Background
In Turkey, there are approximately 3,000 pw CF. CF centers are supported by the Turkish Ministry of Health (MoH). CF clinical outcomes in Turkey have not followed those reported in the USA and Western European Countries. CF in Turkey shows a disproportionate rate of morbidity and mortality, as indicated by the current number of CF patients at Marmara CF Center (Ma CFC) with most patients are below 18 years of age (297 patients are 0-17 years of age and only 70 patients are 18 years and above in 2020). Most patients with CF in Turkey are children and their quality of life is poor. They are likely to have low body mass index (BMI) and low forced expiratory volume at 1 second % predicted (FEV1% predicted (FEV1pp)). Ma CFC is the largest in Istanbul and second largest in Turkey. The center has infrastructure support including a CF nurse, Physical therapist, and a part time dietitian, sweat testing lab, genetic testing, and microbiology laboratory. However, the allied health support was inadequate for the size of that center.
A newborn screening program for CF started in 2015 which uses immunoreactive trypsinogen (IRT) combined with repeat IRT, followed by sweat testing, and DNA sequencing. Sweat testing laboratories are using the coulometric method, and it was certified by MoH. This method was found to be compatible with classic sweat testing methods using Gibson & Cooke and Chloridometer Methods.7 F508del homozygous mutations is present in only 28% of the patients. The most common mutations are F508del, 1677delTA, N1303K. Ma CFC has been reporting to the ECFSPR since 2017. Median age at diagnosis and follow-up duration of the patients was 0.3 (IQR:0.2-0.9) and 11.9 (IQR:5.4-16.1) years, respectively. Most CF medications are available in Turkey, covered by the government and free for patients. There is a Turkish CF patient and family organization (KIFDER). This organization has been very active in lobbying on behalf of pw CF and CF centers with the government and, also is very involved in CF education, in collaboration with CF centers across Turkey.
UoM CFC follows approximately 275 pediatric patients and over 600 total pw CF. The center data has been above average for USA centers and is well supported with adequate staffing. The center is very active in research and quality improvement (QI) work.