Choosing against invasive long-term mechanical ventilation in congenital central hypoventilation syndrome. Is pediatric tracheostomy for long- term mechanical ventilation ever obligatory?

Elizabeth Lanphier – Cincinnati Children's Hospital Medical Center

Abstract: Congenital central hypoventilation syndrome (CCHS) is a rare genetic disorder affecting the autonomic nervous system and control of breathing. In its most severe form, it is characterized by chronic respiratory failure requiring tracheostomy for long-term mechanical ventilation. This presentation will discuss a case in which the parents of an infant diagnosed with CCHS initially refused tracheostomy resulting in multidisciplinary team disagreement about range of clinically and ethically acceptable options to present to family. Deliberation was complicated by limited published literature on ethical considerations of the management of CCHS, especially as the only available manuscript argues that provision of non-invasive mechanical ventilation is in the child’s best interest and should be pursued over parental objection. The expectation that the child would have minimal to no neurocognitive impairment featured prominently in conversations among the subspeciality teams involved as providers tried to weigh concerns for the child’s best interest against the risks of treatment, the expected burden of care placed on family, and known limitations in the out-of-hospital support infrastructure. From a disability ethics standpoint, it has been argued that the presence of neurocognitive disability should not preclude the option of tracheostomy for long-term mechanical ventilation, but does the absence make it ethically impermissible to discuss comfort care within a shared decision-making framework? The unique pathophysiology of CCHS presents an opportunity to evaluate the impact of ableism on provider counseling around tracheostomy for long-term mechanical ventilation, a treatment decision which typically falls within the zone of parental discretion when recommended for other indications.

Keywords: Pediatric decision making, Ableism, Long-term mechanical ventilation