Helping Children Come Home the Use of Non Invasive Ventilation in Life Limiting Conditions

Abstract

Introduction Non-invasive ventilation (NIV) therapy at home to support paediatric patients with complex life limiting conditions is increasingly recognized. Goals include improving patient comfort alleviating dyspnea, reducing hypoventilation and facilitating discharge home. Patients and families report positive experiences. However, practices vary worldwide and there remains ethical debate of NIV in this context. The Queensland Children's Hospital (QCH) is the only tertiary children's hospital within Queensland, Australia and has the largest paediatric home ventilation program in Australia. Three cases are discussed, describing the role of NIV in the palliative care setting, highlighting benefits for patients and demonstrating the multi-disciplinary collaboration involved. Description Case 1: 7-month-old, ex 30+3-week male infant, who transitioned from neonatal care to QCH. Lung biopsy confirmed alveolar growth disorder. Interdisciplinary collaboration with paediatric intensive care (PICU), sleep medicine, palliative care and family resulted in commencing NIV therapy for sleep. This respiratory support facilitated discharge home for 6 weeks prior to the child's death. Case 2: 11-year-old girl with Rett syndrome with restrictive lung disease, and scoliosis. Progressive type 2 respiratory failure was observed with multiple hospital admissions. Early multidisciplinary (MDT) discussion and collaboration with the family allowed NIV to be established during PICU admission with transition to home care. Subsequently, there was 9 months without hospitalization. NIV was continued at the end of life, alleviating respiratory distress and facilitating the parents' preference for death at home. Case 3:16-year-old boy with Sotos syndrome, seizures, pulmonary aspiration and hospitalization. CPAP was trialed for severe obstructive sleep apnea and hypoventilation. This provided comfort, and improved hypoventilation. Collaboration with regional centres developed a clear management plan, avoiding hospital admission and supporting end of life care at home. Families expressed gratefulness to have NIV at home, reporting reduced hospitalization and improved quality of life (QoL) for their child. Discussion These cases demonstrate how NIV therapy in children with life limiting conditions is feasible and beneficial for children and families at the end of life. Positive outcomes included reduced hospitalization, improved respiratory symptoms, QoL, and the ability to support home death. Early MDT approach and setting clear goals with families in this context, are essential to ensure success. Liaison with regional centres empower them to retain involvement and support greater access to end-of-life care at home. Further prospective studies of NIV use in this context are required to guide the role of NIV in a palliative care setting and understand the experiences of families.