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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Deficient Knowledge

Nursing Diagnosis

May be related to

  • First exacerbation/hospitalization experience.
  • Ineffective or incomplete education in past.
  • Rapidly expanding clinical knowledge base with new interventions available.

Possibly evidenced by

  • Anxiety related to hospitalization
  • Frequent exacerbation
  • Inability to verbalize treatment regimen or rationale
  • Nonadherence to therapy
  • Uncertainty or questions
  • Verbalized misconceptions

Desired Outcomes

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  • Client and family will verbalize understanding of disease process, treatment recommendations, and goals of therapy.
Nursing Interventions Rationale
Assess the client’s baseline knowledge of the disease pathophysiology. Cystic fibrosis is a disease of the secretory glands, including mucus, and sweat glands, and affects many organs, especially the lungs and pancreas.
Assess the client’s knowledge of nutritional needs. An understanding of nutritional needs and options for meeting those needs promotes self-management.
Assess the client’s knowledge of infection control recommendations for individuals with cystic fibrosis. Individuals with cystic fibrosis should have a good understanding of infection prevention recommendations for their own protection and that of the greater CF community.
Assess the client’s knowledge of the genetics of cystic fibrosis. Cystic fibrosis is caused by a defect in the CF transmembrane conductance regulator (CFTR) gene, a gene that makes a protein that controls the movement of salt and water into and out of body cells. Children who inherit a defective CFTR gene from each parent will have CF.
Assess the client’s knowledge of medications, airway clearance therapies, and equipment. Complex treatment regimens in CF can be difficult to maintain, and the regular assessment of knowledge can identify gaps in therapy and sources of nonadherence.
Assess the client’s knowledge of diagnostic testing, including laboratory evaluations, pulmonary function testing, airway cultures, and pathogens. Client understanding of individual baseline lung function and usual airway pathogens helps with the understanding of treatment recommendations and goals of therapy.
Provide basic information on CF pathophysiology and treatment goals using reliable resources. Currently, there is no cure for CF. Goals of treatment are to prevent lung infections, remove thick mucus from the lungs, prevent intestinal blockage, enhance nutrition, and reduce the risks of dehydration. With meticulous self-management and medical care, clients are living longer (many into their 40s and 50s).
Provide information about support groups. Living with a chronic disease can be emotionally challenging. Groups that share common experiences can be helpful.
Review pulmonary function testing and individual trends. These data provide a guide for progression or improvement in the disease.
Review ACT and breathing techniques to dislodge mucus. Chest physiotherapy is one of the mainstays of treatment. Clients often find it difficult to perform; strategies or adjunct aids to assist in effective performance need to be discussed.
Review infection control recommendations. Care providers have a responsibility to ensure that clients and families are informed about infection control strategies for optimal prevention of new pathogen acquisition.
Review pulmonary medications and nutritional/digestive medications and their rationales. Complex medication regimens in CF change over time, and frequent review helps to clarify their purpose and supports adherence. Pulmonary medications may include antibiotics, bronchodilators, anti-inflammatory medications, and mucus thinners. Nutritional medications may include pancreatic enzymes, vitamin supplements, and salt.
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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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