Spinal Muscular Atrophy Symptoms and Treatment
Progressive weakness and immobility of the arms and legs in spinal muscular atrophy patients may predispose to other orthopedic problems including tightness of the joints (contractures). Physical therapists can instruct children and their families in range of motion techniques to help prevent these problems. Night splints of the ankles and wrist may also be useful in preventing contractures. Facilitated independent sitting or standing with a special chair or a standing frame may be an important part of the child’s daily therapy program.
Children with Spinal Muscular Atrophy Type I and Type II are especially vulnerable to respiratory complications. A regular program of respiratory therapy and breathing exercises may be very helpful for these youngsters. Precautions to avoid illness such as yearly influenza immunization should be given to most of these youngsters. The most emotionally charged issues in the management of children with SMA concern whether or not to initiate aggressive mechanical support when the breathing muscles begin to fail. Although the medical options are reasonably simple, the emotional turmoil of placing a child on a “breathing machine” is invariably painful for parents. Although many parents (and physicians alike) carry an image of a mechanical ventilator as a huge and unwieldy device, advances in technology have made ventilator use considerably easier and more convenient. Less invasive means of dealing with respiratory problems include many options. Parents should be trained in simple chest physiotherapy to help tide youngsters through their usual “coughs and colds”. Because of difficulty with coughing, even a simple cold may result in saliva and mucous obstructing a child’s airway. Techniques of postural drainage and chest percussion may prevent this complication.
Some children benefit from supplemental oxygen usually delivered through a small nasal tube. This is often helpful at night. Before initiating oxygen treatment, however, it must be determined that the brain is not using low concentrations of oxygen in the blood to determine how rapidly a child must breathe. Some of the normal regulation of breathing may be altered in children with chronic neuromuscular diseases. The decision to use supplemental oxygen should, therefore, be preceded by an evaluation by a pulmonary specialist.
Although the “standard” means of mechanical breathing support involves the surgical placement of a tube in the wind pipe (trachea), other less invasive means of breathing support are now being used. This includes devices termed “negative pressure” ventilators and external positive airway pressure support systems. The use of a BI-PAP has been especially helpful for many of our children with SMA. Instead of a tracheostomy, air is mechanically directed through the nostrils through a firmly, but gently fitted mask. This affords many of the benefits of a mechanical ventilator. It can only be emphasized that the decision to use any or all of these forms of respiratory support are emotionally charged and highly personal. Parents and children would be well advised to speak with other parents who have made this decision and to visit with children who have opted to utilize BI-PAP or tracheostomy based respiratory support
The information on this page was written for Fight SMA by: Robert T. Leshner, M.D., Professor, Neurology and Pediatrics, Children’s National Medical Center.
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