The impact of severe rare chronic neurological disease in childhood on the quality of life of families—a study on MLD and PCH2

Table 2 PedsQL™ Family Impact Module

Sample	Total Impact Score	Parent HRQOL	Family Functioning
MLD and PCH2 (n = 43/43/42)	57.3*** (17.5)	60.8*** (18.3)	49.8*** (22.2)
Late-infantile MLD (n = 8/8/8)	48.3*** (15,3)	54.8*** (20.8)	35.4*** (19.2)
Juvenile MLD (n = 19/19/18)	59.1*** (19.6)	61.1** (20.9)	56.2** (22.0)
PCH2 (n = 16/16/16)	59.7*** (15.5)	63.4* (15.2)	49.9*** (21.7)
MLD Mothers (n = 25/25/24)	53.1*** (18.8)	55.6*** (19.7)	47.8*** (24.3)
MLD Fathers (n = 22/22/22)	58.6*** (19.3)	63.0* (21.5)	51.9*** (21.5)
PCH2 Mothers (n = 16/16/16)	55.9*** (17.9)	58.6** (17.8)	47.4*** (23.6)
PCH2 Fathers (n = 13/13/13)	67.7 (16.1)	73.6 (16.6)	56.4* (24.3)
No chronic condition (n = 546) [26]	73.2 (13.6)	71.2 (14.9)	67.6 (18.4)

Standard deviation presented in parentheses. Different case numbers result from the fact that only sufficiently completed scales could be used. Higher values equal better functioning/less negative impact
PedsQL™, Pediatric Quality of Life Inventory [23]; MLD, metachromatic leukodystrophy; PCH2, pontocerebellar hypoplasia type 2; HRQOL, health-related quality of life
^*P value is significant at < 0.05 level; **P value is significant at < 0.01 level; ***P value is significant at < 0.001 level. Comparison between individual samples and the “no chronic condition” sample

ISSN: 1750-1172