Our Iron Will: Sickle cell disease
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Sickle cell disease (SCD) is a severe haematological disorder, caused by an inherited mutation of the beta globin gene.10
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Sickle cell disease (SCD) is a chronic haemolytic anaemia.
Normal red blood cells are round and flexible, which lets them travel through small blood vessels. In SCD red blood cells are crescent shaped, like a sickle, that sticks to the walls of blood vessels, blocking the blood flow.10
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Sickle cell disease symptoms can vary from person to person and can change over time. 11
Blood transfusions increase the oxygen carrying capacity of blood and reduce the complications of vaso-occlusion.12

Red blood cell transfusions have become the standard of care in sickle cell disease treatment.13
The lack of adherence to iron chelation therapy will result in iron overload.12

As iron continues to build up in the body, complications happen more often

People with sickle cell disease and iron overload§ had higher rates of:13

Death (64% vs. 5%)

organ failure (71% vs. 19%)

pain episodes (64% vs. 38%)

§Defined as serum ferritin levels >1,500 ng/mL and transferrin saturation >50%

Possible effects on your kidneys

Kidney complications are common in people with sickle cell disease – affecting 30-50% of adults. Careful monitoring of kidney function is important to prevent further damage to the kidneys. Your doctor will give you all the information about kidneys, but also liver complications, and how to take care of their health. Your doctor may recommend that you see a specialist to evaluate your kidney function.14

Having your iron levels checked regularly is the way to know if your iron chelation therapy is working

Iron chelation needs to be adjusted regularly or even switched depending on iron level, weight, and tolerance to side effects.1,2
There are a number of parameters that need to be monitored during the iron chelation therapy, such as:1,15,16
Serum ferritin: Liver iron concentration: Cardiac MRI T2*:
ferritin plays an important role in the storage of intracellular iron and is widely used in diagnosing and monitoring iron overload in SCD.15 one of the liver functions is to store iron for when the body needs it. Liver is also a primary target for iron toxicity and its functions need to be monitored regularly.1 when the storage capacity of ferritin is exceeded, iron is deposited in the myocardium with the risk of cardiac failure. Cardiovascular magnetic resonance imaging (cardiac MRI) is a useful noninvasive tool for evaluating the amount of iron in the heart.16

Your doctor will regularly check your values for each of these parameters, according to requirements and with the aim to assure that your chelation treatment maintains iron levels within recommended ranges.

Patients & Caregivers: in case of need to report an adverse drug reaction, please refer to your physician, asking him to fill in and submit the relevant case report to the concerned Health Authority, according to the Pharmacovigilance requirements in force in your Country. Nevertheless, please be kindly reminded that each patient can report any such cases directly to the national reporting system.