Cystic Fibrosis

Cystic Fibrosis

Cystic Fibrosis (CF) is an inherited disease that affects approximately 30,000 people in the US. It is typically diagnosed at birth and is a progressive genetic disorder that results in persistent lung infections and reduced lung capacity over time.

People with CF have mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. When the CFTR protein is not working correctly, there is an imbalance of salt and chloride which results in the mucus in various organs becoming thick and sticky. In the lungs, the mucus clogs the airways and traps bacteria which leads to infections.

Early Signs and Symptoms

The CF gene was first identified in 1989. There’s a 1 in 4 chance that a child will be born with CF when both parents carry the gene and pass it to their offspring. CF can affect the lungs, digestive system, pancreas and reproductive system. Signs include:

  • Salty-tasting skin
  • Persistent coughing
  • Frequent lung infections including pneumonia or bronchitis
  • Wheezing or shortness of breath
  • Poor growth or weight gain

Disease progression


Recognizing which individuals have more aggressive disease characteristics and predicting their risk of lung disease progression is an important step in managing and extending the life expectancy of CF patients. Disease progression can vary greatly. It also depends on which organs are affected. Very often those affected suffer from a constant cough and recurrent respiratory and lung infections. Digestive disorders are also common and can cause young patients to become underweight or fail to thrive.

Medical advances


Therapies for the treatment of cystic fibrosis focus on the symptoms of the disease, but in recent years there has also been a focus on the genetic cause of CF. For the most part patients require treatment that resolve the symptoms of CF. These treatments have advanced over many years. As a result, patients spend several hours per day managing their disease as a part of normal life. More recently, causal therapies have targeted the genetic cause of CF thus making symptomatic management less challenging.

Cystic fibrosis symptoms

Cystic fibrosis symptoms

CF affects the lungs, pancreas, digestive, and reproductive systems.

Find out more about symptoms and the disease progression

Relief for your airways: inhalations for cystic fibrosis

Inhalation therapy for Cystic Fibrosis

PARI devices and medications provide valuable support to patients with Cystic Fibrosis who require inhalation therapy. Whether your goal is to fight bacterial infections or loosen mucus in your lungs, PARI has a comprehensive product portfolio to best support your needs.

Find out more about diagnosis and treatment of CF

The eRapid® Nebulizer System – Modern inhalation for fast and efficient treatments

More about the eRapid

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