TY - JOUR
T1 - Behavioral functioning in cardiofaciocutaneous syndrome
T2 - Risk factors and impact on parenting experience
AU - Pierpont, Elizabeth I.
AU - Wolford, Melinda
N1 - Publisher Copyright:
© 2016 Wiley Periodicals, Inc.
PY - 2016/8/1
Y1 - 2016/8/1
N2 - The present study is an investigation of behavioral functioning in children with cardiofaciocutaneous syndrome (CFC). CFC is a rare single-gene disorder associated with cardiac disease, characteristic skin and facial features, intellectual disability, and neurological complications such as seizures and structural brain anomalies. Emotional and behavioral features of CFC have not been systematically investigated. We aimed to identify key variables that contribute to psychopathology during childhood and adolescence, and to examine the impact of challenging behaviors on the caregiving experience. Parents of 34 children and adolescents with CFC completed standardized broadband measures of child emotional and behavioral functioning, as well as measures of sensory modulation, functional communication, and caregiver stress. Results indicate that children with CFC syndrome are at heightened risk for psychopathology, with attention problems, social difficulties, and unusual behaviors (e.g., obsessive thoughts, strange behaviors, repetitive acts) found to be especially prevalent. Behavioral challenges in children with CFC syndrome were significantly associated with a history of obstetric complications and with problems modulating sensory information. With regard to the impact of child neurocognitive and behavioral issues on the caregiving experience, parent self-reported stress was significantly higher among parents of children who engaged in more problem behaviors, and lower among parents whose children could communicate effectively with others. Results of this study suggest avenues to help families cope with CFC-related stressors and enhance overall functioning. In particular, this study highlights the need for educational and treatment interventions aimed at addressing sensory needs, increasing functional communication, and identifying and managing challenging behaviors.
AB - The present study is an investigation of behavioral functioning in children with cardiofaciocutaneous syndrome (CFC). CFC is a rare single-gene disorder associated with cardiac disease, characteristic skin and facial features, intellectual disability, and neurological complications such as seizures and structural brain anomalies. Emotional and behavioral features of CFC have not been systematically investigated. We aimed to identify key variables that contribute to psychopathology during childhood and adolescence, and to examine the impact of challenging behaviors on the caregiving experience. Parents of 34 children and adolescents with CFC completed standardized broadband measures of child emotional and behavioral functioning, as well as measures of sensory modulation, functional communication, and caregiver stress. Results indicate that children with CFC syndrome are at heightened risk for psychopathology, with attention problems, social difficulties, and unusual behaviors (e.g., obsessive thoughts, strange behaviors, repetitive acts) found to be especially prevalent. Behavioral challenges in children with CFC syndrome were significantly associated with a history of obstetric complications and with problems modulating sensory information. With regard to the impact of child neurocognitive and behavioral issues on the caregiving experience, parent self-reported stress was significantly higher among parents of children who engaged in more problem behaviors, and lower among parents whose children could communicate effectively with others. Results of this study suggest avenues to help families cope with CFC-related stressors and enhance overall functioning. In particular, this study highlights the need for educational and treatment interventions aimed at addressing sensory needs, increasing functional communication, and identifying and managing challenging behaviors.
KW - RASopathies
KW - attention
KW - behavior
KW - cardiofaciocutanous syndrome
KW - communication
KW - parenting stress
KW - sensory modulation
KW - sensory processing
KW - social
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U2 - 10.1002/ajmg.a.37725
DO - 10.1002/ajmg.a.37725
M3 - Article
C2 - 27149079
AN - SCOPUS:84965019197
SN - 1552-4825
VL - 170
SP - 1974
EP - 1988
JO - American Journal of Medical Genetics, Part A
JF - American Journal of Medical Genetics, Part A
IS - 8
ER -