Skip to main content

Cystic Fibrosis in Adolescents

  • 1411 Accesses

Folklore from central Europe recounts that infants who taste salty with their mother’s kiss will not survive infancy. This is felt to be the first description of cystic fibrosis. Centuries later in 1938, the first description of CF in the medical literature noted pancreatic abnormalities, malnourishment, and associated pneumonia in infants who died usually in the first year of life (Andersen, 2008).

Keywords

  • Cystic Fibrosis
  • Cognitive Behavioral Intervention
  • Pancreatic Insufficiency
  • Cognitive Restructuring
  • Cystic Fibrosis Transmembrane Regulator

These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

This is a preview of subscription content, access via your institution.

Buying options

Chapter
USD   29.95
Price excludes VAT (USA)
  • DOI: 10.1007/978-0-387-87687-0_17
  • Chapter length: 14 pages
  • Instant PDF download
  • Readable on all devices
  • Own it forever
  • Exclusive offer for individuals only
  • Tax calculation will be finalised during checkout
eBook
USD   109.00
Price excludes VAT (USA)
  • ISBN: 978-0-387-87687-0
  • Instant PDF download
  • Readable on all devices
  • Own it forever
  • Exclusive offer for individuals only
  • Tax calculation will be finalised during checkout
Softcover Book
USD   139.99
Price excludes VAT (USA)
Hardcover Book
USD   179.99
Price excludes VAT (USA)
Fig. 1

References

  • Abman, S. H., Ogle, J. W., Harbeck, R. J., Butler-Simon, N., Hammond, K. B., Accurso, F. J. (1991). Early bacteriologic, immunologic, and clinical courses of young infants with cystic fibrosis identified by neonatal screening. Journal of Pediatrics, 119, 211–217.

    PubMed  CrossRef  Google Scholar 

  • Andersen, D. H. (1949). The present diagnosis and therapy of cystic fibrosis of the pancreas. Proceedings of the Royal Society of Medicine, 42, 25–32.

    Google Scholar 

  • Andersen, D. H. (2008). Cystic fibrosis of the pancreas and its relation to celiac disease. American Journal of Diseases of Children, 56, 344.

    Google Scholar 

  • Ballestero, Y., Hernandez, M. I., Rojo, P., Manzanares, J., Nebreda, V., Carbajosa, H., et al. (2006). Hyponatremic dehydration as a presentation of cystic fibrosis. Pediatric Emergency Care, 22, 725–727.

    PubMed  CrossRef  Google Scholar 

  • Bartholomew, L. K., Czyzewski, D. I., Parcel, G. S., Swank, P. R., Sockrider, M. M., & Mariiotto, M. J. (1997). Self-management of cystic fibrosis: Short-term outcomes of the Cystic Fibrosis Family Education Program. Health Education & Behavior, 24, 652–666.

    CrossRef  Google Scholar 

  • Blair, C., Cull, A., & Freeman, C. P. (1994). Psychosocial functioning of young adults with cystic fibrosis and their families. Thorax, 49, 798–802.

    PubMed  CrossRef  Google Scholar 

  • Carew, L. D. (2001). The adolescent with cystic fibrosis: A psychosocial perspective. South African Journal of Child and Adolescent Mental Health, 13, 23–29.

    Google Scholar 

  • Collins, F. S. (1992). Cystic fibrosis: molecular biology and therapeutic implications. Science, 256, 774–779.

    PubMed  CrossRef  Google Scholar 

  • Corbett, K., Kelleher, S., Rowland, M., Daly, L., Drumm, B., Canny, G., et al. (2004). Cystic fibrosis-associated liver disease: a population-based study. Journal of Pediatrics, 145, 327–332.

    PubMed  CrossRef  Google Scholar 

  • D’Auria, J. P., Christian, B. J., Henderson, Z. G., & Haynes, B. (2000). The company they keep: The influence of peer relationships on adjustment to cystic fibrosis during adolescence. Journal of Pediatric Nursing, 15, 175–182.

    PubMed  Google Scholar 

  • Davis, M. A., Quittner, A. L., Stack, C. M., Yang, M. C. (2004). Controlled evaluation of the STARBRIGHT CD-ROM program for children and adolescents with cystic fibrosis. Journal of Pediatric Psychology, 29, 259–267.

    PubMed  CrossRef  Google Scholar 

  • de Jong, W., Grevink, R. G., Roorda, R. J., Kaptein, A. A., & van der Schans, C. P. (1994). Effect of a home exercise training program in patients with cystic fibrosis. Chest, 105, 463–468.

    PubMed  CrossRef  Google Scholar 

  • DeLambo, K. E., Ievers-Landis, C. E., Drotar, D., & Quittner, A. L. (2004). Association of observed family relationship quality and problem-solving skills with treatment adherence in older children and adolescents with cystic fibrosis. Journal of Pediatric Psychology, 29, 343–353

    PubMed  CrossRef  Google Scholar 

  • DiGirolamo, A. M., Quittner, A. L., Ackerman, V., & Stevens, J. (1997). Identification and assessment of ongoing stressors in adolescents with a chronic illness: An application of the behavior-analytic model. Journal of Clinical Child Psychology, 26, 53–66.

    PubMed  CrossRef  Google Scholar 

  • Dik, H., Nicolai, J. J., Schipper, J., Heijerman, H.G., & Bakker, W. (1995). Erroneous diagnosis of distal intestinal obstruction syndrome in cystic fibrosis: Clinical impact of abdominal ultrasonography. European Journal of Gastroenterology & Hepatology, 7, 279–281.

    Google Scholar 

  • Drotar, D. (2006). Psychological interventions in childhood chronic illness. Washington DC: American Psychological Association.

    CrossRef  Google Scholar 

  • Feindler, E. L., Ecton, R. B., Kingsley, D., & Dubey, D. (1986). Group anger control training for institutionalized psychiatric male adolescents. Behavior Therapy, 17, 109–123.

    CrossRef  Google Scholar 

  • Feranchak, A. P., Sontag, M. K., Wagener, J. S., Hammond, K. B., Accurso, F. J., & Sokol, R. J. (1999). Prospective, long-term study of fat-soluble vitamin status in children with cystic fibrosis identified by newborn screen. Journal of Pediatrics, 135, 601–610.

    PubMed  CrossRef  Google Scholar 

  • FitzSimmons, S. C. (1993). The changing epidemiology of cystic fibrosis. Journal of Pediatrics, 122, 1–9

    PubMed  Google Scholar 

  • Flume, P. A., O'Sullivan, B. P., Robinson, K. A., Goss, C. H., Mogayzel, P. J., Willey-Courand, D. B., et al. (2007). Cystic fibrosis pulmonary guidelines: chronic medications for maintenance of lung health. American Journal of Respiratory & Critical Care Medicine, 176, 957–969.

    CrossRef  Google Scholar 

  • Foster, C., Eiser, C., Oades, P., Sheldon, C., Tripp, J., Goldman, P., et al. (2001). Treatment demands and differential treatment of patients with cystic fibrosis and their siblings: Patient, parent and sibling accounts. Child: Care Health & Development, 27, 349–364.

    CrossRef  Google Scholar 

  • Glasscoe, C. A., & Quittner, A. L. (2003). Psychological interventions for cystic fibrosis. Cochrane Database of Systematic Reviews. Retrieved April 4, 2008, from http://web.ebscohost.com.

  • Gudas, L. J., Koocher, G. P., & Wypij, D. (1991). Perceptions of medical compliance in children and adolescents with cystic fibrosis. Journal of Developmental & Behavioral Pediatrics, 12, 236–242.

    CrossRef  Google Scholar 

  • Gulmans, V.A. M., de Meer, K., Brackel, H. J. L., Faber, J. A. J., Berger, R., & Helders, P. J. M. (1999). Outpatient exercise training in children with cystic fibrosis: Physiological effects, perceived competence, and acceptability. Pediatric Pulmonology, 28, 39–46.

    PubMed  CrossRef  Google Scholar 

  • Hains, A. A., Berlin, K. S., Davies, W. H., Parton, E. A., & Alemzadeh, R. (2006). Attributions of adolescents with Type 1 diabetes in social situations: Relationship with expected adherence, diabetes stress, and metabolic control. Diabetes Care, 29, 818–822.

    PubMed  CrossRef  Google Scholar 

  • Hains, A. A., Berlin, K. S., Davies, W. H., Smothers, M. K., Sato, A. F., & Alemzadeh, R. (2007). Attributions of adolescents with Type 1 diabetes related to performing self-care around friends and peers: The moderating role of friend support. Journal of Pediatric Psychology, 32, 561–570.

    PubMed  CrossRef  Google Scholar 

  • Hains, A. A., Davies, W. H., Behrens, D., & Biller, J. A. (1997). Cognitive behavioral interventions for adolescents with cystic fibrosis. Journal of Pediatric Psychology, 22, 669–687.

    PubMed  CrossRef  Google Scholar 

  • Hains, A. A., Davies, W. H., Behrens, D., Freeman, M. E., & Biller, J. A. (2001). Effectiveness of a cognitive behavioral intervention for young adults with cystic fibrosis. Journal of Clinical Psychology in Medical Settings, 8, 325–336.

    CrossRef  Google Scholar 

  • Hains, A. A., Davies, W. H., Parton, E., & Silverman, A. H. (2001). A cognitive behavioral intervention for distressed adolescents with Type 1 diabetes. Journal of Pediatric Psychology, 26, 61–66.

    PubMed  CrossRef  Google Scholar 

  • Johnson, K. B., Ravert, R. D., & Everton, A. (2001). Hopkins teen central: Assessment of an internet-based support system for children with cystic fibrosis. Pediatrics, 107, e24.

    PubMed  CrossRef  Google Scholar 

  • Kendall, P. C. (1994). Treating anxiety disorders in children: Results of a randomized clinical trial. Journal of Consulting and Clinical Psychology, 62, 100–110.

    Google Scholar 

  • Kendall, P. C., Chansky, T. E., Kane, M. T., Kim, R. S., Kortlander, E., Ronan, K. R. et al. (1992). Anxiety disorders in youth: Cognitive-behavioral interventions. New York: Allyn & Bacon.

    Google Scholar 

  • Kendall, P. C., Flannery-Schroeder, E., Panichelli-Mindel, S. M., Southam-Gerow, M., Henin, A., Warman, M. (1997). Therapy for youths with anxiety disorders: A second randomized clinical trial. Journal of Consulting & Clinical Psychology, 65, 366–380.

    CrossRef  Google Scholar 

  • Kerem, B., Rommens, J. M., Buchanan, J. A., Markiewicz, D., Cox, T. K., Chakravarti, A., et al. (1989). Identification of the cystic fibrosis gene: Genetic analysis. Science, 245, 1073–1080.

    PubMed  CrossRef  Google Scholar 

  • Konstan, M. W., & M. Berger. (1992).  Infection and Inflammation of the lung in cystic fibrosis. In P. Davis (Ed.), Cystic fibrosis: Lung biology in health and disease (pp. 219–276). New York: Marcel Dekker.

    Google Scholar 

  • Kopelman, H., Durie, P., Gaskin, K., Weizman, Z., & Forstner G. (1985). Pancreatic fluid secretion and protein hyperconcentration in cystic fibrosis. New England Journal of Medicine, 312, 329–334.

    PubMed  CrossRef  Google Scholar 

  • Kotloff, R. M., FitzSimmons, S. C., & Fiel, S. B. (1992). Fertility and pregnancy in patients with cystic fibrosis. Clinics in Chest Medicine, 13, 623–635.

    PubMed  Google Scholar 

  • La Greca, A. M., & Bearman, K. J. (2003). Adherence to pediatric treatment regimens. In M. C. Roberts (Ed.), Handbook of pediatric psychology (3rd ed., pp. 119–140). New York: Guilford Press.

    Google Scholar 

  • Lanng, S., Hansen, A., Thorsteinsson, B., Nerup, J., & Koch, C. (1995). Glucose tolerance in patients with cystic fibrosis: Five year prospective study. British Medical Journal, 311, 655–659.

    PubMed  Google Scholar 

  • Lewinsohn, P. M., Clarke, G. N., Hops, H., & Andrews, J. (1990). Cognitive-behavioral treatment for depressed adolescents. Behavior Therapy, 21, 385–401.

    CrossRef  Google Scholar 

  • Littlewood, J.M. (1992). Gastrointestinal complications in cystic fibrosis. Journal of the Royal Society of Medicine, 85(Suppl. 19), 13–19.

    PubMed  Google Scholar 

  • Main, E., Prasad, A., & Schans, C. (2005). Conventional chest physiotherapy compared to other airway clearance techniques for cystic fibrosis. Cochrane Database of Systematic Reviews, (1):CD002011

    Google Scholar 

  • Modi, A. C., & Quittner, A. L. (2006). Barriers to treatment adherence for children with cystic fibrosis and asthma: What gets in the way? Journal of Pediatric Psychology, 31, 846–858.

    PubMed  CrossRef  Google Scholar 

  • Mullins, L. L., Pace, T. M., & Keller, J. (1994). Cystic fibrosis: Psychological issues. In R. A. Olson, L. L. Mullins, J. B. Gillman, & J. M. Chaney (Eds.), The sourcebook of pediatric psychology (pp. 204–217). Boston: Allyn & Bacon.

    Google Scholar 

  • Passero, M. A., Remor, B., & Solomon, J. (1981). Patient reported compliance with cystic fibrosis therapy. Clinical Pediatrics, 20, 264–268.

    PubMed  CrossRef  Google Scholar 

  • Poustie, V. J., Russell, J. E., Watling, R. M., Ashby, D., & Smyth, R. L. (2006) Oral protein energy supplements for children with cystic fibrosis: CALICO multicentre randomised controlled trial. British Medical Journal, 332, 632–636.

    PubMed  CrossRef  Google Scholar 

  • Quinton, P. M. (1990). Cystic fibrosis: A disease in electrolyte transport. The FASEB Journal, 4, 2709–2717.

    PubMed  Google Scholar 

  • Quittner, A. L., Drotar, D., Ievers-Lands, C., Slocum, N., Seidner, D., & Jacobsen, J. (2000). Adherence to medical treatments in adolescents with cystic fibrosis: The development and evaluation of family based interventions. In D. Drotar (Ed.), Promoting adherence to medical treatment in childhood chronic illness (pp. 383–407). Mahwah, NJ: Lawrence Erlbaum Assoc.

    Google Scholar 

  • Quittner, A. L., Tolbert, V., Regoli, M. J., Orenstein, D., Hollingsworth, J. L., & Eigen, H. (1996). Development of the Role-Play Inventory of Situations and Coping Strategies (RISCS) for parents of children with cystic fibrosis. Journal of Pediatric Psychology, 21, 209–235.

    PubMed  CrossRef  Google Scholar 

  • Ramsey, B. W. (1996). Management of pulmonary disease in patients with cystic fibrosis. New England Journal of Medicine, 335, 179–188.

    PubMed  CrossRef  Google Scholar 

  • Robin, A. L., & Foster, S. L. (1989). Negotiating parent-adolescent conflict: A behavioral family systems approach. New York: Guilford Press.

    Google Scholar 

  • Rosenstein, B. J., & Cutting, G. R. (1998). The diagnosis of cystic fibrosis: A consensus statement. Journal of Pediatrics, 132, 589–595.

    PubMed  CrossRef  Google Scholar 

  • Selvadurai, H. C., Blimkie, C. J., Meyers, N., Mellis, C. M., Cooper, P. J., & Van Asperen, P. P. (2002). Randomized controlled study of in-hospital exercise training programs in children with cystic fibrosis. Pediatric Pulmonology, 33, 194–200.

    PubMed  CrossRef  Google Scholar 

  • Stark, L. J., Mackner, L. M., Patton, S. R., & Acton, J. D. (2003). Cystic fibrosis. In M. C. Roberts (Ed.), Handbook of pediatric psychology (3rd ed., pp. 286–303). New York: Guilford Press.

    Google Scholar 

  • Stark, L. J., Opipari, L. C., Spieth, L. E., Jelalian, E., Quittner, A. Q., Higgins, L., et al. (2003). Contribution of behavior therapy to dietary treatment in cystic fibrosis: A randomized controlled study with a 2-year follow-up. Behavior Therapy, 34, 237–258.

    CrossRef  Google Scholar 

  • Taussig, L. M., Lobeck, C. C., di Sant'Agnese, P. A., Ackerman, D. R., & Kattwinkel, J. (1972). Fertility in males with cystic fibrosis. New England Journal of Medicine, 287, 586–589.

    PubMed  CrossRef  Google Scholar 

  • Thomas, A. M., Peterson, L., & Goldstein, D. (1997). Problem solving and diabetes regimen adherence by children and adolescents with IDDM in social pressure situations: A reflection of normal development. Journal of Pediatric Psychology, 22, 541–561.

    PubMed  CrossRef  Google Scholar 

  • Thompson, R. J., Gustafson, K. E., George, L. K., & Spock, A. (1994). Change over a 12-month period in the psychological adjustment of children and adolescents with cystic fibrosis. Journal of Pediatric Psychology, 19, 189–203.

    PubMed  CrossRef  Google Scholar 

  • Thompson, R. J., Gustafson, K. E., Hamlett, K. W., & Spock, A. (1992). Psychological adjustment of children with cystic fibrosis: The role of child cognitive processes and maternal adjustment. Journal of Pediatric Psychology, 17, 741–755.

    PubMed  CrossRef  Google Scholar 

  • Wang, X., Moylan, B., Leopold, D. A., Kim, J., Rubenstein, R. C., Togias, A., et al. (2000). Mutation in the gene responsible for cystic fibrosis and predisposition to chronic rhinosinusitis in the general population. Journal of the American Medical Association, 284, 1814–1819.

    PubMed  CrossRef  Google Scholar 

  • Welsh, M. J., & Smith, A. E. (1995). Cystic fibrosis. Scientific American, 273, 52–59.

    PubMed  CrossRef  Google Scholar 

  • Welsh, M. J., Tsui, L. C., Boat, T. F., & Beaudet, A. L. (1995). Cystic fibrosis. In C. R. Scriver, A. L. Beaudet, W. S. Sly, & D. Valle (Eds.), The metabolic and molecular basis of inherited disease (7th ed., pp 3799–3876). New York: McGraw-Hill.

    Google Scholar 

  • Wysocki, T., Harris, M. A., Buckloh, L. M., Mertlich, D., Lochrie, A. S., Taylor, A., et al. (2008). Randomized, controlled trial of behavioral family systems therapy for diabetes: Maintenance and generalization of effects on parent-adolescent communication. Behavior Therapy, 39, 33–46.

    PubMed  CrossRef  Google Scholar 

  • Zuelzer, W. W., & Newton, W. A. (1949). The pathogenesis of fibrocystic disease of the pancreas: A study of 36 cases with special reference to the pulmonary lesions. Pediatrics, 4, 53–69.

    PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Anthony A. Hains .

Editor information

Editors and Affiliations

Rights and permissions

Reprints and Permissions

Copyright information

© 2009 Springer Science+Business Media, LLC

About this chapter

Cite this chapter

Hains, A.A., Davies, W.H., Quintero, D., Biller, J.A. (2009). Cystic Fibrosis in Adolescents. In: O'Donohue, W. (eds) Behavioral Approaches to Chronic Disease in Adolescence. Springer, New York, NY. https://doi.org/10.1007/978-0-387-87687-0_17

Download citation