Early Screening and Treatment of Heart Complication in Sickle Cell Disease

Early Detection and Management of Cardiac Iron Overload in Sickle Cell Disease Using Multimodal Imaging for Improved Clinical Outcomes

This study tests whether early heart screening and treatment for iron overload in subjects with sickle cell disease can prevent heart problems and reduce hospitalizations.

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