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

Rebecca S. Morse

Advisor: Johannes Rojahn, PhD, Department of Psychology

Committee Members: Robert Pasnak, Theodore Hoch

Psychological Clinic, Conference Room
June 17, 2011, 03:00 PM to 11:00 AM


Smith Magenis Syndrome (SMS) is a rare genetic syndrome (1/15,000) 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 (Dykens & Smith, 1998). Earlier research has shown increased parental stress with increased maladaptive behaviors of the child with SMS, and lack of support resources for the family. This study attempted to extend those earlier research findings. 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 (Sparrow, Balla, & Cicchetti, 1984),  the Achenbach Child Behavior Checklist (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). Hierarchical multiple regression was used to examine the effect of the children’s maladaptive behavior as the predictor variables of parental stress after controlling for age and cognitive level. To examine the relationship between parent stress, family functioning and parental coping strategies, parent stress levels were used as the predictor, and parent coping and family functioning as the outcome variables. Maladaptive behaviors (CBCL) did predict parent stress over and above age (p<.05); however, it was not predictive for life stress. Maladaptive behaviors (CBCL total score and externalizing) did predict parent stress over and above IQ (p<.05). Only internalizing behaviors were predictive for life stress, when accounting for IQ (p<.05). Adaptive functioning (VABS) was not predictive for either parent or life stress, when accounting for age or IQ. Similarly, parent stress levels did not predict overall family adjustment, or parent coping style. We assume that the lack of consistent predictive power was due to the restricted range in all dependent variables.