Movement therapies

Charlie Fairhurst

Evelina Children’s Hospital & Chailey Heritage

(Surrey under 14s Cricket Coach)

1

• Introduction

• Movement therapy as part of holistic care

• Specific interventions

– General

– Focal

Neurodisability - medical pathways

• Emergency care - medical stabilisation

• Diagnosis

• Long term Management ~

– Child ~ Quality of life • Think holistically

• Nutrition

• Seizures / Spasms

• Movement / Mobility / Posture

• Management - Family & interdisciplinary support ~ Quality of life

Independence

Participation

Experience

Dependence

Core

Hierachy of

Clinical

Need

Developing a structure for review in Neurodisability

PAIN FREE

COMFORT

SECURITY

ATTATCHMENT

No child can perform at

any level of ability unless

they have adequate

physical and

psychological comfort

Basic Clinical

…needs met?

Psychological

Self identity

Physically

•minimise negative impact of

medical disorders seizures /

infections

•adequate nutrition?

Psychologically

•encourage self identity, whilst

acknowledging total

dependence

Passive and

active

participation

Active

decision

making

Facilitating self control within

environments

•Home

•Play

•School

INPUT

•Communication

•Movement

•Education

•Physical needs

•Emotional needs

•Operational level

•Change of environment

Allowing the individual child to

make choice about their lives

Treatment options in motor disorders

Holistic Medical Management

• Motor difficulty – Movement and Posture

– Spasms

– Contractures

– Pain

• Co-morbidities – Seizures

– Chest

– Nutrition

– GI tract

9

Principles of Motor Management

• Respond to the dynamic process encountered with growth to minimise secondary problems

• Respond to + and – effects of UMN syndrome

• Movement

• Posture

• Development

• Multidisciplinary approach

10

Adaptive approach

~ over time

Upper Motor Neurone Syndrome

• Overactivation • Spasticity

– increased muscle tone – hyperreflexia – clonus – abnormal co-contraction

• Released flexor reflexes – mass synergy patterns

• Reduction in motor activity

• Loss of dexterity

• Weakness – inadequate force generation

– slow movements

• Loss of selective control

+ -

More amenable to

intervention

Usually more disabling but

less focussed on

Dystonic Cerebral Palsy

• Confused terminology

– Dystonia / dyskinesis / athetosis / chorea

• ~10% of all Cerebral Palsy

– 67 % Term peri-natal hypoxic ischaemic encephalopathy

– 21% pre-natal or premature damage

– 12 % uncertain aetiology

• Atrophy and sclerosis of basal ganglia

Therapy

• Function

• Neurodevelopmental practice

• Strength

• Stretch

• Uni vs Bilateral modalities

Medical management of tone problems

General

Focal

Permanent Reversible

Oral

Therapy

Surgery

SDR

BTX-A

ITB DBS

ITB – Intrathecal Baclofen pumps

SDR – Selective Dorsal Rhizotomy

DBS – Deep Brain Stimulation

BTX-A – Botulinum Toxin A injections

Spinal level

Locomotor Driving

System

Adaptive

system Equilibrium

system

BTX-A

SDR

Baclofen

Tizanidine

Antidystonic treatment

Oral

DBS

Dantrolene

Benzodiazepines

Anticonvulsants

ITB

Benzodiazepines

Oral therapy Muscle relaxants • Baclofen

– Pre and Post synaptic GABA

– Use since 1970’s

– Poor lipid solubility, X cross Blood Brain Barrier ++

• Tizanidine – Adrenergic

– Drowsiness, Hypotension

• Clonazepam / Diazepam – Increase pre-synaptic inhibition spinal cord level

– Sedation

• Dantrolene – Prevents Calcium release from Sarcoplasmic reticulum

– Medicine of the devil

Tone management

• Baclofen-GABA Agonist

-inhibits transmission at spinal level

-Reduces spasticity

-Depresses the central nervous system

-SE: Drowsiness and hypotonia, may lower the seizure threshold

• Dose 5mg Three times daily

– Increase to effect / side effect

Oral therapy Movement modifiers – Anti-dystonic agents • Trihexyphenidyl

– Anticholinergic, basal ganglia – Medicine of first choice – High doses necessary – GI tract disturbance, Irritability, Urinary retention, Dry mouth & eyes

• Laevo-DOPA plus Carbidopa – Basal ganglia, increase L-DOPA – Insomnia, Nausea, Vomiting, Chorea

• Clonazepam – Central Nervous system – Drowsiness, Hypotension

• Carbamazepine • Dopamine depletor plus blocker

– Tetrabenazine – Depression ++, Drowsiness

Trihexyphenidyl

• Drug most likely to have a favourable effect in non-progressive secondary Dystonia

• Start at 0.5 mg (infant), 1 mg (child) or 2 mg (adolescent) three times a day

• Increase by 0.5, 1 or 2mg per day each week until:

– It is effective

– Side effects occur

– Total daily dose of 9, 30 or 90 mg is reached

Trihexyphenidyl .........................................................................

July 2007 – Oct 2011 153 children age 3-19 (69f, 84m) Start dose 0.5 mg (infant), 1 mg (child), 2 mg (adolescent) three times per day

Increase by 0.5, 1 or 2 mg per day each week until Maximum dose 1 mg/kg/day

134 Tolerated Dose

36 Stopped treatment

49 Continue low dose 2mg tds (no.=9), intermediate dose (no.=40)

6 Continue intermediate dose plus DOPA

43 Continue maximum dose

Beneficial effects in treatment group / 98

Longer tolerance of seating system 45 (46%)

COMFORT 57 (58%)

Improved oromotor skills / swallowing 25 (26%)

Reduced power of extensor thrust 31 (32%)

Improved saliva control 43 (44%)

Other - combined 97

Side effect in treatment group / 98

Transient agitation 37 (38%)

Loss of short term memory ?>7 (>7%)

Transient dyskinesis 13 (13%)

Constipation 20 (20%)

Urinary retention 5 (5%)

Blurred vision ?>8 (>8%)

Xerostomia – dry mouth 22 (22%)

Laevodopa plus Carbidopa Start dose 1 mg / kg L-DOPA per day in four divided doses

Weekly increase 1 mg / kg Maximum dose 10 mg / kg, Continue for 3 months for therapeutic trial

October 1998 – October 2011

37 children, age 5-19 (10f, 25m)

8 stopped at low dose

• 3 Aggression

• 2 Depression

• 3 Chorea

29 that had L-DOPA for more than 3 months

13 Stopped – insufficient benefit

8 Continued mono-therapy

6 Continued plus Trihexyphenidyl

Benefits / 29

Improved swallowing 11

Reduced dysarthria 14

Reduced extensor spasm 12

Reduced upper limb dystonia 9

Better tolerance equipment 16

Other 6

None 7

Side effects / 29

Nausea 13

Altered liver profile 4

Aggression 3

Sleepiness 0

Insomnia 0

Spinal level

Locomotor Driving

System

Adaptive

system Equilibrium

system

BTX-A

SDR

Baclofen

Tizanidine

Antidystonic treatment

Oral

DBS

Dantrolene

Benzodiazepines

Anticonvulsants

ITB

Benzodiazepines

Botulinum Toxin type A

Medico legal and medico-economic factors

Limited license – accepted practice

Integrated therapy & Common indications

Doseage and safety

Assessment, administration, re-evaluation, continuation and dis-continuation

•Benefits vs Risks – Reduced spasticity / spasms vs weakness

•Ambulant vs Non-ambulant children – long term studies??

•vs Pain – Significant benefit for up to six months

•Functional benefit – Posture, ADL, QOL

•Upper limb •Neck & Spine •Saliva Control

Botulinum Toxin in Hip spasticity

• 6 months

• 27 new children, adolescents in pain

• PPP pre average 39 (range 24 – 59)

• Functional challenges – Toileting

– Cleanliness

– Dressing

– Sleeping

• USS guided injections to – Iliopsoas, Medial Hamstrings, Adductor Magnii

Botulinum Toxin in Hip spasticity

• 6 months

• 27 new children, adolescents

• PPP pre average 39 (range 24 – 59)

• PPP 1 month post average 9 (range 0 – 23)

• Functional improvements

– Toileting

– Cleanliness

– Dressing

– Sleeping

PRE 1 MONTH POST

60

30

10

20

40

50

Paediatric Pain

Profile Scores

Pre and Post targeted

Botulinum Toxin

Injections SCORE

UK Audit 2010 - 11

• TOTAL 60 +15

• South West 3+1

• South Thames 9+4

• North Thames 7

• Oxford and Anglia 8+3

• Trent / East Midlands 2+2

• West Midlands 8+2

• North West 4

• Northern and Yorkshire 7+2

• Wales 2+1

• Scotland 5

• Northern Ireland 2

• Ireland 3 32

Injection Numbers in 2010 Population 1 - 16

• North Thames 811 1.924m

• South Thames 782 1.588m

• South West 426 0.927m

• West Midlands 546 1.067m

• Oxford and Anglia 485 0.724m

• East Midlands / Trent 335 0.822m

• North West 430 1.338m

• North East and Yorkshire 507 1.460m

• Wales 115 0.567m

• Scotland 214 0.929m

• Ireland 224 1.246m

• TOTAL 4875 12.592m

34

Maximum doses used

Botox - iu ~

100 = 1 4/kg = 2 200 = 9 6/kg = 1 300 = 1 8/kg = 3 350 = 1 10/kg = 3 400 = 22 12/kg = 14 600 = 1 13/kg = 1 700 = 1 15/kg = 2 800 = 1 16/kg = 4 1000 = 1 18/kg = 1

25/kg = 6 27/kg = 1

Dysport - iu ~

20/kg = 1

900 = 1 25/kg = 2

1000 = 11 30/kg = 11

1200 = 5 35/kg = 4

2000 = 1 40/kg = 1

50/kg = 1

Injection sites • Upper limb 39 / 60

• Shoulder 29

• Elbow 39

• Wrist 36

• Fingers / thumb 32 / 31

• Lower limb 58 / 60 • Hips 56

• Hamstrings 53

• Gastroc-Soleus 56

• Small muscles 39

• Neck / Back 17 / 60

• Saliva glands 12 / 60

Spinal level

Locomotor Driving

System

Adaptive

system Equilibrium

system

BTX-A

SDR

Baclofen

Tizanidine

Antidystonic treatment

Oral

DBS

Dantrolene

Benzodiazepines

Anticonvulsants

ITB

Benzodiazepines

Movement therapies

Invasive techniques

Selective Dorsal (Posterior) Rhizotomy

Intrathecal Baclofen pumps

Deep Brain Stimulation

Selective Dorsal Rhizotomy

UK & Ireland centres SDR

• Robert Jones and Agnes Hunt, Oswestry

• Bristol

• Nottingham

• St Louis

Intrathecal Baclofen Combination of Dystonia and

Hypertonia

AACPDM 2000 – 25 trials

17 trials showed benefits

•Movement, posture

•Pain, spasms

•ADL, QOL

•Reduced Orthopaedic

intervention

ITB withdrawal syndrome

•Increasing spasms, seizures and pain

•Agitation, Hallucinations, Itching, Hyperthermia

Infection

•Acute, Long term

Overdose – Hypotonia, drowsiness, respiratory depression

ITB UK & Ireland centres • Nottingham

• Sheffield

• Evelina Children’s Hospital

• Bristol

• Birmingham

• Alder Hey

• Newcastle

• Edinburgh

• Dundee

• Dublin

DBS UK & Ireland

• Evelina Children’s Hospital

• Bristol

• Oxford

• Edinburgh

44

Choices, choices, choices….. -Spasticity Dystonia Both

-Tightness Weakness Co-ordination

-Focal General Problem

-Functional difficulties Co-morbidities

-Hopes and expectations Goal setting

-Health Economics Experience Availability