Thalassemia Carriers Differ from One Another.

Thalassemia

Thalassemia is a hereditary disease in which blood cells break down too quickly, causing anemia. In this disease, complications occur in the globin chain of hemoglobin that carries oxygen in the blood.

A child can be born as a thalassemia patient only if both the father and mother are thalassemia carriers. If either the mother or father is a carrier, there is a risk of the child becoming a carrier. Thalassemia carriers and patients are not the same.

Symptoms:


Symptoms of thalassemia begin to appear four to six months after birth. Such as paleness of the baby, weakness, frequent infections, jaundice, normal growth of the body is disturbed, the spleen and liver are slowly enlarged, puberty symptoms are not manifested, etc.

Treatment of thalassemia is complex.

  •  Bone Marrow Transplantation and Gene Therapy: Bone marrow transplantation and gene therapy are not yet practiced in our country.
  •  Regular Blood Cell Transfusion (RCC) and Iron Chelation: With regular transfusions of safe blood and proper iron chelation, a thalassemia patient can lead a normal life like 10 others. They will need some other medicines, like folic acid, vitamin C for iron extraction, vitamin D and calcium for parathyroid failure, and endocrine replacements, regularly.

Every month, a patient spends 5–15 thousand rupees in the medical sector, including blood collection and necessary medicines. Which is expensive for most thalassemia patients in Bangladesh.

Patient Food:

Carriers have no restrictions on iron-rich foods. However, a thalassemia patient who has regular blood transfusions is prohibited from consuming iron-containing foods. Liqueur tea is said to be consumed.

Blood Circulation:

  • A planned blood transfusion enables a thalassemia patient to lead an almost normal life.
  • It helps patients live longer by reducing the incidence of hypertrophied marrow-related problems and cardiac failure.
  • Moderate blood circulation reduces abnormal blood formation.
  • Just as much blood should be given as needed, so that iron overload is less.
  • Small-volume, short-interval blood transfusions should be our goal. Usually, 1 bag of blood is given every 2–4 weeks.
  • The amount of blood needed for transfusion depends on the patient’s hemoglobin level, weight, physical condition, and how much hemoglobin the doctor wants to keep for the patient, etc.

Management:

  • Regular blood donor blood should be used. Because using your own blood increases the risk of serious diseases like graft versus host disease (GVHD),
  • A blood-dependent thalassemia patient should first know their blood hemoglobin level.
  • The patient’s ABO, Rh genotype, and phenotype should be known.
  • Kell, Kidd, and Duffy antibodies should be known if possible.
  • The type of thalassemia, thalassemia major or E-beta thalassemia, should be known through an electrophoresis test.
  • Blood-enhancing drugs are effective in some cases. As a result, they need less blood. Special attention should be paid to this.
  • Red Cell Concentrate (RCC) should be used. Because using whole blood can increase the amount of fluid in the body and cause cardiac failure.
  • A leukocyte filter should be used later in cases of fever for blood transfusion.

Professor Dr. Syeda Masuma Rahman, Consultant, Safe Blood Transfusion Programme, Directorate of Health

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