Inadequate vs. appropriate iron chelation in thalassemia

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

About 70%

of deaths in transfusion-dependent thalassemia patients are due to heart complications and disease.

Despite improvements in care, cardiac iron overload is the leading cause of death among people with transfusion-dependent thalassemia

% of people with transfusion dependent thalassemia chart

Don’t forget your other organs!

Careful monitoring of liver and kidney function is important to prevent further damage to the organs. 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.

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

Lowering serum ferritin and liver iron concentration are not enough. Reducing cardiac iron is the priority in thalassemia.
Serum ferritin = test to assess iron level in the blood	<1,000 ng/mL	Monthly
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.	>20 milliseconds (ms)	Yearly (or every 6 months if your T2* is <10 ms)
Liver iron concentration = amount of iron in the liver	2-5 mg/g dry weight	Yearly

Lowering serum ferritin and liver iron concentration are not enough.
Reducing cardiac iron is the priority in thalassemia.

Parameter

Target

Recommended monitoring

<1,000 ng/mL

Monthly

>20 milliseconds (ms)

Yearly (or every 6 months if your T2* is <10 ms)

2-5 mg/g dry weight

Yearly

† MRI = magnetic resonance imaging

What to expect during an MRI T2* scan

MRI stands for “magnetic resonance imaging”. T2* is 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.

The MRI scanner is a large, donut-shaped magnet that has a tunnel in the middle. You will lie on a table that slides into the tunnel so a technician can take pictures. The scan is painless and takes anywhere from 20 to 90 minutes. It is important that you lie perfectly still during the scanning process. You may be given medications to help you relax or even fall asleep so that it’s easier to stay still. The machine is noisy so you might be given headphones or music to listen to.

Reducing iron overload in the heart is critical for people with thalassemia syndromes