Pancreatic insufficiency and nutritional support

• Preventative counselling - high fat, high calorie healthy diet
• Pancreatic Enzyme Replacement Therapy (PERT)
• Salt tablets and vitamins
• Dietary Supplements
• Artificial feeding with gastrostomy (PEG) tube or button gastrostomy

90% of children with cystic fibrosis have pancreatic insufficiency, this means they are unable to extract enough nutrition from their diet without medical help. The level of intervention required is based on assessment of weight for height as a percentage of normal. This table shows what type of nutritional support is required by weight/height assessment, details of treatments are listed below: Children who are well nourished have better outcomes and better lung function during childhood.

Preventative counselling - high fat, high calorie healthy diet

Typical energy needs for a child with cystic fibrosis are higher than for a normal child, in fact up to 150% of normal. This means for every 2 meals a healthy child needs a child with cystic fibrosis needs 3 to maintain normal growth and development. A healthy diet for a child with cystic fibrosis would be unhealthy for a normal child. Children with cystic fibrosis should not eat diet foods, should eat full fat versions of milk and dairy products. Ordinary meals may need to be bulked up with fat and calories by adding extra butter or fats. Homemade milkshakes and high calorie meals and snacks including fast food can be very helpful. As this type of diet is very different to the notion of a healthy diet careful education of the parent/carer and child are required. It is also important to ensure the child is having the recommended intake of dietary calcium. 90% of children have pancreatic insufficiency and will require the following supplements or treatments:

Pancreatic Enzyme Replacement Therapy (PERT)

Pancreatic enzymes are not produced and so must be taken by mouth every time fat containing foods or drinks are consumed. They should be swallowed prior to the meal and cannot be sprinkled on food. The capsules are large and can either be swallowed whole or opened and the granules mixed with the first mouthful of food. A further dose might be needed during a meal. If not enough PERT is taken symptoms of fat malabsorption including bloating, flatulence and diarrhoea will be present. Most children will take Creon 10,000 capsules and typical doses are listed below: (Approximately one 10,000 unit capsule per Kg of body weight per day but this varies greatly between patients). Babies and toddlers can take creon micro (5,000 units per scoop). This is usually administered on a teaspoon with a drop of milk or puree food.

Brand names of PERT

Pancreatin is a mixture of enzymes including lipase, protease and amylase used in pancreatic insufficiency. There are several brands that provide pancreatin in different strengths. Any of them may be used although Creon is prescribed most commonly. Brand names include:

• Creon Micro 5000 per scoop Creon 10,000, Creon 25,000, Creon 40,000
• Nutrizym 10, Nutrizym 22
• Pancrex, Pancrex V
• Pancrease HL

Salt tablets and vitamins

Salt depletion may be treated with salt supplements, brand name: Slow Sodium. Chronic salt deficiency may be severe in children with cystic fibrosis and can be a cause of failure to grow – this is called ‘Pseudo-Bartter Syndrome’.

Vitamin supplements (A, D, E, K) – children are likely to take multivitamins such as Abidec or Dalivit that contain vitamins A to D and separate preparations of vitamins E and K (menadiol).

Dietary Supplements

The principle of supplements is to provide concentrated calories in a palatable form. There are many flavours and presentations:

• High energy milk-based sip feeds, e.g. Paediasure Plus, Fortini, Frebini Energy,
• High energy fruit juice based feeds, e.g. Enlive Plus, Fortijuice, Provide Xtra
• High energy infant milks, e.g. SMA High Energy, Infatrini
• Powdered energy sources, e.g. Maxijul and Caloreen (carbs), Calogen and Liquigen (fats) and a mixture of fats and carbs – Duocal

Pancreatic enzymes need to be taken with most but not all of these. Powdered energy sources require mixing before use and the others can be drunk straight from the carton with a straw. Babies can be fed with a bottle. Children with loss of appetite who struggle to eat meals are likely to struggle with supplements as well.

Artificial feeding with gastrostomy (PEG) tube or button gastrostomy

Children who fail to gain weight despite supplements or who are very underweight will have artificial feeding. Getting their weight up reduces the number of chest exacerbations and improves their ability to recover from these. It can improve well-being and energy levels and may be used over the short or longer term. A Percutaneous-Endoscopic- Gastrostomy (PEG) tube is an indwelling thin plastic tube from the abdominal wall to the stomach. This is connected to a feed pump overnight and improves calorie intake without reducing appetite for food during the day which can be problem with supplements. Enzymes need to be taken beforehand but children should not be woken during the night to take a further dose of enzymes. Some feeds do not need to be taken with enzymes. Brand names of feeds include:

• Infant feeds – SMA high energy, Infatrini, Neocate, Pregestimil (enzymes not required)
• Older children – Paediasure Plus, Nutrini, Tetrini, Frebini, Emsogen (enzymes not required)