Non-invasive Ventilation for Children With Chronic Lung Disease

Abstract

Advances in medical care and supportive care options have contributed to the survival of children with complex disorders, including children with chronic lung disease. By delivering a positive pressure or a volume during the patient's inspiration, NIV is able to reverse nocturnal alveolar hypoventilation in patients who experience hypoventilation during sleep, such as patients with chronic lung disease. Bronchopulmonary dysplasia (BPD) is a common complication of prematurity, and despite significant advances in neonatal care over recent decades its incidence has not diminished. Most affected infants have mild disease and require a short period of oxygen supplementation or respiratory support. However, severely affected infants can become dependent on positive pressure support for a prolonged period. In case of established severe BPD, respiratory support with non-invasive or invasive positive pressure ventilation is required. Patients with cystic fibrosis (CF) and advanced lung disease develop hypoxaemia and hypercapnia during sleep and hypoventilation during sleep usually predates daytime hypercapnia. Hypoxaemia and hypercapnia indicates poor prognosis and prompts referral for lung transplantation. The prevention of respiratory failure during sleep in CF may prolong survival. Long-term oxygen therapy has not been shown to improve survival in people with CF. A Cochrane review on the use NIV in CF concluded that NIV in combination with oxygen therapy improves gas exchange during sleep to a greater extent than oxygen therapy alone in people with moderate to severe CF lung disease. Uncontrolled, non-randomized studies suggest survival benefit with NIV in addition to being an effective bridge to transplantation. Complications of NIV relate mainly to prolonged use of a face or nasal mask which can lead to skin trauma, and neurodevelopmental delay by acting as a physical barrier to social interaction. Another associated risk is pulmonary aspiration caused by vomiting whilst wearing a face mask. Adherence to NIV is one of the major barriers to treatment in children. This article will review the current evidence for indications, adverse effects and long term follow up including adherence to NIV in children with chronic lung disease.

Recent advances in the use of high flow nasal oxygen therapies

“…Hypercapnia is relatively rare in children and is typically associated with advanced lung diseases, such as cystic fibrosis or neuromuscular diseases ( 67 – 69 ). Affected children may require NIV to offset the effects of nocturnal hypercapnia to reduce the risk of alveolar hypoventilation during sleep ( 67 , 69 ).…”

“…Hypercapnia is relatively rare in children and is typically associated with advanced lung diseases, such as cystic fibrosis or neuromuscular diseases ( 67 – 69 ). Affected children may require NIV to offset the effects of nocturnal hypercapnia to reduce the risk of alveolar hypoventilation during sleep ( 67 , 69 ). Unfortunately, complications from NIPPV-induced pressure can lead to gastric distention, gastroesophageal reflux, pulmonary aspiration, and other adverse effects ( 67 , 70 ).…”

“…Affected children may require NIV to offset the effects of nocturnal hypercapnia to reduce the risk of alveolar hypoventilation during sleep ( 67 , 69 ). Unfortunately, complications from NIPPV-induced pressure can lead to gastric distention, gastroesophageal reflux, pulmonary aspiration, and other adverse effects ( 67 , 70 ). Few studies have analyzed the effectiveness of HFNO compared to other methods in children with hypercapnia.…”

Bilevel Positive Airway Pressure Versus Nasal Continuous Positive Airway Pressure for Prevention of Extubation Failure in Infants After Cardiac Surgery: A Randomized Controlled Trial

“…NIV has previously been widely used in the eld of premature babies and newborns. Studies have con rmed that BiPAP and NCPAP can be used to treat neonatal respiratory failure, respiratory distress syndrome (RDS) and reduce the incidence of bronchopulmonary dysplasia [2,[5][6][7][8]. However, few studies have focused on the application of NIV in infants with CHD after extubation.…”

“…Infants with congenital heart disease (CHD) need respiratory support after surgery because of their young age and poor independent mobility. Noninvasive ventilation (NIV) has been proven to be useful for respiratory support in pediatric patients and can effectively reduce the extubation failure rate in high-risk infants [1][2][3][4][5]. In recent years, the technique of NIV has become increasingly mature, especially the application of bilevel positive airway pressure (BiPAP) and nasal continuous positive airway pressure (NCPAP), which have signi cantly reduced the application of invasive ventilation and shortened the time of mechanical ventilation.…”