Environment Effect on Six-Minute Walk Test Performance

Participants will be asked to come to the Clinical Research Center (CRC) for a visit lasting
about one hour. Participants will be asked to review and sign an informed consent document to
participate in this trial. If participants agree to participate and sign the consent, the
following procedures will be done. If they have not had pulmonary function testing performed
in the past 18 months, standard spirometry testing will be performed. Participants will
perform two six-minute walk tests, one indoors and one outdoors. First, they will be randomly
assigned to perform either the indoor or outdoor testing first. Then participants will have
their vital signs taken, perform the first six-minute walk study and immediately after have
their vital signs taken again. Participants will have a rest period between the two
six-minute walk tests of at least 30 minutes. Participants will then perform the second
six-minute walk test with the same above procedures for the first test.

Inclusion Criteria:

1. Age above 18 years.

2. Have a diagnosis of congenital Alpha-1 Antitrypsin Deficiency with an allelic
combination of ZZ.

3. Have a Forced Expiratory Volume in 1 second (FEV1)< 50% of predicted and FEV1/forced
vital capacity (FVC) < 70% (Global Initiative for Chronic Obstructive Lung Disease
[GOLD] stage II, III, or IV).

4. Not currently prescribed Oxygen replacement at a rate of greater than 2 liters.

5. Willing and able to perform two six-minute walk tests.

6. Willing and able to provide written informed consent.

Exclusion Criteria:

1. Subject has had a moderate or severe pulmonary exacerbation during the 4 weeks before
the study visit.

2. History of lung transplant.

3. Severe concomitant disease (e.g., congestive heart failure, clinically significant
pulmonary fibrosis, malignant disease [with the exception of skin cancers other than
melanoma], history of acute hypersensitivity pneumonitis reaction, or current chronic
hypersensitivity pneumonitis).

4. Unwilling or unable to perform six-minute walk testing.

5. Mentally challenged adult subjects who cannot give independent informed consent.

6. In the opinion of the investigator the subject may have compliance problems with the
protocol and the procedures of the protocol.

7. American Thoracic Society listed absolute contraindications to six-minute walk test:
unstable angina or myocardial infarction during previous month.