Symptoms and Management of Sickle Cell Anemia

If your child gets swellings over his hand, foot or any other part of the body, one of the cause to consider is sickle cell anemia.

Common Presentations

  • The patient may be symptom less in early years of life. Later on, as he grows up, he may have unexplained swellings over his peripheral areas. These include the hands, foot, vaginal lips, penile region or any other.
  • Episodes of excruciating pain in the legs or arms is another common way the disease presents. These episodes may occur 4 to 12 times a year. It may be even more in frequency, and severity, where immediate treatment is required.
  • Affected individuals are anemic, that is, they look pale. The oxygen carrying capacity of blood is greatly affected. Patients complain of weakness, early fatigue on doing any strenuous activity.
  • Affected individual contract infections, frequently. Common among them are the upper respiratory, urinary and stomach infections.

What Is Sickle Cell Anemia?

Normal red blood cells or erythrocytes are dough-nut shaped, without a central hole. They are flexible in structure. They flow smoothly in blood and are able to pass through tiny blood vessels of the peripheries. They are produced in the marrow region of the long bones of the body. RBCs have an average life of 120 days.

In sickle cell anemia, the RBCs have a different (mutated) hemoglobin than the normal RBC. This hemoglobin is called hemoglobin S. It is inherited by the individual from his parents.

To get a sickle cell condition, it’s important that the child gets this defective gene for hemoglobin making from both his parents. Inheriting a normal gene from any one parent would prevent him from getting the sickle cell condition. Medically speaking, this happens because this character is autosomal recessive.

The altered form of hemoglobin in sickle cell condition changes the RBC shape into the shapes of sickles. They also become rigid and sticky.

As the flexibility of these blood cells is lost, their movement in the blood vessels is affected. They do not flow smoothly and make clutter together in narrow vessels or tortuous vessels causing blockage of those vessels.

Sequence of Events in Sickle Cell Condition

The sickle shaped RBCs are not able to flow smoothly in the blood vessels. The get cluttered up in the narrow passages of peripheral vessels. They also tend to stick to the walls of blood vessels.

Due to this, there may be a complete or partial blockage in the blood vessels. This may give the patient an excruciating pain in the affected portion of the body, caused to due reduced blood supply to the affected portion. This painful crisis is usually temporary, and is relieved by taking pain medications, and easing up the blocked vessel.

Swellings may appear in different peripheral portions of the body. The cause here to is blockage of blood vessels draining the organ.

Sickle cells get entangled in the narrow passages of the spleen. There is, therefore, pooling of blood in the spleen. The spleen may enlarged and give you pain.

This affects the immune system of the body. The patient becomes prone to infections. Most kids, by the age of 8 have a scarred spleen, caused due to repeated sequestrations of RBCs in the organ.

Symptoms

  • Anemia is the commonest symptom to be seen. The affected child is pale to look at, complains of weakness and early fatigue. This is because there is always a scarcity of RBCs and total oxygen carrying capacity of blood. The sickle shaped RBCs survive for 2o days on a average. Their production in the bone marrow is unable to meet the demand.
  • Jaundice may be visible. This is caused due to massive loss of RBCs in the blood. Bilirubin pigment, which gives a yellow tinge to the skin in jaundice patients, is produced due to destruction of red blood cells.
  • Pain crisis, also called the sickle crisis may occur many times in a year, up to 10-12 or even more. The affected portion of the body is deprived of blood supply due to blockage of blood vessels by the cluttered sickle shaped RBCs. There is very intense pain in the affected portion. Most commonly involved portions of the body are the peripheral portions, like the arm, legs, fingers, penis or the chest.
  • Swellings over the hands, legs, foot, vaginal lips, fingers etc.
  • Increased frequency of infections is seen. The affected child frequently catches fever, upper respiratory infections, urinary or GI infections. This is due to low immune status of the body.
  • Sudden stroke is a rare but serious symptom of the condition. The occurs when a blood vessel supplying a part of the brain suddenly gets blocked. The part of the brain it was draining is deprived of blood supply and becomes non functional. There may be temporary or permanent neurological impairment.

Diagnosing Sickle Cell Anemia

If your child has any of the above mentioned symptoms, sickle cell state may be suspected. To diagnose, a blood sample is taken and smear slide is appeared. Sickle shaped RBCs are visible under the microscope.

Management

The child suffering from sickle cell anemia would benefit from an early diagnosis. The management aims at giving the child a healthy life, free from complications.

Managing painful crisis– Pain medications are given immediately.

If the frequency of these episodes is much, one may opt for regular blood transfusions. This would dilute the blood, mixing normal RBCs with the sickle shaped ones.

This step also guards against splenic sequestrations and other painful emergencies.

Managing recurrent infections– Vaccinations are given to the child for most common infections. Prophylactic antibiotic treatment may be started if any infection is suspected in the community.

Managing Anemia– A healthy diet is encouraged. Iron and folic acid supplements need to be given to meet the increased demand of RBC production.

Hydroxyurea Therapy

This is given in recent times and has been seen to reduce all complications of sickle cell anemia. It promotes the production of a different type of hemoglobin called the fetal hemoglobin, which has much less tendency to sickle up.