Development and psychometric validation of measures to assess the impact of phenylketonuria and its dietary treatment on patients’ and parents’ quality of life: the phenylketonuria – quality of life (PKU-QOL) questionnaires

Regnault, Antoine; Burlina, Alberto; Cunningham, Amy; Bettiol, Esther; Moreau-Stucker, Flavie; Benmedjahed, Khadra; Bosch, Annet M

doi:10.1186/s13023-015-0261-6

Orphanet Journal of Rare Diseases

Table 5 Spearman correlation coefficients between Parent PKU-QOL and CHQ-PF28 scores in the parent evaluable population (n = 253)

From: Development and psychometric validation of measures to assess the impact of phenylketonuria and its dietary treatment on patients’ and parents’ quality of life: the phenylketonuria – quality of life (PKU-QOL) questionnaires

	CHQ-PF28 scores
PKU-QOL scores	PF	RP	GH	BP	PiT	PiE	RE	SE	MH	Be	FA	FC	CH
Child health status	−0.24	−0.24	−0.43	−0.26	−0.34	−0.39	−0.09	−0.37	−0.23	−0.37	−0.37	−0.26	0.15
Headaches	−0.06	−0.10	−0.29	−0.32	−0.18	−0.21	−0.12	−0.25	−0.29	−0.25	−0.22	−0.16	0.16
Stomach aches	−0.11	−0.06	−0.18	−0.40	−0.11	−0.23	−0.02	−0.08	−0.27	−0.15	−0.17	−0.03	0.17
Tiredness	−0.24	−0.21	−0.28	−0.28	−0.34	−0.36	−0.19	−0.26	−0.35	−0.28	−0.23	−0.11	−0.00
Lack of concentration	−0.10	−0.07	−0.23	−0.23	−0.20	−0.22	−0.28	−0.36	−0.33	−0.57	−0.22	−0.17	−0.06
Slow thinking	−0.16	−0.10	−0.29	−0.20	−0.20	−0.23	−0.40	−0.37	−0.36	−0.47	−0.26	−0.17	0.03
Irritability	−0.12	−0.09	−0.28	−0.16	−0.28	−0.36	−0.23	−0.42	−0.45	−0.54	−0.27	−0.29	0.03
Aggressiveness	−0.16	−0.22	−0.19	−0.12	−0.26	−0.28	−0.31	−0.31	−0.37	−0.49	−0.28	−0.24	−0.09
Moodiness	−0.14	−0.18	−0.21	−0.26	−0.32	−0.43	−0.31	−0.40	−0.43	−0.53	−0.37	−0.24	−0.07
Sadness	−0.16	−0.10	−0.25	−0.32	−0.36	−0.30	−0.36	−0.38	−0.43	−0.35	−0.28	−0.16	0.03
Anxiety	−0.18	−0.15	−0.24	−0.18	−0.24	−0.16	−0.34	−0.39	−0.41	−0.20	−0.21	−0.21	0.01
Emotional impact of PKU	−0.14	−0.15	−0.33	−0.21	−0.23	−0.40	−0.04	−0.22	−0.29	−0.26	−0.29	−0.27	−0.03
Practical impact of PKU	−0.20	−0.18	−0.35	−0.21	−0.33	−0.39	−0.15	−0.24	−0.37	−0.34	−0.42	−0.25	0.07
Social impact of PKU	−0.20	−0.18	−0.29	−0.24	−0.26	−0.35	−0.20	−0.25	−0.33	−0.31	−0.37	−0.36	−0.03
Overall impact of PKU	−0.21	−0.20	−0.39	−0.26	−0.31	−0.46	−0.12	−0.29	−0.37	−0.35	−0.44	−0.34	−0.02
Anxiety – blood test	−0.15	−0.13	−0.14	−0.05	−0.21	−0.22	−0.03	−0.01	−0.15	−0.08	−0.27	−0.06	0.08
Impact of anxiety – blood test	−0.19	−0.20	−0.24	−0.10	−0.26	−0.26	−0.09	0.02	−0.14	−0.05	−0.29	−0.03	0.26
Anxiety – blood Phe levels	−0.06	−0.06	−0.25	−0.13	−0.13	−0.42	−0.09	−0.06	−0.28	−0.24	−0.19	−0.09	0.05
Financial impact of PKU	−0.12	−0.05	−0.20	−0.19	−0.19	−0.29	−0.06	−0.05	−0.15	−0.26	−0.24	−0.20	0.03
Information on PKU	−0.14	−0.19	−0.25	−0.26	−0.14	−0.24	−0.14	−0.30	−0.16	−0.24	−0.19	−0.27	0.00
Adherence to Phe-free protein supplements	−0.09	−0.16	−0.10	−0.13	−0.11	−0.08	−0.22	−0.24	−0.21	−0.28	−0.12	−0.07	0.03
Management of Phe-free protein supplements	−0.03	−0.12	−0.09	−0.10	−0.28	−0.21	−0.05	−0.07	−0.18	−0.28	−0.22	−0.14	−0.06
Practical impact of Phe-free protein supplements	−0.15	−0.23	−0.19	−0.12	−0.17	−0.18	−0.04	−0.06	−0.25	−0.10	−0.38	−0.26	−0.03
Guilt if poor adherence to Phe-free protein supplements	−0.14	−0.07	−0.27	−0.09	−0.10	−0.27	−0.11	0.08	−0.20	−0.10	−0.14	−0.05	0.15
Relationships within family because of Phe-free protein supplements	−0.17	−0.21	−0.15	−0.26	−0.33	−0.22	−0.15	−0.16	−0.24	−0.34	−0.32	−0.23	−0.02
Adherence to dietary protein restriction	−0.04	−0.09	−0.17	−0.08	−0.19	−0.11	−0.14	−0.15	−0.09	−0.24	−0.16	−0.10	0.07
Management of dietary protein restriction	−0.12	−0.16	−0.26	−0.25	−0.30	−0.40	−0.24	−0.34	−0.35	−0.49	−0.41	−0.29	−0.04
Practical impact of dietary protein restriction	−0.15	−0.24	−0.24	−0.28	−0.30	−0.33	−0.10	−0.30	−0.32	−0.33	−0.46	−0.39	−0.14
Food enjoyment	−0.11	−0.06	−0.19	−0.28	−0.21	−0.14	−0.07	−0.26	−0.27	−0.24	−0.28	−0.28	0.07
Guilt if dietary protein restriction not followed	−0.15	−0.04	−0.15	−0.07	−0.05	−0.31	−0.12	−0.00	−0.15	−0.07	−0.12	−0.04	0.04

CHQ-PF: Child Health Questionnaire–Parent Form; Phe: phenylalanine; PKU: phenylketonuria; QOL: quality of life; PF: Physical functioning; RP: Role/Social Physical; GH: General health; BP: Bodily Pain; PiT: Parent impact Time; PiE: Parent impact Emotional; RE: Role/Social Emotional; SE: Self-Esteem; MH: Mental Health; Be: Behaviour; FA: Family Activity; FC: Family Cohesion; CH: Change in Health.
Moderate correlations (>0.4) are shown in bold.

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ISSN: 1750-1172

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