Cystic Fibrosis (CF), Causes, Symptoms, Treatment & Life Expectancy

Clinical picture (symptoms and complications) of cystic fibrosis

All manifestations of cystic fibrosis are due to the thick sticky mucous -that your body secretes- and its effects on the different organs. Symptoms of cystic fibrosis vary between patients, according to the disease severity. They also differ in the same patient; symptoms improve and worsen over time.

Cystic fibrosis causes symptoms and complications that are related to the affected organs, as follows:

Cystic fibrosis and respiratory system

Lung disease in cystic fibrosis results from the thick mucous that block the tubes that carry the air to and from the lungs. Also, this thick stagnant mucous makes a suitable environment for harmful bacteria.

The lung symptoms include the following:

  • A persistent cough that produces thick profuse mucoid sputum
  • Wheezes (A high-pitched sound that you hear during breathing)
  • Shortness of breathing, which usually leads to exercise intolerance

As the disease progresses, the respiratory condition worsens, and further complications occur, as follows:

  • Recurrent lung infections: As we said, stagnant thick mucous is the preferred environment for infections. Lung infections cause symptoms like fever, productive cough, wheezes, and difficult breathing. But, the major problem for lung infections in cystic fibrosis is that it is difficult to treat due to antibiotic resistance and may need hospitalization for weeks.
  • Bronchiectasis (damaged airways): It is an abnormal dilation of the bronchial tubes and scarring of their walls. In this condition, the lung loses its elasticity, and it becomes hard to move air or clear mucous. This condition means structural damage to the lungs.
  • Hemoptysis (coughing blood): Bronchiectasis damages the bronchial wall and extends to the adjacent blood vessels, which causes bleeding into the lungs and hemoptysis. This condition may be mild or life-threatening, but it requires urgent care.
  • Nasal sinuses (infection and polyps): Thick mucous also builds up in the paranasal sinuses and blocks them, which causes sinusitis. Sinusitis causes fever, headache, facial pain, and nasal discharge. Recurrent and chronic sinusitis causes overgrowths of the nasal tissue (nasal polyps) that block the nasal passages and worsen the breathing difficulties. About 10% to 20% of patients with CF have recurrent nasal polyps.
  • Pneumothorax: It means the presence of air in the pleural space that surrounds the lung tissue. It is more common in adults with CF and causes acute chest pain and difficulty breathing.
  • Respiratory failure: As the disease progresses, it damages more lung tissue, and the lung functions worsen more. Finally, the lung can’t work, which is a life-threatening condition. It is the most common cause of death in cystic fibrosis.

Written by Martin Davis