Tuesday, 28. May 2024
What is it like to grow up with the hereditary disease cystic fibrosis (CF) and to be an adult with the disease? Thomas Malenke, himself a CF patient, shares his experience in an interview.
Thomas Malenke: Growing up and living with CF is very different from growing up and living healthy. At least that applies to me, born in 1966. Back then, the medical and therapeutic options for CF were still very limited and in no way comparable with today. It affects everything – including your outlook on life. The main thing was always “focus on staying alive”, which used to and still does take some of the lightness out of life.
My healthy brother always lived his life more freely and unconstrained than me. When you have cystic fibrosis, you tend to become a manager of your health. My daily routine is strictly planned with fixed times for respiratory therapy – that means inhalation and mobilisation exercises – and taking medication. I also have to see my lung specialist at least every three months, which means I have to go to the CF outpatient department.
Thomas Malenke: Over time, the treatment options for cystic fibrosis have continuously improved. For many years now there have been good enzymes for digestion, antibiotics, efficient inhalation devices and also inhalation solutions. None of that was around when I was a child. I spent three months in hospital before the age of one. Then I really did very narrowly escape death.
Otherwise, I didn’t have many problems as a child. Apart from the fact that I was terribly thin, caused in part by the strictly low-fat diet, which you really have to stick to without the effective enzymes you have today. The lung problems started when I was 16. I coughed a lot and picked up every infection doing the rounds. When I was 20, I spent six or seven weeks flat out with a lung infection.
It wasn’t until I was 20 that I started inhalation therapy. With the availability of better enzymes and the resulting weight gain, and with generally better medications and treatments, my health has stabilised since the 1990s. I have recently become very hopeful about the CFTR modular therapy. I have not been taking the tablets for long, but they are already having an effect.
Thomas Malenke: I have been taking the CFTR modulators for about two months now. My lung function has significantly improved, and so has my blood sugar. But I have developed high blood pressure. I can breathe more freely and need less insulin (I have been suffering from CF-related diabetes for several years now).
Although my breathing has improved, I am still carrying on with my inhalation and breathing therapy. I don’t want to risk anything. I also heard about a recent study where lung function remained stable if you continue with the inhalation therapy despite taking CFTR modulators – and that it tends to drop if you stop the inhalation therapy. The modulators have increased life expectancy significantly.
Thomas Malenke: It is a challenge because when you have CF you can’t plan like a healthy person can. For instance, I only plan three years ahead. This is why my ex-wife and I decided not to have children because it was too great a responsibility. The statistical life expectancy back then was very low.
But aside from that, partners of someone with CF have to be robust and resilient and come to terms with the fact that come rain or shine, you have to consistently and unwaveringly have your therapy and that you need time for that. That is a challenge.
Common sense and health take centre stage. I don’t take risks such as travelling to distant countries where the medical standards are far below those of Germany. I’m often not as relaxed as healthy people are, which is something my girlfriend has to take on board.
Thomas Malenke: Because I always only planned three years ahead, given how uncertain my future was, I completely ruled out having a stellar career with overtime and the like. And that’s why I didn’t go to university either. But I have been working since I was 19, currently as a project leader, and I work a 40-hour week.
Thomas Malenke: In the back of my mind is the firmly held belief that my days are numbered. But life is always unpredictable, even for healthy people. The main thing is that you make the most of it and that you take whatever life throws at you in your stride.
Thomas Malenke: I have my inhalation therapy twice a day. I start with a hypertonic, 3% saline solution. Then I do my stretching and mobilization exercises for the chest. In the mornings, I then also inhale an antibiotic.
I also make sure that I fit exercise into my daily routine. That’s why I cycle to work and don’t take the lift, but take the stairs to my office instead. It is on the eighth floor. Regular rehab at the North Sea also really helps.
Thomas Malenke: It is very helpful to talk to other people affected by cystic fibrosis. You can manage many day-to-day challenges better if you benefit from other people’s experience. I have been working as a volunteer for 35 years to promote this exchange.
The CFTR modulators are an enormous step forward in the treatment of cystic fibrosis. Children born today with cystic fibrosis have much, much better prospects than I did back then. Of course, they do not cure the disease, but the chance of living a long and normal life is much, much better. I think that this is giving families hope.
Thomas Malenke was born in 1966 with the genetic disease cystic fibrosis (CF). He has set out to motivate others with CF and to promote an exchange between those affected. He has been involved in this endeavour for 35 years now: for example at the “CF-Selbsthilfe Köln e. V.”, and with the newsletter “CF-Freundesbrief”, webinars and blog articles at muko.info. In 2018, he was awarded the honorary certificate of the Cystic Fibrosis Association for his services to self-help. In 2022 he was awarded the Order of Merit of the Federal Republic of Germany.
Notes: The statements made in the report are the individual view of the persons reporting. They do not necessarily reflect the PARI view or the general state of science.
CFTR modulators improve the impaired function of the CFTR channel in patients with cystic fibrosis. A triple combination of CFTR modulators was first approved in Germany in 2020. Since then, CF therapy has been changing and is now more individually tailored than it used to be to the situation and needs of the patient.
An article written by the PARI BLOG editorial team.
© 2024 PARI GmbH Spezialisten für effektive Inhalation
