DEPARTMENT
Case Study—Primary Care
Asthma Management in Children With Autism Spectrum Disorders: Pearls for a Successful Clinical Encounter Sophie Lu, MN, ARNP, Jennifer Sonney, MN, ARNP, PPCNP-BC, & Gail M. Kieckhefer, PhD, ARNP, PPCNP-BC, C-AE
KEY WORDS Section Editors Jo Ann Serota, DNP, RN, CPNP Corresponding Editor Ambler Pediatrics Ambler, Pennsylvania Beverly Giordano, MS, RN, ARNP, PMHS University of Florida, Gainesville Gainesville, Florida Donna Hallas, PhD, PNP-BC, CPNP New York University College of Nursing New York, New York Sophie Lu, Alumna, School of Nursing, University of Washington, Seattle, WA. Jennifer Sonney, Director, Pediatric Graduate Specialty, School of Nursing, University of Washington, Seattle, WA. Gail M. Kieckhefer, Professor, School of Nursing, University of Washington, Seattle, WA. Conflicts of interest: None to report. Correspondence: Jennifer Sonney, MN, ARNP, PPCNP-BC, School of Nursing, University of Washington, Box 357262, Seattle, WA 98195; e-mail: [email protected]. J Pediatr Health Care. (2014) -, ---. 0891-5245/$36.00 Copyright Q 2014 by the National Association of Pediatric Nurse Practitioners. Published by Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.pedhc.2014.04.002
www.jpedhc.org
Autism spectrum disorders, communication, shared management, asthma
A 6-year-old boy with a diagnosis of autism spectrum disorder (ASD) since age 36 months presents to the pediatric primary care clinic because of 4 days of upper respiratory tract infection (URI) symptoms and wheezing that began today. The patient’s mother also reports copious rhinorrhea, a mild sore throat, and a low-grade fever. The wheezing began this morning and was accompanied by increased respiratory rate, cough, and decreased activity. The patient’s mother unsuccessfully attempted to give the patient a steamy bath to thin his secretions, but this attempt caused severe agitation and was abandoned. No other medications or herbal remedies have been administered. His mother also reports that the patient has been anxious and upset as a result of the disruption in his routine because he was kept home from school the past 2 days. The mother experienced extreme difficulty in bringing the patient for today’s appointment because he cried and resisted being placed in the car for travel. DEVELOPMENTAL HISTORY This child’s developmental history is significant for deficits in social-emotional reciprocity and an expressive and receptive language delay; he did not utter his first word until age 3 years. He met gross and fine motor milestones as expected. As an infant, he seldom -/- 2014
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showed a social smile or cooed. In his older infant and toddler years he had no interest in playing peek-a-boo, did not respond to his name, did not imitate others, and did not participate in proto-declarative pointing. He participated in an early intervention program at a local birth-to-three center when he was 31 months old, and he was formally diagnosed with ASD at age 36 months by an interdisciplinary team at a local child development center. He has since attended an integrated preschool and kindergarten with peer models in the classroom. He struggles with conversational skills, expressing and understanding emotions, and developing relationships with peers. He has accompanying language impairment and speaks primarily in phrases. He is now in first grade, has an individualized education plan, and is integrated into a regular classroom. His academic performance is average, and he responds best to simple, concrete instructions. Furthermore, he attends speech-language therapy and after-school social skills classes twice a week. He has no accompanying intellectual impairment, medical or genetic conditions, or behavioral disorders. MEDICAL HISTORY The patient’s medical history is significant for a diagnosis of intermittent asthma at age 5 years, for which he has been prescribed an albuterol nebulizer to use as needed. This patient has a history of being very resistant to receiving nebulizer treatments because of
heightened sensitivities to auditory and tactile stimulation. His last wheezing episode was approximately 1 year ago and did require several days of nebulizer treatments. His history is positive for occasional atopic dermatitis flares, which are well controlled with daily emollients and an as-needed low-potency topical corticosteroid cream. He also has seasonal allergic rhinitis that is well controlled with as-needed oral antihistamine chewable tablets. PHYSICAL EXAMINATION Upon entering the room, the patient is holding a small toy fire engine and appears alert and in no acute distress. His height and weight were both in the 6th percentile. His vital signs, which were difficult to obtain, were as follows: temperature, 37.9 C; heart rate, 126 beats per minute; respiratory rate, 30 breaths per minute; blood pressure, 96/62 mm Hg (approximately 50th percentile); and oxygen saturation, 96% on room air. Physical examination was complicated by resistance from the patient, but revealed pink and moist nasal mucosa with clear rhinorrhea. He has scattered expiratory bibasilar wheezes that do not clear with cough and prolonged exhalation but good aeration to all lobes upon auscultation. No retractions, dyspnea, or accessory muscle use are noted with an anterior-posterior to lateral diameter of 1:2 (normal). The remainder of his physical examination is noncontributory.
CASE STUDY QUESTIONS 1. 2. 3. 4.
What are the core symptoms and associated characteristics of ASD? What is the association between autism and asthma? What strategies can be used when communicating with and providing care for children who have ASD? What are some specific asthma management techniques and long-term management considerations for children with the dual diagnosis of asthma and ASD?
CASE STUDY ANSWERS Core Symptoms and Associated Characteristics of ASD 1. What are the core symptoms and associated characteristics of ASD? ASD is a neurodevelopmental disorder that is distinguished by impaired social interaction and communication, repetitive or stereotyped behaviors, and clinically significant impairment in functioning (American Psychiatric Association [APA], 2013). Sensory integration problems are common among children with ASD (APA, 2013; Scarpinato et al., 2010). As is denoted by the name, ASD comprises a wide gamut of characteristics with severity levels that can vary with context as well as time. Furthermore, ASD may also be associated with intellectual impairment, language impairment, other medical or genetic conditions, and/
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or other neurodevelopmental, mental, or behavioral disorders. Some individuals with ASD are nonverbal, whereas others utilize an extensive vocabulary to provide impromptu didactic orations to anyone willing to listen. However, despite this variance between individuals with ASD, there are commonalities in the challenges that these children face in social interactions and in communication with regard to health care. Children with ASD have trouble reading and understanding nonverbal cues such as facial expressions, gestures, and body language (Lee, Walter, & Cleary, 2012; Scarpinato et al., 2010; Venkat, Jauch, Russell, Crist, & Farrell, 2012). It is also common for these children to have speech and language delays that limit their ability to communicate with words that contribute to
Journal of Pediatric Health Care
reduced health literacy. Furthermore, autistic children frequently find it difficult to be flexible when it comes to changes in routine and often are averse to new or unexpected situations (Beard-Pfeuffer, 2008; Lee et al., 2012; Scarpinato et al., 2010). The environment of the primary care office can be a daunting and overstimulating place for a child with ASD (Venkat et al., 2012), and the situation can further be exacerbated when invasive or unpleasant treatments are needed. Therefore health care providers must take into account special considerations when caring for children with ASD in the clinic and utilize a patient and family-centered approach for chronic disease management. Association Between Autism and Asthma 2. What is the association between autism and asthma? Asthma is the exemplar in this article for a chronic condition in a child with ASD. Although some data exist regarding the incidence of asthma and ASD, the focus of this article shall be on communication with and chronic condition management in children with ASDs. Data from 2008 through 2010 indicate that asthma affects about 9.5% of children in the United States (Akinbami et al., 2012). Children with ASDs have a modestly higher rate of asthma in comparison with children who develop typically. The National Health Interview Survey (Schieve et al., 2012) revealed that 21.6% of children with ASD were reported to have asthma, and of those children, 49.5% had experienced an asthma attack in the past 12 months. In comparison, only 38.7% of the referent group of children without developmental disabilities were reported to have had an attack within the past 12 months (adjusted odds ratio = 1.7; 95% confidence interval = 1.0-3.0). The link between asthma and autism remains unexplained, although we presume that the higher rates of asthma exacerbation may be related to poor adherence to asthma treatment plans, leading to inadequate disease control. The patient in this case has a history of intermittent asthma that is triggered by URIs and seasonal allergic rhinitis, which coincides with his current presentation of wheezing with URI symptoms. The main challenge in the management of his asthma is his sensitivity to tactile sensations, which has made it difficult for him to take his medication via a nebulizer. Another crucial element for his care is to discuss the triggers for his asthma and to develop a feasible plan to prevent exacerbations. Communication and Care Strategies 3. What strategies can be used when communicating with and providing care for children who have ASD? The first step in communicating with children with ASD is to assess their developmental level (Scarpinato et al., 2010). Given the wide spectrum of ASD presentawww.jpedhc.org
tions, it is not possible to use a singular approach for a particular age group when working with autistic children. Furthermore, gauging developmental capabilities is especially important because of the higher Given the wide prevalence of intellecspectrum of ASD tual disability, neuropresentations, it is logic abnormality, and language delay within not possible to use this population coma singular approach pared with children for a particular age who develop in a typical manner. The higher group when prevalence of intellecworking with tual disability, neuroautistic children. logic abnormality, and language delay can result in greater likelihood of a discrepancy between the physical age and the developmental and cognitive level of the child with ASD (Schieve et al., 2012; Venkat et al., 2012). The developmental level of a child with ASD can be determined by taking a thorough developmental history, reviewing evaluations from school and neurodevelopmental specialists, and observing the child in the examination room. Perhaps the most critical consideration for approaching the care of children with ASDs is to consult the parent or caregiver to learn the most effective methods for communication, the child’s interests, what situations or actions upset the child, and whether the child has sensitivities to specific sounds, tastes, or tactile sensations (Beard-Pfeuffer, 2008; Blake, 2010; Johnson, Lashley, Stonek, & Bonjour, 2012; Lee et al., 2012; Scarpinato et al., 2010; Venkat et al., 2012). Many parents of children with special health care needs develop and carry a document with lists of medical diagnoses, medications, and known allergies. These lists are especially useful for first-time encounters with unfamiliar providers or settings, and their use by parents of children with chronic conditions should be encouraged. For the child with ASD, this document could be modified to include a section on ‘‘What approaches work best with my child.’’ Pertinent information might include any light or sound sensitivities, if the patient prefers sitting on the examination table versus with the parent, any component of the physical examination that is especially distressing and should be performed last, or if the child has a particular interest in videos, songs, or objects that could be used as a means of distraction. The information provided will give the health care provider a brief overview on situations or actions to avoid if possible and tools that might work well to encourage cooperation or provide comfort. The environment of the office can then be adjusted accordingly. For example, for a patient with sound sensitivities, time spent in the waiting room can be limited by placing the patient in a quieter examination room, -/- 2014
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allowing him or her to wait in the car, or scheduling appointments first thing in the morning or immediately after lunch, and blood pressure can be obtained manually rather than with use of an automated machine (Venkat et al., 2012). Having patience and allowing extra time for the visit are key principles for providing care to children with autism. Children with autism often have difficulty with changes and transitions and may need flexibility in arrival times, as well as extra time to acclimate once in the examination room. Explaining step by step what to expect during the office visit and providing the opportunity to explore any equipment that will be used can help mitigate the anxiety caused by activities that are outside of the patient’s normal routine (Beard-Pfeuffer, 2008; Johnson et al., 2012; Scarpinato et al., 2010). Some children might be interested in helping with the examination, such as by holding the stethoscope in position during the lung examination or deciding on the sequence of the examination or ‘‘what part to check next.’’ Because many children with autism have difficulty with communication and reading social cues, a care provider should use familiar words, simple sentences, and straightforward language when speaking to the child. Additionally, the use of visuals such as pictures, checklists, color codes, and hand gestures have been noted to be effective in communicating with children with ASD (Lee et al., 2012; Scarpinato et al., 2010). The care provider should ask the parent if certain types of visuals are used at home or at school to gauge which visual tools are likely to be familiar and effective. Consultation with a child life specialist may be beneficial. As is the case with children who develop in a typical manner, the use of rewards as motivation for cooperative behavior is a helpful strategy when providing care for autistic children. Children with Children with ASD ASD often have reoften have stricted interests that restricted interests coincide with the tendency toward aversion that coincide with to change and inflexithe tendency bility; however, these toward aversion to interests can also be used to gain the child’s change and compliance. The item inflexibility; used as a reward may however, these differ for a child with ASD compared with interests can also children who develop be used to gain the in a typical manner. A child’s compliance. sticker may be an appealing reward for a child without autism, whereas a chance to click the print button on the computer and watch the printer 4
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spokes turn while the page prints may be the preferred reward for a child with ASD who has an interest in computers and machinery. Lastly, when possible and appropriate, provide the opportunity for children with ASD to make decisions and participate in their care. This approach helps create the sense of control so needed by these children and may contribute to greater cooperation (Lee et al., 2012; Scarpinato et al., 2010). This subject will be explored in more detail in the next section. Specific Asthma Management Techniques and Long-term Management Considerations 4. What are some specific asthma management techniques and long-term management considerations for children with the dual diagnosis of asthma and ASD? A useful and fitting model to apply for the management of asthma and other chronic conditions for children with ASDs is the shared management model (Kieckhefer, Trahms, Churchill, & Simpson, 2009; Sonney, 2012). Facilitating parent-child shared management is now listed as a primary care pediatric nurse practitioner competency in the 2013 PopulationFocused NP Competencies (National Organization of Nurse Practitioner Faculties, 2013). The ultimate goal of the shared management model is to help guide and support the child to obtain the highest level of independence possible in managing his or her chronic condition given his or her unique capabilities. The concept of parent-child shared management encompasses three themes arising from the literature. The first theme is that children will take on more responsibility for managing their chronic condition with increasing age. For children with ASD, the acquisition of greater responsibility would correlate with increasing developmental capabilities rather than physical age. The second theme involves providing multiple transition points for children to gradually take on more responsibility in the management of the chronic condition, assessing evidence of success at each transition. This approach is in contrast to an abrupt delegation of responsibilities to the child as he or she reaches adolescence or young adulthood. The third theme highlights the importance of the parent’s role as a leader in guiding and monitoring his or her child through the steps of shared management (Kieckhefer et al., 2009). The application of the shared management model to children with ASD begins with an important foundational framework rooted in the principle that even children with neurodevelopmental disorders such as autism ought to be encouraged, supported, and empowered to gain greater self-efficacy, self-confidence, and independence within the scope of their cognitive and physical capabilities. The shared management model allows the child with ASD to be assessed and understood at his or her current level of ability. Additionally, it sets forth a pathway and helps identify goals for the child Journal of Pediatric Health Care
that can be achieved with the parent’s leadership and guidance and the support of the health care provider. The shared management model proposes a logical, developmental progression of health management with a focus on maximizing the child’s potential (Kieckhefer et al., 2009; Sonney, 2012). This progression should be concurrent with progression in other self-care skills (e.g., eating and dressing) and maximize individual cognitive and emotional capabilities. Although the child with ASD may not reach independence, the provider can help parents facilitate an increase in the child’s responsibility for his or her own care. Initially, parents are the sole providers of the child’s care. However, many children progress to the point that they help hold or prepare any equipment and cooperate with treatments through the development of rituals (Sonney, 2012). Children with ASD often adhere strictly to rituals and might thrive with a structured approach to their chronic illness care. With further progress a parent might be able to take on a supervisory role while the child provides some self-care. This self-care might include concrete monitoring of the chronic problem, such as knowing to pack a rescue inhaler before activities or trips, reporting symptoms such as cough or chest pain, and counting the number of puffs of a medication. Later the child may be able to premedicate before physical education class or even start speaking with health care providers about symptoms that he or she Children with ASD might be experiencing (Sonney, 2012). The often adhere strictly next step of progresto rituals and might sion involves having thrive with a the child be the manager of care and the structured parent being the superapproach to their visor. This stage might chronic illness care. include having the child taking daily medications while the parent observes. In the final stage of the shared management model the child is the supervisor and is primarily responsible for all care, with the parent serving as a consultant. In circumstances of illness, it is common for children to show regression in their ability to cooperate and assist in management (Kieckhefer et al., 2009; Sonney, 2012). Parents and health care providers need to be patient and anticipate this regression. However, it is also necessary to work with the child to restore his or her skills. Some defined role for the child always exists in the shared management model, even when the child is ill or has cognitive and psychomotor limitations. The child is expected to cooperate with the treatment routine and aid in preparing the medication, even if that aid is confined to shaking the medication prior to use. These limited actions can bring a sense of ongoing success to the child and www.jpedhc.org
parent, thereby encouraging future steps in effective asthma control. The child in the case presentation has a history of expressive and receptive language delay, currently receives speech-language therapy services, and also has tactile sensitivities. These factors present several challenges for the management of asthma, both in the child’s ability to understand the necessity of pharmacologic therapy and in the administration of inhaled medication, but the child’s average cognitive capabilities support attempts to involve him in his own care. Using the shared management developmental perspective model (Kieckhefer & Trahms, 2000), one strategy in advancing shared management is to ritualize treatments, which would be particularly helpful for children with ASD, who tend to prefer routines. A visual schedule is a useful tool for establishing a routine to ritualize treatment. The visual schedule should depict information such as when it is necessary to use an inhaler and the steps of taking an inhaler. Additionally, the visual schedule can be used to encourage the child’s participation. For instance, the visual schedule could show a picture of a person, ideally a peer model or even the child himself or herself, holding the inhaler with their finger over the top. The next step in the visual schedule could show the person pressing down and actuating the inhaler. The child could then be prompted to take on the role of holding and actuating the inhaler by use of verbal coaching reinforced by examining the visual schedule. Another way the child can participate is to learn the parts of the body involved with the condition and begin to identify what symptoms and triggers require attention. To help the child with ASD learn this information, a storybook that includes pictures and relatable narration could be used. These visual strategies would provide an alternative means to communicate with the child and help him or her understand the disease and medications, despite speech and language issues. The child in this case presentation had a particular interest in firefighters and fire trucks. The health care provider allowed the child to try both a nebulizer and a metered dose inhaler (MDI) with a spacer and mask in the office to determine which delivery method he preferred; the child clearly was less agitated with the MDI and spacer with face mask, which is logical considering his sensitivity to auditory stimulation. The health care provider enlisted the help of the local fire department, who donated a firefighter hat and badge to the family. A firefighter also posed in a photograph that depicted the firefighter holding an MDI with a spacer and mask to his face. These props were used to encourage the patient to cooperate with taking his inhaler medication. When it was time to use the albuterol, the patient would first examine his visual schedule depicting the circumstances of asthma exacerbation and the steps of taking his medication. He would then get to wear -/- 2014
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the firefighter hat and badge as he went through the steps of using his MDI with spacer and mask. Furthermore, he understood that his role was to actuate his inhaler when he was shown the picture of the firefighter holding the MDI. These creative strategies resulted in successful medication administration and wellcontrolled asthma symptoms for this child. CONCLUSION The management of chronic conditions in children with ASD requires a customized approach tailored to each individual child. The role of the health care provider is to understand the characteristics of ASD, the challenges associated with this neurodevelopmental condition, and its unique manifestations in each child. To provide effective chronic disease management for the child with ASD, the provider should creatively utilize the strategies discussed and support parents in being leaders and guides for their child to achieve their optimal shared management capability. REFERENCES Akinbami, L. J., Moorman, J. E., Bailey, C., Zahran, H. S., King, M., Johnson, C. A., & Liu, X. (2012). Trends in asthma prevalence, health care use, and mortality in the United States, 2001-2010. NCHS Data Brief, (94), 1-8. American Psychiatric Association. (2013). Diagnostic and statistical manual of mental disorders (5th ed.). Arlington, VA: Author. Beard-Pfeuffer, M. (2008). Understanding the world of children with autism. RN, 71(2), 40-45, quiz 46. Blake, K. E. (2010). Spectrum disorders: A new generation of complex patients. The Pennsylvania Nurse, 65(4), 9-11, 15.
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Johnson, N. L., Lashley, J., Stonek, A. V., & Bonjour, A. (2012). Children with developmental disabilities at a pediatric hospital: Staff education to prevent and manage challenging behaviors. Journal of Pediatric Nursing, 27(6), 742-749. Kieckhefer, G. M., Trahms, C. M., Churchill, S. S., & Simpson, J. N. (2009). Measuring parent-child shared management of chronic illness. Pediatric Nursing, 35(2), 101-108, 127. Kieckhefer, G. M., & Trahms, C. M. (2000). Supporting development of children with chronic conditions: From compliance toward shared management. Pediatric Nursing, 26(4), 354-363. Lee, C., Walter, G., & Cleary, M. (2012). Communicating with children with autism spectrum disorder and their families: A practical introduction. Journal of Psychosocial Nursing and Mental Health Services, 50(8), 40-44. National Organization of Nurse Practitioner Faculties. (2013). Population-focused nurse practitioner competencies. Retrieved from http://www.nonpf.org/associations/10789/files/PopulationFocus NPComps2013.pdf Scarpinato, N., Bradley, J., Kurbjun, K., Bateman, X., Holtzer, B., & Ely, B. (2010). Caring for the child with an autism spectrum disorder in the acute care setting. Journal for Specialists in Pediatric Nursing, 15(3), 244-254. Schieve, L. A., Gonzalez, V., Boulet, S. L., Visser, S. N., Rice, C. E., Van Naarden Braun, K., & Boyle, C. A. (2012). Concurrent medical conditions and health care use and needs among children with learning and behavioral developmental disabilities, National Health Interview Survey, 2006-2010. Research in Developmental Disabilities, 33(2), 467-476. Sonney, J. (2012). Parent-child shared management: The path to independence. Paper presented at the Duncan Seminar: Adolescent Health Transition for Youth with Special Health Care Needs. Seattle, WA: Seattle ChildrenÕs Hospital. Venkat, A., Jauch, E., Russell, W. S., Crist, C. R., & Farrell, R. (2012). Care of the patient with an autism spectrum disorder by the general physician. Postgraduate Medical Journal, 88(1042), 472-481.
Journal of Pediatric Health Care
Case Study—Primary Care
Asthma Management in Children With Autism Spectrum Disorders: Pearls for a Successful Clinical Encounter Sophie Lu, MN, ARNP, Jennifer Sonney, MN, ARNP, PPCNP-BC, & Gail M. Kieckhefer, PhD, ARNP, PPCNP-BC, C-AE
KEY WORDS Section Editors Jo Ann Serota, DNP, RN, CPNP Corresponding Editor Ambler Pediatrics Ambler, Pennsylvania Beverly Giordano, MS, RN, ARNP, PMHS University of Florida, Gainesville Gainesville, Florida Donna Hallas, PhD, PNP-BC, CPNP New York University College of Nursing New York, New York Sophie Lu, Alumna, School of Nursing, University of Washington, Seattle, WA. Jennifer Sonney, Director, Pediatric Graduate Specialty, School of Nursing, University of Washington, Seattle, WA. Gail M. Kieckhefer, Professor, School of Nursing, University of Washington, Seattle, WA. Conflicts of interest: None to report. Correspondence: Jennifer Sonney, MN, ARNP, PPCNP-BC, School of Nursing, University of Washington, Box 357262, Seattle, WA 98195; e-mail: [email protected]. J Pediatr Health Care. (2014) -, ---. 0891-5245/$36.00 Copyright Q 2014 by the National Association of Pediatric Nurse Practitioners. Published by Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.pedhc.2014.04.002
www.jpedhc.org
Autism spectrum disorders, communication, shared management, asthma
A 6-year-old boy with a diagnosis of autism spectrum disorder (ASD) since age 36 months presents to the pediatric primary care clinic because of 4 days of upper respiratory tract infection (URI) symptoms and wheezing that began today. The patient’s mother also reports copious rhinorrhea, a mild sore throat, and a low-grade fever. The wheezing began this morning and was accompanied by increased respiratory rate, cough, and decreased activity. The patient’s mother unsuccessfully attempted to give the patient a steamy bath to thin his secretions, but this attempt caused severe agitation and was abandoned. No other medications or herbal remedies have been administered. His mother also reports that the patient has been anxious and upset as a result of the disruption in his routine because he was kept home from school the past 2 days. The mother experienced extreme difficulty in bringing the patient for today’s appointment because he cried and resisted being placed in the car for travel. DEVELOPMENTAL HISTORY This child’s developmental history is significant for deficits in social-emotional reciprocity and an expressive and receptive language delay; he did not utter his first word until age 3 years. He met gross and fine motor milestones as expected. As an infant, he seldom -/- 2014
1
showed a social smile or cooed. In his older infant and toddler years he had no interest in playing peek-a-boo, did not respond to his name, did not imitate others, and did not participate in proto-declarative pointing. He participated in an early intervention program at a local birth-to-three center when he was 31 months old, and he was formally diagnosed with ASD at age 36 months by an interdisciplinary team at a local child development center. He has since attended an integrated preschool and kindergarten with peer models in the classroom. He struggles with conversational skills, expressing and understanding emotions, and developing relationships with peers. He has accompanying language impairment and speaks primarily in phrases. He is now in first grade, has an individualized education plan, and is integrated into a regular classroom. His academic performance is average, and he responds best to simple, concrete instructions. Furthermore, he attends speech-language therapy and after-school social skills classes twice a week. He has no accompanying intellectual impairment, medical or genetic conditions, or behavioral disorders. MEDICAL HISTORY The patient’s medical history is significant for a diagnosis of intermittent asthma at age 5 years, for which he has been prescribed an albuterol nebulizer to use as needed. This patient has a history of being very resistant to receiving nebulizer treatments because of
heightened sensitivities to auditory and tactile stimulation. His last wheezing episode was approximately 1 year ago and did require several days of nebulizer treatments. His history is positive for occasional atopic dermatitis flares, which are well controlled with daily emollients and an as-needed low-potency topical corticosteroid cream. He also has seasonal allergic rhinitis that is well controlled with as-needed oral antihistamine chewable tablets. PHYSICAL EXAMINATION Upon entering the room, the patient is holding a small toy fire engine and appears alert and in no acute distress. His height and weight were both in the 6th percentile. His vital signs, which were difficult to obtain, were as follows: temperature, 37.9 C; heart rate, 126 beats per minute; respiratory rate, 30 breaths per minute; blood pressure, 96/62 mm Hg (approximately 50th percentile); and oxygen saturation, 96% on room air. Physical examination was complicated by resistance from the patient, but revealed pink and moist nasal mucosa with clear rhinorrhea. He has scattered expiratory bibasilar wheezes that do not clear with cough and prolonged exhalation but good aeration to all lobes upon auscultation. No retractions, dyspnea, or accessory muscle use are noted with an anterior-posterior to lateral diameter of 1:2 (normal). The remainder of his physical examination is noncontributory.
CASE STUDY QUESTIONS 1. 2. 3. 4.
What are the core symptoms and associated characteristics of ASD? What is the association between autism and asthma? What strategies can be used when communicating with and providing care for children who have ASD? What are some specific asthma management techniques and long-term management considerations for children with the dual diagnosis of asthma and ASD?
CASE STUDY ANSWERS Core Symptoms and Associated Characteristics of ASD 1. What are the core symptoms and associated characteristics of ASD? ASD is a neurodevelopmental disorder that is distinguished by impaired social interaction and communication, repetitive or stereotyped behaviors, and clinically significant impairment in functioning (American Psychiatric Association [APA], 2013). Sensory integration problems are common among children with ASD (APA, 2013; Scarpinato et al., 2010). As is denoted by the name, ASD comprises a wide gamut of characteristics with severity levels that can vary with context as well as time. Furthermore, ASD may also be associated with intellectual impairment, language impairment, other medical or genetic conditions, and/
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or other neurodevelopmental, mental, or behavioral disorders. Some individuals with ASD are nonverbal, whereas others utilize an extensive vocabulary to provide impromptu didactic orations to anyone willing to listen. However, despite this variance between individuals with ASD, there are commonalities in the challenges that these children face in social interactions and in communication with regard to health care. Children with ASD have trouble reading and understanding nonverbal cues such as facial expressions, gestures, and body language (Lee, Walter, & Cleary, 2012; Scarpinato et al., 2010; Venkat, Jauch, Russell, Crist, & Farrell, 2012). It is also common for these children to have speech and language delays that limit their ability to communicate with words that contribute to
Journal of Pediatric Health Care
reduced health literacy. Furthermore, autistic children frequently find it difficult to be flexible when it comes to changes in routine and often are averse to new or unexpected situations (Beard-Pfeuffer, 2008; Lee et al., 2012; Scarpinato et al., 2010). The environment of the primary care office can be a daunting and overstimulating place for a child with ASD (Venkat et al., 2012), and the situation can further be exacerbated when invasive or unpleasant treatments are needed. Therefore health care providers must take into account special considerations when caring for children with ASD in the clinic and utilize a patient and family-centered approach for chronic disease management. Association Between Autism and Asthma 2. What is the association between autism and asthma? Asthma is the exemplar in this article for a chronic condition in a child with ASD. Although some data exist regarding the incidence of asthma and ASD, the focus of this article shall be on communication with and chronic condition management in children with ASDs. Data from 2008 through 2010 indicate that asthma affects about 9.5% of children in the United States (Akinbami et al., 2012). Children with ASDs have a modestly higher rate of asthma in comparison with children who develop typically. The National Health Interview Survey (Schieve et al., 2012) revealed that 21.6% of children with ASD were reported to have asthma, and of those children, 49.5% had experienced an asthma attack in the past 12 months. In comparison, only 38.7% of the referent group of children without developmental disabilities were reported to have had an attack within the past 12 months (adjusted odds ratio = 1.7; 95% confidence interval = 1.0-3.0). The link between asthma and autism remains unexplained, although we presume that the higher rates of asthma exacerbation may be related to poor adherence to asthma treatment plans, leading to inadequate disease control. The patient in this case has a history of intermittent asthma that is triggered by URIs and seasonal allergic rhinitis, which coincides with his current presentation of wheezing with URI symptoms. The main challenge in the management of his asthma is his sensitivity to tactile sensations, which has made it difficult for him to take his medication via a nebulizer. Another crucial element for his care is to discuss the triggers for his asthma and to develop a feasible plan to prevent exacerbations. Communication and Care Strategies 3. What strategies can be used when communicating with and providing care for children who have ASD? The first step in communicating with children with ASD is to assess their developmental level (Scarpinato et al., 2010). Given the wide spectrum of ASD presentawww.jpedhc.org
tions, it is not possible to use a singular approach for a particular age group when working with autistic children. Furthermore, gauging developmental capabilities is especially important because of the higher Given the wide prevalence of intellecspectrum of ASD tual disability, neuropresentations, it is logic abnormality, and language delay within not possible to use this population coma singular approach pared with children for a particular age who develop in a typical manner. The higher group when prevalence of intellecworking with tual disability, neuroautistic children. logic abnormality, and language delay can result in greater likelihood of a discrepancy between the physical age and the developmental and cognitive level of the child with ASD (Schieve et al., 2012; Venkat et al., 2012). The developmental level of a child with ASD can be determined by taking a thorough developmental history, reviewing evaluations from school and neurodevelopmental specialists, and observing the child in the examination room. Perhaps the most critical consideration for approaching the care of children with ASDs is to consult the parent or caregiver to learn the most effective methods for communication, the child’s interests, what situations or actions upset the child, and whether the child has sensitivities to specific sounds, tastes, or tactile sensations (Beard-Pfeuffer, 2008; Blake, 2010; Johnson, Lashley, Stonek, & Bonjour, 2012; Lee et al., 2012; Scarpinato et al., 2010; Venkat et al., 2012). Many parents of children with special health care needs develop and carry a document with lists of medical diagnoses, medications, and known allergies. These lists are especially useful for first-time encounters with unfamiliar providers or settings, and their use by parents of children with chronic conditions should be encouraged. For the child with ASD, this document could be modified to include a section on ‘‘What approaches work best with my child.’’ Pertinent information might include any light or sound sensitivities, if the patient prefers sitting on the examination table versus with the parent, any component of the physical examination that is especially distressing and should be performed last, or if the child has a particular interest in videos, songs, or objects that could be used as a means of distraction. The information provided will give the health care provider a brief overview on situations or actions to avoid if possible and tools that might work well to encourage cooperation or provide comfort. The environment of the office can then be adjusted accordingly. For example, for a patient with sound sensitivities, time spent in the waiting room can be limited by placing the patient in a quieter examination room, -/- 2014
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allowing him or her to wait in the car, or scheduling appointments first thing in the morning or immediately after lunch, and blood pressure can be obtained manually rather than with use of an automated machine (Venkat et al., 2012). Having patience and allowing extra time for the visit are key principles for providing care to children with autism. Children with autism often have difficulty with changes and transitions and may need flexibility in arrival times, as well as extra time to acclimate once in the examination room. Explaining step by step what to expect during the office visit and providing the opportunity to explore any equipment that will be used can help mitigate the anxiety caused by activities that are outside of the patient’s normal routine (Beard-Pfeuffer, 2008; Johnson et al., 2012; Scarpinato et al., 2010). Some children might be interested in helping with the examination, such as by holding the stethoscope in position during the lung examination or deciding on the sequence of the examination or ‘‘what part to check next.’’ Because many children with autism have difficulty with communication and reading social cues, a care provider should use familiar words, simple sentences, and straightforward language when speaking to the child. Additionally, the use of visuals such as pictures, checklists, color codes, and hand gestures have been noted to be effective in communicating with children with ASD (Lee et al., 2012; Scarpinato et al., 2010). The care provider should ask the parent if certain types of visuals are used at home or at school to gauge which visual tools are likely to be familiar and effective. Consultation with a child life specialist may be beneficial. As is the case with children who develop in a typical manner, the use of rewards as motivation for cooperative behavior is a helpful strategy when providing care for autistic children. Children with Children with ASD ASD often have reoften have stricted interests that restricted interests coincide with the tendency toward aversion that coincide with to change and inflexithe tendency bility; however, these toward aversion to interests can also be used to gain the child’s change and compliance. The item inflexibility; used as a reward may however, these differ for a child with ASD compared with interests can also children who develop be used to gain the in a typical manner. A child’s compliance. sticker may be an appealing reward for a child without autism, whereas a chance to click the print button on the computer and watch the printer 4
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spokes turn while the page prints may be the preferred reward for a child with ASD who has an interest in computers and machinery. Lastly, when possible and appropriate, provide the opportunity for children with ASD to make decisions and participate in their care. This approach helps create the sense of control so needed by these children and may contribute to greater cooperation (Lee et al., 2012; Scarpinato et al., 2010). This subject will be explored in more detail in the next section. Specific Asthma Management Techniques and Long-term Management Considerations 4. What are some specific asthma management techniques and long-term management considerations for children with the dual diagnosis of asthma and ASD? A useful and fitting model to apply for the management of asthma and other chronic conditions for children with ASDs is the shared management model (Kieckhefer, Trahms, Churchill, & Simpson, 2009; Sonney, 2012). Facilitating parent-child shared management is now listed as a primary care pediatric nurse practitioner competency in the 2013 PopulationFocused NP Competencies (National Organization of Nurse Practitioner Faculties, 2013). The ultimate goal of the shared management model is to help guide and support the child to obtain the highest level of independence possible in managing his or her chronic condition given his or her unique capabilities. The concept of parent-child shared management encompasses three themes arising from the literature. The first theme is that children will take on more responsibility for managing their chronic condition with increasing age. For children with ASD, the acquisition of greater responsibility would correlate with increasing developmental capabilities rather than physical age. The second theme involves providing multiple transition points for children to gradually take on more responsibility in the management of the chronic condition, assessing evidence of success at each transition. This approach is in contrast to an abrupt delegation of responsibilities to the child as he or she reaches adolescence or young adulthood. The third theme highlights the importance of the parent’s role as a leader in guiding and monitoring his or her child through the steps of shared management (Kieckhefer et al., 2009). The application of the shared management model to children with ASD begins with an important foundational framework rooted in the principle that even children with neurodevelopmental disorders such as autism ought to be encouraged, supported, and empowered to gain greater self-efficacy, self-confidence, and independence within the scope of their cognitive and physical capabilities. The shared management model allows the child with ASD to be assessed and understood at his or her current level of ability. Additionally, it sets forth a pathway and helps identify goals for the child Journal of Pediatric Health Care
that can be achieved with the parent’s leadership and guidance and the support of the health care provider. The shared management model proposes a logical, developmental progression of health management with a focus on maximizing the child’s potential (Kieckhefer et al., 2009; Sonney, 2012). This progression should be concurrent with progression in other self-care skills (e.g., eating and dressing) and maximize individual cognitive and emotional capabilities. Although the child with ASD may not reach independence, the provider can help parents facilitate an increase in the child’s responsibility for his or her own care. Initially, parents are the sole providers of the child’s care. However, many children progress to the point that they help hold or prepare any equipment and cooperate with treatments through the development of rituals (Sonney, 2012). Children with ASD often adhere strictly to rituals and might thrive with a structured approach to their chronic illness care. With further progress a parent might be able to take on a supervisory role while the child provides some self-care. This self-care might include concrete monitoring of the chronic problem, such as knowing to pack a rescue inhaler before activities or trips, reporting symptoms such as cough or chest pain, and counting the number of puffs of a medication. Later the child may be able to premedicate before physical education class or even start speaking with health care providers about symptoms that he or she Children with ASD might be experiencing (Sonney, 2012). The often adhere strictly next step of progresto rituals and might sion involves having thrive with a the child be the manager of care and the structured parent being the superapproach to their visor. This stage might chronic illness care. include having the child taking daily medications while the parent observes. In the final stage of the shared management model the child is the supervisor and is primarily responsible for all care, with the parent serving as a consultant. In circumstances of illness, it is common for children to show regression in their ability to cooperate and assist in management (Kieckhefer et al., 2009; Sonney, 2012). Parents and health care providers need to be patient and anticipate this regression. However, it is also necessary to work with the child to restore his or her skills. Some defined role for the child always exists in the shared management model, even when the child is ill or has cognitive and psychomotor limitations. The child is expected to cooperate with the treatment routine and aid in preparing the medication, even if that aid is confined to shaking the medication prior to use. These limited actions can bring a sense of ongoing success to the child and www.jpedhc.org
parent, thereby encouraging future steps in effective asthma control. The child in the case presentation has a history of expressive and receptive language delay, currently receives speech-language therapy services, and also has tactile sensitivities. These factors present several challenges for the management of asthma, both in the child’s ability to understand the necessity of pharmacologic therapy and in the administration of inhaled medication, but the child’s average cognitive capabilities support attempts to involve him in his own care. Using the shared management developmental perspective model (Kieckhefer & Trahms, 2000), one strategy in advancing shared management is to ritualize treatments, which would be particularly helpful for children with ASD, who tend to prefer routines. A visual schedule is a useful tool for establishing a routine to ritualize treatment. The visual schedule should depict information such as when it is necessary to use an inhaler and the steps of taking an inhaler. Additionally, the visual schedule can be used to encourage the child’s participation. For instance, the visual schedule could show a picture of a person, ideally a peer model or even the child himself or herself, holding the inhaler with their finger over the top. The next step in the visual schedule could show the person pressing down and actuating the inhaler. The child could then be prompted to take on the role of holding and actuating the inhaler by use of verbal coaching reinforced by examining the visual schedule. Another way the child can participate is to learn the parts of the body involved with the condition and begin to identify what symptoms and triggers require attention. To help the child with ASD learn this information, a storybook that includes pictures and relatable narration could be used. These visual strategies would provide an alternative means to communicate with the child and help him or her understand the disease and medications, despite speech and language issues. The child in this case presentation had a particular interest in firefighters and fire trucks. The health care provider allowed the child to try both a nebulizer and a metered dose inhaler (MDI) with a spacer and mask in the office to determine which delivery method he preferred; the child clearly was less agitated with the MDI and spacer with face mask, which is logical considering his sensitivity to auditory stimulation. The health care provider enlisted the help of the local fire department, who donated a firefighter hat and badge to the family. A firefighter also posed in a photograph that depicted the firefighter holding an MDI with a spacer and mask to his face. These props were used to encourage the patient to cooperate with taking his inhaler medication. When it was time to use the albuterol, the patient would first examine his visual schedule depicting the circumstances of asthma exacerbation and the steps of taking his medication. He would then get to wear -/- 2014
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the firefighter hat and badge as he went through the steps of using his MDI with spacer and mask. Furthermore, he understood that his role was to actuate his inhaler when he was shown the picture of the firefighter holding the MDI. These creative strategies resulted in successful medication administration and wellcontrolled asthma symptoms for this child. CONCLUSION The management of chronic conditions in children with ASD requires a customized approach tailored to each individual child. The role of the health care provider is to understand the characteristics of ASD, the challenges associated with this neurodevelopmental condition, and its unique manifestations in each child. To provide effective chronic disease management for the child with ASD, the provider should creatively utilize the strategies discussed and support parents in being leaders and guides for their child to achieve their optimal shared management capability. REFERENCES Akinbami, L. J., Moorman, J. E., Bailey, C., Zahran, H. S., King, M., Johnson, C. A., & Liu, X. (2012). Trends in asthma prevalence, health care use, and mortality in the United States, 2001-2010. NCHS Data Brief, (94), 1-8. American Psychiatric Association. (2013). Diagnostic and statistical manual of mental disorders (5th ed.). Arlington, VA: Author. Beard-Pfeuffer, M. (2008). Understanding the world of children with autism. RN, 71(2), 40-45, quiz 46. Blake, K. E. (2010). Spectrum disorders: A new generation of complex patients. The Pennsylvania Nurse, 65(4), 9-11, 15.
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Johnson, N. L., Lashley, J., Stonek, A. V., & Bonjour, A. (2012). Children with developmental disabilities at a pediatric hospital: Staff education to prevent and manage challenging behaviors. Journal of Pediatric Nursing, 27(6), 742-749. Kieckhefer, G. M., Trahms, C. M., Churchill, S. S., & Simpson, J. N. (2009). Measuring parent-child shared management of chronic illness. Pediatric Nursing, 35(2), 101-108, 127. Kieckhefer, G. M., & Trahms, C. M. (2000). Supporting development of children with chronic conditions: From compliance toward shared management. Pediatric Nursing, 26(4), 354-363. Lee, C., Walter, G., & Cleary, M. (2012). Communicating with children with autism spectrum disorder and their families: A practical introduction. Journal of Psychosocial Nursing and Mental Health Services, 50(8), 40-44. National Organization of Nurse Practitioner Faculties. (2013). Population-focused nurse practitioner competencies. Retrieved from http://www.nonpf.org/associations/10789/files/PopulationFocus NPComps2013.pdf Scarpinato, N., Bradley, J., Kurbjun, K., Bateman, X., Holtzer, B., & Ely, B. (2010). Caring for the child with an autism spectrum disorder in the acute care setting. Journal for Specialists in Pediatric Nursing, 15(3), 244-254. Schieve, L. A., Gonzalez, V., Boulet, S. L., Visser, S. N., Rice, C. E., Van Naarden Braun, K., & Boyle, C. A. (2012). Concurrent medical conditions and health care use and needs among children with learning and behavioral developmental disabilities, National Health Interview Survey, 2006-2010. Research in Developmental Disabilities, 33(2), 467-476. Sonney, J. (2012). Parent-child shared management: The path to independence. Paper presented at the Duncan Seminar: Adolescent Health Transition for Youth with Special Health Care Needs. Seattle, WA: Seattle ChildrenÕs Hospital. Venkat, A., Jauch, E., Russell, W. S., Crist, C. R., & Farrell, R. (2012). Care of the patient with an autism spectrum disorder by the general physician. Postgraduate Medical Journal, 88(1042), 472-481.
Journal of Pediatric Health Care
