Screening is Crucial
During the same event, Dr. dr. Teny Tjitra Sari, Sp.AK, MPH, a member of the Indonesian Pediatricians’ Association (Ikatan Dokter Anak Indonesia – IDAI)’s Hematology-Oncology Special Work Unit, emphasizes the fact that as an abnormality in the red blood cells, caused by insufficient or no production of the globin protein chains that make up primary hemoglobin, thalassemia might hinder a child’s proper growth and development. These abnormal red blood cells become fragile, which in turn leads to chronic anemia, a condition requiring patients undergo routine blood transfusions, so that they can have sufficient oxygen and nutrients needed by their bodies to survive.
“One bag of blood will insert 250 mg iron into a patient’s body, with only 2 mg a day or 60 mg a month being excreted by the body. One transfusion a month will leave the patient with 190 mg of waste iron, which will accumulate in the blood. This accumulation must be cleaned out, using chelation therapy in order to prevent the patient’s heart, liver, endocrine glands, bones, and skin from malfunctioning. Complications caused by excess iron in the body will lead to death from organ failure – about 71% because of cardiac malfunction, 12% with infections, 6% because of liver malfunction, and 11% suffering other organ malfunctions,” dr. Teny reported.
There were 10,555 thalassemia cases in 2019, rising to 10,955 cases in 2020, and again to 10,973 in 2021. In 2019-2020, West Java became the province with the biggest thalassemia rate at 3,636 cases, followed by DKI Jakarta (2,200) and Central Java (937). The nation’s biggest hospital, Cipto Mangunkusumo in Jakarta, recorded 497 cases among both children and adults, with an addition of 20-50 new patients a year in 2023. If a single patient needs 2-4 transfusions a month, then the estimated amount of blood required by the Thalassemia Center for transfusions is 4,383,309 ml – or no fewer than 22,000 bags – a year!
She went on to describe the grim reality that Sumba region, in East Nusa Tenggara, has a 1:3 thalassemia carrier rate, or about 30% of the population. As the disease is inherited, the best way to sever the thalassemia chain is by marrying a non-carrier.
“The comparison in costs between preventive efforts in the form of peripheral blood screening, or hemoglobin analysis, and curative efforts, or regular lifetime blood transfusions and chelation, is over one thousand-fold. In other words, the money spent to treat a single thalassemia patient is equal to the amount needed to screen 1,000 people for the disease. As thalassemia is extremely expensive to treat, please have yourself screened for the disease before you marry. Or marry anyway, but please have no biological children with that particular partner.” (est)