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Optimal growth and nutrition status predict better lung function and longevity for children with cystic fibrosis (CF). Daily nutrition therapy for children with CF requires adequate food resources, parental knowledge of nutrition and behavior management, and confidence in one’s ability to apply the skills. The Mountain West Cystic Fibrosis Consortium Questionnaire (MWCFC-Q) was designed to identify educational intervention targets to improve the growth and nutrition of children with CF. Parents of children with CF returned 305 anonymous MWCFC-Qs. Data analyzed included household food security, knowledge of nutrition and general CF therapies, and self-confidence in one’s ability to manage components of CF care. Factors associated with food insecurity were reported by 26.3% of respondents. The median accuracy for questions regarding nutrient content of commonly used foods was 71.4% and 57.9% for CF nutrition therapy. Parents’ self-confidence in overall CF management was relatively high at a mean value 8.28 ± 1.22 of 10 possible. However, mean self-confidence in the CF nutrition domain was significantly less than mean self-confidence for the CF-related tasks domain, which included chest physiotherapy and medication administration (7.75 ± 1.56, 8.62 ± 1.24, P < .001, respectively). Parental knowledge of nutrition for CF and confidence in the application of this knowledge can improve the growth and nutrition status of children with CF. Identification of food security issues may enable health care professionals to adjust nutrition interventions and direct families to appropriate food resources. The MWCFC-Q could be useful for designing and testing educational interventions for nutrition management of CF.

The effectiveness of enteral and parenteral nutrition regimens in preventing or reversing protein-energy malnutrition (PEM) and in preventing treatment delays was evaluated in 32 children receiving treatment for newly diagnosed Stage 111 (3 patients) and IV (29 patients) neuroblastoma. Ten of 18 malnourished patients were randomized to central parenteral nutrition (CPN) and 8 to peripheral parenteral nutrition (PPN) plus enteral nutrition for 4 weeks and then received enteral nutrition (EN: intense nutrition counselling, oral foods and supplements) for weeks 5 through 10. Ten of 14 nourished patients received EN and 4 CPN for 4 weeks and EN thereafter. Dietary, anthropometric and biochemical measurements were determined for weeks 0, 1,2,3,4,7, and 10 for 24 patients who completed the protocols. In malnourished patients, both CPN (seven patients) and P P N (seven patients) were effective in reversing P E M in the first 4 weeks; thereafter, EN effectively maintained nutritional gains in both groups. In nourished patients, EN (seven patients) was not as effective as CPN (three patients) in preventing P E M during the first 4 weeks; afterwards, EN maintained gains in the CPN group but did not promote needed increases in weight nor fat reserves in the EN group. Patients supported by parenteral nutrition (PN, weeks 1-4) had fewer treatment delays (2/17, 12%) than EN patients (4/7,57%, P < 0.05). These data indicate that PN reverses or prevents P E M and prevents treatment delays during the first 4 weeks of intense oncologic treatment and provides nutritional benefits which can be maintained with EN thereafter.Cancer 56:2881-2897. 1985.lTHIN the last decade, multimodal treatment has W vastly improved the outcome of many children with cancer. Prognosis for children with advanced neuroblastoma, however, continues to be discouragingly poor.

Benefits and risks of nutrition support were evaluated in 31 malnourished children with newly diagnosed Wilms' tumor managed according to the third National Wilms' Tumor Study protocol. Patients were classified at diagnosis as being at high nutritional risk (HNR, n = 19) or low nutritional risk (LNR, n = 12). Ten HNR patients were randomized to central parenteral nutrition (CPN) and nine HNR patients were randomized to peripheral parenteral nutrition (PPN) plus enteral nutrition (EN) for 4 weeks of initial intense treatment and EN (nutritional counseling, oral foods and supplements) thereafter. Thirteen HNR patients (seven CPN, six PPN) completed the protocol. Twelve LNR patients received EN; 11 Stage I malnourished patients were randomized to 10 or 26 weeks of chemotherapy. Dietary, anthropometric, and biochemical data were determined for HNR patients at weeks 0-4, 6, 13, 19, and 26 and for LNR patients at weeks 1, 2, 5, and 26. In HNR patients, adequate parenteral nutrition support reversed protein energy malnutrition (PEM), and prevented chemotherapy and radiotherapy delays due to granulocytopenia. CPN was superior to PPN in reversing PEM: energy intake, weight gain, and retinol binding protein were higher (P less than 0.05). LNR patients lost weight and fat reserves in the first 2 weeks of treatment; depletion persisted at week 5, and 25% had chemotherapy delays. Thereafter, EN reversed PEM in patients with both chemotherapy regimens. These data suggest that CPN is preferable during initial intense treatment for HNR patients, and that, although EN is ineffective in preventing depletion and treatment delays in the first 5 weeks of treatment for LNR patients, it is effective thereafter.

Cystic fibrosis (CF) is an inherited disease affecting the respiratory, gastrointestinal, hepatobiliary, and reproductive systems. Nutrition status in persons with CF is often compromised due to increased energy needs, frequent infections, pancreatic insufficiency, lung disease, or CF-related diabetes. Maintaining good nutrition status has been associated with better pulmonary function, reduced hospitalizations, and increased longevity. Nutrition support as oral supplementation (used in >37% of the CF population) or tube feeding (used in >13% of the CF population) is often required for children and adults with CF. The purpose of this update is to describe current consensus and evidence for enteral nutrition support guidelines, reported complications of enteral feeding in the CF population, evidence of expected outcomes, and to discuss related areas requiring further research. A case report is provided to illustrate potential outcomes of aggressive enteral support.

The short-term and long-term effectiveness of central parenteral nutrition (CPN) versus peripheral parenteral nutrition (PPN) in improving muscle mass (arm muscle area [AMA]) was evaluated for 24 malnourished children with newly diagnosed Stage IV neuroblastoma (n = 14) or Stages II-V Wilms' tumor (n = 10). Patients were randomized to either CPN or PPN plus enteral nutrition (EN: intense nutrition counseling, oral foods, and supplements) for 4 weeks followed by EN until week 10. Oncologic treatment was similar for each tumor type. Dietary, anthropometric, and biochemical measurements were obtained at weeks 0, 4, and 10. During weeks 1 through 4, energy (CPN: means 100 +/- 4; PPN: means 96 +/- 4% of healthy children) and protein (CPN: means 2.5 +/- 0.1; PPN means 2.7 +/- 0.2 g/kg) intakes of the two groups did not differ. The AMA increased (P less than 0.05) with 4 weeks of CPN but not with PPN; changes thereafter with EN were not significant. Weight (P less than 0.05) and triceps skinfolds (P less than 0.01) increased with 4 weeks of PN in both groups and decreased with EN thereafter (P less than 0.01) but were higher at week 10 than diagnosis. Increases in albumin in both groups reached significance at week 10 (P less than 0.05). These data show that CPN improves AMA in malnourished children with neuroblastoma or Wilms' tumor when energy and protein intakes are adequate. The AMA gains can be maintained thereafter with EN.

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