Cystic Fibrosis — NCLEX Cheat Sheet
Sweat chloride > 60 mEq/LAlso known as: CF · salty baby · thick mucus disease · 65 roses
Use this quick-reference guide to spot, treat, and prevent Cystic Fibrosis on the NCLEX. Keep it handy during review and on exam day!
🩺📒 The 1-minute cheat sheet
⚠️ Causes
- Autosomal recessive, CFTR gene
- Thick mucus → lungs + pancreas
🩺 Signs
- Salty skin, chronic cough
- Steatorrhea, FTT, clubbing
- Meconium ileus in newborn
✅ Do
- Chest physiotherapy + postural drainage
- Pancreatic enzymes w/ all meals
- High-cal, high-protein, fat-sol vits
🚩 Report
- ↑ resp distress, hemoptysis
- Bowel obstruction signs
📚 Cystic Fibrosis — full study notes
The cheat sheet above is your quick recall card. These notes go deeper — what it is, what to do first, what must be reported, and what to teach.
Key points
Understand these first- Thick mucus obstructs airways, causing chronic cough, wheezing, recurrent respiratory infections, and eventually bronchiectasis.
- Pancreatic duct blockage prevents enzymes from reaching the intestine, causing malabsorption of fats and fat-soluble vitamins.
- Stools are frequent, bulky, greasy, and foul-smelling (steatorrhea) with failure to thrive despite a good appetite.
- Sweat contains excess sodium and chloride, producing salty skin and risk of electrolyte loss in heat.
- Newborns may present with meconium ileus; older children may develop clubbing and barrel chest.
- The sweat chloride test is the gold standard for diagnosis.
Nursing priorities
What to do, in order- Perform chest physiotherapy, postural drainage, and airway clearance techniques to mobilize thick secretions.
- Give pancreatic enzyme replacement with every meal and snack to aid digestion and absorption.
- Provide a high-calorie, high-protein diet with fat-soluble vitamin (A, D, E, K) supplements.
- Administer bronchodilators, mucolytics (dornase alfa), and antibiotics as ordered, and encourage fluids.
- Encourage extra salt and fluids during hot weather, exercise, or fever to replace sweat losses.
Red flags — report now
Escalate immediately- Increased respiratory distress, fever, change in sputum color, or hemoptysis signals pulmonary infection or exacerbation.
- Signs of bowel obstruction (abdominal distension, vomiting, no stool) require immediate attention.
- Heat exhaustion or electrolyte depletion can develop rapidly from excessive sweating.
- Never give pancreatic enzymes without food, and report failure to gain weight or worsening respiratory symptoms.
Labs & values
Numbers to know- Sweat chloride: normal <40 mEq/L; >60 mEq/L is diagnostic of CF
- Oxygen saturation: normal 95-100%
- Fat-soluble vitamins (A, D, E, K): monitored for deficiency
Patient teaching
What patients must know- Take pancreatic enzymes with every meal and snack, never on an empty stomach.
- Perform daily airway clearance and chest physiotherapy as prescribed to keep lungs clear.
- Eat a high-calorie, high-protein, high-fat diet with vitamin supplements to maintain weight.
- Add extra salt and fluids in hot weather and report any increase in cough, fever, or breathing trouble.
❓ Cystic Fibrosis: NCLEX FAQs
What are the priority nursing interventions for Cystic Fibrosis?
Perform chest physiotherapy, postural drainage, and airway clearance techniques to mobilize thick secretions. Give pancreatic enzyme replacement with every meal and snack to aid digestion and absorption. Provide a high-calorie, high-protein diet with fat-soluble vitamin (A, D, E, K) supplements. Administer bronchodilators, mucolytics (dornase alfa), and antibiotics as ordered, and encourage fluids.
What are the warning signs of Cystic Fibrosis a nurse must report?
Increased respiratory distress, fever, change in sputum color, or hemoptysis signals pulmonary infection or exacerbation. Signs of bowel obstruction (abdominal distension, vomiting, no stool) require immediate attention. Heat exhaustion or electrolyte depletion can develop rapidly from excessive sweating. Never give pancreatic enzymes without food, and report failure to gain weight or worsening respiratory symptoms.
What do I need to know about Cystic Fibrosis for the NCLEX?
Thick mucus obstructs airways, causing chronic cough, wheezing, recurrent respiratory infections, and eventually bronchiectasis. Pancreatic duct blockage prevents enzymes from reaching the intestine, causing malabsorption of fats and fat-soluble vitamins. Stools are frequent, bulky, greasy, and foul-smelling (steatorrhea) with failure to thrive despite a good appetite. Sweat contains excess sodium and chloride, producing salty skin and risk of electrolyte loss in heat.
What patient teaching is important for Cystic Fibrosis?
Take pancreatic enzymes with every meal and snack, never on an empty stomach. Perform daily airway clearance and chest physiotherapy as prescribed to keep lungs clear. Eat a high-calorie, high-protein, high-fat diet with vitamin supplements to maintain weight. Add extra salt and fluids in hot weather and report any increase in cough, fever, or breathing trouble.
Quick Tip
Thick mucus obstructs airways, causing chronic cough, wheezing, recurrent respiratory infections, and eventually bronchiectasis.
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