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Management

Management. Airway clearance Ineffective airway clearance can accelerate the onset of respiratory failure [Bach et al 1997] Peak cough flows < 160 L/min → ineffective airway clearance [Bach and Saporito 1996] DMD: Peak cough flow < 270 L/min or MEP < 60 cmH 2 O [ATS consensus 2004]

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Management

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  1. Management • Airway clearance • Ineffective airway clearance can accelerate the onset of respiratory failure [Bach et al 1997] • Peak cough flows < 160 L/min → ineffective airway clearance [Bach and Saporito 1996] • DMD: Peak cough flow < 270 L/min or MEP < 60 cmH2O [ATS consensus 2004] • 3 types • Manual techniques – glossopharyngeal breathing, air stacking [Kang and Bach 2000] • Mechanical techniques – Insufflator-exsufflators [Bach 1994] • Mucus mobilization devices – High frequency chest wall oscillator, intrapulmonary percussive ventilation

  2. Peak cough flow in NMD children C C NMD NMD a,c = unassisted cough b,d = insufflation/exsufflation cough Chatwin et al 2003

  3. Chatwin et al 2003 a=ped NMD b=ped control c=adult NMD d=adult control UAC = unassisted cough PAC = physiotherapy assisted cough EAC = exsufflation assisted cough IEAC = insufflation-exsufflation assisted cough

  4. Patient with SMA using the MI-E via a mouthpiece Miske, L. J. et al. Chest 2004;125:1406-1412

  5. Left, A: chest radiograph of 22-month-old girl with SMA type I and right upper lobe density Miske, L. J. et al. Chest 2004;125:1406-1412

  6. Management • Supplemental oxygen • REM-related hypoxia • May prolong duration of apnea and hypopnea [Smith et al 1989] • Mechanical ventilatory support • Negative pressure ventilation (NPV) • Plexiglass lung, Cuirass shell, Pulmowrap • Collapse of upper airway [Levy et al 1989, Hill et al 1992]

  7. Chest cuirass

  8. Management • Positive pressure ventilation • Via tracheotomy • NIPPV: nasal mask ventilation • Normalize blood gas and alleviate symptoms of hypercapnia [Heckmatt et al 1990, Hill et al 1992] • Stabilize declining lung function and prolong life expectancy [Vianello et al 1994] • Preventive nasal ventilation

  9. Management • NIPPV indication • Daytime hypercapnia (PCO2>50 mmHg) [ATS consensus 2004, European Consortium 1996] • Nocturnal hypoventilation (PCO2>50 mmHg and/or hypoxemia (<92%) • Timing of initiation of NIPPV remains controversial • One multi-center study found no evidence of lung function preservation in NIPPV patients [Raphael et al 1994] • Those with nocturnal hypoventilation are likely to develop daytime hypercapnia within 2 years [Ward et al 2005]

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