Before starting NIV
When should the patient be referred to the respiratory service?
Patients should be referred to the respiratory service at diagnosis or as soon as possible thereafter to allow time to prepare the patient and reduce delays in identifying and treating respiratory insufficiency.
How often should people living with MND be monitored to check their respiratory function?
Patients should be monitored every three months as a minimum. However, it is important to note that the timing of monitoring will vary based on the patient’s symptoms, speed of progression and their preferences.
How should we assess respiratory function?
Respiratory function should be determined by assessing the full clinical picture. This includes checking for signs and symptoms of potential respiratory insufficiency, carrying out respiratory function tests and the patient’s speed of deterioration.
Respiratory function tests should include respiratory muscle strength testing and assessment of blood gases. Learn more about the different tests:
What are the signs and symptoms to look out for that indicate respiratory insufficiency?
Disturbed or non-refreshing sleep
Difficulty getting comfortable in bed
Poor concentration or memory
Weak cough or sniff
What are the signs and symptoms that suggest the need to commence NIV immediately or to palliate?
Increases respiratory rate at rest
Abdominal paradox (inward movement of the abdomen during inspiration)
Use of accessory muscles of respiration
Reduced chest expansion on maximal inspiration
Hypoxia or raised daytime PaCO2 on blood gases
My patient has symptoms of respiratory insufficiency but their respiratory muscle strength tests and blood gases only show a mild decline, do they need NIV?
It is important not to dismiss the need for NIV if the patient only has one marker indicating respiratory insufficiency. You should talk to the patient and explain that NIV may help with their symptoms and explore whether they wish to trial NIV.
What tests should I carry out to assess respiratory function?
You should carry out respiratory muscle strength testing and this should involve a measure of inspiratory pressure, vital capacity and peak cough flow. You should also perform some blood gas analyses. Learn more about the different tests:
Can I carry out respiratory function tests in patients with bulbar impairment?
Yes, but you must consider the factors such as the patient’s ability to do the tests, their risk of aspiration and concerns surrounding the validity of the tests. You should consider different steps to take in order to overcome the barriers of testing patients with bulbar impairment such as using different types of mask interfaces, prescribing medication to control secretions and seeking expert help and advice from respiratory specialists.
When should discussions about respiratory support and NIV begin?
Discussions should begin early, ideally at diagnosis as this allows the patient to plan their care.
What should I do if the patient does not want to discuss respiratory support and NIV?
It is important that you respect the patient’s decision not to talk about respiratory support and NIV. However, it is crucial that you show willingness to have these conversations and provide regular opportunities to open up conversations about them in order to review and re-discuss the patient’s wishes. Providing information leaflets can be helpful when patients do not want to talk about breathing support straight away.
When introducing the idea of NIV, what should I talk about?
It is important when introducing the idea of NIV to make sure the discussions are honest and comprehensive. You should discuss the benefits of using NIV including that it can help improve quality of life and may help to prolong survival but the emphasis should be on quality of life.
Patients should be made aware when starting NIV that they can stop using NIV at any time and they should be reassured that this is ethical and legal, and that healthcare professionals will support them. You should also discuss the limitations of NIV and that the patient’s use of NIV might change with disease progression.
My patient does not want to trial NIV, what should I do?
Not everyone will want to trial NIV and it is important that you respect the patient’s choice. It is important that you explore the reasons why patients may not want to trial NIV as they could be struggling to accept the diagnosis or have not been fully informed about NIV. You should also revisit the patient’s wishes as they may change.
You should reassure them that you will support them and discuss other treatment options. When discussing other treatment options it is important that you explain that these will not improve breathing and gas exchange in the way that NIV can do.