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 (CHP) (SCD) is contracted by the commonwealth of Pennsylvania for the notification and follow-up of newborn screening results for hemoglobinopathies and to provide comprehensive care for children with sickle cell disease, for the 32 county region of Western Pennsylvania and follows 233 patients under the age of 21 years with SCD. Approximately 245,000 persons of African descent reside in this region. Every year over 600 newborns are detected with hemoglobinopathy trait and 12-15 are diagnosed with sickle cell anemia. 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 cultural, health beliefs related or psychosocial reasons there may be social and economic barriers to healthcare that may affect uptake of extended family testing and genetic counseling.

Goals and Objectives

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. Objectives of this project are:

1. Training: to enhance knowledge and awareness of counseling and education for SCD 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 of the impact of screening and counseling on those identified with a hemoglobinopathy.

Methodology

The 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. A cooperative community based network involving all the major providers of care for patients with SCD in Western Pennsylvania has been established. Comprehensive care is being further enhanced by means of intensive case management and structured telephone-delivered reinforcement of health behaviors. Follow-up of sickle cell or other abnormal hemoglobin trait is being further enhanced by 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.

Evaluation

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

1. Impact on health outcomes such as effectiveness of follow-up and counseling of individuals with sickle cell disease or trait, compliance with recommended treatment, retention of health messages and impact of counseling on personal lives of those counseled
2. Effectiveness of the project in meeting the learning needs of individuals who participate in courses and other training activities
3. Effectiveness of the overall organizational and administrative structure and various components of the project consortium.

Experience to Date

1. Confirmatory testing, initiation of penicillin prophylaxis and comprehensive care for 100% of children diagnosed to have SCD on the newborn screen in western Pennsylvania since 1992, 96.7% of whom remain in comprehensive care.
2. Since September 2002, 97% of families with a newborn with sickle cell trait have been successfully notified by mail, 50% have also been contacted by telephone. Among the patients contacted, 92% received counseling over the phone, 73% have received a special educational video on hemoglobinopathy trait, 48 % expressed interest in formal genetic counseling and 25% have already scheduled appointments for genetic counseling.
3. Intensive case management and telephone delivered reinforcement of health messages has been implemented. Of 46 patients who were found to be noncompliant with comprehensive care visits for 2 years or more 44(95%) have been contacted and 22(48%) have already been seen in comprehensive care clinic.
4. Comprehensive databases on individuals with sickle cell disease or trait have been adapted for education, intensive case management and to facilitate communication among healthcare providers
5. Implementation of a transition program to adult sickle cell program.

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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