Self‐Evaluation of Quality of Life Among Patients Receiving Home Parenteral Nutrition: A Validation Study

Background : Home parenteral nutrition (HPN) can increase survival and quality of life (QOL) in cases of chronic intestinal failure. The present study compares a gold standard—a validated QOL assessment by Baxter et al (hereafter, HPN‐QOL)—with a shorter questionnaire for QOL evaluation (hereafter, New QOL). Materials and Methods : Both questionnaires were completed by 58 patients attending the nutrition clinic of the Rabin Medical Center (Israel). A z score was used to equate means and variances. Statistical analysis was based on Pearson correlation between variances. Stepwise regression was used to evaluate the best value predictor in the New QOL for QOL during HPN. Cronbach’s α was used for internal consistency, and confirmatory factor analysis was used for structural validity. Results : Results from the sections of the New QOL that relate to the patient’s general health, level of independence, and experience with handling the parenteral nutrition therapy are highly correlated with the HPN‐QOL (r = 0.43 and r = 0.50, respectively). In addition, there was good correlation between the questionnaires with reference to the patient’s social state, mental state, and everyday functioning (r = ?0.43, New QOL; r = ?0.39, HPN‐QOL). However, the New QOL results show no correlation between the patient’s physical symptoms and QOL during HPN. This was hypothesized to result from the nature of the study population. Conclusion : The results of our new questionnaire are well correlated with the gold standard HPN‐QOL. The New QOL appears to be simple and easy to use.     

Management of Recurrent Catheter‐Related Bloodstream Infections in an Adult Patient Receiving Home Parenteral Nutrition: Dramatic Effect of Ethanol Lock Therapy

We report the use of ethanol lock therapy to dramatically reduce the incidence of catheter‐related bloodstream infections (CRBSIs) in a long‐term adult home parenteral nutrition (HPN) patient. This case study demonstrates the efficacy of ethanol lock therapy in eliminating CRBSIs when other treatments have been unsuccessful. We suggest that ethanol lock therapy has an important role in decreasing CRBSI in HPN patients with recurrent CRBSIs.     

Clinical Application of Magnetic Resonance Spectroscopy of the Liver in Patients Receiving Long‐Term Parenteral Nutrition

Background: Intestinal failure–associated liver disease (IFALD) may have progressed to an advanced stage by the time it becomes evident via laboratory or physical signs. A safe, noninvasive technique for assessing the liver could significantly aid in monitoring the effects of therapeutic intervention, improve the timing of liver and small intestinal transplantation, and increase our understanding of the causes of IFALD. Methods: Six female patients fed intravenously for >1 year and 6 controls matched for body mass index (BMI) underwent liver magnetic resonance scanning with acquisition of ¹H and ³¹P resonance spectra. Areas under the curve for lipid (the sum of CH, CH₂, and CH₃), water, and choline peaks were calculated and expressed semi‐quantitatively as ratios of lipid:water and choline:lipid. Phosphomonoester (PME) and phosphodiester (PDE) peak areas were similarly expressed as a ratio. Controls and cases were compared using Mann‐Whitney U test; least squares regression analysis was used to compare the effect of measured variables on the lipid:water peak area ratio. Results: Patients and controls were well matched for BMI. Parenteral feeding was associated with a highly significant increase in lipid:water peak ratio (P < .005). Choline:lipid (P < .05) and choline:water (not significant) ratios were reduced in patients compared with controls. The increase in lipid:water ratios in patients was independent of BMI and choline:water ratios. A ratio of PME:PDE of >0.3 (and >3 SD from the control mean) predicted the 2 patients at most risk of advanced liver disease. Conclusions: This pilot study confirms the potential of magnetic resonance spectroscopic techniques in evaluating IFALD and could contribute significantly to our understanding and management of this condition.     

Laboratory Monitoring of Children on Home Parenteral Nutrition: A Prospective Study

Background: The primary hypothesis of this article is that a team approach in creating a protocolized laboratory monitoring schedule for home parenteral nutrition (PN) patients improves patient safety by decreasing the occurrence of nutrition deficiencies and is cost‐effective. Methods: In this prospective cohort study of home PN patients, each patient followed an established protocol of laboratory monitoring and weekly review by an interdisciplinary team of dietitians, nurses, and physicians. Data collected included anthropometric measurements, laboratory results, deviations from laboratory protocols, laboratory charges, PN shortage information, and means of ameliorating such shortages. Cost‐effectiveness analysis was only performed for nonmicronutrient laboratory tests. Results: Fifteen children (male, n = 6) with a median age of 59 months (range, 19–216) were included in this study. Primary diagnoses included short bowel syndrome (47%) and intestinal pseudo‐obstruction (40%). Patients received PN mixtures from 6 different infusion companies and experienced 60 different shortages in the PN formulation requiring adjustments or substitutions (mean, 4 shortages per patient). All patients had appropriate growth and complete micronutrient monitoring. No patient experienced any clinical symptoms due to shortages. The median number of laboratory draws/patient per month was 2.9 preprotocol compared with 1.14 postprotocol (P = .003). The median per patient per month charges were $2014 (interquartile range [IQR], 1471–2780) preprotocol compared with $792 (IQR, 435–1140) postprotocol (P = .002). Conclusions: A structured team approach to laboratory monitoring of home PN patients can simplify PN management, significantly decrease monthly laboratory costs, and lead to fewer laboratory draws while improving micronutrient monitoring and preventing deficiencies.     

A Rare Case of Hyperammonemia Complication of High‐Protein Parenteral Nutrition

Hyperammonemia is a metabolic derangement that can be potentially fatal. Primary hyperammonemia due to urea cycle enzyme deficiency is usually discovered in neonates but rarely can present in adulthood. Late‐onset manifestations of urea cycle disorders can go unnoticed, until they become life threatening. The authors report a 28‐year‐old man who developed hyperammonemia in the hospital following parenteral nutrition (PN), leading to cerebral edema, which was fatal despite resolution of the hyperammonemia with cessation of PN and the use of continuous renal replacement therapy.     

Plasma and Red Blood Cell PUFAs in Home Parenteral Nutrition Paediatric Patients—Effects of Lipid Emulsions

Background: Mixed lipid emulsions (LE) containing fish oil present several advantages compared to the sole soybean oil LE, but little is known about the safety of essential fatty acids (EFA) profile in paediatric patients on long-term Parenteral Nutrition (PN). Aim of the study: to assess glycerophosfolipid polyunsaturated fatty acids (PUFA) levels on plasma and red blood cell (RBC) membrane of children on long term PN with composite LE containing fish oil (SMOF), and to compare it with a group receiving olive oil LE (Clinoleic^®) and to the reference range for age, previously determined on a group of healthy children. Results: A total of 38 patients were enrolled, median age 5.56 (0.9–21.86) years, 15 receiving Clinoleic^®, 23 receiving SMOF. Patients on SMOF showed significantly higher levels of eicosapentaenoic acid (EPA) and docosahexaenoic acid (DHA), lower levels of arachidonic acid (ARA) and Mead acid (MEAD)/ARA ratio in plasma and RBC compared with patients on Clinoleic^® and with healthy children. Triene:tetraene (T:T) ratio of both groups of patients did not differ from that of healthy children-median plasma (MEAD/ARA: 0.01, interquartile rage (IQR) 0.01, p = 0.61 and 0.02, IQR 0.02, p = 0.6 in SMOF and Clinoleic^® patients, respectively), and was considerably lower than Holman index (>0.21). SMOF patients showed no statistically significant differences in growth parameters compared with Clinoleic^® patients. Patients of both groups showed stiffness class F0-F1 of liver stiffness measure (LSM) 5.6 (IQR 0.85) in SMOF patients and 5.3 (IQR 0.90) in Clinoleic^® patients, p = 0.58), indicating absence of liver fibrosis. Conclusions: Fatty acids, measured as concentrations (mg/L), revealed specific PUFA profile of PN patients and could be an accurate method to evaluate nutritional status and eventually to detect essential fatty acid deficiency (EFAD). SMOF patients showed significantly higher EPA, DHA and lower ARA concentrations compared to Clinoleic^® patients. Both LEs showed similar hepatic evolution and growth.     

Effect of Hyperalimentation and Insulin‐Treated Hyperglycemia on Tyrosine Levels in Very Preterm Infants Receiving Parenteral Nutrition

Background: Hyperalimentation describes the increase in glucose, amino acids (AAs), and lipid intake designed to overcome postnatal growth failure in preterm infants. Preterm infants are dependent on phenylalanine metabolism to maintain tyrosine levels because of tyrosine concentration limits in parenteral nutrition (PN). We hypothesized that hyperalimentation would increase individual AA levels when compared with the control group but avoid high phenylalanine/tyrosine levels. Aim: To compare the plasma AA profiles on days 8–10 of life in preterm infants receiving a hyperalimentation vs a control regimen. Methods: Infants <29 weeks’ gestation were randomized to receive hyperalimentation (30% more PN macronutrients) or a control regimen. Data were collected to measure macronutrient (including protein) intake and PN intolerance, including hyperglycemia, insulin use, urea, and AA profile. Plasma profiles of 23 individual AA levels were measured on days 8–10 using ion exchange chromatography. Results: One hundred forty‐two infants were randomized with 118 AA profiles obtained on days 8–10. There were no differences in birth weight or gestation between groups. There was an increase (P < .05) in 8 of 23 median individual plasma AA levels when comparing hyperalimentation (n = 57) with controls (n = 61). Only tyrosine levels (median; interquartile range) were lower with hyperalimentation: 27 (15–52) µmol/L vs 43 (24–69) µmol/L (P < .01). Hyperalimentation resulted in more insulin‐treated hyperglycemia. No difference between the groups was apparent in tyrosine levels when substratified for insulin‐treated hyperglycemia. All insulin vs no insulin comparisons showed lower tyrosine levels with insulin treatment (P < .01). Conclusion: Hyperalimentation can result in paradoxically low plasma tyrosine levels associated with an increase in insulin‐treated hyperglycemia.     

Prevention of Subsequent Catheter‐Related Bloodstream Infection Using Catheter Locks in High‐Risk Patients Receiving Home Parenteral Nutrition

Introduction:Catheter‐related bloodstream infection (CRBSI) is a serious complication in patients receiving home parenteral nutrition (HPN). Antibiotic lock therapy (ALT) and ethanol lock therapy (ELT) can be used to prevent CRBSI episodes in high‐risk patients. Methods: Following institutional review board approval, all patients enrolled in the Mayo Clinic HPN program from January 1, 2006, to December 31, 2013, with catheter locking were eligible to be included. Patients without research authorization and <18 years old at the initiation of HPN were excluded. Total number of infections before and after ALT or ELT were estimated in all patients. Results: A total of 63 patients were enrolled during the study period. Of 59 eligible patients, 29 (49%) were female, and 30 (51%) were male. The median duration of HPN was 3.66 (interquartile range, 0.75–8.19) years. The mean age ± SD at initiation of HPN was 49.89 ± 14.07 years. A total of 51 patients were instilled with ALT, and 8 patients were instilled with ELT during their course of HPN. A total of 313 CRBSI episodes occurred in these patients, 264 before locking and 49 after locking (P < .001). Rate of infection per 1000 catheter days was 10.97 ± 25.92 before locking and 1.09 ± 2.53 after locking (P < .001). Discussion: The major findings of the present study reveal that ALT or ELT can reduce the overall rate of infections per 1000 catheter days. ALT or ELT can be used in appropriate clinical setting for patients receiving HPN.     

Noninvasive Quantitative Assessment of Hepatic Steatosis by Proton Magnetic Resonance Spectroscopy Among Adult Patients Receiving Home Parenteral Nutrition

Background: Intestinal failure–associated liver disease is a frequent complication in patients with chronic intestinal failure (CIF), with steatosis as a dominant feature in adults. Proton magnetic resonance spectroscopy (1H‐MRS) is a noninvasive method to quantify liver fat content (LFC). In this study, LFC was assessed with 1H‐MRS, taking into account the possible accumulation of paramagnetic components of home parenteral nutrition (HPN) that may disturb these measurements. Methods: LFC was measured in 15 adult CIF patients who had been receiving HPN for >6 months. 1H‐MR spectra were obtained with a 3 Tesla magnetic resonance (MR) system, with a method correcting for the presence of paramagnetic ions. Patients with low (<5%) versus high (≥5%, steatosis) LFC were compared with nonparametric statistical tests. Results: 1H‐MRS analysis revealed steatosis in 5 patients (median, 10.3%), while 10 patients had normal LFC (median, 0.9%). In all patients, the 1H‐MRS results indicated the presence of various amounts of paramagnetic constituents in the liver. Patients with steatosis had higher alanine aminotransferase values than patients without steatosis (median, 60 vs 28 U/L). Unexpectedly, in the steatosis group, the frequency of HPN use was lower, with significant lower total HPN and carbohydrate calories. In 1 patient, MR spectra were of inferior quality, with broadened resonances after infusion with a ferric compound. Conclusion: 1H‐MRS enables reliable noninvasive assessment of LFC in patients receiving long‐term HPN, if correcting for possible accumulation of paramagnetic components in the liver. However, LFC determination by 1H‐MRS is not recommended after a recent ferric compound infusion.     

A Qualitative Study of the Perceived Value of Membership in The Oley Foundation by Home Parenteral and Enteral Nutrition Consumers

Background: Support and educational organizations have been shown to improve quality of life of consumers of home nutrition support. One such organization, The Oley Foundation, offers resources for the home parenteral and enteral nutrition (HPEN) consumer. While research has shown proven benefits to HPEN consumers affiliated with The Oley Foundation, no studies have investigated the perceived value of membership to the consumer or the way in which consumers are introduced to the organization. Methods: Qualitative methodology was used to gain a deeper understanding of the perceived value of membership in The Oley Foundation. Audiotaped, in‐depth, semistructured telephone interviews were conducted to explore participants' experiences with The Oley Foundation and HPEN. Inductive content analysis was used to analyze data and identify themes associated with membership value. Results: The value of The Oley Foundation lies in programs and resources and the competency, inspiration, normalcy, and advocacy gained from membership, helping individuals adjust to life with HPEN dependency. More than half of participants found the organization through self‐initiated Internet searches, but all participants clearly expressed the desire “I wish I knew about it sooner.” Conclusion: This study identifies the value of membership in The Oley Foundation and the important role the organization has in the lives of HPEN‐dependent consumers. Nutrition support clinicians should introduce the organization to patients when the need for HPEN is established and prior to hospital discharge.     

Risk Factors for the Development of Catheter‐Related Bloodstream Infections in Patients Receiving Home Parenteral Nutrition

Background: Risk factors for development of catheter‐related bloodstream infections (CRBSI) were studied in 125 adults and 18 children who received home parenteral nutrition (HPN). Methods: Medical records from a national home care pharmacy were reviewed for all patients that had HPN infused at least twice weekly for a minimum of two years from January 1, 2006‐December 31, 2011. Infection and risk factor data were collected during this time period on all patients although those patients who received HPN for a longer period had data collected since initiation of HPN. Results: In adults, 331 central venous catheters (CVCs) were placed. Total catheter years were 1157. Median CVC dwell time was 730 days. In children, there were 53 CVCs placed. Total catheter years were 113.1. Median CVC dwell time was 515 days. There were 147 CRBSIs (0.13/catheter year;0.35/1000 catheter days). In children there were 33 CRBSIs (0.29/catheter year;0.80/1000 days; P < .001 versus adults). In adults, univariate analysis showed use of subcutaneous infusion ports instead of tunneled catheters (P = .001), multiple lumen catheters (P = .001), increased frequency of lipid emulsion infusion (P = .001), obtaining blood from the CVC (P < 0.001), and infusion of non‐PN medications via the CVC (P < .001) were significant risk factors for CRBSI. Increased PN frequency was associated with increased risk of CRBSI (P = .001) in children, but not in adults. Catheter disinfection with povidone‐iodine was more effective than isopropyl alcohol alone. There were insufficient patients to evaluate chlorhexidine‐containing regimens. Conclusion: Numerous risk factors for CRBSI were identified for which simple and current countermeasures already exist.     

Effects of ω‐3 Fish Oil Lipid Emulsion Combined With Parenteral Nutrition on Patients Undergoing Liver Transplantation

Background: The effect of parenteral nutrition (PN) support supplemented with ω‐3 fatty acids was investigated in a randomized, controlled clinical trial at the Affiliated Drum Tower Hospital, Medical School of Nanjing University. Materials and Methods: Ninety‐eight patients with the diagnosis of end‐stage liver disease or hepatic cellular carcinoma were admitted for orthotopic liver transplantation at the Affiliated Drum Tower Hospital. The patients were randomly divided into 3 groups: diet group (n = 32), PN group (n = 33), and polyunsaturated fatty acid (PUFA) group (n = 33). Patients in the PN and PUFA groups received isocaloric and isonitrogenous PN for 7 days after surgery. Venous heparin blood samples were obtained for assay on days 2 and 9 after surgery. A pathological test was performed after reperfusion of the donor liver and on day 9. Results: Alanine aminotransferase levels were improved significantly by PUFA treatment compared with traditional PN support (P < .05). Compared with the results on day 9 in the PN group, a significant difference was seen in the extent of increase of the prognostic nutrition index and prealbumin in the PUFA group. The pathological results also showed that ω‐3 fatty acid supplementation reduced hepatic cell injury. PUFA therapy also decreased the incidence of infectious morbidities and shortened the posttransplant hospital stay significantly. Conclusion: Posttransplant PN support can greatly improve metabolism of protein and nutrition states of patients. ω‐3 fatty acid–supplemented PN significantly reduces injury of the transplanted liver, decreases the incidence of infectious morbidities, and shortens posttransplant hospital stay.     

Epidemiology of Infectious and Noninfectious Catheter Complications in Patients Receiving Home Parenteral Nutrition: A Systematic Review and Meta‐Analysis

Patients receiving parenteral nutrition (PN) as their primary source of nutrition are at high risk for both infectious and noninfectious catheter complications (catheter‐related infections, catheter occlusion, and venous thrombosis). The aim of this review was to synthesize and evaluate what is known about catheter complications and prevention strategies in the PN population. Three electronic databases (Medline, Embase, and CINAHL) were screened for studies published between January 2012 and February 2019 regarding infectious and noninfectious catheter complications in patients receiving PN. Rates of infectious and noninfectious catheter complications, prevalence of causative pathogens, potential risk factors, and prevention strategies via the use of antimicrobial lock therapy (ALT) were assessed. Fifty‐three catheter complication studies and 12 ALT studies were included. Studies were grouped by definition of complication: catheter‐related bloodstream infections (CRBSI) or central line–associated bloodstream infections (CLABSI). Random effects summary rates per 1000 catheter days were 0.85 CRBSI episodes (95% CI 0.27–2.64) and 1.65 CLABSI episodes (95% CI 1.09–2.48). Use of taurolidine or ethanol ALT was efficacious in reducing infectious catheter complications; however, several studies had concerns for adverse mechanical complications. Potential risk factors for catheter complications were highly varied and often contradictory between studies. The rates of catheter complications were higher among catheterized patients receiving PN compared with nationally reported rates of complications in all catheterized patients. Risk factors for catheter complications need to be better understood for targeted prophylactic use of ALT. Future studies are warranted; however, they should be conducted using more standardized definitions and criteria.     

Strong Variability of Di(2‐ethylhexyl)phthalate (DEHP) Plasmatic Rate in Infants and Children Undergoing 12‐Hour Cyclic Parenteral Nutrition

Background: Medical devices such as perfusion materials in polyvinyl chloride may contain di(2‐ethylhexyl)phthalate (DEHP). Several studies have questioned the harmlessness of phthalates, which have been shown to have toxic effects on the reproductive system and general development. This study was designed to assess DEHP exposure in infants and children benefitting from cyclic parenteral nutrition (PN). The results are compared with those obtained from children used as controls and receiving no PN, to estimate the potential risk to this pediatric population, taking into account exposure levels and already published data. Methods: Plasmatic concentrations of DEHP were assessed by high‐performance liquid chromatography from blood samples taken from 22 children at the start and finish of a 12‐hour cyclic PN period and compared with those obtained from 20 control children of comparable age and gender. Results: After a 12‐hour cyclic PN period, DEHP migration varied widely among the patients. The concentrations were not quantifiable in 4 children at the start of PN. In 1 child, they were quantifiable neither at the start nor at the end of PN. However, for 17 children, DEHP concentrations were quantifiable at the start of PN and were very variable from one child to another. At the end, DEHP concentrations had significantly but variably increased in these children. No trace of DEHP was found in the blood samples from 20 healthy controls. Conclusion: Considering published data on phthalate toxicity, it would appear advisable to encourage the use of medical devices that are either phthalate or DEHP free.