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976 SECTION 12: Pediatrics The Child With Special Healthcare Needs Thomas Abramo Nicholas W. Porter Samuel T. Selby INTRODUCTION AND EPIDEMIOLOGY Children with special healthcare needs (CSHCN) have or are at increased risk for chronic physical, developmental, behavioral, or emotional conditions and require health and related services of a type or amount beyond those required by children generally. 1,2 This group of children encom passes a wide spectrum of medical complexity, functional limitations, and resource utilization. 3,4 A subset of the CSHCN population, children with medical complexity have substantial healthcare service and resource needs for their chronic conditions. 4,5 Their response to illness and trauma may be unpredictable compared with the normal pediatric patient, sometimes leading to a rapid and unexpected deterioration. This requires a personalized approach that addresses their special needs and chronic medical problems in addition to the emergency medical condition at hand. This unique response to illness and injury accounts for the majority of pediatric hospital days and deaths in this group. 4,6 CSHCN is a growing population, accounting for 15% to 18% (11.2 million) of children in the United States7,8 and for 40% to 80% of all pediatric healthcare utilization and costs.7,9,10,11 These trends are largely due to increased survival of premature infants and improved medical or surgical treatment of conditions that were once terminal. 5,6 Compared to typical children, children with chronic conditions tend to be older, male, and from white racial or ethnic groups. 8,10-13 The clinical spectrum of CSHCN conditions is diverse, and complex medical devices are often required for care ( Tables 148-1 and 148-2). 10-12,14-17 Because of the increasing prevalence of this patient population and their high rate of healthcare utilization, emergency medical practices must adapt to meet the needs of this complex group. EMERGENCY INFORMATION FORM An emergency information form provides information and instructions to optimize emergency care for complex medical conditions. 16,22 Components of a comprehensive medical plan include information about the patient’s chronic conditions, special instructions for devices or in case of emergencies, and supplies that might be required for routine or emergency medical care. The individualized information form can be provided at the ED visit by the patient’s caregiver. 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; vitals; 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 and access to 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 emer gency care for the child. 15,16 A “go-bag” or kit with specialized equipment should accompany the child.

he 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 emer gency care for the child. 15,16 A “go-bag” or kit with specialized equipment should accompany the child. These kits can contain needed supplies such as extra tracheostomy tubes and equipment for replacement, appropriate size suction catheters, syringes and adapters to decompress feeding tubes, a bag-valve mask resuscitator, and needles to access cen tral lines. 23 It is important for medical providers to ask for this special equipment in emergency situations. GENERAL CLINICAL APPROACH As in typical pediatric emergencies, a standardized approach is needed. Attention to airway, breathing, and circulation is paramount to caring for any child; however, special consideration for known chronic conditions CHAPTER will drive the practitioner’s workup and resuscitation. For example, a child with history of comorbidities and resulting temperature instability may present to the ED with hypothermia (<36.5° C) in the setting of infection. 20 Active rewarming and consideration of sepsis even in absence of more classical indicators such as fever and tachycardia are important for approaching the child’s treatment. Ancillary clinical data such as cardiac monitoring, pulse oximetry, capnography, continuous temperature monitoring, and near-infrared spectrometry may provide important information to the practitioner. Access to multiple informational resources including family, primary care physicians, specialists, and home health nurses is helpful in tailoring the approach to the individual child (Table 148-3). 15,16,23 Long-term caregivers often 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 ele ments of the history and subtle symptoms of illness 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 advance 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 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 medical complexity 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. Baseline pulse oximetry may be lower in children with congenital heart disease. 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 spe cial attention to medical devices and equipment: palpate the scalp for ventricular shunts for swelling or tenderness; check patency of trache ostomy tubes; assess central or peripherally inserted vascular catheters for patency and signs of infection; and examine gastrostomy sites and feeding tubes. The physical exam should include evaluating the back of nonmobile patients for signs of infection or breakdown.

or swelling or tenderness; check patency of trache ostomy tubes; assess central or peripherally inserted vascular catheters for patency and signs of infection; and examine gastrostomy sites and feeding tubes. The physical exam should include evaluating the back of nonmobile patients for signs of infection or breakdown.  LABORATORY TESTING AND IMAGING The specific evaluation depends on the presenting problem and under lying special healthcare needs. Perform bedside glucose testing in all complex patients with altered mental status. Check serum drug levels (e.g., anticonvulsants) as appropriate. Children with complex needs are more likely to have electrolyte disturbances than typical children. Compare plain radiographs and CT scans with prior imaging results to distinguish acute from chronic changes. TREATMENT Although treatment is tailored to the presenting condition, the uni versal 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. ”  CONGENITAL HEART DISEASE Many children presenting to the ED will have one of a broad range of congenital heart diseases. The practitioner may notice cyanosis, fin gernail clubbing, or a loud murmur on physical exam. It is important to understand each patient’s special cardiac needs. They may have been completely surgically repaired with no residual functional limita tions due to their heart condition. Alternatively, they may be awaiting Tintinalli_Sec12_p0669-0996.indd 976 8/2/19 7:59 PM

lubbing, or a loud murmur on physical exam. It is important to understand each patient’s special cardiac needs. They may have been completely surgically repaired with no residual functional limita tions due to their heart condition. Alternatively, they may be awaiting Tintinalli_Sec12_p0669-0996.indd 976 8/2/19 7:59 PM CHAPTER 148: The Child With Special Healthcare Needs 977 TABLE 148-1 Congenital or Developmental Disorders and Associated Medical Conditions 19,20 Chromosomal disorders10,21 Down syndrome (trisomy 21 syndrome) Seizure disorder (12%–15%); atlantoaxial instability (14%–22%); cataracts (15%); serous otitis media (50%–70%); deafness (75%); congenital heart disease (50%): ASD, VSD, AV canal, PDA, tetralogy of Fallot, pulmonary hypertension; GI atresias (12%); Hirschsprung’s disease (1%); constipation, fecal impaction from medications or hypothyroidism; thyroid disease (15%); diabetes mellitus; leukemia (1%); acquired hip dislocation (6%); and psychiatric disorders (22%) Fragile X syndrome Recurrent serous otitis media (60% in males), strabismus (30%–56% in males), seizures (14%–50% in males), autism (16% in males), self-abusive, and mitral valve prolapse (22%–77% in males) Trisomy 18 Congenital heart disease (99%): VSD, ASD, and PDA Turner’s syndrome (XO syndrome) Short stature in females, horseshoe kidney, heart disease (bicuspid aortic valve in 30%, coarctation of the aorta in 10%, valvular aortic stenosis, mitral valve prolapse, aortic dissection later in life, and hypertension) Noonan’s syndrome Webbing of the neck, pectus excavatum, cryptorchidism, and pulmonic stenosis Disorders with facial defects as major feature Pierre Robin syndrome Micrognathia, glossoptosis, and cleft soft palate; primary defect: early mandibular hypoplasia Waardenburg’s syndrome Lateral displacement of medial canthi, partial albinism, and deafness Occasional associations: VSD, Hirschsprung’s disease, esophageal atresia, and anal atresia Treacher Collins syndrome Malar hypoplasia with downward-slanting palpebral fissures, defect of lower lid, and malformation of the external ear Disorders with limb defects as major feature Holt-Oram syndrome Upper limb defect, cardiac anomaly (ASD, VSD, arrhythmia), and narrow shoulders Fanconi’s pancytopenia syndrome Radial hypoplasia, hyperpigmentation, pancytopenia, and renal anomaly Radial aplasia thrombocytopenia (TAR syndrome) Inherited metabolic disorders Phenylketonuria (autosomal recessive) Light pigmentation, eczema (33%), poor coordination, seizures (25%), and autistic behavior Hunter’s syndrome (X-linked recessive) Developmental lag after age 6–12 mo, coarse facies, growth deficiency, stiff joints by age 2–4 y, clear corneas, and hepatosplenomegaly Hurler’s syndrome (autosomal recessive) Developmental lag after age 6–10 mo, coarse facies, stiff joints, mental deficiency, cloudy corneas by age 1–2 y, hepatosplenomegaly, and rhinitis Connective tissue disorders Marfan’s syndrome Arachnodactyly with hyperextensibility, lens subluxation, and aortic dilatation Ehlers-Danlos syndrome Hyperextensibility of joints, hyperextensibility of skin, and poor wound healing with thin scar Osteogenesis imperfecta congenital Short, broad, long bones; multiple fractures; and blue sclera Hamartoses Sturge-Weber sequence Flat facial hemangiomata and meningeal hemangiomata with seizures Tuberous sclerosis syndrome Hamartomatous skin nodules (thumb print macules), seizures, angiomyolipomata (45%–81%), phakomata, and bone lesions Neurofibromatosis syndrome Multiple neurofibromata, café-au-lait spots, presence or absence of bone lesions, seizures and/or EEG changes in 20%, cerebrovascular compromise, and headaches Environmental agents (toxins) Fetal alcohol syndrome Vision problems (94%), recurrent serous otitis (93%), hearing loss (66%), hear

s Neurofibromatosis syndrome Multiple neurofibromata, café-au-lait spots, presence or absence of bone lesions, seizures and/or EEG changes in 20%, cerebrovascular compromise, and headaches Environmental agents (toxins) Fetal alcohol syndrome Vision problems (94%), recurrent serous otitis (93%), hearing loss (66%), hear t defects (29%–41%), renal hypoplasia, duplication of the kidney and collecting system, and bladder diverticula (10%) Other environmental exposures include fetal hydantoin syndrome, fetal trimetha dione syndrome, fetal valproate syndrome, fetal warfarin syndrome, and retinoic acid embryopathy Trauma Traumatic brain injury Visual and hearing disturbances; cranial nerve damage; spasticity, incoordination, ataxia, and feeding disorders; GERD; neuropsychiatric disturbances Cerebral palsy Seizures (33%), strabismus (50%), hearing loss (10%), hip dislocation, scoliosis, contractures, gait disorder, GERD (8%–10%), chronic aspiration and recurrent RAD, pulmonary fibrosis, and bronchiectasis Miscellaneous Angelman’s syndrome (happy puppet syndrome) Puppet-like gait, paroxysms of laughter, and characteristic features; seizures vary from major motor to akinetic, beginning usually at age 18–24 mo Beckwith-Wiedemann syndrome Macroglossia, omphalocele, macrosomia, and ear creases; neonatal polycythemia and hypoglycemia in early infancy; associated with Wilms’ tumor CHARGE syndrome Coloboma, heart disease (tetralogy of Fallot, PDA, double-outlet right ventricle with an atrioventricular canal, VSD, ASD, and right-sided aortic arch), atresia choa nae, retarded growth and development and/or CNS anomalies, genital anomalies and/or hypogonadism, and ear anomalies and/or deafness Prader-Willi syndrome Mental retardation, hypotonia, hypogonadism, obesity, hyperphagia, gastric perforation, hypoventilation, obstructive sleep apnea, cor pulmonale, NIDDM, scoliosis, strabismus, inability to vomit, decreased sensitivity to pain, seizure disorder, hypoxia, right-sided heart failure, and pulmonary hypertension Rett’s syndrome Hyperventilation, breath holding, air swallowing, bruxism, ataxia, muscle wasting, poor circulation, scoliosis, seizures, and intermittent flushing VATER syndrome Vertebral defects and VSD, imperforate anus, tracheoesophageal fistula, renal anomalies, and single umbilical artery Williams’ syndrome Elfin-like syndrome, cardiovascular disease, supravalvular aortic stenosis, pulmonic stenosis, coarctation of the aorta, strabismus, joint contractures, hypertension, ure thral stenosis, vesicoureteral reflux, constipation, ulcers, and hypercalcemia Abbreviations: ASD = atrial septal defect; AV = atrioventricular; EEG = electroencephalogram; GERD = gastroesophageal reflux disease; NIDDM = non–insulin-dependent diabetes mellitus; PDA = patent ductus arteriosus; RAD = reactive airway disease; TAR = thrombocytopenia-absent radius; VSD = ventricular septal defect.

mia Abbreviations: ASD = atrial septal defect; AV = atrioventricular; EEG = electroencephalogram; GERD = gastroesophageal reflux disease; NIDDM = non–insulin-dependent diabetes mellitus; PDA = patent ductus arteriosus; RAD = reactive airway disease; TAR = thrombocytopenia-absent radius; VSD = ventricular septal defect. Tintinalli_Sec12_p0669-0996.indd 977 8/2/19 7:59 PM

mia Abbreviations: ASD = atrial septal defect; AV = atrioventricular; EEG = electroencephalogram; GERD = gastroesophageal reflux disease; NIDDM = non–insulin-dependent diabetes mellitus; PDA = patent ductus arteriosus; RAD = reactive airway disease; TAR = thrombocytopenia-absent radius; VSD = ventricular septal defect. Tintinalli_Sec12_p0669-0996.indd 977 8/2/19 7:59 PM 978 SECTION 12: Pediatrics surgical correction or be somewhere in a series of staged surgical corrections. Depending on the patient’s current cardiac physiology, the patient with cyanotic congenital heart disease may have some degree of venous mixing or shunting causing relatively low oxygen saturations. In some cases, a pulse oximetry reading of 70% may be normal, such as in a child with partially repaired hypoplastic left heart syndrome. The same child will have saturations above 90% after completion of their Fontan surgical correction at 3 years of age. At the other end of the spectrum, the child with pulmonary hypertension depends on a normal oxygen saturation (97% to 100%) to ensure adequate pulmonary blood flow. These children will often be on supplemental oxygen at baseline, and it is essential to correct hypoxemia immediately. Ask the patient’s caregiver or consult with a cardiologist to determine an acceptable oxygen saturation for each patient and the patient’s specific heart defect. A thorough evaluation of a patient with congenital heart disease may include careful physical examination, vital signs, pulse oximetry, chest radiography, electrocardiography, and consultation with the patient’s cardiologist (see Chapter 129, “Congenital and Acquired Pediatric Heart Disease”). Admission to the hospital or transfer to a pediatric center may be warranted for conditions that might otherwise be treated on an outpatient basis in typical children.  RESPIRATORY DISORDERS Children with complex medical histories frequently present with respiratory problems. Chronic lung disease due to prematurity, reactive airway disease, restrictive lung disease due to scoliosis, dysphagia, and trache ostomies are common in this population. It is important to ask about special respiratory needs, such as bronchodilator use, home oxygen supplementation, thickened diets, airway clearance, and infection history. To evaluate acute respiratory complaints, obtain a thorough history of past respiratory function, especially in patients with a history of pre maturity: escalating home therapy including supplemental oxygen use; increased, colored, or thickened secretions from a tracheostomy tube; bronchodilator and inhaled steroid use in reactive airway disease; or baseline stridor in patients with tracheomalacia. Compare the radiology and laboratory results obtained to previous studies. Capillary blood gases may be an important indicator of current and chronic respiratory status. 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. A nasal trumpet or cervical collar may help maintain airway position, alleviating obstruction. 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 con sultation for airway foreign bodies. Admission is generally warranted for worsening respiratory status, pneumonia, increased home oxygen requirement, persistent respiratory distress, or removal of a foreign body.

cheostomy tubes, ventilators), β-agonists for bronchospasm, antibiotics for pneumonia, and subspecialty con sultation for airway foreign bodies. Admission is generally warranted for worsening respiratory status, 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 146, “Metabolic Emergencies in Infants and Children”). Although a detailed understanding of individual bio chemical 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. A bedside glucose at presentation is essential because many of these disorders lead to hypoglycemia. 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 (see Chapter 146, “Metabolic Emergencies in Infants and Children). 24 Consult a biochemical geneticist for further diagnostic evaluation and management guidance.  SEIZURES The prevalence of epilepsy is estimated at 6.3 cases per 1000 children and seizures are a frequent cause for admission. 4,25-29 In general, the nature and complexity of the seizure syndromes differ considerably between patients, but seizure syndromes are often consistent in the individual. The initial evaluation and management follow the standard approach to seizures (see Chapter 138, “Seizures in Infants and Children”), with a few caveats in children with medical complexity. 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 about medication or dose changes and 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 to determine if therapeutic adjustments are required. Keep in mind a broad differential including shunt malfunction, infection, electrolyte abnormality, and subtherapeutic medication doses. 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, may be associated with underlying disorders or syndromes. Patients with refractory seizures, including those with epileptic encephalopathies (e.g., Lennox-Gastaut or Dravet’s syndrome), 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 to maintain ketosis. Therefore, ask caregivers about baseline serum glucose levels before administering dextrose because dextrose may increase seizures previ ously 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.

t 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 TABLE 148-2 Common Home Medical Devices and Equipment •   Apnea monitors •   Central venous lines or percutaneous IV catheters and pumps •   Colostomies •   Feeding tubes and pumps •   Internal pacemakers or defibrillators •   Nebulizers •   Oxygen/suction •   Tracheostomies •   Vagus nerve stimulators •   Ventilators •   Ventricular peritoneal shunts TABLE 148-3 Checklist for Emergency Care of Children With Special Healthcare Needs •  Fully  involve the family and caregivers and use the care plan. •  Inquire  about advance directives and family goals of emergency care. •  Use  family information when assessing child’s pain, mental status, and symptoms. •  Anticipate  latex allergy. •  Identify  proper equipment sizes (e.g., tracheostomies, gastrostomy tubes, colostomies). •   Anticipate difficult intubation in the child with craniofacial or cervical vertebrae anomalies or contractures. •  Recognize  and treat comorbid states. •  Consider  physical or sexual abuse in the child with special needs. Tintinalli_Sec12_p0669-0996.indd 978 8/2/19 7:59 PM

tomies, gastrostomy tubes, colostomies). •   Anticipate difficult intubation in the child with craniofacial or cervical vertebrae anomalies or contractures. •  Recognize  and treat comorbid states. •  Consider  physical or sexual abuse in the child with special needs. Tintinalli_Sec12_p0669-0996.indd 978 8/2/19 7:59 PM CHAPTER 148: The Child With Special Healthcare Needs 979 motor dysfunction, or increased fluid losses from infection or gastric tube output. 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 intraosse ous access (see Chapter 114, “Vascular Access in Infants and Children”). Constipation may be associated with nonspecific symptoms such as abdominal pain, change in bowel habits, anorexia, or overflow diar rhea. 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 normal handling, therapy, falls, or accidents, practitioners must also consider abuse. 30 Assess for ortho pedic 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 greater than three times more likely to be abused (sexually, physically, and/or via neglect) and endure multiple abuse events when compared with children without disabilities (Sullivan 2000). 31,32 They often place higher emotional, physical, economic, and social demands on their families.33,35 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 (see Chapter 150, “Child Abuse and Neglect”). TECHNOLOGY-DEPENDENT CHILDREN 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. 36 Such patients account for 70% to 90% of technology use, and malfunction accounts for nearly 10% of this population’s admissions. 4 Devices include ventilators, pacemakers, tracheostomies, gastrostomy tubes, central venous catheters, ventriculoperitoneal shunts, and vagus nerve stimulators. 36,37 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 tube type, size, and most recent change.

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 tube type, size, and most recent change. 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 pneu mothorax. Granuloma or stricture formation at the stoma or tracheal wall can cause bleeding with cannula manipulation or suctioning. Ero sion 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, bron chitis, 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, a new or increasing oxygen requirement, or a change in secretions.  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%. 38 Ventilator-related complications, such as pneumothorax or machine failure, are managed according to standard principles. Provide bag ventilation for total ventilator failure. Table 148-4 provides a checklist for ventilator troubleshooting.  FEEDING TUBES Feeding tubes are commonly present. 4 Complications of a nasogastric tube include sinusitis, nasal and esophageal irritation, tube dislodge ment 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. Feeding tubes may become dislodged, break, or otherwise malfunc tion. It is important to know what size and type of tube to replace. Also knowing the position of feeding tube present (gastric tube, gastrojejunal tube) will help to ensure proper placement. Gastrojejunal tubes will often require a consult to interventional radiology for replacement. Gastrostomy tubes that become displaced require prompt replace ment, because a stoma without a tube will close in a matter of hours. A Foley catheter can be placed in a stoma while awaiting appropriate gastrostomy tube replacement. Narrowed stomas may require dilation in order to pass a tube. Confirm proper placement with aspiration of gastric contents. For difficult replacements, stomas that have been present for less than 12 weeks, or uncertainty about position, it is best to confirm placement with a radiographic dye study (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.  INDWELLING VENOUS CATHETERS 39 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.

be cauterized using silver nitrate sticks.  INDWELLING VENOUS CATHETERS 39 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. Complications include occlusion, breakage, TABLE 148-4 Ventilator Troubleshooting Alarm Possible Causes Interventions High pressure Plugged or obstructed airway Coughing/bronchospasm Clear obstruction Suction tracheostomy Administer bronchodilator Low pressure/apnea Loose or disconnected circuit Leak in circuit Leak around tracheostomy site Ensure all circuits are connected Check tracheostomy balloon Ensure tracheostomy is well seated Low power Internal battery depleted Plug the ventilator into a power outlet Setting error Settings incorrectly adjusted Manually ventilate patient Transport ventilator and patient Power switchover Unit switched from AC to internal battery Press “alarm silent” button after ensuring battery is powering ventilator Tintinalli_Sec12_p0669-0996.indd 979 8/2/19 7:59 PM

ventilator into a power outlet Setting error Settings incorrectly adjusted Manually ventilate patient Transport ventilator and patient Power switchover Unit switched from AC to internal battery Press “alarm silent” button after ensuring battery is powering ventilator Tintinalli_Sec12_p0669-0996.indd 979 8/2/19 7:59 PM 980 SECTION 12: Pediatrics dislodgement, air embolism, phlebitis, and infection. Parenteral feed ing complications include cholestasis that may lead to irreversible liver disease or metabolic bone disease. 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 consulta tion 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. 39 For suspected infection, obtain blood cultures and administer antibiotics to cover gram-positive and gram-negative organisms (Gominet 2017). Fever in patients with externalized catheters represents a medical emergency due to risk for line infection. These patients should have central and peripheral blood cultures and be started on broad-spectrum IV antibiotics to cover both gram-positive and gram-negative organisms. Admission to the hospital is warranted in even well-appearing patients with central line and fever.  CEREBROSPINAL FLUID SHUNTS Cerebrospinal shunt placement is the most common neurosurgical procedure performed in children. 39 Most commonly, cerebrospinal fluid is diverted from the ventricles to the peritoneal cavity, but occasionally the atria and pleural spaces may be used. 4 Complications include separation or disruption of the tubing or device, infection, and overdrainage 41,42 (Table 148-5). Shunt malfunction occurs most often in the year follow ing placement,43 with approximately 40% of standard shunts malfunctioning during this time. 44 After the first year, the annual rate of shunt malfunction is about 5%.45 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 by tapping 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. After 3 months of shunt placement, infection rates fall to a very low occurrence. Infection rates vary from 1% to 40%, with most in the range of 5% to 15%. 46 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, coagulasenegative Staphylococcus, Staphylococcus aureus, and Streptococcus ) and gram-negative (Escherichia coli, Enterococcus species, and Haemophilus influenzae) species.

dominal pain. Infected shunts may be accompanied by mechanical shunt failure. Antibiotics should be selected to treat gram-positive (most commonly, coagulasenegative 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. 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 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. SPECIAL POPULATIONS  DEVELOPMENTAL DISABILITY/DELAY Developmental disability is a broad term encompassing conditions such as attention-deficit/hyperactivity disorder, cerebral palsy, seizures, stammering, and other developmental delays in children. Developmental disability is occurring with increased frequency and affects approximately one in six children. 48 Children with intellectual disability can be difficult to approach, may have impaired social and communication skills, and may become aggressive or combative when confronted by new or pain ful stimuli. The parent or primary caregiver is the best source of infor mation 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 chil dren. Review medication profiles to prevent drug interactions and to identify potential drug side effects.  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. 49 The disorder has a wide range 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. 50 The current prevalence is approximately 1 in 68 children. 51,52 The cause is unknown, but there is no relationship with childhood immunizations.

s a wide range 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. 50 The current prevalence is approximately 1 in 68 children. 51,52 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 aggres sive 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 TABLE 148-5 Symptoms and Signs of Ventriculoperitoneal Shunt Obstruction or Malfunction Symptom Signs Headache Shunt site swelling or normal exam Visual disturbances Papilledema Nausea or vomiting Bulging fontanelle, enlarged head, or normal exam Lethargy and/or irritability Engorged head veins Lack of developmental progress and/or poor school performance McEwen sign (cracked-pot sound during skull percussion) Note: Neurologic findings include increased deep tendon reflexes or lower extremity tone, positive Babinski sign, lateral (sixth) or upward (fourth) gaze palsy (sun-setting), and respiratory compromise. Tintinalli_Sec12_p0669-0996.indd 980 8/2/19 7:59 PM

rformance McEwen sign (cracked-pot sound during skull percussion) Note: Neurologic findings include increased deep tendon reflexes or lower extremity tone, positive Babinski sign, lateral (sixth) or upward (fourth) gaze palsy (sun-setting), and respiratory compromise. Tintinalli_Sec12_p0669-0996.indd 980 8/2/19 7:59 PM CHAPTER 148: The Child With Special Healthcare Needs 981 are unfamiliar to the child. Sedation may be needed for examination and procedures.  DOWN SYNDROME Down 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 (Table 148-1) is characterized by developmental delay, intellectual disability, congenital heart defects, GI abnormalities, thyroid dysfunction, insulin-dependent diabetes mel litus, increased risk of upper respiratory and ear infections, atlantoaxial instability, and leukemia. Congenital heart defects affect approximately 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 approximately 5% of cases 53 and include esophageal atresia, tracheoesophageal fistula, pyloric stenosis, duodenal atresia, Meckel’s diverticulum, Hirschsprung’s disease, and imperforate anus. Therefore, feeding difficulties or vomit ing in the infant with Down syndrome requires thorough investiga tion. Gastroesophageal reflux responds to standard therapy. Midface malformations affect the normal functioning of eustachian tubes, lead ing to recurrent and chronic infections of the ear, sinuses, and upper respiratory tract. Asymptomatic atlantoaxial instability is present in approximately 13% of children with Down syndrome. Consider cervical spine injury after trauma, specifically with deceleration injuries, which can cause atlantoaxial subluxation or dislocation. Children with Down syndrome, compared to those without, have a 10- to 20-fold increased risk of developing leukemia, 54 most commonly acute lymphoblastic leukemia. CEREBRAL PALSY The term cerebral palsy describes a collection of nonprogressive dis orders of movement and posture originating from injury sustained by the developing brain within the first 3 to 5 years of life. The prevalence is 3 to 4 cases per 1000 individuals. 48 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 dis ability. Cerebral palsy is often classified by the type of motor abnormality, its distribution, and the degree of involvement (Table 148-6). Prenatal injury may occur from teratogen exposure, genetic syn dromes, intrauterine infections, brain malformations, intrauterine cerebrovascular accidents, or fetal-placental malfunctions. The perinatal period may be affected by preeclampsia, 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. Approximately 25% of cases have no obvious cause. 55,57 Children with severe spasticity may have an intrathecal baclofen pump. Baclofen pump complications include malfunction, dislodgement, infection, or acute medication withdrawal.

p cerebral palsy as a result of meningitis, traumatic brain injury, or toxic exposures. Approximately 25% of cases have no obvious cause. 55,57 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 lifesaving supportive measures, oral baclofen and benzodiazepines are used to treat symptoms.  NEURAL TUBE DEFECTS AND SPINAL CORD INJURY 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. 58 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. Patients with neural tube defects are often allergic to latex . 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 148-7). Spinal cord injury results in a similar loss of motor and sensory control below the level of the lesion. In these patients, significant osteopenia in their non functioning limbs can lead to underlying fractures presenting as extremity swelling. This can occur with their routine physical therapy and care. Autonomic dysreflexia is a serious, potentially life-threatening complication of neural tube defects and spinal cord injuries. Auto nomic dysreflexia consists of paroxysmal sympathetic and parasympa thetic 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, 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.

. 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 148-7 Complications of Neural Tube Defects •   Autonomic dysreflexia •   Chiari II malformation •   Cognitive impairment •   Constipation •   Contractures •   Gastroesophageal reflux •   Growth failure •   Hydrocephalus •   Latex allergy •   Neurogenic bowel and bladder dysfunction •   Osteoporosis •   Recurrent urinary tract infections •   Respiratory compromise •   Scoliosis •   Seizures •   Spinal cord syrinx •   Tethering of the spinal cord •   Vesicoureteral reflux •   Vision impairment TABLE 148-6 Cerebral Palsy Motor abnormality Spastic Dyskinetic Ataxic Hypotonic Increased tone; muscle stiffness Involuntary, nonpurposeful, uncoordinated movements Wide-spaced, unsteady gait; movement uncoordinated and clumsy Floppy; generalized muscle weakness and flaccidity Distribution Diplegia Paraplegia Hemiplegia Quadriplegia Paralysis affecting symmetrical parts of the body Paralysis of both legs Involvement of one half of the body Four-limb paralysis Tintinalli_Sec12_p0669-0996.indd 981 8/2/19 7:59 PM