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Info About Cystic Fibrosis

Cystic Fibrosis (CF) is the most common life threatening genetic condition in Australia. CF affects many of the body’s systems, including lungs and digestion. Improved medication and treatments have seen life expectancy extend considerably. 

Babies are born with the disease. CF is an inherited genetic condition, and both parents have to carry the gene. 

For a child to be born with CF both parents must carry the CF gene; they usually do not know they are carriers of the CF gene. One CF gene from each parent will result in a pregnancy with a : - One-in-four chance a child will have CF - Two-in-four chance a child will not have CF – but will carry the CF gene - One-in-four chance each baby will NOT have CF and NOT be a CF carrier

In cystic fibrosis there is a problem with the protein that controls the movement of salt in and out of the cells. Too much salt in the cells causes mucus to be very thick and sticky and to build up in organs like the lungs. The mucus clogs the tiny air passages in the lungs and traps bacteria. Repeated infections and blockages can cause irreversible lung damage and death. Mucus can also cause problems in the pancreas, preventing the release of enzymes needed for the digestion of food. This means that people with CF can have problems with nutrition. 

People with CF may have the following symptoms: 

persistent cough, particularly with physical effortsome difficulty in breathing or wheezing with efforttiredness, lethargy or an impaired exercise abilityfrequent visits to the toiletsalt loss in hot weather which may produce weaknesspoor appetite. 

Treatment for CF can be intensive and time consuming. At present there is no cure for CF and treatment is therefore aimed at slowing progression of the condition.

Treatment has improved greatly in recent years and advances continue to be made. Studies have proved that regular attendance at a major CF centre or clinic is beneficial. This enables people to experience the expertise of the CF team which includes doctors, physiotherapists, dieticians, social workers and nurses.

The treatment for lung problems includes:

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.

It is common for people with CF to encounter some difficulties with their lungs. A combination of airway clearance techniques and medication can help control lung infections and prevent lung damage. To avoid the risk of cross infection, people with CF need to ensure they do not come into close contact with others with CF.

CF affects the pancreas (which produces the enzyme needed to digest food) and makes it difficult for people with CF to absorb food. This can cause malnutrition, which can lead to poor growth, physical weakness and delayed puberty. There is medication that can compensate for the failure of the pancreas.

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

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

Some people may also require supplementary vitamins.

In older patients, insulin production can become deficient due to increasing pancreatic disease.

Some develop CF related diabetes and their blood sugar levels are no longer controlled. However, this rarely happens to children with CF. Common symptoms of diabetes include thirst, hunger, weight loss and excessive need to urinate, but some people do not show obvious symptoms of diabetes.

Types if Cystic Fibrosis:

Pneumothorax related to cystic fibrosisNasal polps related to cystic fibrosisChronic sinusitis related to cystic fibrosisMeconium ileus related to cystic fibrosisIntussusception related to cystic fibrosisUndescended testis associated to cystic fibrosisHydrocele associated with cystic fibrosis

Rare Types of Cystic Fibrosis:

Nasal polyps related to cystic fibrosisPancreatic insufficiency related cystic fibrosisSinusitis related cystic fibrosisBronchiectasis related cystic fibrosisIntussusception related to cystic fibrosisUndescended testis associated to cystic fibrosisHydrocele associated with cystic fibrosis