General measures
- Inhalation of hypertonic saline solutions is a useful and effective measure
- To maintain stable lung function, respiratory training with the flutter valve should be performed several times a day.
- Observance of the general hygiene measures!
- Nicotine restriction (refrain from tobacco use).
- Aim for normal weight!Determination of BMI (body mass index, body mass index) or body composition using electrical impedance analysis.
- Falling below the BMI lower limit (from the age of 19: 19; from the age of 25: 20; from the age of 35: 21; from the age of 45: 22; from the age of 55: 23; from the age of 65: 24) → Participation in a medically supervised program for the underweight.
- Avoidance of environmental stress:
- Carry out residential renovation to avoid harmful influences such as dust, moisture or other allergens.
- Travel recommendations:
- Participation in a travel medical consultation required!
- Air travel only with an additional oxygen supply
Conventional non-surgical therapy methods
If pulmonary function deteriorates, oxygen administration and noninvasive ventilation may be required.
Vaccinations
The following vaccinations are advised, as infection can often lead to worsening of the presenting disease:
- Influenza vaccination (starting from the 6th month of life).
- Pneumococcal vaccination
Regular checkups
- Regular medical check-ups (at least every 3 months):
- Length and weight
- Microbiological examinations (throat swab/possible sputum).
- Pulmonary function (at least from 6 years of age).
- Control examinations (at least once a year)
- Abdominal sonography (ultrasound of the abdominal organs).
- Inflammatory parameters (e.g., CRP)
- Transaminases
- Oral glucose tolerance test (oGTT; from 10 years of age).
- Serum levels of fat-soluble vitamins
- Chest X-ray (chest; regular; interval controversial).
Nutritional medicine
- Nutritional counseling based on nutritional analysis
- Observance of the following specific nutritional recommendations:
- To be able to meet the increased energy requirements in cystic fibrosis – for example, due to the increased work of breathing as well as the severe chronic inflammation – attention should be paid to an adequate high-energy diet. Nutrient intake recommendations:
- 35-40% of energy in the form of fat with a high content of polyunsaturated fatty acids (omega-3 and 6 fatty acids).
- Fat content of each meal adapted pancreatic enzyme substitution due to exocrine pancreatic insufficiency (disease of the pancreas associated with insufficient production of digestive enzymes).
- 40% of energy in the form of carbohydrates – possibly additional intake of maltodextrin (glucose mixture).
- 20-25% of the energy in the form of protein
- 35-40% of energy in the form of fat with a high content of polyunsaturated fatty acids (omega-3 and 6 fatty acids).
- If necessary, offer additional food; if underweight despite optimal nutritional therapy → installation of a percutaneous endoscopic gastrostomy tube (PEG tube; endoscopically created artificial access from the outside through the abdominal wall into the stomach) for weight gain.
- A total of 5 servings of fresh vegetables and fruits daily (≥ 400 g; 3 servings of vegetables and 2 servings of fruits)
- High-fiber diet (whole grain products).
- Use multivitamin preparations (micronutrient preparations; vital substance preparations): Supplementation of fat-soluble vitamins A, D, E, K and water-soluble vitamin B12.
- In case of insufficient nutrition plant of a feeding tube
- To be able to meet the increased energy requirements in cystic fibrosis – for example, due to the increased work of breathing as well as the severe chronic inflammation – attention should be paid to an adequate high-energy diet. Nutrient intake recommendations:
- Selection of appropriate food based on the nutritional analysis
- See also under “Therapy with micronutrients (vital substances)” – if necessary, taking a suitable dietary supplement.
- Detailed information on nutritional medicine you will receive from us.
Sports Medicine
- Endurance training (cardio training).
- Preparation of a fitness or training plan with suitable sports disciplines based on a medical check (health check or athlete check).
- Supervised strength and endurance training (three times 30-60 minutes each week for six months) resulted in significant improvement in lung function (measured in FVC/forced expiratory vital capacity and FEV1/expiratory one second capacity or forced expiratory volume)
- For detailed information on sports medicine, please contact us.
Physical therapy (including physiotherapy)
The following supportive (assisting) measures should be performed several times a day to maintain stable lung function:
- Inhalation therapy with hypertonic saline (= secretolytic treatment):
- Checklists and videos on inhalation technique s. u. of the website of the German Respiratory League: www.atemwegsliga.de/richtig-inhalieren.htm
- Baby with cystic fibrosis benefit from early onset 2 times daily inhalation therapy with hypertonic saline (improvement in lung function measured by Lung Clearance Index (LCI); better weight gain after one year: on average 500 grams heavier and 1.5 centimeters taller)
- Tapping massage of the thorax (chest).
- Physiotherapy (optimally combined with sports): used Pezzi ball, ergometer (bicycle ergometer, treadmill) and respiratory therapy equipment (flutter; PEP system). This serves:
- The elimination of viscous secretions through special breathing techniques (autogenous drainage; self-help technique).
- Improvement of thoracic mobility
- Reduction of hyperinflation (emphysema).
