| Sickle cell disease (SCD) is a term for a group of genetic disorders characterized by the production of hemoglobin S, anemia, and acute and chronic tissue damage. Primarily African Americans are affected by SCD in the United States with an estimated incidence of 1 in every 375 live births for the most common form, homozygous SS disease (Sickle Cell Disease Guideline Panel, 1993). The course and severity of SCD are highly unpredictable. Morbidity and complications directly impact the quality of life of affected individuals and their families. A diagnosis of SCD no longer means an early death. The average life span of a person with SS disease has increased from about 14 years in 1973 to a median age of death in the 1990's among males at 42 years and females at 48 years (Bloom, 1995; Platt et al., 1994). This increased longevity has created a new patient population: adults with SCD.; Preparing adolescents to transfer from pediatric to adult care settings is an important component of care. Little is known about factors that may influence this process and enable adolescents to achieve successful transition. The Transition Knowledge Questionnaire, a Severity of Disease Index, the Family APGAR Scale, and the Adjective Checklist Autonomy Scale were used to determine the relationship(s) between knowledge about SCD, severity of disease, and family relationships to the level of independence in an adolescent with SCD. Age and gender were also considered.; The study was conducted at the Comprehensive Sickle Cell Center of The Children's Hospital of Philadelphia. The sample included 74 adolescents aged 14 through 21 years (X = 16.58, SD = 1.98) who regularly received care at the center; 77% had SS disease. Multiple regression statistics were used for analyses. Although knowledge, severity and family relationships accounted for only 25% of the variance for independence in the final model, the overall regression was statistically significant, F(3, 70) = 7.85, p < .00001. Age and gender were excluded. These findings provide guidelines to use in assessing, evaluating, developing and implementing individual transition needs for adolescents with SCD. |
