Collection of Airway, Blood and/or Urine Specimens From Subjects for Research Studies

This is a protocol to obtain blood, urine and/or airway specimens from normal individuals and
individuals with lung disorders in order to carry out laboratory studies looking at genetic
expression, gene transfer, infection, proteins, human genes, and to store specimens for
future genetic studies. Specimens collected may include blood, urine and airway samples
(nasal, airway brushing, biopsy and/or washings) from consenting subjects. Subjects will
include both individuals diagnosed with lung disease and healthy control subjects. The
purpose of this study is to obtain biologic materials from the blood, airways and/or urine of
normal individuals and individuals with lung disease. The normal are used to establish a set
of normal ranges for various parameters. These provide control information when compared to
individuals with various pulmonary diseases, and will help in understanding of the etiology
and pathogenesis of various lung diseases. The investigators will use bronchoscopy (inserting
a scope into the lungs) to obtain airway cells by brushing, biopsy and/or washings in
individuals with lung disease and in healthy controls. By studying those cells, the
investigators hope to learn more about the specific causes of lung disease, how lung disease
manifests and progresses, and how lung disease can be treated.

Inclusion Criteria:

Group 1.1 - HEALTHY VOLUNTEER RESEARCH SUBJECTS (Healthy as defined by those not having
lung disease and inclusive of: all races, ethnicities, sex, HIV status, smoking status and
multiple birth status, etc., within the general population. Smoking status is defined for
individuals that may use any of the following: cigarettes, pipes, E-cigarettes, waterpipe,
shisha, etc.):

- All study subjects should be able to provide informed consent

- Males or females ages 18 years and older

- Must provide HIV informed consent

Group 1.2 - VOLUNTEER RESEARCH SUBJECTS WITH LUNG DISEASE (As defined by those having lung
disease or symptoms of lung disease and inclusive of: all races, ethnicities, sex, HIV
status, smoking status and multiple birth status, etc., within the general population.
Smoking status is defined for individuals that may use any of the following: cigarettes,
pipes, E-cigarettes, waterpipe, shisha, etc.):

- Must provide informed consent

- Males and females age 18 years and older

- Lung disease proven by at least one of the following: symptoms consistent with
pulmonary disease; (2) chest X-rays consistent with lung disease; (3) pulmonary
function tests consistent with lung disease; (4) lung biopsy consistent with lung
disease; (5) family history of lung disease; and/or (6) diseases of organs with known
association with lung disease

- Must provide HIV informed consent

Additional Inclusion criteria for CF subjects:

• All CF subjects will be homozygous for the ΔF508 mutation, with mild-moderate lung
disease as defined by FEV1 ≥ 50%

Group 2 - WCMC/NYPH CLINICAL PATIENTS

- Must provide informed consent

- Males and females, age 18 years and older

- Lung disease proven by at least one of the following: (1) symptoms consistent with
pulmonary disease; (2) chest X-rays consistent with lung disease; (3) pulmonary
function tests consistent with lung disease; (4) lung biopsy consistent with lung
disease; (5) family history of lung disease; and (6) diseases of organs with known
association with lung disease

Group 3 - PCNY CLINICAL PATIENTS VOLUNTEER RESEARCH SUBJECTS WITH LUNG DISEASE

- Must provide informed consent

- Males and females, age 18 years and older

- Lung disease proven by at least one of the following: symptoms consistent with
pulmonary disease; (2) chest X-rays consistent with lung disease; (3) pulmonary
function tests consistent with lung disease; (4) lung biopsy consistent with lung
disease; (5) family history of lung disease; and (6) diseases of organs with known
association with lung disease

Exclusion Criteria:

Group 1.1 - HEALTHY VOLUNTEER RESEARCH SUBJECTS

- Individuals not deemed in good overall health by the investigator during screening
visits and/or prior to the bronchoscopy will not be accepted into the study.

- Individuals with history of chronic lung disease, including asthma or with recurrent
or recent (within three months) acute pulmonary disease will not be accepted into the
study.

- Individuals with allergies to atropine or any local anesthetic will not be accepted
into the study.

- Individuals with allergies to pilocarpine, isoproterenol, terbutaline, atropine or
aminophylline will not be accepted into the study.

- Females who are pregnant or nursing will not be accepted into the study

Group 1.2 - VOLUNTEER RESEARCH SUBJECTS WITH LUNG DISEASE

- Any history of allergies to xylocaine, lidocaine, versed, valium, atropine,
pilocarpine, isoproterenol, terbutaline, aminophylline, or any local anesthetic will
not be included in the study

- Individuals deemed unable to complete the bronchoscopy due to health concerns by the
investigator.

- Females who are pregnant or nursing

Additional Exclusion criteria for CF subjects:

- Experienced a recent (≤ 8 weeks) pulmonary exacerbation of their disease

Group 2 - WCMC/NYPH CLINICAL PATIENTS

- Patient refuses consent

Group 3 - PCNY CLINICAL PATIENTS

- Patient refuses consent