Diet and Exercise in Cystic Fibrosis

Book 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.