Please note - The Cystic Fibrosis Knowledge Centre is for Doctors and other Healthcare Professionals.
Enter the Cystic Fibrosis Knowledge Centre.
Cystic Fibrosis (CF) is caused by a defect in a gene known as the cystic fibrosis transmembrane conductance regulator (CFTR) gene. This gene makes a protein that controls the movement of ions, such as chloride, and water, across cell membranes.
CF is caused by hundreds of different gene mutations. The most common mutation is delta F508, usually written as ΔF508.
- Persistent cough, often with phlegm
- Frequent lung infections
- Wheezing or shortness of breath
- Very salty-tasting skin
- Poor growth or weight gain despite a good appetite
- Greasy, bulky, foul-smelling stools Stomach pain and discomfort caused by too much gas in the intestines
Because CF is a multisystem disease, treatment must be multidisciplinary, with a team of healthcare professionals providing comprehensive management of the patient.
Current therapy for CF is targeted at prevention and treatment of exacerbations. Care at the CF center includes inpatient as well as outpatient clinical care.
What’s in the Cystic Fibrosis Knowledge Centre?
- Causes of Cystic Fibrosis (CF)
- Physiologic Consequences
- Incidence and Prevalence
- Clinical Consequences
- Effects on the Respiratory System
- Effects on the Digestive system
- CF- related Diabetes Mellitus
- Effects on the Heart
- Other Effects of Cystic Fibrosis
- Early screening and Diagnosis
- Clinical Manifestation
- Multiple Organ Involvement
- Progression of Respiratory Clinical Features
- Complications of Lung Disease
- Other Symptoms Affected by CF
- Diagnostic Approach
- Lung Function Tests
- Spirometry Testing
- Pulmonary Function Tests
- Management of Cystic Fibrosis
- Nutritional Support
- Lung Management
- Management of the Digestive System
- Management of the Gallbladder and Liver
- Management of the Pancreas
- Antibiotic Therapy
- Emerging Pathogens
- Anti-inflammatory Therapy
- Severe Disease
- Resources - FAQ