Cystic Fibrosis PRegnancy-Related Outcome data to Support PERsonal choices
About the CF-PROSPER project
Aim(s) of the research
This project aims to empower women with Cystic Fibrosis of reproductive age to negotiate the complex choices they and their families face when considering starting a family. We aim to:
- Find out more about pregnancy in women with Cystic Fibrosis using data from registries in the United Kingdom and in the United States of America, including how many women get pregnant and how pregnancy affects women’s health
- Develop a ‘decision aid’ to help health professionals, women and their families have informed discussions about their choices when they are considering starting a family
Background to the research
- Cystic Fibrosis is the most common inherited disease and affects around 10,400 people in the UK today. People with cystic fibrosis experience a build-up of thick sticky mucus in the lungs, digestive system and other organs, causing a wide range of challenging symptoms affecting the entire body. Due to advances in the treatment for Cystic Fibrosis over recent years, people with this disease are living longer healthier lives than in previous decades and average life expectancy is 47 years.
- More women in the UK who have Cystic Fibrosis are now considering having families of their own. Health professionals, women and families living with Cystic Fibrosis need high quality evidence, presented in a user-friendly manner to help them discuss these issues and make decisions about starting a family that take into account women’s individual needs, values, and preferences, through a shared decision-making approach.
What we hope to discover
- We will use large national databases of Cystic Fibrosis patients in the UK and USA to find out more about pregnancy and Cystic Fibrosis. We will look at how many women with Cystic Fibrosis become pregnant, whether there are differences in between those that do and do not get pregnant in terms of their disease severity, and what happens to women’s health (including lung function and survival) during pregnancy and following child birth.
- We will work with a Stakeholder group that includes women with Cystic Fibrosis, their partners, and clinical care teams to create a ‘decision aid’ for women with CF who are considering starting a family. Decision aids provide information and tools to help people weigh up their options, think about values and preferences, and develop confidence to discuss their choices. We will carry out an online survey and interviews to find out what information women with Cystic Fibrosis need about starting a family. We will gather feedback on the decision aid from 10 women with Cystic Fibrosis and 10 health professionals.
Public involvement
- Our patient partner is a co-applicant on this bid, has reviewed the project plans outlined here, and will join our Study Management Group. At least three patient and public involvement partners will join our Stakeholder Group. Our patient partners will contribute to guiding the project, co-designing the decision aid, and dissemination activities.
Dissemination
- We will make the decision aid universally accessible free of charge via the Cystic Fibrosis Trust for use by women with CF and their clinical teams. We will publish at least two papers in scientific journals to report our study findings, and will present our work at the European Cystic Fibrosis Society and North American Cystic Fibrosis conferences. We will work with our patient co-applicant and stakeholders to produce public facing summaries of our findings, which will be distributed via social media and the Cystic Fibrosis Trust.
Funding
This project is funded by a Health and Care Research Wales Research for Public and Patient Benefit Grant (RfPPB-18-1497 T).