Article by: Dr Zoe Arugay
Photo: Newscom
 

Cystic fibrosis is an inherited disorder affecting the lungs and digestive system. It is characterized as manifesting with unusually thick, sticky mucus that clogs the lungs which can lead to life-threatening lung respiratory infections. This is brought about by having a defective gene so its protein product is responsible for this kind of secretion. Because it affects the lungs, patients with cystic fibrosis may manifest with cough and wheezing, the most common symptoms. Cough may often be accompanied by sputum, gagging, vomiting, and disturbed sleep.

In addition to affecting the lungs this way, it also obstructs the pancreas and stops the naturally existing enzymes from helping the body digest the food we eat. It typically manifests with abdominal distention, vomiting, and failure to pass meconium in newborn.

Diagnosis of CF is suggested by a positive prenatal or newborn screen, family history, or symptomatic presentation. It is confirmed by a sweat test and sometimes with genetic testing.

 

Treatment involves antibiotics, aerosol drugs and inhaled bronchodilators to aid in respiration, and may even include pancreatic enzyme supplementation.

 

Advances in treatment of CF has lead to a much longer life expectancy compared to some 30 years ago when patients diagnosed with CF die in their teenage years. Today, the median age of survival is more than 37 years, and more than 45 percent of all people with CF in the U.S. are 18 or older, according to the Cystic Fibrosis Foundation.