Cystic Fibrosis Overview

Pathophysiology Overview

• Cystic Fibrosis is characterized by thick mucous that clogs ducts and pathways across multiple organ systems.

Respiratory System

• Clinical Manifestations:
  • Mucus clogs the respiratory tract and alveoli (emphysema).
  • Decreased gas exchange and decreased $\text{O}_2$ saturation.
  • Dyspnea and increased total work of breathing (TWOB).
  • Increased secretions, increased risk for upper respiratory infections (URI), and increased risk for pneumonia.
  • Increased metabolic rate, elevated caloric need, and weight loss.
• Treatments:
  • Mucolytics
  • Bronchodilators
  • Chest PT
  • Aerosol therapy
  • Oxygen ($\text{O}_2$)
  • Antibiotics (Abx)
  • Exercise

Hepatic and Biliary System

• Clinical Manifestations:
  • Mucus clogs hepatic and biliary ducts, causing liver scarring (cirrhosis), liver dysfunction, and portal HTN.
  • Blocked bile leaving the gallbladder leads to cholecystitis (RUQ pain) and reduced fat breakdown (steatorrhea).

Gastrointestinal System

• Clinical Manifestations:
  • Intestines fill up with mucus.
  • Fecal impaction (constipation) and intussusception.
  • Decreased absorption of nutrients resulting in malnourishment.
• Treatment:
  • Increased protein and calorie diet.

Pancreatic System

• Exocrine Pancreas:
  • Pancreatic ducts fill up with mucus, causing decreased excretion of trypsin, amylase, and lipase (pancreatitis).
  • Decreased breakdown and absorption of fats and carbohydrates (steatorrhea).
  • Treatment: Administer pancreatic enzymes (pancrelipase) with food and fat-soluble vitamin (ADEK) supplements.
• Endocrine Pancreas:
  • Decreased release of insulin leading to hyperglycemia and increased risk of DM.
  • Treatment: Give insulin.

Reproductive System

• Clinical Manifestations:
  • Mucus clogs the reproductive tract, leading to infertility.