Discussing respiratory support and NIV

James

“Hello, my name is James. I’m 30-years-old, from Doncaster in South Yorkshire. I was diagnosed with motor neurone disease in November 2017 at the age of 29. It’s one of those things that life puts in front of you, that’s my view of it, and for me it’s helping people in years to come. That’s what I want to achieve from my shorter journey. I know that people will have gleaned a lot of help from my experiences.”

Steph (James’s partner)

“Obviously, when he received the diagnosis on that day, he was very upset. It was strange because I thought that for a long time, you know, he may be quite down about it and struggle. He didn’t. Obviously, we also had just had the baby six days prior to his diagnosis. That was a big thing as well, he said, you know, I want to be positive for the baby and I want to be a role model for the baby and try and be a normal family, if you like, and continue with life.”

James

“I was experiencing a lot of yawning during the day, feeling tired. So, what they said was ‘we’ll just get you checked out. We’ll send you for a 24-hour oximetry which will measure your oxygen levels.’ They looked at the results and it was there that she said ‘your oxygen levels have dipped a little bit.’ I think the lowest one was 83%, which is not good really - as your oxygen levels should be in the high 90’s, or at least the low 90’s.

So, she issued me with it and said ‘I want you to get used to it, the NIV machine and the benefits it can give.’ She explained it really well; what it would do and what it would help with. ‘We just want you to get used to it so that, when eventually you need it a lot more than you need it now, you’re ready for it and you’re not struggling to get used to it when you’re dependent on it and we think it will benefit you a lot more as well now.’

To be honest, I thought it was going to be a lot bigger. I hadn’t really seen one and she brought it out in this little gym bag, as you call it. I was like ‘Well, it’s pretty tiny to be fair’, that’s what I thought. She went though it really thoroughly. I’m really pleased with how she put me at ease with it, and that was it, we brought it home.”

Challenges of introducing NIV to people living with MND

Professor Chris McDermott

“There are many challenges of caring for people with MND and supporting them through their journey. I think that, when we’re beginning to talk about breathing support for patients, introducing the concept of non-invasive ventilation, that’s a particularly challenging time because you’re acknowledging that, perhaps, a new part of their illness with MND has started.

There’s a lot for the patient to take in, they’re taking home equipment that might initially be frightening to them, you know, taking home this equipment, this ventilator, so there’s often anxiety for the patient.”

How to manage early discussions about NIV and the importance of gauging how much understanding the patient has about how breathing is affected in MND

Matt Cox

“A lot of the time, my first questions will be ‘Do you know why you’re here?’ but also ‘What do you understand about the effect that MND has on your breathing?’ and ‘Have you heard of non-invasive ventilation?’ So just try and gauge their level of understanding before you start discussions.

Some patients will come with a lot of prior knowledge and have done a lot of reading and have done a lot of research into their condition and into the respiratory complications of MND. Some patients will come and either won’t have had those discussions or won’t have listened to those discussions.

If those sorts of things have been discussed alongside diagnosis, quite often patients haven’t taken it in and haven’t heard what’s being told to them. So, I think, trying to gauge their level of knowledge and understanding is the place to start with those discussions.”

The importance of having a multi-disciplinary approach when introducing the concept of NIV

Matt Cox

“I think it’s important to recognise that motor neurone disease is a complex condition. Introducing NIV isn’t an easy thing to do; there’s often a lot of things that make it very difficult. So it’s important to involve all members of the multidisciplinary team when trying to troubleshoot and trying to introduce non-invasive ventilation.”

Describing the psychological difficulties that people with MND face, particularly when thinking about NIV

Dr Emily Mayberry

“My name is Emily Mayberry and I’m a clinical psychologist in the motor neurone disease team in Sheffield. As a psychologist, when I see people with MND, they often describe feeling like they’re experiencing multiple losses; those symptoms they have, even before knowing what the symptoms are caused by; and then there’s receiving a diagnosis and all of the loss that brings in terms of thinking about the future and what it means for them and for their health and their family; and then, over time, people continue to experience more losses as their health deteriorates.

They need more help, they lose more independence, lose more mobility and - depending on the person and the things that matter to them and the course that the MND takes - it can continue that way.

I think it can be a really hard thing for people to accept and come to terms with as well. Many people don’t want to think about it or don’t want to talk about it, and in some ways that’s really helpful. If people can focus on the present and the here-and-now, then they aren’t spending their time worrying about the future or grieving for the losses that they’ve had.

But, at the same time, if people don’t spend any time thinking about the future and the things that matter and how they want things to be then, actually, that can cause a lot of problems for them down the line as well. So, it’s a real balancing act, but I think the grief that people experience and the coming to terms with it, and what that means, and getting that balance between being present and focused on what matters now versus planning for the future as well.

NIV can be both an immediate sort of threat to people, in terms of the item itself feeling frightening, but it can also symbolise the loss of independence, the loss of control, the loss of ability to breathe and can therefore remind people of those really deep worries that they have as well.”

How to support patients who choose not to discuss NIV

Debbie Freeman

“Patients don’t always want to talk about the future and we have to go at their pace. I think what’s really important is that you give them the opportunity to engage in those conversations every time you meet them. I often ask them if there’s anything else they want to talk about, do you have any concerns, do you have any new questions that you want to talk about? I also give the carers an opportunity to ask questions.

Very often we find that - at the front door - carers have questions for us, that relate to end of life, that they don’t want to discuss in front of the patient. So you have to manage it very sensitively but, as long as you’re available to deal with that when you can and put things in place to support them, you can manage without them wanting to have direct information.”