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Table 3 Influence of severity of symptoms in PedsQL™ Family Impact Module

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

Sample Total Impact Score Parent HRQOL Family Functioning
Gross motor    
GMFC-MLD ≤ 4 (n = 10/10/10) 61.7 (18.6) 63.3 (19.6) 59.3 (19.6)
GMFC-MLD > 4 (n = 32/32/31) 56.5 (17.4) 60.6 (18.2) 47.3 (22.7)
Complete immobility    
GMFC-MLD < 6 (n = 30/30/30) 61.8 (17.8) 64.2 (18.9) 56.0 (22.0)
GMFC-MLD = 6 (n = 12/12/11) 48.6* (13.5) 54.6 (15.8) 37.2* (18.3)
Speech    
VSS < 4 (n = 10/10/10) 62.5 (16.8) 65.0 (18.0) 60.5 (17.8)
VSS = 4 (n = 32/32/31) 56.6 (17.9) 60.3 (18.6) 47.9 (23.2)
Complete loss of communication    
VSS ≤ 4 (at least ability to express emotions) (n = 34/34/34) 61.5 (16.4) 65.1 (17.1) 54.3 (21.8)
No ability to express emotions (n = 8/8/7) 43.1** (15.3) 45.8** (15.9) 34.5 (19.3)
Visual stimulation    
Visual stimuli perceptible (n = 32/32/32) 62.2 (16.7) 65.8 (17.2) 54.1 (21.5)
Visual stimuli not perceptible (n = 10/10/9) 44.8** (13.9) 47.4** (15.0) 39.6 (23.4)
Independent eating    
Independent eating possible (n = 8/8/8) 66.3 (17.8) 67.7 (18.9) 64.5 (15.9)
No independent eating possible (n = 34/34/33) 56.1 (17.3) 60.0 (18.2) 47.7* (22.8)
PEG tube    
No PEG tube present (n = 23/23/23) 62.7 (15.7) 64.8 (17.9) 56.4 (18.6)
PEG tube present (n = 19/19/18) 52.3* (18.5) 57.3 (18.6) 44.0 (24.5)
Final stage of disease    
Child alive (n = 32/32/31) 61.7 (17.5) 64.7 (18.2) 55.4 (21.9)
Child deceased (n = 11/11/11) 44.5** (9.8) 49.3* (14.0) 34.2** (14.8)
  1. Standard deviation presented in parentheses. Different case numbers result from the fact that one Family Functioning scale was insufficiently completed. Higher values equal better functioning/less negative impact
  2. PedsQL™, Pediatric Quality of Life Inventory [23]; HRQOL, health-related quality of life; GMFC-MLD, Gross Motor Function Classification-MLD [16]; MLD, metachromatic leukodystrophy; VSS, The Viking Speech Scale [24]; PEG, percutaneous endoscopic gastrostomy
  3. *P value is significant at < 0.05 level; **P value is significant at < 0.01 level. Comparison between every pair of a sub-item