Survival of individuals with cerebral palsy receiving continuous intrathecal baclofen treatment: a matched‐cohort study

Abstract

AIM To determine whether intrathecal baclofen (ITB) changes mortality risk in persons with cerebral palsy (CP).METHOD Records were reviewed for all persons with CP who were managed with ITB for hypertonicity at a specialty hospital in Minnesota between May 1993 and August 2007. A comparison cohort was randomly selected from clients of the California Department of Developmental Services who were initially evaluated between 1987 and 1990 and were matched to those with ITB for age, sex, Gross Motor Function Classification System (GMFCS) level, presence or absence of epilepsy, and feeding-tube use. Survival probabilities were estimated using the Kaplan-Meier method, and differences were tested via log-rank.RESULTS Three hundred and fifty-nine persons with CP (202 males, 157 females) receiving ITB for hypertonicity (mean age 12y 8mo, SD 7y 9mo, range 3y 1mo to 39y 9mo) were matched to 349 persons without ITB pumps (195 males, 154 females; mean age 12y 7mo, SD 8y 4mo, range 2y 7mo to 40y). The proportion of patients at different GMFCS levels in the ITB and in the non-ITB cohorts, respectively, was as follows: level II 3% and 3%, level III 16% and 16%, level IV 38% and 37%, and level V 43% and 44%. Survival at 8 years of follow-up was 92% (SD 1.9%) in the ITB cohort and 82% (SD 2.4%) in the non-ITB cohort (p<0.001). After adjustment to account for recent trends in improved survival in CP, 8-year survival in the non-ITB cohort was 88%, which was not significantly different from the ITB cohort (p=0.073).INTERPRETATION ITB therapy does not increase mortality in individuals with CP and may suggest an increase in life expectancy.Cerebral palsy (CP) is the most common congenital cause of disability in children, affecting approximately 2 to 3 per 1000 live births. 1 Spasticity is reported in approximately 70% of those with CP and is thought to interfere with function and comfort. Baclofen is a derivative of gamma-aminobutyric acid that is used to treat spasticity. The effectiveness of oral baclofen is limited by its sedating side effects, so the drug is often administered intrathecally by continuous infusion to deliver it to the site of action, the spinal cord. Intrathecal baclofen (ITB), which was approved by the US Food and Drug Administration in 1996 for use in individuals with CP, is effective in the reduction of spasticity as well as dystonia and is frequently used to treat hypertonicity associated with CP. [2][3][4] In addition to the long-term reduction of hypertonicity, authors have reported improvement in comfort, positioning, ease of care provision, and motor function in select groups of individuals, and a reduction in the anticipated need for orthopedic surgery. 2,5,6 Progression of hip dislocation may be reduced with ITB, although the effect of ITB on the progression of scoliosis is controversial. [7][8][9][10] ITB therapy involves the surgical implantation of a programmable pump with a reservoir for the continuous delivery of baclofen to the intrathecal space. As such, it is associated with risks related to surge...

Setting a patient‐centered research agenda for cerebral palsy: a participatory action research initiative

“…Some work has been done in this area, including studies of selective dorsal rhizotomy 14,15 and intrathecal baclofen. 16 Much more research needs to focus on this area, including studies of intensive therapy interventions and other treatments into which families heavily invest their time and resources with the hope of maximizing their child's potential as an adult. Thirdly, there were many questions about physiology of the ageing process combined with a developmental neurological disorder such as CP.…”

Polyanalgesic Consensus Conference 2012: Recommendations for the Management of Pain by Intrathecal (Intraspinal) Drug Delivery: Report of an Interdisciplinary Expert Panel

“…Cerebral Palsy Results from a number of studies suggest that IT baclofen therapy is useful in reducing spasticity, decreasing pain frequency and severity, and improving gross motor function in patients with cerebral palsy (207–211). One review of records from IT baclofen‐treated patients with cerebral palsy and patients with cerebral palsy who did not receive IT baclofen suggested that IT baclofen therapy does not increase mortality and may improve life expectancy (212). This suggestion is based on a small retrospective single center study and more research is needed.…”

Nursing Care of the Pediatric Neurosurgery Patient

“…Recent longitudinal studies support both ef fi cacy and safety in the continuous use of ITB for children with spasticity as well as other movement disorders caused from various underlying conditions, including cerebral palsy, stroke, multiple sclerosis, myelomeningocele, and head or spinal injury (Bergenheim et al 2003 ;Krach et al 2010 ;Morton et al 2011 ;Ramstad et al 2010 ;Ward et al 2009 ) . Recent longitudinal studies support both ef fi cacy and safety in the continuous use of ITB for children with spasticity as well as other movement disorders caused from various underlying conditions, including cerebral palsy, stroke, multiple sclerosis, myelomeningocele, and head or spinal injury (Bergenheim et al 2003 ;Krach et al 2010 ;Morton et al 2011 ;Ramstad et al 2010 ;Ward et al 2009 ) .…”