Sickle Cell Anemia - Symptoms Causes Diagnosis Treatment and Complications

By trima sandhyka - Friday, April 14, 2017 No Comments

Understanding Sickle Cell Anemia


Anemia Sickle cell anemia is a condition where there are abnormalities in the shape of red blood cells, of which should be rounded and flexible, become sickle-shaped and hard. In sickle cell anemia, the body becomes normal red blood cell deficiency to meet the transport of nutrients and oxygen throughout the body.
Sickle Cell Anemia - Symptoms Causes Diagnosis Treatment and Complications

Normal red blood cells contain enough hemoglobin capable of carrying 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 the turn quickly when damaged blood cells earlier.
While on sickle cell anemia, a condition experienced by inverted. The red blood cells are sickle-shaped does not have sufficient amount of hemoglobin to carry oxygen the body needs. In addition, the blood cells have a short age range that runs slow cell renewal.
In contrast to normal red blood cells are able to flow smoothly for the round and flexible, blood cells sickle shape often stick to each other and 'stuck' in the blood vessels and cause blockages.

Symptoms of Sickle Cell Anemia

Patients with sickle cell anemia may show a variety of symptoms due to deficiency of red blood cells (anemia), the form of the body feels tired and underpowered, irregular heartbeat, and shortness of breath (especially after physical activity).
Blood clots can cause damage to the blood vessels so that patients with sickle cell anemia can feel the pain that is unbearable. This pain relapse episode when called sickle cell crisis. It is estimated that patients with sickle cell anemia may develop this condition for up to 14 times a year (although generally 1-2 times), the duration of pain about 5-7 days.
In children, the appearance of episodes of sickle cell crisis can be recognized from the swelling of the hands and feet. As the growth of age, the pain may spread to other body parts, such as to the abdomen, sternum, spine, pelvis, and ribs.
The growth of children suffering from sickle cell anemia risk is hampered because of deficiency of red blood cells that supply nutrients and oxygen needed by the body. These conditions are also at risk of slowing down puberty they are in their teens.
In addition, patients may experience:
  • Impaired vision due to damage to the retina as the effects of the blockade to blood flow in the eye.
  • Susceptible to bacterial or viral infection due to damage to the spleen (the organ that fights infection).
  • Body appears yellow (jaundice). Caused by the buildup of bilirubin may result from damage to red blood cells quickly. In addition, high bilirubin levels can also cause gallstone disease when a pile of these substances crystallize and clog the bile ducts.
  • Injuries to the skin due to blockage in the blood vessels of the skin.
  • Priapism or prolonged erection that cause pain and risk of causing damage to the penis as well as infertility. Priapism occurs due to blockage of blood flow in the penis.
If you have children who suffer from sickle cell anemia, supervise them closely because this condition could potentially lead to complications that endanger lives. Immediately take your child to the hospital if it shows signs of increasing levels of severity of symptoms, such as:
  • Hard to breathe
  • looks confused
  • High fever
  • Dizziness and neck stiffness
  • Severe headache
  • Priapism experienced for more than two hours (the boy)
  • Belly swollen and extremely painful
  • Still feel the pain in the sickle cell crisis despite being given painkillers.
  • Convulsions
 

Causes of Sickle Cell Anemia

Sickle cell anemia is not contagious. This condition is caused by a gene mutation that makes hemoglobin molecules stick together and harden when deoxygenation process (oxygen release and absorption of carbon dioxide). The clumps of hemoglobin is then damaging the original shape of red blood cells, from which should round to crescent.
Sickle cell anemia is an autosomal recessive. This means that a person at risk of developing symptoms of sickle cell anemia when both parents are carriers of this disease. If only one course, then the child born will only be a carrier, not the disease.
Actually, the proportion of affected or not affected by sickle cell anemia in children with both parents of the nature of the disease is the same, namely 25 percent. This means that 1 in 4 children likely to inherit a defective gene or gene pair could also normal. While the proportion for the child to remain a carrier is 50 percent.
In addition to Asians, sickle cell anemia disease experienced by the Middle East, Africa, the Eastern Mediterranean, and the Caribbean.

Diagnosis of Sickle Cell Anemia

A child who is born of the seed of both parent carrier of sickle cell anemia need to undergo in order to know whether he be a patient or carrier. One type of inspection that is quite accurate in this case is hemoglobin electrophoresis. Through this examination, the patient or the carrier can be determined from the calculation of the amount of sickle hemoglobin in the blood. In addition to hemoglobin electrophoresis, other tests are quite accurate in the diagnosis of sickle cell anemia is a DNA testing fetal cells.

Treatment of Sickle Cell Anemia

Currently the treatment of sickle cell anemia are generally only intended to prevent the recurrence of sickle cell crisis, relieve symptoms and prevent complications. As for the treatment of sickle cell anemia can include:
  • Bone marrow transplant . The only method of treatment that can cure this condition once and for all only through bone marrow transplants. Through this method, the bone marrow of patients will be replaced with donor bone marrow to produce red blood cells healthy. But not easy to find a bone marrow donor. In addition, this method also has risk of complications, such as seizures, stroke, and tumors.
  • Overcoming sickle cell crisis . To cope with sickle cell crisis that often recur (more than six times a year), the doctor will usually prescribe a drug is able to stimulate the body to produce a type of hemoglobin that is not affected by sickle-cell mutation called hemoglobin fetus (HbF) to replace the hemoglobin after birth ( Hb) were broken. Although quite effective, the use of hydroxycarbamide can cause various side effects. Besides being able to thwart contraception and increases the risk of defects in pregnant women, the use of hydroxycarbamide without a prescription from a doctor feared could lower the level of blood viscosity at a dangerous level and lower levels of white blood cells in the body that makes a person consuming it prone to infection.
  • Pain management. If you feel pain that interferes when undergoing a period of sickle cell crisis, try to relieve a way to compress the affected part with warm towels, take his mind off the pain (eg, playing video games, watching movies, or reading favorite stories), drink plenty fluids to increase blood flow is blocked, and taking pain medication that is sold at pharmacies (eg paracetamol. If the pain has not disappeared and even more disturbing, see your doctor. Your doctor will probably prescribe pain medication at a dose of stronger containing the combination of codeine and paracetamol or morphine.
  • Overcoming anemia. To overcome the symptoms of blood deficiency or anemia in patients with sickle cell anemia, the doctor will usually prescribe supplements of vitamin B9 or folic acid and recommend the types of food that is needed to increase the production of red blood cells. The food group can include liver, green beans, peas, potatoes, and spinach).
  • Coping with stunted growth. For children who have problems of growth, the possibility of the doctor will prescribe supplements of zinc (zinc) in order to stimulate the growth of bone and muscle. Meanwhile, to overcome the problem of puberty in adolescents with sickle cell anemia, the doctor will likely perform hormone therapy.
  • Prevention of infection. To prevent the risk of infection, a doctor may recommend that patients with sickle cell anemia to be immunized, the immunization hepatitis and meningitis. In addition to immunizations, the doctor will probably prescribe some antibiotics.
  • Prevention of stroke. To prevent the risk of stroke, it is recommended to undergo transcranial doppler scan (TCD scan) every year. Through ultrasound assisted examination methods, the rate of blood flow in the brain can be seen. If doctors suspect a blockage of blood flow by blood cells sickle risk of causing a stroke, then the doctor may recommend blood transfusion to increase blood supply to the brain.
In addition to the drug through doctors, we can also implement a healthy lifestyle in everyday life in order to minimize the symptoms of sickle cell anemia. Examples of healthy lifestyles in these conditions is to regularly eat healthy foods with nutritionally balanced (carbohydrates, protein, vegetables, and fruit), exercise the recommended doctor (usually mild exercise that is not too drained of energy), and drink plenty of fluids.
You are also advised to stay away from the factors that can trigger a sickle cell crisis, such as smoking, excessive consumption of alcohol, use illegal drugs, stressed, are in high areas with low oxygen levels, and are in areas of extreme temperature.

Complications of Sickle Cell Anemia

To blockage of blood vessels can reduce the function or even damage the body's organs (such as kidneys, spleen, liver, and brain) due to the inhibition of blood flow. The result can occur:
  • Blindness due to damage to the retina as the effects of the blockade to blood flow in the eye.
  • Acute chest syndrome and pulmonary hypertension due to blockage of sickle cells in the blood vessels of the lungs. Both conditions are characterized by symptoms of shortness of breath is quite lethal.
  • Stroke due to obstruction of blood flow in the brain.

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