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:

Pain episodes (64% vs. 38%)

Organ failure (71% vs. 19%)

Death (64% vs. 5%)

Defined as

Serum ferritin = test to assess iron level in the blood

levels >1,500 ng/mL and

Transferrin saturation = amount of iron bound by proteins (transferrin) in the blood

>50%

Consider 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. Talk to your doctor and ensure you are on an appropriate iron chelator at the right dosing level that doesn’t impact your kidneys or liver. Your doctor may recommend that you see a specialist to evaluate your kidney function.

The only way to know if your iron chelator is working is to have your iron levels checked regularly

Iron chelation needs to be adjusted regularly or even switched depending on your iron level, weight, and tolerance to side effects

Sickle Cell Disease treatment guidelines support regular monitoring of serum ferritin, and MRI T2* of the heart and liver
ParameterTargetRecommended Monitoring

Serum ferritin = test to assess iron level in the blood

25-300 ng/mLMonthly

Liver iron concentration = amount of iron in the liver

0.8-3.5 µg/g dry weightEvery 1-2 years

T2* = a type of MRI sequence that uses relaxation time of two protons to measure visible differences on images. In this case T2* represents the amount of iron build-up. T2* scans can be conducted on the heart and liver and the same time.

>30 milliseconds (ms)Performed if you have:
  • sickle cell disease with a high iron load (liver iron concentration of >15 mg/g [dry weight (dw)]) for 2 consecutive years or more
  • signs of organ damage because of transfusional iron overload
  • or signs of damage to heart function

MRI = magnetic resonance imaging

With the right iron chelation for you, treatment helps to maintain iron levels within recommended ranges.


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