Recent trends in cerebral palsy survival. Part II: individual survival prognosis.

AIM: The aim of the study was to determine survival probabilities and life expectancies for individuals with cerebral palsy based on data collected over a 28-year period in California. METHOD: We identified all individuals with cerebral palsy, aged 4 years or older, who were clients of the California Department of Developmental Services between 1983 and 2010. Kaplan-Meier survival curves were constructed for 4-year-old children, and the estimated survival probabilities were adjusted to reflect trends in mortality by calendar year. For persons aged 15, 30, 45, and 60 years, separate Poisson regression models were used to estimate age-, sex-, and disability-specific mortality rates. These mortality rates were adjusted to reflect trends of improved survival, and life expectancies were obtained using life table methods. RESULTS: The sample comprised 16,440, 14,609, 11,735, 7023, and 2375 persons at ages 4, 15, 30, 45, and 60 years, respectively. In 1983, 50% of 4-year-old children who did not lift their heads in the prone position and were tube fed lived to age 10.9 years. By 2010, the median age at death had increased to 17.1 years. In ambulatory children the probability of survival to adulthood did not change by more than 1%. Life expectancies for adolescents and adults were lower for those with more severe limitations in motor function and feeding skills, and decreased with advancing age. Life expectancies for tube-fed adolescents and adults increased by 1 to 3 years, depending on age and pattern of disability, over the course of the study period. INTERPRETATION: Over the past three decades in California there have been significant improvements in the survival of children with very severe disabilities. There have also been improvements to the life expectancy of tube-fed adults, though to a lesser extent than in children.

Recent trends in cerebral palsy survival. Part I: period and cohort effects.

AIM: To determine whether the trend of improved survival among individuals with cerebral palsy (CP) in California during the 1980s and 1990s has continued during the most recent decade. METHOD: In an observational cohort study we evaluated individuals with CP, aged 4 years and older, who were clients of the California Department of Developmental Services. Medical diagnoses, functional disabilities, and special health care requirements were assessed with Client Development Evaluation Reports made between 1983 and 2010. Trends in birth cohort survival were analyzed with Kaplan-Meier curves and Cox regression. Calendar year period effects were analyzed with Poisson regression. RESULTS: A total of 51,923 persons with CP (28,789 males [55%], 23,134 females [45%]; mean age 14y 11mo, SD 14y 1mo, range 4y 0mo to 96y 10mo) collectively contributed 662,268 years of follow-up. There were 7690 deaths for an overall mortality rate of 11.6 per 1000 persons per year. No significant birth cohort effects on survival were observed in 4-year-olds who had no severe disabilities. By contrast, children who did not lift their heads in prone position who were born in more recent years had significantly lower mortality rates (Cox hazard ratio 0.971, p<0.001) than those with comparable disabilities born earlier. With regard to calendar year period effects, we found that age-, sex-, and disability-specific mortality rates declined by 1.5% (95% CI 0.9-2.1) year-over-year from 1983 to 2010. The estimate increased to 2.5% (95% CI 1.9-3.1) per year when we additionally controlled for tube-feeding status. Mortality rates in tube fed adolescents and adults, ages 15 to 59 years, declined by 0.9% (95% CI, 0.4-1.4) per year. No improvement was observed for adolescents or adults who fed orally or for those over age 60. In fact, the ratio of age-specific mortality rates for these latter groups to those in the general population, increased by 1.7% (95% CI 1.3-2.0) per year during the study period. INTERPRETATION: The trend toward improved survival has continued throughout the most recent decade. Declines in CP childhood mortality are comparable to the improvements observed in the United States general population (i.e. the mortality ratio in childhood has remained roughly constant over the last three decades). In contrast, the mortality ratio for most adolescents and adults with CP, relative to the general population, has increased.

Life expectancy in cerebral palsy: an update.

This paper clarifies and updates some issues of life expectancy in cerebral palsy. These are: (1) the definition of life expectancy and how it is calculated; (2) the secular trends that have occurred since the data for the 1998 paper were collected; (3) revised estimates reflecting improvements of some of the analytical methods and statistics provided in that paper; (4) comparison of life expectancies among countries; (5) issues regarding quality of care; and (6) consideration of prospective life expectations in addition to current life expectancy.

Growth patterns in a population of children and adolescents with cerebral palsy.

This study examined growth of children and adolescents with cerebral palsy (CP) who received services from the California Department of Developmental Services from 1987 to 2002. In all, 141 961 measurements of height and weight were taken from 24920 patients with CP (14103 males, 10817 females). Centiles of weight and height were determined by age, sex, and five levels of functional ability ranging from fully ambulatory to unable to walk, crawl, or feed self, and fed via gastrostomy tube. Resulting charts of height and weight centiles were compared with Centers for Disease Control and Prevention weight and height charts for the general population of the US. Centiles of height and weight of patients with CP were close to those of the general population for the highest functioning groups with CP, but lagged substantially for other groups. Presence of a feeding tube was associated with greater height and weight in the lowest functioning groups, with centiles for weight being 2 to 5 kg higher for those with gastrostomy tubes. The charts may assist in early identification of nutritional or metabolic difficulties beyond what might be expected for patients with similar functional disabilities.

Survival in cerebral palsy in the last 20 years: signs of improvement?

This study investigated the possibility of improved survival in cerebral palsy (CP) over a 20-year period. Participants were 47 259 persons with CP receiving services from the State of California between 1983 and 2002. The person-year approach was used. This asks whether the probability of dying in a given calendar year changes over the study period after age and severity of disability are taken into account. An appreciable improvement over time was found in children with severe disabilities and in adults who required gastrostomy feeding. In these groups, mortality rates fell by 3.4% per year. Therefore, life expectancies reported in earlier studies should be increased by approximately 5 years if adjustments to 2002 mortality rates are made. For other persons with CP there was, at most, a small improvement over the 20-year period. The results suggest there have been improvements in the treatment and care of the most medically fragile children. Gastrostomy feeding has become much more widespread over the past two decades, and the improved survival of persons with gastrostomies may reflect better understanding of their requirements.

Decline in function and life expectancy of older persons with cerebral palsy.

The authors studied the pattern of functional abilities and decline of skills in adults with cerebral palsy. The data source was the California Developmental Disabilities data base, which included 904 subjects of age 60. For those individuals who are mobile when they become adults, there is a marked decline in ambulation, especially in late adulthood, and few of the 60 year-olds who walked well preserved this skill over the following 15 years. Older subjects frequently also lost the ability to dress themselves. Many other skills, however, seemed to be well preserved, including speech, self-feeding and the ability to order meals in public. Whereas the great majority of young adults lived in their families' home or in small private group homes, 18% of the 60 year-olds lived independently or semi-independently, and 41% resided in facilities providing a higher level of medical care. Survival rates of the ambulatory older adults were only moderately worse than the general population. Survival was, however, much poorer among those who had lost mobility.