Can you really drop your inhalation therapy altogether if you are on CFTR modulators? A cystic fibrosis patient shares her experience

CFTR modulators, which you take as a tablet, are considered a milestone in the treatment of the hereditary disease cystic fibrosis (CF). They often improve lung function and the general health of those affected. Even though the current recommendation is to continue basic symptomatic therapy, which also includes the inhalation of hypertonic saline solution (e.g. MucoClear 6%) and antibiotics, many patients wonder whether they might be able to skip the daily inhalation.

Anne*, who has cystic fibrosis, and who has had to have inhalation therapy twice a day since birth, tried it out. She shares her experience in an interview.

PARI blog: When did you start taking CFTR modulators?

Anne: I have been taking CFTR modulators since about 2019 because I was lucky enough to take part in a trial.

PARI blog: How was your health, your lungs and your physical performance before taking CFTR modulators?

Anne: My health was not particularly good. I only just made it into the study with my very restricted FEV1 values. I was often tired, I couldn’t really do any sport, and sometimes even going for a walk was a challenge. I often had lung infections and often had to have intravenous antibiotics. I still somehow plodded along with a part-time job, a young child, housework and therapy. But things were going downhill rather than uphill.

PARI blog: What was your inhalation and respiratory therapy like before you started on CFTR modulators?

Anne: I had to do my inhalation therapy twice a day for about an hour using drugs to open up the airways, antibiotics, mucus-thinning inhalation solutions – including hypertonic saline solution – and I had to do breathing exercises with PEP systems and mobilisation exercises. I have been following this programme more or less since my birth in 1977. I would never have even dreamt of skipping my inhalation therapy.

PARI blog: And yet you still dropped the inhalation therapy when you started taking CFTR modulators. How come?

Anne: Yes, that was back in October 2022. Since I have been taking CFTR modulators, my health and lungs have not only stabilised, but have actually improved. Since then, I have hardly coughed anymore – during the day or at night. My FEV1 has improved and fluctuates between 52 and 57 percent. I have almost as much energy as I did before my health did a nosedive back in 2014. I have only needed intravenous antibiotic therapy once since then, and I can even jog again at a slow pace.

Because of this and because a lot of people have said that you can give up the inhalation without any negative consequences for your health if you are on CFTR modulator therapy, I asked my lung specialist about it. He said I could try it and reduce the inhalation therapy to inhalation with antibiotics and a spray that widens the airways (bronchodilator). So I left out inhalation with a nebuliser with bronchodilators, hypertonic saline solution and other mucus-thinning inhalation drugs. I did it for a week and then I gave up on the experiment.

PARI blog: Why did your start your inhalation therapy again?

Anne: I noticed that too much mucus had built up in my lungs and I had to cough more. Coughing is always unpleasant and bad for the lungs. It is also embarrassing for me if I get a coughing fit in public or have problems with breathing.

I also didn’t want to risk more secretions building up in the time between inhalations. The more secretions, the greater the drop in performance and the risk of a lung infection, which would then be difficult and take a long time to shift. I was afraid and had the feeling that reducing inhalation with mucus thinners would put my health and the stable condition I had reached at risk.

Trying it out in October, i.e. autumn and the season for airway infections, was probably not a good idea either. But I still wouldn’t repeat the experiment. I have to inhale hypertonic saline solutions even with the CFTR modulator therapy to keep my lungs sufficiently clear of secretions. My doctor says that I cannot drop the inhalative antibiotic therapy.

PARI blog: What is your inhalation therapy like now?

Anne: As it always was. But one thing that has changed is that I can occasionally skip an inhalation, without having to pay for it the next day. If I get home unexpectedly late at night and am tired, I can now just go to bed. I used to have to force myself to do the inhalation therapy, no matter how late it was.

Or if I have to leave the house very early in the morning, I can now reduce the therapy from an hour to 30 minutes without it having serious consequences for my lungs. I wasn’t able to do that before I started on CFTR modulators. I preferred to get up at 3:30 in the morning so that I could do my hour of inhalation therapy before, for example, catching a flight to go on holiday.

PARI blog: Many thanks for taking the time to talk to us.

* Name changed by the editorial board

More articles on cystic fibrosis in adults

Relationship and parenting with cystic fibrosis

Sport and cystic fibrosis

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.

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