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In our blog series, we are handing over to our wonderful Patient and Public Involvement and Engagement (PPIE) group members. They share their experiences of using NIV, and what it is like to work alongside our researchers to help shape a more inclusive NHS system for people living with MND.
My first experiences of NIV - Peter Camburn
My first experiences of NIV - Peter Camburn
At first, I recoiled at the very thought of NIV, but now I would be lost without it.
The Motor Neuron Disease Clinic at The Royal Stoke Hospital has different specialist teams. One area is Sleep and Ventilation. This department started an investigation into how well I was sleeping. The somewhat alarming conclusion was that I had severe obstructive sleep apnoea in addition to MND! The treatment would be to use a non-invasive ventilator (NIV) at night. I didn’t like the prospect of wearing a mask all night. Also, I felt that I wasn’t experiencing symptoms like falling asleep often in the day or feeling drowsy. So I rejected the offer of an NIV machine.
The turning point
An incident one evening changed my mind. After my evening meal, I fell off my chair, cut my head on the corner of the sideboard and ended up on the floor, unable to move, and with my face in a pool of blood. I managed to grab a phone and call for an ambulance. The call handler asked several questions; one sticks in my mind, “How much blood is there, a mug full or less than a mug full?”
The upshot of all this was that I changed my dining room chairs for ones with arms, and I decided to accept the offer of the NIV! I was convinced that I had fallen asleep sitting at the table, and that sleep apnoea was to blame. In addition, every time I started working at my desk, I became sleepy. I collected my NIV machine from the hospital on the 5th of July 2023. After instruction, I’ve used it every night since.
Getting used to NIV
There were, of course, a few teething problems. One morning, I awoke to a mask with blood in it. I had bitten my cheek. There were several symptoms that affected the use of the NIV. The saliva secretions would almost fill the bottom of the mask. I remember dabbing them up several times before deciding to finally put on the mask. The main problem initially was that the full-face mask, covering my mouth and nose, made a wound on top of my nose. The District Nurse dressed the wound, and the GP was involved. After consulting the helpful Sleep and Ventilation Team at the hospital, a different mask was suggested, one that covered my mouth but fitted under my nose. I still use this (F40) mask today.
At first, my NIV was set in CPAP mode, which meant that the flow of air was at a constant pressure. The NIV machine was checked at the regular MND clinics, and any problems were discussed with the ventilation nurse and with the consultant doctor. About a year after starting to use the NIV, I started to become drowsy in the day again. It was decided to change the CPAP mode to one where a pressure was set for breathing in (IPAP) and a different pressure for breathing out (EPAP). This mode is still in use today, although the pressures are checked and changed if needed. Also, a second NIV was provided for use downstairs in the daytime, and in case a backup machine was needed if the nighttime NIV developed a fault. Throughout this period, I had the help and support of the MND Specialist Nurse who visited monthly.
Using NIV gives me energy to enjoy my MND garden (Image copyright: Peter Camburn)
Benefits of NIV
The introduction of NIV certainly resolved the sleep apnoea symptoms, but it also had wider benefits for my MND. The saliva secretions have been with me now for over three years. They cause coughing and choking, and there can be chest infections too. When I started to use the NIV, the coughing was significantly reduced, and I generally get a good night’s sleep. There appear to be wider benefits as well. I get less fatigued in the day and, therefore, can tackle the many difficulties MND presents better than before.
My involvement in the DENIM trial
The opportunity to be part of the DENIM Patient and Public Involvement Group has been so rewarding. DENIM is such a good example of where a research project can help people with MND. I have been fortunate to have the backing of the Sleep and Ventilation Department at The Royal Stoke Hospital, together with the MND Coordinator and Specialist Nurse. If there is an equipment problem, I phone the NIV Helpline and leave a message. A return call lets me know that a new mask or other item will be sent in the post. I’m aware that not all NIV users are so fortunate. DENIM sets out to deal with that inequality.
There is, of course, more to be done. The potential for NIV to enhance the quality of life for MND patients is huge. It might even extend life! So, DENIM matters for the whole MND community.
Eric: Our MND Journey
Colin is a member of the Patient and Public Involvement (PPI) advisory group for the DENIM trial. DENIM stands for Delivering Effective Non-Invasive Ventilation in Motor Neuron Disease. The trial aims to change how NHS teams provide non-invasive ventilation support for people with MND. Colin told us about his and Eric’s journey with MND and using non-invasive ventilation (NIV).
Eric and Colin were together / married for nearly 37 years. Eric sadly passed away in 2024.
Receiving a diagnosis
Eric was diagnosed with Motor Neuron Disease (MND) in 2015. He started to experience some unusual symptoms around 18 months before being diagnosed. These included walking differently, tripping over things and falling over. Despite knowing he shouldn’t, Eric Googled his symptoms, and a feeling began to form that he had multiple sclerosis or MND. His GP, on the other hand, suggested that Eric had pulled a muscle. Eric knew this wasn’t the case, but his GP still sent him for further assessment.
Eric eventually saw a specialist, who confirmed that he had MND. He had already come to terms with his diagnosis, but he was concerned about the lack of available support. Fortunately, Eric was seen by several professionals, including speech and language therapists, physiotherapists, occupational therapists, specialist nurses and social workers, who collectively offered him the assistance he needed.
Future-proofing their home
Eric and Colin started thinking about their living situation. Recognising that the stairs would become a problem if Eric would no longer be able to walk, they decided to move into a bungalow in an area they liked.
They started making changes to their new home, including bathroom adaptations and putting in track and hoist systems. Eric was shocked at how much it cost, but the changes to their home were invaluable. The local council’s Early Intervention Team was brilliant. Alongside a blue badge and some portable equipment, the team provided them with walking sticks to begin with, and then a walking frame, and then a manual wheelchair.
The “ups and downs” of disability
Assistive technology: the boundaries are endless
Eric started to lose the use of his hands, prompting a referral to the Regional Adaptation Team. The team aimed to help him find comfortable ways to continue using his phone and to explore the use of voice-activated systems on his work computer. They were able to set up a system where Eric could access the TV, lights and his work computer, using a joystick on his wheelchair. The system was helpful at first, but as Eric continued to lose the use of his hands, using the joystick on his wheelchair became too difficult, and he decided to stop working in 2019.
The Adaptation Team acted swiftly to find another solution for Eric. They introduced him to EyeGaze (now called MyGaze), and he took to it in seconds. It enabled him to switch on the TV, watch videos, create music playlists and open the front door, all by simply moving his eyes. Additionally, the technology had a voice-output function, which Eric used to preserve his breath and communicate with his family, friends and lovely carers.
The use of assistive technology proved invaluable for Eric, and the Regional Adaptation Team introduced him to technologies he didn’t know existed. While there were some limitations in terms of what the technologies could do, Colin feels that, with the advancement of the field, especially artificial intelligence (AI), the boundaries are endless.
Using Non-Invasive Ventilation (NIV)
Eric was admitted to hospital due to erratic blood gas reading in July 2019. Shortly after this stay, he started using NIV. From Colin’s perspective, Eric took to it easily. If something could help, Eric was determined to use it. He tried on different masks and preferred the one that covered his nose and mouth. Eric went from using NIV for just a few hours a day to using it almost around the clock. He had two NIV machines, so he could use them in different rooms, and it was reassuring to have a spare.
Starting to use NIV involved a steep learning curve, but it proved invaluable. Eric and Colin faced some practical challenges. These included figuring out how long the batteries lasted, what would happen if the power went down, whether the machine was waterproof and how to clean the NIV machine and the hose. Their local health care team was very responsive and answered their questions as they arose.
Eric faced another issue: air was leaking from his mask. This can happen if the mask doesn't fit snugly, resulting in a small gap between the mask and the face. The leak was often worse when he was reclining or sleeping and would cause the machine to beep. The beeping rarely woke Eric, but it often kept Colin awake. Their local health care team provided Eric with another strap, which helped achieve a tighter fit of the mask. He also started to use a silicone nose bridge strap which helped. However, sometimes using NIV was uncomfortable, and Eric would get sores on the bridge of his nose.
Eric and Colin also received support from the NIV machine company. A phone number to reach their call-out service was written on the back of the machine. The team were able to advise, troubleshoot and arrange for a technician to visit. Their service was excellent, and they were able to help at any moment, even in the middle of the night.
Using NIV helped Eric and Colin to continue doing things they loved - meeting up with friends, going to the cinema, seeing exhibitions and going to the theatre. The NIV machine was portable and went everywhere with Eric whenever he left the house.
Though it was a bit challenging at first, using NIV was invaluable. Eric and Colin are so grateful for the equipment and the support received for its use and maintenance.
Top: Eric using NIV, Bottom: Eric with his NIV machine
Eric and Colin enjoying life with MND. Bottom left: On a flight to one of their final holidays. Right: “The world is your oyster”
Eric and Colin’s top ten tips for using NIV:
1. Get the necessary training that is offered for using the NIV. Ensure that all informal and formal carers attend.
2. Keep any guidance notes to hand near the NIV.
3. Keep the emergency call-out number to hand, in case of difficulties.
4. Try to have a second spare NIV machine…ideal for different parts of the home (e.g. bathroom); travel; and also for emergencies.
5. Keep the NIV machines charged at all times.
6. Clean all equipment, including hoses, thoroughly as per instructions.
7. Consider nose strips if air is leaking from the mask.
8. Masks come in different sizes; find the most suitable and don't be afraid to ask for another one to try.
9. Ensure the provided service/maintenance date is adhered to.
10. Fully appreciate the freedom and quality of life that NIV brings!
We’d like to thank Colin for sharing his and Eric’s MND journey with us!
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