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RCOG World Congress 2023
June 12 - 14, 2023 | London, UK
PP.0072
Obstetric and neonatal outcomes in women with CF (wwCF)
Poster Presenter
Part of Topic
Maternal Medicine
Obstetric and neonatal outcomes in women with CF (wwCF)
A Downes1, F Flynn1, R Robinson1, W Banya1, N Singh2, B Belkarty1, A Jones1, NJ Simmonds1, E Ukor1, S Madge1, S Collins1, R Scott2, 3, I Felton1
1. Department of Adult Cystic Fibrosis, Royal Brompton Hospital, part of Guy’s and St Thomas’ NHS Foundation Trust, London, UK. 2. Department of Obstetrics, Chelsea and Westminster Hospital NHS Foundation Trust, London , 3. Department of Diabetes & Endocrinology Chelsea & Westminster Hospital NHS Foundation Trust, London
Background and Aims
•Historically pregnancy in women with cystic fibrosis (wwCF) has been considered high-risk requiring management in specialist centres1, with guidelines stipulating that lung function (based on percentage predicted forced expiratory volume in 1 second, FEV1pp) <50% should be viewed as an absolute contraindication to pregnancy1.
•In August 2020 a new generation of treatment, highly effective CF-genotype specific modulator therapies (specifically elexacaftor-tezacaftor-ivacaftor (ETI), brand name Kaftrio®) were approved and funded for use by NHS England. 90-95% of adults with CF are eligible, and since its availability there has been a significant increase in pregnancy rates with a fourfold rise in this large adult CF centre, a trend also reflected in UK CF patient registry reports2.
•ETI confers both direct (better general health/nutrition, decreased cervical mucus viscosity & increase in pH) and indirect (increased quality of life, expectation of increased life expectancy) pro-fertility effects.
•Increasing pregnancy rates and increased stability since the introduction of ETI warrants review of these outcomes according to pre-conception lung function.
Method and Design
•Single-centre patient records review of all wwCF who completed pregnancies from Sept 2020-Nov 2022.
•All completed pregnancies were categorised into three groups according to maternal FEV1pp at conception:
•Mild/no impairment (>80% FEV1pp) n= 13 (median 93% (89, 99))
•Moderate impairment (50-80% FEV1pp) n= 16 (median 65.5% (59, 71.5))
•Severe impairment (<50% FEV1pp) n = 5 (median 42% (38, 43))
Results
•51 pregnancies were identified.
•Eight first trimester miscarriages(no later miscarriages) and nine terminations for social indication were excluded – live birth rate: 83.3%.
•32 women completed 34 pregnancies over the study period (35 live births – one twin pregnancy).
•Pre-existing CF-Diabetes was present in seven wwCF prior to pregnancy, of the 27 remaining 13 (48%) went on to develop gestational diabetes.
•Overall there was no maternal or infant mortalities, and no maternal peripartum respiratory support was required.
•5 (16%) infants required brief neonatal ICU support for: prematurity (n=1), hypoglycaemia (n=1), transient tachypnoea of the newborn (n=2) and circulatory shock post-delivery (n=1).
•One infant was found to have simple dextrocardia, there were no other abnormalities regardless of ETI use, including no cases of cataracts on routine neonatal screen.
•Rates of pre-term delivery were higher with lower lung function but did not reach statistical significance (p=0.51)
•Mild: n=2/13 (15.4%)
•Moderate: n=5/16 (31.25%)
•Severe: n= 2/5 (40%)
•Median gestational age (weeks+days) was similar between all groups (p=0.27)
•Mild: 38 (37+1, 38+1)
•Moderate: 37 (36+6, 37+6)
•Severe: 37+3 (35+1, 37+4)
•Mean birth weight (kg) proportionately decreased with lung function but birth centiles were similar, and neither was statistically significant (p=0.33 and p=0.98 respectively)
•Mild: 3.11 ± 0.44kg; 50th (25, 75)
•Moderate: 2.89 ± 0.56kg; 50th (31, 75)
•Severe: 2.70 ± 0.52kg; 50th (25, 83)
•Caesarean section delivery rate was lowest in those with most severe lung function impairment (p=0.19)
•Mild: 8/13 (61%)
•Moderate 10/16 (62.5%)
•Severe: 1/5 (20%)
Conclusions
•Since the introduction of ETI the majority of pregnancies in this large adult CF centre are among wwCF with moderate-severe pre-conception lung function impairment.
•Overall, obstetric and neonatal outcomes are good with low incidence of complications.
•Women with low lung function can go on to have successful pregnancies without complications.
•There is a high rate of diabetes (CF-Diabetes and Gestational diabetes) in wwCF who become pregnant, with a higher rate of gestational diabetes than the general UK population (48% vs 5%)3.
•However, there does appear to be a trend towards lower birth weight with more severe lung function impairment, and larger studies with greater numbers of pregnancies in women with pre-conception FEV1pp <50% would be helpful to further explore this.