SESSION TITLE: Pediatric Medicine Posters
SESSION TYPE: Original Investigation Poster
PRESENTED ON: Wednesday, October 29, 2014 at 01:30 PM - 02:30 PM
PURPOSE: Clinical guidelines for males with DMD recommend serial pulmonary function testing to guide use of noninvasive respiratory devices, decrease morbidity, prevent or delay tracheostomy, and prolong life with quality. The impact of these guidelines has not been studied.
METHODS: The Muscular Dystrophy Surveillance, Tracking, and Research Network (MD STARnet) is a population-based surveillance system that identifies all males, born on or after 1/1/1982, who have DMD in Arizona, Colorado, Georgia, Iowa and Western New York. We analyzed MD STARnet respiratory care data in 27 affected males who received tracheostomies and asked: (1) whether tracheostomy was performed during an acute respiratory illness; (2) which tests of respiratory muscle strength, including peak cough flow (PCF), forced vital capacity (FVC), sleep hypoventilation testing, and awake hypoventilation testing (end-tidal or blood gas carbon dioxide) were performed in the year before tracheostomy; and (3) whether males had home mechanical insufflation/exsufflation (MI/E) and/or noninvasive ventilation (NIV; bilevel positive airway pressure or mechanical ventilators with nasal mask or sip interface) before tracheostomy.
RESULTS: Between 1/1/2006 and 12/31/2011, there were 456 males between the ages of 14 - 27 years old with DMD. Of those, 27 had tracheostomies performed. Eight, 9, and 10 tracheostomies were performed in 2006-2007, 2008-2009, and 2010-2011, respectively. In each of these 2-year periods: 50% or more of tracheostomies were performed during acute respiratory illnesses; and in the year before tracheostomy: 60% or less had a FVC measurement, 10% or less had PCF testing or awake hypoventilation testing, and 30% or less had sleep hypoventilation testing. In 2006-2007 and 2008-2009, 50% or less had home MI/E or NIV before tracheostomy. In 2010-2011, 60% had MI/E and 100% had NIV before tracheostomy.
CONCLUSIONS: At least half of tracheostomies were performed with acute respiratory illnesses. In the year before tracheostomy, evaluation of respiratory muscle strength and awake hypoventilation is limited. Though home NIV is possibly increasing, males with DMD often do not have MI/E before tracheostomy.
CLINICAL IMPLICATIONS: Wider use of current guidelines might improve respiratory care and prevent or delay tracheostomy in males with DMD.
DISCLOSURE: David Birnkrant: Consultant fee, speaker bureau, advisory committee, etc.: Paid Consultant to Hill-Rom Corporation The following authors have nothing to disclose: Daniel Sheehan, Christina Westfield, Anil Kumar, Elinora Price, Jennifer Andrews, Deborah Fox, Christopher Cunniff
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