Acute neuromuscular respiratory failure

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Target exams

Red flags

• A normal oxygen saturation does not exclude neuromuscular respiratory failure; carbon dioxide climbs while oxygenation is preserved, so the child can arrest with a reassuring saturation
• Measure bedside forced vital capacity every two to six hours; a value under twenty millilitres per kilogram, a fall of over thirty percent, or maximum inspiratory pressure weaker than minus thirty centimetres of water predict the need for ventilation
• Bulbar weakness with a weak cough, pooled secretions, and a soft voice predicts aspiration; this is an indication for intubation, not non-invasive ventilation
• Never give suxamethonium to a child with a denervating or chronic neuromuscular disease; it can trigger life-threatening hyperkalaemia and cardiac arrest; use rocuronium one milligram per kilogram
• Non-invasive ventilation supports pump failure only when bulbar function is intact; it does not protect the airway and will fail when bulbar weakness develops
• Do not wait for a rising carbon dioxide on the blood gas before escalating; by the time the gas is abnormal the child is already decompensating

Life stages

Care settings

Clinical exam formats

Board mappings

• Recognises that acute neuromuscular respiratory failure is a failure of the respiratory pump rather than the lungs, presents with hypercapnia before hypoxaemia, and is most often due to Guillain-Barre syndrome in a previously well child
• Monitors bedside forced vital capacity every two to six hours and states that a value under twenty millilitres per kilogram, a fall of over thirty percent, or bulbar weakness with a weak cough are the triggers for intensive care and airway protection
• States that non-invasive ventilation can support a child with intact bulbar function but that bulbar weakness with aspiration is an indication for intubation, and that suxamethonium must be avoided because of dangerous hyperkalaemia in denervated muscle
• Applies the Lawn twenty-thirty-forty monitoring framework using forced vital capacity, maximum inspiratory pressure, and maximum expiratory pressure, and the Durand and Sharshar predictors of ventilation, and justifies when to intubate before blood-gas decompensation
• Distinguishes pump failure causing hypoventilation and hypercapnia from bulbar failure causing aspiration and hypoxaemia, and runs the differential across the motor unit from anterior horn cell to muscle including spinal muscular atrophy, Guillain-Barre syndrome, myasthenia gravis, botulism, and muscular dystrophy
• Manages cough augmentation with mechanical insufflation-exsufflation, times non-invasive ventilation versus intubation and tracheostomy, and coordinates retrieval, intensive care, disease-modifying therapy, and the long-term ventilation decision
• The forced vital capacity threshold under twenty millilitres per kilogram, the over thirty percent decline rule, and the maximum inspiratory and expiratory pressure thresholds for intensive care escalation
• The two mechanisms of failure, hypercapnic pump failure and hypoxaemic bulbar aspiration, and the differential of acute flaccid weakness across the motor unit
• The avoidance of suxamethonium and use of rocuronium for rapid sequence intubation, the role of non-invasive ventilation when bulbar function is intact, and cough augmentation with mechanical insufflation-exsufflation
• Runs the structured assessment, respiratory monitoring, and airway decision for a child with progressive neuromuscular weakness while excluding the mimics and arranging tertiary retrieval
• Decides on and times non-invasive ventilation versus intubation using serial forced vital capacity and bulbar assessment, and coordinates intensive care, disease-specific therapy, and rehabilitation
• Counsels the family on the cause, the projected course from days to months depending on the diagnosis, the long-term ventilation and quality-of-life decisions in chronic disease, and the safety-net
• Level 1: Recognition that a child with progressive weakness, a weak cough, and shallow breathing has neuromuscular respiratory failure and that a normal oxygen saturation does not exclude it
• Level 2: Application of bedside spirometry with forced vital capacity thresholds, the distinction between pump and bulbar failure, and the timing of intensive care escalation
• Level 3: Coordination of non-invasive ventilation, intubation avoiding suxamethonium, cough augmentation, disease-specific therapy, and the long-term ventilation decision in chronic neuromuscular disease
• Acute neuromuscular respiratory failure as a failure of the respiratory pump causing hypercapnia before hypoxaemia, with Guillain-Barre syndrome the commonest acute cause in children
• The forced vital capacity monitoring thresholds under twenty millilitres per kilogram and the over thirty percent decline rule, and the indication for intubation in bulbar weakness
• The avoidance of suxamethonium in denervated muscle, the role of non-invasive ventilation when bulbar function is intact, and the differential across the motor unit including botulism and myasthenia gravis
• Structured assessment of a child with progressive neuromuscular weakness including serial bedside spirometry, bulbar and cough assessment, and arterial blood-gas interpretation
• Recognition of the intensive care and intubation triggers and the timing of disease-specific therapy, and the exclusion of acute flaccid myelitis, transverse myelitis, and cord compression
• Communication of the diagnosis, the projected recovery, and the long-term ventilation decision to the family with honesty about uncertainty
• Acute neuromuscular respiratory failure as respiratory pump failure with hypercapnia preceding hypoxaemia, and the common causes in United States children including Guillain-Barre syndrome and acute flaccid myelitis
• Forced vital capacity monitoring with the twenty millilitres per kilogram threshold and the role of non-invasive ventilation and intubation, and the avoidance of suxamethonium
• The differential across the motor unit, the role of cough augmentation, and the disease-specific therapy for spinal muscular atrophy, myasthenia gravis, and infant botulism
• Recognition of acute neuromuscular respiratory failure as pump failure with hypercapnia before hypoxaemia and the serial forced vital capacity monitoring that drives the airway decision
• Stepwise management with bedside spirometry, non-invasive ventilation versus intubation, rocuronium for rapid sequence induction, and cough augmentation
• Multidisciplinary care of the underlying cause, intensive weaning, rehabilitation, and the long-term ventilation decision in chronic disease
• Canadian approach to acute neuromuscular respiratory failure and acute flaccid paralysis surveillance, with serial forced vital capacity monitoring driving the intensive care and airway decision
• Non-invasive ventilation when bulbar function is intact, intubation with rocuronium avoiding suxamethonium for bulbar weakness, and cough augmentation with mechanical insufflation-exsufflation
• Coordination of disease-specific therapy, intensive care, rehabilitation, and the family-centred long-term ventilation decision