The Obstetrician & Gynaecologist 11.1576/toag.11.1.19.27464 www.rcog.org.uk/togonline 2009;11:19–24 Review
Review Cystic fibrosis and
pregnancy
Authors Julie Goddard / Stephen J Bourke
Key content:
• Cystic fibrosis is the most common inherited life-threatening disease.
• Advances in treatment have led to increased life expectancy.
• Increasing numbers of women with cystic fibrosis are choosing to embark on
pregnancy; for most, it does not appear to have a detrimental effect on long-term
health.
• Pregnancy outcomes are generally good but 20% of women do not live to see their
child’s tenth birthday.
Learning objectives:
• To be aware of the effects of cystic fibrosis on pregnancy.
• To understand the importance of preconceptual counselling.
• To appreciate that pregnancy management requires a multidisciplinary approach.
Ethical issues:
• Should doctors advise women with cystic fibrosis and poor lung function against
undertaking pregnancy?
• Should assisted reproductive technology be available for women with life-limiting
diseases?
Keywords breastfeeding / contraception / diabetes / life expectancy / lung function
Please cite this article as: Goddard J, Bourke SJ. Cystic fibrosis and pregnancy. The Obstetrician & Gynaecologist 2009;11:19–24.
Author details
Julie Goddard MRCOG Stephen J Bourke MD FRCPI FRCP FCCP DCH
Specialist Registrar Consultant Physician
Wansbeck General Hospital, Woodhorn Lane, Cystic Fibrosis Centre, Royal Victoria Infirmary,
Ashington, Northumberland NE63 9JJ, UK Queen Victoria Road, Newcastle upon Tyne
Email: NE1 4LP, UK
(corresponding author)
© 2009 Royal College of Obstetricians and Gynaecologists 19
, Review 2009;11:19–24 The Obstetrician & Gynaecologist
Introduction studies4,7 have shown that women and girls with
Cystic fibrosis is an autosomal recessive disease, cystic fibrosis are not well informed about
caused by mutations of a gene on chromosome 7 reproductive issues.
which encodes for a chloride channel in the
membranes of epithelial cells. Reduced chloride Contraception is important for those wishing to
conductance results in viscid secretions and organ avoid pregnancy and this needs to be discussed
damage in the respiratory and reproductive tracts early, as unplanned pregnancy can be disastrous.
and the pancreatic and hepatobiliary ducts. Life Choice of contraceptive method can be difficult
expectancy is reduced and the main cause of death because of potential interactions with cystic fibrosis
is respiratory failure due to progressive and its treatment but the risks of contraception
bronchiectasis and persistent lung infections with need to be balanced against the risk of unplanned
virulent organisms such as Pseudomonas pregnancy. The combined oral contraceptive pill
aeruginosa, Staphylococcus aureus and Burkholderia (COC) is the most frequently used method.4 There
cepacia. Other complications of cystic fibrosis are concerns that it could be less effective in women
include pancreatic insufficiency, diabetes, with cystic fibrosis, in view of the frequent courses
osteoporosis, liver disease and gallstones. Patients of antibiotics required; however, there have been no
are treated in specialist centres with a complex pregnancies reported as a result of COC failure. It is
treatment regimen that includes chest important to advise on the use of additional
physiotherapy, nutritional supplements, pancreatic contraception (for example, condoms) when a
enzymes, mucolytics (e.g. nebulised DNase), long- woman is taking antibiotics. Long-term methods,
term oral and nebulised antibiotics (e.g. colistin, such as Depo-Provera® (Pfizer Ltd, Walton-on-the
tobramycin) and frequent courses of intravenous Hill, Surrey) , Implanon® (Organon, Cambridge,
antibiotics (e.g. ceftazidime, meropenem, Cambs) and the Mirena® (Bayer plc, Newbury,
tobramycin). Berks) intrauterine system, are highly effective and
avoid concerns regarding malabsorption. Long-
There are approximately 8000 people living with term use of Depo-Provera can, however, aggravate
cystic fibrosis in the UK and 1 in 25 of the osteoporosis, a common complication of cystic
population is a carrier of the cystic fibrosis gene fibrosis. If termination of pregnancy is required, the
mutation.1 The outlook has improved dramatically usual methods (surgical or medical) can be used.
because of improved diagnosis and management:
median survival in the UK is now 31 years. It is Historical perspective on
estimated that the life expectancy for a child born cystic fibrosis and pregnancy
with cystic fibrosis in 2000 will be 50 years.2 The first successful pregnancy in a woman with
Survival into adulthood brings with it issues of cystic fibrosis was reported in 1960.8 She delivered a
sexuality, fertility and potential pregnancy. This healthy premature baby but died 5 weeks
paper provides an overview of the current issues postpartum from respiratory failure. Initial
surrounding cystic fibrosis and pregnancy. reports9,10 suggested high maternal and perinatal
mortality and pregnancy generally was
Sexuality and fertility discouraged. In the subsequent four decades,
In the 1980s, surveys3 showed that most people with however, the prognosis of cystic fibrosis has
cystic fibrosis were not married or sexually active. improved and attitudes towards pregnancy have
Recent studies4 have shown, however, that sexual gradually changed. There is an increasing trend for
adaptation is normal and that sexual activity is not women with cystic fibrosis to undertake pregnancy.
delayed when compared with adolescents without This can pose a challenge to the medical team but
cystic fibrosis. the emphasis now is on respecting women’s
autonomy and providing information, advice and
Menstruation generally occurs slightly later in girls support in helping them make decisions regarding
with cystic fibrosis and bodyweight is the most pregnancy and motherhood.11
significant determinant of menarche. Once
menstruation is established, the majority have The effects of cystic fibrosis
regular cycles, with amenorrhoea most likely to
occur in girls with poor lung function.5 Nearly all on pregnancy
men with cystic fibrosis are infertile but women Approximately 30–40 women with cystic fibrosis
with cystic fibrosis have relatively normal fertility, undertake pregnancy each year in the UK.12 Early
despite having thickened cervical mucus which reports9,13 suggested that pregnancy was associated
does not show the usual cyclical variation. An with high rates of miscarriage, premature delivery
Aberdeen study6 highlighted that 72% of women and stillbirth. Recent data, however, demonstrate
with cystic fibrosis thought that having children good fetal outcomes (Table 1). The proportion of
was important but that 26% had never discussed live births is now 70–90% and the spontaneous
fertility issues with a health professional. Other miscarriage rate is no higher than in the general
© 2009 Royal College of Obstetricians and Gynaecologists
20
Review Cystic fibrosis and
pregnancy
Authors Julie Goddard / Stephen J Bourke
Key content:
• Cystic fibrosis is the most common inherited life-threatening disease.
• Advances in treatment have led to increased life expectancy.
• Increasing numbers of women with cystic fibrosis are choosing to embark on
pregnancy; for most, it does not appear to have a detrimental effect on long-term
health.
• Pregnancy outcomes are generally good but 20% of women do not live to see their
child’s tenth birthday.
Learning objectives:
• To be aware of the effects of cystic fibrosis on pregnancy.
• To understand the importance of preconceptual counselling.
• To appreciate that pregnancy management requires a multidisciplinary approach.
Ethical issues:
• Should doctors advise women with cystic fibrosis and poor lung function against
undertaking pregnancy?
• Should assisted reproductive technology be available for women with life-limiting
diseases?
Keywords breastfeeding / contraception / diabetes / life expectancy / lung function
Please cite this article as: Goddard J, Bourke SJ. Cystic fibrosis and pregnancy. The Obstetrician & Gynaecologist 2009;11:19–24.
Author details
Julie Goddard MRCOG Stephen J Bourke MD FRCPI FRCP FCCP DCH
Specialist Registrar Consultant Physician
Wansbeck General Hospital, Woodhorn Lane, Cystic Fibrosis Centre, Royal Victoria Infirmary,
Ashington, Northumberland NE63 9JJ, UK Queen Victoria Road, Newcastle upon Tyne
Email: NE1 4LP, UK
(corresponding author)
© 2009 Royal College of Obstetricians and Gynaecologists 19
, Review 2009;11:19–24 The Obstetrician & Gynaecologist
Introduction studies4,7 have shown that women and girls with
Cystic fibrosis is an autosomal recessive disease, cystic fibrosis are not well informed about
caused by mutations of a gene on chromosome 7 reproductive issues.
which encodes for a chloride channel in the
membranes of epithelial cells. Reduced chloride Contraception is important for those wishing to
conductance results in viscid secretions and organ avoid pregnancy and this needs to be discussed
damage in the respiratory and reproductive tracts early, as unplanned pregnancy can be disastrous.
and the pancreatic and hepatobiliary ducts. Life Choice of contraceptive method can be difficult
expectancy is reduced and the main cause of death because of potential interactions with cystic fibrosis
is respiratory failure due to progressive and its treatment but the risks of contraception
bronchiectasis and persistent lung infections with need to be balanced against the risk of unplanned
virulent organisms such as Pseudomonas pregnancy. The combined oral contraceptive pill
aeruginosa, Staphylococcus aureus and Burkholderia (COC) is the most frequently used method.4 There
cepacia. Other complications of cystic fibrosis are concerns that it could be less effective in women
include pancreatic insufficiency, diabetes, with cystic fibrosis, in view of the frequent courses
osteoporosis, liver disease and gallstones. Patients of antibiotics required; however, there have been no
are treated in specialist centres with a complex pregnancies reported as a result of COC failure. It is
treatment regimen that includes chest important to advise on the use of additional
physiotherapy, nutritional supplements, pancreatic contraception (for example, condoms) when a
enzymes, mucolytics (e.g. nebulised DNase), long- woman is taking antibiotics. Long-term methods,
term oral and nebulised antibiotics (e.g. colistin, such as Depo-Provera® (Pfizer Ltd, Walton-on-the
tobramycin) and frequent courses of intravenous Hill, Surrey) , Implanon® (Organon, Cambridge,
antibiotics (e.g. ceftazidime, meropenem, Cambs) and the Mirena® (Bayer plc, Newbury,
tobramycin). Berks) intrauterine system, are highly effective and
avoid concerns regarding malabsorption. Long-
There are approximately 8000 people living with term use of Depo-Provera can, however, aggravate
cystic fibrosis in the UK and 1 in 25 of the osteoporosis, a common complication of cystic
population is a carrier of the cystic fibrosis gene fibrosis. If termination of pregnancy is required, the
mutation.1 The outlook has improved dramatically usual methods (surgical or medical) can be used.
because of improved diagnosis and management:
median survival in the UK is now 31 years. It is Historical perspective on
estimated that the life expectancy for a child born cystic fibrosis and pregnancy
with cystic fibrosis in 2000 will be 50 years.2 The first successful pregnancy in a woman with
Survival into adulthood brings with it issues of cystic fibrosis was reported in 1960.8 She delivered a
sexuality, fertility and potential pregnancy. This healthy premature baby but died 5 weeks
paper provides an overview of the current issues postpartum from respiratory failure. Initial
surrounding cystic fibrosis and pregnancy. reports9,10 suggested high maternal and perinatal
mortality and pregnancy generally was
Sexuality and fertility discouraged. In the subsequent four decades,
In the 1980s, surveys3 showed that most people with however, the prognosis of cystic fibrosis has
cystic fibrosis were not married or sexually active. improved and attitudes towards pregnancy have
Recent studies4 have shown, however, that sexual gradually changed. There is an increasing trend for
adaptation is normal and that sexual activity is not women with cystic fibrosis to undertake pregnancy.
delayed when compared with adolescents without This can pose a challenge to the medical team but
cystic fibrosis. the emphasis now is on respecting women’s
autonomy and providing information, advice and
Menstruation generally occurs slightly later in girls support in helping them make decisions regarding
with cystic fibrosis and bodyweight is the most pregnancy and motherhood.11
significant determinant of menarche. Once
menstruation is established, the majority have The effects of cystic fibrosis
regular cycles, with amenorrhoea most likely to
occur in girls with poor lung function.5 Nearly all on pregnancy
men with cystic fibrosis are infertile but women Approximately 30–40 women with cystic fibrosis
with cystic fibrosis have relatively normal fertility, undertake pregnancy each year in the UK.12 Early
despite having thickened cervical mucus which reports9,13 suggested that pregnancy was associated
does not show the usual cyclical variation. An with high rates of miscarriage, premature delivery
Aberdeen study6 highlighted that 72% of women and stillbirth. Recent data, however, demonstrate
with cystic fibrosis thought that having children good fetal outcomes (Table 1). The proportion of
was important but that 26% had never discussed live births is now 70–90% and the spontaneous
fertility issues with a health professional. Other miscarriage rate is no higher than in the general
© 2009 Royal College of Obstetricians and Gynaecologists
20
