Chronic Respiratory Conditions

Asthma

Chronic inflammatory airway disorder involving bronchoconstriction, airway edema/inflammation, and ↑ mucus

Why can asthma eventually cause permanent lung-function changes?

Repeated airway inflammation can cause airway remodeling, leading to permanent decrease in lung function

Why is silent chest more concerning than wheezing in a child with asthma?

Airway obstruction is so severe that there may not be enough airflow to produce wheezing sound

What is peak-flow meter used in asthma?

Helps children with asthma monitor airway narrowing and recognize signs early

Status asthmaticus

Prolonged, severe asthma attack that does not respond adequately to usual treatment (e.g. albuterol), and can progress to respiratory failure

Complications of status asthmaticus

Severe respiratory distress, SpO2 <92%, restlessness, diaphoresis, can’t speak full words, wheezing or silent chest

What monitoring is needed for severe status asthmaticus?

Continuous pulse ox and cardiopulmonary monitoring (electrolyte imbalance), with frequent reassessment of respiratory status

What are corticosteroids used for in someone with severe status asthmaticus?

Reduces inflammation

What is magnesium sulfate used for in someone with severe status asthmaticus?

Relaxes bronchial smooth muscles

What is ketamine used for in someone with severe status asthmaticus?

Used in severe cases when sedation/intubation may be required

Heliox

Lightweight helium-oxygen mixture given w/ nonrebreather that decreases turbulent/unstable airflow → ↓ airway resistance →  ↓ work of breathing

Cystic Fibrosis (CF)

Autosomal recessive disorder affecting the CFTR gene → abnormal chloride and water transport → abnormally thick, sticky secretions that interfere with exocrine glands

How does CF affect the GI system?

Thick mucus blocks enzymes from pancreatic ducts → poor digestion, malabsorption, abdominal distention, large greasy bad smelling poop

How does CF affect the integumentary system?

Sweat will contain abnormally high amount of chloride → salty tasting skin and increased risk for fluid/electrolyte loss (esp. in hot weather)

How does CF affect the respiratory system?

Thick mucus plugs small airways → recurrent respiratory infections, chronic cough, airway obstruction, impaired gas exchange

How does CF affect reproductive system for boys?

Thick/tenacious seminal secretions and vas deferens abnormalities → usually infertile

How does CF affect the reproductive system for girls?

Thick cervical secretions can make sperm penetration more difficult

What newborn findings may suggest cystic fibrosis?

Failure to regain birth weight by 4-6 weeks, failure to pass stool within 24 hrs, and salty tasting skin

Why is failure to pass meconium (first stool ever) within 24 hrs concerning for CF?

May indicate meconium ileus where thick intestinal contents obstruct the bowel → abdominal distention, vomiting, failure to pass

What CF findings may appear around 1-2 months?

Constant hunger, feeding problems, vomiting, abdominal distention, and large greasy foul smelling poop

What CF findings may appear around 4-6 months?

Thick mucus in airways lead to recurrent respiratory infections (trapped bacteria not excreted)

What is a key/confirmatory diagnosis test for cystic fibrosis?

Sweat chloride test, which measure abnormally high chloride concentration in sweat

What might chest x-ray show in a child with CF?

Hyperinflation, thickened bronchial walls, atelectasis, or infiltrates

When should pancreatic enzymes be given to child with CF?

Give before, with, or within 30 minutes after meals/snacks to replace pancreatic enzymes to digest and absorb nutrients

What type of diet is recommended in children with CF?

High calorie+protein, fat-soluble vitamins, extra salt

What vitamins are commonly supplemented in CF?

A, D, E and K

Why should diapers be changed promptly after bowl movements in infants with CF?

CF stools contain fat which can irritate skin and cause breakdown of skin integrity

What do pancreatic enzymes do?

Breaks down fat, carbs, and protein so intestines can absorb nutrients