Purpose

This project is aimed at improving the processes which link newborn screening for sickle cell disease (and related hemoglobinopathies and traits) to subsequent care including family support, patient and carrier education, hematology assessment, and genetic counseling.

Challenges

The main purpose of sickle cell disease newborn screening is intervention to reduce mortality (through immediate penicillin prophylaxis and education). Screening also allows identification and education of families with sickle cell trait and other hemoglobinopathies. When a newborn with sickle cell disease is identified by the New England Newborn Screening Program (NENSP), the program contacts the primary care provider to arrange confirmatory testing and initiation of penicillin therapy. One significant challenge had been “closing the loop” to assure that patients identified by newborn screening make it to a hematologist. Recent clarifications of HIPAA regulations/public health exemptions have made this tracking more feasible. While Massachusetts, through NENSP, offers in-home counseling to all families of babies identified with S trait, only one in three families takes advantage of the offered counseling. Massachusetts has no formal linkage between the Newborn screening program activities and subsequent care. Unlike many other states, MA has no chapter of the SCDAA, and community support agencies are not directly involved with follow up of newborn screening.

Goals and Objectives

1. Provide ongoing provider training
   1. materials for community health center nurses, health educators;
   2. materials for pediatricians, family practitioners;
   3. materials for community support groups.
2. Refine an ongoing feedback system from hematology providers to NENSP for patients identified by newborn screening with sickle hemoglobinopathies and related disorders, termed “closing the loop.”
3. .Establish close interactions to bridge the gap among specialists, generalists, community groups, newborn screening and reference labs, and the National Coordinating Center.
   A Regional Conference at the end of Grant Year I will be a major activity for this purpose.
4. Provide direct services to clients via community agencies.

Methodology

The project is coordinated by the New England Pediatric Sickle Cell Consortium (NEPSCC), which represents all of the pediatric hematology centers in the state. Project Director Ms. Ryan is the facilitator for the NEPSCC, and a leader in providing educational materials and treatment guidelines for sickle cell diagnosis and treatment in Massachusetts.

1.   
   1. For provider education about newborn screening, we will use the curriculum (Powerpoint presentation and printed materials) developed during the current project year.
   2. Treatment guidelines/pathways of the NEPSCC will be used for training about sickle cell treatment.
2. For feedback from providers to NENSP, we will use a fax notification system developed during the present grant year. We will devise a scoring system to report to MCHB and State Title V representatives the fraction of patients who made it to optimum care. 2002 and 2003 data will be used as a pilot during project year 1, with the aim of collecting further retrospective data (before 2002) and prospective data (2004 and beyond) in year two of this project.
3. The Executive Committee of the project brings together stakeholders to improve interactions. Quarterly meetings are planned. In addition, a regional conference is planned for the end of project year 1, to discuss the relationship of sickle cell newborn screening and subsequent care issues, with experts and providers from New England, and experts from outside the region.
4. We will provide direct support for the community outreach and patient service activities of the Community Sickle Cell Support Group, as a collaborative effort directed by Dr. Oyeku.

Evaluation

Goal IA. We will measure how many practitioners are reached with the newborn screening curriculum, and what they know and what they have learned using the knowledge/attitude surveys developed in the current grant year.

Goal IB. We will assess how many centers have adopted (or considered adopting) the treatment guidelines promulgated by NEPSCC or by other groups for care of children with sickle cell disease.

Goal II. The key measure of this goal is what fraction of children identified by newborn screening with sickle cell disease have made it to appropriate care. A subsidiary measure is to attempt to understand process issues in this measure – can we account for each screened child? If not, why not?

Goal III. We will report the attendance at coordination activities, and at the planned regional conference. A key aim of year 2 evaluations, if funded, will be to take stock of recommendations from the conference and determine how these might be implemented locally, within the state and region, and nationally.

Goal IV. We will report on the number of clients served by the community agency collaborators, and on the types of services provided.

Experience to Date

The applicants of this proposal have substantial experience in key aspects.

• This center is a current MCHB grantee for sickle cell/newborn screening. Project Director Ms. Ryan developed the curriculum and handouts for our current education effort at community health centers. Evaluation committee head Dr. Oyeku developed the knowledge surveys used.
• The MCHB-funded “Boston Center for Genetic Blood Diseases,” (E. Neufeld, PI, 2003-‘06) links expert hematologists with community providers and a national support group (Cooley’s Anemia Foundation) for comprehensive, coordinated, care in thalassemia.
• The New England Pediatric Sickle Cell Consortium develops ‘pathways’ for care of specific medical issues in sickle cell disease, and provides care for more than 500 sickle cell patients.
• Project co-Directors Drs. Space, Sprinz, Heeney, Neufeld, are pediatric hematologists expert in sickle cell care and family support, with specific experience in the issues of feedback to newborn screening based on the current grant year. Dr. Oyeku is an HRSA-funded health services fellow.
• NENSP performs over 80,000 newborn hemoglobinopathy screens each year, identifying approximately 30-40 patients with sickle disorders, and hundreds with S trait annually.
• Community outreach collaborator, the Community Sickle Cell Support Group has more than ten year’s experience providing support to patients and families with sickle cell disease.

Text of Annotation

The purpose of this project is to unite the providers and community resources involved in newborn screening and care for patients and families with sickle hemoglobinopathies. The New England Pediatric Sickle Cell Consortium will coordinate outreach, education and evaluation. NEPSCC will use an “educate the educator” strategy, and offer the curriculum (as conferences, outreach teaching sessions, and written materials) to members of the Mass. League of Community Health Centers, community pediatricians, and support groups in the community. With the Newborn Screening Program, we will develop a feedback system to allow the public health system to assess the fraction of infants identified with sickle cell disease who achieve the recommended goal of seeking care with comprehensive sickle cell treatment centers.

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