Quality of life in adult home parenteral nutrition patients

BACKGROUND: Home parenteral nutrition (HPN) is life-sustaining therapy in some disease states. Patients, however, report alterations in physical, psychologic, and social function that negatively affect perceived quality of life (QOL). Many generic tools have been used to evaluate QOL during HPN, but there is no gold standard measurement. QOL improvement can only result from identifying and addressing patient-specific problems. The purpose of this study was to identify the tools used by others to measure QOL in adults receiving long-term HPN and to identify factors that affect QOL in this population. METHODS: An electronic search of CINAHL, MEDLINE, and Health and Psychosocial Instruments databases was conducted to identify studies of HPN and QOL in adults. RESULTS: Thirty-four publications on HPN and QOL were identified. Twenty-four studies of HPN and QOL were included in this review; 10 papers were review articles or editorials. QOL was worse in HPN patients compared with healthy populations. Impaired QOL was associated with decreased physical, psychologic, and social function. Depression, drug dependency, sleep disturbance, frequent urination, fear of therapy-related complications, and inability to eat negatively affected QOL. CONCLUSIONS: Use of different QOL instruments, scales, and lifestyle domains limited comparison among studies. QOL is poor in patients receiving HPN and worse in the presence of depression and narcotic dependency. The technical aspects of HPN administration interfere with routine activities. It is difficult to determine whether HPN itself or the impact of the disease affected QOL. Development of an HPN-specific QOL tool may help in this differentiation.     

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.     

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.     

Sleep patterns of cyclic parenteral nutrition,a pilot study: are there sleepless nights?

BACKGROUND: Cyclic home parenteral nutrition (HPN) is 1 of the few medical therapies given during normal nocturnal sleep hours, and it is possible that infusion may alter normal sleep patterns. The aim of this pilot study was to evaluate the sleep patterns of 5 patients receiving HPN. METHODS: An Epworth sleep questionnaire was completed and wrist Actigraph data were collected before admission to the sleep laboratory. Formal overnight polysomnography was then preformed for 3 consecutive nights. The first night served as the acclimatization period. On the second and third nights, patients were randomized to receive either no infusion or infusion of their standard parenteral nutrition. Results were reported as the median and range and were compared with historical aged-matched controls. RESULTS: Five patients (3 women and 2 men) with a mean age of 61 years (40 to 73 years) were studied. Patients had been HPN-dependent for a median of 23 months (4 to 60 months). Patients were receiving HPN because of short bowel syndrome (2), chronic pancreatitis (2), and intestinal pseudoobstruction (1). A 1.5-liter HPN formula, infused over 10 hours, included approximately 25 kcal/kg/d with 30% lipid and 1.0 to 1.5 g/kg/d of protein. All solutions included multiple trace elements and standard multivitamins. During total parenteral nutrition (TPN) infusion, the percent of sleep efficiency was higher than without infusion, 81% versus 72%. Sleep efficiency in age-matched controls was approximately 88%. Sleep latency was longer in patients compared with controls, and longer in patients during infusion than without infusion, 35 versus 28 minutes. During TPN infusion, the percent of stage-1 (2%), stage-2 (52%), slow-wave (24%) and random eye movement (REM) sleep (21%) was similar to values during the night without infusion. Controls had lower slow-wave and REM times. The median Epworth sleep score was 3, which is the normal reported range. CONCLUSIONS: Although sleep quality is reduced in patients receiving HPN compared with aged-matched controls, sleep quality does not seem to be negatively effected by cyclic HPN infusion.     

Survival of Patients with Multi-Level Malignant Bowel Obstruction on Total Parenteral Nutrition at Home

Home parenteral nutrition (HPN) may improve the survival in selected patients with malignant bowel obstruction. This retrospective, medical registry-based study aimed to identify clinical and laboratory markers predicting short survival, which would allow a more accurate selection of patients that would benefit from HPN in inoperative bowel obstruction. In a retrospective analysis of 114 patients receiving HPN, the median survival was 89 days after discharge home, and the three and six-month survival probability was 48% and 26%, respectively. Parenteral nutrition was provided during 98% of overall survival time and ended on a median of one day before the patient’s death. Discontinuing chemotherapy, anemia, severe hypoalbuminemia, and water retention appeared correlated with survival shorter than three months. In these cases, routine initiation of HPN should be discouraged, as it may not bring any benefits to the patient. The decision on the initiation of HPN should be made along with continuing or initiating chemotherapy.     

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.     

Vitamin D deficiency in patients receiving home parenteral nutrition

Background: In addition to its role in bone metabolism, vitamin D has important immunomodulatory and antineoplastic effects. Patients on home parenteral nutrition (HPN) receive most of their vitamin D from intravenous (IV) supplementation. Vitamin D deficiency is common in the general population, and the adequacy of vitamin D supplementation in HPN patients is unclear. The purpose of this study is to determine the vitamin D status of patients on HPN. Methods: Consecutive patients seen in a regional home nutrition program had their oral and IV vitamin D intakes determined. Plasma 25‐hydroxyvitamin D levels were measured in all patients. Intake of calcium, magnesium, and phosphate were also determined. Results: The mean 25‐hydroxyvitamin D level in 22 patients receiving HPN for a mean of 33.5 months (range, 1–177) was 42 nmol/L. Vitamin D deficiency was present in 15 (68%) patients and vitamin D insufficiency in 6 (27%) patients. The mean dietary vitamin D intake was 79.5 IU per day, while the mean IV supplementation was 166 IU per day. Conclusions: In this study of a regional Canadian HPN program, there was a high prevalence of vitamin D deficiency/insufficiency affecting virtually all patients. All patients receiving HPN should be supplemented with vitamin D and have their 25‐hydroxyvitamin D levels monitored. Further studies are required to determine optimal methods and dosing of vitamin D replacement using oral supplements or ultraviolet light therapy.     

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.     

Effects of home parenteral nutrition on resting metabolic rate: a case study.

Resting metabolic rate (RMR) measurement by indirect calorimetry is often used in acute care; however, indirect calorimetry is rarely performed on patients treated at home with complex therapies such as home parenteral nutrition (HPN). A new hand-held indirect calorimeter is now available to measure RMR and oxygen consumption (VO2). This is a case study of a 35-year-old female who had her RMR and VO2 measured at rest while receiving cyclic HPN and while off of HPN over a period of 8 days. Results indicate that her RMR was higher while receiving HPN. Measuring RMR in patients receiving HPN may be very beneficial to avoid underfeeding and overfeeding. It will be important for the clinician to note if the patient is receiving HPN at the time of the measurement because RMR may be affected by HPN infusion.     

Development of Sustain™: A.S.P.E.N.'s National Patient Registry for Nutrition Care

The American Society for Parenteral and Enteral Nutrition (A.S.P.E.N.) developed a Web-based registry for nutrition care launched in early 2011, initially focusing on the home parenteral nutrition (HPN) patient population. The purpose of Sustain is to collect information regarding the patients and populations who require HPN in the United States, measure outcomes associated with HPN, allow institutional benchmarking against the aggregate data, and publish the findings to improve the quality of care for patients receiving HPN. The registry is open to all sites (hospital and home based) who care for new or existing HPN patients. Preliminary data should be available in early 2012.     

Transient Elastography (FibroScan) Is Not Correlated With Liver Fibrosis but With Cholestasis in Patients With Long‐Term Home Parenteral Nutrition

Background: Long‐term home parenteral nutrition (HPN) may induce liver disorders. Transient elastography (TE) has been proposed as a noninvasive alternative to liver biopsy analysis for assessment of the progression of hepatic fibrosis to cirrhosis. The goal of this study was to compare values from TE measurements to biopsy‐determined stages of histologic fibrosis in patients receiving HPN. Methods: In this multicenter prospective study, patients receiving long‐term HPN (≥6 months) who required a liver biopsy for clinical reasons were included. TE (FibroScan) values for each patient were compared with the degree of hepatic fibrosis measured from biopsy specimens based on the Brunt classification. TE values were also correlated to biochemical and histologic cholestasis. Two noninvasive indices for predicting liver fibrosis (APRI and FIB‐4) were also evaluated. Results: Thirty patients were included in this study (mean age, 42.1 years; 63% male). The mean duration of HPN was 100.7 months; 25 patients had a short bowel and 13 had an intestinal stoma. Biochemical cholestasis was described in 22 patients. Liver histologic features varied among these patients. There was no correlation between the values of TE and the stages of histologic fibrosis, but TE values were significantly correlated to serum bilirubin level and the severity of histologic cholestasis as well as APRI and FIB‐4 scores. Conclusions: In patients with long‐term HPN, TE failed to assess the degree of hepatic fibrosis. This could be due to the heterogeneity of liver histologic features observed in these patients and the presence of chronic cholestasis.     

Plasma vitamin and mineral status in home parenteral nutrition patients

Home parenteral nutrition (HPN) provides long-term nutritional support for persons whose absorptive capacity is compromised by a variety of intestinal malabsorption problems. However, the presence of vitamin and mineral deficiency syndromes that normally would not have time to develop in the hospitalized patient receiving total parenteral nutrition has been reported in patients receiving HPN. This study entails a longitudinal survey of plasma concentrations of vitamins A, E, and 1,25-dihydroxyvitamin D, as well as the minerals zinc, copper, and selenium, in patients receiving HPN. Plasma samples from eight patients who had been on HPN for 1-92 months before the study began were obtained once a month over a 12-month period. The blood was drawn immediately before their evening infusion of TPN in order to approximate fasting plasma nutrient concentrations. Patient values were compared to fasting control values and to published norms. Values for vitamin A, 1,25-dihydroxyvitamin D, and zinc all were within the normal range, and there was no evidence of metabolic bone disease. Plasma vitamin E and copper concentrations exceeded the normal range for most of the 12-month period. Of all of the nutrients studied, only plasma selenium concentrations were consistently in the low-normal to below-normal range. Selenium levels in patients on HPN should be monitored regularly, and supplementation may be necessary if clinical conditions warrant.     

Iron deficiency anemia in patients receiving home total parenteral nutrition

BACKGROUND: Certain patients receiving home total parenteral nutrition (HPN) are likely to develop iron-deficiency anemia because of inadequate absorption or chronic iron loss from gastrointestinal lesions. The objective of this study was to examine the incidence and prevalence of iron deficiency anemia in patients on long-term HPN (>6 months) and to investigate both the efficacy of and rate of adverse reactions to parenteral iron dextran therapy. METHODS: The records of 55 patients treated with HPN for >6 months between January 1, 1994 and December 31, 1999 were examined. RESULTS: Thirty patients (55%) had evidence of iron-deficiency anemia. Ten patients were diagnosed at the initiation of HPN, and in 20 patients, iron deficiency developed after receiving HPN. The time between initiation of HPN and development of anemia ranged from 2 to 97 months (mean 28.8+/-26.2 months). Mild iron loss from the gastrointestinal tract seemed to be the predominant reason for iron deficiency. Regular treatment with small amounts of iron in HPN appeared to be safe and efficacious, with no reported side effects. Total dose infusion of iron was associated with adverse reactions in as many as 25% of these patients, although all reactions were mild and self-limited. CONCLUSIONS: Iron-deficiency anemia is common in patients receiving chronic HPN. Regular small doses of iron in HPN formula, rather than total dose infusion, is the preferred treatment.     

肠外营养输液途径合理性和适应证分析--3979例患者回顾性调查

目的 探讨肠外营养（PN）输液途径的合理性及适应证。方法 回顾性分析3979例患者的营养状况、科室分布、PN使用时间、输液途径等，对其输液途径合理性、适应证等进行分析。结果 使用PN患者主要为普通外科、胸心外科等13个科室，外科系统占93．2％，内科系统占6．8％，其中胃肠外科接受PN治疗的患者营养不良发生率为14．7％。PN治疗时间大多数≤7天，没有严格的掌握适应证。中心静脉插管占89％，外周静脉插管占11％。在中心静脉置管方式上以锁骨下静脉为主。结论 多数应用中心静脉营养没有严格掌握适应证，营养处方没考虑到患者个体差异，中心静脉插管以锁骨下静脉为主。应加强全面临床营养知识培训，推广简单的营养不良及营养不良风险的评估方法，提高对PN治疗的适应证认识，采用推荐的经周围静脉中心静脉插管途径。     

Psychosocial complaints are associated with venous access-device related complications in patients on home parenteral nutrition

Background: Complications related to venous access devices (VADs) remain the major drawback of home parenteral nutrition (HPN) support. In addition to technical issues, patients also experience psychosocial problems. The aim of this study is to present an overview of VAD‐related complications in patients on long‐term HPN and to assess whether these adversities are related to experienced psychosocial problems and quality of life (QOL). Methods: Information on VAD‐related complications was collected from the medical charts of 110 adult HPN patients who were followed by the 2 major referral centers in the Netherlands. In addition, a survey was conducted in this group to characterize psychosocial problems and assess their association with technique‐related complications; 75 patients (68%) responded. Results: At the time of survey, the majority of patients (76%) had developed 1 or more episodes of catheter‐related sepsis at some point during their HPN treatment. The overall incidence of VAD‐related blood stream infections (BSIs) was 3 per 1,000 venous access days. The incidence of VAD occlusions was 0.8 per 1,000 venous access days. During the observation period, there was a highly significant association between the incidence of VAD‐related complications and the occurrence of psychosocial complaints (eg, depression, fatigue, social impairment, and decreased QOL). Conclusions: Psychosocial complaints are associated with previously experienced VAD‐related complications in patients on HPN therapy. Although only an association, and not a causal relationship is demonstrated by these findings, our results underscore the need for preventive and therapeutic measures regarding both types of problems in these patients.     

Characterization of posthospital bloodstream infections in children requiring home parenteral nutrition

Background: Home parenteral nutrition (HPN) is lifesaving for children with intestinal failure. Catheter‐associated bloodstream infections (CA‐BSI) are common in hospitalized patients receiving parenteral nutrition (PN), but data evaluating CA‐BSI in children receiving HPN are limited. Objective: To determine the incidence and characteristics of CA‐BSI in children receiving HPN. Methods: Medical records of 44 children receiving HPN during a 3‐year period were reviewed. End points were CA‐BSI during the initial 6 months after discharge. CA‐BSI was defined as isolation of pathogens from blood requiring antimicrobial therapy. Results: The primary indication for HPN was short bowel syndrome (46%), and 59 BSI were documented during the initial 6 months of HPN in 29 (66%) children. Of CA‐BSI, polymicrobial infections accounted for 52%; gram‐positive, 29%; gram‐negative, 17%; and fungal, 2%. CA‐BSI incidence per 1000 catheter‐days was highest during the first month posthospital discharge (72 episodes; 95% confidence interval [CI], 45.4–109.6). CA‐BSI incidence density ratio for children receiving HPN for >90 days compared with those receiving HPN for <30 days was 2.2 (P < .05). Logistic regression revealed that Medicaid insurance and age <1 year were associated with increased risk for CA‐BSI (odds ratio [OR], 4.4 [95% CI, 1.13–16.99] and 6.6 [1.50–28.49], respectively; P < .05). Conclusions: The incidence of CA‐BSI in children receiving HPN is highest during the first month posthospital discharge. Strategies to address care in the immediate posthospital discharge period may reduce the burden of infectious complications of HPN.     

Iron Deficiency in Long‐Term Parenteral Nutrition Therapy

Background: Iron is not routinely added to parenteral nutrition (PN) formulations in the United States because of the risk of anaphylaxis and concerns about incompatibilities. Studies have shown that iron dextran in non‐lipid‐containing PN solutions is safe. Data are limited on iron status, prevalence of iron deficiency anemia (IDA), and efficacy of intravenous iron infusion in long‐term home PN (HPN). We aimed to determine the incidence of IDA and to examine the effectiveness of parenteral iron replacement in patients receiving HPN. Methods: Medical records of patients receiving HPN at the Mayo Clinic from 1977 to 2010 were reviewed. Diagnoses, time to IDA development, and hemoglobin, ferritin, and mean corpuscular volume (MCV) values were extracted. Response of iron indices to intravenous iron replacement was investigated. Results: Of 185 patients (122 women), 60 (32.4%) were iron deficient. Five patients were iron deficient, and 18 had unknown iron status before HPN. Of 93 patients who had sufficient iron storage, 37 had IDA development after a mean of 27.2 months (range, 2–149 months) of therapy. Iron was replaced by adding maintenance iron dextran to PN or by therapeutic iron infusion. Patients with both replacement methods had significant improvement in iron status. With intravenous iron replacement, mean ferritin increased from 10.9 to 107.6 mcg/L (P < .0001); mean hemoglobin increased from 11.0 to 12.5 g/dL (P = .0001); and mean MCV increased from 84.5 to 89.0 fL (P = .007). Conclusions: Patients receiving HPN are susceptible to IDA. Iron supplementation should be addressed for patients who rely on PN.     

Denosumab Improves Bone Mineral Density in Patients With Intestinal Failure Receiving Home Parenteral Nutrition: Results From a Randomized,Controlled Clinical Trial

Aim : Low bone mineral density (BMD) is commonly reported in patients receiving home parenteral nutrition (HPN). Oral and intravenous calcium, vitamin D, and bisphosphonates have been used to treat BMD but with low efficiency due to their limited absorption and patient compliance. Denosumab is a new drug that helps prevent osteoclast development and activation and led to decreased bone resorption in some studies. The aim of this study was to assess its value in HPN patients. Methods : Between November 2011 and March 2013, 49 patients receiving HPN (29 women, 20 men, mean age 55.3 years) who met the eligibility criteria were randomly assigned to a denosumab or control group. Regional dual‐energy x‐ray absorptiometry of the spine and hip was performed before therapy and after 12 months. BMD, T score, and z score were assessed. Results : Fifteen patients received 2 doses of therapy and were fully reassessed after 1 year. At baseline and after 12 months, the absorptiometry revealed T scores of ?3.439 standard deviations (SD) vs ?2.33 SD at lumbar segment 2 (L2) and ?2.957 SD vs ?2.067 SD at lumbar segment 3 (L3), z scores of ?2.24 SD vs ?1.36 SD at L2 and ?1.995 vs ?1.067 SD at L3, and BMD of 0.801 vs 0.946 at L2 and 0.857 vs 0.979 at L3, respectively. Two serious outcomes were reported, without any correlation to the intervention. Two patients were weaned off HPN and hence discontinued. One patient experienced sciatica, resulting in discontinuation of the intervention. Conclusions : This study showed that denosumab may be a valuable treatment option for improving BMD in HPN patients.     

A review of the quality of life of adult patients treated with long-term parenteral nutrition

BACKGROUND AND AIMS: Some previous studies have assessed quality of life (QoL) in home parenteral nutrition (HPN) using generic instruments or non-validated questionnaires. A systematic search of electronic databases and relevant publications identified 50 publications. This paper reviews the QoL of patients receiving HPN and discusses the factors affecting QoL. RESULTS: There is little available data about the QoL of HPN patients. Both HPN and the underlying disease may affect QoL, and an evaluation of QoL requires the separation of these two issues. CONCLUSIONS: There is a need for a standardised, scientifically validated, treatment-specific instrument to measure QoL in this population. The use of a treatment-specific QoL questionnaire should become part of the routine clinical management of HPN patients.