Thalassemia (Thalassemia) is a hereditary blood disease. Hemoglobin is a very important component of blood. The oxygen that we carry with breathing, the work of hemoglobin is carried on all parts of the body. There is a defect in the production of these hemoglobin particles transmitting oxygen to the blood. Hemoglobin is produced by two alpha proteins and two beta proteins.
If the production of these proteins decreases, then the production of hemoglobin in the body also decreases and thalassemia occurs. Alpha and beta proteins are made from genes. Synthesis of the two group’s hemoglobin chain is basically genetically controlled. Thalassemia occurs mainly when someone receives a defective gene from their parents in their genealogy.
Types of Thalassemia
Thalassemia is mainly of two kinds. Such as:
The alpha thalassemia section (chain) is formed by four genes. We get this gene from parents from two to each. Alfa thalassemia occurs when one or more of these genes is defective. The more serious the gene is, the more serious the problem will be.
There are four types of alpha thalassemia, such as:
Carrier or carrier: If a gene is defective, there will be no signs and symptoms of thalassemia. However, the infected person will act as the carrier and the affected person can spread the disease to his child.
Alpha Thalassemia Minor: If two genes are defective, then light symptoms can be seen. This condition is called alpha-thalassemia minor or alpha-thalassemia trait. It can not be seen in any of these symptoms. However, the affected person may have light anemia, which can easily be found to be weak.
Hemoglobin H. Disease: If the three genes are defective, its symptoms are medium to severe. This condition is called hemoglobin H. Disease. This results in anemia and may lead to further complications.
Alpha Thalassemia Major: If four genes are defective, then Alpha Thalassemia major or Hydrops fetalis. Due to this, delivery may occur before the child is severely infected with anemia, or a baby may die after birth or the child may have to undergo a blood transfusion treatment.
Beta Thalassemia is made up of chains, with two genes. We get two genes from each of our parents. Beta-thalassemia occurs when one or both genes are defective.
Beta-thalassemia can be of three types:
Beta Thalassemia Minor: If a gene is defective, then mild symptoms can be seen. This condition tells of beta-thalassemia major or beta-thalassemia trait. This can lead to light anemia and its carrier can spread the disease among her child.
Beta thalassemia intermedia: If two genes are defective, sometimes there may be symptoms of moderate encephalitis. This is called beta thalassemia intermedia.
Beta Thalassemia Major: Fatal symptoms are seen when two genes are defective. This condition is called beta-thalassemia major or Cooley’s anemia. Newborn babies who have this problem are very healthy during birth. But its symptoms are seen within the first two years of birth. If they are not treated, they may cause liver, bone and heart problems.
Thalassemia Miner does not usually have any major symptoms except for anemia or anemia. Thalassemia Major is usually pronounced with symptoms in two years of childbirth. Symptoms include:
- Make more cry.
- A little bit scary.
- More infectious diseases are more affected.
- Hunger strike or unwillingness to eat.
- Plagiarism Grows.
- The face is pale.
- The skin is yellow (jaundice).
- Mouth bone distortion.
- Slow physical growth.
- Stomach stretches outward or growing.
- Dark color urine.
How is thalassemia diagnosed?
If doctors suspect Thalassemia by diagnosing the history, signs, and physical examination of the disease, then see the complete blood count and see the amount of hemoglobin in the blood. If necessary, hemoglobin electrophoresis test is also done.
X-rays are seen to be damaged by the bone. Usually, the X-ray of the head and face is done. Chest X-ray exams have many times seen ribs bone distortion. Many of the heart increases in size.
Red blood cell levels in the blood through blood tests, changes in the size of red blood cells, pale red blood cells, red blood cells contain hemoglobin, Assam, iron, and iron the baby’s blood, the amount of hemoglobin is known.
It is possible to determine if there is a patient with thalassemia through DNA examination or whether the patient is carrying a hemoglobin genome. Thalassemia is initially suspected and subsequently monitored by some symptoms and blood tests. For example, blood-type blood count (CBC), blood film (PBF) etc.
Who is more at thalassemia risk?
Having Thalassemia in the Family: If you have a thalassemia in your family/family, you are at risk of this disease.
Certain nations include: Thalassemia is more common among some people such as South Asia, Southeast Asia, Africa, Italy, Greek, and Middle Eastern people.
If both parents and parents are not carriers of thalassemia, then children cannot be born with thalassemia. If parents or parents are the thalassemia carriers, then there will be 50 percent chance that the child will be the thalassemia-like mother or father. But this disease will not be affected.
If both parents and mothers are the thalassemia carriers
- In 25 percent of cases, their child may develop thalassemia due to Major Disease.
- 50 percent of which can be born as carriers, no symptoms can be expressed.
- 25 percent can be born as healthy children.
What complications may arise due to thalassemia?
Increase in Iron: Increased amount of iron in the blood due to blood transfusion or disease. If the number of iron increases in blood, it damages the heart, liver and endocrine system.
Increase the risk of infection: Thalassemia patients increase the risk of infection. Especially those who have been expelled from the body, this risk is much more. Because the limb fighting with infections is no longer in the body. Due to blood transfusion, various types of blood-borne diseases such as hepatitis are infected.
Bone Problems: The bone gets stretched and the bone stretches out to become thin and fragile. It is more likely to break the bones.
Problems of Heart Problems: Heart-to-heart problems can occur due to severe thalassemia. This can also lead to heart failure.
Spleen or Spleen: Playa protects our body from infection and filters out old and had blood clots. Because of the thalassemia, there is a lot of red blood cells in the human body, so Spleen has to do a lot of work. This results in spleen enlargement.
Baby growth is low: In the case of a healthy-normal child, where 70-80% of the amount of fatty hemoglobin in the blood is born, the amount of hemoglobin in the blood decreases to 2-3% in less than six months after the baby is born. So the child becomes anemic. Besides, the age at which children want to stand, learn to sit and talk, is not in accordance with these normal development rules.
Thalassemia major patients should be given regular blood for the treatment of hemoglobin deficiency in the blood so that the level of hemoglobin is above 9 grams/deciliter (56/56%).Separate blood components for thalassemia patients only to give red cells or red cells. For thalassemia intermediate, most patients do not require regular blood. Many patients do not have to donate blood. Be sure to confirm thalassemia type before beginning blood transfusion.
Beta-thalassemia major patients usually have to donate blood after 2 to 4 weeks. If Thalassemia Intermedia and E Beta Thalassemia, if there is a severe anemia like Thalassemia Major, after 2 to 4 weeks, otherwise blood will be given more.
In order to find additional iron deposited due to regular blood transfusion in the body of thalassemia patients, it is necessary to receive regular iron ovens such as Desefral, Kelfber. Also, a new mouth-watering drug called Deferasirox is now being used. Any medicines are to be consumed according to the doctor’s advice.
Iron heart disease is the main cause of death due to thalassemia. International research has found that in the long-term treatment, Kelfber has more heart disease protection than Desefral. Due to this, joint treatment recommendations of Desafral and Kalfour were made. Research has shown that the effectiveness of each drug in joint treatment increases with therapeutic and it is possible to effectively eliminate iron.
Replacement of bone marrow
Thalassemia can be cured by bone marrow transplantation. Since it is a congenital problem, if its genetic problem is changed, that is, the organ that has problematic hemoglobin is created, it is possible to completely cure Bomnarro (bone marrow), if it can be modified by bone marrow, it can be completely cured. For this process, a match requires an athlete who is rare. The process is also risky, costly and not successful in cent percent.
Another process is to collect stem cells from Cord Blood Transplantation (newborn’s placenta or embryo and ambulatory cord). Most recently this process has been used successfully in the treatment of the therapeutics of thalassemia in some countries of the world. Research is also underway on gene therapy and it is expected that it will make a breakthrough in future treatment or treatment of thalassemia.
Folic Acid Supplement
The child may need folic acid supplements to prevent encephalitis caused by thalassemia. Folic Acid Hole Vitamin B, which is essential for the normal and healthy structure of each blood cell.
To prevent thalassemia
Prevention of thalassemia is very possible. This is a preventable disease. If both husband and wife are the thalassemia carriers or a thalassemia carrier and a hemoglobin e, then there is the possibility of child thalassic. If any of the two husband and wife are completely healthy, then there is no possibility of being newborn’s thalassemia. However, the newborn may be the thalassemia carrier, which is not a disease. Therefore, discouraging and preventing marriage between the carriers of the disease can reduce the birth of new thalassic children in society.
Thalassemia is expanding rapidly in these countries due to non-awareness of genetic disease and marriage between relatives in Bangladesh, Pakistan. Thalassemia is born in about eight to ten thousand children every year in our country. Meanwhile, there are more than three and a half million children in the country. There are approximately 1.5 million people in this country. So, everyone should be cautious about preventing this disease. If we want, we can eliminate this disease completely from our society.
What should be the life of thalassemia affected people?
- Regular blood tests.
- Try to keep the amount of hemoglobin in the blood 10 g or deciliter.
- Immunization of meningitis and hepatitis B virus.
- Examine height, weight, liver function every three months in the child’s field.
- After blood transfusion of eight to 10 bags, it is necessary to determine the amount of iron in the blood.
- Observe the child’s intelligence and development every year.
- If the blood level of iron is above one thousand nanograms or milliliters, then the patient is discharged.
- Ensure pure blood filtration.
- Keep in mind that children are not infected.
- To protect the body bone, adequate vitamin D calcium-rich foods should be taken. Do not give iron-rich vitamins without consulting the doctor.
Regular hands should be kept clean and washed with soap.