Respiratory Dysfunction - Outline

🫁 GENERAL ASPECTS OF RESPIRATORY INFECTIONS (PEDIATRIC)

🔹 1. Respiratory Tract Overview

Upper Respiratory Tract:

• Includes: Oronasopharynx, pharynx, larynx, and trachea

• Common infections: colds, sinusitis, pharyngitis, laryngitis

Lower Respiratory Tract:

• Includes: Bronchi, bronchioles, alveoli

• Common infections: bronchitis, bronchiolitis, pneumonia

Croup Syndromes:

• Involve infections of the epiglottis and larynx

• Can cause airway obstruction, stridor, and respiratory distress

🔹 2. Infectious Agents

Viral Agents (Most Common):

• Respiratory Syncytial Virus (RSV)

• Parainfluenza

Bacterial and Other Agents:

• Group A ß-hemolytic streptococcus – common in pharyngitis

• Staphylococci

• Chlamydia trachomatis, Mycoplasma pneumoniae, Pneumococci

• Haemophilus influenzae – can cause epiglottitis, otitis media

🔹 3. Age-Related Susceptibility

🔹 4. Pediatric vs. Adult Airway Differences

• Smaller airway diameter = more prone to obstruction

• Shorter airway length = faster spread of infections

• Smaller lungs = less oxygen reserve

• Obligate nose breathers (infants)

• Shorter, horizontal Eustachian tubes = frequent ear infections

• Fewer alveoli = less efficient gas exchange

🧠 Remember: Children decompensate faster than adults!

🔹 5. Seasonal Variations

• Most respiratory infections: Winter & Spring

• Mycoplasma infections: more common in Fall & Winter

• Asthmatic bronchitis: worse in cold weather

• RSV season: peaks in Winter and Spring

🔹 6. Clinical Manifestations

General Symptoms (Age-Dependent):

• Fever: Rare in newborns, peaks from 3 months to 3 years

• GI symptoms: Anorexia, vomiting, diarrhea, abdominal pain

• Respiratory symptoms:

  • Cough

  • Nasal congestion/discharge

  • Sore throat

  • Chest pain

  • ↑ HR and RR

  • Nasal flaring

  • Color changes (cyanosis, pallor)

  • Restlessness, irritability, confusion

  • Clubbing (chronic hypoxia)

Breath Sounds to Recognize:

• Crackles (rales) – fluid in alveoli (e.g., pneumonia)

• Wheezing – narrowed airways (e.g., asthma, bronchiolitis)

• Stridor – upper airway obstruction (e.g., croup, epiglottitis)

Retractions (signs of increased effort):

• Suprasternal (above sternum)

• Clavicular (above collarbone)

• Intercostal (between ribs)

• Substernal (below sternum)

• Subcostal (below ribs)

🔹 7. Nursing Considerations (ADPIE)

A. Assessment

• Vital signs, lung sounds, signs of distress

• Hydration status, behavioral changes

B. Diagnosis

• Ineffective airway clearance

• Impaired gas exchange

• Risk for dehydration

C. Planning

• Set realistic, age-appropriate goals

• Prioritize airway and hydration

D. Implementation

• Administer oxygen, antipyretics

• Encourage fluids and rest

• Isolate if contagious

• Educate family

E. Evaluation

• Reassess respiratory effort

• Monitor for resolution of symptoms

• Evaluate parent understanding

🔹 8. Assessment in Respiratory Distress

✅ Key Signs to Watch:

• Respiration quality: rate, depth, sounds

• Pulse: fast, weak?

• Skin color: pale, cyanotic?

• Cough: dry, wet, barking?

• Behavior: irritability, confusion, lethargy

• Dehydration: dry mucosa, sunken fontanel, ↓ urine output

🚨 Red Flag: Children can deteriorate rapidly—monitor closely!

🔹 9. Nursing Interventions for Respiratory Infection

• Ease breathing: humidified air, elevate HOB

• Manage fever: antipyretics (acetaminophen/ibuprofen), cool compress

• Promote rest & comfort: quiet environment, cluster care

• Prevent infection spread: hand hygiene, mask, isolation if needed

• Hydration & nutrition: encourage small, frequent fluids

• Educate & support family: explain signs to monitor, meds, return precautions

• Discharge Planning: follow-up care, home environment, when to seek help

🤧 UPPER RESPIRATORY TRACT INFECTIONS (URI)

🔹 1. Nasopharyngitis = “Common Cold”

• Most common type of upper respiratory infection

• Affects nasal passages and pharynx

🔹 2. Causes (Viral Agents)

• RSV (Respiratory Syncytial Virus)

• Rhinovirus

• Adenovirus

• Influenza virus

• Parainfluenza virus

🦠 Note: Caused by many viruses—this is why antibiotics are not effective.

🔹 3. Clinical Manifestations

Symptoms vary by age but commonly include:

• Fever: May or may not be present; higher in younger children

• Irritability, fussiness, and restlessness

• Nasal inflammation and congestion

• Decreased appetite and fluid intake

• Vomiting and diarrhea (often due to swallowed mucus)

🔹 4. Home Management

🏠 Depends on the child’s age and symptoms, but generally includes:

• Rest

• Fluids: Encourage hydration

• Nasal suctioning for infants

• Humidified air (cool mist vaporizer)

• Monitor for complications: ear infections, worsening cough, high fever

🔹 5. Pharmacologic Treatment

🚫 NOT Recommended:

• OTC pediatric cold remedies

  • Ineffective and may cause harmful side effects

• Antihistamines

  • Little to no benefit for viral URI symptoms

✅ Recommended:

• Antipyretics (e.g., acetaminophen, ibuprofen)

  • For fever and discomfort

• Cough suppressants (for dry cough only)

  • Use with caution due to potential alcohol content

• Decongestants (to reduce nasal swelling)

  • Nose drops/sprays (short-term use only; more effective than oral)

  • Avoid prolonged use due to rebound congestion

😷 ACUTE STREPTOCOCCAL PHARYNGITIS

🔹 Overview

• Infection of the upper airway caused by Group A β-hemolytic Streptococcus (GABHS)

• Highly contagious

• Risk for serious complications if untreated

⚠ Sequelae (Potential Complications)

• Acute Rheumatic Fever – can affect the heart, joints, skin

• Acute Glomerulonephritis – kidney inflammation

🩺 Clinical Manifestations

• Sudden sore throat

• Fever

• Headache

• Abdominal pain

• Enlarged, red tonsils with white patches

• Painful swallowing

• Rash (in some cases – scarlet fever)

🧪 Diagnostics

• Rapid Strep Test

• Throat Culture (gold standard if rapid test is negative)

💊 Pharmacologic Treatment

Penicillin (Drug of choice):

• Oral Penicillin V – for 10 days

  • Must complete full course to prevent rheumatic fever and kidney issues

  • Poor compliance in some children

• IM Penicillin G (one-time injection)

  • Solves compliance issue

  • Painful

  • Procaine Penicillin G – less painful alternative

  • ❌ NEVER give IV Penicillin G (can cause cardiac arrest)

Penicillin Allergy:

• Use Erythromycin

🧑‍⚕ Nursing Considerations

• Encourage medication compliance

• Educate family about completing antibiotics

• Monitor for complications: rash, joint pain, hematuria

• Promote hydration and rest

👅 TONSILLITIS

🔹 Pathophysiology & Etiology

• Inflammation of the palatine tonsils

• Often caused by viral or bacterial agents (commonly GABHS)

😷 Clinical Manifestations

• Tonsillar edema → obstructs air/food passage

• Difficulty swallowing and breathing

• Mouth breathing if adenoids also enlarged

• Sore throat, bad breath, fever, muffled voice

🩺 Therapeutic Management

• Tonsillectomy if:

  • ≥3 infections per year despite appropriate treatment

🧑‍⚕ Nursing Considerations

General Care:

• Encourage fluids

• Pain management

• Monitor for signs of bacterial infection

Postoperative Care:

• Airway management – position on side or stomach to facilitate drainage

• Bleeding risk:

  • Observe for frequent swallowing, pallor, restlessness, or throat clearing

  • Avoid suctioning

• Keep quiet environment

• Minimize crying/agitation (can increase bleeding)

• Comfort measures:

  • Ice collar

  • Pain medication (NO aspirin)

🦠 INFLUENZA (“FLU”)

🔹 Basics

• 3 Types: A, B, C

• Spread: Direct contact or contaminated surfaces

• Peak season: Winter

• Infectious 24 hrs before and 5–7 days after symptom onset

🤒 Clinical Features

• High fever

• Chills

• Cough

• Headache

• Sore throat

• Body aches

• Fatigue

• GI symptoms (esp. in children)

💊 Pharmacologic Treatment (Antivirals)

Must be started within 48 hours of symptom onset:

• Oseltamivir (Tamiflu) – oral, for children >2 weeks old

• Zanamivir (Relenza) – inhaled, for children >7 years

• Rimantadine – used for Type A (less common now)

⚠ Avoid Aspirin in children with influenza → risk of Reye syndrome (fatal liver and brain damage)

👂 OTITIS MEDIA (OM) – EAR INFECTION

🔹 Pathophysiology & Etiology

• Infection/inflammation of the middle ear

• Often follows an upper respiratory infection (URI)

• Caused by blocked Eustachian tubes → fluid buildup

• Common organisms: Streptococcus pneumoniae, Haemophilus influenzae

🔹 Types of Otitis Media

1. Acute Otitis Media (AOM):

• Sudden onset, fever, ear pain, irritability, pulling at ears

• Red, bulging tympanic membrane

• Altered cone of light

2. Otitis Media with Effusion (OME):

• Fluid without infection

• Hearing loss, fullness

• Tympanic membrane shows air bubbles or fluid line

• Tympanometry shows non-mobile tympanic membrane

🔹 Diagnostics

• Otoscopy: visual signs of effusion or inflammation

• Pneumatic otoscopy / tympanometry: test tympanic mobility

🔹 Therapeutic Management

Pharmacologic:

• Watchful waiting (≥6 months, non-severe symptoms): 72 hours

• Antibiotics required if:

  • <6 months of age

  • <2 years with persistent pain and fever

• Topical pain relief: benzocaine drops (Rx), warm compress

Antibiotic Therapy:

• 1st line: Amoxicillin PO (divided BID x 10 days)

• 2nd line: Augmentin (Amoxicillin + Clavulanate), Azithromycin, Cephalosporins IM

Pain/fever management:

• Acetaminophen

• Ibuprofen (only >6 months)

🚫 Avoid: Steroids, antihistamines, decongestants, antibiotic ear drops

🔹 Surgical Management

• Myringotomy with tube insertion (M&T): for recurrent OM or hearing loss

🔹 Nursing Considerations

• Relieve pain

• Facilitate drainage

• Prevent recurrence (reduce smoke/allergen exposure)

• Teach parents signs of ear infections

• Support with medication adherence and follow-up

🗣 CROUP SYNDROMES

🔹 General Characteristics

• Affect larynx, trachea, bronchi

• Hoarseness, barking cough, inspiratory stridor, varying respiratory distress

🔹 Types:

1. Epiglottitis (Supraglottitis) – medical emergency

2. Laryngitis

3. Laryngotracheobronchitis (LTB) – most common

4. Tracheitis

🛑 ACUTE EPIGLOTTITIS (Medical Emergency)

🔹 Clinical Manifestations

• Sudden sore throat

• Tripod position

• Drooling, dysphagia

• Inspiratory stridor, muffled voice

• No cough

• Retractions, restlessness

• Risk for complete airway obstruction

🔹 Management

• Do NOT examine throat unless emergency equipment is ready

• Airway management priority

• Antibiotics, IV fluids

• Prevention: Hib vaccine

🔹 Nursing Considerations

• Calm environment

• Do not agitate or lay child flat

• Monitor for increasing respiratory distress

🦭 ACUTE LARYNGOTRACHEOBRONCHITIS (LTB)

🔹 Most common croup syndrome (usually <5 years old)

🔹 Causative Agents

• RSV

• Parainfluenza virus

• Influenza A & B

• Mycoplasma pneumoniae

🔹 Clinical Manifestations

• Barking “seal-like” cough

• Inspiratory stridor

• Suprasternal retractions

• Hoarseness

• Hypoxia, respiratory distress

• May progress to respiratory acidosis, failure, or death

🔹 Therapeutic Management

• Airway management (priority)

• Maintain hydration (oral or IV)

• Cool mist/humidified air

• Nebulized treatments:

  • Epinephrine – reduces edema

  • Steroids – reduce inflammation

⚠ SIGNS OF INCREASING RESPIRATORY DISTRESS IN CHILDREN

🔹 Red Flags:

• Restlessness

• Tachycardia

• Tachypnea

• Retractions:

  • Substernal

  • Suprasternal

  • Intercostal

🌙 ACUTE SPASMODIC LARYNGITIS (Spasmodic Croup / Midnight Croup)

🔹 Overview

• Sudden, paroxysmal attacks of laryngeal obstruction

• Usually viral

• Common in ages 1–3

• Occurs suddenly at night, resolves by morning

🔹 Inflammation

• Mild or absent

• No fever or systemic illness

🔹 Clinical Features

• Barking cough (sudden onset)

• Hoarseness

• Noisy breathing (stridor)

• Child appears well between episodes

🔹 Therapeutic Management

• Cool mist/humidified air

• Calm environment (crying worsens symptoms)

• If severe: racemic epinephrine or steroids

• Often managed at home unless severe

🦠 BACTERIAL TRACHEITIS

🔹 Overview

• Bacterial infection of upper trachea

• Has features of both croup and epiglottitis

• May be a complication of LTB

🔹 Clinical Manifestations

• Similar to LTB, but more severe

• Thick purulent secretions

• High fever

• Respiratory distress

• Stridor unrelieved by typical croup treatments

🔹 Therapeutic Management

• Humidified oxygen

• Antipyretics (e.g., acetaminophen)

• IV antibiotics

• Possible intubation → airway obstruction risk

🫁 INFECTIONS OF THE LOWER AIRWAYS

• Affects bronchi and bronchioles

• Called the reactive portion of the airway (prone to spasm, edema)

• Airway narrowing common due to:

  • Incomplete cartilaginous support

  • Inflammation and mucous

🗣 BRONCHITIS (Tracheobronchitis)

🔹 Definition

• Inflammation of trachea and bronchi

🔹 Cause

• Mostly viral

🔹 Clinical Manifestations

• Persistent dry, hacking cough

  • Becomes productive in 2–3 days

• Tachypnea

• Low-grade fever

• Chest soreness from coughing

🔹 Treatment

• Supportive care

  • Fluids

  • Rest

  • Antipyretics

  • Humidified air

• No need for antibiotics unless bacterial superinfection

👶 BRONCHIOLITIS

🔹 Overview

• Starts as URI with clear nasal drainage

• Caused primarily by RSV (~80% of cases)

🔹 Symptoms

• Gradual onset of:

  • Fever

  • Wheezing

  • Tachypnea

  • Nasal flaring, retractions

  • Non-productive, paroxysmal cough

  • Poor feeding, lethargy

🦠 RESPIRATORY SYNCYTIAL VIRUS (RSV)

🔹 Transmission

• Direct contact with secretions

• Can live for hours on surfaces

🔹 Pathophysiology

• Virus → swelling of bronchiole walls

• ↑ Mucus production → airway obstruction

🔹 Treatment

• Supportive care only

  • Humidified O2

  • Fluids

  • Suctioning

  • Antipyretics

🔹 Prevention

• Palivizumab (Synagis): monthly IM injection during RSV season

  • Given to high-risk infants (e.g., premature, congenital heart disease)

🔹 Nursing Considerations

• Monitor for hypoxia

• Use contact precautions

• Educate parents on home care and hydration

• Elevate HOB, frequent suctioning

🦠 SARS-CoV-2 (COVID-19 in Children)

🔹 Cause

• Caused by Severe Acute Respiratory Syndrome Coronavirus 2

🔹 Clinical Manifestations

• Often mild in children

• May include:

  • Fever

  • Cough

  • Fatigue

  • GI symptoms (nausea, vomiting, diarrhea)

  • Loss of taste/smell

  • Rare: respiratory distress or pneumonia

🔹 Diagnosis

• PCR or rapid antigen test (nasal/throat swab)

🔹 Treatment

• Supportive in most cases

  • Fluids, antipyretics, rest

• Hospitalization for severe cases

🔹 Prevention

• COVID-19 vaccine (approved for children ≥6 months depending on brand)

🔹 Nursing Care

• Isolate infected child

• Educate family on symptom monitoring

• Emotional support, especially if child is isolated or hospitalized

🧠 MULTISYSTEM INFLAMMATORY SYNDROME IN CHILDREN (MIS-C)

🔹 Overview

• Post-COVID hyperinflammatory syndrome

• Typically occurs 2–6 weeks after COVID-19 infection

🔹 Clinical Manifestations

• Persistent high fever

• Abdominal pain, vomiting, diarrhea

• Rash, conjunctivitis

• Low blood pressure

• Cardiac involvement (myocarditis, coronary artery changes)

🔹 Prognosis

• Most children recover with prompt treatment

• May require ICU care

🔹 Management

• Hospitalization

• IV fluids, IVIG, steroids

• Monitor cardiac function

🫁 PNEUMONIA

🔹 Types of Pneumonia

🔹 Etiology of Pneumonias

• Infectious causes:

  • Bacterial (e.g., Streptococcus pneumoniae)

  • Viral (e.g., RSV, influenza)

  • Atypical: Mycoplasma pneumoniae (common in school-age children)

  • Fungal: Histoplasmosis, coccidioidomycosis

• Non-infectious causes:

  • Aspiration pneumonia (food, liquid, vomit)

  • Environmental toxins, chemicals

🔹 Etiology & Epidemiology (Pediatric Focus)

• Common in infants and young children

• Viral causes more frequent in <5 years

• Mycoplasma and bacterial causes increase with age

🔹 Clinical Manifestations

• General signs:

  • Fever

  • Malaise

  • Tachypnea

  • Cough (productive or dry)

• Other symptoms:

  • Chest pain

  • Abdominal pain (often referred pain)

  • Nausea, vomiting

  • Crackles, dullness to percussion, decreased breath sounds

🔹 Diagnostic Evaluation

• Chest X-ray

• CBC (elevated WBC for bacterial)

• Sputum culture (if productive cough)

• Pulse oximetry (oxygen saturation)

• Blood cultures if sepsis suspected

🔹 Therapeutic Management

• Antibiotics for bacterial pneumonia

• Antipyretics for fever

• Oxygen therapy if hypoxic

• IV fluids if dehydrated

• Chest physiotherapy (if indicated)

Prevention:

• Pneumococcal conjugate vaccine (PCV13)

Complications:

• Pleural effusion

• Empyema

• Atelectasis

• Sepsis

🔹 Nursing Care Management

• Respiratory support: monitor work of breathing, oxygen needs

• Fever control

• Hydration

• Positioning: semi-Fowler’s to ease breathing

• Parental education on meds and signs of worsening condition

• Encourage coughing and deep breathing if age-appropriate

🚫 FOREIGN BODY ASPIRATION

🔹 Risk Factors

• Common in infants/toddlers (especially ages 1–3)

• High risk in children with feeding/swallowing difficulties

🔹 Prevention

• Cut food into small pieces

• Avoid nuts, popcorn, grapes, hard candy

• Proper feeding position (upright)

• Supervise mealtimes

• Avoid risky items:

  • Oily nose drops

  • Talcum powder

  • Solvents

🔹 Clinical Signs

• Sudden coughing, gagging

• Wheezing, stridor, cyanosis

• Decreased breath sounds on affected side

🔹 Management

• Bronchoscopy to remove object

• Supportive care post-removal

• Education for caregivers on aspiration prevention

🔥 INHALATION INJURY (Smoke & Carbon Monoxide)

🔹 Severity Depends On:

• Type of substance (smoke, chemicals, CO)

• Environment (enclosed space = worse)

• Duration of exposure

🔹 Types of Injury

1. Local (upper airway)

   • Burns, edema, airway obstruction

   • Hoarseness, stridor, singed nasal hairs

2. Systemic (lower airway + CO poisoning)

   • Hypoxia, confusion, headache

   • Cherry-red skin (late sign of CO poisoning)

🔹 Therapeutic Management

• 100% humidified oxygen

• Intubation if airway edema present

• Monitor ABGs, carboxyhemoglobin levels

• Bronchodilators

• Fluid resuscitation if burns present

🔹 Nursing Considerations

• Airway is priority

• Frequent assessment for respiratory distress

• Monitor LOC and SpO₂

• Emotional support for patient and family

🚬 PASSIVE SMOKING (Secondhand Smoke)

🔹 Scope of the Problem

• Children exposed to tobacco smoke:

  • ↑ risk of asthma

  • ↑ frequency/severity of respiratory infections

  • ↑ risk of otitis media

  • Sudden Infant Death Syndrome (SIDS) in infants

🔹 Nursing Considerations

• Assess exposure during health visits

• Provide education on health risks

• Encourage smoke-free homes and cars

• Support for smoking cessation (refer to programs)

🌬 ASTHMA (Pediatric)

🔹 Definition

• Chronic inflammatory disorder of the airways

• Characterized by:

  • Airway inflammation

  • Bronchial hyper-responsiveness

  • Intermittent airflow obstruction

🔹 Key Features

• Wheezing

• Breathlessness

• Chest tightness

• Cough (especially at night or early morning)

• Obstruction is reversible—spontaneously or with treatment

🔹 Etiology & Risk Factors

• 60–80% allergen-related

• Some genetic predisposition

Risk Factors:

• Age

• Atopy (allergic tendency)

• Family history (especially maternal asthma)

• Maternal age <20 years

• Smoking (maternal/grandmaternal)

• African American ethnicity

• History of life-threatening attacks

• Limited healthcare access

• Psychosocial stressors

🔹 Pathophysiology

Airway narrowing caused by:

1. Inflammation and mucosal edema

2. Excessive mucus secretion

3. Bronchospasm from smooth muscle contraction
   ⬇ Leads to reduced airway diameter → limited airflow

🔹 Common Triggers

• Allergens (dust mites, pollen, pets, mold)

• Irritants (smoke, pollution)

• Cold air, weather changes

• Exercise

• Strong emotions (laughing, crying)

• Medications (NSAIDs, beta-blockers)

• Food additives, certain foods

• Respiratory infections

• Endocrine changes (e.g., menstruation)

🔹 Diagnosis

• Based on:

  • Clinical history

  • Physical exam

  • Family history

  • Pulmonary function tests

  • Peak expiratory flow rate (PEFR)

🧠 ASTHMA MANAGEMENT GOALS

• Prevent exacerbations

• Control symptoms

• Avoid known triggers/allergens

• Relieve bronchospasm quickly

• Monitor with peak flow meter

• Teach self-management (devices, medication use)

🛠 STEPWISE TREATMENT APPROACH

🔸 Medications

Rescue (Quick-Relief) Meds:

• β2-agonists: Albuterol, Metaproterenol, Terbutaline

• Xopenex: Less side effects, nebulized only

Controller (Long-Term) Meds:

• Inhaled corticosteroids: Pulmicort, Flovent

• Leukotriene modifiers: Singulair, Accolate, Zyflo

• Cromolyn sodium (inhaled NSAID)

Rarely Used:

• Theophylline (methylxanthine): monitor serum levels

• Anticholinergics: Atropine

🔸 Asthma Severity Classification (Age ≥5 Years)

🧴 TREATMENT BY STEP

Step 1:

Mild Intermittent

• Albuterol PRN (inhaler or nebulizer)

• No daily meds

Step 2:

Mild Persistent

• Low-dose corticosteroids

  • Pulmicort, Flovent (inhaler/nebulizer)

• Leukotriene modifiers

• Rescue meds PRN

Step 3–4:

Moderate Persistent

• Medium-dose corticosteroids

• Add long-acting β2-agonists (e.g., Serevent)

• Rescue meds PRN

Step 5–6:

Severe Persistent

• High-dose corticosteroids

• Long-acting β2-agonists

• Oral corticosteroids

• Rescue as needed

🧪 Monitoring & Action Plans

Peak Flow Meter

• Used daily to monitor airway status

• Has 3 color-coded zones:

  • Green = 80–100% personal best (good control)

  • Yellow = 50–79% (caution, use rescue meds)

  • Red = <50% (emergency)

Written Asthma Action Plan

• Created for home/school use

• Lists medications, triggers, when to seek help

🏃 ASTHMA & EXERCISE

• Exercise-induced asthma is common

• Use short-acting bronchodilator (2–3 hrs prior)

• Long-term bronchodilator (10–12 hrs prior) if needed

• Encourage activity with proper prep

🚨 STATUS ASTHMATICUS

Definition:

• Severe respiratory distress that doesn’t respond to initial treatments

Emergency Management:

• Epinephrine 0.01 mL/kg SQ (max: 0.3 mL)

• IV magnesium sulfate

• IV corticosteroids

• IV ketamine

• Treat underlying infections if present

❗ SIGNS OF SEVERE RESPIRATORY DISTRESS

• Child refuses to lie down

• Sudden agitation

• Agitated → quiet (bad sign)

• Diaphoresis

• Pallor/cyanosis

• Use of accessory muscles, nasal flaring, retractions

🧬 CYSTIC FIBROSIS (CF)

🔹 Definition & Overview

• Autosomal recessive genetic disorder

• Affects exocrine glands, leading to thick, sticky secretions

• Main systems involved:

  • Respiratory

  • Gastrointestinal (GI)

  • Reproductive

  • Skin

🔹 Pathophysiology (Simplified)

• Glands make thick, sticky mucus

• Mucus causes blockages in:

  • Airways → lung infections

  • Pancreas → prevents enzyme release → malabsorption

• Sweat glands lose salt → “salty-tasting skin”

• Leads to organ damage over time

🔹 Effects by System

🫁 Respiratory System

• Thick mucus traps bacteria → chronic infections

• ↓ O2/CO2 exchange → hypoxia, acidosis

• Complications:

  • Atelectasis

  • Barrel chest, clubbing, cyanosis

  • Respiratory failure, cor pulmonale

  • Pneumothorax, hemoptysis

🍽 GI System

• Blocked pancreatic ducts → enzyme deficiency

• Impaired digestion/absorption → malnutrition

• Steatorrhea (fatty stools)

• 4 F’s of stools: Frothy, Foul-smelling, Fatty, Float

• Risk for rectal prolapse, biliary cirrhosis

• May develop CF-related diabetes

💧 Sweat Glands

• ↑ salt in sweat

• Sweat chloride test = most reliable diagnostic tool

  • Na+ and Cl– are 2–5x higher than normal

🔁 Reproductive System

• Delayed puberty (females)

• Sterility (males – blocked vas deferens)

🔹 Clinical Presentation

• Recurrent respiratory infections (e.g., pneumonia, bronchitis)

• Wheezing, dry cough

• Poor weight gain despite good appetite

• Failure to thrive

• Salty-tasting skin

• Meconium ileus (newborns)

🔹 Infectious Agents in CF

• Pseudomonas aeruginosa

• Burkholderia cepacia (very serious in CF)

• Staph aureus

• H. influenzae

• E. coli, Klebsiella

🔹 Diagnosis

• Sweat chloride test (gold standard)

• Chest x-ray

• Pulmonary Function Tests (PFTs)

• Stool fat/enzyme analysis

• Barium enema (GI complications)

• Common triad for diagnosis:

  • Meconium ileus

  • Failure to thrive/malabsorption

  • Chronic respiratory infections

🎯 GOALS OF CF MANAGEMENT

• Prevent/minimize pulmonary complications

• Ensure adequate nutrition

• Support child & family in adapting to chronic illness

🔸 Respiratory Management

• Chest physiotherapy (CPT): daily, use of ThAIRapy vest

• Bronchodilators before CPT

• Pulmozyme (DNase) to thin mucus

• Antibiotics (inhaled, IV, or PO)

• Supplemental oxygen if needed

• Forced expirations

• Lung transplant for end-stage disease

🔸 GI Management

• Pancreatic enzyme replacement (before meals/snacks)

• High-protein, high-calorie diet (up to 150% RDA)

• Salt supplementation

• Fat-soluble vitamins (ADEK)

• Treat obstruction, prevent rectal prolapse

• May need insulin for CFRD (CF-related diabetes)

🔸 Prognosis

• Life expectancy improving with advanced care

• Factors that improve outcome:

  • Nutrition

  • Early infection treatment

  • Good pulmonary hygiene

  • New advances: gene therapy, lung transplant

🔸 Family Support

• Frequent treatments and hospitalizations

• Emotional strain; offer resources and counseling

• Genetic implications—future family planning

❤ PEDIATRIC CARDIOPULMONARY RESUSCITATION (CPR)

🔹 Key Differences from Adult CPR

• Pediatric arrests often due to respiratory failure or shock, not sudden cardiac collapse

• Early recognition and airway management is critical

🔹 Common Causes of Cardiac Arrest (By Setting)

Out-of-Hospital:

• Trauma

• SIDS

• Choking

• Severe asthma

• Drowning

• Poisoning

In-Hospital:

• Progressive respiratory failure

• Underlying chronic conditions

🔹 Chain of Survival (Pediatric Focus)

1. Prevention

2. Early recognition & call for help

3. High-quality CPR

4. Rapid PALS (Pediatric Advanced Life Support)

5. Post-resuscitation care

🔹 Standard of Care

• CPR:

  • 30:2 compressions to breaths (single rescuer)

  • 15:2 (two rescuers)

  • Use 2 fingers (infants), 1 hand (small child), or 2 hands (larger child) for compressions

• PALS:

  • Advanced airway, IV access, medications

  • Defibrillation if shockable rhythm