Congenital Heart Defects and Receipt of Special Education Services.

BACKGROUND: We investigated the prevalence of receipt of special education services among children with congenital heart defects (CHDs) compared with children without birth defects. METHODS: Children born from 1982 to 2004 in metropolitan Atlanta with CHDs (n = 3744) were identified from a population-based birth defect surveillance program; children without birth defects (n = 860 715) were identified from birth certificates. Cohorts were linked to special education files for the 1992-2012 school years to identify special education services. Children with noncardiac defects or genetic syndromes were excluded; children with CHDs were classified by presence or absence of critical CHDs (ie, CHDs requiring intervention by age one year). We evaluated the prevalence of receipt of special education services and prevalence rate ratios using children without birth defects as a reference. RESULTS: Compared with children without birth defects, children with CHDs were 50% more likely to receive special education services overall (adjusted prevalence rate ratio [aPRR] = 1.5; 95% confidence interval [CI]: 1.4-1.7). Specifically, they had higher prevalence of several special education categories including: intellectual disability (aPRR = 3.8; 95% CI: 2.8-5.1), sensory impairment (aPRR = 3.0; 95% CI: 1.8-5.0), other health impairment (aPRR = 2.8; 95% CI: 2.2-3.5), significant developmental delay (aPRR = 1.9; 95% CI: 1.3-2.8), and specific learning disability (aPRR = 1.4; 95% CI: 1.1-1.7). For most special education services, the excess prevalence did not vary by presence of critical CHDs. CONCLUSIONS: Children with CHDs received special education services more often than children without birth defects. These findings highlight the need for special education services and the importance of developmental screening for all children with CHDs.

Use of special education services among children with and without congenital gastrointestinal anomalies.

Our objective was to evaluate the relationship between congenital gastrointestinal anomalies requiring neonatal surgery and neurodevelopmental outcome. Among the children born in metropolitan Atlanta during 1982-2001 who survived to age 1 year (N = 762,824), we identified children with congenital gastrointestinal anomalies via linkage with the Metropolitan Atlanta Congenital Defects Program and children who received special education services via linkage with the Special Education Database of Metropolitan Atlanta. Several modest increases in special education service use were observed among children with isolated congenital gastrointestinal anomalies; no association was statistically significant. Among children with Hirschsprung disease, gastroschisis, esophageal atresia, intestinal malrotation, bowel atresia, or imperforate anus who had multiple anomalies, we observed statistically significant increases in special education service use.

Increased risk of death among children with Lennox-Gastaut syndrome and infantile spasms.

The magnitude and causes of death among a cohort of children with epilepsy were determined. A follow-up study with a population-based cohort of 10-year-old children in the metropolitan Atlanta area with epilepsy was conducted. The National Death Index and linkage to State of Georgia death certificates were used to identify deaths. The authors estimated the expected numbers of deaths by applying mortality rates adjusted by age, race, and sex for the entire state of Georgia to the population for the follow-up period. Among the 688 children who were in the final epilepsy cohort, 64 deaths occurred; 20.6 deaths were expected (mortality ratio adjusted for age, race, and sex = 3.11). The mortality ratios for children with Lennox-Gastaut syndrome and infantile spasms were 13.92 and 11.91, respectively. Children and adolescents with epilepsy, especially those with Lennox-Gastaut syndrome or infantile spasms, have an increased risk of death.

The co-occurrence of autism and birth defects: prevalence and risk in a population-based cohort.

AIM: To estimate the prevalence of major birth defects among children with autism, the prevalence of autism in children with birth defects, and the risk for autism associated with having birth defects. METHOD: Retrospective cohort including all children born in Atlanta, GA, USA, 1986 to 1993, who survived to age 3 years and were identified through Georgia vital records. Children with autism and other developmental disabilities residing in Atlanta at ages 3 to 10 years in 1996 were identified through the Metropolitan Atlanta Developmental Disabilities Surveillance Program. Children with major birth defects through age 6 years were identified by the Metropolitan Atlanta Congenital Defects Program. RESULTS: Birth defects were found among 6% of children with autism (total n=617; 488 males, 129 females) and was associated with a near twofold increased risk for autism overall. However, the risk magnitude and statistical significance varied by type of birth defect. With any type of birth defect, the risk for autism accompanied by intellectual disability or other developmental disabilities was typically higher than the risk for autism alone. A 6:1 to 8:1 male bias was observed among children with autism and a birth defect. INTERPRETATION: Investigation of the association between autism and birth defects is warranted, especially for the role of birth defects in autism among sex-specific or autism subgroups.

Use of special education services by children with orofacial clefts.

BACKGROUND: Our objective was to evaluate the use of special education services by children with orofacial clefts (OFCs). METHODS: We linked the birth certificates of children born from 1982-2001 in five counties of metropolitan Atlanta to a population-based birth defects surveillance system to identify children with OFCs, and to the special education files for the school years 1992-2004 to identify children who used special education services. The special education data contained exceptionalities and services rendered for each school year. Prevalence ratios (PRs) and 95% CIs were calculated. The data were stratified by race/ethnicity, maternal education, type of OFC, and the presence of associated major malformations. In addition, we assessed the age at which special education began and the amount of time spent receiving services. RESULTS: Of the 777 children with OFCs, 201 (26%) were in special education at least 1 year compared with 8% of the children who had no major birth defects, yielding a PR of 3.2 (95% CI: 2.9-3.6). The most common exceptionality or service for children with an OFC was speech and language services. Compared with children with no birth defects, children with an OFC were four times more likely to be in this exceptionality (PR 3.8; 95% CI: 3.3-4.3). After excluding children in speech and language services, children with OFCs were still more likely to use special education services (PR 2.4; 95% CI: 1.7-3.2). CONCLUSIONS: Children with OFCs used special education services more often than children without birth defects. This information can help in planning for future population needs.

A population-based study of the recurrence of developmental disabilities--Metropolitan Atlanta Developmental Disabilities Surveillance Program, 1991-94.

Serious developmental disabilities (DD) are quite common and affect approximately 2% of all school-aged children. The impact of DDs with respect to the need for special education services, medical care and the demand on family members can be enormous. While this impact can be magnified for families with more than one child with a DD, little is known regarding the epidemiology of recurrence of DDs. When the cause of a DD is unknown, genetic counsellors rely on recurrence risk estimates which for DDs are over 10 years old. The objectives of our study were to: (1) assess the contribution of recurrent cases to the prevalence of DDs; (2) provide current, population-based recurrence risk estimates; and (3) examine characteristics of the first affected child as predictors of recurrence. Two population-based data sources were used to identify all children born to the same mother during the period 1981-91 in the five-county metropolitan Atlanta area with at least one of four DDs: mental retardation (MR), cerebral palsy, hearing loss, or vision impairment. Recurrence risk estimates for these DDs ranged from 3% to 7% and were many times higher than the background prevalences. The risk of recurrence of DDs was greatest for MR - approximately eight times greater than the baseline MR prevalence. Isolated mild MR (IQ 50-70) was highly concordant between siblings with MR. Sex, race, and birthweight of the index child, maternal education, and maternal age were not significantly associated with recurrence risk. Further research is needed to investigate the roles of genetic and environmental factors on the recurrence of DDs, particularly isolated mild MR.

Trends in the prevalence of cerebral palsy in a population-based study.

OBJECTIVE: To determine trends in the prevalence of congenital cerebral palsy (CP) over a 16-year period for 1-year survivors using a large, population-based surveillance program. METHODS: We determined birth weight-specific trends in the prevalence of CP in live birth and 1-year survivor cohorts of children in a 5-county metropolitan Atlanta area for the periods from 1975-1977, 1981-1985, and 1986-1991. We ascertained children with CP in metropolitan Atlanta by record review as part of an ongoing developmental disability surveillance program conducted by the Centers for Disease Control and Prevention and the Georgia Department of Human Resources. A total of 110, 262, and 443 cases of congenital CP were identified for the birth years 1975-1977, 1981-1985, and 1986-1991, respectively. Data were analyzed by birth weight, race, subtypes of CP, and whether the CP existed as an isolated disability or was accompanied by another disability. RESULTS: There was a modest increase in the overall prevalence of congenital CP from 1.7 to 2.0 per 1000 1-year survivors during the period from 1975-1991. This trend was primarily attributable to a slight increase in CP in infants of normal birth weight--CP rates in moderately low and very low birth weight infants did not show consistent trends. There was an increase in the proportion of children who had CP and no other disabilities that was most apparent in infants of normal birth weight from 17% in 1975-1977 to 39% in 1986-1991. For children weighing <1500 g, the proportion of children with spastic diplegic CP increased over time (7% of cases in 1975-1977, 36% in 1985-1988, and 32% in 1986-1991). CONCLUSIONS: In the only ongoing population-based study of CP in the United States, there has been a modest increase in the prevalence of CP in 1-year survivors born from 1975-1991. This increase however was seen only in infant survivors of normal birth weight. No change was seen in the trends in CP prevalence in low birth weight and very low birth weight infant based on infant survivors.

Increased mortality in children and adolescents with developmental disabilities.

A population-based cohort of 10-year-old children with mental retardation, cerebral palsy, epilepsy, hearing impairment or vision impairment, who were ascertained at 10 years of age in a previous study conducted in metro Atlanta during 1985-87, was followed up for mortality and cause of death information. We used the National Death Index to identify all deaths among cohort members during the follow-up period (1985-95). We estimated expected numbers of deaths on the basis of actual age-, race- and sex-specific death rates for the entire Georgia population for 1989-91. The objective was to quantify the magnitude of increased mortality and evaluate the contribution of specific disabilities to mortality among children and adolescents with one or more of five developmental disabilities. A total of 30 deaths were observed; 10.1 deaths were expected, yielding an observed-to-expected mortality ratio of almost three to one. The numbers of observed deaths exceeded those of expected deaths, regardless of the number of disabilities present, but the ratios were statistically significant (at the 95% confidence level) only in children with three or more co-existing disabilities. In general, the magnitude of the mortality ratios was directly related to various measures of the severity of the person's disability. An exception to this pattern was the elevated mortality from cardiovascular disease among cohort members with isolated mental retardation (three observed deaths vs. 0.2 expected). The specific underlying causes of death among other deceased cohort members included some that were the putative cause of the developmental disability (e.g. a genetic syndrome) and others that could be considered intercurrent diseases or secondary health conditions (e.g. asthma). Prevention efforts to decrease mortality in adolescents and young adults with developmental disabilities may need to address serious conditions that are secondary to the underlying disability (i.e. infections, asthma, seizures) rather than towards injuries, accidents and poisonings, the primary causes of death for persons in this age group in the general population.