Cystic fibrosis is a relatively common hereditary or genetic disease, which can have a devastating effect on health.

Today survival of this previously lethal genetic disorder has improved greatly due to early detection and the use of intensive treatments, including nutrition interventions.

People with cystic fibrosis need to be under the management of a medical team that usually includes a physician, a physiotherapist and a dietician.

Medical treatment may include all of the following; antibiotics (oral and inhaled) to treat respiratory and other infections, inhalants to assist with breathing, physiotherapy to help move mucus out of the lungs and other organs.

Also, additional foods by mouth or tube, particularly when the person has to be hospitalized are needed.

Diet therapy in cystic fibrosis is one of the most important aspects of treatment to ensure normal growth and development.

In case a member of the family is affected by this genetic disease, then it’s important to consult a clinical dietician to assist you with the correct diet for various stages of your life.

For example to meet the increased requirement for energy and protective nutrients during childhood and the teenage years, this helps to increase resistance to infections and supply enough energy for the body needs despite poor digestion and absorption.

It is important to mention that due to problems associated with blocked pancreas and liver ducts, people with cystic fibrosis do not have sufficient enzymes and bile salts in the small intestine to absorb enough energy from foods for their daily needs.

Chronic under nutrition and feeding is one of the most common features of this disease and diet therapy must aim to boost energy intake.

If the patient has no appetite, is underweight and experiences a host of symptoms that interfere with eating, caretakers are faced with a seemingly insurmountable problem.

These individuals often have salty sweat, while their fingers may have clubbed ends and look slightly blue. They are also sensitive to heat exhaustion and dehydration.

In some cases, cystic fibrosis can lead to sterility in males. Diabetes and liver complications are also common.

Problems with digestion caused by a lack of digestive enzymes can lead to poor absorption of fat, abdominal pain and even intestinal obstruction. These individuals may also suffer from reflux and heartburn, vomiting caused by excessive coughing, loss of appetite, constipation and bloating.

However, there are ways of ensuring that people with cystic fibrosis have an adequate energy intake. One solution is to increase the fat content of the diet.

In contrast to most people who need to cut down on fat intake, cystic fibrosis sufferers should be encouraged to add cream or butter to foods such as cereals, potatoes, desserts and fruit. They should also use full cream milk and dairy products instead of the fat free varieties.

High energy snacks between meals can also assist to increase the energy density of the diet  for example full cream yoghurt, cream cheese or ice cream, bread with butter, jam and peanut butter, milk shakes made with full cream milk, pureed fruit and honey, or cheese with whole wheat crackers.

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