introduction:"CFTR modulators represent a significant advancement in CF treatment since they are small molecules improving the function of the defective CFTR protein in a mutation-specific way, which helps restore chloride ion transport across cell membranes. Notable pharmaceutical agents include Trikafta®, Symdeko®, Orkambi® and Kalydeco® [1]. These medications have been demonstrated to significantly improve lung function and reduce pulmonary exacerbations. However, they are expensive and may cause side effects such as liver enzyme elevations and cataracts in pediatric patients [2]. Furthermore, they are not suitable for all CF patients since only mutations which produce a CFTR channel can be supported by CFTR modulators, not those mutations which lead to a missing CFTR channel (knock out) [1], e.g. stop-mutations including p.Arg553Ter or p.Gly542Ter [3]. ",
introduction:"CFTR modulators represent a significant advancement in CF treatment since they are small molecules improving the function of the defective CFTR protein in a mutation-specific way, which helps restore chloride ion transport across cell membranes. Notable pharmaceutical agents include Trikafta®, Symdeko®, Orkambi® and Kalydeco® [1]. These medications have been demonstrated to significantly improve lung function and reduce pulmonary exacerbations. However, they are expensive and may cause side effects such as liver enzyme elevations and cataracts in pediatric patients [2]. Furthermore, they are not suitable for all CF patients since only mutations which produce a CFTR channel can be supported by CFTR modulators, not those mutations which lead to a missing CFTR channel (knock out) [1], e.g. stop-mutations including p.Arg553Ter or p.Gly542Ter [3]. ",
introduction:"Mucolytics help thin and loosen the mucus in the lungs, making it easier to cough up and clear the airways. These therapies are typically administered via wet or dry inhalation, providing direct delivery to the lungs. In the case of wet inhalation, the medication is inhaled as an aqueous solution and nebulized, while in the case of dry inhalation, the medication is inhaled as a powder. [1] Key Therapies include mannitol, Pulmozyme® and hypertonic saline.",
introduction:"Mucolytics help thin and loosen the mucus in the lungs, making it easier to cough up and clear the airways. These therapies are typically administered via wet or dry inhalation, providing direct delivery to the lungs. In the case of wet inhalation, the medication is inhaled as an aqueous solution and nebulized, while in the case of dry inhalation, the medication is inhaled as a powder. [1] Key Therapies include mannitol, Pulmozyme® and hypertonic saline.",
introduction:"Antibiotics are crucial for treating bacterial infections in CF patients since the mucus serves as an optimal environment for pathogens. A huge variety of antibiotics can be inhaled, oral, or intravenous, depending on the severity and kind of the infection [12]. Commercially available medications include TOBI® and CAYSTON®. A major problem associated with the application of antibiotics is the fact that long-term use can lead to antibiotic resistance and potential side effects like kidney damage and hearing loss [12].",
introduction:"Antibiotics are crucial for treating bacterial infections in CF patients since the mucus serves as an optimal environment for pathogens. A huge variety of antibiotics can be inhaled, oral, or intravenous, depending on the severity and kind of the infection [12]. Commercially available medications include TOBI® and CAYSTON®. A major problem associated with the application of antibiotics is the fact that long-term use can lead to antibiotic resistance and potential side effects like kidney damage and hearing loss [12].",
introduction:"The digestive process is impaired in 80% of patients with cystic fibrosis (CF), as a result of pancreatic insufficiency, which in turn leads to difficulties in digesting food and absorbing nutrients. Enzyme supplements like Creon® are therefore essential [16]. Moreover CF patients are also advised to eat a balanced and energy-rich diet, as the increased work of breathing and increased coughing, as well as infections, fever and diarrhoea, consume more energy than a healthy person. [1] It is also an option for patients to use nutritional supplements. Electrolyte preparations are also used in this context to compensate for the increased need for fluids and the required salts.",
introduction:"The digestive process is impaired in 80% of patients with cystic fibrosis (CF), as a result of pancreatic insufficiency, which in turn leads to difficulties in digesting food and absorbing nutrients. Enzyme supplements like Creon® are therefore essential [16]. Moreover CF patients are also advised to eat a balanced and energy-rich diet, as the increased work of breathing and increased coughing, as well as infections, fever and diarrhoea, consume more energy than a healthy person. [1] It is also an option for patients to use nutritional supplements. Electrolyte preparations are also used in this context to compensate for the increased need for fluids and the required salts.",
