An insight into Indonesian current thalassaemia care and challenges

Abstract

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, complications 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 cannot 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, thalassaemia 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.

Skrining pranikah untuk pencegahan thalassemia mayor yang efektif dan efisien.

“…Penyakit ini tidak dapat disembuhkan, sehingga bagi penderita thalassaemia mayor memerlukan transfusi darah secara rutin untuk menunjang kelangsungan hidupnya [1; 2]. Di Seluruh dunia, diperkirakan 320.000 bayi lahir dengan Hemoglobinopati setiap tahunnya, dan 80% terjadi di negara berpenghasilan rendah atau menengah, Indonesia merupakan salah satu negara yang termasuk golongan tersebut [1], dengan jumlah populasi penduduk Indonesia lebih dari 270 juta jiwa [3]. Sekitar 5 % dari populasi diperkirakan membawa gen thalassaemia.…”

Study protocol for a cross-sectional study on knowledge, attitude, and practice towards thalassemia among Indonesian youth

“…The copyright holder for this preprint this version posted April 16, 2021. ; https://doi.org/10.1101/2021.04.11.21255264 doi: medRxiv preprint cases into zero cases following a mandatory national thalassemia prevention program which includes screening and prenatal diagnosis [17]. Similar preventive approach may even offer greater benefits to low-to-middle-income countries including Indonesia, where definitive treatments (i.e., bone marrow transplant, hematopoietic stem cell therapy) and supportive treatments (i.e., blood transfusion, iron chelating drugs) for thalassemia remain either expensive, not readily available, or associated with poor compliance [6,18]. The Indonesian Ministry of Health claimed that funding for thalassemia treatment accounted for USD 149 million between 2014 and 2018, putting it as the fifth most expensive spending from the total healthcare budget.…”

“…As stated by Indonesian Ministry of Health, screening for thalassemia aims to identify carriers and inform them about the chances of having a thalassemia-affected offspring as well as the preventive measures. To date, only Jakarta, the capital city of Indonesia, mandates mean corpuscular volume (MCV) and mean corpuscular hemoglobin (MCH) examinations prior to marriage [6]. It is currently unclear whether the Indonesian government plans to extend this policy about mandatory premarital screening to other regions [20].…”

“…However, since Indonesian law currently does not allow abortion following antenatal screening, antenatal screening is not a feasible solution. This dilemma has led experts in Indonesia to suggest that it may be best to conduct thalassemia screening programs on school-aged children and university students, so they can consider potential marriage partners from an early age [6].…”

“…Moreover, the data from 2019 showed that Indonesia has around 10,500 diagnosed cases across the country, and it is projected that the number could reach 20,000 by 2020. Nevertheless, these numbers are likely underestimated since many thalassemia cases in Indonesia remain undiagnosed [5,6].…”