Abstract
Cystic fibrosis is the most common genetic disease in the Caucasian population and can lead to multisystem dysfunction, significant respiratory morbidity, and limited life span. The treatment burden to maintain health in this condition is significant and doesn’t guarantee longevity. This chapter reviews cystic fibrosis and its impact on the human body as well as the history of care in this patient population. It also highlights the progress that has been made in the care of people with CF, which now obviates other needs such as preparing these patients to be successful adults.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
Similar content being viewed by others
References
Andersen DH. Cystic fibrosis of the pancreas and its relation to celiac disease. Am J Dis Child. 1938;56:344.
Sanders DB, Fink AK. Background and epidemiology. Pediatr Clin N Am. 2016;63(4):567–84.
Find a CF Care Center. https://www.cff.org/ccd/. Accessed 29 June 2020.
Cystic Fibrosis Foundation. Patient registry 2018 annual report. Bethesda, MD: Cystic FIbrosis Foundation; 2019.
Stoltz DA, Meyerholz DK, Welsh MJ. Origins of cystic fibrosis lung disease. N Engl J Med. 2015;372(4):351–62.
Elborn JS. Cystic fibrosis. Lancet. 2016;388(10059):2519–31.
Davies JC, Ebdon AM, Orchard C. Recent advances in the management of cystic fibrosis. Arch Dis Child. 2014;99(11):1033–6.
Gilligan PH. Infections in patients with cystic fibrosis: diagnostic microbiology update. Clin Lab Med. 2014;34(2):197–217.
Pittman JE, Calloway EH, Kiser M, et al. Age of Pseudomonas aeruginosa acquisition and subsequent severity of cystic fibrosis lung disease. Pediatr Pulmonol. 2011;46(5):497–504.
Bar-on O, Mussaffi H, Mei-zahav M, et al. Increasing nontuberculous mycobacteria infection in cystic fibrosis. J Cyst Fibros. 2015;14(1):53–62.
Ledder O, Haller W, Couper RT, Lewindon P, Oliver M. Cystic fibrosis: an update for clinicians. Part 2: hepatobiliary and pancreatic manifestations. J Gastroenterol Hepatol. 2014;29(12):1954–62.
Olivier AK, Gibson-corley KN, Meyerholz DK. Animal models of gastrointestinal and liver diseases. Animal models of cystic fibrosis: gastrointestinal, pancreatic, and hepatobiliary disease and pathophysiology. Am J Physiol Gastrointest Liver Physiol. 2015;308(6):G459–71.
Ode KL, Frohnert B, Laguna T, et al. Oral glucose tolerance testing in children with cystic fibrosis. Pediatr Diabetes. 2010;11(7):487–92.
Ode KL, Moran A. New insights into cystic fibrosis-related diabetes in children. Lancet Diabetes Endocrinol. 2013;1(1):52–8.
Colombo C, Battezzati PM, Crosignani A, et al. Liver disease in cystic fibrosis: a prospective study on incidence, risk factors, and outcome. Hepatology. 2002;36(6):1374–82.
Debray D, Kelly D, Houwen R, Strandvik B, Colombo C. Best practice guidance for the diagnosis and management of cystic fibrosis-associated liver disease. J Cyst Fibros. 2011;10(Suppl 2):S29–36.
Van der Doef HP, Kokke FT, Van Derent CK, Houwen RH. Intestinal obstruction syndromes in cystic fibrosis: meconium ileus, distal intestinal obstruction syndrome, and constipation. Curr Gastroenterol Rep. 2011;13(3):265–70.
Sathe M, Houwen R. Meconium ileus in cystic fibrosis. J Cyst Fibros. 2017;16(Suppl 2):S32–9.
Van der Doef HP, Slieker MG, Staab D, et al. Association of the CLCA1 p.S357N variant with meconium ileus in European patients with cystic fibrosis. J Pediatr Gastroenterol Nutr. 2010;50(3):347–9.
Dray X, Bienvenu T, Desmazes-dufeu N, Dusser D, Marteau P, Hubert D. Distal intestinal obstruction syndrome in adults with cystic fibrosis. Clin Gastroenterol Hepatol. 2004;2(6):498–503.
Ahmad A, Ahmed A, Patrizio P. Cystic fibrosis and fertility. Curr Opin Obstet Gynecol. 2013;25(3):167–72.
Sueblinvong V, Whittaker LA. Fertility and pregnancy: common concerns of the aging cystic fibrosis population. Clin Chest Med. 2007;28(2):433–43.
Boyd JM, Mehta A, Murphy DJ. Fertility and pregnancy outcomes in men and women with cystic fibrosis in the United Kingdom. Hum Reprod. 2004;19(10):2238–43.
Osmundo Junior GS, Athanazio RA, Rached SZ, Francisco RPV. Maternal and perinatal outcomes in pregnant women with cystic fibrosis. Rev Bras Ginecol Obstet. 2019;41(4):230–5.
GotTransition.org. Got Transition®—Health Care Providers. 2020. https://gottransition.org/providers/index.cfm. Accessed 21 June 2020.
Tuchman L, Schwartz M. Health outcomes associated with transition from pediatric to adult cystic fibrosis care. Pediatrics. 2013;132(5):847–53.
Weissberg-benchell J, Shapiro JB. A review of interventions aimed at facilitating successful transition planning and transfer to adult care among youth with chronic illness. Pediatr Ann. 2017;46(5):e182–7.
Meadows AK, Bosco V, Tong E, Fernandes S, Saidi A. Transition and transfer from pediatric to adult care of young adults with complex congenital heart disease. Curr Cardiol Rep. 2009;11(4):291–7.
Tuchman LK, Schwartz LA, Sawicki GS, Britto MT. Cystic fibrosis and transition to adult medical care. Pediatrics. 2010;125(3):566–73. https://doi.org/10.1542/peds.2009-2791.
Duguépéroux I, Tamalet A, Sermet-gaudelus I, et al. Clinical changes of patients with cystic fibrosis during transition from pediatric to adult care. J Adolesc Health. 2008;43(5):459–65.
Mclaughlin SE, Diener-west M, Indurkhya A, Rubin H, Heckmann R, Boyle MP. Improving transition from pediatric to adult cystic fibrosis care: lessons from a national survey of current practices. Pediatrics. 2008;121(5):e1160–6.
Cooley WC, Sagerman PJ. Supporting the health care transition from adolescence to adulthood in the medical home. Pediatrics. 2011;128(1):182–200.
Cystic Fibrosis Foundation. Appendix 2. Revised requirements: models for adult CF programs. www.portcf.org/Resources/CFFCareNet/Accreditation%20 Criteria/ADULT%20Program%20Requirements %20and%20Models%2Epdf. Accessed 15 Jan 2010.
Hanghøj S, Boisen KA. Self-reported barriers to medication adherence among chronically ill adolescents: a systematic review. J Adolesc Health. 2014;54(2):121–38.
Britto MT, Garrett JM, Dugliss MA, Johnson CA, Majure JM, Leigh MW. Preventive services received by adolescents with cystic fibrosis and sickle cell disease. Arch Pediatr Adolesc Med. 1999;153(1):27–32.
Sawicki GS, Sellers DE, Robinson WM. High treatment burden in adults with cystic fibrosis: challenges to disease self-management. J Cyst Fibros. 2009;8(2):91–6.
Callahan ST, Cooper WO. Continuity of health insurance coverage among young adults with disabilities. Pediatrics. 2007;119(6):1175–80.
Dziuban EJ, Saab-Abazeed L, Chaudhry SR, et al. Identifying barriers to treatment adherence and related attitudinal patterns in adolescents with cystic fibrosis. Pediatr Pulmonol. 2010;45:450e8.
Kazmerski TM, Sawicki GS, Miller E, et al. Sexual and reproductive health care utilization and preferences reported by young women with cystic fibrosis. J Cyst Fibros. 2018;17(1):64–70.
Kazmerski TM, Sawicki GS, Miller E, et al. Sexual and reproductive health behaviors and experiences reported by young women with cystic fibrosis. J Cyst Fibros. 2018;17(1):57–63.
Kazmerski TM, Borrero S, Sawicki GS, et al. Provider attitudes and practices toward sexual and reproductive health care for young women with cystic fibrosis. J Pediatr Adolesc Gynecol. 2017;30(5):546–52.
Author information
Authors and Affiliations
Corresponding author
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2023 Springer Nature Switzerland AG
About this chapter
Cite this chapter
Baumann, T., Barto, T.L. (2023). Cystic Fibrosis: A Successful Model of Transition of Care and Lessons Learned. In: Sharafkhaneh, A., Gozal, D. (eds) Sleep Medicine. Springer, Cham. https://doi.org/10.1007/978-3-031-30010-3_15
Download citation
DOI: https://doi.org/10.1007/978-3-031-30010-3_15
Published:
Publisher Name: Springer, Cham
Print ISBN: 978-3-031-30009-7
Online ISBN: 978-3-031-30010-3
eBook Packages: MedicineMedicine (R0)