Blog post #14 SMA Awareness Month: Personal reflections on mouthpiece ventilation

• I describe Spinal Muscular Atrophy (SMA)
• I talk about how I think knowledge about the condition has developed
• I discuss my recent experience of starting daytime ventilation using ‘sip and puff

August marks Spinal Muscular Atrophy (SMA) Awareness Month. SMA “is a rare, neuromuscular condition. It causes progressive muscle wasting (atrophy) and weakness. It may affect crawling and walking ability, arm, hand, head and neck movement, breathing and swallowing. How severely people are affected, and in what way, varies greatly”(SMA UK, 2025). 

I was diagnosed with SMA just after my 1st birthday at a time where there was very little known about the condition and even less known about symptom management. Over the years, my muscles have become progressively weaker meaning that I need more support to carry out my day-to-day activities and generally stay well.

Due to research and knowledge into SMA developing over the past couple of decades, the treatment and support I receive from people (in the form of social care), medication (drugs such as Risdiplam) and technology (such as ventilation and respiratory devices like a cough assist) has enabled me to live a fulfilling life. Collectively, the treatments and supports have kept me alive when a common cold has turned into pneumonia and prevented many other serious illnesses.

As I have talked about elsewhere, I love research. I am the lead Community Researcher on Cripping Breath, a project based at the University of Sheffield, funded by the Wellcome Trust.  The aim of this project is to develop a new analysis of respiration and ventilation, centring people who have had their lives saved and sustained by ventilatory medical technologies (NIV/HMV). People like me. 

Cripping Breath is centred around arts-informed, archival, narrative and ethnographic approaches, to develop Crip perspectives - forms of knowledge production that emerge from lived experiences of disability and chronic illness. I primarily work on the narrative stream of the research alongside the Community Researchers and Jen Kettle, the Research Associate on this stream. We are aiming to collect people’s ventilation stories, including how different people come to use mechanical ventilation, their everyday experiences, and their thoughts on respiration and ventilation in a wider context.

I wrote this blog post for SMA Awareness month as my own ventilation story has recently changed. In a previous blog post I mentioned that I was a night-time ventilation user and I described what this meant to me. However,I have recently started using daytime ventilation. This comes in the form of a “sip and puff” ventilator. For me, this is a ventilator with the same settings as my night-time ventilator but is attached to my wheelchair to use throughout the day. Rather than having a mask on my face,  there is a long tube with a straw like interface. When I feel like I need to take a deep breath or speak clearly, I simply move my mouth to the straw, I begin to take a breath and the ventilator supports the rest of the breath. Making it a much longer and deeper breath than what I could ever manage even with the greatest amount of effort.

Hearing about commencing daytime ventilation felt quite shocking. I imagined it would happen much later in my life.  I'm also well rehearsed in having to persuade medical teams that I need help with something or advocate precisely that a part of my body isn’t working like it normally does. Although it is often incredibly frustrating and tiring, this process of advocating gives me time to adjust to change.  

I was also scared when it was suggested by my respiratory team that I should also use ventilation during the day. I felt like my body was failing because it couldn’t breathe properly when I was awake. I, being the sole person responsible for my breathing abilities, wasn't functioning at 100% when I was awake and aware. My initial reaction was that I was a failure.

I had to grapple with the idea of losing another part of my independence as breathing is something we do instinctively and naturally, not with machines, right? Another part of my life was about to be taken over by technology. Technology will inevitably become faulty, another ‘thing’  requiring charging daily and the prompt need to  organise my family and carers to be trained in using the new ventilator. 

I had  plenty of tears, some anxiety and a feeling of utter deflation. I had my family, friends, care team, work colleagues and of course canine friends to support me.  Apart from a couple of friends who have used this type of ventilation, I found it difficult to find information, personal stories and advice on using it. I searched tictok for some quick and accessible explanations of the "sip and puff" to share with my care team. I wanted something that explained the machine and its potential benefits without them needing to read a medical journal. I was aware that they needed time to adjust and come to terms with the change in my condition. But I found very little available.

So, how has the introduction of day time ventilation gone? I don’t wish to be a Pollyanna (a person characterized by irrepressible optimism and a tendency to find good in everything) after describing some difficult reactions to the change. However, I do want to raise awareness of SMA, progression, embracing respiratory technology and make it okay to have ‘a wobble’ when things change.

Perhaps I came to the realisation that research on neuromuscular respiratory care is moving forward. Likewise doctors could be embracing new technology to enhance well-being as well as prolong life? Perhaps it’s about acceptance of SMA and its realities. I have experienced subtle and small changes in my wellbeing that are useful for engaging in everyday life. For example, it has certainly helped to manage some of the tricky symptoms of SMA - fatigue and breathlessness. Learning to conserve energy and prioritise social interactions was ironically becoming exhausting. I have a little bit of extra ‘wiggle room’ when it comes to energy supplies.

Can you help to raise awareness of SMA this August? 

You can also join the Cripping Breath and Pathfinders team online Monday 22nd September 6:30 PM – 8 PM.  We want to bring the talk of ventilation out of the clinic and into real life - into our travelling ambitions, love lives, parenting journeys and plain old everyday lives. We want to create a space to learn from others by sharing tips and experiences and to talk about what being a ventilator user really means in society Sign up here