Abstract
Smith Magenis Syndrome (SMS) is a rare genetic syndrome most commonly caused by a microdeletion on chromosome 17 p11.2. It is associated with a pattern of physical, developmental, and behavioral characteristics including intellectual disability, sleep disturbance, and a variety of behavior problems. The purpose was to examine the relationship between maladaptive behaviors in children with SMS and parent stress, and to determine whether family functioning and effective parent coping strategies may alleviate the impact of child maladaptive behaviors on parent stress. Data were collected on 25 individuals with SMS ages 1.4 to 19.4 years old. Parents were interviewed using the Vineland Adaptive Behavior Scale (VABS; Sparrow et al. 1984), the Achenbach Child Behavior Checklist (CBCL; Achenbach 1991), the Family Assessment Device-General Functioning Scale (Epstein et al. 1983), the Family Crisis Oriented Personal Evaluation Scales (McCubin et al. 1991), the Parental Stress Index-3rd edition (Abidin 1995), and the Stress Index for Parents of Adolescents (Abidin 1995). Using multiple hierarchical regression analyses we found that maladaptive behaviors (CBCL internalizing and externalizing behaviors) affected parent stress; however, we also saw that parent stress was alleviated in well-functioning families. Maladaptive behaviors did not have a significant negative impact on life stress.
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Author Note
This study was made possible by a data sharing agreement between the National Human Genome Research Institute and George Mason University. This study was supported by the Intramural Research Programs of the National Human Genome Research Institute, NIH, USDHHS. The author’s gratefully recognize Parents and Researchers Interested in Smith–Magenis syndrome (PRISMS) for partial sponsorship of a doctoral student fellowship (R.M.). The authors wish to express their gratitude to the families who actively participated in this study.
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Morse, R.S., Rojahn, J. & Smith, A.C.M. Effects of Behavior Problems, Family Functioning, and Family Coping on Parent Stress in Families with a Child with Smith-Magenis Syndrome. J Dev Phys Disabil 26, 391–401 (2014). https://doi.org/10.1007/s10882-014-9367-3
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DOI: https://doi.org/10.1007/s10882-014-9367-3