Despite universal newborn screening and scientific advances, US children continue to experience disabilities and death from sickle cell disease related causes. From 1999-2003, there were 275 deaths due to sickle cell disease among children through age 19 (USDHHS 2006). Significant variations in survival have been noted by state; these differences have not been explained fully by sociodemographic and health care system characteristics. Persistent variations in access to high quality care and public health services may contribute, but this issue has not been systematically addressed.
This project addresses a major public health concern—how public health systems influence the health of people. We are examining how newborn screening for sickle cell disease influences survival of children with the disease. Findings from the study will inform the development of programs and policies aimed to promote the well-being of children and families with sickle cell disease.
The 2 specific study aims are:
To assess how varying capacities of public health systems influence survival of children with sickle cell disease in the US;
To identify facilitators and barriers for developing public health infrastructure related to newborn screening and follow up for sickle cell disease.
For Aim 1, using a survey, we will relate organizational structures and functions of public health activities organized at the state level with sickle cell outcomes, specifically childhood mortality among states with a minimum number of sickle cell births per year. We also will explore whether variations within states in public health infrastructure are associated with varying mortality by county in selected states with many childhood sickle cell deaths. For Aim 2, key informant interviews will enable us to identify what prompted and facilitated states to build their particular systems of care for newborn screening and follow up for sickle cell disease, barriers identified, and strategies for overcoming these barriers.
This project is one component of the Basic and Translational Research Program grant awarded by the NIH to Johns Hopkins University. Funding is for four years, with the start date of June 2008.
This WCHPC study team is led by Cynthia Minkovitz (PI), with Holly Grason as the collaborating investigator. Our WCHPC colleague Marjory Ruderman, and doctoral candidate Elizabeth Harrison, are providing critical research support. A national project advisory committee has been constituted to review sampling criterion and categorization of states, draft instruments, identification of state respondents, and preliminary findings. In addition, members of the advisory committee will be critical to dissemination of study findings to national and state organizations and diverse constituents.