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Sickle cell anemia is a blood disorder that results from a genetic disorder, which is characterized by an abnormal form of red blood cells. The abnormal form of red blood cells usually resembles a crescent moon.
This disease often occurs in Africa and America. Sickle cell anemia occurs since childhood and continues into adulthood.
Cause
Sickle cell anemia is caused by a mutation of the gene that causes the emergence of different hemoglobin, called HbS. The cause of this gene mutation is unknown.
Sickle cell anemia can tighten the condition in conditions:
- Cold air
- On long haul flights
- Infection
- Dehydration
- Drink too much alcohol
- Emotional stress
- Pregnancy
Diagnosis
The incidence of sickle cell anemia is not a common condition. Many tests are needed to ensure the diagnosis and find out the complications that occur.
At the initial stage, the doctor will conduct a complete interview and a thorough physical examination of the patient. Then proceed with a laboratory examination in the form of hemoglobin examination, leukocytes, platelets, kidney function (urea, creatinine, and urine examination), liver function (SGOT, SGPT, and bilirubin), electrolytes, and oxygen saturation in the blood.
If it is suspected that there is a crescent cell anemia from various examinations, then the next step is to make sure by doing an electrophoresis examination. Examination is done by taking the patient's blood, then dropping it into electrophoresis so as to see what 'ribbon' will appear. If high levels of HbS are obtained, it is certain that there is a sickle cell anemia.
To see if there are complications of sickle cell anemia, doctors will sometimes also ask for various radiological examinations such as:
- Chest x-ray to see the condition of the lung, especially if it is suspected that the patient has acute chest syndrome which is characterized by severe chest pain and tightness.
- CT scan to see bone abnormalities caused by the death of many bone cells because the bone marrow is experiencing 'fatigue'
- Doppler ultrasound for early detection of stroke risk
- Echocardiography to see heart swelling and hypertension in pulmonary arteries
Symptoms
Symptoms of crescent anemia seen from childhood, can even be seen from infancy. The anemia that is experienced is severe enough so that the sufferer looks pale, easily tired, weak, lethargic. In children, development can be slower than other children his age.
Even though the disease is called crescent anemia, the symptoms are not just anemia. Other symptoms that often occur in sufferers are:
- Bone pain, especially in the bones in the lower limbs.
- Very easy to get an infection
- Heart swelling is characterized by tightness and swelling in both lower limbs
- Gallstones that occur since childhood, marked by right upper abdominal pain arise
- Erections that occur continuously without sexual stimulation
- Late sexual development
- Stature is smaller than people his age
- Thin body
- Stroke at a young age
- Severe pain in the tips of the fingers and toes due to constriction of blood vessels that occur suddenly. In conditions of severe narrowing, deep wounds can occur and are not easily cured in the legs.
- Acute chest syndrome, which is characterized by fever, coughing, severe chest pain
- Protruding eyes or vision sharply slowly
Treatment
Until now sickle cell anemia treatment is more to overcome the symptoms that appear, not to eliminate disease.
Treatment to improve the condition of blood cells generally uses hydroxyurea. If patients with crescent cell anemia experience severe pain, then drugs containing opioids or the like may be given, for example methadone, morphine, fentanyl, or codeine. Sometimes antidepressants also need to be given to help reduce pain.
Because sickle cell anemia sufferers are susceptible to infection, the sufferer needs a vaccine to prevent some diseases. Among them are pneumococcus, meningococcus, and influenza vaccines. If an infection has occurred, the doctor will consider giving antibiotics that can kill various types of bacteria. Vitamins such as folic acid are also needed for the formation of red blood cells.
If there are injuries to the fingertips, good wound care according to the doctor's instructions is very necessary. In addition, if anemia is severe enough (especially if the hemoglobin level is below 6 mg / dl), red blood cell transfusion is also needed.
Now there is hope to cure sickle cell anemia, which is a bone marrow transplant. The effectiveness of this treatment is still being investigated.
Prevention
Until now sickle cell anemia has not been prevented. However, it is important to know that sickle cell anemia sufferers can reduce the genes of the disease to their children.
Therefore, if sickle cell anemia sufferers want to have children, you should first do genetic counseling with a doctor to discuss the possibility of the child experiencing the same disease as well.
