Diseases

Cystic Fibrosis

Lung disease

Cystic fibrosis (CF) is a hereditary congenital disease, in which there is a defect in the production of mucus in the body. The mucus is tougher and more saline and can accumulate in the lungs. This accumulation can cause inflammation and recurrent respiratory infections. CF is a serious and incurable disease, which is eventually fatal.


Cause

Cystic fibrosis is caused by a defect of the genes. The child has to get an abnormal gene from both the father and mother to get sick. The father and mother are carriers of the CF gene, without being sick. If both parents are carriers, their children have twenty-five percent chance of getting CF.

Due to the defect, some organs discharge thick and tough mucus. Mucus normally carries waste products, such as inhaled dust particles and bacteria. It also transports digestive substances from the pancreas to the intestines. If the mucus is tough, these functions are in danger. Several organs operate less well. Apart from the lungs, the pancreas and the liver may also be affected by the disease. CF is not contagious.


Symptoms

The first problems often arise at young age. The main signs and symptoms are:

Not all symptoms occur simultaneously. Over the years, the symptoms generally become worse. Due to loss of function of the pancreas, a special type of diabetes may arise.


Diagnosis

The diagnosis of cystic fibrosis is usually made in the first two years of life. If the doctor suspects that the patient has CF, several tests can be done:


Treatment

The treatment of CF takes place in three different areas:

Treatment is important for the rest of the patient’s life. In case of severe lung damage, lung transplant is the last solution.


Prognosis

CF is not curable yet. It’s a delibitating disease, because the symptoms are becoming more severe with age. At this moment, the average life expectancy for patients is about forty years. Life expectancy can vary by patient, because the progression of the disease is different in every person. By intensive (medical) care, the quality of life of CF patients has nowadays greatly improved.


Considerations

In people with CF, the bacterium Pseudomonas aeruginosa (PA) causes most of the respiratory infections. This bacterium lives mainly in a warm, humid environment. For example, in the water well of the shower or bath, the drain of the toilet and the sink, in coughed up phlegm and in dishcloths. But also in lakes and rivers. The following precautions may be wise:


Facts


See also

Diabetes Mellitus

Biopsy

DNA Test

Intestinal Current Measurement

Sweat Test

Healthy Food

Physical Exercise

Vitamin

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