5 Cystic Fibrosis Nursing Care Plans

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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 adequate nutrition, and preventing complications.

Here are five (5) nursing care plans (NCP) and nursing diagnosis (NDx) for cystic fibrosis:

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  1. Impaired Gas Exchange
  2. Ineffective Airway Clearance
  3. Imbalanced Nutrition: Less Than Body Requirements
  4. Deficient Knowledge
  5. Infection
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Ineffective Airway Clearance

Nursing Diagnosis

May be related to

  • Increased mucopurulent production and mucous plugging in response to new or increased airway bacterial growth.

Possibly evidenced by

  • Abnormal chest x-ray film
  • A decline in pulmonary function testing (spirometry)
  • An ineffective cough
  • Chest pain
  • Crackles, rhonchi
  • Dyspnea
  • Fatigue
  • Labored breathing
  • Reduced breath sounds

Desired Outcomes

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  • Client will be proficient in using effective airway clearance therapies to clear secretions on a daily basis, as evidenced by decreased work of breathing and improved pulmonary function.
  • Client will maintain clear, open airway as evidenced by normal breath sounds, normal rate and depth of respirations, and an airway free of secretions, with an effective cough.
Nursing Interventions Rationale
Assess cough for effectiveness. Habitual cough suppression is common in clients with cystic fibrosis; an effective mucus-clearing is important for adequate airway clearance.
Assess respiratory rate, work of breathing, use of accessory muscles, and the presence of retractions. Infection, inflammation, and mucous plugging will cause an increase in the respiratory effort to compensate for airway obstruction. As moving air into and out of the lungs becomes more difficult, the breathing pattern alters to include the use of accessory muscles and retractions.
Assess the chest wall for even chest expansion. Unequal chest expansion can indicate a pneumothorax– a complication of cystic fibrosis.
Auscultate lung for adventitious sounds, and the degree of aeration. Crackles, rhonchi, and decreased aeration signify an ongoing infection and inflammation.
Assess sputum for color, amount, and consistency. A decreasing amount and frequency of sputum production, lighter color, and thinner consistency of sputum indicate improvement in exacerbation. Scant amounts or streaking of blood in the sputum can occur from airway inflammation; however, major hemoptysis can occur and is a life-threatening emergency.
Monitor oxygen saturation. Hypoxemia may result from impaired gas exchange and from the buildup of secretions and bronchial constriction.
Monitor pulmonary function testing. Improvement in spirometry signifies improved airflow and aeration, effective airway clearance, and treatment of infection.
Assess the client’s comfort level with and adherence to recommended airway clearance therapy (ACT). Client satisfaction and proficiency with the selected ACT regimen is essential for adherence.
Assess for barriers to ongoing airway clearance. The identification of barriers to ACT can guide interventions to improve adherence.
Provide the opportunity for exercise and physical therapy. Exercise supports the goal of airway clearance by loosening the mucus and facilitating effective coughing. It also improves overall physical conditioning and recovery.
Encourage a frequent and effective cough- particularly around airway clearance therapy (ACT). Habital cough suppression results in the retention of mucus; clients should be encouraged to actively and intentionally use effective coughing to clear airway mucus.
Administer bronchodilators before ACT as ordered. Bronchodilators are administered to treat hyperactivity and to reverse bronchospasm.
Administer mucolytics as ordered in concert with ACT. Mucolytics decrease the viscosity of the sputum and promote expectoration of secretions.
Administer pain medication as needed. Attention to pain relief facilitates the client being able to cooperate comfortably with ACT and exercise/physical therapy.
Collaborate with the client and staff to determine the preferred ACT. Options include:

  • Autogenic or postural drainage,
  • Handheld oscillating devices.
  • Mechanical chest wall percussion (vest).
  • Traditional chest physiotherapy.
The Cystic Fibrosis Transmembrane Conductance Regulator defect causes mucus to become dehydrated. Secretions in cystic fibrosis are generally thick, sticky, and more difficult to clear. Frequent airway clearance is a mainstay in the treatment of acute exacerbations, as well as an integral part of health maintenance in cystic fibrosis. Individualized care and client choice in therapy will affect adherence and the effectiveness of treatment. A variety of supportive devices is available. A respiratory therapist can be helpful in these selections.
Collaborate with the client and staff to ensure that the schedule for therapy is amenable to all and does not interfere with meals, rest times, or medications. ACT is time-consuming and must be part of a balanced treatment plan. ACT should not be done immediately after meals, nor should it interfere with rest or other required therapies such as medication and physical therapy; it therefore requires careful scheduling.
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See Also

You may also like the following posts and care plans:

Respiratory Care Plans

Care plans about respiratory system disorders:

Pediatric Nursing Care Plans

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Nursing care plans for pediatric conditions and diseases: 

Paul Martin is a registered nurse with a bachelor of science in nursing since 2007. Having worked as a medical-surgical nurse for five years, he handled different kinds of patients and learned how to provide individualized care to them. Now, his experiences working in the hospital is carried over to his writings to help aspiring students achieve their goals. He is currently working as a nursing instructor and have a particular interest in nursing management, emergency care, critical care, infection control, and public health. As a writer at Nurseslabs, his goal is to impart his clinical knowledge and skills to students and nurses helping them become the best version of themselves and ultimately make an impact in uplifting the nursing profession.

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