Children with special needs have a wide variety of physical and developmental challenges. These children often have medical devices, subtle presentations, and behavioral or psychiatric issues that demand consideration when delivering emergency care. Some of the more common issues include the evaluation and management of complications in devices such a gastrostomy/jejunostomy feeding tubes, cerebroventricular shunts, and tracheostomy tubes, as well as impediments to the performance of common emergency procedures (eg, laceration repair, sedation for imaging) on children with developmental or behavioral disorders. This review will examine each of these circumstances and provide guidance on the best approaches to managing these patients.
A 7-year-old girl with an intellectual disability arrives to the ED with her parents, who state that the girl has been lethargic all day, is not tolerating her gastrostomy tube feeds, and is having some leakage and redness around the exit site of her tube. She has been vomiting nonbloody, nonbilious material with every feeding attempt. Her parents describe her breathing as “harder than usual,” with an increase in her baseline home oxygen requirement. In addition, they noticed some scant blood from her tracheostomy tube after suctioning thick whitish-yellow secretions from it. Her urine output has decreased from 4 times per day to 1 time per day. She has a low-grade fever to 38.2°C. Her past medical history includes premature birth at 25 weeks with a complicated NICU stay, including hypoxic-ischemic encephalopathy, grade IV intraventricular hemorrhage requiring placement of a ventriculoperitoneal shunt, gastrostomy tube placement, prolonged intubation with chronic lung disease, tracheostomy tube placement, a seizure disorder, and significant developmental delay (the child is nonverbal) with static encephalopathy. With her many problems, how do you evaluate the cause of this child’s vomiting, lethargy, and difficulty breathing?
A 3-year-old boy with Down syndrome is brought to the ED 1 hour after pulling out his percutaneous endoscopically placed gastrostomy tube, which was placed 2 years ago. The mother believes he broke the balloon when he yanked the tube out. His past medical history includes repair of a ventricular septal defect and failure to thrive. What are the best ways to manage the dislodged tube?
An 8-year-old boy with autism arrives to the ED with a 5-cm scalp laceration extending to the temporal area of the head that he sustained after falling from a 5-foot height. His parents report a loss of consciousness for 1 to 2 minutes as well as abnormal behavior. You decide he needs a CT scan to evaluate for intracranial injury, and the laceration will need to be repaired. How do you approach the sedation of this child so he can safely and effectively undergo the interventions he requires?
Children with special healthcare needs are defined as children “who have or are at increased risk for a chronic physical, developmental, behavioral, or emotional condition and who also require health and related services of a type or amount beyond that required by children generally.”1 An estimated 12% to 18% of children in the United States have special needs,2-5 and they account for 5 times the number of doctor visits as children without special needs.
Children with pervasive neurodevelopment disorders, autistic spectrum disorder (ASD), and developmental disorders, and behaviorally complex children are frequent patients in the emergency department (ED). These children often have complex medical histories and various comorbid conditions that complicate their care. In addition, these children may be limited in their capacity for cooperation and communication, making diagnosis and treatment difficult. In a Canadian study of patients with ASD who presented to the ED at a tertiary care center, 33 of 160 patients (20%) were admitted, 71% had an emergency triage score of 1 to 2 (suggesting high-acuity problems), and 79% had > 1 chronic health problem. Furthermore, the study found that patients with ASD comprised 2% of all ED visits. Parents of children with developmental disorders may also be more likely to use the ED as their medical home, especially if they do not have a well-established relationship with a primary care provider. In a survey of ED usage by children in the United States with developmental disorders, children with ≥ 3 ED visits were found to report less access to primary care sources than patients with no ED visits, who often reported well-established primary care.7
The term “children with special needs” covers a broad array of children, including those born prematurely, children who suffered hypoxia in the perinatal period, children with developmental and behavioral conditions, and medically complex patients, such as those with congenital cardiac and brain disorders, genetic disorders, inborn errors of metabolism, and mitochondrial diseases. This review focuses on identification and management of common problems with enteral feeding tubes, tracheostomies, and ventricular shunts, and will include a discussion on performing emergency procedures in children with autism and other cognitive disabilities.
An extensive literature search was conducted on PubMed using combinations of the following search terms: special needs, children, disabled, technology dependent, epidemiology, prevalence, emergency department, gastrostomy, percutaneous endoscopic, compli-cations, jejunostomy, migration, ventriculoperitoneal shunt, infection, cellulitis, malfunction, tracheostomy, tracheoinnominate fistula, tracheitis, mucociliary clearance, emergency procedure, bleeding, sedation, autism, developmental delay, virtual reality, child life specialist, autism, behavior, procedure, management, acute setting, imaging, MRI, agitation , and aggressive . Only articles whose subjects included children aged 0 to 18 years were identified. Abstracts were reviewed for relevance to the topic. Supporting articles were gathered from related articles and the reference lists of review articles. All relevant articles were selected, reviewed, and included in the bibliography. Overall, there is a dearth of literature in this patient population, and more studies are needed.
Evidence-based medicine requires a critical appraisal of the literature based upon study methodology and number of subjects. Not all references are equally robust. The findings of a large, prospective, randomized, and blinded trial should carry more weight than a case report.
To help the reader judge the strength of each reference, pertinent information about the study, such as the type of study and the number of patients in the study will be included in bold type following the references cited in this paper, as determined by the author, will be noted by an asterisk (*) next to the number of the reference.
Solomon Behar, MD; John Cooper, DO
June 2, 2015