Paediatric Cystic Fibrosis (CF) – diet in children with CF

A guide to eating well for children with CF

Eating well is important for everyone, and is especially important for children with CF.

Having a well-balanced and varied diet will help your child get the nutrients they need to stay healthy and grow, and prevent vitamin or mineral deficiencies.

The balanced diet (Eat Well Guide)

The Eatwell Guide is an example of a balanced diet; offer your child a variety of food from each of the main food groups each day.

The proportions needed for children with CF will vary; this is because some children with CF have higher energy requirements compared to their peers.

Potatoes, bread, rice, pasta and other starchy carbohydrates

Starchy foods release energy gradually, include a portion of these with each meal.

Fortified breakfast cereals are a good choice as many have added B vitamins and iron and some have added vitamin D and calcium.

Beans, pulses, fish, eggs, meat and other proteins

Protein foods include meat, fish, eggs, nuts, beans and pulses and plant based alternatives, such as Quorn or soya mince. Many of these are also good sources of zinc and iron and Vitamin B12.

Include a portion of protein food with each main meal. If you eat fish, aim to have two portions a week, one of which should be naturally oily such as salmon, or mackerel.

Milk and dairy foods

Milk and dairy foods such as cheese and yogurt contain calcium and protein as well as vitamins A and D, these nutrients that are important for bone development.

Children over one year old can have full fat cow’s milk as their main drink. Children over two years old who are growing well can use semi skimmed milk. Plant-based milks can be used if needed; your dietitian will be able to advise you on the most suitable alternatives.

Fruits and vegetables

These provide vitamins and minerals and are an excellent source of fibre. Aim for five portions a day, try to include both a vegetable and a fruit at mealtimes. A portion of fruit or vegetables is equal to the amount that fits into the palm of your child’s hand.

Choose fresh, frozen, canned (in their own juice), dried or juice or smoothies. Fruits and vegetables can make useful snacks.

Fat

Fats are a good source of energy, they contain essential fatty acids needed for the body and brain to function, and are a source of fat-soluble vitamins (Vitamins A, E, D and K).

There are three main types of dietary fat, saturated, monounsaturated, and polyunsaturated fats. Monounsaturated and polyunsaturated fats can help protect against heart disease, so choose these where possible.

Saturated fats

Found in meat, butter, cream, palm oil and coconut oil

Monounsaturated fats

Found in olive and rapeseed oils, avocado, seeds and nuts such as hazelnuts, almonds and pecans.

Polyunsaturated fats

Found in oily fish such as salmon, mackerel and sardines, nuts such as walnuts, almonds and peanuts and vegetable oils such as sunflower oil.

Omega 3 polyunsaturated fats

Found in olives, oily fish, nuts, seeds and rapeseed oil, these are especially good fats to include in your child’s diet as these may also help brain development. They can possibly play a role in reducing inflammation in cystic fibrosis.

Calcium

Calcium requirements are higher in children with CF as there is a greater risk of them developing weaker bones later in life, due to either malabsorption or the side effects of some medications.

Children’s calcium requirements increase, as they get older. Children under eight years old should aim for at least three portions of dairy foods a day, children over eight should aim for at least four to five portions of dairy foods a day. A calcium supplement may be advised if required.

Iron

Iron is especially important in making a component of red blood cells called haemoglobin, this helps to carry oxygen to every cell of the body. Iron deficiency anaemia can make your child feel tired and breathless, low in energy and irritable, and can reduce their appetite.

Children with CF are at greater risk of iron deficiency (anaemia), this can be due to malabsorption or not having enough iron in the diet or having an infection or as a side effect of some medications.

Try to include a number of sources of iron in your child’s diet each day. The best sources of iron are red meats such as beef or lamb, other sources of iron include fortified breakfast cereals, pulses and beans, eggs, whole grains, nuts, green leafy vegetables and dried fruit.

Iron from red meats is more easily absorbed than iron from other sources; therefore, children following a vegetarian or vegan diet are at more risk of iron deficiency. Vitamin C helps the body absorb iron; offer your child a glass of fruit juice or some fruit or vegetables at the same time as iron rich foods.

Vitamins and minerals

Including a variety of foods from each of the main food groups each day will help to make sure your child’s diet provides the vitamins and minerals they need to stay healthy.

Children with CF who need to take enzymes (Creon) will also need to take a fat-soluble vitamin supplement each day, containing vitamins A, D, E, and K, as these vitamins are not always fully absorbed.

Children who do not need to take enzymes and who are under 5 year old should take a vitamin supplements containing vitamins A, C and D every day, (unless they are having over 500mls infant formula). Your child’s fat-soluble vitamin levels will be checked each year at their annual review.

Fibre

Fibre is a type of carbohydrate not digested by the body. In CF a good intake of fibre and fluid is especially important to help keep bowel movements healthy, to help to prevent constipation or DIOS (distal intestinal obstruction syndrome).

DIOS is unique to cystic fibrosis, it occurs when the bowel becomes partially or completely blocked and causes symptoms such as stomach pain, bloating and nausea and needs special medical treatment.

Include foods that are good sources of fibre in your child’s diet each day. These include wholegrain starchy foods such as wholemeal bread, pasta or breakfast cereals, as well as fruit and vegetables. Having too much fibre can reduce your child’s appetite; especially in younger children, so try to have a mixture of high fibre foods and lower fibre alternatives.

Salt

Children with CF will lose more salt through their sweat, especially during hot weather or when exercising. Children can get extra salt in their diet by eating salty foods such as cheese or ham, or salty snacks such as crisps, or by adding extra salt during cooking or at the table.

Salt supplements are usually recommended during heat waves, or when travelling abroad, during intense exercise or exercising more than an hour, or if your child sweats a lot or if you notice the presence of salt crystals in their hairline.

Symptoms of salt depletion include tiredness, lethargy, loss of appetite, muscle cramps, poor concentration and sunken eyes.

Fluid

Children with CF need a good intake of fluid, this is especially important in hot weather and while exercising. Having a good intake of fluid and keeping well hydrated can help keep the mucus in the lungs thinner. Having a good intake of fluid and fibre helps to keep food moving through their gut and helps to prevent constipation and DIOS.

The following is a guide to fluid amounts recommended for different ages:

1-3 years 1200ml 5 cups 5-10 years 1800ml 7 cups

3-5 years 1500ml 6 cups >10 years 2300ml 8-10 cups

Your child’s urine is a good indicator of their hydration. Ideally, it should be a pale straw colour with no smell; darker, stronger smelling urine suggests your child needs more fluid.

A healthy balance

Children with CF who are a healthy weight tend to have better lung function, fewer chest infections, stronger bones and better long-term health.

Having three balanced meals a day with two or three small snacks in between meals is usually sufficient to meet the energy requirements associated with CF.

If your child is gaining weight and growing well, try to limit processed foods such as biscuits, sweets and chocolate, crisps and sugary drinks, as these are high in fat and sugar and provide extra energy but few nutrients.

Offer healthier snacks such as fruit or dried fruit, or nuts (over four years), breakfast cereal, yoghurts, fromage frais, crackers, crumpets or toast with peanut butter or cheese.

High-energy diets

Sometimes children with CF need extra energy to fuel their body and extra protein to help them grow and to repair their cells, for example:

• When your child is unwell, or has a chest infection and the lungs are working hard, to help fight infection and to prevent weight and muscle loss.
• If your child is underweight, losing weight or not growing well.

If your child is unwell or needs to gain weight, offering foods high in fat and sugar will help them get the energy they need. If your child has a small appetite offer them smaller portions initially, then offer a second course or extra helpings if these are needed.

Offer full fat dairy foods such as a milk, yogurt and milky puddings or custard. Try to include foods high in unsaturated fats such as olives, nuts or nut butters, avocado and use rapeseed, olive or sunflower oils or spreads.

Add extra energy to savoury dishes by grating cheese on top or adding extra butter or margarine or oil to toast or sandwiches, vegetables or potatoes or pasta (use olive, rapeseed or sunflower oils spreads where possible).

Higher energy drinks be useful, such as full sugar squash, full fat milk or milkshakes, hot chocolate or malted milk.

Nutritional supplements

Your dietitian may recommend a nutritional supplement to boost your child’s intake. These are high in energy and are available as milk, yoghurt and juice style drinks.

Schools and nurseries

Most schools and nurseries encourage children to eat fruit at break time and offer healthy meal options at lunch. Children with CF should be encouraged to eat the same foods as other children. If more energy dense snacks are needed these can be eaten at home.

Enzymes (Creon)

If you child takes enzymes, it is important they take the appropriate amount with food and milky drinks to ensure the nutrients are absorbed; this helps with weight gain and growth.

Signs of not having enough enzymes include

• More frequent or, pale, loose, oily or offensive smelling stools, wind or abdominal pain.
• Poor weight gain, or poor growth over time; this can happen even if a change in bowel movements is not seen, this is sometimes called low level malabsorption.
• An increased appetite, as children will eat more to compensate for malabsorption.

Modulators

Some children with CF take medications called modulators; examples include Orkambi, Kalydeco or Kaftrio. These medications must be taken with a food or a drink that contains fat. If your child takes enzymes, these must be given at the same time as the modulators.

Modulators should be taken approximately 12hrs apart, for example at breakfast time and with an evening meal or bedtime snack. Many children find that once they start taking modulators their health improves, they may also start gaining more weight. Some children taking modulators need to modify their diets to avoid gaining excess weight.

My child is not eating well

When young children are not eating well, meal times can be stressful. If your child has CF, this can be particularly challenging.

Offer meals and snacks at regular times, this way your child learns to expect when they are going to eat.

Eat together as a family as often as possible, aim to make mealtimes a pleasant and social experience.

Remember, your child will copy your behaviour; if you eat well and enjoy your food, your child has an excellent role model.

If you require any further information, please contact your dietitian

Tel: 01223 216655