How do young people with a hereditary cancer predisposition syndrome understand and experience cancer survivorship? "With Li-Fraumeni syndrome, it's just an intermission"

Allison Werner-Lin; Rowan Forbes Shepherd; Camella J Rising; Ashley S Thompson; Chloe Huelsnitz; Catherine Wilsnack; Patrick Boyd; Alix G Sleight; Sadie Hutson; Payal P Khincha

doi:10.1002/pon.6080

How do young people with a hereditary cancer predisposition syndrome understand and experience cancer survivorship? "With Li-Fraumeni syndrome, it's just an intermission"

Psychooncology. 2023 Mar;32(3):375-382. doi: 10.1002/pon.6080. Epub 2023 Jan 3.

Authors

Affiliations

¹ School of Social Policy and Practice, University of Pennsylvania, Philadelphia, Pennsylvania, USA.
² Clinical Genetics Branch, Division of Cancer Epidemiology and Genetics, National Cancer Institute, Rockville, Maryland, USA.
³ Behavioral Research Program, Division of Cancer Control and Population Sciences, National Cancer Institute, National Institutes of Health, Rockville, Maryland, USA.
⁴ Steve Hicks School of Social Work, University of Texas at Austin, Austin, Texas, USA.
⁵ Department of Physical Medicine and Rehabilitation, Cedars-Sinai Medical Center, Los Angeles, California, USA.
⁶ Department of Nursing, University of Tennessee, Knoxville, Tennessee, USA.

PMID: 36514197
DOI: 10.1002/pon.6080

Abstract

Objectives: Adolescents and young adult (AYA) cancer survivors face unique medical and psychosocial sequalae, including chronic health conditions, late effects of treatment and fear of recurrence. The meaning of cancer survivorship may be further complicated for AYAs with hereditary cancer predisposition syndromes. This study used a patient-centered framework to investigate how AYAs with Li-Fraumeni syndrome (LFS) consider cancer survivorship.

Methods: An interprofessional team conducted 30 semi-structured interviews with AYAs (aged 18-41, mean 31 years) enrolled in the National Cancer Institute's LFS Study (NCT01443468). Twenty had experienced at least one cancer diagnosis. Interview data were thematically analyzed by an inter-professional team using interpretive description and grounded theory methods.

Findings: Participants viewed "survivorship" as a period marked by no evidence of formerly diagnosed disease. By contrast, participants felt the label "survivor" was tenuous since LFS is characterized by multiple primary malignancies and uncertainty about intervals between one diagnosis and the next. Many AYAs viewed survivorship as requiring a high degree of suffering. Though many personally rejected "survivor" identities, almost all articulated its various functions including positive, negative, and more complicated connotations. Instead, they chose language to represent a range of beliefs about survival, longevity, prognosis, and activism.

Conclusions: AYAs with LFS struggle with the term "survivor" due to their multi-organ cancer risk, short intervals between malignancies, and evolving identities. Loved ones' cancer-related suffering informed perspectives on survivorship. Survivorship care for AYAs with cancer risk syndromes requires interprofessional interventions that address their unique biomedical and psychosocial needs.

Keywords: Li-Fraumeni syndrome; adolescents and young adults (AYA); cancer; cancer care; genetic cancer risk; inherited cancer predisposition syndromes; survivor; survivorship.

Publication types

Clinical Trial
Research Support, Non-U.S. Gov't

MeSH terms

Adolescent
Cancer Survivors* / psychology
Emotions
Genetic Predisposition to Disease
Humans
Li-Fraumeni Syndrome* / diagnosis
Li-Fraumeni Syndrome* / psychology
Neoplasms* / psychology
Survivors
Young Adult

Grants and funding

National Cancer Institute, Division of Cancer Epidemiology and Genetics Intramural Research Program