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Cystic Fibrosis

Cystic Fibrosis. Gina Brandl, RN BSN Nursing Instructor, Pediatrics. Cystic Fibrosis – Overview of Disease. Cystic fibrosis (CF) - inherited autosomal recessive disorder in children Most common cause of chronic lung disease in children – 30,000 children & adults in US Incidence

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Cystic Fibrosis

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  1. Cystic Fibrosis Gina Brandl, RN BSN Nursing Instructor, Pediatrics

  2. Cystic Fibrosis – Overview of Disease • Cystic fibrosis (CF) - inherited autosomal recessive disorder in children • Most common cause of chronic lung disease in children – 30,000 children & adults in US • Incidence • Annually 1,000 children diagnosed • 70% of patients are diagnosed by age 2 • 40% of CF population is age 18 and older • Median life span

  3. Genetically transmitted disease Genes (DNA) From parents Gene located on 7th chromosome 2 copies of genes needed to inherit disease Carrier states Etiology (National Institutes of Health, n.d.)

  4. Defective gene Related to protein involved in chloride ion transport Body produces thick, sticky mucus Clogs the lungs Stagnant mucus Obstructs the pancreas Malabsorption & malnutrition Pathophysiology • (MedlinePlus, 2006)

  5. Symptoms • Symptoms may include • Meconium ileus • Appears at birth • Salty-tasting skin • When newborn is kissed • Steatorrhea • Greasy, bulky and foul smelling • Poor growth/weight gain in spite of good appetite • Chronic coughing, at times with phlegm • Frequent lung infections

  6. Sweat Test Measures sodium or chloride in person’s sweat Two samples Ensure false-positive does not occur Not reliable on newborns Genetic Analysis Newborn with signs and symptoms may confirm diagnosis with blood test. Inherited disease Recommend checking family members and first cousins Diagnostic Tests

  7. Common Nursing Diagnoses • Ineffective airway clearance r/t tracheobronchial secretions and obstruction • Imbalanced nutrition: less than body requirements r/t inability to digest food or absorb nutrients • Risk for infection r/t chronic pulmonary disease • Interrupted family processes r/t chronic illness

  8. Planning & Interventions • Provide respiratory therapy • Administer medications • Meet nutritional needs • Provide psychosocial support • Discharge planning and home teaching

  9. Treatments • Aimed at relieving symptoms and complications • Antibiotics • Aerosolized • Mucus-thinning drugs • Thins secretions • Easier to cough up • Bronchodilators • Relaxes smooth muscles in the airways

  10. Treatments • Bronchial airway drainage • Postural drainage • Oral enzymes and better nutrition • High calorie diet • Special vitamins & pancreatic enzymes • Lung transplant • Pain relievers • Ibuprofen

  11. Expected Outcomes • The expected outcomes of nursing care include • Family and/or child demonstrate proficiency in • Providing pulmonary care • Reducing pulmonary infections • Developing a schedule for pulmonary cares that fits into family needs • Adequate calories and pancreatic enzymes are consumed to support growth of the child to stay within developmental weight ranges

  12. Respiratory infections Avoid exposure Chest percussion & postural drainage Diet Community resources Genetic counseling Written information Home care Support services Parent and Child Education

  13. Critical Thinking Exercise • A 9 month-old infant has a pattern of recurrent hospitalizations of respiratory difficulty, recurrent bronchitis with thick mucus production and poor weight gain with thin extremities. His history revealed a meconium ileus at birth. • VS: 101.8 (F)-144-54. A sweat chloride test confirms a diagnosis of cystic fibrosis. The physician suggests genetic testing.

  14. Critical Thinking Exercise • The family asks why genetic testing is needed. How should the nurse respond to the parent’s question? • Describe the significance of the sweat chloride test in relation to the disease process of cystic fibrosis. • What are the issues related to having a child with a chronic respiratory illness? • The family asks why their child has not been gaining weight. How would you respond to this question? • Diagnosed during infancy or early childhood, what are the three major presentations of a child with cystic fibrosis to a primary care provider?

  15. References • Cystic Fibrosis Foundation. (2007). About cystic fibrosis: Frequently asked questions. Retrieved November 18,2007, from the website http://www.cff.org/AboutCF/Faqs/ • Mayo Clinic. (2007). Cystic fibrosis – diseases and conditions. Retrieved November 23, 2007, from the website http://www.mayoclinic.com/health/cystic-fibrosis/DS00287/DSECTION=6

  16. References • MedlinePlus. (2006). Cystic fibrosis. Retrieved November 18, 2007, from the website https://www.nlm.nih.gov/medlineplus/ency/imagepages/18135.htm • National Institutes of Health. (2006). What causes cystic fibrosis?. Retrieved November 21, 2007, from the website www.nhlbi.nih.gov/.../Diseases/cf/cf_causes.html

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