Introduction
Methods
Numbers of deaths
Causes of deaths
Calculation of death rates
Calculation of life-expectancy
CMR
Deployment of deferiprone and CMR
Results
Survival analysis
Diagnosis | Alive | Dead | Total |
---|---|---|---|
Beta thalassaemia major | 644 | 206 | 850 |
Beta thalassaemia intermedia | 138 | 20 | 158 |
Hb E/beta thalassaemia | 73 | 7 | 80 |
Alpha thalassaemia major | 1 | 0 | 1 |
Total | 856 | 233 | 1,089 |
Period | |||||||
---|---|---|---|---|---|---|---|
Cause of death
| 1950 – 59 | 1960 – 69 | 1970 – 79 | 1980 – 89 | 1990 – 99 | 2000 – 03 | Total |
Iron overload | 1 | 21 | 37 | 46 | 6 | 111 | |
Anaemia | 6 | 17 | 9 | 1 | 33 | ||
BMT complication | 5 | 15 | 1 | 21 | |||
Infection | 1 | 4 | 5 | 6 | 3 | 19 | |
Malignancy | 1 | 1 | 2 | 1 | 5 | ||
Other | 2 | 2 | 8 | 4 | 16 | ||
Unknown | 1 | 1 | |||||
TOTAL | 6 | 22 | 37 | 56 | 74 | 11 | 206 |
% iron overload
|
0
|
4.5
|
56.8
|
66.1
|
62.2
|
54.5
|
53.9
|
CMR and iron chelator
Death rates
All Causes | Patient years | Deaths, all causes | |||||||||||||
---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
Year of birth | Births | Before 1960 | 1960–69 | 1970–79 | 1980–89 | 1990–99 | 2000–03 | Total | Before 1960 | 1960 – 69 | 1970 – 79 | 1980 – 89 | 1990 – 99 | 2000 – 03 | Total |
Before 1960 | 55 | 263 | 454 | 312 | 186 | 140 | 48 | 1403 | 6 | 7 | 17 | 10 | 3 | 0 | 43 |
1960–69 | 166 | 842 | 1,452 | 1,205 | 948 | 346 | 4,793 | 15 | 15 | 32 | 19 | 2 | 83 | ||
1970–79 | 216 | 1,107 | 2,081 | 1,814 | 665 | 5,667 | 5 | 8 | 35 | 3 | 51 | ||||
1980–89 | 212 | 1,234 | 1,982 | 750 | 3,966 | 6 | 15 | 5 | 26 | ||||||
1990–99 | 172 | 952 | 683 | 1,635 | 2 | 1 | 3 | ||||||||
2000–2003 | 29 | 92 | 92 | 0 | 0 | ||||||||||
TOTAL | 850 | 263 | 1,296 | 2,871 | 4,706 | 5,836 | 2,584 | 17,556 | 6 | 22 | 37 | 56 | 74 | 11 | 206 |
All Causes | Deaths, all causes per 1,000 patient years | |||||||
---|---|---|---|---|---|---|---|---|
Year of birth | Births | Before 1960 | 1960 – 69 | 1970 – 79 | 1980 – 89 | 1990 – 99 | 2000 – 03 | Total |
Before 1960 | 55 | 22.8 | 15.4 | 54.5 | 53.8 | 21.4 | 0.0 | 30.6 |
1960–69 | 166 | 17.8 | 10.3 | 26.6 | 20.0 | 5.8 | 17.3 | |
1970–79 | 216 | 4.5 | 3.8 | 19.3 | 4.5 | 9.0 | ||
1980–89 | 212 | 4.9 | 7.6 | 6.7 | 6.6 | |||
1990–99 | 172 | 2.1 | 1.5 | 1.8 | ||||
2000–2003 | 29 | 0.0 | 0.0 | |||||
TOTAL | 850 | 22.8 | 17.0 | 12.9 | 11.9 | 12.7 | 4.3 | 11.7 |
Iron overload | Patient years | Deaths from iron overload | |||||||||||||
---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
Year of birth | Births | Before 1960 | 1960–69 | 1970–79 | 1980–89 | 1990–99 | 2000–03 | Total | Before 1960 | 1960 – 69 | 1970 – 79 | 1980 – 89 | 1990 – 99 | 2000 – 03 | Total |
Before 1960 | 55 | 263 | 454 | 312 | 186 | 140 | 48 | 1403 | 0 | 1 | 14 | 6 | 2 | 0 | 23 |
1960–69 | 166 | 842 | 1,452 | 1,205 | 948 | 346 | 4,793 | 0 | 7 | 27 | 15 | 1 | 50 | ||
1970–79 | 216 | 1,107 | 2,081 | 1,814 | 665 | 5,667 | 0 | 4 | 23 | 1 | 28 | ||||
1980–89 | 212 | 1,234 | 1,982 | 750 | 3,966 | 0 | 6 | 4 | 10 | ||||||
1990–99 | 172 | 952 | 683 | 1,635 | 0 | 0 | 0 | ||||||||
2000–2003 | 29 | 92 | 92 | 0 | 0 | ||||||||||
TOTAL | 850 | 263 | 1,296 | 2,871 | 4,706 | 5,836 | 2,584 | 17,556 | 1 | 21 | 37 | 46 | 6 | 111 |
Iron overload | Deaths from iron overload per 1,000 patient years | |||||||
---|---|---|---|---|---|---|---|---|
Year of birth | Births | Before 1960 | 1960 – 69 | 1970 – 79 | 1980 – 89 | 1990 – 99 | 2000 – 03 | Total |
Before 1960 | 55 | 0.0 | 2.2 | 44.9 | 32.3 | 14.3 | 0.0 | 16.4 |
1960–69 | 166 | 0.0 | 4.8 | 22.4 | 15.8 | 2.9 | 10.4 | |
1970–79 | 216 | 0.0 | 1.9 | 12.7 | 1.5 | 4.9 | ||
1980–89 | 212 | 0.0 | 3.0 | 5.3 | 2.5 | |||
1990–99 | 172 | 0.0 | 0.0 | 0.0 | ||||
2000–2003 | 29 | 0.0 | 0.0 | |||||
TOTAL | 850 | 0.0 | 0.8 | 7.3 | 7.9 | 7.9 | 2.3 | 6.3 |
Deaths per 1,000 patient years (95% confidence Interval) | |||||||
---|---|---|---|---|---|---|---|
Decade | All deaths | Iron overload deaths | Deaths other causes | Patient years | All deaths | Iron overload deaths | Deaths other causes |
Before 1960 | 6 | 0 | 6 | 263 | 22.8 (8.4,49.7) | 0.0 (0.0,14.0) | 22.8 (8.4,49.7) |
1960–69 | 22 | 1 | 21 | 1,296 | 17.0 (10.6,25.7) | 0.8 (0.0, 4.3) | 16.2 (10.0,24.8) |
1970–79 | 37 | 21 | 16 | 2,871 | 12.9 (9.1,17.7) | 7.3 (4.5,11.1) | 5.6 (3.2,9.1) |
1980–89 | 56 | 37 | 19 | 4,706 | 11.9 (9.0,15.4) | 7.9 (5.5,10.8) | 4.0 (2.4,6.3) |
1990–99 | 74 | 46 | 28 | 5,836 | 12.7 (10.0,15.9) | 7.9 (5.8,10.5) | 4.8 (3.2,6.9) |
2000–2003 | 11 | 6 | 5 | 2,584 | 4.3 (2.1,7.6) | 2.3 (0.9,5.1) * | 1.9 (0.6,4.5) |
TOTAL | 206 | 111 | 95 | 17,556 | 11.7 (10.2,13.5) | 6.3 (5.2,7.6) | 5.4 (4.4,6.6) |
1980–99 | 130 | 83 | 47 | 10,542 | 12.3 (10.3,14.6) | 7.9 (6.3,9.8) | 4.5 (3.3,5.9) |
2000–03 | 11 | 6 | 5 | 2,584 | 4.3 (2.1,7.6) | 2.3 (0.9,5.1) * | 1.9 (0.6,4.5) |