Sickle cell anemia

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Normal red blood cell and sickle cell
Normal red blood cell and sickle cell

Sickle Cell Disease is a serious disorder affecting the integrity of red blood cells. Its name is derived because because the cells become round and flat resembling a sickle.[1] The change in shape affects the ability of the cells to carry oxygen, but they may also become stuck blood vessels preventing flow. When the red blood cells fail to carry oxygen to parts of the body it can cause damage to the heart, brain, and kidneys.[2]

Contents

Cause

Sickle cell inheritance.
Sickle cell inheritance.

Sickle cell diseases have different types and are determined by the gene inherited from their parents. If someone has inherited sickle cell directly from their parents it is known as hemoglobin SS disease, or sickle cell anemia. A person who gets gene from their parents and also abnormal gene from the other got hemoglobin SC disease or hemoglobin S-thalassemia.[3]

The causal mutations are those within genes responsible for beta globins (blood proteins). These sickle shaped red blood cell are sticky and inflexible, which block the blood flow. As a result, they can’t carry oxygen to other part and cause serious damages to organs and cause pain. [4]

Sickle cell trait is different from the sickle cell disease. Both hemoglobin A and S are produced in the red blood cell. There are always more A than S. Most people who have sickle cell trait are healthy like others. [5]

There is 50% chance to get a baby sickle cell disease or trait if one of the parents has sickle cell anemia and another has sickle cell trait. And if they both have sickle cell trait, it is 25% chance to get sickle cell disease. [6]

Symptoms

Symptoms depend on the different type of the sickle cell disease [7] , it might hurts or not and can stay for minutes or a few days. [8] Commonly, it causes the acute chest syndrome which causes pain in the chest, coughing, difficulty breathing, and fever. Hand-foot syndrome is when the hands and foot become swelled and painful. A person who has the sickle cell disease can get infection easily than others. When you have fever and the body temperature goes up to 38 Celsius you should consider it as sickle cell disease. Splenic sequestration crises is a symptoms cause paleness, weakness, an enlarged spleen, and pain in the abdomen. Also it can cause stroke because of the poor oxygen in the brain occurs headache, seizures, weakness of the arms and legs, speech problems, a facial droop, and loss of consciousness. [9]

Treatment

Treatment for sickle cell
Treatment for sickle cell

Most kids with the disease take a drug, penicillin, to prevent other infections. Folic acids, vitamin supplement are also helpful because it increases new red blood cells in bodies.[10] Normal blood cell lives about 120 days but the sickle cell lives only for 10 to 20 days because the bone marrow cannot make new red blood cells that fast.[11] Some kid need to do blood transfusions to give them fresh and new red blood cell which decrease the chance to block the blood vessel. These are only temporary treatment but not the absolute cure.[12]

Because the bone marrow makes the red blood cell, a bone marrow transplant is the only way to cure sickle cell disease. If they don’t have your family like brother and sister it is hard to find the donor who has close to theirs. [13] But it also takes a lot of risk in the operation. Although the operation has been successful to several affected kids, adults have more of a chance to reject the bone marrow transplant. [14]

It is important to care for your kid with proper care from a hematologist, a blood specialist or sickle cell clinic. Because of development of medicines, most people with sickle cell disease can live about mid-40s or more. Kids need to drink lots of fluids, get lots of sleep and keep in the average temperature all the time. [15]

References

Misshapen red blood cells characteristic of sickle cell anemia
Misshapen red blood cells characteristic of sickle cell anemia

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