Newborn screening may be the single most important recent advance in the treatment of sickle cell disease. However, the efficacy of newborn screening depends on adequate patient education, counseling, and follow-up medical care. In large part, it is the nurse's responsibility to ensure that these follow-up services are provided.
The Northern California Comprehensive Sickle Cell Center at Children's Hospital, Oakland, has a regional newborn hemoglobinopathy screening program that identifies approximately 15 newborns with sickle cell disease annually. Obstetrical nurses collect an umbilical cord blood sample from all babies born at the ten participating institutions. Samples are sent to the reference hemoglobinopathy laboratory at Children's Hospital, and diagnostic results are available within 1 week of birth.
When a diagnosis of sickle cell disease is made, the genetic counselor discusses the case with the sickle cell nurse coordinator and physician. The responsible private physician and obstetrician are personally notified. In most cases, the family receives a telephone call from the genetic counselor approximately 1 week after the birth. An appointment is then made in the next week for laboratory confirmation of diagnosis and initiation of counseling. At the confirmation appointment, the family and patient are introduced to the sickle cell nurse coordinator, and a comprehensive sickle cell clinic appointment is made for the following week. At this appointment, a "new family letter" introducing the rest of the sickle cell staff is reviewed with the family by the nurse, and a copy is given to the family. This letter outlines the comprehensive services available to patients and contains information to familiarize them with our particular clinical services and policies.
Translation, and interobserver and test–retest reliability of the Brazilian Portuguese version of Children's Hospital Oakland Hip Evaluation Scale for patients with sickle cell disease
