Chapter 146: The Child With Special Healthcare Needs

Children with special healthcare needs are children with chronic physical, developmental, behavioral, or emotional conditions that require health and related services beyond what is required by children in general.^1,2 This population accounts for about 16% of children in the United States,^3,4 accounts for 40% to 80% of all pediatric healthcare utilization and costs, and is the fastest-growing group in pediatrics.^3,5,6,7,8,9 Compared to children without chronic conditions, children with chronic conditions tend to be older, male, and from non-Hispanic white racial/ethnic groups.^4,6,10,11 The clinical spectrum of conditions is diverse, and complex medical devices are often required for care (Tables 146-1 and 146-2).^{6,8,9,10,12,13,14}

An emergency care plan provides information and instructions for emergency care. Components of the plan include an information form, special instructions for devices or emergency conditions, and ancillary replacement supplies.

The individualized emergency information form should be provided at the ED visit (http://www.childrensnational.org/files/PDF/EMSC/PubRes/EIForm.pdf). Informational elements of the form include demographic and emergency contact information; names and contact information for the primary and specialty care physicians; diagnoses, past procedures, and baseline examination findings; allergies; immunizations; procedures to avoid; common acute problems and suggested management; and comments on other specific medical issues or special instructions for device malfunction. Information should be regularly updated. Copies of the form should be available at the home, physician's office, and care facilities.

The care plan should include special instructions for EMS personnel, first responders, or family members who may need to provide emergency care for the child.^12,13 A "go-bag" or kit with specialized equipment should accompany the child. Such kits contain extra tracheostomy tubes, appropriate size suction catheters, equipment to change a tracheostomy tube, syringes and adapters to decompress a feeding catheter, a bag-valve mask resuscitator, and needles to access central lines.¹⁷

Optimal emergency care requires access to multiple healthcare resources, including family, primary care physicians, specialists, and home health nurses (Table 146-3).^12,13,17 Long-term caregivers know the child's baseline status and are familiar with medications and supportive equipment.

HISTORY AND PHYSICAL EXAMINATION

The child may not be able to communicate directly. Elicit essential elements of the history and subtle symptoms from family or care providers; occasionally, a call to a chronic care facility or review of past medical records is necessary to obtain important details. Inquire about advanced directives, limitations of care, and family goals as well as treatments prior to ED arrival. Carefully review medications including recent changes or missed doses, allergies (e.g., latex), and comorbidities. The physical examination, too, often relies on accompanying family or care providers, particularly with regard to mental status, assessment of pain, and changes from baseline. Obtain vital signs. Baseline vital signs for children with complex medical conditions may be out of the range of normal. Core temperature may be low at baseline, and a "normal" temperature may represent fever; baseline values for heart rate, respiratory rate, and oxygen saturation may be abnormal; vital signs may be controlled by a pacemaker or ventilator. Obtain an accurate weight to calculate weight-based medication dosing. To obtain a current weight, if a conventional floor scale is not appropriate, ask caregivers for the most recent weight, use a bed scale, or consider a length-based tape to estimate weight. When performing a head-to-toe evaluation, pay special attention to medical devices and equipment: palpate ventricular shunts for swelling or tenderness; check patency of tracheostomy tubes; assess central or peripherally inserted vascular catheters for patency and signs of infection; and examine gastrostomy sites and feeding tubes.

LABORATORY TESTING AND IMAGING

The specific evaluation depends on the presenting problem and underlying special healthcare needs. Perform bedside glucose testing in all complex patients with altered mental status. Check serum drug levels (e.g., anticonvulsants) as appropriate. Compare plain radiographs and CTs with prior imaging results to distinguish acute from chronic changes.

Although treatment is tailored to the presenting condition, the universal approach of prioritizing airway, breathing, circulation, and disability remains the same. A general overview of common respiratory, metabolic, neurologic, GI, and musculoskeletal problems and suspected abuse follows. A review of the complications common to children dependent on medical technology is discussed separately later, under "Technology-Dependent Children."

RESPIRATORY DISORDERS

Oral motor dysfunction and gastroesophageal reflux can lead to aspiration, wheezing, pneumonia, and chronic congestion. Scoliosis and neuromuscular disease may compromise pulmonary mechanics and lead to functional restrictive lung disease with limited respiratory reserve. To evaluate acute respiratory complaints, obtain a thorough history of past respiratory function, especially in the situation of prematurity; escalating home therapy including supplemental oxygen use; reactive airway disease; or tracheomalacia. Airway instability can develop in the child with poor head control, and simple head repositioning or jaw thrust may improve airway mechanics and decrease pooling of saliva in the oropharynx. Suction and change tracheostomies as needed. Consider foreign body aspiration in appropriate clinical circumstances. Treatment of respiratory distress in this population may include deep suctioning, replacement of malfunctioning equipment (tracheostomy tubes, ventilators), β-agonists for bronchospasm, antibiotics for pneumonia, and subspecialty consultation for airway foreign bodies. Admission is generally warranted for pneumonia, increased home oxygen requirement, persistent respiratory distress, or removal of a foreign body.

METABOLIC DISORDERS

This diverse category of disorders is due to genetic defects that lead to abnormalities in the metabolic pathways of proteins, carbohydrates, and lipids (see chapter 144, "Metabolic Emergencies in Infants and Children"). Although a detailed understanding of individual biochemical pathways is not necessary for emergency management and evaluation, general familiarity with presenting signs and symptoms is necessary, because recognition in the ED could be lifesaving. Initial laboratory studies should include a CBC, serum glucose, electrolytes with anion gap, blood gas, ammonia, liver function tests, urine ketones, and lactate dehydrogenase, aldolase, and creatine kinase if there are muscular symptoms. After verifying adequate airway, breathing, and circulation, direct care to correct acute metabolic abnormalities such as hypoglycemia (Table 146-4).¹⁸

Consult a biochemical geneticist for further diagnostic evaluation and management guidance.

SEIZURES

The prevalence of epilepsy is estimated at 4 to 9 cases per 1000 children but is as high as 35% among children with special needs.^19,20,21 In general, the nature and complexity of the seizure syndromes differ considerably between patients, but seizure syndromes are generally consistent in the individual. The initial evaluation and management follow the standard approach to seizures (see chapter 135 "Seizures in Infants and Children"), with a few caveats in the child with special healthcare needs: note anomalies or comorbid conditions that might lead to airway or respiratory compromise with the use of respiratory depressant anticonvulsants; obtain a careful history including the baseline frequency and type of seizures and efficacy of past medications (ask the child's family what has and has not worked previously); confirm allergies and potential drug interactions; and consult with the primary neurologist if multiple medications are needed for seizure control and determine if therapeutic adjustments are required. Obtain serum anticonvulsant medication levels as indicated.

Adequate seizure control may require multiple medications. IntracTable seizures, those that do not respond to at least two anticonvulsants, affect approximately 1 in 10 children with epilepsy²² and may be associated with underlying disorders or syndromes. Patients with refractory seizures, including those with epileptic encephalopathies (e.g., Lennox-Gastaut and Dravet's syndromes) may be placed on a ketogenic diet or have an implanted vagus nerve stimulator. For children on a ketogenic diet, it is critical to avoid sugars and maintain ketosis. Therefore, ask caregivers about baseline serum glucose levels before administering dextrose, as dextrose may increase seizures previously controlled by therapeutic ketosis. In addition, consider working with a pharmacist to ensure that the vehicle in which a medication is provided is not contraindicated for a ketogenic diet. For example, most chewable and suspension medications and common medications such as acetaminophen and ibuprofen contain sugar.

GI DISORDERS

GI complaints may include dehydration, feeding tube complications, and constipation. Dehydration may result from poor oral intake, oral motor dysfunction, or increased fluid losses from infection. Children with growth retardation (in particular below the fifth percentile) or marginal reserves may dehydrate even from minimal vomiting and diarrhea. In addition to the clinical signs of dry mucous membranes, tachycardia, and oliguria, ask the family about weight loss, changes in baseline function, and ability to orally or enterally hydrate. IV access may be difficult, and the child may require central or intraosseous access (see chapter 112, "Intravenous and Intraosseous Access in Infants and Children"). Constipation may be associated with nonspecific symptoms such as abdominal pain, change in bowel habits, anorexia, or overflow diarrhea. Treat acute constipation with enemas, suppositories, polyethylene glycol, and dietary adjustments. Avoid mineral oil in children with aspiration potential to avoid pulmonary complications and impaired fat-soluble vitamin absorption. Do not recommend chronic use of Fleet enemas because electrolyte abnormalities may result.

MUSCULOSKELETAL/DERMATOLOGIC DISORDERS

Children with disorders that require orthopedic braces, have prolonged periods of immobility, or result in wheelchair dependence may develop painful skeletal or cutaneous complications. The risk of pathologic fractures increases from disuse and nutritionally induced osteopenia. Although fractures may occur from therapy, falls, or accidents, also consider abuse, particularly for comminuted fractures. Assess for orthopedic injuries in the irritable, severely impaired, or nonverbal child by palpating all extremities and checking range of motion. Inspect the skin, especially in dependent areas, for pressure ulcers or skin breakdown. Evaluate wheelchair-dependent children on stretchers and out of their chairs to check the entire body.

NEGLECT AND ABUSE

Children with disabilities are three to four times more likely to be neglected or abused when compared with children without disabilities.²³ They often place higher emotional, physical, economic, and social demands on their families.^24,25,26 The added attention required, coupled with limited caregiver resources, can result in failure of the child to receive needed medications, medical care, and appropriate educational placement. Maintain a heightened awareness for potential abuse, and work closely with the primary physician who is most familiar with the child and his or her care providers.

A technology-dependent child is one who needs a medical device to compensate for the loss of a vital body function and substantial nursing care to avert death or further disability.²⁷ Devices include ventilators, pacemakers, tracheostomies, gastrostomy tubes, central venous catheters, ventriculoperitoneal shunts, and vagus nerve stimulators.^27,28 Contact the child's primary physician and home health nurse early in the evaluation process to avoid unnecessary tests, to determine need for inpatient admission, and to simplify care.

TRACHEOSTOMY CARE

There are many types and sizes of tracheostomy tubes, so clarify the exact type and size of tube. Common complications related to tracheostomy tubes include decannulation, bleeding at the cannulation site, obstruction, and tube reinsertion into a false passage, resulting in subcutaneous emphysema, pneumomediastinum, or pneumothorax. Granuloma or stricture formation at the stoma or tracheal wall can cause bleeding with cannula manipulation or by suctioning. Erosion into the innominate artery is rare and is usually related to an inferiorly placed tracheostomy stoma. Consider otolaryngology consultation and urgent bronchoscopy for bleeding.

Infections associated with tracheostomies include tracheitis, bronchitis, and pneumonia. Normal secretions are generally clear to white and thin in consistency. Abnormal secretions are thick and yellow to green in color. Obtain a tracheal aspirate and culture in children with fever, respiratory distress, or a change in secretions or oxygen requirement.

MECHANICAL VENTILATION

Chronic respiratory support is necessary for a variety of conditions: neurologic and neuromuscular disease accounts for about half, and chronic lung disease accounts for only 7%.²⁹ Ventilator-related complications, such as pneumothorax or machine failure, are managed according to standard principles. Provide bag ventilation for total ventilator failure. Table 146-5 provides a checklist for ventilator troubleshooting.

FEEDING TUBES

Complications of a nasogastric tube include sinusitis, nasal and esophageal irritation, tube dislodgement or clogging, and pulmonary aspiration. Gastrostomy tubes can be associated with gastroesophageal reflux, tube dislodgement or clogging, peristomal wound infection, abdominal wall abscess, peritonitis, gastric perforation or hemorrhage, gastrocolic fistula, gastric ulceration, and gastric outlet obstruction. The gastrostomy tube may become dislodged or broken and can be difficult to reinsert if left out for any period of time. Insert a small feeding tube or Foley catheter to prevent stoma closure while arranging for the appropriate tube to be reinserted (see chapter 86, "Gastrointestinal Procedures and Devices"). Additional complications of gastrojejunostomy tubes include diarrhea, tube migration, small bowel perforation, and intussusception (see chapter 87, "Complications of General Surgical Procedures").

Stomal complications include dermatitis, allergic hypersensitivity, granulation, cellulitis, and fungal infections. Superficial bleeding due to granulation tissue can be cauterized using silver nitrate sticks.

Complications of parenteral feeding include catheter obstruction or occlusion, air embolism, catheter breakage, catheter displacement, or catheter-related infection. Other complications include cholestasis that may lead to irreversible liver disease or metabolic bone disease.

INDWELLING VENOUS CATHETERS

Indwelling catheters, often tunneled central venous catheters, can be of multiple types but will be accessible through either externalized catheter tubing, such as a Hickman device, or a subcutaneous reservoir, such as a Port-a-Cath device.³⁰ Complications include occlusion, breakage, dislodgement, air embolism, and infection. Various agents can be used to relieve catheter obstruction in the ED, such as tissue plasminogen activator in the instance of intraluminal coagulated blood, but protocols are typically institution dependent. Surgical or interventional radiology consultation may be necessary if initial attempts at removing the obstruction are unsuccessful. For breakage or dislodgement, clamp externalized catheters proximal to the affected segment and repair with a special kit if available. For nonexternalized catheters, especially when there is concern for dislodgment, seek surgical consultation promptly. Air embolism may be associated with symptoms of respiratory distress and hemodynamic instability. If air embolism is suspected, clamp the catheter and place the child in left-sided Trendelenburg position with supplemental oxygen.³⁰ For suspected infection, obtain blood cultures and administer antibiotics to cover gram-positive organisms, which are the most common pathogens.

CEREBROSPINAL FLUID SHUNTS

Cerebrospinal shunt placement is the most common neurosurgical procedure performed in children.³⁰ Complications include separation or disruption of the tubing or device, infection, and overdrainage^31,32 (Table 146-6). Shunt malfunction occurs most often in the year following placement,³³ with approximately 40% of standard shunts malfunctioning during this time.³⁴ After the first year, the annual rate of shunt malfunction is about 5%.³⁵

Seizures are generally accompanied by other signs and symptoms and are seldom, if ever, the only sign of shunt malfunction. Patients with impending herniation may develop Cushing's triad: hypertension, bradycardia, and irregular respirations. This is a late sign of impending herniation and a true neurosurgical emergency. When neurosurgical consultation is not immediately available and the patient is experiencing symptoms of herniation, removing cerebrospinal fluid through the shunt reservoir may be lifesaving.

Shunt infections occur most commonly within a few months of shunt placement, many within the first few weeks, and they generally decrease in frequency with time. Infection rates vary from 1% to 40%, with most in the range of 5% to 15%.³⁶ Signs and symptoms of shunt infection may be nonspecific, such as fever, behavior change (irritability or lethargy), vomiting, and/or abdominal pain. Infected shunts may be accompanied by mechanical shunt failure. Antibiotics should be selected to treat gram-positive (most commonly, coagulase-negative Staphylococcus, Staphylococcus aureus, and Streptococcus) and gram-negative (Escherichia coli, Enterococcus species, and Haemophilus influenzae) species. Intra-abdominal cerebrospinal fluid loculations or peritoneal pseudocysts can occur at the distal tip of the catheter with or without peritonitis. Less common intra-abdominal complications are bowel obstruction due to adhesions, subphrenic abscess, and cerebrospinal-enteric fistula.³⁷

The evaluation for mechanical shunt malfunction includes plain radiographs of the skull, neck, chest, and abdomen to evaluate continuity of shunt hardware, and head CT or quick-brain MRI to assess ventricular size and shunt positioning. Increased cerebral ventricle size, particularly when compared with previous studies, indicates shunt malfunction. However, malfunction may be present with unchanged ventricular size due to a loss of surrounding tissue compliance. A "negative" neuroimaging study does not rule out shunt malfunction or obstruction. Approximately 11% of shunt failures present with small ventricles, and neuroimaging should not be used as the sole or definitive diagnostic modality when evaluating shunt function. If shunt malfunction or infection is suspected, consult neurosurgery for further evaluation, including shunt aspiration, nuclear medicine flow studies, admission, and monitoring. Programmable shunts will need to be reprogrammed after MRI because the magnet alters the set pressure level. Programming requires an on-site, trained provider.

URINARY DIVERSIONS

Urinary diversions include vesicostomies, ureterostomies, ileal loop conduits, and bladder augmentations. Complications include prolapse and stomal stenosis in vesicostomies, stenosis of ureterostomies, pyelonephritis, stricture of the ureteroileal anastomosis, peristomal hernia, or stenosis in ileal loop conduits. Small bowel obstruction can be a complication of constriction of the ileal conduit. Most complications are managed in consultation with the child's urologist.

AUTISM SPECTRUM DISORDER

Autism spectrum disorder is a category of disorders characterized by restrictive, repetitive, and stereotyped patterns of behaviors with impaired social interaction and communication.³⁸ The disorder has a wide variety of clinical expressions, severity, and levels of function. The increase in prevalence over the past few decades may be due to improved and earlier detection.³⁹ The current prevalence is about 1 in 88 children.⁴⁰ The cause is unknown, but there is no relationship with childhood immunizations. In general, most children with autism spectrum disorder do not have associated medical disorders and have medical needs similar to those of age-matched, normally developing children. Sensory defensiveness, unusual social behaviors, and aggressive self-protective responses to medical procedures and examinations can complicate medical interactions. Always ask the parents or primary caregivers about the most effective means of communication for each child, as well as specific "do's and don'ts." Consider reducing auditory, visual, and tactile stimulation, and allow a period of adjustment to the ED environment; if possible, limit the number of medical providers who are unfamiliar to the child. Sedation may be needed for examination and procedures.

INTELLECTUAL DISABILITY AND GLOBAL DEVELOPMENTAL DELAY

Intellectual disability occurs in approximately 1% to 3% of the population⁴¹ and is defined by cognitive impairment and decreased adaptive behaviors resulting from injury, disease, or abnormality occurring before the age of 18. Global developmental delay refers to intellectual disability in children <5 years old and is characterized by significant deficits in learning and adaptive behaviors.⁴² Intellectual disability is associated with disorders such as Down's syndrome, Rett's syndrome, Williams' syndrome, fragile X syndrome, and fetal alcohol syndrome, but has no disorder-specific associations in most cases. Children with intellectual disability can be difficult to approach, have impaired social and communication skills, and may become aggressive or combative when confronted by new or painful stimuli. The parent or primary caregiver is the best source of information for effective interaction strategies. The various medical problems found in these patients can be specific to the underlying syndrome, but treatment is generally the same as with developmentally normal children. Review medication profiles to prevent drug interactions and to identify potential drug side effects.¹⁶

DOWN'S SYNDROME

Down's syndrome is a relatively common genetic disorder, most often resulting from a trisomy of chromosome 21, that occurs in just over 1 in 1000 live births. The syndrome (see Table 146-1) is characterized by developmental delay, intellectual disability, congenital heart defects, GI abnormalities, thyroid dysfunction, insulin-dependent diabetes mellitus, increased risk of upper respiratory and ear infections, atlantoaxial instability, and leukemia. Congenital heart defects affect about half of children and include atrioventricular canal defects, ventricular and atrial septal defects, tetralogy of Fallot, and patent ductus arteriosus. These defects generally are detected in early infancy, but pulmonary hypertension and congestive heart failure can develop over time if treatment is not successful. GI abnormalities occur in about 5% of cases⁴³ and include esophageal atresia, tracheoesophageal fistula, pyloric stenosis, duodenal atresia, Meckel's diverticulum, Hirschsprung's disease, and imperforate anus. Therefore, feeding difficulties or vomiting in the infant with Down's syndrome requires thorough investigation. Gastroesophageal reflux responds to standard therapy. Midface malformations affect the normal functioning of eustachian tubes, leading to recurrent and chronic infections of the ear, sinuses, and upper respiratory tract. Asymptomatic atlantoaxial instability is present in about 13% of children with Down's syndrome. Maintain a high index of suspicion for cervical spine injury after trauma. Specifically, deceleration injuries raise concern for atlantoaxial subluxation or dislocation. Children with Down's syndrome, compared to those without, have a 10- to 20-fold increased risk of developing leukemia,⁴⁴ most commonly acute lymphoblastic leukemia.

CEREBRAL PALSY

The term cerebral palsy describes a collection of nonprogressive disorders of movement and posture originating from injury sustained by the developing brain within the first 3 to 5 years of life. The prevalence is 2 to 4 cases per 1000 individuals. Although motor deficits are the primary feature, cerebral palsy can also be associated with seizures, cognitive impairments, and sensory, communication, and behavioral abnormalities. There is a wide spectrum of intellectual and physical function, ranging from normal intelligence and mild motor deficits to severe disability. Cerebral palsy is often classified by the type of motor abnormality, its distribution, and the degree of involvement (Table 146-7).

Prenatal injury may occur from teratogen exposure, genetic syndromes, intrauterine infections, brain malformations, intrauterine cerebrovascular accidents, or fetal-placental malfunctions. The perinatal period may be affected by pre-eclampsia, complications of labor and delivery, sepsis or CNS infection, asphyxia, or prematurity. Older infants and children can develop cerebral palsy as a result of meningitis, traumatic brain injury, or toxic exposures. About 25% have no obvious cause.^45,46,47 Children with severe spasticity may have an intrathecal baclofen pump. Baclofen pump complications include malfunction, dislodgement, infection, or acute medication withdrawal. Life-threatening baclofen withdrawal can occur and may present with malignant hyperthermia, hypotension, myoclonus, seizures, rhabdomyolysis, disseminated intravascular coagulation, multisystem organ failure, cardiac arrest, coma, and death. In addition to life-saving supportive measures, oral baclofen and benzodiazepines are used to treat symptoms.

NEURAL TUBE DEFECTS

Neural tube defects result from the failure of the neural tube to close during the early embryonic stage of development and account for most congenital abnormalities of the CNS. The cause is multifactorial, but folic acid deficiency is associated with an increased risk, and maternal supplementation is currently recommended as early as possible in pregnancy.⁴⁸ The most common and most severe form of neural tube defect is meningomyelocele, which involves the spinal cord, meninges, and vertebral column. Spina bifida and spina bifida occulta are other examples of neural tube defects. Complex medical problems result from the impairment of sensory and motor control of voluntary and autonomic activities at or below the site of the lesion (Table 146-8).

Autonomic dysreflexia is a serious, potentially life-threatening complication of neural tube defects. Autonomic dysreflexia consists of paroxysmal sympathetic and parasympathetic hyperactivity initiated by stimuli below the level of the lesion, such as bladder overdistention, fecal impaction, or fracture. The symptoms of autonomic dysreflexia are sweating, flushing, pounding headache, hypertension, bradycardia, and piloerection. The primary intervention is to determine and eliminate the offending stimulus through bladder emptying, disimpaction with local anesthesia, discontinuation of painful procedures, or repositioning. If elevated blood pressure does not respond to repositioning or stimulus reduction, institute standard treatment for hypertensive emergencies.

Chiari Malformation

Chiari malformation consists of a downward displacement of the cerebellar tonsils through the foramen magnum, frequently causing obstructive hydrocephalus. Although mild tonsillar herniation seen in type I Chiari malformation can be asymptomatic, the more severe Chiari II is common in children with meningomyelocele and consists of malformation of the cerebellum, hindbrain, and brainstem (Table 146-9), requiring early ventricular shunt placement. Even minor cervical hyperflexion and extension injuries can cause symptoms in children with Chiari malformation; any deceleration injury, even mild, requires cervical spine stabilization and assessment for spinal cord injury due to instability of the craniocervical junction. Consider Chiari II malformation as a possible cause of new-onset stridor in the child with meningomyelocele. Monitor closely for progression to complete airway obstruction. Diagnosis requires emergent MRI of the craniocervical junction. If respiratory function is not severely compromised and the airway remains stable, outpatient disposition may be considered in consultation with subspecialists caring for the child.

URINARY TRACT

Complications of a neurogenic bladder include recurrent urinary tract infection, urinary retention or incontinence, and complications of self-catheterization. Medications or prophylactic antibiotics may be prescribed to enhance continence and minimize damage to the upper urinary tract. Because bacterial colonization of the urine is common, only treat symptomatic infection with antibiotics. Suspect formation of a false passage during self-catheterization with difficult catheter passage, pain, or urethral bleeding. Do not attempt recatheterization in the ED for these symptoms, for fear of compounding the underlying injury, but obtain urologic consultation. Long-term indwelling catheterization predisposes the patient to the development of latex allergy.

Acknowledgment The author gratefully acknowledges the contributions of Douglas R. Trocinski and Donna Moro-Sutherland, the lead authors of this chapter in previous editions.