Hereditary Hemochromatosis (HH) is a genetic disorder of Northern European descent resulting in dysregulation of iron homeostasis through one of several possible mutations that leads to unchecked absorption of dietary iron and can result in long-term damage including:

• Liver failure and cirrhosis

• Elevated cancer risk

• Significant joint pain

• Cardiac complications

• Early onset dementia and other CNS-related complications

Iron is only eliminated through tissue loss (via bleeding or sloughing of skin and intestinal lining), making overaccumulation a significant problem.

Therapeutic phlebotomy can be effective, but suffers from multiple drawbacks:

• 50% of patients require more than six phlebotomies per year.

• A lifetime of phlebotomies carries significant patient burden.

• Studies show less than 25% long-term patient adherence.

• Patients are at risk of accumulating iron between procedures.

• Lack of iron control shows significant survival decrease and increase in hospital utilization.

Phlebotomy alone is inadequate to address the needs of HH patients, providers, and payers.

BBI-001 may be used in conjunction with, or in place of, phlebotomy maintenance therapy. The safety, tolerability, and convenience of BBI-001 oral maintenance therapy should facilitate adherence, and provide a therapy to maintain normal iron levels and improve health outcomes in hemochromatosis patients.

The use of BBI-001 may significantly reduce or altogether eliminate the need for phlebotomy.