(89) A Novel CF-Pregnancy Service: Two year review following introduction of elexacaftor/tezacaftor/ivacaftor (Kaftrio)

Background: As a quality-service improvement response following availability of elexacaftor/tezacaftor/ivacaftor (Kaftrio) and the significant increase in yearly average CF-pregnancies (n=7 pre-2020, to n=33 in 2021) at this adult CF centre (~600 pwCF), a monthly multidisciplinary CF-Maternal Health Virtual Clinic was established with antenatal virtual CF-exercise classes dedicated to provide adaptive, specialist support to this cohort as well as outreach guidance and education to local obstetric teams.

Methods: Single centre retrospective review of Royal Brompton Hospital CF-Maternal Health MDT clinic records and patient survey from March 2020-22.

Results: A total of 41 females (median age=30yrs), involving 47 pregnancies, were eligible for elexacaftor/tezacaftor/ivacaftor at start of pregnancy, of which 40% (n=19/47) were unplanned and 19% (n=9/47) via assisted conception. Three females with a history of infertility conceived naturally having required assisted conception for previous pregnancies and n=5 females had multiple pregnancies during study period.
Elexacaftor/tezacaftor/ivacaftor was continued in 60%(n=28), delayed in 28%(n=13) and stopped in 13%(n=6) pregnancies through maternal choice and careful clinical counselling. Pre-pregnancy pulmonary status was poorer in women who ‘continued’ versus ‘delayed/stopped’ (Table 1). Of those who stopped, 85% (n=5/6) restarted due to pulmonary deterioration by third trimester.
Antenatal CF-complications included at least one episode of minor haemoptysis in 21% (n=9/41), at least one infective exacerbation in 55% pregnancies (n=26/47), and non-invasive ventilation in one case. Other pregnancy-associated complications included one case of ovarian hyperstimulation syndrome, one sub-segmental pulmonary embolism, and pregnancy induced hypertension in 4% (n=2/47).
Excluding 10 first trimester terminations, 10 current pregnancies and one patient relocation, obstetric outcomes available for 26 pregnancies confirmed a live birth rate of 85% (n=22/26) and 15% first trimester miscarriage rate (n=4/26). Obstetric complications included preterm delivery rate of 23% (n=6/26) including two cases COVID-infection resulting in two neonatal ICU admissions, one case of endometritis post-C-section, and a fourth-degree perineal tear. There were no ectopic pregnancies, maternal or neonatal deaths, nor reports of infant cataracts or congenital malformations. Median gestational age was 37/40 weeks (range: 29-40). Mode of delivery was via caesarean section in 45% (n=10/22, of which two were emergency) and vaginal in 55% (n=12/22) of which 83% (n=10/12) were via induction of labour for diabetes (CF or gestational) indication. Deliveries were supported and occurred equally at local obstetric units and in tertiary-care obstetric hospital settings (50%, n=11/22).
Patient-experience survey responses cited high levels of confidence in health optimisation and prioritisation during pregnancy and praised excellent inter-healthcare provider communication and peer-to-peer emotional support provided among expectant mothers in the virtual antenatal-exercise groups.

Conclusions: In the absence of clinical trial safety data, the novel approach of a dedicated CF-Maternal Health MDT clinic with local obstetric outreach support has ensured regular, specialist clinical and emotional peer-to-peer support for this cohort of females eligible for elexacaftor/tezacaftor/ivacaftor to ensure optimal outcomes and experiences of their pregnancies, and where appropriate close to home.

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