what causes sickle cell anemia? Sickle cell anemia is an anemic condition in which abnormal red blood cell formation, from which it should be round and flexible, becomes sickle 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.
Sickle-shaped red blood cells do not have enough hemoglobin to transport oxygen according to the body’s needs. In addition, the crescent-shaped blood cells have a shortage range so that sickle cell updates are slow
Anemia Cell Sabit
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 a blockage.
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).
Blockage in blood vessels can cause damage to blood vessels so that people with sickle cell anemia can feel unbearable pain. The episode when this pain relapse is called a sickle cell crisis. It is estimated that sickle cell anemia patients may experience this condition up to 14 times a year (though generally 1-2 times), with a duration of 5-7 days.
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.
Patients with sickle cell anemia in children will inevitably delay their growth. This is due to deficiency of red blood cells in charge of supplying the nutrients and oxygen needed by our 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 a bacterial or viral infection due to damage to the spleen (an organ that works against infection).
- Body looks yellow (jaundice). Occurs due to the accumulation of bilirubin substances can occur due to damage to red blood cells rapidly. In addition, high levels of bilirubin can also cause gallstone disease if the pile of the substance crystallizes and clogs the bile ducts.
- Injury to the skin due to blockage of the blood vessels of the skin.
- Prolonged or painful erectile or prolonged pain causing damage to the penis and sterility.
take care of your children strictly if they suffer from sickle cell anemia 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.
Causes of Sickle Cell Anemia
Sickle cell anemia is not a contagious disease. This condition is caused by the mutation of genes that make the hemoglobin molecules stick together and harden when deoxygenation (release of oxygen and carbon dioxide absorption). hemoglobin is what will then damage the original form of red blood cells to be sickle-shaped
Actually, the proportion of sickle cell anemia affected in children with both carriers of this disease is the same, that is 25 percent. This means that 1 in 4 children have the chance to inherit a pair of defective genes or it could be a pair of normal genes. While the proportion of the child to remain a carrier is 50 percent.
In addition to Asians, sickle-cell anemia affects many people in the Middle East, Africa, the Mediterranean, and the Caribbean.
Treatment of Sickle Cell Anemia
Usually Handling sickle cell anemia only to prevent the recurrence of sickle cell crisis, symptoms, and prevent the emergence of complications. The treatment of sickle cell anemia may include:
Bone marrow transplantation.
The only method of treatment that can cure this condition completely is through a transplant or bone marrow transplant. Through this method, the bone marrow of the patient will be replaced by a donor bone marrow that can produce healthy red blood cells. But it is not easy to find a bone marrow donor. In addition, this method is also at risk of complications, such as convulsions, strokes, and tumors.
Overcoming sickle cell crisis.
To overcome the recurrent sickle cell crisis (more than six times a year), doctors will usually prescribe this drug to stimulate the body to produce one type of hemoglobin unaffected by sickle cell mutation called hemoglobin fetus (HbF) to replace postpartum hemoglobin ( Hb) is broken. Although quite effective, the use of hydroxycarbamide can cause various side effects. In addition to thwarting contraception and increasing the risk of infant defects in pregnant women, the use of hydroxycarbamide without a prescription from doctors feared could reduce the level of blood viscosity at dangerous levels and reduce levels of white blood cells in the body that actually makes someone who consumes them susceptible to infection.
Handling of pain.
If you feel a disturbing pain during a sickle cell crisis period, try to relieve it by compressing the affected area with a warm towel, diverting the mind from the pain (eg by playing video games, watching movies, or reading favorite stories), drinking a lot liquids to facilitate blocked blood flow, and take over-the-counter painkillers at pharmacies (eg paracetamol.If the pain is not lost and even more disturbing, see your doctor immediately.) The doctor may prescribe pain medication with a stronger dose containing a combination of codeine and paracetamol or inject morphine.
To overcome the symptoms of blood deficiency or anemia in patients with sickle cell anemia, doctors will usually prescribe vitamin B9 or folic acid supplements and suggest the types of foods needed to increase the production of red blood cells. The food group may include liver, green beans, peas, potatoes, and spinach).
Overcoming stunted growth.
For children with growth problems, doctors may prescribe zinc supplements to stimulate bone and muscle growth. Meanwhile, to overcome the problem of puberty in adolescents with sickle cell anemia, doctors are likely to perform hormone therapy.
Prevention of infection.
To prevent the risk of infection, doctors usually recommend sickle cell anemia patients to be immunized, namely hepatitis and meningitis immunization.
Prevention of stroke.
To prevent the risk of stroke, it is recommended to undergo transcranial doppler scan (TCD scan) each year. If doctors suspect blockage of blood flow by sickle-cell blood at risk of stroke, doctors usually suggest blood transfusions to increase blood supply to the brain.
In addition to drug doctors, we can also apply a healthy lifestyle in everyday life in order to minimize the symptoms of sickle cell anemia. Examples of healthy lifestyles in these conditions are to regularly eat healthy foods with balanced nutrients (carbohydrates, proteins, vegetables, and fruits), exercise the doctors recommend (usually mild exercise that is not too draining energy), and drink enough fluids.
You are also advised to stay away from factors that can trigger a sickle cell crisis, such as smoking, excessive alcohol consumption, using drugs, experiencing stress, being in high areas with low oxygen levels, and are in extreme temperatures.