Home Parenteral Nutrition in Adult Patients With Chronic Intestinal Failure: Catheter‐Related Complications Over 4 Decades at the Main Danish Tertiary Referral Center

Background/Aims: Catheter‐related complications (CRCs) cause mortality and morbidity in patients dependent on parenteral support at home (HPN) due to intestinal failure (IF). This study describes the incidences of CRCs in an adult IF cohort over 40 years. It illustrates the evolution and consequences of CRCs, their association to demographic characteristics, and potential risk factors in an effort to provide the rationale for preventive precautions to the relevant patients with IF at risk. Methods: All patients with IF discharged with HPN from 1970–2010 were included. Patient and treatment characteristics were extracted from the Copenhagen IF database. The incidences were given per 1000 central venous catheter (CVC) days. Results: The 1715 CRCs occurred in 70% of the 508 patients with IF (56% of the 2191 CVCs). The incidence of catheter‐related bloodstream infections (CRBSIs) was 1.43. Higher age, HPN administration by community home nurses, and prior CRBSIs significantly raised the hazard for CRBSIs. In the 1970s, catheters were generally replaced following CRBSIs, whereas catheter salvage was the norm in the 2000s. The incidences of mechanical complications, tunnel infections, and catheter‐related venous thromboses were 0.80, 0.25, and 0.11, respectively. The overall CRC incidence was 2.58, decreasing the first 3 decades but peaking in the last (2.84). The deaths related to CRCs were low (0.018). Conclusion: Even in an experienced IF center of excellence, the incidence of CRCs increased over the 4 decades. This increase could be explained by the expansion of the indication of HPN to a more elderly and frail patient population.     

Single‐Center,Adult Chronic Intestinal Failure Cohort Analyzed According to the ESPEN‐Endorsed Recommendations,Definitions, and Classifications

Background/Aims: The objective of this study was to describe a clinically well‐defined, single‐center, intestinal failure (IF) cohort based on a template of definitions and classifications endorsed by the European Society for Clinical Nutrition and Metabolism (ESPEN). Methods: A cross‐sectional, retrospective, adult IF cohort, receiving parenteral support (PS), was extracted from the Copenhagen IF database at the tertiary IF center, Copenhagen University Hospital, Rigshospitalet, Denmark. Results: Rigshospitalet provided PS to 188 adult patients with IF on December 31, 2011. Six patients received only fluids and electrolytes, while 97% required parenteral energy (17 ± 12 kcal/kg/d). Although 92% of the cohort had undergone intestinal resection, only 53% were classified as patients with short bowel syndrome (SBS) according to the pathophysiological classification. In the remaining cohort, patients were distributed as 5% with intestinal fistula, 12% with intestinal dysmotility, 5% with mechanical obstruction, and 14% with mucosal diseases. Twelve percent had a combination of pathophysiological causes. The patients with SBS (n = 100) were subdivided according to bowel anatomy into group 1 (jejuno/ileostomy, n = 82), group 2 (jejuno‐colonic‐anastomosis, n = 16), and group 3 (jejuno‐ileo‐colonic‐anastomosis, n = 2). When evaluating the cohort requirements for PS using the ESPEN chronic IF classification based on the need for fluid volume and energy, 53% of the patients with IF were distributed in the maximum categories. Conclusion: The orphan condition of IF with its large patient heterogeneity mandates establishment of uniform definitions and a harmonization of classifications. As illustrated, the ESPEN‐endorsed definitions and classifications are well designed and may serve as a common uniform template to facilitate both intra‐ and intercenter comparisons between reference centers and thus outcome results.     

Vitamin D Deficiency in Children With Intestinal Failure Receiving Home Parenteral Nutrition

Background: Vitamin D plays important roles in both skeletal and nonskeletal health. Limited data suggest that patients with intestinal failure (IF) receiving home parenteral nutrition (PN) are at risk for vitamin D deficiency due to inadequate oral intake, poor absorption, and chronic illness. The purpose of this study was to document vitamin D status in pediatric patients with IF receiving home PN. Materials and Methods: We performed a 2‐year retrospective review of children with IF followed at our center who had been on home PN for ≥6 months and had ≥1 serum 25‐hydroxyvitamin D (25‐OHD) level checked as part of routine clinical care. Patients were then categorized as deficient (<20 ng/mL), insufficient (20–29 ng/mL), or normal (≥30 ng/mL) based on their lowest vitamin D level. Demographic data and clinical characteristics were also assessed. Results: Eleven of 27 children (41%) had ≥1 insufficient 25‐OHD level, including one child with vitamin D deficiency. Diagnosis of short bowel syndrome (compared with dysmotility or malabsorption syndromes) was associated with decreased likelihood of suboptimal vitamin D status, with an odds ratio of 0.12 (95% confidence interval, 0.02–0.8, P = .028). Osteopenia was noted in 59% of the cohort. There was a trend toward higher risk for osteopenia in patients with low 25‐OHD levels compared with those with normal 25‐OHD levels (82% vs 44%, P = .109). Conclusion: Suboptimal 25‐OHD levels are common in children with IF on home PN. This emphasizes the critical importance of routine surveillance of serum vitamin D levels and consideration of enteral supplementation when indicated.     

Expediting Transition to Home Parenteral Nutrition With Fast‐Track Cycling

Background. Delivery of home parenteral nutrition (PN) is typically cycled over 12 hours. Discharge to home on PN is often delayed due to potential adverse events (AEs) associated with cycling PN. The purpose was to determine whether patients requiring long‐term PN can be cycled from 24 hours to 12 hours in 1 day instead of 2 days without increasing the risk of PN‐related AEs. Methods. Hospitalized patients receiving PN at goal calories infused over 24 hours without severe electrolyte or blood glucose abnormalities were eligible. Patients were randomly assigned to a 1‐step “fast‐track” protocol or 2‐step “standard” protocol. AEs were defined as hypoglycemia or hyperglycemia, new‐onset or worsening dyspnea, tachycardia, tachypnea, lower extremity or sacral edema, pulmonary edema, or abdominal ascites and were graded as minor or major. Results. In the 63 patients studied, the most prevalent PN‐related AE was hyperglycemia, occurring in 24.2% and 30.0% of patients in the fast‐track and standard groups, respectively. Overall, there was no significant difference in the prevalence of PN‐related minor AEs between fast‐track and standard groups (33.3% and 53.3%, P = .5). No major PN‐related AEs occurred in the fast‐track group, while 1 major PN‐related AE (pulmonary edema) occurred in the standard group. Conclusions. Fast‐track cycling is as safe as standard cycling in patients without diabetes mellitus or major organ dysfunction requiring long‐term PN. Fast‐track cycling could potentially expedite hospital discharge, resulting in decreased healthcare costs and improved patient satisfaction.     

Long-Term Outcomes in Patients with Intestinal Failure Due to Short Bowel Syndrome and Intestinal Fistula

Short bowel syndrome (SBS) and enterocutaneous or enteroatmospheric fistulas are common indications for home parenteral nutrition (HPN). However, there are few data describing factors influencing surgical decision-making or outcomes particularly following fistula development. We aimed to compare outcomes between patients with SBS and fistulas and explore surgical decision-making. HPN-dependent adults from 2001–2018 at a national reference centre were included in this study. HPN cessation was analysed using death as competing risk. In total, 465 patients (SBS (62%), fistula (38%)) were included, with median HPN dependency of 2.6 years. In total, 203 patients underwent reconstructive surgery; while frailty was the commonest reason for not undergoing surgery (49.2%), 22.7% declined surgery. Overall, 170 ceased HPN, with a probability of 13.8%, 34.1% and 38.3% at 1, 5 and 10 years, respectively. Patients undergoing surgery had higher nutritional autonomy rates (109.8 incidences/1000 patient years) compared to those not undergoing surgery (18.1 incidences/1000 patient years; p < 0.001). A total of 295 patients (63.4%) were predicted to cease HPN based on gastrointestinal anatomy but only 162/295 (54.9%) achieved this; those unable to do so were older with a higher comorbidity index. There were no differences in long-term nutritional and survival outcomes or surgical decisions between patients with SBS and fistulas, or between enterocutaneous and enteroatmospheric fistulas. This study represents one of the largest datasets describing the ability of HPN-dependent patients with SBS or fistulas to achieve nutritional autonomy. While reconstructive surgery facilitates HPN cessation, approximately one-fifth of patients declined surgery despite HPN dependency. These data will better inform patient expectation and help plan alternative therapies.     

Management of Parenteral Nutrition in Hospitalized Adult Patients

Despite the high prevalence of malnutrition in adult hospitalized patients, surveys continue to report that many clinicians are undertrained in clinical nutrition, making targeted nutrition education for clinicians essential for best patient care. Clinical practice models also continue to evolve, with more disciplines prescribing parenteral nutrition (PN) or managing the cases of patients who are receiving it, further adding to the need for proficiency in general PN skills. This tutorial focuses on the daily management of adult hospitalized patients already receiving PN and reviews the following topics: (1) PN basics, including the determination of energy and volume requirements; (2) PN macronutrient content (protein, dextrose, and intravenous fat emulsion); (3) PN micronutrient content (electrolytes, minerals, vitamins, and trace elements); (4) alteration of PN for special situations, such as obesity, hyperglycemia, hypertriglyceridemia, refeeding, and hepatic/renal disease; (5) daily monitoring and adjustment of PN formula; and (6) PN‐related complications (PN‐associated liver disease and catheter‐related complications).     

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.     

Vitamin Serum Level Variations Between Cycles of Intermittent Parenteral Nutrition in Adult Patients With Short Bowel Syndrome

Background: An alternative form of nutrition therapy for patients with short bowel syndrome (SBS) who do not have home parenteral nutrition (PN) available is the administration of the PN cycle during regular hospital admissions and oral vitamin supplement at home. The aim of this study was to evaluate serum levels of folic acid and vitamins A, B₁₂, C, and E before and after the PN cycle in patients with SBS. Materials and Methods: This research was conducted with 10 patients with SBS (less than 15 minutes of total barium transit time) who were receiving the PN cycle and oral vitamin supplement at home. Patients were evaluated regarding total food ingestion and oral vitamin supplement intake. Serum levels of vitamins were evaluated immediately after the end of each PN cycle (phase 1) and before the beginning of the next PN cycle (phase 2). Results: Patients’ nutrient ingestion was in accordance with recommendations for healthy individuals. Regarding the oral vitamin supplement intake, 20% of the patients presented low adherence. Although all patients had a normal serum level of folic acid and vitamin B₁₂, PN vitamin infusion during hospitalization and home oral vitamin supplement were not enough to make the serum levels of vitamins A, C, and E achieve normal values. Conclusion: This study documented that patients did not receive an adequate administration of oral vitamin supplements of vitamins A, C, and E through PN. More studies need to be conducted investigating higher doses of vitamin administration (oral or intravenous administration), assessing differences between water and liposoluble supplements.     

Longitudinal Bone Mineralization Assessment in Children Treated With Long‐Term Parenteral Nutrition for Severe Intestinal Failure

Background: Metabolic bone disease is common in children receiving home parenteral nutrition (HPN) for intestinal failure (IF). Long‐term evolution of bone mass in pediatric IF is poorly documented. The aims of this study were (1) to determine the prevalence of low bone mass (LBM) in children receiving HPN for IF, (2) to evaluate the evolution of total bone mineral content (TBMC) during HPN with dual‐energy x‐ray absorptiometry (DXA), and (3) to identify related factors. Methods: All children referred in our HPN center from 2004 to 2014 were eligible. Inclusion criteria were HPN dependence due to noninflammatory IF, at least 2 TBMC assessments, and HPN duration of at least 2 years at last DXA. TBMC was expressed in z score for ideal weight for height (WFH). LBM was defined by a TBMC WFH z score ≤–2 standard deviations (SD). Results: A total of 175 DXAs for 31 children were performed, mean of 5.6 ± 2.9 assessments per child. The median time between first and last DXA recorded was 6.2 years (0.7–16.6). At the first DXA, 14 children (45%) had a LBM. TBMC increased by +0.1 ± 0.04 SD per year of HPN (P = .012). The risk of LBM decreased with an odds ratio of 0.9 per year of HPN (95% confidence interval, 0.92–0.99; P = .018). Lean mass z score and calcium parenteral intakes were related to the TBMC improvement. Conclusion: LBM is common in pediatric IF, but bone status could improve during HPN in these children.     

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.     

Poor Social Support and Unemployment Are Associated With Negative Affect in Home Parenteral Nutrition–Dependent Patients With Chronic Intestinal Failure

Background: Home parenteral nutrition (HPN) provides life‐sustaining treatment for people with chronic intestinal failure (CIF). Poor quality of life is reported in association with the burden of HPN and the underlying condition leading to intestinal failure (IF). However, levels of negative affect (NA), incorporating anxiety and depression, have not been reported in CIF. This study examined the occurrence and risk factors for NA in a large CIF population using the validated Hospital Anxiety & Depression Scale (HADS) and Multidimensional Scale of Perceived Social Support (MSPSS) questionnaires. Methods: A survey pack including HADS and MSPSS were distributed to patients managed by a specialist IF center. Data from these measures were analyzed against factors including underlying disease, length of time receiving HPN, employment status, and demographics. Results: Of 85 patients who completed this study, 56% had clinical levels of anxiety and/or depression. Linear regression analysis confirmed significant correlations between social support and HADS scores, with poorer perceived social support associated with higher levels of NA (?0.26 MSPSS + 33.24; R² = 0.29; P < .0001). There were also significant associations between unemployment and higher anxiety (P = .004), depression (P = .008), and NA scores (P = .003). By contrast, there were no significant associations between patient age, duration of time receiving parenteral nutrition, pathophysiological mechanisms of CIF, and NA levels. Conclusions: Clinical anxiety and depression are common, affecting more than half of patients with CIF. Patients with poor social networks and those of working age who have ceased employment are particularly vulnerable and should be prioritized for psychological support because of their elevated risk.     

The Gut Microbiome in Patients with Intestinal Failure: Current Evidence and Implications for Clinical Practice

Intestinal failure (IF) is the reduction of gut function or mass below a minimum needed to absorb nutrients and fluids, such that patients are dependent on parenteral nutrition (PN). Patients with IF have an altered gut microbiome. Our aim was to review and evaluate the current evidence on gut microbiome and its metabolic activity, as well as its association with disease characteristics in adults and children with IF. We performed a PubMed literature search for articles published after 2000 using the following terms: intestinal, microbiome, microbiota, short‐chain fatty acids, short bowel syndrome, and PN. Literature search was restricted to human studies only. The gut microbiome diversity is remarkably reduced, and community structure is altered with a noticeable overabundance of Proteobacteria, especially the Enterobacteriaceae family. A substantial increase in Lactobacillus level is often reported in patients with IF. Gut microbiome characteristics have been associated with poor growth, liver disease, D‐lactic acidosis, and duration of intestinal adaptation. Differences in microbiome characteristics have been found between patients receiving PN and those whose guts have adapted and have been weaned off PN. Future research with prospective sample collection should explore the value of the gut microbiome as a biomarker to guide clinical practice and as a modifiable therapeutic target to optimize outcomes of patients with IF.     

The Association Between Home Parenteral Nutrition and Patients With FAP‐Associated Intra‐Abdominal Desmoids

Background: Intra‐abdominal desmoid tumors (IADTs) are a common complication of familial adenomatous polyposis (FAP). Treatment is not standardized for advanced disease. Medical and surgical treatments may be ineffective in preventing complications, which can cause intestinal failure. Home parenteral nutrition (HPN) can be a life‐saving treatment in these patients. The aim of this study was to investigate the association with HPN in FAP‐IADTs. Methods: A retrospective review of FAP patients with IADTs at the Cleveland Clinic (CC) between 1980 and 2009 was performed. Patients and tumor characteristics were retrieved from the CC Jagelman Registry for Inherited Neoplasms and CC HPN database. Inclusion criteria were FAP‐IADTs and 6‐month follow up at CC. Exclusion criteria were <6‐month follow‐up, lack of 3‐dimensional lesion or sheet desmoid, and/or incomplete medical records. Kaplan‐Meier curves were analyzed for HPN and non‐HPN groups. Results: One hundred fifty‐four patients were included and divided into 2 groups: HPN (n = 41, 26.6%) and non‐HPN (n = 113, 73.4%). The HPN group was more likely to have advanced‐stage disease and significantly higher incidence of chronic abdominal pain, narcotic dependency, bowel obstruction, ureteral obstruction, deep vein thrombosis, pulmonary embolism, fistulae, and sepsis (P < .05). The need for HPN represented a strong predictor of mortality (5‐year survival HPN = 72% vs non‐HPN = 95%), but duration of HPN did not affect mortality. Conclusion: HPN, although a life‐saving treatment, is an independent poor prognostic factor associated with high morbidity and mortality.