Survival and mortality in cerebral palsy: observations to the sixth decade from a data linkage study of a total population register and National Death Index

Abstract

Background Likely duration of survival of children described as having cerebral palsy is of considerable interest to individuals with cerebral palsy, their families, carers, health professionals, health economists and insurers. The aim of this paper is to describe patterns of survival and mortality to the sixth decade in a geographically defined population of people with cerebral palsy stratified according to the clinical description of their impairments in early childhood. Methods Identifiers of persons born in Western Australia 1956–2011, registered with cerebral palsy on the Western Australian Register of Developmental Anomalies and surviving at least 12 months, were linked to the Australian National Death Index in December 2014. Patterns of mortality were investigated using survival analysis methods. Results Of 3185 eligible persons, 436 (13.7%) had died. Of that sample the 22% with the mildest impairment had survival patterns similar to the general population. Mortality increased with increasing severity of impairment. Of 349 (75%) with available cause of death data, 58.6% were attributed to respiratory causes, including 171 (49%) to pneumonia at a mean age of 14.6 (sd 13.4) years of which 77 (45%) were attributed to aspiration. For the most severely impaired, early childhood mortality increased in succeeding decades of birth cohorts from 1950s to 1990 with 20% dying by 4 years of age in the 1981–1990 birth cohort; it then decreased for subsequent birth cohorts, 20% mortality not being attained until 15 years of age. However by 20 years of age mortality of the most severely impaired born in the 1991–2000 birth cohort exceeded that of all other birth cohorts. Remaining life expectancies by age to 50 years have been estimated for two strata with more severe impairments. Conclusion For 22% of individuals with cerebral palsy with mild impairment survival to 58 years is similar to that of the general population. Since 1990 mortality for those with severe cerebral palsy in Western Australia has tended to shift from childhood to early adulthood.

State of the Evidence Traffic Lights 2019: Systematic Review of Interventions for Preventing and Treating Children with Cerebral Palsy

“…One in 15 will require non-oral tube feeding [262]. Dysphagia management is extremely important because aspiration resulting in respiratory complication is a leading cause of death in individuals with cerebral palsy (45%) [266]. Experts have called for greater attention to respiratory health given the lack of preventative strategies and low levels of evidence for management strategies (airway clearance techniques, oral sensorimotor therapy, compensatory strategies such as positioning and thickening fluids, sialorrhea management, upper airway interventions, antibiotics, gastro-intestinal interventions, and spinal surgery) (yellow lights) [22].…”

“…Almost half of all children with cerebral palsy have cooccurring intellectual disability (46%) of varying severities, but notably, the prevalence of this comorbidity declining [1,2,262]. Co-occurring intellectual disability, coupled with severe physical disability, is known to elevate the risk for premature death during childhood [266]. With the shift in thinking about early motor interventions, the field has also started to explore whether the cognition of children with cerebral palsy can be modified and optimized.…”

Health service use among adults with cerebral palsy: a mixed methods systematic review protocol

“…The majority of people with CP will survive to at least 60 years of age. 10 Many adults with CP experience secondary conditions with age that require intervention and support from clinicians working across multiple health and social care disciplines. 12 Previous research has demonstrated that young people with CP experience challenges in accessing health services, as they move from child to adult health services.…”

“…[6][7][8][9] Many people with CP survive well into adulthood with a recent study reporting more than 80% of people with CP have a life expectancy, beyond 58 years. 10 People with CP continue to present with ongoing health issues from Strengths and limitations of this study ► This mixed method systematic review will include quantitative, qualitative and mixed methods studies in order to provide a comprehensive overview of the current literature relating to health services for adults with cerebral palsy. ► This review process is strengthened by utilisation of the widely used Andersen-Newman behavioural model of health service utilisation as an overarching framework to guide data extraction and analysis.…”

Nomogram for pneumonia prediction among children and young people with cerebral palsy: A population-based cohort study

“…The primary reason for hospitalizations [ 12 ] and the leading cause of death among younger individuals with CP is pneumonia [ 4 ]. More than half (53%–58.6%) of the deaths among children with CP were attributed to respiratory infection and failure [ 13 , 14 ].…”