Mucoviscidosis (cystic fibrosis)

Mucoviscidosis or cystic fibrosis is a disease based on genetic mutations. A dysfunction in certain glands causes the increased production of viscous mucus in the lungs, among other things.
As a result of this mucus production, the organs are unable to function properly. Productive coughing is hindered as a result, patients suffer from shortness of breath and dry coughing, the susceptibility to respiratory tract infections such as bronchitis or pneumonia is increased. The life expectancy of affected people is still significantly lower than it is for healthy persons. However, with proper and timely treatment, patients can now live significantly longer lives than they could 20 years ago.
The life expectancy of affected persons is significantly lower than it is for healthy persons. However, with proper and timely treatment, patients can now live significantly longer lives than they could 20 years ago.
Mukoviszidose - Schleimbildung in den Bronchien
Cystic fibrosis patients suffer from the formation of stubborn phlegm which affects the passage of mucus in the lungs, among other things. This results in shortness of breath and coughing.

Cause of cystic fibrosis

The cause of cystic fibrosis is a defect in chromosome 7. The affected gene is a protein that acts in the cell membrane as a chloride channel. Because of the gene defect, the protein is altered and the channel function is impaired. To date, more than 1000 different mutations of the CFTR gene are known. Cystic fibrosis only arises when both parents inherit a mutated gene. If both parents are carriers of one mutated and one healthy gene, the probability of a child receiving two intact gene copies is 25%.

The probability that a child with one intact and one mutated copy is healthy, but can pass on the mutation is 50%, while the probability that the child is ill is 25%. If both parents are diseased, all children will inherit the illness.

To date, science is unable to provide a conclusive explanation as to why a defect that engenders such a severe illness, is so prevalent and does not disappear over time. Therefore, a conclusion has been drawn that the cystic fibrosis illness must carry with it an evolutionary advantage.

First, it was suspected that the disease progression of cholera is positively affected thanks to the gene defect. The dissemination of CF does not support this theory, however, as cystic fibrosis often appears in areas in which cholera is seldom diagnosed, and vice versa.

A connection to typhoid was also suspected, but could not be proven. The bacterium depends on the CFTR channels in order to arrive in the intestinal cells.

The most possible explanation is a protection against tuberculosis, which is accompanied by a predisposition to cystic fibrosis. This correlation has been relatively well demonstrated in clinical tests.

The main symptoms of cystic fibrosis are a chronic dry cough, advancing shortness of breath, mucus in the lungs and pancreas, digestive disorders, all the way to delayed growth, intestinal obstructions, infertility, and jaundice.

The symptoms are more or less pronounced depending on the severity of illness.
Women can learn while pregnant whether their child is affected by CF, by undergoing a genetic analysis of the amniotic fluid. Later on, the illness can be diagnosed through sweat tests that point to elevated levels of salt in the sweat, and radiographic images that indicate blocked airways.

There are several options for therapy; recommended among them is respiratory physiotherapy, which loosens mucus and pus from the lungs. Also recommended is the intake of medication via inhalation, which can dilate the lungs and loosen mucus.

The following products can help you in cases of cystic fibrosis:

Atemtherapiegerät RC‑Cornet® PLUS

RC-Cornet® PLUS

A PLUS for added quality of life

The new RC-Cornet® PLUS pep therapy device produces a positive exhalation pressure that loosens up stubborn bronchial phlegm in the event of chronic respiratory diseases, and reduces coughing and shortness of breath.
Atemtherapiegerät RC‑Cornet® PLUS

RC-Cornet® (Base cornet)

Pep therapy for the lower respiratory tract – loosens phlegm, reduces coughing and shortness of breath.
Inhalierhilfe RC-Chamber®


The spacer for inhalation for all ages

The RC-Chamber® spacer for inhalation simplifies the use of metered-dose inhalers and minimises side effects due to drug residue in the throat area.
Atemtherapiegerät RC‑Cornet® PLUS


Effective loosening of bronchial phlegm

On exhalation, the PEP mask ensures that the airways dilate and that stubborn phlegm is loosened.