Anxiety, Fears, and Phobias in Persons With Williams Syndrome

Although much research has focused on the cognitive-linguistic profile associated with Williams syndrome, studies have yet to follow up on preliminary observations suggesting increased anxiety and fears in persons with this disorder. To this aim, Study 1 compared fears in 120 participants with Williams syndrome to 70 appropriately matched persons with mental retardation of mixed etiologies. Study 2 assessed differences in parent versus child reports of fears in 36 Williams syndrome and 24 comparison group parent-child dyads. In Study 3, rates of phobia and other anxiety disorders were assessed in standardized psychiatric interviews with the parents of 51 individuals with Williams syndrome. Relative to their counterparts, persons with Williams syndrome had significantly more fears as well as a wider range of frequently occurring fears, as reported by either parents or participants themselves. Children in both groups reported more fears than their parents. Whereas generalized and anticipatory anxiety were found in 51% to 60% of the sample with Williams syndrome, specific phobia was more prevalent, with 96% showing persistent and marked fears and 84% avoiding their fears or enduring them with distress. The feasibility of cognitive-behavioral treatments for phobia is discussed, as are implications for future research.     

Anxiety,Fears, and Phobias in Persons With Williams Syndrome

Although much research has focused on the cognitive-linguistic profile associated with Williams syndrome, studies have yet to follow up on preliminary observations suggesting increased anxiety and fears in persons with this disorder. To this aim, Study 1 compared fears in 120 participants with Williams syndrome to 70 appropriately matched persons with mental retardation of mixed etiologies. Study 2 assessed differences in parent versus child reports of fears in 36 Williams syndrome and 24 comparison group parent-child dyads. In Study 3, rates of phobia and other anxiety disorders were assessed in standardized psychiatric interviews with the parents of 51 individuals with Williams syndrome. Relative to their counterparts, persons with Williams syndrome had significantly more fears as well as a wider range of frequently occurring fears, as reported by either parents or participants themselves. Children in both groups reported more fears than their parents. Whereas generalized and anticipatory anxiety were found in 51% to 60% of the sample with Williams syndrome, specific phobia was more prevalent, with 96% showing persistent and marked fears and 84% avoiding their fears or enduring them with distress. The feasibility of cognitive-behavioral treatments for phobia is discussed, as are implications for future research.     

Affective disorder in the parents of a clinic sample of children with anxiety disorders

BACKGROUND: Family history studies in adults reveal strong familiality for the anxiety disorders with some specificity. The aim of the current study was to establish whether there was an elevated rate of anxiety disorders in the parents of children with anxiety disorders, and whether there was intergenerational specificity in the form of disorder. METHODS: The mental state of a clinic sample of 85 children with anxiety disorder and their parents was systematically assessed, together with a comparison sample of 45 children with no current disorder and their parents. RESULTS: Compared to the rate of anxiety disorder amongst parents of comparison children, the rate of current anxiety disorder in mothers of anxious children was significantly raised, as was the lifetime rate of anxiety disorder for both mothers and fathers. The mothers of children with generalised anxiety disorder, social phobia, specific phobia and separation anxiety disorder all had raised lifetime rates of the corresponding disorder, but also raised rates of others disorders. LIMITATIONS: Only 60% of the fathers of the anxious children were assessed. CONCLUSIONS: Strong familiality of anxiety disorders was confirmed, especially between child and maternal anxiety disorder. All child anxiety disorders were associated with several forms of anxiety disorder in the mother. Some specificity in the form of anxiety disorder in the child and the mother was apparent for social phobia and separation anxiety disorder. The findings have implications for the management of child anxiety.     

Cognitive therapy for depressed adults with comorbid social phobia

BACKGROUND: Evidence suggests that comorbid depression influences the outcome of cognitive-behavioral treatment for patients presenting with social phobia. Little is known, however, about the influence of comorbid social phobia on the response to cognitive therapy (CT) for depression among adults presenting with recurrent major depressive disorder (MDD). These analyses seek to clarify this relationship. METHODS: Patients (N=156) with recurrent DSM-IV MDD entered CT (20% also met DSM-IV criteria for social phobia). Every week during the course of CT, clinicians assessed depressive symptoms and patients completed self-report instruments measuring severity of depression and anxiety. RESULTS: At presentation, outpatients with comorbid social phobia reported greater levels of depressive symptoms and clinicians rated their impairment as more severe, compared to their counterparts without social phobia. Patients with or without comorbid social phobia did not differ significantly in (1) attrition rates; (2) response or sustained remission rates; (3) time to response or sustained remission; or (4) rate of improvement in symptoms of depression or anxiety. LIMITATIONS: The lack of domain-specific measures limits inference with respect to the improvements in social anxiety that occur with CT of depression. CONCLUSIONS: These findings introduce the hypothesis that CT for depression may be flexible enough to treat the depressive symptoms of patients presenting with MDD who also suffer from social phobia.     

Illness fears in the general population

OBJECTIVES: Because relatively little is known about illness fears, we sought to estimate the prevalence, risk factors, and morbidity associated with such fears in the community. METHODS: We conducted a brief telephone survey of persons aged 40 to 65 years from randomly selected households in the Johnson County, Iowa, area. Respondents were asked whether a series of illness and medical care items made them no more nervous, somewhat more nervous, or much more nervous than other people. Those who reported more discomfort were asked to what extent this interfered with medical care or caused impairment or distress. Information about demographic and health characteristics was also obtained. RESULTS: Five hundred persons, 62% of those contacted, responded to the survey. A factor analysis revealed four fear dimensions: illness/injury, medical care, blood/needle, and aging/death. Five percent of respondents reported much more nervousness in relation to at least four of six illness/injury items, 4% indicated that such fears interfered with their medical care, and 5% reported some negative effect on their life. Similarly, 5% of respondents reported much more nervousness in relation to at least two of four medical care items. Illness/injury fears were somewhat more common in persons with lower income and education and in those with medical conditions. CONCLUSIONS: This survey shows that fears of illness and medical care are common in the general population and indicates that lower socioeconomic status and experience with illness are associated with these fears. The findings also suggest that interference with care occurs among those with the strongest fears.     

Fears and phobias: reliability and heritability.

BACKGROUND: Familial factors, which are partly genetic, influence risk for phobias. Prior family and twin studies, however, were based on a single lifetime assessment, which may be only moderately reliable. METHODS: We obtained, 8 years apart, two assessments of lifetime history of five unreasonable fears and phobias (agoraphobia and social, situational, animal and blood-injury phobia) from face-to-face and telephone interviews from 1708 individual female twins from a population-based registry. We also obtained, 1 month apart, test retest reliability on 192 twins. We fitted, using the program Mx, a measurement model that estimates the role of genetic and environmental risk factors correcting for measurement error. RESULTS: Short-term reliability of the five phobias was modest (mean kappa = 0.46), but higher than long-term stability (mean kappa = 0.30). Unreliability occurred both for subject recall of unreasonable fears and for interviewer assessment of which fears constituted phobias. Examining fears and phobias together, in a multiple threshold model, results suggested that twin resemblance was due solely to genetic factors, with estimated total heritabilities, corrected for unreliability, of: any 43%, agoraphobia 67%, animal 47%, blood/injury 59%, situational 46% and social 51%. With the exception of animal phobia, similar results were obtained analysing phobias alone. CONCLUSIONS: Lifetime histories of unreasonable fears and phobias assessed at personal interview have substantial unreliability. Correcting for unreliability, the liability to fears and their associated phobias is moderately heritable. Individual-specific environmental experiences play an important role in the development of phobias, while familial-environmental factors appear to be of little aetiological significance.     

Relaxation therapy, desensitization, and the treatment of anxiety-based disorders.

Evaluated systematic desensitization and relaxation training for the treatment of snake phobia and test anxiety as representatives of two classes of anxiety-based disorders. Treatment outcomes were assessed by examining situational and dispositional components of anxiety as related to these disorders and by behavioral measures of performance in relevant anxiety-provoking situations. Analyses of variance revealed that more pervasive anxiety reductions occurred for the more focalized animal phobia and that there was little difference in the effectiveness of desensitization and relaxation training. The generalizability of research findings based on the treatment of animal phobias was questioned, and the possible role of nonspecific factors in determining success was considered.     

Prevalence and genetic and environmental influences on anxiety disorders in 6-year-old twins

BACKGROUND: Prevalence of childhood anxiety disorders at specific ages and genetic etiological influences on anxiety disorders in young children have been little studied. The present study reports prevalence estimates in a community sample of 6-year-old twins, and patterns of genetic and environmental influences on these early-onset anxiety disorders. METHOD: Using a two-phase design 4,662 twin-pairs were sampled and 854 pairs were assessed in the second phase by maternal-informant diagnostic interview using DSM-IV criteria. RESULTS: The most common conditions were separation anxiety disorder (SAD) [2.8%, 95% confidence interval (CI) 2.1-3.8, for current disorder] and specific phobia (10.8%, 95% CI 8.4-13.6, for current disorder). Behavioral genetic modeling was feasible for these two conditions, applied to two phenotypes: symptom syndrome (regardless of impairment) and the narrower one of diagnostic status (symptom syndrome with associated impairment). The heritability estimate for SAD diagnostic status was high, 73%, with remaining variance attributed to non-shared environment. The heritability estimates for specific phobia were also high, 80% for the symptom syndrome and 60% for diagnostic status, with remaining variance attributed in both cases to non-shared environment. CONCLUSIONS: Compared with previous epidemiological surveys of children and adolescents in wide age-bands, the current estimates suggest that rates of anxiety disorders assessed in young childhood are generally at least as high and perhaps higher compared with those found in older children. The heritability estimates suggest that the genetic effects on these early-onset anxiety disorders are substantial and more significant than environmental effects, whether shared or non-shared.     

The burden of chronic disease,multimorbidity, and polypharmacy in adults with Down syndrome

Data on clinical characteristics of adults with Down syndrome (DS) are limited and the clinical phenotype of these persons is poorly described. This study aimed to describe the occurrence of chronic diseases and pattern of medication use in a population of adults with DS. Participants were 421 community dwelling adults with DS, aged 18 years or older. Individuals were assessed through a standardized clinical protocol. Multimorbidity was defined as the occurrence of two or more chronic conditions and polypharmacy as the concomitant use of five or more medications. The mean age of study participants was 38.3 ± 12.8 years and 214 (51%) were women. Three hundred and seventy‐four participants (88.8%) presented with multimorbidity. The most prevalent condition was visual impairment (72.9%), followed by thyroid disease (50.1%) and hearing impairment (26.8%). Chronic diseases were more prevalent among participants aged >40 years. The mean number of medications used was 2.09 and polypharmacy was observed in 10.5% of the study sample. Psychotropic medications were used by a mean of 0.7 individuals of the total sample. The high prevalence of multimorbidity and the common use of multiple medications contributes to a high level of clinical complexity, which appears to be similar to the degree of complexity of the older non‐trisomic population. A comprehensive and holistic approach, commonly adopted in geriatric medicine, may provide the most appropriate care to persons with DS as they grow into adulthood.     

Sleep-Related Problems and the Effects of Anxiety Treatment in Children and Adolescents

This study examined (a) demographic and clinical characteristics associated with sleep-related problems (SRPs) among youth with anxiety disorders, and (b) the impact of anxiety treatment: cognitive-behavioral therapy (CBT; Coping Cat), medication (sertraline), their combination, and pill placebo on SRPs. Youth (N?=?488, ages 7–17, 50% female, 79% White) with a principal diagnosis of generalized anxiety disorder, separation anxiety disorder, or social phobia participated. SRPs were reported by parents and youth. Findings differed by informant and by type of SRP, with evidence that SRPs are associated with age, anxiety severity, externalizing problems, functional impairment, and family burden at pretreatment. Anxiety treatment reduced SRPs; effect sizes were small to medium. Reductions in parent-reported separation-related sleep difficulties were significantly greater in active treatment than in the placebo condition, with the greatest reductions reported by parents of youth whose active treatment was multimodal or included sertraline. Youth whose anxiety treatment involved CBT reported significantly greater decreases in dysregulated sleep (e.g., sleeplessness). Both CBT for anxiety and sertraline appear to be somewhat effective in reducing SRPs, and multimodal treatment may be preferable depending on the symptom presentation. To inform practice, future research should examine a broad range of SRPs, incorporate objective measures of sleep, and evaluate the impact of behavioral strategies that directly target SRPs in youth with anxiety disorders.     

When distraction fails: parental anxiety and children's responses to distraction during cancer procedures

OBJECTIVE: To examine parental anxiety in the context of successful and unsuccessful distraction treatment of preschool aged children undergoing chemotherapy procedures. METHODS: Twenty-nine children (M age = 42 months) experiencing intramuscular or portacatheter injections participated in the study. Parents and children were shown how to use a portable electronic toy as a distractor during chemotherapy injections. Parental anxiety was assessed at baseline and child distress was coded during each procedure. RESULTS: Parents' baseline state anxiety accounted for 17% of the variance in changes in children's distress following distraction intervention. Parents of children who did not benefit from distraction reported significantly higher state anxiety at baseline than parents of the other participants. CONCLUSIONS: Results highlight the importance of examining individual outcomes in intervention studies and suggest that parents' emotional states may moderate distraction treatment outcome in young children. Future research formally testing parent anxiety as a moderator is recommended.     

Parent Expectancies and Preferences for Mental Health Treatment: The Roles of Emotion Mind-Sets and Views of Failure

Because parents are primary gatekeepers to mental health care for their children, parental expectations that mental health treatment is ineffective may undermine treatment seeking, retention, and response. Thus, a need exists to understand parents’ expectations about treatment and to develop scalable interventions that can instill more favorable views. We examined parents’ treatment expectancies and preferences for their offspring and themselves in relation to two global beliefs: mind-sets (malleability beliefs) of emotions and anxiety, and views of failure as enhancing versus debilitating. Study 1 (N = 200; 49.5% fathers; 70.4% Caucasian) examined associations among parents’ emotion mind-sets, anxiety mind-sets, failure beliefs, and treatment expectancies and preferences. Study 2 (N = 430; 44.70% fathers; 75.80% Caucasian) tested whether online inductions teaching “growth emotion mind-sets” (viewing emotions as malleable), adaptive failure beliefs, or both improved parents’ treatment expectancies and hypothetical preferences for treatment (vs. no-treatment). Participants received one of three 8- to 15-min inductions or a psychoeducation control, rating treatment expectancies. and preferences pre- and postinduction. In Study 1, fixed emotion mind-sets and failure-is-debilitating beliefs were associated with lower parent psychotherapy expectancies for offspring and themselves and stronger “no-treatment” preferences for offspring. In Study 2, inductions teaching (a) growth emotion mind-sets only and (b) growth emotion mind-sets and failure-is-enhancing beliefs improved parents’ psychotherapy expectancies for themselves (ds = .38, .51) and offspring (ds = .30, .43). No induction increased parents’ hypothetical preferences for treatment (vs. no-treatment). Findings suggest scalable strategies for strengthening parents’ psychotherapy effectiveness beliefs for themselves and their children.     

Anxiety in Wiedemann–Steiner syndrome

This study examined anxiety in Wiedemann–Steiner syndrome (WSS). Eighteen caregivers and participants with WSS completed the parent- and self-report versions of the Screen for Child Anxiety Related Disorder or the adapted version of the Screen for Adult Anxiety Related Disorder. Approximately 33.33% of parents and 65% of participants with WSS rated in the clinical range for overall anxiety. Across anxiety subtypes, parents primarily indicated concerns with Separation Anxiety (72%), which was also endorsed by the majority of participants with WSS (82%). The emergent trend showed Total Anxiety increased with age based on parent-informant ratings. The behavioral phenotype of WSS includes elevated anxiety. Clinical management should include incorporating early behavioral interventions to bolster emotion regulation given the observed risk of anxiety with age.     

Autosomal dominant supravalvular aortic stenosis: large three-generation family

Supravalvular aortic stenosis (SVAS) can be inherited as an isolated autosomal dominant trait or can be a component manifestation of the Williams syndrome. Some consider the Williams syndrome to be due to more severe expression of the gene defect that causes isolated SVAS. We describe a family with isolated SVAS that is the largest thoroughly studied family with this disorder to our knowledge; no patients in this family had Williams syndrome. Five members of this family were reported by Lewis et al. (Dis Chest 55:372-379, 1969). We reevaluated this family and now include examinations of the parents, additional sibs and children of the original 5 patients. Twenty relatives had physical and echocardiographic examinations. In addition, information from outside sources was obtained on 7 relatives not personally evaluated. The SVAS showed marked variability of expression and was not associated with mental retardation or with the facial manifestations of Williams syndrome. We think that previous reports of Williams syndrome reputedly occurring within the same family as isolated autosomal dominant SVAS were inadequately documented. Based on our family and review of the literature, we suggest that isolated SVAS and Williams syndrome represent clinically distinct entities.     

Social phobia in the Australian National Survey of Mental Health and Well-Being (NSMHWB)

BACKGROUND: This article reports data on social phobia from the first large scale Australian epidemiological study. Prevalence rates, demographic correlates and co-morbidity in the sample that met criteria for social phobia are reported and gender differences examined. METHOD: Data were obtained from a stratified sample of 10641 participants as part of the Australian National Survey of Mental Health and Well-Being (NSMHWB). A modified version of the Composite International Diagnostic Interview (CIDI) was used to determine the presence of social phobia, as well as other DSM-IV anxiety, affective and substance use disorders. The interview also screened for the presence of nine ICD-10 personality disorders, including anxious personality disorder, the equivalent of DSM-IV avoidant personality disorder (APD). RESULTS: The estimated 12 month prevalence of social phobia was 2.3%, lower than rates reported in several recent nationally representative epidemiological surveys and closer to those reported in the Epidemiological Catchment Area study (ECA) and other DSM-III studies. Considerable co-morbidity was identified. Data indicated that the co-morbidity with depression and alcohol abuse and dependence were generally subsequent to onset of social phobia and that the additional diagnosis of APD was associated with a greater burden of affective disorder. Social phobia most often preceded major depression, alcohol abuse and generalized anxiety disorder. CONCLUSIONS: Social phobia is a highly prevalent, highly co-morbid disorder in the Australian community. Individuals with social phobia who also screen positively for APD appear to be at greater risk of co-morbidity with all surveyed disorders except alcohol abuse or dependence.     

Working alliance in online cognitive behavior therapy for anxiety disorders in youth: comparison with clinic delivery and its role in predicting outcome

Background

Substantial evidence exists that positive therapy outcomes are related to the therapist–client working alliance.

Objectives

To report two studies that examined (1) the quality of the working alliance in online cognitive behavior therapy (CBT), with minimal therapist contact, for anxiety disorders in youth, and (2) the role of working alliance and compliance in predicting treatment outcome.

Methods

Study 1 participants were 73 adolescents aged 12 to 18 years who met diagnostic criteria for an anxiety disorder, plus one or more of their parents. Participants were randomly assigned to clinic or online delivery of CBT, with working alliance being assessed for youth and parents after session 3. Study 2 participants were 132 children and adolescents aged 7 to 18 years who met diagnostic criteria for an anxiety disorder, plus one or more of their parents. Youths and parents participated in a minimally therapist-assisted online CBT program supported by brief, weekly emails and a single, short phone call.

Results

Study 1 revealed a strong working alliance for both online and clinic CBT, with no significant difference in working alliance between conditions for adolescents (F _1,73 = 0.44, P = .51, η_p ² = 0.006, Cohen d = 0.15). Parents also reported high working alliance in both conditions, although a slight but significantly higher working alliance in clinic-based therapy (F _1,70 = 6.76, P = .01, η_p ² = 0.09, Cohen d = 0.64). Study 2 showed a significant and substantial decrease in anxiety symptoms following online therapy (P < .001 for all outcome measures). Adolescents improved significantly more in overall functioning when working alliance (beta = .22, t ₇₉ = 2.21, P = .03) and therapy compliance (beta = .22, t ₈₄ = 2.22, P = .03) were higher, with working alliance also predicting compliance (beta = .38, F _1,80 = 13.10, P = .01). No such relationships were evident among younger children.

Conclusions

Working alliance is important in determining clinical outcome for online treatment for anxiety among adolescents, with minimal therapist assistance, although this was not the case for younger children.

Trial Registration

Australian New Zealand Clinical Trials Registry: ACTRN12611000900910; http://www.anzctr.org.au/trial_view.aspx?ID=343375 (Archived by WebCite at http://www.webcitation.org/674C4N3JJ)     

Parent perceptions,beliefs, and fears around genetic treatments and cures for children with Angelman syndrome

Genetic therapies have shown recent promise in alleviating some of the cognitive issues associated with some genetic disorders; however, these therapies may come with significant health and socio‐ethical concerns, particularly when they involve child participants. Little is known about what parents of children with genetic disorders think about genetic therapies, or about their knowledge of how genetic‐based therapy might treat their child's symptoms. Forty‐two parents of children with Angelman syndrome (AS) and 27 parents of a mixed etiology comparison group completed an online survey reporting on their perceptions of, and priorities for, genetic therapy. Almost all parents of children with AS (95%) and the comparison group (89%) agreed that treatments aiming to reduce symptoms associated with their child's syndrome were positive. However, significantly more parents of children with AS (95%) than the comparison group (56%) felt that genetic treatment trials aiming to “cure” their child should be a research priority. AS parent priorities for the focus of clinical trials were neurology/seizures, communication skills, and motor skills/mobility. For the comparison group, the priorities were IQ, immune response, and expressive speech. Parents of both groups did not want treatments to change their child's personality or their happiness. Global assumptions cannot be made about targets for therapy between syndromes, about parental understanding of genetics, or about research evidence across syndromes. This study highlights the need for true family and patient engagement in all stages of the research design and treatment evaluation.     

Anxiety Reporting and Culturally Associated Interpretation Biases and Cognitive Schemas: A Comparison of Mexican,Mexican American,and European American Families

This study examined whether Mexican (n = 53), Mexican American (n = 50), and European American (n = 51) children differed in their reporting of anxiety symptoms and whether parental influence and specific cognitive schemas associated with Mexican culture were related to differences in anxiety reporting. As expected, Mexican and Mexican American children reported significantly more physiological and worry symptoms than the European American children. Mexican and Mexican American children endorsed collectivism as a cultural value more strongly than European American children, and the Mexican children evidenced greatest use of social strategies reflecting simpatia. In family discussions of ambiguous, potentially anxiety-arousing situations, Mexican and Mexican American parents verbalized a greater percentage of somatic interpretations than the European American parents. Results indicate potential linkages between cultural values, socialization practices, and anxiety reporting.     

Anxiety reporting and culturally associated interpretation biases and cognitive schemas: a comparison of Mexican, Mexican American, and European American families.

This study examined whether Mexican (n = 53), Mexican American (n = 50), and European American (n = 51) children differed in their reporting of anxiety symptoms and whether parental influence and specific cognitive schemas associated with Mexican culture were related to differences in anxiety reporting. As expected, Mexican and Mexican American children reported significantly more physiological and worry symptoms than the European American children. Mexican and Mexican American children endorsed collectivism as a cultural value more strongly than European American children, and the Mexican children evidenced greatest use of social strategies reflecting simpatia. In family discussions of ambiguous, potentially anxiety-arousing situations, Mexican and Mexican American parents verbalized a greater percentage of somatic interpretations than the European American parents. Results indicate potential linkages between cultural values, socialization practices, and anxiety reporting.