Newborn with Ohtahara Syndrome and absence of respiratory trigger: ethical issues in the NICU

Introduction: Ohtahara syndrome (OS) is a rare drug-resistant epileptic encephalopathy characterized by frequent tonic spasms or focal motor seizures associated with the specific electroencephalographic pattern of burst suppression. It begins in the first months of life and represents less than 4% of childhood epilepsy cases. The prognosis depends from the aetiology and is generally poor with death in most cases during early childhood. The management of severe encephalopathies associated with OS is controversial, especially regarding the strategies to ensure the well-being of young patients and their families, requiring a continuous modulation of medical interventions: from the intensity of care waiting for the diagnosis, to palliative care where a prognosis of incurability, up to the possible desist from intensive/invasive treatments when they are no longer proportionate to the current clinical situation. Objective: Through the case of M, a 38-week-old newborn, intubated and mechanically ventilated since birth due to the absence of spontaneous respiratory activity, for whom the clinical diagnosis of OS was reached at about 4 months of life, we intend to investigate the related ethical issues: a. the proportionality and ethical-clinical appropriateness of treatments in patients for whom a poor prognosis quoad vitam is reached; b. the decision-making process to be shared with the parents, those were unable to accept the irreversibility of their daughter's illness, despite the psychological support offered to them from the beginning. Discussion: In a situation of probably irreversible pathology, such as severe encephalopathy with cortico-subcortical dysfunction and electrical-clinical picture of OS, associated with the absence of respiratory trigger, in which elements capable of modifying the prognosis quoad vitam do not emerge, and it is difficult for the parents to elaborate awareness about the seriousness of the disease, it becomes difficult to identify the limits of treatments, which could take the form of unreasonable obstinacy and a painful prolongation of the dying process. In the case of M, during his hospitalization in the Neonatal Intensive Care Unit (NICU) at "A. Gemelli" Teaching Hospital IRCCS of Rome (Italy), neonatologists have constantly involved child neuropsychiatrists to search for an etiological cause and viable therapeutic options, and have requested the support in several phases of clinical ethics consultants to evaluate the proportionality and ethical appropriateness in an interdisciplinary way of the treatments in place (particularly the mechanical ventilator) and to be implemented. Conclusions: The limits of treatments and more generally questions related to the end of life are increasingly frequent ethical issues, even in the NICU, and must be constantly discussed, explored and shared within the medical team and with parents, even when communication is difficult. In the case of M, only after several interdisciplinary evaluations, a decision was reached which was also shared with the parents only after various interdisciplinary evaluations: a. to continue the palliative care already in place; b. to abstain from intensive/invasive interventions in the event of critical events; c. and to favor the welfare of the whole family, transferring M to a Pediatric Hospice, where the death occurred when the little girl was 11 months old.