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Neuromuscular Disorders A Primer For School Psychologists
Neuromuscular Disorders A Primer For School Psychologists
Neuromuscular Disorders: over 4 million births in the United States each year, about 400 to 600 boys with DBMD
are born every year (Centers for Disease Control and Prevention, 2006; Muscular Dys-
trophy Association, 2006).
A Primer for School Infantile progressive spinal muscular atrophy (SMA) is a genetic disease in which
loss of motor neurons in the spinal cord affects the part of the nervous system that
Psychologists controls voluntary muscle movement. SMA is caused by a deficiency of a motor neuron
protein called SMN, necessary for survival of motor neurons, resulting from a faulty
BY KRISTINE AUGUSTYNIAK & LISA KILANOWSKI-PRESS gene on chromosome five. Onset of the disease is before birth to 18 months of age.
The symptoms of SMA are generalized muscle weakness, weak cry, trouble with swal-
mproved medical care and legislative mandates have increased the degree to lowing as well as sucking, and breathing distress. Babies cannot sit without support.
© 2008, N atio nal As s o ciatio n o f S chool Psyc h ologi st s December 2008, Volume 37, Number 4 | Communiqué | 23
(MDS); mitochondrial encephalomyopathy; lactic acidosis and stroke-like episodes psychological problems, a rate twice that of nonafflicted peers. The psychosocial diffi-
(MELAS); myoclonus epilepsy with ragged red fibers (MERRF); mitochondrial neuro- culties faced by individuals with neuromuscular disorders include depression, negative
gastrointestinal encephalomyopathy (MNGIE); neuropathy, ataxia, and retinitis pig- body image, social isolation and inhibition, anger, anxiety, and feelings of helplessness
mentosa (NARP); and progressive external ophthalmoplegia (PEO). MITO is caused by and hopelessness. Caregivers are at increased risk for poorer psychological adjustment
defective genes that produce proteins that process food into energy within cells. The pro- marked by higher stress, less family support, greater intrafamilial conflict, feelings of
tein affected determines which of the mitochondrial diseases is present. Onset, symp- isolation, and extended bereavement from multiple losses (Livench & Antonak, 1994;
toms, and progression of the condition vary according to disease. Mitochondrial myo- Polakoff et al., 1998). School psychologists, counselors, and teachers must be diligent
pathies are relatively rare, having an incidence of approximately 6 out of every 100,000 in their evaluation of behavioral or emotional difficulties, particularly in reference to
individuals to as high as 16 out of 100,000 individuals (Cobb, 2005; MDA, 2006). potential classroom-based challenges such as inattention, lack of motivation, or non-
Medical evaluation and diagnosis of NMDs is complex and often complicated by compliance, which are strongly linked to the effects of pain and fatigue. Furthermore,
the vague nature of initial presenting concerns, such as muscle weakness, fatigue, and although family systems intervention may be outside of the purview of school-based
double vision, among others, that often overlap with other medical conditions. Dur- service delivery in many districts, evaluation of family functioning in terms of the
ing the course of evaluation, physicians may rely on diagnostic tools such as electrodi- student’s psychological well-being presents as a relevant consideration. Despite the
agnostic examination (EDX), spinal fluid availability of counseling and behavioral intervention supports in the schools, parents
analysis, muscle and nerve biopsies, skin surveyed as part of a study of school-based service provision to students with NMDs
biopsies, and the assessment of the auto- Neglecting the rated counseling services least favorably of all service areas assessed. Lack of initial
nomic nervous system (including cardio- importance of pain evaluation for counseling services, lack of clarity about counseling goals and objectives
vascular and sensory systems, among oth- management leads to for IEP counseling, as well as inattention to social and emotional needs by school staff
ers; Cleveland Clinic, 2008). Functional were reported as areas of concern by respondents (Murphy et al., 2006). Such findings
limitations resulting from the NMDs are
critical omissions of suggest a need to increase counseling support for students with NMD and to improve
typically assessed by related practitioners service that may be the quality and scope home–school collaboration.
such as occupational therapists, physical provided to the student,
therapists, and speech therapists. Due to while also reducing the DIRECTIONS FOR SCHOOL PSYCHOLOGISTS
AND ALLIED EDUCATION PROFESSIONALS
the progressive nature of most NMD’s, likelihood that proper
youth who have been recently diagnosed Given the low incidence of the disabilities and potentially limited exposure to work-
may not demonstrate a strong degree of
accommodations, ing with NMD populations, school psychologists and other school-based service pro-
functional impairment. Visits to neurolo- modifications, and viders may need additional training to provide effective services. The following serve
gists and other practitioners may initially strategies related as suggestions to increase quality of supports provided to students with neuromus-
occur infrequently but increase as the dis- to the presence cular disorders.
ease progresses. of chronic pain are ■ School psychologists should seek to conduct comprehensive needs assessments
At present, there is no cure for any of of any student recently diagnosed with neuromuscular disorder. Such assess-
the NMDs, leaving disease management
included in the ments need not be contingent upon special education eligibility determination
and remediation of functional limitations comprehensive IEP. procedures and should be designed to identify areas of potential concern and
the primary course of action for physi- intervention outside of the realm of special education classification. Needs as-
cians. Despite the lack of a cure, many sessments should include evaluation for informal classroom modifications and
treatments designed to slow the progres- informal counseling supports, if available, as well as referrals to community sup-
sion of the disease, as well as its symptomatic presentation, are regularly employed, port services. Consideration of Section 504 plans for students not in need of for-
and include gene therapy, use of steroids, intravenous immunoglobulin treatment, and mal special education support is critical.
therapeutic apherisis, among others. Increasing functional skills, improving quality of ■ When conducting comprehensive psychoeducational evaluations of students
life, and working to ensure longevity are the primary aims of medical intervention. diagnosed with NMD, or in suspected cases of NMD, practitioners involved in
the evaluation process must be sensitive to their own professional limitations.
CONSIDERATIONS FOR SCHOOL PSYCHOLOGISTS In cases where any set of presenting concerns extend beyond the expertise of
Cognitive Functioning. Though cognitive issues related to increased pain and dis- staff, including school psychologists, counselors, occupational and physical
comfort may affect a child’s academic achievement, current research suggests that therapists, and nurses, outside evaluations should be conducted. Recommenda-
students with neuromuscular diseases most often evidence only a mild degree of im- tions from outside service providers skilled in working with students with NMD,
pairment in cognitive function (slightly below average cognitive ability). In addition including suggestions regarding classroom modifications and assistive technolo-
to mild disruptions in language ability and speech production, decreased sustained gies, should be explored.
attention, variable memory performance, and slowing of information processing are ■ Parent reports regarding lack of timely identification of NMD symptoms and
the most frequently cited areas of decline (Botez-Maquard & Botez, 1993; Dorman, functional impairment by school districts is of concern. Such oversights in de-
Hurley, & D’Avignon, 1988; Flood & Perlman, 1987; Hart, Henry, Kwentus & Leshner, tection of dysfunction have led to delays in diagnosis of neuromuscular disorder
1986; Zeigler & Wessel, 1996). Consideration of the aforementioned domains is essen- as well as the assignment of appropriate interventions and supports. As such,
tial when developing appropriate special education programming, particularly if it is school psychologists may proactively seek to present information relevant to
hoped to continue appropriate cognitive stimulation, communication skill develop- the diverse presentation of NMD to school faculty and staff via inservice presen-
ment, and educational attainment despite decline in physical functioning (Polakoff, tations, workshops, or newsletters. Increasing awareness of the various features
Morton, Koch, & Rios, 1998). of the disorder may lead to increased early detection and intervention efforts.
Pain Management. One of the most frequently overlooked yet critical aspects of Urging staff to report any concerns regarding observations of deviations from
work with students with neuromuscular disorder involves the need to address the in- normative functioning or changes in student motor functioning may assist in
fluence that pain exerts on student well-being and educational attainment. Although early diagnosis and treatment. Additionally, given the ability of school psycholo-
school-based professionals are typically inexperienced in this domain, studies by Ze- gists to research, analyze, synthesize, and disseminate information, practitio-
bracki and Drotar (2008) and McKenzie et al. (2000) highlight the importance of edu- ners are well positioned to conduct disability awareness trainings for school
cators’ understanding of the relationship between pain management, activity limita- professionals already working with children diagnosed with NMD, thus increas-
tions, fatigue, and emotional distress among youth with NMDs. The acute or chronic ing the overall responsiveness of the school community. Within such a forum,
presentation of pain yields a variety of implications for educational progress and at- it may also be possible for misconceptions about the disability, including the re-
tainment, all of which should be considered and addressed in the student’s Individu- lationship between physiological function and overall well-being, as well as the
alized Education Plan (IEP). Routine assessment of pain and its sequelae can provide influence of pain on functioning, to be resolved directly and immediately, while
valuable information to medical professionals as they consider pharmacological and providing opportunities for open discourse regarding the best means to inter-
orthotic options and to mental health professionals as they refine cognitive behavioral vene on behalf of the child.
strategies to increase the child’s ability to cope with pain. Neglecting the importance ■ School psychologists are well poised to perform roles as liaisons and service
of pain management leads to critical omissions of service that may be provided to coordinators among home, school, medical, and community environments.
the student, while also reducing the likelihood that proper accommodations, modi- While the school nurse is an invaluable resource in terms of medical manage-
fications, and strategies related to the presence of chronic pain are included in the ment, school psychologists are skilled in their ability to synthesize medical and
comprehensive IEP. functional information into meaningful, educationally relevant terms. Desig-
Social and Emotional Needs. The social and emotional consequences of NMD may nation of the school psychologist as a critical point of contact, or “case man-
also have debilitating affects on children and their families. Twenty to thirty percent ager,” for students with NMD may serve to ensure greater continuity in care
of children with chronic illness or disability will experience significant behavioral or and intervention between outside service providers and school-based inter-
24 | Communiqué | December 2008, Volume 37, Number 4 © 2 0 0 8 , Nat io nal A sso c iat io n o f Sc ho o l P syc ho lo g ist s
ventionists. Given the breadth of knowledge that school psychologists possess ease Center. Retrieved September 28, 2008, Students with neuromuscular disorders: A sur-
from http://my.clevelandclinic.org/ vey of parental satisfaction with school based
regarding intervention and consultation, practitioners may serve as key contact
neuromuscular_center/default.aspx services. International Journal of Special Educa-
persons responsible for sharing information regarding changes in student func- tion, 12, 68–76.
Cobb, B. R. (2005). Mitochondrial myopathies.
tioning and progress, as well as student needs across home, school, and clini- In S. L. Chamberlin & B. Narins (Eds.), The Muscular Dystrophy Association (2006) Facts
cal domains. Similarly, school psychologists are ideally suited to ensure that Gale encyclopedia of neurological disorders about Duchenne & Becker Muscular Dystrophies.
appropriate referrals to outside agencies (e.g., family support and counseling; (Vol.2), (pp. 534–538). Detroit: Thomson Retrieved September 20, 2008, from http://
Gale. www.mda.org/publications/fa-dmdbmd-what
individual counseling; support groups; community associations well versed in .html
NMDs, respite, assistive technology) occur when necessary, both within and Dawson, S., & Kristjanson, L. J. (2003). Mapping
the journey: Family carers’ perceptions of National Institute of Neurological Disorders and
outside of the initial referral process. issues related to end-stage care of individuals Stroke (2007). Friedreich’s Ataxia fact sheet.
■ Given the variety of social and emotional challenges faced by students with NMD, with muscular dystrophy or motor neuron Retrieved Sep 20, 2008, from http://www
any student with a neuromuscular disorder who is receiving special education disease. Journal of Palliative Care, 19, 36–42. .ninds.nih.gov/disorders/friedreichs_ataxia/
detail_friedreichs_ataxia.htm
supports should be evaluated for counseling services. Service providers engaged Dorman, C., Hurley, A. D., & D’Avignon, J.
in providing IEP-related counseling supports to students with NMD should strive (1988). Language and learning disorders of Polakoff, R. H., Morton, A. A., Koch, K. D., &
older boys with Duchenne muscular dystro- Rios, C. M. (1998). The psychosocial and cog-
to identify clear goals and objectives within the counseling arena and should seek phy. Developmental Medicine and Child nitive impact of Duchenne’s muscular dystro-
to share information regarding student functioning and progress with parents. Neurology, 30, 316–327. phy. Seminars in Pediatric Neurology, 5, 116–123.
Domains of social and emotional functioning that may warrant exploration during Flood, M. K., & Perlman, S. L. (1987). The men- Sandoval, J. (2006). Neuromuscular diseases.
counseling sessions include depression, anxiety, peer relationships, body image, tal status of patients with Friedreich’s ataxia. In L. Phelps (Ed.), Health-related disorders in
Journal of Neuroscience Nursing, 19, 251–255. children and adolescents: A guidebook for under-
changes in physical functioning, changes in academic performance, stigma, fear standing and educating (pp. 193–211). Washing-
of the future, perceived needs at school, and family relationships in the face of de- Hart, R. P., Henry, G. K., Kwentus, J. A., & Leshner,
ton, DC: American Psychological Association.
R.T. (1986). Information processing speed of
generative functioning (Livench & Antonak, 1994; Polakoff, et al., 1998). Although children with Friedreich’s ataxia. Developmental SMA Foundation (2006). Retrieved Sep 20,
school districts may not provide family based counseling supports, consideration Medicine & Child Neurology, 28, 310-313. 2008, from http://www.smafoundation.org/
of familial challenges from a family-systems perspective may prove helpful when index.php?option=com_faq&Itemid=32&
Livench, H., & Antonak, R. F. (1994). Review of
Itemid=32#answer3
serving as a consultant between home and school. Referrals to community family research on psychosocial adaptation to neuro-
counseling centers and support groups, if available, may benefit families experi- muscular disorders: I. Cerebral palsy, muscu- Strong, K., & Sandoval, J. (1999). Mainstreaming
lar dystrophy and Parkinson’s disease. Journal children with a neuromuscular disease: A map
encing acute and prolonged illness-related distress. ■ of Social Behavior and Personality, 9, 201–230. of concerns. Exceptional Children, 65, 353–366.
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© 2008, N atio nal As s o ciatio n o f S chool Psyc h ologi st s December 2008, Volume 37, Number 4 | Communiqué | 25
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