TY - JOUR T1 - Cystic Fibrosis Patients Eligible for Modulator Drugs: Data from Cystic Fibrosis Registry of Turkey JF - European Respiratory Journal JO - Eur Respir J DO - 10.1183/13993003.congress-2019.PA4518 VL - 54 IS - suppl 63 SP - PA4518 AU - Fatma Nazan Çobanoğlu AU - Deniz Doğru Ersöz AU - Erkan Çakır AU - Tuğba Şişmanlar Eyüboğlu AU - Sevgi Pekcan AU - Güzin Cinel AU - Ebru Yalçın AU - Nural Kiper AU - Velat Şen AU - Hadice Selimoğlu Şen AU - Ömür Ercan AU - Özlem Keskin AU - Sevgi Bilgiç Eltan AU - Lina Muhammed Al Shadfan AU - Hakan Yazan AU - Derya Ufuk Altıntaş AU - Şenay Şaşihüseyinoğlu AU - Nihat Sapan AU - Şükrü Çekiç AU - Haluk Çokuğraş AU - Ayşe Ayzıt Atabek AU - Tuğba Ramaslı Gürsoy AU - Ayşe Tana Aslan AU - Ayşen Bingöl AU - Abdurrahman Erdem Başaran AU - Ali Özdemir AU - Mehmet Köse AU - Melih Hangül AU - Nagehan Emiralioğlu AU - Gökçen Tuğcu AU - Hasan Yüksel AU - Özge Yılmaz AU - Fazıl Orhan AU - Zeynep Gökçe Gayretli Aydın AU - Erdem Topal AU - Zeynep Tamay AU - Ayşe Süleyman AU - Demet Can AU - Cem Murat Bal AU - Gönül Çaltepe AU - Uğur Özçelik Y1 - 2019/09/28 UR - http://erj.ersjournals.com/content/54/suppl_63/PA4518.abstract N2 - Better understanding of CFTR biology has led to the development of modulator drugs. Lumacaftor/Ivacaftor (L/T) is approved for people ages ≥2 who have two copies of the F508del mutation. Tezacaftor/Ivacaftor (T/I) is approved for people ages ≥12 who have two copies of the F508del mutation and also approved for people who have a single copy of one of 26 specific mutations. Ivacaftor (I) is approved for people ages ≥1 who have a single copy of one of the mutations approved for T/I and additionally for 12 specific mutations. We aimed to find out the number of patients recorded in Cystic Fibrosis Registry of Turkey (CFRT) in 2018 who are eligible for modulator therapy. Of 1170 patients, 128 are homozygote F508del (22 mths-36 yrs), and 123 (10.51%) are aged ≥2 yrs and eligible for L/T. Among 128 patients with homozygote F508del, 48 are aged ≥12 yrs and among 42 patients (1-31 yrs) who have one or two copies of 3849+10kbC→T, A455E, D110H, D579G, F1052V, R74W, R347H, S945L, 13 are aged ≥12 yrs, and totally 61 (5.21%) patients are eligible for T/I. Finally, total 68 (5.81%) patients (1-31 yrs) have one or two copies of D110H, G178R, G1069R, G1349D, R117H and S549R mutations in addition to the mutations stated in T/I group and all of them are older than one year old and eligible for I therapy. According to the data, approximately one fifth of CF patients are eligible for modulator drug therapy. Compared to North European and North American CF patients, less Turkish patients are eligible for modulator therapies, so new treatment modalities are necessary for them.FootnotesCite this article as: European Respiratory Journal 2019; 54: Suppl. 63, PA4518.This is an ERS International Congress abstract. No full-text version is available. Further material to accompany this abstract may be available at www.ers-education.org (ERS member access only). ER -