Hematology

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Sickle Cell Anemia … Symptoms, Prevention, Treatment

Sickle Cell Anemia:

Sickle cell anemia is an inherited blood disorder (IBD) that affects red blood cells; due to disorder in the gene responsible for the formation of hemoglobin, causing sticking to the walls of the small blood vessels, and thus reducing the flow of the blood and oxygen into the body organs, the matter which leads to the appearance of symptoms suffered by the sickle cell patients, including severe pain, dyspnea and other symptoms.

What Are the Mutations on the Red Cell When There Is a Case of Sickle Cell Anemia?
In normal conditions, the red blood cells are disk-shaped and flexible and can move through the blood vessels easily.
But in case of infection with the sickle cell anemia, the red cells become rigid, sticky and crescent-shaped, or what is called the "sickle". Such an abnormal shape of the red blood cells make them unable to move flexibly inside the blood vessels and they become more sticking to the vessels wall affecting the movement of blood in the vessels, whereas the blood flow is slow or the blood may tumble in the vessels due to the red blood cells sticking and obstructing of the blood vessels. The most important thing is that the oxygen will not reach the body organs in adequate amounts, making the body prone to serious health problems, including inflammations and stroke.

Sickle Cell Symptoms:
Normally, the sickle cell anemia symptoms begin when the infant reaches four months, and include the following:

A repeated spells of pain in different parts of the body, where the blood cells are broken and small vessels are blocked, such as: pain in the stomach, joints or limbs.
Chronic anemia.
Repeated inflammation.
Symptoms of malnutrition, short stature and slow growth.
Abnormalities in bones.
Drowsiness and fatigue.
Vision problems.

Examinations to Detect an Infection with the Sickle Cell Anemia:

The sickle cell anemia may be detected through the routine blood test after child birth.
The sickle cell anemia may also be discovered before delivery through examination of the amniotic fluid surrounding the fetus in the mother's womb.
The pre-matrimonial tests reveal any infection of a parent, so they are so important to avoid giving birth to children prone to the infection with the genetic blood diseases, including the sickle cell anemia.

Treatment:

The Main Objectives of Treating the Sickle Cell Anemia:

Minimizing rates of the ambulatory and emergency cases of the infected persons.
Limiting the occurrence of the complications.
Reducing the pain.
Improving the infected person's ability for living with the disease.

Ways of Treatment:

The person infected with the sickle cell anemia is in need of continuous care to prevent the recurrence of complications and deterioration of his health condition.
During the spells, the infected person is treated with pain relievers and by supplying his body with fluids.
For treating the pain, some cases may respond to the over-the-counter painkillers, while others require using more powerful pain relievers, such as morphine, meperidine and others. This is to be carried out under the medical supervision in the hospital.
Some of the infected persons are given (Hydroxyurea) as a preventive medicine to prevent the occurrence of spells, particularly the symptoms of the respiratory system, such as chest pain and dyspnea.
The anemia during the spells is treated by transfusing of blood to compensate the shortage of the red-colored fluid.
It is important to take routine and annual vaccines, especially for the infected children, such as the seasonal vaccines against influenza to prevent the infection.
The sickle cell anemia may be treated with the marrow transplant.

Ways for Living with the Disease:

The patient is given the folic acid tablets to help in treating the anemia cases.
The patient must take rest and avoid daily stress.
The regular practice of exercises helps the patient to rest and reduces chances of his exposure to pain spells.
The patient should regularly drink an adequate amount of water, since being dehydrated could make the condition worse.
The patient should avoiding exposure to extreme heat or cold.

Sickle Cell Anemia Complications:
Complications

Cause

Stroke

It occurs because of the obstruction of the brain's blood vessels. The infected may also have epilepsy spells, acroanesthesia, speech difficulty and loss of consciousness.

Acute Chest Syndrome
It is a fatal condition, during which the person suffers from (a cute chest pain, high temperature and breathing difficulty), and it occurs when the blood vessels in the lungs are obstructed by the sickle cell or because of the inflammation of the lungs.

Pulmonary Hypertension

The condition is characterized by shortness of breath, which necessitates an immediate relief.

Organ Damage

This occurs due to the obstruction of the blood vessels in any of the body organs, and the inability of this organ to get the sufficient amount of oxygen.

Gallstones
The breakdown of the blood red cells produces Bilirubin; and the rise of the rate of this substance in the body causes the formation of the gallstones.

Skin Ulcers
The sickle cell anemia may cause ulcers in the skin, especially in the feet.

Penis Vessels Blockage
Men with the sickle cell anemia are suffering from prolonged and painful erection due to the obstruction of the penis's blood vessels by the red blood cells.

Prevention of Sickle Cell Anemia:
Committing to do the pre-matrimonial comprehensive medical checkup helps to limit the transmission of the sickle cell disease between generations, whereas medical examinations reveal the likelihood of the presence of defected genes among men or women, particularly those on whom the symptoms don't show up.

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