Lesson Objectives for Cystic Fibrosis

• Understanding Cystic Fibrosis (CF) Basics:
  • Gain knowledge about the etiology, pathophysiology, and clinical manifestations of cystic fibrosis, including the impact on the respiratory and digestive systems.
• Caring for Patients with CF:
  • Learn the principles of providing holistic care for individuals with cystic fibrosis, including the management of respiratory and nutritional challenges, infection prevention, and psychosocial support.
• Medication Management:
  • Understand the various medications used in the treatment of cystic fibrosis, including bronchodilators, mucolytics, antibiotics, and pancreatic enzymes, and learn the appropriate administration and monitoring procedures.
• Nutritional Support and Monitoring:
  • Explore strategies for promoting optimal nutrition in individuals with CF, emphasizing the importance of pancreatic enzyme replacement therapy, nutritional supplements, and dietary modifications.
• Psychosocial Considerations:
  • Recognize the psychosocial aspects of living with cystic fibrosis, including the impact on mental health, coping mechanisms, and strategies for providing emotional support to individuals and their families.

Pathophysiology of Cystic Fibrosis (CF)

• Defective CFTR Gene:
  • Cystic fibrosis results from mutations in the CFTR (cystic fibrosis transmembrane conductance regulator) gene located on chromosome 7, leading to a dysfunctional CFTR protein.
• Impaired Chloride Transport:
  • The CFTR protein is responsible for regulating chloride ion transport across cell membranes. Mutations cause reduced chloride secretion and increased sodium absorption, leading to thick, sticky mucus production.
• Viscous Mucus Production:
  • Abnormal CFTR function results in the production of thick, sticky mucus in various organs, particularly affecting the respiratory and digestive systems. This mucus obstructs airways and ducts, causing complications.
• Respiratory Complications:
  • In the respiratory system, the thick mucus obstructs airways, promoting bacterial colonization and recurrent infections. Chronic inflammation and damage to lung tissue contribute to progressive respiratory decline.
• Digestive System Involvement:
  • In the digestive system, the thick mucus obstructs pancreatic ducts, leading to insufficient release of digestive enzymes. This impairs nutrient absorption and causes malnutrition. Liver and gallbladder complications may also occur.

Etiology of Cystic Fibrosis (CF)

• Genetic Inheritance:
  • Cystic fibrosis is an autosomal recessive genetic disorder, meaning that an individual must inherit two defective CFTR genes (one from each parent) to develop the condition.
• CFTR Gene Mutations:
  • The etiology is linked to mutations in the CFTR gene, specifically in the code for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. Various mutations can occur, affecting the functionality of this protein.
• Carrier Parents:
  • Parents who carry a single mutated CFTR gene are carriers and do not exhibit symptoms. When both parents are carriers, there is a 25% chance with each pregnancy that the child will inherit two mutated genes and develop cystic fibrosis.
• Ethnic and Geographic Variations:
  • The prevalence of specific CFTR gene mutations can vary among different ethnic groups and geographic regions. Certain mutations are more common in specific populations.
• New Mutations:
  • In rare cases, individuals may develop cystic fibrosis due to new mutations in the CFTR gene that occur spontaneously. This can happen even if there is no family history of the condition.

Desired Outcome for Cystic Fibrosis (CF) Management

• Optimal Respiratory Function:
  • Improve and maintain optimal lung function to minimize respiratory symptoms, reduce the frequency of respiratory infections, and enhance overall respiratory well-being.
• Nutritional Adequacy:
  • Achieve and maintain proper nutritional status by addressing malabsorption issues and ensuring adequate caloric intake. This includes promoting healthy growth and development in pediatric patients.
• Effective Mucus Clearance:
  • Enhance and facilitate mucus clearance from the airways to reduce the risk of airway obstruction, infections, and respiratory complications.
• Quality of Life Improvement:
  • Enhance the overall quality of life by managing symptoms effectively, promoting independence, and addressing psychosocial aspects of living with cystic fibrosis.
• Prevention of Complications:
  • Minimize and manage complications associated with cystic fibrosis, such as diabetes, liver disease, and osteoporosis, through comprehensive care and proactive interventions.

Cystic Fibrosis Nursing Care Plan

Nursing Interventions and Rationales

• Assess respiratory status; note rate, rhythm, and quality of breathing; auscultate lungs

• Review growth charts, assess changes or decrease in height or weight

• Assess abdomen
  • Look for distention
  • Listen for active bowel sounds and gas pattern
  • Feel – palpate for mass or signs of constipation

• Insert and monitor nasogastric feeding tubes

• Monitor nutritional status
  • Caloric intake
  • Excessive thirst or hunger
  • Fiber intake
  • Use of feeding tube

• Monitor blood glucose

• Assess for signs of infection
  • Monitor temperature
  • Note cough with or without sputum
  • Obtain sputum culture and sensitivity

• Monitor for signs of dehydration and encourage fluid and salt intake

• Assess for bone malformation, deformity or fractures

• Administer medications and supplements appropriately

• Suction secretions as necessary per facility protocol

• Perform chest physical therapy or vest therapy 2 – 4 times per day per facility protocol

• Provide education for home treatment and resource information for patients and parents

Evaluation for Cystic Fibrosis (CF)

• Respiratory Function:
  • Monitor lung function through regular pulmonary function tests to assess the effectiveness of respiratory treatments.
  • Evaluate the frequency and severity of respiratory infections and the need for antibiotic interventions.
• Nutritional Status:
  • Assess weight gain and growth patterns, monitoring for improvements in nutritional status.
  • Review enzyme replacement therapy effectiveness in supporting nutrient absorption.
• Medication Adherence:
  • Evaluate the patient’s adherence to the prescribed medication regimen.
  • Assess the impact of medications on symptom management and overall well-being.
• Psychosocial Well-being:
  • Assess the emotional well-being of the patient and family, considering the impact of CF on their daily lives.
  • Monitor participation in support groups and utilization of mental health resources.
• Self-Management Skills:
  • Evaluate the patient’s ability to independently perform airway clearance techniques.
  • Assess the understanding and application of information provided regarding early signs of respiratory infections and appropriate actions.

References

• https://kidshealth.org/en/parents/cf.html
• https://childrensnational.org/choose-childrens/conditions-and-treatments/airway-lungs/cystic-fibrosis
• https://www.cincinnatichildrens.org/health/c/cystic-fibrosis
• https://www.mayoclinic.org/diseases-conditions/cystic-fibrosis/diagnosis-treatment/drc-20353706