5.3 Chronic Respiratory Failure & Pulmonary Conditions

Chronic Respiratory Failure & Pulmonary Conditions

BPD / Chronic Lung Disease

Bronchopulmonary dysplasia (BPD), often called chronic lung disease of prematurity (CLD), is a sequela of preterm birth, surfactant deficiency, oxygen toxicity, volutrauma/barotrauma, infection, and disrupted alveolarization. Older children with BPD who present to the PICU may still have baseline oxygen need, wheeze, pulmonary hypertension, poor lung compliance, and heightened vulnerability to viral illness. Their “normal” SpO2 target may be lower than that of a healthy child—confirm goals with the chronic care plan rather than chasing adult norms.

Acute viral infections are the usual tip into failure. Nursing care emphasizes lung-protective support, avoidance of large tidal volumes, careful fluid balance (these lungs leak easily), nutrition, and early recognition of cor pulmonale signs: hepatomegaly, rising oxygen need, and right-heart strain on echo when obtained. Bronchodilators and steroids are used selectively based on phenotype and team plan—not automatically for every desaturation. Infection prevention and vaccination status matter enormously for this population.

Neuromuscular Chronic Ventilator Dependence

Children with spinal muscular atrophy, Duchenne muscular dystrophy, congenital myopathies, and high spinal cord injury develop progressive or static weakness of inspiratory muscles and cough. Chronic hypercapnic failure appears first at night; many use nocturnal NIV at home, then progress to more continuous support or tracheostomy ventilation. Ineffective cough leads to atelectasis and recurrent pneumonia—secretion clearance (cough assist, vest, manual techniques as ordered) is as important as the ventilator mode.

In acute illness, a neuromuscular child may look “comfortable” while PaCO2 climbs because they cannot generate retractions. Rely on EtCO2/ABG, sleepiness, and home caregiver reports. Do not attribute hypoventilation to “anxiety.” When intubated, plan extubation carefully with secretion load optimized and NIV bridge ready if that is the child’s chronic strategy.

Tracheostomy Care

A tracheostomy secures a long-term airway for upper-airway obstruction, chronic ventilation, or secretion management. Daily nursing competencies include confirming tie security (usually snug enough for one finger), inspecting the stoma for granulation or infection, providing humidity to prevent thick mucus plugs, and suctioning to the appropriate depth—not routinely deep beyond the tube unless indicated. Keep emergency equipment at the bedside: same-size and smaller trach tubes, obturator, suction, and bag-mask with tracheal and facial interfaces as appropriate.

Tube change is a two-person procedure when possible; know whether the tract is mature. Accidental decannulation in a fresh trach is an emergency—cover the stoma and ventilate from above if the child has a patent upper airway, or replace the tube per protocol. Speaking valves and caps are used only when assessed safe (cuff deflated when required, adequate leak, and team clearance). Decannulation is a planned process after tolerance of capping trials and airway evaluation—not an impulsive bedside decision.

Home-Ventilator Escalation in the PICU

Children arrive from home on portable ventilators with individualized modes (often pressure control or pressure support with backup rates). First actions in deterioration: assess airway patency (trach tube position, plug, cuff status), confirm circuit integrity, and compare device settings to the written home prescription. Do not “start from scratch” with adult ICU defaults that under- or over-ventilate the child. Then identify the acute driver—viral infection, aspiration, pneumothorax, equipment failure, or progression of neuromuscular disease.

Escalation may mean increasing rate or pressure within ordered limits, converting to a hospital ventilator for advanced monitoring, adding oxygen, starting NIV interfaces for children without trachs, or intubating when the home artificial airway strategy fails. Families are clinical partners: ask baseline SpO2, usual peak pressures, alarm patterns, and what looked different today. Document home settings clearly on admission so nights and handoffs remain safe.

Discharge planning starts on admission for technology-dependent children: confirm caregiver competency for suctioning, trach changes, and ventilator alarms; arrange durable medical equipment checks; and document the exact home prescription that will travel with the child. During the PICU stay, protect sleep, nutrition, and skin around the stoma and mask interfaces—these chronic details prevent the next preventable readmission. If pulmonary hypertension accompanies BPD or chronic hypoxemia, avoid abrupt hypoxia, acidosis, and high mean airway pressures that spike pulmonary vascular resistance, and coordinate vasodilator therapies already part of the child’s regimen.

Chronic respiratory failure care is longitudinal. The CCRN Pediatric exam tests whether you can protect fragile BPD lungs, support neuromuscular ventilation and cough, manage trach emergencies, and stabilize home-vent children without erasing the settings that keep them alive every day.