PFT Analysis Report (Revised 13-Jul-2018)

Sponsor: Novartis Protocol: CQAW039A2315 ERT Account: 800387

Investigator: Atienza, Tito Sponsor Site ID: 3553 Measurement Type: Forced Spirometry
Patient ID: 3553002 SYS#: 800387-040928-000007
Visit: V1 Timepoint: PRE PFT Transmittal Number: 42134303
Ethnicity: South East Asian Sex: Male Height: 155.0cm
Age: 21.00
Collection Date: 06-Jul-2018 Collection Time: 07:48:42 Collected by: Tabag, Oliver
Original Data
QC Statements:
[Forced Spirometry All Efforts] Data meets ATS/ERS criteria for acceptable quality; patient might be randomized. Please check
other screening visit PFT data for quality messages and observe study inclusion/exclusion criteria.
[Forced Spirometry Best FEV1] Subject did not exhale for longer than 6 seconds.
[Forced Spirometry QC Grade] Acceptable Forced Spirometry measurement received.
Parameter Pred 1st 2nd 3rd 4th 5th 6th 7th 8th Best %Pred
Trial Time 07:49:18 07:50:41 07:52:08 07:53:36
FEV1 [L] 3.152 2.041 1.989 *2.057 2.047 2.057 65
FVC [L] 3.524 2.481 2.481 *2.509 2.484 2.509 71
FEV1/FVC 0.898 0.823 0.802 0.820 0.824 0.820 91
Error Codes D D
Operator Comment: acceptable
After BTR
BTR Statements:
[Forced Spirometry BTR] Forced Spirometry measurement unchanged.
[Forced Spirometry BTR] Data meets ATS/ERS criteria for acceptable quality; patient might be randomized. Please check other
screening visit PFT data for quality messages and observe study inclusion/exclusion criteria.
[Comments Acceptable Grade after BTR] FEV1 is acceptable however FVC is underestimated (Technician must enter a comment
and FEV1 must be valid in order for the effort to be considered Acceptable).
[Forced Spirometry BTR Grade] Acceptable Forced Spirometry measurement after BTR.
[OR Note] Grades and comments revised per email from site "patient cannot exhale longer"
[OR Note] Please remember to always provide a comment when the subject cannot exhale longer than 6 seconds
Parameter Pred 1st 2nd 3rd 4th 5th 6th 7th 8th Best %Pred
Trial Time 07:49:18 07:50:41 07:52:08 07:53:36
FEV1 [L] 3.152 2.041 1.989 *2.057 2.047 2.057 65
FVC [L] 3.524 2.481 2.481 *2.509 2.484 2.509 71
FEV1/FVC 0.898 0.823 0.802 0.820 0.824 0.820 91
Error Codes D D
OverReader: Brissenden, Debbie Date: 06-JUL-2018 15:02:08 EDT -04:00
QC By: Austin, Carrie Date: 13-JUL-2018 17:25:03 EDT -04:00
Error Codes:
A No repeatability; Less than 3 accepted forced measurements E No plateau was detected at the end of the expiration
B FEV1 repeatability is unacceptable F Back extrapolation volume was too large
C FVC repeatability is unacceptable H Late peak flow detected
D Expiration time was too short I Coughing was detected in the first part of the expiration
The following analysis information was modified from the previous report:
QA Statement Added: [Forced Spirometry All Efforts] Data meets ATS/ERS criteria for acceptable quality; patient might be
randomized. Please check other screening visit PFT data for quality messages and observe study inclusion/exclusion criteria.
QA Statement Added: [Forced Spirometry QC Grade] Acceptable Forced Spirometry measurement received.
QA Statement Deleted: [Forced Spirometry All Efforts] Patient CANNOT be randomized, because data does not meet the
ATS/ERS criteria for acceptable quality PFT data at screening visit.
QA Statement Deleted: [Forced Spirometry QC Grade] Unacceptable Forced Spirometry measurement received.
BTR Statement Deleted: [Forced Spirometry BTR] Patient CANNOT be randomized, because data does not meet the ATS/ERS
criteria for acceptable quality PFT data at screening visit.
BTR Statement Added: [Forced Spirometry BTR] Data meets ATS/ERS criteria for acceptable quality; patient might be
randomized. Please check other screening visit PFT data for quality messages and observe study inclusion/exclusion criteria.
BTR Statement Added: [Comments Acceptable Grade after BTR] FEV1 is acceptable however FVC is underestimated
(Technician must enter a comment and FEV1 must be valid in order for the effort to be considered Acceptable).
BTR Statement Deleted: [Comments Unacceptable Grade after BTR] Subject did not exhale for longer than 6 seconds.
BTR Statement Deleted: [Forced Spirometry BTR Grade] Unacceptable Forced Spirometry measurement after BTR.
BTR Statement Added: [Forced Spirometry BTR Grade] Acceptable Forced Spirometry measurement after BTR.
BTR Statement Added: Grades and comments revised per email from site "patient cannot exhale longer"
BTR Statement Added: Please remember to always provide a comment when the subject cannot exhale longer than 6
seconds
BTR Statement Deleted: please provide a comment subject cannot exhale any longer
QC By: Taylor, Colleen Date: 08-JUL-2018 22:58:20 EDT -04:00

Generated on: 13-JUL-2018 17:30:08 EDT -04:00 Page 1 of 4

 PFT Analysis Report (Revised 13-Jul-2018)

Sponsor: Novartis Protocol: CQAW039A2315 ERT Account: 800387

Investigator: Atienza, Tito Sponsor Site ID: 3553 Measurement Type: Forced Spirometry
Patient ID: 3553002 SYS#: 800387-040928-000007
Visit: V1 Timepoint: PRE PFT Transmittal Number: 42134303
Ethnicity: South East Asian Sex: Male Height: 155.0cm
Age: 21.00
Collection Date: 06-Jul-2018 Collection Time: 07:48:42 Collected by: Tabag, Oliver
For Site Use Only: Please sign and date and file with your source documents.

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Printed Name of Investigator Signature Date

Comments

Generated on: 13-JUL-2018 17:30:08 EDT -04:00 Page 2 of 4

 PFT Analysis Report (Revised 13-Jul-2018)

Sponsor: Novartis Protocol: CQAW039A2315 ERT Account: 800387

Investigator: Atienza, Tito Sponsor Site ID: 3553 Measurement Type: Forced Spirometry
Patient ID: 3553002 SYS#: 800387-040928-000007
Visit: V1 Timepoint: PRE PFT Transmittal Number: 42134303
Ethnicity: South East Asian Sex: Male Height: 155.0cm
Age: 21.00
Collection Date: 06-Jul-2018 Collection Time: 07:48:42 Collected by: Tabag, Oliver

Original Original

After BTR After BTR

Generated on: 13-JUL-2018 17:30:08 EDT -04:00 Page 3 of 4

 PFT Analysis Report (Revised 13-Jul-2018)

Sponsor: Novartis Protocol: CQAW039A2315 ERT Account: 800387

Investigator: Atienza, Tito Sponsor Site ID: 3553 Measurement Type: Forced Spirometry
Patient ID: 3553002 SYS#: 800387-040928-000007
Visit: V1 Timepoint: PRE PFT Transmittal Number: 42134303
Ethnicity: South East Asian Sex: Male Height: 155.0cm
Age: 21.00
Collection Date: 06-Jul-2018 Collection Time: 07:48:42 Collected by: Tabag, Oliver
Original

After BTR

Generated on: 13-JUL-2018 17:30:08 EDT -04:00 Page 4 of 4