Cystic Fibrosis and Pneumothorax

Pneumothorax is also known as a collapsed lung. It is caused by damage to the lung which then releases air into the chest cavity. For those living with cystic fibrosis (CF), pneumothorax is the result of weakened lung tissue which begins to leak. This specific type of pneumothorax is known as secondary spontaneous pneumothorax (SSP). As CF treatments improve and people live longer, the occurrence of SSP may increase. But, with proper medical care, a full recovery can be achieved.^1-3

How does it occur?

Pneumothorax occurs when the chest cavity lining and surface of the lung are damaged. The weakened tissue allows air to escape from the lung into the chest cavity. The damage may be due to inflammation, infection, or stress on the tissue.^1-3

How/when do people with CF experience a collapsed lung?

Adults living with cystic fibrosis tend to develop pneumothorax more often than children. This may be due to stress placed on the lung tissue as people get older.⁴

There are also other risk factors that may play a role, such as:^1-3

• Bacterial or fungal infection in the lung
• Use of feeding tubes
• Use of non-invasive positive pressure ventilation (NIPPV)
• Excessive coughing up of blood
• Poor pancreatic function
• Poor lung function
• Use of inhaled medications
• Smoking
• Thoracic endometriosis, a disease in which uterine-like tissue grows abnormally outside the uterus.

What are the symptoms of pneumothorax?

Chest pain is often the first symptom noticed by the person experiencing a pneumothorax. Other symptoms may include:^1,3

• Sharp, stabbing chest pain when inhaling
• Shortness of breath
• Bluish skin color, due to decreased oxygen to the skin
• Fatigue, a severe feeling of tiredness
• Rapid breathing and heart rate
• Dry, hacking cough

If you experience these symptoms, contact your doctor. A pneumothorax that is not treated properly can be a life-threatening experience.^1,3

How is it diagnosed?

During a physical exam, your doctor will listen for hollow sounds in your chest. Arterial blood gas tests will show lower oxygen and higher carbon dioxide levels. Finally, a chest x-ray will confirm the presence of the pneumothorax. The image will show the location that has been damaged and presence of air in the chest cavity.⁵

How is it treated?

The presence of a pneumothorax is very serious and may be life-threatening if large and left untreated. However, when treated properly a person can typically make a complete recovery. The goal is to provide safe, effective treatment that eliminates repeat occurrences.¹

The severity of the pneumothorax will determine whether hospital care is required. Treatment may include:^2,5,6

• Observation only
• Chest tube placement
• Pleurodesis, a procedure where your doctor places a talc powder in the space between your lung and chest wall lining. This causes irritation and swelling between these 2 layers, causing them to stick together, stopping the fluid or air from escaping.

The size and frequency of the pneumothorax will also help determine the correct treatment plan.²

If the pneumothorax persists or reoccurs, your doctor may recommend either medical or surgical procedures that will aid by healing or removing the damaged tissue.²

Pneumothorax can be a life-threatening medical emergency. If you are experiencing any of these symptoms, seek medical attention right away. If you have a history of pneumothorax, do not assume your symptoms are mild. Only a trained medical professional can determine the severity of your collapsed lung.^1,5

Have you or your loved one with cystic fibrosis experienced pneumothorax? Share your experience in the comments below.