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.
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:
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.
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.
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