Sheree L. Boulet

Trends in the Prevalence of Developmental Disabilities in US Children, 1997–2008

OBJECTIVE: To fill gaps in crucial data needed for health and educational planning, we determined the prevalence of developmental disabilities in US children and in selected populations for a recent 12-year period. PARTICIPANTS AND METHODS: We used data on children aged 3 to 17 years from the 1997–2008 National Health Interview Surveys, which are ongoing nationally representative samples of US households. Parent-reported diagnoses of the following were included: attention deficit hyperactivity disorder; intellectual disability; cerebral palsy; autism; seizures; stuttering or stammering; moderate to profound hearing loss; blindness; learning disorders; and/or other developmental delays. RESULTS: Boys had a higher prevalence overall and for a number of select disabilities compared with girls. Hispanic children had the lowest prevalence for a number of disabilities compared with non-Hispanic white and black children. Low income and public health insurance were associated with a higher prevalence of many disabilities. Prevalence of any developmental disability increased from 12.84% to 15.04% over 12 years. Autism, attention deficit hyperactivity disorder, and other developmental delays increased, whereas hearing loss showed a significant decline. These trends were found in all of the sociodemographic subgroups, except for autism in non-Hispanic black children. CONCLUSIONS: Developmental disabilities are common and were reported in ∼1 in 6 children in the United States in 2006–2008. The number of children with select developmental disabilities (autism, attention deficit hyperactivity disorder, and other developmental delays) has increased, requiring more health and education services. Additional study of the influence of risk-factor shifts, changes in acceptance, and benefits of early services is needed.

Trends in the postfortification prevalence of spina bifida and anencephaly in the United States

BACKGROUND The prevalence of NTDs in the US declined significantly after mandatory folic acid fortification; however, it is not known if the prevalence of NTDs has continued to decrease in recent years relative to the period immediately following the fortification mandate. METHODS Population-based data from 21 birth defects surveillance systems were used to examine trends in the birth prevalence of spina bifida and anencephaly during 1999-2000, 2001-2002, and 2003-2004. Prevalence data were stratified by non-Hispanic White, non-Hispanic Black, and Hispanic race or ethnicity. Prevalence ratios were calculated by dividing the birth prevalences during the later time periods (2001-2002 and 2003-2004) by the birth prevalences during 1999-2000. RESULTS During 1999-2004, 3,311 cases of spina bifida and 2,116 cases of anencephaly were reported. Hispanic infants had the highest prevalences of NTDs for all years. For all infants, the combined birth prevalences of spina bifida and anencephaly decreased 10% from the 1999-2000 period to the 2003-2004 period. The decline in spina bifida (3%) was not significant; however the decline in anencephaly (20%) was statistically significant. CONCLUSIONS While the prevalences of spina bifida and anencephaly in the United States have declined since folic acid fortification in the food supply began, these data suggest that reductions in the prevalence of anencephaly continued during 2001-2004 and that racial and ethnic and other disparities remain.

Health Care Use and Health and Functional Impact of Developmental Disabilities Among US Children, 1997-2005

OBJECTIVE To present nationally representative estimates of health-related limitations, needs, and service use among US children with and without developmental disabilities (DDs). DESIGN Retrospective analysis of data from a sample of US households from the 1997-2005 National Health Interview Surveys. PARTICIPANTS Children aged 3 to 17 years (n = 95 132). MAIN OUTCOME MEASURES Parents or other knowledgeable adults reported on their childrens DDs, health needs, and use of health and education services. Developmental disabilities included attention-deficit/hyperactivity disorder, autism, blindness, cerebral palsy, deaf/a lot of trouble hearing, learning disability, mental retardation, seizures, stuttering/stammering, and other developmental delay. RESULTS Among children with 1 or more DDs, prevalence estimates for limitations in movement (6.1%), needed help with personal care (3.2%), needed special equipment (3.5%), received home health care (1.4%), and regularly took prescription medication(s) (37.5%) were 4 to 32 times higher than for children without DDs. Children with DDs were 2 to 8 times as likely to have had more than 9 health care visits (14.9%), received special education (38.8%), had a surgical or medical procedure (7.5%), and recently visited a medical specialist (23.9%), mental health professional (26.6%), therapist/allied health professional (19.6%), and/or emergency department (10.3%). Effects were generally stable during the study interval and independent of age, race, sex, and family income. Cerebral palsy, autism, mental retardation, blindness, and deafness/a lot of trouble hearing were associated with the highest levels of health and functional impact indicators. CONCLUSIONS Developmental disabilities profoundly affect childrens health and functioning. These data can inform evidence-based targeted prevention strategies for minimizing functional limitations and lifetime disability. Additional study of unmet needs and access to care is needed.

Concurrent Medical Conditions and Health Care Use and Needs among Children with Learning and Behavioral Developmental Disabilities, National Health Interview Survey, 2006-2010.

Studies document various associated health risks for children with developmental disabilities (DDs). Further study is needed by disability type. Using the 2006-2010 National Health Interview Surveys, we assessed the prevalence of numerous medical conditions (e.g. asthma, frequent diarrhea/colitis, seizures), health care use measures (e.g. seeing a medical specialist and >9 office visits in past year), health impact measures (e.g. needing help with personal care), and selected indicators of unmet health needs (e.g. unable to afford needed prescription medications) among a nationally representative sample of children ages 3-17 years, with and without DDs. Children in four mutually exclusive developmental disability groups: autism (N = 375), intellectual disability (ID) without autism (N = 238); attention-deficit/hyperactivity disorder (ADHD) without autism or ID (N = 2901); and learning disability (LD) or other developmental delay without ADHD, autism, or ID (N = 1955); were compared to children without DDs (N = 35,775) on each condition or health care measure of interest. Adjusted odds ratios (aORs) were calculated from weighted logistic regression models that accounted for the complex sample design. Prevalence estimates for most medical conditions examined were moderately to markedly higher for children in all four DD groups than children without DDs. Most differences were statistically significant after adjustment for child sex, age, race/ethnicity, and maternal education. Children in all DD groups also had significantly higher estimates for health care use, impact, and unmet needs measures than children without DDs. This study provides empirical evidence that children with DDs require increased pediatric and specialist services, both for their core functional deficits and concurrent medical conditions.

Perinatal outcomes of twin births conceived using assisted reproduction technology: a population-based study†

BACKGROUND Approximately 18% of multiple births in the USA result from assisted reproduction technology (ART). Although many studies comparing ART and naturally conceived twins report no difference in risks for perinatal outcomes, others report slight to moderate positive or protective associations. METHODS We selected twin deliveries with and without indication of ART from Massachusetts live birth-infant death records from 1997 to 2000 linked to the US ART surveillance system. The sample was restricted to deliveries by mothers with increased socioeconomic status, private health insurance and intermediate/plus prenatal care use. Our final sample included 1446 and 2729 ART and non-ART twin deliveries, respectively. Odds ratios (OR) for associations between ART and perinatal outcomes were adjusted for maternal demographic factors, smoking, prenatal care and hospital care level. RESULTS ART twin deliveries were less likely than non-ART to be very preterm (adjusted OR 0.75; 95% confidence interval 0.58-0.97) or include a very low birthweight (<1500 g) infant (0.75; 0.58-0.95) or infant death (0.55; 0.35-0.88). In stratified analyses, these findings were observed among primiparous deliveries, but there were no risk differences among multiparous ART and non-ART twin deliveries. CONCLUSIONS ART treatment was not a risk factor for adverse perinatal outcome, and risks for several outcomes were somewhat lower among ART twin deliveries. Nonetheless, ART is strongly associated with twinning and twins remain a high-risk group, relative to singletons. Promoting singleton gestation in assisted conception is an important strategy for reducing adverse outcomes.

Health of Children 3 to 17 Years of Age With Down Syndrome in the 1997-2005 National Health Interview Survey

OBJECTIVE. This study provides population-based estimates of recent medical conditions, concurrent developmental disorders, and health impact and utilization indicators for US children with and without Down syndrome. METHODS. The sample included children 3 to 17 years of age in the 1997–2005 National Health Interview Survey Child Sample Core and specifically included 146 children with Down syndrome, 604 children with mental retardation but without Down syndrome, and 95 454 children without either condition reported. Developmental and medical conditions, health status, and service use were reported by parents or other knowledgeable caregivers. RESULTS. After adjustment for demographic factors, children with Down syndrome had higher odds, compared with children without mental retardation, of recent food/digestive allergy, frequent diarrhea/colitis, ≥3 ear infections in the previous year, very recent head/chest cold, and developmental disabilities other than mental retardation. They had increased odds that approached significance for recent seizures, very recent stomach/intestinal illness, and asthma. They had substantially higher rates (threefold or higher, compared with children without mental retardation) for nearly all health impact and health and special education service use measures. Of note, >25% of children with Down syndrome needed help with personal care, regularly took prescription medications, had recently seen a medical specialist, and received physical therapy or related therapy. The comparison group with mental retardation without Down syndrome represented many children with multiple serious disabilities who also had high rates of medical conditions and high levels of health impact and service use. CONCLUSION. These findings provide empirical, population-based data to inform guidelines for frequent monitoring and support for children with Down syndrome.

Heme oxygenase-1 gene promoter polymorphism is associated with reduced incidence of acute chest syndrome among children with sickle cell disease.

Sickle cell disease is a common hemolytic disorder with a broad range of complications, including vaso-occlusive episodes, acute chest syndrome (ACS), pain, and stroke. Heme oxygenase-1 (gene HMOX1; protein HO-1) is the inducible, rate-limiting enzyme in the catabolism of heme and might attenuate the severity of outcomes from vaso-occlusive and hemolytic crises. A (GT)(n) dinucleotide repeat located in the promoter region of the HMOX1 gene is highly polymorphic, with long repeat lengths linked to decreased activity and inducibility. We examined this polymorphism to test the hypothesis that short alleles are associated with a decreased risk of adverse outcomes (hospitalization for pain or ACS) among a cohort of 942 children with sickle cell disease. Allele lengths varied from 13 to 45 repeats and showed a trimodal distribution. Compared with children with longer allele lengths, children with 2 shorter alleles (4%; ≤ 25 repeats) had lower rates of hospitalization for ACS (incidence rate ratio 0.28, 95% confidence interval, 0.10-0.81), after adjusting for sex, age, asthma, percentage of fetal hemoglobin, and α-globin gene deletion. No relationship was identified between allele lengths and pain rate. We provide evidence that genetic variation in HMOX1 is associated with decreased rates of hospitalization for ACS, but not pain. This study is registered at www.clinicaltrials.gov as #NCT00072761.

Children with Orofacial Clefts: Health-Care Use and Costs Among a Privately Insured Population

Objectives. Orofacial clefts are common birth defects that often require multiple surgeries and medical treatments during childhood. We used healthcare insurance claims data to estimate health-care expenditures for infants and children ≤10 years of age with an orofacial cleft. Methods. The data were derived from the 2000–2004 MarketScan® Commercial Claims and Encounters databases, which include person-specific information on health-care use, expenditures, and enrollment for approximately 50 large employers, health plans, and government and public organizations. Health insurance claims data from 821,619 children ≤10 years of age enrolled in employer-sponsored plans during 2004 were analyzed. Expenditures for inpatient admissions, outpatient services, and prescription drug claims were calculated for children with and those without an orofacial cleft. Results. The difference in annual mean costs (i.e., incremental costs) between children aged 0 through 10 years with an orofacial cleft and those without an orofacial cleft was

Health Care Expenditures for Infants and Young Children with Down Syndrome in a Privately Insured Population

13,405. The mean and median costs for children ≤10 years of age with an orofacial cleft were eight times higher than for children of the same age without an orofacial cleft. Mean costs for infants with a cleft and another major, unrelated defect were 25 times higher than those for an infant without a cleft, and five times higher than for infants with an isolated cleft. Conclusion. These findings document substantially elevated medical care costs for privately insured children with an orofacial cleft. Additional study of the economic burden associated with this condition should include a broader range of economic costs.

Mode of delivery and birth outcomes of macrosomic infants

OBJECTIVE To use health care insurance claims data from a privately insured population to estimate health care use and expenditures for infants and children aged 0 to 4 years with Down syndrome. STUDY DESIGN Data from the 2004 Medstat MarketScan database were used to estimate medical care use and expenditures related to inpatient admissions, outpatient services, and prescription drug claims for children with and those without Down syndrome. Costs were further stratified by the presence or absence of a congenital heart defect (CHD). RESULTS The mean medical costs for infants and children with Down syndrome were