Pregnancy and cystic fibrosis: Approach to contemporary management

Abstract

Over the previous 50 years survival of patients with cystic fibrosis has progressively increased. As a result of improvements in health care, increasing numbers of patients with cystic fibrosis are now considering starting families of their own. For the health care professionals who look after these patients, the assessment of the potential risks, and the process of guiding prospective parents through pregnancy and beyond can be both challenging and rewarding. To facilitate appropriate discussions about pregnancy, health care workers must have a detailed understanding of the various important issues that will ultimately need to be considered for any patient with cystic fibrosis considering parenthood. This review will address these issues. In particular, it will outline pregnancy outcomes for mothers with cystic fibrosis, issues that need to be taken into account when planning a pregnancy and the management of pregnancy for mothers with cystic fibrosis or mothers who have undergone organ transplantation as a result of cystic fibrosis.

Cystic Fibrosis-Related Diabetes and Subclinical Hypothyroidism in Pregnancy

“…Pregnancies of women with CF are managed as high risk (premature delivery often occurs in 9% to 35% of pregnancies with CF), especially when they are complicated by diabetes [6]. In these rare cases, the most difficult task for the health care provider is to balance the nutritional requirements of pregnancy in a woman with CFRD, and at the same time to maintain glycemic control [7]. In our case, the gradual switch in basal insulin administration with more units in the morning than in the evening improved the variance of glycemia in the 24-hour period, and resolved the hypoglycemic episodes.…”

“…In order to control the glycemic variance resulting from this effect, it is recommended to hospitalize patients especially when they are on insulin therapy [8,9]. The rate of weight change was also improved with a satisfactory total gain of 7 kg in pregnancy, considering that weight gain is often difficult among pregnant women with CFRD [3,4,7]. The recommendation for CF in pregnancy is to eat a high-fat and energy diet (120%-150% of the recommended energy intake for pregnant women without CF) in order to increase the maximum absorption rate as much as possible, aiming for a weight gain of 11 kg [7].…”

“…CFRD is a co-morbidity factor to the already established pulmonary disease of CF with adverse effects on maternal survival (especially when forced expiratory volume in 1 second [FEV1] is less than 60%), short-term fetal outcomes, related to preterm delivery (10%-25% of cases), and increased rates of low birth weight (<2.5 kg). Pregnancy in women with CFRD is relatively rare, requiring intensive monitoring due to the disease's unique pathophysiologic characteristics [1][2][3][4][5][6][7].…”

The experience of men and women with cystic fibrosis who have become a parent: A qualitative study

“…Their desire for children, however, often outweighs known risks (Ahluwalia et al., ). Best care outcomes are achieved at a tertiary centre with an experienced multidisciplinary team (Deighan, Ash, & McMorrow, ; Geake, Tay, Callaway, & Bell, ), involving the woman in frank discussions and decisions to balance risks of premature birth with impending maternal respiratory failure (Deighan et al., ), and facing unpredictable complications of pregnancy in CF (Cheng et al., ). However, pregnancy care in CF is documented as both rewarding and challenging (Deighan et al., ; Geake et al., ), difficulties being lessened with careful planning (Edenborough et al., ).…”

“…Given the growing trend towards parenthood, it is requisite for CF care teams to be well‐informed regarding new health problems and issues that arise (Geake et al., ; Quon & Aitken, ), informed by current data regarding parenthood outcomes (Boyd et al., ; Hubert et al., ). Ullrich, Bobis, and Bewig () recommend that CF clinics routinely discuss aspirations of becoming a parent, but conclude there are limited data on the experience of parenting, leaving care teams to offer counsel sometimes beyond their familiar territory.…”