Cystic Fibrosis

Why is nutrition important for individuals with Cystic Fibrosis?

Due to the build-up of sticky mucus in the lungs, there is an increase in the risk of lung infections, inflammation, and other complications with this group. People with cystic fibrosis need to eat more calories to cope with the breathing difficulties and fight off infection¹.  Mucus also clogs the pancreas and stops the release of enzymes (juices that help digestion). This affects how the food travels through the gut, is broken down (particularly fat, protein, and vitamins), digested, and nutrients absorbed. Therefore, children with cystic fibrosis may have difficulty growing and gaining weight².
 

What symptoms are associated with Cystic Fibrosis that can cause reduced dietary intake?

Symptoms of cystic fibrosis include:

• frequent chest infections
• wheezing, coughing, difficulty breathing
• difficulty gaining weight and growing
• yellowing of the skin and the whites of the eyes (jaundice)
• diarrhoea, constipation, or large, smelly poo
• a bowel obstruction in new-born babies (meconium ileus) – surgery may be needed

These symptoms can often result in poor dietary intake so it is important for children with cystic fibrosis and their families to work with a dietitian to ensure that all of their nutrition requirements are being met.

How to help prevent and/or manage malnutrition?

There is no cure for cystic fibrosis, however people are cared for by a team of specialist healthcare professionals. The care provided helps to prevent lung infections and reduce symptoms. As already discussed, the mucus blocks the pancreas which can affect the absorption of nutrients. Therefore, an adequate, balanced diet is vital. Cystic fibrosis can result in the needs for energy increasing by 100-200% and this increases as lung function drops³. To prevent or manage problems with growth and to stay healthy, a dietitian may advise a high-calorie diet and a vitamin and mineral supplement. Increasing calorie intake can be difficult, if a person is feeling unwell, tired or has a poor appetite. Specialist cystic fibrosis dietitians are part of every specialist hospital team for this patient group. The prescription of enzyme capsules may also be part of the care plan to help the digestion of nutrients. Treatments can also include medications to treat infections, thin the mucus, widen airways (physiotherapist also provide support with this) and removal of small growths (polyps) in the nose. 
 

Ways to use Pro-Cal™ powder and/or Pro-Cal shot™ to increase nutritional intake of children with cystic fibrosis?

If a person’s weight is lower than recommended, the body has less energy to fight infection and improve lung function. Therefore, a healthy body weight can help support you if there is any sudden worsening of lung health. For a person with cystic fibrosis, an extra 1000kcal or more daily may be required for weight gain. Adding extra nutrients to foods and fluids is a practical way to increase calories, without increasing volume excessively. Pro-Cal shot can be taken as a low volume shot or added to food and fluid e.g., desserts, milk, cereals, scrambled egg, baked goods etc.

• Pro-Cal shot can also be frozen into ice cubes or lollies
• Pro-Cal shot and Pro-Cal powder can also be given via tube feeding as advised by a healthcare professional
• Pro-Cal powder can be sprinkled into foods and drinks e.g., mashed potato, soup, hot chocolate etc.

Top Tips to aid dietary intake:

• Adding full fat dairy products, sugary products, eggs and other fats and oils to meals and snacks.
• Check labels for higher calorie options when food shopping. It is easier to check food and fluid’s nutritional information using per 100g or 100ml to compare foods and fluids. However, also check the advised portion size and think about how much you would eat and drink as a portion.
• If you are finding managing your diet difficult, contact your specialist team to support you.
• Plan your meals and snacks to make sure you are including all your oral nutritional supplements (e.g., Pro-Cal shot, Pro-Cal powder).
• Set reminders for yourself for meals and snacks if your appetite is low.
• The Cystic Fibrosis Trust is another great source for tips and useful information.⁴

References:

1. National Health Service (NHS) Overview Cystic Fibrosis https://www.nhs.uk/conditions/cystic-fibrosis/
2. British Lung Foundation (BLF) What is cystic fibrosis? https://www.blf.org.uk/support-for-you/cystic-fibrosis/what-is-it
3. Cystic Fibrosis Foundation (CFF) What is cystic fibrosis?  https://www.cff.org/intro-cf/about-cystic-fibrosis
4. Cystic Fibrosis Trust https://www.cysticfibrosis.org.uk/

This piece was written in conjunction with a specialist cystic fibrosis dietitian from the UK. 

Pro-Cal shot and Pro-Cal powder are Foods for Special Medical Purposes and must be used under medical supervision. Suitable from 3 years of age onwards. Pro-Cal shot contains Milk (Milk Protein, Lactose) and Soya (Soya Lecithin). Pro-Cal powder contains Milk (Milk protein, lactose)