Maintaining Respiratory Health in Cystic Fibrosis Patients

Author

Pediatric pulmonologist, Pulmonary Department, Children’s Medical Center,Tehran University of Medical Sciences, Tehran, Iran.

Abstract

Cystic fibrosis (CF) is an inherited disease that primarily affects the lungs and the digestive system, however, it also affects a number of other organs and systems. More than 90% of mortality of  CF patients is due to lung complications.  Healthy lungs are important for a long life for people with CF, We will discuss two important topics for maintaining respiratory health.
Chronic use of drugs for maintaining respiratory health
There are a number of drugs available to keep CF lungs healthy. We will discuss the science behind the recommendations for use of:
Inhaled antibiotics
Dornase alfa
Azithromycin
Hypertonic saline
High-dose ibuprofen
Ivacaftor
CF Airway Clearance Therapies
Airway Clearance therapy is very important to keeping CF lungs healthy. Our discussions cover the following topics such as the:
Daily airway clearance
Different techniques of airway clearance
Effect of aerobic exercise on airway clearance
 

Keywords