Cystic Fibrosis in Pregnancy

doi:10.1017/9781108163705.008

8 - Cystic Fibrosis in Pregnancy

from Section 3 - Pulmonary Conditions Not Specific to Pregnancy

Published online by Cambridge University Press: 14 April 2020

Melanie Chin and

D. Elizabeth Tullis

Edited by

Stephen E. Lapinsky and

Lauren A. Plante

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Stephen E. Lapinsky: Affiliation:
Mount Sinai Hospital, Toronto
Lauren A. Plante: Affiliation:
Drexel University Hospital, Philadelphia

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Summary

Cystic fibrosis (CF) is an autosomal recessive genetic disorder caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene that results in a multisystem disease. CF most commonly affects the respiratory, hepatobiliary, digestive and reproductive systems, though the majority of morbidity and mortality results from progressive obstructive lung disease and chronic pulmonary infection. In recent years, the survival of patients with CF has increased significantly with a recently reported median survival of up to 51.8 years of age.

Type: Chapter
Information: Respiratory Disease in Pregnancy , pp. 73 - 81

DOI: https://doi.org/10.1017/9781108163705.008 [Opens in a new window]

Publisher: Cambridge University Press

Print publication year: 2020

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References

Elborn, JS. Cystic fibrosis. Lancet. 2016 388(10059):2519–31.CrossRef Google Scholar PubMed

The Canadian Cystic Fibrosis Registry 2014 Annual Report. Toronto, Canada: Cystic Fibrosis Canada. 2016.Google Scholar

Cystic Fibrosis Foundation. Patient Registry 2015 Annual Data Report. Bethesda, maryland: © 2016 Cystic Fibrosis Foundation. 2016. www.cff.org/our-research/cf-patient-registry/2015-patient-registry-annual-data-report.pdf (accessed November 2019.)Google Scholar

Kaplan, E, Shwachman, H, Perlmutter, AD, et al. Reproductive failure in males with cystic fibrosis. N Engl J Med. 1968 279(2):65–9.Google Scholar

Edenborough, FP. Women with cystic fibrosis and their potential for reproduction. Thorax. 2001 56(8):649–55.Google Scholar

Siegel, B, Siegel, S. Pregnancy and delivery in a patient with cystic fibrosis of the pancreas. Obstet Gynecol. 1960 16:438–40.Google Scholar

Goss, CH, Rubenfeld, GD, Otto, K, Aitken, ML. The effect of pregnancy on survival in women with cystic fibrosis. Chest. 2003 124(4):1460–8.Google Scholar

Edenborough, FP, Borgo, G, Knoop, C, et al. Guidelines for the management of pregnancy in women with cystic fibrosis. J Cyst Fibros. 2008 7(Suppl 1):S2-32.CrossRef Google Scholar PubMed

Tizzano, EF, Silver, MM, Chitayat, D, Benichou, JC, Buchwald, M. Differential cellular expression of cystic fibrosis transmembrane regulator in human reproductive tissues: clues for the infertility in patients with cystic fibrosis. Am J Pathol. 1994 144(5):906–14.Google Scholar

Kopito, LE, Kosasky, HJ, Shwachman, H. Water and electrolytes in cervical mucus from patients with cystic fibrosis. Fertil Steril. 1973 24(7):512–16.Google Scholar

Wang, XF, Zhou, CX, Shi, QX, et al. Involvement of CFTR in uterine bicarbonate secretion and the fertilizing capacity of sperm. Nat Cell Biol. 2003 5(10):902–6.CrossRef Google Scholar PubMed

Schram, CA, Stephenson, AL, Hannam, TG, Tullis, E. Cystic fibrosis (cf) and ovarian reserve: a cross-sectional study examining serum anti-mullerian hormone (amh) in young women. J Cyst Fibros. 2015 14(3):398–402.Google Scholar

Johannesson, M, Landgren, BM, Csemiczky, G, Hjelte, L, Gottlieb, C. Female patients with cystic fibrosis suffer from reproductive endocrinological disorders despite good clinical status. Hum Reprod. 1998 13(8):2092–7.Google Scholar

Stallings, VA, Tomezsko, JL, Schall, JI, et al. Adolescent development and energy expenditure in females with cystic fibrosis. Clin Nutr. 2005 24(5):737–45.CrossRef Google Scholar PubMed

Hubert, D, Patrat, C, Guibert, J, et al. Results of assisted reproductive technique in men with cystic fibrosis. Hum Reprod. 2006 21(5):1232–6.Google Scholar

McCallum, TJ, Milunsky, JM, Cunningham, DL, et al. Fertility in men with cystic fibrosis: an update on current surgical practices and outcomes. Chest. 2000 118(4):1059–62.CrossRef Google Scholar PubMed

Ahmad, A, Ahmed, A, Patrizio, P. Cystic fibrosis and fertility. Curr Opin Obstet Gynecol. 2013 25(3):167–72.CrossRef Google Scholar PubMed

Geake, J, Tay, G, Callaway, L, Bell, SC. Pregnancy and cystic fibrosis: approach to contemporary management. Obstet Med. 2014 7(4):147–55.Google Scholar

Committee on Genetics. Committee Opinion No. 691: carrier screening for genetic conditions. Obstet Gynecol. 2017 129(3):e41-55.CrossRef Google Scholar

US CF Foundation, John Hopkins University, The Hospital for Sick Children. The Clinical and Functional Translation of CFTR (CFTR2). Available from: http://cftr2.org/ (accessed November 2019).Google Scholar

Ratjen, F, Bell, SC, Rowe, SM, et al. Cystic fibrosis. Nat Rev Dis Primers. 2015 1:15010.Google Scholar

Watson, MS, Cutting, GR, Desnick, RJ, et al. Cystic fibrosis population carrier screening: 2004 revision of American College of Medical Genetics mutation panel. Genet Med. 2004 6(5):387–91.CrossRef Google Scholar PubMed

Rohlfs, EM, Zhou, Z, Heim, RA, et al. Cystic fibrosis carrier testing in an ethnically diverse US population. Clin Chem. 2011 57(6):841–8.CrossRef Google Scholar

Harper, JC. Preimplantation genetic screening. J Med Screen. 2018 25(1):1–5.CrossRef Google Scholar PubMed

Gilljam, M, Antoniou, M, Shin, J, et al. Pregnancy in cystic fibrosis: fetal and maternal outcome. Chest. 2000 118(1):85–91.Google Scholar

Lau, EM, Barnes, DJ, Moriarty, C, et al. Pregnancy outcomes in the current era of cystic fibrosis care: a 15-year experience. Aust N Z J Obstet Gynaecol. 2011 51(3):220–4.Google Scholar

Jones, AM, Dodd, ME, Govan, JR, et al. Burkholderia cenocepacia and Burkholderia multivorans: influence on survival in cystic fibrosis. Thorax. 2004 59(11):948–51.CrossRef Google Scholar PubMed

Cheng, EY, Goss, CH, McKone, EF, et al. Aggressive prenatal care results in successful fetal outcomes in CF women. J Cyst Fibros. 2006 5(2):85–91.Google Scholar

Edenborough, FP, Stableforth, DE, Webb, AK, Mackenzie, WE, Smith, DL. Outcome of pregnancy in women with cystic fibrosis. Thorax. 1995 50(2):170–4.CrossRef Google Scholar PubMed

Thorpe-Beeston, JG, Madge, S, Gyi, K, Hodson, M, Bilton, D. The outcome of pregnancies in women with cystic fibrosis – single centre experience 1998–2011. Br J Obstet Gynaecol. 2013 120(3):354–61.Google Scholar

Stephenson, AL, Stanojevic, S, Sykes, J, Burgel, PR. The changing epidemiology and demography of cystic fibrosis. Presse Med. 2017 46(6 Pt 2):e87-95.CrossRef Google Scholar PubMed

Stallings, VA, Stark, LJ, Robinson, KA, et al. Evidence-based practice recommendations for nutrition-related management of children and adults with cystic fibrosis and pancreatic insufficiency: results of a systematic review. J Am Diet Assoc. 2008 108(5):832–9.CrossRef Google Scholar PubMed

Canadian Cystic Fibrosis Registry 2013 Annual Report. Toronto, Canada: Cystic Fibrosis Canada. 2014.Google Scholar

Wilson, RD, Genetics, C, Wilson, RD, et al. Pre-conception folic acid and multivitamin supplementation for the primary and secondary prevention of neural tube defects and other folic acid-sensitive congenital anomalies. J Obstet Gynaecol Can. 2015 37(6):534–52.Google Scholar PubMed

Rasmussen, KM, Yaktine, AL, eds. Weight gain during pregnancy: reexamining the guidelines. In: The National Academies Collection: Reports Funded by National Institutes of Health. Washington, D.C.: National Academies Press. 2009.Google Scholar

Moran, A, Dunitz, J, Nathan, B, et al. Cystic fibrosis-related diabetes: current trends in prevalence, incidence, and mortality. Diabetes Care. 2009 32(9):1626–31.Google Scholar

McMullen, AH, Pasta, DJ, Frederick, PD, et al. Impact of pregnancy on women with cystic fibrosis. Chest. 2006 129(3):706–11.Google Scholar

Giacobbe, LE, Nguyen, RH, Aguilera, MN, et al. Effect of maternal cystic fibrosis genotype on diabetes in pregnancy. Obstet Gynecol. 2012 120(6):1394–9.CrossRef Google Scholar PubMed

Miller, RJ, Tildesley, HD, Wilcox, PG, Zhang, H, Kreisman, SH. Sex disparities in effects of cystic fibrosis-related diabetes on clinical outcomes: a matched study. Can Respir J. 2008 15(6):291–4.Google Scholar

Moran, A, Brunzell, C, Cohen, RC, et al. Clinical care guidelines for cystic fibrosis-related diabetes: a position statement of the American Diabetes Association and a clinical practice guideline of the Cystic Fibrosis Foundation, endorsed by the Pediatric Endocrine Society. Diabetes Care. 2010 33(12):2697–708.Google Scholar

Canadian Diabetes Association Clinical Practice Guidelines Expert Committee, Thompson, D, Berger, H, et al. Diabetes and pregnancy. Can J Diabetes. 2013 37 (Suppl 1):S168-83.Google Scholar

Konstan, MW, Flume, PA, Kappler, M, et al. Safety, efficacy and convenience of tobramycin inhalation powder in cystic fibrosis patients: The EAGER trial. J Cyst Fibros. 2011 10(1):54–61.Google Scholar

Padberg, S, Wacker, E, Meister, R, et al. Observational cohort study of pregnancy outcome after first-trimester exposure to fluoroquinolones. Antimicrob Agents Chemother. 2014 58(8):4392–8.CrossRef Google Scholar PubMed

Schaefer, C, Amoura-Elefant, E, Vial, T, et al. Pregnancy outcome after prenatal quinolone exposure: evaluation of a case registry of the European Network of Teratology Information Services (ENTIS). Eur J Obstet Gynecol Reprod Biol. 1996 69(2):83–9.Google Scholar

Bourget, P, Fernandez, H, Delouis, C, Taburet, AM. Pharmacokinetics of tobramycin in pregnant women: safety and efficacy of a once-daily dose regimen. J Clin Pharm Ther. 1991 16(3):167–76.Google Scholar

Hernandez-Diaz, S, Werler, MM, Walker, AM, Mitchell, AA. Folic acid antagonists during pregnancy and the risk of birth defects. N Engl J Med. 2000 343(22): 1608–14.Google Scholar

Committee on Obstetric Practice. Committee Opinion No. 717: sulfonamides, nitrofurantoin, and risk of birth defects. Obstet Gynecol. 2017 130(3):e150-2.Google Scholar

Kline, AH, Blattner, RJ, Lunin, M. Transplacental effect of tetracyclines on teeth. J Am Med Assoc. 1964 188:178–80.Google Scholar

Kaminski, R, Nazareth, D. A successful uncomplicated CF pregnancy while remaining on ivacaftor. J Cyst Fibros. 2016 15(1):133–4.Google Scholar

Ladores, S, Kazmerski, TM, Rowe, SM. A case report of pregnancy during use of targeted therapeutics for cystic fibrosis. J Obstet Gynecol Neonatal Nurs. 2017 46(1):72–7.Google Scholar

Muammar, M, Marshall, P, Wyatt, H, Skelton, V. Caesarean section in a patient with cystic fibrosis. Int J Obstet Anesth. 2005 14(1):70–3.Google Scholar

Butler, JA, Restrick, LJ, Esmond, GM, Mickelsons, C, Empey, DW. Pregnancy assisted by nasal intermittent positive pressure ventilation in a patient with cystic fibrosis. J R Soc Med. 1997 90(4):222–3.Google Scholar

Patel, EM, Swamy, GK, Heine, RP, et al. Medical and obstetric complications among pregnant women with cystic fibrosis. Am J Obstet Gynecol. 2015 212(1):98.e1-9.CrossRef Google Scholar PubMed

Deighan, M, Ash, S, McMorrow, R. Anaesthesia for parturients with severe cystic fibrosis: a case series. Int J Obstet Anesth. 2014 23(1):75–9.Google Scholar

Della Rocca, G. Anaesthesia in patients with cystic fibrosis. Curr Opin Anaesthesiol. 2002 15:95–101.Google Scholar

Schechter, MS, Quittner, AL, Konstan, MW, et al. Long-term effects of pregnancy and motherhood on disease outcomes of women with cystic fibrosis. Ann Am Thorac Soc. 2013 10(3):213–19.Google Scholar

Stephenson, AL, Sykes, J, Berthiaume, Y, et al. Clinical and demographic factors associated with post-lung transplantation survival in individuals with cystic fibrosis. J Heart Lung Transplant. 2015 34(9):1139–45.Google Scholar

Moritz, MJ, Constantinescu, S, Coscia, LA, et al. National Transplantation Pregnancy Registry: 2015 Annual Report. Philadelphia PA: Gift of Life Institute. 2016.Google Scholar

Gyi, KM, Hodson, ME, Yacoub, MY. Pregnancy in cystic fibrosis lung transplant recipients: case series and review. J Cyst Fibros. 2006 5(3):171–5.CrossRef Google Scholar PubMed

Shaner, J, Coscia, LA, Constantinescu, S, et al. Pregnancy after lung transplant. Prog Transplant. 2012 22(2):134–40.Google Scholar

Vos, R, Ruttens, D, Verleden, SE, et al. Pregnancy after heart and lung transplantation. Best Pract Res Clin Obstet Gynaecol. 2014 28(8):1146–62.Google Scholar

McKay, DB, Josephson, MA, Armenti, VT, et al. Reproduction and transplantation: report on the AST Consensus Conference on Reproductive Issues and Transplantation. Am J Transplant. 2005 5(7):1592–9.CrossRef Google Scholar PubMed

American College of Medical Genetics. Standards and Guidelines for Clinical Genetics Laboratories: Technical Standards and Guidelines for CFTR Mutation Testing. Bethesda, MD: American College of Medical Genetics. 2011.Google Scholar

International Association of Diabetes and Pregnancy Study Groups Consensus Panel, Metzger, BE. International Association Of Diabetes And Pregnancy Study Groups recommendations on the diagnosis and classification of hyperglycemia in pregnancy. Diabetes Care. 2010 33(3):676–82.Google Scholar