Diet and Exercise in Cystic Fibrosis

Ronald Ross Watson

• Publisher: Academic Press
• Year: 2014
• Print ISBN: 9780128000519
• eBook ISBN: 9780128005880
• Topic: Biological Sciences
• Subtopic: Human Anatomy & Physiology
• Index: Biological Sciences

Diet and Exercise in Cystic Fibrosis, a unique reference edited by distinguished and internationally recognized nutritionist and immunologist Ronald Ross Watson, fills the gap in the current dietary modalities aimed at controlling cystic fibrosis. Using expert evaluation on the latest studies of the role of food and exercise in lifelong management of cystic fibrosis, this valuable resource shows how to maintain intestinal, hepatic, and pulmonary high quality function for improving quality of life for those with cystic fibrosis. A helpful tool for researchers and clinicians alike, this reference helps refine research targets, and provides the beginning of a structured dietary management scheme for those with cystic fibrosis. - Provides a detailed resource that reviews the health problems occurring in Cystic Fibrosis relative to dietary, complementary, and alternative therapies - Contains expert evaluation on the role of foods and exercise for lifelong management of Cystic Fibrosis to maintain intestinal, hepatic, and pulmonary high quality function for improved quality of life - Defines and evaluates various nutritional and dietary approaches to the unique problems of those with Cystic Fibrosis

Section A

Overview of Nutrition and Diets in Cystic Fibrosis

Outline

Chapter 1. Nutrition for Pregnant Women Who Have Cystic Fibrosis

Chapter 2. Disordered Eating and Body Image in Cystic Fibrosis

Chapter 3. Neonatal Screening and Nutrition/Growth in Cystic Fibrosis: A Review

Chapter 4. Cystic Fibrosis Nutrition: Outcomes, Treatment Guidelines, and Risk Classification

Chapter 5. Clinic, Nutrition, and Spirometry in Cystic Fibrosis

Chapter 6. Family Mealtimes and Children with Cystic Fibrosis

Chapter 7. Disturbed Sleep Behaviors and Melatonin in Sleep Dysfunction and Treatment of Cystic Fibrosis

Chapter 8. Age at Diagnosis and Disease Progression of Cystic Fibrosis

Chapter 9. The Effects of Caffeine, Alcohol, and Tobacco in Cystic Fibrosis

Chapter 10. Eating Disorders and Disturbed Eating Attitudes and Behaviors Typical in CF

Chapter 1

Nutrition for Pregnant Women Who Have Cystic Fibrosis

Suzanne H. Michel¹, and Donna H. Mueller^{2 1}Clinical Assistant Professor, Medical University of South Carolina, Charleston, SC, USA ²Nutrition Sciences Department, Drexel University, Philadelphia, PA, USA

Abstract

More women with cystic fibrosis (CF) are reaching reproductive age, becoming pregnant, and delivering babies. Available evidence based on retrospective chart reviews or national registries reveals that pregnancy does not affect survival. Some evidence indicates that women who, before conception, are at goal weight, with good pulmonary function, and do not have diabetes may have better pregnancy outcomes. Optimal nutrition, both before and during pregnancy, is essential for the well-being of the woman and her infant. Vitamin and mineral nutrition management in CF requires assessment and supplementation of the fat-soluble vitamins and minerals as indicated. Women who have CF are at risk for developing diabetes during pregnancy and evaluation is recommended throughout pregnancy. Limited information is available describing nutrition management of the pregnant woman who has CF. This chapter reviews available evidence describing pregnancy in women who have CF and provides information about nutrition management.

Keywords

Blood glucose screening; Cystic fibrosis; Diabetes; Minerals; Nutrition; Nutrition support; Pancreatic enzymes; Pregnancy; Vitamins; Weight

1.1. Introduction

Nutrition before and during pregnancy is fundamental for all women. Determinants of pregnancy outcome for women and their babies include weight and nutrient status before conception and weight gain and overall nutrient stores and intake during gestation [1]. For women who have CF, pregnancy heightens their need for optimal nutrition and is regarded as an essential factor influencing pregnancy and fetal outcome [2].

Median age of survival for all people who have CF is 41.1 years with women having a median age of survival of 38 years [3]. More women with CF are reaching reproductive age, becoming pregnant, and delivering babies. On the basis of the 2012 U.S. Cystic Fibrosis Foundation (CFF) Patient Registry, 249 pregnancies were reported in women who have CF, with 2 live births per 100 women. Of the 249 pregnancies, 145 resulted in live births, 1 was a stillborn event, 23 were spontaneous abortions, and 7 were therapeutic abortions. At the time of data analysis, 71 women had yet to deliver their babies [3]. Although pregnancy increasingly is common among women who have CF, few facts are available about nutrition management. This chapter summarizes evidence related to outcomes for pregnant women who have CF, available nutrition recommendations, and applications by clinicians.

1.2. Historical Perspective

In 1960, at which time the median age of survival for people who had CF was 10 years of age, the first report of pregnancy in a woman who had CF was published. The woman died 6 weeks following delivery [4]. In 1966, Grand et al. evaluated information available for 10 women who became pregnant and concluded that substantive factors to consider during pregnancy should include the following: (1) comprehensive prenatal evaluation and therapy, including cor pulmonale; (2) careful management of electrolytes; (3) optimal nutrition and enzyme therapy; (4) diagnosis of maternal diabetes, proper labor management, and care of the newborn; and (5) assessment of the infant for CF [5]. Cohen et al. described 84 pregnancies. Between conception and 6 months postpartum, there were 10 maternal deaths and significantly increased morbidity, with 60% of the women experiencing pulmonary symptomatology and an increase of 14% for cor pulmonale [6]. Palmer et al. performed a retrospective chart review of eight women who had 11 pregnancies between 1974 and 1981. Fifty-two percent of the women were pancreatic sufficient (PS) and at 95% of ideal body weight. Four had diabetes either before their pregnancy or developed it during pregnancy [7]. Corkey et al. described 11 pregnancies in seven women with 10 live births. Two of the women were pancreatic insufficient (PI) and four of the pregnancies occurred before the women knowing they had CF [8]. In a review of pregnancy occurring between 1963 and 1990, Canny et al. reported outcomes for 25 women with CF who had 38 pregnancies. Two women were diagnosed with CF after their pregnancies. Twelve of the women were PS, one woman developed pancreatitis following delivery, one was using insulin at the time of the pregnancy, and three developed gestational diabetes. Of the 38 pregnancies, 4 were interrupted by abortion, 3 were therapeutic, and 1 was spontaneous. Mean weight gain for 24 of the women for whom the information was available was 10.5 ± 5.7 kg [9].

As more women with CF had babies, reports provided survival data, but few described management techniques, including nutrition management. Case reports and epidemiological studies showed that the long-term outcome of women with CF who become pregnant was not worse than that of CF women who did not become pregnant [2,10–15]. Some data indicated that women with better pulmonary function might do better [12,13,16]. Using CFF Patient Registry data, Fitzsimmons et al. found no differences in CF status up to 18 months following delivery [17]. McMullen et al. found no difference in rate of CF decline in women who had babies versus those who did not. No information was provided regarding fetal outcome [2]. In another review of pregnancy, Gilljam et al. found that women who were PS; did not have Burkholderia cepacia; and, measured by standardized spirometry, had values of forced expiratory volume at one second (FEV1%) greater than 50% demonstrated better outcome than those who did not. Postdelivery pulmonary decline was similar to those women who did not have babies [12]. In a retrospective chart review of pregnancies occurring between 1990 and 2009 in the United Kingdom, Etherington et al. reported the presence of preexisting cystic fibrosis-related diabetes (CFRD), coupled with a prepregnancy FEV1% less than 60% predicted, were associated with significantly worse outcomes [18]. Thorpe-Beeston et al. reviewed 48 pregnancies in 41 women. There were 2 miscarriages, 44 singleton births, and 2 twin births. Forty-six percent of the deliveries were preterm deliveries and all but two were delivered preterm because of medical concern for maternal well-being. Caesarean sections were performed for 48% of the deliveries. The authors noted that women should receive optimal antenatal counseling to ensure that they understand the implications of their disease on overall survival [13]. Using data from the 1994–2005 Epidemiologic Study of CF, Schechter et al. studied long-term physiologic and functional effects of pregnancy. They concluded that although pregnancy and motherhood do not appear to accelerate disease progression, these two situations may lead to more illness-related visits, pulmonary exacerbations, and a decrease in some domains of quality of life [15].

Early reports of pregnancy described poor outcomes for both the women and their babies [4–7]. The majority of women in the studies with good outcome were PS, with optimal weight and pulmonary function before conception [8,9]. On the basis of the evidence available at the time, a major recommendation was that pregnancy be attempted only by women in excellent health. Reviews of more recent population data and clinical data do not indicate a survival difference in CF women between those who became pregnant and had a baby and those who had not become pregnant [15,17]. On an individual basis, however, women with inadequate weight, inadequate pulmonary function tests, or diabetes before pregnancy may have a mo...

Table of contents

• Cover image
• Title page
• Table of Contents
• Copyright
• Dedication
• Preface
• Acknowledgments
• Biography
• List of Contributors
• Section A. Overview of Nutrition and Diets in Cystic Fibrosis
• Section B. Vitamin D Deficiency and Supplementation in Growth and Health in Children with Cystic Fibrosis
• Section C. Vitamin Deficiency, Antioxidants, and Supplementation in Cystic Fibrosis Patients
• Section D. Management of Diabetes and Celiac Disease Associated with Cystic Fibrosis: Role of Nutrition and Food
• Section E. Nutrition and Pulmonary Function in Cystic Fibrosis Patients
• Section F. Exercise and Behavior in Management of Cystic Fibrosis
• Section G. FAT and LIPID Metabolism in Cystic Fibrosis
• Index