Jusqu'où soigner les pathologies pulmonaires dont souffrent les enfants atteints de maladies chroniques à risque vital ? Les enjeux médicaux et éthiques autour du « temps qui reste à vivre »

Children with life-limiting condition require specific management, including palliative care. These patients develop major respiratory morbidity and eventually die from bronchopulmonary diseases. When the primary diagnosis is not airway or lung disease, they progressively develop a chronic respiratory condition, characterised by its own course, including chronic infectious complications and chronic respiratory insufficiency, eventually leading to acute respiratory failure. New respiratory technologies are now available: dyspnea scales, cough-assist devices, clearance of airway secretions, non invasive ventilation during night sleep, invasive ventilation with tracheotomy and use of microphone… The process of integrating these diagnostics and treatment tools into the management of the chronic respiratory condition of these patients raises important ethical issues, with regard to uncertainty about the child's prognosis and remaining life time, improved life expectancy due to treatment of chronic respiratory insufficiency, the value of months or years in the child's lifetime. We are aware that children with life limiting conditions benefit from respiratory treatment mainly during acute exacerbations, but do not receive preventive treatments aimed at preventing acute exacerbations, slowing the process of respiratory insufficiency. Access to palliative care, as soon as possible, should include specific management of the chronic respiratory condition, with a strategic, thoughtful, multidisciplinary approach integrating the child and his/her family.