ISBT Science Series (2020) 0, 1–8
CONGRESS REVIEW
© 2020 International Society of Blood Transfusion
DOI: 10.1111/voxs.12544
An insight into Indonesian current thalassaemia care and
challenges
Pustika Amalia Wahidiyat,
1
Teny Tjitra Sari,
1
Ludi Dhyani Rahmartani,
1
Iswari Setianingsih,
2
Stephen Diah Iskandar,
3
Anastasia Michelle Pratanata,
3
Ivana Yapiy,
3
Mikhael Yosia
1
& Fernando Tricta
4
1
Department of Child Health, Faculty of Medicine, Universitas Indonesia-Cipto Mangunkusumo Hospital, Jakarta, Indonesia
2
The Eijkman Institute for Molecular Biology, Jakarta, Indonesia
3
Faculty of Medicine Universitas Indonesia, Jakarta, Indonesia
4
ApoPharma Inc., Toronto, Ontario, Canada
Received: 30 December 2019,
revised 30 January 2020,
accepted 4 February 2020
Thalassaemia is one of the most prevalent inherited blood disorders in Indonesia,
with highly diverse mutations ranging from mild to severe that can be found
across the nation. Nevertheless, thalassaemia management in Indonesia is still
limited to supportive treatment, such as blood transfusion, iron chelation, com-
plications monitoring, psychosocial support and a comfortable transition from
child to adult clinic. However, these managements are still suboptimal in most
parts of the nation. Indonesia still has a long way from implementing the optimal
curative treatment for thalassaemia. Iron chelators are sometimes not available,
especially in rural areas. The cost for optimal dosages of iron chelation also can-
not be fully covered by the current national health insurance scheme. However,
it still benefits our patients, considering it is the only treatment to decrease iron
deposition in organs. With that situation, our patients, both paediatric and adults,
have normal cardiac haemosiderosis, moderate-to-severe hepatic haemosiderosis
and normal to mild pancreatic haemosiderosis. Therefore, the number of deaths,
especially those due to heart failure and infection, was significantly reduced. An
improvement in thalassaemia supportive treatments is in line with the increase in
patients’ life expectancy. Without curative treatment options, the lifelong cost for
treatment will extremely burden the national health budget. To date, thalas-
saemia stands for the 5th most costly disease in Indonesia. Therefore, a screening
programme must be realized soon; hence, the treatment cost can be allocated for
initiating the transplant unit or improving other important areas.
Key words: blood transfusion, complications, epidemiology, Indonesia, iron chela-
tion, thalassaemia.
Introduction
Thalassaemia is a hereditary condition where a mutation
in the globin gene gives rise to depletion or complete
absence of either a-globin or b-globin chain of haemo-
globin, leading to a-thalassaemia and b-thalassaemia,
respectively [1,2]. It was first discovered among people
living around the Mediterranean Sea by Dr. Cooley and
Lee dating back almost a century ago [3]. This geographi-
cal pattern is now known as ‘thalassaemia belt’ which
spans countries from the Mediterranean ocean to Asia.
With the increasing number of migrations, thalassaemia
cases are not limited to its initial niche but also expand-
ing to America and Australia, contributing to a global
health problem that escalates along with its improving
survival [2,4].
Worldwide, it is estimated that 320 000 babies are born
with haemoglobinopathies each year, and 80% of these
Correspondence: Pustika Amalia Wahidiyat, Division of Hematology and
Oncology, Department of Child Health, Cipto Mangunkusumo Hospital -
Faculty of Medicine Universitas Indonesia, Jalan Pangeran Diponegoro
No. 71, Kenari, Senen, Jakarta Pusat 10430. E-mail:
pa.wahidiyat@gmail.com
1