Quality of life of children with tuberous sclerosis complex

Abstract

AimEvaluation of impaired quality of life (QOL) of Malaysian children with tuberous sclerosis complex (TSC) and its possible risk factors.MethodCross-sectional study on 68 parents of Malaysian children aged 2–18 years with TSC. QOL was assessed using proxy-report Paediatric Quality of Life Inventory (PedsQL) V.4.0, and scores compared with those from a previous cohort of healthy children. Parents also completed questionnaires on child behaviour (child behaviour checklist (CBCL)) and parenting stress (parenting stress index-short form). Multiple regression analysis was used to determine sociodemographic, medical, parenting stress and behavioural factors that impacted on QOL.ResultsThe mean proxy-report PedsQL V.4.0 total scale score, physical health summary score and psychosocial health summary score of the patients were 60.6 (SD 20.11), 65.9 (SD 28.05) and 57.8 (SD 19.48), respectively. Compared with healthy children, TSC patients had significantly lower mean PedsQL V.4.0 total scale, physical health and psychosocial health summary scores (mean difference (95% CI): 24 (18–29), 20 (12–27) and 26 (21–31) respectively). Lower total scale scores were associated with clinically significant CBCL internalising behaviour scores, age 8–18 years and Chinese ethnicity. Lower psychosocial health summary scale scores were associated with clinically significant CBCL internalising behaviour scores, Chinese ethnicity or >1 antiepileptic drug (AED).ConclusionParents of children with TSC reported lower PedsQL V.4.0 QOL scores in all domains, with psychosocial health most affected. Older children, those with internalising behaviour problems, of Chinese ethnicity or on >1 AED was at higher risk of lower QOL. Clinicians need to be vigilant of QOL needs among children with TSC particularly with these additional risk factors.

Quality of life in children with tuberous sclerosis complex: A pediatric cohort study

“…Our findings indicate that neuropsychiatric disorders seriously affect patients' QOL and require careful attention from neurologists. However, our results are quite different from those of previous studies: There were no significant differences in any of the QOL domains by age 20 . It was first found that the TSC1/TSC2 group reported significantly worse QOL.…”

“…Notably, the psychosocial health scores were significantly lower than the physical health scores, which was consistent with previous studies 20,21 . As in previous studies, TANDs can exist at all ages 5 .…”

“…Patient QOL is affected by the interaction of all phenotypes 29,30 . This study is probably the most comprehensive study on risk factors for QOL to be conducted thus far 20,21,31 . Multiple linear regression models were adopted to identify possible risk factors independently related to the low proxy‐report PedsQL scores.…”

Evaluating Health-related Quality of Life Among Tuberous Sclerosis Complex Individuals Using Health Utilities Index: a Local Study and Comparison With Existing Data

“…There are two recent studies conducted in Asia assessing the QoL of TSC patients in Malaysia and mainland China respectively (29,30). We compared these two studies with our study and identi ed 3 distinctive differences.…”

“…Data regarding HRQoL in Asian TSC population is limited. Two cross-sectional studies evaluated the QoL of TSC patients using PedsQL in China and Malaysia respectively, but their study populations were limited to the pediatric age group (29,30). Another retrospective analysis study documented the QoL of postepileptic surgery TSC patients in China (31).…”