Reproductive health considerations for IDH-mutant glioma patients considering IDH inhibitor therapy: A retrospective cohort study.

People with myotonic dystrophy type 1 (DM1) often develop sleep-disordered breathing requiring non-invasive ventilation (NIV). Between 20 and 54% of people with DM1 will not use or stop NIV for reasons that are not fully understood. This is important because 45% will die of respiratory failure, and non-adherence to NIV is associated with increased mortality. A retrospective cohort study of people with DM1 by Boussaïd et al. showed the following characteristics are associated with adherence to NIV: symptomatic sleep-disordered breathing, a history of acute respiratory failure, professional education, absence of excessive mask leak, and well-functioning ventilators. Unfortunately, this does not give the clinician much guidance on how to improve adherence for an individual patient, except to ensure the ventilator and mask are working appropriately. Subsequently, Vosse . showed that cognitive impairment, depressive symptoms, and CTG repeat length do not correlate with NIV adherence. As part of a quality assurance initiative, we sought to determine if psychosocial factors might contribute to non-adherence to NIV in our patients. In this case, additional supports could be offered to address social isolation and mental health to improve adherence and reduce mortality. We examined the charts of all patients with DM1 currently enrolled in our multidisciplinary clinic for adults with hereditary neuromuscular disorders in northern Alberta. We made use of the provincial electronic medical record, Connect Care, which is used by all hospitals and hospital-affiliated clinics in Alberta. We identified 97 patients with DM1, of which 41 were prescribed NIV and 56 were not. Of those prescribed NIV, 3 were prescribed continuous positive airway pressure (CPAP), and 32 bilevel positive airway pressure (BPAP), and both modalities were considered NIV for the purposes of this chart review. Two individuals had only recently started BPAP (within the last 2 months) and there was insufficient information to determine adherence. Of the remaining 39 people prescribed NIV, the mean age was 45 years at the time of last Pulmonary Medicine assessment (standard deviation 11 years), and 55% were female.