CF PROSPER: Cystic Fibrosis Pregnancy Related Outcome data to Support PERsonal choices

End of project summary

Main messages 

Work package 1:  

• Pregnancy rates were over three times lower in women with Cystic Fibrosis (CF) in the UK than the general population with about 70% resulting in a live birth between 2003 - 2017.  
   
• Pregnancy rates were highest for women aged 25 - 29 and 30 - 34 years for both women with CF and the general population and lowest for those aged 15 - 19 and 40 - 44 years.  
   
• Live births mirrored pregnancy rates with a 3.5-fold difference in the live-birth rate (17.4 versus 61.4 per 1000 woman-years). 
   
• The availability of ivacaftor for 6.2% of women with CF of childbearing age increased the pregnancy rate for those eligible. Extrapolating this result to the much larger adult CF population now eligible for modulator therapy (90%), we can expect improved health outcomes and survival in CF and an increase in pregnant women with CF in Obstetric departments.  
   
• On comparison of women with CF who were pregnant and those who were not in both the UK and in the US between 2003-2017, pregnant women were more likely to be diagnosed with CF later, have improved nutritional status (BMI), higher lung function (%FEV1), less likely to have Pseudomonas aureginosa infection, using hypertonic saline, not have CF-related diabetes and be pancreatic sufficient. 
   
• Pregnancy increased the rate of annual lung function decline by 12% for women with CF in the USA with a slightly higher rate of 19% for women with CF in the UK. 
   
• It is not clear if modulator therapy will change this as the majority of the cohort from both countries would not have benefitted from modulator therapy.  
   
• It is important that obstetricians are aware of the current and expected future trends of pregnancy and CF to help women with CF, their partners and clinical teams in the decision process on whether to start a family. It is important adult CF centres are aware of the impact of pregnancy on lung function when sharing information with women with CF who may be considering having families of their own.  

Work package 2:  

• Women with CF were highly motivated to participate in shared decision-making (SDM) about reproductive health  
   
• Lack of information specific to people with CF about reproductive choices compromised capability to engage in SDM. Women reported a particularly high need for information about how to plan for pregnancy, options for giving birth and for breastfeeding, likely health outcomes for their children (including pregnancy loss and CF status), fertility (impact of CF on fertility, options for fertility treatment, fertility preservation options), and options for having a family other than pregnancy (e.g. surrogacy, fostering, adoption, step-children)   
   
• Women with CF reported that there were insufficient opportunities for discussions with their healthcare teams about their reproductive goals 
   
• Women’s social-support networks were associated with their self-efficacy for engaging in SDM. The quality of relationships with healthcare professionals and the importance of hearing about the experiences of other women with CF in relation to making decisions about reproductive goals and of (not) having children were salient themes.  
   
• The ‘My Voice CF’ decision aid was perceived to be useful and acceptable by healthcare professionals and women but required modification of content to make it suitable for UK use, and needed to be implemented as part of a wider package of decision support that included clinician training and re-design of healthcare systems to provide opportunities for high-quality conversations about reproductive health  
   
• Multi-level interventions are needed to support person-centred care about reproductive goals, informed by high-quality evidence relating to health and wellbeing outcomes for women with CF and their children.