Smith-Magenis Syndrome: Maladaptive Behaviors and Effects on Parent Stress, Coping, and Family Adjustment




Morse, Rebecca S.

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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. Data were collected on 25 individuals with SMS, 10 males (mean IQ 72; SD 13), and 15 females (mean IQ 68; SD 22.2); ages 1.4-19.4 (mean 6.56; SD 4.71). Parent reports were obtained using the Vineland Adaptive Behavior Scale (VABS; Sparrow, Balla, & Cicchetti, 1984), the Achenbach Child Behavior Checklist (CBCL; Achenbach, 1991), the Family Assessment Device-General Functioning Scale (Epstein, Baldwin, & Bishop, 1983), the Family Crisis Oriented Personal Evaluation Scales (McCubin, Olson, & Larson, 1991), the Parental Stress Index-3rd edition (Abidin, 1995), and the Stress Index for Parents of Adolescents (Abidin, 1995). We found positive linear relationships between maladaptive behaviors and parent stress, but no association between maladaptive behaviors and overall life stress. We also found that parent stress levels were not associated with measures of family adjustment or specific parent coping strategies.



Developmental, Syndrome, Behavior, Disabilities