Sex and Relationships Education for Individuals with Cystic Fibrosis: A Service-Based Approach

Emily Norris,Catherine Butler,Kirsty James,Samantha Phillips

doi:10.1007/s11195-018-9535-y

Abstract

Increasing life expectancy within cystic fibrosis (CF) raises challenges around previously neglected topics such as sexual and reproductive health (SRH). The study aimed to gather retrospective experiences of service provision around SRH to consider the role of the CF service, age of information provision and unmet needs highlighting possible improvements to provision. A mixed-methods retrospective survey-based design was employed. An Adult CF team participated in a consultation session generating survey questions around SRH. A 20-item online survey was constructed and disseminated to adult CF patients. Unmet needs were found in SRH provision in pediatric and adult CF services, with further information required by patients on topics including parenthood and fertility. Results support previous research findings highlighting the need for standardized provision around SRH. Age of SRH provision suggested individual differences in need within the pediatric service. Further research could explore format and specific age of SRH information provision.

Highlights

Cystic fibrosis is a recessive genetic disorder caused by an abnormality of the CFTR gene, resulting in production of thick mucus in the lungs and pancreas and leading to recurrent infections, breathing difficulties and digestive problems [12]
Patients reported a lack of information provision in pediatric and adult services, which in some cases had resulted in negative effects such as spending “too long thinking the worst” about being unable to have children due to infertility
Barriers to information seeking such as embarrassment and a desire for the clinicians working within the cystic fibrosis (CF) service to initiate conversations highlight the role of the CF service in meeting patient need in this area

Summary

Introduction

Cystic fibrosis is a recessive genetic disorder caused by an abnormality of the CFTR gene, resulting in production of thick mucus in the lungs and pancreas and leading to recurrent infections, breathing difficulties and digestive problems [12]. CF is life-limiting, with median predicted survival in the UK estimated at 45 years (Cystic Fibrosis Trust [1]), this is increasing yearly, with a median predicted survival of more than 50 years for children born in 2000 [4, 19]). Increased life expectancy raises issues such as pregnancy and parenthood, which were not previously considered. As CF is a recessive disorder, both parents must carry the CFTR gene for a child to inherit the disorder, highlighting issues around reproductive decision-making.

Methods

Discussion

Conclusion