Table of contents:
- Get to know thalassemia
- Symptoms of thalassemia
- The risk of thalassemia in the Kajang and Bugis tribes
Although it is still a rare disease, thalassemia is quite a lot in Indonesia. Indonesia still occupies one of the countries with the highest risk of thalassemia in the world. According to data collected by the World Health Organization or WHO, out of 100 Indonesians, 6 to 10 people have the genes that cause thalassemia in their bodies.
According to the Chairperson of the Indonesian Thalassemia Foundation, Ruswadi, quoted from Republika, until now, thalassemia major patients who constantly need regular blood transfusions have reached 7,238 patients. Of course, that's only based on data from hospitals. Apart from that, there could be some things that are not recorded so that the number is bigger.
According to Pustika Amalia Wahidayat, a doctor from the Hematology-Oncology Division of the Department of Child Health, Faculty of Medicine, University of Indonesia, quoted by Detik, countries in the Middle East, Mediterranean countries, Greece, and Indonesia are in the thalassemia belt area. This is why the number of patients is quite large.
Still according to Pustika, quoted from the Indonesian Pediatrician Association, this condition is not seen based on the number of patients. This is seen through the frequency of gene abnormalities found.
The provinces with the highest rates in Indonesia for thalassemia cases are West Java and Central Java. However, there are several ethnic groups in Indonesia that are known to have a high risk of thalassemia, namely the Kajang and the Bugis.
Get to know thalassemia
Thalassemia is a genetic disease that is passed on through the blood of the family. Gene mutations that occur make the body unable to produce the perfect form of hemoglobin. Hemoglobin located in red blood cells functions to carry oxygen. This causes the blood to be unable to carry oxygen properly.
The cause of thalassemia is caused by two types of protein that make up hemoglobin, namely alpha globin and beta globin. Based on these proteins, there are two types of thalassemia. The first is alpha thalassemia which occurs because the gene forming alpha globin is missing or mutated. The second is beta thalassemia, which occurs when genes affect the production of the protein beta globin.
Typically, alpha thalassemia attacks people in Southeast Asia, the Middle East, China, and Africa. Meanwhile, beta thalassemia is found in many areas of the Mediterranean.
Symptoms of thalassemia
Based on the appearance of symptoms, thalassemia is divided into two, namely thalassemia major and thalassemia minor. Thalassemia minor is only a carrier of the thalassemia gene. Their red blood cells are smaller, but most of them have no symptoms.
Thalassemia major is thalassemia which will show certain symptoms. If the father and mother have the thalassemia gene, their fetus is at risk of dying in late gestational age.
But for those who do survive, they will develop anemia and constantly need blood transfusions to support the need for hemoglobin in the blood.
The following are symptoms of thalassemia that are commonly found.
- Facial bone abnormalities
- Fatigue
- Growth failure
- Shortness of breath
- Yellow skin
The best prevention for thalassemia is screening before marriage. If both partners carry the thalassemia gene, it is almost certain that one of their children will have thalassemia major and will need to have a lifelong blood transfusion.
The risk of thalassemia in the Kajang and Bugis tribes
Indeed, there are several ethnic groups in Indonesia who are at risk of beta thalassemia. The Kajang and Bugis tribes have high potential. For those of you who come from the Kajang and Bugis tribes or have blood of their descendants, there is nothing wrong with screening for thalassemia.
Research conducted by Dasril Daud and his team from Hasanuddin University Makassar in 2001 shows interesting findings. This research involved 1,725 tribal people in South Sulawesi and 959 people from other ethnic groups in Indonesia such as Batak, Malay, Javanese, Balinese, Sumba, and Papuan. There were 19 people with beta thalassemia in the Bugis and Kajang tribes, but none from other tribes.
In 2002, a research was also conducted and the result was that there was a frequency of beta thalassemia gene carriers in the Bugis tribe of 4.2%.
According to Sangkot Marzuki in his book Tropical Disease: From Molecule to Bedside, based on the investigation found mutations in the beta globin gene in the Bugis tribe that were not found at all in the Javanese.
Why is the high risk of thalassemia in the Kajang tribe? It could be that this has something to do with the customs of the Kajang tribe. The indigenous people of the Kajang tribe, located in Bulukumba Regency, South Sulawesi, have a binding custom to marry other people in their customary area. If not, they have to live outside their customary area. Therefore, fathers and mothers from the same customary area are more likely to give birth to children with thalassemia. After that the child will marry someone who has the same condition.
However, there has been no further research on this matter. Experts are still monitoring and studying the risk of thalassemia in the two tribes in Indonesia so that they can take effective preventive and treatment measures.
