Therapiegrenzen hei Mukoviszidase: Medizinische Kriterien

Summary

As it is not yet possible tocure cystic fibrosis (CF), the most urgent aim is toarrest the progress of the disease as far as possible by an optimal treatment. But there are limits which are set by a late diagnosis (due to unsatisfactory diagnostic tools or limited experience of many physicians with respect to CF), by age-dependentshortcomings (e. g., physiotherapy and inhalation therapy in early childhood), by an insufficient efficacy of drugs (e. g., mucolytics), by unsatisfactory knowledge about appropriate antiinflammatory treatment, by inadequate preventive measures, and by emotional difficulties in receiving a regular, time-consuming therapy which impairs quality of life or to bear special therapeutic measures such as O₂ insufflation, noninvasive intermittant ventilation, or PEG. Acute complications, development of germs which are resistant to all available antibiotics, and — most frequently — the progress of the bronchopulmonary manifestation of CF lead to the final stage of the disease. If this is not combined with a rapid elevation of arterial pCO₂, the patient will suffer from tachydyspnea or even orthopne, sleeplessness in spite of deep exhaustion, pain, and fear of death. He expectssupport and relief. In this extraordinary situation the physician is influenced by ethical, religious and legal aspects. He will also be influenced by the parents of the patient, the nurses, colleagues, and the nonmedical staff. However, mainly, the physician is responsible for the suffering patient and for himself: He must be aware of the fact that he has to live with his decisions lifelong. Under these circumstances it may be helpful to know that it is legal to discontinue the maximal therapy (i. e., intubation and artificial ventilation) if it serves the patient and to use all drugs which are necessary to lessen the suffering in the final painful stage of the disease, even if — to a small extent — life is shortened.