Cystic fibrosis: Nutritional issues
- Robert D Baker, MD, PhD
Robert D Baker, MD, PhD
- Professor of Pediatrics
- University at Buffalo, the State University of New York
- Chris Coburn-Miller, MSRD, CSP
Chris Coburn-Miller, MSRD, CSP
- CF Dietitian
- Women and Children's Hospital of Buffalo
- Susan S Baker, MD, PhD
Susan S Baker, MD, PhD
- Professor of Pediatrics
- University at Buffalo, the State University of New York
- Section Editors
- Kathleen J Motil, MD, PhD
Kathleen J Motil, MD, PhD
- Section Editor — Pediatric Nutrition
- Professor of Pediatric Nutrition
- Baylor College of Medicine
- George B Mallory, MD
George B Mallory, MD
- Section Editor — Pediatric Pulmonology
- Associate Professor of Pediatrics
- Baylor College of Medicine
- Melvin B Heyman, MD, MPH
Melvin B Heyman, MD, MPH
- Section Editor — Gastroenterology
- Professor of Pediatrics
- University of California, San Francisco
Children and adolescents with cystic fibrosis (CF) frequently have growth failure caused by the combination of malabsorption, increased energy needs, and reduced appetite. Nutrient delivery and correction of maldigestion and malabsorption are essential to achieve normal growth to support optimal pulmonary function and to prolong life.
The CF Foundation (CFF) patient registry has documented substantial improvement in life expectancy of patients with CF (figure 1) . To a large degree, the longer life achieved by patients with CF can be ascribed to improved treatment of lung disease, pulmonary toilet, potent and tailored antibiotics, dornase alfa (DNase), and lung transplantation. However, greater emphasis on CF nutrition is considered important to improve longevity and quality of life. As a result, the CFF created a consensus report on practical aspects of nutrition in pediatric CF , and a more theoretic report on gastrointestinal outcomes and confounders in CF .
The evaluation, monitoring, and treatment of nutritional problems will be addressed here. The diagnosis and management of CF-related pancreatic insufficiency, and screening for CF-related comorbidities that affect nutritional status will also be discussed briefly here and in detail in separate topic reviews:
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- PANCREATIC INSUFFICIENCY
- ASSESSING AND MONITORING NUTRITION
- Blood tests
- Evaluation for comorbidities
- - Bone disease
- - CF-related diabetes mellitus
- - Pulmonary function testing
- - Cystic fibrosis-related liver disease
- - Small intestine bacterial overgrowth
- - Pancreatitis
- - Intestinal resection
- NUTRIENT DEFICITS AND GOALS
- Fat soluble vitamins
- - Vitamin A
- - Vitamin D
- - Vitamin E
- - Vitamin K
- Essential fatty acids
- NUTRITION SUPPORT
- Pharmacological options
- Enteral feedings
- - Route
- - Schedule
- - Formula
- - Enzyme replacement
- Parenteral nutrition
- INFORMATION FOR PATIENTS
- SUMMARY AND RECOMMENDATIONS