STUDY NUMBER: 
CONTINUATION SHEET for either: 
NIH 2514-1, Consent to Participate In A Clinical Research Study 
NIH 2514-2, Minor Patient’s Assent to Participate In A Clinical Research Study 
CONTINUATION: page_Lof 1L pages. 
least 1.2 liters. Such individuals should have typical respiratory 
manifestations of CF but still be able to undergo the various tests such as 
bronchoscopy which are needed to prove effectiveness yet require a certain 
minimum lung function to be performed safely. 
Participants must have a Shwachman score of 40 to 70 (mild to moderate 
disease; see protocol section). This is a measure of the severity of CF based 
on various clinical criteria. If there is gastrointestinal (digestive) or 
liver disease present, it must be mild and stable with appropriate therapy. 
The Shwachman score criteria is required so that typical individuals with CF 
are studied but whose disease is sufficiently mild (or moderate) that they are 
able to undergo the somewhat lengthy protocol without excessive problems. 
Stable gastrointestinal and liver status are necessary to ensure that changes 
in the patient's non- respiratory status do not interfere with the evaluation 
of the impact of this experimental treatment with AdCFTR on the respiratory 
manifestations of CF. 
Patients with diabetes mellitus which is well controlled, that is, no episodes 
of diabetic ketoacidosis, hyperosmolar coma or hypoglycemic attacks 
(exceedingly high or low blood sugar levels) requiring hospitalization within 
the previous three years, are acceptable for inclusion. This criterion is 
intended to ensure that changes in the subjects non-respiratory status do not 
interfere with the evaluation of the impact of the experimental treatment on 
the respiratory manifestations of CF. 
Participants must be able to undergo nasal brushing without complications. 
Nasal brushing is a technique used to obtain lining cells from the nose. This 
is a critically important part of the protocol because evaluation of these 
cells will form one of the measures of success of transferring the normal CFTR 
gene to the participant's cells. It is important that the subject be able to 
undergo this procedure without the prospect of complications, for example a 
history of recurrent nosebleeds, abnormal anatomy of the nose or allergies to 
local anesthesia (numbing medicine placed in the nose to prevent discomfort 
when the brushing is performed). Should the subject have these or other 
problems which preclude the safe brushing of the nose they would not be able 
to participate in the protocol. 
Participants must be able to undergo fiberoptic bronchoscopy with brushing of 
the large airways and brochoalveolar lavage without complications . Fiberoptic 
bronchoscopy with brushing and bronchoalveolar lavage is an important part of 
the protocol without which it is impossible to measure the safety and 
effectiveness of this treatment in those areas of the lung most severely 
affected. These procedures are safe and relatively easily tolerated. They 
are described in more detail later in this consent form. Some subjects, 
however, may feel unable to undergo the procedures for either personal or 
I 
PATIENT IDENTIFICATION 
CONTINUATION SHEET for either: 
Recombinant DNA Research, Volume 16 
[777] 
