Ongoing monitoring and optimisation of NIV

Increasing dependence on NIV

Whilst non-invasive ventilation (NIV) can help improve survival and improve patients’ quality of life, it will not prevent the disease from causing progressive respiratory muscle weakness.

As the patient’s respiratory muscles deteriorate, the patient may use NIV for longer amounts of time and may eventually need to use it continuously. It is important that patients are made aware of this when healthcare professionals discuss NIV with them. They should be reassured that there is no evidence that NIV weakens the respiratory muscles.

In the following video, Julie talks about the thoughts and emotions she had when she started using NIV for 24 hours per day.

With increased dependency, patients begin using NIV both during the nighttime and during the day. Initially for naps or after exertion but later more frequently. The mask and machine set ups may need to vary to allow for daytime activities. Particular care should be taken to ensure patients can communicate, eat and drink (if able) and that pressure sores are avoided and treated promptly.

In the following video, Julie highlights the importance of using different machines, settings and masks for daytime and nighttime use. She also highlights the importance of using different masks at different times to prevent pressure sores.

As the patients use NIV more, the multidisciplinary team should discuss the likelihood of further deterioration and how this might be managed. Discussions should occur frequently but might include when the patient commences NIV, when they start to use it during the day or when they develop daytime breathlessness or have a respiratory infection.

Patients and carers may start to recognise that removing NIV, even for a short period, may cause them to develop distressing symptoms. Patients and carers need to feel confident that they can deal with routine and emergency situations.

Patients need sufficient equipment including back-up equipment in the event of equipment or electricity failure and to allow for travel outside the home and cleaning of the devices. They also should have an action plan should they encounter problems which includes out-of-hours support which should be easily accessible.

In the following video, Matt Cox, an Extended Scope Respiratory Physiotherapist describes how he recognises increased dependence and the importance of providing more equipment.

Patient wearing a mask while asleep

It is also important to be aware that the patient’s carers and family members may feel distressed about their loved one’s decline. In the following video Dr Emily Mayberry, a Clinical Psychologist, describes the importance of recognising that carers may experience distress and being able to support them as well as the patient.

Good practice points