Physical Medicine Interventions for Duchenne Muscular Dystrophy, the Including Avoiding Invasive Airway Tubes

22 V. KEY POINTS 1. No one needs a tracheotomy tube for only being too weak to breathe. 2. “NIV (CPAP and low span bi-level PAP),” rather than “NVS,” is inadequate for DMD. 3. Patients are left to go into acute respiratory failure and undergo tracheotomies although it was known that this could be avoided by CNVS and MIE since 1953. People with DMD continue to be extubated to oxygen and NIV rather than NVS and MIE, and when failing extubation as a result, and ventilator unweanable, they are re-intubated and trached despite publications indicating that they can be extubated to CNVS and MIE, known since 1996. NMD specialists need knowledge of CNVS and MIE. 4. Since routine polysomnograms do not distinguish central and obstructive events from inspiratory muscle weakness, people with weak muscles are often treated inappropriately with NIV instead of NVS. 5. After more than one hundred thousand hours of CNVS and MIE use by peo- ple with NMD, without barotrauma, critical care physicians still use permissive hypercapnia, that is, intentionally underventilate the lungs of people, and even when extubating to CNVS, are afraid to discharge CNVS users home. 6. Sophisticated ventilators are unnecessary for self-directed CNVS users for whom the only important alarm is oximetry. 7. Decannulation to CNVS facilitates weaning and eating. Airway tubes hinder or prevent weaning and usually necessitate stomach tubes that would otherwise be unnecessary. 8. Since 1957 over 2000 patients with NMD have been using portable ventilators with active ventilator circuits for CNVS, volume preset at 700 to 1400 ml or pressure control at 18 to 25 cm H2O, without O2, EPAP, PEEP, bi-level, or polysomnographies. Most begin NVS at 650 to 700 ml but use 1100-1300 ml after a few months. Some have continued CNVS for over 70 years. Therefore, EPAP, PEEP, bi-level PAP, and O2 therapy, are unnecessary for patients with ventilatory pump failure. Bilevel can be used at NVS settings but is still suboptimal. 9. “Pulmonary function testing,” polysomnographies, intubations, bronchos- copies, tracheotomies, often gastrostomies, and ongoing nursing care for invasive airway tubes can be avoided for DMD patients. 10. Few lessons are when the effort to learn them is not buoyed by financial gain. 11. EPAP increases the risk of aspiration, reflux, cardiac arrhythmias, disrupts sleep, and is counterproductive for NVS users.