Date of Award

5-2018

Document Type

Dissertation

Degree Name

Doctor of Philosophy (PhD)

Program

Nursing Science

Research Advisor

Carolyn Graff, Ph.D.

Committee

Alicia Diaz-Thomas, M.D. Donna Hathaway, Ph.D. Tamekia Jones, Ph.D. Satish Kedia, Ph.D. Mona Wicks, Ph.D.

Keywords

congenital adrenal hyperplasia, disorders of sexual development, female children, health-related quality of life, mixed methods

Abstract

Introduction. Health-related quality of life (HRQOL) has become a meaningful area in research, medical management, and in the evaluation of health outcomes. However, in children with congenital adrenal hyperplasia (CAH), HRQOL is a long-neglected subject. Previous research in individuals with CAH typically focused on medical treatment and outcomes, which suggested that HRQOL is influenced by personal factors such as genital ambiguity, impaired growth, higher incidence of obesity, metabolic syndrome, hypertension, acute adrenal crisis, as well as losses in psychosexual milestones and psychological functioning. Social contexts, such as family, were identified as factors that contribute to an individual’s perceived HRQOL. Today, there is some agreement that non-disclosure, shame, secrecy, and stigma affect psychological well-being. Despite research efforts, there is limited information about which factors influence HRQOL and which factors are most relevant to female children with CAH. This indicates the need to go beyond identifying single factors, and address the complex relationship of factors that impact HRQOL, and individuals’ perception of these factors. Therefore, the bioecological model of human development was used to understand the different factors that impact HRQOL in female children with CAH.

Methods. A convergent mixed methods design was selected whereby the quantitative and qualitative results were merged to provide a comprehensive understanding of HRQOL of female children with CAH. Qualitative methods (QUAL) involved using semi-structured interviews with child-caregiver dyads (N=20) to explore HRQOL and describe factors that impact HRQOL of female children with CAH. Member checking procedures were implemented to ensure trustworthiness or credibility and rigor of the QUAL component of this study. NVivo 10 was used to analyze the QUAL data. Additionally, the full sample of 25 child-caregiver dyads completed KINDL-R questionnaires, which provided a quantitative (QUAN) measure of HRQOL of female children with CAH. QUAN data were analyzed using SPSS version 24. Individual analysis of both QUAL and QUAN data were merged to ascertain findings for a mixed methods interpretation.

Results. Children with CAH and their caregivers reported having good overall HRQOL. Children scored significantly lower on the School subscale compared to their caregivers (Mdn= 12.5, IQR= 18.75) Examination of the data revealed significant relationships between other health issues and how caregivers rated children’s HRQOL, p <.05. The following themes emerged from the child and caregiver interviews: 1) living with CAH; 2) normalcy; 3) disclosure of diagnosis; 4) feelings of the child/caregiver; 5) what should we know; and 6) improvements. Merging of data showed that not all of QUAN variables are reflected in the QUAL themes. However, many of the QUAL categories and comments elucidated information about the spectrum of children’s and caregiver’s perceptions of the HRQOL of children with CAH.

Discussion. Using a comprehensive approach offered an understanding of what it is like for children to live with CAH from the perspective of the child and caregiver. By quantitatively measuring HRQOL and using qualitative interview data, the information in this study may shed light on children’s and caregivers’ needs that are not met by current guidelines. Although the overall child- and caregiver-reported HRQOL of female children was not impaired, children’s and caregiver’s lived experiences highlight the many factors that impact HRQOL including the importance of friends and family, personal characteristics (i.e., the ability to adapt and self-esteem), the environment (i.e., home, school), and the impact of time (i.e., developmental time, past vs present). Knowing this, clinicians may better support children and families by expanding the focus beyond sex development to include: 1) information about the different ways of knowing (i.e., knowing how to talk with others and with their child); 2) self-management and independence (i.e., learning how to administer medication and handling responsibility as children grown older); and 3) psychological support (i.e., coping with a new diagnosis, adapting to the disorder.)

ORCID

http://orcid.org/0000-0002-3989-1352

DOI

10.21007/etd.cghs.2018.0452

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