draft (version 3) - the british academy of childhood disability

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Cerebral Palsies Pathway, Camden Community Child Health Team

Contents

Pages Content

1. 1 Contents

2. 2 Flow chart: overall pathway process

3. 3-19 Pathway mapping by age group:

4. 3 Initial assessment and diagnosis (any age)

5. 3 Birth – 3 months

6. 4 3 months

7. 5 6 months

8. 6 12 months

9. 8 18 months

10. 9 2 years

11. 11 3 years

12. 12 4 years

13. 14 5 – 6 years

14. 16 7 – 11 years

15. 17 11 – 18 years

16. 19 Appendix 1: Physiotherapy Input according to GMFCS level

17. 24 Appendix 2: Occupational Therapy Packages of Care

18. 28 Appendix 3: Standardised and Non-standardised assessments administered by Occupational Therapists

19. 29 Appendix 4:Gross Motor Function Classification System (GMFCS)

20. 31 Appendix 5: Manual Ability Classification System (MACS)

21. 32 Appendix 6: Assisted Hand Assessment (AHA)

22. 33 Appendix 7: Audit of Need Scores

23. 33 Appendix 8: Goal Attainment Scaling (GAS) scores

24. 34 Appendix 9: Communication Function Classification System (CFCS)

25. 35 Appendix 10: Guide to levels of risk of negative health consequences from dysphagia

26. 46 Appendix 11: Assessment of the child with suspected Cerebral Palsy

27. 53 Appendix 12: Cerebral palsy growth charts

28. 54 Appendix 13: Guidelines for Hip Surveillance in Children with Cerebral Palsy

29. 58 Appendix 14: Guidelines for the management of osteopaenia

30. 59 Appendix 15: Management of gastro-oesophageal reflux

31. 61 Appendix 16: Management of constipation

32. 62 Appendix 17: Management of drooling

33. 64 Appendix 18: Medical management of spasticity

34. 70 Appendix 19: Referral to Camden Dental Services

35. 71 Appendix 20: End of life plans

36. 73 References

37. 78 Contributors & Thanks

2

Flowchart to demonstrate overall pathway, Camden Child Development Services:

GP / HV

High risk groups, including perinatal network

Therapy Services

Secondary Care Services

Out of borough

Private healthcare

Referral of child with

suspected CP or evolving motor disorder

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

Normal Development Other developmental disorder

Suspected CP or evolving motor disorder

Discharge Multi-disciplinary / specialist referrals

Follow-up appointment: with or without child present

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

Transition to Adult Services

Referral

Initial

Assessment

Intervention Assessment & Intervention:

Family Support

Development, motor skills &

play

Language & communication

Feeding

Education

Physical Health

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

Paediatrician OT PT

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

Paediatrician OT PT

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

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

HV / SHV

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

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

OT

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


PT

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

Language & Communication

Information from support organisations provided e.g. Bliss guide for feeding premature babies

Awareness of newborn hearing results

SLT

Feeding

MDT liaison re: feeding with acute and community services

Home based support provided as needed

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

HV

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

Paediatrician

5




General advice given to promote all areas of development.

Ensure Early Support materials provided where appropriate.

HV

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

Consider multi-disciplinary assessment with OT






PT




OT & PT


Advice for promoting early language/communication skills

SLT

Feeding

Ongoing support for feeding needs if appropriate

SLT

Education


SEN


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

HV

Physical Health

Growth & nutrition (weight, length, BMI)



Consider blood test screening for osteopaenia









Paediatrician

6




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


OT






PT

Home Visit if required to look at equipment needs




OT & PT


Review of SLT needs as part of developmental review

Sessions/ programme to promote early language/communication skills

SLT

If complex medical needs to be jointly managed by ISDC SLT

ISDC SLT

Education


SEN


EYIT



HV

Physical Health

Consider GMFCS classification

Growth & nutrition (weight, length, BMI): plot on appropriate growth chart


Assessment of hips and spine: initial hip surveillance with X-ray at 12-24 months of age









Consider referral for dental review

Paediatrician

7





Offer 12 month Bayley Scales of Infant and Toddler Development assessment and Anticipatory Guidance.

OT & SLT


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

OT





PT

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.



Consider picture programmes for advice on activities and posturing (GMFCS III, IV & V)

OT & PT


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


SEN


EYIT

8



Parent and toddler groups / playgroups discussed

HV

Physical Health

Reconsider GMFCS classification

Growth & nutrition (weight, length, BMI): plot on appropriate growth chart


Assessment of hips and spine: hip surveillance X-ray may be required













Paediatrician


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 Seating clinic if appropriate


Hemiplegics: Assisting Hand Assessment

OT






PT






Consider picture programmes for advice on activities and posturing (GMFCS III, IV & V)

OT & PT

9


Communication based sessions focused on verbal and non-verbal systems as appropriate

SLT

Feeding



SLT

Education


SEN


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.


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

HV

Physical Health


Growth & nutrition (weight, height/knee height, BMI): plot on appropriate growth chart















Paediatrician


Offer 2 year Bayley Scales of Infant and Toddler Development Assessment and offer Anticipatory Guidance.


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

OT

10





Assessment of tone (severity, distribution & dynamic tone using Tardieu scale), posture and pattern of movement



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

Provide therapy input as part of multi-disciplinary team



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.


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

OT & PT


Communication based sessions focused on both verbal and non-verbal 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)

SLT

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


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

Any professional (Children’s Centre services may be able to give an overview if required)

Ongoing facilitation of support in nursery as necessary EYIT

11



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

HV

Physical Health

















Paediatrician


If appropriate offer 3 year Bayley Scales of Infant and Toddler Development assessment and offer Anticipatory Guidance.



Home Visit if required to look at equipment Needs

Nursery visit to provide OT Programme and advise on equipment needs




Refer to Social Care OT for housing adaptations / equipment needs if appropriate.

OT

Assessment of tone (severity, distribution & dynamic tone using Tardieu scale), posture and pattern of movement



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

12


MACS score




OT


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


SLT

Feeding



SLT

Education

Contribute to IEP +/- statutory assessment as needed

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.

SLT, PT, OT

Provide training for nursery staff if needed.

Advice provided to nursery/school on activities to be incorporated into play / physical exercise sessions to maximise gains of therapy

OT & PT

Share information from EYFS (Early Years Foundation Stage) assessment with other education and health professionals

Nursery keyworker

Consider referral to Educational Psychologist SENCO

SEN section consider request for statutory assessment if appropriate

SEN section




HV

Physical Health













Paediatrician

13




Consider handover to school health service



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.


Advise re home and school equipment if necessary

Review Equipment needs at home





OT

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

Joint mobility assessment (home & school)

Joint assessment for suitable chair provision if needed



PT & OT


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)

SLT

Education

Parents apply for school reception place via admissions section

Parents & SEN

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



Transfer to school nurse

HV

Physical Health Annual review:




Assessment of hips and spine: 6- 12 monthly hip surveillance X-ray may be required

Consider annual blood test screening for osteopaenia










When appropriate, child should be given the opportunity to discuss their condition: diagnosis, explanation, prognosis and any other questions


With consent, correspondence to be copied to SEN section at Crowndale and SENCO

Paediatrician / School Paediatrician



Administer any standardised assessments as appropriate


Review Equipment needs at home.





OT

Annual assessment Hypertonicity Assessment Tool (HAT)

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 & OT


School based SLT input (in line with child’s statement) regardless of placement


SLT

Feeding



SLT

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

Education professionals

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

OT & PT

Discuss with child, family & school re: realistic activity levels, possible modifications and pacing (GMFCS III)

PT

16



Ongoing support for family and ensure referrals to relevant services have been made, depending on needs of the family

?Named professional





Assessment of hips and spine: 12 monthly hip surveillance X-ray may be required









Consider referral for dental review (see referral form: Camden and Islington Primary Care Trusts Community Dental Service)






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)




Refer to Lycra Clinic if appropriate





OT

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

PT

17

Consider implications of growth on ROM, strength & control





Refer to Wheelchair clinic for buggy/ wheelchair if needed

Review Risk assessment and Manual handling plan

PT & OT




SLT

Feeding



SLT

Education




Education Professionals

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)

OT & PT



Ongoing support for family and ensure referrals to relevant services have been made, depending on needs of the family

Transfer to secondary school nurse

Named professional














18







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)




Refer to Lycra Clinic if appropriate





OT

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




PT & OT




SLT

Feeding



SLT

19

Education


Year 9: transition-focussed annual review to take place



Education Professionals

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)

OT & PT

>18 years Transition to adult services – see transition pathways

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

Family centred approach with SMART goals set in liaison with

parents


Aquatic therapy


parents

24 hour postural management


Aquatic therapy


parents

24 hour postural management


Aquatic therapy

2-4 years Neurodevelopmental therapy

Strengthening through functional

activities

Aquatic therapy


Strengthening through functional

activities

Aquatic therapy

Mobility aids

Orthotics

Night time splinting

Lycra

Botox


Postural management

Orthotics

Lycra

Aquatic therapy

Botox

Mobility aids


Postural management

Orthotics

Lycra

Aquatic therapy

Botox

Mobility aids

Standing frame & seating

4-6 years Orthotics

Lycra

Serial casting

Botox

Strength training

Groups

Trampolining

Gait aids

Orthotics

Lycra

Serial casting

Botox

Strength training

Groups

Trampolining


Orthotics

Lycra

Aquatic therapy

Botox

Strength training

Groups

Serial casting

Rebound therapy

Mobility aids


Orthotics

Lycra

Aquatic therapy

Botox

Mobility aids

Gaitors

Postural management

Wheelchair assessment

Manual handling advice

Strengthening programme

7-11 years Orthotics

Botox

Serial casting

Lycra

Groups

Trampolining

Orthotics

Botox

Serial casting

Lycra

Groups

Trampolining

Gait aids

Orthotics

Lycra

Strength training

Groups

Botox

Serial casting

Rebound therapy

Activity modification

Walking aids & mobility devices

Strengthening

Orthotics

Equipment monitoring

11-18 years Orthotics

Botox

Serial casting

Lycra

Orthotics

Botox

Serial casting

Lycra

Gait aids

Orthotics

Lycra

Botox

Serial casting

Activity modification

Mobility devices

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

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

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

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.

Level 1 Package Enhanced Level 1 Package Level 2 Package: Level 3 Package:

Mosaic (pre-school)

1. Initial OT assessment as part of the multi-disciplinary assessment, to include functional assessment in the following areas:

Motor abilities – both fine and 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 multi-disciplinary 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

This package is offered to children who need individually tailored early intervention to be delivered on a regular basis. This involves demonstrating handling techniques to parents and providing advice. 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 40-60 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

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)


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

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 40-50 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)


10. Liaison time made available for carers

11. Liaison time made available for

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.

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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

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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.

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.

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Appendix 5: Manual Ability Classification System (MACS) Fig 5.1: Supplementary MACS Level Identification Chart (from www.macs.nu)

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.

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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

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

(From http://faculty.uca.edu/sharonr/Documents/CFCS_English_2011_09_01.pdf)

35

Appendix 10 (from www.npsa.nhs.uk/EasySiteWeb/GatewayLink.aspx?alId=7034)

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

Indicators associated with high risk

Level of learning

disability/cognitive function

• Person able to understand risks associated with their dysphagia.

• Person able to understand and implement their management strategies.

• Person reliant on others to implement dysphagia management strategies.

• Person is cognitively dependent on others to eat and drink.

• 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).

Alertness and

cooperation

• 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 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.

• 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



• 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).

• 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.

Person is occasionally distractible during mealtimes.

Person is highly distracted by environmental sounds or activity.

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.

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 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.

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 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

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



make decisions because of decompensation (e.g. volume or rate intake).

Seizure activity

Person does not have seizures. Person’s seizures are well

controlled by medication or very infrequent and easy to recognise.

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’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.

Oral health problems

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 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 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.

Underlying respiratory problem

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

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

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



being improved via, for example, exercises and postural management.

Person has active and effective cough reflex.

activities to improve respiratory function.

Person has delayed cough reflex and/or less effective cough.

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.

Postural control

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.

Person’s postural stability deteriorates during and after eating and drinking (e.g. head tilting forward or backwards or changes to hand to mouth co-ordination 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.

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).

Behavioural difficulties

Person has no behavioural problems.

Person is able to eat and drink safely with appropriate support.

Person’s behaviour affects the efficiency and safety of their eating and drinking (e.g. increasing level of agitation or wanting to move when eating and drinking).

Person’s management strategies impact on the safety of eating and drinking.

Person’s behaviour is incompatible with safe eating and drinking.

Person’s management strategies are incompatible with safe eating and drinking.

Person does not have any Person is likely to or does Person is likely to or does experience

39



Unmanaged pain

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).

experience unmanaged pain which may distract them during eating and drinking.

Person has limited ability to communicate about pain experienced.

unmanaged pain which distracts them during eating and drinking.

Person cannot communicate pain experienced.

Mental health problems

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.

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).

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

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



selecting the form and administering medications.

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).

Physical environment

Person needs no environmental adaptations for safe eating and drinking.

Person’s environment is appropriate and adapted to their management needs.

Person’s environment is temporary (e.g. respite or hospital) and hence not adapted to their specific management needs.

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.

Person can only be safe eating and drinking with specified

41



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’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.

specialised equipment (e.g. slow-flow 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.

Staffing level

Person does not require staff support to eat and drink safely.

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.

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.

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 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.

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.

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.

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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.

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.

Staff fail to implement guidelines or implement them inconsistently.

Staff are unaware of the health risks associated with non-adherence.

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 adherence to plan

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

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

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



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.

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

Situations associated with increasing risk

Situations associated with high risk

Carers take into account the above factors associated with quality of life and dignity when supporting the person during meals and drinks.

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).

Carers do not consider the above factors when supporting the person during meals and drinks.

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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

Situations associated with increasing risk

Situations associated with high risk

Carers take into account the above factors when supporting the person during meals and drinks.

Carers find it difficult to consider these factors when supporting the person during meals and drinks.

Carers do not consider the above factors when supporting the person during meals and drinks.

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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.

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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 non-progressive disturbances that occurred in the developing fetal or infant brain” (Bax et al, 2005).

CP affects 2-2.5 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 (www.scope.org.uk)—A UK based organisation concerned with the

needs of people with cerebral palsy. ii. American Academy for Cerebral Palsy and Developmental Medicine

(www.aacpdm.org)—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: www.canchild.ca/en/childrenfamilies/cerebralpalsy.asp

iv. Contact a Family: www.cafamily.org.uk v. Early support booklets

6. Follow-up:

http://www.cafamily.org.uk/

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

48

Flowchart to show assessment process (1): Initial Assessment

Child presents with suspected cerebral palsy

Initial multi-disciplinary assessment: paediatrician, PT, OT

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.

History – include:

prenatal and birth histories

family history

developmental history

(including any concern about regression)

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

49

Flowchart to show assessment process (2): Investigations

Result of MRI brain +/- spine

Normal

Abnormal

No further investigations at present but child

should continue to be monitored

clinically

No further investigations at present but child

should continue to be monitored

clinically

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)

Reconsider diagnosis: Could the child have

hereditary spastic paraparesis

primary dystonia

metabolic condition?

NO

YES

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

Are the MRI results suggestive of focal

infarction or haemorrhage?

NO

YES

Consider screening for coagulation

disorder or vascular

malformation

Are the MRI results suggestive of either?

Developmental malformation of the brain

An underlying genetic or metabolic disorder

YES

NO

Does the child have any of the following?

History suggestive of deterioration or metabolic decompensation

Family history of childhood neurologic disorder

YES

NO

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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 http://motorgrowth.canchild.ca/en/MotorGrowthCurves/resources/MotorGrowthCurves.pdf)

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Figure 11.2: Differential Diagnosis of Chronic Motor Disorders in Children

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)

Differential

Diagnosis

Progressive Dystonia Ataxia Spasticity

Spinocerebellar degeneration (e.g. ataxia telangiectasia)

Hereditary spinocerebellar ataxia

Metabolic (e.g. SSADH deficiency, hexosaminidase A & B deficiency)

Leucodystrophy

Lesch-Nyhan

Wilsons

Arginase deficiency

Metabolic (e.g. glutaric aciduria type 1, mitochondrial disorders)

Neurodegenerative

Genetic (e.g. MCT8 mutations, Pelizaeus-Merzbacher disease)

Dopamine responsive

Prader-Willi syndrome

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)

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Figure 11.4: Metabolic investigations to be considered The following tests can be considered based on clinical features:

The following is a comprehensive list of tests which could be considered: 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)

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

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

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: http://www.lifeexpectancy.org/articles/GrowthCharts.shtml Figure 12.1, Example growth chart:

http://www.lifeexpectancy.org/articles/GrowthCharts.shtml

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Appendix 13: Guidelines for Hip Surveillance in Children with Cerebral Palsy

1. Background:

Migration and deformity of the femoral head are common in children with cerebral palsy; estimated prevalence varies but has been recently estimated as one third (Soo et al, 2006; Hagglund et al, 2005).

The likelihood of displacement is directly related to function as determined by the Gross Motor Function Classification System (GMFCS), see appendix 2 (Soo et al, 2006).

Hip dislocation is associated with functional impairment and with pain, even into adulthood (Boldingh et al, 2005).

Hip asymmetry can lead to pelvic obliquity which is strongly associated with an increased risk of spinal scoliosis (Letts et al, 1984).

Hip subluxation & dislocation often occurs at a young age (Soo et al, 2006).

Evidence suggests that if the femoral head is well located at age 5 years there is less chance that hip instability will develop (Scrutton & Baird, 1997).

2. Why is surveillance needed?

In some centres, it has been shown that referral for surgical intervention occurs too late for soft tissue surgery to be successful (Morton et al, 2006).

Hip surveillance using a combination of clinical and radiological screening, can detect hip disease at an early stage and thus offer early surgical intervention (Dobson et al, 2002).

Using effective screening programmes has been shown to decrease the incidence of hip dislocation

(Hagglund et al, 2005) and the need for reconstructive surgery (Dobson et al,

2002).

3. General Principles: i. Hip surveillance is the process of identifying and monitoring the early indicators of progressive

hip displacement (Wynter et al, 2008). ii. Early identification is an essential part of the strategy for prevention of hip displacement and

its sequelae therefore every child should be referred for hip surveillance at the time cerebral palsy (CP) is identified.

iii. Although the relative risk of hip displacement is directly related to the GMFCS level, hip surveillance is required for every child with CP regardless of gross motor functional ability.

iv. Clinical examination alone is insufficient - all children with bilateral lower limb involvement require an X-ray (Wynter et al, 2008).

v. Children with hemiplegia and WGH IV (see figure 2) gait have the potential for late onset hip dislocation regardless of GMFCS (Wynter et al, 2008). If a child with hemiplegia has hip asymmetry in lying, hip surveillance is required 12 monthly until skeletal maturity.

4. How to conduct surveillance (Wynter et al, 2008)

The commencement of hip surveillance is dependent on corrected age.

The frequency of on-going hip surveillance is determined by radiological measures (see figure 3) including migration percentage (MP), GMFCS level and clinical assessment (see figure 1).

Despite recommendations for frequency increased surveillance will be required for any child in whom there is:

o Reduced range of movement at the hip o Pain referable to the hip o Functional deterioration o Presence of spinal deformity, pelvic obliquity or leg length discrepancy.

5. Prevention and treatment of hip subluxation and dislocation (Pountney & Green, 2006;

Association of Paediatric Chartered Physiotherapists, 2001)

Postural management:

1. For children with GMFCS IV-V Start 24 hour postural management in lying as soon as possible after birth From age 6 months – in sitting From age 12 months – in standing

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2. If MP ≥ 15%: use positioning equipment and refer to orthopaedic team

Weight Bearing Programmes: o Should be started at the usual chronological age of standing of between 12 -15

months o Children should weight bear for 60 minutes 4-5 times per week

Botulinum Toxin: o Can be used to manage hip migration and pain in hip subluxation. o When decisions are made about treatment, take into account clinical status, function,

pain levels, sleep and long term prognosis as well as radiological findings. Orthopaedic intervention:

o MP is unstable (progressing more than 10% per year) and/or progresses to greater than 35%

o Pain referable to the hip o Identification of other orthopaedic conditions. o Orthopaedic treatment may include soft tissue surgery (e.g. adductor releases) or

bone surgery (e.g. femoral osteotomy, pelvic osteotomy or open reduction) the aim of which is to restructure the proximal femoral and acetabular anatomy to maintain the position of the hip (Pountney & Green, 2006).

Table 13.1: Quick reference guide – Management Options Based on Migration Percentage on Hip X-ray (Poutney, T., 2008 & Cliffe, L. et al, 2011)

Migration Percentage Intervention

≥ 15% Postural management +/- botox to facilitate this

≥ 25% Annual spine X-ray to screen for development of scoliosis is required

≥ 35% Referral to orthopaedic team – consider adductor tenotomy

≥ 50% Referral to orthopaedic team – consider femoral osteotomy. If surgery is not appropriate, consider botox

56

Figure 13.1: Table to show strategy for hip surveillance according to GMFCS (adapted from Wynter et al, 2008)

All children: initial assessment at age 12-24 months (or when identified): Clinical assessment, verify GMFCS level & AP X-ray

GMFCS I GMFCS II GMFCS III GMFCS IV † GMFCS V

†

* If GMFCS level changes at any stage, on-going surveillance should be determined by new classification † Independent of MP, if clinical and/or radiographic evidence of scoliosis or pelvic obliquity is present, 6 monthly surveillance is required until skeletal maturity

Age 3y: clinical assessment & verify GMFCS*

Age 5y: clinical assessment &

verify GMFCS*

Discharge if: - remains GMFCS I - no other significant signs

12 months later: clinical assessment & AP X-ray

Age 4-5 years: clinical assessment, verify GMFCS* & AP X-ray

Age 8-10y: clinical assessment, verify GMFCS* & AP X-ray

Review 12 mthly until stabilised

Continue 12 monthly surveillance until MP stabilised

Stable Unstable

MP

Discharge if MP stable

6 months later: clinical assessment, verify GMFCS* & AP X-ray

MP

Stable Unstable

12 mthly surveillance

6 mthly surveillance (until stabilised)

Age 7 years: clinical assessment, verify GMFCS* & AP X-ray

If: - MP stable & <30 % - GM function is stable → discharge until pre-puberty

Pre-puberty until skeletal maturity: 12 mthly X-rays


MP

Stable Unstable

12 mthly surveillance

6 mthly surveillance (until stabilised)


If: - MP stable & < 30% - GM function is stable → discharge until pre-puberty


Continue 6 mthly surveillance


If: - MP stable & <30 % - GM function is stable → 12 monthly surveillance

Discharge at skeletal maturity

Pre-puberty until skeletal maturity: 12 mthly X-rays

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Figure 13.2: Winters, Gage and Hicks Hemiplegia Group IV (WGH IV) - usually clinically apparent by age 4-5 years

Hemiplegia Gait Patterns (from Wynter et al, 2008)

Figure 13.3: Radiological Measures

Migration percentage (MP): is measured by drawing a horizontal line through the most superior medial point of each triradiate cartilage (H-line) and a vertical line (P-line) drawn perpendicular to it at the lateral margin of the acetabulum; then calculated as shown above

A hip is usually considered to be subluxed if the migration percentage is equal to or greater than 33%. Changes in, or stability of MP over time, are more relevant than a single MP measure, hence the recommendation for repeated measures at specific intervals.

58

Appendix 14: Guidelines for the management of osteopaenia (for children at risk due to motor disability aged 0-19 years)

Baseline bloods done at diagnosis of motor disability- Calcium, phosphate, PTH, Alkaline phosphatase, 25-OH Vitamin-D, creatinine.

25-OH Vitamin D <50 nmol/L 25-OH Vitamin D >50 nmol/L

Treatment dose of Vitamin D as Ergocalciferol or Cholecalciferol Age <1yr 1500units a day 1yr-12yrs 3000units a day >12 yrs 6000units a day Treat for 3 months

Recheck level, aim for Vitamin D 100nmol/L (high end of normal range 70-100nmol/L)

Start Vitamin D supplementation 1000units/day (ongoing prophylactic dose)

- Optimise Ca intake with help from dietician

- Promote weight bearing with help from physiotherapists and occupational therapist

Repeat blood tests annually ideally or opportunistically. Monitor for fractures and bone pain

Fracture or bone pain despite adequate supplementation, refer to Calcium clinic at GOSH for further assessment and treatment.

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Appendix 15: Management of gastro-oesophageal reflux History and Examination In most infants and children, history and examination are sufficiently reliable in diagnosing GOR, recognising complications and initiating management. Consider the following:

Feeding history

Pattern of vomiting

Evidence of pain

Growth

Dietary intake

Previous treatment and response to this Investigations If there is diagnostic uncertainty or the child fails to respond to simple measures the child may require further investigation or drug therapy. 1. Combined pH/Impedance 24hrs monitoring Valid and reliable tool in measuring reflux correlation to symptoms. It combines a traditional pH study with Impedance analysis. This test can be performed while patient is on treatment.

Every patient must be reviewed by Paed Gastro Team for test requesting.

Indications

Non GI symptoms of GORD – laryngeal symptoms/ atypical chest pain

Unexplained recurrent pneumonia/ poorly controlled asthma

Suspicion that GORD cause of ALTE (Acute Life Threatening Episode)

Symptomatic on maximum doses of medical treatment

Prior to surgical intervention

To determine if a patient’s symptoms are temporally associated with reflux. 2. Endoscopy and Biopsy Assess presence and severity of oesophagitis, strictures and Barrett’s oesophagus Exclude other disorders such as Crohn’s, webs eosinophilic or infective oesophagitis Indications

Symptomatic on maximal first line medical treatment

Unclear diagnosis

Prior to surgical intervention 3. Video fluoroscopy Episodes of aspiration, assessment of swallow, not for GORD 4. Barium Study If suspicion of anatomical abnormalities such as malrotation, hiatus hernia, oesophageal strictures etc. Not useful in diagnosing GORD (PPV 80 –82%, sensitivity 31 – 86%, specificity 21 -83%) 5. Urine Culture to rule out UTI 6. Blood tests Not indicated for GORD but may be useful to rule out other diagnosis – anaemia, inflammatory markers, specific IgE 7. Other Gastric emptying studies for suspected gastroparesis

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Treatment Medical Treatment Ranitidine Neonates start at 2 mg/kg TDS (max 3mg/kg TDS)

1-6 months 3 mg/kg TDS (max 10mg/kg/day) >6 months 2 - 4mg/kg BD (max 300mg a day)

Doses should be decreased with renal insufficiency Lansoprazole Only for those > 1 year

< 30 kg: 15mg OD. Dose can be increased to 15mg BD > 30 kg: 30mg OD. Dose can be increased to 30mg BD Omeprazole Under 1 year all infants should receive omeprazole suspension/Omeprazole

MUPS All ages: 1 – 3 mg/kg/day

(max 40mg/day, round doses to the nearest tablet/capsule/ml) Domperidone: 200 - 400mcg/kg/day QDS Surgical Treatment Persistent GORD symptoms despite being on maximum doses of medical treatment Nissen Fundoplication – either endoscopically or surgically. (Need to ensure correct diagnosis – endoscopy and pH/impedance prior to surgery)

Algorithm for < 1 year

Posseting/ Mild Reflux

↓ 1. Reassurance/ Simple Measures

Milk Thickening agents Antacids (e.g. Infant Gaviscon)

Formal Dietetic review ↓

Unresponsive to 1 or Symptomatic/ complicated GORD

↓ 2. Trial of Ranitidine ± Domperidone

↓ Unresponsive to treatment (6- 8 weeks)

↓

REFER TO PAED GASTROENTEROLOGIST

Algorithm for > 1 year

Symptoms suggestive of GORD

↓ 1. Trial of Ranitidine ± Domperidone*

↓ Unresponsive to treatment (6- 8 weeks)

2. Lansoprazole (stop Ranitidine) ± Domperidone

↓ No significant resolution of symptoms

↓


Algorithm for > 5 year

Symptoms suggestive of GORD ↓

8 week course of Lansoprazole ± Domperidone ↓

Unresponsive to treatment or symptoms return on stopping treatment

↓


*In patients with severe cerebral palsy with a high reflux index on pH study, start with Lansoprazole and Domperidone.

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Appendix 16: Management of constipation General principles

1. All doses must be tailored to patient response.

Stool softeners must achieve passage of soft stools without straining or pain

Stimulant laxatives must result in increased passage of stool within 24 hours 2. Disimpaction must be assured before maintenance therapy is begun 3. Frequent review after disimpaction to ensure effective maintenance therapy 4. All patients must receive appropriate dietary and behavioural information 5. Simple drug regimens to allow families to manage medication themselves

Disimpaction Algorithm Maintenance Algorithm Aims:

Minimum 2 bowel motions/ week

No soiling

No straining

Pain free passage of stools

Alternatives: Second line stimulant: Docusate Third Line stimulant Sodium Picosulphate Second line softener: Liquid paraffin, Lactulose

Transit studies – if indicated refer to local unit Indications:

1. Following third disimpaction protocol 2. Prior to referral for full-thickness rectal biopsy 3. In cases of doubtful history

Microlax / Fleet enemas for 2-3/7 or until good result

Movicol Paediatric

No clear result within 1-2/52 Clear result achieved within 1-2/52

Maintenance algorithm

OR

Refer to inpatient unit

Movicol Paediatric: 1-4 sachets (or more), dose to be adjusted according to patient’s response Senna: dose per formulary every second day if no bowel motion (BM)

1-2 week clinic review: aim to achieve minimum 2 pain free BM/week and no soiling

2-4 week clinic review: continue Movicol / Senna combination (try to gradually reduce use of stimulant)

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Appendix 17: Management of drooling (Adapted from Fairhurst & Cockerill, 2011) Background:

Drooling beyond the age of 4 years is neurodevelopmentally abnormal

Management of the problem should be multi-disciplinary

Medically, the focus of treatment is to modify the neuroglandular control of saliva with anticholinergic drugs

There is a poor published evidence base for many of the treatments used History:

Oral-motor control

Problems that may exacerbate drooling including posture, medication, dental health, ENT problems, neurological status

Quantify the problem: o How disruptive is the drooling to daily life? o How much is the drooling? Number of bibs used throughout the day can be used as

a rough measure.

Examination:

Observe feeding, drinking & swallowing when the child is at rest and when distracted by a simple task.

Physical examination to include dental health, postural control and neurology of the tongue, cranial nerves, bulbar region, swallowing and respiratory system.

Figure 17.1: Flowchart to demonstrate an approach to management (Reproduced with kind permission from Fairhurst & Cockerill, 2011, page 29):

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Management: 1. Conservative treatment

a. Consider postural control of the head, neck and trunk b. Promote dabbing rather than wiping across the mouth (promotes less salivary gland

stimulation) c. Consider protection of clothing that is age appropriate e.g. sports towelling wrist

bands, bandanas d. Prevent excessive mouthing with simple distraction therapy e. Avoid sweet fizzy drinks and acidic foodstuffs

2. Oral motor programme:

Likely only to be useful in children with mild to moderate oral dysfunction, good cognitive skills and a high level of motivation.

Tongue and mouth exercises as advised by SLT aiming to improve oral-facial tone, increase sensory awareness and develop voluntary control of movement.

3. Intra-oral device: palatal training devices

4. Medical options:

All medicines are used off license and careful discussion about potential side effects must take place between the assessing team, child, families and local prescriber before the initiation of therapy.

Common side effects of anti-cholinergics include: o Blurred vision due to accommodative difficulties o Constipation and urinary retention due to relaxation of bowel smooth muscle and the

detrusor muscle of the bladder o Sedation o Irritability o Headache o Increase in frequency of seizures

Cautions with anti-cholinergics: o Hot weather: as sweating is mediated by the parasympathetic nervous system o Individuals with severe gastrointestinal disorders, including gastro-oesophageal reflux

and/or constipation: a cautious dose implementation regime should be used

Absolute contra-indications for using anti-cholinergics: o Glaucoma o Myasthenia gravis o History of urinary retention.

Glycopyronnium Bromide Benzhexol Hyoscine patches

<15kg start 0.25mg od <25kg start 0.5mg od >25kg start 1mg od

Infant start 0.5mg od Child start 1mg od

<8y start with ½ patch

Increment slowly to twice daily dosing by 2 weeks

Increase 0.5-1mg bd for a week, then

Place patch behind the ear and observe for adverse skin reaction

After a further 1-2 weeks, increase up to tds

Increase 0.5-1mg tds for a week, then

Alternate sites to minimise the risk

Can be further increased slowly to a max of 0.04mg/kg/dose tds

Increase by 0.5-1mg alternate dose each week to initial max of 2mg tds

Typically patch is replaced every 2-3 days

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Appendix 18: Medical Management of Spasticity

1. Background

These guidelines are for use in children and young people up to 19 years of age with spasticity

They cover the use the pharmacological agents

They do not cover the important role of physiotherapy & orthotics (see cerebral palsy pathway) or the use of orthopaedic surgery

They do not cover selective dorsal rhizotomy or deep brain stimulation which are carried out rarely and in highly specialist centres

2. Aims To prevent:

Musculoskeletal complications of spasticity To reduce:

Impairment of motor function

Pain from muscle spasms

3. Principles

Management should be based on individual needs and preferences of young person and their family

4. Outline See figure 1: Medical Management of tone problems 5. Use of drugs See table 1: Summary of Drugs Used in Management of Spasticity Remember: a. All drugs have side effects and a limited evidence base b. Truncal hypotonia can be exacerbated by muscle relaxants and this can be very disabling c. Increased tone can be functionally useful, e.g. in standing and therefore reducing it should be

done cautiously.

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Figure 18.1: (from Fairhurst, 2011)

Selective Dorsal Rhizotomy (SDR)

Usually for children with spasticity at GMFCS level II or worse secondary to periventricular leucomalacia

Aim is to reduce sensory input to the sensory–motor reflex arcs responsible for increased muscle tone

Selected lumbar sensory rootlets are divided, preserving some sensory supply and the motor roots responsible for voluntary movements

Intensive physiotherapy and aftercare is usually given for several months after the procedure

Positive outcomes: ↓ LL spasticity, ↓ orthopaedic procedures, ↑GM function, ↑QoL

Side effects: ↓ GM function, ↓ bladder function, spinal deformity

Deep Brain Stimulation (DBS)

Usually for children with severe dystonic bilateral CP (if all other avenues of treatment have not helped)

Aim is to make aberrant signals from the basal ganglia( more organised)

Electrodes are implanted within the basal ganglia and deliver a continuous electrical signal

Positive outcomes: ↓ dystonia, ↑QoL

Contraindications: Extensive damage of corticospinal tract (consider ITB instead)

Intrathecal Baclofen (ITB)

Usually for bilateral spasticity affecting upper and lower limbs GMFCS level III, IV or V.

If spasticity or dystonia are causing difficulties with any of the following:

1. pain or muscle spasms 2. posture or function 3. self-care (or ease of care by

parents or carers). Contraindications:

1. child is too small to accommodate an infusion pump

2. local or systemic intercurrent infection.

Cautions:

1. co-existing medical conditions (for example, uncontrolled epilepsy or coagulation disorders)

2. previous spinal fusion procedure

3. malnutrition 4. respiratory disorders with a

risk of respiratory failure. 5. muscle weakness

Side Effects: Hypotonia, sleepiness, respiratory depression, negative effect on scoliosis, withdrawal

Referral pathway for ITB / DBS / SDR 1. Initial referral to local motor disorders clinic (Dr Neil

Wimalasundera, GOSH) 2. GOSH team will then refer onto specialist centre if appropriate 3. Specialist centres are as follows:

SDR – GOSH ITB & DBS – Evelina Children’s Hospital

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Table 18.1: Summary of Drugs Used in Management of Spasticity Drug Route of

administration Indications Contraindications Cautions Administration

(also check paediatric BNF)

Side Effects

Further Information

Diazepam Oral Where a rapid effect is required (e.g. a pain crisis) and: 1. Spasticity contributing

to discomfort or pain, 2. Muscle spasms OR 3. Functional disability

secondary to spasticity

1. Respiratory depression 2. Sleep apnoea syndrome

Respiratory disease

As a bedtime dose Start: 0.25mg/kg twice daily Max: infant 2.5mg twice daily child 5mg twice daily >12 yrs 10mg twice daily If response to initial dose is unsatisfactory consider:

increasing the dose

consider a trial of combined treatment with baclofen after 4-6 week

Highly sedating Tolerance easily developed & possibility of BDZ withdrawal symptoms

Consider changing from diazepam to baclofen if long-term treatment is indicated Think about stopping the treatment whenever the child or young person's management programme is reviewed and at least every 6 months. Stopping treatment: if the child has been receiving diazepam for several weeks, reduce the drug in stages to avoid withdrawal symptoms.

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Drug Route of administration

Indications Contraindications Cautions Administration (also check paediatric BNF)

Side Effects

Further Information

Baclofen Oral Where a sustained long-term effect is desired and: 1. Spasticity contributing

to discomfort or pain, 2. Muscle spasms, OR 3. Functional disability

secondary to spasticity

1. Respiratory impairment

2. epilepsy 3. peptic ulcer 4. diabetes 5. hypertonic

bladder sphincter

6. liver or renal impairment

Start: 5mg three times a day (halved in very small children) Increase: step wise over about 4 weeks to achieve optimum therapeutic benefit Max dose: 0.3mg/kg/dy. upper limits up to 5mg/kg/dy If response to initial dose is unsatisfactory consider a trial of combined treatment with diazepam after 4-6 weeks

Truncal hypotonia Sedation Increased seizures

Think about stopping the treatment whenever the child or young person's management programme is reviewed and at least every 6 months. Stopping treatment: if the child has been receiving baclofen for several weeks, reduce the drug in stages to avoid withdrawal symptoms.

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Drug Route of administration

Indications Contraindications Cautions Administration (also check paediatric BNF)

Side Effects

Further Information

Botulinum Toxin A

Intramuscular injection under ultrasound guidance

Focal spasticity of the upper limb that is:

1. impeding fine motor function

2. compromising care and hygiene

3. causing pain 4. impeding

tolerance of other treatments, such as orthoses

5. causing cosmetic concerns

Focal spasticity of the lower limb that is:

1. impeding gross motor function

2. compromising care and hygiene

3. causing pain 4. impeding

tolerance of other treatments, such as orthoses and use of equipment to support posture

5. causing cosmetic concerns

6. disturbing sleep Focal dystonia causing serious problems such as postural or functional difficulties or pain.

1. severe muscle weakness

2. previous adverse reaction or allergy to botulinum toxin type A

3. aminoglycoside treatment

1. Bleeding disorder

2. Generalised spasticity

3. Fixed muscle contractures

4. Marked bony deformities

5. Concerns about child or young person's likelihood of engaging with the post-treatment adapted physical therapy programme

Refer for Baseline assessment of tone, range of movement, motor function & proceed down treatment plan as needed (Referral letter to Dr Dulmini Birkett & Madeleine Szadurski, c/o/RFH)

Cold-like symptoms Swallowing difficulties Breathing difficulties Loss of urinary control (reversible) – only with adductor injections Cardiac arrhythmias

Duration of effect is approx. 3-6 months (or more).

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Appendix 19: Referral to Camden Dental Services

Referrals to Mohita Lall, Kentish Town Health Centre

(Contact details [email protected])

Camden and Islington Primary Care Trusts

Community Dental Service

To: Senior Dental Officer Specialist in Paediatric Dentistry.

From: ………………………………………………………………………………..Date:………………………..

Organisation:………………………………………………………………………………………………………….

Patient’s name: ………………………………………………………………D.O.B………………………

Address and Phone Number:

…………………………………………………………………………………………………………………………………………………

……………………………………………………………………………………………………………………………

………………………………………………………………………………………………………………………………………

Reason for referral:

…………………………………………………………………………………………………………………………………………………

…………………………………………………………………………………………………………………………..

……………………………………………………………………………………………………………………………………..

Social History:

……………………………………………………………………………………………………………………………………..

………………………………………………………………………………………………………………………………………

Medical History:

…………………………………………………………………………………………………………………………………… ..

………………………………………………………………………………………………………………………………………

Other Information:

If interpreter required, please state language …………………………………………..

Will the family travel to Finsbury Health Centre Yes/No

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Appendix 20: End of life plans (Adapted from Wolff, Browne & Whitehouse, 2011).

The Personal Resuscitation Plan (PRP) is an emergency medical care plan which supports the provision of the most appropriate level of intervention for the child whether they are at home, school, short break unit or hospital.

The PRP supports early discussions about the provision of appropriate care.

It is written by the Consultant Paediatrician in conjunction with the family and other key healthcare professionals.

The PRP is a positive approach, emphasising what should be done rather than what not to do.

Once the PRP is completed it should be held by the family with copies in school, short break facility and healthcare facilities.

The PRP is not a legal contract; it is a medical care plan as recommended by the child’s consultant; the parents (or guardian) can change their mind at any time.

The opportunities for introducing the idea of a PRP are varied and include: o When a child has had a first life-threatening illness, e.g. chest infection o When a child starts to be left in the care of others, e.g. nursery or short break facility o If the child’s condition deteriorates o If there is the death of another child in the family

Template for a Personal Resuscitation Plan (PRP) for Child or Young Person available at http://ep.bmj.com/content/suppl/2011/03/01/adc.2010.185272.DC1/EandP_adc_2010_185272

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Contributors: Dr Ellie Day, Paediatric Registrar, Camden Community Child Health Team Dr Dulmini Birkett, Consultant in Paediatric Neurodisability, Camden Community Child Health Team Madeleine Szadurski, Lead Physiotherapist, Camden Community Child Health Team Camden Paediatric Physiotherapy Team Betty Hutchon, Lead Occupational Therapist, Camden Community Child Health Team Natasha Patten, Occupational Therapist, Camden Community Child Health Team Camden Paediatric Occupational Therapy Team Chloe Selby, Speech & Language Therapist, Camden Community Child Health Team Camden Speech & Language Therapy Team Jennifer Fraser, Camden MOSAIC Team Leader Elizabeth Mudiywa, Specialist Health Visitor, Camden Community Child Health Team Early Years Intervention Team, Camden Special Educational Needs Department Samantha Sergeant, Paediatric Dietician, Camden Community Child Health Team Camden Child & Adolescent Mental Health Service Camden Social Care Disabled Children’s Team Chloe Benn, Paediatric Pharmacist, Royal Free London NHS Foundation Trust Paediatric Gastroenterology Team, Royal Free London NHS Foundation Trust With thanks to: Dr Caroline Brain, Consultant Paediatric Endocrinologist, Great Ormond Street Hospital for Children NHS Foundation Trust Dr Lucinda Carr, Consultant Paediatric Neurologist, Great Ormond Street Hospital for Children NHS Foundation Trust Dr Charlie Fairhurst, Consultant in Paediatric Neurodisability, Evelina Children’s Ms Olivia Malaga-Shaw, Consultant Orthopaedic Surgeon, Royal Free London NHS Foundation Trust Prof Peter Rosenbaum, McMaster University and CanChild Centre for Childhood Disability Research Prof David Strauss & Dr Jordan Brooks, Life Expectancy Research Group Dr Georgina Williams, Paediatric SHO, Camden Community Child Health Team Hospital