A patient empowerment program for primary immunodeficiency improves quality of life in children and adolescents

Figures & data

Table 1. Baseline characteristics of the study population.

	All subjects (n = 19)
Age (years)   Gender   – Male   – Female	10 (3)   12 (63)   7 (37)
Duration of IgG treatment (years)	3 (3)
Ethnic background   – Caucasian   – Asian   – Other	17 (90)   1 (5)   1 (5)
IgG treatment ever and current   – Intravenous and subcutaneous   – Intravenous   – Subcutaneous	9 (47)   5 (26)   5 (26)
IgG treatment location ever and current   – Hospital/ outpatient care unit   – Hospital/ at home   – At home	5 (26)   13 (68)   1 (5)
Type of immunodeficiency   – CVID   – CID   – APDS   – ARA   – Complement deficiency   – Specific antibody deficiency   – XLF/Cernunnos deficiency   – Other primary antibody deficiency	5 (26)   3 (16)   3 (16)   1 (5)   1 (5)   1 (5)   1 (5)   4 (21)

Values for mean (SD) or total number (percentage) are shown.

APDS: Activated PI3K delta syndrome; ARA: Autosomal recessive agammaglobulinaemia; CID: Combined immunodeficiency; CVID: Common variable immune deficiency; XLF: XRCC4-like factor.

Table 2. KIDSCREEN-27 dimensions in children/adolescents and parents/proxy before and 6 months after PID-PEP kids.

	Before	After	p-value
Children/adolescents (n = 13)
Physical Well-Being	48 (15)	50 (11)	0.110
Psychological Well-Being	52 (13)	56 (14)	0.017
Parents & Autonomy	53 (13)	57 (12)	0.007
Social Support & Peers	49 (15)	53 (10)	0.168
School Environment	52 (16)	57 (12)	0.012
Parents/proxy (n = 19)
Physical Well-Being	43 (14)	45 (10)	0.313
Psychological Well-Being	46 (11)	50 (12)	0.033
Parents & Autonomy	54 (6)	58 (9)	0.033
Social Support & Peers	46 (12)	53 (10)	0.011
School Environment	50 (10)	53 (10)	0.116

Values for mean (SD) and number of subjects being evaluated (n) are shown. Statistical significance was tested by Wilcoxon signed-rank test and bold values indicate p < 0.05.

Table 3. DISABKIDS-37 dimensions in children/adolescents and parents/proxy before and 6 months after PID-PEP kids.

	Before	After	p-value
Children/adolescents (n = 13)
Total	70 (20)	78 (17)	0.006
Independence	70 (21)	77 (17)	0.032
Emotion	71 (26)	79 (17)	0.036
Social Inclusion	68 (20)	78 (14)	0.002
Social Exclusion	78 (26)	84 (21)	0.007
Physical	69 (27)	79 (21)	0.012
Medication	65 (27)	72 (22)	0.091
Parents/proxy (n = 18)
Total	69 (17)	74 (18)	0.020
Independence	77 (15)	76 (17)	0.849
Emotion	67 (22)	76 (17)	0.022
Social Inclusion	66 (17)	68 (23)	0.392
Social Exclusion	78 (18)	81 (21)	0.074
Physical	67 (20)	73 (19)	0.050
Medication	57 (24)	69 (21)	0.003

Values for mean (SD) are shown. Statistical significance was tested by Wilcoxon signed-rank test and bold values indicate p < 0.05.

Figure 1. Mean KIDSCREEN-27 dimensions in a German population-representative study, as well as in (A) children/adolescents with PID (n = 13) and (B) their parents/proxy (n = 19) before and 6 months after PID-PEP kids.

Data taken from [19].

Germany National Normdata KIDSCREEN females & males, children & adolescents age 8–18 (self report & proxy report; Table A7_B-32; KIDSCREEN-27) [Internet]. Hamburg: Universitätsklinikum Hamburg-Eppendorf; 2011–2023 [ cited 2024 May 18]. Available from: https://www.kidscreen.org/deutsch/auswertung/manual-anh%C3%A4nge/

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