Module 1: Living with

It’s just a matter of making the best of the situation and asking yourself whether you will actually become more handicapped by using a breathing machine.

This might sound like a cliché, but the values and attitudes inherited from parents greatly affect ones mentality and ability to independently achieve what one wants. You can enter adulthood thinking: “I’m handicapped, and as such, so are my abilities. I’ve got enough challenges in my life, I don’t need more. In short: “I’ve enough with just being handicapped.”

Or you can look upon yourself as equal to your brother or sister, male or female friend, and disregard the word handicap and say to yourself: “When I grow up, I’m going to have my own apartment, live where I want to live, have the occupation I’ve always wanted, and be independent of my parents.”

If you look upon yourself as handicapped and constantly hear that you are handicapped, you get brainwashed by the word ‘handicapped’. Think about how many times you heard the word ‘handicapped’ during your childhood. Probably so many times that you don’t need to remind yourself of it. This was how I tried to think for many years prior to leaving home. I can’t say that everything went according to plan, but I can say that I entered adulthood with my head held high. For that, I give my mother and father credit for the attitude instilled in me.

Living with respiratory failure means that I need assistive devices to compensate for my body not being able to breathe by itself. Assistive respiratory aids can be compared to assistive mobility devices, for example, a wheelchair for people who have difficulty in walking.  It is important that the person with respiratory failure and those close to that person, are aware of what it means to have respiratory failure and what needs to be done to live a good life with assistive respiratory devices.

But why am I qualified to say so much about this? In both my childhood and adult life with continual confirmation about my deteriorating health every single day, I can claim that there are two choices: You can ask yourself, “Why me? It’s so unfair!” Or you can accept the new challenge with open arms and adapt accordingly. The first alternative could lead you into a vicious circle where negativity breeds negativity.

In my situation, a good life meant accepting the situation and disease. Acceptance has developed in several stages. As a young boy, I didn’t have the life experience to understand the severity of the situation. Just the same as any other child, reflecting over life wasn’t one of my strongest points. I wanted to do fun things.  My parents hadn’t told me everything about the disease and prognosis. Therefore, it was probably easier for me than my parents to accept the situation. The question is whether acceptance has become more relevant to me as an adult in that I can now reflect over how it potentially affected me, not just at the time, but in later years as well.

In 1999, when I was 18-years-old, I came across some old statistics about the life span of Duchenne patients . They stated that people with such a diagnosis often didn’t live beyond the age of 23! It took this very literally and I remember that day very well. Talk about terrifying to read! It felt like a proper slap in the face. I believed this for a year and generally thought that I only had five years to live. I’d heard that the survival rate was lower than that of ‘fit’ people, but the fact that it was so low came as a huge shock. This was one of the few times that I’ve really had to put my values and attitudes to the test.

My life was not turned upside down by one isolated event during childhood. It has been a process with developments that have gradually led to a change in how my family and I have had to do things. My parents made the necessary decisions and changes at different points in my childhood and adolescence. For me, that was just ‘the way it was’. A quick and radical development would probably have been harder to process.

I was the middle child of three siblings and grew up in rural surroundings in West Norway. The nearest city was Bergen, a few hours’ drive away. My childhood was safe and secure in a small and orderly environment with nine pupils in my class. Everyone knew each other and it was easy to form new friendships.

I was diagnosed with Duchenne muscular dystrophy at the age of four. At that time I could walk and stand, but could not run. It prevented me from playing with other children and it stressed me out trying to keep up with them. Nonetheless, I managed to establish contact with some of the other children in the village.

I got an electric wheelchair when I was eight and I used to sit on the sideline whilst the other children played football in the school playground. It didn’t bother me, as I didn’t think that I was the only one who couldn’t play football. The wheelchair enabled me to accompany my family for walks in the woods. I was more independent and could move faster outdoors.

Family togetherness was always important. Even though I was more challenged than my siblings in general, my parents always focused on treating us quite equally. To the extent possible, they wanted us to do the same activities whether it was a fishing trip, walk in the woods and fields or just playing with the other children in the neighbourhood.  The main focus was my parents’ idea that my handicap wasn’t going to signify my daily life. I was going to do what other children could do as much as possible. I wasn’t going to sit indoors and think about everything I couldn’t do. My mother ordered me to go outside and play if had been sitting indoors too much.

In hindsight, I think my parents thought that no one was to be too concerned about the fact that I was handicapped. But, of course, my handicap was the focus of attention when I had to adapt to assistive devices, meet medical personnel in connection with outpatient check-ups, and training and physiotherapy, etc.

The values and attitudes I possess today mainly come from my parents, but something else also turned out to be an important source of inspiration.
Having a handicapped child leads to limitations. A parent can feel that many things have to be taken into consideration, which can also affect the relationship between the mother and father. However, there was one solution that worked very well for both my parents and me. When attending the outpatient clinic for a check up at the age of ten, we were offered respite care.

Parents of handicapped children are offered respite care to give them a rest and break. The children, however, get to experience a different type of day with several mandatory or voluntary activities. Although, my respite care was not entirely different to my normal daily life. I took my school homework with me and the special education department at the respite care centre followed me up. I also met others in a similar situation and gradually became well-acquainted with four other users. Even though it was very important to me to have friends that were not handicapped, I also learned a lot from others in the same situation as me. At that time, it was immensely inspiring. In that way, I learned how to be more independent and I believed more and more that I could manage on my own. It could just be a simple thing like going to the shop and asking a shop assistant for help if I couldn’t find a product. Activities were also staged, making it possible to visit the city centre (Bergen). For me who came from a small village with few services, this was a huge change of scenery. I soon discovered that this was where I wanted to live when I grew up.

If it is possible to meet like-minded people in similar places, I look upon it as a perfect opportunity and one that I wholeheartedly recommend.

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.”

There comes a time in every typical Norwegian family for young people to leave home. This should also apply to young people with functional impairments. I look upon this as essential in order to become independent and to attain self-accomplishment. I left home in Year 2000 to live by myself and to be nearer to a place with more opportunities and services. When looking back, I wasn’t very independent at that time. I knew nothing or very little about what it entailed to be responsible for the management of my own daily life without the help of my parents to guide and adapt things for me. I didn’t have the competence to manage my own personnel to cover my needs nor to obtain the assistive devices I needed or to keep track of financial support schemes, write applications or manage my own private finances. For someone who thought it was scary to telephone the doctor’s surgery to make an appointment (I got my mother to do it), it was like wheeling a wheelchair backwards on the edge of the Pulpit Rock.

In order to develop, you can’t stay inside your comfort zone forever. It might be the most comfortable thing to do, but if you want to get anywhere in life, you must be prepared for the occasional uncomfortable situation. It’s difficult to do something for the first time, often you’ll wish you were somewhere else, but such experiences have given me so much confidence. In addition, I have released some of the fear I had of failing.

I’m one of those people who needs to be pushed and pushed and put in a position where I have to do something in order to learn as much as possible from a situation or event. The experiences I gained when I relied on myself for the very first time are those from which I have learnt the most during my life. I think it’s easy to underestimate your own capacity, at least in situations where you feel insecure.

But, of course, it hasn’t always been plain sailing. I’ve got into situations where I had to fly half way around the world in search of elegance and perfection. There will always be bumps in the road on the way achieving what you want in life. It’s just a matter of equipping yourself with the best shock absorbers as possible.

Deciding over one’s own body and health is indisputably a human right. During my life, I have met many doctors, nurses and representatives of other health professions within both the municipal and specialist health services, all of whom have wanted to help me with advice about my health. More often than not it would have been foolish to ignore the advice. They have studied for many years and I do not doubt their competence.

On the other hand, it is still important to remember that as a patient, I always have the last word about decisions concerning my health. We don’t always want to do what we should. I should be under medical observation 24/7, live in a sterile room with access to vegetables only in a place where authorised health personnel can only enter wearing airtight space suits. But who wants to live that way?

All independent adults have the right to decide whether they want to spend the whole day eating French fries or chicken salad (without dressing) or whether they want to consume alcohol four days per week. This is rarely a topic for functionally fit people, but I’ve experienced such situations several times.

If someone was to continually decided over my health and said they knew what and wasn’t better for me, I wouldn’t be an independent human being with my own options. What pride would I have? Taking such choices away from an astute adult person disrespects the person concerned. For example, if I lived in an institution I wouldn’t have all the choices I deem necessary to experience personal freedom.

Fortunately, I now have user-controlled personal assistance (BPA) during the day and evening. This scheme allows me to make my own choices, whether good or bad, and enables me to not only decide over my health, but also how I want to live my life. Sadly, I know that many people do not have the freedom I frequently take for granted.

Self-pity is a human mechanism that everyone has tapped into at some point. Perhaps, it’s more natural to be self-pitying if you are functionally impaired. It’s easy to confine yourself in a world where your thoughts revolve around on might-have-beens and why things are as they are. I usually say: “Don’t overthink too much.” If you over-analyse such questions, it’s easy to end up in a vicious circle where self-pity breeds bad thought patterns and a negative mentality that affects each situation you find yourself in.

It’s important to have a realistic approach towards what you can and cannot do. This means that you must not choose unattainable goals. Even if you were to have the opportunity to jump after Wirkola, it doesn’t mean that you should. Having said that: it doesn’t mean that you can’t move mountains. It’s important to set goals, and to create a plan for self-achievement and where you want to go in life. As a daily wheelchair user, physical obstacles are continually encountered, but often there’s an alternative route to take.

From early childhood, I have been able to conquer the high mountains in my home municipality. Some might think that it’s impossible for a handicapped child to go up steep, hilly forest terrain, but my father didn’t think that way. His ability to ignore obstacles is a quality that I now possess. A positive attitude forms the foundation for seeking opportunities and achieving your desired quality of life. In the media, one hears of functionally impaired people doing the so-called impossible for someone in such a situation. I doubt that these people sat down and said to themselves, “It can’t be done, imagine everything that could go wrong,” before going out and accomplishing that which seemed impossible later. I’m sure their approach was completely different. You only have to look at Cato Zahl Pedersen or Stephen Hawking, who had a tracheostomy in 1985. Each person has their limits and as a functionally impaired person, it is important to challenge these boundaries. Not everyone can be like Stephen Hawking or Zahl Pedersen. We all have to overcome challenges, whether big or small.