ECHO – PART B

ABCI is being developed for the treatment of cystic fibrosis (CF). CF is a genetic condition that can affect many body systems, but it mainly affects the lungs, liver, and pancreas. CF affects the cells in the body that produce mucus, sweat and digestive fluids. People with CF are lacking an ion channel that plays an important role in balancing salts and water on the surface of cells in the body. Normally secreted fluids are thin and slippery, but in people with CF, the defective gene causes the secretions to become sticky and thick. In people with CF, these secretions plug up tubes, ducts, and passageways in the body, especially in the lungs and pancreas. CF affects approximately 81,000 individuals worldwide, and more than 540 people in New Zealand.

ABCI is a new inhaled formulation of the antifungal drug amphotericin B (AmB) and works by creating new ion channels and helps to correct the salt and water balance on cell surfaces, reducing the thickness of secretions. It is hoped that, by targeting the lungs directly with an inhaler and by helping to normalise lung secretions, ABCI may be an effective treatment for CF. Other formulations of AmB have been approved and used around the world.

Study Visits: 2 x 2-night stays (4 nights total)

Outpatient Visits: 34 clinic visits

Reimbursement: $7,500