Quality of Life and Associated Factors in Japanese Children With Inborn Errors of Metabolism and Their Families

Yamaguchi, Keiko; Wakimizu, Rie; Kubota, Mitsuru

doi:10.1177/2326409818755799

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Journal of Inborn Errors of Metabolism and Screening

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Original Article • J. inborn errors metab. screen. 6 • 2018 • https://doi.org/10.1177/2326409818755799 copy

Quality of Life and Associated Factors in Japanese Children With Inborn Errors of Metabolism and Their Families

Authorship SCIMAGO INSTITUTIONS RANKINGS

Abstract

To reveal the associated factors of quality of life (QoL) in children with inborn errors of metabolism (IEM), their siblings, and their primary caregivers and partners, we conducted an anonymous questionnaire survey in Japan. Descriptive, correlation, and multiple regression analyses were performed. Fifty-six children with IEM, 35 siblings, 143 primary caregivers, and 86 partners completed our questionnaires. There were significant positive correlations between higher QoL in children with IEM and lower disease influence (r ¼0.46) and higher perceived emotional support (r ¼0.67). We could not find any associated factor of siblings’ QoL. Lower parental distress, higher family empowerment, and higher household income contributed to higher QoL in primary caregivers (adjusted R² ¼ 0.636). Higher household income, lower anxiety about childrearing, and higher satisfaction in the relationship with the child and entire family contributed to higher QoL of partners (adjusted R² ¼ 0.398). We concluded that developing effective interventions to improve QoL is needed for the entire family in future outpatient settings.

Keywords
inborn errors of metabolism; quality of life; children; family; questionnaire survey

KINDL Scores	Children With IEM (n = 56)					Siblings of a Child With IEM (n = 35)
KINDL Scores	Age	Number of Siblings	Birth Order	Disease (KINDL)	Emotional Support Resources Score	Age	Number of Siblings	Birth Order	Emotional Support Resources Score
Physical	—0.268^a a P < .05.	0.017	0.009	0.417^b b P < .01.	0.417^b b P < .01.	—0.325	—0.021	0.098	0.491^b b P < .01.
Emotional	—0.23	0.008	—0.041	0.383^a a P < .05.	0.415^b b P < .01.	—0.069	0.098	—0.041	0.445^b b P < .01.
Self-esteem	—0.071	—0.297^a a P < .05.	—0.23	0.181	0.505^b b P < .01.	—0.126	0.035	—0.011	0.155
Family	0.015	—0.058	—0.04	0.316^a a P < .05.	0.443^b b P < .01.	0.216	0.213	—0.068	0.505^b b P < .01.
Friends	—0.107	—0.005	0.001	0.336^a a P < .05.	0.492^b b P < .01.	—0.469^b b P < .01.	-0.126	0.058	0.164
School	—0.128	—0.081	—0.097	0.455^b b P < .01.	0.426^b b P < .01.	—0.15	-0.139	0.042	—0.047
Total	—0.153	—0.076	—0.054	0.460^b b P < .01.	0.673^b b P < .01.	—0.238	0.021	0.042	0.291

		Children With IEM (n = 56)		Siblings of a Child With IEM (n = 35)
		n/M (SD)	%/ range	n/M (SD)	%/ range
Age		12.0 (3.1)	7-20	12.4 (3.3)	7-20
	7-12 years	31	54.4%	20	57.1%
	13-17 years	23	41.0%	13	37.1%
	18-20 years	2	3.6%	2	5.7%
Gender^a a Gender: male (boy) and female (girl).
	Male	28	50.0%	11	31.4%
	Female	28	50.0%	22	62.9%
	NA	-	-	2	5.7%
Birth order
	First	27	48.2%	19	54.3%
	Second	21	37.5%	14	40.0%
	Third	8	14.3%	2	5.7%
The number of siblings
	Zero	11	19.0%	0	-
	One	17	48.2%	17	48.6%
	Two	17	30.4%	14	40.0%
	Three	1	1.8%	3	8.6%
	Four	0	0.0%	1	2.9%
Disease^b b Comorbidities of children with IEM (n): heart disease (1) and atopic dermatitis (1).
	With	56	100.0%	7	20.0%
	Without	0	0.0%	31	88.6%
Diagnosis^c c Siblings’ diagnosis (n): allergic rhinitis (1), spina bifida (1), Basedow disease (1), and NA (1).
	Amino acid disorders	31	55.4%	-	-
	Lysosomal storage disease	6	10.7%	-	-
	Organic acidemias	9	16.1%	-	-
	Carbohydrate metabolism	4	7.1%	-	-
	disorders
	Metal metabolism disorders	4	7.1%	-	-
	Fatty acid metabolism	2	3.6%	-	-
	disorders

		Primary Caregivers (n = 143)		Partners (n = 86)
		M (SD)	Range	M (SD)	Range
Age		42.4 (6.0)	27-58	43.3 (6.8)	26-59
	20s	5	3.5%	2	2.3%
	30s	36	25.2%	23	26.3%
	40s	85	59.5%	45	51.5%
50s		17	11.9%	17	19.4%
Gender^a a Gender: male (father) and female (mother).
	Male	11	7.7%	76	88.4%
	Female	132	92.3%	10	11.6%
Occupation
	Full-time housewife	65	45.5%	4	4.7%
	Part-time job	29	20.3%	5	5.8%
	Full-time job	34	23.8%	66	76.7%
	Self-employed	10	7.0%	11	12.3%
	Other	5	3.5%	0	0.0%
Disease
	With	27	18.9%	12	14.0%
	Without	116	81.1%	74	86.0%

Scores of the Japanese Version of the KINDL			Children With IEM (n = 56)		Siblings (n = 35)
Subscales	No of Items	Score Range	M (SD)	Range	M (SD)	Range
Physical	4	0.0-100	75.9 (19.0)	25.0-100	77.1 (19.5)	18.8-100
Emotional	4	0.0-100	81.5 (14.2)	43.8-100	81.1 (13.1)	50.0-100
Self-esteem	4	0.0-100	51.8 (26.0)	0.0-100	58.8 (22.6)	0.0-100
Family	4	0.0-100	71.6 (20.7)	6.3-100	73.9 (17.3)	25.0-100
Friends	4	0.0-100	69.6 (20.7)	25.0-100	77.1 (16.1)	37.5-100
School	4	0.0-100	65.7 (19.3)	25.0-100	62.3 (18.9)	25-100
Total	24	0.0-100	69.2 (14.4)	29.2-100	71.7 (12.6)	28.1-90
Disease	6	0.0-100	75.0 (15.1)	37.5-100	79.2 (7.2) n = 4	71-83
Emotional support resources in daily life scores	4	0.0-100	76.0 (19.4)	0.0-100	80.7 (17.0)	37.5-100

				Primary Caregivers (n = 143)		Partners (n = 86)
		No of Items	Score Range	M (SD)	Range	M (SD)	Range
Scores of the Japanese version of WHOQOL-26		26	1.0-5.0	3.29 (0.62)	1.54-4.73	3.38 (0.48)	2.19-4.42
	Physical health	7	1.0-5.0	3.33 (0.73)	1.29-4.86	3.50 (0.62)	2.14-4.71
	Psychological health	6	1.0-5.0	3.27 (0.73)	1.17-5	3.44 (0.59)	1.67-5.00
	Social relationships	3	1.0-5.0	3.38 (0.68)	1.0-5.0	3.29 (0.51)	2.00-4.33
	Environment	8	1.0-5.0	3.27 (0.66)	1.25-4.75	3.32 (0.56)	1.75-4.50
	Two items for total QoL	2	1.0-5.0	3.17 (0.74)	1.0-5.0	3.20 (0.670	1.50-5.00
Scores of the Japanese version of PSI-SF		19	19-95	45.2 (10.6)	22-82	-	-
	Difficult child	9	10-50	21.6 (5.8)	9-40	-	-
	Parental distress	10	9-45	23.6 (6.9)	10-44	-	-
Scores of the J-FES		34	34-170	103.3 (0.7)	14-60	-	-
	Family	12	12-60	39.6 (7.6)	21-60	-	-
	Service system	12	12-60	38.5 (7.5)	10-50	-	-
	Social politics	10	10-50	25.1 (6.2)	45-170	-	-

Dependent Variable: QoL Scores Explanatory variables		Primary Caregivers (n = 143)		Partners (n = 86)
Dependent Variable: QoL Scores Explanatory variables		sb	P	sb	P
	Current age of children with IEM	-0.033	.522	0.121	.166
	Age of onset	-0.047	.377	-	-
	The number of times visiting a hospital per year	-0.062	.23	-	-
	Age of primary caregivers	-0.014	.784	0.171	.056
	The number of difficult things related to raising a child with IEM	-0.05	.37	-	-
	The number of anxieties related to raising a child with IEM	-0.015	.781	-0.273	.003^a a P<.01.
	The number of eligible information resource of their child’s disease and treatment	0.052	.308	-	-
	Degree of distress related to caring for a child with IEM (1 = strongly disagree, 2 = disagree, 3 = undecided, 4 = agree, 5 = strongly agree)	-0.062	.24	-0.163	.083
	Satisfaction with the relationship with the child with IEM (1 = very satisfied, 2 = satisfied, 3 = neither, 4 = dissatisfied, 5 = very dissatisfied)	0.01	.848	0.259	.01^b b P < .05.
	Satisfaction with the relationships within the family (1 = very satisfied, 2 = satisfied, 3 = neither, 4 = dissatisfied, 5 = very dissatisfied)	-0.033	.545	0.208	.04^b b P < .05.
	Degree of understanding their child with IEM by people around them (1 = bad, 2 = not good, 3 = normal, 4 = good, 5 = very good)	-0.044	.428	-	-
	Household income (1 = wealthy, 2 = slightly wealthy, 3 = average, 4 = slightly poor, 5 = poor)	0.147	.006^a a P<.01.	0.282	.002^a a P<.01.
PSI-SF
	Difficult child	0.029	.612	-	-
	Parental distress	-0.586	<.001^c	-	-
J-FES scores
	Family	0.248	<.001^c	0.024	.794
	Service system	-0.024	.721	0.016	.859
	Social politics	-0.1	.103	0.009	.916
R²		0.644		0.426
Adjusted R²		0.636		0.398

Latin American Society Inborn Errors and Neonatal Screening (SLEIMPN); Instituto Genética para Todos (IGPT) Rua Ramiro Barcelos, 2350, CEP: 90035-903, Porto Alegre, RS - Brasil, Tel.: 55-51-3359-6338, Fax: 55-51-3359-8010 - Porto Alegre - RS - Brazil
E-mail: rgiugliani@hcpa.edu.br

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[1] Corresponding Author:Keiko Yamaguchi, University of Tsukuba, 1-1-1 Tennodai, Tsukuba, Ibaraki305-8575, Japan. Email: yamaguchi.keiko.xa@alumni.tsukuba.ac.jp