Life span improves for those with cystic fibrosis

People with cystic fibrosis (CF) are living longer than ever before. The average life span has increased from 33 years in 2000 to 47 years in 2016. Those 14 extra years are making a difference and its because of:

• Early detection of CF leads to earlier disease control, 
• Improved disease management
• Closer monitoring  
• Improved therapy

Lung infections are still challenging. When they happen, they can lead to airway damage. But increased attention to keeping the lungs healthy has helped to prevent infections and improve life expectancy.

Other therapies that are working:

• Managing liver disease. Liver disease in CF comes from an underlying gene defect but it can also come from malnutrition. Providing good nutrition, preventing repeated infections, better pancreas control and preventing worsening of liver disease are important.
• Pancreatic enzyme replacement therapy (PERT) helps to reduce malnutrition, vitamin deficiency, and growth failure. 
• Recent advances in the medicines help the CFTR protein work properly in certain patients. 
• For those who progress to severe lung disease, lung transplantation can prolong life. 
• Testing for colon cancer. Patients with CF can develop colon cancer at higher rates. Testing for hidden blood and routine colonoscopies is essential.