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Know About Sickle Cells Disease

by Health Please


Posted on 10:09AM Jun 16, 2018 in General


Know About Sickle Cells Disease



What is Sickle-cell disease?

Sickle-cell disease (SCD) is a group of blood disorders typically inherited from a person's parents. The most common type is known as sickle-cell anaemia (SCA). It results in an abnormality in the oxygen-carrying protein haemoglobin (haemoglobin S) found in red blood cells. This leads to a rigid, sickle-like shape under certain circumstances. Problems in sickle cell disease typically begin around 5 to 6 months of age.

Sickle Cells Disease

A number of health problems may develop, such as attacks of pain ("sickle-cell crisis"), anemia, swelling in the hands and feet, bacterial infections, and stroke. Long term pain may develop as people get older. The average life expectancy in the developed world is 40 to 60 years.

Symptoms

Signs and symptoms of sickle cell anemia, which vary from person to person and change over time, include:

1) Anemia:

Sickle cells break apart easily and die, leaving you without enough red blood cells. Red blood cells usually live for about 120 days before they need to be replaced. But sickle cells usually die in 10 to 20 days, leaving a shortage of red blood cells (anemia).

Without enough red blood cells, your body can't get the oxygen it needs to feel energized, causing fatigue.

2) Painful swelling of hands and feet:

The swelling is caused by sickle-shaped red blood cells blocking blood flow to the hands and feet.

3) Episodes of pain:

Periodic episodes of pain, called crises, are a major symptom of sickle cell anemia. Pain develops when sickle-shaped red blood cells block blood flow through tiny blood vessels to your chest, abdomen and joints. Pain can also occur in your bones.

The pain varies in intensity and can last for a few hours to a few weeks. Some people have only a few pain episodes. Others have a dozen or more crises a year. If a crisis is severe enough, you might need to be hospitalized.

Some adolescents and adults with sickle cell anemia also have chronic pain, which can result from bone and joint damage, ulcers and other causes.

4) Vision problems:

Tiny blood vessels that supply your eyes may become plugged with sickle cells. This can damage the retina the portion of the eye that processes visual images, leading to vision problems.

5) Frequent infections:

Sickle cells can damage an organ that fights infection (spleen), leaving you more vulnerable to infections. Doctors commonly give infants and children with sickle cell anemia vaccinations and antibiotics to prevent potentially life-threatening infections, such as pneumonia.

6) Delayed growth:

Red blood cells provide your body with the oxygen and nutrients you need for growth. A shortage of healthy red blood cells can slow growth in infants and children and delay puberty in teenagers.

Diagnosis

A blood test can check for hemoglobin S — the defective form of hemoglobin that underlies sickle cell anemia. In the United States, this blood test is part of routine newborn screening done at the hospital. But older children and adults can be tested, too.

In adults, a blood sample is drawn from a vein in the arm. In young children and babies, the blood sample is usually collected from a finger or heel. The sample is then sent to a laboratory, where it's screened for hemoglobin S.

If the screening test is negative, there is no sickle cell gene present. If the screening test is positive, further tests will be done to determine whether one or two sickle cell genes are present.

Additional tests

If you or your child has sickle cell anemia, a blood test to check for a low red blood cell count (anemia) will be done. Your doctor might suggest additional tests to check for possible complications of the disease.

Medications

Medications used to treat sickle cell anemia include:

Antibiotics:

Children with sickle cell anemia may begin taking the antibiotic penicillin when they're about 2 months old and continue taking it until they're at least 5 years old. Doing so helps prevent infections, such as pneumonia, which can be life-threatening to an infant or child with sickle cell anemia.

Pain-relieving medications:

To relieve pain during a sickle cell crisis, your doctor might prescribe pain medications.

Hydroxyurea (Droxia, Hydrea):

When taken daily, hydroxyurea reduces the frequency of painful crises and might reduce the need for blood transfusions and hospitalizations. Hydroxyurea seems to work by stimulating production of fetal haemoglobin, a type of hemoglobin found in newborns that helps prevent the formation of sickle cells.



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