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World Thalassemia Day to be observed today Prof. Nadeem Samad says Thalassemia patients on rise in Balochistan; awareness needed to be created among masses about blood disorder; bone marrow transplantation only treatment currently available.

QUETTA -- International Thalassemia Day is being observed today (Tuesday) in Quetta as elsewhere on the globe. The Pakistan Thalassemia Federation and other non-governmental organizations working in the field of Thalassemia have planned various activities including lecture and walk today to mark the Day in the metropolis.

The focus would be on the complicated blood disease, how it is caused and how it can be prevented.

The Incharge Thalassemia Care Center, Bolan Medical Complex Hospital and Hematologist, Professor Dr. Nadeem Samad told that the disease is increasing in the province mainly due to the lack of awareness.

He said that in 2010, a total of 644 Thalassemia patients were registered with the TCC of BMC while their number has reached to 1,000 plus now.

He said that since main reason behind increasing the number of patients is lack of awareness so it is desperately needed to be created among the people about the disease.

According to the experts, Thalassemia is an inherited blood disorder in which the red blood cells contain a reduced amount of hemoglobin, or in severe cases, a lack of hemoglobin in the cells. Hemoglobin is the oxygen carrying protein part of the red blood cell and a decreased level is the cause of most types of anemia.

Thalassemia can range from mild to life threatening and therefore should be monitored.

Patients with Thalassemia face two problems, either they cannot produce enough red blood cells or many cells are destroyed; therefore, there is a shortage of red blood cells in the blood stream. This in turn decreases the amount of oxygen which can be carried effectively throughout the body to vital organs.

Hemoglobin contains two different kinds of protein chains named alpha and beta chains. Any deficiency in these chains can cause abnormalities in the formation, size, and shape of RBCs. Also known as Cooley's anemia, beta globin anemia is more common and can range in severity. According to a report by Baker H. Al-Awamy, MD, PhD. in the Saudi Medical Journal, 2000; Vol. 21 (1): 8-17, the Alpha-thalassemia gene is highly prevalent in Saudi Arabia and the interaction with the sickle cell gene is commonly observed.

Additionally, Beta thalassemia is also common in Saudi Arabia along the coastal strip of the Red Sea and in the Eastern province of Al Ahsa. Therefore, in 2003 a royal decree was announced in December and implemented by a February 2004 order for the Saudi Ministry of Health to design a protocol for premarital testing in an effort to reduce possible future genetic problems in families.

Thalassemia can result in loss of iron through absorption in the intestinal tract which can cause Hemosiderosis. This in turn is probably responsible for damage to the heart, liver, pancreas, endocrine glands and other organs. Due to the presence of a large amount of fetal hemoglobin, a situation arises where there is not enough oxygen in the cells to function properly. Beta Thalassemia can cause shortness of breath, heart palpitations, fatigue, and enlarged bones due to the increased production of red blood cells in the bone marrow. In severe cases, patients may need blood transfusions.

If left untreated, severe anemia can result in insufficient growth and development, as well as other common physical complications that can lead to a dramatically decreased life-expectancy. According to G. Lucarelli in the New England Journal of Medicine 1987; 316: 1050-1055, bone marrow transplantation (BMT) is the only treatment currently available to cure Thalassemia.
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Publication:Balochistan Times (Baluchistan Province, Pakistan)
Date:May 8, 2012
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