1. No category

Disruptive Classroom Behavior in an Amish School-Aged Child With Muscular
Dystrophy
Pediatrics 2004;114;1501
DOI: 10.1542/peds.2004-1721U
The online version of this article, along with updated information and services, is
located on the World Wide Web at:
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PEDIATRICS is the official journal of the American Academy of Pediatrics. A monthly
publication, it has been published continuously since 1948. PEDIATRICS is owned,
published, and trademarked by the American Academy of Pediatrics, 141 Northwest Point
Boulevard, Elk Grove Village, Illinois, 60007. Copyright © 2004 by the American Academy
of Pediatrics. All rights reserved. Print ISSN: 0031-4005. Online ISSN: 1098-4275.
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Disruptive Classroom Behavior in an Amish School-Aged Child With
Muscular Dystrophy*
CASE
Will is an Amish school-aged boy with Duchenne
muscular dystrophy (DMD). After reading about attention-deficit/hyperactivity disorder (ADHD) in
the local newspaper, his mother asked Will’s pediatrician, “Do you think my son has this (condition)?”
She noted that Will fidgets in different settings and
has difficulty focusing on his work when in school.
She also reported that his teacher in the Amish
school does not want him in her classroom because
he is disruptive and noncompliant. The teacher tried
incentives to encourage Will to complete his schoolwork but without success. Subsequently, his teacher
prevented Will from participating in recess activities;
at other times, she sent him home as punishment for
noncompliance in the classroom. During the past
week, Will did not attend school while his teacher
sent assignments home with his sister. Will’s mother
reported that he seemed to be happy, with a normal
appetite and sleep pattern. She was not aware of any
behavior problems with other children.
Will’s father, who also attended the pediatric appointment, added that he did not feel that the teacher
was patient with Will. He stated that Will has friends
but may have a difficult time keeping up with them
due to his physical weakness secondary to muscular
dystrophy. He also believed that due to motor limitations, writing was difficult for Will. The parents
did not provide consequences for Will’s insistence on
not attending school nor did they positively reward
him for school attendance. When at home, Will did
his work and kept to himself.
Will was quiet and motionless in his seat. He
stated that he liked school and playing with friends
and that the schoolwork was not too difficult.
INDEX TERMS. Duchenne muscular dystrophy, Amish culture, learning disability.
Dr Martin T. Stein
The clinical information available on this schoolaged boy with muscular dystrophy is sparse. However, there is a sufficient medical and social history
to suggest several directions for further investigation. A medical understanding of the natural history
of DMD and the effect it has on motor and cognitive
development only begins the process of inquiry.
Will’s pediatrician must use her knowledge of cultural values and educational expectations in the
* Originally published in J Dev Behav Pediatr. 2004;25:280 –284.
doi:10.1542/peds.2004-1721U
PEDIATRICS (ISSN 0031 4005). Copyright © 2004 by the American Academy of Pediatrics and Lippincott Williams & Wilkins.
Amish community in order to communicate effectively with Will, his parents, and teacher.
The commentaries highlight the importance of incorporating a cultural, psychological, and educational focus during the evaluation process. The patient, family, school, and extended community are
individual components of Will’s life; to provide a
therapeutic intervention, each component must be
addressed.
Dr Lee Pachter is Professor of Pediatrics and Anthropology at the University of Connecticut School of
Medicine, where he directs the Division of General
Pediatrics. Dr Pachter’s education in anthropology
and pediatrics is highlighted in his commentary,
where he explores the unique characteristics of the
Amish culture and their impact on educational and
family values. Lisa Schwartz, a doctoral student in
clinical psychology at Case Western Reserve University, writes about the importance of a comprehensive
psychosocial evaluation that moves beyond the initial concern with ADHD. Dr Howard Taras is Professor of Pediatrics at the University of California,
San Diego, where he is Acting Director of the Division of Community Pediatrics. Dr Taras is a medical
consultant for several school districts and Chair of
the American Academy of Pediatrics Committee on
School Health. His commentary addresses medical,
behavioral, legal, and cultural aspects of the case.
Martin T. Stein, MD
Professor of Pediatrics
University of California
Children’s Hospital San Diego
San Diego, California
Dr Lee M. Pachter
This case brings up many interesting issues with
regard to child behavior and the importance of context (or setting). There are various contexts that pertain to this particular case, including culture, the
educational system, community, chronic illness, and
child development.
The Amish are a religious and cultural group that
stems from the Anabaptist movement in Europe in
the 1500s and 1600s. Amish society is based on simple (plain) living and a literal interpretation of the
Bible.1 Most Amish communities are agricultural and
based around family farms. The pace of life is guided
by the belief that modernity, in the form of high
technology, is unnecessary for leading a good and
pious life. Most traditional (Old Order) Amish do not
own cars or have electricity on their farms and
homes (generators are common, though). Motorpowered farm equipment is often used— but drawn
behind a horse. “It’s not the technology that we are
against,” said one farmer, “but the pace of life that
PEDIATRICS Vol. 114 No. 5 November 2004
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1501
often goes with the technology.” Although change—
in the form of acculturation— does occur, the centrality of maintaining a simple life based on tradition
does not allow for much variation within the community. There are numerous sects or orders of
Amish, each with differing perspectives on what is
acceptable with regard to use of modern technology.
Children are socialized to value community
strength and cohesion. Individual advancement and
mobility are not highly regarded normative values in
traditional Amish society. Children are taught to be
obedient, well-mannered, quiet, and humble. Parents
are responsible for their children’s behavior.
Traditional Amish children are educated in
schools in the community. Formal education ends at
the 8th grade. In 1972, the Supreme Court exempted
Old Order Amish from compulsory education past
this grade. The traditional Amish school is the
1-room schoolhouse in which all children are taught
in the same space by a single teacher.2 Socialization
with children of different ages is the typical pattern
in most Amish schools. The teacher rotates time,
actively teaching with each grade while the other
grades do readings and textbook exercises. The
teacher often instructs the older students, who then
work with the younger students. This further enhances the socially normative value of interdependence, group cohesion, and community.
The social context of growth and development in
childhood is considerably different compared to typical mainstream (what the Amish call “English”) settings. A behavior considered normal in a mainstream
family and school setting may be perceived differently in the traditional Amish context described
above. A child whose behaviors are interpreted as
being “disruptive” and “noncompliant” not only
harms his/her own reputation but also may be seen
as problematic in the social environment of a 1-room
schoolhouse that requires interpersonal cooperation
as well as self-management skills. Also, perceived
deviant behavior may be seen as a stain on the parent’s reputation.
Will’s behaviors are likely related to his muscular
dystrophy. Symptoms often become more pronounced at this age, and depression may accompany
the loss of motor skills and independence. Also, intellectual impairment may become pronounced at
this age. He may eventually require individualized
educational resources. At first, I thought that if ever
there was a poor “goodness of fit” between child and
environment, here it is! Will is a child with increasing
special needs in a community that values simplicity
and conformity. He lives in a community where the
educational expectations include attention, self-motivation, and discipline, especially challenging for a
child with possible depression and mental impairment. The limited capacity of the local school system
for the provision of specialized services or an individualized educational plan is an additional barrier.
However, after thinking about this for a while, I
began to see that this setting might have some particular advantages. Will lives in a community that
takes direct responsibility for its members. As an
example, many self-employed Amish do not partic1502
ipate in Social Security since they feel that it is the
responsibility of the community, not the government, to take care of its own members. The closeness
of relationships inherent in a small community and a
1-room schoolhouse could provide Will with the
strong social support system he will need as his
disease progresses. Perhaps he will require more
1-on-1 teaching and individual attention, which
might be possible in a 1-room schoolhouse by assigning an older student as a tutor.
The pediatrician can provide information and
guidance to the parents, teacher, and community. An
understanding that Will’s behaviors are not due to
an inherent “stubbornness,” nonconformity, or individualism (all stigmatized behaviors) but are a result
of his medical condition will be useful. When framed
in this way, I suspect that there will be greater tolerance for other behavioral and educational issues that
may develop and a greater effort to do whatever
possible to help this child successfully fit into the
social and educational environment. A health-beliefs
history will facilitate the pediatrician’s understanding about which issues are particularly concerning to
the family, as the parents reveal their beliefs regarding causes and meanings of the issues from their
perspective.3 The role of Will’s pediatrician in explaining the connection between the behaviors and
the chronic illness, and in negotiating potential solutions, is crucial.
Lee M. Pachter, DO
Professor of Pediatrics and Anthropology
University of Connecticut School of Medicine
Farmington, Connecticut
REFERENCES
1. Hostetler JA. Amish Society. 4th ed. Baltimore, MD: The Johns Hopkins
University Press; 1993
2. Hostetler JA, GE Huntington. Children in Amish Society: Socialization and
Community Education. New York, NY: Holt, Rinheart and Winston; 1971
3. Pachter LM. Ethnic and cultural influence on child health and health
services. In: Green M, Haggerty RJ, Weitzman M, eds. Ambulatory Pediatrics. 5th ed. Philadelphia, PA: W. B. Saunders; 1999:105–109
Lisa Schwartz
Pediatricians frequently are asked to evaluate children for ADHD when parents or teachers report
symptoms of hyperactivity, impulsivity, and inattention. This is a unique case in that the child has muscular dystrophy and lives in an Amish community.
Following Will’s initial visit to a referral clinic,
consultation with the teacher was challenging in that
Will attended an Amish school without a phone.
Will’s mother was asked to have the teacher call the
clinic in order to discuss Will’s classroom behaviors.
The teacher’s observations were consistent with
the behaviors reported by Will’s mother. The teacher
added that he does well in school when he is able
to complete his work. However, the young Amish
teacher was unfamiliar with the symptoms of ADHD
and could not provide insight into the reason for
Will’s symptoms.
When presented with a child who is disruptive,
hyperactive, and not completing schoolwork, the assessment should include depression, anxiety, and a
cognitive deficiency. Will’s parents were hesitant
DISRUPTIVE BEHAVIOR IN AN AMISH SCHOOL-AGED CHILD WITH DMD
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and uncomfortable when the possibility of a psychological problem was raised. The stigma of mental
illness, common among some Amish individuals,
may have hindered the teacher’s ability to assess
Will’s symptoms.1 Although his mother seemed to
accept a diagnosis of ADHD, which she perceived to
be a medical diagnosis associated with a pharmacological treatment, both parents were uncomfortable
with the standardized behavioral questionnaires to
assess psychological symptoms.
Will’s physical limitations associated with muscular dystrophy may have contributed to some of his
symptoms. As his father observed, the progressive
nature of the disease limited Will’s ability to physically keep up with his peers and to write his assignments. The teacher’s punishment for his behaviors,
taking away recess time, and sending him home,
reinforced the notion that he is no longer able to
participate in peer and academic activities. It did not
leave room for an alternative contingency management program. With an emerging realization of his
physical limitations, feelings of sadness and withdrawal may have led to Will’s inattentive behavior
and lack of interest in school.2 In addition, DMD is
associated with cognitive impairments that may account for some of Will’s symptoms at school.3 However, considering the teacher’s report of Will’s
academic achievement when he does complete assignments, it is unlikely that he has a significant
cognitive deficiency.
It is more likely that Will’s current symptoms can
be attributed to a new awareness of his condition and
the limitations related to it. He is at an age when
physical limitations in children with DMD are likely
to occur4 and may be associated with difficulties
with peer and academic activities. In this context, it is
not surprising that he has become withdrawn and
unmotivated about participation in peer and academic activities at school. Will may have a secondary
gain from his inattentive and noncompliant behavior
at school; loss of recess privileges and being sent
home removed him from the school environment.
Furthermore, without appropriate reinforcement for
his behaviors (eg, lack of rewards for school attendance and punishment for misbehaving in school),
Will currently has no motivation to change his behavior.
Effective intervention should be focused on educating the parents and teacher about the potential
psychological, physical, and cognitive manifestations
of muscular dystrophy and the importance of participating in normal childhood activities (eg, school and
peer play) consistent with his abilities. Will’s parents
and teacher can provide reinforcement for school
attendance and attentive and compliant behaviors at
school. The teacher also can offer appropriate reinforcements for Will’s behaviors that do not involve
removing him from academic or peer activities. Finally, in the context of an Amish family culture,
Will’s parents may not be comfortable with or have
access to psychosocial services.1,5 Potential barriers
to psychosocial services should be discussed with the
parents. Helping them to understand their son’s behaviors in the context of the muscular dystrophy and
potential benefits from counseling may be helpful.
Identification of culturally sensitive psychosocial services close to their Amish community should be a
priority for the clinician and family.
Lisa Schwartz, MA
Department of Psychology
Case Western Reserve University
Cleveland, Ohio
REFERENCES
1. Cates JA, Graham LL. Psychological assessment of the older order
Amish: unraveling the enigma. Prof Psychol Res Pr. 2002;33:155–161
2. Thompson RJ, Zeman JL, Fanurik D, Sirotkin-Roses M. The role of parent
stress and coping and family functioning in parent and child adjustment
to Duchenne muscular dystrophy. J Clin Psychol. 1992;48:11–19
3. Wicksell RK, Kihlgren M, Melin L, Eeg-Olofsson O. Specific cognitive
deficits are common in children with Duchenne muscular dystrophy.
Dev Med Child Neurol. 2004;46:154 –159
4. Warschausky S, Kewman DG, Bradley A, Dixon P. Pediatric neurological
conditions: brain and spinal cord injury and muscular dystrophy. In:
Roberts MC, ed. Handbook of Pediatric Psychology. 3rd ed. New York, NY:
Guilford Press; 2003:375–391
5. Greska LP, Korbin JE. Amish. In: Ember CR, Ember M, eds. Encyclopedia
of Medical Anthropology. Vol. 2: Cultures. New York, NY: Kluwer
Academic/Plenum Press; 2004:557–564
Dr Howard Taras
There are a number of issues to consider related to
how physicians communicate with their patients’
schools. Figuratively, a “triangle of communication”
exists for school-age health problems. A parent, a
physician, and a school representative comprise 3
corners of the triangle, and the child is in the center.
Reasons for the line of communication between physician and school to be the weakest are: (a) schools
without nurses often have no designated health representative, (b) teachers rarely feel comfortable telephoning doctors, (c) confidentiality concerns inhibit
communication, (d) physicians have neither time nor
reimbursement to attend school meetings, (e) primary care doctors’ offices are not organized to return
calls at precisely the time when teachers are available
to receive them, and (f) physicians not familiar with
the legal rights of students may restrict effective
advocacy. Only informed, intelligent, assertive, objective, and highly verbal parents can mitigate the
need for direct doctor-school communication. I will
address this boy’s circumstances through this lens of
communication with schools.
Medical. As a child with DMD enters third grade,
he may begin to experience fine and gross motor
problems that are debilitating enough to interfere
with class work but not yet obvious to school staff.
Even if his doctor educated Will’s 2nd grade teacher
last year, it would be erroneous to assume that the
school retained this knowledge. Physicians should
coordinate with parents to update schools annually
and whenever the medical condition changes. I recommend sending to the school a 1-page description
(using nonmedical terms) of a chronic disease that
may affect academic performance and behavior in
school and request that the correspondence become
part of the student’s permanent record. Annually
request parents of school-aged patients to complete a
form that permits you to interact with the child’s
school on health matters. There is a standard form
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SUPPLEMENT
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that is specific to school-related communication and
complies with federal regulations on exchange of
information (Health Insurance Portability and Accountability Act).1
Behavioral. ADHD does not sound likely here. Maladaptation to stress of a progressive muscular disorder is a more likely diagnosis. Schoolteachers need to
know that neurobehavioral changes are a part of the
phenotype of DMD. Decreased social awareness, affective problems (eg, labile mood, depression, and
anxiety), social isolation, aggression, and impairment
in some memory functions have been documented.2–4
Legal. Ideally, a school’s awareness of health and
neurobehavioral problems will lead to further school
investigation and accommodations. The teacher’s observations of Will’s behavior combined with her
knowledge of the disease should have led to a special
education evaluation that included physical therapy
and occupational therapy assessments and targeted
psychological testing. The federal law known as the
Individuals With Disabilities Education Act should
be in place to establish an individualized educational
program.5 Misbehavior, when attributable to a disability, prohibits use of punishment as a response
(although behavioral therapeutic responses are permitted as long as this is written into the individualized educational program).
If Will’s level of achievement is not significantly
different from his intellectual ability to qualify him
for special education, then the Rehabilitation Act
(Section 504) guarantees that reasonable accommodations will be made.6 Examples might be more time
to write assignments and individualized instructions
on school work in a manner that accommodates his
potential memory deficits. Most clinicians would be
able to advocate to this point, but it sounds as if this
child attends a private, Amish school. Schools that
do not receive public funds are not required to perform evaluations for special education or to offer
special education programs. Private religious schools
are exempt from even more basic accommodations
(like building a ramp for a child with a wheel chair or
granting untimed testing). The public school district
nearest Will’s community is responsible for his special education evaluation and providing some services. However, not all these services must be provided at the site of his Amish school.
Cultural. Will’s family and teacher are Amish.
They belong to a culture that emphasizes that all
children must be taught personal responsibility and
accountability— highlighting the needs of the group
rather than of the individual.7 Will’s physician
should explore whether Will’s educational needs are
being subjugated for the greater good of the classroom and, if so, work within the Amish culture and
community to modify how his needs can be met in
the cultural milieu of that school.
Howard Taras, MD
Professor of Pediatrics
University of California
Medical Director of Physician Consultation to San
Diego School Districts
San Diego, California
1504
REFERENCES
1. American Academy of Pediatrics. Health Insurance Portability and Accountability Act form for communication with schools. Available at:
www.schoolhealth.org. Accessed May 16, 2004
2. Hinton VJ, De Vivo DC, Nereo NE, Goldstein E, Stern Y. Selective deficits
in verbal working memory associated with a known genetic etiology: the
neuropsychological profile of Duchenne muscular dystrophy. J Int Neuropsychol Soc. 2001;7:45–54
3. Roccella M, Pace R, De Gregorio MT. Psychopathological assessment in
children affected by Duchenne de Boulogne muscular dystrophy. Minerva Pediatr. 2003;55:267–273, 273–276
4. Hinton VJ, De Vivo DC, Nereo NE, Goldstein E, Stern Y. Poor verbal
working memory across intellectual level in boys with Duchenne dystrophy. Neurology. 2000;54:2127–2132
5. Altshuler SJ, Kopels S. Advocating in schools for children with
disabilities: what’s new with IDEA? Soc Work. 2003;48:320 –329
6. Betz CL. Use of 504 plans for children and youth with disabilities:
nursing application. Pediatr Nurs. 2001;27:347–352
7. McGonigal K. Durkheimian explanations of key aspects of Amish culture. 2001. Available at: www.missouri.edu/⬃rsocjoel/rs150/papers/
endpaper.html. Accessed May 16, 2004
Dr Martin T. Stein
DMD was described in 1868 by a French neurologist as a muscular paralysis associated with muscle
hypertrophy and accumulation of large amounts of
fat and connective tissue in muscle. The X-linked
pattern of inheritance has been known for over a
century. A mutation in the translation of the gene on
the X chromosome that controls the synthesis of the
muscle protein, dystrophin, was discovered in 1986.
Dystrophin is found in concentrations less than 3% of
normal in muscle biopsies of children with DMD. A
dystrophin deficiency is associated with a membrane
defect that allows leakage of cytoplasmic components such as creatine kinase and influx of excessive
calcium. An excess of intracellular calcium causes the
muscle necrosis in patients with DMD. The serum
creatine phosphokinase is elevated at birth and during the preclinical phase of disease.1
DMD is a hereditary myopathy associated with
progressive weakness. Most patients are apparent to
clinicians by 3 to 4 years old with delayed walking
(half of patients) or an abnormal gait in association
with a markedly elevated level of serum creatine
phosphokinase. Weakness of the gluteal and hip extensor muscles cause a waddling gait and a compensatory exaggeration of lumbar lordosis. Proximal
arm weakness appears later. Although the rate of
progression varies, by 13 years of age all patients
have lost the ability to walk and use a wheel chair.
Kyphoscoliosis and weakness of respiratory muscles
are associated with recurrent pulmonary infections
and the most patients die before 20 years of age; 25%
of patients with DMD live beyond 21 years of age.
Cognitive deficiency is common in children with
DMD. Approximately 30% of patients have some
degree of mental retardation with the range of intelligent quotient between 46 and 134. Many patients
with average or above-average cognitive abilities
have deficits in immediate memory, verbal learning,
abstract concept formation, and flexibility. The
brains of patients with DMD demonstrate variable
alterations including pachygyria (widened gyri and
decreased number of sulci), a low brain weight, and
heterotopias (abnormal neuronal migration).
Knowledge about the inheritance, progressive
DISRUPTIVE BEHAVIOR IN AN AMISH SCHOOL-AGED CHILD WITH DMD
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weakness, and potential cognitive deficiency in children and adolescents with DMD is critical to the
provision of comprehensive care for Will, his parents, and his extended community. It guides a pediatrician’s recommendations about predictable
changes in physical activity, respiratory care, and
ongoing educational needs. Patients with DMD benefit from a comprehensive care team including a
neurologist, orthopedic surgeon, physical and occupational therapists, and a mental health professional.
Although Will’s father demonstrated some understanding of the disorder, further education might
enhance the family’s adaptive strategies at home and
their communication with the school. Education of
parents and patients must be ongoing and responsive to changes in developmental maturation. Are
there other children in Will’s family? What is his
mother’s understanding of the condition? Will a consultation visit with a genetic counselor be useful?
How will genetic counseling be perceived in the
Amish culture? Is Will’s experience of increasing
weakness and limitation of activities associated with
feelings of depression? His pediatrician is in a position to explore these issues, educate, guide, refer
when appropriate, and treat intercurrent problems at
an early stage. In addition, this case highlights the
importance of close attention to cultural variations
and determinants that may impact on the explanatory model of illness as perceived by the family and
community.
REFERENCE
1. Smith SA, Swaiman KF. Muscular dystrophies. In: Swaiman KF, Ashwal
SA. Pediatric Neurology: Principles and Practice. 3rd ed. St Louis: Mosby;
1999:1235–1242
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SUPPLEMENT
1505
Disruptive Classroom Behavior in an Amish School-Aged Child With Muscular
Dystrophy
Pediatrics 2004;114;1501
DOI: 10.1542/peds.2004-1721U
Updated Information &
Services
including high resolution figures, can be found at:
http://pediatrics.aappublications.org/content/114/Supplement
_6/1501.full.html
References
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_6/1501.full.html#ref-list-1
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PEDIATRICS is the official journal of the American Academy of Pediatrics. A monthly
publication, it has been published continuously since 1948. PEDIATRICS is owned, published,
and trademarked by the American Academy of Pediatrics, 141 Northwest Point Boulevard, Elk
Grove Village, Illinois, 60007. Copyright © 2004 by the American Academy of Pediatrics. All
rights reserved. Print ISSN: 0031-4005. Online ISSN: 1098-4275.
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