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P60 A REGIONAL SURVEY OF PAEDIATRIC CONSULTANTS’ PRACTICES AND ATTITUDES IN THE MANAGEMENT OF SPINAL MUSCULAR ATROPHY TYPE 1
1J. L. Heraghty, 2T. N. Hilliard, 2A. Majumdar, 1P. Jardine, 1P. J. Fleming, 1A. J. Henderson. 1University of Bristol, Bristol, UK, 2Bristol Royal Hospital for Children, Bristol, UK
The management options for spinal muscular atrophy (SMA) are changing with an increase in the use of non-invasive and invasive ventilation. However, there are few empirical data to support the practice of long-term ventilation in improving quality of life in these patients. Without active respiratory management, children with SMA usually die within the first 2 years of life. We designed a survey to assess current attitudes and practices in the management of this condition in a single geographical region of the UK.
Methods In November 2008 a web-based anonymous survey was sent to all paediatric consultants within the region who would have potential contact with a child with SMA. Following a brief clinical scenario of an infant with SMA type 1, a number of management options were suggested regarding general health care, antibiotics for infection, feeding options, immunisations, ventilation for acute illness and long-term home ventilation. For each option, respondents were asked if they would (a) not discuss, (b) discuss but not recommend or (c) recommend the intervention.
Results 72% (133/185) of consultants completed the survey. They were representative of the surveyed population in terms of place of work and specialty practice. 83% of respondents would recommend nasogastric feeding, 79% oral antibiotics and 39% intravenous antibiotics during infections. 73% would recommend influenza and pneumococcal vaccination and 44% would recommend RSV prophylaxis. Non-invasive ventilation (NIV) would be recommended for the acute management of a respiratory infection by 52% but only 14% would recommend intubation and ventilation, although 82% said they would discuss this with the family. A high proportion of respondents would discuss long-term ventilation …