What is cystic fibrosis?

BIG IDEA:
Cystic fibrosis is a genetic disease with multiple etiologies. Typically, patients with cystic fibrosis have a thick buildup of mucus in their lungs, which can clog their airways and lead to persistent lung infections, and eventually respiratory failure. Treatments can vary greatly from person to person depending on disease etiology, but many patients rely on drugs that a ect the function of the cystic brosis transmembrane conductance regulator (CFTR). The CFTR protein is defective in patients with cystic fibrosis, and proper treatment can increase CFTR function and prevent symptoms associated with cystic fibrosis.
ESSENTIAL QUESTION:
Why are CFTR drugs prescribed to treat some patients with cystic fibrosis while other patients are treated only with airway clearance techniques?
THE CHALLENGE:
We need to answer the questions from patients with cystic fibrosis in a format that is easy to digest and understand. Many patients do not understand why they are prescribed different drugs than other patients with cystic fibrosis.
Ex. Handout, pamphlet, youtube video, script for healthcare provider, etc.
GUIDING QUESTIONS:
Here are the most common patient questions:
1. Why they have cystic fibrosis?
2. What is cystic fibrosis?
a. What is the cause?
b. What proteins are mutated?
3. Potential symptoms associated with their disease?
4. Treatment options.
a. Explain how the drugs work (mechanism of action)
b. Who is prescribed CFTR drugs and why?

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