Life expectancy in cerebral palsy.
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Biomedical subjects
Publications and source records attributed to R Shavelle.
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It is well known that individuals with cerebral palsy (CP) are subject to higher mortality than the general population but the causes of this have not been systematically analysed. This study investigates mortality in a Californian population of 45,292 individuals with CP, 4028 of whom died during the 1986 to 1995 study period. The aims of this study were to identify diseases that may be causally linked with CP, and diseases whose diagnosis and/or treatment need improvement. Overall, the standardized mortality ratio (SMR) was 8.4. Mortality from breast cancer was three times that of the general population, suggesting poorer detection and/or treatment. The dramatic elevation of mortality due to brain cancer, especially in children (SMR=24), raises the possibility of a link between this and CP. Cause of death was non-specific in some individuals. Therefore, these SMRs are conservative. As expected, SMRs due to respiratory diseases were very high but, contrary to anecdotal reports, such diseases did not account for most deaths. High SMRs were also found for diseases of the circulatory and digestive systems. Finally, a marked elevation of deaths was due to external causes, including drowning and being hit by motor vehicles.
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More than 2,000 persons with developmental disabilities have recently been transferred from California institutions into community care. Using data on 1,878 clients moved between April 1993 and December 1995, Strauss et al. (1998) found a corresponding increase in mortality rates. In the present report we update that study by analyzing 1996 data. There were 36 deaths, an 88% increase in risk-adjusted mortality over that expected in institutions, p < .01. We again found that persons transferred later were at higher risk than those moving earlier, even after adjustment for differences in risk profiles. In the highest functioning group, the community mortality rate was tripled. Death certificate information was also analyzed.
We develop an extension of the Kaplan-Meier estimator for the case of multiple live states. The method can be used to construct prognostic charts for tracking individuals initially in a given condition. It is also the key component in constructing a longitudinal version of the multistate life table.
The authors analyzed death rates from external causes (accidents, injuries, homicides, etc.) for persons with developmental disability in California. There were 520 such deaths during the 1981-1995 study period, based on 733,705 person-years of exposure; this represents all persons who received any services from the state. Compared with the general California population, persons with developmental disability were at lower risk of homicide, suicide, and poisonings (standardized mortality ratios, 0.31-0.68), but higher risk of pedestrian accidents, falls, fires, and, especially, drowning (standardized mortality ratio=6.22). A major focus of the study was comparisons between different residential settings. Persons in semi-independent living had significantly higher risk than did those in their family home or group homes, with homicides rates being three times higher and pedestrian accidents rates being doubled, while persons in institutions had much lower risks with respect to most causes. Of the 28 deaths due to drug and medication overdoses, 79 percent occurred in supported living or small-group homes. Avoidable deaths could be reduced by making direct care staff more aware of the risks and better trained in acute care, along with improved monitoring of special incidents.
OBJECTIVES: The purpose of this study was to compare quantitative estimates of coronary calcification with traditional coronary risk factors to determine their independent predictive power for the diagnosis of obstructive angiographic coronary artery disease in symptomatic patients. METHODS: Three hundred sixty-eight symptomatic patients underwent coronary angiography and electron beam computed tomography at four different centers between April 1989 and December 1993. A blinded cardiologist interpreted the electron beam computed tomograms. Coronary risk factors were obtained in all 368 patients. Both bivariate and multivariate analyses were used to investigate the relation between risk factors and angiographic disease. RESULTS: One hundred fifty-eight patients (43%) had angiographically obstructive coronary artery disease (>50% luminal stenosis) and 297 (81%) had coronary calcification. At the bivariate level, only male sex and log-transformed coronary calcification were predictive of angiographic disease (p = 0.008, p = 0.001). By multivariate analysis, only male sex and coronary calcification were predictive (p = 0.001, p = 0.001). Sixty-four of the 71 patients without coronary calcification did not have disease, yielding a negative predictive value of 90%. Receiver operating characteristic curve analysis showed that the amount of coronary calcium was a significantly better discriminator of disease than were the other risk factors. CONCLUSIONS: Coronary calcification is a stronger predictor of angiographic coronary artery disease in symptomatic patients undergoing angiography than are standard risk factors.
We compared risk factor-adjusted mortality for California adults with developmental disabilities based on 22,576 adults receiving services in California, 1985-1994. Mortality rates were adjusted for factors such as age and level of functioning. Risk factor-adjusted mortality was 72% higher in community care than in institutions. The mortality pattern over the years 1993-1994, which had not previously been studied, was comparable to that of 1985-1992. The substantially increased risk in community care suggests that community settings may be less effective in preventing mortality in this population.
Between 1993 and 1995, 1,878 persons transferred from California institutions into the community. By early 1996, 45 had died--significantly more than expected (Strauss, Shavelle, Baumeister, and Anderson, 1998). We report here on the death certificates for this group, using a comparison group of 45 certificates for institutional residents. Thirty-two of the community deaths versus 10 of the institution deaths were "sudden" or "subacute." All of the institution deaths versus 79% of the community deaths were reported to the coroner, and 55% of the institutional deaths were followed by autopsy compared to 33% in the community. Six deaths were due to perforated bowels, 5 of which were in individuals residing in the community.
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More than 2,000 persons with developmental disabilities have been transferred from California state institutions into community care in recent years. We investigated whether this has been accompanied by a change in mortality rates, using data on 1,878 clients moved since April 1993. Mortality rates were compared to those expected for comparable persons in institutions. Risk-adjusted mortality rates for movers exceeded institutional rates by 51%, p < .05. After removal of cancer deaths in both groups, this increased to 67%. The effect was largest shortly after the move and in the subjects who had moved most recently. The deinstitutionalization process in California appears to have resulted in a substantial increase in mortality, indicating the need to ensure continuous, consistent, and competent medical care and supervision in the community.
The life expectancy is an important summary measure of an individual's prognosis for survival. The life table is the preferred method for computing life expectancies, but it is not always feasible. We show that for several chronic disabilities, the logarithms of the age-specific mortality ratios (relative to the general population) decline linearly with age, reaching parity at age 85 or older. This, combined with a standard modeling of an individual's current mortality rate, yields a set of age-specific mortality rates that can be used to produce a "customized" life table. The life expectancy is then immediately available. In a series of empirical comparisons the method performed better than an assumption of constant excess death rate (EDR), and much better than one of constant mortality ratio (MR). The method may be useful for a variety of non-progressive disabilities, such as cerebral palsy and injuries of the brain or spinal cord.
BACKGROUND: The large database of the California Department of Developmental Services provides a data source for mortality rates in persons with mental retardation by age, sex, severity, cause and associated conditions. This study involves patients with a diagnosis of cerebral palsy. RESULTS: After a table of demographic data, four tables are used to show detailed age-related observed and expected mortality rates for Cerebral Palsy patients by sex and a severity factor that divides the patients into two groups of approximately equal size. The factor used was quadriplegia (all four limbs involved in motor dysfunction). Spasticity was the predominant feature of the motor dysfunction. CONCLUSION: Excess mortality was moderate in the less severe Cerebral Palsy patients, but was higher in those with quadriplegia (overall EDR--Excess Death Rate--about 6 per 1000 and 16 per 1000, respectively). In less severe cases EDR was higher at ages 1-4 years, the almost constant to age 49, then rose with advancing age. In case with quadriplegia EDR decreased in childhood and young adults to a relatively stable minimum at ages 25-49, then increased at older ages. There was little sex difference in EDR.
To determine the predictors of mortality and find the life expectancies of adults with cerebral palsy (CP), data on 24,768 individuals aged 15 years and over who received services in California between January 1980 and December 1995 were analyzed. Multivariate statistical methods to identify mortality predictors, and actuarial methods to determine corresponding life expectancies were used. The key predictors were lack of basic functional skills: mobility and feeding. Adults lacking these skills had much reduced life expectancies, as short as 11 years for the worst functioning groups. By contrast, survival of high-functioning adults was close to that of the general population. The influence on survival of cognitive skills, type of CP, and other factors appears to be expressed largely through their effect on basic functioning. Life expectancies of adults of a given age can differ by 40 years or more, according to their functional level.
PURPOSE: To determine the predictive value of coronary calcifications for coronary heart disease events in high-risk, asymptomatic adults: PATIENTS AND METHODS: A prospective cohort study of 1,461 high-risk, asymptomatic subjects were followed for 55 months with a 98% success rate. Coronary risk factor assessment and cardiac fluoroscopy with digital subtraction enhancement were performed to determine the number of calcified coronary arteries. RESULTS: Fifty-eight percent of this cohort (852 subjects) had fluoroscopically detectable coronary calcification: 437 (30%) had calcium in one, 253 (17%) in two, and 162 (11%) in all three coronary vessels. There were 90 (6%) deaths, 35 (39%) attributable to coronary heart disease, and 43 (3%) nonfatal myocardial infarctions. Subjects with calcification in more than one major coronary artery were 2.2 times more likely to suffer coronary death or nonfatal infarction (P = 0.001) than were subjects with one or no calcified arteries. Multivariable logistic regression analysis showed that only the number of calcified arteries, age, total cholesterol, history of diabetes, and left ventricular hypertrophy by electrocardiogram were associated independently with the incidence of coronary death or infarction in these subjects. CONCLUSIONS: Coronary calcification predicts coronary heart disease death or infarction in high-risk asymptomatic adults as well as do standard risk factors.
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OBJECTIVE: To derive prognostic data for survival and clinical improvement in children with severe developmental disabilities. STUDY DESIGN: A 13-year follow-up study of several cohorts of children initially evaluated before their first birthday. The outcomes studied were survival and improvement in condition. Methods were used to overcome limitations in previously published work on the same California data base. Of the 11,912 children who received services from the California Department of Developmental Services between January 1980 and December 1993, we focused on three cohorts defined according to mobility and need for tube feeding. RESULTS: Children who were tube fed and unable to lift their heads by ages 3 to 12 months were at high risk for early death, with a median remaining life expectancy of 3.2 years. Of those who survived an additional 2 years, the condition of about one third improved. A substantial majority of those who either showed improvement or died had done so by that age. CONCLUSION: By age 5 years, the prognoses for survival and improvement have to a large extent been clarified. For children who survive to age 5 years, even those in the lowest functioning cohort have a 60% chance of surviving an additional 5 years. Detailing the probabilities of various outcomes at various ages should be useful to parents, pediatricians, and others concerned with children with developmental disabilities.