scholarly journals Examination of Correlates to Health-Related Quality of Life in Individuals with Fragile X Syndrome

2020 ◽  
Vol 10 (4) ◽  
pp. 213
Author(s):  
Marika C. Coffman ◽  
Rebecca C. Shaffer ◽  
Lauren M. Schmitt ◽  
Kelli C. Dominick ◽  
Ernest Pedapati ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Fragile X Syndrome ◽  
Fragile X ◽  
Parent Report ◽  
Health Related Quality ◽  
Related Quality ◽  
Health Related ◽  
Pedsql 4.0 ◽  
Degree Of Association

Health-related quality of life (HRQoL) is a multidimensional concept involving physical, psychological, social, and cognitive aspects of life. Individuals with Fragile X syndrome (FXS) experience a life-long disorder that impacts the HRQoL of the affected individual and their family. Thus, HRQoL may be an important outcome measure following intervention. However, it is yet not known whether HRQoL concerns relate to observed impairments in FXS. In the present study, we examined the nature and degree of association between HRQoL and established measures of functioning in FXS using the Parent Report for Children version of the PedsQL 4.0 Generic Core Scales and Cognitive Functioning Scale. We observed significant relationships between HRQoL a nd measures of adaptive behavior, maladaptive behaviors, and social functioning. The present study has implications for treatment outcomes for clinical trials in FXS.

Social/economic costs and health-related quality of life in patients with fragile X syndrome in Europe

2016 ◽  
Vol 17 (S1) ◽  
pp. 43-52 ◽  
Author(s):  
Karine Chevreul ◽  
◽  
Coralie Gandré ◽  
Karen Berg Brigham ◽  
Julio López-Bastida ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Fragile X Syndrome ◽  
Social Economic ◽  
Fragile X ◽  
Health Related Quality ◽  
Economic Costs ◽  
Related Quality ◽  
Health Related

Health-related quality of life in Chinese boys with Duchenne muscular dystrophy and their families

2019 ◽  
Vol 23 (3) ◽  
pp. 495-506 ◽  
Author(s):  
Rui Liang ◽  
Sophelia Hoi Shan Chan ◽  
Frederick Ka Wing Ho ◽  
Oi Ching Tang ◽  
Sharon Wan Wah Cherk ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Duchenne Muscular Dystrophy ◽  
Parent Report ◽  
Self Report ◽  
Disease Stage ◽  
Health Related Quality ◽  
Related Quality ◽  
Health Related ◽  
Pedsql 4.0

This study aims to assess the family functioning and health-related quality of life (HRQOL) in Chinese boys with Duchenne muscular dystrophy (DMD) and their parents using Pediatric Quality-of-Life Family Impact Module ( PedsQL FIM) and Pediatric Quality-of-Life Inventory (PedsQL) 4.0. Findings from 15 families with DMD were compared with 15 unaffected families. The HRQOL, as measured by the mean PedsQL 4.0 Generic Core Scale scores for the boys with DMD were significantly lower than those of age-matched healthy boys, for overall ( p < 0.05, parent-report; p <0.001, self-report), physical ( p < 0.001, parent-report and self-report), and social ( p < 0.05, parent-report) functioning, but the emotional functioning is not affected. The parent–child concordance of our affected DMD families was generally in the moderate-to-good agreement range (intraclass correlation coefficients from 0.51 to 0.73), except for emotional (0.28) and social (0.31) functioning. The PedsQL FIM total score showed an inverse relationship with the affected child’s age (correlation coefficient: −0.55; p < 0.01) and the disease stage (correlation coefficient: −0.63; p < 0.01) confirming that parental HRQOL and overall family functioning worsened as the child increased in age with advancing disease stage.

Generic Health-Related Quality of Life after Repair of Esophageal Atresia and Its Determinants within a German–Swedish Cohort

2018 ◽  
Vol 29 (01) ◽  
pp. 075-084 ◽  
Author(s):  
Sofie Flieder ◽  
Michaela Dellenmark-Blom ◽  
Stefanie Witt ◽  
Carmen Dingemann ◽  
Julia Quitmann ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Esophageal Atresia ◽  
Healthy Population ◽  
Health Related Quality ◽  
German Sample ◽  
Generic Hrqol ◽  
Related Quality ◽  
Health Related ◽  
Pedsql 4.0

Aim Despite advances of outcomes of esophageal atresia (EA), knowledge on patients' health-related quality of life (HRQoL) is sparse. Due to the heterogeneity of EA, larger cohorts need to be investigated to ensure reliability of data. Aim was to determine generic HRQoL after EA repair in a Swedish–German cohort. Patients and Methods Ethical approval was obtained. A total of 192 patients (2–18 years; 134 Swedish; 58 German) were included. Clinical data were reviewed. EA was classified in “severe” and “mild/moderate.” Pediatric Quality of Life Inventory 4.0 Generic Core Scale (PedsQL 4.0) was used in appropriate versions (2–7 years; 8–18 years; self- [SR] and proxy report [PR]) to determine generic HRQoL. Results Swedish and German samples were clinically and demographically comparable. HRQoL was lower in “severe EA” versus “mild/moderate” (2–18 years; total score; PR 85.6 vs. 73.6; p < 0.001) and Gross A versus Gross C type EA (2–7 years; total score; PR 61.0 vs. 79.3; p = 0.035). Total HRQoL was higher in the Swedish versus German sample (2–18 years; total score; PR 82.3 vs. 72.7; p = 0.002). HRQoL was impaired in the German sample versus healthy population (2–18 years; total score; PR 72.7 vs. 82.7; p = 0.001). In German patients (8–18 years), HRQoL was higher in SR versus PR (80.7 vs. 74.7; p = 0.044). Patients' age and presence of VACTERL association or isolated anorectal malformations did not affect HRQoL. Various differences were detected regarding different dimensions of PedsQL 4.0. Conclusion In this first international study, we found several differences in perception of generic HRQoL. HRQoL appears to be determined by the type of EA and severity rather than patients' age or the presence of typical associated malformations. Country-specific differences may be culturally dependent, but further investigations are suggested. A condition-specific instrument validated for EA may provide additional insights.

scholarly journals Assessment of Quality of life Among Children with Cancer, Children Undergoing Hemodialysis and Children with Thalassemia: A Comparative Study

2020 ◽  
Author(s):  
Tayseer Afifi ◽  
Khamis Elessi ◽  
Obay Baraka ◽  
Mohammed Omar ◽  
Israa Ahmad ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Chronic Conditions ◽  
Poor Quality ◽  
Health Related Quality ◽  
Children With Cancer ◽  
Related Quality ◽  
Health Related ◽  
Pedsql 4.0 ◽  
Assess Quality

Abstract Purpose This study aims to evaluate health-related quality of life among children with different chronic conditions and make a comparison among them. In addition, the study will draw a comparison between children’s perspective about their quality of life and parents’ perspective. Furthermore, the study will address the associations between quality of life with disease severity, duration and the presence of other co-morbidities. Methods This was a prospective, analytical, correlational study design in which Pediatric Quality of Life Inventory (PedsQL 4.0 generic core scale) was administered to assess quality of life on 110 children; 50 children with cancer, 30 children with thalassemia and 30 children undergoing hemodialysis. Researchers interviewed children as well as their parents. Results Results showed that children with Thalassemia had the lowest scores and suffered from poor quality of life compared to the other two groups of children. Interestingly, quality of life level among children undergoing dialysis was lower than children with cancer. However, there were marked discrepancy between child’s and parents’ answers on scale questions. The level of quality of life, in all children, were statistically significant in relation with family size, income and parents’ education. Conclusion We identified high prevalence of poor level of health-related quality of life among children included in this study. The findings support that the quality of life among children with chronic conditions should receive more attention in our local medical settings.

Validity of the health-related quality of life assessment in the ADORE study: Parent Report Form of the CHIP-Child Edition

2006 ◽  
Vol 15 (S1) ◽  
pp. i63-i71 ◽  
Author(s):  
Anne W. Riley ◽  
David Coghill ◽  
Christopher B. Forrest ◽  
Maria J. Lorenzo ◽  
Stephen J. Ralston** ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Parent Report ◽  
Life Assessment ◽  
Health Related Quality ◽  
Quality Of Life Assessment ◽  
Related Quality ◽  
Health Related

scholarly journals A.01 The relationship between fatigue and health-related quality of life in a clinical trial population of Duchenne muscular dystrophy patients

2017 ◽  
Vol 44 (S2) ◽  
pp. S8-S8
Author(s):  
Y Wei ◽  
B El-Aloul ◽  
C Nguyen ◽  
E Zapata-Aldana ◽  
C Campbell
Keyword(s):  
Quality Of Life ◽  
Clinical Trial ◽  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
Parent Report ◽  
Health Related Quality ◽  
Patient Report ◽  
Related Quality ◽  
Health Related

Background: Fatigue was recently reported to be the largest contributor to poor health-related quality of life (HRQOL) in paediatric Duchenne muscular dystrophy (DMD). Additional studies are necessary to confirm the generalizability of this finding. Our objective was to explore the longitudinal relationship between fatigue and HRQOL in an additional cohort of DMD patients. Methods: We performed a secondary analysis of data from a clinical trial (NCT00592553), which enrolled patients with nonsense mutation DMD, aged 5–20 years, from 37 sites in 11 countries (N=174). Fatigue and HRQOL were assessed using the PedsQLTM Multidimensional Fatigue Scale and Generic Core Scales, respectively, by patient- and parent-report at baseline and over 48 weeks. Results: Patients reported greater fatigue than healthy controls from published data. There was no significant difference between patient- and parent-reported fatigue. Fatigue was significantly correlated with worse HRQOL at baseline, by patient-report (r=0.70, P<0.001) and parent-report (r=0.70, P<0.001); and at 48 weeks, by patient-report (r=0.79, P<0.001) and parent-report (r=0.74, P<0.001). Change in fatigue was significantly correlated with change in HRQOL over 48 weeks, by patient-report (r=0.64, P<0.001) and parent-report (r=0.67, P<0.001). Conclusions: Fatigue is a major contributor to HRQOL in DMD. The strong association between fatigue and HRQOL corroborates previous studies, and suggests that reducing fatigue may improve HRQOL.

Health-related Quality of Life of Children Treated for Non-syndromic Craniosynostosis

2021 ◽  
Author(s):  
Marizela Kljajic ◽  
Giovanni Maltese ◽  
Peter Tarnow ◽  
Peter Sand ◽  
Lars Kölby
Keyword(s):  
Quality Of Life ◽  
Health Related Quality ◽  
Syndromic Craniosynostosis ◽  
Proxy Reports ◽  
Surgical Methods ◽  
Patient Reported ◽  
Related Quality ◽  
Health Related ◽  
Pedsql 4.0

Abstract Background: Assessing health-related quality of life (HRQoL) allows acquisition of the subjective perspective of patients regarding their health and functioning; however, little is known about the experiences of patients living with treated craniosynostosis (CS). Methods: School-aged children (7–16 years) treated for non-syndromic CS were assessed using the Pediatric Quality of Life Inventory (PedsQL) 4.0 Generic Core Scales via both self- and proxy reports.Results: Seventy-three patients and their parents responded to the PedsQL (response rate: 80.2%). Patients generally estimated HRQoL as high, with no difference in HRQoL found between treated sagittal (SS) or metopic (MS) synostosis. In the SS group, surgical methods involving spring-assisted surgery and pi-plasty were unrelated to HRQoL outcomes. Additionally, HRQoL was highly correlated with intelligence quotient (IQ) and adaptive behavior skills (ABAS). Furthermore, we observed differences in estimated HRQoL between self- and proxy reports (i.e., parents estimated child HRQoL as higher than did the children). Conclusions: Children treated for CS have a generally high HRQoL, and neither CS type nor surgical method influenced HRQoL outcomes. Moreover, children and parents estimated HRQoL differently, suggesting the importance of using both self- and proxy reporting in patient-reported measures. We found that HRQoL was strongly related to IQ and ABAS, indicating that the PedsQL can be used as a screening instrument to identify craniofacial patients in need of further psychological assessment.

scholarly journals Child-related characteristics predicting subsequent health-related quality of life in 8- to 14-year-old children with and without cerebellar tumors: a prospective longitudinal study

2014 ◽  
Vol 1 (3) ◽  
pp. 114-122 ◽  
Author(s):  
Kim S. Bull ◽  
Christina Liossi ◽  
David Culliford ◽  
Janet L. Peacock ◽  
Colin R. Kennedy ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Parent Report ◽  
Regression Modeling ◽  
Self Report ◽  
Health Related Quality ◽  
Emotional Function ◽  
Related Quality ◽  
Health Related ◽  
Z Scores

Abstract Background We identified child-related determinants of health-related quality of life (HRQoL) in children aged 8–14 years who were treated for 2 common types of pediatric brain tumors. Methods Questionnaire measures of HRQoL and psychometric assessments were completed by 110 children on 3 occasions over 24 months. Of these 110, 72 were within 3 years of diagnosis of a cerebellar tumor (37 standard-risk medulloblastoma, 35 low-grade cerebellar astrocytoma), and 38 were in a nontumor group. HRQoL, executive function, health status, and behavioral difficulties were also assessed by parents and teachers as appropriate. Regression modeling was used to relate HRQoL z scores to age, sex, socioeconomic status, and 5 domains of functioning: Cognition, Emotion, Social, Motor and Sensory, and Behavior. Results HRQoL z scores were significantly lower after astrocytoma than those in the nontumor group and significantly lower again in the medulloblastoma group, both by self-report and by parent-report. In regression modeling, significant child-related predictors of poorer HRQoL z scores by self-report were poorer cognitive and emotional function (both z scores) and greater age (years) at enrollment (B = 0.038, 0.098, 0.136, respectively). By parent-report, poorer cognitive, emotional and motor or sensory function (z score) were predictive of lower subsequent HRQoL of the child (B = 0.043, 0.112, 0.019, respectively), while age at enrollment was not. Conclusions Early screening of cognitive and emotional function in this age group, which are potentially amenable to change, could identify those at risk of poor HRQoL and provide a rational basis for interventions to improve HRQoL.

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