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Cystic fibrosis

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Pharmacology for Nurses

Definition

Cystic fibrosis is a genetic disorder that affects the respiratory and digestive systems, leading to the production of thick and sticky mucus. This condition can cause severe respiratory infections and complications.

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5 Must Know Facts For Your Next Test

  1. Cystic fibrosis is caused by mutations in the CFTR gene, affecting chloride ion transport across cell membranes.
  2. Patients with cystic fibrosis often require mucolytic agents to help break down and clear thick mucus from their airways.
  3. Common mucolytics used for cystic fibrosis include dornase alfa and hypertonic saline.
  4. Expectorants like guaifenesin may be used to loosen mucus, making it easier to cough up.
  5. Managing cystic fibrosis typically involves a multidisciplinary approach, including respiratory therapy, medications, and nutritional support.

Review Questions

  • What genetic mutation leads to cystic fibrosis?
  • How do mucolytics benefit patients with cystic fibrosis?
  • Name two common medications used as mucolytics in the treatment of cystic fibrosis.
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