Introduction
Methods
Results
Characteristic | N = 271 |
---|---|
Mean (±SD) | |
Duration of the disease (years) | 9.8 (±3.8) |
Average pretransfusion haemoglobin (g/dL) | 8.4 (±1.1) |
Annual transfusion requirement (ml/kg/year) | 235 (±76) |
Average serum ferritin (ng/mL) | 1993 (±1844) |
Duration of hospital stay (days) | 2.29 (±1.0) |
Distance from home to hospital (km) | 54.8 (±48) |
Frequency (%) | |
Age groups | |
5–7 years | 68 (25.1%) |
8–12 years | 116 (42.8%) |
13–18 years | 87 (32.1%) |
Sub-type of thalassaemia | |
β-thalassaemia major | 228 (84.1%) |
HbE β-thalassaemia | 43 (15.9%) |
Thalassaemia treatment centre | |
Kurunegala | 155 (57.2%) |
Anuradhapura | 79 (29.1%) |
Ragama | 37 (13.7%) |
Frequency of blood transfusions | |
> 4 weekly | 29 (10.7%) |
4 weekly | 208 (76.8%) |
3 weekly | 33 (12.2%) |
< 3 weekly | 1 (0.4%) |
Average pretransfusion haemoglobin | |
< 7.0 g/dl | 32 (11.8%) |
7.0–8.9 g/dl | 132 (48.7%) |
9.0–10.5 g/dl | 99 (36.5%) |
> 10.5 g/dl | 8 (3.0%) |
Annual transfusion requirementa | |
< 200 ml/kg/year | 109 (42.6%) |
201–250 ml/kg/year | 41 (16.0%) |
251–300 ml/kg/year | 55 (21.5%) |
> 300 ml/kg/year | 51 (19.9%) |
Spleen status | |
No splenomegaly | 179 (66.1%) |
Splenomegaly of 1–3 cm | 74 (27.3%) |
Splenomegaly of > = 4 cm | 11 (4.1%) |
Splenectomised | 7 (2.5%) |
Liver status | |
No hepatomegaly | 188 (69.3%) |
Hepatomegaly of 1–2 cm | 67 (24.7%) |
Hepatomegaly > = 3 cm | 16 (5.9%) |
Serum Ferritinb | |
< 1000 ng/mL | 83 (31.6%) |
1001–2500 ng/mL | 122 (46.4%) |
2501–5000 ng/mL | 38 (14.4%) |
> 5000 ng/mL | 20 (7.6%) |
Iron chelator medication | |
No chelation | 1 (0.4%) |
Deferasirox | 163 (60.1%) |
Deferoxamine | 29 (10.7%) |
Deferiprone | 3 (1.1%) |
Deferasirox + Deferoxamine | 75 (27.7%) |
Complications | |
Thalassaemia facies | 92 (33.9%) |
Skin pigmentation | 57 (21.0%) |
Short staturec | 117 (48.0%) |
Undernutritiond | 85 (35.1%) |
Type 1 diabetes | 5 (1.8%) |
Hypothyroidism | 11 (4.1%) |
Cardiomyopathy | 2 (0.7%) |
Elevated transaminases | 58 (21.4%) |
Cirrhosis | 0 |
Allergic reaction to transfusion | 60 (22.1%) |
Hepatitis C infection | 60 (22.1%) |
Abdominal scars | 66 (24.4%) |
Hearing impairment | 4 (1.5%) |
Visual impairment | 19 (7.0%) |
Health-related quality of life among patients with transfusion dependent β-thalassaemia and controls
Health-related quality of life among patients with β-thalassaemia major and haemoglobin E β-thalassaemia
Correlation between socio-demographic and clinical characteristics and health-related quality of life
Total quality of life score | Physical functioning score | Psychosocial health summary score | Emotional functioning score | Social functioning score | School functioning score | |
---|---|---|---|---|---|---|
Age$ (n = 271) | 0.029 | 0.082 | (−) 0.004 | 0.126* | (−) 0.046 | (−) 0.084 |
Duration of disease$ (n = 271) | 0.060 | 0.075 | 0.053 | 0.171** | 0.034 | (−) 0.088 |
Average pretransfusion haemoglobin$ (n = 271) | 0.056 | (−) 0.014 | 0.086 | 0.083 | 0.072 | 0.032 |
Annual transfusion requirement$ (n = 256) | 0.075 | 0.024 | 0.095 | 0.157* | 0.041 | 0.016 |
Average serum ferritin$ (n = 263) | 0.050 | 0.105 | 0.008 | 0.044 | 0.048 | (−) 0.071 |
Spleen size# (n = 271) | (−) 0.038 | 0.008 | (−) 0.054 | (−) 0.057 | (−) 0.021 | (−) 0.059 |
Liver size# (n = 271) | (−) 0.044 | (−) 0.005 | (−) 0.062 | (−) 0.025 | (−) 0.034 | (−) 0.082 |
Duration of hospital stay# (n = 242) | (−) 0.064 | (−) 0.009 | (−) 0.073 | (−) 0.018 | (−) 0.097 | (−) 0.106 |
Distance from home to hospital$ (n = 244) | (−) 0.068 | (−) 0.002 | (−) 0.079 | (−) 0.007 | (−) 0.053 | (−) 0.143* |
Clinical determinants of health-related quality of life
Total quality of life score (Mean ± SD) | Physical functioning score (Mean ± SD) | Psychosocial health summary score (Mean ± SD) | Emotional functioning score (Mean ± SD) | Social functioning score (Mean ± SD) | School functioning score (Mean ± SD) | |
---|---|---|---|---|---|---|
Average pretransfusion haemoglobin | ||||||
> 9.0 g/dl (n = 107) | 73.9 (±12.4) | 74.6 (±15.6) | 73.6 (±12.4) | 71.8 (±15.3) | 82.6 (±15.9) | 66.3 (±17.6) |
< 9.0 g/dl (n = 164) | 72.2 (±12.5) | 74.5 (±15.4) | 71.0 (±12.7) | 69.7 (±17.0) | 79.6 (±14.6) | 64.3 (±17.1) |
p-value |
0.26
|
0.97
|
0.10
|
0.31
|
0.10
|
0.36
|
Annual transfusion requirement | ||||||
< 250 ml/kg/year (n = 150) | 71.8 (±13.0) | 73.9 (±16.3) | 70.8 (±12.7) | 68.9 (±16.4) | 80.5 (±15.6) | 63.4 (±17.7) |
> 250 ml/kg/year (n = 106) | 73.8 (±12.1) | 74.8 (±15.1) | 73.3 (±12.6) | 72.3 (±16.6) | 81.1 (±15.2) | 66.4 (±16.3) |
p-value |
0.20
|
0.64
|
0.13
|
0.10
|
0.75
|
0.16
|
Liver status | ||||||
No hepatomegaly (n = 188) | 73.5 (±11.9) | 74.8 (±15.0) | 72.8 (±12.2) | 71.0 (±16.1) | 81.4 (±14.4) | 66.3 (±17.4) |
Hepatomegaly (n = 83) | 71.5 (±13.6) | 74.0 (±16.6) | 70.4 (±13.4) | 69.4 (±17.0) | 79.3 (±16.7) | 62.3 (±16.9) |
p-value |
0.22
|
0.70
|
0.15
|
0.47
|
0.28
|
0.08
|
Spleen status | ||||||
No splenomegaly (n = 179) | 73.7 (±12.1) | 74.8 (±15.5) | 73.0 (±13.3) | 71.8 (±16.1) | 81.6 (±14.1) | 66.2 (±17.6) |
Splenomegaly (n = 85) | 72.0 (±13.1) | 74.8 (±14.9) | 70.8 (±13.3) | 69.1 (±16.4) | 80.0 (±16.6) | 63.2 (±16.7) |
p-value |
0.30
|
0.98
|
0.17
|
0.21
|
0.41
|
0.18
|
Splenectomy status | ||||||
No splenomegaly (n = 179) | 73.7 (±12.1) | 74.8 (±15.5) | 73.0 (±13.3) | 71.8 (±16.1) | 81.6 (±14.1) | 66.2 (±17.6) |
Splenectomised (n = 7) | 62.4 (±8.1) | 64.4 (±20.5) | 61.7 (±6.7) | 55.7 (±14.5) | 70.1 (±20.7) | 59.2 (±15.3) |
p-value |
< 0.05
|
0.08
|
< 0.05
|
< 0.05
|
< 0.05
|
0.30
|
B | (−)12.0 | (−)12.0 | (−)18.0 | (−)10.5 | ||
p-value (adjusted) |
< 0.05
|
< 0.05
|
< 0.01
|
< 0.05
| ||
Serum ferritin | ||||||
< 1000 ng/mL (n = 83) | 72.0 (±12.8) | 73.6 (±15.3) | 71.1 (±12.8) | 69.9 (±16.6) | 79.4 (±14.8) | 64.3 (±18.1) |
> 1000 ng/mL (n = 180) | 73.1 (±12.2) | 74.8 (±15.7) | 72.3 (±12.4) | 70.7 (±16.4) | 81.4 (±15.1) | 65.0 (±16.9) |
p-value |
0.49
|
0.57
|
0.49
|
0.72
|
0.30
|
0.78
|
Skin pigmentation | ||||||
No (n = 214) | 73.0 (±12.5) | 74.5 (±15.5) | 72.4 (±12.5) | 71.2 (±16.2) | 80.7 (±15.0) | 65.5 (±16.5) |
Yes (n = 57) | 72.4 (±12.4) | 74.7 (±15.5) | 70.6 (±12.8) | 68.2 (±17.0) | 81.1 (±15.8) | 63.7 (±20.2) |
p-value |
0.75
|
0.91
|
0.32
|
0.22
|
0.85
|
0.49
|
Diabetes | ||||||
No (n = 266) | 73.0 (±12.4) | 74.7 (±15.5) | 72.2 (±12.6) | 70.6 (±16.4) | 80.9 (±15.1) | 65.2 (±17.3) |
Yes (n = 5) | 66.5 (±14.7) | 68.1 (±16.5) | 66.3 (±15.1) | 68.0 (±17.5) | 73.0 (±19.2) | 58.0 (±19.5) |
p-value |
0.24
|
0.34
|
0.30
|
0.72
|
0.24
|
0.35
|
Hypothyroidism | ||||||
No (n = 260) | 73.0 (±12.5) | 74.9 (±15.4) | 72.1 (±12.7) | 70.7 (±16.4) | 80.7 (±15.3) | 65.2 (±17.0) |
Yes (n = 11) | 69.3 (±12.2) | 67.2 (±16.7) | 70.5 (10.0) | 67.2 (±16.7) | 82.2 (±11.4) | 61.4 (±23.9) |
p-value |
0.33
|
0.11
|
0.68
|
0.49
|
0.74
|
0.47
|
Short stature | ||||||
No (n = 127) | 74.8 (±13.1) | 75.9 (±16.8) | 74.4 (±13.0 | 71.2 (±17.0) | 83.9 (±14.5) | 68.0 (±17.5) |
Yes (n = 117) | 71.0 (±12.1) | 72.9 (±14.6) | 69.9 (±12.1) | 70.1 (±16.0) | 77.9 (±15.6) | 61.8 (±16.7) |
p-value |
< 0.05
|
0.14
|
< 0.01
|
0.58
|
< 0.01
|
< 0.01
|
B | (−)4.7 | (−)5.3 | (−)6.4 | (−)6.3 | ||
p-value (adjusted) | < 0.01 | < 0.01 | < 0.01 | < 0.01 | ||
Undernutrition | ||||||
No (n = 157) | 74.3 (±12.7) | 75.9 (±16.3) | 73.7 (±12.4) | 71.4 (±15.9) | 82.1 (±14.7) | 67.4 (±17.3) |
Yes (n = 85) | 70.7 (±12.4) | 72.1 (±14.6) | 69.9 (±13.1) | 69.7 (±17.4) | 79.3 (±16.2) | 60.6 (±16.9) |
p-value |
< 0.05
|
0.07
|
< 0.05
|
0.42
|
0.17
|
< 0.01
|
B | (−)3.7 | (−)3.7 | (−)6.5 | |||
p-value (adjusted) |
< 0.05
|
< 0.05
|
< 0.01
| |||
Hepatitis C infection | ||||||
No (n = 211) | 71.7 (±12.0) | 73.6 (±15.0) | 70.7 (±12.3) | 69.3 (±16.0) | 79.1 (±15.2) | 63.8 (±17.4) |
Yes (n = 60) | 77.2 (±13.1) | 77.9 (±16.8) | 76.9 (±12.4) | 74.7 (±17.2) | 86.5 (±13.8) | 69.6 (±16.3) |
p-value |
< 0.01
|
0.05
|
< 0.01
|
< 0.05
|
< 0.01
|
< 0.05
|
B | 5.5 | 6.3 | 5.1 | 7.5 | 6.3 | |
p-value (adjusted) |
< 0.01
|
< 0.01
|
< 0.05
|
< 0.01
|
< 0.05
| |
Abdominal scars | ||||||
No (n = 205) | 72.8 (±13.0) | 74.5 (±15.8) | 71.8 (±13.1) | 70.1 (±16.9) | 80.3 (±15.8) | 65.4 (±17.3) |
Yes (n = 66) | 73.2 (±10.7) | 74.8 (±14.7) | 72.7 (±11.0) | 71.8 (±14.6) | 82.1 (±13.0) | 64.1 (±17.5) |
p-value |
0.78
|
0.86
|
0.64
|
0.47
|
0.41
|
0.59
|
Thalassaemia facies | ||||||
No (n = 179) | 71.0 (±12.5) | 72.5 (±16.2) | 70.3 (±12.4) | 68.5 (±16.4) | 79.7 (±15.8) | 63.1 (±16.5) |
Yes (n = 92) | 76.5 (±11.6) | 78.5(±13.2) | 75.4 (±12.3) | 74.4 (±15.6) | 82.8 (±13.6) | 68.9 (±18.2) |
p-value |
< 0.01
|
< 0.01
|
< 0.01
|
< 0.01
|
0.10
|
< 0.01
|
B | 5.6 | 5.7 | 5.5 | 5.8 | 6.6 | |
p-value (adjusted) |
< 0.001
|
< 0.01
|
< 0.01
|
< 0.01
|
< 0.01
| |
Hearing problems | ||||||
Yes (n = 4) | 73.9 (±12.7) | 75.0 (±8.8) | 73.3 (±15.3) | 80.0 (±10.8) | 83.7 (±17.0) | 56.2 (±18.8) |
No (n = 267) | 72.9 (±12.5) | 74.5 (±15.6) | 72.0 (±12.6) | 70.4 (±16.4) | 80.7 (±15.2) | 65.2 (±17.3) |
p-value |
0.87
|
0.95
|
0.84
|
0.24
|
0.69
|
0.30
|
Visual problems | ||||||
No (n = 252) | 73.0 (±12.5) | 74.4 (±15.7) | 72.2 (±12.6) | 70.7 (±16.5) | 80.7 (±15.5) | 65.5 (±16.5) |
Yes (n = 19) | 72.0 (±12.0) | 76.0 (±11.8) | 70.0 (±12.8) | 68.4 (±15.0) | 81.3 (±9.9) | 60.0 (±25.4) |
p-value |
0.75
|
0.68
|
0.47
|
0.55
|
0.88
|
0.18
|
Elevated transaminases | ||||||
No (n = 213) | 73.2 (±12.6) | 74.4 (±15.8) | 72.6 (±12.7) | 70.2 (±16.4) | 81.5 (±15.1) | 66.3 (±17.1) |
Yes (n = 58) | 71.9 (±11.8) | 75.1 (±14.2) | 70.2 (±12.2) | 71.7 (±16.2) | 78.1 (±15.2) | 60.7 (±17.7) |
p-value |
0.48
|
0.75
|
0.20
|
0.54
|
0.12
|
< 0.05
|
B | (−)5.2 | |||||
p-value (adjusted) |
< 0.05
| |||||
Iron chelator medication | ||||||
Desferioxamine (n = 29) | 74.2 (±12.2) | 79.3 (±13.9) | 71.7 (±13.7) | 71.4 (±15.5) | 79.1 (±15.9) | 64.7 (±17.5) |
Deferasirox (n = 163) | 71.9 (±12.8) | 72.9 (±16.4) | 71.4 (±12.5) | 69.7 (±17.1) | 79.7 (±15.3) | 65.0 (±17.4) |
p-value |
0.37
|
0.05
|
0.88
|
0.61
|
0.84
|
0.92
|
Socio-demographic determinants of health-related quality of life
Total quality of life score (Mean ± SD) | Physical functioning score (Mean ± SD) | Psychosocial health summary score (Mean ± SD) | Emotional functioning score (Mean ± SD) | Social functioning score (Mean ± SD) | School functioning score (Mean ± SD) | |
---|---|---|---|---|---|---|
Education level of the mother | ||||||
Below O/L (n = 184) | 72.8 (±12.9) | 74.8 (±15.8) | 71.9 (±12.9) | 70.7 (±17.0) | 80.4 (±15.6) | 64.9 (±17.9) |
A/L or higher (n = 87) | 73.0 (±11.4) | 74.1 (±14.9) | 72.4 (±12.0) | 70.2 (±15.1) | 81.6 (±14.3) | 65.4 (±16.0) |
p-value |
0.88
|
0.73
|
0.75
|
0.84
|
0.55
|
0.84
|
Education level of the father | ||||||
Below O/L (n = 199) | 71.5 (±13.2) | 73.1 (±16.1) | 70.6 (±13.3) | 69.4 (±17.3) | 79.5 (±16.0) | 63.4(±18.3) |
A/L or higher (n = 72) | 76.8 (±9.3) | 78.6 (±12.7) | 75.9 (±9.4) | 73.6 (±13.0) | 84.3 (±11.9) | 69.8 (±13.4) |
p-value |
< 0.001
|
< 0.01
|
< 0.001
|
< 0.05
|
< 0.01
|
< 0.01
|
B | 5.1 | 5.5 | 5.1 | 3.9 | 4.7 | 6.2 |
p-value (adjusted) |
< 0.01
|
< 0.01
|
< 0.01
|
< 0.05
|
< 0.05
|
< 0.01
|
Mother’s occupation | ||||||
Housewife/ Unemployed (n = 228) | 72.9 (±12.5) | 74.7 (±15.1) | 72.0 (±12.8) | 70.4 (±16.7) | 81.0 (±15.6) | 65.0 (±17.5) |
Employed (n = 43) | 72.6 (±12.5) | 73.8 (±17.6) | 72.4 (±11.6) | 71.1 (±14.7) | 79.8 (±12.9) | 65.6 (±16.6) |
p-value |
0.85
|
0.72
|
0.84
|
0.78
|
0.64
|
0.81
|
Father’s occupation | ||||||
Unemployed/ Unskilled (n = 187) | 71.7 (±12.3) | 73.6 (±15.6) | 70.8 (±12.5) | 70.0 (±16.6) | 79.3 (±15.8) | 63.2 (±16.8) |
Skilled/ Professional (n = 84) | 75.4 (±12.4) | 76.6 (±15.1) | 74.8 (±12.4) | 71.6 (±15.9) | 83.9 (±13.2) | 69.2 (±17.7) |
p-value |
< 0.05
|
0.14
|
< 0.05
|
0.47
|
< 0.05
|
< 0.05
|
B | 3.7 | 4.0 | 4.4 | 5.7 | ||
p-value (adjusted) |
< 0.05
|
< 0.05
|
< 0.05
|
< 0.05
| ||
Monthly family income (LKR) | ||||||
< 50,000 (n = 253) | 72.6 (±12.4) | 74.2 (±15.4) | 71.7 (±12.6) | 70.4 (±16.6) | 80.4 (±15.2) | 64.5 (±17.3) |
> 50,000 (n = 17) | 77.6 (±12.6) | 79.9 (±16.1) | 76.1 (±12.3) | 71.7 (±13.4) | 85.0 (±14.6) | 72.6 (±15.8) |
p-value |
0.10
|
0.14
|
0.16
|
0.75
|
0.23
|
0.06
|
Average duration of hospital stay | ||||||
1 day (n = 41) | 75.9 (±12.2) | 77.2 (±15.1) | 75.1 (±12.5) | 71.3 (±18.7) | 83.2 (±15.9) | 71.0 (±12.3) |
> 1 day (n = 230) | 72.3 (±12.4) | 74.1 (±15.5) | 71.5 (±12.6) | 70.4 (±15.9) | 80.3 (15.0) | 64.0 (±17.9) |
p-value |
< 0.05
|
0.23
|
0.09
|
0.73
|
0.27
|
< 0.01
|
B | (−)4.2 | (−)8.5 | ||||
p-value (Adjusted) |
0.05
|
< 0.01
| ||||
Distance from home to hospital | ||||||
< 100 km (n = 206) | 74.1 (±12.3) | 75.7 (±15.3) | 73.3 (±12.4) | 71.4 (±16.1) | 81.6 (±15.2) | 67.2 (±15.7) |
> 100 km (n = 38) | 70.8 (±12.7) | 74.14.7) | 69.1 (±13.1) | 71.0 (±16.4) | 80.1 (±14.5) | 55.4 (±22.1) |
p-value |
0.13
|
0.78
|
0.06
|
0.88
|
0.59
|
< 0.01
|
B | (−)11.0 | |||||
p-value (adjusted) |
< 0.001
|