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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Infection

May be related to

  • Growth plume of known colonized CF airway pathogens
  • Invading viral organism
  • New acquisition of common cystic fibrosis airway pathogens

Possibly evidenced by

  • Decline in pulmonary function testing (spirometry)
  • Dyspnea/wheezing
  • Elevated white blood cell count
  • Fever
  • Increased sputum
  • Increased or a new cough
  • Malaise
  • Pathogenic microbes on airway culture

Desired Outcomes

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  • Client will experience improvement in infection and suppression of bacterial growth, as evidenced by a decrease in a cough, mucus production to baseline, normothermia, and normal white blood cell count.
Nursing Interventions Rationale
Assess for the general signs and symptoms of infection such as fever, cough, malaise, increased sputum, weight loss, tachypnea, and tachycardia. Pulmonary infections are associated with general symptoms of infection, along with an increase in respiratory rate and heart rate.
Obtain a sputum specimen for culture and sensitivity as ordered. The acquisition of new pathogens is often associated with CF pulmonary exacerbation. Common CF pathogens include Staphylococcus aureus, Staphylococcus aureus, Pseudomonas aeruginosa, Methicillin-resistant Staphylococcus aureus (MRSA), Achromobacter xylosoxidans, Burkholderia cepacia, and Stenotrophomonas Maltophilia. Airway culture and sensitivities guide infection control measures, as well as antimicrobial selection.
Monitor oxygen saturation. Oxygen requirements can increase during acute respiratory infections or in response to fever. The noninvasive measurement of oxygen saturation by pulse oximetry provides for the early recognition of impaired oxygen saturation.
Monitor the client’s immunoglobulin E (IgE) as ordered. CF pulmonary exacerbations can result from allergic bronchopulmonary aspergillosis (ABPA), an allergic response to Aspergillus in the airways. IgE will be significantly elevated.
Monitor pending culture and sensitivity results for drug resistance. Drug resistance may necessitate a change in antibiotics. A new organism may alter infection control precautions.
Monitor viral studies as ordered. Pulmonary exacerbations can occur in response to or concurrently with a viral illness.
Monitor white blood cell count as ordered. WBC can be elevated in response to pulmonary exacerbation or infection with a new organism.
Monitor the client’s antibiotic levels as ordered. Inadequate dosing can result in resistance and/or a poor therapeutic response. Toxicity can result in poor outcomes or adverse events.
Ensure that clients with CF are not cohorted. The cohorting of clients with CF is not recommended based on published CF Infection Control Consensus Guidelines.
Administer antipyretics as ordered, avoiding nonsteroidal anti-inflammatory drugs (NSAIDs) in clients receiving IV aminoglycosides. Antipyretics maintain normothermia and reduce metabolic needs. Aminoglycosides and NSAIDs in combination can cause renal toxicity.
Administer IV antibiotics as ordered in a timely manner. Benchmark institutions set the standard for the first dose of IV antibiotics within 4 hours of admission.
Consider an audiology evaluation in clients who have a history of frequent IV aminoglycoside use. Ototoxicity is a common adverse effect of aminoglycoside use.
Institute the appropriate infection control precautions. Many CF pathogens require clients to be in contact isolation based on the Centers for Disease Control and Prevention (CDC) and Cystic Fibrosis Foundation (CFF) Consensus Guidelines.

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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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