6 Cystic Fibrosis Nursing Care Plans

Impaired Gas Exchange

Most deaths associated with cystic fibrosis (CF) result from progressive and end-stage lung disease. In individuals diagnosed with CF, the lungs are normal in utero, at birth, and after birth, before the onset of infection and inflammation. Shortly after birth, many persons with CF acquire a lung infection, which incites an inflammatory response (Sharma & Haver, 2022). Small and large airways are obstructed by the thick secretions, resulting in difficulty in breathing. The accumulation and stasis of the thick secretions create a medium for the growth of organisms that cause repeated respiratory infections.

Nursing Diagnosis

• Impaired Gas Exchange

May be related to

• Airway obstruction by nasal obstruction
• Airway and alveoli inflammation
• Bronchiectasis with decreased surface area for gas exchange and loss of lung function
• Infection with lung consolidation, alveolar collapse

Possibly evidenced by

• Activity intolerance
• Cough
• Dyspnea
• Hypercapnia
• Hypoxemia
• Irritability
• Pale, cyanotic skin color
• Restlessness
• Tachypnea
• Tachycardia

Desired Outcomes

• The client will maintain optimal gas exchange as evidenced by oxygen saturation of 90% or greater, arterial blood gases (ABGs) within the client’s usual range, relaxed breathing, baseline heart rate, alert response mentation and no further deterioration in the level of consciousness.
• The client will verbalize understanding of causative factors and appropriate interventions.
• The client will participate in the treatment regimen within the ability or situation.

Nursing Assessment and Rationales

1. Monitor vital signs, especially respiratory and heart rates for any changes.
There will be an increase in the respiratory and heart rate as a way of compensation for early hypoxia, as well as chest retractions, increased work of breathing, nasal flaring, and use of accessory muscles of respiration. These are signs of respiratory distress necessitating prompt intervention or treatment.

2. Assess for changes in respiratory status such as cyanosis, pallor, changes in the level of consciousness, labored breathing, and tachypnea.
The client will adapt their breathing pattern over time to facilitate gas exchange. Abnormalities may indicate respiratory compromise, hypercarbia, or hypoxia. A child with cystic fibrosis often has a barrel chest (anterior-posterior diameter approximates transverse diameter). Clubbing of nail beds might also be present, a compensatory response indicating a chronic lack of oxygen.

3. Monitor transcutaneous carbon dioxide as ordered.
Chronic hypercarbia can be present in clients with moderate to severe cystic fibrosis lung disease; increasing levels can indicate the progression of acute infection and pending respiratory failure. Like hypoxemia, chronic hypercarbia is also associated with mortality in CF and can be detected on screening, and its presence in CF is cited as an indication for a lung transplant referral. It is recommended that individuals with advanced cystic fibrosis lung disease (ACFLD) undergo annual screening for hypercarbia using venous blood gas (Kapnadak et al., 2020).

4. Monitor arterial blood gases and oxygen saturation as indicated.
Increasing PaCO2 and decreasing PaO2 are signs of respiratory failure. Severe hypoxemia and hypercarbia can lead to hypotension, dysrhythmias, and failing respiratory effort. In accordance with US Center for Medicare and Medicaid Services and most third-party payer requirements, if PaCO2 is >56 mm Hg on venous blood gas, a confirmatory arterial blood gas should be obtained (Kapnadak et al., 2020).

5. Auscultate for breath sounds and percuss for chest resonance.
Auscultation may reveal a variety of adventitious breath sounds. Fine or coarse crackles and scattered or localized wheezing might be present. With progressive obstructive pulmonary involvement, breath sounds might be diminished. Percussion over the lung fields usually yields hyper resonance due to air trapping.

6. Assess the client’s mental status and general appearance.
Restlessness, irritability, confusion, and somnolence may reflect hypoxemia or decreased cerebral oxygenation. The client may become irritable and tired easily. There is a gradual change in physical appearance.

Nursing Interventions and Rationale

1. Provide adequate rest between activities during the day, with minimal nighttime interruption in sleep.
Activity should be paced appropriately to avoid increase in oxygen demand and avoid fatigue. Individuals diagnosed with cystic fibrosis may have sleep problems due to abnormalities with the airway, as well as impaired gas exchange. CF has been shown to predispose individuals to hypoxemia, or low levels of oxygen in the blood, which is associated with sleep disruption and daytime impairment (Editorial Team, Cystic-Fibrosis.com, 2019).

2. Place the client in a semi-Fowler’s position or elevate the head of the bed.
The semi-Fowler position promotes lung expansion and decreases airway collapse, dyspnea, and breathwork through gravity. The client’s head of the bed may be elevated to promote maximal inspiration and enhance the expectoration of secretions to improve ventilation.

3. Assist in performing postural drainage and chest physiotherapy.
Postural drainage and chest-clapping therapy are also of value. When postural drainage and chest physiotherapy are done properly, the secretions in the chest are moved up and out.

4. Instruct the client in performing breathing exercises.
Breathing exercises may also be recommended for the older child. Pursed-lip breathing is one technique that is simple and effective. The client is instructed to inhale through the nose, then exhale through the mouth with lips pursed as if whistling. Exhalation should be at least twice as long as inhalation. The client is taught not to force air out but let it escape naturally.

5. Administer hypertonic saline solution via nebulization.
Hypertonic saline inhalation has been proposed as a therapy to increase the hydration of airway surface liquid in clients diagnosed with CF. Hypertonic saline was not associated with worsening bacterial infections or inflammation. The Pulmonary Therapies Committee of the Cystic Fibrosis Foundation recommends long-term use of hypertonic saline for clients with CF aged six years or older to improve lung function and reduce the number of exacerbations (Sharma & Haver, 2022).

6. Administer oxygen therapy as indicated. Avoid giving high oxygen concentrations to clients with chronic carbon dioxide retention.
Supplemental oxygen maintains adequate oxygenation, decreases work of breathing and calorie expenditure, relieves dyspnea, and increases the level of comfort. Maintaining an oxygen saturation greater than 90% is the goal. Both nocturnal and exertional supplemental oxygen in CF improves oxygenation with the potential for slight worsening of hypercarbia. Oxygen use in advanced cystic fibrosis lung disease (ACFLD) also improves exercise capacity and reduces absenteeism from school or work (Kapnadak et al., 2020).

7. Collaborate with respiratory care personnel in the monitoring and administration of noninvasive ventilation as indicated.
Bilevel-positive airway pressure is noninvasive ventilation indicated for clients with severe lung disease and a superimposed acute illness or for assistance with nighttime ventilatory needs. Nocturnal NIV for hypercarbia in CF was shown in one high-quality randomized crossover trial to improve partial pressure of carbon dioxide, dyspnea, and exercise tolerance. Other series similarly demonstrate improvements in symptoms, lung function, or utility in bridging to lung transplant (Kapnadak et al., 2020).

8. Promote participation in a pulmonary rehabilitation program.
Multiple studies have evaluated exercise programs at home and in healthcare settings in individuals with CF including advanced cystic fibrosis lung disease (ACFLD). Both strength and aerobic training improve exercise capacity and quality of life, and some studies demonstrate small improvements or slowing in the rate of decline in lung function. Enrollment in pulmonary rehabilitation programs may also be beneficial in preparation for lung transplantation and is required by some transplant programs (Kapnadak et al., 2020).

9. Prepare for transfer to the intensive care unit if indicated.
Survival in CF after an ICU admission has improved compared to previous decades. Survival to hospital discharge is reported as high as 55% when a lung transplant is an option and 10 to 55% when a transplant is not an option. The CF Foundation recommends that individuals with advanced cystic fibrosis lung disease (ACFLD) and acute respiratory failure be considered eligible for ICU management regardless of transplant status if congruent with the goals of care (Kapnadak et al., 2020).

10. Assist in the preparation of lung transplantation, as appropriate.
Although outcomes have improved, advanced cystic fibrosis lung disease (ACFLD) is associated with significant morbidity and reduced quality of life and remains the most common cause of death in CF. Lung transplant outcomes have also improved and multiple cohort studies demonstrate improved quality of life and survival with transplant for individuals with ACFLD. The benefits of lung transplant are likely most pronounced in those who are severely impaired and have additional predictors of mortality (Kapnadak et al., 2020).