Respiratory Complications
Drooling
Difficulty in managing oral secretions in children with disabilities results from poor oral motor control. Parents may express concern over their child's drooling, frequent cough, or increased upper airway congestion.
Management of drooling can be pharmacologic or surgical. Treatment is recommended when drooling causes significant skin irritation, social problems, or the child is having recurrent respiratory infections secondary to poor secretion management (46).When oral secretions are copious, use of a suction catheter by caretakers can keep the oral cavity and upper airway clear. Families should be instructed in the appropriate technique of oral cavity suctioning and have a portable suction machine that can be used when out of the home setting. Use of medications such as glycopyrolate or the scopolamine patch can decrease the volume of secretions. The use of botulinum toxin injections into the submandibular and parotid glands is being recommended more frequently for children with cerebral palsy (47). Surgical ligation of the glands is typically reserved for cases that are unresponsive to medications.
Drooling may indicate that a child is having difficulty with eating, drinking, or swallowing. In a child with a degenerative neuromuscular disease, such as spinal muscular atrophy, the development of increased drooling or difficulty managing oral secretions should prompt a further investigation into his or her feeding status. A referral to a feeding team should be made for consideration of performing a modified barium swallow. Alternative feeding modalities, such as a nasogastric tube or a gastrostomy tube, may be necessary. Use of medications to dry secretions in a child with muscle weakness may be counterproductive, as thicker secretions may be more difficult to clear.
Respiratory Distress
Children with upper respiratory infections commonly present to their primary care provider with fever, increased work of breathing, and tachypnea.
The evaluation and treatment of a child with a disability who presents with these symptoms should be similar to a typical child. However, the deterioration may be accelerated, requiring a rapid diagnosis and initiation of treatment. The assessment should begin with a review of vital signs, including pulse oximetry. The physical examination focuses on assessing the child's level of alertness, his or her work of breathing, and a chest examination. Children with neuromuscular diseases will frequently increase their respiratory rate in order to maintain oxygen saturation. Unfortunately, a child can decompensate quickly in this situation as a result of significant fatigue. Oxygen saturations can be falsely reassuring in the face of hypoventilation.Diagnostic testing may include pulse oximetry, chest x-ray, venous or arterial blood gas, sputum culture looking for a bacterial etiology, and viral studies for identification of common viruses such as influenza and respiratory syncytial virus (RSV). Viral etiologies are the most common causes of upper respiratory infections in both disabled and typical children.
Use of antiviral medications should be considered in children with disabilities because of their high risk for significant morbidity. Enteral or parenteral antibiotics should be reserved for suspected bacterial etiologies. Coverage for anaerobic bacteria should be initiated when aspiration pneumonia is suspected.
A child with a neuromuscular disease, such as spinal muscular atrophy or Duchenne muscular dystrophy, may need assistance with secretion mobilization and airway clearance. Secretion mobilization can be addressed with chest percussion or a vibratory vest, skills that a family should be comfortable performing. Airway mobilization can be accomplished with the use of a cough-assist machine. The In-Exsufflator, a commercially available device that provides a positive pressure breath followed by a large exhalation, improves peak cough expiratory flow rates (48,49). Children and caregivers should be familiar with the different techniques and initiating their use at the first signs of a respiratory illness. When symptoms increase and evidence of hypoventilation is present, use of noninvasive and invasive respiratory support may be necessary. Noninvasive support may include negative pressure ventilation or positive pressure ventilation with bilevel positive airway pressure (BiPaP) (50). It is important for primary care providers to have knowledge of the various options for respiratory support and to understand the family's wishes on the extent of treatment the family wants in the case of acute decompensation. Acute events are less stressful when families and their primary care providers have discussed their wishes while the child is well and prior to the event.