Tuesday, 11. June 2024
Many patients with cystic fibrosis are chronically infected with the bacterium Pseudomonas aeruginosa. Chronic infection and inflammation can lead to fibrotic changes and respiratory failure of the lungs – the most common cause of death in cystic fibrosis.
How do patients manage the bacterium? In an interview, Marlene* explains what life is like with a pseudomonas infestation. She has cystic fibrosis (CF) and has had the bacteria in her lungs for almost 40 years.
Marlene: When I was 9, I had to go to hospital to have a splinter surgically removed. I spent about a week in hospital at the time. The next time my sputum was tested, the bacterium was identified for the first time. So, it is fairly certain that I picked it up in hospital.
Marlene: Not at all. I carried on taking my long-term prophylaxis against Staphylococci, carried on with the inhalation therapy, and that was that. Back then there weren’t any inhalative antibiotics. I can’t remember if it was normal at that time to immediately blast the infection with intravenous (IV) antibiotics, or if I didn’t want to be on my own in hospital for two weeks. IV therapy at home wasn’t an option back then.
Marlene: When I was 12, I went to hospital on the recommendation of the lung specialist I was seeing at the time, and started on IV penicillin – unfortunately, nobody knew that I was allergic to it. So, after just a few minutes I went into anaphylactic shock and nearly died. My arms and legs were completely blue, my face was swollen up. And because of the extreme allergic reaction, my lungs also swelled up.
I also developed allergic asthma and occasionally had acute attacks of suffocation in the years that were to follow. This experience meant that my attitude towards IV therapy, which was pretty low to start with, reached rock bottom, as you can imagine. It wasn’t until years later – I think from the age of 16 – that I was able to bite the bullet and have a standard IV therapy about once a year.
Marlene: I don’t have any record of my lung function (FEV1) from when I was a baby to now. I can remember that when I was a child and adolescent, it was always between about 60 and 68%. As a young adult I once almost touched on 80%. At that time I did about an hour and a half of sport a day, six days a week – karate and swimming. So far, my personal best in the lung function test is 79% FEV1.
My case shows that the value does not always have to drop even with a chronic pseudomonas colonisation. But it did drop lower than I could ever have imagined for a few years.
Marlene: There were two key episodes in my life. The first was when I was 19 years old. Because I had an allergy to mould, I was prescribed a high dose of cortisone, that was actually too high for my body weight. As cortisone suppresses the immune system, the bacteria were able to proliferate really fast in my lungs.
Within just a few days, my FEV1 plummeted from 68% to 42%. I had a high fever and had to spend three weeks in hospital, where I was given three different antibiotics intravenously. As it had been when I went into anaphylactic shock, there was a moment where I thought: That’s my life at an end, then. I mentally said goodbye to my family and told them I loved them very much. I wanted that to be the last thing they heard me say.
Fortunately, I turned a corner: The antibiotics did actually work after about a week, and I was able to escape the clutches of death once again. Intensive inhalation and breathing therapy were important for my recovery from this severe lung infection.
In hospital, I used every spare moment I had to use an oscillating breathing device and went up and down the stairs. I did twist exercises to mobilise the secretions so I could cough them up. A few weeks after my stay in hospital, I was back to my old FEV1.
Marlene: When I started work, things got gradually worse. I was working in an advertising agency and doing lots of overtime. There was not much time for sport and plenty of sleep, which meant that my health deteriorated.
But it had always been important for me to lead a “normal” life and keep up with “healthy people”. That is why I was prepared to put up with it. The pseudomonas bacteria really caused me lasting problems when my child started in childcare.
Here, I was again unlucky, and several unfortunate factors came into play all at once. My new outpatient CF reduced my inhalation therapy and my child kept bringing respiratory infections back home. That took a toll on my immune system, which had already been worn down by lack of sleep and the double hit of motherhood and work. This meant that I kept getting lung infections caused by the pseudomonas, which I had to fight off with ever longer and more frequent IV therapies.
After a while I went back to my old inhalation therapy. That improved things a little. But because I had had so many lung infections, the tissue had already sustained quite a bit of damage and was covered with bronchiectasis. My new doctor told me that I probably had around another 10 years at this low level and then I would either die or have to have a lung transplant. In December 2017 my lowest FEV1 reached: 35%.
Marlene: Fortunately! I feel much better nowadays. My FEV1 is around 56% at the moment. That is clearly down to the fact that I have been able to take CFTR modulators since 2020 – initially in a clinical trial. I was just about eligible for enrolment back then with an FEV1 of 40%.
Since taking the CFTR modulators, I have been feeling better and better. The pseudomonas is almost no bother at all now, even though it has colonised my lungs. I have had two IV therapies since 2020 – one of which wasn’t actually necessary. I took it as a prophylaxis.
Marlene: Taking CFTR modulators does not change my previous inhalation and breathing therapy. I have carried on with everything as I used to do, and have nebuliser therapy twice a day for around an hour. I use the eFlow®rapid for this, where I inhale a hypertonic saline solution with bronchodilators, and then another mucolytic and antibiotics.
I would never completely give up on the nebuliser therapy with saline solution. I see it as a prophylaxis to protect and moisten my airways. I also make sure that no secretions remain in my lungs. The less mucus there is in the bronchial tubes, the less breeding ground there is for the pseudomonas.
I also cannot take the antibiotics currently available for dry inhalation. They either make me cough or make me feel as if my lungs are closing up. Sometimes I have the feeling that too much medication lingers in my mouth and throat. With nebuliser therapy, I believe that the active substances safely reach the bronchial tubes.
But I am less strict about the inhalation therapy nowadays. I used to get up at 3 in the morning to do the inhalation if I had to leave for the airport at 5, for example, if I was going on holiday. That’s not something I do nowadays, and I treat myself to an extra hour in bed. But even now, I can count the times I have skipped the inhalation therapy in the last year, on one hand.
* The name has been changed by the editorial team at the request of the interviewee to protect her privacy.
Notes: The statements made in the report are the individual view of the person 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.
© 2024 PARI GmbH Spezialisten für effektive Inhalation