Imagine you have a disease in which your red blood cells last less than a month
, as opposed to a normal person’s which last for three months. Imagine that he needs blood transfusions for as long as two years to have a good life. But for many Malaysians, this is their destiny. In fact, about 5% of the population suffers from thalassemia, an inherited blood disorder that dramatically changes a person’s lifestyle. To mark World Thalassemia Day, FMT Lifestyle spoke to hematologist Dr Henning Loo about this common hereditary blood disorder.
Loo explained that thalassemia is a red blood cell disorder in which the patient’s hemoglobin production is affected by a deletion or mutation in their alpha or beta gene. Hemoglobin is a protein found in red blood cells that carries oxygen to various parts of the body. “If there is no beta chain, we call it beta thalassemia. Absence of the alpha chain is called alpha thalassemia,” he said. In patients with thalassemia, their red blood cells break down more quickly than expected. Normal red blood cells can last about three months, but a thalassemia patient’s cells have a delayed lifespan. Since a person’s hemoglobin is made up of two beta chains and four alpha chains, the lack of either is diagnosed as alpha or beta thalassemia, based on the chain deficiency. Loo added that when it comes to the severity of this disorder, it depends on whether a person suffers from thalassemia minor or thalassemia major.
“Patients with only the thalassemia trait usually do quite well and can live normal lives. However, when they get sick, they may experience symptoms of anemia like fatigue, reduced exercise tolerance, and even palpitations. They may also develop gallbladder stones and jaundice, he added. Thalassemia major patients, on the other hand, require lifelong blood transfusions starting at age two because their red blood cells last less than a month. These bloods should also be pumped regularly.
Unfortunately, blood transfusions often carry risks. First, it is concerned about the mass of iron in their hearts are necessary as their hearts, to fill them with their gland.
When this happens, there is a problem of many organs dysfuunton, which makes the patient want to cover the metal of iron. This blood is common is common among the Chinese and people, as some people in the Saraawak. “Regardless of the type or type of thalassemia a person has, it takes a family history of thalassemia for this to happen.”
Loo also said that it is feared that a carrier of thalassemia will not be able to have children with thalassemia disease again, due to the fear that their children will also be born with the disease.
“Although this is true, with advances in technology, even two women with thalassemia traits can still get married and have children.” However, he stressed that they would need the help of a vet, who would extract the right sperm and eggs from them. parents and their fertilization in vitro, after which it is transferred back to the mother’s uterus. Loo said this process would prevent the baby from getting thalassemia major. The other alternative would be to conceive naturally and obtain a sample of amniotic fluid for testing. Overall, Loo concluded that the public should be aware of this blood disorder to reduce the chances of giving birth to babies with thalassemia major. “Previously, thalassemia patients only lived 20 or 30 years, but today,
with better medical treatment, they can live up to 40 or 50 years. However, this is still quite short compared to the adult population normal.
