NIV dependent child....when to refer paediatric respiratory physician

There is an exponential increase in the use of non-invasive ventilation (NIV) and invasive-ventilation in children globally. The lack of accessibility to Paediatric Respiratory Physicians, high cost to provide this treatment and huge burden of care to the families, are issues in Malaysia that hinder the early and efficient provision of this service.

Any child who requires respiratory support after about 2-4 weeks of being in a stable clinical state, without any acute diseases that need treatment should be considered for home NIV.

LT-NIV is considered for conditions that affect respiratory-muscle performance (alterations in central respiratory drive or neuromuscular function) and/or impose an excessive respiratory load (airway obstruction, lung disease, or chest-wall anomalies).

The following is a list of respiratory diseases and recommended respiratory support

1. Lung parenchymal disease (e.g. bronchopulmonary dysplasia, chILD, severe bronchiectasis: oxygen ± NIV
2. Neuromuscular disease( e.g. Duchenne’s muscular dystrophy, Spinal Muscular Atrophy): NIV
3. Alveolar hypoventilation ( e.g. severe kyphoscoliosis, spondylothoracic dysplasias , post-severe pneumonia ): NIV
4. Upper airway obstruction (e.g. Down syndrome, Craniosynostosis, Pierre Robin): CPAP
5. Lower airway obstruction (e.g. tracheobronchomalacia, post-infectious bronchiolitis obliterans, vascular ring ) : CPAP/NIV  ±oxygen
6. Central hypoventilation (e.g. Congenital Central Hypoventilation Syndrome): tracheostomy+ ventilator

Relative contraindications for home NIV include the inability of the local medical infrastructure to support home NIV and poor motivation or inability of the patient/caregivers to cooperate or understand recommendations. Anatomic abnormalities that interfere with interface fitting, inability to protect the lower airways due to excessive airway secretions and/or severely impaired swallowing, or failure of LTNIV to support respiration can lead to considering invasive ventilation via tracheostomy

Conclusion:  General paediatricians need to learn how to identify patients who will benefit from home NIV as the hospital is not a suitable place for a child to grow in.