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.
TABLE 181-1Range of Comorbid Conditions in Children with Neurologic Complexity ||Download (.pdf) TABLE 181-1 Range of Comorbid Conditions in Children with Neurologic Complexity
|Affected System ||Potential Complications |
|Neurologic || |
|Development/neuropsychiatric || |
|Sensory || |
Cortical visual impairment
Sensorineural hearing loss
Conductive hearing loss
|Growth, nutrition, and gastroenterology || |
Poor weight gain/malnutrition
Poor gastric emptying
Gastroesophageal reflux disease
|Pulmonary || |
Restrictive lung disease/hypoventilation
Chronic inflammatory lung disease
Recurrent aspiration pneumonias
|Upper airway || |
Obstructive sleep apnea
Upper airway anomalies
|Musculoskeletal || |
Altered muscle tone
Progressive neuromuscular scoliosis
Spasticity related discomfort
|Endocrine || |
Hypothalamic-pituitary axis disturbances
Growth hormone deficiency
|Renal/urologic || |
Recurrent urinary tract infections
|Skin ||Decubitus ulcers |
|Dental || |
Poor oral hygiene
COMMONLY ASSOCIATED COMORBID CONDITIONS BY SYSTEM
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.
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.
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.
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 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.
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.
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.
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
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.
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.
TABLE 181-2Common Causes of Chronic Pain ||Download (.pdf) TABLE 181-2 Common Causes of Chronic Pain
|System ||Specific Cause |
|Musculoskeletal ||Fractures, scoliosis, hip dislocations |
|Gastrointestinal ||Gastritis, gastric ulcer, esophagitis, chronic constipation, gallstones, gas pain |
|Neurology ||Spasticity, headaches, seizures, neuropathic |
|Somatological ||Dental caries |
|Gentitourinary ||Urinary retention, renal stones, menstrual cramps |
|Iatrogenic ||Multiple surgeries and therapies |
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-documented31,32 (Table 181-3). Assessing the need for additional psychosocial support for family members must be a routine part of evaluation.
TABLE 181-3Common Familial Stresses in Children with Neurologic Complexity ||Download (.pdf) TABLE 181-3 Common Familial Stresses in Children with Neurologic Complexity
|General Stressor ||Specific Issues |
|Financial hardship || |
Increased medical costs
Durable medical equipment
Copayment for medical visits
Family job loss
|Coordination and communication challenges ||Multiple providers (appointments), agencies, and insurers |
|Medical concerns || |
Waxing and waning symptoms
Lack of certainty
|Physical and emotional exhaustion || |
Child may have sleep disturbance
|Lack of privacy || |
Therapists and nurses in home
Publically shared information about child
|Lack of community supports/services ||Constant need for advocacy |
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.
Among children with severe neurologic impairment, even when the underlying diagnosis is not secure, providers can conduct broad reviews for the commonly identified comorbid conditions, intervene as appropriate, advocate for therapeutic and educational services for patients, and work to promote a sense of patient and family wellbeing. Ultimately, most of the underlying neurologic conditions in this population are not reversible, and can be challenging to manage. Successful care for these children requires integration of a multidisciplinary team with the family and primary provider(s) at the hub, with multiple spokes to subspecialists, ancillary medical staff, therapists, educators, and community agencies (Figure 181-1). With diligent, thoughtful care and teamwork, providers can work to prevent or slow complications and maximize each child’s quality of life.
IJ. Autonomic complications following central nervous system injury. Semin Neurol. 2008;28(5).
MJ, Van Cleave
A. Understanding factors associated with work loss for families caring for CSHCN. Pediatrics
M, Lorenz, Reimnitz
H; German Lung Abscess Study Group. Moxifloxacin
/sublactam in aspiration pneumonia and primary lung abscess. Infection
IJ. Paroxysmal sympathetic hyperactivity after acquired brain injury: a review of diagnostic criteria. Brain Injury
R. Clinical prognostic messages from a systematic review on cerebral palsy. Pediatrics. 2012;130(5):1285–1312.
PW. The changing landscape of disability in childhood. Future Child
TG. Treatment of epilepsy in children with developmental disabilities. Dev Disabil Res Rev
C. Self-injurious behavior in people with intellectual disability. Curr Opin Psychiatry
C. Oromotor dysfunction and communication impairments in children with cerebral palsy: a register study. Dev Med Child Neurol
GN. Evidence that children with special needs all require visual assessment. Arch Dis Child
PB. Gastrointestinal disorders in children with neurodevelopmental disabilities. Devel Disabil Res Rev. 2008;14:128–136.
RD. Growth and nutrition disorders in children with cerebral palsy. Dev Disabil Res Rev
JH. Pulmonary care of children and adolescents with developmental disabilities. Pediatr Clin North Am
PB. Fundoplication versus postoperative medication for gastro-oesophageal reflux in children with neurological impairment undergoing gastrostomy. Cochrane Database Syst Rev. 2007;(1):CD006151.
et al. Impact of fundoplication versus gastrojejunal feeding tubes on mortality and in preventing aspiration pneumonia in young children with neurologic impairment who have gastroesophageal reflux disease. Pediatrics
J, Van Aspergen
PP. Assessing and managing lung disease and sleep disordered breathing in children with cerebral palsy. Pediatr Respir Rev. 2009;10:18–24.
H. Management of drooling in children. Arch Dis Child Educ Pract Ed.
I. Use of botulinum neurotoxin A in uncontrolled salivation in children with cerebral palsy: a pilot study. Int J Oral Maxillofac Surg
J. Salivary gland surgery for control of chronic pulmonary aspiration in children with cerebral palsy. In J Pediatr Otorhinolarynogol. 2009;73:1192–1194.
JL. Laryngotracheal separation surgery for intractable aspiration: our experience with 12 patients. Int J Pediatric Otorhinolaryngolog. 2011;75(73):931–934.
A. Management of spasticity in children with cerebral palsy. Semin Pediatr Neurol.
P. Growth hormone deficiency and cerebral palsy. Therapeut Clinical Risk Manage. 2010;6:413–418.
SB. Bone health in children with cerebral palsy and epilepsy. J Pediatr Health Care
E. Urodynamic findings in children with cerebral palsy. Int J Urol
Oliveira CA, de Souza
MB, de Aruajo
Castro GF. Dental needs and management of children with special health care needs according to type of disability. J Dent Child. 2012;79(303):165–169.
et al. Unmet dental needs and barriers to care for children with significant special health care needs. Pediatr Dent
K. Sleep in children with autistic spectrum disorder. Sleep Med
K. Sleep problems and the need for parental night-time attention in children with physical disabilities. Child Care Health Dev
et al. The efficacy of melatonin for sleep problmes in children with autism, fragile X syndrome, or autism and fragile X syndrome. J Clin Sleep Med.
S. Update on the use of melatonin in pediatrics. J Pineal Res
E. Pain in cognitively impaired children: a focus for general pediatricians. Eur J Pediatr
C. Care coordination for CSHCN: association with family-provider relations and family/child outcomes. Pediatrics. 2009;124(S4):S430–434.
et al. The health of primary caregivers of children with cerebral palsy: how does it compare with that of other Canadian caregivers? Pediatrics. 2004;144(2):e182–191.
A. Sublingual atropine
drops for the treatment of pediatric sialorrhea. J Pain Symptom Manage.