Tracheostomy

A personal perspective: The decision for a Tracheostomy (trache*)…

Today, we can see all the benefits of a trache* – our son Erdi (who has RYR1 centronuclear myopathy) can now concentrate all of his energy on just enjoying life, instead of trying to breathe.

But it wasn’t always like this because the first thing my husband Ed and I thought when we opened up the Myotubular Trust website and saw the beautiful photos of the affected children, was ‘hopefully Erdi won’t have to have a trache*’. For us, we seemed to be accepting about everything else his disability may bring, like having to use a wheelchair for example – but just not the trache. The idea of a ‘hole’ in his throat scared us so much.

(* tracheostomy or trache is a small incision in the windpipe used for ventilation and breathing.)

In the early days, Erdi did cope using just bi-pap ventilation through a nose mask, and was even able to take some time off it for a short time. We are glad we tried this route of ventilation, but his breathing ability gradually changed over time and as he grew bigger – to the point where he began to rely on bipap more and more. Eventually he was using 24/7 bipap ventilation through his nose mask – which was also causing problems with the shape of his face because we had to pull it tight to stop any leaks. Leaks caused him to desaturate and affect his safe blood gas levels. He also still kept having periods when he was becoming unwell and needed to be intubated (a breathing tube placed into the lungs via nose or mouth for ventilation) because of respiratory infections and desaturation. Looking back, it was such a difficult time and often really upsetting.

Then when he was 11 months old, the time came that we had to make a decision about giving Erdi a trache. It felt very hard to decide because Ed and I thought ‘are we just making him struggle and suffer more?’. I also didn’t know how the trache was going to help Erdi.

Because I had witnessed Erdi needing to be intubated following illness to help him breathe and allow his body to rest, that made me reflect a lot. I decided that I didn’t want him to be going through this experience again and again – it felt so shocking to see that as it was usually an emergency situation. I also felt I had a responsibility not to allow it to happen again. But still I struggled to decide what to do, for another two weeks.

Then my mind was suddenly changed, when I had a meeting with Professor Heinz Jungbluth – Erdi’s neuromuscular doctor. Dr Jungbluth chatted to me for about an hour about Erdi and the options we had available to help him. But it was one single sentence from him made me decide that the tracheostomy was the right thing to do. He said ‘if that wasn’t an option for Erdi, we wouldn’t have given you this opportunity. Because we think it will help him to have a trache’. It totally changed my mind – to think that he was being given an opportunity and that it could work for him. It helped too that I could trust the doctor and what he was saying to me – he showed real empathy.

Dr Jungbluth had clarified my thoughts and helped me decide straightaway that we were going to go for it. I knew that with a trache came lots of responsibilities – and I knew that wouldn’t be daunting for me. I decided that I would have to take care of it for Erdi; to learn to change it; deal with it in any emergency and learn to do the trache ties.

On the day of trache surgery – Erdi stayed in theatre for about 3 hours. It felt so scary. But he came out of surgery smiling – with his sweet face beaming up at me. At that point – a whole burden was lifted off my shoulders – too see him just smiling up at me free from any mask and strap restrictions over his face. It was the first time I felt weightless – and in that moment I knew that we had also reached the final point we could reach for Erdi to essentially give him his best fighting chance to live with his muscle condition. I wanted to keep that last hope for Erdi locked away in my heart – we had given him a chance.

Choosing for your child to have a trache is very hard and comes with mixed emotions – but quickly after he was given a trache – it made me realise how beneficial it can be. There are so many more positives to hold onto. For example, you can have direct access to the airways more quickly when they are unwell. But the most positive thing for Erdi is that we saw how much he improved, it was so fast as well! He could talk, move more and had more energy to do everything. He would wake up from rest fully alert and expressing himself in everything. The trache has made such a major difference to his life.

One lesson I would pass on to others is not to be scared, but listen to a doctor you can trust and talk to other parents if you can. For me, it quickly became easy to manage – you do get used to it. But the best thing about a trache for Erdi is he is more comfortable, we can easily care for him and do things for him to give him a happy and comfortable life. I feel more confident to take him out because a bipap seemed more difficult to manage outside the home. He can go to nursery and walk to school with his brother and sister sometimes, or go to the park. He is so much less tired because he is so much better ventilated with a trache. Before, with a bipap, he didn’t have the strength to do any of these things – he just concentrated all of his strength on his breathing.

I just can’t wait to show Dr Jungbluth our little Erdi today. He deserves to see the opportunity that was given to him and the big difference this decision has made for Erdi. (And also maybe some of the doctors who were so negative about Erdi’s condition). He enjoys the world now and is achieving so much. He is stronger than ever!