Date of Award

2023

Degree Type

Dissertation

Degree Name

Doctor of Philosophy in Nursing

Department

Nursing

First Advisor

Mary Sullivan

Second Advisor

Jung Eun Lee

Abstract

Background: Sickle cell disease (SCD) is a hereditary blood disorder that affects millions globally. In Saudi Arabia, it manifests in severe or moderate forms, affecting 24 of 10,000 children and adolescents. This places a substantial burden on families, necessitating intricate care. While existing literature has primarily focused on medical management and quality of life, there is a gap in understanding Saudi adolescents’ experiences living with SCD and their self-management behaviors. Given the cultural emphasis on family in Saudi Arabia, this study was guided by the Individual and Family Self-management Theory (IFSMT), emphasizing collaborative efforts involving individuals and their families.

Objectives: This study sought to (1) understand adolescents’ subjective experience of living with SCD; (2) describe the SCD self-management behaviors and condition-specific contextual factors with the IFSMT; and (3) determine associations between SCD, treatment complexity, and adolescents’ self-management behaviors as perceived by adolescents and their families.

Methods: A convergent mixed-method approach involved: 1) Qualitative descriptive phenomenology through in-depth, semi-structured interviews, 2) Quantitative data from medical records and surveys completed by adolescents and their parents, including demographics, and questionnaires on SCD severity (PSISCP), Illnesses perception (IPQ-R), and SCD self-management behaviors (SMSC). A hybrid thematic analysis encompassed inductive and deductive steps followed by correlation of quantitative data. Convergent and divergent triangulation synthesized qualitative and quantitative data.

Results: Thirty-five adolescents and parents were recruited from three hospitals in Jeddah and Makkah, Saudi Arabia. All completed surveys and 21 adolescents participated in interviews. Four themes characterized their experience with SCD: (1) Adolescents experience SCD as “pain” that ranges from “normal” to “pain attacks” (2) Some adolescents feel that others disbelieve them and may blame them for pain experiences; (3) SCD and its pain affect every aspect of adolescents’ lives; and (4) Adolescents use multiple ways of managing and coping with SCD and its pain to minimize the effects on their lives. Adolescents with higher SCD severity had more negative perceptions, and their higher self-management knowledge was associated with higher severity and negative illness perception. Parents’ perceptions about their adolescents’ knowledge and skills were higher than their adolescents’. A predominant convergence was found between the qualitative and quantitative data on SCD severity, disease perception, and self-management knowledge. However, a divergence was noted between reported self-management skills in qualitative and quantitative data.

Conclusion: There were five overarching conclusions: (a) adolescents’ pain perception is tied to perceived SCD severity; (b) SCD impacts every aspect of their lives; (c) SCD severity and perceptions shape self-management knowledge; (d) discrepancies emerge in self-management skills, with variations between adolescent and parent perceptions; and (e) family support is indispensable for adolescent SCD self-management. Future research should focus on refining pain assessment tools for Saudi adolescents with SCD, aiming to facilitate the development of family-centered, culturally responsive, and individually tailored self-management strategies for this population.

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