Drepanocytic Anemia: Definition, Symptoms, Treatment, Causes and Prevention

It is a condition in which there are not enough healthy red blood cells to carry adequate oxygen throughout the body.

Sickle or sickle cell anemia is a hereditary form of anemia. Usually, your red blood cells are flexible and round, moving quickly through your blood vessels. In sickle-cell anemia, the red blood cells become stiff and sticky and have the shape of sickles or growing moons.

These irregularly shaped cells can get trapped in small blood vessels, slowing down or blocking blood flow and oxygen in some body parts.

There is no cure for most people with sickle cell anemia. However, treatments can relieve pain and help prevent problems associated with the disease.

Symptoms of Drepanocytic Anemia

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

Anemia

Sickle cells separate easily and die, leaving you without enough red blood cells. Red blood cells usually live about 120 days before they need to be replaced. However, 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 not get the oxygen it needs to feel energized, causing fatigue.

Episodes of pain

Periodic episodes of pain, called crises, are a significant symptom of sickle cell anemia.

Pain develops when sickle-shaped red blood cells block blood flow through small blood vessels to the chest, abdomen, and joints. Pain can also occur in your bones.

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

Some teens and adults with sickle cell anemia also have chronic pain, resulting from damage to bones and joints, ulcers, and other causes.

Painful swelling of the hands and feet

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

Frequent infections

Sickle cells can damage an organ that fights infections (spleen), leaving it more vulnerable to infections. Doctors commonly give infants and children immunizations against sickle cell anemia and antibiotics to prevent life-threatening conditions, such as pneumonia.

Stunted growth

Red blood cells provide your body with the oxygen and nutrients it needs to grow. The shortage of healthy red blood cells can delay growth in babies and children and delay puberty in adolescents.

Eyesight problems

The small blood vessels that irrigate your eyes may become clogged with sickle cells. This can damage the retina, the portion of the eye that processes the visual images and causes vision problems.

Treatment

This type of sickle cell anemia, as we have said, produces pains that can vary from person to person and according to the tolerance levels of each of these in the face of the pain present. Therefore, a first step to treating this type of anemia is vital:

  • Treat the patient with analgesics of different spectra; we have the classic ibuprofen among the most common.
  • Appeal blood transfusions to dispel the possibilities of strokes.
  • The intake of much liquid.

Well, you can supplement the treatment with medications such as:

Hydroxycarbamide to counteract thoracic neuralgia and even difficulty breathing. Also, taking antibiotics, especially in children, is essential to combat bacteria that cross with sickle cell disease.

To reduce excessive iron production caused by sickle cell diseases, a pharmacological treatment previously prescribed by a specialist is necessary.

When to see a doctor

Although sickle cell anemia is usually diagnosed in childhood, if you or your child have any of the following problems, consult your doctor immediately or seek emergency medical attention:

  • There are unexplained episodes of severe pain, such as pain in the abdomen, chest, bones, or joints.
  • Swelling in the hands or feet.
  • Abdominal swelling, especially if the area is sensitive to touch.
  • Fever, people with sickle cell anemia have an increased risk of infection, and fever may be the first sign of infection.
  • Pale skin or nail beds.
  • Yellow dye on the skin or the whites of the eyes.

Signs or symptoms of stroke

If you notice unilateral paralysis or weakness in the face, arms, or legs, confusion, trouble walking or talking, sudden vision problems or unexplained numbness, or a headache, call 911 or the local emergency number immediately.

Causes of Sickle Cell Anemia

Sickle cell anemia is caused by a mutation in the gene that tells your body to make the red, iron-rich compound that gives blood its red color ( hemoglobin ).

Hemoglobin allows red blood cells to carry oxygen to all body parts from your lungs. In sickle cell anemia, abnormal hemoglobin causes red blood cells to become stiff, sticky, and misshapen.

The gene for sickle cell anemia is transmitted from generation to generation in a pattern of inheritance called autosomal recessive inheritance. This means that both the mother and the father must send the defective form of the gene for a child to be affected.

If only one parent passes the sickle cell anemia gene to the child, that child will have the sickle cell trait.

With a normal hemoglobin gene and a defective gene form, people with the sickle cell trait produce normal hemoglobin and sickle cell hemoglobin.

Your blood may contain some sickle cells, but they usually have no symptoms. However, they are carriers of the disease, which means that they can transmit the gene to their children.

Prevention

If you have the sickle cell trait, you should consult a genetic counselor before trying to conceive can help you understand your risk of having a child with sickle cell anemia. They can also explain possible treatments, preventive measures, and reproductive options.