Thalassemia, a type of anemia characterized by abnormal red blood cells, is a hereditary disease caused by inheriting abnormal genes from parents. In Thailand, there are over 500,000 thalassemia carriers and more than 18 million people have been identified as potential carriers. However, with early detection and prevention, the spread of the disease can be reduced.
There are two groups of thalassemia patients: carriers who are healthy and show no symptoms, and those with obvious symptoms such as paleness, yellow eyes, fatigue, swollen stomach, and enlarged liver and spleen. Approximately 30-40% of the Thai population are carriers, with 1% representing severe thalassemia patients who are at risk of death for both the mother and child.
There are three groups of thalassemia severity syndromes. The severe syndrome mostly affects babies, with symptoms including edema, difficulty in labor, pale complexion, and enlarged liver and spleen. The very severe syndrome is asymptomatic at birth, but symptoms such as yellow eyes, fatigue, short stature, facial changes, and organ enlargement appear at 3-6 months of age. The minor severe syndrome is characterized by slight paleness, jaundice, and frequent illness.
In severe cases, blood transfusions may be required. High transfusion, used for pediatric patients with severe thalassemia, prevents abnormal blood formation and allows for healthy growth. However, iron accumulation in the body is a concern and iron pills are often prescribed. Supportive blood transfusion, on the other hand, is given when the patient is exhausted due to pallor from the original condition.
Accumulated iron from blood transfusions and excess absorption in the gastrointestinal tract can cause severe complications such as heart failure, liver damage, cirrhosis, and diabetes. Regular assessments for iron overload are necessary to prevent these complications.
Patients with thalassemia who have a serum ferritin level above 1,000 ng/ml and have been receiving regular blood transfusions for more than a year, received blood approximately 10-20 times, or have undergone splenectomy may need iron chelation tablets. Prior to splenectomy, it is recommended to receive anti-infective vaccination to prevent infection.
Hematopoietic stem cell transplantation is currently the only method that can potentially cure thalassemia diseases. However, it is not suitable for all patients due to the risks and high cost involved. Pediatric patients with Thalassemia Major who have siblings with a histological match offer the best chance of success.
Treatment for thalassemia includes a balanced diet from all food groups, avoidance of iron-rich foods, discontinuation of iron-containing drugs and vitamins, moderate exercise, regular dental check-ups, and strict adherence to prescribed folic acid.
For couples planning to have a baby, it is important to undergo a health check to determine if both spouses carry the same type of thalassemia. If so, there is a 25% chance that the child will have thalassemia disease. Consulting with a doctor during pregnancy or considering artificial insemination can help ensure the health of the baby.
It is crucial to raise awareness about thalassemia and take appropriate measures to prevent its spread. By detecting and managing the condition early on, individuals can lead healthier lives and reduce the impact of thalassemia on future generations.
Note: The information in this article has been sourced from Phayathai Hospital and the Ministry of Public Health.
Thalassemia (Thalassemia) is a type of anemia. caused by abnormal red blood cells causing chronic pallor The disease can be inherited. People with thalassemia inherit abnormal genes from their parents. As for people who carry thalassemia, they will inherit the abnormal gene from one or the other of the parents and find that from statistics inThailand has almost thalassemia cases 500,000 Thai people are thalassemia carriers, more than 18 million people can see that Thalassemia is close to you. But if detected and prevented, it can reduce infections and carriers.
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There are two groups of thalassemia patients:
carrier group Healthy, normal and perfect in everything. but hereditary thalassemia is latent and can be passed on to offspring Only a blood test in a hospital is needed to know if you are a thalassemia carrier. group with obvious symptoms such as paleness, yellow eyes, fatigue, swollen stomach, swollen liver and spleen, he must receive blood every month
30-40 percent of the Thai population, or 18-24 million people, are thalassemia carriers. who are ready to pass on the abnormality to their children as thalassemia, 1 per cent or 630,000 people Severe thalassemia patients There can be a risk of death for the mother and the child.
Three groups of thalassemia severity syndromes
severe syndromeMost of the babies are oedematous, labor difficult, pale, enlarged liver and spleen, and most of the children die in the mother’s womb or after birth . and can die The very severe syndrome at birth is asymptomatic. But at 3-6 months of age, babies will have yellow eyes, fatigue, short stature, dwarfism, enlarged liver and spleen, facial changes. The forehead rises, the cheekbones are high, the bridge of the nose is flat, and the teeth protrude. if not treated Continues to die Minor Severe SyndromeThis group is normal. slightly pale yellow Frequent illness and jaundice
When severe… blood transfusions may be required.
Blood transfusion to prevent abnormal blood formation (high transfusion) is commonly used in pediatric patients with severe thalassemia. newly diagnosed to be healthy growing up with age Iron absorption from the gastrointestinal tract is reduced, the spleen is not enlarged, and facial bones are prevented from changing. But with this method, the patient will have a lot of iron accumulated in the body. Therefore, it is necessary to have an iron pill along with it. Supportive blood transfusion (low transfusion) where blood is given only when the patient is exhausted due to pallor from the original condition. This usually happens after an infectious fever.
Patients with thalassemia have accumulated iron from the blood received (1 ml of red blood cells contains 1.16 mg of iron) and from absorption of excess iron from the gastrointestinal tract. The iron build-up can destroy the heart. It can cause heart failure, liver damage, and cirrhosis. and destroying the pancreas can cause diabetes So it is necessary to be regularly assessed for iron overload.
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Who needs iron chelation tablets? When is thalassemia?
Serum ferritin level more than 1,000 ng/ml Thalassemia patients receiving blood regularly for more than a year Thalassemia patients receiving blood about 10-20 times Treated with splenectomy Only when the doctor considers using the this method of treatment The spleen is so large that it causes pressure on other internal organs. or the size is more than 6 cm, the patient needs blood transfusions more often than before
It is recommended to receive the anti-infective vaccination at least two weeks before splenectomy. to prevent infection
Hematopoietic stem cell transplantation alternatives for thalassemia patients
This is currently the only method that can cure 75-92% of thalassemia diseases, but it cannot be used to treat all patients. Because there is a risk of harm from the treatment and the cost is very high. Patients who can be treated with this method are: Pediatric Patients with Thalassemia Major and have siblings from the same parents or if the donor has an HLA or histological match to the patient The chances of siblings from the same parents match with the patient in 1 in 4 because the donor has a 1 in 10,000 to 1 in 100,000 chance of matching with the patient
Thalassemia is treated.
Eat food from all 5 food groups, avoid foods high in iron, including blood, liver, organs. Discontinue iron-containing drugs and vitamins. In case the patient is a child he should be fully vaccinated as a normal child exercise, but moderately When you feel tired, take a break immediately. Avoid high impact exercise. Because the bones can break easily. Refrain from smoking and refrain from all types of alcoholic beverages. Because it will worsen your health. Teeth should be checked every 6 months as it can easily cause tooth decay. If you have a high fever, you should see a doctor immediately. Because it may increase the risk of serious infections Take folic acid strictly as prescribed by your doctor.
Why do I need a health check before planning to have a baby? ?
if both spouses carry the same type of thalassemia There is a 25% chance that the child will have thalassemia disease, so when pregnant a doctor should be consulted to diagnose the baby before it is born or the couple will chooses the artificial insemination method to help have children.
Thanks for information from: Phayathai Hospital and Ministry of Public Health
Photos from: Shutterstock.
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