Cystic fibrosis: Symptoms, causes, and management

Cystic fibrosis is a hereditary disease that affects the lungs and digestive system. The body peou to produce thick and sticky mucus that can clog the lungs and obstruct the pancreas.

Cystic fibrosis (CF) can be life-threatening, and people with the condition tend to have a shorter-than-normal life span.

Sixty years ago, many children with CF died before reaching elementary school age. However, advances in treatment mean that people with CF often live into their 30s, 40s, and beyond.

There is currently no cure for CF. It affects some 30,000 people in the United States with around 1,000 new cases diagnosed each year.

Of these new diagnoses, 75 percent are made in children under the age of 2 years.

Fast facts on cystic fibrosis

Here are some key points about cystic fibrosis. More detail is in the main article.

Cystic fibrosis (CF) involves the production of mucus that is much thicker and more sticky than usual.

It mainly affects the lungs and digestive system.

CF is a hereditary condition that occurs in a child when both parents have the defective gene.

All newborns in the U.S. are screened for CF.

There is no cure, but good nutrition and taking steps to thin mucus and improve mucus expectoration can help.

What is cystic fibrosis?

Cystic fibrosis
Cystic fibrosis mainly affects younger children. People with cystic fibrosis can still lead an active life when the condition is properly managed.

CF is a genetic disease that mainly affects the lungs and digestive system, but it can result in fatal complications such as liver disease and diabetes.

The defective gene responsible for CF leads to the creation of thicker, stickier mucus than is usual. This mucus is difficult to cough out of the lungs. This can make breathing difficult and lead to severe lung infections.

The mucus also interferes with pancreatic function by preventing enzymes from properly breaking down food. Digestive problems result, potentially leading to malnutrition.

This thickening of mucus can also cause male infertility by blocking the vas deferens, or the tube that carries the sperm from the testes to the urethra.

CF is serious, with potentially life-threatening consequences. The most common cause of death in people with CF is respiratory failure.

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Symptoms

The most common symptoms of CF are:

salty-tasting skin

persistent coughing

shortness of breath

wheezing

poor weight gain in spite of excessive appetite

greasy, bulky stools

nasal polyps, or small, fleshy growths found in the nose

CF’s obstruction of the lungs increases the risk of lung infections such as bronchitis and pneumonia, as it creates optimal conditions for the growth of pathogens.

Obstruction in the pancreas can lead to malnutrition and poor growth. It has also been associated with an increased risk of diabetes and osteoporosis.

Treatment

There is currently no cure for CF. Treatment can manage the symptoms of the disease, however, and improve quality of life. Symptoms can vary and treatment plans will be individualized.

Airway clearance

It is crucial for people with CF to get rid of mucus from their lungs to allow clear breathing and minimize lung infections.

Airway clearance techniques (ACT) can help people with CF to loosen and get rid of mucus from their lungs.

An example of ACT would be postural drainage and percussion. A therapist claps the patient’s chest and back while they sit, stand, or lie in a position that should help to free up mucus.

Inhaled medication is effective at reaching the airways and commonly used. The medication can be given by aerosol or as a metered dose inhaler. These medications can thin mucus, kill bacteria, and mobilize mucus to improve airway clearance.

Antibiotics are an important part of regular care. These can be taken orally, intravenously, or through inhalation.

Other drugs, such as ibuprofen and azithromycin, have been found to preserve and improve lung function, and are now considered to be a part of standard therapy for people with CF.

People with CF can also help reduce their risk of lung infection by taking the following steps:

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