Respiratory Management
Since respiratory management can impact lifespan considerably, physicians should support families in implementing a proactive approach. While pulmonary management is often demanding, it is also the therapeutic modality that will most likely enhance quality of life and prolong lifespan.
To maintain lung capacity, breathing exercises and supplementary aids may be helpful. Incentive spirometry and "breath-stacking" can be implemented at an early age but require discipline to perform on a daily basis. Aerosol therapy with nebulizers may be helpful in some settings and can be initiated at the onset of respiratory symptoms.
Benefit from the routine use of mucolytics, bronchodilaters or steroid treatments is unclear and should be dictated by individual circumstances. Cough assist devices such as the inexsufflator (cough machine) are essential when ineffective cough inhibits adequate removal of bronchial secretions in the lower airways. Vest therapy or manual percussion techniques to help mobilize secretions, when used in conjunction with the cough assist machine, can be additionally helpful in some patients. Regular use of such therapies, most importantly the cough assist machine, are invaluable in the setting of a superimposed respiratory illness, and can help prevent a simple “cold” or “bronchitis” from evolving into pneumonia or a collapsed lung resulting in respiratory crisis.
Even when children are well, daily use of such a regimen can help minimize atelectasis and chest wall contractures and deformity. In many children and young adults, nocturnal hypoventilation with and without obstructive apnea necessitates assisted ventilation.
BiPAP is recommended whenever the vital capacity falls to < 40%. However, in infants and young children who can’t cooperate with formal pulmonary function testing, doctors must use other signs and symptoms to help decide when a child might benefit from BiPAP support. Recurrent nighttime awakenings are often an indication that patients may not be optimally breathing during sleep. A sleep study may help to determine whether or not there is an obstructive component (due to low muscle tone or enlarged tonsils or adenoids), or whether nocturnal hypoventilation (shallow-breathing resulting in low oxygen levels or increased carbon dioxide levels) is present. BiPAP is often recommended for use only at night, but can be invaluable to use for longer periods of time when an upper respiratory infection or other illness results in increased work of breathing and fatigue.
Flu prophylaxis is recommended annually. In younger infants and children with significant intercostal weakness (all type I and weak type II subjects), prophylaxis for respiratory syncytial virus (RSV) is also recommended.
Pulmonary medicine consultation is recommended to assist in making decisions regarding long-term respiratory management. Aggressive treatment of respiratory infection is essential. Antibiotic use is of value when symptoms arising from a presumed viral upper respiratory infection persist longer than expected or new fever or altered secretions appear in the midst of an apparent viral illness. Since recurrent or prolonged antibiotic treatment can predispose patients to yeast infections or even enterocolitis, a balanced approach is needed.
In the severely compromised infant or child, a lower threshold for administering antibiotics may be warranted. If illness results in persistent hypoxemia below 93%, the need for hospitalization and potential intubation should be discussed, although many such patients can be managed effectively in hospital using a non-invasive respiratory protocol.
Oxygen therapy should only be used in conjunction with assisted ventilation in such patients as it can suppress respiratory drive, resulting in atelectasis and hypercarbia.
- Care Guidelines for Children with Neuromuscular Disease with Acute Respiratory Illness
- View Daily Respiratory Management Strategies video
Dr. Mary Scroth, MD, Associate Professor of Pediatric Pulmonology at the University of Wisconsin School of Medicine and Public Health has written several booklets funded by Families of SMA, regarding SMA respiratory care for parents, families and health care providers involved in SMA care.
