Cystic Fibrosis

What is Cystic Fibrosis?
Cystic Fibrosis (CF) is an inherited genetic condition that mainly affects the lungs, digestive system and the sweat glands.  It is the most common life threatening genetic condition affecting Australian children.

How common is CF?
Amongst people of Caucasian ancestry 1 out of 25 are genetic carriers for CF.  If two people are genetic carriers for CF and they have a child there is:
• a 1 in 4 (25%) chance that the child will have CF
• a 2 in 4 (50%) chance that the child will be a genetic carrier
• a 1 in 4 (25%) chance that the child will not have CF and will not be a genetic carrier for CF

How does CF affect the body?
CF affects the exocrine glands, which secrete body fluids such as sweat, mucus and enzymes.

Lungs
People with CF produce abnormally thick sticky mucus which blocks small air passages in the lungs, causing difficulty in clearing infections and can result in lung damage over a period of time.

Digestion
In CF the sticky mucus can make it difficult for enzymes produced by the pancreas to reach the digestive system and as a result food that is eaten cannot be fully digested.  Children with CF may therefore have difficulty in gaining weight and the undigested food results in large bulky bowel actions.

Sweat Glands
People with CF do not sweat more than other people but they do lose more salt and potassium in their sweat.

What are the symptoms of CF?
People with CF may have the following symptoms
• Persistent cough, particularly with physical effort
• Some difficulty in breathing or wheezing with effort
• Tiredness, lethargy or an impaired exercise ability
• Frequent visits to the toilet
• Salt loss in hot weather which may produce muscle cramps and weakness
• Poor appetite

How is CF Treated?
Treatment for CF can be intensive and time consuming.  At present thee is no cure for CF and treatment is therefore aimed at slowing the progress of the condition.

Lungs
Treatment for lung problems include:
• Chest physiotherapy
• Antibiotics
• Inhalations via compressed air pump and nebuliser

Most people will require this treatment on a regular daily basis and some may require aerosol medications to assist breathing.  Exercise is encouraged on a regular basis to maintain a healthy lung capacity.  Sport and aerobic exercise are recommended for all age groups of people with CF.

Digestion
To improve absorption of food most people with CF require enzyme replacement capsules with meals and snacks.

To improve nutrition and maintain bodyweight a well balanced diet high in protein, fat and calories is required.

People with CF will also require supplementary vitamins

Sweat Glands
Salt supplements and additional fluids are required in hot weather, during strenuous exercise or in cases of fever.