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Biomedical subjects

Sadie P Hutson

Publications and source records attributed to Sadie P Hutson.

3 recordsLinked to original sources
PubMed2025

Concerns about the consequences of cancer predisposition and relationships with quality of life in young adults with Li-Fraumeni syndrome.

Li-Fraumeni syndrome (LFS) is an inherited condition associated with high multi-organ cancer risks from birth. Young adults (YAs; 18-39 years) with LFS experience psychosocial challenges that may negatively affect health-related quality of life (HRQOL). This study aimed to describe a specific psychosocial challenge, concerns about the consequences of LFS (CC-LFS), and investigate relationships among CC-LFS and HRQOL among YAs. An online survey assessed CC-LFS and HRQOL among YAs with LFS enrolled in a National Cancer Institute study. Regression analyses examined relationships between CC-LFS and HRQOL scores, adjusted for age, cancer, and history of mental health challenges. Of 37 total respondents (78% female; Mage&#x2009;=&#x2009;31 years), 51% had a cancer history. Many reported past mental health challenges, including emotional problems (70%), depression/anxiety disorders (68%), and suicidal ideation (43%). Mean scores for mental (M&#x2009;=&#x2009;65.8), physical (M&#x2009;=&#x2009;67.5), and general (M&#x2009;=&#x2009;63.9) HRQOL were lower than in the general population. Higher CC-LFS scores (M&#x2009;=&#x2009;31/50, SD&#x2009;=&#x2009;7.2) were associated with lower scores for each HRQOL domain. Adjusting for covariates, higher CC-LFS scores were associated with lower physical HRQOL (&#x3b2;&#x2009;=&#x2009;-0.491, 95% CI -0.76, -0.22, p&#x2009;<&#x2009;0.001) and general HRQOL (&#x3b2;&#x2009;=&#x2009;-0.466, 95% CI -0.77, -0.16, p&#x2009;=&#x2009;0.004), but not mental HRQOL (p&#x2009;=&#x2009;0.102). CC-LFS accounted for significant proportions of unique variance in general (R2adj&#x2009;=&#x2009;17.7%, p&#x2009;=&#x2009;0.004) and physical HRQOL (R2adj&#x2009;=&#x2009;20.4%, p&#x2009;<&#x2009;0.001). Findings suggest YAs with LFS may experience substantial concerns, mental health challenges, and diminished HRQOL. Concerns about LFS consequences appear to be a psychosocial risk factor that clinicians and researchers might address through targeted interventions to improve YAs' psychosocial health.

Humans↗
PubMed2007

Experiences of siblings of patients with Fanconi anemia.

BACKGROUND: Clinical management of families with autosomal recessive genetic disorders focuses almost exclusively on the affected family members. However, clinically unaffected members of such families may also be severely troubled by the serious illness in a family member. The purpose of this study was to explore the experiences of healthy siblings of patients with a chronic genetic disease, Fanconi Anemia (FA). PROCEDURE: We used a qualitative, descriptive design, which consisted of in-depth, semi-structured interviews. A convenience sample of nine siblings of patients with FA was recruited from a National Cancer Institute clinical research protocol, which targets families with inherited bone marrow failure syndromes. NVivo 2.0 software facilitated qualitative content analysis of the data. RESULTS: Siblings' rich descriptions provided novel insights into the intricate hardships of living within a family in which a rare, life-threatening, chronic genetic illness in one member is the focus of daily life. Four major themes of the sibling experience emerged from the interview data: (1) containment, (2) invisibility, (3) worry, and (4) despair. CONCLUSIONS: Our data suggest that unrecognized psychosocial issues exist for the apparently healthy siblings of patients with FA. This study explores the psychosocial consequences of living in a family with FA and one of only a few studies to explore the sibling experience of chronic illness using a contemporaneous approach. These findings support the need for an increased awareness among health care providers; future hypothesis driven investigation, and improved assessment of problems with potential psychological morbidity.

Adolescent↗