History of chronic respiratory symptoms

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Isabella Cooper has a history of chronic respiratory symptoms, including frequent coughing, recurrent lung infections, and poor weight gain. After undergoing diagnostic tests, he was diagnosed with cystic fibrosis (CF) at a young age. Genetic screening revealed that Isabella had one copy of the F508del mutation in the cystic fibrosis transmembrane conductance regulator (CFTR) gene.

Isabella is now receiving comprehensive management for CF, which includes airway clearance techniques and treatment with Trikafta, which is a fixed-dose combination medication composed of a chloride channel opener and CFTR modulators. She also visits a specialised CF clinic regularly for monitoring and support.

1. How does mucous build up in CF contribute to respiratory complications?

2. What role does the periciliary layer play in lung function and how is it affected in cystic fibrosis?

3. How does Trikafta help maintain proper periciliary layer function?

Reference no: EM133491509

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