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Intrathecal baclofen in children and adolescents with cerebral palsy Dr Ram Kumar Consultant Paediatric Neurologist Alder Hey, Liverpool May 2012.

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Presentation on theme: "Intrathecal baclofen in children and adolescents with cerebral palsy Dr Ram Kumar Consultant Paediatric Neurologist Alder Hey, Liverpool May 2012."— Presentation transcript:

1 Intrathecal baclofen in children and adolescents with cerebral palsy Dr Ram Kumar Consultant Paediatric Neurologist Alder Hey, Liverpool May 2012

2 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

3 Case A: seen March 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

4 Case A: Supine spine x-ray 51 deg Cobb angle Previous adductor tenotomies, botulinum toxin lower limbs, bilateral hip reconstruction surgery

5 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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10 Refresher on ITB Titanium 40 ml capacity 175 g 8.8 cm diameter 7 year life except at high infusion rates Connector Opaque one-piece catheter 89 cm long; internal volume ~ 1 day’s worth of infusion

11 Refill through the central port

12 Programmer and wand Various ways of programming infusion e.g. simple continuous, variable rate continuous, complex bolus dosing regime

13 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

14 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

15 Usual process of assessment Consensus on the appropriate use of ITB in paediatric spasticity. Eur J Paed Neurol 2009 Diagnosis and prognosis Co-morbidities Past, current and impending physical, drug and surgical treatments Other professionals involved Non-medical factors Physical examination at baseline Questionnaire tools as appropriate Follow-up: Symptoms Focussed physical examination Other professionals involved/missing from follow-up Changes in non-medical situation Questionnaire tool as appropriate Response to previous changes in treatment

16 The updated European Consensus 2009 on the use of Botulinum toxin for children with cerebral palsy. Eur J Paed Neurol 2009

17 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

18 Dec 2008: Age 13 Mar 2010: Age 15

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20 76 degree Cobb angle Spine flexible under traction March 2012: Age 17 years ITB dose 210mcg/day Posterior instrumentation ; anterior approach not required Out of hospital within 9 days

21 Post-operative problems Headaches, flashing lights Has the ITB pump stopped working? Has the catheter been cut or blocked? Neuropathic pain and behaviour change

22 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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25 Pre-ITB, T-1 (9 months prior to ITB implantatio n) Pre-ITB pump implantatio n, T0 T1 (2 months post- implantatio n) T2 (27 months post- implantation) ITB infusion dose (mcg/day) PPP Pain A (hip pain) PPP Good day MAS Oral medications Diclofenac 40mg tds, Tramadol 50mg tds Diclofenac 40mg tds, intramuscular botulinum toxin injections

26 Patient number Age at ITB pump implant Co-morbiditiesMedication related to tone or pain management, at implantation 114 LD, EPILEPSY, SCOLIOSIS, VI, BEHAVIOURCodeine, Paracetamol, Gabapentin Botulinum Toxin, Baclofen 216 LDBaclofen 312 LD, EPILEPSY, PEG, RESP, SCOLIOSIS, HIPSTramadol, Paracetamol, Tetrabenazine 413 LD, EPILEPSY, PEG, SCOLIOSIS, VIDantrolene, Paracetamol, Nitrazepam 514 LD, EPILEPSY, SCOLIOSIS, HIPS, VIDiclofenac, Paracetamol, Tramadol, Botulinum Toxin, Baclofen 616 LD, SCOLIOSIS, VIDiclofenac, Baclofen, Trihexiphenidyl 714 LD, EPILEPSY, BEHAVIOURDiclofenac, Baclofen, Trihexiphenidyl 813 LD, EPILEPSY, PEG, VI, BEHAVIOURParacetamol, Baclofen

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28 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”

29 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

30 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

31 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 (1): 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): 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): 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.


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