Cystic fibrosis (CF) is a rare, genetic, and progressive disease that primarily affects the pulmonary and digestive system but may involve multiple organ systems. Cystic fibrosis is often diagnosed as a newborn. There is no cure for CF, and patients may have a limited lifespan.
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.
In this article:
- Nursing Process
- Nursing Assessment
- Nursing Interventions
- Nursing Care Plans
- Impaired Gas Exchange
- Impaired Spontaneous Ventilation
- Ineffective Airway Clearance
- Risk For Infection
Nursing Process
CF is rare, and nurses in specialized settings such as pulmonology offices or pediatric units may 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 Assessment
The first step of nursing care is the nursing assessment, during which the nurse will gather physical, psychosocial, emotional, and diagnostic data. In this section, we will cover subjective and objective data related to cystic fibrosis.
Review of Health History
1. Assess the patient’s general symptoms.
Clinical manifestations of cystic fibrosis include the following:
- Salty-tasting skin
- Cough with thick mucus
- Wheezes
- Dyspnea
- Frequent lung or sinus infections
- Nasal polyps (noncancerous growths in the nose)
- Delayed growth and development
- Inability to gain weight
- Steatorrhea (greasy, foul-smelling stools)
- Constipation
- Male infertility
2. Note the patient’s age.
Two-thirds of patients with cystic fibrosis are diagnosed by the age of one year. The average age of diagnosis is 6 to 8 months. Symptoms may vary with age.
3. Review the patient’s medical history.
In neonates, meconium ileus is one of the earliest symptoms of CF. Other GI manifestations may include intestinal obstruction, volvulus, perforation, etc.
4. Ask the parents about the patient’s health.
Wheezing, coughing, recurrent respiratory infections, and pneumonia can occur in patients under the age of one year. Early infancy GI tract symptoms can include steatorrhea, failure to thrive, or both.
5. Review the patient’s family and genetic history.
CF is an autosomal-recessive disease. For a child to have CF, they must have one copy of the gene from each parent. Children cannot develop cystic fibrosis if they only get one gene copy. However, they will be carriers and may pass the gene to their offspring.
6. Note the patient’s race.
Although CF affects people of many races, white people with Northern European ancestry are the most likely to have it.
Physical Assessment
1. Perform physical examination.
The condition’s progression influences physical manifestations and how many different organ systems are involved.
- HEENT: rhinitis, nasal polyps, inflammation of the submandibular or parotid gland
- Respiratory: rapid breathing, respiratory distress, chest retractions, dry or productive cough, increased chest diameter
- Integumentary: finger clubbing, cyanosis, aquagenic wrinkling of the palms (AWP)
- Gastrointestinal: abdominal distention, liver and spleen enlargement (hepatosplenomegaly), rectal prolapse, cheilosis (swelling and fissuring of the lips), steatorrhea, chronic constipation, poor weight gain
- Musculoskeletal: scoliosis, kyphosis
2. Auscultate the lung sounds.
Patients presenting with pulmonary symptoms may display adventitious lung sounds such as wheezing or crackles.
3. Percuss the chest.
Percussion may reveal a chest that is hyperresonant to percussion, indicating hyperinflation.
4. Investigate airway inflammation.
In CF patients, airway inflammation is a distinguishing characteristic of lung disease. Even in the absence of infection, airway inflammation is still present.
5. Assess digestive symptoms.
CF causes thick mucus to block the ducts in the pancreas, preventing necessary enzymes from digesting food and absorbing nutrients. This leads to foul-smelling, greasy stools (steatorrhea), poor weight gain and growth, intestinal blockages, and chronic constipation.
6. Assess male genitalia and fertility.
75-80% of males who possess the gene mutation for CF present with congenital bilateral absence of vas deferens (CBAVD). In the adult male, obstructive azoospermia, or the absence of sperm, is evidence of CF.
Diagnostic Procedures
1. Diagnose CF based on symptoms and a positive sweat test.
The following criteria can be used to diagnose CF:
- Suspected CF:
- Sibling with CF
- Positive newborn screen
- Presence of symptoms affecting one or more organ systems
- Chronic sinopulmonary disease
- Gastrointestinal or nutritional abnormalities
- Salt loss syndromes
- Evidence of CFTR dysfunction:
- High sweat chloride more than 60 mEq/L on two occasions
- Two disease-causing CFTR mutations
- Abnormal nasal potential difference
2. Perform prenatal, neonatal, and postnatal screening tests.
The ability to diagnose before, during, and after delivery allows one to plan for the physical and mental needs of the parents and newborn.
- Prenatal: Amniocentesis can offer further fetal assessment when both parents have CF mutations.
- Neonatal: Immunoreactive trypsinogen (IRT) testing is required in the United States for newborn CF screening.
- Postnatal: Perform diagnostic tests as soon as possible on newborns with intestinal obstruction and suspected CF.
3. Prepare the patient for the sweat chloride test.
A result of 60 mmol/L or greater on two separate occasions diagnoses CF.
4. Assist the patient with a chest X-ray.
A chest radiograph can detect the following:
- Pulmonary nodules
- Bronchiectasis
- Hyperinflation
- Infiltrates
5. Prepare the patient for pulmonary function testing.
An essential approach for assessing and monitoring the CF status is pulmonary function testing. PFT can demonstrate airway obstruction in young infants, though standard spirometry may not be reliable until children are 5-6 years old.
6. Assist the patient with imaging scans.
The following imaging scans are used for further evaluation of CF:
- Chest CT scanning: used to diagnose lung involvement (such as bronchiectasis)
- Abdominal radiography: detects meconium ileus
- Prenatal ultrasound: sonographic characteristics revealing hyperechoic masses indicate an increased risk of meconium ileus
7. Discuss genotyping.
Advise genotype testing for patients with a positive family history preparing for pregnancy.
8. Assess pancreatic enzymes.
The pancreatic enzyme immunoreactive trypsinogen coupled with a sweat test increases the sensitivity and specificity of screening for CF.
9. Investigate the sputum sample.
Patients with CF present with bacterial pathogens in their sputum. Bronchoalveolar lavage fluid usually shows a high percentage of neutrophils, and the presence of Pseudomonas aeruginosa supports the diagnosis of CF.
10. Prepare for contrast barium enema.
When a meconium ileus is suspected or observed via radiography, a contrast barium enema can be performed for diagnosis.
Nursing Interventions
Nursing interventions and care are essential for the patients recovery. In the following section, you will learn more about possible nursing interventions for a patient with cystic fibrosis.
1. Administer medications as ordered.
These are the following medications given to patients with CF:
- Pancreatic enzyme supplements
- Multivitamins
- Mucolytics
- Antibiotics
- Bronchodilators
- Anti-inflammatory agents
- Medications treating related conditions or complications (such as insulin, bisphosphonates)
- Medications that can counteract chloride transport abnormalities (such as ivacaftor)
2. Clear the airway.
CF patients secrete viscous lung secretions. Administer mucolytic medications to improve secretion clearance and lessen sputum thickness. Consider other techniques like positive expiratory pressure (PEP) using a PEP mask, flutter devices, or a high-frequency chest wall oscillation vest.
3. Loosen lung secretions.
Collaborate with the respiratory therapist in performing chest physical therapy and postural drainage. Educate the parents on correctly performing these techniques to loosen lung secretions for easy removal and airway clearance.
4. Manage mild pulmonary exacerbations.
The family can be instructed on how to manage mild pulmonary symptoms at home by:
- Increasing airway clearance techniques
- Administering inhaled bronchodilators
- Performing chest PT and postural drainage
- Increasing dornase alfa (mucolytic) use
- Administering antibiotics
5. Administer hypertonic saline.
For patients with CF aged six years and older, the long-term administration of hypertonic saline is advised to enhance lung function and prevent exacerbations by increasing the hydration of airway surface liquid.
6. Assist the patient and the parents in creating a meal plan.
Advise the patient and the parents to follow a regular diet (as prescribed) with increased calories and unrestricted fat intake to compensate for malabsorption of increased energy demand. Nutritional supplements and supplemental vitamins are commonly required.
7. Instruct on pancreatic enzymes.
Pancreatic enzymes are taken before meals to help patients digest their food and absorb nutrients. Remind patients and parents to take enzymes before all meals and snacks that contain protein, fat, or complex carbs and to swallow capsules whole or open and sprinkle the beads on an acidic food if the child cannot swallow capsules.
8. Consider surgical treatment.
For the treatment of the following respiratory conditions, surgery may be necessary:
- Air accumulation in the pleural cavity (pneumothorax)
- Persistent hemoptysis (coughing up blood)
- Growths in the nose (nasal polyps)
- Chronic sinusitis
- End-stage lung disease (consider lung transplant)
The following are GI tract conditions that require surgical treatment:
- Obstruction in the newborn’s intestine (meconium ileus)
- Telescoping of intestines (intussusception)
- G-tube placement for supplemental feeding
- When part of the intestine slips into the rectum (rectal prolapse)
9. Manage diabetes.
Diabetes mellitus develops in 8–12% of CF patients older than 25 due to pancreatic tissue damage. Insulin therapy may be necessary.
10. Advise the patient to comply with long-term monitoring.
The following objectives are attained through patient monitoring at the CF clinic every two to three months:
- Growth and development promotion
- Lung function enhancement
- Lung disease prevention
- Nutritional support
- Treatment and complication monitoring
- Psychosocial counseling
11. Encourage routine vaccinations.
Patients with CF should receive routine vaccines, such as the seasonal influenza vaccine. Advise the patient to avoid crowds and sick people to prevent respiratory infection.
Nursing Care Plans
Once the nurse identifies nursing diagnoses for cystic fibrosis, nursing care plans help prioritize assessments and interventions for both short and long-term goals of care. In the following section, you will find nursing care plan examples for cystic fibrosis.
Imbalanced Nutrition: Less Than Body Requirements
The buildup of mucus also prevents the release of digestive enzymes from the pancreas causing malnutrition.
Nursing Diagnosis: Imbalanced Nutrition
Related to:
- Malabsorption
- 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
Expected outcomes:
- 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.
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.
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.
Impaired Gas Exchange
Nursing Diagnosis: Impaired Gas Exchange
Related to:
- Accumulation of tenacious secretions
- Infectious process
- Inflammation of airways
As evidenced by:
- Abnormal ABGs
- Tachypnea
- Dyspnea
- Hypoxia
- Restlessness
- Tachycardia
- Altered respiratory pattern
Expected outcomes:
- Patient will display adequate ventilation and oxygenation as evidenced by blood gas levels within normal limits.
- Patient will display clear lung sounds and remain free of respiratory distress.
Assessment:
1. Assess oxygen saturation through pulse oximetry.
Oxygen saturation of less than 95% may indicate failing respiratory effort.
2. Monitor mental status.
Confusion, restlessness, irritability, and lethargy are concerning signs of poor gas exchange.
3. Monitor respiratory pattern.
Note changes in respiratory depth, rate, and characteristics that signal distress.
Interventions:
1. Administer oxygen.
Supplemental oxygen maintains adequate oxygenation and decreases the work of breathing. Consider humidified oxygen to loosen and thin respiratory secretions.
2. Cluster care to maximize rest.
Minimize interruptions by clustering nursing care and interventions to allow for rest and reduce oxygen demand.
3. Administer medications.
Administer bronchodilators to open the airways, mucolytics to break up thick mucus and facilitate effective clearance, and antibiotics to treat pulmonary infections.
4. Continually monitor ABGs.
ABGs should be assessed routinely to monitor for worsening hypoxia or alterations in acid-base balance.
Impaired Spontaneous Ventilation
Cystic fibrosis is a life-long condition affecting the airways. Respiratory infections can have serious consequences on ventilation.
Nursing Diagnosis: Impaired Spontaneous Ventilation
Related to:
- Excessive mucus production
- Respiratory fatigue
- Airway blockage
- Respiratory infections
As evidenced by:
- Accessory muscle use
- Shortness of breath
- Apprehension
- Decreased cooperation
- Restlessness
Expected outcomes:
- Patient will be free of signs of respiratory distress, such as restlessness or accessory muscle use.
- Patient will demonstrate effective airway clearance techniques.
- Patient will exhibit clear breath sounds.
Assessment:
1. Closely monitor the respiratory status.
Accessory muscle use, retractions, nasal flaring, and cyanosis are severe signs of respiratory distress.
2. Auscultate for adventitious breath sounds.
Adventitious breath sounds such as wheezes or crackles are an indication of narrowed airways or increased secretions.
Interventions:
1. Administer oxygen.
If oxygen saturation drops below 95% or the client displays symptoms of impaired ventilation, apply supplemental oxygen.
2. Maintain head of bed elevation with regular position changes.
Keep the head of the bed elevated to achieve maximum chest expansion and encourage repositioning and ambulation as applicable to prevent atelectasis.
3. Provide suctioning.
Suction oral or oropharyngeal secretions as necessary to prevent accumulation that could result in aspiration.
4. Assist with airway clearance techniques.
Airway clearance is crucial in CF. Chest PT with postural drainage and using devices like positive expiratory pressure therapy (PEP) helps clear mucus.
5. Discuss the possibility of lung transplantation.
End-stage lung disease with CF often requires lung transplantation, which may not lengthen survival but improves quality of life.
Ineffective Airway Clearance
Thick and sticky mucus clogging airways is a defining characteristic of cystic fibrosis.
Nursing Diagnosis: Ineffective Airway Clearance
Related to:
- Increased mucus production
- Infectious process
- Inflammation of the bronchioles
As evidenced by:
- Persistent cough
- Productive cough
- Wheezing
- Shortness of breath
- Nasal congestion
- Sinusitis
- Abnormal chest x-ray
- Adventitious breath sounds
Expected outcomes:
- 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.
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.
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.
Risk For Infection
Thick mucus in the airways creates an environment that promotes the growth of bacteria.
Nursing Diagnosis: Risk for Infection
Related to:
- Thick mucus causing pneumonia or bronchitis
- Antibiotic resistance causing MRSA
- Weakened immune system
- Poor environmental conditions
As evidenced by:
A risk diagnosis is not evidenced by signs and symptoms as the problem has not yet occurred. Nursing interventions are aimed at prevention.
Expected outcomes:
- 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.
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.
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.
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