Workshop report117th ENMC Workshop: Ventilatory Support in Congenital Neuromuscular Disorders — Congenital Myopathies, Congenital Muscular Dystrophies, Congenital Myotonic Dystrophy and SMA (II) 4–6 April 2003, Naarden, The Netherlands
Section snippets
Session 1—Assessment methodologies in childhood respiratory impairment
The first session addressed the rationale for commonly performed measurements of various aspects of respiratory function in order to generate recommendations for assessment of respiratory function in children with NMD (Appendix A).
Session 2—Treatment of respiratory impairment in congenital neuromuscular diseases
The whole impetus of investigations is to highlight patients who will respond to treatment modalities. This session reviewed the evidence for the range of treatments available, including physiotherapy techniques to enhance respiratory muscle function or cough and techniques of assisted ventilation. The recommendations of the group in this area are summarised in Appendix B.
Session 3—Specific issues relating to the natural history of respiratory involvement in these disorders
The next session of the meeting focused on the disease subgroups under discussion and specific features of their natural history suggesting that recommendations for management should be more specific than those for the group as a whole. The burden of the respiratory complications in these diseases is summarised in Table 1.
Session 4—Discussion and opportunities for further study
The final session of the workshop allowed time to debate various issues of specific importance and milestones in these diseases. For neonates with a need for prolonged ventilatory support, discussion with centres with experience in these disorders is imperative before decisions about continuing or discontinuing treatment are made. In neonates and at all ages, very clear discussion of the endpoint of ventilation will allow appropriate management of the terminal stages of these disorders.
Dr
Acknowledgements
This Workshop was made possible thanks to the financial support of the European Neuromuscular Centre (ENMC) and its main sponsors:
Association Française contre les Myopathies (France)
Deutsche Gesellschaft für Muskelkranke (Germany)
Telethon Foundation (Italy)
Muscular Dystrophy Group of Great Britain and Northern Ireland (UK)
Muskelsvindfonden (Denmark)
Prinses Beatrix Fonds (Netherlands)
Schweizerische Stiftung für die Erforschung der Muskelkrankheiten (Switzerland)
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