World Thalassemia Day: All you need to know

With International Thalassemia Day being observed across the world today to raise awareness, here are certain things you'd like to know about it.

Written By: Agencies | Published : May 8, 2016 5:41 PM IST

Thalassemia, a blood disorder that originated in the Mediterranean region, involves the production of abnormal amounts of hemoglobin in the body. Also called the Mediterranean anemia, this disorder is known to be most widespread in African, Mediterranean, and Asian countries.Read: Donate blood. Help people with thalassemia.

Dr. Mandeep Singh, Sr. Consultant, Fortis Escorts Bone and Spine Institute, said, Thalassemia is a blood disorder, which is an inherited autosomal disorder where abnormal hemoglobin is formed in the body. This is due to a variant or the missing gene that determines how the body makes hemoglobin. The result is the improper transport of oxygen to the body and destruction of red blood cells. People suffering from thalassemia have less hemoglobin and fewer red blood cells which translate into anemia or anemic conditions.

Thalassemia is known to cause complications like an iron overload in the body, bone deformities, and cardiovascular illnesses. Research showed that this disease had resulted in 25,000 deaths in 2013. About to get married? Go and get yourself tested for thalassemia.

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Dr. Singh added, Presented as anemia, thalassemia is a condition that requires repeated blood transfusion. The combination of repeated blood transfusion and chelation therapy has dramatically increased the life expectancy of thalassemic patients. On the other hand repeated blood transfusion leads to iron overload.

Leads to these frequent endocrine changes:

Hypogonadism
Short stature
Diabetes mellitus
Hypoparathyroidism
Hypothyroidism
Bony changes
Skull: classic "hair-on-end "appearance
Facial bones: rodent facies
Ribs: "rib-within-a-rib" appearance
Premature fusion of the growth plate
Short stature
Bone pain
Vertebral fractures
High prevalence of osteoporosis and osteopenia is seen in these patients. BMD (bone mineral density) is done to assess the severity of osteoporosis. Zinc and vitamin D deficiency are reported frequently in these patients.

He further informed, Bone mass is reduced in children. Peak bone mass is suboptimal in adults. Fracture is common in these patients. Bone pain is more common in females. Treatment usually aims at improving BMD, by the use of calcium, vitamin D, and gonadal steroid replacement therapy. Read: Scientists develop a new treatment for Thalassemia.

Source: ANI News

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