Increasing numbers of children are dependent on a medical device to compensate for the loss of a bodily function.

Palliative care and technology dependence may exist in tandem, and often do. Non-invasive ventilation (NIV ) may play an important role in symptom relief and improving quality as well as duration of life particularly as it may improve fatigue, sleep and in consequence pain. That said the requirement of NIV is in itself a significant predictor of disease progression. The challenge of starting, continuing, changing (setting and time on/off) and stopping NIV is individual to the child, their condition and perceived benefit.

Symptoms potentially relieved by NIV

• Early morning headaches
• Feeling fatigue on waking
• Nocturnal arousals with daytime somnolence or hyperactivity
• Poor concentration

Potential burdens of: NIV

• Skin irritation (without tracheostomy)– use Duoderm® or Cavilon® on the affected areas during ventilation (the bridge of the nose is the most vulnerable area)
• Dry lips – use lipsalve and ensure adequate hydration (NB. Substances containing petroleum jelly should not be used on children receiving oxygen via a ventilator)
• Gastric distension – May improve with changing to a nasal mask or a change of sleeping position

End of life care

When a child or young person is identified as entering an end of life phase; with the focus of care moving away from prolonging life, patients and families may be given the option of discontinuing supportive therapy. As a child deteriorates or deals with an acute infection the number of hours that NIV is used in a day will often be increased. While NIV continues to relieve symptoms it may be appropriate for it to continue if its benefit outweighs the burden, but this balance is not an easy one to evaluate.

Patients and carers should understand that whilst NIV will support breathing it will not keep a child alive in the case of cardio-respiratory arrest.