Historically, midwives have been considered experts in normality, acting as the primary carers for clients experiencing uncomplicated pregnancy and birth (Royal College of Midwives, 2005). As a result of changes in public health, advancements in modern medicine, increased recognition of risk factors and the growing medicalisation of birth, the UK childbearing population are presenting to maternity services with a rising incidence of medical and social complexities (Smith and Dixon, 2008). Accordingly, the knowledge base and skills required for health professionals to provide safe, holistic midwifery care to the diverse UK childbearing population is growing (Smith and Dixon, 2008).
The traditional definition of midwives as experts in normality could now be considered too narrow, and modern midwifery can be defined as working in partnership with women to optimise physiological mechanisms of childbirth, while prioritising safe and holistic care and promoting cultural experiences of childbearing (International Confederation of Midwives, 2017). Seemingly, modern midwives must exist in both the medical and social models of care, but stay true to the woman-centred principles of midwifery. Caseload midwifery refers to a small team continuity model where 4–6 midwives, known to the woman and family, provide care throughout the antenatal, intrapartum and postnatal continuum (Cumberlege, 2016). With NHS care moving towards small team, caseload midwifery, what will the growing population of women with very complex pregnancies experience? Arguably, all women benefit from midwifery care, regardless of risk status (World Health Organization, 2019).
A key example of a population for whom modern medicine has made childbearing a possibility is those with cystic fibrosis. Over the past 60 years, median life expectancy has increased from a few months to in excess of 50 years (Dodge et al, 2007; Elborn, 2016). The first successful pregnancy took place in 1960, with pregnancy since becoming commonplace (Lau et al, 2011). From clinical experience, pregnancy associated with cystic fibrosis is heavily medically managed by specialist doctors with little input from midwifery. This article will examine whether there is an argument to invest in protected caseload midwifery contacts for these women, in line with the National Institute for Health and Care Excellence (2008a), in addition to the care they receive from the obstetric and specialist teams.
Cystic fibrosis and pregnancy
Paramothayan (2019) describes cystic fibrosis as an autosomal recessive disorder affecting 1 in 2500 live births and cystic fibrosis is the most common inherited genetic disorder in the Caucasian population. A mutation within chromosome 7 results in a defective cystic fibrosis transmembrane conductance regulator, which impacts the respiratory, gastrointestinal and reproductive systems. The aim of cystic fibrosis care is to prevent respiratory infections, known as exacerbations, thus reducing the progression of bronchiectasis (widening of the bronchi(oles) eventually resulting in respiratory failure) (Burden et al, 2012). Additionally, treatment includes nutritional supplementation and psychological support. Simply, cystic fibrosis treatment is often a mixture of time-consuming physiotherapy, oral medication, nebulisers, nutritional supplements and intravenous therapy (Paramothayan, 2019).
Geake et al (2014) explain that the impact of pregnancy on lung function for those with cystic fibrosis is highly variable. Like all women, physiological adaptations to pregnancy occur, including increases in blood volume, cardiac output, resting ventilation and insulin resistance. Additionally, in later pregnancy the gravid uterus causes a decrease in total lung capacity and residual lung volume. The unpredictable variability in pathophysiological response to pregnancy with cystic fibrosis necessitates a multidisciplinary team approach across the cystic fibrosis and obstetric specialties, and individualised care. Complications associated with pregnancy are pulmonary exacerbations, diabetes (pre-existing and gestational) and pre-eclampsia (Burden et al, 2012).
Historically, it was thought that pregnancy in cystic fibrosis would carry greater risk of spontaneous miscarriage, premature delivery and stillbirth; however, evidence indicates these rates are comparable to the general population (Ciavattini et al, 2012). Pregnancy is a greater clinical risk for those with poor lung function and low body mass index (Whitty, 2010); however, pregnancy has been shown not to impact long-term survival (Elborn, 2016.). Furthermore, Goss et al (2003) investigated 12-year survival for women with cystic fibrosis following pregnancy. A total of 680 participants demonstrated that pregnancy did not impact 10-year survival, compared with 3327 non-pregnant controls, although the pregnant participants utilised more health resources, indicating poorer physical health.
Undoubtedly, the clinical management decisions for those with cystic fibrosis fall widely outside a midwife's sphere of practice. However, the clinical presentation of a woman is just one aspect; midwives must view the full holistic picture. From clinical experience, all antenatal care has been provided by doctors, focused primarily on physical health. However, cystic fibrosis is known to be associated with an increased rate of depression and anxiety (Quittner et al, 2014), and pregnancy is known to have the potential to exacerbate these issues (Renton et al, 2015).
The psychosocial impact of cystic fibrosis
Individuals living with cystic fibrosis are expected to complete burdensome and time-consuming daily treatment regimes and may suffer poor physical health, regardless of adherence (Bishay and Sawicki, 2016). As a result, those with cystic fibrosis may have significant psychological, physical and social challenges to participating in daily life (Yohannes et al, 2012). Recognition of the importance of these factors has led to the development of the health-related quality of life instrument. This instrument considers the physical, social and psychological functioning of the individual in a bid to viewing holistic wellbeing (Cronly et al, 2019). Arguably, for individuals living with a chronic life-limiting condition, health-related quality of life is more important than physical health alone.
A systematic review of 23 studies examining physical health variables suggests that pulmonary exacerbations are the strongest predictors of self-reported health-related quality of life (Cronly et al, 2019). Conversely, Yohannes et al (2012) examined the impact of depression and anxiety variables on health-related quality of life and found them to have a more significant impact than physical health variables. However, the study may be limited, as the 129 participants all originated from the same clinic. In support of this argument, Cronly et al (2019) conducted a national, cross-sectional study in Ireland and found that mental health was more strongly associated with health-related quality of life than physical health. Crucially, when mental health was controlled for, physical health had little independent effect. However, the sample size was only 174 participants.
Quittner et al (2014) conducted a large epidemiological study across nine countries, including 6088 individuals with cystic fibrosis, to find that rates of depression and anxiety were 2–3 times higher than community controls. Recommendations suggested those with cystic fibrosis are frequently screened for depression and anxiety, and treatment offered as appropriate (Quittner et al, 2014). The relationship demonstrated between mental health and health-related quality of life supports the importance of this intervention (Cronly et al, 2019).
Contributing to the psychosocial impact of living with cystic fibrosis is the arduous daily regime of physiotherapy and treatments prescribed (Cronly et al, 2019). Adherence to this regime is vital in maintaining a good degree of physical health, yet compliance is notoriously poor (Llorente et al, 2008). Llorrente et al (2008) found that of the treatments prescribed, adherence to physiotherapy was poorest for children and adults, as participants self-reported only 41.2% of the regime performed. Additionally, adherence to treatment regimens decreased with increasing age and severity of disease. Although, it must be noted the sample was exclusively female and only included 34 participants.
Oates et al (2019) objectively recorded the ratio of performed to prescribed daily physiotherapy using high-frequency chest wall compression vests in paediatric patients. The study found that for 110 participants, the mean adherence to the prescribed physiotherapy regime was 61%, with 29% of the participants performing <35% of the daily regime. Interestingly, this study compared objective records with self-reports. Self-reports suggested only 8% of participants performed <35% of the regime. Conversely, 66% self-reported adherence of >80% but, when objectively measured, only 35% of participants reached this threshold.
The study by Oates et al (2019) only included paediatric patients, and Llorente et al (2008) demonstrated a decline in self-reported compliance with increasing age and disease severity. Therefore, compliance is likely to be even lower in the adult population if measured objectively. Llorente et al (2008) found that self-reported adherence to nebulised medication was 61.8% across children and adults. Conversely, Hoo et al (2016) found that adherence to nebulisers in the adult population was as low as 33% when measured objectively. Adherence to treatment remains a major barrier to preserving good physical health in the cystic fibrosis population.
Smith and Wood (2007) argue that in children and adolescents, psychosocial factors and treatment adherence are interrelated. Recommendations to improve adherence include involving the patient and family in treatment planning, shared decision making and improved relationships between healthcare practitioners and families (Smith and Wood, 2007). A report by Siracusa et al (2014) documents extensive work to transform care provision and patient outcomes at a centre over a 10-year period. Pivotal to this change was incorporating patients and carers into the development team, as well as a collective vision to focus on the priorities and wellbeing of the families accessing the service (Siracusa et al, 2014).
Merely considering physical health in cystic fibrosis is insufficient; a holistic view is necessary. Arguably, mental health plays one of the most important roles in an individual's health-related quality of life. Additionally, prescribing an individualised regime to optimise physical health and anticipating compliance is futile without considering the individual holistically.
The psychosocial impact of childbearing
Jomeen (2017) describes pregnancy, childbirth and the puerperium as major life events that can have a significant impact on psychosocial wellbeing. This period is associated with major changes to lifestyle, social role, responsibilities and financial demands. Childbearing is considered a normal life event but can be experienced as a life crisis, especially for those already vulnerable to stress
Perinatal mental health refers to any mental health condition that occurs during pregnancy or within the first year postpartum (Viveiros and Darling, 2019). Noonan et al (2018) demonstrate the prevalence of perinatal mental health, estimating that within the perinatal period, 25–45% of women experience anxiety and 10–25% experience depression; this does not include other perinatal mental health conditions. Furthermore, maternal suicide is the leading cause of direct maternal death within the perinatal period, accounting for deaths in 2.9 of every 100 000 maternities (Mothers and Babies: Reducing Risk through Audits and Confidential Enquiries across the UK, 2018). Madden et al (2018) suggest that midwives are uniquely positioned to recognise and facilitate treatment for perinatal mental health. Cumberlege (2016) agrees and states that perinatal mental health recognition and treatment is a key element of care that must be improved.
Nagle and Farrelly (2018) conducted qualitative research to investigate women's experience of having their perinatal mental health assessed. Women felt that screening tools appeared to be a ‘tick-box exercise’ and preferred being given time to discuss their perinatal mental health with care professionals. Additionally, perceived busy staff workloads and lack of continuity of carer were considered barriers to disclosing information (Nagle and Farrelly, 2018). Cumberlege (2016) supports this notion, suggesting that individualised care and continuity of carer may facilitate trusting relationships, and improve access to perinatal mental health services.
Women with cystic fibrosis who become mothers are vulnerable to psychosocial issues related to both cystic fibrosis and pregnancy, in addition to further issues specific to their situation. In a study by Renton et al (2015), 37.5% of cystic fibrosis participants were recognised to go on to develop postnatal depression (this did not include other perinatal mental health conditions). Much of the literature concerning pregnancy and cystic fibrosis focuses on physical health outcomes. Cammidge et al (2016) considers the psychosocial impact of becoming a mother with cystic fibrosis. They used a grounded theory approach in a study with 11 participants who described feelings of guilt at the prospect of parental absence for hospital stays or mortality. Furthermore, Jessup et al (2018) performed a phenomenological study, including mothers and fathers with cystic fibrosis, and reported that parents described a huge adjustment when their children were born, and similar guilt at the prospect of the burden their children may experience.
Cammidge et al (2016) found that during pregnancy, women felt grateful for the care received surrounding physical health but wanted more emotional support. Moreover, women suggested that conversations sometimes felt impersonal or too centralised on physical health, and they wished for more discussion surrounding new life as a mother. Kazmerski et al (2016) supports this view. Their qualitative investigation of 22 participants found that women with cystic fibrosis were seeking some experience of normalcy surrounding childbearing, and excitement at becoming parents. Cammidge et al (2016) recognised the importance of also supporting the transition to parenthood for the partner of someone with cystic fibrosis, who may become the child's primary carer. In terms of treatment adherence, Cammidge et al (2016) and Jessup et al (2018) found that following initial disruption, compliance improved beyond pre-pregnancy rates following childbirth and the child was described as a motivating factor to stay well.
In summary, women with cystic fibrosis have higher rates of mental health issues (Renton et al, 2015) and have a desire for improved emotional support and a sense of normalcy surrounding pregnancy (Cammidge et al, 2016). Adherence to treatment is thought to be interrelated with psychosocial factors and overcome by placing the patients at the centre of care (Siracusa et al, 2014). Additionally, adherence is improved when a child is introduced, as they can be a motivating factor (Jessup et al, 2017).
Cumberlege (2016) states personalised care, with true woman's choice and continuity of carer, improves women's childbearing experiences and physical and mental health outcomes. Therefore, caseload midwifery, in addition to multidisciplinary team care, is a legitimate intervention to address the issues discussed above. Conversely, pregnant women with cystic fibrosis already have an arduous timetable of healthcare appointments and the time taken to see a midwife may be viewed as superfluous. In line with recommendations produced by Cumberlege (2016), pregnancy and birth care should be planned based on the individual woman and her choice, and caseload midwifery should be offered.
Birthing with cystic fibrosis
Factors relating to cystic fibrosis can occasionally necessitate preterm delivery by caesarean section; however, most women choose vaginal birth at term (Burden et al, 2012). Thorpe-Beeston et al (2013) performed a retrospective study on a single centre from 1998–2011, investigating 48 pregnancies in 41 women. Half of these women went on to have a vaginal birth, Caesarean section was associated with women with poorer lung function and obstetric necessity, and 46% of the babies were born prematurely. In two cases, premature labour was spontaneous, which is consistent with the rate in the general population; the remainder were as a result of preterm induction of labour. Over a third (35.4%) of women required insulin to manage diabetes within pregnancy.
Conversely, Burden et al (2012) performed a retrospective study on a single centre from 2003–2011 and demonstrated a 100% vaginal birth rate, although this study only included 15 pregnancies with 12 women. Diabetes was more prevalent than in Thorpe-Beeston et al's (2013) study; it was observed in 57% of participants. It is possible the 5-year discrepancy in the commencement of the studies could account for such different birth outcomes. However, Renton et al (2015) performed a retrospective review from 2003–2013 investigating 15 pregnancies in eight women and reported a 54% rate of vaginal delivery. Arguably, the sample sizes for these studies are too small to draw conclusions.
In Burden et al's (2012) study, 71% of participants underwent induction of labour by 38 weeks for either worsening lung function or because of geographical location. The rate of induction of labour was not provided by the other studies. Geake et al (2014) suggested that delivery be planned in an obstetric unit with a co-located specialist cystic fibrosis centre. There are only 26 specialist cystic fibrosis centres across the UK, with just one in Northern Ireland and Wales respectively (Cystic Fibrosis Trust, 2019). The data from Burden et al (2012) indicate that it is accepted practice to induce labour in women who must travel to specialist cystic fibrosis centres. When compared with spontaneous labour, induction of labour is associated with reduced efficiency of labour, leading to more medical intervention and increased pain (National Institute for Health and Care Excellence, 2008b). This represents a health inequality based on geographical location: if you happen to live further from a cystic fibrosis specialist centre you are more likely to experience induction of labour and the associated risks. The relatively small prevalence of cystic fibrosis in the UK makes this a difficult issue to resolve; however, it is possible as knowledge within the field grows, the induction of labour rates within pregnancies with cystic fibrosis may change.
Vaginal delivery is commonplace for those with cystic fibrosis, often complicated by lung function, diabetes and obstetric intervention (induction of labour). From clinical experience, intrapartum care is provided by midwives in a high-risk obstetric unit, under the supervision of the obstetric team. Childbirth has been described as fear-inducing for women with cystic fibrosis (Kazmerski et al, 2016). Fear in childbirth has not been shown to impact birth outcomes (Heimstad et al, 2006; O'Connell et al, 2019); however, fear is associated with quality of birth experience (Hildingsson et al, 2018).
Hildingsson et al (2018) found caseload midwifery to be beneficial and preferable for women experiencing fear surrounding birth. In their study, caseload midwifery was offered as an intervention, and the 10 participants reported their fear of birth was reduced or alleviated, and they had a good birth experience. Sandall et al (2016) conducted a large Cochrane review of 15 trials and 17 674 women, and found case-loading midwifery was recognised as best practice, beneficial in improving maternal and neonatal safety, reduced medical intervention, and improved client satisfaction. However, this review only included women considered ‘low-risk’ or those who were ‘high-risk’ but not currently experiencing problems.
Women with cystic fibrosis have been demonstrated to be capable of vaginal birth; however, they frequently undergo obstetric intervention (Burden et al, 2012) and little research has investigated their quality of birth experiences. Sandall et al (2016) demonstrated that for the low-risk population, caseload midwifery is best practice, noticeably improving experience and health outcomes. Investigation must follow investment in caseload midwifery for women with cystic fibrosis, to assess experience and health outcomes. Audit will inform whether increasing the breadth of midwifery services is compensated for in the long-term, by a reduction in incidence of medical intervention, perinatal mental health issues and associated costs.
Conclusions
In summary, cystic fibrosis is a life-limiting disorder affecting multiple organ systems. A significant increase in the life expectancy of those with cystic fibrosis over recent years has led to pregnancy becoming commonplace in this population. Pregnancy has a variable impact on the physical health of those with cystic fibrosis and specialist multidisiplinary team care is required.
The psychosocial impact of cystic fibrosis is extensive and mental health plays a crucial role in quality of life. Adherence to treatment is notoriously poor but improved when patients feel at the centre of their care. Pregnancy, childbirth and the transition to parenthood pose their own unique challenges, and women with cystic fibrosis express a desire to experience normalcy and excitement surrounding their pregnancy. Caseload midwifery may support mental health, treatment adherence and emotional support for women with cystic fibrosis.
Vaginal birth is a viable option for most women with cystic fibrosis, although women express fear surrounding birth. Medical intervention is readily performed, with induction of labour taking place in 71% of pregnancies. Caseload midwifery may reduce fear and improve birth experience for those with cystic fibrosis, and advocate for promotion of physiological birth where appropriate.
Modern midwives need to exist in both the medical and social model of care, advocating for optimisation of physiological processes of childbirth and holistic care. The author recommends that small team, caseload midwifery, in line with the ‘Better Births’ report, be offered to women with cystic fibrosis, in addition to multidisciplinary team care. Midwifery contacts should be protected and available regardless of the frequency of obstetric contacts.
The discussion surrounding caseload midwifery in cystic fibrosis may be extended to all women with very complex pregnancies, for whom midwifery contact is often limited. Arguably, all women are entitled to holistic care, support for emotional wellbeing and a sense of normalcy. It should be investigated whether investment in caseload midwifery, in addition to obstetric care, for all women regardless of risk status may improve short-term and long-term outcomes for families.