Cystic fibrosis is an autosomal recessive genetic disorder that is caused by a mutation in the gene for the protein cystic fibrosis transmembrane conductance regulator (CFTR) present in the mucus-secreting cells of the body, primarily affecting the respiratory, reproductive, and gastrointestinal tracts.
The hallmark manifestation of CF is chronic, progressive lung disease, resulting from secretion of dehydrated mucus with airway obstruction, and malnutrition from pancreatic insufficiency. However, there is a wide range of disease variety. Pulmonary symptoms can range from nearly asymptomatic disease with undetectable changes in the lung function to severe obstructive disease early in childhood, whereas gastrointestinal symptoms may range from mild constipation, normal liver function, and intermittent pancreatitis to severe loss of pancreatic function, malabsorption with subsequent malnutrition, CF-related diabetes, and end-stage liver diseases.
The sweat glands and reproductive glands are also affected; almost all males with CF are sterile because of absence or impairment of vas deferens, whereas females have reduced fertility. The impact on the sweat gland give rise to abnormally high chloride secretion; a simple analysis of sweat chloride content continues to be the gold standard for CF diagnosis.
Nursing Care Plans
Nursing care plan for clients with cystic fibrosis includes maintaining adequate oxygenation, promoting measures to remove pulmonary secretions, emphasizing the importance of adequate fluid and dietary intake, ensuring an adequate nutrition, and preventing complications.
Here are five (5) nursing care plans (NCP) for cystic fibrosis:
- Impaired Gas Exchange
- Ineffective Airway Clearance
- Imbalanced Nutrition: Less Than Body Requirements
- Deficient Knowledge
- See Also and Further Reading
Impaired Gas Exchange: Excess or deficit in oxygenation and/or carbon dioxide elimination at the alveolar-capillary membrane.
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
- Pale, cyanotic skin color
- Client will maintain optimal gas exchange as evidenced by oxygen saturation of 90% or greater, arterial blood gasses (ABGs) within the client’s usual range, relaxed breathing, baseline heart rate, alert response mentation and no further deterioration in the level of consciousness.
|Monitor respiratory and heart rate for any changes.||There will be an increase in the respiratory and heart rate as a way of compensation for early hypoxia.|
|Assess for changes in respiratory status such as cyanosis, pallor, changes in the level of consciousness, labored breathing and tachypnea.||Client will adapt their breathing pattern over time to facilitate gas exchange. Abnormalities may indicate respiratory compromise, hypercarbia, or hypoxia.|
|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.|
|Monitor arterial blood gasses 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.|
|Provide for adequate rest between activities during the day, with a minimal nighttime interruption in sleep.||Activity increases oxygen needs and should be paced appropriately to avoid fatigue.|
|Place the client in a semi-Fowler’s position.||Semi-Fowler position promotes lung expansion and decreases airway collapse, dyspnea, and breath work through gravity.|
|Administer oxygen therapy as indicated. (Avoid giving high oxygen concentration in clients with chronic carbon dioxide retention).||Supplemental oxygen maintains adequate oxygenation, decreases the work of breathing and calorie expenditure, and relieves dyspnea, increasing the level of comfort. Maintaining an oxygen saturation greater than 90% is the goal.|
|Collaborate with respiratory care personnel in the monitoring and administration of noninvasive ventilation as indicated.||Bilevel positive airway pressure is a noninvasive ventilation indicated for clients with severe lung disease and a superimposed acute illness or for assistance with nighttime ventilatory needs.|