IRON OVERLOAD IN SICKLE CELL DISEASE
There is a lot of text on the topic of iron overload in patients with SCD that I can't possibly hope to cover in depth here yet it should be stated that this is a major and unavoidable complication of transfusions that can not be ignored.
Already addressed in my last two posts is what blood transfusions do for people with sickle cell and how in many cases it saves lives via its treatment of of acute chest syndrome (ACS) and stroke. Transfusions typically involve red blood cells which naturally will provide additional iron to the body and as a result, long term transfusion therefore becomes associated with significant iron overload.
For patients with Thalassemia Major (TM), long term transfusion causes iron overload that result in cardiac damage, liver fibrosis, gonadal dysfunction and growth retardation. However, for patients with SCD, they are relatively protected from iron-induced cardiac and endocrine damage as compared with TM patients. This is because sickle cell disease patients do not hyper-absorb iron as is the case in thalassemia and so there remains a level of uncertainty of transfusion history in SCD. As a result of this, 'ferritin' levels are/should be obtained annually in any SCD patient who has been transfused to screen for possible iron overload.
FERRITIN IS A BLOOD CELL PROTEIN THAT CONTAINS IRON. A FERRITIN TEST HELPS YOUR DOCTOR UNDERSTAND HOW MUCH IRON YOUR BODY IS STORING.
I recall saying in my previous post that my sister gets 'exchange transfusions' once a month but has to go in 2 days ahead of her appointment for all the necessary checks to ensure it is safe for her to get transfused. Her ferritin levels and iron concentration levels are checked and if there is any appearnace of of iron overload or build up, iron chelation therapy is employed (which is the use of drugs to remove the extra iron in her body). Symptoms of iron overload include tiredness/weakness, joint aches or pain, liver disease, shortness of breath, diabetes among several others. For some people it can take many years for the build up of iron to cause problems yet for others, iron overload can happen quickly and because symptoms can resemble that of other diseases, iron overload can be difficult to detect. If left untreated, can cause damage and one can become sick very quickly.
It is important to keep track of how many units of blood you receive with every transfusion and may need to get tested for iron overload after only 10 episodes of transfusion. Remember to always talk with your doctor about any symptoms you experience after transfusion because as already seen, iron overload in SCD can be dangerous and may become lethal.
Always Love 💘