Spinal Muscular Atrophy (SMA) with Acute Respiratory Failure Clinical Pathway — PICU

Considerations for Tracheostomy in Patients with SMA

When to Consider a Tracheostomy

Indications are not absolute, but are general thoughts as to when to consider tracheostomy discussion. This discussion should only be undertaken in collaboration with patient’s primary pulmonologist and PACT team if they are involved.

Indication	Specific Considerations
Inability to provide adequate mechanical ventilation/airway clearance non-invasively	Profound bulbar weakness Tracheomalacia Craniofacial abnormalities that precludes adequate use of a noninvasive interface for ventilation or oro-nasal mask for MI-E (CoughAssist) use
Inability to extubate patient after acute illness after multiple attempts despite:	Patient near baseline status before extubation attempt No need for supplemental oxygen at time of extubation attempt Atelectasis reversed if present Not requiring excessive suctioning of tracheal secretions No acute intercurrent illness Cardiovascular status stable Adequate bulbar function
Patient family amenable to tracheostomy	Recurrent illness Patient does not want continuous NIV or use of continuous NIV would pose significant developmental impediment Family expresses willingness to have child undergo trach Suitable discharge plan (home, residential facility) can be expected

Considerations for Tracheostomy

Consider tight to shaft (TTS) cuffed tube to provide for adequate ventilation during sleep but leak to support phonation when awake
Speaking valve should be introduced whenever possible

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