PURPOSE:Duchenne patients eventually require 24 hour ventilatory support. Many centers recommend tracheostomy rather than noninvasive ventilation (NIV). We report the outcome of 19/23 adult DMD patients on nocturnal NIV plus daytime mouthpiece ventilation (MPV).
METHODS:16/23 patients used nocturnal bilevel NIV, avg. pressure 17/6 cmH2O. 3/23 used volume-targeted ventilators, Vtavg 900 ml, RRavg 13/min. Outpatient-initiated 24 hour NIV patients (12/23), used MPV, a chair-mounted ventilator and mouthpiece. Oximetry, downloads and clinical assessment assured adequate ventilation. All patients used lung volume recruitment and 16/23 had CoughAssistTM devices.
RESULTS:23 DMD patients were followed at the Ottawa Hospital Rehabilitation Centre. Nocturnal NIV was used in 19/23 patients. Average values at initiation of nocturnal NIV were; FVC 1.08L (25%pred.), PaCO2 52mmHg, age 17.5 years. Duration on nocturnal NIV was 1 to 11.3 years with one death after 2 years on NIV. 12 patients subsequently required 24 hour support, indications; dyspnea, tachypnea and hypercapnea despite adequate nocturnal NIV. Average values at initiation of 24 hour support were; FVC 0.57L (13.2%pred.), PaCO2 53mmHg, age 19.3 years. PaCO2 was reduced to 48mmHg with Nocturnal NIV and to 43mmHg with 24 hour NIV. Duration on 24 hour NIV/MPV is up to 12 years with two deaths to date (after 3.75 and 4 years on NIV). No patient required a permanent tracheostomy. In 81 patient-years on NIV there have been only 4 respiratory-related hospitalizations.
CONCLUSION:To date all of our patients with DMD have been managed long beyond nocturnal NIV with MPV. No patient required a tracheostomy for respiratory failure.
CLINICAL IMPLICATIONS:MPV should be considered as an alternative to tracheostomy for 24 hour support in DMD.
DISCLOSURE:Douglas McKim, No Financial Disclosure Information; No Product/Research Disclosure Information