Thalassemia is a blood disorder that is inherited from parents to children. Thalassemia affects the ability to produce hemoglobin which results in anemia.
Hemoglobin is a protein in red blood cells that carries oxygen and other nutrients to other cells in the body. This disease is inherited by parents who have a mutated gene or thalassemia gene mutation. A child who inherits a mutation gene is called a carrier or carier , or also called thalassemia trait (thalassemia).
Some Thalassemia Classifications
Thalassemia classification, namely: Thalassemia minor, intermediate and major. Children with thalassemia major show symptoms of the disease at the age of the first two years. This child looks pale, lethargic and has a low appetite, which causes stunted growth. People with this disease need blood transfusion and intensive care or antibiotic injections are needed.
Routine blood transfusion keeps hemoglobin levels close to normal. However, repeated blood transfusions also have the side effect of iron deposition in the body that can cause damage to the liver, heart and organs, so that in children with thalassemia who get blood transfusions routinely or repeatedly indicated to be given therafi desperal. Disferal therapy is a liquid drug that is given under the skin which has the aim to prevent the accumulation of FE in the body either hemochromatosis (accumulation of FE under the skin) or hemosiderosis (accumulation of FE in the organ). This installation is given 4-7 times per week after blood transfusion.
Now, let’s get to know a little more about blood
Blood is a liquid that is found in all living things (except plants) which functions to send substances and oxygen needed by the body, transporting chemicals produced by metabolism and also as a body’s defense against viruses or bacteria. While blood transfusion in language comes from English, namely ” Blood transfusion “, which means moving blood pour. Whereas in terms of blood transfusion is the process of transferring or entering blood from a donor into the recipient’s body (patient) through a vein that matches his blood type to save lives.
The purpose of the transfusion is:
Restores less blood volume.
Increase friction / blood components that are lacking
And we know about blood is “Blood Type”
Sometimes we ask friends, what is your blood type? Usually related to the zodiac, funny funny about it. Heh heh .. but of course in the context of serious blood type questions are important when we want to donate blood, need blood donors even when at the Blood Bank or PMI. For this reason, what is familiar to us is the ABO system
- First discovered by Karl Landsteiner 1901
- An antigen which is very important for blood type
- Each person’s serum contains antibodies to antigens that are not present in their red blood cells.
- Giving ABO incompatible blood transfusions causes intravascular hemolysis.
- Hemolytic reactions can occur in compatible cross-tests
in the chart can be seen like this (of course you still remember it, biology lesson?):
|Antigen cell typing||Serum typing||Interpretation|
|Anti-A||Anti-B||Anti-AB||Cell A||Cell B||Cell O||Blood Type|
There’s more according to Rhesus. Lah, what is that?
Rhesus or Rh is another antigen found in red blood cells. The term Rh is derived from “rhesus” , because this antigen was first discovered in 1940 by Landsteiner and AS Wenner in the blood of Mocacus rhesus apes . Blood cells that have Rh antigens are called Rh + (Rhesus positive), whereas those without Rh antigens are called Rh- (Rhesus negative). If a person who has Rh negative blood is transfused with Rh positive (Rh +) blood, the Rh negative (Rh-) blood group will immediately form anti-Rh antibodies, resulting in blood agglutination.
Problems will arise if a mother who is Rh negative has a baby with Rh positive blood. Even though the maternal and child circulatory system is separate, there are often very few blood cells that enter the maternal circulatory system through the placenta. This event usually occurs at the last moment of pregnancy. In response to these foreign blood cells, maternal blood will form antibodies. These antibodies enter the baby’s circulatory system through the placenta. Baby’s blood is a foreign protein (antigen) for antibodies, so antibodies will react to the baby’s blood, resulting in agglutination. The presence of agglutination in blood cells will cause anemia, and the name of the disease is called foetalis erythroblastosis. If this disease cannot be treated, the baby can die. The following is a table of Rhesus blood groups with Antigen / Agglutinogen and Aglutinin / Antibody
|Rhesus Positive (Rh +)||there is a Rhesus antigen||there are anti-Rh antibodies|
|Rhesus negative (Rh -)||no Rhesus antigen||no anti-Rh antibodies|
In WHO guidelines (Sibinga, 1995) stated:
- Transfusions should not be given without strong indications.
- Transfusion is only given in the form of a replacement blood component that is missing / lacking.
Based on the above objectives, blood transfusions tend to use blood components according to their needs. For example the need for red blood cells, granulocytes, platelets, and blood plasma that contains protein and clotting factors. Guidelines are needed in administering blood components to patients who need them, so that the side effects of transfusion can be reduced to a minimum. Lansteiner, the pioneer of transfusion said: “Blood transfusion must not be given, unless the benefits outweigh the risks” .
In anemia, a new transfusion is appropriate if the patient shows “Oxigen Need” signs of tightness, dizziness, palpitations, dizziness, anxiety or Hb <6 g / dl. Provision of red blood cells, often used if the Hb level is less than 6 gr%, and almost not necessary if the Hb is more than 10 gr% and if the Hb level is between 6-10gr%, then the red blood cell transfusion is an indication of the patient’s oxygenation. It is important to remember that Hb levels are not the only parameter, but physiological factors and surgical risks that affect oxygenation of the patient must also be considered. Losses of up to 30% EBV can generally be treated with electrolyte fluids alone. Losing more than that, after being given electrolyte fluids need to be continued with transfusion if Hb <8 g / dl.
Habibi et al. Gave a clue that giving one unit of PRC would increase hematocrit 3-7%. The indications are:
- Blood loss> 20% and blood volume more than 1000 ml.
- Hemoglobin <8 gr / dl.
- Hemoglobin <10 g / dl with major diseases: (for example, emphysema, or ischemic heart disease).
- Hemoglobin <10 gr / dl with autologous blood.
- Hemoglobin <12 gr / dl and depends on the ventilator.
It can be mentioned that blood hemoglobin (Hb):
- Hb around 5 is CRITICAL
- Hb around 8 is TOLERABLE
- Hb around 10 is OPTIMAL
Five common indications of blood transfusion:
- Acute blood loss / acute anemia, if 20-30% of the total volume of blood is lost and bleeding continues and is preceded by fluid volume replacement.
- Severe and chronic anemia if HB cannot be increased by other means.
- Septic shock (if intravenous fluids are unable to cope with impaired blood circulation and in addition to giving antibiotics)
- Provides plasma and platelets in addition to clotting factors, because there are no other specific blood components
- Exchange transfusion in neonates with severe jaundice.
Okay we skip first until there. We return to Thalassemia
Thalassemia is a hereditary disorder in the form of a deficiency of one of the globin chains in hemoglobin so that it can cause erythrocyte immature (rapid lysis) and cause anemia.
- Thalassemia minor, usually does not show clear clinical symptoms, mild anemia
- Intermediate thalassemia, characterized by splenomegaly and anemia that appears at the age of 2-4 years, thus requiring blood transfusion.
- Thalassemia major, usually characterized by the appearance of symptoms of face cooley, hepatosplenomegaly, severe anemia, growth disorders and bone deformity, where these symptoms appear earlier since the age of 2-12 months and are very dependent on blood transfusions.
And we know that inevitably, handling children with Thalassemia is a blood transfusion, with transfusions it is hoped we can replace the blood elements that cannot be produced by the body from people with thalassemia. And indeed, like lifestyle changes, transfusion is a routine for sufferers, so many benefits are felt so that they can work as before.
Care must be taken when choosing the right blood, body condition and laboratory results. In addition, we also need to know the side effects of blood transfusion in the long term.
Complications of routine (repeated) blood transfusions:
Blood transfusion needed by thalassemia clients in the form of PRC (Packed Red Cell), which is given routinely every time the client’s Hb levels fall below normal (<10 mg / dl) as much as 10-20 cc / kgBB.
Giving blood transfusions will cause the breakdown of Hb that produces Fe needed for the formation of new erythrocytes, but by giving blood transfusions routinely (repeatedly) will cause complications from the breakdown of excess Hb which can produce excessive amounts of Fe so that the remaining Fe will accumulate or buried in the human body, including:
- Hemosiderosis , which is the accumulation of Fe in the good organs in the liver (resulting in hepatomegaly), spleen (resulting in splenomegaly), heart, pancreas, or hypofise glands (decreased growth hormone).
- Hemocromatosis , which is the accumulation of Fe under the skin so the skin color appears grayish black.
The accumulation of Fe can be reduced or prevented by giving chelating agent that is by installing desferal, where the excess Fe will be wasted through urine and feces.
The conclusions or conclusions are:
Blood transfusion in language comes from English, which is ” Blood transfusion “, which means the transfer of blood. Whereas in terms of blood transfusion is the process of transferring or entering blood from a donor into the recipient’s body (patient) through a vein that matches his blood type to save lives.
The purpose of blood transfusion is to restore the volume of blood that is lacking, adding friction / components of the blood that is lacking and as a therapeutic action in certain cases one of them in the case of thalassemia.
Routine blood transfusion can maintain hemoglobin levels and close to normal. However, repeated blood transfusions also have the side effect of iron deposition in the body that can cause damage to the liver, heart and other organs, so that in children with thalassemia who get regular or repeated blood transfusions are indicated to be given therafi desperal. Disperal therapy is a liquid drug given under the skin that has the aim to prevent the accumulation of FE in the body either hemochromatosis (accumulation of FE under the skin) or hemosiderosis (accumulation of FE in the organ). This installation is given 4-7 times per week after blood transfusion.
And let’s donate our blood, because so many need blood help. Is not someone has a high quality of self if it can provide benefits for themselves, others and the environment.
May be useful. Thanks.
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Howard, Martin R., and Peter J. Hamilton. 2008. Haematology Third Edition. Elsevier.
Markum AH, Ismail S.AlatasH. 1991. Buku ajar Ilmu Kesehatan anak. Jakarta: Bagian IKA FKUI.
Rakhmawati, Windy. 2009. Buku Ajar Keperawatan Pemasangan Desferal. Bagian Ilmu Keperawatan Anak Fakultas Ilmu Keperawatan Universitas Padjadjaran.
Available at : http://pustaka.unpad.ac.id/wp-content/uploads/2010/03/pemasangan_desferal.pdf (diakses pada tanggal 20 Mei 2015). Sembiring, Samuel P. K. 2010. Bisakah thalassemia disembuhkan?.Medan. [online].
Available at :http://www.morphostlab.com/artikel/bisakah-thalassemia-disembuhkan.html (diakses pada tanggal 20 Mei 2015)