ERS/TSANZ Task Force Statement on the management of reproduction and pregnancy in women with airways diseases

Fertility in asthma patients

Different studies have found varied associations between the presence of asthma and subfertility in women [3, 5, 6]. More recent studies have suggested that asthma negatively influences fertility with increased time to pregnancy and reduced birth rate [3, 6]. The time to pregnancy in women with asthma increases with maternal age and use of fertility treatment increases especially in those over 35 years of age [3], which may suggest that the severity of the disease or the duration of exposure to low-grade inflammation has a negative influence on fertility.

Epidemiological data attempting to clarify the relative effects of asthma medications and underlying asthma severity on fertility is inconclusive. Gade et al. [3] found an association between untreated asthma (defined as no regular inhalers) and time to pregnancy, as well as between use of daily inhaled corticosteroid (ICS) and longer time to pregnancy, although it was not possible to differentiate between asthma severity per se and the level of treatment [3]. However, this was not found in a subsequent clinical study [7]. Recently, interesting data from the Screening for Pregnancy Endpoints (SCOPE) study have shown that women with self-reported asthma only using short-acting β₂-agonists (SABAs) had prolonged time to pregnancy and reduced fertility [6], yet those with self-reported asthma who were prescribed ICS had no reduction in fertility. This suggests that undertreatment of asthma with systemic inflammation may reduce fertility [6]. Given this data, women with asthma should be informed of the risk of reduced fertility especially if not on maintenance ICS therapy and particularly above 35 years of age [7, 8].

Fertility in CF and bronchiectasis

The life expectancy of people with CF has been increasing due to improved treatments [4, 9], which has resulted in an increasing number of men and women with CF living into adulthood and wanting to have children. In both cases, it is recommended that the partner is tested for cystic fibrosis transmembrane conductance regulator (CFTR) mutations to allow appropriate discussions regarding prenatal testing and pre-implantation genetic diagnosis [10, 11].

It is well known that most men with CF (95%) are infertile because of blockage or absence of the vas deferens, which requires in vitro fertilisation (IVF) intervention for pregnancy [10]. In contrast, although CFTR is expressed in the cervix [12] and the water content of cervical mucous does not show the normal menstrual cycle variation, the fallopian tubes remain patent in the majority of adult women with CF [13]. Fertility in women with CF can be worsened by poor nutrition leading to irregular ovulation or secondary amenorrhoea. A recent multinational survey showed that 35% of women with CF experienced subfertility compared with 5–15% in the general population [14]. Older age and pancreatic insufficiency were associated with subfertility, while lung function and body mass index were not associated with subfertility. CF-related diabetes (CFRD) was present in 16% of women with normal fertility and 23% of the subfertile group (p=0.02), and those with CFRD were significantly less likely to attempt conception. In cases of reduced fertility, IVF may be necessary [14]. Recently, a French registry study of women with CF who reported a pregnancy between 2001 and 2012 found that 14 out of 29 women with pre-gestational diabetes reported medically assisted conception compared with 51 out of 160 women without CFRD (p=0.06) [15].

In women with CFRD it is expected that good glycaemic control will improve the ability to conceive and reduce the risk of miscarriage and congenital malformations, as found in pre-conception care for non-CF women with diabetes [16]. Gestational diabetes is common, so current guidelines recommend an oral glucose tolerance test (OGTT) should be performed in all women with CF before pregnancy and repeated during pregnancy [17–19].

In contrast to the knowledge concerning fertility issues in asthma and CF, little is known about these issues in bronchiectasis due to other causes. In general, most clinicians will manage fertility using a similar approach of maximising lung function and nutrition, and minimising chronic infection.

Considerations from the Task Force

Tables 2–5 present the Task Force summaries for airways medications [23–81]. Please note the summaries are not based on randomised controlled trials.

• Compatible. In general, this option is considered the drug of first choice, which should be tolerated during pregnancy and/or breastfeeding, as there is sufficient anecdotal evidence through use during human pregnancies that the embryo/fetal risk is very low or nonexistent.

• Probably safe. In general, there is limited trial experience during human pregnancy (or during breastfeeding), but the characteristics of the drug and/or drugs in the same class suggest low risk.

• Possibly safe. This drug is considered for second-line use, if better-tested treatment options fail. Direct maternal benefit is thought likely to outweigh potential risk during pregnancy and/or breastfeeding, although exact risks are unknown.

• Breastfeeding. Please note that all listed refer to use in full-term, healthy, exclusively breastfed babies from mothers taking a listed drug at the standard recommended dose(s) and duration(s).

In all cases, the clinician should compare the benefits and risks of each medication.

In conclusion, the use of any drugs during pregnancy and lactation needs to balance the maternal risk of therapy versus no therapy and the fetal risk of uncontrolled maternal disease with the risk of therapy on the newborn.

In women with asthma, inhaled medications have been used for many years without documentation of adverse effects on the fetus. In contrast, the effect of undertreated asthma resulting in a severe exacerbation with maternal hypoxia is well described to have adverse effects on the fetus.

Monoclonal antibodies are not likely to cross the placenta in sufficient quantities to cause fetal harm and should be continued during pregnancy if required for asthma control in the mother.

For women with bronchiectasis and CF, routine therapy with inhaled bronchodilators, corticosteroids antibiotics and mucolytics should continue to maintain maternal stability throughout pregnancy. Any risks associated with systemic antibiotics vary depending on the agent and selection should be based on clinical grounds for the mother balanced with risk to the fetus.

Natural history of pregnancy in women with asthma

Asthma severity can change during pregnancy [92], with a later retrospective cohort of 1087 pregnancies showing 36% had improved asthma symptoms, 41% had unchanged symptoms and 23% had worsened symptoms during pregnancy [93]. Previous data suggested that during pregnancy asthma was unchanged in 33% and asthma was improved in 28% [94]. This has led to the concept that “one-third improve, one-third are unchanged and one-third experience increased asthma symptoms” [93, 95, 96]. In a recent study of 92 women, the proportion who deteriorated was smaller than this one-third hypothesis [97]. Prospective studies have shown a higher frequency of asthma exacerbations among those with more severe underlying disease. In general, the first trimester and the last month of pregnancy are relatively free of exacerbations. During the second and third trimesters increased asthma symptoms and need for medications is common [96, 98].

The change during pregnancy might be related to specific phenotypes, such as allergic or eosinophilic phenotypes, with rhinitis as an indicator of atopy associated with worsening asthma during pregnancy [85]. Asthma exacerbations in overweight or obese women during pregnancy are twice as frequent as in healthy weight women with asthma [86].

Miscarriage, premature delivery and other obstetric complications associated with asthma

International guidelines on the management of asthma during pregnancy are consistent in their main recommendations [99, 100]. Most recommend monthly monitoring of symptoms due to the unpredictable course of asthma during pregnancy, which should include both obstetric care providers and a multidisciplinary respiratory team. A qualitative study reported that Australian midwives lacked confidence and certainty about their role in asthma management during pregnancy [101]. Barriers to midwife involvement included a lack of knowledge about asthma during pregnancy, lack of time and a lack of referral pathways to asthma specialists [101].

Maternal asthma is associated with reduced fetal growth (low birthweight) due to a combination of pre-term delivery and intra-uterine growth restriction (small for dates). Following pregnancies with a moderate-to-severe asthma exacerbation, there is an increased risk of pre-term birth, low birthweight [102, 103] and small for gestational age [104]. A meta-analysis of 13 studies found a 46% increased risk of low birthweight compared with women without asthma [102]. Maternal asthma is associated with increased risks of pre-eclampsia [102], gestational diabetes [105], caesarean section [105], perinatal mortality (driven by neonatal death) [27] and neonatal hospitalisation at birth [27].

Active asthma management during pregnancy was found to lessen the increased risk of pre-term birth [102], neonatal hospitalisation [27] and gestational diabetes [105]. Exhaled nitric oxide fraction-guided management was reported to reduce asthma exacerbations [83], reduce neonatal hospitalisation [83], reduce infant bronchiolitis [106], and reduce the later development of childhood wheeze and asthma [106]. There is a small but statistically significant increased risk of congenital malformations (relative risk 1.11, 95% CI 1.02–1.21) in women with asthma [27]. This increased risk does not appear to be associated with maintenance asthma medication, with large studies demonstrating the relative safety of ICS use in relation to congenital malformations [107, 108].

Exercise, nutrition, asthma and pregnancy

At present the recommendations for pregnant women with asthma are based on those for the general population [109]. Maternal exercise during pregnancy is beneficial for reducing the incidence of low fetal birthweight and is likely to be important for pregnancy in overweight or obese women [110].

Most pregnant women with asthma have vitamin D levels <75 nmol·L⁻¹ throughout gestation, which are associated with a greater risk of adverse respiratory outcomes in the infants. Those women with lower levels of vitamin D are at higher risk of having infants who later develop childhood asthma [111]. Vitamin D exposure during fetal development influences the immune system of the neonate, which can contribute to protection from asthma-related outcomes in early life [112].

Breastfeeding

A systematic review of observational studies on breastfeeding shows that breastfeeding is effective at preventing allergy in infants, with the most pronounced effect in infants at higher risk of allergy, when breastfed for at least 4 months [113]. However, longer follow-up studies of older children and adults found no protective effect against atopy and asthma, suggesting that the protective effect of breastfeeding delays rather than prevents development of atopy and asthma [114].

Summary

Most cohort studies demonstrate that asthma is associated with adverse outcomes during pregnancy that are related to poor asthma control, especially acute exacerbations. Some of these adverse outcomes can be reduced by good asthma management. More randomised controlled trials of pregnant women with asthma will strengthen the recommendations [115].

Natural history of pregnancy in women with CF

While the older literature outlines increased risks of pregnancy-associated complications and miscarriages, this may have reflected worse nutrition and lung function in previous series. Since the initial case report in 1960 of a pregnancy in a woman with CF [117] with delivery of a viable fetus, it was soon noted that maintenance of the mother's health pre- and post-delivery was crucial. During the 1970s, Cohen et al. [118] collected data from 129 pregnancies among 100 women with a mean age of 20.7 years. Medical abortion was undertaken in 19% with an additional 5% having a spontaneous miscarriage. Maternal mortality in this original series was 12% within 6 months of delivery and 18% within 2 years. More recent studies have shown similar levels of medical abortions, but better maternal survival [119].

Case series of women with CF have shown increased risk of miscarriages [120], both early and mid-pregnancy, with the chance of live birth reduced in mothers with worse lung function. For that reason, some authors have suggested that a pre-pregnancy forced expiratory volume in 1 s (FEV₁) >60% predicted should be present before pregnancy is undertaken [121], while others suggest a cut-off of FEV₁ >70% predicted [122] and others have shown that those women with CF with an FEV₁ >80% predicted have better outcomes [123]. Clearly risks are lower in those with better lung function pre-pregnancy, but there have been successful pregnancies in women with FEV₁ <40% predicted [119, 124]. As maternal and fetal risks are increased in those with lower pre-conception FEV₁, pregnancy in a woman with CF and advanced lung disease can be difficult to manage.

Pre-existing CFRD is an important consideration during pregnancy. A recent analysis of the French CF Registry compared outcomes of pregnancy in women with CF with and without pre-gestational diabetes, noting that women with CFRD were older at the time of first pregnancy (29 versus 26 years; p=0.05) and had worse lung function (FEV₁ 50% versus 67% predicted; p=0.03) [15]. There was an increased rate of caesarean section in diabetic women compared with nondiabetic women (48% versus 21%; p=0.005) [15]. However, there was no significant impact on fetal growth or pre-term delivery and there was no difference in long-term outcomes in the two groups [15].

Risks to the fetus

While there has long been an increase in the risk of miscarriages in women with CF having pregnancies, earlier studies did not demonstrate any difference in congenital anomalies [17, 116]. However, a recent population study of pregnancies in California between 2005 and 2008 compared the outcomes of pregnancies from mothers with CF versus those of the general population. They reported a significant increased risk of congenital anomalies (14.3% versus 6.4%; p<0.005) [120].

Long-term maternal outcomes

Overall, the US Cystic Fibrosis Foundation National Patient Registry demonstrated that women with CF who reported a pregnancy showed no reduction in overall survival compared with age-matched women with CF who did not report a pregnancy [124]. Data from the Epidemiologic Study of Cystic Fibrosis (ESCF) showed that pregnancy and motherhood were not associated with accelerated loss of lung function, but were linked to more pulmonary exacerbations and increased clinic visits [125]. However, a single-centre review has shown that following pregnancy, of 15 women with FEV₁ <50% predicted, seven died within 8 years of delivery [126].

There are multiple cases of women colonised with Burkholderia species, in particular the Burkholderia cepacia complex, who have had poor outcomes following pregnancy [127, 128]. Tanser et al. [129] noted a particularly difficult combination of poor lung function (FEV₁ <50% predicted) with B. cepacia colonisation where maternal mortality was high. At present, there is little information concerning pregnancy in women colonised with mycobacterial species, but many of the drugs used to treat these organisms are not recommended for use during pregnancy.

CFTR modulators

With the recent introduction of ivacaftor and lumacaftor as CFTR modulators, there have been a number of reports of these agents being used during pregnancy and during breastfeeding [56, 58, 130, 131]. To date, there have been no reports of abnormalities associated with these agents, but the relative benefits of remaining on CFTR modulator therapy before, during and after pregnancy would need to be balanced against any theoretical risks.

Suggestions for management during pregnancy

Women with CF who are pregnant should be reviewed in a multidisciplinary CF clinic at least monthly for the first and second trimesters, increasing to every 2 weeks or more frequently as necessary. It is recommended that more frequent follow-up during pregnancy be undertaken in those with more severe disease. As many women with CF can develop gestational diabetes and require insulin, the European Cystic Fibrosis Society recommends that an OGTT should be performed pre-pregnancy and repeated at 20 weeks, and again at 28 weeks if necessary [17]. The US [18] and Australian guidelines [19] suggest an OGTT should be performed pre-pregnancy and at 12–16 and 24–28 weeks gestation in all women with CF.

Nutritional considerations

Women with CF are expected to gain an overall 10–12 kg during pregnancy, as would the general obstetric population [17]. Fat-soluble vitamins and other supplements should continue during pregnancy in order to maintain normal levels of all vitamins [17]. In addition to folate and iron supplements as recommended for all pregnant women, calorie supplements may also be required to provide sufficient energy intake to allow for normal weight gain during pregnancy.

Increased intensity of maintenance treatments to maintain stability during pregnancy

During pregnancy the ongoing routine care of the CF adult needs to be encouraged. As reflux increases during pregnancy [17], adjustments to physiotherapy regimens may be necessary. Many women change their daily routine to have more frequent smaller meals and time their physiotherapy before meals to minimise reflux. A high degree of individualisation is required as there are no studies confirming the “best option”.

Loss of lung function

In general, it is expected that women with CF will transiently lose lung function to a similar level as other pregnant women. However, there are very few studies outlining the relative drop in lung function for women with CF with differing levels of lung function pre-conception. Many studies have compared pregnancy outcomes against pre-pregnancy lung function. In general, higher values of FEV₁ pre-pregnancy are associated with better outcomes and complete return of lung function after pregnancy [123]. Importantly, long-term lung function in the group of women with CF who have had a pregnancy is similar to those who have not been pregnant [125].

Physiotherapy issues: exercise, airway clearance and pelvic floor

Regular airway clearance and exercise should be an established routine before pregnancy and maintained through pregnancy, with modification as necessary, including avoiding contact sports [132, 133]. Although there are no trials comparing physiotherapy interventions in pregnant women with CF, options include the active cycle of breathing technique, autogenic drainage, positive expiratory pressure (PEP) therapy, oscillating PEP and intrapulmonary percussive ventilation including the MetaNeb device [134]. Therapy may need to be adjusted as the fetus grows.

Upright sitting is often the most comfortable position for airway clearance during pregnancy, and instruction to maintain a neutral lumbar spine during airway clearance for optimal bladder control and prevention of back problems may be necessary [134]. Left and right side lying, horizontal or head up may be more effective positions in some women, but most clinicians avoid the supine horizontal position, especially during the second and third trimesters to minimise pressure from the gravid uterus on the inferior vena cava, which may decrease venous return and cardiac output.

The growing fetus results in upward pressure on the diaphragm and downward pressure on the pelvic floor. Particularly during the third trimester, increased stress on the pelvic floor may result in urinary leakage, especially during coughing. In most clinics, women with CF are taught effective pelvic floor strength and endurance exercises with referral to a physiotherapist experienced in this area.

Post-transplant: is this a second chance?

An important paper from Gyi et al. [135] in 2006 outlined 10 CF lung transplant recipients who became pregnant. There were nine live births and one therapeutic abortion. Five of the nine births were premature. Four women died of rejection within 3 years of pregnancy. More recently, a retrospective review of pregnancies reported to the US National Transplant Registry detailed 30 pregnancies in 21 women post-transplant [136]. They described 18 live deliveries at an average gestation of 34 weeks, with a weight of 2.2 kg. There were 11 complications including two neonatal deaths in the offspring and five of the 21 mothers died during follow-up. In summary, these studies have shown that pregnancy in lung transplant recipients is “feasible, but should still be regarded as a risky undertaking” [135].

Summary

In contrast to the literature concerning randomised trials of management of pregnancy in women with asthma, most studies in women with CF considering pregnancy are observational, without any prospective evaluation of any particular therapies. The increasing number of women with CF who have relatively preserved lung function and nutrition achieving adulthood means that there is an ever-growing number of women considering pregnancy. Management during pregnancy is generally optimised by cooperation of the CF multidisciplinary team in conjunction with the obstetric team and most established adult CF centres have close links with their local obstetric colleagues using “high-risk pregnancy clinics” to coordinate the complex management required.

Natural history of pregnancy

In contrast to other airways diseases, the natural history of pregnancy in women with bronchiectasis is much less documented. One of the early case series described three women with idiopathic bronchiectasis who successfully completed term pregnancies [137]. Women with primary ciliary dyskinesia are often subfertile, but not completely infertile, as there are case reports of natural pregnancies [138]. Women with bronchiectasis secondary to other diseases such as immunodeficiency will require consideration of both their primary underlying disease as well as their bronchiectasis when contemplating pregnancy.

Miscarriage rates

Although women with asthma have a slightly increased risk of miscarriage, the risks in women with bronchiectasis are at present unknown. Further studies are now necessary to document whether there is an increased risk of miscarriage in women with established lung disease and whether the different causes of bronchiectasis have different risks during pregnancy. Extrapolating from other airways diseases, it would be expected that all pregnancy-related problems would be increased, reflecting the lack of respiratory reserve dependent on lung function.

Suggestions for follow-up

As there is little literature available, management during pregnancy must be made on clinical acumen. Overall, it is expected that increased clinic visits would be necessary in the second and third trimesters as respiratory compromise from the growing fetus limits airway clearance.

Exercise and nutrition

Clinical experience suggests that exercise and nutritional requirements for optimisation of lung function during pregnancy would be similar to those of CF, except with regard to pancreatic dysfunction. Maintenance of regular moderate intensity exercise and good nutrition throughout pregnancy would be recommended, in keeping with the general population [132].

Physiotherapy

Similar to the issues for women with CF, the growing fetus will impact on the respiratory system and pelvic floor. Optimal airway clearance should continue throughout pregnancy to prevent sputum retention. Modification to airway clearance techniques and physical exercise will be required as pregnancy progresses. No specific programmes for women with bronchiectasis are available in the literature, so most physiotherapists would tailor their therapy to reflect the management of women with CF who become pregnant. Management of the pelvic floor to prevent urinary leakage especially during airway clearance techniques and coughing will most likely require similar interventions as outlined in the section on pregnancy in women with CF.

Introduction

Labour is often divided into three stages. The first stage of labour involves the gradual shortening and dilatation of the cervix up to 10 cm. This process may take up to 36 h in the primipara and is shorter in those who have previously had children. The second stage of labour involves the descent of the fetus through the birth canal and delivery, which may take between 30 min and 2 h. The third stage of labour comprises the delivery of the placenta, which may take up to 1 h if not induced.

Labour

During labour, the resting hyperventilation of pregnancy increases further. This still exceeds the increased oxygen demand of labour and delivery, so most healthy women will not have hypoxia or hypercapnia. However, pain and anxiety can lead to rapid shallow breathing and a decrease in alveolar gas exchange. As expected, hypoxia, hypercapnia and respiratory acidosis occur more readily in women with more severe pulmonary disease [139].

There are no published studies specifically addressing intra-partum management in women with respiratory disease. Most applicable guidelines suggest evaluation prior to pregnancy or in early pregnancy, to develop a detailed and individualised management plan for birth, including place and mode of delivery, appropriate monitoring during delivery (e.g. arterial oxygen saturation measured by pulse oximetry), provision of necessary pulmonary interventions (e.g. inhalers for asthma, physiotherapy for CF and supplemental oxygen as required), and invasive and noninvasive monitoring during and after birth [17, 100]. Adequate pain relief during labour is a high priority for women with CF and other chronic suppurative lung diseases, with a normal vaginal delivery being highly desirable in order to minimise post-delivery complications [140].

Obstetric considerations

As noted in the section on pregnancy in women with asthma, some studies have shown an association between asthma and increased caesarean section rate [141–144], but this may be due to planned caesarean section or an increased rate of induction of labour rather than due to any direct effect of asthma. Moreover, there are no trials to address whether there is any clinical benefit of caesarean section versus vaginal birth in women with respiratory disease. Advantages of natural vaginal delivery include quicker recovery in those with bronchiectasis or CF and some studies suggested that children born by caesarean section in mothers with asthma may have an increased risk of subsequent asthma [145], while other studies did not [146].

Most guidelines suggest that the decision for caesarean section should be made for obstetric indications [17, 147]. However, modification of the birth plan in women with significant airways disease may be required [2]. This may include elective delivery to ensure specialist staff are available, modifications to the second stage of labour to ensure the expulsive phase of labour is not prolonged, adequate analgesia and oxygen as required, and avoidance of general anaesthetic if possible. In women with CF, adequate maintenance of hydration, nutrition and blood glucose control also need to be considered [17].

The use of prostaglandin E₂ for induction of labour or oxytocin (for augmentation of the second and third stages) has not been associated with worsening lung function or asthma exacerbation [148]. However, a recent case report outlined a woman with severe bronchiectasis (FEV₁ 32% predicted) who developed acute hypoxaemia when given oxytocin for uterine contraction [149]. As the hypoxaemia was resistant to supplemental oxygen it was suggested that the oxytocin had increased shunting through the damaged lung. We could find no studies to assess active versus physiological management of the third stage of labour in women with respiratory disease; however, the NICE guidelines advocate an active third stage of labour for women as it is associated with a lower risk of post-partum haemorrhage and/or blood transfusion [150]. As discussed in the following, several of the uterotonics used for post-partum haemorrhage have significant adverse effects on the respiratory system; ergotamine may cause bronchospasm, particularly in association with general anaesthesia [151], so oxytocin is the uterotonic of choice for the active third stage of labour.

Anaesthetic considerations

Pre-delivery evaluation and multidisciplinary planning is the cornerstone in managing pregnant women with respiratory disease. Adequate pain relief will reduce anxiety and maternal stress. Early epidural analgesia with local anaesthetics (with or without opioids) for labour pain is the preferred method. Lumbar epidural analgesia can be extended into safe and proper anaesthesia in the case of an emergency caesarean section, thereby avoiding the need for airway management. Systemic opioids can provide moderate pain relief, but will suppress cough, suppress ventilation and may induce bowel obstruction especially in women with CF. A cautiously titrated epidural for analgesia is generally preferred [152].

Respiratory considerations

Exacerbations of asthma are uncommon during labour [98]. It is generally accepted that usual asthma medications should be continued during childbirth [147]. Inhaled or parenteral β-agonists have been used as a tocolytic in women with threatened pre-term labour [153] or uterine hyperstimulation, but there is no published evidence that inhaled β-agonists used for asthma treatment affect the course of labour. In women receiving oral steroids (7.5 mg daily for at least 2 weeks) there is a potential risk of maternal hypothalamic–pituitary–adrenal axis suppression, which has led to the widespread practice of giving “stress dose” hydrocortisone intravenously during active labour and during caesarean section [147]. For women with more severe disease, assisted ventilation strategies may be considered with appropriate intensive care unit backup. In women with established bronchiectasis, positive end-expiratory pressure may assist by splinting open smaller airways to prevent dynamic airway closure and hence better mobilisation of secretions.

Role of physiotherapy

Pain, shortness of breath and low oxygen saturation during labour are reported in some healthy women during parturition. Women with chronic airways disease would be expected to experience these symptoms at an increased frequency or severity during labour, although this has not yet been documented. Oxygen therapy should be provided to maintain normal saturations as per standard care. Bronchodilator therapy and assistance with sputum clearance may be required by some women during labour.

Summary

There is little evidence at present to guide management decisions in the intra-partum period. Most disease-specific guidelines offer recommendations based on low-grade evidence or consensus opinion. Caesarean section appears to be more common in this population, likely for multifactorial reasons. Active management of the third stage of labour may be considered using oxytocin without ergotamine in women with lung disease.

Introduction

The post-partum period is a particularly vulnerable time for the medically unwell woman, with risks of cardio-respiratory decompensation, infection, sepsis and haemorrhage. Guidelines advocate that the birth management plan includes the first few days post-partum, with recommendations on the location for post-delivery care in the maternity unit or respiratory/high dependency unit balancing the individual needs of the woman and her baby.

Obstetric considerations

Post-partum haemorrhage, defined as ≥500 mL of bleeding within 24 h of birth, occurs in 2.5–5% of women post-delivery. While observational studies have suggested that there is no increase in post-partum haemorrhage in women with lung disease, a meta-analysis showed that there were significant increases in both ante-partum and post-partum haemorrhage [105].

Prostaglandin F_2a used to treat post-partum haemorrhage due to uterine atony may cause bronchoconstriction [151]. Although not associated with clinical exacerbations [143], it is recommended not to be used in women with asthma [2]. Ergometrine may also cause bronchospasm particularly in association with general anaesthetics [151]. Women on long-term systemic corticosteroids have an increased risk of poor wound healing and infection, which is especially important after caesarean section in those with suppurative lung disease who require daily airway clearance to maintain health. As the risk of post-partum venous thromboembolic disease is increased in those with immobility [154], it is important that women are mobilised or thromboprophylaxis with low-molecular-weight heparin may need to be considered.

Anaesthetic considerations

Optimal post-partum analgesia allows early mobilisation with minimal side-effects. Pain should be assessed and managed in a multimodal way. The respiratory depressant effect of opioids and suppression of cough are particularly important in women with respiratory diseases. The level of care and monitoring post-partum should be considered in a multidisciplinary setting balancing both obstetric and airway risks for the mother with any risks for the newborn.

Respiratory considerations

Some older studies have noted an increased risk of post-partum asthma exacerbation in women having caesarean sections [143], but this group was generally not taking ICSs. For CF and bronchiectasis, the outcomes are less well known.

Physiotherapy in the postnatal period

Physical support for the mother is important after birth to allow sufficient time to inhale medications, perform airway clearance therapy and exercise to prevent exacerbations. These factors are more important after caesarean section, especially in those with underlying airways disease (see previous sections).

Breastfeeding issues

Breastfeeding is universally advocated in international maternity guidelines. However, for some women with significant airways disease, nutritional, pharmacological and other factors need to be considered. Medications commonly used in women with airways disease and their evidence for use during breastfeeding are described in tables 2–5.

It is generally accepted that the composition of salts and proteins in the breast milk from a woman with CF are within normal limits [155]. As breastfeeding increases maternal protein, calcium and overall caloric requirements by ∼500 kcal·day⁻¹, an individualised approach is necessary especially for women with CF. These concerns should be balanced with the beneficial effects of breastfeeding overall (see tables 2–5).

Summary

While there is little evidence to guide management decisions in the immediate post-partum period and most disease-specific guidelines offer recommendations based on low-grade evidence or consensus opinion, post-partum haemorrhage may be increased in women with asthma and medications used for the management of post-partum haemorrhage may affect the respiratory system. Close collaboration between the obstetric and respiratory teams is necessary to optimise management.

Asthma

The decision to get pregnant was not influenced by having asthma and many women felt that it did not affect their pregnancy adversely. Some were very concerned about their asthma worsening during pregnancy or were worried about their ability to take care of their current offspring. Respondents were not confident that they could recognise the signs of asthma deterioration. Those women with a respiratory specialist valued having this input and many of those without a respiratory specialist would have liked to have seen one while pregnant. Many felt that primary care was not able to answer all questions about continuing medications and their effects on the offspring while pregnant and breastfeeding. Women accessed information from patient organisations and felt that asthma was often not adequately addressed during their pregnancy. Furthermore, options concerning birth and postnatal care were not adequately covered in all cases.

Cystic fibrosis

Women with CF are very aware of the difficulty in getting pregnant, the need for genetic testing and the impact of pregnancy on their health. Many respondents reported not getting pregnant due to fears of their child having CF and the impact on the health of the offspring. Some felt the risk of not being well enough to care for their child or potentially dying before their child was an adult was too big a chance to take. Some were worried that they would regret not trying to have children but felt conflicted due to these concerns. Many women reported that they could not afford fertility treatments, and some felt the length of time needed to get genetic tests and find a maternity unit that understands CF should be flagged with patients earlier. Some women felt surrogacy was their best chance of becoming a parent, although this is not available in all countries. Many women felt that having CF would be an issue for adoption. Many women felt that too much emphasis was placed on pregnancy and not enough priority was given to being a parent while living with CF. Women described the impact of having a child on their health, which was similar to the experience of the respondents with bronchiectasis described below.

Those who were or had been pregnant acknowledged that their pregnancy was not without worry, but that they were very motivated to have a child. Some reported having their children early due to concerns about their life expectancy. Several women stated that due to the impact of their first child on their health, they had chosen not to have another child. Women reported that they would appreciate networking with other mothers living with CF to discuss the practical issues of parenting. A recent online survey in Australia using closed-group moderated discussions showed that women with CF desired more information about childbearing, expressing concern about the safety of their CF medications during pregnancy [156].

Bronchiectasis

Overall, women reported that they did not give their condition much thought and were more focused on getting pregnant. However, some did report that they would not have more children due to the impact on their lungs. They described feeling “well looked after” with involvement and coordination between their respiratory and obstetrics teams. Some emphasised the impact as a parent with a chronic lung condition of having a small child who brings illnesses into the home. They felt that there should be more support about how to manage this issue. Women also related that the strain of caring for a newborn was very difficult given their condition and felt that further support for mothers with bronchiectasis was needed to mitigate these difficulties.

Summary

The patients’ perspective concerning conception, pregnancy and the postnatal period differs markedly between the three conditions. Those with asthma and bronchiectasis often do not consider their condition when deciding whether to try to conceive, in contrast to the protracted decisions for those with CF. While respondents with asthma reported not having access to respiratory professionals during their pregnancy, those with bronchiectasis did not have this problem. For CF, the issue raised was often the difficulty finding obstetric specialists who had experience of dealing with pregnancy in women with CF and the length of time taken for genetic testing and fertility treatments. In general, women with chronic airways disease felt that they were not adequately prepared for the postnatal period and parenthood in general.