Wednesday, 29. June 2022
Cystic fibrosis, or CF for short, is a hereditary metabolic disorder. The first symptoms start early on, in infancy and childhood. Many children affected suffer from frequent bacterial respiratory infections, brought on by thick mucus. As it is difficult to cough up, breaking down the mucus with inhalation is an important part of therapy in patients with cystic fibrosis. We have compiled the FAQs and answers to all your questions about inhalation for cystic fibrosis.
Mucus that is stubbornly lodged in the bronchial tubes and that is also an ideal breeding ground for bacteria is one of the main problems patients with cystic fibrosis face. Inhaling saline solution moistens the mucous membranes and also liquifies the secretions, which make them easier to cough up. Antibiotic inhalations can also fight off bacteria.
Essentially, treatments of the respiratory passages in patients with cystic fibrosis focus on the following: breaking down the stubborn mucus to make it easier to cough up, and fighting inflammation and bacterial infections. Inhalation therapy has an important role to play here.
Inhalation with a nebuliser is a tried and tested method to treat respiratory problems and diseases. The therapy uses “aerosols”, tiny particles of air, water, saline or medication.
Often, respiratory physiotherapy is recommended alongside inhalation therapy. Both of these therapies in tandem can make it easier to cough up mucus.
People who have cystic fibrosis inhale using special, high performance inhalation devices and systems. These inhalation devices with nebulisers generate very small aerosols. Not only do they nebulise saline solutions – certain nebulisers have also been approved for the inhalation of medications especially developed for cystic fibrosis.
An inhalation system that offers short inhalation times and silent operation improves quality of life: That leaves you with more time for all those things that have nothing to do with the illness or its treatment. Inhalation devices for the treatment of cystic fibrosis also have to be light, small and mobile. They can be used anywhere – not just in your own four walls. That makes you more independent. They should also be easy to clean and disinfect. This saves time and improves hygiene.
The eFlow®rapid inhalation system with a vibrating membrane nebuliser, for instance, is an extremely efficient device for the treatment of respiratory diseases such as cystic fibrosis. During the development, special care was taken to ensure that the device could offer efficient, safe and fast inhalation therapy.
Cystic fibrosis treatment firstly involves inhaling saline solutions. These ensure that the thick mucus in the airways is liquified and is easier to cough up.
People with cystic fibrosis inhale hypertonic saline solutions (MucoClear 3% or MucoClear 6%). They have a higher salt content and so break down the mucus very effectively. Secondly, the devices are also used to inhale drugs designed to dilate the airways or to fight bacteria.
Yes, as already described above, people with cystic fibrosis can and should inhale medication because this route delivers the active ingredient directly and quickly where it is needed: in the lungs or the bronchial system. This reduces potential side effects, and less medication is needed for the desired effect – compared to taking a tablet.
Patients with cystic fibrosis inhale medication that dilates the bronchial tubes or antibiotics that fight off airway infections. People with cystic fibrosis are very prone to infections. Bacterial infections tend to cause permanent damage to the lung tissue, which it why it is really important to avoid them. For this reason, the treatment is targeted at fighting infectious pathogens. Usually antibiotics are inhaled in long-term treatment regimens.
NB: The attending doctor will decide which medications the patient should take, and will prescribe these accordingly.
People with cystic fibrosis generally inhale several times a day to moisten the lungs and to dislodge the thick mucus, taking between two and three hours a day. This makes efficient inhalation systems all the more important, to save as much time as possible.
Taking this triple combination can reduce the development of the thick mucus in patients with cystic fibrosis. It is not yet clear which patients are then able to drop the saline inhalation therapy. This is currently being investigated in studies. What is important is that the pivotal studies for Kaftrio/Trikafta were conducted with the basic therapy in place (inhalation), and so almost all doctors currently recommend sticking to this.
Note: The information in this blog post is not a treatment recommendation. The needs of patients vary greatly from person to person. The treatment approaches presented should be viewed only as examples. PARI recommends that patients always consult with their physician or physiotherapist first.
An article written by the PARI BLOG editorial team.
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