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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

Cystic Fibrosis

Gina Brandl, RN BSN

Nursing Instructor, Pediatrics

cystic fibrosis overview of disease
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
etiology
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.)

pathophysiology
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)
symptoms
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
diagnostic tests
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
common nursing diagnoses
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
planning interventions
Planning & Interventions
  • Provide respiratory therapy
  • Administer medications
  • Meet nutritional needs
  • Provide psychosocial support
  • Discharge planning and home teaching
treatments
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
treatments10
Treatments
  • Bronchial airway drainage
    • Postural drainage
  • Oral enzymes and better nutrition
    • High calorie diet
    • Special vitamins & pancreatic enzymes
  • Lung transplant
  • Pain relievers
    • Ibuprofen
expected outcomes
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
parent and child education
Respiratory infections

Avoid exposure

Chest percussion & postural drainage

Diet

Community resources

Genetic counseling

Written information

Home care

Support services

Parent and Child Education
critical thinking exercise
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.
critical thinking exercise14
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?
references
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
references16
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