Quality of life in patients with Tuberous Sclerosis Complex (TSC).
Burden
Complications
Quality of life
TSC
Journal
European journal of paediatric neurology : EJPN : official journal of the European Paediatric Neurology Society
ISSN: 1532-2130
Titre abrégé: Eur J Paediatr Neurol
Pays: England
ID NLM: 9715169
Informations de publication
Date de publication:
Nov 2019
Nov 2019
Historique:
received:
17
05
2019
revised:
07
09
2019
accepted:
08
09
2019
pubmed:
24
9
2019
medline:
12
2
2020
entrez:
24
9
2019
Statut:
ppublish
Résumé
The quality of life (QoL) of patients with Tuberous Sclerosis Complex (TSC) has not been studied before. We aimed to investigate the impact of the disease on QoL. We studied the QoL of 91 TSC patients who have attended the Bath TSC clinic, UK over 6 months. QoL was evaluated using the PedsQL for children, and SF-36 for adults. Impaired QoL is found in all patients with TSC regardless of the presence of epilepsy and learning disabilities (LD). Total mean self-reported score for children was 71 out of 100, compared to a UK norm of 84, p < 0.000. The proxy mean score was 48, (UK norm 85, p < 0.000). Physical Functioning score for adults with TSC was 70, compared to a UK norm of 94, p < 0.000. The Social Functioning score for adults with TSC was 71, (UK norm 88, p < 0.000). Impaired QoL is found in all patients with TSC regardless of the presence of epilepsy and learning disabilities. The psychosocial domain is most affected. The quality of life of children with TSC is lower than children who suffer from asthma, diabetes, cancer and inflammatory bowel disease. To improve health related quality of life in TSC, a focus on patient's physical health, educational performances, and overall quality of life is crucial. In order to achieve this, coordinated medical care across disciplines, and psychosocial and social support is necessary.
Sections du résumé
BACKGROUND
BACKGROUND
The quality of life (QoL) of patients with Tuberous Sclerosis Complex (TSC) has not been studied before. We aimed to investigate the impact of the disease on QoL. We studied the QoL of 91 TSC patients who have attended the Bath TSC clinic, UK over 6 months. QoL was evaluated using the PedsQL for children, and SF-36 for adults.
RESULTS
RESULTS
Impaired QoL is found in all patients with TSC regardless of the presence of epilepsy and learning disabilities (LD). Total mean self-reported score for children was 71 out of 100, compared to a UK norm of 84, p < 0.000. The proxy mean score was 48, (UK norm 85, p < 0.000). Physical Functioning score for adults with TSC was 70, compared to a UK norm of 94, p < 0.000. The Social Functioning score for adults with TSC was 71, (UK norm 88, p < 0.000).
CONCLUSIONS
CONCLUSIONS
Impaired QoL is found in all patients with TSC regardless of the presence of epilepsy and learning disabilities. The psychosocial domain is most affected. The quality of life of children with TSC is lower than children who suffer from asthma, diabetes, cancer and inflammatory bowel disease. To improve health related quality of life in TSC, a focus on patient's physical health, educational performances, and overall quality of life is crucial. In order to achieve this, coordinated medical care across disciplines, and psychosocial and social support is necessary.
Identifiants
pubmed: 31543331
pii: S1090-3798(19)30181-3
doi: 10.1016/j.ejpn.2019.09.006
pii:
doi:
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
801-807Commentaires et corrections
Type : CommentIn
Informations de copyright
Copyright © 2019 European Paediatric Neurology Society. Published by Elsevier Ltd. All rights reserved.