Living with cystic fibrosis: “Not normal, but extraordinary and in some ways more positive”


Miriam is 29 and married. She has a secure job. She and her husband are currently looking for a house and are planning to start a family. Sound normal? But Miriam does not think her life is normal, but extraordinary. The reason is the incurable disease she has: cystic fibrosis. An interview.

PARI blog: Miriam, your “general set-up” – job, married, planning a family, looking for a house – sound like a pretty “normal” life. But you say that you don’t lead a normal life, that it is an extraordinary one because you have the incurable disease cystic fibrosis. What do you mean by that?

Miriam: That’s right. I do not feel normal, I feel extraordinary. Thanks to my awareness, my particular attitude toward life. And I have this because I have cystic fibrosis. Because of this disease, I have often had to deal with a fear of dying. I can never really shake it. No matter how happy and nice a moment is, death is always lurking in a corner of the room and I can see him. While I lead a normal life in that I have a job and a relationship, this feeling never leaves me. I am sure it is different for healthy people than for me. This attitude toward life that I have is not normal, it is extraordinary and I think that is better than “normal”.

What is good exactly about this extraordinary attitude toward life?

Miriam: Despite my cystic fibrosis I lead a happy and contented life. Of course sometimes I think that having cystic fibrosis wasn’t really necessary. But I think I am more contented than some healthy people. I think this is because my experiences and this disease have made me very aware of two important things. For one, I appreciate my health and capabilities at any given moment more than most healthy people. I am very aware that things could be different. Healthy people often do not know what a valuable commodity their health is and are reckless with it. They work so much they get sick. They ignore their limits, do not listen to their bodies, work until they are sick, although they could be healthy. They make the same mistakes over and over. I see this happening a lot with the people around me. I look after my body, and I am happy that I am still doing so well and do not want to put my health at risk.

For two, for me, death is not something theoretical that will happen at some point in the future, instead it is palpable and could happen very quickly. So I enjoy everything more. I treasure every day. I make a conscious attempt to see the beautiful things in life. I go through life with my eyes open, and like a child, every day I find joy in the little things: the beauty of nature, a little dog or a bright rainbow. I practically breathe in this positive energy. Other people do not pay attention to these things. Many go through life grimly determined and in a bad mood. They get upset by small things like red lights. My time on earth is too valuable to live like this. I focus on the good things. I think this unusual view of life is valuable.

PARI blog: How did you come to see life this way? What role did cystic fibrosis play?

Miriam: I would not be the person I am today without cystic fibrosis. It is what brought about this special view of life. I was 19 years old when I first felt mortal fear. This was after my school-leaving exams. I had an extreme breakdown of my health. It was slowly but surely building up for months. First, preparing for my exams was a ton of work. Then after passing my exams I celebrated non-stop. Right after that I started my job, and wanted to prove myself and so I took part in a one-week team-building training course put on by my employer even though I could already sense I was not well enough. I ignored my body’s signs. During a scavenger hunt my colleagues had to take turns carrying me because I couldn’t make it on my own. That’s why I found myself in A&E. I needed oxygen and was in a wheelchair because I was too weak to walk on my own. I was like this for three weeks. For the first time, I was truly scared about having this disease and dying from it. Two years ago, I felt this same mortal fear again. I woke up at night coughing. I was coughing up pure blood, a lot and only blood. At A&E they told me that parts of my lungs were in a catastrophic condition. They could see there were enlarged blood vessels. They said that if one of these vessels tore, I could be gone in an instant. For several months, I lived in constant fear of dying suddenly. These situations have intensified my awareness of the present.

PARI blog: How do you deal with the fear you have because of cystic fibrosis?

Miriam: In both situations when I had this fear, one of the reactions was to increase my respiratory therapy. At 19, I got even more serious about inhalation and my therapy. Two years ago, I worked harder on my problem of hyperinflation of my lungs and used the PEP System at least 30 minutes a day for weeks. I even had it in my mouth while I was doing chores. I like to do inhalation and respiratory therapy because I know it is good for me. My (at the time) new specialist was able to still my fear I felt then. She viewed my condition more holistically and put the statements made at the emergency department in perspective. However, the fear I felt at the age of 19 gripped me for many years. That was very stressful for me. Five years ago a psychologist explained to me that the body produces the stress hormone adrenaline when it experiences fear. She said I could break down this hormone by exercising and at the same time increase the hormones that produce a feeling of happiness, to confine the fear and promote well-being. Every time I started getting anxious, I exercised. My mood and my lungs thanked me. Now I exercise at least three times a week. Today my pulmonary function is just below normal values.

PARI blog: Increasing inhalation and respiratory therapy was part of how you coped with your fear. Why?

Miriam: That’s right. Fear is paralysing. You become passive and are not in control of your life. Therefore, I always try to be active when I am afraid. Doing inhalation and respiratory therapy is something active. Instead of the cystic fibrosis controlling me through my body, I am having an effect on the cystic fibrosis. I am turning the tables. I do not suffer from cystic fibrosis, I carry cystic fibrosis instead. Therefore, I increase my inhalation and respiratory therapy during phases when I experience frustration and fear. Also, exercise and sport are important to me. By doing respiratory therapy and exercising, I have the hope that I can help improve my situation myself. And hope is the feeling that is most often stronger than fear.

PARI blog: Miriam, thank you for this frank and open discussion.

Miriam: You’re welcome

Note: The statements made in the interview are the individual views of the interviewee. They do not necessarily reflect the PARI view or the general state of science.

An article written by the PARI BLOG editorial team.

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