Dear Alpha-1 Research Registry Member,

We are contacting you to follow-up about a research study sponsored by KAMADA High Quality Pharmaceuticals. As you may know the current treatment for individuals who have been diagnosed with Alpha-1 deficiency is augmentation therapy. Augmentation therapy involves weekly infusions of the alpha-1 antitrypsin protein; and until other therapies become available it is considered ongoing and lifelong. This study will be looking at an inhaled form of the human Alpha- 1 antitrypsin (AAT) protein. This is a double-blind study of the daily use of “Kamada- API for inhalation” that will require approximately 8 visits to a study center. Double blind means that Alpha-1 antitrypsin deficient individuals with lung disease will be randomized to receive the study medication at one of two doses (80mg/day or 120 mg/day) or a placebo (a substance containing no study drug). Inhalation of the drug will be done at least once, but up to two times a day. Study procedures include a bronchoscopy at the beginning and end of the study period, blood tests, and physical exams.

Eligible participants must:  Be 18-65 years of age  Have a diagnosis of Alpha-1 deficiency- ZZ or ZNull (have an AAT level of less than 11 uM)  Not have any Alpha-1 augmentation therapy for at least 8 weeks before the first dose of study drug

Many exclusions to participation exist, including having had a lung transplant, or lung surgery within the last 2 years, current use of high dose steroids, active smoking within 12 months of screening visit, pregnancy, or a COPD exacerbation within 6 weeks of the screening visit. More of these will be explained to you when you contact a participating site. Travel reimbursement may be available through AlphaNet or the participating medical center, please ask the center you would like to visit for details.

There are 2 medical centers currently enrolling participants; University of Texas Health Science at Tyler and the University of Florida. Please see the enclosed letter for more detailed information.

To contact the University of Texas Health Science Tyler, please call 903-877-5518 or email Jan Hoeft at [email protected].

To contact the University of Florida, please call 866-229-6312 or email Pam Schreck at [email protected] or Joanna Nolte at [email protected].

Thank you for your consideration of this study and your participation in the Alpha-1 Research Registry. If you have any questions please feel free to respond to this email or call us toll free at 1-877-886-2383.

Sincerely,

Charlie Strange, M.D. Director, Alpha-1 Foundation Research Registry Professor, Medical University of South Carolina