The Role of a Nutrition Support Team in the Management of Intestinal Failure Patients

Parenteral nutrition (PN) is a complex and specialized form of nutrition support that has revolutionized the care for both pediatric and adult patients with acute and chronic intestinal failure (IF). This has led to the development of multidisciplinary teams focused on th

Standardized and Individualized Parenteral Nutrition Mixtures in a Pediatric Home Parenteral Nutrition Population

OBJECTIVES: Studies evaluating efficacy or safety of standardized parenteral nutrition (PN) versus individualized PN are lacking. We aimed to assess effects on growth and safety of standardized PN compared with individualized PN in our Home PN group. METHODS: Descriptive cohort study in Dutch children on Home PN, in which standardized PN was compared with individualized PN. Both groups received similar micronutrient-supplementation. Primary outcome was growth over 2 years, secondary outcomes were electrolyte disturbances and biochemical abnormalities. Additionally, patients were matched for age to control for potential confounding characteristics. RESULTS: Fifty patients (50% girls, median age 6.5 years) were included, 16 (32%) received standardized PN mixtures. Age (11 vs 5 years), gestational age (39.2 vs 36.2 weeks) and PN duration (97 vs 39 months) were significantly higher in the group receiving standardized PN (P: ≤0.001; 0.027; 0.013 respectively). The standardized PN group showed an increase in weight-for-age (WFA), compared with a decrease in the individualized PN group (+0.38 SD vs -0.55 SD, P: 0.003). Electrolyte disturbances and biochemical abnormalities did not differ. After matching for age, resulting in comparable groups, no significant differences were demonstrated in WFA, height-for-age, or weight-for-height SD change. CONCLUSIONS: In children with chronic IF, over 2,5 years of age, standardized PN mixtures show a comparable effect on weight, height, and weight for height when compared with individualized PN mixtures. Also, standardized PN mixtures (with added micronutrients) seem noninferior to individualized PN mixtures in terms of electrolyte disturbances and basic biochemical abnormalities. Larger studies are needed to confirm these conclusions. TRIAL REGISTRATION: Academical Medical Center medical ethics committee number W18_079 #18.103

"MY PKU": increasing self-management in patients with phenylketonuria. A randomized controlled trial

<p>Abstract</p> <p>Background</p> <p>Phenylketonuria (PKU) is an autosomal recessive disorder of phenylalanine metabolism. The inability to convert phenylalanine (Phe) into tyrosine causes Phe to accumulate in the body. Adherence to a protein restricted diet, resulting in reduced Phe levels, is essential to prevent cognitive decline. Frequent evaluation of plasma Phe levels and, if necessary, adjustment of the diet are the mainstay of treatment. We aimed to assess whether increased self-management of PKU patients and/or their parents is feasible and safe, by providing direct online access to blood Phe values without immediate professional guidance.</p> <p>Methods</p> <p>Thirty-eight patients aged ≥ 1 year participated in a 10 month randomized controlled trial. Patients were randomized into a study group (1) or a control group (2). Group 2 continued the usual procedure: a phone call or e-mail by a dietician in case of a deviant Phe value. Group 1 was given a personal "My PKU" web page with a graph of their recent and previous Phe values, online general information about the dietary treatment and the Dutch PKU follow-up guidelines, and a message-box to contact their dietician if necessary. Phe values were provided on "My PKU" without advice. Outcome measures were: differences in mean Phe value, percentage of values above the recommended range and Phe sample frequency, between a 10-month pre-study period and the study period in each group, and between the groups in both periods. Furthermore we assessed satisfaction of patients and/or parents with the 'My PKU' procedure of online availability.</p> <p>Results</p> <p>There were no significant differences in mean Phe value, percentage of values above recommended range or in frequency of blood spot sampling for Phe determination between the pre-study period and the study period in each group, nor between the 2 groups during the periods. All patients and/or parents expressed a high level of satisfaction with the new way of disease management.</p> <p>Conclusions</p> <p>Increased self-management in PKU by providing patients and/or parents their Phe values without advice is feasible and safe and is highly appreciated.</p> <p>Trial registration</p> <p>The trial was registered with The Netherlands National Trial Register (<a href="http://www.trialregister.nl/trialreg/admin/rctview.asp?TC=1171">NTR #1171</a>) before recruitment of patients.</p

An international study of the quality of life of adult patients treated with home parenteral nutrition

Background & aims: Home parenteral nutrition-quality of life (HPN-QOL©) is a self-assessment tool for the measurement of QOL in patients on HPN. The aims of this study were: to re-assess the basic psychometric properties of the HPN-QOL© in a multinational sample of adult patients; to provide a description of QOL dimensions by short and long HPN treatment duration; to explore clinical factors potentially associated to QOL scores. Methods: Patients (n = 699) from 14 countries completed the HPN-QOL©. The questionnaires were analysed to evaluate data completeness, convergent/discriminant validity and internal-consistency reliability. The association of overall QOL and HPN treatment duration as well as other clinical factors were investigated using multivariable linear regression models. Results: The analysis of the multitrait-scaling and internal consistency indicates a good fit with the questionnaire structure for most items. Item discriminant validity correlation was satisfactory and psychometric evaluation of the HPN-QOL© in the different English, French and Italian language patient sub-groups confirmed psychometric equivalence of the three questionnaire versions. The results of the multivariable linear regression showed that QOL scores were significantly associated with HPN duration (better in long-term), underlying disease (better in Crohn's disease and mesenteric ischaemia) and living status (worse in living alone) and, after adjusting for the other factors, with the number of days of HPN infusion per week. Conclusions: The HPN-QOL©, is a valid tool for measurement of QOL in patients on HPN, to be used in the clinical practice as well as in research

Intestinal failure in adults: recommendations from the ESPEN Expert Groups

Background and aims: Intestinal failure (IF) is defined as “the reduction of gut function below the minimum necessary for the absorption of macronutrients and/or water and electrolytes, such that intravenous supplementation is required to maintain health and/or growth”. Functionally, it may be classified as type I acute intestinal failure (AIF), type II prolonged AIF and type III chronic intestinal failure (CIF) The ESPEN Workshop on IF was held in Bologna, Italy, on 15-16 October 2017 and the aims of this document were to highlight the current state of the art and future directions for research in IF. Methods: This paper represents the opinion of experts in the field, based on current evidence. It is not a formal review, but encompasses the current evidence, with emphasis on epidemiology, classification, diagnosis and management. Results: IF is the rarest form of organ failure and can result from a variety of conditions that affect gastrointestinal anatomy and function adversely. Assessment, diagnosis, and short and long-term management involves a multidisciplinary team with diverse expertise in the field that aims to reduce complications, increase life expectancy and improve quality of life in patients. Conclusions: Both AIF and CIF are relatively rare conditions and most of the published work presents evidence from small, single-centre studies. Much remains to be investigated to improve the diagnosis and management of IF and future studies should rely on multidisciplinary, multicentre and multinational collaborations that gather data from large cohorts of patients. Emphasis should also be placed on partnership with patients, carers and government agencies in order to improve the quality of research that focuses on patient-centred outcomes that will help to improve both outcomes and quality of life in patients with this devastating condition

Nutrition and the circadian timing system

Life on earth has evolved under the daily rhythm of light and dark. Consequently, most creatures experience a daily rhythm in food availability. In this review, we first introduce the mammalian circadian timing system, consisting of a central clock in the suprachiasmatic nucleus (SCN) and peripheral clocks in various metabolic tissues including liver, pancreas, and intestine. We describe how peripheral clocks are synchronized by the SCN and metabolic signals. Second, we review the influence of the circadian timing system on food intake behavior, activity of the gastrointestinal system, and several aspects of glucose and lipid metabolism. Third, the circadian control of digestion and metabolism may have important implications for several aspects of food intake in humans. Therefore, we review the human literature on health aspects of meal timing, meal frequency, and breakfast consumption, and we describe the potential implications of the clock system for the timing of enteral tube feeding and parenteral nutrition. Finally, we explore the connection between type 2 diabetes and the circadian timing system. Although the past decade has provided exciting knowledge about the reciprocal relation between biological clocks and feeding/energy metabolism, future research is necessary to further elucidate this fascinating relationship in order to improve human healt

Evaluation of 6 years use of sodium hydroxide solution to clear partially occluded central venous catheters

BACKGROUND & AIMS: Central venous catheter occlusion is a frequently occurring complication during home parenteral nutrition (HPN). The aim of the study was to investigate the effectiveness of sodium hydroxide (NaOH) administration to clear an occluded central venous catheter especially in HPN. METHOD: Retrospective study to the use of NaOH in partially occluded central venous catheters. About 45 patients with HPN treated in the Academic Medical Center of the University of Amsterdam (AMC) were included in this study between January 1997 and April 2003. Excluded from the study were patients under the age of 18 at the start of HPN and/or patients who use parenteral nutrition for less than 3 months. Partial catheter occlusion was defined as a spontaneous flow less than 60 drops/min. RESULTS: In total, 130 occlusions were registered in 29 HPN patients. The other 16 HPN patients did not report any occlusion. The incidence of occlusions in fat containing total parenteral nutrition (TPN) was 1 occlusion in 167 feeding days. TPN without fat showed only one occlusion (incidence 1 in 7126 feeding days). The use of a lipid emulsion proved an important risk factor for catheter occlusion in this study (P <0.05, RR=43). Ninety-five central venous catheter occlusions were treated with NaOH 0.1M. The remaining occlusions were total or mechanical occlusions making NaOH treatment impossible. In 73 out of 95 partial occlusions treatment with NaOH was effective (P <0.05). Using NaOH extended the use of 32 catheters (range 7-1592 days, mean 328). Twenty-one out of 32 catheters could be used for more than 3 months after using NaOH (P <0.05). CONCLUSION: We conclude that perfusion of a partial occluded central venous catheter (defined as 25-60 drops/min) used for parenteral nutrition with 0.1N NaOH is safe and shows a significant long term improvement in catheter care, by preventing total occlusion and operative remova

Results of an International Survey on Feeding Management in Infants With Short Bowel Syndrome-Associated Intestinal Failure

Objectives: Short bowel syndrome (SBS) is a complex and rare condition (incidence 1200/100,000 live births) that requires a multidisciplinary team approach to management. In January 2019, the first European Reference Network on Rare and Inherited Congenital Anomalies (ERNICA) Intestinal Failure (IF) workshop was held. Several questions about the strategies used in managing IF associated with SBS were devised. The aim of our study was to collect data on the enteral feeding strategies adopted by the ERNICA centres. Methods: A questionnaire (36 questions) about strategies used to introduce enteral nutrition post-operatively and start complementary food/solids in infants with SBS associated IF was developed and sent to 24 centres in 15 countries that participated in the ERNICA-IF workshop. The answers were collated and compared with the literature. Results: There was a 100% response rate. In infants, enteral nutrition was introduced as soon as possible, ideally within 24–48 hours post-small intestinal surgical resection. In 10 of 24 centres, bolus feeding was used, in nine continuous, and in five a combination of both. Twenty-three centres used mothers’ own milk as the first choice of feed with extensively hydrolysed feed, amino acid-based feed, donor human milk or standard preterm/term formula as the second choice. Although 22 centres introduced complementary/solid food by 6 months of age, food choice varied greatly between centres and appeared to be culturally based. Conclusions: There is diversity in post-surgical enteral feeding strategies among centres in Europe. Further multi-centre studies could help to increase evidence-based medicine and management on this topic