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Translational Research and Clinical Applications in the Management of Cystic Fibrosis

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Family Resilience and Chronic Illness

Part of the book series: Emerging Issues in Family and Individual Resilience ((EIIFR))

Abstract

Cystic fibrosis (CF) is a life-shortening and recessive genetic disorder that affects approximately 1 in 2500 live births worldwide. The disorder affects the functions of multiple organs including the lungs, pancreas, liver, and reproductive system and requires adherence to a time-consuming and burdensome treatment regimen. This chapter reviews literature regarding adherence to this treatment regimen, the role of the family in treatment adherence, the impact of CF on family functioning and psychological health, the effect of infection control guidelines on CF patient social support and functioning, and the processes associated with the transition from pediatric to adult care. Empirically evaluated interventions targeting each of these challenges are discussed. Reviewed literature points to the importance of: 1) considering the changing needs of individuals with CF in light of developments in medical practices and policy, 2) the need for supportive interventions for families and individuals with CF to improve family functioning, 3) treatment adherence and mental health, and 4) the necessity of programs that facilitate the transition of treatment responsibility from parents to their children with CF.

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Author information

Authors and Affiliations

  1. Department of Psychology and Pediatrics, University of Miami, Coral Gables, FL, 33146, USA

    Alexandra L. Quittner Ph.D.

  2. Department of Psychology, University of Miami, Coral Gables, FL, USA

    Christina J. Nicolais M.S., Estefany Saez-Flores B.A. & Ruth Bernstein M.S.

Authors
  1. Alexandra L. Quittner Ph.D.
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  2. Christina J. Nicolais M.S.
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  3. Estefany Saez-Flores B.A.
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  4. Ruth Bernstein M.S.
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Correspondence to Alexandra L. Quittner Ph.D. .

Editor information

Editors and Affiliations

  1. College of Human Sciences, Oklahoma State University, Stillwater, Oklahoma, USA

    Ginger L. Welch

  2. Oklahoma State University, Stillwater, Oklahoma, USA

    Amanda W. Harrist

Discussion Questions

Discussion Questions

  1. 1.

    What Family Adaptive Systems (FAS), as described in Henry, Morris, and Harrist (2015), might be most relevant to the issue of treatment adherence in families with a child with CF? Give hypothetical examples to support your choice.

  2. 2.

    For Human Science students: What normative developmental issues of adolescence are likely involved in the transition of disease management from parents to self for CF patients?

  3. 3.

    For Health Science students: The authors state that “…patient-provider communication appears to be a major barrier to adequate implementation” of infection control. Given limited time available during patient visits, how might communication about this topic be improved?

  4. 4.

    The authors report that recent studies found that “Approximately half of women with CF reported not using contraceptives, compared to 25% of women without CF.” Why might that be the case?

  5. 5.

    Summarize what you learned in this chapter using “resilience” terminology as described in Luthar, Cicchetti, and Becker (2000; e.g., risk status, protective factors, protective-stabilizing factors, protective-enhancing factors, protective-reactive factors, vulnerable-stable factors, vulnerable-reactive factors).

    References for Discussion Questions

    • Henry, C. S., Morris, A. S., & Harrist, A. W. (2015). Family resilience: Moving into the third wave. Family Relations, 64, 22–43. doi:10.1111/fare.12106

    • Luthar, S. S., Cicchetti, D., & Becker, B. (2000). The construct of resilience: A critical evaluation and guidelines for future work. Child Development, 71, 543–562. doi:10.1111/1467-8624.00164

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Quittner, A.L., Nicolais, C.J., Saez-Flores, E., Bernstein, R. (2017). Translational Research and Clinical Applications in the Management of Cystic Fibrosis. In: Welch, G., Harrist, A. (eds) Family Resilience and Chronic Illness. Emerging Issues in Family and Individual Resilience. Springer, Cham. https://doi.org/10.1007/978-3-319-26033-4_4

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  • DOI: https://doi.org/10.1007/978-3-319-26033-4_4

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