Maternal and fetal morbidity in pregnancies of Norwegian and Swedish women with cystic fibrosis

Abstract

Women with cystic fibrosis with mild to moderate disease may safely go through pregnancy. In those with severe disease, preterm delivery is common. A team approach is necessary to provide the best medical care for pregnant women with cystic fibrosis.

Aggressive prenatal care results in successful fetal outcomes in CF women

“…However, the pulmonary function of our patients (median FEV 1 of 56%) was lower than in prior obstetric case series (61 -76% predicted) [6,8,10 -12]. Unlike our case series, in these prior studies only a small minority of patients had severe pulmonary impairment (defined as less than 50% predicted normal) [6,8,10,11,16]. Thus, with the increased survival of CF patients, and improved obstetric care, CF women with severe disease are choosing to reproduce.…”

“…Almost half of our patients were in nutritional failure at the time of conception and thus are representative of the U.S. CF population. The median weight gain of the patients in our series (10.4 kg) was as high as any prior CF population series (lowest in France, 5.7 kg and equally high in Norway and Sweden, 10.4 kg) [6,8,10,11]. With aggressive oral supplementation and with TPN in a third of our patients, all delivered fetuses of appropriate weight for gestational age.…”

“…The lung function and BMI of CF women who chose to have obstetric care at the UWMC were the same as women not choosing to have their obstetric care at the referral center and thus are a representative CF population of the Pacific Northwest. Although there have been obstetric CF case series from France, Britain, Norway and Sweden, Israel and Canada, there are no detailed case series from the United States [8][9][10][11][12][13]. Moreover, these prior obstetric series date back as far as 1963 when both obstetric and CF care were markedly different from current clinical practice.…”

“…Three/six patients had weight loss of 0.9 kg in 17 weeks, 1.4 kg over 30 weeks and 1.8 over 19 weeks. TPN was commenced at gestational week 17, 25, 27, 27, 29 and 30 (mean 26 T 5, range of 17 -30 weeks) and TPN was delivered to the patients for 5,7,8,9,11 and 16 weeks (mean 10 T 4, range 5-16 weeks). The daily calories delivered were 1.2 to 1.3 the basal energy expenditure.…”

Update on cystic fibrosis epidemiology

“…Four recent cohort studies have clarified the frequency and outcomes of pregnancy in patients with CF [28][29][30][31]. These studies represent data from four countries (United States, France, Norway, and Sweden).…”