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

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  1 , Rowan Forbes Shepherd  2 , Camella J Rising  2 , Ashley S Thompson  2 , Chloe Huelsnitz  3 , Catherine Wilsnack  4 , Patrick Boyd  3 , Alix G Sleight  2   5 , Sadie Hutson  6 , Payal P Khincha  2
Affiliations
Clinical Trial

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 et al. Psychooncology. 2023 Mar.

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.

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References

REFERENCES

    1. Fidler MM, Frobisher C, Hawkins MM, Nathan PC. Challenges and opportunities in the care of survivors of adolescent and young adult cancers. Pediatr Blood Cancer. 2019;66(6):e27668. https://doi.org/10.1002/pbc.27668
    1. Cheung CK, Zebrack B. What do adolescents and young adults want from cancer resources? Insights from a Delphi panel of AYA patients. Support Care Cancer. 2017;25(1):119-126. https://doi.org/10.1007/s00520-016-3396-7
    1. Smith AW, Parsons HM, Kent EE, et al. Unmet support service needs and health-related quality of life among adolescents and young adults with cancer: the AYA HOPE study. Front Oncol. 2013;3:75. https://doi.org/10.3389/fonc.2013.00075
    1. Siembida EJ, Reeve BB, Zebrack BJ, Snyder MA, Salsman JM. Measuring health-related quality of life in adolescent and young adult cancer survivors with the National Institutes of Health Patient-Reported Outcomes Measurement Information System: comparing adolescent, emerging a. Psycho Oncol. 2021;30(3):303-311. https://doi.org/10.1002/pon.5577
    1. Keegan TH, Ries LA, Barr RD, et al. Comparison of cancer survival trends in the United States of adolescents and young adults with those in children and older adults. Cancer. 2016;122(7):1009-1016. https://doi.org/10.1002/cncr.29869

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