ECFS Patient Registry Variables Conformity Survey

Thank you for sending your data to the ECFSPR.

To identify any differences in the variables definitions/coding between your national database and the ECFSPR we kindly ask you to fill in this questionnaire. The information will help us in the data analyses, and differences will be highlighted in the annual data report as appropriate. Your help and collaboration in this survey is greatly appreciated.

NB You do not need to login to submit this information.

Introductory Information

This data is submitted by (submitter's name) *

Email *

Please enter the email address where we can contact you.

Country *

Please report the estimated coverage of your national CF population attained by your registry, if known *

Please indicate briefly how the coverage estimation is made (e.g. ad hoc study, expert judgement etc.) *

Which year does your follow-up data refer to? *

1. DEMOGRAPHIC

1.1 Year of Birth

1.1.1 Did you send the variable to the ECFSPR? *

Yes

1.2. Month of birth

1.2.1 Did you send the variable to the ECFSPR? *

Yes

The ECFSPR requires this variable to be coded as follows: 2 digits (1 to 12) If unknown use 7

1.2.2 In the Excel file, did you code this variable according to the coding above? *

Yes

1.2.3 What is the difference in your coding? *

1.3. Day of birth

1.3.1 Did you send the variable to the ECFSPR? *

Yes

The ECFSPR requires this variable to be coded as follows: For data protection issues this field should be set to 15. We do not collect day of birth, only month and year.

1.3.2 In the Excel file, did you code this variable according to the coding above? *

Yes

1.3.3 What is the difference in your coding? *

1.4 Gender

1.4.1 Did you send the variable to the ECFSPR? *

Yes

The ECFSPR requires this variable to be coded as follows: 1 male 2 female

1.4.2 In the Excel file, did you code this variable according to the coding above? *

Yes

1.4.3 What is the difference in your coding? *

1.5 Status of patient on 31st December of the year of follow-up

1.5.1 Did you send the variable to the ECFSPR? *

Yes

The ECFSPR requires this variable to be coded as follows: -1 unknown or variable not collected 0 he/she died during the year of follow-up 1 he/she is alive on 31st December of the year of follow-up 2 not seen during the year of follow-up 3 lost to follow-up

1.5.2 In the Excel file, did you code this variable according to the coding above? *

Yes

1.5.3 What is the difference in your coding? *

1.6 Year of Death

1.6.1 Did you send the variable to the ECFSPR? *

Yes

The ECFSPR requires this variable to be coded as follows: 4 digits If patient is alive, leave blank If unknown use 9999

1.6.2 In the Excel file, did you code this variable according to the coding above? *

Yes

1.6.3 What is the difference in your coding? *

1.7 Month of Death

1.7.1 Did you send the variable to the ECFSPR? *

Yes

The ECFSPR requires this variable to be coded as follows: 2 digits (1 to 12) If unknown or patient is alive leave blank

1.7.2 In the Excel file, did you code this variable according to the coding above? *

Yes

1.7.3 What is the difference in your coding? *

1.8 Day of Death

1.8.1 Did you send the variable to the ECFSPR? *

Yes

The ECFSPR requires this variable to be coded as follows: 2 digits (1 to 31) If unknown or patient is alive leave blank

1.8.2 In the Excel file, did you code this variable according to the coding above? *

Yes

1.8.3 What is the difference in your coding? *

1.9 Cause of Death

1.9.1 Did you send the variable to the ECFSPR? *

Yes

The ECFSPR requires this variable to be coded as follows: -1 unknown -99 missing because the patient is alive or variable not collected 1 respiratory 2 liver GI 3 trauma 4 suicide 5 transplantation 6 non-CF related 7 cancer 8 other CF-related

1.9.2 In the Excel file, did you code this variable according to the coding above? *

Yes

1.9.3 What is the difference in your coding? *

2. DIAGNOSIS

2.1 Diagnosis confirmed according to the criteria for including patients in the ECFSPR

2.1.1 Did you send the variable to the ECFSPR? *

Yes

The ECFSPR requires this variable to be coded as follows: -1 unknown -99 missing (variable not collected) 0 no 1 yes 2 diagnosis to be confirmed

2.1.2 In the Excel file, did you code this variable according to the coding above? *

Yes

2.1.3 What is the difference in your coding? *

2.1.4 Does your registry definition meet the ECFSPR definition? *

Yes

The ECFSPR definition for this variable is the following: 1. Two sweat tests value > 59 mmol/L chloride: CF diagnosis accepted. 2. One sweat test value > 59 mmol/L chloride + DNA Analysis/Genotyping – two identified disease-causing CF mutations: CF diagnosis accepted. 3. If the sweat value is less than or equal to 60 mmol/L chloride, or not reported, then at least 2 of the following must be fulfilled: a. DNA Analysis/Genotyping: two identified disease-causing CF mutations; b. NPD (Transepithelial (Nasal) Potential Difference) or ICM (Intestinal current measurement): result consistent with a diagnosis of CF; c. Clinical presentation: typical features of CF.

2.1.5 What is the difference in your definition? *

2.2 Age at diagnosis

2.2.1 Did you send the variable to the ECFSPR? *

Yes

The ECFSPR requires this variable to be coded as follows: Age of diagnosis in years (two decimals) If the age at diagnosis is unknown or the variable is not collected, use value -1

2.2.2 In the Excel file, did you code this variable according to the coding above? *

Yes

2.2.3 What is the difference in your coding? *

2.3 Neonatal screening

2.3.1 Did you send the variable to the ECFSPR? *

Yes

The ECFSPR requires this variable to be coded as follows: -1 unknown -99 missing (variable not collected) 0 not done 1 performed, result positive 2 performed, result negative 3 performed, result unknown

2.3.2 In the Excel file, did you code this variable according to the coding above? *

Yes

2.3.3 What is the difference in your coding? *

2.4 Meconium Ileus

2.4.1 Did you send the variable to the ECFSPR? *

Yes

The ECFSPR requires this variable to be coded as follows: -1 unknown -99 missing (variable not collected) 0 no 1 yes, operated 2 yes, not operated 3 yes, don't know if operated

2.4.2 In the Excel file, did you code this variable according to the coding above? *

Yes

2.4.3 What is the difference in your coding? *

2.5 First mutation

2.5.1 Did you send the variable to the ECFSPR? *

Yes

The ECFSPR requires this variable to be coded as follows: If genotyping was not performed, then use "not done" for both mutations. If genotyping was performed, but none of the two mutations were identified, please use "unknown" for both mutations. If genotyping was performed, but one of the two mutations was not identified, please use "unknown" for that mutation, do not leave it blank. If the spelling of the mutation name is not in the list at the end of this document, please use "Other" and write the mutation name in the variable "Other first mutation".

2.5.2 In the Excel file, did you code this variable according to the coding above? *

Yes

2.5.3 What is the difference in your coding? *

2.6 Second mutation

2.6.1 Did you send the variable to the ECFSPR? *

Yes

2.6.2 In the Excel file, did you code this variable according to the coding above? *

Yes

2.6.3 What is the difference in your coding? *

2.7 Type of sweat test

2.7.1 Did you send the variable to the ECFSPR? *

Yes

The ECFSPR requires this variable to be coded as follows: -1 unknown -99 missing (variable not collected) 0 not done 1 titration 2 conductivity

2.7.2 In the Excel file, did you code this variable according to the coding above? *

Yes

2.7.3 What is the difference in your coding? *

2.7.4 Does your registry definition meet the ECFSPR definition? *

Yes

The ECFSPR definition for this variable is the following: If patient has not undergone sweat testing, record as “not done”. If a sweat test is “not done” then two known genotype mutations must be reported. i. Sweat Test: record the patient's sweat test; ii. Electrolytes: Chloride concentration measurement is the preferred analysis; iii. Chloride value: report the Chloride value in millimols per litre (mmol/L). If duplicate tests were carried out on the same day, report the highest positive value. NOTE: The acceptable range for Chloride values is 1-160 mmol/L. Anyone who has a Chloride value above 160 mmol/L must be re-tested.

2.7.5 What is the difference in your definition? *

2.8 Electrolytes

2.8.1 Did you send the variable to the ECFSPR? *

Yes

The ECFSPR requires this variable to be coded as follows: -1 unknown -99 missing (variable not collected) 0 not done 1 chloride 4 other

2.8.2 In the Excel file, did you code this variable according to the coding above? *

Yes

2.8.3 What is the difference in your coding? *

2.8.4 Does your registry definition meet the ECFSPR definition? *

Yes

2.8.5 What is the difference in your definition? *

2.9 Sweat test value

2.9.1 Did you send the variable to the ECFSPR? *

Yes

The ECFSPR requires this variable to be coded as follows: If the value is unknown or if the variable is not collected, use value -1

2.9.2 In the Excel file, did you code this variable according to the coding above? *

Yes

2.9.3 What is the difference in your coding? *

2.9.4 Does your registry definition meet the ECFSPR definition? *

Yes

The ECFSPR definition for this variable is the following: If patient has not undergone sweat testing, record as “not done”. If a sweat test is “not done” then two known genotype mutations must be reported. i. Sweat Test: record the patient's sweat test; ii. Electrolytes: Chloride concentration measurement is the preferred analysis; iii. Chloride value: report the Chloride value in millimols per litre (mmol/L). If duplicate tests were carried out on the same day, report the highest positive value. NOTE: The acceptable range for Chloride values is 1-170 mmol/L. Anyone who has a Chloride value above 170 mmol/L must be re-tested.

2.9.5 What is the difference in your definition? *

2.10 Nasal Potential Difference - NPD

2.10.1 Did you send the variable to the ECFSPR? *

Yes

The ECFSPR requires this variable to be coded as follows: -1 unknown -99 missing (variable not collected) 0 not done 1 test done

2.10.2 In the Excel file, did you code this variable according to the coding above? *

Yes

2.10.3 What is the difference in your coding? *

2.11 CF-typical NPD

2.11.1 Did you send the variable to the ECFSPR? *

Yes

The ECFSPR requires this variable to be coded as follows: -1 unknown -99 missing (variable not collected. Use if NPD= -1, -99 or 0) 0 no, not CF-typical NPD measurement 1 yes, CF-typical NPD measurement 2 indeterminate measurement

2.11.2 In the Excel file, did you code this variable according to the coding above? *

Yes

2.11.3 What is the difference in your coding? *

2.12 Date of NPD Year

2.12.1 Did you send the variable to the ECFSPR? *

Yes

Enter year of NPD measurement (4 digits); if year is unknown, use 9999. If NPD = -1/-99/0, leave blank

2.12.2 In the Excel file, did you code this variable according to the coding above? *

Yes

2.12.3 What is the difference in your coding? *

2.13 Date of NPD Month

2.13.1 Did you send the variable to the ECFSPR? *

Yes

Enter number of month of NPD measurement, max 2 digits; If month unknown, use 7. If patient born after 1/07, use date of diagnosis or 31/12; If NPD = -1/-99/0, leave blank.

2.13.2 In the Excel file, did you code this variable according to the coding above? *

Yes

2.13.3 What is the difference in your coding? *

2.14 Date of NPD Day

2.14.1 Did you send the variable to the ECFSPR? *

Yes

Enter day of the month of NPD measurement, max 2 digits. If day is unknown but month is known, use 15 for the day; if both month & day unknown, use 01 (01/07) or date of diagnosis or 31 (31/12); If NPD = - 1/-99/0, leave blank.

2.14.2 In the Excel file, did you code this variable according to the coding above? *

Yes

2.14.3 What is the difference in your coding? *

2.15 Intestinal Current Measurement

2.15.1 Did you send the variable to the ECFSPR? *

Yes

The ECFSPR requires this variable to be coded as follows: -1 unknown -99 missing (variable not collected) 0 test not done 1 test done

2.15.2 In the Excel file, did you code this variable according to the coding above? *

Yes

2.15.3 What is the difference in your coding? *

2.16 CF-typical ICM

2.16.1 Did you send the variable to the ECFSPR? *

Yes

The ECFSPR requires this variable to be coded as follows: -1 unknown -99 missing (variable not collected. Use if ICM = -1, -99 or 0) 0 no, not CF-typical ICM measurement 1 yes, CF-typical ICM measurement 2 indeterminate measurement

2.16.2 In the Excel file, did you code this variable according to the coding above? *

Yes

2.16.3 What is the difference in your coding? *

2.17 Date of ICM Year

2.17.1 Did you send the variable to the ECFSPR? *

Yes

Enter year of ICM measurement; if year is unknown, use 9999 . If ICM = -1/-99/0, leave blank

2.17.2 In the Excel file, did you code this variable according to the coding above? *

Yes

2.17.3 What is the difference in your coding? *

2.18 Date of ICM Month

2.18.1 Did you send the variable to the ECFSPR? *

Yes

Enter number of month of ICM measurement, max 2 digits. If month unknown, use 7. If patient born after 1/07, use date of diagnosis or 31/12. If ICM = -1/-99/0, leave blank.

2.18.2 In the Excel file, did you code this variable according to the coding above? *

Yes

2.18.3 What is the difference in your coding? *

2.19 Date of ICM Day

2.19.1 Did you send the variable to the ECFSPR? *

Yes

Enter day of the month of ICM measurement, max 2 digits; If day is unknown but month is known, use 15 for the day; if both month & day unknown, use 01 (01/07) or date of diagnosis or 31 (31/12); If ICM = - 1/-99/0, leave blank.

2.19.2 In the Excel file, did you code this variable according to the coding above? *

Yes

2.19.3 What is the difference in your coding? *

3. THERAPY

3.1 Inhaled continuous (>3 months) hypertonic NaCl during the year of follow-up

3.1.1 Did you send the variable to the ECFSPR? *

Yes

The ECFSPR requires this variable to be coded as follows: -1 unknown -99 missing (variable not collected) 0 no 1 yes

3.1.2 In the Excel file, did you code this variable according to the coding above? *

Yes

3.1.3 What is the difference in your coding? *

3.2 Inhaled continuous (>3 months) Mannitol during the year of follow-up

3.2.1 Did you send the variable to the ECFSPR? *

Yes

The ECFSPR requires this variable to be coded as follows: -1 unknown -99 missing (variable not collected) 0 no 1 yes

3.2.2 In the Excel file, did you code this variable according to the coding above? *

Yes

3.2.3 What is the difference in your coding? *

3.3 Inhaled continuous (>3 months) antibiotic during the year of follow-up

3.3.1 Did you send the variable to the ECFSPR? *

Yes

The ECFSPR requires this variable to be coded as follows: -1 unknown -99 missing (variable not collected) 0 no 1 yes

3.3.2 In the Excel file, did you code this variable according to the coding above? *

Yes

3.3.3 What is the difference in your coding? *

3.4 Inhaled continuous (>3 months) bronchodilators during the year of follow-up

3.4.1 Did you send the variable to the ECFSPR? *

Yes

The ECFSPR requires this variable to be coded as follows: -1 unknown -99 missing (variable not collected) 0 no 1 yes

3.4.2 In the Excel file, did you code this variable according to the coding above? *

Yes

3.4.3 What is the difference in your coding? *

3.5 In oxygen therapy the year of follow-up

3.5.1 Did you send the variable to the ECFSPR? *

Yes

The ECFSPR requires this variable to be coded as follows: -1 unknown -99 missing (variable not collected) 0 no 1 yes

3.5.2 In the Excel file, did you code this variable according to the coding above? *

Yes

3.5.3 What is the difference in your coding? *

3.6 Continuous (>3 months) NIPPV during the year of follow-up

3.6.1 Did you send the variable to the ECFSPR? *

Yes

The ECFSPR requires this variable to be coded as follows: -1 unknown -99 missing (variable not collected) 0 no 1 yes – CPAP 2 yes - BiPAP

3.6.2 In the Excel file, did you code this variable according to the coding above? *

Yes

3.6.3 What is the difference in your coding? *

3.7 Use of rhDNase during the year of follow-up

3.7.1 Did you send the variable to the ECFSPR? *

Yes

The ECFSPR requires this variable to be coded as follows: -1 unknown -99 missing (variable not collected) 0 no 1 yes

3.7.2 In the Excel file, did you code this variable according to the coding above? *

Yes

3.7.3 What is the difference in your coding? *

3.8 Continuous (>3 months) inhaled steroids during the year of follow-up

3.8.1 Did you send the variable to the ECFSPR? *

Yes

The ECFSPR requires this variable to be coded as follows: -1 unknown -99 missing (variable not collected) 0 no 1 yes

3.8.2 In the Excel file, did you code this variable according to the coding above? *

Yes

3.8.3 What is the difference in your coding? *

3.9 Continuous (>3 months) oral steroids during the year of follow-up

3.9.1 Did you send the variable to the ECFSPR? *

Yes

The ECFSPR requires this variable to be coded as follows: -1 unknown -99 missing (variable not collected) 0 no 1 yes

3.9.2 In the Excel file, did you code this variable according to the coding above? *

Yes

3.9.3 What is the difference in your coding? *

3.10 Use of continuous (>3 months) Azithromicin (or other macrolide) during the year of follow-up

3.10.1 Did you send the variable to the ECFSPR? *

Yes

The ECFSPR requires this variable to be coded as follows: -1 unknown -99 missing (variable not collected) 0 no 1 yes

3.10.2 In the Excel file, did you code this variable according to the coding above? *

Yes

3.10.3 What is the difference in your coding? *

3.11 Use of ursodeoxycholic acid during the year of follow-up

3.11.1 Did you send the variable to the ECFSPR? *

Yes

The ECFSPR requires this variable to be coded as follows: -1 unknown -99 missing (variable not collected) 0 no 1 yes

3.11.2 In the Excel file, did you code this variable according to the coding above? *

Yes

3.11.3 What is the difference in your coding? *

3.12 Use of pancreatic enzymes during the year of follow-up

3.12.1 Did you send the variable to the ECFSPR? *

Yes

The ECFSPR requires this variable to be coded as follows: -1 unknown -99 missing (variable not collected) 0 no 1 yes

3.12.2 In the Excel file, did you code this variable according to the coding above? *

Yes

3.12.3 What is the difference in your coding? *

3.13 Continuous (>3 months) Proton Pump Inhibitors during the year of follow-up

3.13.1 Did you send the variable to the ECFSPR? *

Yes

The ECFSPR requires this variable to be coded as follows: -1 unknown -99 missing (variable not collected) 0 no 1 yes

3.13.2 In the Excel file, did you code this variable according to the coding above? *

Yes

3.13.3 What is the difference in your coding? *

3.14 Ivacaftor during the year of follow-up

3.14.1 Did you send the variable to the ECFSPR? *

Yes

The ECFSPR requires this variable to be coded as follows: -1 unknown -99 missing (variable not collected) 0 no 1 yes

3.14.2 In the Excel file, did you code this variable according to the coding above? *

Yes

3.14.3 What is the difference in your coding? *

3.15 Start Year of Ivacaftor Treatment

3.15.1 Did you send the variable to the ECFSPR? *

Yes

The ECFSPR requires this variable to be coded as follows: If no Ivacaftor, leave blank; If “yes” to Ivacaftor, start year is required and must be equal to or before the year of follow-up; Use start year - max 4 digits; If you do not know the year therapy started but you know that it is before the current year of follow-up (CYFU) and therapy has continued to the CFYU, 01/01/XXXX (where XXXX = CYFU) should be used for the date of start of Ivacaftor. If no is selected at 3.15.1, box to open with this text:

3.15.2 In the Excel file, did you code this variable according to the coding above? *

Yes

The ECFSPR requires this variable to be coded as follows: If no Ivacaftor, leave blank; If “yes” for Ivacaftor, start year is required. See above, including defaults for unknown dates.

3.15.3 What is the difference in your coding? *

3.16 Start Month of Ivacaftor Treatment

3.16.1 Did you send the variable to the ECFSPR? *

Yes

The ECFSPR requires this variable to be coded as follows: If no Ivacaftor, leave blank; If “yes” for Ivacaftor, start month is required; Use start month - max 2 digits (1-12); Month unknown: if the start year is before the current year of follow up and only the month is unknown, 7 should be used for the month (see previous, if both start year and month are unknown); If the start year is the same as the year of follow-up, 7 should be used for the month;

3.16.2 In the Excel file, did you code this variable according to the coding above? *

Yes

The ECFSPR requires this variable to be coded as follows: If no Ivacaftor, leave blank; If “yes” for Ivacaftor, start month is required. See above, including defaults for unknown dates.

3.16.3 What is the difference in your coding? *

3.17 Start Day of Ivacaftor Treatment

3.17.1 Did you send the variable to the ECFSPR? *

Yes

The ECFSPR requires this variable to be coded as follows: If no Ivacaftor, leave blank; If “yes” for Ivacaftor, start day is required; Use start day - max 2 digits; Use day of the month Ivacaftor therapy started (max 2 digits); Day unknown: if only the day is unknown, 15 should be used for the day; If both day and month are unknown, 1 should be used for the day and 7 for the month (01/07); If day, month and year are unknown, 01/01/XXXX should be used (where XXXX = current year of follow up).

3.17.2 In the Excel file, did you code this variable according to the coding above? *

Yes

The ECFSPR requires this variable to be coded as follows: If no Ivacaftor, leave blank; If “yes” for Ivacaftor, start day is required. See above, including defaults for unknown dates.

3.17.3 What is the difference in your coding? *

3.18 Stop Year of Ivacaftor Treatment

3.18.1 Did you send the variable to the ECFSPR? *

Yes

The ECFSPR requires this variable to be coded as follows: If no Ivacaftor, leave blank; If treatment stopped in current year of follow-up (CYFU), current year should be used; 4 digits; If the treatment stopped before CYFU, "no" for Ivacaftor should be used and no dates should be provided; If the therapy started during or before CYFU and continued to the next year, no year should be entered (you are asked to confirm in ECFSTracker that no stop date = continuing to the next year); If you know patient was taking therapy in CYFU but you don’t know if the therapy continued to the next year you should really try to recover this information. If you really cannot recover this information use CYFU (full unknown stop date 31/12/2019);

3.18.2 In the Excel file, did you code this variable according to the coding above? *

Yes

The ECFSPR requires this variable to be coded as follows: If no Ivacaftor, leave blank; If “yes” for Ivacaftor, stop year is required. See above, including defaults for unknown dates.

3.18.3 What is the difference in your coding? *

3.19 Stop Month of Ivacaftor Treatment

3.19.1 Did you send the variable to the ECFSPR? *

Yes

The ECFSPR requires this variable to be coded as follows: If no Ivacaftor in current year of follow-up (CYFU), leave blank; If stopped in current year of follow up (CYFU), use the number of the month (max 2 digits); If stopped in CYFU and the month is unknown please recover this information. If you really cannot recover this information 12 should be used (31/12 is unknown stop date);

3.19.2 In the Excel file, did you code this variable according to the coding above? *

Yes

The ECFSPR requires this variable to be coded as follows: If no Ivacaftor, leave blank; If “yes” for Ivacaftor, stop month is required. See above, including defaults for unknown dates.

3.19.3 What is the difference in your coding? *

3.20 Stop Day of Ivacaftor Treatment

3.20.1 Did you send the variable to the ECFSPR? *

Yes

The ECFSPR requires this variable to be coded as follows: If no Ivacaftor in current year of follow-up (CYFU), leave blank; If stopped in current year of follow up (CYFU), use the number of the day (max 2 digits); If stopped in CYFU and only the day is unknown please recover this information. If you really cannot recover this information 15 should be used; If stopped in CYFU and both month & day are unknown, but year is known, use 31/12.

3.20.2 In the Excel file, did you code this variable according to the coding above? *

Yes

The ECFSPR requires this variable to be coded as follows: If no Ivacaftor, leave blank; If “yes” for Ivacaftor, stop day is required. See above, including defaults for unknown dates.

3.20.3 What is the difference in your coding? *

3.21 Lumacaftor / Ivacaftor during the year of follow-up

3.21.1 Did you send the variable to the ECFSPR? *

Yes

The ECFSPR requires this variable to be coded as follows: -1 unknown -99 missing (variable not collected) 0 no 1 yes

3.21.2 In the Excel file, did you code this variable according to the coding above? *

Yes

3.21.3 What is the difference in your coding? *

3.22 Start Year of Lumacaftor / Ivacaftor Treatment

3.22.1 Did you send the variable to the ECFSPR? *

Yes

The ECFSPR requires this variable to be coded as follows: If no Lumacaftor / Ivacaftor, leave blank; If “yes” to Lumacaftor / Ivacaftor, start year is required and must be equal to or before the year of follow-up; Use start year - max 4 digits; If you do not know the year therapy started but you know that it is before the current year of follow-up (CYFU) and therapy has continued to the CFYU, 01/01/XXXX (where XXXX = CYFU) should be used for the date of start of Lumacaftor / Ivacaftor.

3.22.2 In the Excel file, did you code this variable according to the coding above? *

Yes

The ECFSPR requires this variable to be coded as follows: If no Lumacaftor / Ivacaftor, leave blank; If “yes” for Lumacaftor / Ivacaftor, start year is required. See above, including defaults for unknown dates.

3.22.3 What is the difference in your coding? *

3.23 Start Month of Lumacaftor / Ivacaftor Treatment

3.23.1 Did you send the variable to the ECFSPR? *

Yes

The ECFSPR requires this variable to be coded as follows: If no Lumacaftor / Ivacaftor, leave blank; If “yes” for Lumacaftor / Ivacaftor, start month is required; Use start month - max 2 digits (1-12); Month unknown: if the start year is before the current year of follow up and only the month is unknown, 7 should be used for the month (see previous, if both start year and month are unknown); If the start year is the same as the year of follow-up, 7 should be used for the month;

3.23.2 In the Excel file, did you code this variable according to the coding above? *

Yes

The ECFSPR requires this variable to be coded as follows: If no Lumacaftor / Ivacaftor, leave blank; If “yes” for Lumacaftor / Ivacaftor, start month is required. See above, including defaults for unknown dates.

3.23.3 What is the difference in your coding? *

3.24 Start Day of Lumacaftor / Ivacaftor Treatment

3.24.1 Did you send the variable to the ECFSPR? *

Yes

The ECFSPR requires this variable to be coded as follows: If no Lumacaftor / Ivacaftor, leave blank; If “yes” for Lumacaftor / Ivacaftor, start day is required; Use start day - max 2 digits; Use day of the month Lumacaftor / Ivacaftor therapy started (max 2 digits); Day unknown: if only the day is unknown, 15 should be used for the day; If both day and month are unknown, 1 should be used for the day and 7 for the month (01/07); If day, month and year are unknown, 01/01/XXXX should be used (where XXXX = current year of follow up).

3.24.2 In the Excel file, did you code this variable according to the coding above? *

Yes

The ECFSPR requires this variable to be coded as follows: If no Lumacaftor / Ivacaftor, leave blank; If “yes” for Lumacaftor / Ivacaftor, start day is required. See above, including defaults for unknown dates.

3.24.3 What is the difference in your coding? *

3.25 Stop Year of Lumacaftor / Ivacaftor Treatment

3.25.1 Did you send the variable to the ECFSPR? *

Yes

The ECFSPR requires this variable to be coded as follows: If no Lumacaftor / Ivacaftor, leave blank; If treatment stopped in current year of follow-up (CYFU), current year should be used; 4 digits If the treatment stopped before CYFU, "no" for Lumacaftor / Ivacaftor should be used and no dates should be provided; If the therapy started during or before CYFU and continued to the next year, no year should be entered (you are asked to confirm in ECFSTracker that no stop date = continuing to the next year); If you know patient was taking therapy in CYFU but you don’t know if the therapy continued to the next year you should really try to recover this information. If you really cannot recover this information use CYFU (full unknown stop date 31/12/2019);

3.25.2 In the Excel file, did you code this variable according to the coding above? *

Yes

The ECFSPR requires this variable to be coded as follows: If no Lumacaftor / Ivacaftor, leave blank; If “yes” for Lumacaftor / Ivacaftor, stop year is required. See above, including defaults for unknown dates.

3.25.3 What is the difference in your coding? *

3.26 Stop Month of Lumacaftor / Ivacaftor Treatment

3.26.1 Did you send the variable to the ECFSPR? *

Yes

The ECFSPR requires this variable to be coded as follows: If no Lumacaftor / Ivacaftor in current year of follow-up (CYFU), leave blank; If stopped in current year of follow up (CYFU), use the number of the month (max 2 digits); If stopped in CYFU and the month is unknown please recover this information. If you really cannot recover this information 12 should be used (31/12 is unknown stop date;

3.26.2 In the Excel file, did you code this variable according to the coding above? *

Yes

The ECFSPR requires this variable to be coded as follows: If no Lumacaftor / Ivacaftor, leave blank; If “yes” for Lumacaftor / Ivacaftor, stop month is required. See above, including defaults for unknown dates.

3.26.3 What is the difference in your coding? *

3.27 Stop Day of Lumacaftor / Ivacaftor Treatment

3.27.1 Did you send the variable to the ECFSPR? *

Yes

The ECFSPR requires this variable to be coded as follows: If no Lumacaftor / Ivacaftor in current year of follow-up (CYFU), leave blank; If stopped in current year of follow up (CYFU), use the number of the day (max 2 digits); If stopped in CYFU and only the day is unknown please recover this information. If you really cannot recover this information 15 should be used; If stopped in CYFU and both month & day are unknown, but year is known, use 31/12.

3.27.2 In the Excel file, did you code this variable according to the coding above? *

Yes

The ECFSPR requires this variable to be coded as follows: If no Lumacaftor / Ivacaftor, leave blank; If “yes” for Lumacaftor / Ivacaftor, stop day is required. See above, including defaults for unknown dates.

3.27.3 What is the difference in your coding? *

3.28 Tezacaftor / Ivacaftor during the year of follow-up

3.28.1 Did you send the variable to the ECFSPR? *

Yes

The ECFSPR requires this variable to be coded as follows: -1 unknown -99 missing (variable not collected) 0 no 1 yes

3.28.2 In the Excel file, did you code this variable according to the coding above? *

Yes

3.28.3 What is the difference in your coding? *

3.29 Start Year of Tezacaftor / Ivacaftor Treatment

3.29.1 Did you send the variable to the ECFSPR? *

Yes

The ECFSPR requires this variable to be coded as follows: If no Tezacaftor / Ivacaftor, leave blank; If “yes” to Tezacaftor / Ivacaftor, start year is required and must be equal to or before the year of follow-up; Use start year - max 4 digits; If you do not know the year therapy started but you know that it is before the current year of follow-up (CYFU) and therapy has continued to the CFYU, 01/01/XXXX (where XXXX = CYFU) should be used for the date of start of Tezacaftor / Ivacaftor.

3.29.2 In the Excel file, did you code this variable according to the coding above? *

Yes

The ECFSPR requires this variable to be coded as follows: If no Tezacaftor / Ivacaftor, leave blank; If “yes” for Tezacaftor / Ivacaftor, start year is required. See above, including defaults for unknown dates.

3.29.3 What is the difference in your coding? *

3.30 Start Month of Tezacaftor / Ivacaftor Treatment

3.30.1 Did you send the variable to the ECFSPR? *

Yes

The ECFSPR requires this variable to be coded as follows: If no Tezacaftor / Ivacaftor, leave blank; If “yes” for Tezacaftor / Ivacaftor, start month is required; Use start month - max 2 digits (1-12); Month unknown: if the start year is before the current year of follow up and only the month is unknown, 7 should be used for the month (see previous, if both start year and month are unknown); If the start year is the same as the year of follow-up, 7 should be used for the month;

3.30.2 In the Excel file, did you code this variable according to the coding above? *

Yes

The ECFSPR requires this variable to be coded as follows: If no Tezacaftor / Ivacaftor, leave blank; If “yes” for Tezacaftor / Ivacaftor, start month is required. See above, including defaults for unknown dates.

3.30.3 What is the difference in your coding? *

3.31 Start Day of Tezacaftor / Ivacaftor Treatment

3.31.1 Did you send the variable to the ECFSPR? *

Yes

The ECFSPR requires this variable to be coded as follows: If no Tezacaftor / Ivacaftor, leave blank; If “yes” for Tezacaftor / Ivacaftor, start day is required; Use start day - max 2 digits; Use day of the month Tezacaftor / Ivacaftor therapy started (max 2 digits); Day unknown: if only the day is unknown, 15 should be used for the day; If both day and month are unknown, 1 should be used for the day and 7 for the month (01/07); If day, month and year are unknown, 01/01/XXXX should be used (where XXXX = current year of follow up).

3.31.2 In the Excel file, did you code this variable according to the coding above? *

Yes

The ECFSPR requires this variable to be coded as follows: If no Tezacaftor / Ivacaftor, leave blank; If “yes” for Tezacaftor / Ivacaftor, start day is required. See above, including defaults for unknown dates.

3.31.3 What is the difference in your coding? *

3.32 Stop Year of Tezacaftor / Ivacaftor Treatment

3.32.1 Did you send the variable to the ECFSPR? *

Yes

The ECFSPR requires this variable to be coded as follows: If no Tezacaftor / Ivacaftor, leave blank; If treatment stopped in current year of follow-up (CYFU), current year should be used; 4 digits If the treatment stopped before CYFU, "no" for Tezacaftor / Ivacaftor should be used and no dates should be provided; If the therapy started during or before CYFU and continued to the next year, no year should be entered (you are asked to confirm in ECFSTracker that no stop date = continuing to the next year); If you know patient was taking therapy in CYFU but you don’t know if the therapy continued to the next year you should really try to recover this information. If you really cannot recover this information use CYFU (full unknown stop date 31/12/2019);

3.32.2 In the Excel file, did you code this variable according to the coding above? *

Yes

The ECFSPR requires this variable to be coded as follows: If no Tezacaftor / Ivacaftor, leave blank; If “yes” for Tezacaftor / Ivacaftor, stop year is required. See above, including defaults for unknown dates.

3.32.3 What is the difference in your coding? *

3.33 Stop Month of Tezacaftor / Ivacaftor Treatment

3.33.1 Did you send the variable to the ECFSPR? *

Yes

The ECFSPR requires this variable to be coded as follows: If no Tezacaftor / Ivacaftor in current year of follow-up (CYFU), leave blank; If stopped in current year of follow up (CYFU), use the number of the month (max 2 digits); If stopped in CYFU and the month is unknown please recover this information. If you really cannot recover this information 12 should be used (31/12 is unknown stop date;

3.33.2 In the Excel file, did you code this variable according to the coding above? *

Yes

The ECFSPR requires this variable to be coded as follows: If no Tezacaftor / Ivacaftor, leave blank; If “yes” for Tezacaftor / Ivacaftor, stop month is required. See above, including defaults for unknown dates.

3.33.3 What is the difference in your coding? *

3.34 Stop Day of Tezacaftor / Ivacaftor Treatment

3.34.1 Did you send the variable to the ECFSPR? *

Yes

The ECFSPR requires this variable to be coded as follows: If no Tezacaftor / Ivacaftor in current year of follow-up (CYFU), leave blank; If stopped in current year of follow up (CYFU), use the number of the day (max 2 digits); If stopped in CYFU and only the day is unknown please recover this information. If you really cannot recover this information 15 should be used; If stopped in CYFU and both month & day are unknown, but year is known, use 31/12.

3.34.2 In the Excel file, did you code this variable according to the coding above? *

Yes

The ECFSPR requires this variable to be coded as follows: If no Tezacaftor / Ivacaftor, leave blank; If “yes” for Tezacaftor / Ivacaftor, stop day is required. See above, including defaults for unknown dates.

3.34.3 What is the difference in your coding? *

3.35 Elexacaftor / Tezacaftor / Ivacaftor during the year of follow-up

3.35.1 Did you send the variable to the ECFSPR? *

Yes

The ECFSPR requires this variable to be coded as follows: -1 unknown -99 missing (variable not collected) 0 no 1 yes

3.35.2 In the Excel file, did you code this variable according to the coding above? *

Yes

3.35.3 What is the difference in your coding? *

3.36 Start Year of Elexacaftor / Tezacaftor / Ivacaftor Treatment

3.36.1 Did you send the variable to the ECFSPR? *

Yes

The ECFSPR requires this variable to be coded as follows: If no Elexacaftor / Tezacaftor / Ivacaftor, leave blank; If “yes” to Elexacaftor / Tezacaftor / Ivacaftor, start year is required and must be equal to or before the year of follow-up; Use start year - max 4 digits; If you do not know the year therapy started but you know that it is before the current year of follow-up (CYFU) and therapy has continued to the CFYU, 01/01/XXXX (where XXXX = CYFU) should be used for the date of start of Elexacaftor / Tezacaftor / Ivacaftor.

3.36.2 In the Excel file, did you code this variable according to the coding above? *

Yes

The ECFSPR requires this variable to be coded as follows: If no Elexacaftor / Tezacaftor / Ivacaftor, leave blank; If “yes” for Elexacaftor / Tezacaftor / Ivacaftor, start year is required. See above, including defaults for unknown dates.

3.36.3 What is the difference in your coding? *

3.37 Start Month of Elexacaftor / Tezacaftor / Ivacaftor Treatment

3.37.1 Did you send the variable to the ECFSPR? *

Yes

The ECFSPR requires this variable to be coded as follows: If no Elexacaftor / Tezacaftor / Ivacaftor, leave blank; If “yes” for Elexacaftor / Tezacaftor / Ivacaftor, start month is required; Use start month - max 2 digits (1-12); Month unknown: if the start year is before the current year of follow up and only the month is unknown, 7 should be used for the month (see previous, if both start year and month are unknown); If the start year is the same as the year of follow-up, 7 should be used for the month;

3.37.2 In the Excel file, did you code this variable according to the coding above? *

Yes

3.37.3 What is the difference in your coding? *

3.38 Start Day of Elexacaftor / Tezacaftor / Ivacaftor Treatment

3.38.1 Did you send the variable to the ECFSPR? *

Yes

The ECFSPR requires this variable to be coded as follows: If no Elexacaftor / Tezacaftor / Ivacaftor, leave blank; If “yes” for Elexacaftor / Tezacaftor / Ivacaftor, start day is required; Use start day - max 2 digits; Use day of the month Elexacaftor / Tezacaftor / Ivacaftor therapy started (max 2 digits); Day unknown: if only the day is unknown, 15 should be used for the day; If both day and month are unknown, 1 should be used for the day and 7 for the month (01/07); If day, month and year are unknown, 01/01/XXXX should be used (where XXXX = current year of follow up).

3.38.2 In the Excel file, did you code this variable according to the coding above? *

Yes

3.38.3 What is the difference in your coding? *

3.39 Stop Year of Elexacaftor / Tezacaftor / Ivacaftor Treatment

3.39.1 Did you send the variable to the ECFSPR? *

Yes

The ECFSPR requires this variable to be coded as follows: If no Elexacaftor / Tezacaftor / Ivacaftor, leave blank; If treatment stopped in current year of follow-up (CYFU), current year should be used; 4 digits If the treatment stopped before CYFU, "no" for Elexacaftor / Tezacaftor / Ivacaftor should be used and no dates should be provided; If the therapy started during or before CYFU and continued to the next year, no year should be entered (you are asked to confirm in ECFSTracker that no stop date = continuing to the next year); If you know patient was taking therapy in CYFU but you don’t know if the therapy continued to the next year you should really try to recover this information. If you really cannot recover this information use CYFU (full unknown stop date 31/12/2019);

3.39.2 In the Excel file, did you code this variable according to the coding above? *

Yes

The ECFSPR requires this variable to be coded as follows: If no Elexacaftor / Tezacaftor / Ivacaftor, leave blank; If “yes” for Elexacaftor / Tezacaftor / Ivacaftor, stop year is required. See above, including defaults for unknown dates.

3.39.3 What is the difference in your coding? *

3.40 Stop Month of Elexacaftor / Tezacaftor / Ivacaftor Treatment

3.40.1 Did you send the variable to the ECFSPR? *

Yes

The ECFSPR requires this variable to be coded as follows: If no Elexacaftor / Tezacaftor / Ivacaftor in current year of follow-up (CYFU), leave blank; If stopped in current year of follow up (CYFU), use the number of the month (max 2 digits); If stopped in CYFU and the month is unknown please recover this information. If you really cannot recover this information 12 should be used (31/12 is unknown stop date;

3.40.2 In the Excel file, did you code this variable according to the coding above? *

Yes

The ECFSPR requires this variable to be coded as follows: If no Elexacaftor / Tezacaftor / Ivacaftor, leave blank; If “yes” for Elexacaftor / Tezacaftor / Ivacaftor, stop month is required. See above, including defaults for unknown dates.

3.40.3 What is the difference in your coding? *

3.41 Stop Day of Elexacaftor / Tezacaftor / Ivacaftor Treatment

3.41.1 Did you send the variable to the ECFSPR? *

Yes

The ECFSPR requires this variable to be coded as follows: If no Elexacaftor / Tezacaftor / Ivacaftor in current year of follow-up (CYFU), leave blank; If stopped in current year of follow up (CYFU), use the number of the day (max 2 digits); If stopped in CYFU and only the day is unknown please recover this information. If you really cannot recover this information 15 should be used; If stopped in CYFU and both month & day are unknown, but year is known, use 31/12.

3.41.2 In the Excel file, did you code this variable according to the coding above? *

Yes

The ECFSPR requires this variable to be coded as follows: If no Elexacaftor / Tezacaftor / Ivacaftor, leave blank; If “yes” for Elexacaftor / Tezacaftor / Ivacaftor, stop day is required. See above, including defaults for unknown dates.

3.41.3 What is the difference in your coding? *

3.42 Other CFTR Modulator during the year of follow-up

3.42.1 Did you send the variable to the ECFSPR? *

Yes

The ECFSPR requires this variable to be coded as follows: -1 unknown -99 missing (variable not collected) 0 no 1 yes

3.42.2 In the Excel file, did you code this variable according to the coding above? *

Yes

3.42.3 What is the difference in your coding? *

3.43 Start Year of Other CFTR Modulator Treatment

3.43.1 Did you send the variable to the ECFSPR? *

Yes

The ECFSPR requires this variable to be coded as follows: If no Other CFTR Modulator, leave blank; If “yes” to Other CFTR Modulator, start year is required and must be equal to or before the year of follow-up; Use start year - max 4 digits; If you do not know the year therapy started but you know that it is before the current year of follow-up (CYFU) and therapy has continued to the CFYU, 01/01/XXXX (where XXXX = CYFU) should be used for the date of start of Other CFTR Modulator.

3.43.2 In the Excel file, did you code this variable according to the coding above? *

Yes

The ECFSPR requires this variable to be coded as follows: If no Other CFTR Modulator, leave blank; If “yes” for Other CFTR Modulator, start year is required. See above, including defaults for unknown dates.

3.43.3 What is the difference in your coding? *

3.44 Start Month of Other CFTR Modulator Treatment

3.44.1 Did you send the variable to the ECFSPR? *

Yes

The ECFSPR requires this variable to be coded as follows: If no Other CFTR Modulator, leave blank; If “yes” for Other CFTR Modulator, start month is required; Use start month - max 2 digits (1-12); Month unknown: if the start year is before the current year of follow up and only the month is unknown, 7 should be used for the month (see previous, if both start year and month are unknown); If the start year is the same as the year of follow-up, 7 should be used for the month;

3.44.2 In the Excel file, did you code this variable according to the coding above? *

Yes

The ECFSPR requires this variable to be coded as follows: If no Other CFTR Modulator, leave blank; If “yes” for Other CFTR Modulator, start month is required. See above, including defaults for unknown dates.

3.44.3 What is the difference in your coding? *

3.45 Start Day of Other CFTR Modulator Treatment

3.45.1 Did you send the variable to the ECFSPR? *

Yes

The ECFSPR requires this variable to be coded as follows: If no Other CFTR Modulator, leave blank; If “yes” for Other CFTR Modulator, start day is required; Use start day - max 2 digits; Use day of the month Other CFTR Modulator therapy started (max 2 digits); Day unknown: if only the day is unknown, 15 should be used for the day; If both day and month are unknown, 1 should be used for the day and 7 for the month (01/07); If day, month and year are unknown, 01/01/XXXX should be used (where XXXX = current year of follow up).

3.45.2 In the Excel file, did you code this variable according to the coding above? *

Yes

The ECFSPR requires this variable to be coded as follows: If no Other CFTR Modulator, leave blank; If “yes” for Other CFTR Modulator, start day is required. See above, including defaults for unknown dates.

3.45.3 What is the difference in your coding? *

3.46 Stop Year of Other CFTR Modulator Treatment

3.46.1 Did you send the variable to the ECFSPR? *

Yes

The ECFSPR requires this variable to be coded as follows: If no Other CFTR Modulator, leave blank; If treatment stopped in current year of follow-up (CYFU), current year should be used; 4 digits If the treatment stopped before CYFU, "no" for Other CFTR Modulator should be used and no dates should be provided; If the therapy started during or before CYFU and continued to the next year, no year should be entered (you are asked to confirm in ECFSTracker that no stop date = continuing to the next year); If you know patient was taking therapy in CYFU but you don’t know if the therapy continued to the next year you should really try to recover this information. If you really cannot recover this information use CYFU (full unknown stop date 31/12/2019);

3.46.2 In the Excel file, did you code this variable according to the coding above? *

Yes

The ECFSPR requires this variable to be coded as follows: If no Other CFTR Modulator, leave blank; If “yes” for Other CFTR Modulator, stop year is required. See above, including defaults for unknown dates.

3.46.3 What is the difference in your coding? *

3.47 Stop Month of Other CFTR Modulator Treatment

3.47.1 Did you send the variable to the ECFSPR? *

Yes

The ECFSPR requires this variable to be coded as follows: If no Other CFTR Modulator in current year of follow-up (CYFU), leave blank; If stopped in current year of follow up (CYFU), use the number of the month (max 2 digits); If stopped in CYFU and the month is unknown please recover this information. If you really cannot recover this information 12 should be used (31/12 is unknown stop date;

3.47.2 In the Excel file, did you code this variable according to the coding above? *

Yes

The ECFSPR requires this variable to be coded as follows: If no Other CFTR Modulator, leave blank; If “yes” for Other CFTR Modulator, stop month is required. See above, including defaults for unknown dates.

3.47.3 What is the difference in your coding? *

3.48 Stop Day of Other CFTR Modulator Treatment

3.48.1 Did you send the variable to the ECFSPR? *

Yes

The ECFSPR requires this variable to be coded as follows: If no Other CFTR Modulator in current year of follow-up (CYFU), leave blank; If stopped in current year of follow up (CYFU), use the number of the day (max 2 digits); If stopped in CYFU and only the day is unknown please recover this information. If you really cannot recover this information 15 should be used; If stopped in CYFU and both month & day are unknown, but year is known, use 31/12.

3.48.2 In the Excel file, did you code this variable according to the coding above? *

Yes

The ECFSPR requires this variable to be coded as follows: If no Other CFTR Modulator, leave blank; If “yes” for Other CFTR Modulator, stop day is required. See above, including defaults for unknown dates.

3.48.3 What is the difference in your coding? *

4. LUNG FUNCTION AND NUTRITION

4.1 Weight measured at date of FEV1 (of best FEV1% of predicted) or, if no lung function test, last weight of the year of follow-up

4.1.1 Did you send the variable to the ECFSPR? *

Yes

The ECFSPR requires this variable to be coded as follows: Weight in kg (one decimal) If the value is unknown or if the variable is not collected, use value -1

4.1.2 In the Excel file, did you code this variable according to the coding above? *

Yes

4.1.3 What is the difference in your coding? *

4.1.4 Does your registry definition meet the ECFSPR definition? *

Yes

The ECFSPR definition for this variable is the following: Measurements: weight and height are measured according to EuroCareCF guidelines: a. weight: removal of outer clothing, shoes and socks; b. height: without shoes and socks - stadiometer - top of head in contact with head board, slight pressure; c. it should be the value at the day of the recorded FEV1; or if no lung function test, last weight of the year of follow-up Z-scores for height, weight and BMI will be calculated using the CDC reference values [Kuczmarski et al (2002)].

4.1.5 What is the difference in your definition? *

4.2 Height measured at date of FEV1 (of best FEV1% of predicted) or, if no lung function test, last height of the year of follow-up

4.2.1 Did you send the variable to the ECFSPR? *

Yes

The ECFSPR requires this variable to be coded as follows: Height in cm (no decimals) If the value is unknown or if the variable is not collected, use value -1

4.2.2 In the Excel file, did you code this variable according to the coding above? *

Yes

4.2.3 What is the difference in your coding? *

4.2.4 Does your registry definition meet the ECFSPR definition? *

Yes

The ECFSPR definition for this variable is the following: Measurements: weight and height are measured according to EuroCareCF guidelines: a. weight: removal of outer clothing, shoes and socks; b. height: without shoes and socks - stadiometer - top of head in contact with head board, slight pressure; c. it should be the value at the day of the recorded FEV1; or if no lung function test, last height of the year of follow-up. Z-scores for height, weight and BMI will be calculated using the CDC reference values [Kuczmarski et al (2002)].

4.2.5 What is the difference in your definition? *

4.3 Month of FEV1 measurement

4.3.1 Did you send the variable to the ECFSPR? *

Yes

The ECFSPR requires this variable to be coded as follows: 2 digits (1 to 12) If unknown use 7 (unknown date of FEV1 measurement is 01-07) If no lung function nor growth data is present, leave blank

4.3.2 In the Excel file, did you code this variable according to the coding above? *

Yes

4.3.3 What is the difference in your coding? *

4.4 Day of FEV1 measurement

4.4.1 Did you send the variable to the ECFSPR? *

Yes

The ECFSPR requires this variable to be coded as follows: 2 digits (1 to 31) If the day is unknown, bu the month is known, use 15. If the day and the month are unknown, use 1 (unknown date of FEV1 measurement is 01-07) If no lung function nor growth data is present, leave blank

4.4.2 In the Excel file, did you code this variable according to the coding above? *

Yes

4.4.3 What is the difference in your coding? *

4.5 Value of FEV1 of best FEV1% of predicted during the year of follow-up

4.5.1 Did you send the variable to the ECFSPR? *

Yes

The ECFSPR requires this variable to be coded as follows: Value in litres (2 decimals) of the FEV1 of the highest FEV1% of predicted of the year according to local reference values. If the value is unknown or if the variable is not collected, use value -1

4.5.2 In the Excel file, did you code this variable according to the coding above? *

Yes

4.5.3 What is the difference in your coding? *

4.5.4 Does your registry definition meet the ECFSPR definition? *

Yes

The ECFSPR definition for this variable is the following: a. for values reported to national registries or to centres and sent to the ECFSPR, it is the value in litres of the FEV1 of the highest FEV1% predicted (according to local references) of the year which should be extracted; b. each patient's FVC and FEV1 measurements must be reported in litres (L), with up to two places to the right of the decimal; c. the FVC measurement must be greater than or equal to the FEV1 measurement; d. for the reported spirometry value, the date of the test and the patient’s height at that date should be reported in order to perform the calculation of the percent of predicted values; e. only tests deemed valid according to ATC/ERS guidelines should be reported.

4.5.5 What is the difference in your definition? *

4.6 Value of FVC of the reported FEV1 of highest FEV1% of predicted during the year of follow-up

4.6.1 Did you send the variable to the ECFSPR? *

Yes

The ECFSPR requires this variable to be coded as follows: Value in litres (2 decimals) of the FVC of the reported FEV1 of the highest FEV1% of predicted of the year according to local reference values. If the value is unknown or if the variable is not collected, use value -1

4.6.2 In the Excel file, did you code this variable according to the coding above? *

Yes

4.6.3 What is the difference in your coding? *

4.6.4 Does your registry definition meet the ECFSPR definition? *

Yes

The ECFSPR definition for this variable is the following: a. for values reported to national registries or to centres and sent to the ECFSPR, litres of the FVC (according to local references) of the recorded FEV1 of the highest FEV1% of predicted of the year which should be extracted; b. each patient's FVC and FEV1 measurements must be reported in litres (L), with up to two places to the right of the decimal; c. the FVC measurement must be greater than or equal to the FEV1 measurement; d. for each reported spirometry value, the date of the test and the patient’s height at that date should be reported in order to perform the calculation of the percent of predicted values; e. only tests deemed valid according to ATC/ERS guidelines should be reported.

4.6.5 What is the difference in your definition? *

4.7 Lowest Lung Clearance Index 2,5% during the year of follow-up

4.7.1 Did you send the variable to the ECFSPR? *

Yes

The ECFSPR requires this variable to be coded as follows: Record value of lowest LCI 2.5% during the year of follow-up; if unknown or not collected use -1. The value should be between 2 and 25.

4.7.2 In the Excel file, did you code this variable according to the coding above? *

Yes

4.7.3 What is the difference in your coding? *

4.8 Date of lowest LCI 2,5% this year - month

4.8.1 Did you send the variable to the ECFSPR? *

Yes

The ECFSPR requires this variable to be coded as follows: Enter number corresponding to month of lowest LCI2.5%; If month is unknown, use '7'. If the patient was born after 1/7 of the year of follow-up use 12 (for 31/12); If no LCI 2.5% (LCI2.5% = -1) do not enter a date; leave blank.

4.8.2 In the Excel file, did you code this variable according to the coding above? *

Yes

4.8.3 What is the difference in your coding? *

4.9 Date of lowest LCI 2,5% this year - day

4.9.1 Did you send the variable to the ECFSPR? *

Yes

The ECFSPR requires this variable to be coded as follows: Enter day of the month of lowest LCI2.5%; If only the day is unknown but the month is known, use 15 for the day; If both day & month are unknown, use 1 (for 1/7); If the patient was born after 1/7 of the year of follow-up use 31 (for 31/12); If no LCI 2.5% do not enter a date - leave blank.

4.9.2 In the Excel file, did you code this variable according to the coding above? *

Yes

4.9.3 What is the difference in your coding? *

4.10 Device used for lowest LCI 2.5%

4.10.1 Did you send the variable to the ECFSPR? *

Yes

The ECFSPR requires this variable to be coded as follows: -1 device unknown -99 missing (variable not collected/LCI = -1) 1 Ecomedics Exhalyzer D N2-Washout 2 Ecomedics Exhalyzer SF6 (Tracergas 4%). 3 Innovision Innocor 4 NDD Easyone Pro 5 Other, not listed

4.10.2 In the Excel file, did you code this variable according to the coding above? *

Yes

4.10.3 What is the difference in your coding? *

5. MICROBIOLOGY

5.1 Chronic Pseudomonas aeruginosa

5.1.1 Did you send the variable to the ECFSPR? *

Yes

The ECFSPR requires this variable to be coded as follows: -1 unknown -99 missing (variable not collected) 0 no 1 yes

5.1.2 In the Excel file, did you code this variable according to the coding above? *

Yes

5.1.3 What is the difference in your coding? *

5.1.4 Does your registry definition meet the ECFSPR definition? *

Yes

The ECFSPR definition for this variable is the following: 1. Chronic Pseudomonas aeruginosa infection: A patient should be considered chronically infected if the modified Leeds criteria are met (a) below, and/or anti-pseudomonas antibodies are detected (b) below. A patient should be defined as chronically infected if he/she fulfils the criteria now, or has done so in recent years, and the physician has no reason to think that the status has changed: a) Modified Leeds criteria - chronic infection: >50% of the samples (sputum/other) collected during the last 12 months should be positive; at least 4 samples collected. b) Significantly raised levels of anti-pseudomonas antibodies according to local laboratories. 2. Chronic infection with other gram-negative or gram-positive bacteria should meet the same criteria as described above.

5.1.5 What is the difference in your definition? *

5.2 Chronic Staphylococcus aureus

5.2.1 Did you send the variable to the ECFSPR? *

Yes

The ECFSPR requires this variable to be coded as follows: -1 unknown -99 missing (variable not collected) 0 no 1 yes

5.2.2 In the Excel file, did you code this variable according to the coding above? *

Yes

5.2.3 What is the difference in your coding? *

5.2.4 Does your registry definition meet the ECFSPR definition? *

Yes

The ECFSPR definition for this variable is the following: 1. Chronic Staphylococcus aureus infection should be defined by the local physician in accordance with the modified Leeds criteria and/or the presence of anti-Staphylococcus aureus antibodies. Patient should be defined as chronically infected if he/she fulfils the criteria now or has done so in recent years and the physician has no reason to think the status has changed- a. modified Leeds criteria, chronic infection: >50% of the sputum samples positive, collected during the last 12 months. At least 4 sputum samples during that period; b. and/or significantly raised anti-Staphylococcus aureus antibodies according to local laboratories; 2. Chronic infection with other gram-negative bacteria should be recorded using the same criteria as above.

5.2.5 What is the difference in your definition? *

5.3 Chronic Burkholderia cepacia

5.3.1 Did you send the variable to the ECFSPR? *

Yes

The ECFSPR requires this variable to be coded as follows: -1 unknown -99 missing (variable not collected) 0 no 1 yes

5.3.2 In the Excel file, did you code this variable according to the coding above? *

Yes

5.3.3 What is the difference in your coding? *

5.3.4 Does your registry definition meet the ECFSPR definition? *

Yes

The ECFSPR definition for this variable is the following: 1. Chronic Burkholderia cepacia infection should be defined by the local physician in accordance with the modified Leeds criteria and/or the presence of anti- Burkholderia cepacia antibodies. Patient should be defined as chronically infected if he/she fulfils the criteria now or has done so in recent years and the physician has no reason to think the status has changed- a. modified Leeds criteria, chronic infection: >50% of the sputum samples positive, collected during the last 12 months. At least 4 sputum samples during that period; b. and/or significantly raised anti- Burkholderia cepacia antibodies according to local laboratories; 2. Chronic infection with other gram-negative bacteria should be recorded using the same criteria as above.

5.3.5 What is the difference in your definition? *

5.4 Haemophilus influenzae this year

5.4.1 Did you send the variable to the ECFSPR? *

Yes

The ECFSPR requires this variable to be coded as follows: -1 unknown -99 missing (variable not collected) 0 no 1 yes, at least once during the year of follow-up

5.4.2 In the Excel file, did you code this variable according to the coding above? *

Yes

5.4.3 What is the difference in your coding? *

5.5 Nontuberculous mycobacteria during the year of follow-up

5.5.1 Did you send the variable to the ECFSPR? *

Yes

The ECFSPR requires this variable to be coded as follows: -1 unknown -99 missing (variable not collected) 0 never during the year of follow-up 1 yes, at least once during the year of follow-up

5.5.2 In the Excel file, did you code this variable according to the coding above? *

Yes

5.5.3 What is the difference in your coding? *

5.6 Stenotrophomonas maltophilia during the year of follow-up

5.6.1 Did you send the variable to the ECFSPR? *

Yes

The ECFSPR requires this variable to be coded as follows: -1 unknown -99 missing (variable not collected) 0 never during the year of follow-up 1 yes, at least once during the year of follow-up

5.6.2 In the Excel file, did you code this variable according to the coding above? *

Yes

5.6.3 What is the difference in your coding? *

5.7 Achromobacter spp (species) this year

5.7.1 Did you send the variable to the ECFSPR? *

Yes

The ECFSPR requires this variable to be coded as follows: -1 unknown -99 missing (variable not collected) 0 no 1 yes, at least once during the year of follow-up

5.7.2 In the Excel file, did you code this variable according to the coding above? *

Yes

5.7.3 What is the difference in your coding? *

5.8 Methicillin-resistant Staphylococcus aureus - MRSA - this year

5.8.1 Did you send the variable to the ECFSPR? *

Yes

The ECFSPR requires this variable to be coded as follows: -1 unknown -99 missing (variable not collected) 0 no 1 yes, at least once during the year of follow-up

5.8.2 In the Excel file, did you code this variable according to the coding above? *

Yes

5.8.3 What is the difference in your coding? *

5.9 Total days on intravenous (IV) antibiotics (for CF-related reasons), at home + in hospital, this year

5.9.1 Did you send the variable to the ECFSPR? *

Yes

The ECFSPR requires this variable to be coded as follows: enter the total number of days on intravenous antibiotics, (CF-related reasons), at home and in hospital during the year of f.u. -1 number of days unknown / variable not collected 0 no IV antibiotics during the year of f.u. Include the first and last day.

5.9.2 In the Excel file, did you code this variable according to the coding above? *

Yes

5.9.3 What is the difference in your coding? *

5.10 Total days on intravenous (IV) antibiotics (for CF-related reasons), in hospital only, this year

5.10.1 Did you send the variable to the ECFSPR? *

Yes

The ECFSPR requires this variable to be coded as follows: enter number of days on intravenous antibiotics, (CF-related reasons), in hospital only, during the year of f.u. -1 number of days unknown / variable not collected 0 no IV antibiotics during the year of f.u. Include the first and last day.

5.10.2 In the Excel file, did you code this variable according to the coding above? *

Yes

5.10.3 What is the difference in your coding? *

5.11 Total days in hospital this year, for any reason, not only CF-related, excluding routine checkup days

5.11.1 Did you send the variable to the ECFSPR? *

Yes

The ECFSPR requires this variable to be coded as follows: enter total number of days in hospital (incl. non-CF related but excluding routine check-up), during year of f.u. -1 number of days unknown / variable not collected. 0 zero days in hospital - patient was not recovered for any reason during year of f.u.

5.11.2 In the Excel file, did you code this variable according to the coding above? *

Yes

5.11.3 What is the difference in your coding? *

6. COMPLICATIONS

6.1 Allergic broncho-pulmonary aspergillosis (ABPA) during the year of follow-up

6.1.1 Did you send the variable to the ECFSPR? *

Yes

The ECFSPR requires this variable to be coded as follows: -1 unknown -99 missing (variable not collected) 0 no ABPA during the year of follow-up 1 current ABPA (diagnosed or still treated during the year of follow-up)

6.1.2 In the Excel file, did you code this variable according to the coding above? *

Yes

6.1.3 What is the difference in your coding? *

6.1.4 Does your registry definition meet the ECFSPR definition? *

Yes

The ECFSPR definition for this variable is the following: 1. Acute or subacute clinical deterioration (cough, wheeze, exercise intolerance, exercise-induced asthma, change in pulmonary function, or increased sputum production) not attributable to another etiology; 2. Total IgE > 500 IU/ml; 3. Positive skin prick test for Aspergillus antigen (> 3 mm) or positive specific IgE for A. fumigatus; 4. Either: a. precipitins to A. fumigatus or in vitro demonstration of IgG antibody to A. fumigatus; b. or new or recent abnormalities on chest radiography (infiltrates or mucus plugging) or chest CT (characteristic changes) that have not cleared with antibiotics and standard physiotherapy.

6.1.5 What is the difference in your definition? *

6.2 Distal intestinal obstruction syndrome - DIOS - this year

6.2.1 Did you send the variable to the ECFSPR? *

Yes

The ECFSPR requires this variable to be coded as follows: -1 unknown -99 missing (variable not collected) 0 no 1 yes

6.2.2 In the Excel file, did you code this variable according to the coding above? *

Yes

6.2.3 What is the difference in your coding? *

6.3 Salt Loss Syndrome this year

6.3.1 Did you send the variable to the ECFSPR? *

Yes

The ECFSPR requires this variable to be coded as follows: -1 unknown -99 missing (variable not collected) 0 no 1 yes

6.3.2 In the Excel file, did you code this variable according to the coding above? *

Yes

6.3.3 What is the difference in your coding? *

6.4 Diabetes and type of treatment during the year of follow-up

6.4.1 Did you send the variable to the ECFSPR? *

Yes

The ECFSPR requires this variable to be coded as follows: -1 unknown -99 missing (variable not collected) 0 no 1 yes, treated with daily insulin. 2 yes, treated with oral hypoglycaemic agents 3 yes, dietary advice only 4 yes, therapy unknown

6.4.2 In the Excel file, did you code this variable according to the coding above? *

Yes

6.4.3 What is the difference in your coding? *

6.5 Pneumothorax requiring chest drain during the year of follow-up

6.5.1 Did you send the variable to the ECFSPR? *

Yes

The ECFSPR requires this variable to be coded as follows: -1 unknown -99 missing (variable not collected) 0 no 1 yes

6.5.2 In the Excel file, did you code this variable according to the coding above? *

Yes

6.5.3 What is the difference in your coding? *

6.6 Liver disease during the year of follow-up

6.6.1 Did you send the variable to the ECFSPR? *

Yes

The ECFSPR requires this variable to be coded as follows: -1 unknown -99 missing (variable not collected) 0 no 1 cirrhosis with hypertension/hypersplenism 2 cirrhosis without hypertension/hypersplenism 3 cirrhosis, hypertension unknown 4 liver disease without cirrhosis 5 variceal bleeding

6.6.2 In the Excel file, did you code this variable according to the coding above? *

Yes

6.6.3 What is the difference in your coding? *

6.6.4 Does your registry definition meet the ECFSPR definition? *

Yes

The ECFSPR definition for this variable is the following: We adopt the definitions for Liver Disease used by the UK Registry. These definitions discriminate patients with severe liver disease (with portal hypertension) from milder cases (cirrhosis without portal hypertension): a) Cirrhosis with Hypertension: scarring of the liver related to underlying CF, typically in a biliary pattern. Severe liver disease may include portal hypertension and/or hypersplenism; b) Cirrhosis without Hypertension: scarring of the liver relating to underlying CF; c) Liver disease without cirrhosis: this includes fatty liver or viral hepatitis but not biliary cirrhosis.

6.6.5 What is the difference in your definition *

6.7 Hemoptysis - major over 240 ml - during the year of follow-up

6.7.1 Did you send the variable to the ECFSPR? *

Yes

The ECFSPR requires this variable to be coded as follows: -1 unknown -99 missing (variable not collected) 0 never during the year of follow-up 1 yes, at least once during the year of follow-up

6.7.2 In the Excel file, did you code this variable according to the coding above? *

Yes

6.7.3 What is the difference in your definition *

6.7.4 Does your registry definition meet the ECFSPR definition? *

Yes

Hemoptysis major over 250 ml, (in one day) this year

6.7.5 What is the difference in your definition *

6.8 Occurrence of malignancy during the year of follow-up

6.8.1 Did you send the variable to the ECFSPR? *

Yes

The ECFSPR requires this variable to be coded as follows: -1 unknown -99 missing (variable not collected) 0 no 1 colorectal cancer 2 small bowel cancer 3 lymphoid leukemia 4 testicular cancer 5 breast cancer 6 thryoid gland cancer 7 other/type unknown

6.8.2 In the Excel file, did you code this variable according to the coding above? *

Yes

6.8.3 What is the difference in your coding? *

6.9 Pancreatic status: faecal elastase

6.9.1 Did you send the variable to the ECFSPR? *

Yes

The ECFSPR requires this variable to be coded as follows: -1 unknown -99 missing (variable not collected) 0 no 1 <200 µg/g once 2 <200 µg/g twice 3 ≥200 µg/g once 4 ≥200 µg/g twice

6.9.2 In the Excel file, did you code this variable according to the coding above? *

Yes

6.9.3 What is the difference in your coding? *

6.9.4 Does your registry definition meet the ECFSPR definition? *

Yes

The ECFSPR definition for this variable is the following: Stool fat (van de Kamer) > 4-5 g/d in young children, > 7g/d in children above 10 yrs and adults and/or faecal pancreatic elastase-1 < 200 ug/g. Two determinations are mandatory: faecal fat excretion values of infants below 3 months are contradictory; causes of steatorrhoea, other than those which are pancreas-related, must have been excluded. Pancreatic status will be assessed at the registry level, according to the following: a) Pancreatic insufficiency Faecal elastase <200 µg/g (twice) and Faecal fat high* (twice); b) Pancreatic sufficiency Faecal elastase ≥200 µg/g (twice) and Faecal fat normal* (twice). *according to the definition above.

6.9.5 What is the difference in your definition? *

6.10 Pancreatic status: faecal fat

6.10.1 Did you send the variable to the ECFSPR? *

Yes

The ECFSPR requires this variable to be coded as follows: -1 unknown -99 missing (variable not collected) 0 not done 1 high once 2 high twice 3 normal once 4 normal twice

6.10.2 In the Excel file, did you code this variable according to the coding above? *

Yes

6.10.3 What is the difference in your coding? *

6.10.4 Does your registry definition meet the ECFSPR definition? *

Yes

6.10.5 What is the difference in your definition? *

7. TRANSPLANTS

7.1 Last liver transplant

7.1.1 Did you send the variable to the ECFSPR? *

Yes

The ECFSPR requires this variable to be coded as follows: -1 unknown -99 missing (variable not collected) 0 no 1 yes

7.1.2 In the Excel file, did you code this variable according to the coding above? *

Yes

7.1.3 What is the difference in your coding? *

7.2 Year of last liver transplant

7.2.1 Did you send the variable to the ECFSPR? *

Yes

The ECFSPR requires this variable to be coded as follows: 4 digits If LiverTransp = 1 but year of latest liver transplant is unknown, use 9999 If LiverTransp = -1/-99/0, leave blank

7.2.2 In the Excel file, did you code this variable according to the coding above? *

Yes

7.2.3 What is the difference in your coding? *

7.3 Last lung transplant

7.3.1 Did you send the variable to the ECFSPR? *

Yes

The ECFSPR requires this variable to be coded as follows: -1 unknown -99 missing (variable not collected) 0 no 1 yes

7.3.2 In the Excel file, did you code this variable according to the coding above? *

Yes

7.3.3 What is the difference in your coding? *

7.4 Year of last lung transplant

7.4.1 Did you send the variable to the ECFSPR? *

Yes

The ECFSPR requires this variable to be coded as follows: 4 digits If LungTransp = 1 but year of latest lung transplant is unknown, use 9999 If LungTransp = -1/-99/0, leave blank

7.4.2 In the Excel file, did you code this variable according to the coding above? *

Yes

7.4.3 What is the difference in your coding? *

7.5 Last kidney transplant

7.5.1 Did you send the variable to the ECFSPR? *

Yes

The ECFSPR requires this variable to be coded as follows: -1 unknown -99 missing (variable not collected) 0 no 1 yes

7.5.2 In the Excel file, did you code this variable according to the coding above? *

Yes

7.5.3 What is the difference in your coding? *

7.6 Year of last kidney transplant

7.6.1 Did you send the variable to the ECFSPR? *

Yes

The ECFSPR requires this variable to be coded as follows: 4 digits If KidneyTransp = 1 but year of latest kidney transplant is unknown, use 9999 If KidneyTransp = -1/-99/0, leave blank

7.6.2 In the Excel file, did you code this variable according to the coding above? *

Yes

7.6.3 What is the difference in your coding? *

7.7 Last other transplant

7.7.1 Did you send the variable to the ECFSPR? *

Yes

The ECFSPR requires this variable to be coded as follows: -1 unknown -99 missing (variable not collected) 0 no 1 yes

7.7.2 In the Excel file, did you code this variable according to the coding above? *

Yes

7.7.3 What is the difference in your coding? *

7.8 Year of last other transplant

7.8.1 Did you send the variable to the ECFSPR? *

Yes

The ECFSPR requires this variable to be coded as follows: 4 digits If OtherTransp = 1 but year of latest other transplant is unknown, use 9999 If OtherTransp = -1/-99/0, leave blank

7.8.2 In the Excel file, did you code this variable according to the coding above? *

Yes

7.8.3 What is the difference in your coding? *

Any Additional Comments

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