Revised 8-26-94 
oxygen gas through the nose. The bronchoscope will be introduced into the nose 
(or mouth if necessary) and passed down the breathing tubes into the lungs; 
you may cough; this is normal and will be controlled as much as possible by 
the doctor and nurse performing the procedure. Both the vehicle alone and the 
AdsvCFTR.lO will be administered through the bronchoscope at the appropriate 
times during the protocol. When the bronchoscope is in place a small brush may 
also be placed through the bronchoscope onto the surface of the airways and 
the airway gently brushed. This is a painless procedure and is very safe, the 
only common problem being some slight bleeding at the site of the brushing. It 
is unlikely this will affect your health. When the brushing has been performed 
the patient may then undergo bronchoalveolar lavage and bronchial biopsy. 
Bronchoalveolar lavage involves wedging the bronchoscope into the air passages 
of a small part of the lung and instilling salt water through the bronchoscope 
so as to gently wash the inner surface of the airways. Once the salt water has 
been instilled it is then gently suctioned out of the lung and collected. This 
procedure is safe. In over 2000 bronchoalveolar lavages performed by the in- 
vestigators over more than a decade, there have been no life threatening com- 
plications. However, individuals with CF have a higher incidence of mild com- 
plications with bronchoalveolar lavage than others. These complications in- 
clude fevers (about 65% of CF individuals, usually self -limiting or relieved 
by anti-fever medications) and chills (about 20% of CF individuals, again 
self - limiting or relieved by anti-fever medications), more infrequently (less 
than 5%) infections or coughing up blood. Bronchial biopsy is a procedure 
where a small sharp clamp is placed down the bronchoscope to snip a small 
piece of tissue of the inside surface of the wall of a large airway. This is 
not painful. The tissue is then prepared and examined under a microscope. It 
is a routine test that is used for diagnosis of lung disorders and for re- 
search purposes. The risks to this procedure are very low, but include bleed- 
ing or leak of air from the wind pipe into the chest cavity. 
In individuals with cystic fibrosis, electrical charges on the inside of the 
airway are significantly more negative than in normal individuals. It is thus 
important to measure these charges during this protocol to see whether they 
return towards normal following administration of AdcvCFTR.10. An intravenous 
("i.v.") catheter will be placed just beneath the surface of the skin in your 
arm and connected to an infusion of salt water. A second catheter, constantly 
rinsed with salt water and medications to modify the movement of salt and 
water in the respiratory tissues, will be placed on the surface of the airways 
to measure the charge there. This second catheter is placed on the airways 
through the bronchoscope and constantly rinsed with a small amount of a salt 
solution and medications that will stimulate the movement of salt and water in 
the cells lining and airways. Both catheters are connected to a chart recorder 
which records the difference in electrical charge between the two surfaces. No 
hazards are anticipated or special precautions required. 
All the bronchoscopy procedures combined will last between 35-50 minutes. Once 
all the tests are performed the tube will be removed and you will return to 
his or her room. For three hours after the bronchoscopy you will not be al- 
lowed to eat or drink while then medicine and effects of the bronchoscopy wear 
off. Bronchoscopy may be performed several times during the protocol, includ- 
ing: one to two times in the baseline period, one time in the vehicle control 
period, and eight to twelve times in the 6 -month period of the experimental 
therapy period. 
Recombinant DNA Research, Volume 20 
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