Module 1: Living with

At the age of 21, I started using a ventilator at night, as my sleep gradually became worse. I was waking up every 30 minutes due to poor breathing and I felt that I had to turn around in bed to move my body in order to get more air into my lungs. The quality of my sleep was very poor and led to many hours of fatigue during the day.

Since my sleep gradually became worse, I contacted my GP. He referred me to a lung specialist, who gave me a ventilator and mask.
We put the ventilator on the bedside table at home and connected it to the mask, which covered my nose up to the middle of my eyes. Having a ventilator at night was more of a relief than a problem. It was something I had to accept. Going to bed knowing that I will have a good night’s sleep is something I will never take for granted.

In the beginning, I didn’t protect the skin around my nose, and as time went on, sores began to develop where the mask caused pressure. I tried different types of gauze and wound dressings, but nothing worked very well. The solution was a mask that was melded to the shape of my nose. It takes a bit longer to put on and fit this type of mask, so it sits comfortably and tightly, but so far this has been the best solution for me. The disadvantage is that it hasn’t been standardised, so if it becomes damaged it will take longer to replace. I developed a tiny sore on the end of my nose with this mask as well, a small piece of gauze covered with transparent film took care of that.

In cooperation with the municipal health service, I was responsible for making the small adaptations to protect my skin. I soon realised that the borough’s home care service were qualified to treat sores and that they could help with equipment.

I started using a ventilator during the day when I was 27-years-old. My lung specialist had hinted that it was perhaps time to do so a few years earlier. I was sceptical, as I wanted to breathe independently for as long as possible. I thought that the ability to breathe on my own would be greatly reduced over time if I started using a ventilator during the day. However, another user of home mechanical ventilation informed me that frog breathing over-compensated for a lack of oxygen. In addition, I also noticed that my daily fitness had significantly deteriorated. I had problems with concentration and was exhausted after spending an hour in my wheelchair. The days were very long. I just had to do something!

The lung department was contacted and I was given an appointment at the outpatient clinic. I wanted a device on my wheelchair that would enable me to use the breathing machine during the day. My doctor gave me three different mouthpieces to try. However, I was wary even before walking through the door and I didn’t think that any of the mouthpieces functioned satisfactorily either. In hindsight, I must admit that I could have had a better attitude and been more positive. Nonetheless, I did find a suitable mouthpiece during the appointment.

In order to use the machine for long period of time, the mouthpiece had to be attached to the chair in a fixed position, but I found that the specialist health service didn’t have a good enough solution. Having obtained information from other users, I purchased black electrician’s tape, some copper thread and a swing arm with an attached crutch holder, which I got from the Assistive Technology Centre. Out of this, we made a device that supported the tubes so that the mouthpiece was immediately in front of my mouth. I expect there are more solutions on the market today.

My condition has always deteriorated at a slow place and I have never felt that my disease has caused any major upheavals in my daily life. Therefore, the changes never felt like confirmation that I was getting worse. However, this changed when it became necessary to use a breathing machine during the day. Many questions arose: How much fuss will it cause? Do I look more handicapped? Will I seem weaker? How will it affect my daily life? Even though I don’t always like to admit it, I do want to appear strong in front of other people, which is something I have probably always wanted to do.

Looking back, I can conclude that the two times I had to employ the use of a breathing machine, my life was affected in a positive way. My energy levels shot up during the day, and my concentration and ability to focus on constructive things improved immensely. I got more pleasure out of following a conversation, watching a film or reading a newspaper article. Even mealtimes became a daily event instead of hard work.

It only took a couple of weeks to adapt, so I was fully comfortable with my new situation. In hindsight, I realise that I should have started therapy in the daytime sooner. But I suppose it’s only natural to ask critical questions about something that makes you uneasy and anxious. It was necessary for me to experience it in order to learn!

As mentioned earlier in the online course, I started ventilator treatment with a nasal mask (specially molded nasal mask/mouthpiece) at nights when I was 21-years-old. The need for respiratory support gradually changed and I have needed around-the-clock support since I was 27-years-old.

As time progressed, mask therapy became a problem. I soon developed sores, swelling and chaffing on the bridge of my nose and it became more difficult to talk. I also noticed that I was producing more mucus. As a result, in autumn 2016, I started to think about whether I wanted a tracheostomy or whether I should indeed have one.

I searched for information about tracheostomies on the Internet, Pust (an online course) and I spoke to users who had undergone a tracheostomy. In addition, I contacted my pulmonologist at the hospital. I received in-depth information about tracheostomies and the potential advantages and disadvantages that might apply to me. I was also informed that a doctor and nurse could visit me at home to give me advice, guidance and follow-up. This information made me feel confident and it played a significant role in my decision.

In December 2016, after a thorough review and assessment, I decided to have the operation and, in February 2017, I was admitted to hospital for a tracheostomy. For the first few days after the operation, I had quite a lot of pain in my throat where the cannula had been inserted. It felt strange to have a foreign body in my windpipe/throat. A standard plastic cannula with an inner cannula and cuff had been inserted. They gradually found the cannula that best suited me and it also had an extra suction channel to remove mucus over the cuff.

At the beginning, I had more mucus in my lungs/throat, but this gradually subsided. I use a cough assist machine and suction to clear the mucous daily.

I was admitted to hospital for three weeks before I was allowed to go home. At which time, my team and I had received in-depth training on how to handle the tracheostomy. We were also given written instructions on usage, cleaning, cannula replacement and unexpected events.

Initially, after being discharged, a doctor and nurse from the specialist health service replaced the cannula at home. My team will gradually receive training, so that we/they can do the procedure.

One of the disadvantages of having a tracheostomy is that procedures and the training of new personnel is more time-consuming, as I rely on assistants around the clock. I have also been given more technical medical equipment and consumables that need monitoring and depend on logistics.

The biggest advantage of the tracheostomy is that I do not need to wear a mask or mouthpiece and my voice gradually improved as well. It is also easier to get rid of the mucus/clear the mucus from my lungs with a tracheostomy compared to when I used a mask. I had respiratory infections every other year with mask therapy. Hopefully, I will not have as many infections, as it is easier to get rid of the mucus with a tracheostomy.

My voice is now better than when I used a mask/mouthpiece. Even though I had a few starting problems with pain and increased mucus production, I do not want to go back to having mask or mouthpiece therapy.

The decision to have a tracheostomy was difficult, but in hindsight it seems like it was unavoidable due to my situation and future prospects. Perhaps it would have been better if I had requested and received more information about all the therapy options appropriate for me with the diagnosis of Duchenne muscular dystrophy.

“It’s easy to look back in retrospect, but if I had known what I now know, I might have decided to have a tracheostomy earlier.”