Is Having Thalassemia Dangerous?
Yes, having thalassemia is indeed dangerous as it can cause organ failure and death. Thalassemia is the name of a genetic blood illness. Those who have this illness by birth are unable to produce normal hemoglobin that is required for producing healthy red blood cells. This illness is not infectious and does not pass between persons by contact. This illness is prevalent in families of South Asian, Chinese, Mediterranean, Middle Eastern, or African lineage.
The blend of a gene from the mom and a gene from the dad causes three diverse sorts of thalassemia. They are Thalassemia Minor, Intermediate, or Major.
Systematic blood transfusions happen to be the sole treatment for those who have thalassemia. It lets all patients of thalassemia live fairly normal lives. Nonetheless, a cure is yet to be found for this illness. The genetic reason for thalassemia was among the foremost genes discovered during the 1970s. However, more than 40 years have passed and there is no gene therapy for thalassemia patients.
A good number of patients of thalassemia major need transfusions at intervals of 2-4 weeks, based on the amount of infused cells that an individual is capable of consuming. It is fact that systematic transfusions contribute much to how well and for how long a patient of thalassemia major lives. However, they also leave them with a surplus of iron. This is a dangerous side effect and is referred to as “iron overload.”
How Do Blood Transfusions Leave The Patients Of Thalassemia With Surplus Iron?
Systematic blood transfusions offer thalassemia patients the red blood cells that they require for surviving. After the breakdown of these red blood cells, the body has a surplus of iron.
Iron overload ensues very quickly in patients who are undergoing prolonged transfusion programs. Human beings do not have a mechanism besides the shedding of the mucosa in their menstruation or gastrointestinal tracts for excreting surplus iron. Thus, patients who are getting transfusions at intervals of three to four weeks gain iron of 0.5 kg daily besides natural losses.
Surplus Iron Is Dangerous
Despite iron being vital to the body, surplus iron can be the cause of organ failure and passing away. It is compulsory that this surplus iron is got rid of as it gets stored in the important organs of the human body, examples being the liver and heart.
The sole treatment options for the removing of surplus iron are phlebotomy & chelation. Although phlebotomy is a successful way of doing away with iron it is not the good one for thalassemia patients.
The main treatment for thalassemia patients with iron overload is chelation.
Under regular circumstances, in a human being, iron is carried bound to a transferrin, a carrier protein. Transferrin carries iron into definite tissues. As it is bound to this particular protein, other tissues do not get the noxious effects of free iron. A Patient of thalassemia on prolonged transfusion rapidly obtains much more iron compared to what transferrin can bind, and the blood has more free iron. This iron is directly noxious to one’s heart and more tissues.
Iron chelation therapy has two objectives, namely, the binding of noxious iron inside the plasma and the elimination of iron from one’s body.
Usually, it takes between three and ten years of prolonged exposure to high iron levels before quantifiable organ dysfunction occurs. The good thing is that this implies that there is time for implementing treatment strategies for reducing iron loading.
However, based on the organ, the time taken for significantly reducing iron can be long. Thus, the finest tactic is to act fast and tries to stop considerable iron loading right from the beginning.
Generally, considerable liver iron loading is detectable approximately after monthly transfusions have been done for six months. However, cardiac loading takes approximately between eight and ten years.
Generally, chelation must be begun right after a patient becomes considerably iron loaded. As the elimination of iron from regular tissues can cause toxicity due to over-chelation, it is important to postpone the commencement of chelation until a patient is considerably iron loaded. As iron loading ensues much faster than the development of toxicity, this postponement will not put a patient in danger.