The purpose of this project has been to reduce mortality and morbidity resulting from sickle cell disease and other hemoglobinopathies. The focus is to improve screening, education, treatment and follow-up especially for sickle cell disease patients. In 1972, the Mississippi legislature enacted Chapter 24, Section 41-24-1 of the Mississippi Code, which gives the State Department of Health the authority to promulgate rules and regulations necessary for the establishment of a program for testing preschool age children for sickle cell anemia and sickle cell trait. In 1985, testing for phenylketonuria and hypothyroidism were mandated, and in 1987, other hemoglobinopathies and galactosemia were instituted. In 1987, 41,865 newborns were screened for all four disorders, representing 99.6 percent of all live births in the state that year. Hemoglobinopathies, including the sickle cell and thalassemia variants, are tested for by electrophoresis and other hematological tests.
In 1982 with hospitals joining the program on a volunteer basis. The filter screening was collected by each hospital enrolled in the program and sent to the Tennessee Department of Health and Environment Laboratory. Special legislation was necessary in order to establish a contract between the two states. Mississippi has an average birth rate of 42,000 live births per year. Regional screening is much more cost effective and when a laboratory handles a minimum of 50,000 to 100,000 samples per year the results are more accurate.
The Mississippi State Department of Health bills the hospital $20.00 for each child screened and reimburses the Tennessee laboratory. The fee includes administrative and follow-up costs. If the specimen is repeated due to technical error or lack of information on the lab slip, the hospital is charged an additional $20.00 for the repeat test.
The Sickle Cell Satellite Clinics are in Greenville, Pontotoc, and in south central Mississippi. Each clinic is staffed by a physician, a nurse and a social worker. Counseling and educational literature are provided to each patient. These clinics provide the majority of tertiary care and also provide other benefits such as an opportunity for clinic physicians and local physicians to interact and form patient centered working relationships. The clinic physicians educate the local physicians on current sickle cell treatment.
State Sickle Cell project coordinators are responsible for follow- up on all newborn screening repeats and confirmatory tests; case management of all patients; chart review; provision of education, counseling, and referral as appropriate; and assisting the central or state program office by alerting them to needs in the field, such as the need for hospital in-service in each district, and public health and general public questions.
The first two years of Mississippi's hemoglobinopathy statistical results appeared to result in numbers that were higher than the national average for sickle cell trait and disease, but after evaluation of five years data the following results were observed: FS and FSC = 1/331, FAS = l/14.5, FAC = 1/48, and FAC = 1/2359. Of the number screened with abnormal hemoglobins, 92% were located and had confirmatory tests obtained. Further testing through University of Mississippi Medical Center Sickle Cell Clinic, Mid- South Sickle Cell Center in Memphis, Tenn., and the Sickle Cell Center of Mobile, Alabama from 1989 through 1993 revealed the following: Sickle/Beta+Thal = 21, Sickle/Beta O Thal - 10, Sickle/Delta Beta Thal = 4, C/Beta + Thal = 9, C/Beta O Thal = 2.
The Mississippi State Department of Health Genetics Program has worked closely with the Division of Vital Records over the past five years in order to investigate the number of deaths related to sickle cell disease. 99 deaths were reported in Mississippi from 1988 through 1992 related to sickle cell disease; 24 of these deaths were under twenty one years of age, and 10 of these deaths were under one year of age.
The Newborn Screening Program in Mississippi includes testing for phenylketonuria (PKU), hypothyroidism (T4, TSH), galactosemia (Gal), and hemoglobinopathies (Hgb). With approximately 42,000 newborns delivered in Mississippi each year, it is expected that 4 PKU confirmed cases, 10 T4, TSH confirmed cases, and at least 60 Hgb confirmed cases will be identified at an early age. With the incidence of galactosemia being one in every 50,000 births, it is expected that only one Gal case every l l/2 to 2 years will be identified. "Newborn Screening" is more correctly identified as a comprehensive program that accounts for the prevention of infant death, mental retardation and life shortening illnesses of newborns in Mississippi.
