The Aug. 15, 2004, issue of the American Journal of Respiratory and Critical Care Medicine, published by the American Thoracic Society (ATS), contains a set of guidelines about respiratory care in Duchenne muscular dystrophy (DMD) prepared by a special ATS working group. Members of this group are experts in DMD respiratory care, generally managing the respiratory care of patients being seen at Muscular Dystrophy Association (MDA) clinics.
The development of these guidelines was supported in part by MDA, through a grant to Jonathan Finder in the Department of Pulmonology at Children’s Hospital of Pittsburgh.
Below is a summary of the statement’s recommendations to physicians caring for patients with Duchenne muscular dystrophy (DMD) and their families. It says that such physicians should provide:
- Baseline respiratory status evaluation early in the disease course (between ages 4 and 6).
- Regular consultations with a physician specializing in pediatric respiratory care twice a year after starting wheelchair use, reaching a vital capacity (maximal amount of air that can be exhaled after a maximal inhalation) that’s below 80 percent of predicted (normal), or reaching age 12.
- Consultations every three to six months after starting mechanically assisted ventilation or airway clearance device.
- Tests to evaluate pulmonary function at each clinic visit.
- Education about assisted ventilation options well before an emergency occurs.
- Nutritional guidance and support, including the placement of a feeding (gastrostomy) tube when indicated.
- Regular evaluations of sleep quality and sleep-disordered breathing.
- Regular cardiac evaluations, including annual electrocardiograms and echocardiograms, starting at least by school age.
- Regular evaluations of the ability to clear secretions (cough).
- Manually assisted cough techniques or mechanical cough assistance with an insufflator-exsufflator (positive and negative pressure) device when secretion clearance becomes less than adequate.
- Education in the use of pulse oximetry (measurement of the amount of oxygen in the blood through the skin, via a painless sensor) at home to monitor the effectiveness of airway clearance.
- Noninvasive ventilatory support via nasal intermittent positive pressure ventilation, either with a bilevel (using different pressures for inhalation and exhalation) airway pressure device, or with a mechanical ventilator, when disrupted or inadequate breathing during sleep or low blood oxygen levels during sleep are detected.
- Avoidance of supplemental oxygen to treat sleep-related hypoventilation (inadequate breathing) unless ventilatory assistance is also being used.
- Noninvasive daytime ventilation when breathing becomes inadequate during the day, using intermittent positive pressure ventilation through a mouthpiece, or an inflatable bladder that provides intermittent abdominal pressure simulating breathing.
- Education in glossopharyngeal breathing (a “gulping” type of breathing) to use during short periods when off mechanical ventilation.
- The option of ventilation via tracheostomy (surgical opening into the trachea in the neck) if noninvasive ventilation isn’t feasible or isn’t desired, with appropriate education for the patient and family.
- Avoidance of preventive (before required) mechanically assisted ventilation, unless and until it is proven useful, since it may lead to a false sense of security and inadequate respiratory function monitoring.
- Evaluation of pulmonary and cardiac function and of breathing during sleep before scoliosis surgery, and airway clearance and respiratory support in the postoperative period.
- The option of oral steroid therapy with prednisone or deflazacort as a possible (though not proven) means to preserve lung function.
- Education about respiratory function and treatment, including end-of-life care options, for the patient and family.
- End-of-life care that includes treatment of pain or difficulty breathing, while attending to the psychosocial and spiritual needs of the patient and family and respecting their choices concerning tests and treatments.
©2004, Muscular Dystrophy Association