Symptoms of Cystic Fibrosis

The type and severity of CF symptoms varies from person to person. In some people not identified with CF through newborn screening, there may be health problems that indicate the presence of CF, such as:

Salty-tasting skin
Slow weight gain, failure to thrive, even with good appetite
Wheezing, coughing, pneumonia
Abnormal bowel movements

These symptoms are not unique to CF, but when many of these symptoms are present a doctor may want to do a sweat test for CF, to obtain a conclusive diagnosis.

Lungs

Almost everyone with Cystic Fibrosis will sooner or later develop lung disease. However, when it occurs and how bad it is differs from person to person.

The thickened mucus builds up in the bronchioles and reduces airflow, making the patient short of breath and wheeze with exercise. This mucus can build up to such an extent that it plugs the smaller airways of the lungs and can then harbor bacteria which cause infection.

Effective Airway Clearance Techniques are required to help remove this mucus reducing the chances of infection.

See Treatment-Physiotherapy for more information on airway clearance techniques.

Digestive System

Cystic Fibrosis mainly affects the digestive system by mucus blocking the pancreas, stopping the digestive enzymes produced from making their way to the gut to digest food.

This complication can cause malnutrition and malabsorption even though the patient has good appetite.

Symptoms of malabsorption include poor weight gain, frequent foul-smelling stools, stomach ache, and excessive gas.

Enzyme supplements, supplementary feeding, and vitamin replacement all assist with compensating for decreased activity of the pancreas.

In older CF patients, the pancreas may become further inactive and reduce or stop the production of insulin, resulting in the CF-related diabetes (CFRD). This rarely occurs in children. CFRD is treated in much the same way as Type 1 diabetes, but varies between individuals.

See Treatment-Nutrition for more information.

Other Affected Organs

Cystic Fibrosis has no effect on brain or sensory function, or mental acuity.

A small percentage of the CF population may be affected by mucus blockage in the small ducts within the liver, reducing liver function.

Adults with CF are also prone to osteoporosis (thin, brittle bones) due to nutritional problems and the adverse effects of steroid medications.

In most men with CF, the tubes that carry the sperm are blocked or have never developed (Absence of the Vas Deferens), this can cause fertility problems, but does not cause sexual impotency.

Women with CF may have irregular menstrual cycles related to nutritional issues, but they do produce healthy fertile eggs, so effective contraception is necessary.

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