Cystic Fibrosis Treatment and Survival

Dr. Mael Mala, a pediatric pulmonologist, discusses cystic fibrosis, a genetic disease affecting the lungs, sinuses, and digestive tract. He highlights the introduction of CFTR modulators, which have significantly improved lung function, reduced complications, and increased the median survival age for patients. These therapies have led to fewer hospitalizations and removed some patients from lung transplant lists.

What is the primary role of Mael Mala?

• A pediatric neurologist

• A pediatric pulmonologist

• A general practitioner

• A pediatric cardiologist

Which systems of the body does cystic fibrosis primarily affect?

• Endocrine and lymphatic systems

• Nervous and circulatory systems

• Digestive and respiratory systems

• Muscular and skeletal systems

What does CFTR stand for in the context of cystic fibrosis treatment?

• Cystic Fibrosis Transmembrane Conductance Regulator

• Cystic Fibrosis Transcription Regulator

• Cystic Fibrosis Treatment Regimen

• Cystic Fibrosis Transmembrane Receptor

How do CFTR modulators help patients with cystic fibrosis?

• By increasing the need for antibiotics

• By fixing the CFTR channel to improve lung function

• By increasing the production of mucus

• By reducing the size of the lungs

What significant change has been observed in patients due to CFTR modulators?

• Decreased lung function

• Increased need for lung transplants

• Increased hospitalization rates

• Improved lung function and reduced hospitalizations

What percentage of cystic fibrosis patients qualify for CFTR modulators?

• 5 out of 10

• 7 out of 10

• 9 out of 10

• 10 out of 10

What was the median age of survival for cystic fibrosis patients a few years ago?

• 50s

• 40s

• 30s

• 20s

What is the current median age of survival for cystic fibrosis patients?

• 60

• 30

• 40

• 50

What has contributed to the increase in the median age of survival for cystic fibrosis patients?

• Improved care and new therapies

• Higher rates of lung transplants

• More frequent hospitalizations

• Increased use of antibiotics