Sheet 1
Cystic Fibrosis
A multi-system genetic disease, primarily affecting lungs and digestive system
  Etiology
    How common is it?
      1 : 3600
      One person per week dies from CF
      50% dx by age six months
    Risk Factors
      Inheriting 2 defective CF genes
      1 : 25 is a carrier of CF gene
      When 2 carriers have a child:
25% chance of CF
50% no CF; but a carrier
25% no CF; not a carrier
  Pathophysiology
    - Mutations in the CFTR gene = cAMP problems = poor chloride transport across epithelial cells on mucosal membranes
- Decreased Cl secretion + increased Na reabsorption = thickness of mucus
  Manifestations
    Dyspnea
    Severe respiratory infections
    Chronic lung infections
    Wet, rattling productive cough
    Poor weight gain or growth
    Difficulty digesting food
  Diagnostics
    Tests
      Sweat Test; measures level of salt in sweat (high is a concern)
      Genetic tests (DNA)
      Newborn screening program (blood test)
    Nursing Care
      Nothing specific
  Treatment
    Medical
      Airway Clearance
- postural drainage with percussion is most common
http://www.cysticfibrosis.ca/assets/files/pdf/List_Airway_Clearance_TechniquesE.pdf
      Transplant
- Lung
- Liver
- Heart
    Pharmacological
      Mucolytics
- Break up mucus
(acetylcysteine; Mucomyst or guiafenesin; Robitussin)
      Bronchodilators
- open AW
(Ventolin
- Nebulized treatment
      Steroids
- decrease inflammation
(prednisone or Flovent)
      Antibiotics
    Nutrition
(high calorie + high protein + dash of salt)
      Pancreatic Enzymes
- aid in digestion and absorption
- Without use = abdo pain, gas, diarrhea
- Most often enteric coated; powder available
      High Calorie
- Poly/Omega 3 (fish, flaxseed, canola oil, nuts)
- Monounsaturated (olive oil, peanut oil, peanut butter)
- Saturated (red meat, pork, chicken, butter, 3.25% milk, cheese)
- Trans (milk, cheese)
      Vitamin Supplements
- Mostly A, D, E, K (fat soluble)
  Nursing Process
    Assessment
1. Breathing
2. GI
3. Psychosocial
    Interventions
1. Chest physio, deep breathing, bronchodilators, infection control, antibiotics
2. Frequent weight, pancreatic enzymes, vitamins
3. Parents present, support, advocacy, refer
    Education
1. Infection control
2. Nutritional intake
3. Organization referral, social worker, listening
  Medical Complications
    Allergic Bronchopulmonary Aspergillosis (ABPA)
- allergic reaction to a fungus (occurs in 25-35%)
- treated with anti-fungal and corticosteroids
    Pancreatitis
- treated with IV antibiotics and/or surgery
    Liver Disease
- occurs in 25%
- r/t increased bile = thick fluids in liver = poor flow in and out
- treated with transplant
    CF Related Diabetes (CFRD)
- occurs in 40-60%
    Nasal Polyps
- occurs in 10-50%
- r/t chronic inflammation
  Psychosocial Considerations
    Anxiety & Fear
    Body image
    Guilt
    Financial