Quality of life and neurological disability in children and young people with ataxia telangiectasia.
Eur J Paediatr Neurol
; 40: 34-39, 2022 Sep.
Article
en En
| MEDLINE
| ID: mdl-35932633
ABSTRACT
AIM:
To explore neurological factors affecting quality of life (QoL) in children and young people with ataxia-telangiectasia (A-T), from both child and parent perspective.METHOD:
24 children/young people with A-T (mean age 11.2 ± 3.5 years; 13 males) and 20 parents were recruited, and 58% were reassessed after an average interval of 3.4 years. Participants completed the PedsQL QoL assessment. Participants with A-T underwent structured neurological examination. QoL data from 20 healthy controls and their parents was used for comparison.RESULTS:
Children/young people with A-T rated their QoL higher than parental ratings across time points, with no longitudinal change. Higher age of the child participant correlated with lower parental (r = -0.43, p = .008) but not child ratings of QoL (r = -0.16, p = .380). Child and parent QoL ratings from the A-T group were lower than respective ratings from controls (ηp2 = 0.44 and ηp2 = 0.75 respectively, both p < .0005, controlled for socioeconomic status). Parental, but not child, ratings of QoL was predicted by a regression model based on neurological scores (R2 = 0.44, p=<.001).INTERPRETATION:
Neurological disability does not determine child/young person QoL ratings in A-T. While certain aspects of neurological disability predict parent-proxy ratings, there is no decline in QoL over time. These results may reflect resilience in the face of a complex life-limiting disorder.Palabras clave
Texto completo:
1
Colección:
01-internacional
Banco de datos:
MEDLINE
Asunto principal:
Calidad de Vida
/
Ataxia Telangiectasia
Tipo de estudio:
Prognostic_studies
/
Qualitative_research
Límite:
Adolescent
/
Child
/
Humans
/
Male
Idioma:
En
Revista:
Eur J Paediatr Neurol
Asunto de la revista:
NEUROLOGIA
/
PEDIATRIA
Año:
2022
Tipo del documento:
Article