Cystic Fibrosis

Nursingbulletin.com
: One-stop Hub Philippine Nursing News and
Resources, Nursing Licensure Examinations,
Nursing Board Exam Results, Nursing
Updates. 
 Cystic Fibrosis
• - a multi-system disorder (autosomal
recessive trait disorder)
• - Common symptoms:
• pancreatic enzyme deficiency - duct
blockage
• chronic lung disease – infection
• sweat gland dysfunction - increased
NaCl sweat concentrations
• - SWEAT CHLORIDE TEST
 CY STI C F IBROS IS
• RESPIRATORY SYSTEM
• - Stagnation of the mucus in the airway

• - Emphysema & atelectasis

• - Chronic hypoxemia
• - Pneumothorax
• - Wheezing & dry non-productive cough
• - Dyspnea
• - Cyanosis
• - Clubbing of the fingers & toes
• - Repeated episodes of bronchitis & pneumonia
 CY STI C F IBROS IS
• GASTROINTESTINAL SYSTEM
- Meconium ileus in the neonate
- Intestinal obstruction
S/S: pain, abdominal distention N&V
- Stearrhea
- Deficiency of A,D, E & K; Malabsorption
Syndrome
- Malnutrition & failure to thrive
- Rectal prolapse
 CY STI C F IBROS IS
• INTEGUMENTARY SYSTEM
- ↑ of Na & Cl in sweat
- Dehydration & electrolyte imbalances
during ↑ weather
• REPRODUCTIVE SYSTEM
- Delayed puberty in females
- Infertility
- Sterility
 CY STI C F IBROS IS
• DIAGNOSTIC TESTS

• 1. Sweat Chloride Test

• 2. Chest X-ray
• 3. Pulmonary function test
• 4. Stool / fat or Enzyme analysis
 CY STI C F IBROS IS
• NURSING CARE FOR THE RESPIRATORY SYSTEM
A. Preventing pulmonary infection
B. Antimicrobial meds.
C. Chest Physiotherapy
D. Bronchodilators
E. Flutter Mucus Clearance Device
F. Therapy Vest Device
G. Recombinant Human Deoxyribonuclease
(DNASE)
 CY STI C F IBROS IS
• NURSING CARE (RESPIRATORY SYSTEM)
- Do not give cough suppressants
- Forced expiratory technique (huffing)
- Physical exercise
- Antibiotics prophylactically
- O2 as needed
- Monitor for hemoptysis
- Possible lung transplant
 CY STI C F IBROS IS
• NURSING CARE FOR THE GIT SYSTEM
- Pancreatic enzymes
- Encourage a well-balanced
- Monitor for failure to thrive
- Monitor for constipation & intestinal
obstruction
- Supplement salt during extremely hot
weather
 THE UNIVERSE ALWAYS CONSPIRES THE
DREAMER TO ACHIEVE ITS DREAMS!

DREAM, BELIEVE AND REALIZE!

Nursingbulletin.com