Health related quality of life in parents of six to eight year old children with Down syndrome

Raising a child with Down syndrome (DS) has been found to be associated with lowered health related quality of life (HRQoL) in the domains cognitive functioning, social functioning, daily activities and vitality. We aimed to explore which socio-demographics, child functioning and psychosocial variables were related to these HRQoL domains in parents of children with DS. Parents of 98 children with DS completed the TNO-AZL adult quality of life questionnaire (TAAQOL) and a questionnaire assessing socio-demographic, child functioning and psychosocial predictors. Using multiple linear regression analyses for each category of predictors, we selected relevant predictors for the final models. The final multiple linear regression models revealed that cognitive functioning was best predicted by the sleep of the child (β = .29, p < .01) and by the parent having given up a hobby (β = ?.29, p < .01), social functioning by the quality of the partner relation (β = .34, p < .001), daily activities by the parent having to care for an ill friend or family member (β = ?.31, p < .01), and vitality by the parent having enough personal time (β = .32, p < .01). Overall, psychosocial variables rather than socio-demographics or child functioning showed most consistent and powerful relations to the HRQoL domains of cognitive functioning, social functioning, daily activities and vitality. These psychosocial variables mainly related to social support and time pressure. Systematic screening of parents to detect problems timely, and interventions targeting the supportive network and the demands in time are recommended.     

Numeracy for adults with Down syndrome: it's a matter of quality of life

Background Numeracy development is widely accepted as critical for adults in the general community which is equally the case for adults with Down syndrome. This paper reports some of the findings of a study including the research question: What is the justification for numeracy development for adults with Down syndrome? Investigating this question led to the search for a framework to support the ongoing development of numeracy. Method The research used a case study methodology. Five adults were observed and interviewed in two contexts each. The data were analyzed to identify links to the quality of life model. Results The research illustrates how a quality of life approach can be used to justify and guide the lifelong development of numeracy. Data from the case studies linked numeracy to quality of life under the principles of personal contexts, variability, life-span perspective, values, choices and personal control, perceptions and self-image. Conclusions The principles of quality of life can provide a framework for the development of numeracy in the context of adulthood and Down syndrome. Preparation for the numeracy needs of a long and satisfying adulthood should begin in early childhood, continue in schools with the teaching of underlying mathematics concepts and skills, and be modified and refined throughout adulthood by the use of a numeracy development plan. Carers and professionals interacting in the contexts need to adopt a teaching role for numeracy.     

Examining the Down syndrome advantage: mothers and fathers of young children with disabilities

Background Across studies and across outcomes, parents of children with Down syndrome have been found to experience greater well‐being than parents of children with other intellectual disabilities (ID). This study examined the extent to which differences in family income and child temperamental difficulty can explain why parents of children with Down syndrome experience greater well‐being. Method Fifty married couples who were parents of young children with ID completed a set of questionnaires measuring child temperament, parent satisfaction with social support, depression and parenting beliefs. Parents were observed interacting with their children. Results When groups were compared without consideration of demographic differences, the findings generally replicated the Down syndrome advantage found by other researchers. After variance attributable to income was removed, however, the Down syndrome advantage disappeared for all parenting and parent wellness measures. The effects of child temperament were confined to maternal and paternal depression. Conclusions Family income was more important than child etiology in predicting the parent measures used in the study.     

Self-reported and parent-reported quality of life of children and adolescents with new-onset epilepsy

Purpose: To investigate the self‐reported quality of life (QOL) of children and adolescents with new‐onset epilepsy and explore parent’s perceptions of their child’s QOL and the impact of epilepsy on the family. Methods: As part of the Standard and New Antiepileptic Drug (SANAD) trial, 248 children (aged 8–15 years) with new‐onset epilepsy and their parents completed batteries of QOL measures. Children completed the KINDL and subscales of the QOLIE‐AD‐48. Parental questionnaires included the Rutter Parent Scales, Adverse Events Profile, and subscales of the Child Health Questionnaire. Key Findings: Compared with published norms, children with epilepsy had significantly poorer QOL across multiple domains compared with healthy children and children with asthma. Parents, particularly of younger children and those with other long‐term health problems, were affected by their child’s health or behavior, which affected their emotional well‐being and time for their own needs. Significance: Children with new‐onset epilepsy, particularly those with comorbid conditions, are at risk of reduced QOL at the time of diagnosis. It is important that children with epilepsy and their families are assessed for psychosocial problems so that early intervention can be undertaken.     

Impact of family functioning on quality of life in patients with psychogenic nonepileptic seizures versus epilepsy

Purpose: To evaluate different contributions of aspects of family functioning (FF) on health‐related quality of life (HRQOL) in patients with psychogenic nonepileptic seizures (PNES) versus epileptic seizures (ES). Methods: Forty‐five participants with PNES and 32 with ES completed self‐report measures of FF (Family Assessment Device; FAD), HRQOL (Quality of Life in Epilepsy‐31), and depression (Beck Depression Inventory‐II; BDI‐II). The FAD is a self‐report questionnaire that assesses FF along six dimensions and general functioning. Regression analyses were used to evaluate the contribution of FF to HRQOL above and beyond the effects of disease severity and depression. Key Findings: Mean Family General Functioning fell in the unhealthy range in participants with ES or PNES. On further analysis, male participants in each group endorsed unhealthy levels of FF compared to female participants. Patients with PNES reported poorer HRQOL and greater depressive symptoms compared to ES participants; there were no gender differences in HRQOL. Regression analyses indicated that the FAD Roles subscale predicted reduced HRQOL in patients with PNES after controlling for illness duration, seizure frequency, and depression. After controlling for the same factors, Communication and Affective Involvement subscales scores predicted HRQOL in ES participants. Significance: Family dysfunction was reported in both ES and PNES participants, but greater family dysfunction was experienced by male participants in both groups. Aspects of FF predicted HRQOL in patients with PNES and ES differentially. FF may be an important treatment target to enhance coping in these groups, although the treatments may need to target different aspects of FF in PNES versus ES.     

Psychiatric disorders in adolescents and young adults with Down syndrome and other intellectual disabilities

Background

Relative to other aspects of Down syndrome, remarkably little is known about the psychiatric problems experienced by youth and young adults with this syndrome and if these problems differ from others with intellectual disabilities. Yet adolescence and young adulthood are particularly vulnerable time periods, as they involve multiple life transitions in educational, medical, and other service systems.

Methods

This study compared the psychiatric diagnoses of 49 adolescent and young adult patients with Down syndrome to 70 patients with other intellectual disabilities (IDs). The groups were similar in age, gender, and level of intellectual impairment. The 119 participants, aged 13 to 29 years (M = 21) were evaluated in one of two specialized psychiatric clinics.

Results

In contrast to previous literature, those with Down syndrome versus other IDs had significantly higher rates of psychosis NOS or depression with psychotic features (43% versus 13%). Unlike the ID group, psychosis was predominantly seen in females with Down syndrome. Marked motoric slowing in performing routine daily activities or in expressive language was manifested in 17% of patients with Down syndrome. No group differences were found in anxiety or depressive disorders, and the ID group had significantly higher rates of bipolar and impulse control disorders.

Conclusions

These preliminary observations warrant further studies on genetic, neurological, and psychosocial factors that place some young people with Down syndrome or other IDs at high risk for severe psychiatric illness.     

Adjustment of children who have a sibling with Down syndrome: perspectives of mothers, fathers and children

Background A number of methodological weaknesses have contributed to our relatively poor understanding of the impact on children of having a brother or sister with a disability. These include a focus on poor adjustment, using multidiagnostic groups, inadequate matching, and a failure to consider the perspectives of children and parents together. Method This study compared the adjustment of 53 siblings of a child with Down syndrome with a comparison group of siblings of children who were developing typically. Children were matched on a case‐by‐case basis for gender, age and position in family. Families were matched for family size and father’s occupation. The age range of the target siblings was 7–14 years. Data were gathered from mothers, fathers and siblings. Results There were no significant differences between the groups on adjustment measures. These included parent perceptions of externalizing and internalizing behaviours, parent perceptions of sibling competence, and sibling perceptions of their own competence and self‐worth. Associations between measures of adjustment and child reports of their contribution to household functioning depended on sex rather than group membership. There was an association between parental reports of externalizing behaviour and sibling relationships with the brother/sister closest in age. Conclusions Having a brother or sister with Down syndrome does not inevitably lead to poor adjustment. Examination of within‐family processes would appear to be more useful in identifying children at risk than merely group membership.     

Patterns of participation and enjoyment in adolescents with Down syndrome

This study aimed to determine participation and enjoyment in young people with Down syndrome (DS) in Taiwan and to assess how participation varies across gender, cognitive, and motor function variables. Using the Children's Assessment of Participation and Enjoyment, data on participation were collected from 997 adolescents with DS and their families. Findings indicated limited diversity and intensity of participation, with proportionately greater involvement in informal activities. Youth with better cognitive and motor functions participated more often in activities and reported higher enjoyment and social engagement with these. These findings provide a foundation for an improved understanding of activity participation of youth with DS. Service providers and families could provide and plan activities to ensure more satisfying and meaningful participation.     

For fathers raising children with autism,do coping strategies mediate or moderate the relationship between parenting stress and quality of life?

In response to the dramatic change in the perception of fatherhood and the significant expansion in fatherhood research, this study came to fill the gap in literature and examine the possible mediation and moderation effects of coping in the relationship between fathers’ of children with autism parenting stress and quality of life (QoL). Mediation and moderation effects were examined using multiple programs and software which included hierarchical regression, structural equation modeling and special Macros added to the analysis programs to confirm the findings. None of the investigated coping strategies could mediate or moderate the stress–QoL relationship among the 101 participating fathers. This study provides interesting information on how the stress-coping-QoL relationship among fathers of children with autism can be affected by the nature of their stress provoking situation, their individual characteristics, the environment and its demands and resources, and the way fathers perceive and apply their coping responses.     

Health co-morbidities in ageing persons with Down syndrome and Alzheimer's dementia

Background Consideration of the relationship between physical and mental health co-morbidities in ageing persons with Down syndrome (DS) and Alzheimer's dementia (AD) is of clinical importance both from a care and resource perspective. Aim To investigate and measure health co-morbidities in ageing persons with Down syndrome with and without AD. Methods Recorded physical and mental health needs were ascertained for 124 persons with DS > 35 years through a systematic and detailed search of individual medical and nursing case records. Differences in persons with and without AD were investigated, by stage of dementia and by level of intellectual disability (ID). A summed score for health co-morbidities was created and compared using t-tests. Results Persons with AD had significantly higher co-morbidity scores than persons without AD (t = −8.992, d.f. = 121, P < 0.0001). There was also a significant difference in summed co-morbidity scores for persons at end-stage vs. persons at mid-stage AD (t = −6.429, d.f. = 56, P < 0.0001). No differences were found by level of ID. Conclusions Increasing health co-morbidities in persons with DS and AD have important implications for care and resources. Appropriate environmental supports combined with competent skilled staff are crucial and will have an important impact on the quality of life for this increasingly at risk population.     

Prevalence and quality of life and sleep in children and adolescents with restless legs syndrome/Willis-Ekbom disease

ObjectiveTo estimate the prevalence of restless legs syndrome/Willis-Ekbom disease (RLS/WED) and its impact on sleep and quality of life in children and adolescents.MethodsThis was a cross-sectional study conducted in the Municipality of Cássia dos Coqueiros, Brazil. Participants included 383 children and adolescents 5–17 years of age. A comparison group was randomly matched by gender and age with the RLS/WED-affected individuals, pairing one by one.ResultsInterviews were conducted for 383 individuals by a neurologist experienced in sleep medicine. RLS/WED was diagnosed using the essential clinical criteria for definitive RLS/WED in children recommended by the International Restless Legs Syndrome Study Group. Sleep and quality of life were evaluated using the Sleep Behavior Questionnaire (SBQ) and the Health-related Quality of Life Questionnaire─Pediatric Quality of Life Inventory (PedsQL). Comparisons were established with a group of randomly selected individuals without RLS/WED, matched by age and gender (control group). The prevalence of RLS/WED symptoms that manifested at least twice a week was 1.9%. The average age of children with RLS/WED was higher compared to the general population (11.5 ± 2.3 vs 9.9 ± 2.5, p < 0.005). A family history of RLS/WED was detected in 90.9% of the patients. The scores obtained by SBQ were higher (53.9 ± 9.4 vs 47.6 ± 10.9, p < 0.047), whereas the scores achieved by PedsQL were lower (69.8 ± 14.8 vs 81.9 ± 10.4, p < 0.003) in children with RLS/WED compared to controls.ConclusionThe prevalence of RLS/WED symptoms manifested at least twice in the preceding week was 1.9% in children and adolescents. Worsened sleep and quality of life were observed in the study.     

Primary caregivers’ awareness and perception of early-onset dementia conditions in adolescents and young and middle-aged adults with Down syndrome

The present study aims to investigate the onset of dementia conditions using the Dementia Screening Questionnaire for Individuals with Intellectual Disabilities (DSQIID) scale and to identify the possible factors associated with DSQIID scores in people with Down syndrome (DS). The study population was recruited from the voluntary registry members of the Republic of China Foundation for Persons with Down syndrome; primary caregivers provided DSQIID information on 196 adolescents and adults with DS (aged 15–48 years) who were entered into the database and analyzed using SPSS 20.0 software. The results described the distribution of early-onset dementia conditions in 53 adolescents and adults with DS, and 2.6% of the subjects with DS had possible dementia (DSQIID score ≧ 20). Univariate analyses found that older age (p = 0.001) and comorbid conditions (p = 0.003) were significantly associated with DSQIID scores. Older subjects were more likely to have higher DSQIID scores than were younger age groups after ANOVA and Scheffe's tests. Lastly, a multiple linear regression analysis revealed that age (p < 0.01), severe disability level (p < 0.05) and comorbid condition (p < 0.01) significantly explained 13% of the variation in DSQIID scores after adjusting for the factors of gender, education level and multiple disabilities in adolescents and adults with DS. The study highlights that future research should focus on the occurrence of dementia in people with DS and on identifying its influencing factors based on sound measurements, to initiate appropriate healthy aging policies for this group of people.     

早期帕金森病患者健康相关生活质量

目的 研究中国早期帕金森病(PD)患者健康相关生活质量(health related quality of life,HR-QOL)的特点;探讨运动症状和非运动症状对早期PD患者HR-QOL的影响.方法 在全国范围内共筛选出391例早期PD患者入组.采用统一帕金森病评分表(UPDRS)和Hoehn-Yahr评价运动症状,采用流行病学研究中心编制的抑郁量表(CES-D)、匹兹堡睡眠质量指数(PSQI)、疲劳量表(FSS)、阿尔茨海默病评定量表的认知部分(ADAS-Cog)和便秘量表分别对抑郁、睡眠障碍、疲劳、认知功能和便秘等非运动症状进行评价;采用36条目简化医疗结局调查问卷(SF-36)评价HR-QOL.比较PD患者与同龄健康老年人SF-36分值的差异.采用逐步多元线性回归分析深入探讨各种运动及非运动症状变量对HR-QOL的影响.结果 早期PD患者除SF-36躯体疼痛维度外,其余各维度分值较同龄健康老年人均下降.UPDRS第3部分分值(23.8±11.8)、Hoehn-Yahr分期(2.0±0.7)和强直分值(4.4±3.1)仅能解释SF-36总分变化的18.9%(R2=0.189).CES-D、FSS和PSQI分值等非运动症状变量引入回归方程后,SF-36总分可被解释的部分由18.9%增加至61.7%(R2=0.617).并且,引入CES-D分值后,SF-36总分可被解释的部分增加了43.3%(R2=0.433).结论 PD症状严重影响早期患者的HR-QOL.运动症状对HR-QOL存在影响,但影响作用有限.抑郁、疲劳和睡眠障碍这3个非运动症状是导致早期PD患者HR-QOL恶化的主要原因.其中,抑郁症状是HR-QOL恶化的最强预测因素.临床上,应重视非运动症状,运动和非运动症状兼治,才能真正提高疗效显著改善患者的HR-QOL.