A Rare Disorder Called Thalassaemia

Thalassaemia or Thalassemia is a rare case of hemolytic anemia. Normally, iron is good for anemic patients but for people suffering from this health disorder, iron should be reduced. Excessive iron destroys the other body organs of a patient like the spleen. Also, the patient’s bone marrow does not produce enough red blood cells (RBC) and that causes patient to become weaker and gets even paler. The sickness refrain patient from eating foods that are rich in iron and prevents one from taking in any medications with iron as well.

Due to lack of RBC the patient has to undergo a regular monthly blood transfusion. The transfusion requires 100cc of Blood Type-B and a pack of RBC each session. Unfortunately, lots of iron enters patient’s body every time transfusion takes place and because of this, the patient has to undergo an iron-chelating treatment. This is a procedure wherein a 0.5 grams of Desferal (deferoxamine mesylate), an iron-chelating agent mixed with distilled water is infused to patient’s stomach subcutaneously through an infusion pump that looks like a VHS or the old Beta tape for eight to 12 hours. It is a painful process in the beginning that one has to endure but when one gets used to it – it is like a part of his daily routine.

Desferal is a product available only from Ciba-Geigy with its main laboratory located in Basel, Switzerland. For five consecutive days after each blood transfusion patient must consume one Desferal and another one every other day. Excessive irons wash out from the body through urine.

The process is painful because the entry point of this Desferal to patient’s body is through a needle attached to his stomach from the end of the syringe in the infusion pump which normally takes eight long hours to complete the process.

The infusion pump enables the Desferal to get into patient’s body slowly but surely – the one and the only safest way to do it. There is no other way than this because Desferal can not be taken orally otherwise it might cause severe complications to the patient.

This medical disorder is expensive as the infusion pump in the early 80’s cost more or less $500; plus the expenses every transfusion; the Desferal and the distilled water; and other medications necessary to purchase.

From time to time Ciba-Geigy organizes meetings with parents whose child have Thalassaemia. This gives them opportunity to discuss problems their children undergo and share knowledge with one another.

There are actually various types of Thalassemia considering that this is not a single disorder but a group of related disorders that affect the human body in similar ways. The hemoglobin is the oxygen-carrying component of RBC and is consists of two different proteins If the body doesn’t produce enough of either of these proteins, the RBC do not form properly and cannot carry sufficient oxygen. The result is anemia that begins in early childhood and lasts throughout life and might eventually lead to this condition called Thalassemia.