Temporal trends of cystic fibrosis in pregnancy: 2006 to 2017


      Treatment of cystic fibrosis (CF) has changed dramatically in the past decade with the approval of CF transmembrane conductance regulator (CFTR) modulators. Using a large, nationally representative database, we aimed to estimate prevalence and annual trends of pregnancies affected by CF during a 12-year period in the United States.

      Study Design

      We conducted a serial cross-sectional analysis of pregnancy-related hospitalizations from the 2006 to 2017 National Inpatient Sample. Diagnosis of CF in both delivery and non-delivery pregnancy-related hospitalizations were captured using ICD-9 and ICD-10 codes. Sociodemographic data and delivery rates were compared to unaffected pregnancies. Joinpoint regression was used to estimate trends in CF over the study period.


      Of nearly 47 million delivery hospitalizations, 4,020 occurred in patients with CF, corresponding to 9 per 100,000 deliveries. The rate of CF increased from 5.3 to 12.3 per 100,000 deliveries during the study period. Cases of CF at delivery increased annually by 5.8% (95% confidence interval [CI]: 3.5, 8.1%, p < 0.01) from 2006 to 2017. Non-delivery hospitalizations for CF did not increase significantly (3.2%, 95% CI: -1.1,7.7%, p=0.13). Moreover, during the study period, the age distribution of CF deliveries shifted toward older maternal ages. Of all deliveries with CF, the percentage of births to women 25 or older increased from from 58.1% in 2006 to 75.8% in 2017. This was similar to all deliveries, where the percentage of births to women 25 or older increased from 64.5% to 75.2%.


      Prevalence of CF in pregnancy has increased in the era of CFTR modulator therapy. The distribution of delivery rates in CF have shifted toward older age groups, which more closely approximates that of the general population. These trends have not resulted in an increase in antepartum hospitalization. These findings may be indicative of increased fertility and improved quality of life conducive to family building.
      Figure thumbnail fx1
      Figure thumbnail fx2