Conditions › Pediatrics
Cystic Fibrosis Nursing Care Plan and Interventions
Cystic fibrosis nursing care centers on airway clearance every day, inhaled medications, pancreatic enzymes with meals and a high-calorie, high-fat diet with fat-soluble vitamins. Watch for pulmonary exacerbations and track growth to see whether the enzymes are working.
Cystic fibrosis nursing care plan
| Nursing problem | Goal | Key interventions |
|---|---|---|
| Ineffective airway clearance | Airway clearance done daily; lung function stable. | |
| Imbalanced nutrition: less than body requirements | Weight gain or growth on track; stools normal. | |
| Health maintenance: needs teaching | Family states the daily treatment plan. |
Problem wording is plain-language nursing diagnosis style. Your program may require NANDA-I labels and your own assessment data.
What it is
Cystic fibrosis (CF)
An inherited CFTR gene defect. Salt and water can't move normally across cells, so mucus is thick and sticky in lungs, pancreas and gut. 1
Autosomal recessive: two carrier parents have a 1 in 4 chance of a child with CF.
The lungs in CF
Sticky mucus traps bacteria: Staph aureus early, Pseudomonas later. Repeated infection and inflammation scar the airways (bronchiectasis). 2
Later signs: clubbing, barrel chest and falling exercise tolerance.
Assessment: what you will see
Greasy stools, poor growth
Thick mucus blocks pancreatic enzymes, so fat and protein aren't absorbed: bulky, oily, foul stools and poor weight gain. 2
Growth lags even with a big appetite. Vitamins A, D, E and K are lost too.
Early cues in infants
Meconium ileus at birth, salty-tasting skin, poor weight gain and a chronic cough. 1,2
All US newborns are screened with a heel-stick blood test.
Sweat chloride test
Confirms CF after a positive newborn screen. Sweat chloride 60 mmol/L or higher fits CF; 30 to 59 is intermediate. 1,2
A chemical and a tiny electric current make the skin sweat so it can be collected.
Complications and red flags
Pulmonary exacerbation
More cough and sputum, new shortness of breath, lower FEV1 or weight loss compared with the client's own baseline. 2,1
Antibiotics early, picked by sputum culture. Moderate to severe flares get IV antibiotics.
Other complications
CF-related diabetes (treated with insulin), liver disease, bowel blockage (DIOS), hemoptysis, pneumothorax, absent vas deferens in males. 2,1
Heavy sweating in heat or fever can cause low-sodium dehydration.
Priority: what the nurse does first
Airway clearance
Chest physiotherapy (percussion, postural drainage), a vibrating vest or PEP devices, on a regular schedule from diagnosis. 2
Aerobic exercise helps move mucus too, so encourage activity.
Nursing interventions and treatment
Inhaled meds
Dornase alfa or hypertonic saline thins mucus. Usual order: bronchodilator first, then dornase, airway clearance, inhaled antibiotic. 2,3
Dornase alfa breaks down DNA in mucus. Keep it refrigerated and out of light.
Pancreatic enzymes
Pancrelipase with every meal and snack. Swallow capsules whole; never crush or chew them. 2,4
Can't swallow it? Sprinkle the beads on applesauce and eat it right away.
Fat-soluble vitamins
Vitamins A, D, E and K in a water-miscible form at higher doses. Extra vitamin K with antibiotics, liver disease or hemoptysis. 2
Fat malabsorption is why these four vitamins run low.
CFTR modulators
Oral drugs that correct or boost the faulty CFTR protein, such as elexacaftor/tezacaftor/ivacaftor. Only for certain gene variants. 2
They improve lung function and weight. Airway clearance and enzymes still continue.
Modulator safety
Take with fat-containing food. Boxed warning for liver injury: check liver tests before and during. Eye exams in children. 4
Avoid grapefruit. Report jaundice or right upper belly pain right away.
Evaluation: is care working?
Are the enzymes working?
Fewer, less greasy stools and steady weight gain. Report fatty stools, bloating, belly pain or weight loss. 4,2
Doses that are too high can scar the colon (fibrosing colonopathy).
Client teaching
High-calorie, high-fat diet
Often 30 to 50% more calories than usual, mostly from fat, with plenty of protein. Not a low-fat diet. 2
Add extra salt in infancy, hot weather and heavy sweating.
Keep studying cystic fibrosis in NurseLingo Pro
Everything for cystic fibrosis in one place. A free account gets 5 free opens of any condition; Pro opens all 277.
Test yourself: free pediatric practice questions with answers and rationales.
Frequently asked questions
What are the priority nursing diagnoses for cystic fibrosis?
Common nursing problems for cystic fibrosis include ineffective airway clearance; imbalanced nutrition: less than body requirements; health maintenance: needs teaching. Choose the ones your assessment supports and rank airway, breathing and circulation problems first.
What does the nurse do first for cystic fibrosis?
Chest physiotherapy (percussion, postural drainage), a vibrating vest or PEP devices, on a regular schedule from diagnosis.
What are the interventions used to treat cystic fibrosis?
Daily airway clearance (chest physiotherapy or devices), inhaled medications, pancreatic enzymes with every meal and snack, a high-calorie, high-fat diet with fat-soluble vitamins and, for eligible clients, CFTR modulator drugs. Nurses watch for lung infections and track growth.
Sources
- OpenStax Medical-Surgical Nursing, 11.9
- merckmanuals.com
- OpenStax Clinical Nursing Skills, 14.4
- dailymed.nlm.nih.gov
For study only, not medical advice. Follow your program, your facility's policies and the provider's orders.