Catherine M. McDonald

Enteral tube feeding for individuals with cystic fibrosis: Cystic Fibrosis Foundation evidence-informed guidelines

Nutrition is integral to the care of individuals with cystic fibrosis (CF). Better nutritional status is associated with improved pulmonary function. In some individuals with CF, enteral tube feeding can be useful in achieving optimal nutritional status. Current nutrition guidelines do not include detailed recommendations for enteral tube feeding. The Cystic Fibrosis Foundation convened an expert panel to develop enteral tube feeding recommendations based on a systematic review of the evidence and expert opinion. These guidelines address when to consider enteral tube feeding, assessment of confounding causes of poor nutrition in CF, preparation of the patient for placement of the enteral feeding tube, management of the tube after placement and education about enteral feeding. These recommendations are intended to guide the CF care team, individuals with CF, and their families through the enteral tube feeding process.

Correlation of Cholestasis With Serum Copper and Whole-Blood Manganese Levels in Pediatric Patients

Many institutions reduce or eliminate copper (Cu) and manganese (Mn) in parenteral nutrition (PN) solutions when cholestasis develops. Little data exist to support this practice. Fifty-four subjects with known serum Cu, whole-blood Mn, and serum-conjugated bilirubin levels were evaluated in this prospective, observational study. Subjects ranged in weight from 760 g to 65.2 kg. Subjects weighing <25 kg received a daily parenteral dose of 20 microg/kg Cu and 5 microg/kg Mn. Subjects weighing > or =25 kg received a dose of 500 microg/d Cu and 150 microg/d Mn. Cholestasis was defined as a conjugated bilirubin level > or =2 mg/dL. Of the 54 subjects, 20 had cholestasis. Fifteen patients had elevated Cu levels, and 21 had high Mn levels. Seven of the subjects had both high Cu and high Mn levels. The regression model comparing cholestasis as a predictor of high, low, or normal Cu level was not significant (P = .9588). Cholestasis was not a significant predictor of high, low, or normal Mn levels (P = .6533). No correlation between Cu and Mn levels was found. The authors found no significant relationship between conjugated serum bilirubin levels > or =2.0 mg/dL, serum Cu, and whole-blood Mn levels. They found insufficient evidence to support the practice of dosing Mn from a Cu level or vice versa. They recommend obtaining Cu and Mn levels on all pediatric patients who develop cholestasis prior to adjusting parenteral doses and at regular intervals for all long-term PN patients.

Validation of a nutrition risk screening tool for children and adolescents with cystic fibrosis ages 2-20 years.

Objectives: According to the 2002 Cystic Fibrosis (CF) Foundation nutrition consensus report, children with CF should grow normally. Cross-sectional data from the foundations patient registry concluded that a body mass index at or greater than the 50th percentile is associated with better lung function. A consistent, evidence-based screening process can identify those individuals with CF having nutrition risk factors associated with a decrease in pulmonary function, target early intervention, and prevent further decline. A tool for screening nutrition risk is described to identify those children with CF who would benefit from more extensive nutrition intervention. Methods: The proposed screening tool is a risk-based classification system with 3 categories: weight gain, height velocity, and body mass index. The CF Foundation recommendations regarding these parameters are incorporated, with risk points assigned when minimum body mass index, weight gain, and/or height gain standards are unmet. Results: An interrater measure of agreement determined a satisfactory level of reliability (κ = 0.85). Patient records (n = 85) were reviewed to determine nutrition status category (no risk or at risk) of this tool compared with the CF Foundation 2002 Nutrition Consensus, yielding sensitivity and specificity at 84% and 75%, respectively. A second comparison was made with combined, independent nutrition risk factors not included in the screening tool. The sensitivity and specificity of the screening tool compared with the combined risk factors were 86% and 78%, respectively. Conclusions: This tool for screening nutrition risk for CF is reliable and valid, with consistent, reproducible results, free from subject or observer bias.

Nutrition knowledge and confidence levels of parents of children with cystic fibrosis.

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,...

Techniques and Procedures: Pediatric Parenteral Nutrition via Computerized Worksheet and Automated Compounding

Ordering parenteral nutrition (PN) that is nutritionally appropriate, pharmacy acceptable, and physically compatible can be complex and time consuming. The Nutrition Support Service at Primary Childrens Medical Center has developed a user-friendly, single-screen computer program for prescribing individualized PN orders for pediatric patients based on weight and nutrient requirements. Educational windows define dosage guidelines and unacceptable parameters. An internal surveillance process identifies incompatibilities resulting from calcium and phosphate concentrations, fluid restrictions, dosages, and cation and anion imbalances. An incompatible solution is prevented and corrected before it is compounded. The completed PN order generates a medical order, pharmacy order documentation sheet, compounding document, and label. The stored PN order interfaces directly with an automated system for compounding without physical manipulation. The computerized worksheet enhances efficient ordering of individualized ...

Differences between WHO AND CDC early growth measurements in the assessment of Cystic Fibrosis clinical outcomes

BACKGROUND Early childhood growth status has been used to predict long-term clinical outcomes in Cystic Fibrosis (CF) patients. Adulthood CF outcomes based on early weight-for-length (WFL) measurements, using either World Health Organization (WHO) or Centers for Disease Control (CDC) scales, have not been compared. METHODS Cystic Fibrosis Foundation registry patients were studied (n=3014). Participants were categorized at age two years as WFL <50th percentile on both WHO and CDC scales, ≥50th percentile on WHO but not CDC, or ≥50th percentile on both. Pulmonary function and overall survival were assessed at age 18years. RESULTS Stepwise gains in pulmonary function and lung transplant-free survival were noted across the three increasing WFL categories. CONCLUSIONS Children with CF who achieve higher WFL at age two years have improved pulmonary and survival outcomes into adulthood. CF providers should continue to utilize current early growth recommendations, with goal WFL ≥50th percentile on CDC growth charts before age two.

Evaluating Changes in Handgrip Strength in Children With Cystic Fibrosis: A Pilot Study

BACKGROUND Body mass index (BMI) is used to determine nutrition status in children with cystic fibrosis (CF); however, lean body mass (LBM) is more strongly associated with pulmonary function. Handgrip strength (HGS) measures muscle function and is reflective of LBM. The aims of this study were to assess relationships among HGS, nutrition status, and pulmonary function; changes in HGS posthospitalization; and any relationship between HGS and nutrient intake. METHODS Twenty-three children with CF aged 6-18 years participated. BMI z scores, nutrition risk scores, and pulmonary function were assessed about 5 months before, days 5-7 of hospitalization, and about 6 weeks posthospitalization. HGS z scores and arm anthropometrics were measured during and after hospitalization. Nutrient intakes were assessed during hospitalization. RESULTS Mean HGS z score at hospitalization was -1.95 ± 0.92 and posthospitalization was -1.59 ± 1.06 (P = .007). Mean BMI z score prehospitalization was -0.17 ± 0.63, at hospitalization was -0.09 ± 0.64, and posthospitalization was 0.06 ± 0.54 (P = .065). Mean forced expiratory volume in 1 second (FEV1 ) prehospitalization was 93.52 ± 17.35, at hospitalization was 85.65 ± 21.57, and posthospitalization was 95.63 ± 18.18 (P = .001). No significant relationship was found between HGS z scores and BMI z scores (P = .892) or HGS z scores and FEV1 (P = .340). CONCLUSIONS HGS z scores and FEV1 significantly increased at follow-up. HGS z scores were lower than the standard even though mean BMI z scores classified participants as normal nutrition status.