Diet and Exercise in Cystic Fibrosis

By Ronald Ross Watson

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

• Language: English
• Publisher: Academic Press
• Release date: Jul 29, 2014
• ISBN: 9780128005880

Book preview

Diet and Exercise in Cystic Fibrosis

Editor

Ronald Ross Watson

University of Arizona, Mel and Enid Zuckerman College of Public Health, Sarver Heart Center in the School of Medicine, Tucson, AZ

Table of Contents

Cover image

Title page

Copyright

Dedication

Preface

Acknowledgments

Biography

List of Contributors

Section A. Overview of Nutrition and Diets in Cystic Fibrosis

Chapter 1. Nutrition for Pregnant Women Who Have Cystic Fibrosis

1.1. Introduction

1.2. Historical Perspective

1.3. Nutrition: Review of the Literature

1.4. Clinical Guidance

1.5. Conclusion

Chapter 2. Disordered Eating and Body Image in Cystic Fibrosis

2.1. Introduction

2.2. Quality of Life

2.3. Body Image

2.4. Eating Behaviors

2.5. Body Dissatisfaction and Disordered Eating: Identification, Treatment, and Intervention Strategies

2.6. Conclusion

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

3.1. Introduction

3.2. Review of the Literature

3.3. RCTs Studies

3.4. Studies Using CF Registry Data

3.5. Cohort Observational Studies

3.6. Discussion

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

4.1. Nutrition and Outcomes

4.2. Assessing Weight Change and Growth in CF

4.3. Energy Guidelines

4.4. Vitamins and Minerals

4.5. Malabsorption

4.6. Increasing Intake

4.7. Comorbid Diseases/Complications That Affect Nutrition

4.8. Nutrition Screening and Risk Classification in a Clinical Setting

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

5.1. Introduction

5.2. Clinical Characteristics

5.3. Methods of Assessing Growth and Pulmonary Function

5.4. Factors that Interfere with the Growth of CF Patients

5.5. Metabolic Process in Cystic Fibrosis: High Calorie Consumption

5.6. Metabolic and Nutritional Secondary Processes

5.7. Relationship between Endocrine-Metabolic Diseases and Lung Development

5.8. Nutrition and Lung Function

5.9. Conclusion

Chapter 6. Family Mealtimes and Children with Cystic Fibrosis

Conclusion and Future Directions

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

7.1. Introduction

7.2. Sleep Dysfunction in Cystic Fibrosis

7.3. Melatonin and CF

7.4. Conclusion

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

8.1. Age at Diagnosis of CF

8.2. Morbidity at Diagnosis and Introduction to Morbidity

8.3. Nutritional Morbidity

8.4. Lung Morbidity

8.5. Liver Morbidity

8.6. Overall Morbidity

8.7. Discussion

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

Key Points

9.1. Introduction

9.2. Cystic Fibrosis Overview

9.3. Caffeine and Patients with CF

9.4. Alcohol and Patients with CF

9.5. Tobacco and Patients with CF

9.6. Summary

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

10.1. Poor Nutritional Status in CF

10.2. Evidence of EDs in CF

10.3. Disturbed Eating Attitudes and Behaviors in CF

10.4. Measures of DEABs for the CF Population

10.5. Conclusion

Section B. Vitamin D Deficiency and Supplementation in Growth and Health in Children with Cystic Fibrosis

Chapter 11. Vitamin D Bioavailability in Cystic Fibrosis

11.1. Introduction

11.2. Vitamin D Status in Cystic Fibrosis

11.3. Photosynthesis of Vitamin D3

11.4. Vitamin D Digestion and Absorption

11.5. Vitamin D Storage

11.6. Vitamin D Transport

11.7. VITAMIN D Metabolism

11.8. Vitamin D Excretion

11.9. Vitamin D Action

11.10. Importance of Vitamin D for Cystic Fibrosis Patients

11.11. Conclusion and Future Directions

Chapter 12. Vitamin D in Cystic Fibrosis

12.1. Introduction

12.2. Prevalence of Deficiency in CF

12.3. Impact of Vitamin D Deficiency in CF

12.4. Vitamin D Treatments in CF

12.5. Conclusion

Chapter 13. Specialty Foods for Children with Cystic Fibrosis

13.1. Cystic Fibrosis

13.2. Nutritional and Growth Problems Associated with Cystic Fibrosis

13.3. Cystic Fibrosis-Related Diabetes Mellitus

13.4. Specialty Foods and Diets

13.5. Conclusion

Chapter 14. Fat-Soluble Vitamin Deficiency in Cystic Fibrosis

14.1. Introduction

14.2. CF Genetics

14.3. Signs and Symptoms

14.4. Nutrition Overview for CF

14.5. Fat-Soluble Vitamins

14.6. Vitamin A Deficiency

14.7. Vitamin E Deficiency

14.8. Vitamin K Deficiency

14.9. Vitamin D Deficiency

14.10. Treatment and Therapy

14.11. CF and Vitamin Supplementation

Chapter 15. Can Light Provide a Vitamin D Supplement in Cystic Fibrosis?

15.1. Introduction

15.2. Vitamin D Deficiency in CF

15.3. Prevention and Treatment of Vitamin D Deficiency in CF

15.4. Ultraviolet B Radiation in the Management of Vitamin D Deficiency in CF

15.5. Conclusion

Chapter 16. Pediatric Cystic Fibrosis and Fat-Soluble Vitamins

16.1. Introduction

16.2. Vitamin A

16.3. Vitamin D

16.4. Vitamin E

16.5. Vitamin K

16.6. Conclusion

Section C. Vitamin Deficiency, Antioxidants, and Supplementation in Cystic Fibrosis Patients

Chapter 17. Vitamin Supplements: A Role in Cystic Fibrosis Patients?

17.1. Introduction

17.2. Vitamin A

17.3. Vitamin D

17.4. Vitamin E

17.5. Vitamin K

17.6. Water-Soluble Vitamins

Chapter 18. Nutritional Strategies to Modulate Inflammation and Oxidative Stress in Patients with Cystic Fibrosis

18.1. Oxidative Stress and Inflammation in Cystic Fibrosis

18.2. Antioxidant Deficiencies in CF

18.3. Dietary Interventions in CF

Chapter 19. Vitamin A Supplementation Therapy for Patients with Cystic Fibrosis

19.1. Overview of Vitamin A

19.2. Biological Profile of Vitamin A in Cystic Fibrosis

19.3. Effectiveness of Vitamin A Treatments in Preventing Negative Symptoms

Chapter 20. The Emergence of Polyphenols in the Potentiation of Treatment Modality in Cystic Fibrosis

20.1. Introduction

20.2. Role of polyphenols in Other Diseases and Possibly CF

20.3. Therapeutic Capacity of polyphenols to CFTR Function

20.4. Antimicrobial Capacity of polyphenols

20.5. Anti-inflammatory Properties of polyphenols

20.6. Role of polyphenols in CFTR-related Comorbidities

20.7. CF-Related mental Impairments

20.8. Conclusion

Chapter 21. Chronic Infection with Pseudomonas aeruginosa in an Animal Model of Oxidative Stress: Lessons for Patients with Cystic Fibrosis

21.1. Oxidative Stress in CF

21.2. Animal Models of Chronic Lung Infection

21.3. Chronic Lung Infection in Animal Model of Oxidative Stress

21.4. Lessons for CF Patients

Chapter 22. Vitamin K in Cystic Fibrosis

22.1. Vitamin K Supplementation for Cystic Fibrosis

Chapter 23. Vitamin K and Cystic Fibrosis

23.1. Introduction

23.2. Dietary Sources

23.3. Vitamin K Digestion

23.4. Biochemistry

23.5. Vitamin K and CF

23.6. Diagnosis of Vitamin K Deficiency

23.7. Vitamin K Preparations

23.8. Vitamin K Supplementation in CF

23.9. Treatment of Vitamin K Deficiency

23.10. Summary

Section D. Management of Diabetes and Celiac Disease Associated with Cystic Fibrosis: Role of Nutrition and Food

Chapter 24. Insulin, Body Mass, and Growth in Young Cystic Fibrosis Patients

24.1. Insulin Secretion—Normal Physiology

24.2. Progressive Insulin Deficiency Is a Feature of CF

24.3. Normal Physiology of Insulin-Mediated Glucose Uptake

24.4. Implications of Insulin Deficiency on Airway Glucose and Bacterial Colonization

24.5. Normal Physiology of Insulin Action on Growth and Anabolism

24.6. Implications of Insulin Deficiency on Growth in CF

24.7. Summary of Importance of Adequate Insulin Secretion in Cystic Fibrosis

24.8. Current Management Principles of Insulin Therapy in Cystic Fibrosis

24.9. Future Directions of Insulin Therapies

Chapter 25. Low Glycemic Index Dietary Interventions in Cystic Fibrosis

25.1. Introduction

25.2. Evidence for Effect in Non-CF Populations with Diabetes

25.3. Evidence for the Use of a Low GI Diet in CF

25.4. Practical Considerations of a Low GI Dietary Intervention in CF

25.5. Theoretical Applications of a Low GI Dietary Intervention in CF

25.6. Conclusions

Chapter 26. Insulin Resistance in Cystic Fibrosis: Management

26.1. Insulin Resistance in Cystic Fibrosis: An Overview

26.2. Pathophysiology of Insulin Resistance

26.3. Management of Insulin Resistance

Chapter 27. Cystic Fibrosis and Celiac Disease

27.1. Introduction

27.2. Clinical Features

27.3. Testing

27.4. Treatment

27.5. Relation to Cystic Fibrosis

27.6. Conclusion

Section E. Nutrition and Pulmonary Function in Cystic Fibrosis Patients

Chapter 28. Probiotic Supplementation and Pulmonary Exacerbations in Patients with Cystic Fibrosis

28.1. Intestinal Inflammation

28.2. Probiotics

28.3. Summary

Chapter 29. Buteyko: Better Breathing = Better Health

29.1. Introduction

29.2. Breathing

29.3. The Buteyko Method

29.4. Conclusion

Chapter 30. Lactoferrin and Cystic Fibrosis Airway Infection

30.1. Introduction

30.2. Iron Homeostasis in Healthy and CF Airways

30.3. CF Airway Infections

30.4. Inflammation in CF Airways

30.5. Antimicrobials of the Human Airway: Lactoferrin, a Component of Innate Immunity

30.6. Lactoferrin and CF Airways

30.7. Future Approaches in the Therapy of CF Airway Infections

30.8. Concluding Remarks

Chapter 31. Cystic Fibrosis–Related Diabetes: Lung Function and Nutritional Status

31.1. Introduction

31.2. Cystic Fibrosis–Related Diabetes: Physiopathology

31.3. Cystic Fibrosis–Related Diabetes: Screening and Diagnosis

31.4. Cystic Fibrosis–Related Diabetes: Treatment

31.5. Cystic Fibrosis–Related Diabetes: Comorbidity and Complications

31.6. Perspectives and Conclusions

Section F. Exercise and Behavior in Management of Cystic Fibrosis

Chapter 32. Exercise Testing in CF, the What and How

32.1. Utility of Exercise Testing

32.2. Exercise Tests

32.3. The Godfrey Protocol

32.4. The Bruce Protocol

32.5. Field Tests

32.6. Conducting an Exercise Test

32.7. Interpreting an Exercise Test

Chapter 33. Mechanisms of Exercise Limitation in Cystic Fibrosis: A Literature Update of Involved Mechanisms

33.1. Introduction

33.2. Methods

33.3. Results

33.4. Ventilatory Parameters

33.5. Muscle Function

33.6. Cardiac Constraints

33.7. Concluding Remarks

33.8. Clinical Implications

Chapter 34. Physical Activity Assessment and Impact

34.1. Introduction

34.2. Tools for Measuring Physical Activity

34.3. Normative Data for Physical Activity in CF

34.4. Methods of Analyzing and Reporting Physical Activity Data

34.5. Evidence for Impact of Physical Activity on Health of Patients with CF

34.6. Discussion

Chapter 35. Motivating Physical Activity: Skills and Strategies for Behavior Change

35.1. Beyond a Prescription

35.2. A’s of Physical Activity Counseling: A Framework for Behavior Change

35.3. Physical Activity Resources for Patients and Practitioners

Chapter 36. Diet, Food, Nutrition, and Exercise in Cystic Fibrosis

36.1. Nutrition Considerations for Exercise

36.2. Hydration Considerations for Exercise

Chapter 37. Personalizing Exercise and Physical Activity Prescriptions

37.1. Introduction

37.2. Components of a Comprehensive Exercise Prescription

37.3. Individualizing Physical Activity and Exercise Prescription for the Adult with Cystic Fibrosis

37.4. Individualizing Physical Activity and Exercise Prescription in the Child with Cystic Fibrosis

Section G. FAT and LIPID Metabolism in Cystic Fibrosis

Chapter 38. The Pancreatic Duct Ligated Pig as a Model for Patients Suffering from Exocrine Pancreatic Insufficiency—Studies of Vitamin A and E Status

38.1. Introduction

38.2. Advantages of an Animal Model to Study Effects of EPI on Vitamin Status

38.3. Effects of EPI on Serum α-Tocopherol Concentrations in Adult Minipigs, Study 1

38.4. Effects of EPI on Retinol and α-Tocopherol Concentrations in Serum and Liver Tissue of Juvenile Pigs, Study 2

38.5. Discussion

Chapter 39. Unsaturated Fatty Acids in Cystic Fibrosis: Metabolism and Therapy

39.1. Introduction

39.2. Patterns of Polyunsaturated FA Alterations in CF

39.3. Relationship between FA Alterations and Clinical Features of CF

39.4. Mechanisms of Altered FA Metabolism in CF

39.5. Dietary and Therapeutic Implications of FA Metabolism in CF

39.6. Conclusions

Chapter 40. Essential Fatty Acid Deficiency in Cystic Fibrosis: Malabsorption or Metabolic Abnormality?

40.1. Introduction

40.2. Essential Fatty Acids

40.3. Fatty Acids in Cystic Fibrosis

40.4. Issues on Obesity and How to Intervene

40.5. Conclusions

Chapter 41. Persistent Fat Malabsorption in Cystic Fibrosis

41.1. Introduction

41.2. Physiology of Intestinal Dietary Fat Absorption

41.3. Impaired Intestinal Dietary Fat Absorption in Cystic Fibrosis

41.4. Intestinal pH and Bicarbonate Secretion

41.5. Intraluminal Bile Salts

41.6. Intestinal Mucosal Abnormalities

41.7. Small Intestinal Bacterial Overgrowth

41.8. Small Intestinal Transit Time

41.9. Conclusion

Chapter 42. Omega-3 Fatty Acids and Cystic Fibrosis

42.1. Introduction

42.2. Omega-3 Fatty Acid: A Role in Cystic Fibrosis?

42.3. Omega Fatty Acids and CF: Animal Models

42.4. Omega Fatty Acids and CF: Clinical Trials

42.5. Conclusion

Index

Copyright

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Dedication

The book is dedicated to the strong interest of Mr. Elwood Richards in food and supplements to help those with cystic fibrosis. This was possible due to his lifelong interest in components in plants that provide health promotion and disease prevention for those with genetic diseases. He knows and supports friends with cystic fibrosis and this book is an outgrowth of his concern for individuals.

Preface

There is increasing research including clinical trials showing a link between nutrition and food, and many aspects of health in cystic fibrosis. This book brings together many expert basic and clinical researchers to focus on the role of nutrition and diets in disease and therapy of cystic fibrosis. The overall evaluation of the various reviews is that nutrition and food protects and provides health and function to cystic fibrosis with and is important in longevity, cognitive impairment, and lung structure in cystic fibrosis.

In the first section overviews of the role of nutrition and diet on cystic fibrosis (CF) patients are provided. In particular nutrition plays a key role in early life in pregnant women according to Michel and Mueller. Dr. Quick shows that disordered eating changes body image. Screening in neonatal is important to provide optimum nutrition for growth and health. Amanda Leonard describes the various treatment guidelines and risk classifications of children with CF. Torres evaluates the role of nutrition in clinical practice relating to spirometry. Clearly family plays a critical training for CF children at mealtimes according to Hammons, Everhart, and Fiese. As found in many other conditions, the hormone melatonin, available over-the-counter, regulates and treats sleep dysfunction and CF as summarized by Watson. De Monestrol’s review shows the effects of age at diagnosis on CF disease progression. Finally a group of Italian researchers describe the role of lactoferrin in lung and airway infection. The very important actions of CF on digestive enzymes and the role of probiotics and foods in treatment of intestinal dysfunction are reviewed. In particular Pohl reviews the actions of celiac disease on CF and its manifestations. Bryon discusses the evidence that eating disorders and disturbed eating attitudes and behaviors have unique manifestations in CF patients. The Starks describe methods of better airway use in breathing for better health. Alcohol use can play an important role in CF function and is reviewed. Finally specialty foods designed for CF children are helpful according to McGuckin.

In the second section the role of vitamin D deficiency as well as replacement by supplement is reviewed from several aspects. Mailhot shows the effects of the disease on vitamin D bioavailability. Singh describes fat soluble vitamin deficiency in general in CF. Robberecht asks and answers the question about the role of light in providing vitamin D supplementation. Beckett, Shaw, and Sathe define pediatric CFs’ effects on fat-soluble vitamins in children.

The third section continues this theme by evaluating vitamin deficiency, especially antioxidants and then their supplementation in promoting health in cystic fibrosis. Inflammation is a significant problem in CF patients. Sadowska-Bartosz describes nutritional strategies to reduce inflammation and oxidative stress. Similarly Offenberger describes the importance of vitamin A supplementation as therapy for CF. Many dietary supplementations with natural products contain polyphenols which are very functional. Kubow describes the developing sets of studies showing polyphenols in treatment modalities in CF. To study bacterial infections animal models of CF are used. Ciofu demonstrates the role of oxidative stress and their lessons for CF patients. Two different authors, Jagannath and Kleinman review vitamin K in therapy of CF.

The fourth section describes major chronic diseases, particularly diabetes are associated with CF. It covers the role of nutrition and food in CF. Hameed reviews the role of insulin on body mass and growth in young CF patients. Balzer investigates the published information on low glycemic index through dietary interventions in CF patients. Finally Jimenez reviews the management of insulin resistance common in CF.

The fifth section deals with the critical dysfunctions of the lungs and where food and nutrition may play roles. Probiotics are increasingly used to modify and regulate pulmonary functions. Shalem describes the literature on probiotic supplementation as it relates to CF dysfunctional pulmonary exacerbations. Diabetes, according to the review by Frias and Barrio affects lung function and nutritional status, and thus is open to regulation.

The sixth section deals with exercise in the health of CF patients and is a major component of the book for CF patient health. Exercise is something that is affected by lung dysfunction, but within the reach of most patients to include in their lives and lifestyles. Lands and Hebestreit describe methods to test for exercise efficacy including how and what to test. A team of authors, Hulzebos, Werkman, Bongers, Arets and Takken review and update a discussion of the exercise limitations imposed by CF on the lungs. Alarie reviews exercise by assessing physical activity and then discussing its impact on the physiology and functions of CF patients. Clearly for anyone to exercise effectively requires motivation. Chambliss investigates the literature relative to the skills and strategies for behavior change to affect the level of physical activity. Kench takes a broad overview of foods and diet and their roles in exercise in the special population that CF patients are. Finally Nippens reviews literature personalizing exercise and physical activities and how to define that for the CF patient.

The final section focuses on a critical component of the diet for the health of CF patients, lipids and fats. Mosseler, Schwarzmaier, Höltershinken, and Kamphues work together to describe results from the pig, an excellent animal model for human lipid use. They look at pancreatic duct ligation as a model for patients with pancreatic insufficiency with the focus on fat soluble vitamins E and A. Van Biervliet and Strandvick ask and answer the question as to whether essential fatty acid deficiency in CF patients is due to malabsorption and/or metabolic abnormalities. Understanding of this concern is vital for designing methods to treat fat deficiencies. Bodewes and Wouthuyzen-Bakker review the causes and consequences of persistent fat malabsorption. Finally Paul and Watson look at the role of omega-3 fatty acids which are becoming recognized as health promoting and needed in increased amounts in the diets of most children and adults, with an emphasis on CF patients.

Acknowledgments

The work of Dr Watson’s editorial assistant Bethany L. Stevens and the editorial project manager Ms. Shannon Stanton, in communicating with authors and working on the manuscripts, was critical for the successful completion of the book. It is very much appreciated. Support for Ms Stevens’ and Dr Watson’s work was graciously provided by Natural Health Research Institute www.naturalhealthresearch.org. It is an independent, nonprofit organization that supports science-based research on natural health and wellness. It is committed to informing about scientific evidence on the usefulness and cost-effectiveness of diet, supplements, and a healthy lifestyle to improve health and wellness, and reduce disease. Finally the work of the librarian of the Arizona Health Science Library, Mari Stoddard, was vital and very helpful in identifying the key researchers who participated in the book.

Biography

Dr Ronald Ross Watson has studied the role of bioactive nutrients, dietary supplements, and alternative medicines for 40 years. He has been funded to conduct research by grants provided by the U.S. National Institute of Heart, Lung and Blood, the American Heart Foundation, as well as companies and private foundations; in addition, he conducts research for a small company to study effects of novel dietary supplements to modify age and autoimmune diseases in mice and humans, including obtaining patents for the discoveries. Dr Watson has edited 102 biomedical books on topics including aging, dietary supplements, and the role of nutrients in health and prevention of disease. He graduated from Brigham Young University with a degree in Chemistry in 1966 and later completed his PhD from Michigan State University in 1971 with a focus on Biochemistry. Dr Watson’s current appointments are in the School of Medicine and the Department of Nutritional Sciences at the University of Arizona, reflecting a long and distinguished interest in dietary supplements and novel foods in health.

List of Contributors

Nancy Alarie,     Physiotherapy Department, Montreal Children’s Hospital, McGill University Health Centre, Montreal, QC, Canada

H.G.M. Arets,     Department of Pediatric Respiratory Medicine, Cystic Fibrosis Center, University Medical Center Utrecht, Utrecht, The Netherlands

Fiona S. Atkinson,     School of Molecular Bioscience, The University of Sydney, NSW, Australia

Ben W.R. Balzer

Academic Department of Adolescent Medicine, The Children’s Hospital at Westmead, NSW, Australia

Discipline of Paediatrics and Child Health, Sydney Medical School, The University of Sydney, NSW, Australia

Raquel Barrio,     Diabetes Pediatric Unit, Ramón y Cajal Hospital, Alcalá University, Madrid, Spain

Grzegorz Bartosz

Department of Biochemistry and Cell Biology, University of Rzeszów, Rzeszów, Poland

Department of Molecular Biophysics, University of Łódź, Łódź, Poland

Kacie Beckett,     Cystic Fibrosis Clinic, University of Texas Southwestern, Dallas, TX, USA

Kirstine J. Bell,     School of Molecular Bioscience, The University of Sydney, NSW, Australia

Francesca Berlutti,     Department of Public Health and Infectious Diseases, Sapienza University of Rome, Rome, Italy

S. Van Biervliet,     Department of Pediatric Gastroenterology and Nutrition, Ghent University Hospital, Ghent, Belgium

Frank A.J.A. Bodewes,     University Medical Center Groningen, University of Groningen, Groningen, The Netherlands

B.C. Bongers,     Child Development & Exercise Center, Wilhelmina Children’s Hospital, University Medical Center Utrecht, Utrecht, The Netherlands

Aaron Robert Brussels,     Health Sciences Center, School of Medicine, Mel and Enid Zuckerman College of Public Health, University of Arizona, Tucson, AZ, USA

Mandy Bryon,     Cystic Fibrosis Service, Great Ormond Street Hospital for Children, London, UK

Carol Byrd-Bredbenner,     Department of Nutritional Sciences, Rutgers University, New Brunswick, NJ, USA

Amy Cantrell,     Division of Pediatric Endocrinology, Scott and White Hospital, Temple, TX, USA

Angela Catizone,     Department of Anatomy, Histology, Forensic Medicine, and Orthopedics, Sapienza University of Rome, Rome, Italy

Oana Ciofu,     Department of International Health, Immunology and Microbiology, Costerton Biofilm Center, Faculty of Health Sciences, University of Copenhagen, Denmark

M. Francisco Rivas Crespo,     Pediatric Endocrinology, Universidad de Oviedo, Hospital Universitario Central de Asturias, Oviedo, Spain

Isabelle de Monestrol

Stockholm CF Center, Karolinska University Hospital, Stockholm, Sweden

Department of Clinical Science, Intervention and Technology, Division of Pediatrics, Karolinska Institutet, Stockholm, Sweden

Dimitri Declercq,     Cystic Fibrosis Centre, Department of Pediatrics, Ghent University Hospital, Belgium

Ieda Regina L. Del Ciampo,     University of São Paulo, São Paulo, Brazil

Robin S. Everhart,     Psychology, Virginia Commonwealth University, Richmond, VA, USA

Barbara H. Fiese,     Human and Community Development, University of Illinois, Urbana–Champaign, IL, USA

Mary Shannon Fracchia,     Department of Pediatrics, Massachusetts General Hospital, Boston, MA, USA; Harvard Medical School, Boston, MA, USA

Alessandra Frioni,     Department of Public Health and Infectious Diseases, Sapienza University of Rome, Rome, Italy

Manyan Fung,     School of Dietetics and Human Nutrition, McGill University, QC, Canada

Sabina Galiniak,     Department of Biochemistry and Cell Biology, University of Rzeszów, Rzeszów, Poland

Carlos Bousoño García,     Pediatric Gastroenterology and Nutrition Unit, Universidad de Oviedo, Hospital Universitario Central de Asturias, Oviedo, Spain

Shihab Hameed

Endocrinology Department Sydney Children’s Hospital, Randwick, NSW, Australia

School of Women’s and Children’s Health, University of New South Wales, Kensington, NSW, Australia

Amber J. Hammons,     Fresno in the Child, Family, and Consumer Sciences Department, California State University, Fresno, CA, USA

Helge Hebestreit,     Universitaets-Kinderklinik, Josef-Schneider-Strasse 2, Wuerzburg, Germany

Wendy Anne Hermes,     Department of Nutrition, Byrdine F. Lewis School of Nursing and Health Professionals, Georgia State University, Atlanta, GA, USA

M. Höltershinken,     Institute of Animal Nutrition, University of Veterinary Medicine Hannover, Foundation, Hannover, Germany

H.J. Hulzebos,     Child Development & Exercise Center, Wilhelmina Children’s Hospital, University Medical Center Utrecht, Utrecht, The Netherlands

Vanitha Jagannath,     Specialist Pediatrician in American Mission Hospital, Manama, Kingdom of Bahrain

David Gonzalez Jiménez,     Pediatric Gastroenterology and Nutrition Unit, Universidad de Oviedo, Hospital Universitario Central de Asturias, Oviedo, Spain

J. Kamphues,     Institute of Animal Nutrition, University of Veterinary Medicine Hannover, Foundation, Hannover, Germany

Andrea Kench,     Department of Nutrition & Dietetics and Respiratory Medicine, The Children’s Hospital Westmead, Westmead, NSW, Australia

Lisa Kent,     Centre for Health and Rehabilitation Technology (CHaRT), Institute of Nursing and Health Research, University of Ulster, Northern Ireland,UK; Department of Respiratory Medicine, Belfast Health and Social Care Trust, Belfast, Northern Ireland, UK

Ronald E. Kleinman

Department of Pediatrics, Massachusetts General Hospital, Boston, MA, USA

Harvard Medical School, Boston, MA, USA

Stan Kubow,     School of Dietetics and Human Nutrition, McGill University, QC, Canada

Larry C. Lands,     Montreal Children’s Hospital, Pediatric Respiratory Medicine, McGill University, Montreal, QC, Canada

Amanda Leonard,     Division of Gastroenterology and Nutrition, The Johns Hopkins Children’s Center, Baltimore, MD, USA

Amy Lowichik,     Division of Pediatric Pathology, Primary Children’s Medical Center, Salt Lake City, UT, USA

G. Mailhot

Department of Nutrition, Université de Montréal, Montréal, QC, Canada

Gastroenterology, Hepatology and Nutrition Unit, CHU Sainte-Justine Research Center, Montréal, QC, Canada

María Martín-Frías,     Diabetes Pediatric Unit, Ramón y Cajal Hospital, Alcalá University, Madrid, Spain

Megan Elizabeth McGuckin,     University of Arizona, Department of Immunobiology, USA

Suzanne H. Michel,     Clinical Assistant Professor, Medical University of South Carolina, Charleston, SC, USA

Alison Morton,     Regional Adult Cystic Fibrosis Unit and Department of Nutrition and Dietetics, Leeds Teaching Hospitals NHS Trust, St James’s Hospital, Leeds, UK

A. Mößeler,     Institute of Animal Nutrition, University of Veterinary Medicine Hannover, Foundation, Hannover, Germany

Donna H. Mueller,     Nutrition Sciences Department, Drexel University, Philadelphia, PA, USA

Noor Naqvi,     School of Dietetics and Human Nutrition, McGill University, QC, Canada

Matthew Nippins

Northeastern University, Boston, MA, USA

Massachusetts General Hospital, Wang Ambulatory Care Center, Boston, MA, USA

Michael O’Connor,     Department of Pediatrics, Division of Allergy, Immunology, and Pulmonology Medicine, Vanderbilt University School of Medicine, Nashville, TN, USA

Holly M. Offenberger,     College of Science, Mel and Enid Zuckerman College of Public Health, University of Arizona, Tucson, AZ, USA

Gaurav Paul,     College of Public Health, University of Arizona, Tucson, AZ, USA

John F. Pohl,     Department of Pediatric Gastroenterology, Primary Children’s Medical Center, University of Utah, Salt Lake City, UT, USA

Virginia Quick,     Eunice Kennedy Shriver National Institute of Child Health and Human Development/National Institutes of Health, Division of Intramural Population Health Research, Bethesda, MD, USA

Gilbert L. Rivera Jr.,     University of Arizona, College of Public Health, Tucson, AZ, USA

Eddy Robberecht,     Cystic Fibrosis Centre, Department of Pediatrics, Ghent University Hospital, Belgium

Izabela Sadowska-Bartosz,     Department of Biochemistry and Cell Biology, University of Rzeszów, Rzeszów, Poland

Donatello Salvatore,     Cystic Fibrosis Center, Pediatric Center Bambino Gesù Basilicata, Hospital San Carlo, Potenza, Italy

Meghana Sathe,     Department of Pediatric Gastroenterology and Nutrition, University of Texas Southwestern, Dallas, TX, USA

T. Schwarzmaier,     Institute of Animal Nutrition, University of Veterinary Medicine Hannover, Foundation, Hannover, Germany

Adam Seegmiller,     Department of Pathology, Microbiology, and Immunology, Vanderbilt University School of Medicine, Nashville, TN, USA

Hiran Selvadurai,     Respiratory Medicine, The Children’s Hospital Westmead, Westmead, NSW, Australia

Tzippora Shalem

Division of Pediatric Gastroenterology and Nutrition, Edmond and Lily Safra Children’s Hospital, Tel-Hashomer, Israel

Sackler Faculty of Medicine, Tel-Aviv University, Tel-Aviv, Israel

Karyn Shaw,     Department of Clinical Nutrition, Children’s Medical Center, Dallas, TX, USA

Vijay Karam Singh,     Health Sciences Center, School of Medicine, Mel and Enid Zuckerman College of Public Health, University of Arizona, Tucson, AZ, USA

Jennifer Stark

Buteyko Institute of Breathing and Health (Inc), Fitzroy, VIC, Australia

Buteyko Breathing Educators Association, IN, USA

Russell Stark

Buteyko Institute of Breathing and Health (Inc), Fitzroy, VIC, Australia

Buteyko Breathing Educators Association, IN, USA

Katharine S. Steinbeck

Academic Department of Adolescent Medicine, The Children’s Hospital at Westmead, NSW, Australia

Discipline of Paediatrics and Child Health, Sydney Medical School, The University of Sydney, NSW, Australia

B. Strandvik,     Department of Biosciences and Nutrition, Karolinska Institutet, Stockholm, Sweden

T. Takken,     Child Development & Exercise Center, Wilhelmina Children’s Hospital, University Medical Center Utrecht, Utrecht, The Netherlands

Vin Tangpricha,     Division of Endocrinology, Metabolism and Lipids, Department of Medicine, Emory University School of Medicine, Atlanta, GA, USA

Lidia Alice G.M.M. Torres,     University of São Paulo, São Paulo, Brazil

Piera Valenti,     Department of Public Health and Infectious Diseases, Sapienza University of Rome, Rome, Italy

Charles F. Verge

Endocrinology Department Sydney Children’s Hospital, Randwick, NSW, Australia

School of Women’s and Children’s Health, University of New South Wales, Kensington, NSW, Australia

Henkjan J. Verkade,     University Medical Center Groningen, University of Groningen, Groningen, The Netherlands

Ronald Ross Watson,     University of Arizona, Mel and Enid Zuckerman College of Public Health, Sarver Heart Center in the School of Medicine, Tucson, AZ

Batia Weiss

Division of Pediatric Gastroenterology and Nutrition, Edmond and Lily Safra Children’s Hospital, Tel-Hashomer, Israel

Sackler Faculty of Medicine, Tel-Aviv University, Tel-Aviv, Israel

M.S. Werkman,     Child Development & Exercise Center, Wilhelmina Children’s Hospital, University Medical Center Utrecht, Utrecht, The Netherlands

Sue Wolfe,     Regional Paediatric Cystic Fibrosis Unit and Department of Nutrition and Dietetics, Leeds Teaching Hospitals NHS Trust, Leeds Children’s Hospital, Leeds General Infirmary, Leeds, UK

Marjan Wouthuyzen-Bakker,     University Medical Center Groningen, University of Groningen, Groningen, The Netherlands

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. Mueller²     ¹Clinical 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 more compromised pregnancy outcome for both themselves and their infants [12,13,19,20]. Table 1.1 summarizes information provided in the reviewed papers.

1.3. Nutrition: Review of the Literature

1.3.1. Weight

In all women, with or without CF, prepregnancy weight, rate of weight gain, and total weight gain during pregnancy are crucial measures to follow for both the woman’s postpartum health and for her baby. Although historically pregnancy in women who had CF occurred in women who were PS and more nutritionally stable [8,9], more recent studies report pregnancy in both PS and PI women with weight, rather than pancreatic status, considered to be the important factor [2,10].

Two studies provide useful clarification. Cheng et al. reported that 7 of the 16 women he studied had a prepregnancy body mass index (BMI) of <20  kg/m². Lower preconception BMI was associated with more hospitalizations and greater need for intravenous antibiotics. Mean weight gain was 10.4  ±  4.5  kg for the entire group and 11.4  ±  5.0  kg for women who delivered at term. Compromised maternal weight or pulmonary status resulted in nine inductions and was associated with longer postpartum length of stay as well as with a tendency toward lower fetal weight. All of the women returned to prepregnancy weight by 2 months after delivery [10]. Gilljam et al. studied 92 pregnancies in 54 women. The mean BMI both pre- and postpregnancy was 21.6  kg/m², with a mean pregnancy weight gain of 8  ±  5  kg. No difference in the 10-year postpartum survival was identified between women with a prepregnancy BMI <20  kg/m² or >20  kg/m². Yet, PS women had better survival when compared with PI women, with no explanation from the authors as to the cause. Six infants were born before 37 weeks’ gestation, four infants were considered small for gestational age, and three were classified low birth weight [12].

The increased nutritional demands of pregnancy may place women with CF at risk [21]. Lau et al. wrote, Poor nutrition is usually a marker of more severe disease and has been associated with worse outcomes and low birth weights. Severe malnutrition (BMI  <  18  kg/m²) is considered a relative contraindication to pregnancy [20]. Goss et al. followed women for 12 years after delivery. Although the women who became pregnant had a higher BMI when compared with women who did not become pregnant, after adjusting for demographic differences, there was no difference in survival [11]. Using British registry data of 1148 females, Boyd et al. noted that 24.3% had weight below the 10th percentile, which placed them at risk for infertility, preterm birth, and delivery complications [22]. The women assessed by McMullen et al. had a higher total weight but not a higher percent of ideal body weight when compared with women who did not become pregnant. Following delivery, the ideal body weight was greater when compared with preconception weight [2]. In the group studied by Thorpe-Beeston et al., 25 women (52.1%) were PI, with a mean BMI at conception of 21.9  ±  3.6  kg/m². No information was provided describing weight gain throughout the pregnancy [13]. In the retrospective review of 15 pregnancies in 12 women by Burden et al. the mean prepregnancy BMI was 21.4  kg/m² (18.3–24) with a mean weight gain of 5.74  kg (1–10) [14].

TABLE 1.1

Summary of Selected Papers Describing Pregnancy in Women Who Have CF

NI, Not included in paper; IBW, Ideal body weight; BMI, Body mass index; TF, Enteral tube feeding; PN, Parenteral nutrition.

a Number of women/number of pregnancies/number of live births.

Modified from Michel SH, Mueller DH. Nutrition for pregnant women who have CF. J Acad Nutr Diet 2012;112:1943–1948.

In a study of preconception counseling, women who had CF and received preconception counseling had significantly greater mean maternal weight gain and significantly heavier babies when compared with women who did not receive counseling [23]. Edenborough et al. suggested that BMI before pregnancy should be within, and preferably at the upper end, of recommended values for women [24]. Chetty et al. noted that increased preterm delivery and poor fetal growth is associated with low BMI; therefore, careful management of maternal weight is important. The authors noted that BMI of at least 90% of recommended is ideal before conception. [25]

1.3.2. Diabetes

In women without CF, elevated glucose levels during pregnancy is associated with adverse perinatal and maternal outcomes [26]. In these women, aggressive treatment of mild gestational diabetes improved outcomes [27]. Work by Hardin et al. elucidated the metabolic basis that predisposes pregnant CF women to early development of diabetes and poor weight gain, thereby requiring greater intake of calories and protein when compared with the non-CF pregnant women. The researchers noted that pregnancy in CF is associated with decreased insulin sensitivity and high hepatic glucose production, as well as decreased insulin secretion [28]. Along with increased protein turnover, response was lower to insulin’s anticatabolic effect. McMullen et al. noted the most frequently documented complication was the need for the management of diabetes [2]. Nine percent of the women studied by Tonelli et al. were on diabetes treatment before pregnancy, which increased to 21% during pregnancy [29]. Both Cheng et al. and Gilljam et al. found a high rate of diabetes in the populations they studied [10,12]. In the 10 diabetic mothers followed by Gilljam et al., one infant was born at 31 weeks’ gestation and died of sepsis at 18 days of life, two infants were preterm with low birth weight, and one was small for gestational age [12]. Thorpe-Beeston et al. reported that 17 women (35.4%) required insulin, but it is not clear whether the insulin was used before pregnancy or as the result of the pregnancy. Although the women required insulin, the mean birth weight percentile in the series was 31.9. The authors hypothesize that the birth weight may reflect good diabetic control, frequent clinic visits, or other metabolic factors, such as the increased energy needs of CF [13]. In the series described by Burden et al., diabetes was present in 57% (8/14), with preexisting CFRD in 28.5% (4/14) and with diagnosis during pregnancy in 28.5% (4/14) [14]. Schechter et al. reported diabetes at baseline in 11.8% of the women who became pregnant and in 12.5% in the women who did not become pregnant [15].

In women who have CF, information describing the progression of CFRD during pregnancy or the progression of those who develop gestational diabetes is limited. Therefore, it is difficult to determine either maternal or infant risk [10,30]. Data that are available imply a greater risk for both mother and infant when diabetes and poor pulmonary function are present in the mother [31]. As is true for women who do not have CF, either diabetes before pregnancy or gestational diabetes will place the woman and her baby at risk; both situations require careful overall management [13,26]. Balancing the need for maternal weight gain while managing previously existing CFRD or gestational diabetes is challenging and requires the ongoing involvement of a registered dietitian or nutritionist familiar with CF [32].

1.3.3. Vitamins and Minerals

No studies have been conducted specific to the management of vitamin and mineral nutrition for women who have CF, either for those considering pregnancy or during pregnancy. Edenborough et al. suggested folic acid at the dosage recommended for women who do not have CF [31]. Consideration is given to appropriate dosage for women with a previous infant with neural tube defects or women with diabetes [33]. Vitamin A deficiency or excess are teratogenic and associated with adverse reproductive outcomes [34]. Evaluation of serum retinol levels in a small group of pregnant women who had CF found serum levels to be within normal range on usual CF-specific vitamin supplementation [35]. Table 1.2 provides fat-soluble vitamin and zinc content of CF-specific products available for adults at the time this chapter was prepared. Information on the over-the-counter product is provided as a basis of comparison.

1.3.4. Nutrition Support

Pregnancy places an additional nutrition burden on women who have CF. Besides maintaining optimal energy and nutrient intake to meet the demands of CF, women also must meet the increased requirements imposed by pregnancy. Less than optimal prepregnancy weight, coupled with an inability to consume adequate energy for weight gain before pregnancy may be reasons to initiate oral energy supplementation or enteral tube feeding. Enteral tube feeding that is required before pregnancy may be an indicator of the challenges that will face the woman while pregnant [31]. Moreover, during pregnancy, enteral feeding may be necessary for women with inadequate weight gain. Tube feeding combined with the elevated intra-abdominal pressure of the expanding uterus may reveal or exacerbate gastroesophageal reflux. Gastrostomy or jejunostomy tube feeding may be prescribed [19,35,36]. Total parenteral nutrition (TPN) may become necessary [10,19]. Inadequate weight gain, weight loss, intractable nausea, and vomiting were the indications for TPN in the population studied by Cheng et al. Prepregnancy BMI or lung function did not predict who would require TPN. Weight gain was less for those receiving TPN compared with those who did not require it [10]. TPN may be indicated for pregnant women who develop pancreatitis during pregnancy [37,38]. McMullen et al. reported that the percentage of women receiving both oral and parenteral nutritional supplementation increased during pregnancy and continued after pregnancy as well as during lactation [2].

TABLE 1.2

Comparison of CF-Specific Vitamin and Mineral Supplements for Adults in the United States, July 2013a

a The content of this table was confirmed in July 2013. Products also contain water-soluble vitamins.

b MVW Complete Formulation Softgels™ is a trademark of MVW Nutritionals, Inc.

c AquADEKs Softgels® is a registered trademark of Yasoo Health, Inc.

d Centrum Tablets® is a registered trademark of Wyeth Consumer Care, Inc.

e Alpha-tocopherol.

f Product contains mixed tocopherols.

1.4. Clinical Guidance

Managing reproductive nutrition in all women includes preconception, pregnancy, and postnatal care. Nourishment, nutrients, food, and eating are intertwined with other aspects of daily life as well as with CF-related daily treatments. Each woman with CF is different. Although everyone awaits pertinent research discoveries, patients and their professional team members use current knowledge and then modify the approaches for personalized care. Medical nutrition therapy for each patient must be individualized, based on the known and unknown scientific facts, and in consideration of the patient’s current health status and wishes.

Fortunately, in clinical practice for patients with CF, the standard of practice expects the patient to be at the center of an interdisciplinary team model of care and, in the United States, receiving care at a CFF-accredited center. Such centers have core teams, including a physician, registered nurse, registered dietitian, social worker, and physical or respiratory therapist. When possible before conception and during pregnancy, patients and their families are involved with additional team members in endocrinology, gastroenterology, genetics, obstetrics, psychology, and pharmacology.

1.4.1. Specific Nutrition Actions

Women who have CF can become pregnant and deliver healthy infants, although prematurity and low birth weight are possible [10,29,39]. On the basis of currently available research, pregnancy outcome is unpredictable. Designing nutrition care plans is challenging, because currently no standard of care has been established for the nutrition management of pregnant women who have CF. Consequently, nutritionally, each woman must be assessed and managed individually, before, during, and after pregnancy [10]. The specialty-trained and experienced registered dietitian or nutritionist is essential in preparing all women with CF of childbearing age for possible pregnancy by providing nutrition management directed toward optimal weight, serum vitamin and mineral levels, and overall dietary quality [14].

1.4.2. Assessment and Counseling

For women who have CF, little research evidence currently exists. Thus, it seems reasonable that overall medical nutrition therapy should rely on recommendations established for women without CF and be modified for the specific situations experienced by each individual woman who has CF [1,40]. Evidence suggests that prenatal counseling can result in better nutrition status and pregnancy outcomes [23]. For the woman planning pregnancy or who is already pregnant, the registered dietitian or nutritionist should perform a comprehensive nutrition assessment that focuses on (1) historical and current weight status and (2) historical and current dietary nutrient adequacy that includes both the intake levels of known nutrients, as well as the serum levels of glucose, fat-soluble vitamins, folic acid, and minerals such as iron and calcium.

Other recommended topics for assessment and counseling include the use of alcohol and caffeine; methods to avoid foodborne illness; appropriate intake of fish regarding potential heavy metals and toxins; and use of herbals, either as dietary supplements or as medicines [21]. All women may be at risk for developing pregnancy-exacerbated reflux and constipation or pancreatitis in PS women [24]. A woman who is diagnosed with pancreatitis, gestational diabetes, and other concomitant medical conditions, or who is an adolescent, presents additional nutritional challenges.

1.4.3. Weight, Enzymes, and Energy

The importance of optimal weight for all women during pregnancy has been well documented [40]. Weight before pregnancy determines pregnancy weight gain goals. Women at recommended body weight require less weight gain throughout pregnancy when compared with weight gain goals for women not at the recommended weight [41]. The Institute of Medicine considers normal weight for women who do not have CF to be a BMI of 18.5–24.9  kg/m² and recommends a total weight gain of 25–35 pounds. If body weight before conception is less than the recommended level, weight gain to the desired level is suggested before pregnancy; otherwise, the advised weight gain goal during pregnancy is greater [42]. Some women find it helpful to follow weight gain on a prenatal weight-gain curve, because this is a visual guide to actual weight gain compared with weight goals [43].

Central to optimal weight gain for women who are PI is the use of pancreatic enzyme replacement therapy (PERT). Pregnant women who are PI must continue to use PERT with all meals, snacks, and beverages containing fat and protein. One concern for some pregnant women with CF is phthalates. As of 2009, the Food and Drug Administration (FDA) required all pancreatic enzyme preparations to undergo all steps of a new drug application [44]. Before 2009, supplemental pancreatic enzymes contained a variety of phthalates, including dibutyl phthalate and diethyl phthalate. Not only were these forms of phthalates found in pancreatic enzyme preparations, but they also were in other medical and household items. These forms were researched and were found to be concerning. Since the legislation was enacted, these forms of phthalates have been removed from a majority of products, including medications [45] as well as the currently available FDA-approved pancreatic enzyme replacement products. With the exception of one enzyme product, currently available products contain hypromellose phthalate (HP), which is used as a coating around the enzyme beads to delay degradation by gastric hydrochloric acid. HP is a phthalic acid ester of hydroxypropyl methylcellulose and was introduced in 1971 as a cellulose derivative for enteric coating and is part of the U.S. National Formulary [46]. To date, no research has indicated a harmful effect of this form of phthalate. As suggested by the CFF, patients concerned about HP in enzymes should talk to their CF health care team, but they should not stop taking PERT [46].

Defining the energy needs of people who have CF is challenging and depends on such variables as level of maldigestion and resultant malabsorption, pulmonary function, pulmonary inflammation, fat-free mass, gender, genetic mutation, age, and other medical complications [47]. A formula that incorporates some of these variables has been published [48]. Energy recommendations specific for pregnant women who have CF remain unavailable, however. Consensus suggests the standardized formula be used as a baseline estimate, to which is added the individual caloric needs from foods and supplements of each woman with CF during pregnancy. The objective measure to be followed clinically is suitable weight gain throughout pregnancy to achieve the weight goal. If the weight gain remains inadequate, then oral, enteral, or parenteral nutrition supplementation may become necessary [31].

1.4.4. Protein

Specific information describing the protein needs of people who have CF is limited, with none available for women with CF who are pregnant. Published data from the CF population suggests that protein intake is correlated strongly with energy intake and that those people who consume adequate calories also consume adequate protein [48,49,50]. Research by Hardin et al. suggested that women who have CF and are pregnant may require higher intakes of both calories and protein when compared with pregnant women without CF [28].

1.4.5. Vitamins and Minerals

Before pregnancy, CF standard practice for women is that serum levels of fat-soluble vitamins are to be assessed routinely with adjustments made with the use of foods and dietary supplements. Levels usually are rechecked following changes to supplemental vitamin prescription [31]. Some special considerations exist. Assessment of serum retinol level and retinol intake should be evaluated in the preconceptional period and during pregnancy. Total retinol intake, including the contribution of beta-carotene, is adjusted based on serum retinol levels, with the goal of maintaining serum levels within the normal reference range [31,35]. Serum vitamin D levels are evaluated and vitamin D is supplemented as indicated by serum results [31]. There are no CF-specific folic acid supplementation recommendations. For non-CF pregnant women, the usual recommendation is 400 mcg daily and 4000–5000 mcg daily if considered to be at high risk for an affected (neural tube defects) pregnancy [31]. Dosing recommendations for the general public have been published and include levels based on risk, such as insulin-dependent diabetes [33]. Mineral nutrition is a challenge for pregnant women who have CF. Without calcium and iron recommendations specific for women who have CF, initial supplementation of these nutrients may be based on recommendations for pregnant women who do not have CF.

1.4.6. Blood Glucose

Blood glucose screening is recommended before pregnancy for women who have CF, but do not have diabetes, and who have not been screened using a 2-h oral glucose tolerance test (OGTT) in the previous 6  months [51]. The test is repeated twice: during gestation weeks 12–16 and again during weeks 24–28. For women diagnosed with gestational diabetes, another 2-h OGTT is recommended 6–12 weeks after the end of pregnancy. Blood glucose levels are monitored based on CFF recommendations [51]. Because the diet during pregnancy should contain sufficient calories to promote optimal weight gain, exogenous insulin may be required [31].

1.5. Conclusion

Review of peer-reviewed published research, organizational consensus statements, and clinical judgment opinions strongly indicate the enormous imperative for basic and clinical research studies. Historically and currently, the main nutritional variable identified and studied is weight, or a combination of weight and height as designated as BMI. Because anthropometric indicators only provide superficial information about the nutritional status and nutritional requirements of pregnant women who have CF, clinicians who are involved with people who have CF must become engaged actively in the research process. For example, codifying elements for a case study publication provides the underpinnings for further research endeavors. Some research is the purview of dietitians and nutritionists, whereas some research is better conducted by an interdisciplinary team. Areas to be considered for research include dietary intake and nutrition status in women with CF throughout adolescence and the childbearing years, nutrient needs during pregnancy for women who have CF and for women who experience exacerbation or other medical complications during pregnancy, and optimal methods of nutrition counseling to elucidate the best outcomes for women and their babies. Investigations on reproductive nutrition in all women with CF during all phases of life are an opportunity to advance health care practices for women who have CF and their children.

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