Purpose

The primary purpose of the Sickle Cell Disease Association of America-Mobile Chapter (SCDAA-MC) community based newborn screening project is to establish an accessible, comprehensive system of sickle cell counseling services aimed at educating sickle cell patients and their families about sickle cell disease. The project intends to promote patient advocacy and continued disease education through an integrated system of activities and services that are facilitated by the SCDAA-MC. The community-based newborn screening project will improve the quality of educational services offered to clients during counseling sessions, thereby developing a network of resources to support those affected with sickle cell trait or disease.

The primary purpose of the Sickle Cell Disease Association of America-Mobile Chapter community-based newborn screening project is to establish an accessible, comprehensive system of sickle cell counseling services aimed at educating sickle cell patients and their families about sickle cell disease. Evaluation of the program will be performed through a system of counseling assessments, pre and post-tests, age-specific/ data questionnaires.

Challenges

In Mobile County many primary care physicians have limited knowledge of sickle cell disease and other abnormal hemoglobinopathies. Consequently, many of these physicians are not aware of appropriate resources when making referrals for related medical or support services. Several states have effective newborn screening programs for infants diagnosed with the disease, but marked deficiencies exist in programs aimed at infants diagnosed with sickle cell trait and other hemoglobin variants. In most of these programs, parents are notified of the results for their infant’s abnormal test, but they often neglect to seek additional education, testing, and sickle cell counseling. As a result, many parents are rarely tested or counseled. Currently, there is no program in existence that provides test results and their implications to the adolescent as he/she approaches childbearing age. Since the 1960’s a large number of Americans have been tested for sickle cell disease and trait. However, these testing programs are poorly planned and implemented which subsequently leads to apprehension and confusion.

Goals and Objectives

The goal of the SCDAA-MC newborn screening project is to provide a seamless system of sickle cell counseling services to individuals and families diagnosed with positive newborn screening results. This goal will be met by utilizing the following objectives. Objective 1: To provide non-directive, confidential counseling to parents of newborns and others with positive test results. Objective 2: To devise and implement a tracking system focused upon the adolescent population who yielded positive trait results but had not received any subsequent counseling. Objective 3: To utilize data gathered through counseling sessions as a potential source for case management referrals. Objective 4: To provide quality sickle cell testing conducted by two laboratories, directly linked for the purpose of establishing an accurate diagnosis of hemoglobinopathies.

Methodology

The initial step in the provision of counseling is the actual detection of the disease or trait. Once a definitive diagnosis is established, the parents should be provided with appropriate education and counseling regarding the specific type of sickle cell disease. A copy of the diagnostic information should be provided for the parents and made available to health professionals involved in the infant’s care. After a diagnosis has been established, the next step involves decision-making. This process involves providing the family with appropriate information that will allow them to make informed reproductive choices in the future.

Since mandatory newborn screenings in the State of Alabama did not begin until 1988, no data currently exist to support the belief that parents whose children were identified at birth with sickle cell trait were informed of their trait status. Case management services are made available to all diseased clients registered with the community program. The community program will staff a pediatric and adult case manager to provide services for clients diagnosed with sickle cell disease. The case managers are knowledgeable of available resources that can possibly improve the quality of life for these patients.

Since Two laboratories will be directly linked to provide detailed laboratory studies for establishing an accurate diagnosis of hemoglobinopathies. Studies performed at these two laboratories will be centrally coordinated through the laboratory at the SCDAA-MC. A medical laboratory technician will be responsible for coordinating the program under the direction of a medical physician. A counseling session will provide basic trait information for the adolescent and a review for the parent(s). In addition, a survey will be administered in an effort to gauge parental behavior patterns.

Case Managers will complete a data questionnaire and age-specific needs assessment for each client enrolled in the community-based program. The data retrieved will be used to determine age-specific challenges facing sickle cell clients. Presently, laboratory screenings are conducted utilizing on-site alkaline and hemoglobin electrophoresis testing. Additional testing procedures such as HPLC, CBC, KB-stain and DNA analysis can be performed at laboratory affiliates to provide comprehensive hemoglobin diagnosis.

Experience to Date

The SCDAA-MC was founded in 1972 in an effort to provide services for individuals with sickle cell disease. The community-based program has provided counseling and education since its inception. The SCDAA-MC formed a partnership with the University of South Alabama Comprehensive Sickle Cell Center (USA-CSCC) in 1981 and has maintained that relationship over the past 21 years. The Sickle Cell Educator/Counselor training program is offered on a quarterly basis, and is one of only four programs sanctioned by the National Sickle Cell Disease Association to provide this type of training. The SCDAA-MC and the University of South Alabama Comprehensive Sickle Cell Center have collaborated to train health care providers in urban and rural areas for service delivery, thereby targeting newborns and others affected with sickle cell disease. The SCDAA-MC community program continues to provide sickle cell counseling for those individuals identified as “at risk”. Counseling, in addition to other services, are provided to all newborns and their families identified with positive results through the Alabama Newborn Screening Program.

In a one-year period (1999-2000), counseling services were provided for 118 parents. During the same time period, SCDAA-MC case managers provided 1, 809 interventions for clients with sickle cell disease. Client interventions consisted of home visits, clinic visits, hospital visits, and office visits. The SCDAA-MC also completed 3,897 hemoglobin screenings during 1999-2000.

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