�-Adrenergic bronchodilators help control airway constriction. Inhaled recombinant human deoxyribonuclease I improves lung function by reducing sputum viscosity (Sorscher, 2015). Inhaled 7 -percent saline produces short-and long-term benefits (Elkins, 2006). Nutritional status is assessed and appropriate dietary counseling given. Pancreatic insuiciency requires replacement of oral pancreatic enzymes. Promising new therapy to correct CFTR protein dysfunction was recently described by Wainwright and colleagues (2015). Using a combination of lumacaftor and ivacaftor, these investigators showed that patients homozygous for the Phe508del mutation were significantly benefitted. No reports of either drug are available regarding pregnant women.
Infection is heralded by increasing cough and mucus production. Oral semisynthetic penicillins or cephalosporins usually suice to treat staphylococcal infections. Pseudomonas infection is problematic, and inhaled tobramycin and colistin have been used successfully to control this organism. Immediate hospitalization and aggressive therapy are warranted for serious pulmonary infections. he threshold for hospitalization with other complications is low. For labor and delivery, epidural analgesia is recommended (Deighan, 2014).
Earlier reports chronicled the poor maternal and perinatal outcomes of women with cystic ibrosis (Cohen, 1980; Kent, 1993). vlore recent reports describe better outcomes, but there still are serious complications. Disease severity is now quantified by pulmonary function studies, which are the best predictor of pregnancy and long-term maternal outcome. Edenborough and colleagues (2000) reported 69 pregnancies and found that if prep regnancy FEV! was <60 percent of predicted, the risk for preterm delivery, respiratory complications, and death of the mother within a few years of childbirth was substantive. Thorpe-Beeston (2013) and Fitzsimmons (1996) and their associates reported similar indings. Gillet and colleagues (2002) reported 75 pregnancies from the French Cystic Fibrosis Registry. Almost 20 percent of newborns were delivered preterm, and 30 percent had growth restriction. he one maternal death was due to Pseudomonas sepsis in a woman whose prepregnancy FEV! was 60 percent. Long-term, however, 17 percent of women died, and four infants had conirmed cystic fibrosis.
Maternal complications are daunting. Patel and coworkers (2015) recently queried the National Inpatient Sample database and reported that the prevalence of cystic ibrosis in pregnancy had a signiicant linear increase from 2000 to 2010. They analyzed 1119 afected women in more than 12 million births and reported a litany of risks (Table 51-5). In contrast, perinatal outcomes were surprisingly good.
Cystic fibrosis is a common antecedent disease leading to lung transplantation. Gyi and coworkers (2006) reviewed 10 pregnancies in such women and reported nine liveborn neonates. Maternal outcomes were less favorable. Of the three gravidas who developed rejection during pregnancy, all had progressively declining pulmonary function and died of chronic rejection by 38 months after delivery.
TABLE 51-5. Odds Ratios for Maternal Complications in 11e19 Pregnant Women with Cystic Fibrosis Compared with Controls