Section U: Care of the Child with Medical Complexity

BACKGROUND

The care of children with medical complexity (CMC) is an increasingly central component of pediatric hospital medicine. While there is no consensus definition for this population, a recent conceptual framework posits four key domains: high levels of family-identified needs, severe chronic conditions, significant functional limitations, and elevated health resource utilization.¹ CMC include children with static or progressive neurologic, neuromuscular, and genetic or acquired conditions that require the assistance of medical technologies such as gastrostomy or tracheostomy, require care from multiple subspecialists and therapists, and have significant family-identified needs for care coordination across the continuum of care. For example, children with quadriplegic cerebral palsy, muscular dystrophies, congenital myopathies, high myelomeningoceles, and glycogen storage diseases such as Morquio syndrome have medical complexities. For some conditions with a wide variety of severity of manifestations, a subset of more severely affected patients would be considered medically complex. For instance, a child with sickle cell disease with multiple comorbidities (pulmonary hypertension, stroke, etc.) and/or medical fragility (e.g. frequent hospitalizations for crises) would also be considered medically complex. Health services researchers use a variety of methods to identify such children from diagnostic (ICD-9) codes, such as the Complex Chronic Conditions, 3M Clinical Risk Groups (CRG), and Neurological Impairment codes.^2-4 The National Survey of Children with Special Health Care Needs has also been analyzed in a way to identify such children at a population level, suggesting a population prevalence of 0.4%.⁵

Although definitions of children with medical complexity and methods to administratively identify them are still evolving, evidence suggests that this growing pediatric population is demanding our attention in terms of healthcare systems and individual clinical approaches. Children identified as having complex chronic conditions or the highest CRG levels are increasing in inpatient settings.^6,7 Children with complex chronic conditions account for 56% of hospitalizations and 82% of hospital days in US children’s hospitals, demonstrating a 33% increase in the last decade. Having a complex chronic condition or use of medical technology is an independent risk factor for adverse patient safety events.⁸ As payers focus on readmissions, the literature suggests that these children are more likely to be frequent hospital attenders.⁹ In a healthcare environment in which pressure to reduce costs is substantial, CMC, who consume disproportionate quantities of health care resources, are a natural population of focus.

THE HOSPITALIST AND CMC

Pediatric hospitalists may care for CMC in a variety of ways. CMC may be admitted to hospitalist inpatient services. Common reasons for admission include aspiration pneumonia, exacerbations of asthma or chronic lung disease, feeding problems, complex wound care, elective surgical procedures, and malfunction of technology assistance devices. Alternatively, hospitalists may be requested to provide consultations for CMC who are admitted to subspecialty services or present to emergency departments. Often, hospitalists who provide sedation services may be requested to advise on the suitability of sedative agents in the context of multisystem disease.

Ideally, care coordination of CMC is spearheaded by the primary care medical home. However, 53% of US children with special healthcare needs lack medical homes.¹⁰ Moreover, pediatric hospitalists are a key part of the “medical neighborhood” for CMC who require frequent or prolonged hospitalizations. Hospitalists are often the first and last healthcare provider to interact with CMC and their families during hospitalizations, offering continuity and supportive care. Upon discharge, hospitalists must ensure that CMC are linked to their medical homes, knowing that having a medical home is associated with fewer emergency department and inpatient admissions and fewer unmet medical needs.¹¹

Hospitalists, like other healthcare providers, encounter obstacles in caring for CMC. Top barriers perceived by pediatric hospitalists include lack of time, post-discharge resources, evidence-based guidelines, reimbursement. and primary care engagement.¹² Despite the challenges, hospitalists can enjoy long-term therapeutic relationships, intellectual challenges. and interdisciplinary interactions with other hospital-based providers as they care for CMC and their families.

APPROACH TO THE CARE OF CMC

CMC require particular attention at all phases of inpatient hospitalization, beginning at the time of admission or transfer from another service. Hospitalists must take care to not rely excessively on prior documentation when constructing current histories and physicals because plans change, patients may have data from other providers that do not reside in any one electronic health record, and misinformation from prior encounters can be easily propagated. CMC presenting to the hospital will have a chief complaint, but thorough history taking may reveal a series of interdependent conditions that require various levels of attention (see Chapters 180 and 181). Attention to underlying conditions and differentiating among time-limited acute conditions, chronic but stable conditions, and progressive and life-limiting conditions ensures that the care rendered is appropriate to the needs of each child.¹³ Prioritization is a key role of hospitalists who care for children with acute illnesses superimposed on chronic conditions.

Many CMC have intellectual and physical disabilities that affect their overall health and development; hospitalists must remember that they see CMC when they are sick and may lack context about quality of life and function when the child is well. The World Health Organization’s International Classification of Function, Health and Disability (ICF) provides a framework for hospitalists who care for CMC. It broadens the traditional organ–system approach to care by shifting focus to health rather than disease and to ability rather than disability. In partnership with CMC and their families, the ICF can facilitate goal setting for during hospitalizations.¹⁴ This approach involves careful documentation of the baseline level of functioning of each CMC during the initial history with families. This information helps providers to recognize when CMC are approaching their own “normal” or are demonstrating a change in status. For example, sleep patterns, frequency of bowel movements, communication skills, mobility limitations, and general functional abilities should be reviewed on admission. Some CMC might pass stool only once every 2 to 3 days; some might communicate yes and no responses via unique eye movements. A good understanding these individual patterns is integral to the recognition of illness and progression to wellness in many CMC.

Admission medication discrepancies are estimated to occur in 30% of admissions to pediatric tertiary care centers, and children who need four or more medications, particularly antiepileptic drugs, are at greatest risk.¹⁵ Medication reconciliation is the critical responsibility of hospitalists, given that CMC often take a combination of medications prescribed by multiple providers with doses that may have been adjusted verbally and without documentation. Diligence is required to ensure that complicated medication and feeding regimens are accurately communicated through systems of computerized physician order entry. CMC may have detailed home routines that are difficult to replicate on hospital units because of policies, regulations, and nursing staffing ratios. These children may only avoid negative consequences by adherence to specific, seemingly idiosyncratic routines. Partnerships between providers and parents can preserve routines during hospitalizations that promote healing environments that are satisfying and safe, particularly during transitions between home and hospital.

It is the responsibility of the hospitalist to ensure that inpatient care coordination is effective and efficient. Frequent communication with the child’s primary care provider (PCP) is essential. Early establishment of a line of communication with a patient’s PCP can facilitate later care management decisions and discharge planning. Similarly, communication with hospital-based specialists is critical to the provision of coordinated care. Specialists may be consulted for acute management questions; their multiple inputs must be integrated, and if conflicting, diplomatically adjudicated. Hospitalists need to be active and resourceful in their interaction with subspecialists, asking specific questions and suggesting management approaches. CMC and their families are best served when the hospitalist advocates for their needs; for example, hospitalists can coordinate multiple procedures by multiple providers under a single anesthesia. Hospitalists can consult with subspecialists not just for acute illness management, but for the development of a discharge care plan that minimizes readmissions as much as possible. Even when formal inpatient consultation is not indicated, keeping established subspecialists “in the loop” can contribute to effective discharge plans that anticipate needs for care at home. With CMC, communication and coordination with a multitude of allied health professionals is just as important as communication with other physicians. Parents, therapists, care managers, discharge planners, social workers, home care providers, educators, and others play important roles in the care of CMC, and it is critical to incorporate their input into all stages of care planning.

Hospitalists sometimes care for CMC and their families as they contend with major decisions such as choosing gastrostomy, tracheostomy, and other technologies. They often care for families making decisions regarding end-of-life care for their children with life-limiting conditions. Increasingly, families are opting for end-of-life care at home rather than in hospitals, and discharge planning becomes the highest priority.¹⁶ Family-centered, interdisciplinary communication is of utmost importance.

The presence of a CMC in the family affects the physical and emotional health and wellbeing of the parents, siblings, and other family members. Parents of children with disabilities are in poorer physical and emotional health than are parents of typically developing children.¹⁷ The physical health of these parents is directly associated with the physical health of their children, and their mental health is associated with their child’s quality of life.¹⁸ Hospitalists should recognize family stress, and engage family support services as needed. The needs, preferences, and resources of families of CMC should be evaluated well in advance of hospital discharge.¹³ The typical patient handoff process is highly variable, unstructured, and at high risk for communication errors for all children, but particularly for CMC. Patient safety is optimized when transitions between providers is deliberate, proactive, and interactive, with an emphasis on strategies to address ongoing acute issues as well as anticipated condition-specific problems that might arise. Hospitalist schedules in which inpatient attendings rotate frequently can be frustrating to families if plans change with each new attending. It is incumbent on hospitalists to communicate effectively with each other as a team to minimize adverse impact of such transitions.

Hospitalists must proactively plan for hospital discharges of CMC, especially when home health nursing care and equipment must be in place for discharge to occur. For CMC, sending a discharge summary to the patient’s PCP is not sufficient; direct communication via telephone facilitates a safe and efficient transition of care. Because 40% of families of children with special health care needs (CSHCN) experience a financial burden related to their child’s conditions, resources to fill prescriptions, purchase specialized diets, and contend with loss secondary to the need to reduce or eliminate employment should be explored.¹⁹ Home medication and feeding schedules should be developed with consideration of family, school, and child needs in order to maximize opportunities for community participation.

FUTURE DIRECTIONS

With the increasing need for medical homes for CMC, residency training programs must ensure that future pediatricians demonstrate comfort and familiarity with devices and medications, and have both the knowledge and technical skills needed to address ambulatory-sensitive problems and minimize emergency department and hospital admissions.²⁰ To date, pediatric residency programs offer little formal training regarding the lives and needs of CSHCN and their families outside the hospital setting, particularly with regard to identifying community resources and collaborating with community agencies and schools.²¹ Indeed, it has been suggested that the care of CMC, which demands that providers have specialized knowledge and expertise in indwelling catheters, seizure control, treatment of spasticity, management of home ventilators, tube feeding management, dealing with home nursing agencies, advocacy with payers, the care of healthy siblings, among many others, might be its own specialty.²²

Healthcare reform is upon us, and hospitalists, like all providers, need to be increasingly focused on delivering healthcare of the highest quality at the lowest appropriate cost. As we go forward, informatics that transform paper to electronic medical records, privacy-protected health information exchanges that support the organization and real-time sharing of clinical information (including medication reconciliation and comprehensive care plans), telemedicine for immediate clinical assessments, and patient–provider electronic communications might ease some of the challenges that slow and fragment our current healthcare system. Issues of readmission rates, shared accountability, and the delivering the right amount of care in the right setting (inpatient vs. ambulatory settings) demand our attention. Fragmented care is no longer an option, and partnerships between parents, hospitalists, subspecialists, and generalists across the continuum are needed to ensure coordinated care. The key roles of parents, social workers, care managers, homecare providers, educators, and community health providers in the deliberate provision of coordinated care for CMC cannot be underestimated, and team-based care is no longer an option but a necessity. Hospitalists are well positioned to nurture these partnerships as they care for CMC.

REFERENCES

BACKGROUND

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.

MANAGEMENT

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.

SEIZURES

General pediatricians depend on their neurological colleagues for long-term management of seizures. However, many patients with severe central nervous system impairment experience seizures during an acute illness, especially illnesses associated with fever. If anticonvulsant levels are appropriate and no other cause for the flare-up of seizures is found, administer lorazepam or clonazepam in divided doses for 3 to 5 days, either enterally or intravenously, until the illness improves.

VENTRICULOPERITONEAL SHUNT DYSFUNCTION

Children with hydrocephalus typically undergo placement of a VP shunt. When these shunts malfunction, children develop signs of increased intracranial pressure. Initially they may experience headaches or irritability. As intracranial pressure increases, they develop further mental status changes such as lethargy or sleepiness, and begin to vomit. Consider any child with a VP shunt and these symptoms to have shunt malfunction until proven otherwise. Increased intracranial pressure may also cause the Cushing’s triad of bradycardia, hypertension or widening pulse pressure, and irregular respirations. Less frequently, a stiff neck will be present, failure of upward gaze, or even papilledema. To evaluate the shunt, obtain a radiological shunt series to look at integrity of the tubing as well as a head CT to evaluate changes in ventricular size. Palpation of the shunt is not a reliable indicator of a problem. Consultation with neurosurgery is generally warranted. Exposure to tablet computers such as the iPad within 5 cm of a programmable shunt valve has been reported in some cases to alter shunt valve.¹²

Ventriculoperitoneal shunts may also become infected. The vast majority of these infections occur within 3 months of placement, and infection after 6 months is quite unusual unless peritonitis is present.¹³ If an infection is suspected, neurosurgery may be consulted to obtain cerebrospinal fluid (CSF) for culture and analysis. Skin organisms are the most common cause of infection, especially Staphylococcus epidermidis.

IRRITABILITY OF UNKNOWN ORIGIN

Extreme irritability (i.e. screaming for no reason) is one of the most difficult situations the hospitalist may encounter in the child with severe neurologic impairment. This problem leads to extreme parent frustration, exhaustion, and sometimes admission when the emergency department or primary physicians are out of ideas. Table 180-2 lists conditions to consider, with the most common highlighted in bold. The first order of business is to look for clues by taking a detailed history and performing a complete physical exam. The most common identifiable causes of irritability include infection or any condition that could cause pain. Sometimes autonomic storming is involved (see discussion below). In many cases the cause is not easily identified. Frequent culprits include constipation, muscle spasms due to severe spasticity (a markedly elevated CPK can be a marker at times but does not exclude this problem), esophagitis (if a gastrostomy tube is present, check a gastric pH: the goal is a pH of 4.5–7). Not to be missed are renal stones, which are very common due to mobilization of calcium from bones in the nonambulatory patient, as well as side effects of therapies (e.g. the medication topiramate, and the ketogenic diet). Also, consider gallstones; patients who have been on total parenteral nutrition are at increased risk. Palpate bones carefully for signs of a fracture, a common occurrence due to osteopenia. Check for pancreatitis, as some seizure medications, especially valproic acid, increase the risk for this condition. Withdrawal from medications such as baclofen may occur if the dose is inadvertently missed or reduced. Subluxed hips may cause pain, but many patients have this finding with no apparent discomfort. A clue that the hips are playing a role in irritability is if the parent notices discomfort with diaper changes, or if there is obvious pain on movement of the hips on exam. An alteration in the home environment is another cause that may not immediately be apparent. Even children who are markedly developmentally delayed may respond adversely to a change of guardian, home nurse, home location, or to parental strife.

In addition to the history and physical exam, a laboratory workup might include a CBC, C-reactive protein (CRP) or other inflammatory marker, liver function tests, amylase, lipase, urinalysis, and urine culture. An abdominal radiograph is useful to look for constipation, which is a common occult cause and easily treated. Consider an abdominal ultrasound if renal or gallstones are suspected.

PAROXYSMAL SYMPATHETIC HYPERACTIVITY: “AUTONOMIC STORMING”

Many patients with developmental delay suffer from dysfunction of the autonomic nervous system. Fundamental involuntary functions that most of us take for granted are rendered unstable. This seems to be especially a problem for the most neurologically damaged children, such as those with severe hypoxic ischemic encephalopathy. Patients may experience irritability, fever, tachypnea, dystonic posturing, hyperhidrosis, seizure-like episodes, hypertension, tachycardia, vomiting, retching, or diaphoresis. When any of these occur, it is often referred to as “autonomic storming,” paroxysmal autonomic instability with dystonia or paroxysmal sympathetic hyperactivity (PSH).¹⁴ PSH is a diagnosis of exclusion. At times it can be difficult to distinguish from sepsis, although patients with this problem have negative cultures, no source of infection on examination, and normal acute-phase reactants such as CRP or procalcitonin. It may also be confused with seizures and drug withdrawal.

The first step is to look for a cause, and then treat once identified. The most common identifiable cause is pain. The differential diagnosis of this is identical to the search for causes of irritability of unknown origin above. If a source of pain is determined, the obvious solution is to treat the underlying problem. However, many children require additional pharmacological intervention, and often no obvious cause is found. Evidence as to the best treatment is largely anecdotal.¹⁵ A useful approach is to begin with daily controller medications, such as gabapentin for pain, plus clonidine or propranolol for signs of sympathetic overactivity. Baclofen is helpful if the patient has severe muscle rigidity. Benzodiazepines and /or narcotics are then used on a prn basis.¹⁶ In the most severe cases, patients may require heavy sedation with drugs such as dexmedetomidine in an intensive care setting. Do not forget to include nonpharmacological interventions such as cuddling, swaddling, and avoiding noxious stimuli in the treatment plan.

FEEDING

Feeding issues are nearly universal in patients with severe neurological impairment. In fact, feeding problems may herald the diagnosis of central nervous system dysfunction in some as yet undiagnosed children. It is often helpful to inquire how long a parent takes to feed a child. Many parents will state that they are able feed their child by mouth, but they may spend much of their day in this endeavor, taking 45 minutes or longer with each meal, feeding them every couple of hours, and yet on exam the child still appears malnourished.

Aspiration is a risk with oral feedings, so a video swallowing study is often indicated in developmentally delayed patients if the patient is taking oral feedings. Many patients experience silent aspiration. A history of coughing or choking with feedings is not a sensitive indicator of risk.

Gastrostomy tubes can lead to improved nutrition in severely neurologically impaired children and improved quality of life in those caring for them.¹⁷ Parents may need to time to accept the idea of a gastrostomy tube, although the vast majority are happy to have them once placed.¹⁸ Feeding is a psychologically charged issue for parents, and some may falsely see placing a gastrostomy tube as evidence of parental failure. It can help if they discuss the placement of a gastrostomy tube with other families. Once a gastrostomy tube is placed, bolus feedings may provide more physiologic gut hormone secretion but increase the chance of gastroesophageal reflux compared to continuous feedings. If bolus feedings are not tolerated, one approach is to provide nighttime continuous feedings with smaller boluses during the day. A typical regimen might provide half the calories overnight in a continuous drip, and the rest in divided boluses during the waking hours.

Many nonambulatory patients require surprisingly few calories. Excessive weight gain is to be avoided, because it makes transfers between bed and wheelchair or care more difficult. Twenty-five percent of weight for height is a reasonable goal.

Gastroesophageal reflux is extremely common in patients with neurological impairment, and these patients are the most resistant to successful management. On many occasions the family and physician are left to choose between fundoplication with a gastrostomy tube, or GJ tube feedings. The former requires surgery and some risk of failure in this population. However, when successful it allows for bolus feedings. Placement of a GJ tube does not require surgery, but does require continuous feedings. Usually a 4- to 8-hour window without feedings can be created to give parents and children a break from dependence on a pump. However, the GJ tubes are difficult to keep in place and may require frequent trips to the hospital for repositioning. If a patient with a GJ tube develops vomiting, be sure to get an abdominal radiograph or if the position is still unclear, a contrast study to check the tube position. A coiled or out of position tube is a common trigger of vomiting in these patients. An ultrasound maybe a consideration to diagnose intussusception around the distal portion of the GJ tube. There is no clear consensus as to which option—fundoplication with G tube, or GJ tube placement—has better outcomes.¹⁹

An issue that arises in patients with GJ tubes is which port to use to administer medications. Obviously, for patients who can tolerate medications given orally or in the G tube, those routes are preferred. However, patients with J or GJ tubes usually have them in place because they cannot tolerate feedings given more proximally, and J tube administration is the only option.^19-21

CONSTIPATION

Intestinal motility is typically poor in the neurologically impaired child. The lack of exercise and decreased fluid intake contribute to the problem. Although vomiting from constipation is unusual in the normal child who is constipated, it is not unusual in a neurologically impaired child. It can also cause pain and irritability, and predisposes patients to urinary tract infections and retention. Overflow diarrhea is also common, in which loose stools are passed around firm, impacted stool in the colon. An abdominal radiograph can be helpful in evaluating this problem. It may be necessary to specifically ask the radiologist to evaluate the stool burden. In the inpatient setting, patients may require cleanout with a polyethylene glycol/electrolyte solution administered as a nasogastric or G tube infusion. For more on the management of this problem, see Chapter 194.

PITUITARY INSUFFICIENCY

Patients with profound neurological impairment are at risk for associated pituitary and adrenal dysfunction, and those with hypopituitarism require stress doses of steroids during acute illness. Unexplained hypoglycemia can be a clue to this problem. Hypotension and shock after surgical procedures, even mild ones such as dental extraction under anesthesia, can occur when adrenal insufficiency goes unrecognized and untreated.

BEHAVIORAL ISSUES AND AGGRESSION

Developmentally delayed children sometimes become aggressive or uncontrollable when ill or in a strange environment. The hospitalist may be called upon to intervene when a child is biting or hitting caretakers, or pulling out lines or tubes. Sometimes clothing such as Under Armour, onesies for adults, or overalls can keep patients from pulling on tubes in the abdomen and chest. Patient care attendants may be required at times to keep patients safe. Medications such as clonidine or benzodiazepines may be helpful in this situation as well. Although benzodiazepines usually have a calming effect, be aware that some patients may become disinhibited and actually get worse. In the most urgent and severe situations, antihistamines such as benadryl or hydoxyzine may be given IM or po, as well as atypical antipsychotics such as olanzepine. Be aware that atypical antipsychotics can prolong the QT interval. Occasionally restraints are required, but only as a last resort. When necessary, be sure to comply with standardized hospital policies for the use of restraints to ensure patient safety.

SPECIAL CONSIDERATIONS

ETHICAL ISSUES

Children with severe neurologic impairment invariably have illnesses for which no cure exists. Usually the path of least resistance for providers is to treat the current problem. The notion that not doing every possible medical procedure is an option often is not discussed with families. For example, it may take only a few minutes of operating room time for an otolaryngologist to place a tracheostomy, but the care it requires necessitates a major commitment from caretakers, often for years or even for the life of the patient. Placement of a tracheostomy is often the right decision for optimal quality of life, but parents need to understand what they are getting into. In these situations, it is important to talk with families about their goals for the child. Do they prefer survival at all costs or improved quality of life, even if it means taking some risks that an illness could lead to the patient’s demise? These decisions are difficult for parents and providers alike. Some parents are afraid that “do not resuscitate” means “do not care for.” It is useful to reassure these families that the patient will still be attended to and cared for if they decide against aggressive, technology-intensive medical treatment.

CARE COORDINATION AND SHARED DECISION MAKING

Parents who care for a severely neurologically impaired child experience extreme stress. Most are in a constant state of mourning for the child they dreamed of but never had. Many are extremely knowledgeable about their child’s condition and should be considered members of the care team. The wise clinician includes them in decision-making, and listens when they talk about reactions to medications, idiosyncrasies their child may have, and readiness for discharge from the hospital.

Hospital discharge is a challenge and requires a great deal of coordination and planning. Many children with complex medical problems are on multiple medications, and there are multiple opportunities for errors in administration. In addition, there may be a need for durable medical equipment such as feeding pumps, pulse oximeters, oxygen, wheelchairs, and the like. Finally, home nursing orders are often required. The assistance of a nurse care coordinator at the time of hospital discharge can be invaluable. Social work support is required for most families to make sure that they have access the resources they are eligible to receive. The use of complete problem lists, brief chronological summaries of significant procedures and events, and written care plans for families are very helpful in providing care. The child with complex medical illness requires a medical home, even more than do typically developing children. Parents often find themselves caught between well-meaning but conflicting advice from multiple subspecialty providers, and greatly benefit from the care of an experienced generalist and advocate.

REFERENCES

BACKGROUND

Children with severe neurologic impairment comprise a diverse group from an etiological perspective. Yet despite this diversity, there are predictable commonalities in many of the comorbidities and complications they experience. As an example, a child with a history of hypoxic ischemic encephalopathy and another with a genetic disorder associated with cerebral dysgenesis each have different reasons for their presentations but will likely have in common issues such as intellectual disability, risk for seizures, risk for oromotor dysfunction and aspiration, gastrointestinal dysmotility, osteopenia. and altered muscle tone. Please see Chapter 180 for a brief introduction to etiology and comorbidities as well as review of management of common acute care issue in this population. The focus of this chapter is on chronic comorbidities.

The hospitalist who appreciates common comorbid conditions in children with severe neurologic impairment will be prepared to more fully evaluate the child admitted with an acute illness for predisposing conditions which if treated may reduce their risk of recurrent illness and hospitalization. In addition, length of hospital stay is often longer in children with severe neurologic complexity. As a result, chronic issues which may not directly relate to the admission but require attention and management are likely to surface. The range of comorbidities in children with underlying neurologic impairments is quite extensive and can encompass virtually any organ system (Table 181-1). Data relating to prevalence of specific conditions are sparse, and when available usually relate to a specific underlying condition such cerebral palsy.¹ Data that delineate a broader range of associated medical conditions in children with disability do not relate specifically to children with neurologic complexity.² Despite the range of possible comorbidities and the lack of prevalence data, a systematic approach to assessment and management can enable the care provider to feel confident in monitoring for likely complications. What follows is a review of the most common comorbid conditions clinically encountered in children with severe neurologic impairment along with guidelines to their outpatient management.

COMMONLY ASSOCIATED COMORBID CONDITIONS BY SYSTEM

NEUROLOGIC

Many children with severe neurologic impairment have an increased predisposition to seizure disorders. If concern for seizures is identified, an EEG is indicated along with referral to a neurologist for assistance in further evaluation and management. Many children with medical complexity who have with seizure disorders end up on multiple antiepileptic agents which in turn can be associated with detrimental side effects such as somnolence, behavioral change, hepatitis or pancreatitis, or kidney stones.³ For particularly refractory seizure disorders, more radical treatment options are considered. These include the ketogenic diet, vagal nerve stimulator, and on occasion, neurosurgical ablation of the epileptic focus.

Developmental delay is often identified in children with underlying complex neurologic disorders. There may be intellectual disability or learning disabilities that affect social and academic achievement. Obtaining accurate testing for cognitive ability is challenging in the child who may have motor coordination difficulties, dysarthria, or sensory impairments. Testing must be conducted by specialists trained to work with this population of children. Delays are often noted in all streams of development, including speech, gross and fine motor skills, and social/adaptive skills.

Behavioral dysregulation, including self-injurious behaviors, occurs with some frequency in the child with underlying neurologic impairment. Approaches to management include behavioral interventions, and at times, mood stabilizing medications.⁴ It is important to recognize when behaviors reflect frustration with communicative abilities and to work on augmenting options for the child as much as possible. Additionally, if the behavior is self-injurious it is important to rule out pain as an etiology. A thorough multisystem evaluation is warranted in these cases (see Chapter 180, Table 180-2 for a differential diagnosis).

Children may have hydrocephalus secondary to central nervous system (CNS) malformations, cysts, meningitis, tumors, or intraventricular bleeding. Ventriculoperitoneal or ventriculoatrial shunts are often placed in this condition. These devices are prone to dysfunction and may cause acute problems related to increased intracranial pressure or infection, as discussed in Chapter 180.

Many children with severe neurologic impairment demonstrate oromotor dysfunction, which is responsible for significant morbidity.⁵ Dysphagia can result in challenges in chewing and swallowing and increased risk of aspiration of foods and liquids. It can also result in diminished oral intake associated with poor weight gain and malnutrition. Decreased fluid intake can further exacerbate constipation. In addition, oromotor dysfunction can contribute to sialorrhea. The child who drools excessively is usually not producing an excess of saliva but rather has difficulty managing the secretions he or she does produce. Finally, from a developmental perspective, oromotor dysfunction can affect speech. It is extremely important when evaluating the nonverbal or dysarthric child to make no assumptions about underlying cognitive ability, as the two are not always correlated. Augmentative technology can play a critical role in facilitating a child’s communication.

SENSORY IMPAIRMENT

Children with underlying neurologic conditions often have associated sensory impairment. In addition to the routine ophthalmologic conditions of childhood, common concerns in this population include cortical visual impairment (inconsistent visual attentiveness), amblyopia, nystagmus, visual field cuts, refractory errors, and exposure keratosis. Hearing loss may take the form of sensorineural loss or may be conductive, perhaps on the basis of congenital malformation. Early identification of any degree of vision or hearing impairment is important in determining the best mechanisms of communication for a child as well as devising educational plans and interventions.⁶ Children with unidentified sensory impairments can appear to have lower cognitive abilities and decreased social awareness than may be actually true. Exploring their baseline level of cognitive functioning with parents and other caregivers is helpful.

GROWTH, NUTRITION, AND GASTROENTEROLOGY

The enteric nervous system (ENS) governs the function of the gastrointestinal system. Even though the ENS can operate autonomously, it is modulated by extensive input from the central nervous system (CNS). As a result, children who have underlying disorders affecting the CNS end up with dysfunction of the gastrointestinal system.⁷ Dysmotility is thus quite common. Poor nutrition is a complex issue which reflects not only complications of dysmotility but an interplay between neurologic, endocrine, and environmental systems as well.⁸

Disorders of foregut dysmotility are seen in the form of esophageal dysmotility, changes in upper and lower esophageal sphincter function, and gastric dysmotility. This clinically manifests as retching, vomiting, and gastroesophageal reflux disease (GERD). Other factors contribute to GERD in this population as well, including hiatal hernia, prolonged periods of lying supine, and increased intra-abdominal pressure secondary to spasticity. GERD is a clinical diagnosis; however, it is important to establish objective findings in some cases with impedance probe studies. Treatment of GERD should be addressed with a proton pump inhibitor alone or in combination with a histamine type 2 receptor antagonist. There is growing evidence that baclofen can also be used in cases of severe GERD.⁹ If delayed gastric emptying is present, erythromycin 30 minutes prior to meals can help with gastric motility.

Persistent or refractory GERD can lead to pulmonary complications of recurrent pneumonia and progressive lung injury. If medical management does not adequately control symptoms, surgical intervention should be considered. A Nissen fundoplication involves wrapping the fundus of the stomach around the gastroesophageal junction. Fundoplication corrects a hiatal hernia (if present), lengthens the intra-abdominal portion of the esophagus, and increases the pressure of the lower esophageal sphincter, thereby helping to prevent GERD. This procedure is not without complications, and retching, dumping syndrome, and bloating occur with some frequency.¹⁰ A nonsurgical option for children who have a gastrostomy is feeding directly into the jejunum via a gastrojejunal (GJ) tube. GJ tubes are associated with risk of intussusception and dumping. More frequently encountered problems include dislodgement and recurrent clogging of the tube necessitating trips to interventional radiology for replacement. The decision to place a GJ tube versus performing a Nissen fundoplication is still a matter of ongoing research. At this time, the decision should be made on an individual basis, balancing the potential risks and benefits of each procedure and local practice patterns.¹¹

Chronic constipation is a common problem in this population, with an estimated prevalence of 26% to 50%.⁷ Poor colonic motility, physical inactivity, medications, and spasticity are some of the risk factors. Constipation is associated with abdominal pain, discomfort, urinary retention, recurrent urinary tract infections, and may be a cause of autonomic storming (paroxysmal sympathetic hyperactivity). Regular bowel movements are the goal, promoting efficient emptying, minimizing complications, and maximizing comfort. Change in dietary formulation with addition of fiber may be helpful. Polyethylene glycol 3350 powder is a mainstay of treatment and may be dissolved in any noncarbonated liquid. Although it is tasteless, it does alter the viscosity of the fluid in which it is dissolved. Some children resist it for this reason when it is taken by mouth, but it is generally well tolerated. It may be supplemented with senna, bisacodyl suppositories, or other stimulants. Suppositories may help parents regulate the time of bowel movements. Sometimes rectal irrigations or enemas are required. Keep in mind that a stimulant laxative may cause cramping if the patient is impacted. Do not use mineral oil in this patient population because of the risk of aspiration. At times, severe constipation can be refractory to medical management and surgical intervention may be considered. The antegrade colonic enema (ACE) procedure involves using the appendix as a stoma, which can then be used for intermittent colonic irrigation with normal saline or polyethylene glycol, facilitating evacuation.⁷

In addition to primary problems of dysmotility, poor nutrition is another common gastrointestinal disorder in children with neurologic conditions. Established growth curves have not been created for many of these conditions, some of them quite rare or unique, further complicating the creation of nutrition and growth goals. When poor nutrition is present or suspected, the reasons can be far-ranging, from oromotor dysfunction with dysphagia, resulting in diminished intake to dysmotility, resulting in diminished appetite. In the case of dysphagia, feeding therapy to address oromotor coordination and proper seating can be useful. The most concerning complication of dysphagia is the risk for aspiration. Thin liquids are the texture most likely to be aspirated because of the velocity at which they traverse the oral cavity. However, solids that require significant chewing may be swallowed whole and pose significant aspiration risk as well. Careful history must be taken to uncover possible aspiration. While coughing or choking during meals is a helpful sign when present, aspiration can be silent. History should be obtained relating to color change during meals, watery eyes, development of a “wet” voice, or need for frequent throat clearing. If any of these signs are identified, a modified barium swallow study should be obtained. If aspiration is present, frank discussion with the family is necessary to determine alternative feeding options. A child who aspirates thin liquids but not thicker ones may be able to consume an oral diet containing thickened liquids. If all consistencies are aspirated or if a child simply cannot take in enough fluid via a thickened liquid diet, a gastrostomy tube to allow direct enteral access may be indicated.

At times, discomfort secondary to GERD or constipation may lead to diminished appetite, which then leads to poor weight gain or weight loss. In these cases, treating the underlying cause may result in enhanced appetite. Cyproheptadine can occasionally be a useful agent to increase appetite.

There are several other gastrointestinal problems found commonly in this population. Pancreatitis can be a complication of medications. Hepatitis is another common effect of medications, particularly anticonvulsants and baclofen. Finally, gallstones are found on occasion, particularly in the child who has a history of parenteral nutrition in the past. In the child presenting with unexplained irritability, screening labs should include liver function tests, amylase, and lipase. An abdominal ultrasound is also a reasonable screen. If gallstones are identified, elective cholecystectomy is often indicated—particularly in the nonverbal child who is unable to communicate his or her discomfort—in an effort to avoid future emergent surgery.

AIRWAY CONCERNS

Along with complications of the gastrointestinal tract, complications of the airway are among the most prevalent comorbid conditions in children with underlying neurologic impairment. They are also among the conditions most likely to pose life-threatening events and/or impact life expectancy. Respiratory concerns may involve the upper or lower airway.

Upper airway issues include anatomic differences (such as choanal atresia or septal deviation) as well as neuromotor differences resulting in tracheomalacia. Signs of upper airway obstruction can include snoring, stridor, and concerning respiratory patterns, such as pausing or gasping while asleep. Obstructive sleep apnea (OSA) is prevalent. If sleep apnea is suspected, polysomnography should be obtained to identify the nature and extent of the abnormalities. Surgical intervention is indicated if OSA is identified in the presence of adenotonsillar hypertrophy. If there is no anatomic abnormality or if tonsillectomy and/or adenoidectomy does not improve the OSA, then a trial of positive pressure should be undertaken.¹² Continuous positive airway pressure (CPAP) is the usual first step. CPAP requires a child to wear a tightly fitting mask, which can be challenging. Respiratory therapists have expertise in mask fitting and will work with a child and his or her family to find the optimal design. Bilevel positive airway pressure (BiPAP) is used if there is an element of central sleep apnea, and provides a backup rate. Tracheostomy may be indicated for severe upper airway obstruction not restricted to sleep.

Lower tract disease includes both restrictive and chronic parenchymal disease. Restrictive disease may be secondary to hypoventilation in the setting of underlying abnormalities of muscle tone or weakness, such as spinal muscular atrophy. Restrictive disease may also be seen in the setting of progressive neuromuscular scoliosis. Positive airway pressure can be useful for the child with chronic hypoventilation or restrictive disease, as it provides enhanced pulmonary expansion. In cases of severe restrictive disease or hypoventilation, chronic ventilation may be indicated. This necessitates placement of a tracheostomy.⁹

Parenchymal disease is often seen in the setting of inflammatory lung disease secondary to chronic aspiration, which can be mistaken for ordinary asthma. Traditional treatment with inhaled steroids and bronchodilators may be effective in ameliorating symptoms. Recurrent pneumonias are also common. The combination of chronic aspiration resulting in inflammation and acute aspiration resulting in recurrent pneumonias can lead to declining pulmonary function over time. This in turn can lead to respiratory failure, a frequent cause of mortality in this population. Immunization to prevent acute infectious exacerbations from vaccine-preventable diseases (e.g. influenza and pneumococcus) is important.

Treatment of pneumonia should be dictated by the setting in which it occurs, the patient’s general health status, and history of prior pneumonias, as discussed in Chapter 180. It is incumbent upon the medical provider to consider the possible role of aspiration when recurrent or chronic symptoms are present. Aspiration can be antegrade, from secretions or an oral diet, or retrograde, as in GERD. A videofluoroscopic swallowing study can delineate risk of aspirating foods and liquids, while a salivogram can be useful for assessing aspiration of secretions, particularly in the child who does not eat by mouth. Infrequently, a CT scan of the lungs may be ordered to look for evidence of bronchiectasis in children with chronic aspiration.¹² In a child who is known to have poor airway protection it can be important to quantify the presence or absence of GERD via an impedance probe.

Management of recurrent or chronic aspiration must address the underlying cause. If a child has difficulty managing their secretions an anticholinergic agent may be indicated. Glycopyrrolate was recently FDA approved for children over age 3. The most common side effects of glycopyrrolate in this population relate to its anticholinergic effects and include worsening constipation, urinary retention, and secretions that are too viscous, thus posing a risk of mucus plugging. Behavioral changes can also be seen. Higher doses are associated with more side effects. In all cases, the initiating dose should be small, 0.02 mg/kg TID, with slow titration upward to a maximum of 3 mg TID.^13,14 Close monitoring for side effects is indicated. Side effects may at times preclude a child’s ability to take glycopyrrolate. Other treatments for sialorrhea include use of the scopolamine patch (which is variable in its effectiveness and cannot be titrated), or sublingual application of optic atropine.¹⁵

Options for treating secretions refractory to medical intervention include botulinum neurotoxin type A (BNT-A) injections into the submandibular and parotid glands, surgical ligation or removal of the salivary glands, and laryngotracheal separation. The effect of BNT-A has been shown to last for only 3 to 4 months and thus necessitates return to the operating room for repeat injections.¹⁶ Salivary duct ligation or removal addresses sialorrhea on a more permanent basis. The most common surgery is ligation of submandibular and parotid ducts. Decrease in the amount of saliva has been demonstrated to decrease the frequency of episodes of lower respiratory tract infections. Parents should be given adequate anticipatory guidance about maintaining good dental hygiene, since decrease in saliva can lead to increase in dental caries.¹⁷ The most definitive procedure to avoid aspiration is a laryngotracheal separation, in which the trachea is brought to a stoma and the larynx is closed off. It is important to note that a child who has undergone this procedure breathes entirely through the tracheal stoma, as the upper airway ends in a blind pouch. This is a fairly radical procedure reserved for children who have persistent daily symptoms in addition to recurrent pneumonias resulting in repeated hospitalizations, decline in pulmonary baseline, and diminished quality of life. This procedure results in permanent placement of a tracheostomy and loss of voice and phonation, but can have a positive impact on pulmonary health.¹⁸

Aggressive pulmonary toileting is an important aspect of managing chronic and recurrent aspiration. For some patients with weak cough and airway clearance, a cough assist device may be useful. This machine provides a positive inspiratory pressure followed by a negative expiratory pressure. Some patients are prescribed a high-frequency chest wall oscillating vest (commonly known as a “chest PT vest”) in an effort to mobilize airway secretions. The data on effectiveness of these devices is as yet inconclusive.

MUSCULOSKELETAL

Many children with primary neurologic impairment have altered muscle tone. The prevalence of cerebral palsy alone is 2 to 3 per 1000 live births. Abnormalities of tone may manifest as hypotonia, spasticity, and/or dystonia, and are often associated with underlying weakness. Over time, problems with muscle tone can lead to permanent contractures, hip subluxation or dislocation, and progressive neuromuscular scoliosis. Further complications can include difficulty with hygiene (if the hips do not externally rotate or abduct easily), skin breakdown (i.e. between the costal margin and iliac crest), challenges with seating, and pain.

Treatment of altered muscle tone should begin early and aggressively to promote maximal functioning and comfort and minimize longitudinal complications. In the United States, referral to early intervention for children under 3 years of age will allow for in-home physical therapy services, which focus on strengthening and coordination. Physical therapy can then be rendered through an individualized educational plan in the public school system between the ages of 3 and 22. Orthotics, such as ankle foot orthoses, wrist splints, or a thoracolumbar spinal orthosis may be useful for promoting skeletal alignment. Virtually all children with altered muscle tone should be followed by either a physiatrist or an orthopedic surgeon to monitor for and slow the prevention of long-term complications.

Spasticity management poses a particular challenge. If the spasticity is focused, for example the heel cords of a child with spastic diplegia, treatment may also be focused in the form of chemodenervation, in which neuronal–muscular transmission is disrupted. The most common type of treatment involves botulinum toxin, which can be administered to specific muscle groups. Effect sets in after approximately 1 week and lasts for several months. Children are often on a regular schedule to receive injections; for example, every 6 months. If a broader distribution of muscles is affected by spasticity, phenol injections can be added for larger muscle groups. The mechanism of action is one of demyelination. If spasticity is widespread, a more systemic effect is warranted. In this case, commonly used agents include baclofen (a GABA-B agonist) and benzodiazepines. A conservative approach to introducing these agents should be taken. Less often, the calcium channel blocker dantrolene may be used.¹⁹

Baclofen can be extremely effective for spasticity management; however, because higher levels are required to penetrate the blood–brain barrier, side effects are common. Somnolence, lowered seizure threshold, worsening constipation, and nuchal hypotonia are several side effects which frequently complicate clinical care. When a child with significant lower extremity spasticity demonstrates good response to baclofen but is limited in their ability to take it because of systemic effects, an intrathecal baclofen pump may be an alternative. A baclofen pump is inserted in the subcutaneous space in the abdomen (usually the right lower quadrant) and has a reservoir which holds a small volume of baclofen. The drug is titrated at exceedingly low rates through a catheter that is threaded into the intrathecal space. In this manner, the drug is delivered directly to the desired region and relatively small doses garner a much bigger clinical effect. The pump needs to be refilled on a regular basis. Complications include pump malfunction or infection and must be considered if the child with a pump presents with unusual symptoms. Typical signs of baclofen withdrawal include but are not limited to seizures, altered mental status (including anxiety, hallucinations, altered level of consciousness), hyperthermia, spasticity or myoclonus, labile blood pressures, and pruritus. Complications can include rhabdomyolysis, renal failure, and disseminated intravascular coagulation. Acute baclofen withdrawal can result in multiorgan failure and be life threatening. It must be recognized early.

For children with neuromuscular abnormalities orthopedic surgery may ultimately be indicated. The most common procedures involve tendon lengthenings to address contractures, hip surgery to address subluxation or dislocation, and spinal fusion to address scoliosis. Scoliosis is often of a progressive neuromuscular type in this population and can contribute to restrictive lung disease, difficulty with seating, and pain. Fusion is a complex, high-morbidity procedure and requires careful consideration by the patient, caregivers, and the healthcare team.

ENDOCRINOPATHIES

Endocrinopathies are common in children with neurologic disease. Hypothalamic-pituitary axis disturbances often occur in conjunction with other CNS abnormalities and can result in hypothyroidism, adrenal insufficiency, growth hormone deficiency, and pubertal changes. At times, multiple endocrinopathies present simultaneously and panhypopituitarism occurs, necessitating broad supplementation with hormones and steroids. In the child who is not growing well, thyroid hormone and growth factors should be checked.²⁰

Decreased bone density is common in nonambulatory children, resulting in pathologic fractures. Sometimes there is no overt etiology. This is important to recognize, as questions of intentional trauma or neglect can be raised in this setting. While necessary to consider, in this population osteopenia is common and is a frequent explanation. Unfortunately, we are not able to predict accurately who will fracture and who will not. Risks for fracture include medications (especially certain anticonvulsants, antacids, and glucocorticoids), poor nutritional status, calcium and phosphorous homeostasis abnormalities, underlying genetic syndromes, and limited weight-bearing activity.

Monitoring should include 25-hydroxy vitamin D levels, and AAP guidelines recommend aiming for a level greater than 50 nmol/L through supplementation.²¹ There is no consensus in the literature about the standards of obtaining bone density studies or utilizing bisphosphonate treatment. While some centers document bone density at certain ages, others refrain from obtaining the study until a pathologic fracture has occurred. Bisphosphonate treatment is generally administered based on a protocol, and there is variation in frequency ranging from monthly to every 3 months. Zaledronic acid is used every 6 months. Weight-bearing activity is often encouraged (using a gait trainer or stander for the nonambulatory child); however, documentation about length of standing time to affect significant change in bone density remains unclear and is an active area of research.

RENAL/UROLOGIC

Voiding dysfunction in individuals with altered muscle tone occurs with some regularity. Children with spina bifida routinely require clean intermittent catheterization, and voiding dysfunction is seen in more than half of children with cerebral palsy.²² Problems may include urinary incontinence, urinary retention, neurogenic detrusor overactivity (involuntary contractures during bladder filling), detrusor-sphincter dyssynergy, and altered bladder capacity. Monitoring urinary patterns is thus important. If a child is uncomfortable periodically, identifying whether it relates to the need to void can be helpful. A warm water bottle and crude maneuver can stimulate voiding. Persistent urinary retention requires evaluation with urodynamic studies and consultation with a urologist. Clean intermittent catheterization may be necessary if there is no void within a certain timeframe, often 8 to 12 hours, to reduce the risk of urinary tract infection and vesicoureteral reflux. Evaluation with voiding cystourethrogram and ultrasound can guide further management in the case of recurrent urinary tract infections.

Nephrocalcinosis occurs in this population at a higher rate than in typically developing children. Risk factors include certain commonly used medications, such as topiramate, as well as altered calcium metabolism. In the child who presents with irritability, another good reason to obtain an abdominal ultrasound is to evaluate for renal stones. If stones are found, consultation with a nephrologist is indicated.

SKIN

Decubitus ulcers are a common problem for individuals with decreased mobility, and often decreased fat stores. Skin should be looked at carefully during all physical examinations, particularly over bony prominences and dependent areas. Attention should be paid to seating and mobility devices. Specialized cushioning can be ordered for wheelchairs. If there are problems with skin breakdown, an air mattress can reduce risk of recurrence and promote healing.

DENTAL

Maintaining healthy oral hygiene in children with neurologic impairment can be challenging. There may be sensory aversion, making it difficult to enter the mouth with a toothbrush. In the child who aspirates, suction may need to be on hand. Many children with spasticity may have a tonic bite reflex, biting down on a toothbrush and impeding any efforts to brush. As a result, cavitation is a risk. GERD and diminished secretions can further increase risk of caries. Routine evaluation with a dentist is a necessary aspect of preventive health in this population, and often more extensive management is necessary during dental treatment.²³ Obtaining adequate dental care for children with underlying neurologic conditions can be challenging and fraught with barriers.²⁴

COMMON COMORBID CONDITIONS SPANNING MULTIPLE SYSTEMS

SLEEP

While disturbances of sleep are common in all children, among children with neurologic complexity the problem is more prevalent and stems from a broader array of primary concerns. It is also a common source of parental fatigue. Manifestations may present as difficulty falling asleep or sustaining sleep, and likely represent a combination of interactions between biological, psychological, and social/environmental factors.²⁵ Sleep problems often go unrecognized, yet they can be responsible for significant daytime symptoms such as inattention and irritability, and can also cause familial disruption.²⁶

Difficulty with sleep onset or maintenance may be a primary neurologic issue. It may relate to disruption of the circadian rhythm and melatonin production (which can be further exacerbated by cortical vision impairment), seizure activity, or present as central apnea without an epileptic correlate. There may be an airway issue, secondary to either obstruction or hypoventilation. There may be a problem related to pain or discomfort, such as poorly controlled GERD, spasticity, inability to change position, or incontinence. Sleep disturbance may sometimes be behavioral or related to anxiety. Evaluation starts with a thorough history and often requires further investigation via polysomnography to fully elucidate the potential underlying cause. Intervention depends on the etiology.

Of course, as with well children, one should employ sleep hygiene measures as a first step. This includes avoiding long naps during the day, exposure to sunlight, avoiding caffeinated beverages, establishing nightly routines, and avoiding falling asleep under conditions that will not be present when the child awakes, such as being held or rocked to sleep.

For children who have a primary neurologic etiology for their sleep disturbance, there is growing support in the literature for the use of melatonin as a sleep aid.^27,28 Dosing regimens still lack consensus, but a standard of 3 to 6 mg is often used. A combination of antihistamines along with melatonin may also used for more sustained effect. When this is not successful, trazodone can be helpful. Zolpidem, clonidine, eszopiclone, and even flurazepam have been used successfully when parents are desperate for a solution and other interventions have failed.

IRRITABILITY

Given the multisystem involvement in this patient population, pain is common. In a study conducted by Breau et al, it is was concluded that 35% to 52% of children with cognitive impairments had significant pain that lasted for more than 9 hours per week.²⁹ Pain can be secondary to musculoskeletal, gastrointestinal, dental, or neurological etiology, can have a negative impact on the quality of life, and is one of the common reasons for inpatient admission.³⁰ It is difficult to assess pain in children who often have limited communication skills, multisystem involvement, and dependence on caretakers for assessment. Pain and irritability should be addressed regularly on an outpatient basis, as recognition and treatment can have a profound effect on quality of life. Like sleep, the diverse range of potential causative reasons for pain mandates careful history taking and investigation (Table 181-2). Treatment will vary depending on cause. Please see Chapter 180 for further discussion.

SPECIAL CONSIDERATIONS

The very nature of children with neurologic complexity is that they are at risk for multisystemic complications and comorbidities. They are also at increased risk of having unmet healthcare needs. It is incumbent upon providers to be aware of these risks, to monitor for them routinely and intervene early. A systematic approach to evaluation is critical.

FAMILIAL EFFECTS AND GOAL SETTING

In addition to needs surrounding the medical issues addressed in this chapter, children with severe neurologic impairments have tremendous need for educational and therapeutic services, durable medical equipment, and assistance in care provision, such as home nursing or personal care attendants. The elaborate and endless coordination and uncertainty can be challenging, and effects of having a child with complex medical needs on families are well-documented^31,32 (Table 181-3). Assessing the need for additional psychosocial support for family members must be a routine part of evaluation.

Ultimately, a child with severe neurologic impairment has an intricate web of medical concerns which impact development, autonomy, and virtually all aspects of daily living. Questions about quality of life are important to raise with caretakers. Identifying the family’s and child’s goals is critical in carving out a care plan that will be most satisfying to the family and the child. An important variable to consider is the child’s comfort. Families differ in the aggressiveness in which they seek interventions. Navigating those choices, whether or not to place a G-tube or tracheostomy or to do a spinal fusion, for example, and supporting families in their decisions, is one of the most important roles a provider can play. Bringing in palliative care specialists to specifically address physical and psychological comfort can often be useful.

SUGGESTED READINGS

REFERENCES

ENTEROSTOMY TUBE FEEDING

If the gastrointestinal tract is functioning adequately, enteral nutrition is preferable to parenteral nutrition in the child with medical complexity. When compared with parenteral nutrition, enterostomy tubes are easier to handle and less expensive, and several randomized trials have shown that complications (e.g. sepsis, thrombosis, liver dysfunction) are less common. Enteral feeding prevents mucosal atrophy, maintains gut flora, and plays an important role in preserving the enteral immune system. Supplementation can be complete or partial depending on the child’s ability to feed by mouth.

Gastrostomy (G), jejunostomy (J), and gastrojejunostomy (GJ) tubes have replaced nasogastric (NG) and nasojejunal (NJ) tubes when the anticipated length of enteral feeding exceeds 8 to 12 weeks. Tubes through the nose, although simple to place, are not well tolerated in the long term and are cosmetically unappealing. They may lead to increased secretions, nasal ulceration, and sinusitis and could predispose to gastroesophageal reflux, esophagitis, and strictures. They are easily displaced and may result in aspiration.

Concerns about permanence of the tube, relinquishing of normalcy, loss of autonomy, and loss of the ability to orally feed often make this a difficult decision for caregivers.

This first part of this chapter outlines the indications for enterostomy tube placement and the different techniques available as well as some of the complications and outcomes reported.

INDICATIONS

There are a number of reasons to consider placement of an enteral tube:

• Oral motor feeding problems, which can result in aspiration of orally ingested food into the lungs or an inability to maintain hydration (especially in children with neurological impairment [NI])

• Failure to thrive as a result of oral motor feeding problems, malabsorption, or a specific disease process (e.g. congenital heart disease, cystic fibrosis, chronic renal failure, cancer)

• Need for an unpalatable elemental diet (e.g. inflammatory bowel disease, various metabolic diseases)

• Feeding aversion that is resistant to other therapy

• Administration of medications (e.g. for human immunodeficiency virus infection)

• Decompression (“venting”) for functional or mechanical bowel obstruction

• As part of palliative care

On occasion, a J or GJ tube may be more appropriate, for example, in the management of severe gastroesophageal reflux disease (GERD) resistant to maximal medical therapy and still symptomatic with NG or G tube feeding. Anatomic or mechanical issues, such as superior mesenteric artery syndrome or gastroparesis, would be another indication for a GJ or J tube (where the tip of the feeding tube is placed distal to the obstruction).

METHOD OF PLACEMENT

There are three well-accepted techniques for placement of enterostomy tubes in children:

• Surgical (open or laparoscopic)

• Percutaneous endoscopic gastrostomy (PEG)

• Percutaneous radiologic gastrostomy (PRG)

Surgical gastrostomy is technically simple and has been performed since the late 1800s. Since the 1990s, an increasing number of these procedures are being performed laparoscopically. PEG, first performed in 1980, is a quick and low-cost method of placing a tube percutaneously under endoscopic guidance by the “pull” or “push” technique, according to the preference of the endoscopist. Percutaneous nonendoscopic or radiologic gastrostomy done under fluoroscopic guidance was first performed in 1981 and has the advantage of being able to be performed in children without general anesthesia if necessary, and does not require endoscopy. PEG is presently the most common technique used in North America.

No study has compared the three techniques in a pediatric population, but a meta-analysis of all patients in the literature who underwent enterostomy over a 15-year period, largely an adult population, was performed to evaluate the effectiveness and safety of the different techniques.¹ Rates of successful tube placement varied; it was 100% for surgical G tubes, 99.2% for PRG, and 95.7% for PEG. Major complications were most common after surgical G tube placement at 19.9%, lower at 9.4% in PEG, and least common after PRG at 5.9%.¹

The technique used often depends on what is most readily available in the particular institution. However, certain conditions may be important to consider. In some medically complex patients, avoidance of general anesthesia may be of significant benefit, thus arguing against a surgically placed tube. However, patients requiring another surgical procedure, such as fundoplication, are better served through surgical tube placement at the same time. In patients with portal hypertension and gastric varices, simultaneous endoscopy may be helpful to avoid puncturing a varix and causing bleeding. Unfavorable anatomy, such as previous gastric surgery or interposition of the liver resulting in absence of a safe access route, is a relative contraindication to the PEG or PRG technique.

The use of prophylactic antibiotics prior to the procedure is generally recommended in many guidelines, although the efficacy of a single dose versus multiple doses and which particular type of antibiotic is not clear.

Many different types of G tubes are available, and no single catheter is appropriate for all patients. Most are secured by a pigtail, balloon, or bulb in the stomach, which helps prevent accidental dislodgement (Figure 182-1). After 6 to 12 weeks the tube can be changed to a low-profile device (e.g. MIC-KEY, Bard button) (Figure 182-2), although low-profile tubes tend to not be available in smaller sizes. They are more cosmetically appealing, tend to move less, and are more difficult to remove accidentally.

NEED FOR ANTI-REFLUX SURGERY

Although G tubes are often placed because of concern about the risk for direct aspiration with oral feeding, it is also possible to aspirate saliva as well as refluxed gastric contents. Many children requiring a G tube (e.g. those with NI) are likely at high risk of having GERD in addition to their oral motor feeding problems and FTT. The risk of developing or exacerbating pre-existing GERD after G tube placement is controversial and has been the subject of a number of studies with differing results. Unless the child’s GERD symptoms are very severe despite maximal medical therapy, fundoplication at the time of G tube insertion is not usually recommended. If severe GERD is present, another option would be to feed via a GJ or jejunal tube. Recent studies suggest that pulmonary outcomes, particularly aspiration pneumonia, are comparable between fundoplication and jejunal feeding.

The ability of reflux testing, particularly in relatively asymptomatic patients, in predicting the need for conversion to a GJ tube or fundoplication, is not well established at this time. Reflux can, of course, be clinically silent, and investigations for GERD are far from perfect. This makes it very difficult to decide what effect tube placement has on the child’s reflux, and studies thus far remain inconclusive.

COMPLICATIONS

Complications can be classified as early (within 30 days of the procedure) or late (after 30 days). They may also be divided into major and minor complications. One clinically useful definition would be that major complications require a significant medical or surgical intervention. Complication rates vary depending on the technique used, with one large meta-analysis quoting major complications with surgical gastrostomy (19.9%), PEG (9.4%), and PRG (5.9%).¹ Minor complication rates can vary greatly, depending on differences in definition, but the same study calculated figures for surgical gastrostomy (9%), PEG (5.9%), and PRG (7.8%).¹

Some of the major complications reported in various series² include:

• Aspiration (as a direct result of the procedure)

• Peritonitis

• Pneumoperitoneum

• Gastrointestinal bleeding

• Severe wound and abdominal wall infection

• Gastrocolonic fistula

• Sepsis

• Death (very rare)

Peritonitis may result from leakage into the peritoneal cavity from around the G tube site or from a dislodged or misplaced tube. Children in whom peritonitis develops after G tube insertion may present with vomiting, fever, irritability, tachycardia, and progressive peritoneal signs, with progression to sepsis and death if not recognized. Early recognition is especially difficult in younger infants and those with NI. If peritonitis is suspected, feeding should be withheld and an urgent contrast study performed through the tube to rule out leakage and confirm tube position. Peritonitis can usually be managed with bowel rest, total parenteral nutrition, empirical antibiotic therapy, and close observation, but surgical intervention is occasionally required.

Minor complications² may include:

• Tube dislodgement

• Tube leakage

• Tube migration

• Tube blockage

• G tube site infection

• G tube site granuloma

• Intussusception (around tip of GJ tube)

Dislodged tubes should be replaced as promptly as possible. Caregivers can be taught to place a Foley catheter to keep the stoma open once the tract has matured. If this is achieved without difficulty and gastric contents can be aspirated, patients can resume feeding through the Foley catheter.

Pericatheter leakage can sometimes become a problem. If the leakage is the result of excessive external tube motion, external fasteners may successfully stabilize the tube. If the stomal site enlargement is due to skin breakdown as a result of infection, antibiotic therapy and replacement with a GJ tube (to minimize fluid contact at the stomal site) may be helpful.

Tubes can migrate into the duodenum or the esophagus (and in the case of GJ tubes, retrogradely into the stomach) and result in obstruction and vomiting. Migration is generally diagnosed by means of a contrast study through the tube.

Tube blockage usually results from failing to flush the tube after feeding or administration of medications, or from certain medications that are notorious for their ability to clog up G and GJ tubes. Flushing the tube with warm water or a carbonated beverage may unblock the tube. Alternatively, blockage can sometimes be corrected with a guidewire, or else the tube will need to be changed. Tubes should be flushed with a minimum of 5 to 20 mL of water after feeding or medications, and medications should be given in liquid format or, if not feasible, then finely crushed and completely dissolved in water before administration through the tube.

Infections around the G tube site can lead to cellulitis and occasionally a subcutaneous abscess. Infection can result in redness, swelling, tenderness, and increased discharge that may be foul smelling. Children rarely have constitutional symptoms, and the infection usually resolves rapidly with local wound care and topical or oral antibiotics. G tube site infections are often polymicrobial, but Staphylococcus aureus and β-hemolytic streptococci are commonly involved, as is fungal superinfection. It is important to distinguish infection from skin redness and irritation caused by sensitivity to acidic gastric contents. The latter may respond to a nonmedicated barrier cream or benefit from acid-suppressing agents. The skin can also become reddened because of a reaction to the tape used to hold the tube in place. Granulation tissue around the stoma is caused by an overgrowth of dark pink “cauliflower-like” tissue, which bleeds easily but generally responds to repeated applications of silver nitrate.

Intussusception of the small bowel is not uncommonly seen with certain types of GJ tubes, especially in young infants and in the presence of a tube with a distal pigtail. This complication should be suspected in patients with a GJ tube presenting with bilious vomiting and feed intolerance. The diagnosis of intussusception can be made by ultrasonography or fluoroscopy, and it is usually managed by changing the tube and replacing with a shorter GJ tube or removing the distal pigtail, or both.

OUTCOMES

It is generally assumed that enterostomy feeding is a necessary, safe, and effective intervention in the group of complex medical patients with oral motor feeding problems. The finding in some earlier cohort studies of a higher death rate and an increase in GERD in children with cerebral palsy (CP) who were fed by gastrostomy is cause for concern. It has been hypothesized that the increased mortality may be due to an increase in pulmonary disease secondary to overly vigorous nutritional maintenance and subsequent aspiration. Unfortunately, no randomized controlled trials of enterostomy tube feeding have been conducted in children with NI, which raises the question of whether the control group was really equally severely disabled before the intervention. This is supported by noting that the association between feeding and death was substantially reduced when other confounding factors were controlled for. Most studies investigating the risks and benefits of tube feeding in neurologically impaired children are limited by methodologic weaknesses, thus making it difficult to draw any firm conclusions in this regard.

Based on the literature to date, it appears that children generally show improved weight gain and improved triceps skinfold thickness after initiation of G tube feeding. Several case series have suggested that earlier intervention may be associated with greater nutritional benefits. Change in the rate of linear growth and effects on bone mineralization and density is less predictable in the population of children with CP.

Caregivers of children with CP generally report an increase in the ease of feeding and a decrease in the time taken up by feeding after enterostomy tube placement. They usually see an improvement in the child’s disposition and nutrition and often report that they are pleased with the G tube and enhancement of the child’s comfort and abilities. There are also caregivers who report on the difficulty of getting respite care because of the tube feeding, as well as the restricted mobility if pump feeding is required. A recent prospective observational study reported that in a population of children with NI, there was no significant improvement in caregiver perception of their child’s quality of life despite their improved nutritional status.

TRACHEOTOMY

Tracheotomy has been described since antiquity, with sources from ancient Persia and Egypt documenting its use as long as 5000 years ago. The first pediatric report dates from 1808, with most subsequent uses of tracheotomy being in the setting of acute infections such as diphtheria and laryngotracheobronchitis.³ In the early twentieth century, tracheotomy was utilized to manage chronic respiratory failure due to polio, and while the use of tracheotomy for managing upper airway obstruction due to acute infection has declined due to immunization and other advances, the last decades have seen an increase in the utilization of tracheotomy to manage chronic respiratory failure usually from underlying neurological conditions. The following sections outline the indications, complications, and method of replacing a tracheostomy tube.

INDICATIONS

The three main indications for a tracheotomy are to bypass airway obstruction (obstructive and/or central), to facilitate long-term invasive mechanical ventilation, and to provide tracheobronchial toilet. The most common age for a tracheotomy to be performed is in the first year of life usually in the context of airway obstruction. Obstruction may occur above, at, or below the level of the glottis or vocal cords. Obstruction above the level of the glottis may result from various craniofacial abnormalities (e.g. Treacher Collins syndrome). Vocal cord palsy causes obstruction at the level of the glottis, and subglottic stenosis often resulting from prolonged intubation in the neonatal period remains a common indication for tracheotomy. While infants and children are felt to tolerate longer periods of intubation than adults, tracheotomy is considered when it is felt that prolonged endotracheal intubation may interfere with normal development (feeding, expressive communication) and lead to progressive laryngotracheal trauma.⁴ While a tracheotomy does not prevent acute infection and lung damage from aspiration, it does assist suction and clearance of secretions. If prolonged invasive ventilation is desired, tracheotomy facilitates the care of children outside of an institutional setting.⁵

The potential complications that may result from a tracheostomy are outlined in Table 182-1. Table 182-2 highlights the commonly encountered problems and actions for managing frequently observed tracheostomy related concerns. Please see Chapter 197 (“Replacing a Tracheostomy Tube) for guidance in changing a tracheostomy tube.⁶

REFERENCES

BACKGROUND

With medicine’s increasing capabilities, situations arise in which possible interventions will not serve the patient’s or the family’s goals of care. Under such circumstances, the decision to forgo treatment, even potentially lifesaving treatment, may be appropriate. This chapter discusses one means of limiting treatment: do-not-attempt-resuscitation (DNAR) orders. It describes the historical development of DNAR orders, outlines the process of writing such orders, and explains why they should generally not be written unilaterally. The related issues of family presence during resuscitation and DNAR orders in the operating room and outside the hospital are also reviewed.

CARDIOPULMONARY RESUSCITATION AND DO-NOT-ATTEMPT-RESUSCITATION ORDERS

Do-not-resuscitate (DNR) orders developed out of the recognition that cardiopulmonary resuscitation (CPR) lacks efficacy in certain patient populations and that a formal process of advance planning was needed. Although modern CPR was initially developed for patients suffering anesthesia-induced cardiac arrest, it became the standard of care for cardiac arrest in hospitalized patients regardless of their underlying diagnoses. Experience, however, demonstrated that the effects of CPR were often transient. In some institutions, covert decision-making processes evolved to withhold or limit resuscitation efforts. Hospitals developed DNR policies in the 1970s to address the need for both a decision-making process and a means to communicate these decisions.¹

There are limited data in the pediatric literature regarding the efficacy of CPR in hospitalized patients. In reviewing the literature it is important to focus on patient-centered outcomes. A retrospective review of data from the National Registry of Cardiopulmonary Resuscitation (NRCPR) found that 52% of children who experienced an in-hospital cardiac arrest resuscitation (pulseless cardiac arrest requiring chest compressions, defibrillation, or both, that elicited an emergency resuscitation response and resulted in a resuscitation record) had return of spontaneous circulation for greater than 20 minutes and 27% survived to hospital discharge. Of the children who survived to discharge, 65% had a good neurological outcome defined as a Pediatric Cerebral Performance Category of normal functioning, mild disability, or moderate disability.²

A retrospective review of in-hospital cardiac arrests in 15 children’s hospitals within the Pediatric Emergency Care Applied Research Network (PECARN) found that 48.7% of patients between 1 day and 18 years of age who received greater than 1 minute of chest compressions and had return of circulation for at least 20 consecutive minutes survived to hospital discharge.³ This compares to 51% in the NRCPR study.² Using different criteria, the PECARN investigators found a higher rate of good neurological outcomes; among survivors who had prearrest and discharge Pediatric Cerebral Performance Category scores available, 94.3% had discharge scores of normal or mild disability or no change in score.³

Information regarding the efficacy of CPR within specific diagnostic categories is even more limited. The PECARN study found pre-existing hematologic, oncologic, or immunologic disorders and pre-existing genetic or metabolic disorders were associated with increased hospital mortality.³

Based on the limited efficacy of CPR, some authors prefer the term do-not-attempt-resuscitation (DNAR).⁴ Reinforcing that many resuscitation attempts are not successful is important because families may have false high expectations of the efficacy of CPR based on television’s depiction of CPR.^5,6 More recently, the term allow natural death (AND) has been proposed. Proponents argue that DNR sounds cold and cruel, and that AND is warmer and more comforting.⁷ A single study reports that nursing students and other college students, but not nurses, are statistically more likely to endorse an AND order than a DNR order in response to a near-death scenario.⁸ Critics of this proposed change argue that unlike DNAR, AND does not convey which specific procedures will be withheld. For example, it is not clear whether an AND order precludes therapeutic treatments such as vasopressors and antibiotics.^9,10

Even if CPR might be effective, there may be situations in which it is ethical to withhold it. For example, in terminally ill patients, it may only prolong the dying process. More controversially, the patient’s quality of life may be so diminished that it need not be maintained.

The Patient Self-Determination Act, which became effective in 1991, requires most health care institutions to ask adult patients on admission whether they have advance directives; however, there are few mechanisms for parents or guardians to express their wishes for their children or for adolescents to express their wishes should they lose medical decision-making capacity.^11,12 Hospitalists should review the goals of treatment—curative, uncertain, or primarily comfort—with the families of children with special healthcare needs (Table 183-1). The patient’s primary care provider may be a valuable resource, especially if he or she has a longstanding or close relationship with the patient and family and has discussed treatment goals with them. Discussing the goals of treatment is particularly important when significant changes occur in the child’s clinical condition. If there is a possibility of cardiorespiratory arrest, especially if there is a low likelihood of survival or survival would not be in the child’s best interest, the hospitalist should discuss the risks and benefits of CPR, and a DNR order should be considered. This difficult topic can be broached by asking, “What is your hope for your child?”

DNR orders generally prohibit CPR regardless of the cause of the arrest. If the DNR order is based on a low likelihood of survival and if different causes of arrest have different survival rates, this should be discussed.¹³ For example, a family might desire intubation and mechanical ventilation for respiratory depression that is a side effect of anticonvulsants.

Hospitalists should be cautious about permitting families to pick and choose components of CPR. For example, administering medications while withholding chest compressions lacks a pathophysiologic rationale; the medications cannot be effective if they are not circulated. It may, however, be reasonable to specify a limited duration of resuscitation efforts.

The hospitalist should carefully document conversations with the patient and the patient’s family in the medical record. This is particularly important because of the limited continuity within some hospitalist systems. The hospitalist should also write a formal order, ensuring that it is internally consistent. DNAR orders should be reviewed periodically and any time there is a significant change in the patient’s medical condition. Hospitalists should communicate the substance of their discussions to primary care providers and complete out-of-hospital DNAR orders (see later) if appropriate at the time of discharge.

DNAR orders should not be interpreted to represent a broad decrease in the intensity of care, especially when such limitations have not been discussed and agreed upon.¹⁴ DNAR orders should be implemented in the context of palliative care, including managing pain and symptoms and addressing emotional and spiritual needs (see Chapter 10).¹⁵

Hospitalists should be familiar with the policies of the hospitals and the laws of the states where they practice. Hospitals may require the attending physician to write the initial DNAR order, prohibit verbal or telephone orders, and specify how frequently the order must be renewed. States may restrict the circumstances in which a DNAR order can be entered, such as only if the patient is terminally ill. These statutes are controversial; some consider them unconstitutional or unethical.¹⁶

Evidence of low survival rates after CPR in specific patient populations has led some authors to argue that it is futile and that physicians can unilaterally withhold it.¹⁷ Definitions of futility are problematic, however, because they incorporate values that patients and families may not share. The disputed procedure generally has a physiologic effect, but parties disagree on what likelihood of success justifies the procedure or the value of the outcome to the patient. Permitting physicians to unilaterally withhold treatment violates patients’ autonomy. Such decisions are particularly problematic because physicians tend to undervalue the quality of life of individuals with disabilities.^18,19 Disagreements between patients or other decision makers and the medical staff have led to the implementation of specific procedural steps to mediate such disagreements or to facilitate the transfer of care to a physician willing to carry out the family’s wishes.²⁰

FAMILY PRESENCE

There is a growing move to permit families to be present during resuscitation attempts. For example, the American Heart Association’s PALS Provider Manual states, “… healthcare providers should offer the opportunity [for family members to be present during the attempted resuscitation of a loved one] whenever possible.”²¹ It should be noted that family presence protocols are structured and include an assessment of the patient and the family, preparation of the family for the event, and support of the family by a trained facilitator during and after the experience.²² Initial studies report that family presence does not delay essential care and family members believe their presence was valuable.²³

OTHER CONTEXT

ANESTHESIA AND SURGERY

DNR orders during anesthesia and surgery may be problematic because anesthesia promotes some degree of cardiovascular instability, and it can be difficult and artificial to separate anesthesia from resuscitation. Additionally, procedure-specific DNAR orders may restrict anesthesiologists’ and surgeons’ discretion in cases of unexpected but easily reversible events. Professional organizations contend that DNAR orders should not be automatically rescinded in the operating room but should be reevaluated. Hospitalists should review the DNAR order and treatment goals with the patient and his or her parents, as well as the anesthesiologists and surgeons, before sedation or surgery. If the DNAR order is suspended, a temporal endpoint should be established.^24,25

OUTSIDE THE HOSPITAL

Children with special health care needs live in many contexts: they may live at home or in an extended-care facility, they may attend school, and they may be hospitalized intermittently. The efficacy of CPR in the outpatient setting is lower than the inpatient setting; a prospective population-based cohort study in the United States and Canada reports that 6.4% of individuals less than 20 years of age who experienced out-of-hospital nontraumatic cardiac arrest survived to hospital discharge.²⁶ This study unfortunately does not report the patient’s neurological outcomes.

Sometimes the families of children with DNAR orders may legitimately need to activate the emergency medical services (EMS) system. For example, the child could be in status epilepticus or suddenly develop distressing symptoms. Performing CPR is, however, the default for first responders and EMS personnel. Many states authorize “portable” or out-of-hospital DNAR orders that EMS personnel are required to comply with and that protect them from liability for doing so. Some programs are applicable to minors. Hospitalists should be aware of state regulations and complete such documents if available.²⁷ Physician Orders for Life-Sustaining Treatment (POLST) are one example. This form not only includes a section for CPR but also sections for medical interventions, antibiotics, artificially administered nutrition, summary or medical conditions, and contact information. Choices within the medical interventions section include comfort measures only, limited additional interventions, and full treatment.²⁸ If out-of-hospital DNAR orders are not available, hospitalists should discuss the risks and benefits of calling 911 with the family. Code status should also be documented on the child’s emergency information form, which includes a concise summary of his or her medical condition(s), medications, and special health care needs.^29,30 Out-of-hospital DNAR orders and emergency information forms may also facilitate patients’ readmission to the hospital.

Children with chronic or terminal conditions who are at risk of dying may also attend school. The American Academy of Pediatrics recommends that pediatricians work with school nurses to incorporate a specific action plan into the student’s individualized heath care plan.³¹ Hospitalists must continue to define their role in such conversations, particularly when patients do not have a functional medical home.

REFERENCES