Long-Term Ventilation in Children with Medical Complexity: A Challenging Issue

Abstract

Children with medical complexity (CMCs) represent a subgroup of children who may have congenital or acquired multisystemic disease. CMCs are frequently predisposed to respiratory problems and often require long-term mechanical ventilation (LTMV). The indications for LTMV in CMCs are increasing, but gathering evidence about indications, titration, and monitoring is currently the most difficult challenge due to the absence of validated data. The aim of this review was to examine the clinical indications and ethical considerations for the initiation, continuation, or withdrawal of LTMV among CMCs. The decision to initiate long-term ventilation should always be based on clinical and ethical considerations and should be shared with the parents.

Respiratory considerations in patients with neuromuscular disorders

“…NIPPV should be initiated when spirometry or respiratory pressures indicate respiratory weakness or when there are symptoms of nocturnal hypoventilation (Table 4). There is a lack of consensus on a specific threshold for initiation of NIPPV based upon respiratory function testing, although there is evidence of improved outcomes with early initiation 114–119 . Use of NIPPV is associated with slowing the rate of decline of respiratory function and improved quality of life and survival 18,19,113,114,118,120,121 .…”

“…There is a lack of consensus on a specific threshold for initiation of NIPPV based upon respiratory function testing, although there is evidence of improved outcomes with early initiation. [114][115][116][117][118][119] Use of NIPPV is associated with slowing the rate of decline of respiratory function and improved quality of life and survival. 18,19,113,114,118,120,121 NIPPV can be used around the clock for patients with advanced respiratory impairment, even with unmeasurable VCs provided bulbar function is intact.…”

“…[114][115][116][117][118][119] Use of NIPPV is associated with slowing the rate of decline of respiratory function and improved quality of life and survival. 18,19,113,114,118,120,121 NIPPV can be used around the clock for patients with advanced respiratory impairment, even with unmeasurable VCs provided bulbar function is intact. 90 In patients with ALS without bulbar involvement, NIPPV increases survival on average of 205 days.…”

Traqueostomía en Prematuros Con Displasia Broncopulmonar Grave

“…Las indicaciones neonatales más frecuentes son: malformación de la vía aérea alta, enfermedades neuromusculares graves, DBP, y condiciones médicas complejas en las que participan el compromiso neurológico, neuromuscular y respiratorio conjuntamente. Muchos de estos RN son ingresados en programas de asistencia respiratoria crónica, que permiten en una proporción significativa de ellos el alta a domicilio con apoyo multidisciplinario (19)(20).…”

Lung Function in Childhood: Designing the Future Trajectories

“…Children with medical complexity represent a growing population who require significant healthcare resources and multidisciplinary care. Long-term ventilation indications and management guidelines are crucial in following patients and their families [ 14 ]. Our medical era offers new therapies for various respiratory diseases, including the so-called “orphan disease”.…”