Home Parenteral Nutrition Program Research Articles

Management of Short Bowel Syndrome With Chronic Intestinal Failure: A Single-Center Experience in Portugal.

Short bowel syndrome with chronic intestinal failure (SBS/CIF) is the inability to maintain protein-energy, fluid, electrolyte, or micronutrient balance due to a short bowel. Although SBS/CIF is rare, its clinical management is complex, challenging, expensive, and time-consuming. This study aimed to analyze a single center's experience with SBS/CIF in adult patients treated with home parenteral nutrition (HPN). A retrospective single-center analysis of all 13 consecutive adult patients with SBS/CIF was included in an HPN program between January 1994 and August 2023. Between 1992 and 2023, 13 patients were included in an HPN program. The primary underlying pathology was acute mesenteric ischemia. The median age of starting HPN was 44 years. Most were subjected to several surgeries of extensive intestinal resection with posterior intestinal reconstruction. Five of the 13 patients died while on HPN with a median duration of 42 months. The causes of death related to HPN were catheter sepsis, endocarditis with cardiac failure, or hepatic failure. One patient died due to underlying pathology: pelvic abscesses and bleeding related to radiotherapy. Eight patients remainalive, with a median time of HPN of 173 months. During the HPN support, the most frequent complications were venous catheter infection and venous territory thrombosis. None of the eight patients alive have hepatic failure. Two patients recently started teduglutide with good tolerance and need a reduction in HPN support. All eight patients have a satisfactory quality of life (parenteral support needs range between five and two nutrition bags per week). Conclusion: Home parenteral nutrition remains the gold standard of SBS/CIF treatment, although teduglutide may reduce HPN needs and complications and provide a better quality of life. Despite the small number of patients, the results shown in this study are not inferior to those in large-volume centers. The existence of the commitment and interest of professionals involved in SBS/CIF at Centro Hospitalar Universitário de Santo António, Portugal, was a fundamental key to achieving those results. A multidisciplinary healthcare group for HPN support can be essential to ensuring these patients' survival and quality of life.

The Czech Home Parenteral Nutrition Registry REDNUP: Comprehensive Analysis of Adult Patients’ Data

Introduction: Home parenteral nutrition (HPN) is the primary treatment modality for patients with chronic intestinal failure, one of the least common organ failures. This article provides a retrospective analysis of the data collected on HPN patients in the Czech Republic over the past 30 years. Methods: National registry data were collected using a standardised online form based on the OASIS registry (Oley – A.S.P.E.N. Information System) across all centres providing HPN in the Czech Republic. Data collected prospectively from adult patients in the HPN program were analysed in the following categories: epidemiology, demographics, underlying syndrome, diagnosis, complications, and teduglutide therapy prevalence. Results: The registry identified a total of 1,838 adult patient records, reflecting almost 1.5 million individual catheter days. The prevalence of HPN has risen considerably over the last few decades, currently reaching 5.5 per 100,000 population. The majority of patients have short bowel syndrome and GI obstruction, with cancer being the most prevalent underlying disease. Catheter-related bloodstream infections have been the most prevalent acute complication. However, the incidence in 2022 was only 0.15 per 1,000 catheter days. The study also observed an increase in the prevalence of patients on palliative HPN over the last decade. Conclusion: This study presents a thorough analysis of data from the Czech REgistr Domaci NUtricni Podpory (REDNUP) registry. It shows an increasing prevalence of HPN, namely, in the palliative patient group. The sharing of national data can improve understanding of this rare condition and facilitate the development of international guidelines.

Assessment of clinical outcome after sudden alteration of home parenteral nutrition program from customised to commercially premixed admixtures. Experience with short bowel syndrome treatment

Background & AimsDisruptions in the home parenteral nutrition (HPN) process may lead to failure to achieve the intended treatment purposes. This study aimed to evaluate the mid-term clinical outcome in a group of patients with short bowel syndrome (SBS) after a sudden change in the type of home parenteral nutrition programs from customized to commercially premixed admixtures. MethodsThe study conducted in 2020 identified 51 patients with SBS: 27 (53%) women and 24 (47%) men. The SBS were classified as SBS with end jejunostomy 23 (45%) patients, with jejuno-colon anastomosis 23 (45%) patients, with jejuno-ileo anastomosis and total colon in continuity 5 (10%) patients. The following therapeutic program-related data were analysed: changes in nutritional status, body mass index (BMI), Controlling Nutritional Status (CONUT) score, and biochemical assessment. For statistical analysis, the Wilcoxon rank-sum and signed-rank paired tests with continuity corrections were used to compare the results. A p-value of <0.001 was considered statistically significant. ResultsThere was no statistically significant difference between the analysed groups in total energy, amino acid concentrations, and intravenous volume supplementation. BMI and CONUT assessments of nutritional status and selected biochemical parameters were stable during the study period. ConclusionsThe study demonstrated that a sudden change in the HPN therapy program from parenteral admixtures, tailored to meet individual patients’ needs, to commercially premixed admixtures had no significant impact on the mid-term clinical condition of patients with SBS.

Unexpected Serum and Urine Aluminum Concentrations in Pediatric Patients on Home Parenteral Nutrition.

The intravenous supply of aluminum (Al) present in parenteral nutrition solutions poses a high risk of the absorption of this element, which can result in metabolic bone disease, anemia, and neurological complications. The aim of this study is to determine the impact of long-term parenteral nutrition (PN) in children on serum Al concentration and its urinary excretion compared to healthy children. We evaluated serum Al concentrations and its urinary excretion in patients enrolled in the Polish home parenteral nutrition (HPN) program between 2004 and 2022. The study group included 83 patients and the control group consisted of 121 healthy children. In children whose PN was started in the neonatal period, we found higher serum Al concentrations and higher urinary Al excretion than in other subjects whose PN was started later. Only 12% of the children on chronic parenteral nutrition had serum Al concentrations of less than 5 μg/L. Healthy children in the control group had higher serum Al concentrations than those in the parenteral nutrition group, which may indicate the influence of one's environment and diet on Al serum levels.

Efficacy and Safety of a Parenteral Nutrition Program for Patients with RAS Wild-Type Metastatic Colorectal Cancer Administered First-Line Cetuximab Plus Chemotherapy: A Propensity Score Matching Study.

Malnutrition is a common problem in patients with metastatic colorectal cancer (mCRC) receiving targeted therapy plus chemotherapy, resulting in severe toxicity and decreased survival rates. This retrospective study employing propensity score matching (PSM) examined the efficacy and safety of a supplemental home parenteral nutrition (HPN) program for patients with RAS wild-type mCRC receiving cetuximab plus chemotherapy. This retrospective nationwide registry study included data from 14 medical centers/hospitals across Taiwan, and the data period ranged from November 2016 to December 2020. Patients with RAS wild-type mCRC receiving cetuximab plus chemotherapy as their first-line therapy were included and divided into HPN and non-HPN program groups. HPN was initiated based on patient-specific factors, such as baseline nutritional status, treatment-related toxicities, and comorbidities. Clinical outcomes were evaluated using response to therapy, duration of response (DoR), progression-free survival (PFS), and overall survival (OS). This study recruited 758 patients, of whom 110 and 648 were included in the HPN and non-HPN program groups, respectively. After 1:3 PSM, the data of 109 and 327 patients from the HPN and non-HPN program groups were analyzed, respectively. The HPN program group had a higher metastasectomy rate (33.9% vs. 20.2%, p = 0.005), and longer duration of treatment and DoR than the non-HPN program group (13.6 vs. 10.3 and 13.6 vs. 9.9 months, p = 0.001 and < 0.001, respectively). The HPN program group tended to have a longer median PFS (18.2 vs. 13.9 months, p = 0.102). Moreover, we noted a significant improvement in the median OS in the same group (53.4 vs. 34.6 months, p = 0.002). Supplemental HPN programs may be recommended for select patients with mCRC receiving targeted therapy plus chemotherapy to improve oncological outcomes.

Chronic Intestinal Failure in Children: An International Multicenter Cross-Sectional Survey.

Background: The European Society for Clinical Nutrition and Metabolism database for chronic intestinal failure (CIF) was analyzed to investigate factors associated with nutritional status and the intravenous supplementation (IVS) dependency in children. Methods: Data collected: demographics, CIF mechanism, home parenteral nutrition program, z-scores of weight-for-age (WFA), length or height-for-age (LFA/HFA), and body mass index-for-age (BMI-FA). IVS dependency was calculated as the ratio of daily total IVS energy over estimated resting energy expenditure (%IVSE/REE). Results: Five hundred and fifty-eight patients were included, 57.2% of whom were male. CIF mechanisms at age 1–4 and 14–18 years, respectively: SBS 63.3%, 37.9%; dysmotility or mucosal disease: 36.7%, 62.1%. One-third had WFA and/or LFA/HFA z-scores < −2. One-third had %IVSE/REE > 125%. Multivariate analysis showed that mechanism of CIF was associated with WFA and/or LFA/HFA z-scores (negatively with mucosal disease) and %IVSE/REE (higher for dysmotility and lower in SBS with colon in continuity), while z-scores were negatively associated with %IVSE/REE. Conclusions: The main mechanism of CIF at young age was short bowel syndrome (SBS), whereas most patients facing adulthood had intestinal dysmotility or mucosal disease. One-third were underweight or stunted and had high IVS dependency. Considering that IVS dependency was associated with both CIF mechanisms and nutritional status, IVS dependency is suggested as a potential marker for CIF severity in children.

Predicting Factors of Protracted Intestinal Failure in Children with Gastroschisis

To identify prenatal and neonatal predictors of short bowel syndrome-related intestinal failure (SBS-IF) in gastroschisis. This retrospective study included all patients with gastroschisis born between 2000 and 2017 who were enrolled in our home parenteral nutrition program, and all patients with gastroschisis born in our institution who survived 2weeks, during the same time period. Prenatal ultrasound features, neonatal status, anatomic features, oral feeding, and parenteral nutrition dependency were analyzed. Among 180 patients, 35 required long-term parenteral nutrition (SBS-IF group) and 145 acquired full oral feeding within 6months (oral feeding group). The mean follow-up was 7.9years (IQR, 1.6-17.5years) and 5.0years (IQR, 0.1-18.2years), respectively. Both bowel matting (OR, 14.23; 1.07-16.7; P=.039) and secondarily diagnosed atresia or stenosis (OR, 17.78; 3.13-100.98; P=.001) were independent postnatal predictors of SBS-IF. Eighteen children (51% of the SBS-IF group) were still dependent on artificial nutrition at the last follow-up. patients with SBS-IF who achieved full oral feeding had a median residual small-bowel length of 74cm (IQR, 51-160cm) vs 44cm (IQR, 10-105cm) for those still dependent on artificial nutrition (P=.02). An initial residual small bowel length of more than 50cm was the best predictive cut-off for nutritional autonomy, with a sensitivity of 67% and a specificity of 100%. Bowel matting, complex gastroschisis, and secondary intestinal obstruction were associated with SBS-IF in gastroschisis. For patients with SBS-IF, a small bowel length of more than 50cm was predictive of secondary nutritional autonomy.

Pediatric Home Parenteral Nutrition in France: A six years national survey

Home Parenteral Nutrition (HPN) is the cornerstone management for children suffering from chronic intestinal failure (CIF). In France, HPN is organized from a network of 7 certified centers located in University Hospitals spread across the national territory. This study aims to review the data involving children on HPN over a 6-years period in France to outline the global and continuous improvement in care.This cross-sectional study included all children enrolled in any of the 7 French HPN certified centers from January 1st, 2014 to December 31st, 2019. Data was recorded from annual databases provided by each center regarding: age at inclusion, indication and duration of HPN, type of intravenous lipid emulsion (ILE), outcome [PN weaning off, transfer to adult center, death, intestinal transplantation (ITx)], rate of catheter-related bloodstream infections (CRSBIs) for 1000 days of HPN, Taurolidine lock procedure (TLP) use and prevalence of cholestasis defined as conjugated bilirubin ≥20 μmol/l.The number of patients increased by 43.6% from 268 in 2014 to 385 in 2019. According to the year of follow up, the indications for HPN were short bowel syndrome (SBS) (42.3-46.6%), congenital enteropathies (CE) (18.5-22.8%), chronic intestinal pseudo-obstruction syndrome (CIPOS) (13.0-16.3%), long segment Hirschsprung's disease (LSHD) (9.7-13.3%), Crohn's disease (CD) (1.6-2.6%) and other non-primary digestive diseases (NPDD) such as immune deficiency, cancer or metabolic disease (4.0-9.2%). The median age at discharge on HPN decreased from 11.7 months in 2014 to 8.3 months in 2019 (p < .001). By December 31st, 2019, 44.8% of children had left the HPN program after a median duration ranging between 39.9 and 66.4 months. Among these patients, 192 (74.2%) were weaned off PN (94.7% SBS), 41 (15.8%) were transferred to adult centers for CIPOS (42%), SBS (31%) or CE (27%), 21 died (8.1%) - mostly in relation to cancer or immune deficiency - and 5 were transplanted (1.9%): 4 underwent combined liver-intestine transplantation for LSHD (n = 2), SBS, CE and one multivisceral Tx for CIPOS. The use of a composite fish-oil based ILE increased from 67.4% in 2014 to 88.3% in 2019 (p < 0.001). CRBSIs dropped from 1.04 CRSBIs per 1000 days HPN in 2014 to 0.61 in 2019 (p < 0.001) while meantime, the percentage of children receiving TLP increased from 29.4% to 63.0% (p < 0.001). The prevalence of cholestasis (conjugated bilirubin ≥ 20 μmol/l) was low and stable between 4.1 and 5.9% of children during the study period.In France, the number of children enrolled in a HPN program continuously increased over a 6 years period. SBS is the leading cause of CIF requiring HPN. The rate of CRBSIs dropped dramatically as the use of TLP increased. Mortality rate was low and mainly in relation to the underlying disease (cancer, immune deficiency). Cholestasis and intestinal Tx remained very rare.

A22 PREVALENCE OF IRON DEFICIENCY AND SUPPLEMENTATION PRACTICES FOR PATIENTS ON HOME PARENTERAL NUTRITION

Abstract Background Iron deficiency (ID) is common in patients receiving parenteral nutrition (PN), likely due to a lack of iron in the PN formula. There is no clear consensus on how often serum iron should be tested or iron supplementation should be given, at which dose or route, in patients on long-term PN. Within the Hamilton Health Sciences (HHS) home PN (HPN) program, the prevalence of ID or iron deficiency anemia (IDA) is unknown. This knowledge will contribute to better iron prescribing practices with ultimate benefit on patient’s health. Aims To assess the prevalence of ID and IDA in patients enrolled in the HHS HPN Program. The secondary aim was to assess supplementation practices for patients enrolled in the HPN program according to gastrointestinal(GI) diagnosis and duration on PN. Methods We conducted a retrospective study including consecutive adult patients enrolled in the HHS-HPN program from January 2015 to November 2020. We collected data on demographics (age, sex, and GI diagnosis), iron supplementation (dose, duration, and route), and information related to iron-deficiency (hemoglobin, serum iron, ferritin, TIBC, and folate) at pre-set intervals (enrollment, 3, 6, 12, 18, 24, 30, 36, 48, 60 months) and last measured. ID was defined as ferritin ≤45μg/L or serum iron ≤9μmol/L. IDA was defined as hemoglobin &amp;lt;130g/L in men or &amp;lt;120g/L in women in the context of ID. Data were expressed as median (IQR) for continuous variables and n/N(%) for categorical variables. Chi2 was performed to assess differences between groups and logistic regression to assess predictors of ID and IDA. The analysis was conducted using SPSS software(v26). Results The analysis included 125 HPN patients (50 males, median age of 55 (40–65) years). Patients received PN for a median of 195 (83–521) days. The most common diagnoses were malignancy (36.8%) and inflammatory bowel disease (23.2%); the most common indications for HPN was short bowel (29.6%) and bowel obstruction (27.2%). Iron profiles were measured in 77% of patients. At enrollment, 42.2% of patients had ID and 38.9% had IDA. Only 13% of patients with ID and 22.8% with IDA had iron supplementation (Figure 1). A total of 38 patients received iron either oral or IV (oral=44.7% vs IV=55.3%; p=0.66). There was no correlation between low levels of serum iron or ferritin with iron supplementation (p=0.23, 0.45 respectively). Age, sex, diagnosis, or reason for PN did not correlate with ID or IDA at any time point. Conclusions Iron-deficiency and IDA are common in patients enrolled in the HHS HPN program independently of age, sex, diagnosis and reason for PN. Prospective studies are needed to implement the most effective way to ensure proper monitoring and treatment of iron deficiency in this population. Funding Agencies None

Efficacy of 4% tetrasodium ethylenediaminetetraacetic acid (T-EDTA) catheter lock solution in home parenteral nutrition patients: A quality improvement evaluation.

A functioning and reliable central venous access device is fundamental for home parenteral nutrition patients to administer essential nutrition. Complications of central venous access devices including occlusion, microbial colonization, and biofilm formation are problematic and sometimes life-threatening. A novel lock solution, 4% tetrasodium ethylenediaminetetraacetic acid, has properties that may reduce such complications. The aim of this study was to determine the safety, efficacy, and cost implications of implementing 4% tetrasodium ethylenediaminetetraacetic acid to prevent catheter-related complications in home parenteral nutrition patients. A pre- and post-intervention study was carried over 36 months (12 months pre; 24 months post) by the British Columbia Home Parenteral Nutrition Program in Vancouver, Canada, where 4% tetrasodium ethylenediaminetetraacetic acid was implemented for patients at high risk for central venous access device occlusion and catheter-related infection. Patients were included in the study if they had previous central venous access device complications. The outcomes evaluated were central line-associated bloodstream infection, catheter occlusion requiring thrombolytic treatment, and catheter replacements. In total, 22 out of 105 patients met the inclusion criteria. Two patients were excluded from analyses due to non-adherence and concomitant use of other lock solutions. Post intervention, 20 home parenteral nutrition patients experienced significant reduction in the central line-associated bloodstream infection rate (pre = 1.918/1000 catheter days; post = 0.563/1000 catheter days; p = 0.04) There were no occlusion events reported post intervention. For home parenteral nutrition patients, 4% tetrasodium ethylenediaminetetraacetic acid lock solution effectively reduces the risk of central venous access device complications including occlusions and catheter-related infections.

Central venous catheter infection in Canadian home parenteral nutrition patients: a 5-year multicenter retrospective study.

A lower central venous catheter (CVC) infection rate suggests an improvement in practice and education. CVC infection remains a complication that often requires significant health care resources. Use of tunneled CVC and patient education on catheter care reduces CVC infection rates. CVC infection is one of the most frequent, life-threatening complications in home parenteral nutrition (HPN) patients. Our objective was to conduct a 5-year retrospective chart review regarding CVC infections in 3 adult HPN programs. Data were collected from the Canadian HPN Registry and patient charts that include demography, infection diagnosis, blood cultures, and treatments. Results are reported as median (range) ± standard error of mean or population frequency. Eighty-one charts were reviewed. Mean age was 51.98 ± 1.71 years. Short bowel syndrome (54.3%) was the primary diagnosis, with 36 months (range, 1324 months) median length of HPN therapy. Forty-seven subjects (58%) had infections over a 5-year period. Of these, there were 144 sepsis events. There was positive correlation (r=0.423; P<0.001) between number of infections and HPN duration. The median length of time the CVC was in place was 281 (range, 14-4380) days. There were 66.7% tunneled CVCs; 25.9% peripherally inserted central catheters (PICCs), and 7.4% implanted venous port. In this sample, there was no association between line infection and catheter type. Most patients presented with fever (58.3%) and chills (38.2%). Blood cultures were done (89.6%), and coagulase negative Staphylococcus was the resulting pathogen present in 25.7%. Patients with bloodstream infection were treated for 17.9 ± 1.2 days with combination antibiotics (22.2%). Overall, the CVC infection rate was 0.97 per 1000 catheter days. We found the standard approach to infection prevention is comparable to reports in literature. However, a subset of patients with multiple CVC infections require education with an emphasis on preventive techniques in order to reduce the incidence of infection.

P128 INCIDENCE AND OUTCOMES OF HOME PARENTERAL NUTRITION IN PATIENTS WITH CROHN’S DISEASE IN OMLSTED COUNTY, MINNESOTA, USA

Abstract Introduction Patients (pts) with Crohn’s disease (CD) undergoing multiple surgeries are at risk of extensive bowel resections, short bowel syndrome (SBS) and malnutrition and may need home parenteral nutrition (HPN). The aim of our study was to estimate the incidence of HPN use in pts with CD in a population-based cohort, and to assess clinical outcomes and complications associated with nutritional intervention. Methods This study was a retrospective population-based cohort using Rochester Epidemiology Project to identify pts who were diagnosed with CD between 1970 and 2011 in Olmsted County, MN. To identify the individuals who required HPN, these Pts were crossed against the list of those who enrolled in the local HPN program. Descriptive statistics were used to evaluate incidence and clinical outcomes. Results Four hundred twenty nine pts with CD were diagnosed in Olmsted County between 1970 and 2011. Of these, 14 received HPN between 1992 and 2018. The pts were followed for a median of 16.85 years after diagnosis of CD (interquartile (IQR), 12.25–24.70) and required HPN a median of 7.67 years after diagnosis (IQR,1.6–15.89). Among the 14 receiving HPN, 85.71% were females. Eleven (78.57%) had moderate to severe CD and 12 (85.71%) pts involved with fistulizing disease. Crohn’s disease involved the ileum in 92.86%, colon in 71.43%, and proximal GI in 14.29% of the pts. Thirteen (92.86%) pts underwent surgery primarily due to obstruction (84.62%). Other indication included: failure of medical therapy (46.15%), fistulizing disease (46.15%), severe pain (30.77%), abdominal abscess (23.08%), bleeding (15.38%), and necrosis (15.38%). Median number of bowel resections was 4 (range, 0–7). Twelve pts (85.71%) had a stoma placed. The median duration of HPN was 2.48 years (range, 0.11–16.36). Indications for HPN included SBS in 64.28%, malnutrition in 28.57% and bowel rest in 21.43%. The mean number of hospitalizations after the start of HPN was 5 (range, 0–20). Catheter-related bloodstream infections occurred in 10 (71.43%) pts, and 3 (21.43%) had a thrombosis. Four pts had osteoporosis and 2 had osteopenia. Parenteral nutrition-associated liver disease occurred in 2 (14.29%) of pts. The pts’ body weight increased a median of 2.5 kilograms (kg) 6 months after the start of HPN but decreased a median of 1 kg at 12 months. Four (30.77%) pts were on HPN at time of last follow-up. Five (35.71%) pts deceased mainly due to CD. Conclusion Less than 4% of pts with CD need HPN. Most pts on HPN have moderate to severe disease who have undergone extensive bowel resection with resultant SBS and malnutrition. Approximately 70% were able to discontinue HPN. Interestingly, after 6 months, pts lost most of the weight they gained during the first 6 months of HPN. It was possible that this was due to noncompliance, or increased metabolic needs because of active disease.

Learning Gaps and Family Experience, Nurse-Facilitated Home Parenteral Nutrition Simulation-Based Discharge Training: Proof-of-Concept Study.

Home parenteral nutrition (HPN) is a life-sustaining therapy for children and adults suffering with severe digestive diseases, yet complications are commonplace, and predischarge trainings are variable. High-fidelity simulation training provides participants with an immersive experience using realistic equipment, supplies, and scenarios. Simulation training is rapidly becoming a potential gold standard for healthcare but is currently underutilized for families and caregivers. We prospectively collected data on pediatric patients managed at a single HPN program from September 1, 2016, to September 30, 2018. Participants in a pilot simulation-based training program (orientation, high-fidelity mannequin, realistic homelike space, standardized clinical scenarios, and structured debriefing) were compared with historical controls. We excluded patients with short-term HPN use and strictly palliative goals of care. Nineteen (90%) families participated in the pilot initiative with a median (interquartile range) age of 0.9 (3.7) years and diagnosis of short-bowel syndrome in 14 (74%). During teaching scenarios, learning gaps were identified for aseptic needleless changes (53%), HPN equipment setup (84%) with specific difficulty adding multivitamin (32%), and dressing changes (63%). Thirty-day readmission rates in simulation-based training group vs historical cases were 42% vs 63% (P = not significant). There was no difference in length of stay between groups. All (100%) simulation-based training group participants would recommend this learning experience to others. HPN discharge training is a novel use for high-fidelity simulation to address family/caregiver satisfaction and to identify learning gaps. Further studies are needed to refine predischarge training materials and examine the impact on postdischarge outcomes.

P4.49: Home Parenteral Nutrition: www.Necker-HPN-traveling.com

Introduction: In France, the Hôpital Necker-Enfants Malades Home Parenteral Nutrition (HPN) program is the oldest (certified in 1984) and the largest with ~ 40% of the 380 children currently on HPN. We enrolled > 800 infants and children in our program since 1984. In 2018, it supported 156 children (ages: 3mths-17yrs): short bowel syndrome (SBS) (n=78), congenital enteropathy (CE) (n=28), total intestinal aganglionosis (TIA) (n=22) or chronic intestinal pseudo-obstruction (CIPOS) (n=17); miscell.(n=11). We try to offer the most normal life for the child, parents & siblings: family life, school attendance, physical & cultural activities…, and vacations. In France, holidays are sacred…!!. Moreover, some children have their grand-parents living outside France, especially in North Africa, the French islands (Caribean & Indian Ocean), Turkey, Portugal…. Until recent years, holidays outside of France were like an unreachable dream. Thanks to our experience of vacations within France and with our multidisciplinary approach and mutual trust with families, we crossed progressively the French borders. Nowadays, 23 different countries have been visited by 21 families. Methods: the HPN vacations organization involves nurses, MD, pharmacists, psychologist, social worker…. It requires time and should be anticipated at least 2 months before departure. Contacts are established with airlines companies for transporting material in the aircraft both in the cabin and in the hold. Local medical and nursing contacts are established, while medical recommendations are delivered regarding emergency situations and the management of hot weather conditions. Results: In 2018, 20 children went to foreign countries or French Islands. IF causes: SBS (n=9), CE (n=7), TIA (n=4). Children received PN 3 to 7 nights a week, with bags from the French usual provider, completed if needed by a local hospital pharmacy (n=4). Destinations were: Morocco, Algeria, Turkey, Greece, Tunisia, Caribeans, Portugal, Thailand,…Stay in the country ranged from 1 to 8 weeks. Only one child had an harmful event with a dehydration. Conclusion: According to the very successful and enjoyable experience for the families, we do encourage an increasing number of families to do the same whenever financially possible. We suggest our colleagues and friends in charge of a HPN program to set up a “HPN travel agency”…… for offering families great holidays and happiness.

P1.07: THE EFFECTS OF MINIMIZING PHLEBOTOMY BLOOD VOLUME ON ANEMIA IN PEDIATRIC HOME PARENTERAL NUTRITION PATIENTS

Background & Objectives: Anemia is a common morbidity in pediatric patients who require home parenteral nutrition (HPN). One factor that increases the risk for anemia in this patient population is frequent phlebotomy as part of routine monitoring. At Loma Linda University Children’s Hospital (LLUCH), the majority of pediatric patients on HPN have monthly labs drawn as part of the standard monitoring protocol. In June 2016, LLUCH began using micro-tubes for routine blood sampling. Traditional phlebotomy tubes require 15–20 mLof blood. Micro-tubes require less than 2mLfor the same tests. The purpose of this study is to understand the effects of phlebotomy blood volume minimization strategies in reducing anemia in our pediatric patients requiring HPN. Methods: This study was IRB-approved and retrospective in design. Since June 2016, the HPN program at LLUCH began using micro tubes for routine laboratory monitoring. Standard clinical data (age, gender, weight) and laboratory data (CBC, ALT, bilirubin, albumin) were collected prior to June 2016 and after September 2016. The primary outcome of this study was to compare the change in hemoglobin concentrations before and after the use of micro-tubes. The secondary outcome was to estimate the prevalence of anemia for age before and after use of micro-tubes for phlebotomy. Statistical analyses were performed including standard descriptive analysis, t-tests, and bivariate analysis. P-value of <0.05 was considered statistically significant. The sensitivity and specificity of red cell mean corpuscle volume (MCV) and red cell distribution width (RDW) in detecting anemia for ages were also calculated. Results: N = Thirty-eight subjects. Mean age = 6.1 years (SD +/- 4.2y). Prevalence of anemia for age before micro-tube implementation = 22% Prevalence of anemia for age after micro-tube implementation = 14% Mean difference in the hemoglobin of before and after implementation = +0.62g/dL(CI=0.21 to 1.02; p=0.004) Secondary bivariate analysis: No correlation between anemia for age and the following factors: age, gender, weight, ALT, and albumin level. Conclusions: Strategies such as micro-tubes to reduce the volume of routine blood draws in pediatric patients requiring chronic HPN may reduce the prevalence of anemia and improve hemoglobin levels. Age, weight, and liver enzyme levels were not found to be independent risk factors for the presence of anemia for age.

P1.59: Establishing the first national home parenteral nutrition program for paediatric patients with intestinal failure

P1.59: Establishing the first national home parenteral nutrition program for paediatric patients with intestinal failure

Home parenteral nutrition provision modalities for chronic intestinal failure in adult patients: An international survey

The safety and effectiveness of a home parenteral nutrition (HPN) program depends both on the expertise and the management approach of the HPN center. We aimed to evaluate both the approaches of different international HPN-centers in their provision of HPN and the types of intravenous supplementation (IVS)-admixtures prescribed to patients with chronic intestinal failure (CIF). In March 2015, 65 centers from 22 countries enrolled 3239 patients (benign disease 90.1%, malignant disease 9.9%), recording the patient, CIF and HPN characteristics in a structured database. The HPN-provider was categorized as health care system local pharmacy (LP) or independent home care company (HCC). The IVS-admixture was categorized as fluids and electrolytes alone (FE) or parenteral nutrition, either commercially premixed (PA) or customized to the individual patient (CA), alone or plus extra FE (PAFE or CAFE). Doctors of HPN centers were responsible for the IVS prescriptions. HCC (66%) was the most common HPN provider, with no difference noted between benign-CIF and malignant-CIF. LP was the main modality in 11 countries; HCC prevailed in 4 European countries: Israel, USA, South America and Oceania (p<0.001). IVS-admixture comprised: FE 10%, PA 17%, PAFE 17%, CA 38%, CAFE 18%. PA and PAFE prevailed in malignant-CIF while CA and CAFE use was greater in benign-CIF (p<0.001). PA+PAFE prevailed in those countries where LP was the main HPN-provider and CA+CAFE prevailed where the main HPN-provider was HCC (p<0.001). This is the first study to demonstrate that HPN provision and the IVS-admixture differ greatly among countries, among HPN centers and between benign-CIF and cancer-CIF. As both HPN provider and IVS-admixture types may play a role in the safety and effectiveness of HPN therapy, criteria to homogenize HPN programs are needed so that patients can have equal access to optimal CIF care.

Role of pharmacists in providing parenteral nutrition support: current insights and future directions.

BackgroundParenteral nutrition (PN) therapy is a complex and critical therapy that requires special clinical knowledge, skills, and practice experience to avoid errors in prescribing, compounding, and clinical management of patients. Pharmacists with adequate clinical training and expertise in PN therapy can have pivotal role in the care of patients receiving PN therapy.ObjectiveThe aim of this systematic review was to describe and evaluate the different roles of pharmacists and their provided services related to PN therapy.Materials and methodsA comprehensive systematic literature review on the topic was conducted via PubMed database using several keywords related to the topic (from 1975 to 2017). Additional resources included the standards of practice and clinical guidelines from recognized organizations such as the American Society for Parenteral and Enteral Nutrition (ASPEN) and the American Society of Health-System Pharmacists (ASHP).ResultsPharmacists have diverse roles in relation to PN therapy including the following: the assessment of patients’ nutritional needs; the design, compounding, dispensing, and quality management of PN formulations; monitoring patients’ response to PN therapy; supervision of home parenteral nutrition (HPN) programs; education of patients, caregivers, and other health care professionals on nutrition support and conducting PN-related research and quality improvement activities. These services seem to be variable across clinical settings and among different countries depending on the practice environment and pharmacists’ clinical practice in these settings. However, each of these practice domains helps to support the delivery of safe and effective PN therapy to patients.ConclusionPharmacists have been actively participating in providing PN-related services to patients. To fulfill the requirements of their essential role in this area of practice, pharmacists need adequate educational preparation and clinical training on nutrition support. Empowerment of pharmacists to assume a stronger leadership role in this dimension of pharmacy practice will enhance the quality of care provided to patients receiving PN therapy and improve PN services.

Paediatric Home Artificial Nutrition in Italy: Report from 2016 Survey on Behalf of Artificial Nutrition Network of Italian Society for Gastroenterology, Hepatology and Nutrition (SIGENP).

Home Artificial Nutrition (HAN) is a safe and efficacious technique that insures children’s reintegration into the family, society and school. Epidemiological data on paediatric HAN in Italy are not available. Aim: to detect the prevalence and incidence of Home Parenteral Nutrition (HPN) and Home Enteral Nutrition (HEN), either via tube or mouth, in Italy in 2016. Materials and methods: a specific form was sent to all registered SIGENP members and investigators of local HAN centres, inviting them to provide the requested centre’s data and demographics, underlying diseases and HAN characteristics of the patients. Results: we recorded 3403 Italian patients on HAN aged 0 to 19 years from 22 centres: 2277 HEN, 950 Oral Nutritional Supplements (ONS) and 179 HPN programs. The prevalence of HEN (205 pts/million inhabitants) and HPN (16 pts/million inhabitants) has dramatically increased in Italy in the last 9 years. Neurodisabling conditions were the first indication for HEN by tube or mouth while HPN is mainly requested in digestive disorders. Conclusions: HAN is a widespread and rapidly growing treatment in Italy, as well as in other European countries. Awareness of its extent and characteristics helps improving HAN service and patients’ quality of life.

Analysis of patients with pseudo-obstruction syndrome in home parenteral nutrition (HPN) program in warsaw

Rationale: Chronic intestinal pseudo-obstruction (CIP) is a rare disorder, characterized by abnormalities affecting peristalsis of gastrointestinal tract. In HPN program in Children's Memorial Health Institute in Warsaw on 1st of January 2018 out of all 143 patients 10 had CIP.