Feeding strategies should take into account the often multifactorial causes of poor nutrient intake. When oral intake is significantly reduced because of persistent nausea or vomiting or the discomfort of pruritis or unpalatable feeds, supplemental tube feeding may be required. This can be given during the day as bolus feeds and/or as a continuous overnight infusion. Where there is ascites or organomegaly, high-energy, low-volume feeds may help with the problem of reduced gastric capacity. With ascites, a rigid sodium restriction is not recommended, but rather a general reduction is salty foods. In hospital, extended periods of nil by mouth and feeding interruptions due to investigations and procedures should be kept to a minimum, and every effort should be made to make meal times enjoyable.

Where there is fat malabsorption, an energy dense diet is given combined with medium-chain triglyceride (MCT) supplementation. MCTs do not require emulsification with bile, diffusing easily into the intestinal cells, where they are absorbed directly into the bloodstream. There is little evidence regarding how much dietary fat should be MCT. MCTs are not a source of essential fatty acids (EFAs), and as the percentage of MCT increases it is more difficult to meet requirements for EFAs. The European Society for Gastroenterology, Hepatology and Nutrition (ESPGHAN) guidelines are that infant formula should contain 4.5–10.8 % energy as linoleic acid, and the ratio of alpha-linoleic:linolenic should be 5–15:1 [10]. The majority of feeds which are currently available in the UK provide approximately 50 % of the fat as MCT and meet requirements for EFAs (refer to Table 72.2). If an MCT feed with a particularly high MCT content, for example, Emsogen®, is used for a prolonged period of time, supplementing with a source of EFAs is warranted, for example, walnut oil supplemented at 1 ml/100 kcal. In cases where mucosal edema due to portal hypertension may be contributing to malabsorption, it may also be beneficial to give hydrolyzed protein to maximize absorption. Where there is a low stool elastase indicating pancreatic insufficiency, supplementation with pancreatic enzymes may be beneficial.

Dietary strategies are centered on trying to preserve lean body mass, avoid hypoglycemia, and minimize hyperammonemia.

Due to increased resting energy expenditure and nutrient malabsorption, energy and protein requirements for children with chronic liver disease are generally well above the estimated average requirement (EAR). Table 72.3 shows that children may need up to 160 % of the EAR for energy and between 3 and 4 g/kg protein for adequate growth [15–17, 20, 21].

Estimations of energy and protein requirements are used only as a guide; requirements are calculated based on the individual, taking into account age, gender, nutritional status, disease state, and growth. Calculated requirements are not static and should be recalculated as part of monitoring procedures.

To increase the nutrient density of a powder-based formula, the concentration can be increased beyond the standard recommended dilution, usually in 2 % increments. For example, the concentration of Heparon Junior® could be increased in two stages from 18 to 22 % which would increase the energy from 86.4 to 105.6 kcal/100 ml. Feeds should be concentrated cautiously given that as the concentration increases so too do all the micronutrients and macronutrients, the renal solute load, osmolality, and the risk of osmotic diarrhea. When concentrating feeds, explicit instructions should be provided to the carers to avoid errors when making up feeds.

Nutrient dense feeds can be given orally or via feeding tube. Not all of the feeds provide complete nutrition; some supplements contain no fat (e.g., Fortijuce®) or incomplete micronutrients (e.g., Scandishake®) and should be given alongside a balanced diet. See Table 72.4 for examples of standard high-energy sip and tube feeds .

The addition of nonprotein energy to the diet can affect the overall balance of a feed or meal. When adding energy, it is important to maintain a protein:energy ratio of between 7.5 and 12 % for infants and between 5 and 15 % for older children [22]. Energy can be added in the form of glucose polymers, fats, or a combination of the two (refer to Table 72.5). These supplements are usually added in 1 % increments and increased daily depending on tolerance. As a guide, the total carbohydrate concentrations recommended are 10–15 % for infants and up to 30 % in older children. For fat, recommendations are 5–6 % for infants and 7 % for older children [22].