Nutrition proactivity in CF

When was the last time you got your Oral Glucose Tolerance Test and your DEXA (bone density) scan?!

Semin Respir Crit Care Med. 2009 Oct;30(5):579-86. Epub 2009 Sep 16.

Nutrition in cystic fibrosis.

Matel JL, Milla CE.

The Stanford Cystic Fibrosis Center, Center for Excellence in Pulmonary Biology, Department of Pediatrics, Stanford University School of Medicine, Palo Alto, California 94304, USA.

Cystic fibrosis (CF) is mostly recognized for its pulmonary morbidity, but the earliest manifestations of the disease are related to its gastrointestinal and nutritional derangements. Destruction of acinar pancreatic tissue, pancreatic ductular obstruction, and lack of enzymatic activity lead to malabsorption (particularly of fats), diarrhea, and failure to thrive. A minority of CF patients carrying milder CF transmembrane conductance regulator (CFTR) mutations have preserved pancreatic secretory activity and are free from significant malabsorption early in life. However, these patients are at risk for losing pancreatic function over time. Nutritional status plays an important role in the progression of the pulmonary disease in CF. Further, CF patients with better nutritional status have a survival advantage. Several factors contribute to impaired nutritional status in CF (e.g., pancreatic insufficiency, chronic malabsorption, recurrent sinopulmonary infections, chronic inflammation, increased energy expenditure, suboptimal intake). Progressive lung disease further increases calorie requirements by increasing the work of breathing. Treatment programs that place an emphasis on higher caloric intake and more aggressive nutritional management in CF patients report better outcomes. Basic tenets of nutritional repletion in CF include the use of pancreatic enzyme replacement therapy and following a high calorie, high protein, unrestricted diet. At the Stanford Cystic Fibrosis Center, nutritional status is assessed on an ongoing basis through anthropometric parameters and annual assessment of body composition, bone density, glucose tolerance, and various biochemical and micronutrient levels. Based on the anthropometric data obtained on routine clinical encounters, patients are categorized as to their nutritional risk. This proactive approach for the early identification of nutritional risk has become a major theme within the network of US CF centers. Aggressive nutritional support with adequate pancreatic replacement management should lead to both normal growth and lung function preservation. In addition, nutritional status has to be monitored closely during routine encounters to allow for early intervention once derangements are noted. This will include increasing calories in the early stages of lung disease and being vigilant of gastrointestinal symptomatology and complications. Copyright Thieme Medical Publishers.

PMID: 19760545 [PubMed - in process]

standardized nutrition classification plan and its relation to nutritional outcomes in children with cystic fibrosis.

J Pediatr Psychol. 2009 May 6. [Epub ahead of print]


Description of a standardized nutrition classification plan and its relation to nutritional outcomes in children with cystic fibrosis.

Leonard A, Davis E, Rosenstein BJ, Zeitlin PL, Paranjape SM, Peeler D, Maynard C, Mogayzel PJ Jr.

Division of Pediatric Gastroenterology and Nutrition.

OBJECTIVE: Better nutrition enhances lung function and increases survival for children with cystic fibrosis (CF). Therefore, we developed a standardized strategy to evaluate nutritional status and create individualized treatment plans to ensure that all patients received the same high-quality care in a busy CF Center. METHODS: A quality improvement approach was undertaken to develop a novel nutrition classification strategy to identify and treat children with subtle manifestations of nutritional deficits in addition to those with obvious nutritional issues. RESULTS: During the 15-month study period, the median body mass index (BMI) percentile increased from 35.2 (0-95.9) to 42.0 (0-97.7), p < .005. Additionally, the number of children with a BMI >/= 50th percentile increased by 11.8%. CONCLUSIONS: Adoption of a standardized approach to nutritional assessment and treatment led to significant improvement in nutritional outcomes of CF patients, demonstrating that systematic changes in clinical practice can improve clinical outcomes substantially over a short period of time.

PMID: 19420226 [PubMed - as supplied by publisher]