What causes sickle cell anemia? Sickle cell anemia is an anemic condition in which abnormal shapes of red blood cells, out of which should be round and flexible, become crescent and hard. In sickle cell anemia, the body becomes deficient in normal red blood cells to meet the transport of nutrients and oxygen throughout the body.
Normal red blood cells contain enough hemoglobin capable of transporting oxygen from the lungs in the amount needed by the body. In addition, normal red blood cells can live long enough to allow time for the body to produce new cells and make changes quickly when previous blood cells are damaged.
Whereas in sickle cell anemia, the condition is reversed. Sickle-shaped red blood cells do not have enough hemoglobin to transport oxygen according to the body’s needs. In addition, these blood cells have a shortage range so that cell renewal is slow.
In contrast to normal red blood cells that are able to flow smoothly because they are round and flexible, sickle-cell blood cells often stick to each other and ‘get stuck’ in the blood vessels and cause clogging.
What Causes Sickle Cell Anemia?
What causes sickle cell anemia? Sickle cell anemia is not a contagious disease. The cause of sickle cell anemia is the presence of gene mutations that make the hemoglobin molecules stick together and harden when deoxygenation (release of oxygen and carbon dioxide uptake). These hemoglobin clumps then destroy the original form of red blood cells, from which it should be a crescent.
Sickle cell anemia is autosomal recessive. This means that a person is at risk for sickle cell anemia if both parents are carriers of the disease. If only one, then the child who was born will only be the carrier, not the disease.
In addition to Asians, sickle-cell anemia affects many people in the Middle East, Africa, the Mediterranean, and the Caribbean.
Symptoms of Sickle Cell Anemia
People with sickle cell anemia may show symptoms of red blood cell deficiency (anemia), body fatigue and lack of energy, irregular heartbeat, and shortness of breath (especially after physical activity).
Patients with sickle cell anemia will suffer blood vessel damage, this damage is due to the blockage in the blood vessels so that people with sickle cell anemia can feel unbearable pain.
In children, the emergence of sickle cell crisis episodes can be identified by swelling of the hands and feet. As age grows, pain can spread to other parts of the body, such as to the abdominal area, breastbone, spine, pelvis, and ribs.
In children with sickle cell anemia, their growth will be inhibited. This is due to deficiency of red blood cells that supply the nutrients and oxygen needed by the body. This condition also risks slowing down their puberty in adolescence.
In addition, patients may experience:
- Impaired vision due to damage to the retina as a result of inhibition of blood flow in the eye.
- Easy to get the bacterial or viral infection due to damage to the spleen (an organ that works against infection).
- Body looks yellow (jaundice).
- Injury to the skin due to blockage in the blood vessels of the skin.
- Prolonged or painful erectile or prolonged pain causing damage to the penis and sterility. Priapism occurs due to blockage of blood flow to the penis.
If you have children with sickle cell anemia, keep an eye on them strictly because this condition has the potential to cause life-threatening complications. Immediately take your child to the hospital if you show signs of increased severity of symptoms, such as:
- Shortness of breath
- Seem confused
- High fever
- Dizziness and stiff neck
- Great headache
- Experiencing for more than two hours (in boys)
- Abdominal swelling and pain
- Still feel the pain in the sickle cell crisis despite being given painkillers.