Alpha Thalassemia In Children - Symptoms And Treatment

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Thalassemia is one medical word you don’t want to hear. While everyone wants to avoid any kind of connection with the same, millions of people around the globe are affected by this health condition. The sad part is that, despite medical advancements, this condition is not yet completely curable.

What Is Thalassemia?

Thalassemia is a group of blood disorders that cause problems in the supply of oxygen to the body by interfering with the production of haemoglobin. In thalassemia, the red blood cells of the body are destroyed at a faster rate. Children who have thalassaemia cannot produce enough haemoglobin in their blood. While there are many different types of thalassemia, the two major types are alpha and beta thalassemia.

To understand it better, let us take a look at the causes, symptoms and treatment options for alpha thalassemia.

What is Alpha Thalassemia?

Alpha thalassemia is thought to occur when there is an impaired production of alpha globin – a protein that is responsible for carrying oxygen through the blood. This condition is more common among people residing in the Mediterranean region and those from the Middle East, India and Central Asia.

Alpha thalassemia results from deletions that involve the HBA1 and HBA2 gene deletions. Individuals affected by alpha thalassemia may also have mild anaemia Its severity may vary from individual to individual.

Causes of Alpha Thalassemia In Children:

Alpha thalassemia is usually caused due to alteration of genes associated with the production of haemoglobin. It is not an infectious disease that is transmitted through contact with the affected individual.

Parents affected by thalassemia have a 25 percent risk of their kid being affected by the disease.

Symptoms of Alpha Thalassemia In Children:

The signs and symptoms associated with alpha thalassemia may differ from person to person, and also on the type of alpha thalassemia. Some children affected by this condition tend to display no symptoms at all, while many others may display common symptoms such as:

  • Irritability
  • Dark urine
  • Swelling of the abdomen
  • Slow growth
  • Pale appearance of the skin
  • Yellowness of the skin, similar to that during jaundice
  • Fatigue and weakness
  • Deformities of the facial bones

Treatment For Alpha Thalassemia:

The treatment options help handle alpha thalassemia depend upon the severity of the symptoms and also on the cause of the disease.

  • In many children, alpha thalassemia tends to have little or no symptoms, and the children just develop mild anaemia, for which no medical treatment is necessary. This is why alpha thalassemia is often mistaken for iron deficiency anaemia.
  • For kids affected by this disorder, folic acid supplements are usually prescribed, which may help the body make new red blood cells. If the child affected by this condition undergoes surgery, blood transfusion may be necessary.
  • On the other hand, in severe cases of alpha thalassemia, kids may need to undergo regular blood transfusions through the entire life to stay healthy. When done over a period of time, this may also cause build-up of iron in the body. Medication may be needed to remove extra iron from their body.
  • The only cure that currently exists for thalassemia is a procedure known as bone marrow transplant. In a bone marrow transplant, the patient is first given high doses of radiation or medications to destroy the defected bone marrow. It is then replaced by a bone marrow taken from a compatible donor, which is usually a close relative or a sibling. Bone marrow transplants do carry many risks associated, which is why, they are recommended only in severe cases of thalassemia.
  • Living with alpha thalassemia can be tough, which is why, carriers of the disease should seek genetic counselling before having children.

Possible Complications:

An individual affected by severe thalassemia may also develop certain complications including:

1. Infections:

Children with alpha thalassemia are more likely to be affected by infection, especially if they have had their spleen removed.

2. Slower Growth Rate:

Anaemia arising as a result of severe alpha thalassemia may cause the affected children to grow more slowly which may also cause delay in puberty.

[ Read: Vomiting In Children ]

3. Bone Deformities:

Alpha thalassemia causes the bone marrow to expand which can make the bones wider, thinner and more brittle, which makes them more likely to break. Abnormal bone structure is also noted, particularly in the bones of the face and skull.

4. Enlarged Spleen:

Alpha thalassemia can stimulate the red blood cells of the body to die at a faster rate, which may cause the spleen to work harder, and therefore, grow larger.

5. Excess Iron:

Children having alpha thalassemia can end up having too much iron in their body, which can arise due to the disease itself or due to having repeated blood transfusions. This excess iron can in turn, damage the liver, heart and the endocrine system.

[ Read: Iron Overdose in Children ]

Decoding the Myths:

There are many common myths surrounding this condition, and it’s always best to know about them in detail to make sure you don’t fall prey to any of these misconceptions.

  • The most common myth is that routine blood tests can detect thalassemia. The truth is that there is a specific test involved in the detection of thalassemia.
  • There is another belief that if just one parent is a thalassemia carrier, the child’s risk of being affected is low, which is not true. The child may be at a risk of being affected by the disease, and may also carry the defected gene and pass it on to his child in future, who may then suffer from the disease.
  • Another common belief is that white people do not need to worry about being affected by alpha thalassemia, which is untrue. Though thalassemia does affect some ethnic groups in particular, white individuals too, can be affected by the disease.
  • It is also believed that being a thalassemia carrier can cut down an individual’s risk of being affected by malaria, especially during childhood, but experts believe that anti-malarial medications are still required.
  • There is also a belief that a thalassemic major person will die young, which is not true. With the correct treatment approach, an individual affected by the disease can live a long life.

Being affected by thalassemia is certainly a tough condition for a child and the parents. But the right diagnosis at the right time and the correct treatment can help contain the situation. Make sure you immediately consult a doctor in case the child shows any of the said symptoms of alpha thalassemia.

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