1. Childhood Disability

2. Aims What is childhood disability? What is childhood disability? Common causes of and disordered development, learning difficulties and disability and how to assess these children Common causes of and disordered development, learning difficulties and disability and how to assess these children Cerebral palsy Cerebral palsy  Causes  Classification  Areas to be assessed at diagnosis

3. What do you understand by: Disorder Disorder Impairment Impairment Disability Disability Handicap Handicap

4. Disorder Medically definable condition or disease Medically definable condition or disease

5. Impairment Loss or abnormality of psychological, physiological or anatomical structure of function Loss or abnormality of psychological, physiological or anatomical structure of function

6. Disability Any restriction of lack (resulting from an impairment) of ability to perform an activity in the manner or within the range considered normal for a child of that age Any restriction of lack (resulting from an impairment) of ability to perform an activity in the manner or within the range considered normal for a child of that age

7. Handicap – social mod The impact of the impairment or disability on the person’s pursuits or achievement of goals that are desired by him/her or expected by him/her by society. The impact of the impairment or disability on the person’s pursuits or achievement of goals that are desired by him/her or expected by him/her by society.

8. But this is now out of date International classification of function - ICF Runs alongside ICD-10 Disease does not predict difficulty In all lives we will at times have difficulties in function Standard language classify function Joins together medical and social model

10. Disorder or disease

12. Body structure and function Structure Structure  Eyes  Ears  Larynx and pharynx  CVS, RS, GIT, GUT  Endocrine, metabolic  Joints and muscles  skin Function  Mental  Sensory and pain  Voice and speech  Digestive, metabolic, endocirine  Neuoromuscular

14. Activities and Participation Communication Communication Mobility Mobility Self care Self care Domestic life Domestic life Interpersonal interactions and relationships Interpersonal interactions and relationships Learning and applying knowledge Learning and applying knowledge Community, social and civic life Community, social and civic life

16. Contextural factors Environment Environment  Natural environment  Man made environment  Products and technology  Support and relationships  Services and policies  Attitudes Personal Factors  Mental health  IQ  Resilience

17. Examples Health Condition Health Condition Impairment Impairment Activity Limitation Activity Limitation Participation restrictions Participation restrictions CP ASC Sensorineural deafness Diabetes mellitus

18. How can we use ICF? Individual Individual Service providers Service providers Society Society Policy development Policy development Economic analysis Economic analysis Research Research

19. Causes of Handicap Disability Incidence (/1000) Physical Cerebral palsy 2.5 Spina bifida 0.3 Muscular dystrophy 0.2 Severe learning difficulties 4.0 Chromosomal abnormalities 4.0 CNS abnormalities 1-2 Special senses Severe visual impairment 0.4 Severe hearing loss 1.0

20. Presentation Antenatal concerns Antenatal concerns At time of birth At time of birth Due to screening programmes Due to screening programmes Family concerns Family concerns After acute severe illness After acute severe illness

21. Delayed development - warning signs At any age At any age  Parental concern  Regression 10 weeks 10 weeks  not smiling

22. 6 months warning signs  Persistent primative reflexes  Persistent squint  Hand preference  Little interest in people, toys, noises

23. 10-12 month warning signs  Not sitting  No double syllable babble  No pincer grasp

24. 18 month warning signs Not walking independently Not walking independently Fewer than 6 words Fewer than 6 words Persistent mouthing / drooling Persistent mouthing / drooling

25. 2 ½ year warning signs No 2-3 word sentences No 2-3 word sentences

26. 4 year old Unintelligible speech

27. What to look for in the history Parental anxieties Parental anxieties Current developmental skills Current developmental skills  Gross motor  Fine motor and vision  Understanding, communication and hearing  Self care and play skills History of developmental skills History of developmental skills Antenatal and birth history including alcohol and drugs Antenatal and birth history including alcohol and drugs Past medical history Past medical history Family history – esp learning difficulties Family history – esp learning difficulties

28. Examination Dysmorphic features Dysmorphic features Growth including head circumference Growth including head circumference General examination General examination Neurological exam including fundi Neurological exam including fundi Current developmental skills Current developmental skills  Gross motor  Fine motor and vision  Understanding, communication and hearing  Self care and play skills

29. What you might find: Downs

30. Foetal alcohol syndrome

31. And many more!

32. Cerebral Palsy 2-2.5 children /1000

33. Aims What is CP What is CP How do you classify CP How do you classify CP What Causes CP What Causes CP Medical approach to a child with possible CP Medical approach to a child with possible CP

34. Cerebral palsy - definition Cerebral palsy (CP) 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. The motor disorders of cerebral palsy are often accompanied by disturbances of sensation, cognition, communication, perception, and/or behaviour, and/or by a seizure disorder.

35. Cerebral palsy classification Motor impairment Motor impairment Functional impairment Functional impairment Associated impairments Associated impairments Anatomical and radiological changes Anatomical and radiological changes Aetiology Aetiology

36. Motor- type Typesigns Hypertonic(85%) Increased muscle tone Spasticity – abnormal response to stretch Brisk reflexes Dyskinetic (7%) Either dystonic or athetiod Dystonic Fluctuating muscle tone, decreased movt Athetoid Increased activity of rapid uncontrolled movements Ataxic (5%) Loss of orderly coordination

37. Motor - function All body areas, including oromotor and speech function All body areas, including oromotor and speech function  Hemiplegia (30%)  unilateral same arm and leg  Diplgeiga (10-30%)  primarily legs  Triplegia – 3 imbs  Quadriplegia (50%)  whole body

38. Severity – GMFCS Gross Motor Function Classification System (for children 6-12 years) I - Walks without difficulty, but difficulties with more advanced tasks I - Walks without difficulty, but difficulties with more advanced tasks II - Walks without devices, limited outdoor mobility II - Walks without devices, limited outdoor mobility III- Walks with devices III- Walks with devices IV – Self mobile with limitation IV – Self mobile with limitation V – Self mobility severely limited even with aids V – Self mobility severely limited even with aids

39. Associated impairments sensory problems (10%) seizures (20%) severe learning difficulties (30%) attentional behavioural behaviouralcommunicative

40. Anatomical and radiological findings Proposed anatomical site Proposed anatomical site Radiological findings: the neuroanatomic findings on computed tomography or magnetic resonance imaging, such as ventricular enlargement, white matter loss, or brain anomaly Radiological findings: the neuroanatomic findings on computed tomography or magnetic resonance imaging, such as ventricular enlargement, white matter loss, or brain anomaly

41. Causation and timing Underlying cause if known e.g. meningitis / head injury/ brain malformation Underlying cause if known e.g. meningitis / head injury/ brain malformation Presumed timing Presumed timing

42. Aetiology Over emphasis on intra partum asphyxia as a cause Over emphasis on intra partum asphyxia as a cause 68% of children with CP have normal Apgar score and neuro neonatal exam (Nelson & Ellenberg 1988) 68% of children with CP have normal Apgar score and neuro neonatal exam (Nelson & Ellenberg 1988) 13% of those with CP had Apgar<5 and all had abnormal neuro exam 13% of those with CP had Apgar<5 and all had abnormal neuro exam

43. Aetiology Cause of most CP unknown but in 70-80% cases due to pre- natal factors Cause of most CP unknown but in 70-80% cases due to pre- natal factors Intra partum asphyxia can & does occur in 10- 20%, causing hypoxic ischaemic brain injury (HIBI) Intra partum asphyxia can & does occur in 10- 20%, causing hypoxic ischaemic brain injury (HIBI)  If this occurs <20 wksmigrational defects 27-30 wksPVL 27-30 wksPVL 34-40 wks parasagittal injury 34-40 wks parasagittal injury Although obstetric & neonatal care has improved, rates of CP have not fallen Although obstetric & neonatal care has improved, rates of CP have not fallen

44. Contribution of Prematurity Cases of CP/ 1000 live births birth wt<1500g1500–2499  2500 1975–8639.4 10.9 0.5 1975–8639.4 10.9 0.5 1976–81 28.2 6.4 1.2 1976–81 28.2 6.4 1.2 1977–92 29.2 14.1 1.0 1977–92 29.2 14.1 1.0 1982–84 59.2 9.7 1.1 1982–84 59.2 9.7 1.1 1984–91 55.9 10.3 1.4 1984–91 55.9 10.3 1.4 1987–90 62.2 13.9 1.4 1987–90 62.2 13.9 1.4 1987–91 95.5 13.1 1.2 1987–91 95.5 13.1 1.2

45. CP prevalence under 27 weeks by year Robertson, JAMA 2007

46. Risk factor for CP Mother Mother  Pre eclampsia  Multiple pregnancy  Infection  Diabetes  Threatened abortion Baby  Pre term birth  IUGR(13% of those with CP cf 2% in normal children)  Brain malformation or other malformation(10- 20%)  Genetic factors (rarely)

47. TypeAetiology Prognosis Prognosis Diplegia PVL – prematurity good Hemiplegia Intrapartum Intrapartum · IVH or ICH Postnatal Normal IQ 25% visual defect 30% epilepsy Quadriplegia Significant early pregnancy events Poor esp if multicystic change Athetiod Acute asphyxia May have normal IQ Ataxic ¼ genetic ½ unknown 1/3 normal IQ Some will walk

48. Diagnosis CP - History History- mother: History- mother:  Detailed obstetric hx, pregnancy & labour (need maternal notes)  Ante natal – foetal growth, APH, infection, preterm labour  Intra partum asphyxia- delivery, rescus,  Neonatal course -SCBU, discharge exam  Post natal illness – meningitis, head injury  Family history- genetic? metabolic?

49. Diagnosis CP -exam Abnormalities of tone Abnormalities of tone Persistence of primitive reflexes Persistence of primitive reflexes Failure to develop protective reflexes Failure to develop protective reflexes Abnormal neurological signs- brisk reflexes, clonus, Babinski sign Abnormal neurological signs- brisk reflexes, clonus, Babinski sign Developmental assessment Developmental assessment Seizures Seizures

50. Exclusion of other explanations  Dysmorphic & genetic syndromes  CNS abnormality (from MRI scan)  Congenital infection  Metabolic errors

51. Possible investigation MRI scan- abnormal in 80—90%, help with aetiology, prognosis, treatable conditions rarely ( e.g. CNS malformation 10%) MRI scan- abnormal in 80—90%, help with aetiology, prognosis, treatable conditions rarely ( e.g. CNS malformation 10%) Clinical examination. Together with scan should determine aetiology Clinical examination. Together with scan should determine aetiology Metabolic testing (0-4%) rarely helpful Metabolic testing (0-4%) rarely helpful Genetic v rare (except in ataxic CP) Genetic v rare (except in ataxic CP)

52. Prognosis Hemiplegia: Will walk, little motor delay Hemiplegia: Will walk, little motor delay Spastic diplegia: 50% will walk but often at level, GMFCS 11/111 Spastic diplegia: 50% will walk but often at level, GMFCS 11/111 Bilateral spastic CP: 25% will need total care, 33% will walk after 4 years Bilateral spastic CP: 25% will need total care, 33% will walk after 4 years Most children who sit independently by 2 years will go onto walk Most children who sit independently by 2 years will go onto walk

53. Interventions – non operative Exercises Exercises Splints, e.g. AFO's Splints, e.g. AFO's Analgesia Analgesia Oral medication Oral medication Botox injections (Botox will relax muscles but it has no effect on fixed tendons) Botox injections (Botox will relax muscles but it has no effect on fixed tendons) Baclofen pumps Baclofen pumps Dorsal rhizotomy (only in selected children with increased tone but no fixed contractures >7 years.. And live in USA!) Dorsal rhizotomy (only in selected children with increased tone but no fixed contractures >7 years.. And live in USA!)

54. Treatment usage 0 - 3 years3 years – puberty Puberty onwards Physio/OT Oral medications Botulinum Physio/OT Oral Medications Botulinum IT baclofen/SDR Tendon Surgery Life Activities Oral Medications Botulinum IT Baclofen/SDR Surgery

55. CP- Orthopaedic complications Contractures Contractures Dislocated hips Dislocated hips Scoliosis Scoliosis

56. Screening hips Xray Hips if >30 months and non ambulant Xray Hips if >30 months and non ambulant Migration percentage: Migration percentage: <15% minimal risk <15% minimal risk if signs of migration then xray every year if >5% per year then refer orthopod if signs of migration then xray every year if >5% per year then refer orthopod >15% refer orthopod >15% refer orthopod 15-30% = 20 - 80% risk hip problems 15-30% = 20 - 80% risk hip problems >30% = 100% risk hip problems >30% = 100% risk hip problems

57. Interventions – operative Aim of moving up one functional level Hips – when >30% subluxed Hips – when >30% subluxed Soft tissues around hips / knees / ankles Soft tissues around hips / knees / ankles Occasionally upper limbs / feet Occasionally upper limbs / feet Rarely the Spine Rarely the Spine

58. Interventions - operative Realistic about outcome Realistic about outcome Realistic about pain and difficulties and post op therapy Realistic about pain and difficulties and post op therapy Realistic about the risks - there is a 1% mortality risk of surgery in cases of severe CP. Realistic about the risks - there is a 1% mortality risk of surgery in cases of severe CP. Optimise nutritional status Optimise nutritional status

59. This just thinks about the motor dificulties

60. Secondary disabilities Vision Vision Hearing Hearing Learning (30% severe LD with 4 limb CP) Learning (30% severe LD with 4 limb CP) Behaviour Behaviour Sleep Sleep

61. Visual impairment Visual Impairment N=99 49 9 41 CP or structural abnormality Hearing impaired Visually impaired children West Sussex. Fielder, Best, Bax

62. Hearing impairment 70% 1% 7% Hearing impaired Visually impaired Neurological disorders, learning difficulties 11% Other systems 10%

63. 40% of children with learning disabilities have a diagnosable mental health problem

64. Which disorders? For a child with learning difficulties the are: For a child with learning difficulties the are:  33 x ASD  8 x ADHD  6 x conduct disorder  4 x emotional disorders  1.7 x depressive disorder

65. Sleep 23% children with CP have sleep problem cf 5% normal population. 23% children with CP have sleep problem cf 5% normal population. Initiation, maintenance, sleep wake cycle Initiation, maintenance, sleep wake cycle Associations: Associations:  Epilepsy OR 17  Quadriplegia OR 13  Dyskinetic CP OR 20  Visually impaired OR 12.5

66. Secondary medical problems in children with cerebral palsy Epilepsy (20% CP) Epilepsy (20% CP) Feeding difficulties Feeding difficulties GOR GOR Drooling Drooling Constipation Constipation

67. When seeing a child with CP in out patients Current concerns Current concerns Motor difficulties Motor difficulties  therapy plan goals  Would it help to decrease tone? – baclofen, botulinum toxin injections Secondary orthopaedic complications Secondary orthopaedic complications  Contractures  Hips  spine

68. Co-morbidities Co-morbidities  Feeding / reflux  constipation  Seizures  VP shunt Current function Current function  Vision / hearing  communication  Behaviour  Sleep

69. Situtation at home and school Situtation at home and school  Aids inc splints and wheelchair  Adaptations – home / school  Benefits  Support organisations  Short term breaks – if appropriate

70. Summary Many causes of cerebral palsy Many causes of cerebral palsy Underlying cause cannot be treated Underlying cause cannot be treated It is important to address the other issues and complications so these children and families can lead normal lives It is important to address the other issues and complications so these children and families can lead normal lives