Early Screening and Treatment of Heart Complication in Sickle Cell Disease

The goal of the study is to detect iron-related heart problems in subjects with sickle cell disease (SCD) through regular cardiac imaging (electrocardiogram, echocardiogram, cardiac MRI) and lab testing every 3 months during the 12 months period. The treatment will be with iron chelation and guideline-directed heart medications.

Inclusion criteria

• At least 18 years of age
• Diagnosis of sickle cell disease (any genotype)
• Serum ferritin levels ≥ 400 ng/mL (up to 80 patients), or less than 400 ng/mL in those who have cardiac symptoms including shortness of breath and lower extremity edema (up to 20 patients)
• Willingness to undergo regular imaging (echocardiograms, ECG, cardiac MRI)
• ECOG performance status of 0-1
• Able to read, understand and provide written informed consent
• Deemed appropriate for participation by the treating physician

Exclusion criteria

• Unable to schedule and required follow-up visits
• Medical comorbidities including:
• Known heart failure
• Unstable angina
• Uncontrolled dysrhythmias
• Acute pulmonary embolism
• Active infection or severe comorbid conditions that in the view of the investigator would limit participation
• History of hypersensitivity or contraindication to chelation therapy
• Severe renal or hepatic impairment
• Pregnancy of breastfeeding