Pregnancy in females with cystic fibrosis and chronic Burkholderia cepacia complex or non-tuberculous mycobacteria infection: A single centre case series.

Abstract

Widespread availability of cystic fibrosis transmembrane conductance regulator modulator therapy (CFTRm) has resulted in improved health and quality of life for many people with cystic fibrosis (CF) and a subsequent rise in pregnancies among females with CF (FwCF). An increasing number of pregnancies are now occurring in women who are chronically infected with clinically significant respiratory pathogens, in particular the Burkholderia cepacia complex (BCC) and non-tuberculous mycobacteria (NTM), including Mycobacterium abscessus complex (MABC). These pathogens are associated with accelerated pulmonary decline, multi-drug antimicrobial resistance patterns and complex, prolonged treatment regimens making them important considerations in pregnancy management. Historically, chronic BCC infection has been linked to poorer obstetric and perinatal outcomes in FwCF, although much of the available evidence predates the CFTRm era and may not reflect the current clinical landscape. Similarly, while NTM infection, most significantly MABC, remains a major clinical challenge and contraindication for lung transplantation in people with cystic fibrosis (PwCF), there is a paucity of published data describing pregnancy outcomes in FwCF living with chronic NTM infection. As CFTRm has shifted disease trajectories and expanded reproductive possibilities, updated evidence describing risks and outcomes is urgently needed. This case series addresses this gap by examining CF-specific, obstetric, and neonatal outcomes in ten pregnancies: four in FwCF chronically infected with BCC and six with NTM infection. All pregnancies ended in live births: nine infants born at term, and one at 32+5 weeks from spontaneous premature labour, requiring the only admission to neonatal intensive care. CFTRm were used in nine pregnancies, and admission to hospital was required in two pregnancies for infective exacerbations of CF. There were no maternal deaths and no maternal intensive care admissions. These findings contribute essential new insights to guide counselling, risk assessment, and multidisciplinary management for pregnant FwCF with chronic infection by these high-risk pathogens.