Sickle Cell Anemia Symptoms And Treatments

Sickle Cell Anemia Symptoms And Treatments

The Understanding Of Sickle Cell Anemia


Sickle cell anemia anemia is a condition where there is a form of abnormality of red blood cells. The normal form of red blood cells is rounded and flexible, while someone who has sickle cell anemia has crescent shaped blood cell and it is hard. In sickle cell anemia, the body becomes lacking of a normal red blood cell to fulfill nutrients and oxygen transport throughout the whole body.

Normal red blood cells contain sufficient hemoglobin which is quite able to transport oxygen from the lungs in the amount required by the body. In addition, the normal red blood cells can live for quite a while so it gives time for the body to produce new cells and do the replacement quickly when a previous blood cell damaged.

In sickle cell anemia, the condition is the opposite. The crescent shaped red blood cells do not have sufficient amount of hemoglobin to transport the needed oxygen. In addition, these blood cells have a short lifespan so the cell renewal runs slowly.

In contrast to the normal red blood cells which are capable of flowing smoothly due to the spherical-shaped and flexible, the crescent shaped blood cells often stick to one another and ' stuck ' in the blood vessels and cause a blockage.


The Symptoms Of Sickle Cell Anemia


Sufferers of sickle cell anemia can show a variety of symptoms due to a shortage of red blood cells (anemia), the body feels tired and less powerful, irregular heartbeat, and shortness of breath (especially after doing physical activity).

Of blockages in the blood vessels can cause damage to the blood vessels so that sufferers of sickle cell anemia can feel unbearable pain. The time when this pain relapses is known as sickle cell crisis. Sickle cell anemia sufferers is estimated to be experiencing this condition up to 14 times a year (although generally 1-2 times), with the duration of pain about 5-7 days.

In children, the appearance of sickle cell crisis episodes can be identified from the swelling of the hands and feet. Along with the growth of the age, the pain can spread to other parts of the body, such as the abdomen, sternum, spine, pelvis, and ribs.

The growth of children who suffer from sickle cell anemia are at risk of stunted because of red blood cells deficiency that supplies oxygen and nutrients needed by the body. This condition is also at risk of slowing down puberty in their teens.

In addition, sufferers can experience:


  • Eyesight problem due to the damage of the retina as the effects of the blood flow blockage inside the eye.
  • Easily affected by a bacterial or virus infection due to damage of the spleen (an organ that serves to fight infection).
  • The body looks yellow (jaundice). It happens because of the buildup of bilirubin substances that may occur due to damage of red blood cells that happens quickly. In addition, high bilirubin levels can also cause gallstones disease if the pile of the substance is crystallized and clogging the bile duct.
  • Wounds on the skin due to blockages in the blood vessels of the skin.
  • Priapisme or prolonged erections causing pain and also has a risk of causing damage to the penis as well as infertility. Priapisme occur due to blockage of blood flow in the penis.

If you have a child who suffers from sickle cell anemia, watch them closely because this condition could potentially cause complications which can endanger their lives. Immediately take your children to the hospital when showing signs of an increase in the level of severity of symptoms, such as:


  • Shortness of breath
  • Looks confused
  • High fever
  • Stiff neck and dizziness
  • Terrific headache
  • Experience the priapisme for more than two hours (on boy)
  • Swollen stomach and feels very sick
  • Still feel pain in the sickle cell crisis even though they have been given a pain reliever medication.
  • Seizures


The Cause Of Sickle Cell Anemia


Sickle cell anemia is not a contagious disease. This condition is caused by a mutation of a gene that makes hemoglobin molecules stick to each other and hardens when the process of deoxygenation (release of oxygen and carbon dioxide absorption) takes place. This lump of hemoglobin which then damages the original shape of the red blood cells, from round to be a crescent.

Sickle cell anemia is autosomal recessive. This means that someone is at risk of having sickle cell anemia symptoms when both parents are a carrier of this disease. If only one, then the child will be a carrier, not a sufferer of the disease.

Actually, the proportion of being affected or not affected by sickle cell anemia in children, whose parents are carriers, is the same, that is 25 percent. This means that 1 in 4 children could inherit a pair of defective genes or it could be a pair of normal gene. Meanwhile, the proportion for the child to remain a carrier is 50 percent.

In addition to the Asian people, the disease of sickle cell anemia is experienced by many people of the Middle East, Africa, Eastern Mediterranean, and the Caribbean.

The Diagnosis of Sickle Cell Anemia


A child whose parents are carriers of sickle cell anemia will need to undergo the examination in order to know whether he is a sufferer or a carrier. One type of examination that is quite accurate in this case is the hemoglobin electrophoresis. Through this inspection, a sufferer or a carrier can be determined from the calculation of the number of sickle hemoglobin in the blood. In addition to the hemoglobin electrophoresis, another test which is quite accurate in diagnosing sickle cell anemia is a fetal cell DNA tests.

Treatment of Sickle Cell Anemia


At the present, the handling of sickle cell anemia are generally only intended to prevent recurrence of sickle cell crisis, relieve symptoms, and prevent complications. As for the handling of sickle cell anemia may include:

A bone marrow transplant. 


The only method of treatment which can cure this condition up completely is just through a transplant or bone marrow transplant. Through this method, the bone marrow of sufferers is replaced by donor’s bone marrow that can produce healthy red blood cells. However, it is not easy to find a bone marrow donor. In addition, this method also raises the risk of complications, such as seizures, stroke, and tumors.

Handling sickle cell crisis.


To cope with the sickle cell crisis which frequently relapse (more than six times a year), the doctor will usually prescribe drugs. These drugs are able to stimulate the body to produce one type of hemoglobin that is not affected by the mutation of sickle cell hemoglobin called fetus (HbF) to replace the postnatal haemoglobin (Hb) which are broken. Though quite effective, the use of hydroxycarbamide may cause various side effects. In addition, it could fail contraception and increase the risk of defects in the babies of pregnant women. Use of hydroxycarbamide without a prescription from the doctor is feared could lower the level of blood viscosity at a dangerous level and lower the levels of white blood cells in the body that make someone who consumes it vulnerable to infection.

Handling pain.


When you feel the pain that is bothering when experiencing sickle cell crisis, try to relieve it by compressing the painful part of the body with a warm towel, divert your mind from the pain (for example, by playing video games, watch a movie, or reading a favorite story), drink plenty of fluids to improve clogged blood flow, and take any pain reliever (for example paracetamol). if the pain remains or even getting more annoying, see the doctor soon. The doctor will likely prescribe a medicated pain relief with stronger doses containing a combination of codeine and paracetamol or inject morphine.

Resolving anemia.


To overcome the symptoms of blood deficiency or anemia in people with sickle cell anemia, your doctor will usually prescribe vitamin B9 supplements or folic acid and suggest the types of food that are needed to increase the production of red blood cells. The food groups can include liver, green beans, peas, potatoes, and spinach.

Overcoming stunted growth.


For children who experience the problem of growth, it is likely the doctor will prescribe supplements of zinc (zinc) in order to stimulate the growth of bones and muscles. As for addressing the problem of puberty in teenage sufferers of sickle cell anemia, your doctor will likely do a hormone therapy.

Prevention of infection.


To prevent the risk of infection, the doctor will usually recommend that sickle cell anemia patient to be immunized, namely immunization of hepatitis and meningitis. In addition to immunization, the doctor will likely also prescribe a number of antibiotics.

Prevention of stroke.


To prevent the risk of stroke, it is recommended to undergo the checkup of transcranial doppler scan (TCD scan) each year. Through this ultrasound assisted examination method, the level of the smooth flow of blood in the brain can be seen. If doctors suspect a blockage of blood flow by the sickle blood cells which has a risk of causing a stroke, then the doctor will usually recommend doing blood transfusions to increase the blood supply to the brain.

In addition to the drug from a doctor, we can also apply a healthy lifestyle in our life in order to minimize the symptoms of sickle cell anemia. Examples of healthy lifestyle in this condition is with the routine of eating healthful food with balanced nutrients (carbohydrates, proteins, vegetables, and fruits), do sports which are recommended by doctor (usually gentle exercise that is not too draining energy), and drink enough fluids.

You are also advised to stay away from the factors that can trigger the onset sickle cell crisis, such as smoking, excessive alcohol consumption, using illegal drugs, experiencing stress, being in a high area with low levels of oxygen, and being in an extremely low-temperature area.

Complications of Sickle Cell Anemia


Blockage in the blood vessels can lose function or even damage the body's organs (such as the kidneys, spleen, liver, and brain) due to the blockage of blood flow. As a result, the following things may occur:


  • Blindness due to the damage in the retina as the effects of the blood flow blockage inside the eye.
  • Acute chest syndrome and pulmonary hypertension due to sickle cell blockages in the blood vessels of the lungs. Both conditions which are characterized by shortness of breath symptoms are classified as deadly.
  • Stroke due to the blood flow blockage in the brain.


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