to eat and drink safely. Staffing level Adequate staff are available to support the person to eat and drink safely at all times. Person has a staff team of less than seven members which is stable and consistent. Additional staff responsibilities do not interfere with safe management. Staff adherence to plan Staff understand and believe the dysphagia management guidelines are appropriate for the person. Staff are fully trained in dysphagia management by experienced staff. 41 Person s equipment function is dependent upon caregivers checking before use (e.g. correct lid on cup for the person). Person has limited ability to adapt beyond own specialised equipment. Person is partially physically dependent on staff to eat and drink safely. Staffing level is insufficient to meet the support needs of all people in a specific setting. Person has a staff team of more than seven members that is stable or a small but unstable team of less than seven. Staff vacancies leading to cover from a variety of carers. Additional staff responsibilities may interfere with safe management. Staff partially believe in and understand dysphagia management and associated guidelines. Staff are trained by more experienced staff only and do not read the management guidelines. Indicators associated with high risk specialised equipment (e.g. slowflow equipment where valve needs to be in place). Person s access to functional specialised equipment is likely to be compromised. Person has no ability to adapt beyond own specialised equipment. Person is totally physically dependent on staff to eat and drink safely. Staffing levels are not sufficient to provide adequate support and monitoring during mealtimes. Person has large and unstable staff team of more than seven members. Use of unmonitored, untrained, unfamiliar agency staff. Additional staff responsibilities interfere with safe management Staff do not acknowledge or believe the person has dysphagia and do not agree with the guidelines. Staff are untrained and have no knowledge of dysphagia management.

42 Extrinsic factors Indicators associated with low risk Indicators associated with increasing risk Family adherence to plan Staff have read and understood the management guidelines and have a thorough knowledge and understanding of implementing the guidelines. Staff follow the dysphagia management guidelines. Staff have a thorough knowledge of the risks associated with dysphagia and non-adherence to management. Staff are able to empathise with and have a positive attitude to people with dysphagia and their health needs. Staff inform relevant people when the person experiences changes which may impact on the safety of their eating and drinking. Family believe in, understand, follow and agree with the dysphagia management guidelines for the person. Families inform relevant people when the person experiences changes which may impact on the safety of their eating and drinking. Time pressures and organisational issues in the family do not impact on safe eating and 42 Staff do not update their knowledge about dysphagia management. Staff forget important aspects of management whilst maintaining other interventions. Staff do not update their knowledge about dysphagia risks. Staff spend little time empathising with the people with dysphagia that they support. Staff intermittently inform relevant people about changes which may impact on safe eating and drinking. Family past experiences, attitudes and beliefs make it difficult for them to accept and implement the changes necessary for safe eating and drinking. Families intermittently inform relevant people about changes which may impact on safe eating and drinking. Time pressure and organisational issues in the family lead to Indicators associated with high risk Staff fail to implement guidelines or implement them inconsistently. Staff are unaware of the health risks associated with nonadherence. Staff have a negative attitude and do not empathise with people with dysphagia. Staff fail to inform relevant people about changes which may impact upon safe eating and drinking. Family member(s) refuse to engage with dysphagia management. Families do not inform relevant people when the person experiences changes which may impact on the safety of their eating and drinking. Time pressure and organisational issues in the family lead to unsafe practices. Person is supported by many

43 Extrinsic factors Indicators associated with low risk Indicators associated with increasing risk drinking (e.g. management is prioritised). Person is supported at mealtimes by only a small number of experienced family carers. reduced and variable safe support. Person is supported by many different family carers at mealtimes. Indicators associated with high risk inexperienced family carers mealtimes. Additional risks Compromised quality of life and loss of personal dignity These risks increase when too little attention is paid to: communication about food and drink; hygiene and personal care needs around eating and drinking; protection of clothing and suitable clothing protection; cultural needs and age in relation to eating and drinking; food and drink preferences, and choices around eating and drinking; personality and history in relation to food and drink; the way the person is assisted; eating and drinking in an appropriate place; experiences and feelings about dysphagia. Situations associated with low risk Carers take into account the above factors associated with quality of life and dignity when supporting the person during meals and drinks. Situations associated with increasing risk Carers find it difficult consider these factors when supporting the person during meals and drinks (e.g. due to competing time demands and insufficient personal information available). Situations associated with high risk Carers do not consider the above factors when supporting the person during meals and drinks. 43

44 Injury and discomfort Risk of injury may increase due to: actions of the person s carer (e.g. wiped excess saliva rather than dabbing causing sore chin); utensils they have to use (large metal spoon); food and drink (hot food, hard food, food with sharp edges, bones). Risk of discomfort may increase due to: the position the person is in; food, drink or drool around the mouth, chin or neck; the speed at which food or drink is given; oral health problems; debris in the mouth; food or drink temperature engaging in physical activity too soon after eating or drinking. Situations associated with low Risk Carers take into account the above factors when supporting the person during meals and drinks. Situations associated with increasing risk Carers find it difficult to consider these factors when supporting the person during meals and drinks. Situations associated with high risk Carers do not consider the above factors when supporting the person during meals and drinks. 44

45 Proforma for indicating degree of negative health risks for individual clients Negative health consequence: Intrinsic factors Low risk (0) Increasing risk (1) High risk (2) Extrinsic factors Low risk (0) Increasing risk (1) High Risk (2) Level of learning disability/cognitive function Physical environment Alertness/cooperation Social environment Distractibility Access to specialised equipment Fatigue Staffing level Rapid decline in function due to ill health (decompensation) Staff adherence Seizure activity Family adherence Oral health problems Additional risks Underlying respiratory problem Quality of life/loss of dignity Posture control Injury/discomfort Behavioural difficulties Unmanaged pain Mental health problems Medication Totals High risk levels should always be monitored by a specialist dysphagia practitioner. 45

46 Appendix 11: Assessment of the child with suspected Cerebral Palsy Background: Cerebral palsy (CP) is defined as a term which describes a group of disorders of the development of movement and posture, causing activity limitation, that are attributed to nonprogressive disturbances that occurred in the developing fetal or infant brain (Bax et al, 2005). CP affects of every 1000 live born children in the Western world (Stanley, Blair & Alberman, 2000). The incidence is higher in premature infants (Escobar, Littenburg & Petitti, 1991) and twins (Nelson & Elenberg, 1995). Diagnosis is based on history and examination, and laboratory tests are not always necessary to confirm the diagnosis. CP has many different aetiologies including occult antenatal infection and inflammation, perinatal asphyxia, congenital abnormalities of the brain, toxins including bilirubin (kernicterus) and substance misuse, and traumatic brain injury. In some children it is not possible to identify the underlying aetiology. Accurate determination of the diagnosis (in the first instance) and the aetiology of CP has implications for treatment, prognosis, management, recurrence risk, prevention programmes and further research. The risks and benefits of any potential investigations should be fully discussed with parents before a decision is made whether or not to proceed. Aims of diagnostic assessment: 1. Exclude other causes of abnormal motor development and be alert to features which may suggest other causes of disordered motor development (including ataxia). 2. Confirm diagnosis of cerebral palsy and therefore anticipate difficulties and target interventions. 3. Discover aetiology for cerebral palsy which may have implications for the family. Clarity around aetiology may help limit further unnecessary testing. Investigations should be timely in terms of the child s presentation and clinical progression. The wishes of the family and benefits of diagnostic investigation should be balanced against the disadvantages to the child of multiple tests. 4. Classify child according to affected side and motor pattern and identify secondary impairment 5. Give information to the family: a. Diagnosis should be disclosed with the following people present: both parents / caregivers and child, consultant paediatrician, member of the ongoing care team (OT, PT, SLT or HV). b. If there is no doubt, the diagnosis should be communicated to the parents as early as possible. Where there is uncertainty, it should be explained to parents that the condition will be monitored over time and the term evolving motor disorder could be used. c. Communication should be direct, clear, honest, open and sensitive to the needs of the family. Offer to talk to other family members if the parents wish. d. Prognosis: Evidence based prognostication based on the gross motor function classification system (GMFCS) level should be used (see figure 1). MACS & Bayley assessments can give further information. e. Offer support: from other families, groups and web based resources including - i. Scope ( A UK based organisation concerned with the needs of people with cerebral palsy. ii. American Academy for Cerebral Palsy and Developmental Medicine ( The pre-eminent multiprofessional childhood disability organisation in the world. Their website links to resources for parents as well as professionals. iii. CanChild Centre for Childhood Disability Research: iv. Contact a Family: v. Early support booklets 6. Follow-up: 46

47 a. Follow-up appointment (with or without the child present) should be offered within 2 weeks by a member of the multi-disciplinary team b. A letter summarising the discussion should be sent to the family and copied to all involved professionals following the initial assessment and diagnosis 47

48 Flowchart to show assessment process (1): Initial Assessment Child presents with suspected cerebral palsy Initial multi-disciplinary assessment: paediatrician, PT, OT History include: prenatal and birth histories family history developmental history (including any concern about regression) Examination include: neurological examination examination of motor abilities and tone, posture & movements examination of skin facial features (consider dysmorphism) eyes growth (particularly head circumference), and development. Developmental assessment: Consider Bayley assessment Are the history & examination findings suggestive of a motor disorder associated with non-progressive CNS lesion? YES NO Consider alternative diagnoses: see figure 2 Clinical diagnosis of cerebral palsy established Establish GMFCS & MACS classification SEE CEREBRAL PALSY PATHWAY Has the child had previous investigations (imaging +/- other) that confirm the aetiology of CP? No Yes No further investigations at present but child should continue to be monitored clinically Arrange MRI brain & consider MRI Spine (if child having a GA for the procedure) see next page 48

49 Flowchart to show assessment process (2): Investigations Normal Reconsider diagnosis: Could the child have hereditary spastic paraparesis primary dystonia metabolic condition? NO Does the child have any of the following? History suggestive of deterioration or metabolic decompensation Family history of childhood neurologic disorder No aetiology determined by medical evaluation NO YES No further investigations at present but child should continue to be monitored clinically Result of MRI brain +/- spine Abnormal Are the MRI results suggestive of focal infarction or haemorrhage? YES NO YES Are the MRI results suggestive of either? Developmental malformation of the brain An underlying genetic or metabolic disorder YES NO YES Consider metabolic & genetic investigations (see figures 4 & 5) please note a significant number of children with normal MRI may have negative testing (Leonard et al, 2011) Consider screening for coagulation disorder or vascular malformation 49 Does the child have any of the following? History suggestive of deterioration or metabolic decompensation Family history of childhood neurologic disorder NO No further investigations at present but child should continue to be monitored clinically

50 Figure 11.1: Graph to show average development of gross motor ability for each category of GMFCS (level I to IV). Reproduced with kind permission of CanChild and JAMA (copyright). (from 50

51 Figure 11.2: Differential Diagnosis of Chronic Motor Disorders in Children Differential Diagnosis Spasticity Dystonia Ataxia Progressive Injury or neoplasm: brain or spinal Genetic (e.g. hereditary spastic paraplegia, MECP2 duplication, Aicardi-Goutières syndrome) Metabolic (e.g. leukodystrophies, GLUT1 deficiency syndrome, PNP deficiency) Ion channelopathies (e.g. hyperekplexia, hereditary myokymia) Sandifer syndrome Infection (e.g. congential HIV) Metabolic (e.g. glutaric aciduria type 1, mitochondrial disorders) Neurodegenerative Genetic (e.g. MCT8 mutations, Pelizaeus- Merzbacher disease) Dopamine responsive Prader-Willi syndrome Spinocerebellar degeneration (e.g. ataxia telangiectasia) Hereditary spinocerebellar ataxia Metabolic (e.g. SSADH deficiency, hexosaminidase A & B deficiency) Leucodystrophy Lesch-Nyhan Wilsons Arginase deficiency Figure 11.3: MRI patterns in children with cerebral palsy (Bax, Tydeman & Flodmark, 2006) MRI Pattern Number (%) Malformation (including migration defects) 32 (9.1) White-matter damage of immaturity 149 (42.5) Focal infarct 26 (7.4) Cortical / subcortical damage 33 (9.4) Basal ganglia damage 45 (12.8) Miscellaneous 25 (7.1) Normal 41 (11.7) Total 351 (100) 51

52 Figure 11.4: Metabolic investigations to be considered The following tests can be considered based on clinical features: Hypotonia Extrapyramidal signs Cerebellar signs Microcephaly Family history Regression Dysmorphism Organomegaly Eye / skin abnormalities CK Copper Caeruloplasmin Cholesterol Triglycerides Lipoproteins AFP Immunoglobulins TORCH screen Lactate Urate Plasma amino acids Urine amino & organic acids Urine urate/creatinine Maternal PKU Lactate Plasma & urine amino acids Urine organic acids TFTs VLCFAs Lysosomal enzyme analysis The following is a comprehensive list of tests which could be considered: Blood: CK TSH, ft4 Albumin Glucose Lactate Amino acids Pyruvate folate Prolactin Uric acid Vitamin B12, vitamin E Cholesterol, triglycerides, VLCFAs α fetoprotein homocysteine transferrin white cell enzymes biotinidase acylcarnitine mitochondrial mutations Urine: amino acids organic acids purines pyrimidines 3OH glutarate Guanidinoacetic acid CSF studies including: lactate and glucose (paired with plasma) neurotransmitters Neurophysiology: especially EMG & NC studies Skin or muscle biopsy: if considering mitochondrial disease or Niemann Pick C Figure 11.5: Genetic investigations to be considered Genetic testing focuses on the causes for any underlying lesion on MRI (e.g. region of polymicrogyria), any positive family history (or consanguinity) or any dysmorphic features associated with motor signs. Testing should therefore be targeted by these clinical features. Consider chromosomal microarray testing for children with learning difficulties (including autism), dysmorphic features, congenital anomalies and/or dystonia (child will need to be referred to clinical genetics team at GOSH) 52

53 Appendix 12: Cerebral palsy growth charts Growth charts (height, weight and BMI) for boys and girls with cerebral palsy according to five groups of varying motor ability. Reproduced with kind permission from: Figure 12.1, Example growth chart: 53