Initiation of NIV

Dealing with emergencies

The patient should have an individual care plan which details what to do in the event of an acute respiratory deterioration. This should also be in combination with other advanced care planning decisions.

Provision for support and equipment

In-hours telephone support should be available for all patients, as should out-of-hours support. This should include access to a member of staff who is appropriately trained for the role. A plan for providing replacement equipment for emergencies should also be formulated. This plan, including telephone numbers, should be provided in writing to patients and their carers.

Patients who are becoming dependent on NIV should have adequate equipment to deal with all eventualities and enable them to leave the house. This should include a NIV battery, spare equipment and training to use it. They should be advised to contact their energy provider and register for priority services in the event of a power cut. These discussions may trigger discussions about advanced care planning, NIV withdrawal and symptomatic treatments, discussed in the next section.

As outlined in the following clip, it is also important that healthcare professionals prepare patients for infection and emergencies.

Dealing with acute respiratory deterioration

Patients should be advised to have access to the respiratory equipment and care plan at all times. This includes when leaving the house for any patient using NIV who may be at risk of respiratory deterioration.

In an emergency situation patients should be advised should to sat upright and encouraged to use their NIV and any other treatments they have been prescribed such as suction and cough assist.

Pre-emptive medications

Patients should be considered for pre-emptive medications to relieve symptoms which can be given by carers or healthcare professionals. Local palliative care services and the patient’s GP should be involved in drawing up a plan. The MND Association have produced a ‘Just in Case Kit’ to support this.

Use of oxgyen

Respiratory failure in motor neurone disease (MIND) causes a chronic increase in blood carbon dioxide and later, hypoxia. The body adapts to these changes over time meaning they can tolerate these abnormalities. However a sudden deterioration in breathing (such as during a pneumonia or mucus plugging) can cause worsening hypoxia and respiratory distress. Usually in an emergency hypoxia would be treated with oxygen to reach target saturations of 94-98%.

However, in people with chronic respiratory failure this can overcorrect their hypoxia, reducing their body’s drive to breathe causing their breathing to become slower, more shallow or stop completely. They may become drowsy or die.

Patients can order an MND alert wristband or card from the MNDA or save the alert card/image (below) onto their phone to alert healthcare professionals to the risks of oxygen and to provide additional guidance about managing acute deteriorations.

MND Alert

I have motor neurone disease and need specialist care if admitted to hospital.

Caution: I may be at risk with oxygen.

Further information at:

“When my son was using NIV 24 hours a day he was so anxious about something going wrong. His head straps would get sweaty and water would build up in his mask and I would have to clean it all the time. I'd have to take it off quickly and put a new one on straight away so having spare equipment wasn't just a nice thing to have, it was really really important.”

J, ex-carer

Good practice points