Cystic Fibrosis - PEDS

Define Cystic Fibrosis

Respiratory disorder that results from inheriting a mutated gene

Mucus glands secrete an increase in the quantity of thick, tenacious mucus, which leads to mechanical obstruction of organs, increase in sodium & chloride in sweat, & ANS abnormalities

Risk Factors (2)

• Both biological parents carry the recessive trait for cystic fibrosis.

• Non-Hispanic white American ethnicity.

Expected Findings (3)

• Family history

• Medical history of respiratory infections or growth failure.

• Meconium ileus at birth manifested as distention of the abdomen, vomiting, & inability to pass stool; Earliest indication of cystic fibrosis in newborns

Respiratory Findings (Early) (3)

• Stasis of mucus increases the risk for respiratory infections.

• Wheezing, rhonchi

• Dry, nonproductive cough

Respiratory (Increased Involvement) Findings (3)

• Dyspnea

• Paroxysmal cough

• Obstructive emphysema & atelectasis on CXR

Respiratory (Advanced Involvement) Findings (4)

• Cyanosis

• Barrel-shaped chest

• Clubbing of fingers & toes

• Multiple episodes of bronchitis or bronchopneumonia

GI Findings (8)

1. Steatorrhea - Frothy, foul smelling, floating, fatty (greasy)

2. Voracious appetite (early), loss of appetite (late)

3. Failure to gain weight or weight loss

4. Delayed growth patterns

5. Distended abdomen or thin arms & legs (infant)

6. Deficiency of fat-soluble vitamins

7. Anemia & reflux

8. Prolapsed rectum

Integumentary Findings

Sweat, tears, & saliva have an excessively high content of sodium & chloride.

Labs (2)

• Blood specimen - Nutritional panel to detect a deficiency of fat-soluble vitamins (A, D, E, & K)

• Sputum culture for detection of infection

Diagnostic Procedures (7)

1. DNA Testing - Isolates mutation

2. PKU - Newborns

3. PFTs

4. CXR - Can indicate diffuse atelectasis and obstructive emphysema

5. AXR - Detects meconium ileus

6. Stool analysis -For presence of fat and enzymes

7. Sweat Chloride Tests - Expected chloride < 40 & sodium < 70

Diagnostic Confirmation - Sweat Chloride Test

Chloride greater than 40 for infants < 3 months & greater than 60 for all others.

Pulmonary Management (6)

1. Airway Clearance Therapy (ACT

2. Chest Physiotherapy (CPT) - With postural drainage, manual or mechanical percussion

3. Active Cycle Breathing Techniques - Huffing or forced expiration

4. Aerosol Therapy - Bronchodilators, human deoxyribonuclease; Recommended prior to ACT.

5. Physical aerobic exercises

6. O2 as prescribed - For CO2 retention

ACT - Nursing Actions (4)

• Assist in providing ACT & teach parents to promote expectoration of pulmonary secretions.

• Usually prescribed twice a day in the morning & evening.

• Avoid ACT immediately before or after meals.

• Several methods of ACT are available.

GI Management (4)

• Provide a well-balanced diet high in protein & calories.

• Administer pancreatic enzymes within 30 min of eating a meal or snack.

• Administer vitamins- multivitamin, vitamins A, D, E, and K. (fat soluble)

• Consult a dietitian - Child should receive regular nutritional evaluations.

Pancreatic Enzymes (5)

• Pancrelipase treats pancreatic insufficiency associated with cystic fibrosis.

• Gravy analogy - monitor stools for dosing (1-2/day)

• ALL meals & snacks

• Swallow or sprinkle capsules

• Increase dose with high fat foods

Interprofessional Care (4)

• Respiratory

• PT

• Pulmonoligists

• Dieticians