Physical Medicine Interventions for Duchenne Muscular Dystrophy, the Including Avoiding Invasive Airway Tubes
12 Following the loss of ability to walk, the patient reaches a lifetime maximum (plateau) VC followed by a 5 to 7% decrease in VC per year. Below 40% of normal VC cough flows tend to decrease below 270 L/minute and risk of colds causing pneumonia increases. During Stage 2, patients typically need MIE in their homes to prevent colds from developing into pneumonia with shortness of breath causing a visit to an emergency room (ER). With any respiratory symptoms, ER doctors give oxygen that causes blood carbon dioxide (CO2) to soar, the patient to lose consciousness, then stop breathing, get resuscitated, then intubated. Once intubated, weaning from ventilator use often becomes impossible because of the tube itself then the ultimatum of tracheostomy for invasive ventilation (CTMV) for life, almost always along with stomach tube placement since the tube not only makes it far more difficult to breathe but it transpierces the swallowing muscles in the neck to also make it more difficult, if not impossible, to eat. However, with the use of up to continuous NVS (CNVS) and optimal MIE, tracheostomy tubes are only needed for patients who, besides needing continuous ventilatory support, also have severe lung disease, i.e. only 3 out of 486 of our DMD patients in our recent publication. 16,29 During Stage 2, ventilatory support is only needed along with MIE during severe colds. These methods are vital to keep the lungs healthy, grow, and cough effectively. They aid respiratory muscles to maintain chest and lung elasticity and to increase cough peak flows (CPF). The VC plateau is the starting point for prescribing lung volume recruitment (LVR) and assisted coughing. Patients are Evaluated for the Use of Physical Medicine Respiratory Muscle Aids Inspiratory and expiratory muscle aids are devices and techniques that involve the manual or mechanical application of pressure to the body or airways to facilitate inspiratory and expiratory (coughing) muscle function. Body ventilators create pressure changes around the chest and abdomen to force air into the lungs but they cause obstructive sleep apneas and are rarely used today. Negative pressure applied to the airways during expiration increases cough flows (CPF), just as positive pressure applied to the airways during inhalation increases breath volume for NVS. Continuous positive airway pressure (CPAP) is not useful for patients with muscle weakness. Evaluation The key parameters of evaluation are blood CO2 assessed by end-tidal (capnograph) monitor that samples air exhaled out the nose or transcutaneous CO2 monitor sampling the CO2 via skin electrode, pulse oximetry measuring oxyhemoglobin saturation (O2sat) for which normal is 95% and greater, CPF which when below 300 L/min there is increased risk of pneumonia but assisted CPF should also be
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