Date of Award

5-31-2022

Document Type

Open Access Dissertation

Degree Name

Doctor of Philosophy (PhD)

Department

Nursing

First Advisor

Laura L. Hayman

Second Advisor

Terese B. Aronowitz

Third Advisor

Philimon N. Gona

Abstract

Background: Heterozygous Familial Hypercholesterolemia (HeFH) is an underdiagnosed, autosomal dominant, monogenic condition affecting ~1:250 individuals in the United States (U.S.), resulting in cardiovascular events 10-20 years earlier than in unaffected peers.

Sample: Fifty-one parents of youth aged 2-18 years followed for HeFH in a pediatric specialty clinic.

Purpose: Assess parental perceptions of HeFH, child adherence to treatment, and parenting in HeFH-affected households.

Methods: A cross-sectional, descriptive, and correlational survey study congruent with elements of the Family Management Style Framework (FMSF). Pearson’s and Spearman’s correlations assessed linear relationships between parentally observed HeFH treatment adherence measures, parenting style, and parental perceptions of high cholesterol and risk for heart disease as applied to themselves or their families, and their children.

Results: Participating parents were largely middle aged (mean 46.1 years, SD 5.6), mothers (78.4%), Caucasian (86.4%), highly educated (Bachelor’s or higher, 86.3%), and had personally received a diagnosis of genetically elevated high cholesterol (70%). Reference children were mostly adolescents (mean 13.4 years, SD 3.4), diagnosed with HeFH while school-aged (age 6.8 years, SD 4.1), and treated with a statin (80%). Median reported adherence to statins over past month was 94% (IQR 90-100). Missed doses were associated with forgetfulness (56.4%), carelessness (29%), or other reasons (41%). Illness perceptions differed between HeFH sub-concepts (high cholesterol and heart disease risk), respondent cholesterol status (+/-), and family position (parent/child). Patterns of association between illness perceptions and child treatment adherence, and illness perceptions and parenting styles, emerged along the same parameters. Parenting style generally did not directly correlate with observed child adherence, nor did child or family history with HeFH diagnosis and management.

Conclusions: Parents largely reported adequate to excellent adherence to HeFH treatments among their children. High cholesterol and risk for heart disease were perceived differently and may be valuable individually in future research. Consistent with proposed relationships within the FMSF, adherence was correlated with many aspects of illness perceptions and varied by family position. Parenting styles were not directly correlated with adherence but were associated with illness perceptions within family positions, suggesting an avenue for moderation of the illness perceptions/adherence relationships deserving of further research.

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