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Cystic Fibrosis

  • Adrienne P. Borschuk
  • Stephanie S. FilignoEmail author
Chapter
  • 18 Downloads
Part of the Issues in Clinical Child Psychology book series (ICCP)

Abstract

Cystic fibrosis (CF) is a life-shortening genetic disease that results in the overproduction of thick, sticky mucus throughout the body. Symptoms are progressive, and management requires a burdensome daily medical regimen. Individuals with CF also require hospital admissions lasting 10–14 days to treat illness exacerbations. Given that individuals with CF and their caregivers are more likely to develop symptoms of anxiety and depression, inpatient CL providers are in a prime position to deliver psychological treatment to children and families while also interfacing with and educating the medical team (both inpatient and outpatient). Common referral concerns can include, but are not limited to, anxiety and depression, adherence promotion, coping with disease progression, and promoting function in the hospital environment. Working effectively with bedside staff and attending medical providers are a key focus of treatment for individuals with CF and their families. Aspects of interventions should be modified as appropriate for this population, particularly keeping in mind infection control guidelines.

Keywords

CF exacerbation Hospital coping Procedural anxiety Interdisciplinary communication Adherence promotion Psychotherapy 

References

  1. Barker, D. H., & Quittner, A. L. (2016). Parental depression and pancreatic enzymes adherence in children with cystic fibrosis. Pediatrics, 137(2), 1–9.  https://doi.org/10.1542/peds.2015-2296CrossRefGoogle Scholar
  2. Blackwell, L. S., & Quittner, A. L. (2015). Daily pain in adolescents with CF: Effects on adherence, psychological symptoms, and health-related quality of life. Pediatric Pulmonology, 50(3), 244–251.  https://doi.org/10.1002/ppul.23091CrossRefPubMedGoogle Scholar
  3. Boat, T. F., Filigno, S., & Amin, R. S. (2017). Wellness for families of children with chronic health disorders. Journal of the American Medical Association Pediatrics, 171(9), 825–826.  https://doi.org/10.1001/jamapediatrics.2017.1682CrossRefPubMedGoogle Scholar
  4. Borschuk, A. P., Everhart, R. S., Eakin, M. N., Rand-Giovannetti, D., Borrelli, B., & Riekert, K. A. (2016). Disease disclosure in individuals with cystic fibrosis: Association with psychosocial and health outcomes. Journal of Cystic Fibrosis, 15(5), 696–702.  https://doi.org/10.1016/j.jcf.2016.02.011CrossRefPubMedPubMedCentralGoogle Scholar
  5. Comeau, A. M., Accurso, F. J., White, T. B., Campbell, P. W., 3rd, Hoffman, G., Parad, R. B., … Cystic Fibrosis, F. (2007). Guidelines for implementation of cystic fibrosis newborn screening programs: Cystic Fibrosis Foundation workshop report. Pediatrics, 119(2), e495–e518.  https://doi.org/10.1542/peds.2006-1993CrossRefPubMedGoogle Scholar
  6. Cystic Fibrosis Foundation. (2018a, December 2). Bronchodilators. Retrieved from https://www.cff.org/Life-With-CF/Treatments-and-Therapies/Medications/Bronchodilators/
  7. Cystic Fibrosis Foundation. (2018b, December 2). What is cystic fibrosis. Retrieved from https://www.cff.org/What-is-CF/About-Cystic-Fibrosis/
  8. Ernst, M. M., Johnson, M. C., & Stark, L. J. (2010). Developmental and psychosocial issues in cystic fibrosis. Child and Adolescent Psychiatric Clinics of North America, 19(2), 263–283.  https://doi.org/10.1016/j.chc.2010.01.004CrossRefPubMedPubMedCentralGoogle Scholar
  9. Filigno, S., Strong, S., Hente, E., Elam, M., Mara, C., & Boat, T. (2017). Promoting school success for students with cystic fibrosis: A novel empirically-based program. Pediatric Pulmonology, 52, S484–S484.Google Scholar
  10. Filigno, S., Strong, S., Hente, E., Murphy, C., Elam, M., Mara, C., … Boat, T. (2018). We have a lot to learn: School needs and school absences for student with cystic fibrosis. Pediatric Pulmonology, 53, 407–407.Google Scholar
  11. Friedman, D., Pinsky, H., Quittner, A., Salathe, M., Smith, B., & Georgiopoulos, A. (2018). Development of a CF-specific CBT intervention to prevent depression and anxiety in adults with CF. Pediatric Pulmonology, 53, 409–409.CrossRefGoogle Scholar
  12. Gravelle, A. M., Paone, M., Davidson, A. G., & Chilvers, M. A. (2015). Evaluation of a multidimensional cystic fibrosis transition program: A quality improvement initiative. Journal of Pediatric Nursing, 30(1), 236–243.  https://doi.org/10.1016/j.pedn.2014.06.011CrossRefPubMedGoogle Scholar
  13. Grossoehme, D. H., Filigno, S. S., & Bishop, M. (2014). Parent routines for managing cystic fibrosis in children. Journal of Clinical Psychology in Medical Settings, 21(2), 125–135.  https://doi.org/10.1007/s10880-014-9396-1CrossRefPubMedPubMedCentralGoogle Scholar
  14. Jamieson, N., Fitzgerald, D., Singh-Grewal, D., Hanson, C. S., Craig, J. C., & Tong, A. (2014). Children’s experiences of cystic fibrosis: A systematic review of qualitative studies. Pediatrics, 133(6), e1683–e1697.  https://doi.org/10.1542/peds.2014-0009CrossRefPubMedGoogle Scholar
  15. Kazak, A. E., Brier, M., Alderfer, M. A., Reilly, A., Fooks Parker, S., Rogerwick, S., … Barakat, L. P. (2012). Screening for psychosocial risk in pediatric cancer. Pediatric Blood & Cancer, 59(5), 822–827.  https://doi.org/10.1002/pbc.24166CrossRefGoogle Scholar
  16. Li, S. S., Tumin, D., Krone, K. A., Boyer, D., Kirkby, S. E., Mansour, H. M., & Hayes, D., Jr. (2018). Risks associated with lung transplantation in cystic fibrosis patients. Expert Review of Respiratory Medicine, 12(11), 893–904.  https://doi.org/10.1080/17476348.2018.1522254CrossRefPubMedGoogle Scholar
  17. Madan, A. S., Alpern, A. N., & Quittner, A. L. (2014). Transition from pediatric to adult cystic fibrosis care: A developmental framework. European Respiratory Society Monograph, 64, 272–285.Google Scholar
  18. Masson, A., Kirszenbaum, M., & Sermet-Gaudelus, I. (2017). Pain is an underestimated symptom in cystic fibrosis. Current Opinion in Pulmonary Medicine, 23(6), 570–573.  https://doi.org/10.1097/MCP.0000000000000427CrossRefPubMedGoogle Scholar
  19. Modi, A. C., Lim, C. S., Yu, N., Geller, D., Wagner, M. H., & Quittner, A. L. (2006). A multi-method assessment of treatment adherence for children with cystic fibrosis. Journal of Cystic Fibrosis, 5(3), 177–185.  https://doi.org/10.1016/j.jcf.2006.03.002CrossRefPubMedGoogle Scholar
  20. Modi, A. C., Pai, A. L., Hommel, K. A., Hood, K. K., Cortina, S., Hilliard, M. E., … Drotar, D. (2012). Pediatric self-management: A framework for research, practice, and policy. Pediatrics, 129(2), e473–e485.  https://doi.org/10.1542/peds.2011-1635CrossRefPubMedPubMedCentralGoogle Scholar
  21. O’Hayer, C., Aikens, R., O’Loughlin, C., Bennett, D., Lee, H., Hendry, M., & Stephen, M. (2018). ACT with CF: Promising conclusions of a telehealth pilot study of acceptance and commitment therapy for anxiety and depression. Pediatric Pulmonology, 53, 408–S2. Abstract 677.Google Scholar
  22. Powers, S. W., Stark, L. J., Chamberlin, L. A., Filigno, S. S., Sullivan, S. M., Lemanek, K. L., … Clancy, J. P. (2015). Behavioral and nutritional treatment for preschool-aged children with cystic fibrosis: A randomized clinical trial. Journal of the American Medical Association Pediatrics, 169(5), e150636.  https://doi.org/10.1001/jamapediatrics.2015.0636CrossRefPubMedGoogle Scholar
  23. Quittner, A. L., Goldbeck, L., Abbott, J., Duff, A., Lambrecht, P., Sole, A., … Barker, D. (2014). Prevalence of depression and anxiety in patients with cystic fibrosis and parent caregivers: Results of the international depression epidemiological study across nine countries. Thorax, 69(12), 1090–1097.  https://doi.org/10.1136/thoraxjnl-2014-205983CrossRefPubMedGoogle Scholar
  24. Saiman, L., Siegel, J. D., LiPuma, J. J., Brown, R. F., Bryson, E. A., Chambers, M. J., … Society for Healthcare Epidemiology of America. (2014). Infection prevention and control guideline for cystic fibrosis: 2013 update. Infection Control and Hospital Epidemiology, 35(Suppl 1), S1–S67.  https://doi.org/10.1086/676882CrossRefPubMedGoogle Scholar
  25. Sanders, D. B., Zhang, Z., Farrell, P. M., Lai, H. J., & Wisconsin, C. F. N. S. G. (2018). Early life growth patterns persist for 12 years and impact pulmonary outcomes in cystic fibrosis. Journal of Cystic Fibrosis, 17(4), 528–535.  https://doi.org/10.1016/j.jcf.2018.01.006CrossRefPubMedPubMedCentralGoogle Scholar
  26. Smith, B. A., Modi, A. C., Quittner, A. L., & Wood, B. L. (2010). Depressive symptoms in children with cystic fibrosis and parents and its effects on adherence to airway clearance. Pediatric Pulmonology, 45(8), 756–763.  https://doi.org/10.1002/ppul.21238CrossRefPubMedGoogle Scholar
  27. Stark, L. J., Quittner, A. L., Powers, S. W., Opipari-Arrigan, L., Bean, J. A., Duggan, C., & Stallings, V. A. (2009). Randomized clinical trial of behavioral intervention and nutrition education to improve caloric intake and weight in children with cystic fibrosis. Archives of Pediatrics and Adolescent Medicine, 163(10), 915–921.  https://doi.org/10.1001/archpediatrics.2009.165CrossRefPubMedGoogle Scholar
  28. Talwalkar, J. S., Koff, J. L., Lee, H. B., Britto, C. J., Mulenos, A. M., & Georgiopoulos, A. M. (2017). Cystic fibrosis transmembrane regulator modulators: Implications for the management of depression and anxiety in cystic fibrosis. Psychosomatics, 58(4), 343–354.  https://doi.org/10.1016/j.psym.2017.04.001CrossRefPubMedGoogle Scholar
  29. Tluczek, A., Orland, K. M., & Cavanagh, L. (2011). Psychosocial consequences of false-positive newborn screens for cystic fibrosis. Qualitative Health Research, 21(2), 174–186.  https://doi.org/10.1177/1049732310382919CrossRefPubMedGoogle Scholar
  30. VanDevanter, D. R., Kahle, J. S., O’Sullivan, A. K., Sikirica, S., & Hodgkins, P. S. (2016). Cystic fibrosis in young children: A review of disease manifestation, progression, and response to early treatment. Journal of Cystic Fibrosis, 15(2), 147–157.  https://doi.org/10.1016/j.jcf.2015.09.008CrossRefPubMedGoogle Scholar

Copyright information

© Springer Nature Switzerland AG 2020

Authors and Affiliations

  • Adrienne P. Borschuk
    • 1
    • 2
    • 3
  • Stephanie S. Filigno
    • 1
    • 2
    • 3
    Email author
  1. 1.Cincinnati Children’s Hospital Medical CenterCincinnatiUSA
  2. 2.Behavioral Medicine and Clinical PsychologyCincinnati Children’s Hospital Medical CenterCincinnatiUSA
  3. 3.University of Cincinnati College of MedicineCincinnatiUSA

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