Children with medical complexity are growing in number in children’s hospitals. They are at the highest risk for medical errors^1,2 and for readmission after discharge.³ A definition of the patient with medical complexity remains elusive, but these children are characterized by chronic, severe health conditions, substantial health service needs, functional limitations that are often severe, and high health resource utilization.⁴ Often these patients have abnormalities involving multiple organ systems and are dependent on medical technology such as gastrostomy tubes, tracheostomies, ventilators, and ventriculoperitoneal (VP) shunts. Many inpatients with well-defined chronic disease are cared for primarily by subspecialists without input from a general pediatric hospital provider. Typical patients in this group include those with cystic fibrosis, sickle cell disease, inflammatory bowel disease, or malignancy. Their care is better discussed elsewhere. However, many patients with complex chronic disease do not fit neatly into a subspecialty, and hospital-based pediatricians are often asked to care for them. Of this latter group, severe neurologic impairment is a frequent common denominator.⁵ This chapter focuses on the acute care of this type of child. The comorbidities of this population are the focus of Chapter 181.

PATHOPHYSIOLOGY

Once an irreversible brain injury has occurred, the etiology (Table 180-1) often matters little in management. Although these patients may be intimidating to the uninitiated, severe central nervous system dysfunction leads to a predictable set of problems (Figure 180-1). For instance, they are at risk for seizures, particularly during acute illness. Nonambulatory patients develop osteopenia and are susceptible to fractures, which can occur with normally benign interventions such as physical therapy. Renal calcifications and stones are common due to mobilization of minerals from bone. Often unrecognized is a predisposition to bladder dysfunction and urinary tract infections due to dyssynergia of the detrusor and external sphincter muscles. Swallowing problems are common and lead to poor nutrition as well as aspiration of gastric contents or oral secretions. This latter problem, as well as poor ventilatory dynamics, predispose to pneumonia. Decubitus ulcers are a common and often preventable problem. These are often found on bony prominences such as the coccyx, ischium, greater trochanter, heel, and occiput as well as on the ears if the patient lies on one side. They may also be seen at pressure points of splints and wheelchairs. The wise practitioner looks for these when performing a physical examination, especially on malnourished patients. Severely neurologically impaired patients experience intestinal motility problems – gastroesophageal reflux and constipation are extremely common. Central nervous system dysfunction can lead to behavior problems such as self-abuse or aggression. If the hypothalamic pituitary axis is damaged, patients may experience diabetes insipidus, adrenal insufficiency, or hypothyroidism. Hypothalamic dysfunction may cause problems with temperature regulation. Patients with profound impairment may experience autonomic dysfunction, which can occasionally lead to paroxysmal sympathetic hyperactivity, also known as autonomic storming, which is easily confused with sepsis or seizures. Spasticity occurs regularly, along with associated contractures. Scoliosis develops from muscle imbalance. Many children do not sleep well, leading to further disruption of the lives of already overburdened caretakers. Dental and gingival problems due to difficulties with achieving adequate oral hygiene and finding appropriate dental care are commonplace.

A nonphysiological effect of caring for a child with severe brain dysfunction is the effect on the family. Marital relations may be strained, and caretakers of children with severely ill patients are adversely affected financially.^6,7 Siblings too are affected, because they are often neglected due to the overwhelming time demands of caring for these children.

An evidence-based approach to treatment is always desirable. Unfortunately, evidence-based choices are often lacking in the medically complex neurologically impaired population. These patients are precisely the ones often excluded from studies, so many of the recommendations below are based on clinical experience rather than voluminous research evidence.

PNEUMONIA

Pneumonia or respiratory distress is a common reason for admission in children with severe neurological impairment. They are susceptible to the usual causes of lower respiratory infection encountered in the hospital, such as viral, mycoplasma, or pneumococcal infection. Severe pulmonary complications of influenza are a particular risk for this population. In addition, aspiration pneumonia is common. When aspiration pneumonia is a consideration, it is reasonable to treat with an antibiotic that has activity against oral flora and anaerobes as well as Gram-negative bacteria.⁸ An acceptable choice for the ill hospitalized patient is intravenous ampicillin/sulbactam. It is easily transitioned to enteral ampicillin/clavulanate as the patient improves. Other regimens to consider include clindamycin with ceftriaxone, piperacillin/tazobactam, or ticarcillin/clavulanate. Measures to improve pulmonary toilet also make sense, because most of these children suffer from an ineffective cough. Many physicians will also supplement treatment with ipratropium or albuterol nebulizer therapy. There is little if any evidence that traditional chest physiotherapy is effective, even in normal patients. Consider instead a cough assist device, intrapulmonary percussive ventilation (IPV), or vest therapy (high-frequency chest wall oscillation).^9,10 These are usually administered two to four times a day. If used with bronchodilators, administer treatment prior to using the cough assist or vest. Bilevel Positive Airway Pressure or Continuous Positive Airway Pressure may also be used to decrease atelectasis, and seem to reduce secretions. Due to the risk of aspiration, do not administer facial BIPAP to a patient being fed concurrently with a gastrostomy tube. It is permissible, however, to feed with a gastrojejunostomy (GJ) tube while positive pressure is being used. Glycopyrrolate and other anticholinergic medications are effective in reducing oral and respiratory secretion volume, but side effects sometimes limit their use (see Chapter 181).

TRACHEITIS

Bacterial tracheitis is unusual in well children but is frequently diagnosed in children with tracheostomies. Patients with tracheitis or tracheobronchitis may present with increased, thickened, or purulent secretions. They may have symptoms consistent with pneumonia, such as decreased oxygen saturation and fever, perhaps due to associated lower airway inflammation, but no signs of pneumonia on chest x-ray. If the trachea is visualized by otolaryngology, tracheal inflammation will be seen. White blood cells are commonly increased in the tracheal secretions. The trachea may be colonized with Staphylococcus aureus, Moraxella catarrhalis, Streptococcus pneumonia, Haemophilus influenza, and other bacteria, making interpretation of cultures difficult. Although positive tracheal cultures alone do not indicate infection, they may be used to guide antibiotic treatment in the patient in whom an infection is diagnosed. Many clinicians treat for 7 to 10 days with systemic antibiotics.

UPPER AIRWAY OBSTRUCTION

Chronic upper airway obstruction due to abnormal airway tone and malacia is a common problem in patients with severe spasticity and may be misdiagnosed by physicians unfamiliar with this problem. These patients present with stertor on inspiration without other cause for obstruction. Usually the stertor improves temporarily if the examiner places the fingers posterior to the mandible and pulls it anteriorly, which can be helpful in diagnosis. Stertor is similar to stridor but is lower pitched and indicative of obstruction in the upper airway superior to the larynx. Stridor is more musical and high pitched in nature. An upper and lower airway evaluation by otolaryngology and pulmonology is often required, and a sleep study may be needed to rule out obstructive apnea. Treatment options include tonsillectomy and adenoidectomy, tracheostomy, or in some cases mandibular distraction or other surgical procedures.¹¹ These latter options are not always successful, but may be considered in an effort to avoid a tracheostomy.

EMERGENCY CARE OF A TRACHEOSTOMY

The care of a tracheostomy is beyond the scope of this chapter. However, physicians who provide care to this population should be aware that any child with a tracheostomy who develops severe respiratory distress should have the tracheostomy tube changed immediately. This can be life saving. If the patient is moving air but having increased work of breathing, saline and suctioning may be tried first. Some providers fear they may not be able to get the tracheostomy tube back in place if removed. This problem is unusual, but if the tracheostomy cannot be replaced, just cover the tracheostomy with a finger and ventilate from above with a bag and face mask until further help arrives.