F.M. Hollander

Effects of nutritional status and dietetic interventions on survival in Cystic Fibrosis patients before and after lung transplantation

BACKGROUND This study retrospectively investigated nutritional status, dietetic intervention and intake in Cystic Fibrosis (CF) patients before and after lung transplantation (LTX). METHODS Body Mass Index (BMI), Fat Free Mass Index (FFMI) and nutritional intake were retrieved from 75 out-patients aged 15-53 years. Patients were seen every 3-4 months during the waiting list time (range 0-81 months) and up to 116 months after LTX. Survival was measured in months. RESULTS The median BMI at baseline was 19.2 kg/m(2) (range: 15.3 to 28.4 kg/m(2)) with 29 patients (39%) below ≤18.5 kg/m(2). FFMI (measured in 65 patients) had a median of 15.2 kg/m(2) (range: 11.1 to 22.4 kg/m(2)) with 39 patients (60%) ≤16.7 kg/m(2) (men) or ≤14.6 kg/m(2) (women). Median energy intake was 2800 kcal, 239 kcal higher than the estimated energy requirement. However, 8 patients consumed ≥500 kcal less than recommended. Protein intake was 104 (range 60-187) g or 1.9 g/kg per day. Despite dietetic intervention with oral nutritional supplements (ONS) (36 patients), tube feeding (12 patients), or both (13 patients), BMI and FFMI hardly improved pre-LTX. LTX was performed in 51 patients (68%); 10 patients died during follow-up, median survival time was 41 months. A BMI ≤18.5 kg/m(2) was more prevalent in patients who died before LTX (6/9) or who died after LTX (4/10) than in patients who were still alive on the waiting list (5/15) or who survived LTX (14/41). Results for FFMI were comparable. From 6-12 months post-LTX, BMI and FFMI markedly improved, especially in underweight patients. CONCLUSION A BMI ≤18.5 kg/m(2) and an FFMI ≤16.7 kg/m(2) (men) or ≤14.6 kg/m(2) (women) appears to impair survival in LTX candidates with CF. Patients maintained a low body weight before LTX. After LTX weight gain is achieved.

Self-reported use of vitamins and other nutritional supplements in adult patients with cystic fibrosis. Is daily practice in concordance with recommendations?

BACKGROUND In cystic fibrosis (CF), prophylactic supplementation of the fat-soluble vitamins A, D, E, and K is recommended. Limited data is available describing vitamin prescription adherence by adult patients. The aim of this study was to assess the use of prescribed vitamins and other nutritional supplements by adult CF patients. METHODS All adult CF patients (n=111) registered at the Utrecht CF Center were invited to participate in a telephone survey on supplement use. Supplemental vitamin intakes were compared with recommendations. In a subsample, associations between supplemental intake and serum vitamin D and E values were assessed. RESULTS In this study 80% of the patients reported the use of vitamin and/or mineral supplements. Fat-soluble vitamins were used by 43% of pancreatic-sufficient (PS) and 81% of pancreatic-insufficient (PI) patients. Of PI patients reporting supplemental vitamin use, only 9% met the recommendations for vitamin A, 32% for vitamin D, 59% for vitamin E, and 81% for vitamin K. Multivitamin supplements were used by 42% of PI and by 29% of PS patients. Other nutritional supplements were rarely used. CONCLUSION A high percentage of PI patients use fat-soluble vitamins below recommendations. Therefore adequate monitoring of vitamin supplementation and status is warranted.

The optimal approach to nutrition and cystic fibrosis : latest evidence and recommendations

Purpose of review Cystic fibrosis (CF) is a progressive genetic disease that affects multiple organ systems. Therapy is directed to maintain and optimize nutritional status and pulmonary function, as these are key factors in survival. In this review, the most recent findings regarding nutritional management associated with pulmonary function and outcome will be explored. Recent findings Evidence-based and expert-based guidelines emphasize the need for adequate nutritional intake to improve nutritional status. For infants and young children, the aim is to achieve the 50th percentile of weight and length for a healthy same-age population up to age 2 years. For older children and adolescents 2–18 years, the target is a BMI of at or above the 50th percentile for healthy children. For CF adults of at least 18 years, the target is a BMI of at or above 22 kg/m2 for women and at or above 23 kg/m2 for men. Recently, new drugs are developed with the aim to treat the malfunction of the cystic fibrosis transmembrane conductance regulator gene. This potentiator/corrector therapy improves lung function and nutritional status and decreases the number of infective exacerbations. As survival is improving and the CF population is aging, it is important to focus on micronutrient and macronutrient intake of CF patients in different age and disease stages. Summary Recent evidence-based nutritional guidelines and improved medical treatment support the nutritional monitoring and interventions in CF patients. Nutritional care should be personalized and provided by a specialized CF dietitian because patients’ care needs may change dramatically during their disease progress.

Prediction Equations Underestimate Resting Energy Expenditure in Patients With End-Stage Cystic Fibrosis

Background: Resting energy expenditure (REE) is increased in patients with cystic fibrosis (CF) with end-stage lung disease due to chronic inflammation and pulmonary infections. After lung transplantation (LTx), energy expenditure is expected to be lower because inflammation will decrease. We assessed the agreement between measured and predicted REE in pre-LTx CF and post-LTx patients with CF and differences in REE in pre-LTx CF and post-LTx patients with CF in a cross-sectional study. Methods: Included were 12 pre-LTx patients with CF (9 women; median age 31.6 years; interquartile range [IQR], 23.3–40.0) and 12 patients with CF within 2 years after LTx (6 women; median age 33.5 years; IQR, 22.3–40.3). REE was measured in a fasted state using indirect calorimetry. Values were compared with predicted REE calculated by formulas of Harris-Benedict (1919 and 1984), Schofield, and the World Health Organization (1985). A calculated REE between 90% and 110% of REE measured was considered adequate. Results: Prediction equations underestimate REE in at least 75% of pre-LTx and 33% of post-LTx patients with CF. Mean (SD) REE measured by indirect calorimetry was 1735 (251) kcal pre-LTx and 1650 (235) kcal post-LTx (P = .40). REE expressed per kilogram of fat-free mass (FFM) was 40.5 kcal/kg in pre-LTx patients with CF, which was higher than the 34.3 kcal/kg in post-LTx patients with CF (P = .01). Conclusions: Prediction equations underestimate REE in patients with end-stage CF. REE per kg of FFM is lower post-LTx than pre-LTx in patients with CF. Measurement of REE is recommended for patients with CF, especially pre-LTx, to optimize energy requirements for improving nutrition status.

233 Resting energy expenditure in patients with cystic fibrosis decreases after lung transplantation

Objectives The resting energy expenditure (REE) in patients with cystic fibrosis (CF) with end-stage lung disease is increased due to chronic pulmonary infection. After lung transplantation (LTX), the pulmonary infection is decreased and therefore the REE might decrease as well. The aim of this study was to determine the REE in CF patients pre- and post-LTX. For this purpose we both measured and calculated the REE. Methods Included were 12 clinically stable pre-LTX CF patients, (9 females, age 15–50 y) and 12 post-LTX CF patients, (6 females, age 20–56 y). REE was measured in fasted stated by using indirect calorimetry, and calculated by using the prediction equations of Harris & Benedict (HB) 1919 and 1984, Schofield and World Health Organization (WHO) 1985. Results The median REE measured by indirect calorimetry was 1735 kcal (range 1379–2133) before and 1650 kcal (range 1287–1922) after lung transplantation. In 10 out of 12 pre-LTX CF patients (83%) including 2 patients with CF Conclusion After lung transplantation the median REE is lower than before lung transplantation. This possibly is due to a reduction in bacterial infections. Prediction equations do not take this into account. Measurement of REE is a more precise method to determine the energy needs for each individual CF LTX patient.

Does fat free mass index correlate better with pulmonary function than body mass index in adult CF patients before and after lung transplantation

Methods The study population consisted of CF patients who were screened for lung transplantation (mean age 29 y). FFMI, BMI and FEV1 were determined during out clinic visits. FFM was measured using single-frequency (50 kHz) bioelectrical impedance analysis. Of these patients 22 underwent lung transplantation. For this subgroup measurements performed just before and after lung transplantation were also included. Pearson correlation coefficients were calculated between FEV1% predicted and BMI and FFMI. Just before lung transplantation mean FEV1 was 21% (range 13%-41%) for men (n=14) and 24% (range 21%-30%) for women (n=8). Correlations are shown in figure B. After adjustment for sex both BMI and FFMI correlated with FEV1 (r= 0.56, p=0.008 and r= 0.51, p=0.02). After lung transplantation (>1 year, n=15) lung function increased to a mean FEV1 of 80% (range 39109) in men and 79% (range 71-98) in women. BMI and FFMI no longer correlated with FEV1. (Figure C)

Dietary treatment with probiotics in patients with cystic fibrosis

Methods We searched the Medline and Cochrane databases for controlled studies with probiotics published since 1988. If no studies were available in CF patients, we extended the search to other patient groups. We limited our search to probiotics found in Dutch diary products. Results Only 2 studies had been performed with CF patients; both with Lactobacillus GG in children. One study shows a reduction of inflammation in the intestine due to the use of Lactobacillus GG after 4 weeks (Bruzzese et al 2004). Another study suggests that the frequency of respiratory infections is reduced after 6 months using Lactobacillus GG with less clinical exacerbations (Bruzzese et al 2007). These results need confirmation. Well-established effects of probiotics studied in non-CF patients include prevention and enhanced recovery from diarrhoea due to infections or antibiotic use (especially Lactobacillus GG and Lactobacillus casei defensis) and amelioration of constipation (Bifidus ActiRegularis) (Hawrelak et al 2005). These effects should occur within 2 weeks, see Table 1.