Cystic Fibrosis

Bronte Short

What Cause Cystic Fibrosis?

Cystic fibrosis, is an autosomal recessive hereditary disease that mostly affects the lungs, pancreas, liver,

intestines, sinuses, and sex organs.

A defect in the CFTR (Cystic Fibrosis Transmembrane Regulator) gene causes cystic fibrosis.

Scientific Name.Is it infectious?

Cystic Fibrosis (CF) is also known as mucoviscidosis.

Cystic Fibrosis is not infectious it can only be passed genetically. Every person inherits two CFTR genes, one from each parent. Children who inherit a faulty

CFTR gene from each parent will have Cystic Fibrosis.

Symptoms

The symptoms of Cystic Fibrosis vary from person to person and over time. Sometimes people will have few symptoms. Other times, symptoms may become more

severe. CF symptoms include:

• Persistent cough, particularly with physical effort • Some difficulty in breathing or wheezing with physical

effort • Tiredness, lethargy or an impaired exercise ability.

• Frequent visits to the toilet. • Salt loss in hot weather which may produce weakness.

• Poor appetite

One of the first signs of cystic fibrosis that parents may notice is that their baby's skin tastes salty when kissed or the baby doesn't pass stool when first born. Most of

the other signs and symptoms of CF develop later. They are related to how CF affects the respiratory,

digestive, or reproductive systems of the body.

People who have Cystic Fibrosis have thick, sticky mucus that builds up in their airways. This buildup of mucus makes it easier for bacteria to grow and cause infections. Infections can block the airways and cause frequent coughing that brings up thick spit or mucus

that's sometimes bloody.

Treatment for cystic Fibrosis.Cystic fibrosis has no cure. But treatments have greatly

improved in recent years. The goals of Cystic Fibrosis treatment are to:

• Prevent and control lung infections • Loosen and remove thick, sticky mucus from the lungs

• Prevent or treat blockages in the intestines • Provide enough nutrition

• Prevent dehydrationDepending on how severe the disease is, patients may

be treated in a hospital.

As treatments for CF continue to improve, so does life expectancy for those who have the disease. Today,

some people who have CF are living into their forties, fifties, or older.

Early treatment for CF can improve both your quality of life and lifespan. Such early treatment includes nutritional and respiratory therapies, medicines,

exercise, and other treatments.

Immunisation.Is It Life Threatening?

There is no immunisation to prevent cystic Fibrosis. It is a genetic disease.

You cannot recover from Cystic Fibrosis, there is no cure.CF is life threatening, the average age for people to live to

is 35 though some people do live till their 40’s and 50’s or older.

The faulty gene has been identified and doctors and scientists are working to find ways of repairing or

replacing it. With today’s improved treatment most people with CF are

able to lead reasonably normal and productive lives. A great amount of time is being directed towards finding

new and improved ways of treating CF and finally finding a cure.

Is CF a common disease?

CF is the most common life threatening, recessive genetic disease affecting Australian children and young adults

today.There are approximately 3,000 people living with CF in

Australia. There are a million genetic carriers of CF in Australia.

About 30,000 people in the United States have CF. It is one of the most common inherited diseases among

Caucasians.

About 1,000 new cases of CF are diagnosed each year.

Extra Facts• 80% of patients are diagnosed by the age of three.

• Over 90% of the affected infants now survive beyond one year.

• People with CF may need to have up to 2 hours intensive chest physiotherapy (to help break up the

mucus in their lungs) and up to 40 enzyme tablets (to help their digestion) every day just to survive.

• In Australia all babies are screened for CF

Bibliography

• www.nhlbi.hin.gov

• www.cysticfibrosis.org.au

• www.healthinsite.gov.au