Your Most Feared Calls: Assessing & treating pediatric patients with undiagnosed pre-existing conditions

JEMS Clinical Review Features
This clinical review feature article is presented in conjunction with the Department of Emergency Medicine Education at the University of Texas Southwestern Medical Center, Dallas

Course Objectives

>>”žIdentify causes, history components and assessment needs for children presenting with altered mental status and seizures.”ž

>>”žIdentify causes for tachypnea in infants and children.

>>”žIdentify co-existing medical disorders in children with Down syndrome.

>>”žDescribe factors that enhance patient assessment for children with Down syndrome.”ž

Glossary Terms
Atlantoaxial:Pertaining to the first two cervical vertebrae.
Adventitious: Abnormal, extra sounds heard with normal breath sounds, such as rhonchi, wheezes and crackles.”ž
Down syndrome:A congenital, chromosomal disorder characterized by varying levels of developmental delay and multiple physical defects.
Lipolysis: Fat breakdown.
Polydipsia:Increased thirst.
Polyphagia:Increased hunger.
Polyuria:Increased urination.
Tonic-clonic movements:Seizure activity characterized by involuntary stiffening and relaxation of muscles.

During the past several years, EMTs and paramedics have seen more and more children with underlying health conditions, including children with special health-care needs (CSHCN). Advances in medical knowledge, improved technology and more affordable medical equipment have allowed CSHCN to be cared for at home rather than in specialized facilities. As a result, when these children become ill or injured, EMS is called to assist the family in caring for them. The families are typically experts in providing the patient_s history and assisting EMS with equipment and devices.”ž

However, some pediatric calls can keep you guessing. The patient has no known pre-existing conditions, and the family may be extremely panicked and upset by the emergency. What causes tachypnea, altered mental status (AMS) and seizures in children? How do you assess and treat those causes?”ž

The following cases involve actual patients who were seen by an urban ambulance service. Consider how each case presented to the crews, and how you would assess and treat the patients.

Case #1
EMS was called to a residence for an unresponsive infant. On arrival, the infant_s grandmother met the crew at the door and informed them that her granddaughter was put down for bed the previous night at the normal time. However, this morning, the grandmother was unable to awaken the girl, and she called 9-1-1.”ž

The patient was found lying prone in her crib. She was pale and breathing 52 times per minute. Her heart rate was 164 apical. EMS applied oxygen at 12 lpm via a pediatric non-rebreather (NRB) mask. The patient_s oxygen saturation was 98% on oxygen.”ž

The crew found the patient to be a well-developed five-week-old female with no visible trauma on examination. The patient was afebrile, and the grandmother denied any recent illness. The infant had been vaginally delivered at 41 weeks gestation and was 7 lbs. 3 oz. on delivery. The grandmother wasn_t aware of any medical conditions the patient might have had.

The patient_s pupils were equal but slow to react to light. The infant was noted to be smacking her lips but had no other spontaneous movement. She was unresponsive to verbal and painful stimuli. EMS placed the infant in their ambulance and initiated transport to the closest facility.

Following local protocol, EMS checked the patient_s blood glucose level (BGL) and found it to be 83 mg/dL. The infant was”žgiven 0.4 mg naloxone IM as the “Unknown, unconscious” protocol specified. There was no change in the patient_s responsiveness after naloxone.”ž

EMS contacted medical control to report on the patient_s status and was advised to administer 0.8 mg diazepam via IV or 2 mg rectal, based on the infant_s Broselow Pediatric Emergency Tape length, because the patient was most likely having a seizure.”ž

Diazepam was given to the infant, and the patient was beginning to move her arms and grunt when EMS arrived at the hospital. The patient_s respirations were 36, heart rate 140, SaO 100% on oxygen, and she was opening her eyes spontaneously. Transport was completed without complications.

Discussion of Case #1
Epilepsy is a clinical syndrome in which a patient is subject to recurrent seizures, usually as a result of insult to the brain itself. Generalized seizures involve the entire cerebral cortex and usually begin with a loss of consciousness. Generalized seizures can be manifested as either grand mal or petit mal. Grand mal seizures are typically manifested bytonic-clonic movementsof the extremities, whereas petit-mal seizures (aka, absence seizures) may last only a few seconds and may be easily overlooked. In the case of petit mal seizure, the patient may simply “zone out” for a brief period and then resume normal activity without anyone noticing.

As opposed to the generalized seizure, a partial (or focal) seizure is caused by electrical discharges limited to a portion of the cerebral cortex and is often caused by a brain lesion. Patients may exhibit tonic-clonic movement in only one extremity. Visual disturbances or sudden, transient behavioral changes may also be signs of a focal seizure.

Seizures in an infant can also be caused by fever, hypoxia, congenital defect, hypoglycemia, trauma and infection. A complete and thorough history will assist EMS providers in ruling out many of these causes.”ž

In the case described, the grandmother would have known if the patient had congenital defects that may have caused a seizure disorder, if a disorder had been previously identified. The family would also be able to tell responders if the child had any illness recently, which should help rule out the presence of infection, such as meningitis. The child_s BGL was within normal limits, so dextrose would most likely have had little to no effect on the patient. Likewise, the patient_s oxygen saturation was adequate and did not appear to be the cause of the seizure.”ž

If no SaO device is available, traditional means of checking oxygenation, such as skin color (pink), capillary refill”ž(< two seconds), breath sounds (clear), and level of responsiveness (AVPU), are still reliable means of testing oxygenation. When in doubt, it_s prudent to administer oxygen. During assessment, the patient was found to be afebrile, so a febrile seizure was not suspected.

When evaluating an infant with a decreased level of responsiveness, be aware that abuse is a possible cause. It_s a sad truth that more children are being seen”žwho have been physically abused by a caregiver. Good scene evaluation skills are extremely important so that you can give a complete picture of the patient_s residence to the receiving facility. In addition, odd behavior on the part of the caregiver should alert you that the child could be a victim of abuse. In this case, the home was clean and well maintained. The grandmother was very upset, but her concern was appropriate. Therefore, abuse would be lower on your list of possible etiologies.

The physician at the receiving facility informed the EMS crew that infants experiencing grand mal seizures often display seizure activity by lip smacking or eye twitching or merely acute oxygen desaturation. Due to the undeveloped musculature, tonic-clonic movements of extremities may not be seen in an infant during seizure activity, hence presenting with such subtle signs as in this infant. The patient was released home with anti-seizure medication after neurology consult.

Case #2
EMS was called to a local urgent care center for a two-year-old male patient with a decreased level of responsiveness. They were informed by the physician that the patient had been seen at their center three times in the past three days. The patient was being treated for bronchiolitis/possible pneumonia and had been given albuterol nebulizers each time he was seen.”ž

The mother told EMS that the patient complained of stomach pain for two days and vomited twice the previous day. He had been prescribed amoxicillin two days prior, but it didn_t seem to be working, prompting the return visit.

The EMS crew found the patient lying on an exam table with a respiratory rate above 60, and oxygen saturation was 96% with blow-by oxygen in place. His inspiratory phase (I) to expiratory phase (E) ratio was matched at 1:1, and his lungs were clear. His heart rate was 148 at the radial site, blood pressure was 90/60, and skin was hot to the touch.”ž

The patient was irritable and restless. His temperature was 100.7_ orally, according to urgent care center staff. EMS examination of the patient revealed no trauma, but the patient whimpered when his abdomen was palpated. The staff informed the EMS crew that they had drawn blood for lab tests but the results weren_t back yet.

The EMS crew placed the patient on their stretcher and initiated transport to the hospital. The patient was noted to have a sweet odor on his breath, so the crew checked his BGL. The glucometer read “HI,” which indicated a reading greater than 650 mg/dL. The crew asked the mother about a family history. She reported a long history of diabetes in her husband_s family.”ž

The crew initiated an IV of normal saline (NS), and bolused the patient with 240 cc/NS based on a weight of 12 kg. The patient had no change in vital signs or responsiveness after the first bolus and was given a second fluid bolus of 240 cc/NS. The remainder of the transport was without incident.”ž

Discussion of Case #2
During early stages of insulin deficiency in a patient with diabetes, hyperglycemia is the primary feature. During pronounced states,lipolysiswill be noted. Prolonged hyperglycemia leads to tissue necrosis and ultimately to organ and system failure.

Patients with untreated or poorly regulated diabetes can develop renal failure, visual disturbances, vascular dysfunction, cardiac problems, liver dysfunction, stroke and overall circulatory dysfunction.”ž

New onset of juvenile diabetes is most commonly seen in children between the ages of four and six, but in rare cases it_s found earlier. The onset of Type 1 diabetes in the first two years of life has the greatest mortality if the diagnosis is delayed.”ž

This child_s presentation could be initially mistaken as purely respiratory in nature. He was in respiratory distress and tachypneic, but he had a normal, non-obstructive respiratory pattern (I:E ratio) and clear lungs. Metabolic causes for tachypnea are more likely, e.g., poisonings, acidosis, etc.”ž

In light of the high glucose, distinctive odor and abdominal pain, diabetic ketoacidosis (DKA) became the probable condition. Clearing the ketosis and correcting the acidosis are the hallmarks of treatment.”ž

From an EMS standpoint, judicious but sufficient fluid administration was indicated. Fluid administration will help lower the glucose, lower the ketosis and begin to correct the acidosis. The patient will require close monitoring for dysrhythmias and worsening AMS due to the electrolyte shifts that may occur with fluid correction.”ž

In this case, follow-up with the attending physician revealed that the patient had an actual BGL of 843 mg/dL. When the mother was interviewed, it was apparent that the child had been displaying all the recognized symptoms of hyperglycemia for the preceding week, includingpolydipsia, polyphagiaandpolyuria.The patient was diagnosed with juvenile onset diabetes and was started on an insulin regimen.

Case #3
EMS was called to respond to a motor vehicle collision at a major intersection. On arrival, they found two vehicles involved in a moderate speed crash. Their patient was a 14-year-old male who appeared to haveDown syndromeon visual inspection. He was seated in the front passenger seat of a van with his seatbelt still in place. The vehicle had damage to the driver_s side with approximately 6″ of intrusion into the passenger compartment. The patient was awake and complaining of neck pain and shortness of breath. The patient and his mother denied loss of consciousness.

She said her son had an underlying diagnosis of Down syndrome, but he was healthy. The child was afraid of the EMTs and didn_t want them to evaluate him. The mother advised them that he is always apprehensive of strangers. He was crying but able to answer questions appropriately. The crew talked with the patient and allowed him to watch them assess his”žmother so that he would know the assessment wouldn_t harm him. The patient then agreed to allow them to evaluate him.

Primary assessment revealed a respiratory rate of 22 with an SaO of 93% on room air and noadventitioussounds on auscultation. No discernable trauma to the patient_s chest was noted on palpation. The heart rate was 120 at the radial site, and the patient_s skin was warm, dry and pink.”ž

EMS placed a C-collar, moved the patient to a long backboard and placed him in the ambulance. Oxygen was applied at 12 lpm via NRB mask, and he was reassessed. Neurovascular status was present in all extremities and equal bilaterally. His chest was symmetrical, and abdomen was soft and without pain on palpation. His pelvis was stable to rocking, and no pain was elicited on palpation of extremities. He calmed as the EMS crew talked with him. No other significant problems were found on re-examination of the patient.

Transport to nearest trauma center was initiated. En route, the patient began to complain of numbness to his feet and hands. The crew re-evaluated him and found his motor strength weaker than noted immediately after immobilization. His level of responsiveness didn_t change, and his vital signs remained stable. The patient was turned over to the trauma team at the hospital without further incident.

Discussion of Case #3
Patients diagnosed with Down syndrome have a predisposition to several medical disorders, including hypothyroidism, congenital heart defects, hearing loss, visual disorders, seizure disorders,atlantoaxialinstability, developmental delays and digestive disorders. Children with Down syndrome are also 10à15% more likely to develop leukemia than other children and, compared with the general population, have a 12-fold higher mortality rate from infectious diseases if left unmonitored and untreated.

In most cases, EMTs and paramedics are taught to quickly evaluate the patient and get them to the hospital in an expedient and safe manner. In the case of a child with Down syndrome, taking extra time on scene and involving the family in the assessment process will improve the evaluation process.”ž

In order to properly evaluate these patients, EMS personnel must develop a rapport with them. If local protocol allows, keeping the parent with the child in the patient compartment will decrease the child_s anxiety and facilitate treatment.”ž

Family-centered care is becoming more accepted in the prehospital setting and works very well when treating children. Not only do most children calm down when their parent is present, most parents feel more comfortable if they can see their children being taken care of.

Atlantoaxial instability (AAI) is a diagnosis that denotes increased mobility at the articulation of the first and second cervical vertebrate of an affected child. Causes of AAI aren_t well understood but may be caused by abnormalities of the ligaments that maintain the integrity of the articulation, bony abnormalities of the cervical vertebrae, or both. AAI can cause spinal cord compression if not treated properly. Diagnosis of AAI in a patient with Down syndrome isn_t usually made until a traumatic event occurs for which the patient is required to have a CT scan of the cervical spine.

In this case, the patient_s shortness of breath and tachycardia were attributed to anxiety and fear; they resolved without further intervention. At the recommendation of subspecialists, a neck brace was carefully placed on the patient. The numbness of his feet and hands resolved over the following two hours.

An increasing number of children are being diagnosed with conditions that used to be treated in medical facilities but must now be addressed in the field. It_s important for EMS providers to recognize signs of underlying medical conditions that may be contributing to the present emergency. In addition, more children with serious medical problems are being cared for at home, and EMTs and paramedics should be ready to assess and manage children with trach tubes, ventilators, IV infusions, G-tubes, J-tubes, NG-tubes and other devices already in place. Finally, remember that the family is often your best source of patient history.”žJEMS

Linda Warren, EMT-P, is the EMS for Children coordinator at the Department of Emergency Medicine, University of New Mexico, Albuquerque. Contact her

Robert Sapien, MD, is a pediatric emergency medicine physician and is involved with the EMS for Children program at the University of New Mexico, Albuquerque.


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