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

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?

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.