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21st August 2024
A new international protocol to support the structured and optimal transition of children with chronic intestinal failure on home parenteral nutrition from paediatric to adult care has been published based on a consensus of specialist healthcare advice from experts across Europe and North America.
The new practical guidance is designed to be used by clinicians as a formal checklist that can be placed in the patient’s chart to review and track the transition process by chronic intestinal failure multidisciplinary team members.
While the outlook and life expectancy for children with chronic intestinal failure on home parenteral nutrition have improved dramatically, and there is a need to bridge the gap between paediatric and adult healthcare for these patients, the research team said.
Members of the Intestinal Failure working group of the European Reference Network for Rare Inherited Congenital (gastrointestinal and digestive) Anomalies and the European Society for Paediatric Gastroenterology Hepatology and Nutrition’s Network of Intestinal Failure and Intestinal Transplant in Europe group were sent a survey to analyse 20 interventions for transition.
Alongside this, the experts were given an open-ended question to fill in any other suggestion regarding the most effective intervention they had experience with.
Interventions that scored over 80% by all participants were automatically added to the new protocol. These included interventions such as ‘paediatric physician initiates discussion about transfer of care to patients and parents’ and ‘paediatric physician assesses the patient’s understanding of the current health situation’, which scored 95% and 93%, respectively.
Interventions scoring below 50% were excluded, while those scoring between 50 and 80% were discussed in a consensus meeting of experts and patient representatives and included if a unanimous agreement was met. Those discussed at the consensus meeting included interventions such as ‘paediatric physician directs all questions and explanations to patient instead of the parents/caregivers’.
The primary interventions in the final protocol include the assessment of the patient’s transition readiness, provision of knowledge to the patient by the paediatric team, involvement of parents in the transition process and collaboration between the paediatric and adult teams.
The researchers advise that preparation for the transition from paediatric to adult care for children with chronic intestinal failure on home parenteral nutrition should begin one to two years before transition and that the process should be tailored to the individual patient.
They also suggest that future research investigating transition readiness of chronic intestinal failure patients is needed.
Reference
Demirok, A et al. Transition from pediatric to adult care in patients with chronic intestinal failure on home parenteral nutrition: How to do it right? Clinical Nutrition 2024; Jun 20: doi.org/10.1016/j.clnu.2024.06.019.
21st February 2023
Neonatal parenteral nutrition and its delivery is a complex process, with very specific requirements. A multidisciplinary team can offer expertise to ensure adequate nutrition to avoid deficits and promote growth while reducing any associated risks.
Providing parenteral nutrition (PN) to babies is a complex process. Multidisciplinary teams (MDTs) can have a unique understanding of the specific nutritional requirements and can offer added expertise in ensuring adequate nutrition to avoid deficits and promote growth, while reducing the risks associated with PN, including during the transition to full enteral feeding.
Appropriate nutrition is essential for growth and development.1–3 Neonates who are unable to tolerate adequate enteral nutrition will require PN. Total parenteral nutrition (TPN) describes a situation whereby all nutrition is delivered intravenously; however, PN is often used in the neonatal unit in conjunction with enteral feeds, either to maintain nutritional intake as milk feeds are increased or for babies in whom full enteral intake is not tolerated (for example, in cases of short bowel).
PN administration should be based on nationally agreed evidence-based guidelines, recognising that the evidence base for neonatal PN can be limited.4,5 To date there has been no randomised controlled clinical trial of neonatal PN powered to examine longer-term outcomes including neurodevelopment and cardiovascular health. The 2020 National Institute for Health and Care Excellence (NICE) guideline for neonatal parenteral nutrition is the most comprehensive review of the current evidence.6 This guideline covers PN for babies born preterm, up to 28 days after their due birth date and babies born at term, up to 28 days after their birth.
The guideline provides recommendations on:
PN should be considered in any neonate who is unlikely to meet the nutritional requirements via the enteral route, either due to immaturity or illness. Premature infants, and especially those of extremely low birth weight, are particularly vulnerable due to their low nutritional reserves.7 Early provision of nutrition for preterm infants is associated with improved weight gain and head growth.8 When a baby meets the indications for PN, it is advised to start it as soon as possible, and within 8 hours at the latest.6
The absolute indications are summarised in Table 1.
The scope of the NICE guideline6 only covered PN for babies born preterm, up to 28 days after their due birth date and babies born at term, up to 28 days after their birth.
However, there will be babies who will require PN for longer periods due to factors that limit enteral intake. As part of the guideline development, NICE considered whether nutrition care/support teams are effective in providing parenteral nutrition in preterm and term babies. The literature review identified no randomised controlled trials; therefore, observational studies were included to inform decision-making.
Four observational studies were identified by NICE.9–12 Two involved cohorts of surgical patients,9,10 whereas the others looked at cohorts of preterm infants.11,12 The lack of robust trials in this area can be seen from the fact that in all categories considered, the NICE team found the evidence to
be of very low quality; however, experience has shown that the involvement of an MDT can improve outcomes.
Outcomes will potentially be influenced by the composition of the MDT which can include professionals from gastroenterology, neonatology, general surgery, nursing, nutrition, pharmacy, social work, and occupational therapy, depending on the individual patient. Not all disciplines will be involved in all patients. The current evidence does not enable the exact determination of an MDT to be detailed but indicates that there is a role for this team. The composition will likely be determined by the clinical condition of the baby and its severity. The report in 2011 by the Paediatric Chief Pharmacists Group recommended that all children have access to a competent MDT, with a minimum composition of a doctor, pharmacist, nurse and dietitian.4 The exact structure of an MDT will depend on the caseload and the patients that are seen. An MDT can be network-based.
Not all babies will require longer-term management by an MDT. A lot of babies, particularly those not in Level 3 units, will only be on PN for a short period and standard bags are appropriate for these patients. For others, for example, in cases of CDH, there might be fluid restrictions, which together with multiple drug infusions limit the volume for nutrition. In these cases, the MDT has a significant role to play in optimising nutrition.
The neonatal consultant will generally be the team member with an overall view of the patient’s condition and will, generally, be the one to make the decision for commencing PN for patients who fall outside the absolute criteria above. For babies falling under surgical conditions, for example in CDH, then a surgeon will have a major input into the nutrition regime, particularly regarding the introduction of enteral feeds. For babies on long-term PN at risk of PN-associated liver disease (PNALD), then a gastroenterologist will also be required.
The nurse will be the healthcare professional who will be spending the most time with the patient and will be monitoring associated items like fluid balance and line condition. The NICE guideline has given ranges for the osmolality of solutions that can be run peripherally. This will be a change in practice for some units and might require more stringent line management, particularly for peripheral cannulae.
Following the introduction of non-medical prescribing in 2004, prescribing of PN was seen as a natural progression for pharmacists as they had been involved in the formulation of PN for many years. Having a pharmacist as a member of the MDT, particularly attending ward rounds for babies on PN, will help to reduce prescribing errors,6 and ensure suitability, from a chemical compatibility view, of the proposed regimen. This would be done in liaison with a pharmacist in the pharmacy aseptic unit, to discuss the validation and clinical appropriateness of any amendments to the formulation with the prescribing pharmacist.
Enteral feeding was outside the scope of the NICE guideline so there were no recommendations around the transition to enteral feeds from PN. However, nearly all babies on PN will transition to full enteral feeding at some point, and this crossover as feeds increase and PN reduces can lead to a nutritional gap. Dietitians play an important role in recommending optimal enteral nutrition for these patients.
The MDT will not be the only factor influencing PN provision and duration of treatment; for example, gestational age is also a factor. Critical outcomes identified by NICE were anthropometric outcomes, prescribing errors, and achievement of target intake. The latter two, in particular, can be influenced by the MDT. Although the evidence around the benefit of an MDT is low quality, knowledge and experience have shown these teams to be effective, particularly for babies with complex needs.
Access to these core professionals, with access to other fields of expertise where needed, for example, surgeons or gastroenterologists as listed above, to provide additional clinical support, will help to provide optimum PN for neonates. This additional expertise can be network-based as not all units will have this additional support on site, and would be called upon as required for specific patients
So, the question is not whether an MDT is required for neonatal PN – experience has shown that it is beneficial – but what is the ideal composition for such a team. However, further research is required in this field to continue improvement in the provision of PN to neonates.
Peter Mulholland MSc FRPharms
First published on our sister site Hospital Pharmacy Europe
8th May 2017
Short bowel syndrome (SBS) is a rare and potentially life-threatening condition characterised by a reduction in intestinal length, usually as a result of extensive surgical resection. This affects the ability to absorb nutrients, electrolytes and fluid.
Common causes of SBS in children include necrotising enterocolitis (NEC), gastroschisis, intestinal atresia and midgut volvulus.1 SBS is the leading cause of intestinal failure (IF) in children. IF occurs when the gastrointestinal tract is unable to absorb sufficient nutrients, water and electrolytes to maintain adequate growth. NEC is the most common cause of SBS and its incidence is expected to rise due to the increased survival rates of premature neonates.2
In addition to the reduced intestinal surface area for absorption, other mechanisms may contribute to the malabsorption in SBS. Gastric acid hypersecretion inactivates pancreatic enzymes and this can impair the absorption of nutrients.2 Increased gastric acid secretion may also cause diarrhoea1 and increase stoma output. Due to the loss of intestinal mass, there is also a reduction in digestive enzymes responsible for carbohydrate digestion and absorption.2
Following resection, the intestine undergoes a process of adaptation whereby it increases in mass and surface area and this ultimately improves its absorption function.3 Several factors are associated with successful adaptation including length of remaining bowel, presence of ileocecal valve, functional integrity and type of small bowel remaining (the ileum adapts better than the jejunum) and presence of intact colon.2–5 The age at which resection occurs is an important factor affecting adaptation because preterm neonates have a greater potential for bowel growth.6 Due to the differences in intestinal growth, it may be preferable to describe the remaining bowel length as a percentage of the expected length based on the age or size of the child.3 SBS can be defined as a reduction in intestinal length of more than 50%.
The main aims in the management of SBS are to maintain growth and development, promote intestinal adaptation and prevent complications.2,3 This requires a multidisciplinary team, referred to as the nutrition support team (NST), which consists of a paediatric gastroenterologist, dietitian, nurse and pharmacist. Input of a NST has shown to have beneficial effects on patient outcomes, as well as potentially reducing healthcare costs.7
Optimal nutritional support is important for growth and development. The gastrointestinal tract is the most physiological and safest way of feeding and nutrition may be given orally or via an enteral feeding tube. Enteral nutrition has an essential role in promoting intestinal adaptation by stimulating the release of hormones from the gastrointestinal tract,2,3,6 and so it should be started as soon as possible after surgery. Oral feeding has the advantage of maintaining sucking and swallowing functions. However, many infants with SBS develop oral aversion as a result of the numerous interventions during their prolonged hospital stay. Continuous enteral tube feeding is beneficial as it increases contact time with the intestinal mucosa and so absorption may be maximised. Enteral feed tolerance is measured by evaluating stool output and vomiting.
Parenteral nutrition (PN) is the mainstay of treatment in SBS. It enables the provision of adequate fluid, electrolytes, macro- and micronutrients to maintain hydration and electrolyte balance and promote growth and development during the period of intestinal adaptation. As enteral feeds are increased, PN is weaned with careful management from the NST. PN may be required for several years until full enteral autonomy can be achieved, so parents/carers are trained to administer PN at home. Guidelines from the European Society of Paediatric Gastroenterology, Hepatology and Nutrition and the European Society for Clinical Nutrition and Metabolism, published in 2005, detail the current standards for paediatric PN.8 An update to these guidelines is expected to be published soon.
The most important complications of SBS are related to PN. Patients require long-term central venous access for PN administration. Infection is the most common complication associated with central venous catheters (CVCs) and can cause significant morbidity and mortality. Adherence to aseptic procedures when handling CVCs is essential to prevent catheter-related bloodstream infections (CRBSIs). Taurolidine, a derivative of the amino acid taurine, has antimicrobial and antifungal activity and is associated with a decreased incidence of CRBSIs when administered as a line lock to patients at risk of infection.9
IF-associated liver disease (IFALD) is a frequent and severe complication affecting children with SBS who are on long-term PN. Clinically, it can be defined by hyperbilirubinaemia (>50µmol/l) and serum alkaline phosphatase and gamma-glutamyl transferase >1.5-times the upper limit of normal. Hepatic steatosis, cholestasis and hepatic fibrosis can occur, with possible progression to cirrhosis and liver failure.2 Onset of IFALD may be specifically related to the PN composition and administration. Lipids provide a source of non-protein calories and essential fatty acids for patients receiving PN, and also enable fat-soluble vitamins to be included in the PN formulation.
However, use of soybean oil-based intravenous lipid emulsions (ILEs) may contribute to IFALD. They contain a higher proportion of omega-6 fatty acids, leading to the production of pro-inflammatory substances with the potential to cause liver damage. Soybean oil-based ILEs provide long-chain triglycerides which are less easily hydrolysed by lipoprotein lipases than medium-chain triglycerides.10 Fish oil-based ILEs provide a source of anti-inflammatory omega-3 fatty acids, and there is some evidence that the use of a multicomponent fish oil-containing ILE may contribute to a reduction in total bilirubin levels in children with IF on long-term PN.11 Provision of excess glucose and lipid in PN may cause hepatic steatosis, so the PN formulation should be adapted to the needs of the individual patient. Generally, glucose intake should not exceed 18g/kg/day in infants, and lipids should provide between 25% and 40% of non-protein calories.8
It is common practice to give ‘fat-free days’ by omitting lipid from the PN formulation for a number of days each week for patients on long-term PN. Cyclical infusion of PN may also reduce the risk of liver complications and so the infusion time is usually gradually reduced from 24 hours to 12 hours, as tolerated. This requires close monitoring of blood glucose, as well as a stepwise increase and decrease of PN infusion rates to prevent hyper and hypoglycaemia. Infants need to be on sufficient enteral feeds to enable them to maintain normal blood glucose control during the time off from PN. The reduction in infusion time enables PN to be administered overnight and encourages oral feeding during the day. It also enables the child to go to school and participate in other activities.
Other risk factors for IFALD include prematurity, recurrent sepsis and lack of enteral feeding. The immature liver of preterm neonates is more susceptible to damage from hepatotoxic substances. Extrahepatic infections (including CRBSIs) cause a release of pro-inflammatory substances that contribute to liver disease.4,6 Absence of enteral nutrition leads to a lack of stimulation of the enterohepatic circulation and the accumulation of toxic bile salts, causing cholestasis.4
Medication may be required to optimise treatment in SBS. Proton pump inhibitors and histamine H2 antagonists can reduce gastric acid hypersecretion. Loperamide can help to reduce stool output by increasing intestinal transit time, and thereby increasing absorption. Children with cholestasis may benefit from ursodeoxycholic acid to improve bile flow.
In children with SBS, the small bowel may become dilated and lose its normal peristaltic activity.3 Surgery may be indicated to increase the functional surface area of the intestine to enhance absorption. Longitudinal intestinal lengthening and tapering (LILT), often referred to as the ‘Bianchi procedure’, involves dividing the dilated bowel in half longitudinally and then joining the two smaller lumens end-to-end to increase intestinal length and halve the diameter.
Serial transverse enteroplasty (STEP) is a procedure where an endoscopic surgical stapler is applied to alternate sides along the length of the dilated intestinal segment to create a ‘zig-zag’ – a longer and narrower channel. Recurrent dilation of the bowel can occur after both procedures. However, unlike STEP, the LILT procedure can only be performed once. Although, these surgical procedures may not lead to enteral autonomy, they may have a role in certain individuals with dilated small bowel and limited enteral feed tolerance.
Intestinal transplantation is indicated in children with irreversible IF who have recurrent life-threatening CRBSIs, IFALD or loss of vascular access. Patients with mild liver disease are offered isolated intestinal transplantation. However, combined liver and small bowel transplantation is recommended in those with severe IFALD. Isolated liver transplantation is indicated in children with SBS who are unable to achieve intestinal adaptation and enteral autonomy due to end-stage liver disease, provided they have previously tolerated at least 50% of their estimated daily calorie requirements enterally with adequate weight gain.4,10
The criteria of some transplant centres includes a requirement of at least 30cm of functional small bowel, with or without the ileocecal valve, for this type of transplant.4 Data from the two UK paediatric intestinal transplant centres report one-year survival of 85% and five-year survival of 60%.12 Worldwide survival rates have been reported as 76%, 56% and 43% at one, five and 10 years, respectively, which includes both adult and paediatric patients.13
The naturally occurring human glucagon-like peptide-2 (GLP-2) is secreted by L-cells of the intestine. Teduglutide is an analogue of GLP-2 and a designated orphan drug. In non-clinical studies, it has been shown to increase villous height and crypt depth,14 thereby increasing the intestinal absorptive surface area. Carter et al15 conducted a 12-week open-label study that included 42 patients aged 1–17 years with SBS and who were PN-dependent for at least one year.
Participants either received teduglutide 0.0125mg/kg/day (n=8), 0.025mg/kg/day (n=14), 0.05mg/kg/day (n=15), or standard of care (n=5). Patients in the teduglutide group required PN on seven days of the week at baseline.
Four patients were weaned off PN by week 12 in the teduglutide group (0.05mg/kg, n=3; 0.025mg/kg, n=1), and two of these patients restarted PN after a four-week wash-out period. The greatest reduction in mean prescribed PN volume was seen in the 0.05mg/kg and 0.025mg/kg teduglutide groups. Improvement in enteral nutrition was seen in all four groups, and was maintained after teduglutide was discontinued.
Teduglutide has recently been approved for use in the EU for children with SBS aged one year and above, administered as a subcutaneous injection at a dose of 0.05mg/kg once daily. The cost of 28 vials (5mg strength) is approximately £15,000.
A double-blind, controlled trial is currently being conducted in children with SBS who are PN-dependent.16 They are randomised to subcutaneous teduglutide 0.05 or 0.025mg/kg/day, or standard of care for 24 weeks. The primary outcome measures are safety and tolerability of teduglutide treatment, and at least 20% reduction in PN volume from baseline. The estimated completion date of this study is June 2017.
In the future, tissue engineering may provide further treatment options in children with SBS. However, current management of these patients can be optimised with a multidisciplinary approach to promote intestinal adaptation, maintain growth and development and prevent potentially life-threatening complications. The ultimate goal is to achieve enteral autonomy and improve the overall quality of lives of these children and their families.
Key points
Jaini Shah MPharm PG Dip GPP
Highly Specialist Pharmacist – Paediatric & Neonatal Nutrition, The Royal London Hospital, Barts Health NHS Trust, London, UK
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