I decided to write this blog after speaking at a respiratory physiotherapist conference in September where I provided health professionals with a patient experience on using a ventilator or how these issues fit into or around my life.
I thought that before I start I wanted to express my gratitude for the wonderful care and respect that I’ve been shown by my respiratory consultant Professor Morgan, respiratory physiotherapist Jo Smith and the whole wonderful department. Thank you for improving the quality of my life.
As you know Duchenne Muscular Dystrophy (DMD) is a muscle wasting condition so it has slowly deteriorated my lung function along with my heart but definitely not my brain. Over the years I’ve tried (or Mum insisted) to maintain optimum lung function and (mostly) listen to doctors suggestions. At the age of 12, I had a back operation in order to prevent my scoliosis from crushing my lungs any further by using rods and screws to hold my spine straight. This operation was painful and took a year for me to recover fully but it dramatically increased my lung capacity and I also grew a few more inches. This spinal operation is meant to increase life expectancy for DMD patients by about 10 years so (in my opinion) it is an important but necessary decision to consider having it done.
I was introduced to the Nippy ventilator and the LVR (lung volume recruitment) bag when I was 19 at my annual respiratory appointment as my lung function was beginning to show signs of deterioration. I was initially reluctant to the idea of using the Nippy during the night as I saw no benefit but only an unnecessary nuisance. Funnily enough at the next respiratory appointment, I lied to the consultant about using it at night but he found out anyway as the ventilator records the hours it has been used so he told me off. The following issues I had with the ventilator initially appeared to be impossible to cope or deal with:
- The machine was noisy so I couldn’t fall asleep
- The mask was uncomfortable around my nose causing red marks
- I felt claustrophobic with the mask covering my nose
- It was scary to rely on a machine to breathe for me
- The mask was challenging to wear due to only being able to sleep on my left side
- Having to accept tangible evidence of my DMD / symptoms encroaching on my quality of life, my denial over the years had to finally be acknowledged overcome. (This issue was not apparent at the time only now I can look back as an adult)
The respiratory appointments are really vital to managing your DMD, I did not realise how vital the check-ups were until I understood the reasoning behind the
nagging insistence to use a Nippy BiPAP (Bilevel Positive Air Pressure). Blood gas samples are taken so that CO2 levels can be analyzed as high CO2 levels demonstrates type 2 respiratory failure, other symptoms of gradual failing ventilatory capacity are:
- Shortness of breath
- Awareness that extra effort is needed to take breaths
- Breathless when you lay on your back but not when sitting (Orthopnoea)
- Daytime dizziness or sleep disturbance
- Morning headaches and confusion
- Waking up not feeling refreshed
- Irritation all through the day
- Tiredness during the afternoon
The symptoms caused by high CO2 levels in your bloodstream can be assisted by the use of long-term BiPAP ventilation as it fully inflates the lungs thus decreasing high CO2 levels as DMD patients normally shallow breathe.
The time came at age 20 during my A-level year when I was hospitalized for a chest infection that I came to appreciate my nippy ventilator. Without the support of the respiratory department teaching me how to use a cough assist machine, providing chest physio and the realization that I was aspirating on food so I was then given a feeding tube (gastrostomy) which saved my life.
The Nippy ventilator improved my quality of life, I woke up refreshed and at the time able to concentrate on college work. I only saw the improvements once I used night-time ventilation for a few weeks, perseverance is important as initially my chest muscles were stiff from lack of exercise and it was a bit painful. Now it has become an essential part of my daily routine, nighttime ventilation has now become about 3 hours in the daytime, it was difficult to let it intrude into my day but it prolongs my energy to do the things I want to. For example, I wear it at the cinema, the car & at MDUK meetings. My main tip is to use a nasal prong mask rather than a full nose mask, the nasal prongs are less likely to cause pressure sores or red marks, less claustrophobic and more importantly looks much better.
I was also given an LVR (Lung Volume Recruitment) bag, a manual way to exercise your lungs or take deep breaths. I was taught by the physiotherapist to breath-stack using it, it’s a way to hold multiple breaths so you have a boost so you can carry on talking or just enjoying yourself. I wish I used the LVR bag earlier as it is easy to use and effective in improving quality of life and getting you used to respiratory assistance.
Ventilation has helped me medically but also emotionally & mentally. I’ve become more confident to speak because now I can concentrate on the conversation rather than worrying that I’m out of breath. I’ve become a calmer person, less irritable or angry as I have fewer headaches or I’ve become more aware when I need my ventilator so it is a good idea to take more responsibility of your own DMD.
I was worried that having a ventilator would ruin my life, restrict me, or somehow admitting I needed respiratory help meant I failed but it has improved every aspect of my life so I’ll be open to using it as much as possible. I hope this blog has been helped many DMD guys battling with the idea of ventilators.