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.