Sick Thalsssemia

Sickle Thalassemia ~ An Introduction

 

What is Thalassemia? Thalassemia is a genetic blood disorder, that triggers production of defective hemoglobin. Hemoglobin is the protein in red blood cells and responsible for carrying oxygen throughout the body. Instead these cells are destructed in groups, leading to anemia. Therea are different types of Thalassemia including alpha-thalassemia, beta-thalassemia, Cooley's anemia and Mediterranean anemia. While alpha and beta are more common.

What is Sickle Cell Anemia? Sickle cell anemia is another hereditary disease, where abnormal crescent shaped red blood cells are formed. There are times when both these conditions can overlap. Both these conditions cause anemia. There is yet another condition called Hemoglobin sickle-beta Thalassemia, colloquially referred to as Sickle Thalassemia. Also called as sickle beta thalassemia, or sickle cell-beta thalassemia, is a different form of sickle cell anemia. This disorder occurs when a kid gets two tweaked HBB genes, wherein one parent carried beta Thalassemia and other carried sickle cell anemia. It is believed to inherited owing to an autosomal recessive fashion, resulting from mutations in hemoglobin. This condition is more common with those from Mediterranean ancestry, like Greeks, Italians and Turks

Here, the severity of the condition can vary. Symptoms help in determining the severity. When mild, suppression degree of beta-chain synthesis is partial. In this case, red blood cells tend to contain 25% - 35% of the normal hemoglobin content. The treatment is generally easier, with the condition considered mild and often, does not need medical attention. On the other hand, severe form is when the beta-chain synthesis is suppressed in entirety.

The treatment is pretty serious, similar to homozygous sickle cell anemia. For severe form, patients may need blood transfusion at regular intervals. Also, antibiotics are suggested to avert pneumonia and other conditions. Pain killers are prescribed for reducing bouts of pain, though, they just offer relief for the time being. Being a disorder of the blood, spleen is affected as an aftermath. It is enlarged and even when taking treatment, it remains so for few years. Immune protection offered is also less and that's the most important cause for fatalities due to Thalassemia and related disorders.

Sickle beta +-Thalassemia is identified through a test called electrophoresis. This is done when a baby is 2 – 3 months of age. They take blood samples from the baby and send it to a laboratory. This will reveal if the kid has sickle cell trait, or if it has other complications. Based on this, the need for treatment will evaluate. Blood transfusion is one of the most common treatment for most enduring different types of Thalassemia. Other methods include Iron overload and Chelation therapy. The treatment is chosen based on the severity, type of problem and the patient's health.

Problems and symptoms of this condition can be similar to that of sickle cell anemia. Health problems due to this condition can include mild to severe anemia, constant pain in legs, arms, back and stomach. Also there is a risk of blood infections, gall stones, spleen enlargement and abrupt dropping of blood count.

Even now, research is ongoing to determine the cause, treatment and other related issues due to sickle cell Thalassemia.