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Cystic Fibrosis (Reading & Sharing Part 2)

Bronchiectasis develops early in the course of cystic fibrosis, being detectable in infants as young as 10 weeks of age, and is persistent and progressive. Recent data reveal that neutrophil elastase activity in BAL fluid in early life is associated with early bronchiectasis in children with cystic fibrosis.

Nursing Assessment

  • Check for family history of CF, failure to thrive, and unexplained infant death; check the child’s history and physical condition. Carefully listen for subtle information that may suggest CF.
  • Assess respiratory status – respiratory rate, presence of tachypnea, wheeze, cough, character of sputum, and oxygen saturation level.
    1. Increased work of breathing.
    2. Quality of breath sounds by auscultation
    3. Child’ perception of respiratory status
    4. Ability to participate in activities of daily living, exercise tolerance, quality of sleep
    5. Assess for oxygen desaturation with sleep
  • Assess nutritional status and characteristics of stool

Nursing Diagnoses

  • Ineffective Airway Clearance related to thick pulmonary secretions (Nursing Alert: B. cepacia affects 5% to 10% of CF patients, is associated with a rapid decline in pulmonary function, and is multiple antibiotic resistant. Twenty percent of colonized patients develop fulminant septicemia and necrotizing pneumonia leading to death.)
  • Risk for infection related to thick, tenacious secretions
  • Imbalanced Nutrition- less than body requirements related to decreased appetite or inadequate absorption
  • Disturbed body image related to chronic disease process
  • Interrupted family processes related to the child with a chronic disease

Nursing Interventions

  • Promoting Airway Clearance
  • Preventing Infection
  • Promoting Adequate Nutrition
  • Enhancing self-esteem and body image
  • Enhancing family processes

Family Education and Health Maintenance

  1. Teach parents to have a thorough understanding of the dietary regimen and special need for calories, fat, and vitamins. Consultation with a registered dietitian is recommended.
  2. Discuss the child’s need for salt replacement and free access to salt as well as the increased need for salt during hot weather or in the presence of fever, vomiting, or diarrhea.
  3. Help the parents to become skilled at CPT (Chest physiotherapy) and other pulmonary treatments. Demonstrate and explain procedures and evaluate their return demonstration.
  4. Help the family to schedule care for the child within the framework of family life.
  5. Help the parents to provide emotional support to their child. The child needs love, understanding, and security, not overprotection.
  6. Stress the importance of regular medical care.
  7. Inform the family about the CF Foundation Guidelines for CF care.
  8. Discuss with parents the limitations and expectations for the child.
  9. Suggest parents and child meet other CF families
  10. Investigate home care options for families, especially respite services for caregivers.
  11. After the diagnosis is confirmed, refer the parents to genetic counseling. It is important for the parents to realize that the affected child inherits the defective gene from both parents. Each pregnancy will result in a 25% chance that the infant will have CF, a 50% chance that it will be a carrier of the CF gene and not have CF, and a 25% chance that the infant will neither be a carrier of the CF gene nor have CF.
  12. Refer families for additional information and support to agencies such as Cystic Fibrosis Foundation.
  13. The pediatric health care team should discuss with the patient and parents the concept of eventual transitioning of care from a pediatric CF center to an adult CF center as early as possible- an important issue that should not be left until adolescence.

Sadly, at some point, it will become apparent to the health care team that the time has arrived to discuss end-of-life issues. The nursing role is to assist the child and family to cope with the experience in such a way that it will promote growth rather than destroy family integrity and emotional well-being.

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