Steven M Day

Mortality and causes of death in persons with Down syndrome in California

This study investigated mortality and causes of death between 1988 and 1999 in 14781 persons (6702 female) with Down syndrome in California, comparing age, sex, ethnicity, and other factors. Mean age at the start of follow-up was 14 years 8 months (SD 14y 10mo). During the study period 600 persons died. The standardized mortality ratio (SMR) for the population was 5.5. Blacks were at greater risk than whites, Hispanics, or Asians (relative risk = 1.5). Mortality declined during the period, especially for children with congenital heart defects. Leukemia (SMR = 17), respiratory illnesses (SMR = 27), congenital anomalies (SMR = 72), and circulatory diseases (SMR = 5.3) accounted for most of the excess mortality. With the exception of leukemia, cancer mortality was not different from that of the general population.

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 5kg 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.

Long-term causes of Death after traumatic brain injury

Shavelle RM, Strauss D, Whyte J, Day SM, Yu YL: Long-term causes of death after traumatic brain injury. Am J Phys Med Rehabil 2001;80:510–516. ObjectiveTo determine which causes of death are more frequent in persons with traumatic brain injury (TBI), and by how much, compared with the general population. Our focus was the period beginning 1 yr after injury. DesignSubjects were 2320 Californians with long-term mental disability after a TBI at age 10 yr or more, followed up between 1988 and 1997. The units of study were person-years, each linked to the subject’s age, gender, level of ambulation, time since injury, and cause of death (if any) for the specific year. Observed numbers of cause-specific deaths were compared with numbers expected according to general population mortality rates. ResultsMortality was higher between 1.0 and 5.0 yr postinjury than after 5.0 yr and was strongly related to reduced mobility. Death rates were elevated for circulatory diseases, respiratory diseases, choking/suffocation, and seizures, with seizure deaths being relatively frequent, even among the most ambulatory. ConclusionsDeath rates for several causes are elevated in persons with long-term sequelae of TBI. The increased risk of choking/suffocation should be of interest to caregivers. Life expectancy seems to be reduced, even for patients who are fully ambulatory.

Long-Term Survival of Persons Ventilator Dependent After Spinal Cord Injury

Abstract Background/Objective: Identify factors related to long-term survival, and quantify their effect on mortality and life expectancy. Setting: Model spinal cord injury systems of care across the United States. Study Design: Survival analysis of persons with traumatic spinal cord injury who are ventilator dependent at discharge from inpatient rehabilitation and who survive at least 1 year after injury. Methods: Logistic regression analysis on a data set of 1,986 person-years occurring among 319 individuals injured from 1973 through 2003. Results: The key factors related to long-term survival were age, time since injury, neurologic level, and degree of completeness of injury. The life expectancies were modestly lower than previous estimates. Pneumonia and other respiratory conditions remain the leading cause of death but account for only 31 % of deaths of known causes. Conclusions: Whereas previous research has suggested a dramatic improvement in survival over the last few decades in this population, this is only the case during the critical first few years after injury. There was no evidence for such a trend in the subsequent period.

Change in ambulatory ability of adolescents and young adults with cerebral palsy.

This study aimed to determine the probability that a child with cerebral palsy (CP) will lose or gain ambulatory ability through adolescence and young adulthood. We analyzed retrospectively data from 1987 to 2002 on Californians with CP initially aged 10 years (SD 0.9y; n=7550 [4304 males, 3246 females]) and 25 years (SD 0.8y; n=5721 [3261 males, 2460 females]) who had varying levels of ambulatory ability (initial Gross Motor Function Classification System Levels I‐IV). We used the Aalen‐Johansen estimator to estimate probabilities of transition to other levels of ambulatory ability in the future. Those who walked and climbed stairs without difficulty at age 10 had only a 23% chance of decline (to requiring a handrail to manage stairs, or worse) 15 years later. Those who ambulated with some difficulty but did not use a wheelchair had a significant chance (33%) of improvement (to being able to walk unsteadily alone at least 3m or better) and only a small chance (11%) of becoming non‐ambulatory. Those who used a wheelchair were more likely to lose ambulatory ability (34%) or die (6%). Those who walked and climbed stairs well at age 25 were likely to maintain that ability 15 years later (76%), while those needing support to climb stairs were more likely to lose ability. Improvement in ambulation after age 25 was unlikely. Children and young adults with CP are likely to maintain their ambulatory ability during their next 15 years. Some who ambulate with difficulty at age 10 may improve through adolescence, but those who use a wheelchair are more likely to decline. By age 25 improvement in ambulation is unlikely and decline more likely. Most, however, will not change over the next 15 years.

Prognosis for ambulation in cerebral palsy: a population-based study.

Objectives. To determine independent predictors of ambulation among children with cerebral palsy and to develop a simple tool that estimates the probability that a child will walk. Methods. In a retrospective study of all children with cerebral palsy who were not yet walking at 2 to 3 \batchmode \documentclass[fleqn,10pt,legalpaper]{article} \usepackage{amssymb} \usepackage{amsfonts} \usepackage{amsmath} \pagestyle{empty} \begin{document} \({1}/{2}\) \end{document} years of age, while receiving services from the California Department of Developmental Services during the years 1987–1999, we analyzed medical and functional data obtained annually by Department of Developmental Services physicians and social workers. Using logistic regression analyses, we determined independent predictors of a childs ability to walk well alone at least 20 feet, without assistive devices, by age 6. We then estimated the probabilities of walking at various levels of ability over time, using multistate survival analysis. Results. Of 5366 study subjects, 2295 (43%) were evaluated at age 6; 12.8% could walk independently and 18.4% walked with support. Independent predictors of successful ambulation included early motor milestones such as sitting (odds ratio: 12.5; 95% confidence interval: 5.8–27.2) and pulling to a stand (odds ratio: 28.5; 95% confidence interval: 13.4–60.4) when compared with lack of rolling at age 2, cerebral palsy type other than spastic quadriparesis (odds ratio: 2.2; 95% confidence interval: 1.5–3.1), and preserved visual function (odds ratio: 2.4; 95% confidence interval: 1.1–5.4). Our ambulation charts depict the probability of remaining nonambulatory, transitioning to 1 of 3 possible ambulatory states, or expiring at all subsequent ages through age 14. Conclusion. The ambulation charts provide a simple straightforward way to estimate the probability that a child with cerebral palsy who is nonambulatory at 2 to 3 \batchmode \documentclass[fleqn,10pt,legalpaper]{article} \usepackage{amssymb} \usepackage{amsfonts} \usepackage{amsmath} \pagestyle{empty} \begin{document} \({1}/{2}\) \end{document} years of age will eventually walk with or without support.

Causes of death in remote symptomatic epilepsy

Objective: To determine the causes of death of individuals with developmental disabilities that occur more frequently among those with remote symptomatic epilepsy (i.e., epilepsy occurring in persons with developmental delay or identified brain lesions) than for those without. Methods: The authors compared causes of mortality in persons with (n = 10,030) and without (n = 96,163) history of epilepsy in a California population of persons with mild developmental disabilities, 1988 to 2002. Subjects had traumatic brain injury, cerebral palsy, Down syndrome, autism, or a developmental disability with other or unknown etiology. There were 721,759 person-years of data, with 2,397 deaths. Underlying causes of death were determined from the State of California’s official mortality records. Cause-specific death rates and standardized mortality ratios (SMRs) were computed for those with and without epilepsy relative to subjects in the California general population. Comparisons were then made between SMRs of those with and without epilepsy, and CIs on the ratios of SMRs were determined. Results: Death rates for persons with epilepsy were elevated for several causes. The greatest excess was due to seizures (International Classification of Diseases-9 [ICD-9] 345; SMR 53.1, 95% CI 28.0 to 101.0) and convulsions (ICD-9 780.3; SMR 25.2, 95% CI 11.7 to 54.2). Other causes occurring more frequently in those with epilepsy included brain cancer (SMR 5.2, 95% CI 2.2 to 12.1), respiratory diseases (SMR 1.7, 95% CI 1.2 to 2.5), circulatory diseases (SMR 1.3, 95% CI 1.0 to 1.7), and accidents (SMR 2.7, 95% CI 1.9 to 3.7), especially accidental drowning (SMR 12.8, 95% CI 7.0 to 23.2). Conclusions: Remote symptomatic epilepsy is associated with an increased risk of death. Seizures, aspiration pneumonia, and accidental drowning are among the leading contributors.

Remote symptomatic epilepsy: does seizure severity increase mortality?

Objective: To investigate the excess mortality due to remote symptomatic epilepsy, taking account of frequency and type of seizures. Methods: The authors compared mortality in persons with (n = 8,156) and without (n = 72,526) history of epilepsy in a 1988 to 1999 California population of persons with mild developmental disabilities. Subjects had traumatic brain injury, cerebral palsy, Down syndrome, autism, or no identifiable condition. There were 506,204 person-years of data, with 1,523 deaths. Excess death rates and standardized mortality ratios were computed for the persons in the study with epilepsy, relative to those in the study without epilepsy. Controlled comparisons were made using logistic regression on person-years. Results: Compared to subjects with no epilepsy, the excess mortality was six (deaths per 1,000 persons per year) for persons with a recent (<12 months) history of status epilepticus, five for a recent history of generalized tonic-clonic seizure, three for a recent history of nonconvulsive seizures, and less than one for a history of epilepsy but no recent events. Proportion in remission and excess mortality showed no change over the 12-year study period. Conclusions: Persistent seizures are associated with increased mortality in remote symptomatic epilepsy. Mortality is highest among individuals with status epilepticus or generalized convulsions.

Comparison of survival in cerebral palsy between countries.

‘Comparison of survival in cerebral palsy between countries.’ SIR–The authors of the paper ‘Life expectancy among people with cerebral palsy in Western Australia’1 (see pages 508–15, this issue), kindly allowed us use of their database in order to compare with the large Californian database.2, 3 We begin our comparison at age 5 years because measurements of intellectual level and severity of cerebral palsy (CP) in Western Australia are updated only until that age. Overall survival rate in the Western Australian population is 93% compared with 83% in California. This large disparity is not, however, due to differences in care in the two regions. It arises because the Californian data include nearly all the more severely affected individuals but comparatively fewer mild instances whereas the Western Australian database includes everyone with CP (most of whom are only mildly affected). For a valid comparison, we stratified the population into four intellectual levels (IQ<20; 20–34; 25–49; 50–69) and three severities of motor dysfunction (mild, moderate,and severe), creating 12 groups. When just one factor (severity or IQ) was controlled, the gap in survival rates narrowed considerably. When both were controlled, the survival curves became strikingly similar. Figure 1 illustrates two cases: severe impairment with IQ<20 and with IQ in the range 20–34. Data from the UK study by Hutton and coworkers4 of the ‘three severe disabilities’ group was compared to the Californian database in the same way and found to be remarkably similar. To our knowledge, this is the first controlled comparison of survival in CP between countries. Robert M Shavelle PhD MBA David J Straus PhD FASA Steven M Day MS MAT