Cystic Fibrosis

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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 TESTSWEAT CHLORIDE TEST

CYSTIC FIBROSIS• RESPIRATORY SYSTEMRESPIRATORY 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

CYSTIC FIBROSIS• GASTROINTESTINAL SYSTEMGASTROINTESTINAL SYSTEM - Meconium ileus in the neonate - Intestinal obstruction S/S:S/S: pain, abdominal distention N&V - Stearrhea - Deficiency of A,D, E & K; Malabsorption

Syndrome - Malnutrition & failure to thrive - Rectal prolapse

CYSTIC FIBROSIS• INTEGUMENTARY SYSTEMINTEGUMENTARY SYSTEM

- ↑ of Na & Cl in sweat - Dehydration & electrolyte

imbalances during ↑ weather• REPRODUCTIVE SYSTEMREPRODUCTIVE SYSTEM

- Delayed puberty in females - Infertility - Sterility

CYSTIC FIBROSIS• DIAGNOSTIC TESTSDIAGNOSTIC TESTS

• 1. Sweat Chloride Test1. Sweat Chloride Test

• 2. Chest X-ray2. Chest X-ray

• 3. Pulmonary function test3. Pulmonary function test

• 4. Stool / fat or Enzyme analysis4. Stool / fat or Enzyme analysis

CYSTIC FIBROSIS• NURSING CARE FOR THE RESPIRATORY SYSTEMNURSING 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)

CYSTIC FIBROSIS• NURSING CARE (RESPIRATORY SYSTEM)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

CYSTIC FIBROSIS• NURSING CARE FOR THE GIT SYSTEMNURSING 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!

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