Introduction
Methods
Participants
Procedure
Measures
Health-related quality of life (HRQoL)
Socio-demographic, medical and psychosocial variables
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Socio-demographic variables: parental gender, age (parent and child), parental chronic disease (yes/no), parental educational level and parental country of birth.
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Medical variables: disease development (improving, progressive, relapsing, stable) in the previous year, use of tube-feeding (yes/no); dependency on medical equipment (yes/no); problems with interaction (yes/no); and hours of sleep per night (child). Also, care dependency (ranging from 0 to 8) was included, defined as the number of life domains on which the child needs care (physical, mobility, eating and drinking, medication use, coping with devices, entertaining, contact with other children, education).
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Psychosocial variables: emotional support was defined as support from four groups of reference persons (partner, family, friends or neighbors), each scored on a 3-point scale with the following values: 0 = no, 1 = more or less, 2 = good, resulting in an overall score for emotional support from 0 to 8; loss of friendship since illness of child (yes/no), given up hobby since illness of child (yes/no), job adjustment due to illness of child (yes/no), and contact with fellow-sufferers (frequently/often versus sometimes or never).
Statistics
Results
Participants
PKU | Galactosemia | Lyso, org, mitoch | Healthy | |
---|---|---|---|---|
Socio-demographic | ||||
Parental age ** | ||||
Mean (SD) | 40.7 (6.3) | 42.5 (6.5) | 41.5 (7.0) | 43.7 (5.4) |
Range | 28.4–57.4 | 29.4–55.1 | 23.8–60.1 | 20.1–60.5 |
n
| 86 | 41 | 108 | 441 |
Age child | ||||
Mean (SD) | 8.7 (5.0) | 9.8 (5.1) | 8.2 (4.5) | |
Range | 1–19 | 1–19 | 1–18 | |
n
| 109 | 65 | 108 | |
Parental gender: % (n) ** | ||||
Male | 43.1 (50) | 39.1 (27) | 21.3 (23) | 16.7 (74) |
Female | 56.9 (66) | 60.9 (42) | 78.7 (85) | 83.3 (369) |
Parental educational level : % (n) a
| ||||
Low | 25.0 (29) | 23.2 (16) | 23.4 (25) | 20.8 (91) |
Middle | 39.7 (46) | 33.3 (23) | 42.1 (45) | 38.2 (167) |
High | 35.3 (41) | 43.5 (30) | 34.6 (37) | 41.0 (179) |
Parental country of birth (Netherlands): % (n) * | 94.0 (109) | 85.5 (59) | 95.4 (103) | 86.0 (381) |
Parental chronic disease (yes): % (n) | 7.8 (9) | 5.8 (4) | 12.0 (13) | 7.7 (34) |
Medical b
| ||||
Disease development: % (n) | ||||
Improving | 26.7 (31) | 24.6 (17) | 16.7 (18) | |
Progressive | 1.7 (2) | 2.9 (2) | 31.5 (34) | |
Relapsing | 14.7 (17) | 13.0 (9) | 23.1 (25) | |
Stable | 56.9 (66) | 59.4 (41) | 28.7 (31) | |
Tube-feeding (yes): % (n) | 0.9 (1) | 0 | 25.9 (28) | |
Dependency medical equipment (yes): % (n) | 6.0 (7) | 0 | 25.9 (28) | |
Problems making contact (yes): % (n) | 4.3 (5) | 13.0 (9) | 56.3 (58) | |
Hours sleep per night | ||||
Mean (SD) | 9.7 (1.5) | 9.4 (1.7) | 9.0 (2.7) | |
Range | 5–12 | 3–13 | 1–13 | |
n
| 116 | 69 | 101 | |
Care dependency (0-8) | ||||
Mean (SD) | 1.1 (1.1) | 2.0 (1.8) | 5.1 (2.3) | |
Range | 0–5 | 0–7 | 0–8 | |
n
| 116 | 69 | 108 | |
Psychosocial (parent) b
| ||||
Emotional support (0-8) | ||||
Mean (SD) | 5.4 (1.8) | 5.4 (1.5) | 4.9 (1.7) | |
Range | 0–8 | 1–8 | 1–8 | |
n
| 116 | 69 | 108 | |
Loss of friendship (yes): % (n) | 11.2 (13) | 20.3 (14) | 55.6 (60) | |
Given up hobby (yes): % (n) | 3.4 (4) | 10.1 (7) | 45.3 (49) | |
Job adjustment (yes): % (n) | 21.6 (25) | 34.8 (24) | 25.9 (28) | |
Contact fellow sufferers (frequently/often): % (n) | 12.1 (14) | 27.5 (19) | 38.9 (42) |
Parental health related quality of life
PKU n = 115 | Galactosemia n = 69 | Lyso, Org, Mitocha
n = 108 b
| Healthy n ≈ 430 c
| |||||
---|---|---|---|---|---|---|---|---|
Mean | 95% CI | Mean | 95% CI | Meand
| 95% CI | Mean | 95% CI | |
Gross motor | 96.5 | 94.7–98.3 | 81.9 b | 77.7–86.2 | 88.0 | 86.0–89.9 | ||
Fine motor | 96.3 | 92.7–99.8 | 94.1 | 91.3–96.9 | 96.9 | 96.0–97.7 | ||
Cognitive | 82.7 | 78.7–86.7 | 81.4 | 75.1–87.8 | 68.9 a | 63.6–74.2 | 78.9 | 76.5–81.2 |
Sleep | 75.7 | 71.3–80.1 | 59.8 | 72.6–67.7 | 58.2 ab | 52.7–63.7 | 70.7 | 68.3–73.0 |
Pain | 78.3 | 74.6–82.0 | 74.5 | 68.7–80.2 | 62.1 ab | 56.8–67.4 | 71.9 | 69.8–74.0 |
Social | 84.1 | 80.7–87.5 | 84.2 | 80.1–88.4 | 70.9 abc | 66.4–75.3 | 83.5 | 81.6–85.4 |
Daily activities | 87.7 | 83.7–91.7 | 88.6 | 83.5–93.7 | 68.3 abc | 63.0–73.7 | 85.3 | 83.2–87.3 |
Sexuality | 84.6 | 80.1–89.1 | 86.2 | 80.5–92.0 | 70.6 abc | 65.1–76.0 | 84.8 | 82.5–87.0 |
Vitality | 63.8 | 59.1–68.4 | 62.0 | 56.9–67.0 | 47.7 abc | 42.7–52.7 | 63.3 | 61.2–65.4 |
Pos. emotions | 64.6 | 60.8–68.5 | 62.4 | 57.9–67.0 | 56.3 a | 52.5–60.2 | 66.4 | 64.5–68.3 |
Depres. emotions | 78.9 | 75.6–82.2 | 75.2 | 70.6–79.8 | 60.1 abc | 56.2–64.0 | 78.6 | 76.8–80.4 |
Agressiveness | 89.3 | 86.6–91.9 | 90.2 | 87.3–93.1 | 83.0 | 79.8–86.2 | 86.0 | 89.1–89.6 |
Predictors of mental HRQoL in parents of children with PKU or galactosemia
PKU | Galactosemia | |||||
---|---|---|---|---|---|---|
Coefficient β | SE of β | 95% CI | Coefficient β | SE of β | 95% CI | |
Fixed effects | ||||||
Socio-demographic | ||||||
Age child | 0.21* | 0.10 | 0.02; 0.41 | −0.09 | 0.10 | −0.29; 0.10 |
Parental gender (female) | −0.17 | 0.16 | −0.49; 0.15 | −0.38b
| 0.20 | −0.78; 0.02 |
Native country (Netherlands) | 0.45 | 0.34 | −0.22; 1.12 | −0.07 | 0.29 | −0.66; 0.51 |
Chronically ill | −0.20 | 0.32 | −0.83; 0.43 | −0.17 | 0.43 | −1.02; 0.68 |
Medical | ||||||
Disease development (stable) | 0.32a
| 0.17 | −0.03; 0.66 | 0.38c
| 0.20 | −0.02; 0.77 |
Care dependency | −0.17 | 0.11 | −0.40; 0.05 | −0.07 | 0.10 | −0.26; 0.12 |
Psychosocial | ||||||
Emotional support | 0.20* | 0.09 | 0.02; 0.38 | 0.38** | 0.10 | 0.17; 0.58 |
Loss of friendship | −1.2** | 0.32 | −1.8; −0.52 | 0.06 | 0.28 | −0.49; 0.62 |
Contact fellow sufferers | 0.11 | 0.27 | -0.41; 0.64 | 0.18 | 0.22 | -0.25; 0.61 |
Job adjustment | 0.09 | 0.22 | -0.36; 0.53 | −0.41d
| 0.22 | -0.86; 0.04 |
% Explained variance by fixed effects | 31.7 | 30.6 | ||||
Total number of observations | 109 | 65 |