Therapiegrenzen hei Mukoviszidase: Medizinische Kriterien

  • H. Lindemann
Conference paper


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.


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  1. 1.
    App EM (1996) Vergleichende Mukolytikatherapie. Pneumologie 50: 845 – 853PubMedGoogle Scholar
  2. 2.
    Bennett WD, Olivier KN, Zeman KL, Hohnecker KW, Boucher RC, Knowles MR (1996) Effect of uridine 5’-triphospate plus amiloride mocuciliary clearance in adult cystic fibrosis. Am J Crit Care Med 153: 1796 – 1801Google Scholar
  3. 3.
    Cherny NI. Coyle N, Foley KM 1996 Guidelines in the care of the dying patient. Hematol Clin North Am 10:261–286CrossRefGoogle Scholar
  4. 4.
    Davis Pamela B, Drumm M, Konstan MW (1996) Cystic fibrosis. Am J Respir Crit Care Med 154: 1229 – 1256Google Scholar
  5. 5.
    Delaney SJ, Wainright BJ (1996) New pharmaceutical approaches to the treatment of cystic fibrosis. Nature Med 2: 392 – 393PubMedCrossRefGoogle Scholar
  6. 6.
    Fole Kathleen M (1997) Competent care for the dying instead of physician-assisted suicide. N Engl J Med 336: 54 – 58CrossRefGoogle Scholar
  7. 7.
    Gillissen A (1996) Neue Perspektiven der Antioxidanteentherapie mit Thiolen: Nacystelyn. Pneumologie 50: 834 – 837PubMedGoogle Scholar
  8. 8.
    Hartmann F (1996) Gedanken über den Zussammenhang von Hoffnung, Vertrauen, Verantwortung und Scham in den Beziehungen zwischen Kranken und ihren Arsten. Med Klin 91: 660 – 664Google Scholar
  9. 9.
    Herranz G (1994) Euthanasie — Gebote und Verbote der Sterbehilfe. Med Klin 89: 216 – 212Google Scholar
  10. 10.
    Hofmann Th, Senier I, Bittner-Dersch P, Hüls G, Schwandt H-J, Lindemann H (1997) Aerosolized amiloride:Dose effect on nasal bioelectric properties, pharmacokinetics, and effect on sputum expectoration in patients with cystic fibrosis. J. Aeros Med 10: 147 – 158CrossRefGoogle Scholar
  11. 11.
    Howard Marybeth, Frizzell RA, Bbedwell DM (1996) Aminoglycoside antibiotics restore CFTR function by suppressing premature stop mutations. Nature Med 2: 467 – 469PubMedCrossRefGoogle Scholar
  12. 12.
    Knowles MR, Paradiso AM, Boucher RC (1995) In vivo nasal potential difference: Techniques and protool for assessing efficacy of gene transfer in cystic fibrosis. Human Gene Therapy 6: 445 – 455PubMedCrossRefGoogle Scholar
  13. 13.
    Knupp B, Stille W (1996) Sterben und Tod in der Medizin. Wiss Verlagsges StuttgartGoogle Scholar
  14. 14.
    Lindemann H (1996) Amiloridinhalation. Pneumologie 50: 841 – 844Google Scholar
  15. 15.
    Lindemann H (1996) Mukoviszidose/zystische Fibrose. Pneumologie 50: 588 – 591PubMedGoogle Scholar
  16. 17.
    Meran JG (1996) Prinzip der Unantastbarkeit des Lebens. Dt Ärztebl 93: B-2162Google Scholar
  17. 17.
    Paul K (1997) Kriterien zur Vormerkung zur Lungentransplantation. In: Paditz E (ed) Nasale Maskenbeatmung bei Mukoviszedose – Brücke zur Lungentransplantation?’ Springer-Verlag Heidelberg, Berlin, Tokyo, New YorkGoogle Scholar
  18. 18.
    Ramsey BW (1996) Management of pulmonary disease in patients with cystic fibrosis. N Engl J Med 335: 179 – 188PubMedCrossRefGoogle Scholar
  19. 19.
    Rochat TH, Pastore, FD, Schlegel-Haueter SE, Filthuth I. Auckenthaler R, Belli D, Suter S (1996) Aerosolized rhDNase in cystic fibrosis: effect on leucocyte proteases in sputum. Eur Respir J 9: 2200 – 2206PubMedCrossRefGoogle Scholar
  20. 20.
    Senier Ivonne (1997) Der Einfluß nasal inhalierter Kochsalz-und Amiloridlösungen auf die nasale Potentialdifferenz bie Mukoviszidose-Patienten. Med. Dissertation, Universität GießenGoogle Scholar
  21. 21.
    Terbrack HG, Gürtler K-H, Hüls G, Bittner-Dersch P, Klör H-U, Lindemann H, (1996) Human-spezifische fäkale Pankreaselastase bie Kindern. Monatsschr Kinderheilkd 144: 901 – 905CrossRefGoogle Scholar
  22. 22.
    Vogelmeier C, Döring G (1996) Neutrophil proteinases and rhDNase therapy in cystic fibrosis. Eur Respir J 9: 2193 – 2195PubMedCrossRefGoogle Scholar
  23. 23.
    Tümmler B (1996) Molekulare Genetik. 17.CF-Ambulanzärzte-Tagung,Titisee, 1. /2. 11.Google Scholar
  24. 24.
    Veeze HJ, Halley DJJ, Bijman J. de Jongste JC, de Jonge HR, Sinaasappel M (1994) Determinants of mild clinical symptoms in cystic fibrosis patients. Residual chloride secretion measured in rectal biopsies in relation to the genotype. J Clin Invest 93: 461 – 466PubMedCrossRefGoogle Scholar
  25. 25.
    Wagner F, Reichenspurner H, Schüler ST (1997) Lungentransplantation bei CF: Ergebnisse und interdisziplinäre Nachbetreuung. In: Paditz E (ed) Nasale Maskenbeatmung bei Mukoviszidose — Brücke zur Lungentransplantation?’ Springer-Verlag Heidelberg, Berlin, Tokyo, New YorkGoogle Scholar
  26. 26.
    World Health Organization (1989) Cancer pain relief and palliative care. GenevaGoogle Scholar

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© Springer-Verlag Berlin Heidelberg 1997

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  • H. Lindemann

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