Acute Evaluation of the Child with Special Healthcare Needs
Acute Evaluation of the Child with Special Healthcare Needs
Authors: Charles F. Willson, MD; Gerald L. Strope, MD; and Judy W. Wood, MD; Clinical Professors of Pediatrics, Department of Pediatrics, Brody School of Medicine at East Carolina University, Co-directors, Center for Children with Complex and Chronic Conditions, Greenville, NC; and Ronald M. Perkin, MD, MA, Professor and Chairman, Department of Pediatrics, the Brody School of Medicine at East Carolina University, Greenville, NC.
Peer reviewer: Wendalyn King, MD, MPH, Assistant Professor of Pediatrics and Emergency Medicine, Emory University, Atlanta, GA.
With the increasing skill of our NICUs in saving extremely premature babies and the capability of rescuing children with acute illnesses and injuries in our PICUs, children with special healthcare needs are being discharged from tertiary centers and returning to their home communities. Local healthcare systems often are not ready or sometimes not even capable of caring for them. (See Table 1 for definitions of children with disabilities and chronic conditions. ) As a result, the local ED has become the acute care provider when an illness develops.
Depending on the definition used, somewhere between 10% and 20% of children have a chronic medical condition or disease. A recent Department of Defense study of ED visits at military facilities found that while children with special healthcare needs represented 11% of the outpatient dependent population, they represented 25% of ED visits.1
An ED physician faces many challenges when evaluating a child with a complex and chronic condition. The mere scope of primary diagnoses is daunting; it ranges from complex inborn errors of metabolism to the child with autism. Because of compromised biological functions and disordered anatomy, these children often are medically very fragile. Even the child with attention deficit disorder frequently presents to the ED with injuries directly resulting from the impulsivity of the behavioral condition. Sometimes, these children also are prescribed multiple psychoactive medications, and drug interactions must be carefully considered. Additionally, the number of extraordinarily obese children in our society is growing, and ED physicians will be faced with patients with immature bodies and minds but adult morbidities such as type two diabetes mellitus, hypertension, and sleep apnea.
In this article, the authors will suggest a general approach to any child with a complex and chronic condition, focus on issues regarding the technology-dependent child, and then consider end-of-life issues and palliation.
The Editor
 |
General Approach
First and foremost, the primary caregiver is a clinician's best ally and a source of valuable information if the medical record is not available and a written care plan has never been developed. Often, the presenting complaint has occurred previously and the caregiver can guide the work-up. Always take the caregiver's assessment of the degree of illness or change from usual status as accurate. Children with complex and chronic conditions are unique, and the person who lives with them 24 hours a day always knows them best. If the caregiver objects to an intervention, course of action, or disposition, reassess the child and the caregiver's reasoning. Sitting with the caregiver for a few minutes to allow them to talk about their child, experiences, and expectations often will offer an appropriate course of action; the parent will be eternally grateful.
The general approach to the child with special healthcare needs (CSHCN) is quite similar to any other patient. Signs of dehydration and respiratory distress are readily elicited, but each CSHCN is unique and may present a special challenge because of disordered anatomy and activity levels. Metabolic disturbances may complicate the evaluation. The child may be ambulatory, have severely disabled movement, or be confined to a wheel chair. Even weighing a stretcher or wheel-chair-bound child with contractures may be extraordinarily difficult. The CSHCN may not be able to provide a specific complaint or may not demonstrate a specific physical finding. Often, the CSHCN is not able to indicate when he or she is in pain; the caregiver may only have noticed a change from the patient's baseline condition. Frequently, it may be determined that the child has a straightforward and common illness imposed upon an extraordinarily complex medical condition.
Approaching these children may result in considerable anxiety on the part of the medical caregiver because of these unknown factors and the potential interaction of underlying problems. It is important to recognize that the patient's chronic condition may have significant impact on the acute condition; however, the acute evaluation should focus on the reason for the visit and not the cause of the patient's handicap. The assessment and treatment of a CSHCN should begin as it does with any child: prompt attention to the patient's airway, breathing, and circulation.2
How to Obtain Information. Ideally, a CSHCN will have a written care plan that includes lists of diagnoses, current medications, specialist consultants, formal care plans, and recent discharge summaries. Sometimes, the best and most current information is contained in the home nurse's evaluation forms. When technology is involved, it is critical to ascertain the sizes of catheters and tracheostomy tubes and types of monitors and ventilators.
Evaluating Available Care Plans. The caregivers should be able to direct the medical provider to any specific information requested. In some cases, a list of diagnoses can provide assistance with understanding the underlying condition. Care plans can give direction about baseline vital signs, medications, baseline oxygen therapy, equipment needs, and unusual diagnoses that may not be apparent by visual inspection. Names and telephone numbers of any medical care providers, including primary care physicians, subspecialty physicians, private duty nursing agencies, and durable medical equipment providers, may be noted in the available documents.
Understanding a Patient's Baseline. It is important to obtain information about the patient's baseline condition to help determine if there are any acute changes that bring the patient in for evaluation. In some cases, parents might be concerned about stress related to airway obstruction or fever. Due to altered physiology, the stress of an illness may lead to decreased heart rate and respiratory drive. Typically, only the patient's caregiver will most accurately be able to describe the child's true baseline.
Understanding the Caregivers' Skills. Many patients with special needs will require special skills from their caregivers. For example, patients with neurogenic bladder will require intermittent clean catheterization. Patients with tracheostomy tubes also will have caregivers who are skilled in providing care for and changing the tracheostomy tube; when other technology devices are required, caregivers may have special skills involving those as well. Allowing these caregivers to perform these functions while the child is being evaluated often will be comforting to the parent and reassuring to the child. For instance, the parent of a child who requires straight catheterization may be able to provide assistance collecting urine for evaluation of their febrile child. Also, a patient with a tracheostomy tube who is in respiratory distress may have a caregiver who can provide assistance and direction with changing the tracheostomy tube or collecting a sample of mucus for culture or microscopic evaluation. A wise emergency physician will support the care regimens these parents have learned at the tertiary care center and critique or change these regimens only after conferring with the center.
Focusing on the Current Problem. Focusing on the current problem and ascertaining the nature of the precipitating event may be challenging in children who have multiple complex derangements. An accurate historical timing of events by the caregiver is critical. For example, in a child with limited respiratory reserve, the development of fever, regardless of source, may result in severe respiratory compromise. When this patient presents for evaluation, the focus should be on the marked respiratory difficulties even though the originating problem was otitis media or a urinary tract infection.
Evaluating Technologies Necessary for Care
Gastrostomy Tube (GT). GTs may become dislodged and can be difficult to reinsert after a few hours. Insertion of a small feeding tube or a small Foley catheter can help maintain the tissue planes around the stoma until an appropriate tube can be reinserted. Reinserting the GT may be challenging and require the assistance of a surgeon. Abdominal distention in a child with a Foley catheter for a GT may be related to distal migration of an inflated Foley catheter into the small intestine. This can result in considerable distention and discomfort for the patient. Deflating the Foley balloon and repositioning it may solve the problem.
Tracheostomy Tube (TT). TTs frequently become obstructed with secretions. The TT that is obstructed with mucus may not be initially recognized. It is important to change the TT in a child if there is respiratory distress. It also is important to reestablish the position of the TT because the patient may not be able to breathe when he/she falls asleep or changes positions. If the TT is dislodged for several hours, it may be difficult to reinsert and the assistance of a specialist experienced with the care of TT may be required. Normal secretions are thin (those that run through the suction catheter), and range from clear to white. Abnormal secretions are thick, yellow, green, brown, or bloody and can be caused by the lack of humidity or by infection. Bacterial cultures should be obtained if the patient has a fever. When the patient has a positive tracheal aspirate and a fever higher than 38°C, antibiotics are indicated. A tracheal aspirate is not beneficial for diagnosing viruses. To diagnose viral infections associated with the respiratory syncytial virus (RSV) or one of the influenza viruses, a nasal aspirate is required. Bleeding from the TT is another troublesome problem. While mild bleeding often is due to a tracheobronchitis or vigorous suctioning, it also may herald a catastrophic event such as erosion of the tube into a major blood vessel. An urgent bronchoscopy may be required to evaluate where the blood is originating.
Colostomy. Children with a colostomy occasionally present with abdominal distention and decreased stool output. It is important to recognize that the stoma may have constricted, resulting in failure of passage of stool.
Assessing Durable Medical Equipment (DME) Necessary for Care
Always ask about any durable equipment that the child may use at home; it is not always apparent when a child is receiving technological support. Children who are on night time assisted ventilation may present without their ventilators. During the commotion of the acute event, a parent also might forget to inform the medical caregiver that their child is intermittently receiving assisted ventilation. Children with assisted ventilation usually will have a tracheostomy and equipment for humidifying the inhaled air and administering supplemental oxygen. Most children who are on ventilator support will have monitoring equipment available, which might include an apnea-bradycardia monitor and/or oximeter. A download from these monitors may provide acute insights into the course of recent events. The name, address, and phone number of the CME company often is attached to the equipment and it should have knowledgeable representatives on-call 24 hours a day. If the presenting problem is related to malfunctioning equipment, it is very important to enlist the services of the DME company to correct the malfunction.
Avoiding Pitfalls in the Emergency Management of Children with Special Healthcare Needs
Many pitfalls exist when caring for a CSHCN. Some of the more common ones are presented in Table 2 and discussed below.
 |
Failure to Fully Involve the Family and Utilize the Care Plan. The family/caregiver knows the child better than anyone else. They are the primary source of care for the child and can provide invaluable information in an emergency situation. The family can tell you the child's diagnoses and equipment needs and identify subtle changes in the child's normal baseline vital signs that might otherwise be overlooked or not recognized as significant. The caregiver and/or home health provider accompanying the child can note any medications given prior to transport to the hospital and may have important information about previous treatments that have worked well for this particular child.2 The patient's care plan also can be a valuable source of information about the child's diagnoses, baseline vital signs, medications, subspecialty and home health providers, past surgeries, and hospitalizations.
Inaccurate Weight. The child who is non-ambulatory or who has significant physical deformities may not be able to stand on a scale or even lie flat and quiet for his/her weight to be obtained. Accurate weights are a necessary tool in the provision of high-quality, safe care. Ask the parent or caregiver who is accompanying the child about the most recent weight without the wheelchair. Many EDs have special scales available to weigh such children. If the CSHCN has a care plan, this should list both the wheelchair weight and the child's most recent weight without the wheelchair.
Child's Inability to Communicate Pain. Many CSHCN have an inability to fully and specifically communicate pain. This may only be revealed through the parent's recognition of subtle changes in the child's behavior or sleep/wake patterns. When necessary, pain medications should be used as would be appropriate for any child, while watching carefully for drug interactions and hypoventilation.
Medication Errors. The CSHCN is frequently on multiple medications, and the family or caregiver usually knows the medications, dosages, and timing. Often, these items are listed in the child's care plan. If a home health provider accompanies the child, he or she should bring a notebook containing current medications and their timing and dosages, as well as when the medications were last given. This is important information to review to avoid adding medications that may have an adverse reaction with medications the child is currently receiving.
Clinicians should be conscious of continuing time sensitive medications during the course of a prolonged ED visit. Also, a medication review may reveal a medication dosage error or adverse interaction as the source of the child's current medical event.
Latex Allergy. CSHCN, especially those with meningomyelocele, have had multiple procedures; this places them at risk for latex allergy. If a definite tolerance to latex is not documented, treat the child as if he/she has latex allergy.2 All EDs should have non-latex gloves and tubing available.
Methemoglobinemia. Be prepared for rare events related to procedures. A CSHCN undergoing procedures requiring benzocaine or other topical anesthetics could develop methemoglobinemia, which may be mistaken for cyanosis arising from the child's underlying medical condition. Clinicians should consider this diagnosis when there is sudden cyanosis following a procedure requiring the use of a topical anesthetic.3
Intubation in the Child with Fragile Vertebrae or Craniofacial Anomalies
Many CSHCN have craniofacial anomalies or fragile, brittle cervical vertebrae related to their underlying condition. These children may require alternative methods of intubation or securing the airway. Some medical conditions that may be associated with difficult intubation or a high likelihood of cervical vertebrae injury include Treacher Collins, Pierre Robin, and Klippel-Fiel syndromes; craniosynostosis;4 rheumatoid arthritis with cervical instability;5 Down syndrome with cervical spine instability;6 the mucopolysaccharide syndromes due to tissue infiltration;7 and arthrogryposis multiplex congenital with micrognathia.8 Down syndrome patients have a smaller subglottis and trachea than other children their age and require an endotracheal tube two sizes smaller than that recommended on standard charts for normal children of the same age.6,9 A chart showing endotracheal tube sizes for Down syndrome children was published in Laryngoscope.9 It also is important to evaluate Down syndrome children for cervical spine instability prior to intubation to avoid injury to the spinal cord.6
Problems with Technological Equipment
Tracheostomy Tube (TT). When clinical evaluation indicates an obstruction of the TT and suctioning does not relieve the obstruction, it may be necessary to change out the TT. Clinicians should be sure to deflate the cuff if one is present.10 A spare TT may be available in the patient's equipment bag, if they have one. It may be necessary to use a smaller size TT if the stoma is constricted.10 A detailed discussion of the obstructed or dislodged TT and its replacement is provided in Emergency Care of the Technology Assisted Child by Posner and colleagues.10
Gastrostomy Tube (GT). Consult the child's family and the care plan to determine whether the child has a GT or a jejunostomy tube (JT). If the GT is obstructed, a trial of irrigation with warm water may relieve the obstruction.10 If the GT is dislodged and has been in place > 1 month, it is important to replace it promptly so that the stoma will not become constricted. The stoma should be checked first to be sure there are no tears present.10 If it has been in place < 1 month, consult with the specialist who originally placed the GT. The patient's equipment bag may contain an extra GT of the appropriate size.2 If not, the GT size may be obtained from the family or the patient's care plan. A Foley catheter can be inserted to maintain the stoma opening until a correct size GT is located.10 For a detailed, excellent discussion of the plugged or dislodged GT and its replacement, see reference 10.
Bleeding from the Stoma of the GT or TT. Superficial bleeding due to granulation tissue can be alleviated with silver nitrate application. For deeper sources of minor bleeding, consider infection, lower site granulation tissue, or inadequate humidification in the case of TT and gastric irritation or ulceration in the case of GT.10 A large amount of bleeding from either the TT or GT requires immediate intervention. Excessive TT bleeding may indicate that a major blood vessel has been eroded.10
Apnea Monitors. If the child arrives to the ED with an apnea monitor, recorded data from the monitor can be downloaded to assess whether suspected apnea and tachy- or bradycardic events were true events.
Failure to Aggressively Treat Fever in a Child with Sickle Cell Disease
Fever (a temperature greater than 38.4°C) in a child with sickle cell disease must be considered a medical emergency.11,12 These patients should be triaged immediately from the ED desk to be seen by the ED physician. Prompt empiric IV antibiotic therapy should be given as soon as blood cultures, CBC, and reticulocyte count are drawn to prevent what can become fulminant and fatal sepsis.12
Failure to Recognize Maltreatment in a Child with Disabilities
It is important to consider child abuse in the differential diagnosis of a CSHCN who presents to the ED. Recent evidence revealed that children with disabilities were more likely to experience multiple episodes and multiple forms of abuse than their non-disabled peers. They also are nearly four times more likely to be neglected and physically abused and three times more likely to be sexually abused.13
Specific Problems
Respiratory Complications in Children with Neuromuscular Diseases. Respiratory complications are a common cause of morbidity and mortality in children with neuromuscular diseases.14 (See Table 3.) In these children, weakness in the muscles of respiration, including the diaphragm and the chest wall muscles, results in a weak cough, shallow breathing, and potentially hypoventilation. The patient is, therefore, vulnerable to atelectasis, pneumonia, and airway obstruction by mucus plugging.
 |
All children with neuromuscular diseases eventually will develop respiratory disturbance during sleep.15 Often these disturbances, such as alveolar hypoventilation, are the first evidence of respiratory muscle weakness. Sleep-disordered breathing and alveolar hypoventilation can occur even though minimal peripheral muscle weakness is present.16 Vulnerability to respiratory disturbances during sleep in children with neuromuscular diseases is present throughout sleep but is most pronounced during REM (rapid eye movement) sleep. This is the consequence of three physiological factors, which include: 1) marked deregulation of control of respiration that occurs during REM sleep; 2) the atonia of the upper airway and intercostal muscles; and 3) the dependence of respiration on diaphragm function. Although often nonobstructive in origin because of weakness of the respiratory pump muscles, the sleep-related hypoventilation may have an obstructive component because of upper airway tissue laxity, particularly when other predisposing factors, such as obesity, are present. Sleep-related hypoventilation/hypoxemia due to neuromuscular disorders may be accentuated in the presence of obstructive sleep apnea.
Potential consequences of sleep-related hypoventilation include sleep disruption with lethargy, hypoxemia, and hypercarbia. Untreated sleep-related hypoxemia and hypercarbia may have a causal role in the development of respiratory failure because of disordered ventilatory control resulting from adaptation and downregulation of the ventilatory responses to these stimuli.
Any dysfunction of the diaphragm whether neurogenic as in motor neuron disease or phrenic nerve paralysis, neuromuscular as in myasthenia gravis or the muscular dystrophies, or extrinsic as in morbid abdominal obesity will interfere with breathing during REM sleep.17 As a result, REM sleep will be aborted, causing REM sleep deprivation. In less severe cases, it will be fragmented by multiple arousals and awakenings. In general, diaphragmatic insufficiency is more noticeable when the individual sleeps supine because in this position the contents of the abdomen upwardly displace the flaccid, weak, or dysfunctional diaphragm, compressing the lungs and placing an additional burden on ventilation. Aborted REM sleep and fragmentation of sleep are not the only consequences of diaphragmatic insufficiency. Hemoglobin desaturations of oxygen are profound, translating to weak respirations and hypoventilation. Desaturations are so conspicuous that REM sleep can be identified in the polysomnogram and more readily in the hypnogram by the evolutions of the oxygen saturation graph. In addition to hypoventilation, the interplay between weak intrathoracic negative pressures and oropharyngeal muscle collapsibility determines the presence of apnea.
The speed and severity of respiratory and cardiac decompensation with hypoventilation make early diagnosis important; however, patients and their physicians may not be aware of the significance of such symptoms. Many patients have symptoms for weeks or months before seeking help or before the severity of the problem is recognized. Cyanosis, abnormal respiratory muscle activity, and right heart strain are important diagnostic clues. In patients with neuromuscular disorders, vital capacity is an important measurement of respiratory function, and can be conveniently monitored with simple portable equipment.
Abnormalities of chest wall configuration, such as scoliosis and pectus excavation, are very common in patients with neuromuscular disease; this further contributes to poor lung expansion due to chest wall restriction.14,18 In patients with weak bulbar musculature, aspiration of secretions aggravates and accelerates respiratory problems.
In patients with severe muscle weakness, potentially life-threatening complications may occur during minor respiratory illnesses such as upper respiratory infections.19 Acute upper respiratory infections can lead to atelectasis or pneumonia by several mechanisms. The infection itself often is accompanied by an increase in the quantity of nasal and oral secretions. Nasopharyngeal secretions cannot only increase in amount but they also can become thicker, especially if they become purulent. This combination can overwhelm an already compromised swallowing mechanism and, therefore, can lead to an increased risk of aspiration of infected upper airway secretions into the lower respiratory tract. Weakened respiratory muscles result in an impaired cough mechanism; therefore, those secretions are more difficult to clear from the lower airways. Airway obstruction from retained secretions can then result in areas of atelectasis or pneumonia. Acute viral upper respiratory infections also have been shown to cause an acute deterioration in respiratory muscle strength and mucociliary clearance.20 Therefore, a patient with marginal respiratory muscle strength could develop weakness that is severe enough to hamper effective airway clearance during an acute viral illness.
The role of immunization against influenza virus or of immunoprophylaxis against RSV has not been studied in a population of children with neuromuscular weakness. Nevertheless, such patients have been identified as being at increased risk for more severe disease in the event of infection. Severe neuromuscular weakness was recently cited as a risk factor for more severe RSV disease in preterm infants born between 32 and 35 weeks gestation.20-22
Because all respiratory complications of neuromuscular disease are mechanical in nature (i.e., weak cough, dysphagia, hypoventilation), mechanical techniques for clearing the airway and improving ventilation are indicated. The concept of respiratory therapy should be introduced at diagnosis. Therapists can instruct parents in chest percussion and assisted cough. Bronchodilators may improve drainage, as will chest percussion. Intermittent positive-pressure breathing may reverse atelectasis. Percussive vests are an emerging technology.
Ventilatory support, either invasive or non-invasive, can reverse or prevent many of the physiologic difficulties associated with respiratory muscle weakness in patients with neuromuscular diseases.23,24
The application of bilevel positive airway pressure is the accepted non-invasive mode of treatment in individuals with REM sleep-related diaphragmatic insufficiency because continuous positive airway pressure (CPAP) is less well tolerated by patients with neuromuscular diseases.17,23 The suggestion has been made that an auto CPAP, capable of changing pressures from breath to breath, may be more amenable for the treatment of patients whose respiratory pressure requirements change significantly from non-REM to REM sleep. All of these considerations require confirmation with appropriate clinical trials.
Among patients with a neuromuscular disorder and nocturnal ventilatory compromise, positive airway pressure applications during sleep vastly improve the quality of sleep, and as a consequence the overall quality of life.23
Cerebrospinal Fluid Shunt Malfunction
Cerebrospinal fluid (CSF) shunts have become the treatment of choice for pediatric patients with increased intracranial pressure that is primarily due to hydrocephalus. There are approximately 18,000 shunts placed annually in the United States.25 Unfortunately, the complication rate after CSF shunt placement is high, with as many as 25-40% failing during the first year after placement.26 Approximately 56-80% of patients will experience at least one episode of malfunction in the 10 years after insertion, and the annual rate of shunt malfunction is estimated to be 5%.27
Children with CSF shunts often present to the ED with common symptoms such as headache, fever, vomiting, lethargy, or irritability. These symptoms often overlap with common childhood illnesses, and the ED physician must decide whether a shunt malfunction is present.
Shunt Malfunction. Symptoms of shunt malfunction include headache, lethargy, irritability, lack of developmental progress, poor school performance, nausea, vomiting, and forced downgaze (sunsetting sign). It is critical to obtain a history of prior shunt malfunctions and to inquire whether the clinical presentation was the same as it is presently. The most common complaints predictive of shunt malfunction are lethargy and shunt site swelling.25 Patients with increased ICP (intracranial pressure) may present with hypertension, bradycardia, and irregular respirations (Cushing's triad). This is a neurosurgical emergency. Workup of shunt malfunction includes CT (computed tomography) scan to assess ventricular size, shunt series x-rays to rule out disconnection and dislocation of shunt tubing, and potentially a shunt tap or nuclear medicine shunt flow study. Clinicians also should inquire whether the ventricles were previously dilated with a malfunction. An unchanged CT scan in a patient who does not dilate their ventricles with shunt failure does not rule out a shunt malfunction.28 The most common cause of shunt malfunction is ventricular catheter obstruction. Valve and distal catheter obstruction are somewhat less common. The treatment of shunt malfunction is shunt revision, with replacement of the occluded component, and perhaps revision of the valve, to decrease further malfunctions.
Shunt Infection. Infection rates in shunts for hydrocephalus vary from 1% to 40% in published series, with most in the range of approximately 5-10%.26 Greater than two-thirds of all shunt infections are due to staphylococcal species, with Staphylococcus epidermidis being the most common (47-64%) and S. aureus less so (12-29%).29,30 Gram-negative organisms, especially Escherichia coli and Klebsiella species, are responsible for 6-12% of shunt infections. These infections almost always present within 6 months of shunt surgery, with the gram-negative and S. aureus infections seen earliest. These are followed by S. epidermidis, which is most commonly seen 1-3 months following surgery. The incidence of shunt infection decreases markedly after 6 months of no shunt manipulation by either surgery or tapping. In atrial shunts, however, the risk of shunt infection continues due to the presence of hardware in the blood stream and the potential for contamination with bacteremia.
Patients with shunt infection may present with or without fever, and may only manifest shunt malfunction symptoms.10,26 A shunt tap should be performed by a qualified provider in any patient who has presented within 6 months of shunt manipulation, and CSF sent for gram stain, culture, glucose, protein, and cell count. The treatment of shunt infection involves removal of infected hardware, placement of a temporary external ventricular drain to handle CSF drainage, and intravenous antibiotic therapy. Occasionally, intrathecal antibiotic therapy is required as well. The duration of antibiotic therapy for shunt infection is a matter of debate, but usually with sterilization of the CSF, the shunt can be replaced within 7-14 days of removal. Prolonged antibiotic therapy after shunt reinsertion is not required.
Case Example. A 2 ½-year-old female with hydrocephalus and a ventriculoperitoneal shunt presented to the ED with a one-day history of vomiting, fever, and decreasing level of consciousness. Evaluation by the ED physician confirmed altered consciousness as well as documenting otitis media and 10% dehydration. A CT scan was obtained and interpreted as negative (comparison to previous CT scans was not done).
A rapid infusion of 5 cc/kg of 0.9% saline solution was given intravenously and followed by an infusion of D5 0.2% saline solution at approximately twice a maintenance rate. She was given antibiotics for the otitis media and admitted to a general pediatric ward service. Eight hours after admission, she experienced seizures and a respiratory arrest. After successful stabilization, she was found to have hyponatremia (sodium level was 121 mEq/dL) and a shunt infection.
This case example allows the development of key points when evaluating children with cerebrospinal fluid shunts:
- The most important caveat to remember is that in any patient with a CSF shunt in place, the shunt is the source of the problem until proven otherwise.28
- Common signs and symptoms of shunt malfunction include headache, vomiting, nausea, and altered mental status. A caregiver of a child with a CSF shunt who states that the child is not acting or "looking right" often is correct in making the diagnosis of shunt malfunction.
- A "negative" CT scan does not rule out shunt malfunction or obstruction.26,28 Approximately 11% of shunt failures present with small ventricles; therefore, CT scan should not be used as the only or definitive diagnostic modality when evaluating shunt function.31 If prior images exist, it is important to compare them to the current ones. A neurosurgeon should assist in the evaluation of even a negative CT scan or MRI if the child is not improving.
- Utilization of hypotonic fluid at rapid rates should be considered carefully, especially in children with neurologic dysfunction.32,33
Cerebral Palsy (CP)
An international symposium was held by a group of selected leaders in the preclinical and clinical sciences to reconsider the definition and classification of CP. The consensus of the group was that "CP describes a group of disorders of the development of movement and posture, causing activity limitation, that are attributed to non-progressive disturbances that occurred in the developing fetal or infant brain. The motor disorders of cerebral palsy are often accompanied by disturbances of sensation, cognition, communication, perception, and/or behavior, and/or by a seizure disorder."34
CP affects about 1.5-2.0 children per 1,000 live-births per year or about 10,000 children per year in the United States.35 CP is the third most common major developmental disability, after autism and mental retardation.35 The spectrum is wide, ranging from those with normal intelligence and mild motor deficits to severely disabled children who cannot talk or walk. CP can be classified based on the area of brain damage. The four major classifications are spastic, athetoid, ataxic, and mixed. Eighty percent of causes of CP are unknown. Known causes include infection, malnutrition, and head trauma. Most cases are thought to occur prior to birth but a few occur during childbirth or postnatally. Conditions associated with CP include mental retardation, seizures, learning problems, attention deficit hyperactivity disorder, and sensory problems. Sensory problems include vision, hearing, and speech impairments. Some children have tactile hypersensitivity or hyposensitivity; others may be dyspraxic.
Pain Assessment. Assessing pain in children is complex, particularly when the child is cognitively impaired and/or unable to communicate verbally. Neurologic impairments may affect the child's ability to comprehend and communicate pain.36 The assessment of pain in communicating children relies extensively on third party observations. Parents can make important contributions to this process, although their knowledge often is overlooked. Parents of non-communicative children with severe cognitive impairment are confident that they can detect pain, inferring it from indirect cues such as crying, facial expression, body movements, or behavioral and emotional change.36
Initial Approach. Children with CP who present for emergency care may provide a special challenge to the medical caregiver. (See Table 4.) Those with normal cognitive function and mild to moderate motor disabilities are usually straight forward and can be managed in the same manner as a normal child. Those with major disabilities, including seizures, growth retardation, communication disabilities, and flexion contractures (e.g., scoliosis), can present major diagnostic dilemmas. The initial approach to these children can be daunting due to the loss of normal anatomic configurations and physiologic responses. It is important to make specific observation of the child as a history is obtained. The purpose of the encounter is to focus on the particular problem initiating the visit. Associated conditions such as movement disorders and abnormal posturing may provide confusing signs and symptoms but are not usually the reason for the encounter. Certainly, abnormal movements and spastic posturing may be exacerbated by the acute illness.
 |
CP is a nonprogressive disorder; however, some of the associated conditions such as flexion-contractures, scoliosis, and feeding disorders can worsen. This progression may result in increasing respiratory problems because of decreased resistance to infection, increased tendency to aspirate, and inability to ambulate. The progression of these associated problems may be due to increased muscle tightness and progressive musculoskeletal deformities. Respiratory problems may not become very notable until a stressful event occurs. Such stressful events include a fever, a seizure, or an episode of pneumonia/atelectasis.
It is important to recognize that the musculoskeletal abnormalities as well as problems with hydrocephalus may result in breathing difficulties. Progressive hydrocephalus may result in loss of laryngeal function, including vocal cord paralysis that causes stridor. The changes in the spinal column relating to scoliosis may be associated with thoracic lordosis, which also can result in changes in the trachea that are consistent with tracheomalacia. In these individuals, the trachea lies anterior to the spinal column, causing lordosis and stretching of the trachea such that it flattens. This may lead to progressive obstructive lung disease and even respiratory insufficiency or failure. Both of these conditions can necessitate intubation that may be difficult to reverse. Ultimately, tracheostomy may be necessary to provide an adequate airway. This is one of the conditions that suggests that cerebral palsy is a progressive condition; however, the progression appears to be related to acquired secondary problems.
Conclusions and Reflections on the Future Management of the CSHCN
While challenging and complicated, caring for the CSHCN in the ED can be gratifying. Efforts are underway at the national level to create a system of care for the CSHCN that is based on an enhanced primary care medical home. Pediatricians, family medicine clinicians, and pediatric residents are beginning to be trained in the longitudinal care of these children and their families. Continuing medical education programs are being developed to support the needed education about new services and technologies for the CSHCN.
In the ideal healthcare system of the future, every medically complex child presenting to the ED will have an up-to-date care plan that lists all of the medical diagnoses, the current medications and accurate dosages, specialists participating in the patient's care, home nursing services, and ancillary therapeutic services. The medical history generated both locally and at the patient's tertiary medical center will be accessible electronically. Caregivers also will carry a summary of these histories in printed form with the care plan. A "go bag" will be stocked with appropriately sized tubes and catheters.10 A healthcare provider at the primary care medical home will personally know the patient and family and be accessible to the ED physician 24 hours a day.
As these children age into adulthood, transition of the primary care medical home from the pediatrician to a knowledgeable adult provider will occur. Physicians with medicine/pediatric residencies will be especially appropriate for these transitions. When death becomes imminent or appropriate, palliative care services will become available locally. (See Table 5 for information regarding the emergency physician's role.)
 |
As one reads promotional literature from children's hospitals, the term "patient and family centered care" is increasingly promoted as the ideal. This philosophy of care recognizes the primacy of the patient and the patient's family in determining how, where, when, and how much care is needed. Incorporating the parent(s) with the child, if appropriate, in medical decision-making and updating them frequently allows for the development of a respectful and trusting relationship that facilitates the best possible outcome. A frequent mantra at a patient-centered facility is: "Nothing about me, without me."
If these needed advances are not occurring locally at your hospital, the ED physician should become an advocate for change. Contact local child advocacy organizations, parent support groups, and pediatric societies to begin the dialogue of making a seamless system of care for children with special healthcare needs. Often a parent or grandparent of a child who has recently been severely ill or died may become a champion for the cause of an improved system of care for these special children. Pediatricians from the tertiary center closest to you may be able to direct you to support groups established in other communities. Capable and appreciative allies also should be found in your local primary care physicians and parents or caregivers. Clinicians may find themselves looking forward to helping the next child who presents with a complex and chronic problem.
References
1. Williams TV, Schone EM, Archibald ND, et al. A national assessment of children with special health care needs: Prevalence of special needs and use of health care services among children in the military care system. Pediatrics 2004;114:384-393.
2. Smith E, Adirim T, Singh T. Special kids, special care. Emergency management of children with special health-care needs. JEMS 2000; 25:82-93.
3. Dahshan A, Donovan GK. Severe methemoglobinemia complicating topical benzocaine use during endoscopy in a toddler: a case report and review of the literature. Pediatrics 2006;117:e806-e809.
4. Vaughan C. Anesthetic management of children with craniofacial anomalies. CRNA 1997;8:123-134.
5. Bandi V, Munnur U, Braman SS. Airway problems in patients with rheumatologic disorders. Crit Care Clin 2002;18:749-765.
6. Kanamori G, Witter M, Brown J, et al. Otolaryngologic manifestations of Down syndrome. Otolaryngol Clin North Am 2000;33:1285-1292.
7. Kempthorne PM, Brown TC. Anaesthesia and the mucopolysaccharidoses: a survey of techniques and problems. Anaesth Intensive Care 1983;11:203-207.
8. Nguyen NH, Morvant EM, Mayhew JF. Anesthetic management for patients with arthrogryposis multiplex congenita and severe micrognathia: case reports. J Clin Anesth 2000;12:227-230.
9. Shott SR. Down syndrome: analysis of airway size and a guide for appropriate intubation. Laryngoscope 2000;110:585-592.
10. Posner JC, Cronan K, Badaki O, et al. Emergency care of the technology assisted child. Clin Ped Emerg Med 2006;7:38-51.
11. Strandjord SE, Humbert J, Daeschner CW, et al. Hematologic and oncologic emergencies. In: Pediatric Hospital Medicine: Textbook of Inpatient Management. Perkin RM, Swift JD, Newton DA, eds. Lippincott, Williams and Wilkins: Philadelphia; 2003:378-382.
12. Miller ST, Rao SP. Sickle cell anemia with fever. In: Atlas of Pediatric Emergency Medicine. Shah BR, Lucchesi M, eds. McGraw-Hill Companies: New York; 2006:463-465.
13. Hibbard RA, Desch LW; American Academy of Pediatrics Committee on Child Abuse; Neglect and Council on Children With Disabilities. Maltreatment of children with disabilities. Pediatrics 2007; 119:1018-1025.
14. Birnkrant DJ. The assessment and management of the respiratory complications of pediatric neuromuscular diseases. Clin Pediatr (Phila) 2002;41:301-308.
15. Givan DC. Sleep and breathing in children with neuromuscular disease. In: Sleep and Breathing in Children: A Developmental Approach. Loughlin GM, Carroll JL, Marcus CL, eds. Marcel Dekker, Inc.: New York; 2000:691-735.
16. Heckmatt JZ, Loh L, Dubowitz V. Nocturnal hypoventilation in children with nonprogressive neuromuscular disease. Pediatrics 1989; 83:250-255.
17. Culebras A. Diaphragmatic insufficiency in REM sleep. Sleep Med 2004;5:337-338.
18. Kennedy JD, Staples AJ, Brook PD, et al. Effect of spinal surgery on lung function in Duchenne muscular dystrophy. Thorax 1995;50:1173-1178.
19. Bach JR, Ishikawa Y, Kim H. Prevention of pulmonary morbidity for patients with Duchenne muscular dystrophy. Chest 1997;112:1024-1028.
20. Panitch HB. Viral respiratory infections in children with technology dependence and neuromuscular disorders. Pediatr Infect Dis J 2004;23(11 suppl):S222-S227.
21. Izurieta HS, Thompson WW, Kramarz P, et al. Influenza and the rates of hospitalization for respiratory disease among infants and young children. N Engl J Med 2000;342:232-239.
22. Meissner HC, Long SS; American Academy of Pediatrics Committee on Infectious Diseases and Committee on Fetus and Newborn. Revised indications for the use of palivizumab and respiratory syncytial virus immune globulin intravenous for the prevention of respiratory syncytial virus infections. Pediatrics 2003;112(6 Pt 1):1447-1452.
23. Perkin RM, Fontane E. Noninvasive ventilation in pediatrics patients: role as a supportive technique in the ED. Pediatric Emergency Medicine Reports 2006;11:85-96.
24. Niranjan V, Bach JR. Noninvasive management of pediatric neuromuscular ventilatory failure. Crit Care Med 1998;26:2061-2065.
25. Kim TY, Stewart G, Voth M, et al. Signs and symptoms of cerebrospinal fluid shunt malfunction in the pediatric emergency department. Pediatr Emerg Care 2006;22:28-34.
26. Fuchs HE. Hydrocephalus, cerebrospinal fluid shunts and their complications. In: Pediatric Hospital Medicine. Perkin RM, Swift J, Newton DA, et al, eds. Lippincott, Williams, and Wilkins: Philadelphia; 2007 (in press).
27. Madikians A, Conway EE Jr. Cerebrospinal fluid shunt problems in pediatric patients. Pediatr Ann 1997;26:613-620.
28. Perkin RM. Understanding hydrocephalus and the ED management of VP shunts and their complications. Pediatric Emergency Medicine Reports 2005;10:37-48.
29. Ersahin Y, Melone DG, Storrs BB, et al. Review of 3017 procedures for the management of hydrocephalus in children. Concepts Pediatr Neurosurg 1989;9:21-28.
30. Choux M, Genitori L, Lang D, et al. Shunt implantation: reducing the incidence of shunt infection. J Neurosurg 1992;77:875-880.
31. Iskandar BJ, McLaughlin C, Mapstone TB, et al. Pitfalls in the diagnosis of ventricular shunt dysfunction: radiology reports and ventricular size. Pediatrics 1998;101:1031-1036.
32. Holliday MA, Friedman AL, Segar WE, et al. Acute hospital-induced hyponatremia in children: a physiologic approach. J Pediatr 2004;145:584-587.
33. Jackson J, Bolte RG. Risks of intravenous administration of hypotonic fluids for pediatric patients in ED and prehospital settings: let's remove the handle from the pump. Am J Emerg Med 2000;18:269-270.
34. Bax M, Goldstein M, Rosenbaum P, et al. Proposed definition and classification of cerebral palsy, April 2005. Dev Med Child Neurol 2005;47:571-576.
35 Cooley WC; American Academy of Pediatrics Committee on Children with Disabilities. Providing a primary care medical home for children and youth with cerebral palsy. Pediatrics 2004;114:1106-1113.
36 Stallard P, Williams L, Lenton S, et al. Pain in cognitively impaired, non-communicating children. Arch Dis Child 2001;85:460-462.
With the increasing skill of our NICUs in saving extremely premature babies and the capability of rescuing children with acute illnesses and injuries in our PICUs, children with special healthcare needs are being discharged from tertiary centers and returning to their home communities.Subscribe Now for Access
You have reached your article limit for the month. We hope you found our articles both enjoyable and insightful. For information on new subscriptions, product trials, alternative billing arrangements or group and site discounts please call 800-688-2421. We look forward to having you as a long-term member of the Relias Media community.