intrathecal baclofen in children and adolescents with cerebral palsy dr ram kumar consultant...
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Intrathecal baclofen in children and adolescents with cerebral palsy
Dr Ram KumarConsultant Paediatric Neurologist
Alder Hey, LiverpoolMay 2012
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Summary
• Case example: before and after ITB (short term effects)
• Refresher on ITB and basics• Another couple of case examples
demonstrating context of ITB use in teenagers with CP
• Other issues particularly pain• Useful references
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• Case A: seen March 2011• 15• HIE Grade 2 or 3• Athetoid dystonic 4 limb cerebral palsy• GMFCS Level 5; MACS 4• Good cognition, dysarthria• Minimal co-morbidities• Increasing dystonia• Previous hip surgery – pseudoarthrosis on left• Spine X-ray pre-ITB (6 months): 51 deg Cobb angle
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Case A: Supine spine x-ray 51 deg Cobb angle
Previous adductor tenotomies, botulinum toxin lower limbs, bilateral hip reconstruction surgery
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Progress
• Main goals: voluntary upper limb control, pain relief, improve flexibility of spinal curvature
• ITB test dose November 2011 – successful; low pressure headache
• Proceed to ITB implantation Feb 2012• Current dose ITB 180 mcg/day
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?
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Refresher on ITBTitanium40 ml capacity175 g8.8 cm diameter7 year life except at high infusion rates
ConnectorOpaque one-piece catheter89 cm long; internal volume ~ 1 day’s worth of infusion
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Refill through the central port
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Programmer and wand
Various ways of programming infusion e.g. simple continuous, variable rate continuous, complex bolus dosing regime
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Intrathecal baclofen itself
• ITB provides 1000 times the CSF concentration compared to oral baclofen
• Rule of thumb 100mg/day of oral baclofen = 100mcg/day
• Acts on GABA-B receptor – but where?• Volume and flow effects of IT baclofen• Receptor downregulation and tolerance• Overdose and withdrawal effects life-threatening
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IT baclofen• Concentration of 1000mcg/ml to 4000mcg/ml• With 40ml pump reservoir, minimum volume of 3
mls• 1000mcg/ml solution at 250mcg/day. Would
need refill every 4.5 months• Alarm for low reservoir volume and nearing end
of life• Can be removed – not permanent procedure• Implications: 100% commitment and ability to
attend clinics
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Usual process of assessment
Consensus on the appropriate use of ITB in paediatric spasticity. Eur J Paed Neurol 2009
Diagnosis and prognosisCo-morbiditiesPast, current and impending physical, drug and surgical treatmentsOther professionals involvedNon-medical factorsPhysical examination at baselineQuestionnaire tools as appropriate
Follow-up:SymptomsFocussed physical examinationOther professionals involved/missing from follow-upChanges in non-medical situationQuestionnaire tool as appropriateResponse to previous changes in treatment
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The updated European Consensus 2009 on the use of Botulinum toxin for children with cerebral palsy. Eur J Paed Neurol 2009
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Another case
• Case B: seen since 2008, now 17 years• 4 limb spastic-dystonic CP• Prematurity 30 weeks• GMFCS 5; MACS 5• Learning difficulties, dysarthric, oral feeder• Perseverative, anxiety issues• Bilateral hip reconstruction in 2002 and 2008• Oral baclofen 100mg/day – ongoing hip pain and
general discomfort
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Dec 2008: Age 13
Mar 2010: Age 15
Mar 2010: Age 15
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76 degree Cobb angleSpine flexible under traction
March 2012: Age 17 yearsITB dose 210mcg/day Posterior instrumentation ; anterior approach not requiredOut of hospital within 9 days
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Post-operative problems
• Headaches, flashing lights• Has the ITB pump stopped working?• Has the catheter been cut or blocked?• Neuropathic pain and behaviour change
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ITB and pain
• Case C; now 17 year old male• CP due to neonatal meningitis• Asymmetric 4 limb spasticity, left worse• GMFCS 5; MACS 5• Severe intellectual disability, blind, VP shunt• Previous bilateral hip surgery ’03• Increasing pain 2 years – focal and general
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0
1
2
3
4
5
6
7
Pre-ITB ITB 85mcg/day ITB 90mcg/day
Score
ITB dose
CCHQ scores for Case C
Ease of cares
Positioning
Comfort
Interaction
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Pre-ITB, T-1 (9 months prior to ITB implantation)
Pre-ITB pump implantation, T0
T1 (2 months post-implantation)
T2 (27 months post-implantation)
ITB infusion dose (mcg/day)
- 0 90.0 182.0
PPP Pain A (hip pain)
50 54 5 22
PPP Good day
14 - 5 11
MAS 1.08 1.78 0.6 0.16
Oral medications
Diclofenac 40mg tds, Tramadol 50mg tds
Diclofenac 40mg tds, Tramadol 50mg tds
Diclofenac 40mg tds, Tramadol 50mg tds
Diclofenac 40mg tds, intramuscular botulinum toxin injections
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Patient number
Age at ITB pump implant
Co-morbidities Medication related to tone or pain management, at implantation
1 14
LD, EPILEPSY, SCOLIOSIS, VI, BEHAVIOUR Codeine, Paracetamol, Gabapentin Botulinum Toxin, Baclofen
2 16LD Baclofen
3 12
LD, EPILEPSY, PEG, RESP, SCOLIOSIS, HIPS Tramadol, Paracetamol, Tetrabenazine
4 13
LD, EPILEPSY, PEG, SCOLIOSIS, VI Dantrolene, Paracetamol, Nitrazepam
5 14
LD, EPILEPSY, SCOLIOSIS, HIPS, VI Diclofenac, Paracetamol, Tramadol, Botulinum Toxin, Baclofen
6 16
LD, SCOLIOSIS, VI Diclofenac, Baclofen, Trihexiphenidyl
7 14
LD, EPILEPSY, BEHAVIOUR Diclofenac, Baclofen, Trihexiphenidyl
8 13LD, EPILEPSY, PEG, VI, BEHAVIOUR Paracetamol, Baclofen
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Findings
• Works for some types of pain (spasticity, neuropathic) more than others (osteoarthritic, visceral)
• In long-term, new sources of pain arise e.g. scoliosis, GI dysmotility
• Other intervention modalities also have a role, so not just about ITB “ITB is not a panacea”
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Other specific issues
• Early and late complications: neurological, regional and systemic
• Often difficult to identify catheter blockage vs progression of underlying tone disorder vs tolerance vs response shift and mission creep
• Problems specific to standing transfer and indoor walkers (GMFCS 3 verging on 4)
• Athetoid-dystonic patients with and without spasticity
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Summary• Patient selection and feasible goals important• Medical and non-medical factors important• ITB “success” vs “failure” only relevant to a 6
month time-frame• ITB long-term success only makes sense in
context of wider rehabilitative approach• Need help please – tall order for any single
service to do all of this• Additional advances in hardware, software and
pharmaceutics should improve matters
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References• NICE Spasticity in children guidelines – due out soon• Dan et al. Consensus on the appropriate use of intrathecal baclofen (ITB)
therapy in paediatric spasticity. Eur J Paediatr Neurol 2010 14(1): 19-28.• Morton et al. Controlled study of the effects of continuous intrathecal
baclofen infusion in non-ambulant children with cerebral palsy. Dev Med Child Neurol. 2011; 53(8):736-41.
• Pin et al. Use of intrathecal baclofen therapy in ambulant children and adolescents with spasticity and dystonia of cerebral origin: a systematic review. Dev Med Child Neurol. 2011;53(10):885-95.
• Heinen et al. The updated European Consensus 2009 on the use of Botulinum toxin for children with cerebral palsy. Eur J Paediatr Neurol. 2010; 14(1):45-66.