William E. MacLean

Maladaptive behaviors and risk factors among the genetic subtypes of Prader–Willi syndrome

Maladaptive behaviors among 65 people with Prader–Willi syndrome were assessed using the Reiss Screen for maladaptive behaviors. Young adults in their twenties were more likely to display aggressive behavior than adolescents and older adults. Differences in maladaptive behaviors between the typical deletion and uniparental disomy (UPD) subtypes were evaluated. The typical deletion subtype had higher self‐injury and stealing scores than the UPD subtype. Subject characteristics were differentially related to maladaptive behavior among the typical deletion and UPD subtypes. Differences in maladaptive behavior were also examined between the typical deletion type I and type II subtypes. The type I deletion subtype had greater physical depression scores than the type II deletion subtype. The Reiss Screen cut‐off scores were used to determine whether differences occurred between the subtypes at a clinically significant level. These findings offer insight into the health care needs of people with PWS.

Perceptions of Stress and Coping Strategies among Adults with Mild Mental Retardation: Insight into Psychological Distress.

Stress, coping, perceptions of control, and psychological distress of 88 adults with mild mental retardation were assessed. Stressful interpersonal interactions and concerns over personal competencies occurred most frequently. Frequency and stress impact were positively associated with a composite score of psychological distress. Active coping was associated with less psychological distress than distraction or avoidant coping. Perceptions of control were positively related to active coping and negatively related to avoidant coping. Active coping was related to less psychological distress when used with perceptions of high control than with perceptions of low control. Decreasing opportunities for stress and increasing accurate perceptions of control and subsequently active coping may reduce psychological distress among people with mild mental retardation.

Depression in Adults With Mild Intellectual Disability: Role of Stress, Attributions, and Coping

The experience of stressful social interactions, negative causal attributions, and the use of maladaptive coping efforts help maintain depression over time in the general population. We investigated whether a similar experience occurs among adults with mild intellectual disability. We compared the frequency and stress impact of such interactions, identified causal attributions for these interactions, and determined the coping strategies of 47 depressed and 47 nondepressed adults with mild intellectual disability matched on subject characteristics. The depressed group reported a higher frequency and stress impact of stressful social interactions, more negative attribution style, and more avoidant and less active coping strategies did than the nondepressed group. Findings have implications for theory building and development of psychotherapies to treat depression.

Self-Injury among a Community Cohort of Young Children at Risk for Intellectual and Developmental Disabilities

OBJECTIVE To identify risk factors for self-injurious behavior in young children with developmental delay and to determine whether that group is also more likely to exhibit other challenging behaviors. STUDY DESIGN A retrospective chart review of 196 children < 6 years of age referred for comprehensive neurodevelopmental evaluations. We analyzed child developmental level, receptive and expressive communication level, mobility, visual and auditory impairment, and co-morbid diagnoses of cerebral palsy, seizure disorders, and autism. RESULTS Sixty-three children (32%; mean age = 42.7 mo, 63% male) were reported to engage in self-injurious behavior at the time of the evaluation. Children with and without self-injurious behavior did not differ on overall developmental level, expressive or receptive language level, mobility status or sensory functioning, or in rates of identification with cerebral palsy, seizure disorders, or autism. However, the self-injurious behavior group was rated significantly higher by parents on destructive behavior, hurting others, and unusual habits. CONCLUSIONS Although self-injurious behavior was reported to occur in 32% of the cohort, the modal frequency was monthly/weekly and the severity was low. No significant differences were found for risk markers reported for adults, adolescents, and older children with intellectual and developmental disabilities. However, self-injurious behavior was comorbid with other behavior problems in this sample.

Coping Strategies of Adults with Mild Intellectual Disability for Stressful Social Interactions

Adults with mild intellectual disability (ID) experience stressful social interactions and often utilize maladaptive coping strategies to manage these interactions. We investigated the specific types of Active and Avoidant coping strategies reported by 114 adults with mild ID to deal with stressful social interactions. Open-ended responses to a sentence stem task were coded into 5 dimensions of Active and Avoidant coping. Adults with mild ID used Problem-Focused coping most frequently, and this strategy was negatively correlated with psychological distress. Emotion-Focused coping was used infrequently but was also negatively related to psychological distress. Coping accounted for a significant portion of variance in psychological distress after controlling for perceptions of stressful social interactions. Findings have important implications for informing the development of interventions to enhance the ability of adults with mild ID to cope with stressful social interactions.

Stress and incidence of bleeding in children and adolescents with hemophilia

OBJECTIVE To examine the relationship of stress and incidence of bleeding in boys with hemophilia. STUDY DESIGN We conducted a 6-month longitudinal study of 97 subjects (ages 4 to 16 years) from six hemophilia centers. Diaries recorded bleeding episodes (including site and history of previous trauma) and both child and parent daily stress. Parent and child stressful life event measures were obtained monthly. Socioeconomic data and clotting factor level were determined at enrollment. Logistic regression models examined the influence of recent stress on likelihood of bleeding on each day, controlling for factor level and socioeconomic data. We also determined associations of aggregated previous months events with bleeding likelihood in the succeeding month. RESULTS Fifty-eight percent of study participants had severe hemophilia. The sample population averaged nine bleeding episodes per 6 months; of these; two thirds of bleeding incidents occurred into joints and 44% after injury. Factor level strongly predicted bleeding incidence (p < 0.0001). Increased parent stress was associated with increased bleeding in general (odds ratio = 1.37, p < 0.003) and with injury (odds ratio = 1.65, p < 0.001), but not bleeding into joints. Similar findings followed parent reports of positive life events. Increased parent negative life events in 1 month were associated with increased bleeding in the succeeding month (p < 0.05). CONCLUSION Short- and long-term parental stress may lead to increased bleeding incidence in hemophilia, although factor level much more strongly predicts bleeding.

Maladaptive and Repetitive Behaviors in Children With Down Syndrome and Autism Spectrum Disorders: Implications for Screening

Researchers and clinicians are recognizing that a subgroup of children with Down syndrome (DS) also present with clinically significant impairments in social-communication and restricted patterns of behavior, such as those that characterize autism spectrum disorders (ASD). Efforts to better describe the behavioral presentation of children with co-occurring DS and ASD could be useful in developing screening and assessment procedures, which could, in turn, facilitate prompt access to appropriate intervention and supports. This study examines the specificity of various forms of maladaptive behavior (e.g., disruptive, repetitive, etc.) in children with co-occurring DS and ASD (n = 22), relative to children with DS (without ASD; n = 54) and those with a nonspecific developmental disorder (DD; n = 34). Parent report of child behavior was obtained on 110 children, ages 3–11 years (mean age = 73 months), who had completed a comprehensive diagnostic evaluation for autism. Two experienced clinical psychologists reviewed more than 50% of cases and independently determined ASD status. One-way analyses of variance were conducted to examine behavioral variables across diagnostic groups. Children with DS + ASD were reported to exhibit more problem behaviors than children in the DS group (without ASD) as well as more self-absorbed behaviors and poorer social relating. The DS + ASD group and DD group had significantly higher scores than the DS only group on overall repetitive behaviors and on “low forms” of repetitive behaviors. Children with DS + ASD were not significantly different from children in the DD group on any of the behavioral variables. Implications for screening and assessment of autism symptoms in children with DS are discussed.

Reassurance seeking and depression in adults with mild intellectual disability

BACKGROUND Adults with intellectual disability (ID) experience a high prevalence of depression. Yet, little research has investigated interpersonal processes related to depression in this population. In the general population, depressed persons behave in ways that elicit negative and rejecting reactions from others. In particular, excessive reassurance seeking, defined as excessively and persistently seeking assurance from others that one is lovable and worthy, indirectly contributes to depressive symptoms through evoking negative and rejecting social interactions. We examined the relation between excessive reassurance seeking, negative and rejecting social interactions and depression in adults with mild ID. METHOD Eighty-seven adults with mild ID and staff completed the Glasgow Depression Scale for people with a Learning Disorder and the Reassurance-Seeking Scale. In addition, adults with mild ID reported on their experience of negative social interactions, and staff rated their relative preference to interact with the adult with mild ID. A meditational model of the indirect effect of excessive reassurance seeking on depressive symptoms via negative and rejecting social interactions was tested. RESULTS Excessive reassurance seeking was positively related to depressive symptoms. Negative and rejecting interactions partially mediated the relation between excessive reassurance seeking and depressive symptoms. CONCLUSIONS Findings identify an important interpersonal process in depression. Efforts to educate staff and adults with mild ID about excessive reassurance seeking and ways to alter it may be useful in treating depression.

Self-Injury in a Statewide Sample of Young Children With Developmental Disabilities

Self-injurious behavior (SIB) is a devastating condition associated with intellectual and developmental disabilities (IDD). Efforts to understand its development are focused on early childhood when the behavior first emerges. Limited prevalence data on SIB during early childhood are currently available. The purpose of this study was to determine the prevalence of SIB in a sample of children with IDD between the ages of 18 and 72 months and associated demographic, diagnostic, and behavioral characteristics. The prevalence rate was 19.1% for SIB including head banging, self-biting, and hitting self. Factors associated with SIB in adults, such as severe intellectual disability; visual, hearing, or mobility impairment; or diagnoses of autism, cerebral palsy, or seizure disorder were not associated with SIB in these children. However, children with SIB had higher rates of Hurtful to Others than the contrast group. Issues related to the characterization of SIB in young children are also discussed.

Patient and Parent/Guardian Perspectives on the Health Care of Adults with Mental Retardation.

Individuals with mental retardation and their parents/guardians have long been neglected in health care research. In this study we used a qualitative methodology to describe the experiences adults with mental retardation and their parents/guardians have with the delivery of primary care services. Semi-structured in-depth interviews were conducted with 12 adults who had mental retardation and 9 parents/guardians. The grounded theory approach was utilized to guide data collection and analysis. Findings suggest an overall positive experience with primary care services. Unique themes were identified that distinguished parent/guardian experiences from those of adults with mental retardation, who focused on immediate, concrete issues related to service delivery, whereas parents/ guardians focused on more peripheral or abstract issues related to the delivery of primary care.