General measures
- In respiratory insufficiency (disruption of pulmonary gas exchange), long-term oxygen therapy is given (see below).
- If necessary, intensive medical measures in case of increasing respiratory distress.
- Review of permanent medication due topossible effect on the existing disease.
- Travel recommendations:
- Participation in a travel medical consultation required!
- Air travel only with an additional oxygen supply
Conventional non-surgical therapy methods
In patients with chronic hypoxia/oxygen deficiency (chronic hypoxemia at rest: arterial partial pressure of oxygen (pO2) < 55 mmHg), long-term oxygen therapy (LTOT; 16-24 h/d) is indicated. Enough oxygen should be given to raise the pO2 to about 60-70 mmHg.
Humidifiers can be used at flow rates of 2 liters/minute and above.The minimum duration of use for long-term oxygen therapy should be 15 hours daily.
Patients on LTOT should be followed up regularly.
Surgical therapy
- Lung Transplantation (LUTX).
Training
- Exercise training-based pulmonary rehabilitation programs.