Assessment of Family Physicians' and Family Physician Assistants' Knowledge of Cystic Fibrosis Disease and Screening Tests
Öz
ABSTRACT
Aim: In our country, since January 1, 2015, a cystic fibrosis(CF) screening program with heel-prick blood sampling in newborns has been implemented in primary health care. Currently, CF is known as a childhood disease with a median survival of 44.4 years and more than 50% of patients are over 18 years of age. Therefore, the role of family physicians in disease management is not limited to the neonatal period, but also includes the follow-up of adult patients. The aim of this study was to investigate the knowledge of primary care physicians and family medicine residents about CF and screening.
Matherials and Methods: The population of the cross-sectional and descriptive study consisted of family physicians and family medicine residents working in Konya city center. The online questionnaire form included information questions designed to measure the sociodemographic characteristics of the participants and their level of knowledge about CF. A total of 230 volunteer physicians were included in the study. Data from the study were analyzed using the SPSS v.27 statistical program.
Results: The knowledge score of family medicine specialists was higher than that of other participants. The mean knowledge score of the participants was 27.16±5.57. Family physicians had a high level of knowledge about screening test and organ involvement in CF.
Conclusion: Early diagnosis of CF is very important for the preservation of lung function. Therefore, it would be beneficial for primary care physicians to receive regular training in the diagnosis and follow-up of CF and to be informed about the complications in the adult life of CF patients with prolonged life expectancy.
Anahtar Kelimeler
Etik Beyan
In accordance with the Declaration of Helsinki (2013), on 19.07.2024, approval number 2024/5131 was obtained from Necmettin Erbakan University Pharmaceutical and Non-Medical Device Research Ethics Committee.
Destekleyen Kurum
The authors did not receive support from any organization for the submitted work.
Kaynakça
- 1. Stoltz DA, Meyerholz DK, Welsh MJ. Origins of cystic fibrosis lung disease. N Engl J Med. 2015;372(4):351-62.
- 2. Davis PB. Cystic fibrosis since 1938. Am J Respir Crit Care Med. 2006;173(5):475-82.
- 3. Elborn JS. Cystic fibrosis. Lancet. 2016;388(10059):2519-31.
- 4. www.ki̇sti̇kfi̇brozi̇sturki̇ye.org [homepage on the Internet]. Ulusal yenidoğan tarama programı kistik fibrozis taraması ter testi rehberi [updated 2015; cited 15 Apr 2025]. Available from: https://www.kistikfibrozisturkiye.org/wp-content/uploads/2019/12/Ter-testi-rehberi.pdf
- 5. Wagener JS, Sontag MK, Accurso FJ. Newborn screening for cystic fibrosis. Curr Opin Pediatr. 2003;15(3):309-15.
- 6. Thia LP, Hoo A-F, Bush A, et al. Evolution of lung function during the first year of life in newborn screened cystic fibrosis infants. Thorax. 2014;69(10):910-7.
- 7. Dijk FN, McKay K, Barzi F, Gaskin KJ, Fitzgerald DA. Improved survival in cystic fibrosis patients diagnosed by newborn screening compared to a historical cohort from the same centre. Arch Dis Child. 2011;96(12):1118-23.
- 8. Andersen DH. Cystic fibrosis of the pancreas and its relation to celiac disease: a clinical and pathologic study. Am J Dis Child. 1938;56(2):344-99.
- 9. www.ki̇sti̇kfi̇brozi̇sturki̇ye.org [homepage on the Internet]. Cystic Fibrosis Foundation Patient Registry 2018 Annual Report [updated 2019; cited 15 Apr 2025]. Available from: https://www.cff.org/sites/default/files/2021-10/2018-Annual-Report.pdf
- 10.www.ki̇sti̇kfi̇brozi̇sturki̇ye.org [homepage on the Internet]. Ulusal Kistik Fibrozis Kayıt Sistemi 2023 Yılı Verileri [updated 2023; cited 15 Apr 2025]. Available from: https://www.kistikfibrozisturkiye.org/wp-content/uploads/2024/11/2023-UKKS-2.pdf
- 11. Cesur Y, Doğan M, Arıyuca S, et al. Hastaneye başvuran malnutrisyonu ve/veya tekrarlayan akciğer enfeksiyonu olan çocuklarda kistik fibrozis sıklığı araştırılması (The evaluation of cystic fibrosis frequency in children with malnutrition and/or recurrent pulmonary infection). Selçuk Ünv Tıp Derg. 2010;26:138-41.
- 12. Sepe A, Romano C, Landi I, et al. Pseudo‐Bartter syndrome in infant with cystic fibrosis screen positive, inconclusive diagnosis: A case report. Clin Case Rep. 2023;11(11):e8046.
- 13. Sismanlar Eyuboglu T, Dogru D, Çakır E, et al. Clinical features and accompanying findings of Pseudo‐Bartter Syndrome in cystic fibrosis. Pediatr Pulmonol. 2020;55(8):2011-6.
- 14. Gökdemir Y, Doğru Ersöz D, editors. Türk Toraks Derneği Kistik Fibrozis Tanı ve Tedavi Rehberi (Turkish Thoracic Society Cystic Fibrosis Diagnosis and Treatment Guidelines). Ankara;2024. p:6. ISBN: 978-625-6615-08-3
- 15. Moran A, Dunitz J, Nathan B, Saeed A, Holme B, Thomas W. Cystic fibrosis–related diabetes: current trends in prevalence, incidence, and mortality. Diabetes care. 2009;32(9):1626-31.
- 16. Finkelstein SM, Wielinski CL, Elliott GR, et al. Diabetes mellitus associated with cystic fibrosis. The J Pediatr. 1988;112(3):373-7.
- 17. Lanng S, Thorsteinsson B, Nerup J, Koch C. Influence of the development of diabetes mellitus on clinical status in patients with cystic fibrosis. Eur J Pediatr. 1992;151:684-7.
- 18. Asseri AA. Cystic fibrosis knowledge and practice among primary care physicians in southwest region, Saudi Arabia. J Family Med Prim Care. 2020;9(3):1354-61.
- 19. Demirtaş MS, Topal E, Kaplan F, Kılıç T. Aile hekimlerinin kistik fibrozis hastalığı, i̇zlemi ve yenidoğan tarama testi konusundaki bilgi düzeyleri (Family physicians' level of knowledge about cystic fibrosis disease, follow-up and newborn screening test). Turk J Fam Pract. 2019;23(2):64-9.
AİLE HEKİMLERİNİN VE AİLE HEKİMLİĞİ ASİSTANLARININ KİSTİK FİBROZİS HASTALIĞI VE TARAMA TESTİ HAKKINDAKİ BİLGİ DÜZEYLERİNİN ARAŞTIRILMASI
Öz
ÖZET
Amaç: Ülkemizde 1 Ocak 2015 tarihinden itibaren birinci basamak sağlık hizmetlerinde yenidoğanlarda topuktan kanı numunesi ile kistik fibrozis (KF) tarama programı uygulanmaktadır. Günümüzde KF, medyan sağkalım süresi 44,4 yıl olan bir çocukluk çağı hastalığı olarak bilinmektedir ve hastaların %50'den fazlası 18 yaşın üzerindedir. Bu nedenle, aile hekimlerinin hastalık yönetimindeki rolü yenidoğan dönemi ile sınırlı olmayıp, yetişkin hastaların takibini de içermektedir. Bu çalışmanın amacı birinci basamak hekimlerinin ve aile hekimliği asistanlarının KF ve tarama konusundaki bilgilerini araştırmaktır.
Gereç ve Yöntem: Kesitsel ve tanımlayıcı tipteki çalışmanın evrenini Konya il merkezinde görev yapan aile hekimleri ve aile hekimliği asistanları oluşturmuştur. Çevrimiçi anket formu, katılımcıların sosyodemografik özelliklerini ve KF hakkındaki bilgi düzeylerini ölçmek için tasarlanmış bilgi sorularını içermektedir. Çalışmaya toplam 230 gönüllü hekim dahil edilmiştir. Veriler SPSS v27.0 programı ile analiz edilmiştir.
Bulgular: Aile hekimliği uzmanlarının bilgi puanı diğer katılımcılara göre daha yüksekti. Katılımcıların ortalama bilgi puanı 27.16±5.57 idi. Aile hekimlerinin KF'de tarama testi ve organ tutulumu hakkında bilgi düzeyleri yüksekti.
Tartışma: KF'nin erken tanısı akciğer fonksiyonlarının korunması için çok önemlidir. Bu nedenle birinci basamak hekimlerinin KF tanısı ve takibi konusunda düzenli eğitim almaları ve yaşam beklentisi uzamış KF hastalarının erişkin yaşamındaki komplikasyonlar konusunda bilgilendirilmeleri yararlı olacaktır.
Anahtar Kelimeler
Kaynakça
- 1. Stoltz DA, Meyerholz DK, Welsh MJ. Origins of cystic fibrosis lung disease. N Engl J Med. 2015;372(4):351-62.
- 2. Davis PB. Cystic fibrosis since 1938. Am J Respir Crit Care Med. 2006;173(5):475-82.
- 3. Elborn JS. Cystic fibrosis. Lancet. 2016;388(10059):2519-31.
- 4. www.ki̇sti̇kfi̇brozi̇sturki̇ye.org [homepage on the Internet]. Ulusal yenidoğan tarama programı kistik fibrozis taraması ter testi rehberi [updated 2015; cited 15 Apr 2025]. Available from: https://www.kistikfibrozisturkiye.org/wp-content/uploads/2019/12/Ter-testi-rehberi.pdf
- 5. Wagener JS, Sontag MK, Accurso FJ. Newborn screening for cystic fibrosis. Curr Opin Pediatr. 2003;15(3):309-15.
- 6. Thia LP, Hoo A-F, Bush A, et al. Evolution of lung function during the first year of life in newborn screened cystic fibrosis infants. Thorax. 2014;69(10):910-7.
- 7. Dijk FN, McKay K, Barzi F, Gaskin KJ, Fitzgerald DA. Improved survival in cystic fibrosis patients diagnosed by newborn screening compared to a historical cohort from the same centre. Arch Dis Child. 2011;96(12):1118-23.
- 8. Andersen DH. Cystic fibrosis of the pancreas and its relation to celiac disease: a clinical and pathologic study. Am J Dis Child. 1938;56(2):344-99.
- 9. www.ki̇sti̇kfi̇brozi̇sturki̇ye.org [homepage on the Internet]. Cystic Fibrosis Foundation Patient Registry 2018 Annual Report [updated 2019; cited 15 Apr 2025]. Available from: https://www.cff.org/sites/default/files/2021-10/2018-Annual-Report.pdf
- 10.www.ki̇sti̇kfi̇brozi̇sturki̇ye.org [homepage on the Internet]. Ulusal Kistik Fibrozis Kayıt Sistemi 2023 Yılı Verileri [updated 2023; cited 15 Apr 2025]. Available from: https://www.kistikfibrozisturkiye.org/wp-content/uploads/2024/11/2023-UKKS-2.pdf
- 11. Cesur Y, Doğan M, Arıyuca S, et al. Hastaneye başvuran malnutrisyonu ve/veya tekrarlayan akciğer enfeksiyonu olan çocuklarda kistik fibrozis sıklığı araştırılması (The evaluation of cystic fibrosis frequency in children with malnutrition and/or recurrent pulmonary infection). Selçuk Ünv Tıp Derg. 2010;26:138-41.
- 12. Sepe A, Romano C, Landi I, et al. Pseudo‐Bartter syndrome in infant with cystic fibrosis screen positive, inconclusive diagnosis: A case report. Clin Case Rep. 2023;11(11):e8046.
- 13. Sismanlar Eyuboglu T, Dogru D, Çakır E, et al. Clinical features and accompanying findings of Pseudo‐Bartter Syndrome in cystic fibrosis. Pediatr Pulmonol. 2020;55(8):2011-6.
- 14. Gökdemir Y, Doğru Ersöz D, editors. Türk Toraks Derneği Kistik Fibrozis Tanı ve Tedavi Rehberi (Turkish Thoracic Society Cystic Fibrosis Diagnosis and Treatment Guidelines). Ankara;2024. p:6. ISBN: 978-625-6615-08-3
- 15. Moran A, Dunitz J, Nathan B, Saeed A, Holme B, Thomas W. Cystic fibrosis–related diabetes: current trends in prevalence, incidence, and mortality. Diabetes care. 2009;32(9):1626-31.
- 16. Finkelstein SM, Wielinski CL, Elliott GR, et al. Diabetes mellitus associated with cystic fibrosis. The J Pediatr. 1988;112(3):373-7.
- 17. Lanng S, Thorsteinsson B, Nerup J, Koch C. Influence of the development of diabetes mellitus on clinical status in patients with cystic fibrosis. Eur J Pediatr. 1992;151:684-7.
- 18. Asseri AA. Cystic fibrosis knowledge and practice among primary care physicians in southwest region, Saudi Arabia. J Family Med Prim Care. 2020;9(3):1354-61.
- 19. Demirtaş MS, Topal E, Kaplan F, Kılıç T. Aile hekimlerinin kistik fibrozis hastalığı, i̇zlemi ve yenidoğan tarama testi konusundaki bilgi düzeyleri (Family physicians' level of knowledge about cystic fibrosis disease, follow-up and newborn screening test). Turk J Fam Pract. 2019;23(2):64-9.
Ayrıntılar
| Birincil Dil | İngilizce |
|---|---|
| Konular | Çocuk Göğüs Hastalıkları |
| Bölüm | Orjinal Araştırma |
| Yazarlar | |
| Yayımlanma Tarihi | 31 Temmuz 2025 |
| Gönderilme Tarihi | 15 Nisan 2025 |
| Kabul Tarihi | 7 Haziran 2025 |
| Yayımlandığı Sayı | Yıl 2025 Cilt: 15 Sayı: 4 |
