Wednesday, 4. September 2024

Inhalation therapy with cystic fibrosis despite taking CFTR modulators? Cystic fibrosis patients address the question

Young mother has little son on her lap and helps him inhale — Parents of children with cystic fibrosis and adults with cystic fibrosis talk about whether and how they inhale during therapy with CFTR modulators.

Many people with cystic fibrosis (CF) benefit from treatment with CFTR modulators. Many wonder if this means they can give up their inhalation therapy and respiratory physiotherapy. We put this question to adults with cystic fibrosis and the parents of children with cystic fibrosis. Here are their responses.

Thomas Malenke

My lung function has improved significantly since taking CFTR modulators, and so has my blood sugar. I can breathe more freely and need less insulin. But I have developed high blood pressure. Although my breathing has improved, I am still carrying on with my inhalation and respiratory therapy. I don’t want to risk anything.

» more about Thomas Malenke’s experience with CF and therapy

Carola Landerer, no chronic pseudomonas infestation of the lungs

Half close-up of young smiling woman against a blue background, one hand on her chin — Carola Landerer has cystic fibrosis and only inhales under CFTR modulators when she has an infection.

Nowadays, I only have to do my inhalation therapy if I have an infection – thanks to the CFTR modulator therapy. I still have physiotherapy once a week. I do strength training at the gym two to three times a week. I used to go four times a week. Because the new tablets mean that I can now do more endurance sport, I do that too. Since starting the CFTR modulator therapy, there is no further evidence of pseudomonas, not even in the bronchoscopy.

» more about Carola Landerer’s life before the CFTR modulator therapy

Anne, chronic pseudomonas infestation of the lungs

Even with the CFTR modulator therapy, I am still carrying on consistently with my inhalation and respiratory therapy as before. So that involves inhalation therapy every morning and evening with bronchodilators and hypertonic saline solution, other mucolytics and antibiotics. Each of these takes an hour.

But one thing that has changed is that I can occasionally skip an inhalation therapy without having to pay a heavy price 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.

» more on Anne’s experience with CFTR modulators

Markus Hänni, reduced pseudomonas infestation of the lungs

Half close-up of a smiling man against a purple background — Thanks to CFTR modulators, the treatment time for Markus Hänni, who has cystic fibrosis, has been halved.

Since the CFTR modulator therapy, my therapy times have halved from three hours a day down to one and a half hours. Inhalation therapy with antibiotics is no longer part of my long-term therapy, as the pseudomonas infestation in my lungs has considerably reduced and the CRP value that indicates inflammation in my body, tends to be in a normal range.

Inhalative antibiotics are nowadays an option and are always close at hand in case I develop an infection, which is a tremendous relief. Knowing there is now this interim step before I have to resort to intravenous antibiotics is really reassuring. Not having this thick secretion also makes the therapy much less of a strain.

» more about Markus Hänni’s experience of fatherhood and CF
» more about Markus Hänni and relationships when you are living with cystic fibrosis

You may also be interested in

Interview with researcher and patient Dr Zißler: Personalised cystic fibrosis therapy in the age of CFTR modulators
First-hand report: Chronic pseudomonas infestation of the lungs in a patient with cystic fibrosis
What can you use at what age: Inhalation and respiratory therapy – assistive devices for cystic fibrosis
3 tips for patients with lung disease: Save time with breathing therapy

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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