Term Home Parenteral Nutrition Research Articles

Central venous catheter (CVC) salvage in case of central line-associated bloodstream infection (CLABSI): A monocentric prospective study in patients on long-term home parenteral nutrition (HPN)

CLABSI is a major complication in HPN and frequently leads to central venous catheter (CVC) removal. We developed a salvaging attitude in long term HPN patients due to the necessity of venous preservation. The main objective of this study is to determine the prognosis of CLABSI. We followed-up for three months, in an approved HPN centre, a cohort of 250 adult patients receiving HPN with CLABSI from 2018 to 2020. CLABSI was defined by a blood culture growth differential [peripheral blood] - [CVC blood]≥2h. A therapeutic approach to conserve CVC was established according to the department's protocol. The primary endpoint was conservation of CVC with negative CVC and peripheral blood cultures at 3 months without complications. Data from 30 CLABSIs were collected for 22 HPN patients. The incidence rate of CLABSIs was 0.28 infections/1000 catheter days. Sixteen CVCs were removed immediately, with causes due to the type of germ (staphylococcus aureus: n=6, candida parapsilosis: n=4, klebsiella: n=2), chronic colonization (n=4) or initial complications (n=4). Among the 14 non-removed CVC, 11 were maintained at 3 months with blood cultures on CVC and peripheral negative for 9 (80%) of them. 3 CVC were removed during the 3 months follow-up (non-CVC-related sepsis n=2, and resistant pseudomonas aeruginosa n=1). The incidence rate of CLABSIs in an expert HPN centre remains low. In case of CLABSIs, according to specific protocol, approximately 50% of CVC were removed immediately (essentially due to bacteriological characteristics). In case of CLABSIs and without initial complication, 80% of CVCs can be maintained at 3 months. These results justify a conservative attitude according to standardized protocol.

In their shoes: Health care professionals walking a day in the life of patients with short bowel syndrome & intestinal failure

Short bowel syndrome leading to intestinal failure (SBS-IF) is a condition characterised by the inability of the gut to absorb the amount of water, nutrients and micronutrients needed to survive and requires long term home parenteral nutrition (HPN) to sustain life. Health care professionals (HCPs) working with patients with SBS-IF are aware of the daily challenges patients face. However, few have experienced the condition first-hand and put themselves 'in the shoes' of their patients. In Their Shoes (ITS) is an immersive simulation delivered through a smartphone application (app), in which participants experience the typical real-life challenges that somebody with SBS-IF will experience over a 24-h period, including social and psychological challenges. This study evaluated the impact of ITS simulation on HCPs working with adults with SBS-IF. A representative sample of 27 multi-disciplinary team (MDT) members working on an Intestinal Failure Unit completed the ITS simulation over a 24-h period. A mixed methods design was used, which included participants completing a survey pre- and post-ITS experience, followed by focus groups and in-depth qualitative interviews. Pre- and post-intervention questions were analysed quantitively via paired t-tests and qualitative data arising from the focus groups and interviews were analysed using inductive thematic analysis. 25 participants (93%) completed 80% of the ITS challenges whilst 27 (100%) completed more than 50% challenges. Participants reported a 16% increase in knowledge, 18% increase in being able to talk to others about SBS and a 2% increase in empathy between pre- and post-simulation. Nineteen team members participated in focus groups and 10 in-depth interviews; qualitative analysis of digital recordings comprised four overarching themes: empathy; admiration and respect; empathy fatigue; learning experience. Despite the small and insignificant increase in empathy scores quantitatively, analysis of open-ended questions and qualitative data found that 74% of participants reported an increase in empathy levels whereas 26% felt it stayed the same. As a simulation-based learning method, ITS can have a positive impact on HCPs working into the SBS-IF setting. Positive benefits in clinical practice include increased levels of empathy, admiration and respect for patients amongst HCPs. Immersing into the ITS experience also allows HCPs to recognise the function and benefits to MDT working, both in terms of ensuring holistic patient centred care and managing HCP empathy fatigue.

P7.06: Endovascular Thrombectomy in a Chronic Upper Cava Syndrome Due to Long Term Home Parenteral Nutrition. New Hope to Extend Life Support

Introduction: Chronic Intestinal Pseudo Obstruction(CIPO) refers to a heterogeneous group of disorders characterized by symptoms of intestinal obstruction, without evidence of mechanical lesion and is a cause of chronic intestinal failure. Loss of vascular accesses by thrombosis has become the current main indication for intestinal transplant. Materials and Methods: A 28-year-old female patient with a history of myopathic CIPO received Home Parenteral Nutrition(HPN) for 25 years, developing central venous accesses thrombosis. As a consequence of a chronic upper cava syndrome with occlusion of the right jugular and subclavian-axillary vein, an invalidating headache and inability to maintain dorsal decubitus due to retroocular pain evolves overtime, but worsen with HPN infusion, limiting the volume and calories provided. A multidisciplinary case discussion concluded that although chronic, an endovascular thrombectomy should be attempted to improve quality of life and to satisfy nutritional needs. Results: A vascular computer tomography(CT) scan with 3D reconstruction and a digital venography were performed showing complete occlusion of the right jugular, axillary and subclavian(where a picc-line was placed as last access) veins and a partial occlusion of the proximal superior vena cava(SVC) and occlusion of the right iliac vein. An angioplasty trough the right humeral, axillary, innominate trunk axis and superior vena cava using 6 & 9mm balloons was performed. Balloon dilatation of multiple venous strictures was done, followed by placement of two stents, one into the right subclavian vein and SVC, and the other into left subclavian vein, with restitution of venous blood flow. Anticoagulation with Rivaroxaban and Clopidrogel was started. On the 5th postoperative day a new onset of acute pain, paresthesia were reported, followed by edema in the right upper limb. A Doppler ultrasound was positive for an acute thrombosis, confirmed by CT scan. A new phlebography and mechanical thrombectomy using the ASPIREX device followed by a new angioplasty of a partial distal stenosis was done. A new stent implantation into humeral, axillary, and subclavian veins was performed. Outcome was favorable with complete resolution of the symptoms; being discharged on day 6. The patient currently is asymptomatic, receiving HPN without complications. Conclusion: This case is an example of the management of deep venous thrombosis secondary to the chronic use of HPN. A comprehensive multidisciplinary approach is mandatory due to its complexity, requiring to be performed by trained and expert interventionists, to successfully provide new alternatives to extend the possibility for their life support or avoid contraindications to transplant. Peripheral venous hemodynamic is not a common procedure however, is the cornerstone to treat this complication achieving in many cases the recovery of venous blood flow delaying the need of intestinal transplantation.

Salvage of central line in case of catheter - associated blood stream infections (clabsis) a prospective observational study in adult patients on long-term home parenteral nutrition (HPN)

Rationale: CLABSI is a major complication in HPN and frequently leads to central venous catheter (CVC) removal. We developed a salvaging attitude in long term HPN patients due to the necessity of venous preservation. The main objective of this study is to determine the prognosis of CLABSI.

Vitamin D deficiency in patients with chronic intestinal failure on home parenteral nutrition.

Vitamin D deficiency is an important complication of chronic intestinal failure (CIF). Liver steatosis is a known late complication of long-term home parenteral nutrition (HPN) therapy in patients with CIF, which can progress to intestinal failure-associated liver disease (IFALD). The aim of this study was to determine the prevalence of vitamin D deficiency among Slovene HPN patients and determine any potential correlation between serum vitamin D levels and liver steatosis associated with IFALD in adult patients with CIF on HPN therapy. Adult patients, diagnosed with CIF, receiving long term HPN therapy, were included in a cross-sectional study. Vitamin D status was determined by measuring serum levels of 25-hydroxyvitamin D. The presence of liver steatosis was diagnosed using 3TS MRI scanner. The association between serum vitamin D levels and liver steatosis was calculated using univariate logistic regression. We included 63 adult patients with CIF on HPN therapy in the study. The median duration of HPN therapy was 70 weeks. The average serum concentration of 25-hydroxyvitamin D was 41.3nmol/l. Insufficient vitamin D levels were found in 45 (73%) patients. Liver steatosis was present in 18 (28.6%) patients. No statistically significant association between serum vitamin D levels and liver steatosis in the study population was found. The results of this study have shown a high prevalence of vitamin D deficiency in adult patients with CIF on HPN. We failed to demonstrate an association between serum vitamin D levels and the prevalence of liver steatosis.

Is the initial benefit of segmental reversal of the small bowel on parenteral nutrition maintained in the long-term? a study in 35 patients with short bowel syndrome

Rationale: Short bowel syndrome (SBS) is the main cause of chronic intestinal failure. These patients have long term home parenteral nutrition (HPN). In some cases, surgical techniques such as segmental reversal of the small bowel (SRSB) can be done to decrease HPN dependency. The aim of this study is to assess long term results of SRSB in patients with SBS.

Metabolic bone disease in children with intestinal failure is not associated with the level of parenteral nutrition dependency

Children on long-term home parenteral nutrition (HPN) are at increased risk of suboptimal growth and metabolic bone disease (MBD) i.e. decreased bone mineral density (BMD). The aims of this cross-sectional study were to assess growth and bone health in children on long term HPN and to identify risk factors for MBD. Children above the age of 5 years, stable on HPN for more than 2 years were included. Medical files were reviewed retrospectively and included demographics, gestational age, birth weight and height, indication for PN, age at PN start, duration of PN, number of weekly PN infusions, weight-for-age and height-for-age (SD), body mass index (BMI, kg/m2) as well as blood and urine analyses at the time of Dual X-ray absorptiometry (DXA) measurements. All BMD values were adjusted to statural age which corresponds to the 50th percentile of height. Growth failure (height-for-age≤-2SD) and MBD (at least one BMD measurement≤-2SD) were analyzed according to the indication of PN, duration of PN and PN dependency index (PNDI) by comparing means and performing logistic regression analysis. PNDI is the ratio of non-protein energy intake in HPN to resting energy expenditure using Schofield equations. Forty children were assessed at 12.4±4.5 years of age. Mean age at PN start was 1.1±3.6y (median 0.5). The indications for PN were short bowel syndrome (SBS, n=21), chronic intestinal pseudo-obstruction syndrome (CIPOS, n=10) and congenital enteropathies (CE, n=9). The mean number of PN perfusions was 6±1/week. PNDI was 110±30%. The mean serum level of 25-OHD3 was suboptimal at 26.5±9.1ng/mL (66.2±22.8nmol/L). The mean concentrations of calcium, phosphorus, and parathyroid hormone (PTH) were in the normal ranges. Eight children (20%) had PTH levels above normal with low 25-OHD3 levels. The mean weight-for-age and height-for-age Z-scores SDS were 0.4±0.9 and-0.5±1.1 respectively. The actual height was lower than genetic target height (p<0.001). The BMD Z-scores, adjusted to the 50th percentile of height, of the spine, the left femur and the whole body were:-1.1±1.7,-1.2±1.5 and-1.5±1.8 SDS respectively. Children with CE had significantly lower BMD values than those with SBS and CIPOS (p=0.01). Only two children had bone fractures after a mild trauma (5%). All children on long-term PN, are at risk of low BMD. High dependency on PN (PNDI>120%) and very long-term PN (>10 years) do not appear to increase the risk of growth failure nor MBD. PN-related bone fractures were rare. Close follow-up remains mandatory.

P1.20: Quality of life in children after intestinal transplantation: comparison with liver transplantation and home parenteral nutrition

Introduction: Home parenteral nutrition (HPN) and intestinal transplantation (ITx) are efficient techniques of the management of intestinal failure (IF).We realised that duration of life may not always equate to child and parents’ health related quality of life (HRQOL).The aims of this study are to evaluate the HRQOL in children after ITx with a validated questionnaire, and to compare this population to patients after liver transplantation (LTx), and to patients withIF on long term HPN. Methods: It is an on-going prospective study including patients between 10 and 18 years old, receiving ITx at Necker-Enfants Malades Hospital, with at least 2 years graft survival. We compared them to patients who underwent LTx or receiving HPN, paired on age, delay from transplantation, diagnosis for children on HPN. We used the Child Health Questionnaire, child report form (CHQ-CF87) and parent report form (CHQ-PF 50), including 13 HRQOL domains: physical functioning, social emotional status, social behavioral status, social physical status, bodily pain, general behavior, mental health, self-esteem, general health perceptions, parental impact, family activities and family cohesion. Mean values for each item will be calculated and converted to percentages. Two-tailed Student t-test will be used to compare the mean scores of subjects. Results: We enrolled 48 children: 16 after ITx (mean age 14.9 ± 2.7), 16 after LTx (mean age 14 ± 2.9) and 16 on long term HPN (mean age 13.8 ± 3.7). The study just started, with a good participation and feedback from patients and parents. 12 patients and 20 parents already answered. We already observed that in the same subcategory patients and parents scores often differed. Conclusion: We expect to gain important information about the HRQOL of children after ITx compared to other chronic diseases, to improve the early and long term psychological care of these patients. We hope that better understanding of the impact of these procedures on the child as well as the family’s QOL will assist them in the care.

Renal Function in Children on Long Term Home Parenteral Nutrition.

Objectives: To assess renal function in pediatric intestinal failure (IF) patients on long term home parenteral nutrition (HPN).Methods: Children who received HPN for a minimum of 3 years between 2007 and 2017 were identified from the IF clinic of a large tertiary referral center. Estimated glomerular filtration rate (eGFR) was calculated using the Schwartz formula at discharge on HPN, after 6 months, 1, 2, and 3 years.Results: Twenty five patients (40% male) fulfilled the inclusion criteria. The indications for HPN were due to an underlying motility disorder in 56% (14/25), enteropathy in 24% (6/25), and short bowel syndrome in 20% (5/25). At the start of HPN 80% (20/25) had a normal eGFR. Four children (17%) had an abnormal eGFR. In the group of patients with normal eGFR at the start of HPN 30% (6/20) had at least one episode of decreased eGFR in the following 3 years, however there was no significant decline in eGFR at the end of the 3 year study period. Overall there was no statistically significant deterioration of eGFR in the study population (p = 0.7898).Conclusion: In our cohort of children on long term HPN no significant decline of eGFR could be demonstrated within 3 years of starting PN.

Manganese status in patients on long term home parenteral nutrition

Manganese status in patients on long term home parenteral nutrition

Reversal of intestinal failure associated liver disease fibrosis in a patient receiving long term home parenteral nutrition

Intestinal failure associated liver disease (IFALD) is frequent problem encountered when managing patients receiving parenteral nutrition (PN). Its occurrence is often multifactorial and modification of these factors is vital for the management of such hepatic dysfunction. The use of novel lipid preparations can form part of this management strategy. We present a case whereby such modification of contributing factors, including lipid preparations, led to improvements in IFALD and reversal of hepatic fibrosis.

Review of liver parameters among intestinal failure patients within the regional intestinal failure service Northern Ireland when transitioned to smof lipid emulsion

Rationale: The aim of this review was to investigate whether transitioning to SMOFlipid® (soybean/MCT/Olive/Fish) in those on long term home parenteral nutrition (HPN) on a glucose only regime and infrequent 1st generation lipid emulsion will impact liver function parameters among adult Intestinal Failure patients. The development of Intestinal Failure- associated liver disease (INFALD) is multifactorial and displays a broad spectrum ranging from mild cholestasis to cirrhosis and decompensated liver disease. Improved liver function will increase quality of life and life expectancy.

Long Term Home Parenteral Nutrition Versus Intestinal Transplantation

Long term home parenteral nutrition (HPN) and intestinal transplantation (ITx) are the 2 treatments of total and definitive intestinal failure. HPN is performed from 1984 in our hospital. More than 800 children were included in the program from its beginning. In 2016, 136 children, 60% with total and definitive intestinal failure, were on HPN. Our hospital is also the only pediatric center ITx in France. From 1994, 103 children received 113 ITx. Mean age at ITx was 5 years. Indications for ITx have decreased in the last 5 years, with only one or two ITx per year from 2013. One patient only was on the waiting list in 2017. ITx is nowadays proposed when severe complications of HPN arise only, such as severe liver or kidney disease. Duration of HPN, social difficulties, infections, problems with venous access, are no longer a definite indication for ITx. In the last 5 years, we observed: - a decrease in infections due to the use of taurolidine, and an aggressive program of patient’s education. - a systematic and larger evaluation of the possibilities for HPN. - a better adaptation to social and familial difficulties. With case reports, we will describe how HPN is nearly always possible, by pushing the limits but without excessive risks, and with a tailored program of patient’s education.

Current practices and experience of transition of young people on long term home parenteral nutrition (PN) to adult services – A perspective from specialist centres

There has been an estimated fivefold increase in the number of children receiving parenteral nutrition (PN) at home in the past 10 years with some children approaching the age when they should be referred to adult services whilst still on treatment. Models of care for moving young people onto adult providers of PN at home are not yet well developed, and transition is a potentially dangerous time for young people with complex health needs. A questionnaire to ascertain current experience and practices of transition in the context of home PN services was dispatched to 170 consultant gastroenterologists who were members of the British Association of Parenteral and Enteral Nutrition (BAPEN) and also to all 40 members of the Nutrition and IF working group of the British Society of Paediatric Gastroenterology and Nutrition (BSPGHAN). Anonymised returns were received from 12 adult and 18 paediatric centres. We estimate about 50% paediatric IF centres, and the three largest adult IF centres responded to the survey. We identified 14 young adults already transitioned and 43 currently in transition. The practices and processes of transition reported were highly variable. Time taken to achieve transition ranged from under 6 months up to 2 years. The most frequent concerns to be identified were confusion around care routines and psychological problems at the time of transition (in one third of respondents). We conclude that a transition pathway and service standards for adolescents on home PN should be developed, consideration should be given to checklists for practical aspects (e.g. pumps), key worker and psychology input to enhance emotional resilience of the young people and carers.

MON-PP119: Pregnancy is Possible During Long Term Home Parenteral Nutrition in Patients Having Chronic Intestinal Failure

MON-PP119: Pregnancy is Possible During Long Term Home Parenteral Nutrition in Patients Having Chronic Intestinal Failure

Two patients with intestinal failure requiring home parenteral nutrition, a NOD2 mutation and tuberculous lymphadenitis

BackgroundMutations in the NOD2 gene are a significant risk factor to acquire intestinal failure requiring home parenteral nutrition. Tuberculous lymphadenitis is the main manifestation of extrapulmonary tuberculosis. Defects in the innate immunity, including NOD2 mutations, may increase the risk for acquiring infections caused by M. tuberculosis. An association of intestinal failure, mutations in the NOD2 gene and tuberculous lymphadenitis has not been described before.Case presentationWe report of two patients with intestinal failure secondary to mesenteric ischemia. Both patients presented with fever and weight loss while receiving long term home parenteral nutrition. Both of them were found to have mutations in the NOD2 gene. Catheter related infections were ruled out. FDG-PET-CT scans initially obtained in search for another infectious focus that would explain the symptoms unexpectedly showed high FDG uptake in mediastinal lymph nodes. Direct or indirect evidence proved or was highly suggestive for tuberculous lymphadenitis. Intravenous tuberculostatic therapy was started and led to a reversal of symptoms and to resolution of the lesions by FDG-PET-CT.ConclusionMutations in the NOD2 gene may put patients both at an increased risk for acquiring M. tuberculosis infections as well as at an increased risk of intestinal failure after extensive intestinal resection. Thus we suggest to specifically include reactivated and opportunistic infections in the differential diagnosis of suspected catheter related infection in patients with intestinal failure who carry mutations in their NOD2 gene.

Predictors of Vitamin D Deficiency in Patients Receiving Long Term Home Parenteral Nutrition

Purpose: Previous studies have suggested a high prevalence of vitamin D deficiency in patients on long term Home Parenteral Nutrition (HPN). The aim of this study was to determine the predictors of vitamin D deficiency in such patients. Methods: We identified 79 consecutive patients who were enrolled in the Cleveland Clinic HPN program for longer than 6 months duration (median=39 months) between 1989 and 2013. Patients were categorized by vitamin D status as follows: sufficient; insufficient; and deficient with respective levels of 25OHD of ≥ 30ng/ml, 30-20ng/ml, and <20ng/ml. Results: The mean age of the patients studied was 52±13 years and 33% were male. The median HPN duration was 39 months and the most common indication was inflammatory bowel disease (37%). Patients had between 1 and 29 measurements of vitamin D levels done during their HPN therapy with a median of 4 values per patient. Majority (82%) of patients had at least 1 prescription of high dose Vitamin D supplements (50,000 IU) during this time. Mean vitamin D levels were 24.5 ± 12.7 with a mean lowest level of 15.5 ± 9.7. Univariate predictors of vitamin D deficiency were younger age, no history of bisphosphonate use, and history of jejunal resection. For every 5 year increase in the age at time of measurement, the odds of having deficiency decreased by 10% (p=0.043). In addition, patients on bisphosphonates had a 54% lower likelihood of having deficiency (p=0.011). Patients without jejunum had a 6-fold higher likelihood of vitamin D deficiency (p=0.002). In the multivariate model (Table), lack of jejunum was the strongest predictor of deficiency, suggesting that patients without jejunum were 5 times more likely to be deficient (odd's ratio [OR] 5.3; 95 % confidence interval [CI] 1.9-15.1; p=0.002).Table: Table. Factors associated with vitamin D deficiency: multivariable analysisConclusion: Younger age, no history of bisphosphonate use, and absence of jejunum were predictors of vitamin D deficiency in patients on long term HPN.

Prevalence of Vitamin D Deficiency and Response to Oral Vitamin D Supplementation in Patients Receiving Home Parenteral Nutrition

Purpose: To determine the prevalence of vitamin D deficiency in patients on long term Home Parenteral Nutrition (HPN) and assess efficacy of oral vitamin D supplementation. Methods: We performed retrospective chart review of eligible adult HPN patients managed by Cleveland Clinic Home Nutrition Support Services for a minimum of 6 months. Patients were categorized by vitamin D status into sufficient: mean 25OHD≥ 30ng/ml, insufficient: mean 25OHD= 20-30ng/ml and deficient: mean 25OHD < 20ng/ml. Results: A total of 79 patients were retrospectively assessed for sufficiency of vitamin D levels while on HPN. Seventy nine patients were further categorized into deficient: 64 (81%), insufficient: 9 (11%), and sufficient: 6 (8%) groups based on the degree of vitamin D deficiency. The mean age of patients studied was 52 ± 13 years and 33% were male. Median HPN duration was 39 months and the most common indication was inflammatory bowel disease (37%). Patients had between 1 and 29 measurements of vitamin D levels done during their HPN therapy with a median of 4 labs per patient. The average levels were 24.5 ± 12.7 with an average lowest level of 15.5 ± 9.7. Majority (82%) of patients had at least 1 prescription for Vitamin D supplements during this time. On univariable analysis, lack of high dose vitamin D supplementation (50,000IU) was associated with a higher likelihood of having vitamin D deficiency. Patients who had taken supplements during the previous 3 and 6 months had a 59% (p=0.001) and 45% (0.032) lower likelihood of having deficiency, respectively. In the multivariate model (Table), oral supplementation of vitamin D anytime in the previous 3 months (odd's ratio [OR]=0.70, 95 % confidence interval [CI]: 0.52,0.93; p=0.016) also showed significance, suggesting 30% lower odds of deficiency with oral supplementation.Table: Table. Factors associated with vitamin D deficiency: multivariable analysisConclusion: Minority (8%) of the HPN patient population had sufficient vitamin D levels. The likelihood of deficiency decreased with high dose vitamin D supplementation (>50,000IU). Further prospective studies are required to define the adequate amount of oral supplementation to attain sufficient vitamin D levels (>30ng/ml) in this complex population. More studies are needed to assess if lower levels of vitamin D in such patients translate into adverse clinical outcomes.

Can success be achieved in the treatment of short bowel syndrome patients in Hungary?

Aim: Depending on the severity of the bowel deficiency short bowel syndrome patients require Home Parenteral Nutrition (HPN) after their temporary hospital treatment. Our aim was to prove that short bowel syndrome patients can be successfully rehabilitated by means of long term home parenteral nutrition in Hungary, too.

213 Role of Non-Invasive Liver Function Test Indices in the Detection of Liver Fibrosis in Patients on Long Term Home Parenteral Nutrition

213 Role of Non-Invasive Liver Function Test Indices in the Detection of Liver Fibrosis in Patients on Long Term Home Parenteral Nutrition