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Sexual and Reproductive Health in Cystic Fibrosis

  • Erin Crowley
  • Cynthia D. BrownEmail author
Chapter
  • 18 Downloads
Part of the Respiratory Medicine book series (RM)

Abstract

Sexual and reproductive health (SRH) issues are a common yet frequently underreported problem in adolescents and adults with cystic fibrosis (CF). As the CF population continues to experience overall health gains in lung function, nutrition, and life expectancy, it is likely that SRH will become a more prevalent issue, and more research will be needed to better understand the needs of the CF population. The focus of this review is to provide an overview of the current state of the literature regarding SRH in the CF population.

Keywords

Cystic fibrosis Male infertility Pregnancy Contraception Puberty Sexual development 

References

  1. 1.
    Boas SR, Fulton JA, Koehler AN, Orenstein DM. Nutrition and pulmonary function predictors of delayed puberty in adolescent males with cystic fibrosis. Clin Pediatr (Phila). 1998;37(9):573–6.Google Scholar
  2. 2.
    Byard PJ. The adolescent growth spurt in children with cystic fibrosis. Ann Hum Biol. 1994;21(3):229–40.Google Scholar
  3. 3.
    Johannesson M, Gottlieb C, Hjelte L. Delayed puberty in girls with cystic fibrosis despite good clinical status. Pediatrics. 1997;99(1):29–34.Google Scholar
  4. 4.
    Sawyer SM, Phelan PD, Bowes G. Reproductive health in young women with cystic fibrosis: knowledge, behavior and attitudes. J Adolesc Health. 1995;17(1):46–50.Google Scholar
  5. 5.
    Roberts S, Green P. The sexual health of adolescents with cystic fibrosis. J R Soc Med. 2005;98(Suppl 45):7–16.Google Scholar
  6. 6.
    Prasad SA, Balfour-Lynn IM, Carr SB, Madge SL. A comparison of the prevalence of urinary incontinence in girls with cystic fibrosis, asthma, and healthy controls. Pediatr Pulmonol. 2006;41(11):1065–8.Google Scholar
  7. 7.
    Umlawska W, Sands D, Zielinska A. Age of menarche in girls with cystic fibrosis. Folia Histochem Cytobiol. 2010;48(2):185–90.Google Scholar
  8. 8.
    Frayman KB, Sawyer SM. Sexual and reproductive health in cystic fibrosis: a life-course perspective. Lancet Respir Med. 2015;3(1):70–86.Google Scholar
  9. 9.
    Zhang Z, Lindstrom MJ, Lai HJ. Pubertal height velocity and associations with prepubertal and adult heights in cystic fibrosis. J Pediatr. 2013;163(2):376–82.Google Scholar
  10. 10.
    Aswani N, Taylor CJ, McGaw J, Pickering M, Rigby AS. Pubertal growth and development in cystic fibrosis: a retrospective review. Acta Paediatr. 2003;92(9):1029–32.Google Scholar
  11. 11.
    Stead RJ, Hodson ME, Batten JC, Adams J, Jacobs HS. Amenorrhoea in cystic fibrosis. Clin Endocrinol. 1987;26(2):187–95.Google Scholar
  12. 12.
    Leifke E, Friemert M, Heilmann M, Puvogel N, Smaczny C, von zur Muhlen A, et al. Sex steroids and body composition in men with cystic fibrosis. Eur J Endocrinol. 2003;148(5):551–7.Google Scholar
  13. 13.
    Blackman SM, Tangpricha V. Endocrine disorders in cystic fibrosis. Pediatr Clin N Am. 2016;63(4):699–708.Google Scholar
  14. 14.
    Denning CR, Sommers SC, Quigley HJ Jr. Infertility in male patients with cystic fibrosis. Pediatrics. 1968;41(1):7–17.Google Scholar
  15. 15.
    Kaplan E, Shwachman H, Perlmutter AD, Rule A, Khaw KT, Holsclaw DS. Reproductive failure in males with cystic fibrosis. N Engl J Med. 1968;279(2):65–9.Google Scholar
  16. 16.
    Sawyer SM, Tully MA, Dovey ME, Colin AA. Reproductive health in males with cystic fibrosis: knowledge, attitudes, and experiences of patients and parents. Pediatr Pulmonol. 1998;25(4):226–30.Google Scholar
  17. 17.
    Dreyfus DH, Bethel R, Gelfand EW. Cystic fibrosis 3849+10kb C > T mutation associated with severe pulmonary disease and male fertility. Am J Respir Crit Care Med. 1996;153(2):858–60.Google Scholar
  18. 18.
    Stern RC, Doershuk CF, Drumm ML. 3849+10 kb C-->T mutation and disease severity in cystic fibrosis. Lancet. 1995;346(8970):274–6.Google Scholar
  19. 19.
    Sawyer SM, Farrant B, Cerritelli B, Wilson J. A survey of sexual and reproductive health in men with cystic fibrosis: new challenges for adolescent and adult services. Thorax. 2005;60(4):326–30.Google Scholar
  20. 20.
    Sawyer SM, Farrant B, Wilson J, Ryan G, O'Carroll M, Bye P, et al. Sexual and reproductive health in men with cystic fibrosis: consistent preferences, inconsistent practices. J Cyst Fibros. 2009;8(4):264–9.Google Scholar
  21. 21.
    Esteves SC, Miyaoka R, Orosz JE, Agarwal A. An update on sperm retrieval techniques for azoospermic males. Clinics (Sao Paulo). 2013;68(Suppl 1):99–110.Google Scholar
  22. 22.
    Palermo G, Joris H, Devroey P, Van Steirteghem AC. Pregnancies after intracytoplasmic injection of single spermatozoon into an oocyte. Lancet. 1992;340(8810):17–8.Google Scholar
  23. 23.
    Dohle GR, Ramos L, Pieters MH, Braat DD, Weber RF. Surgical sperm retrieval and intracytoplasmic sperm injection as treatment of obstructive azoospermia. Hum Reprod. 1998;13(3):620–3.Google Scholar
  24. 24.
    McCallum TJ, Milunsky JM, Cunningham DL, Harris DH, Maher TA, Oates RD. Fertility in men with cystic fibrosis: an update on current surgical practices and outcomes. Chest. 2000;118(4):1059–62.Google Scholar
  25. 25.
    Muchekehu RW, Quinton PM. A new role for bicarbonate secretion in cervico-uterine mucus release. J Physiol. 2010;588(Pt 13):2329–42.Google Scholar
  26. 26.
    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
  27. 27.
    Shteinberg M, Lulu AB, Downey DG, Blumenfeld Z, Rousset-Jablonski C, Perceval M, et al. Failure to conceive in women with CF is associated with pancreatic insufficiency and advancing age. J Cyst Fibros. 2019;18:525.Google Scholar
  28. 28.
    Cystic Fibrosis Foundation Patient Registry 2016 annual data report. Bethesda: Cystic Fibrosis Foundation; 2017.Google Scholar
  29. 29.
    Thorpe-Beeston J, Madge S, Gyi K, Hodson M, Bilton D. The outcome of pregnancies in women with cystic fibrosis—single centre experience 1998–2011. BJOG. 2013;120(3):354–61.Google Scholar
  30. 30.
    McArdle JR. Pregnancy in cystic fibrosis. Clin Chest Med. 2011;32(1):111–20.Google Scholar
  31. 31.
    Rasmussen KMY, Ann L. Weight gain during pregnancy: reexamining the guidelines. Washington, DC: The National Academies Press; 2009.Google Scholar
  32. 32.
    Stallings VA, Stark LJ, Robinson KA, Feranchak AP, Quinton H. 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.Google Scholar
  33. 33.
    Edenborough FP, Borgo G, Knoop C, Lannefors L, Mackenzie WE, Madge S, et al. Guidelines for the management of pregnancy in women with cystic fibrosis. J Cyst Fibros. 2008;7:S2–S32.Google Scholar
  34. 34.
    Ren CL, Morgan RL, Oermann C, Resnick HE, Brady C, Campbell A, et al. Cystic Fibrosis Foundation pulmonary guidelines. Use of cystic fibrosis transmembrane conductance regulator modulator therapy in patients with cystic fibrosis. Ann Am Thorac Soc. 2018;15(3):271–80.Google Scholar
  35. 35.
    Davies JC, Moskowitz SM, Brown C, Horsley A, Mall MA, McKone EF, et al. VX-659–Tezacaftor–Ivacaftor in patients with cystic fibrosis and one or two Phe508del Alleles. N Engl J Med. 2018;379(17):1599–611.Google Scholar
  36. 36.
    Keating D, Marigowda G, Burr L, Daines C, Mall MA, McKone EF, et al. VX-445–Tezacaftor–Ivacaftor in patients with cystic fibrosis and one or two Phe508del Alleles. N Engl J Med. 2018;379(17):1612–20.Google Scholar
  37. 37.
    Ramsey BW, Davies J, McElvaney NG, Tullis E, Bell SC, Dřevínek P, et al. A CFTR potentiator in patients with cystic fibrosis and the G551D mutation. N Engl J Med. 2011;365(18):1663–72.Google Scholar
  38. 38.
    Kaminski R, Nazareth D. A successful uncomplicated CF pregnancy while remaining on Ivacaftor. J Cyst Fibros. 2016;15(1):133–4.Google Scholar
  39. 39.
    Heltshe SL, Godfrey EM, Josephy T, Aitken ML, Taylor-Cousar JL. Pregnancy among cystic fibrosis women in the era of CFTR modulators. J Cyst Fibros. 2017;16(6):687–94.Google Scholar
  40. 40.
    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
  41. 41.
    Trimble AT, Donaldson SH. Ivacaftor withdrawal syndrome in cystic fibrosis patients with the G551D mutation. J Cyst Fibros. 2018;17(2):e13–e6.Google Scholar
  42. 42.
    Taylor-Cousar JL, Munck A, McKone EF, van der Ent CK, Moeller A, Simard C, et al. Tezacaftor–Ivacaftor in patients with cystic fibrosis homozygous for Phe508del. N Engl J Med. 2017;377(21):2013–23.Google Scholar
  43. 43.
    Wainwright CE, Elborn JS, Ramsey BW, Marigowda G, Huang X, Cipolli M, et al. Lumacaftor–Ivacaftor in patients with cystic fibrosis homozygous for Phe508del CFTR. N Engl J Med. 2015;373(3):220–31.Google Scholar
  44. 44.
    Marie J, Elisabeth E-A, Isabelle M, Inés De M, Nicolas V, Eric C. Bedaquiline and linezolid for extensively drug-resistant tuberculosis in pregnant woman. Emerg Infect Dis. 2017;23(10):1731–2.Google Scholar
  45. 45.
    Van Kampenhout E, Bolhuis MS, Alffenaar J-WC, Oswald LMA, Kerstjens HAM, de Lange WCM, et al. Pharmacokinetics of moxifloxacin and linezolid during and after pregnancy in a patient with multidrug-resistant tuberculosis. Eur Respir J. 2017;49(3):16017124.  https://doi.org/10.1183/13993003.01724-2016.
  46. 46.
    Bookstaver PB, Bland CM, Griffin B, Stover KR, Eiland LS, McLaughlin M. A review of antibiotic use in pregnancy. Pharmacotherapy. 2015;35(11):1052–62.Google Scholar
  47. 47.
    Siegel B, Siegel S. Pregnancy and delivery in a patient with cystic fibrosis of the pancreas. Obstet Gynecol. 1960;16(4):438–40.Google Scholar
  48. 48.
    Edenborough FP, Mackenzie WE, Stableforth DE. The outcome of 72 pregnancies in 55 women with cystic fibrosis in the United Kingdom 1977-1996. BJOG. 2000;107(2):254–61.Google Scholar
  49. 49.
    LAU EMT, BARNES DJ, MORIARTY C, OGLE R, DENTICE R, CIVITICO J, 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
  50. 50.
    Renton M, Priestley L, Bennett L, Mackillop L, Chapman SJ. Pregnancy outcomes in cystic fibrosis: a 10-year experience from a UK centre. Obstet Med. 2015;8(2):99–101.Google Scholar
  51. 51.
    Reynaud Q, Poupon-Bourdy S, Rabilloud M, Al Mufti L, Rousset Jablonski C, Lemonnier L, et al. Pregnancy outcome in women with cystic fibrosis-related diabetes. Acta Obstet Gynecol Scand. 2017;96(10):1223–7.Google Scholar
  52. 52.
    Jelin AC, Sharshiner R, Caughey AB. Maternal co-morbidities and neonatal outcomes associated with cystic fibrosis. J Matern Fetal Neonatal Med. 2017;30(1):4–7.Google Scholar
  53. 53.
    Patel EM, Swamy GK, Heine RP, Kuller JA, James AH, Grotegut CA. Medical and obstetric complications among pregnant women with cystic fibrosis. Am J Obstet Gynecol. 2015;212(1):98.e1–9.Google Scholar
  54. 54.
    Schechter MS, Quittner AL, Konstan MW, Millar SJ, Pasta DJ, McMullen A. Long-term effects of pregnancy and motherhood on disease outcomes of women with cystic fibrosis. Ann Am Thorac Soc. 2013;10(3):213–9.Google Scholar
  55. 55.
    Shaner J, Coscia LA, Constantinescu S, McGrory CH, Doria C. Pregnancy after lung transplant. Prog Transplant. 2012;22(2):134–40.Google Scholar
  56. 56.
    Kazmerski TM, Sawicki GS, Miller E, Jones KA, Abebe KZ, Tuchman LK, 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.Google Scholar
  57. 57.
    Kazmerski TM, Sawicki GS, Miller E, Jones KA, Abebe KZ, Tuchman LK, et al. Sexual and reproductive health behaviors and experiences reported by young women with cystic fibrosis. J Cyst Fibros. 2018;17(1):57–63.Google Scholar
  58. 58.
    Trussell J. Contraceptive failure in the United States. Contraception. 2011;83(5):397–404.Google Scholar
  59. 59.
    Stead RJ, Grimmer SF, Rogers SM, Back DJ, Orme ML, Hodson ME, et al. Pharmacokinetics of contraceptive steroids in patients with cystic fibrosis. Thorax. 1987;42(1):59–64.Google Scholar
  60. 60.
    Plant BJ, Goss CH, Tonelli MR, McDonald G, Black RA, Aitken ML. Contraceptive practices in women with cystic fibrosis. J Cyst Fibros. 2008;7(5):412–4.Google Scholar
  61. 61.
    Simmons KB, Haddad LB, Nanda K, Curtis KM. Drug interactions between non-rifamycin antibiotics and hormonal contraception: a systematic review. Am J Obstet Gynecol. 2018;218(1):88–97.e14.Google Scholar
  62. 62.
    Chotirmall SH, Smith SG, Gunaratnam C, Cosgrove S, Dimitrov BD, O'Neill SJ, et al. Effect of estrogen on pseudomonas mucoidy and exacerbations in cystic fibrosis. N Engl J Med. 2012;366(21):1978–86.Google Scholar
  63. 63.
    Roe AH, Traxler S, Schreiber CA. Contraception in women with cystic fibrosis: a systematic review of the literature. Contraception. 2016;93(1):3–10.Google Scholar
  64. 64.
    Whiteman MK, Oduyebo T, Zapata LB, Walker S, Curtis KM. Contraceptive safety among women with cystic fibrosis: a systematic review. Contraception. 2016;94(6):621–9.Google Scholar

Copyright information

© Springer Nature Switzerland AG 2020

Authors and Affiliations

  1. 1.Indiana University School of Medicine, Division of Pulmonary, Critical Care, Occupational and Sleep MedicineIndianapolisUSA

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