Cystic fibrosis (CF) is a rare, genetic, and progressive disease that damages the lungs, digestive system, and causes further complications to other organs. Cystic fibrosis is normally diagnosed as a newborn. There is no cure for CF and patients usually do not live past their 50s.
Mutations to the CFTR (cystic fibrosis transmembrane conductance regulator) gene prevent the electrolyte chloride (a component of salt) from moving to the surface of cells. If chloride cannot attract water to the cell surface this leads to thick, sticky mucus that builds up in the lungs and pancreas increasing the risk of lung infections and complications with nutrition and digestion.
The Nursing Process
CF is rare and nurses in specialized settings such as pulmonology offices or pediatric units will likely care for these patients. Nurses may treat children with respiratory infections or care for adults who undergo lung or liver transplants and must understand the complex and delicate disease process of CF.
Nursing Care Plans Related to Cystic Fibrosis
Ineffective Airway Clearance Care Plan
Thick and sticky mucus clogging airways is a defining characteristic of cystic fibrosis.
Nursing Diagnosis: Ineffective Airway Clearance
- Increased mucus production
- Infectious process
- Inflammation of the bronchioles
As evidenced by:
- Persistent cough
- Productive cough
- Shortness of breath
- Nasal congestion
- Abnormal chest x-ray
- Adventitious breath sounds
- Patient will maintain a patent airway
- Patient will effectively expectorate/clear secretions
- Patient will demonstrate an improvement in airway clearance as observed by vital signs and chest x-ray within normal limits
Ineffective Airway Clearance Assessment
1. Assess lung sounds.
Wheezing is a common finding with cystic fibrosis. On auscultation, crackles, rales, or stridor may be heard.
2. Assess sputum characteristics.
Observe the amount, color, and consistency of mucus. Mucus production should decrease with treatment. Green or yellow mucus likely signals an infectious process.
3. Monitor respiratory rate and vital signs.
Patients may often present with dyspnea and labored breathing. Assess respiratory rate and rhythm along with O2 saturation to prevent hypoxemia.
Ineffective Airway Clearance Interventions
1. Administer bronchodilators.
Bronchodilators should be given before hypertonic saline as these will open the airways and make coughing up mucus easier.
2. Administer mucolytics.
Hypertonic saline is commonly given inhaled and thins the mucus. Guaifenesin is an expectorant and will help cough up the mucus.
3. Assist with airway clearance.
Airway clearance techniques should be performed last after bronchodilators and mucolytics are administered as the techniques will likely be more effective. The nurse can assist the respiratory therapist with techniques such as chest physiotherapy.
4. Position accordingly.
Proper positioning makes a huge difference in clearing the airways. Chest x-rays can help determine where secretions are in the lungs so the patient can position for the mobilization of mucus. If secretions are in the upper lobes, supine positioning is optimal. If in the middle or lower lobes, upright is best. If in the right or left lungs, the patient should lay on that side.
Imbalanced Nutrition: Less Than Body Requirements Care Plan
The buildup of mucus also prevents the release of digestive enzymes from the pancreas causing malnutrition.
Nursing Diagnosis: Imbalanced Nutrition
- Poor digestion
- Nutrient deficiencies
As evidenced by:
- Delayed growth
- Diarrhea or constipation
- Foul-smelling and greasy stools
- Poor weight gain
- Intestinal obstructions
- Abdominal distention or bloating
- Patient will display improvement in nutritional status as evidenced by lab work
- Patient will demonstrate weight gain of 10% within three months
- Patient will adhere to enzymes and supplements to improve nutrition
Imbalanced Nutrition: Less Than Body Requirements Assessment
1. Assess weight and growth.
Children with CF are often very thin and small for their age. The nurse should closely assess and document height and weight at each visit as these often correlate with CF outcomes.
2. Observe stools.
Patients may struggle with constipation or diarrhea as nutrients are not digested correctly. Stools are often foul-smelling and may have mucus or oil in them. A stool sample can be analyzed to see if the pancreas is producing enzymes.
3. Monitor lab work.
CF can cause complications of the liver as well as CF-related diabetes. The nurse should monitor liver enzymes, glucose levels, and electrolytes levels for complications.
Imbalanced Nutrition: Less Than Body Requirements Interventions
1. Administer pancreatic enzymes.
Pancreatic enzymes help the body digest carbs, fats, and protein. These should be taken before eating to aid digestion.
2. Provide a high protein diet.
Patients require high-calorie and high-protein diets. Encourage dairy, breaded meats, peanut butter, pasta, and eggs.
3. Teach the patient and family.
Children with CF may be less inclined to follow diet and enzyme guidelines. Children should be encouraged to eat anytime they are hungry. Adhering to set mealtimes may not be feasible and flexibility should be encouraged. Involve the child in taking their enzymes by opening the capsules and sprinkling the beads on their food.
4. Collaborate with a dietician.
Dieticians can assist with finding the correct amount of enzymes required as well as recommending additional vitamins and supplements and adding calories to the diet.
Risk For Infection Care Plan
Thick mucus in the airways creates an environment that promotes the growth of bacteria.
Nursing Diagnosis: Risk for Infection
- Thick mucus causing pneumonia or bronchitis
- Antibiotic resistance causing MRSA
- Weakened immune system
- Poor environmental conditions
Note: A risk diagnosis is not evidenced by signs and symptoms as the problem has not occurred yet and the goal of nursing interventions is aimed at prevention.
- Patient will remain free of respiratory infections
- Patient will demonstrate improvement in the infectious process as evidenced by improved white blood count and improved symptoms
- Patient will prevent infection by proper hand hygiene and masking in public places
Risk For Infection Assessment
1. Assess for signs of infection.
Assess for fever, increased cough or sputum production, tachycardia, and increased fatigue.
2. Monitor lab work.
The white blood count will be elevated and sputum cultures may show bacterial organisms.
3. Assess for antibiotic resistance.
CF patients are treated frequently for infections that can lead to antibiotic resistance. This can be dangerous as commonly used antibiotics are no longer effective.
Risk For Infection Interventions
1. Administer antibiotics.
IV antibiotics are the standard of care for many of the infections that plague CF patients, which are often lung infections.
2. Implement infection control precautions.
Strict hand hygiene practices should be followed and nurses should wear gowns and gloves when in the presence of CF patients to prevent transmitting germs. CF patients require single-patient rooms and when multiple CF patients are in a clinic together they should be at least 6 feet apart.
3. Receive vaccinations.
CF patients should receive the same immunizations all children receive. They are more susceptible to influenza and respiratory infections and should receive annual flu vaccines and Covid-19 vaccines.
4. Encourage exercise.
Exercise is often difficult for patients with CF to tolerate. Aerobic exercise helps with atelectasis and the clearing of secretions and research shows frequent exercise improves mortality.
References and Sources
- Chaudary, N., & Balasa, G. (2021, June 14). Airway Clearance Therapy in Cystic Fibrosis Patients Insights from a Clinician Providing Cystic Fibrosis Care. NCBI. Retrieved April 6, 2022, from https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8214208/
- Common Medications | The Cystic Fibrosis Center at Stanford | Stanford Medicine. (n.d.). Stanford Medicine. Retrieved April 6, 2022, from https://med.stanford.edu/cfcenter/education/english/Meds.html
- Cystic Fibrosis Foundation. (n.d.). About Cystic Fibrosis. Cystic Fibrosis Foundation. Retrieved April 6, 2022, from https://www.cff.org/intro-cf/about-cystic-fibrosis
- Cystic Fibrosis Foundation. (n.d.). Enzymes. Cystic Fibrosis Foundation. Retrieved April 6, 2022, from https://www.cff.org/managing-cf/enzymes#how-to-take-enzymes
- Cystic Fibrosis Foundation. (n.d.). Mucus Thinners. Cystic Fibrosis Foundation. Retrieved April 6, 2022, from https://www.cff.org/mucus-thinners
- Cystic Fibrosis Foundation. (n.d.). Why Are Some Germs Particularly Dangerous for People With CF? Cystic Fibrosis Foundation. Retrieved April 6, 2022, from https://www.cff.org/managing-cf/why-are-some-germs-particularly-dangerous-people-cf
- Cystic fibrosis – nutrition Information. (n.d.). Mount Sinai. Retrieved April 6, 2022, from https://www.mountsinai.org/health-library/nutrition/cystic-fibrosis-nutrition
- Cystic fibrosis – Symptoms and causes. (2021, November 23). Mayo Clinic. Retrieved April 6, 2022, from https://www.mayoclinic.org/diseases-conditions/cystic-fibrosis/symptoms-causes/syc-20353700
- Gifford, A. H., Stoudemire, W., & Saiman, L. (2014). Infection Prevention and Control Clinical Care Guidelines. Cystic Fibrosis Foundation. Retrieved April 6, 2022, from https://www.cff.org/medical-professionals/infection-prevention-and-control-clinical-care-guidelines
- Le, T. N., Anabtawi, A., Putman, M. S., Tangpricha, V., & Stalvey, M. S. (2019). Growth failure and treatment in cystic fibrosis. Journal of cystic fibrosis: official journal of the European Cystic Fibrosis Society, 18 Suppl 2(Suppl 2), S82–S87. https://doi.org/10.1016/j.jcf.2019.08.010
- Pool, J. (2019, August 16). What Vaccinations Should a Person with CF Get? Cystic-Fibrosis.com. Retrieved April 6, 2022, from https://cystic-fibrosis.com/vaccinations