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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Imbalanced Nutrition: Less Than Body Requirements

Nursing Diagnosis

May be related to

  • Chronic malabsorption
  • Increased caloric needs
  • Poor nutritional status resulting from anorexia

Possibly evidenced by

  • Abnormal eating behaviors
  • Abnormal stool patterns (diarrhea or constipation)
  • Anemia
  • Chronic abdominal discomfort
  • Gastroesophageal reflux
  • Hypoalbuminemia
  • Low fat-soluble vitamin levels
  • Muscle wasting
  • Weight loss, weight plateau
  • 10% to 20% below ideal weight

Desired Outcomes

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  • Client will maintain adequate nutritional status or demonstrate weight gain on a trajectory to adequate nutritional status.
  • Client will be free of signs/symptoms of malabsorption.
Nursing Interventions Rationale
Assess the abdomen for bloating, fullness, bowel sounds, or palpable stool mass. Monitor stool patterns for frequency, odor, consistency, and the presence of oil or grease. Undertreated malabsorption is common in cystic fibrosis; symptoms may be normalized by clients and families; adjustments in pancreatic enzyme supplementation are warranted with the signs or symptoms of malabsorption.
Assess the skin’s color, integrity, and turgor. Malnutrition can be associated with an alteration in skin integrity. CF-related liver disease can result in jaundice.
Monitor for an increase in weight and appetite. Increasing trends in weight and appetite accompany the resolution of pulmonary exacerbations.
Monitor for excessive thirst, urination, and hunger. Obtain bedside blood glucose readings as ordered. CF-related diabetes occurs in up to 30% of adolescents and adults with CF; glucose intolerance can be present intermittently with pulmonary exacerbations.
Monitor serum chemistry as ordered. Hypoalbuminemia, an elevation in liver enzymes, hyperglycemia, and alterations in electrolyte balances can all represent complication in cystic fibrosis.
Encourage liberal hydration and high fiber intake. Chronic constipation can result from mucus secretion and dehydration in the intestinal lumen (especially when malabsorption is adequately treated and stool consistency is normalized).
Encourage the liberal use of salt or salty food intake. Hyponatremic dehydration and salt loss occur easily in the client with cystic fibrosis as sequelae to CF defect in the sweat glands.
Encourage a high protein, high-calorie diet. Client with cystic fibrosis has increased caloric needs as much as 1.2 to 1.5 of the daily recommended amounts.
Administer all fat-soluble vitamins with meals and enzymes. Fat-soluble vitamin deficits are common in cystic fibrosis because of fat malabsorption. Vitamin supplements must be taken with pancreatic enzymes to be absorbed.
Administer pancreatic enzymes before all meals and snack containing fat or protein as ordered. Client with cystic fibrosis requires pancreatic enzyme supplementation to adequately digest food containing fat or protein.
Collaborate with a registered dietician in obtaining a full nutritional evaluation. Multidisciplinary input and collaboration will optimize therapeutic interventions. Clients with cystic fibrosis usually have a long history of impaired nutrition, and interventions need to be guided by baseline data.
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See Also

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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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