Purpose

To enhance support, including counseling and education to patients and families of infants screened positive for SCD or as carriers of sickle cell or other abnormal hemoglobin.

Challenges

The Pediatric Comprehensive Sickle Cell Program at Children’s Hospital of Pittsburgh actively follows 233 patients under the age of 21 years with sickle cell disease (SCD). The program is contracted by the commonwealth of Pennsylvania for the notification of abnormal newborn screening results suggestive of SCD and to provide comprehensive care for children with these diseases and for the notification of sickle cell trait, for the 32 county region of Western Pennsylvania. Approximately 245,000 persons of African descent reside in this region. Every year over 600 individuals are found to have abnormal newborn screen result for hemoglobinopathy trait and 12-15 are diagnosed with sickle cell anemia. Since 1992, 100% of newborns detected to have SCD on the newborn screen have been started on penicillin. However, less than10% of families notified of sickle cell or other hemoglobinopathy trait in their newborn have taken up the offer of formal counseling and extended family testing. Among children with SCD 70% receive health insurance through one of the managed care plans administered through the PA state Medical assistance. Thus, in addition to other cultural, health beliefs related or psychosocial reasons there may be social and economic barriers to healthcare that may contribute to this poor uptake of extended family testing and genetic counseling.

Goals and Objectives

Specific objectives of this project are:

1. Training: to enhance awareness of and importance of counseling and education for sickle and other major hemoglobinopathies among primary care providers in Western Pennsylvania
2. Family services:
   1. to enhance education and counseling of families with a child identified through newborn screening to have SCD or being a carrier for sickle cell or other abnormal hemoglobin trait and
   2. to provide psychosocial support, intensive case management and telephone delivered reinforcement of health related behaviors with a view to improving compliance with comprehensive and preventive care.
3. Evaluation: to determine the impact of screening and counseling on those identified with a hemoglobinopathy.

The long term goal of this project is to enhance the support, including counseling and education to patients and families of infants screened positive for SCD or as carriers of sickle cell or other abnormal hemoglobin.

Methodology

The Children’s Hospital of Pittsburgh (CHP) SCD program will build upon its experience with follow up, testing, psychosocial support of abnormal newborn screen for SCD and the provision of comprehensive care of infants identified by newborn screening through childhood and adolescence. Currently, the team has three pediatric hematologists, a nurse coordinator, a nurse practitioner a social worker, a part time clinical psychologist and part-time data manager. We propose to enhance the provision of services in the context of a cooperative community based network involving all the major providers of care for patients with SCD in Western Pennsylvania. Comprehensive care is sought to be enhanced by using an approach of intensive case management and structured telephone-delivered reinforcement of health behaviors. Follow-up of sickle cell or other abnormal hemoglobin trait is sought to be enhanced by institution of intensive case management, use of videotape/CD-ROM delivered trait information, professional genetic counseling and integration of primary care providers and community based organizations into systems of care. We anticipate that this approach will reduce the effect of economic, social and cultural barriers and improve uptake of extended family testing and genetic counseling. Professional counseling and psychosocial support will thus be made more accessible and culturally acceptable to families. A modular curriculum of instruction and certification for sickle cell counselor-case workers will be developed and implemented. Elements of this curriculum will be used for sensitizing certified genetic counselors to SCD and to raise awareness of other primary care providers and health professionals regarding the importance of counseling and support for families with a newborn diagnosed with SCD or trait.

Evaluation

The overall objective for the evaluation is to determine the effectiveness of this model in the planning, development, and delivery of services to enhance the support, including counseling and education to patients and families of infants screened positive for SCD or as carriers of sickle cell or other abnormal hemoglobin.

The efficiency and effectiveness of the project will be monitored in the following ways:

1. Effectiveness of the overall organizational and administrative structure of the consortium
2. Measures of compliance with the recommended treatment protocols
3. Evaluation of completeness of follow up of children diagnosed with sickle cell trait including
4. Evaluation of impact of screening and counseling of families with a child with sickle cell trait
5. Evaluation of effectiveness of education of primary care providers as assessed by follow up questionnaires and evaluation by PCPs

Experience to Date

1. Confirmatory testing, initiation of penicillin prophylaxis and comprehensive care for 100% of children diagnosed to have sickle cell anemia on the newborn screen in western Pennsylvania over the last 10 years.
2. 96.7% patients with SCD with sickle cell seen at CHP SCD since 1992 remain in comprehensive care.
3. Successful notification of sickle cell trait status to 97% of families with a newborn detected to have sickle cell trait.
4. Maintenance of comprehensive database records on all sickle cell patients and persons with sickle cell trait detected on newborn screen or seen at CHP since 1992.
5. Implementation of a transition program with the adult sickle cell program at UPMC, Pittsburgh.

Text of Annotation

This community based cooperative project seeks to enhance support, including counseling and education to patients and families of infants screened positive for SCD or as carriers of sickle cell or other abnormal hemoglobin. The program will extend and coordinate care by:

1. Improving support and education by intensive case management and telephone delivered reinforcement of health behaviors to enhance compliance with comprehensive care among patients detected to have SCD on newborn screening.
2. To enhance support and genetic counseling for families with a newborn detected to have a sickle cell or other abnormal hemoglobin trait by intensive case management enhanced education and professional genetic counseling.
3. Improving awareness of the importance of sickle cell trait counseling and education among primary care providers in western Pennsylvania.

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