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

Comparing sickle cell disease patients with iron overload to those without iron overload, a review of clinical trials in 2018 showed that sickle cell patients with iron overload had higher rates of:

Mortality(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%.

Monitor renal toxicity

Kidney complications are common in patients with sickle cell disease – affecting 30-50% of adults. Regular monitoring of both renal and hepatic toxicity can help optimize chelation and ensure patients are on an appropriate chelator.

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

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

Sickle Cell Disease treatment guidelines support regular monitoring of serum ferritin, and cardiac and liver MRI T2*
ParameterTargetExpert panel recommendation
Serum ferritin25-300 ng/mLMonthly
Liver iron concentration (LIC)800-3,500 µg/g dry weightEvery 1-2 years
Cardiac MRI T2*>20 msPerformed for patients with:
  • sickle cell disease with a high iron burden (liver iron content >15 mg/g [dry weight (dw)]) for 2 years or more
  • evidence of end organ damage because of transfusional iron overload
  • or evidence of cardiac dysfunction

Iron chelation therapy results in better overall survival in sickle cell disease, but only if it is instituted early and compliance is maintained