Breaking Down Barriers

Autism is a developmental disability, characterized by abnormal communications and unique social behaviors, that affects one out of every 166 births.1 At least nine out of 10,000 individuals are impacted, and it is four times more common among males.1 The number of cases is increasing by more than 10% each year.1 Autism can be found in any community, regardless of socioeconomic status, lifestyle or ethnicity. In the next decade, it is projected that the number of cases in the United States could reach four million.1,2

     First described in 1943, autism is one of five disorders that fall under the heading of Pervasive Developmental Disorders, or PDD. These include a number of neurologic conditions involved with developmental impairment, including social interaction and communication. The four remaining PDDs include Asperger's Disorder, Childhood Disintegrative Disorder (CDD), Rett's Syndrome, and PDD-Not Otherwise Specified (PDD-NOS).1-3

Cause
     A single specific cause for autism has not been identified, although a number of possible causes have been explored.1,3 Years ago, it was suspected that cold, rejecting mothers, sometimes referred to as "refrigerator mothers," caused autism in children. It was also suspected that autism was the result of parental emotional or psychological deficits. These theories have been proved to be incorrect.1-3

     Contemporary theories for possible causes include genetic predisposition; obstetric complications; administration of vaccinations such as measles, mumps and rubella (MMR); and toxic exposures.1-6 Fragile X syndrome, tuberous sclerosis and congenital rubella syndrome have also been investigated.1-7 It is believed that the symptoms associated with autism result from alterations in cerebral (brain) structure or function.1-6 (Table I) provides a list of resources that can be consulted for additional information regarding causes and effects of autism.

Signs and Symptoms
     Autism is considered a "spectrum" disorder.1-13 Signs and symptoms can range from mild to severe, and no two cases are identical (Table II). A comprehensive assessment will need to be conducted to determine if the symptoms are related to autism or other medical conditions. Factors like the frequency of occurrence of symptoms, their severity, sequence of symptoms, cerebral development, patient's age and patient's baseline health will need to be considered.1-3,12,13

Language, Body Movements, Social Interactions
     Language abnormalities, rigid or repetitive patterns of behavior, and unique social interactions are seen in children with autism.1-3 Language and communication skills may be limited. Nearly 40% of children with autism are either unable to speak or experience significant speaking challenges.3,13 If they are able to speak, the speech may appear to be abnormal.3,13 For example, they may not appear to be able to control the volume of their speech and may experience pronominal reversals, such as saying "you" instead of "I."3,13 When engaged in conversation, they may dwell on the same topic rather than having a back-and-forth dialogue and may exhibit echolalia--the act of repeating what was just asked. Alternative language and communication skills, including sign language and pictures, may be used to support their ability to communicate.3,13

Body movements and behavior
     Abnormal movements and behavior may be observed, such as hand-flapping, when the child raises and lowers his/her arm rapidly. The wrist is often flaccid so it appears to be flapping in the wind. Hand-flapping may be most frequently observed when the individual is happy or excited. Unusual arm movements may also occur in combination with the child moving the entire body, such as jumping or spinning around in circles. Basic gestures like waving hello or good-bye may not make sense to autistic children. At social events, they may display "inappropriate" behaviors, such as wanting to be left alone or masturbating in public. Motor tics and the inability to remain still may occur, as well.3,13

Routines
     Autistic individuals often prefer a routine, and adjusting the routine even slightly may result in disruptive behavior. Changing a child's nighttime routine, for example, may be extremely upsetting and result in emotional or physical outbursts. In some cases, families may need to modify their plans to avoid changing the autistic member's routine.2 When possible, the care provider should be sensitive to the potential effect any change in routine might have on an autistic patient.

Reactions to stimuli
     Reactions to stimuli can range across a wide spectrum. The sense of touch, smell or hearing may appear to be abnormally over- or under-active. Textures normally considered soft or comfortable may be perceived as painful. Odors that are pleasing to most people may be unappealing and may even result in gagging. Normal sounds, such as a vacuum or motorcycle, may be upsetting or even painful. Depending on how he processes stimuli, the child may even respond with aggressive, self-injurious or violent behavior. Because of the extremes in sensory processing and subsequent responses, behaviors associated with autism may be, in part, the result of complications with the individual's ability to integrate and respond to stimuli.1-3,12

Human Contact & Toy Selection
     The autistic individual may avoid making eye contact during a conversation. The child may have difficulty talking about feelings and may not understand or appreciate the feelings of others. Children may resist being held or cuddled by parents or guardians. When the child plays with toys, he may focus on one specific part versus the entire object. For example, instead of playing with the entire toy truck, the child may focus on spinning one wheel. He may be fascinated by and spend hours watching traffic lights, fans, running water, or repeatedly flushing the toilet. Unique toy preferences may include collecting rubber bands, paper clips or pieces of paper.3,12

Stereotypy
     Autistic individuals may also exhibit stereotypies, which are patterned repetitive movements, postures and utterances. Stereotypies can be described as orofacial, extremity, or head and trunk. Orofacial stereotypy involves movements of the tongue, mouth and face, smelling or sniffing motions and making certain sounds. Extremity movements include the hands, fingers, toes and legs. Head and trunk patterns include rolling, tilting, banging the head, or rocking the entire body. In extreme cases, stereotypies can result in self-injurious behavior. Skin-picking, self-biting, head-punching and slapping, head-to-object and body-to-object banging, body-punching and slapping, and poking themselves in the eye are possible. Lip-chewing, removal of hair and nails, and teeth-banging may also occur.3,12

Behavior When Sick Or Injured
     The child with autism may exhibit atypical responses to pain or physical injury. Instead of crying and running to a parent when injured, he might continue with the activities that resulted in the injury. A change in the child's mood may be the only indicator that an injury has occurred. Parents have reported a decrease in self-injurious behaviors, aggression toward others, property destruction, temper tantrums and hyperactivity when the child has a fever. In some cases, when the child appears to be "calmer," this may be a flag to check for illness or injury.3,12

Discovery and Diagnosis
     Autism is frequently identified during childhood, often before the child reaches age three.1-3,8-10 The suspicion of autism may initially be raised during an unrelated health exam, such as a routine physical or well-baby check. During the appointment, the healthcare provider may notice that the child exhibits behavior patterns that are suggestive of autism.1-3 The parents/guardians may also report behavior patterns that can be associated with autism (see Table III).

     The diagnosis of autism is not based on a single patient-provider encounter, nor is there a single diagnostic test that can be processed to determine if an individual has autism. Rather, extensive patient assessments, observation of the patient's communication skills, and evaluations of mental development and behavior are needed. In addition, a variety of providers, such as a psychologist, developmental pediatrician, speech/language therapist, learning consultants and neurologist, may be involved in the assessment.11 Once the assessments have been completed, and after the healthcare team has collaborated, a diagnosis of autism might be determined. A number of other diseases need to be considered in the differential diagnosis (Table IV).

Differential Diagnosis
     When autism-like symptoms are present, you must determine if the symptoms are due to autism or if they are the result of another condition, such as a psychological or seizure disorder. It is estimated that more than 70% of individuals with autism have some form of mental retardation.1,2 Approximately 25% of autistic individuals experience seizure activity.1,2 The presence of co-existing conditions contributes to the complexity of assessing and diagnosing autism. As a result, determining the exact cause of the symptoms can be challenging (see Table V).1,2,11

Prehospital Assessment
     The prehospital assessment should begin before patient contact is made. Start by assessing the environment, which includes observing for the presence of any weapons, signs of substance abuse and any clues regarding the patient's medical history. Note whether the patient's environment is well-kept or disorderly. Because of the potential challenges with an autistic child, there may be a greater risk for abuse. As in all cases involving at-risk populations, the prehospital care provider will need to be particularly sensitive to any signs of potential abuse, as this encounter may be the only opportunity to identify this threat. The patient's overall health, the presence of potentially life-threatening conditions and bystander/guardian input will influence this assessment.3,13

     Once patient contact is made, evaluate mental status and ABCs. The AVPU method (A=patient is alert, V=patient responds to verbal, P=patient responds to painful stimuli, U=patient is unresponsive) and Glasgow Coma Scale (GCS) can be used to determine a rapid baseline neurologic assessment. If the patient appears to have an altered level of consciousness, or if the ABCs are at risk, immediate intervention is indicated.10,14

     Obtain at least one set of vital signs and note the patient's neurologic status, pulse, respirations, blood pressure and skin condition. This can be challenging, especially if the patient is displaying any behavioral episodes or is agitated. If the patient is acting out and obtaining a blood pressure is not possible, it may be necessary to rely upon assessment of the patient's skin to quickly determine perfusion status. If available and practical under the circumstances, cardiac monitoring, pulse oximetry and capnometry can also be used. Reassess vital signs every five to 10 minutes, with more frequent assessments as needed.10,14

     Conduct a head-to-toe survey. Due to the potential for the autistic patient to have self-inflicted or other injuries, be alert for signs of trauma. The DCAP-BTLS system--which looks for the presence of deformity, crepitus, abrasions, punctures, bleeding/burns, tenderness, lacerations or swelling--can be used when assessing for trauma. Any injury or trauma-related finding should be managed according to local protocols. Self-inflicted injury and abuse can be difficult to distinguish.

Prehospital Treatment
     Prehospital treatment will vary with each case and will require an understanding of the types of behaviors and responses that can be expected in autistic patients. It may not be readily obvious that the patient is experiencing an autism-related event. The EMS crew may have been called for a variety of reasons, including seizures, self-mutilation, or sudden and bizarre behavior. In some cases, it may not be immediately clear why EMS was summoned.

     Even though suggested guidelines or protocols for the management of autism in the prehospital setting are available, provider discretion may be necessary for giving optimal care. If the patient presents with an altered mental status, providers will need to investigate and treat (if possible) the potential cause, such as hypoglycemia. If the patient is experiencing violent or abnormal physical behavior, parental assistance and/or restraints may be needed to defuse the situation and avoid injury.10,14

     Administration of medications will be guided by factors such as the patient's condition, age, provider discretion and local protocols. Medication administration may range from supplemental oxygen to chemical restraints, depending on the situation. Providers should ensure that they are familiar with the medication prior to administration, especially in pediatric cases. This includes confirming the appropriate dose, route and indications, and understanding the potential side effects.1,2,10,14 It is important to be aware that there may be ways to calm autistic patients down that do not require restraints. Parents or care providers may be very helpful in suggesting techniques that will have a calming effect without restraining.

     If intravenous access is indicated, several factors will influence the location of the IV, as well as the fluids to be infused. For example, if the patient is volume-depleted or hypovolemic, administer a fluid bolus of 20 ml/kg of normal saline or lactated Ringer's. If the IV is primarily for a route of medication administration, then a dextrose solution (e.g., D5W) or normal saline may be preferred. Intravenous access locations and techniques may range from intraosseous (IO) infusion to the antecubital fossa (A/C) to the external jugular (EJ), depending on the patient's size, age and condition.10,14

     In situations when safety is at risk, physical and/or chemical restraints may need to be considered. If restraints are going to be used, try to let the parents know your intent to use them before they are actually applied. Follow local protocols regarding type and use of the restraint.10,14

     EMS providers should always consult the parents from the start of the call. Talk with the parents or guardians when assessing and treating the autistic patient, especially if the patient is young, as the parents may be the primary source of patient information. The autistic patient may use alternative means (e.g., sign language, pictures) for communication. In these cases, parental assistance may prove to be invaluable.1,2,10,14

Prescribed Medications and Autism
     In general, medications prescribed for autism tend to influence associated symptoms, not the underlying cause or condition. Symptoms that may be targeted include those associated with ADHD, obsessive-compulsive disorder, depression, dysrhymia (depressed moods) and tic disorders. More than 25% of children and adults with autism take psychotropic drugs at some point, including antidepressants, antipsychotics and beta-blockers. Table VI provides a summary of the more commonly prescribed medications.1,2,3,8

Long-Term Care
     Long-term care of the autistic patient will be influenced by a variety of factors, including the patient's baseline state of health, clinician judgment and developments in the management of autism. Depending upon the results of the patient assessment and parents' reports, treatment may be designed around behavior, communication and/or social aspects of the patient's life. For example, if the patient exhibits behavior that poses a threat to the safety of self or others, behavior intervention may be needed. The patient who is experiencing speech or other verbal communication challenges may benefit from focused treatment plans. Patients displaying potentially inappropriate social behaviors (e.g., screaming in public) may benefit from treatment plans that focus on the specific behavior and/or the triggering event (e.g., experiencing frustration). Treatment will be individualized and may need to be altered depending on the outcomes.1-5,8,9

Conclusion
     Autism is a complex disorder. In the prehospital setting, where it may not be obvious if the patient is experiencing autism-related symptoms versus symptoms of another condition, parental input may prove to be invaluable. Having a good understanding of what can be expected when caring for an autistic patient is critically important to successful management. Thoroughly assessing and managing the patient will help to ensure that optimal treatment is provided.

References

1. Defining Autism. Autism Society of America. www.autism-society.org/site/PageServer?pagename=whatisautism.
2. Dagg P. Pervasive Developmental Disorder-Autism. www.emedicine.com/med/topic3202.htm.
3. Brasic J. Pervasive Developmental Disorder-Autism. www.emedicine.com/ped/topic180.htm.
4. National Institute of Health. National Institute of Child Health & Human Development. Autism Research at the NICHD. www.nichd.nih.gov/autism/.
5. National Institute of Health. National Institute of Child Health & Human Development. Autism Overview: What We Know. www.nichd.nih.gov/publications/pubs/autism_overview_2005.pdf.
6. Harris G, O'Connor A. On autism's cause, it's parents vs. research. The New York Times, New England Edition, June 2005.
7. The National Fragile X Foundation. What is Fragile X? www.fragilex.org/html/what.htm.
8. Willemsen-Swinkels S, Buitelaar J. The autistic spectrum: Subgroups, boundaries, and treatment. Psych Clin N Am. Chicago: Elsevier Science, 2002.
9. Deuel R. Autism: A cognitive developmental riddle. J Ped Neurol. Fairfax: IOS Press, 2002.
10. Owley T. Treatment of individuals with autism spectrum disorders in the emergency department: Special considerations. Clinical Pediatric Emergency Medicine. Chicago: Elsevier, 2004.
11. Autism Society of America. Diagnosing Autism. www.autism-society.org/site/PageServer?pagename=autismdiagnosis.
12. Autism Society of America. Autism Characteristics. www.autism-society.org/site/PageServer?pagename=autismcharacteristics.
13. Centers for Disease Control and Prevention. Autism Information Center. www.cdc.gov/ncbddd/dd/aic/about/#symptoms.
14. Bledsoe B, Porter R, Shade B. Paramedic Emergency Care, 3rd Ed. Upper Saddle River: Prentice-Hall, 1991.

Paul Murphy, MA, MSHA, EMT-P, has clinical and administrative experience in healthcare organizations.
Chris Colwell, MD, is the medical director for Denver Paramedics and the Denver Fire Department, as well as an attending physician in the ED at the Denver Health Medical Center, Denver, CO.
Gilbert Pineda, MD, FACEP, is medical director for the Aurora Fire Department and Rural Metro Ambulance, Aurora, CO, as well as an attending physician in the ED at The Medical Center of Aurora and Denver Health Medical Center.
Tamara Bryan, BS, EMT-P, has more than a decade of healthcare experience, including clinical and project management roles.