SB is a monitored condition in birth defects surveillance networks across the U.S. and in most developed countries. A public health emphasis on prevention, early identification, and multidisciplinary treatment has been associated with a decreased prevalence and improved outcomes in individuals with SB in the US. Despite the decline in SB births since the implementation of folic acid fortification of cereals and wheat grains in the US, there are still substantial numbers of SB births that occur each year. Although much is known about the management of SB and the multiple co-morbid conditions and complications that occur in affected individuals, there are few systematic, longitudinal studies on population-based cohorts of children or adults. In 2003, the Centers for Disease Control and Prevention (CDC), the Agency for Healthcare Research and Quality, and the Spina Bifida Association of America hosted a multidisciplinary meeting to review the available research on conditions impacting the lives of individuals with SB and to develop a national research agenda. The purpose of this agenda was to identify research priorities in 16 of the most important co-occurring conditions in SB and advance research efforts to improve the quality of life of those affected, as well as to guide policy makers, educators, clinicians, and caregivers in the development of practice parameters, public policy, and public health activities that address the needs of this population. The hypothesis guiding this research is that population-based surveillance and integrated research programs are effective in determining the prevalence of disorders and establishing data on health outcomes and the types of care for these disorders. Such population-based surveillance for individual disorders is an important first step to understanding the full range of clinical outcomes for affected people. The availability of longitudinal data on these cohorts provides an opportunity to document and assess the natural history of the condition and the effects of various diagnosis and treatment strategies. The project proposes that Arizona contribute population-based data on 3-5 year old children with SB to achieve the following specific aims:
Specific Aim 1 : Generate a population-based dataset that can describe the natural history of SB, co-morbid conditions, and related complications as baseline data in a longitudinal cohort;
Specific Aim 2 : Describe the medical and social services received by individuals with SB and their families, including knowledge of prevention, and whether these vary by race/ethnicity and socioeconomic status.
These specific aims will be achieved through completion of the following research objectives: 1. In collaboration with CDC, refine and finalize the protocol for population-based surveillance and long-term follow up of individuals with SB, building on the results of a CDC-funded pilot project currently underway.2. Identify a sample of children affected by SB using state based Birth Defects Surveillance Systems.3. Recruit children and their families for participation in the study.4. Gather data and information using a combination of methods, including interviews, survey, records abstraction, and neuropsychological testing.5. Assess the effectiveness of data collection methods.6. Assess the outcomes of the project.
