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

11 III. STAGE 2: WHEELCHAIR USE Figure 3. Over 140° of scoliosis because scoliosis surgery was avoided due to fear of respiratory complica- tions but he is now 36 years old, can not sit, and has chronic back and leg pain. Prevention of Severe Spinal Deformity and Preparation for Surgery Weak paraspinal muscles cause the spinal column to collapse, cause chest deformity, scoliosis, and kyphosis. Spinal deformities progress rapidly once muscle weakness prevents a child from walking and progressing throughout life. Left untreated or if only bracing is used, the spine can collapse to curves of over 140° (Figure 3). Although its prevention usually yields no benefits on lung function and may even decrease VC by fixing the rib cage, severe scoliosis can impinge on the heart, cause intolerable back, buttock, and leg pain, necessitate expensive custom seating, and can render ineffective, otherwise practical breathing aids like the ventilation belt (intermittent abdominal pressure ventilator [IAPV]). Some physicians do not consider surgical options because they mistakenly think that the child has little hope of long survival and has a high risk of respiratory and other complications by failing extubation. Surgeons often use interim bracing while applying a 40° criterion for surgically correcting scoliosis. For children with DMD, by the time the curve reaches 40°, the VC, or deepest breath one can take, may be below 23% 13 and most surgeons would be reluctant to operate without prophylactic tracheotomy. This is usually refused so 75% of scoliotic DMD patients never undergo spinal fixation surgery. 13,14 However, even older patients with no strength to breathe can avoid breathing complications by clinicians with knowledge of NVS and mechanical insufflation-exsufflation (MIE). Patients with supine VCs less than 1200 ml are taught NVS and MIE before surgery. Our surgeons have operated on children with 3% of normal VC, 80 ml, without a single pneumonia or need to ever resort to tracheotomy because of NVS and MIE, 15 using the oximetry feedback presented in this monograph. Even patients with no measurable VC can be extubated as soon as fully alert, without resort to tracheotomies, to CNVS and MIE with oximetry feedback. Following surgical intervention the patient is usually discharged within 4 or 5 days. Scoliosis bracing is not warranted for DMD. 15