Objective: 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. Not only are adult health care providers not adequately prepared to deal with these individuals but also the adolescents may not be prepared for this important transition. 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 purpose of this study was to identify through an explanatory model, depicting hypothesized relationships from clinicians as the conceptual framework, predictors of independence in adolescents with SCD. Knowledge about factors that are modifiable will assist providers in developing interventions and transition programs for these adolescents with SCD. Design: A non-experimental descriptive correlational design was used. Population, Sample, Setting, Years: The study was conducted at a comprehensive sickle cell center of a university pediatric hospital in a Northeastern metropolitan area. A convenience sample of 74 adolescents aged 14 through 21 years (X=16.58, SD=1.98), males and females, who regularly received care at the center participated in the study. Seventy-seven percent were diagnosed with homozygous or sickle cell SS disease. Approximately 24% of all subjects had completed high school and 14.9 % were in college at the time of enrollment. Data collection extended over a 13-month period, ending August 2001. Concept or Variables Studied Together: The explanatory model attempted to define 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. Methods: Interviews were conducted at the sickle cell center and in the homes of subjects. Subjects completed four instruments: a Demographic Data Sheet, the Transition Knowledge Questionnaire (TKQ), the Family APGAR Scale, and the Adjective Checklist Autonomy Scale. The researcher calculated the Severity of Disease Index (SDI) after review of each subject's medical record. The TKQ and the SDI are new measures that were developed for use in this study. Multiple regression statistics were used for analyses. Findings: Knowledge, severity and family relationships accounted for only 25% of the variance for independence in the final model but the overall regression was statistically significant, F (3, 70)=7.85, p<.00001. Severity was positively related to independence while knowledge and family relationships were inversely related to independence. Age was significantly correlated to knowledge and there was a statistically significant difference in the mean scores on the SDI between males and females with females having higher SDI scores. Both, however, were excluded from the regression model. Conclusions: A small proportion of the variance for independence in this sample was accounted for by the three variables in the model. According to this model, adolescents with less knowledge, poorer family relationships and greater severity of disease will be more independent. At first seemingly contra-intuitive, this explanation may represent those adolescents who are struggling to manage their disease in the absence of substantial knowledge and family support. Undoubtedly other variables must contribute to independence. Because of sampling methods, these findings cannot be generalized to all adolescents with SCD. Implications: These findings provide useful information for practicing clinicians and can be guidelines to assess individual transition needs and help in developing, implementing and evaluating transition programs for adolescents with SCD. The TKQ and SDI offer foundations for further testing and refinement so that sensitive measures can become available to clinicians and researchers. Qualitative research studies on independence in adolescents with SCD would also enhance current knowledge by identifying factors they perceive as the strongest predictors of independence.
Back to Posters
Back to The Advancing Nursing Practice Excellence: State of the Science