Respiratory support in advanced pulmonary disease

Tan Yee Yen
Tengku Ampuan Rahimah Hospital, Klang, Selangor, Malaysia

Children and adolescents at the advance stage of any chronic lung diseases inevitably progress into respiratory failure with hypoxia and hypercarbia. Oxygen therapy is frequently used to correct hypoxaemia and for comfort. Evidence is emerging on the use of non-invasive ventilation (NIV) in this group of patients. In cystic fibrosis, NIV has been shown to be associated in the stabilization in lung function and a reduction in exacerbation rate. NIV is often employed as a bridging tool to lung transplant. The evidence of benefit of NIV in other lung parenchyma disease affecting the paediatric and adolescent population is scarce. A study from a tertiary paediatric hospital of 33 patients with chronic lung diseases requiring long term ventilation showed improvement in oxygen saturation, lung function and exacerbation rate following the initiation of NIV. However, mortality rate remained high, with 1/3 of the patients dying within the first 5 years of the initiation of NIV, reflecting the severity of the underlying lung diseases. Patient and parental acceptance, practicality and the costs of multiple respiratory support devices for domiciliary use are the issues that need to be considered before the decision to initiate any additional support for this group of patients who is already approaching the end of life.