Kistik Fibrozisli Hastalarda COVID-19’un Etkisi ve Tıbbi Beslenme Tedavisi

Abstract

Kistik fibrozis, epitel hücresi membranındaki Kistik Fibrozis Transmembran Regülator adlı klor kanalında meydana gelen mutasyon sonucu dış salgı bezlerindeki fonksiyon bozukluğudur. Kistik Fibrozis Transmembran Regülatordeki yapısal ve fonksiyonel bozukluk; solunum yolları, pankreas, safra kanalları, üreme organları ve gastrointestinal sistem gibi organların epitel hücre membranlarında klor transportunun bozulmasına neden olur. Kistik fibrozisli hastalarda görülen akciğer enfeksiyonu morbidite ve mortalite prevalansını arttırmaktadır. Kistik fibrozis; hava yollarında oluşan kalınlaşmış sekresyonlar, enfeksiyon artışı ve kalıcılığı için olumsuz bir ortam yaratmaktadır. Viral enfeksiyonlar, kistik fibrozisin seyrinde önemli bir rol oynadığı için, kistik fibrozisli hastalarının şiddetli akut solunum sendromu koronavirüs-2 enfeksiyonu durumunda ciddi belirtiler geliştirme riskinin yüksek olduğu düşünülmektedir. Ancak kistik fibrozisli hastalar arasında koruyucu maske takılması, insanlarla temasın az olması veya konakçı faktörlerden kaçınma gibi davranışlar sayesinde akut solunum sendromu koronavirüs-2 insidansının (%0,07) genel popülasyonlardaki ortalama insidansın (%0,15) altında olduğu görülmüştür. Kistik fibrozisli hastalar ve aileleri, hastalığın yönetilmesinde genel popülasyona kıyasla birçok zorlukla karşı karşıyadır. Özellikle pandemi döneminde ilaç tedavisi, fizyoterapi, egzersiz ve tıbbi beslenme tedavisi gibi yöntemlerle yönetilen kistik fibrozis tedavi sürecinde kişiselleştirilmiş beslenme tedavisi uygulayarak bireylerin sağlığını olumlu yönde etkilemek oldukça önemlidir. Pandemi dönemi; besine ulaşımdaki zorluk, sosyal izolasyon ve gıda güvensizliği gibi nedenler ile kistik fibrozisli hastaların beslenme alışkanlıklarında değişikliklere neden olmuş olabilir. Bu derleme makalenin amacı kistik fibrozisli hastalarda COVID-19’un etkisini incelemek ve pandemi dönemindeki tıbbi beslenme tedavisini değerlendirmektir.

Keywords

• COVID-19
• kistik fibrozis
• beslenme tedavisi.

Effect of COVID-19 and Medical Nutrition Therapy in Patients with Cystic Fibrosis

Abstract

Cystic fibrosis is a dysfunction in the exocrine glands as a result of mutation in the chloride channel called Cystic Fibrosis Transmembrane Regulator in the epithelial cell membrane. Structural and functional disorder in Cystic Fibrosis Transmembrane Regulator; it causes disruption of chloride transport in epithelial cell membranes of organs such as respiratory tract, pancreas, bile ducts, reproductive organs and gastrointestinal tract. Lung infection seen in patients with cystic fibrosis increases the prevalence of morbidity and mortality. Cystic fibrosis; The thickened secretions formed in the airways create a negative environment for the increase and persistence of infection. Because viral infections play an important role in the course of cystic fibrosis, patients with cystic fibrosis are thought to be at high risk of developing severe symptoms in the case of severe acute respiratory syndrome coronavirus-2 infection. However, among patients with cystic fibrosis, the incidence of acute respiratory syndrome coronavirus-2 (0.07%) was found to be below the average incidence (0.15%) in general populations, thanks to behaviors such as wearing a protective mask, little contact with people, or avoiding host factors. Patients with cystic fibrosis and their families face many challenges in managing the disease compared to the general population. It is very important to positively affect the health of individuals by applying personalized nutrition therapy in the treatment process of cystic fibrosis, which is managed by methods such as drug therapy, physiotherapy, exercise and medical nutrition therapy, especially during the pandemic period. Pandemic period; Difficulty in accessing food, social isolation and food insecurity may have caused changes in the nutritional habits of patients with cystic fibrosis. The aim of this review article is to examine the effect of COVID-19 in patients with cystic fibrosis and to evaluate medical nutrition therapy during the pandemic period.

Keywords

• COVID-19
• cystic fibrosis
• nutrition therapy.

References

1. Fanconi G, Uehlinger E, Knauer C. Das Coeliakiesyndrom bei angeborener zystischer Pankreasfibromatose und Bronchiektasien. Wien Med Wochenschr. 1936;86:753-6.
2. Andersen DH. Cystic fibrosis of the pancreas and its relation to celiac disease: a clinical and pathologic study. American journal of Diseases of Children. 1938;56(2):344-99.
3. Naehrig S, Chao CM, Naehrlich L. Cystic Fibrosis. Dtsch Arztebl Int. 2017;114(33-34):564-74.
4. Scotet V, L'Hostis C, Ferec C. The Changing Epidemiology of Cystic Fibrosis: Incidence, Survival and Impact of the CFTR Gene Discovery. Genes (Basel). 2020;11(6).
5. Aydemir Ö, Kiper N. Dünyada ve Ülkemizde Kistik Fibrozis Hastalığı. Türkiye Klinikleri J Int Med Sci. 2007;3(14):1-3.
6. Hangul M, Pekcan S, Kose M, Acican D, Sahlar TE, Erdogan M, et al. The Incidence of Cystic Fibrosis in the Central Region of Anatolia in Turkey Between 2015 and 2016. Balkan Med J. 2019;36(3):179-83.
7. Parkins MD, Somayaji R, Waters VJ. Epidemiology, Biology, and Impact of Clonal Pseudomonas aeruginosa Infections in Cystic Fibrosis. Clin Microbiol Rev. 2018;31(4).
8. Türk Toraks Derneği. Kistik Fibrozis Tanı ve Tedavi Rehberi. Türk Toraks Dergisi. 2011;12(2):1-140.

9. Gibson LE, Cooke RE. A test for concentration of electrolytes in sweat in cystic fibrosis of the pancreas utilizing pilocarpine by iontophoresis. Pediatrics. 1959;23(3):545-9.
10. Rowe SM, Clancy JP, Wilschanski M. Nasal potential difference measurements to assess CFTR ion channel activity. Methods Mol Biol. 2011;741:69-86.
11. National Health Service. Cystic Fibrosis: Treatment. [cited 2022 Mar 16]. Avaible from: https://www.nhs.uk/conditions/cystic-fibrosis/treatment/.
12. Seyed Hosseini E, Riahi Kashani N, Nikzad H, Azadbakht J, Hassani Bafrani H, Haddad Kashani H. The novel coronavirus Disease-2019 (COVID-19): Mechanism of action, detection and recent therapeutic strategies. Virology. 2020;551:1-9.
13. Bain R, Cosgriff R, Zampoli M, Elbert A, Burgel PR, Carr SB, et al. Clinical characteristics of SARS-CoV-2 infection in children with cystic fibrosis: An international observational study. J Cyst Fibros. 2021;20(1):25-30.
14. Stanton BA, Hampton TH, Ashare A. SARS-CoV-2 (COVID-19) and cystic fibrosis. Am J Physiol Lung Cell Mol Physiol. 2020;319(3):L408-L15.
15. Peckham D, McDermott MF, Savic S, Mehta A. COVID-19 meets Cystic Fibrosis: for better or worse? Genes Immun. 2020;21(4):260-2.
16. Fainardi V, Longo F, Chetta A, Esposito S, Pisi G. Sars-CoV-2 infection in patients with cystic fibrosis. An overview. Acta Biomed. 2020;91(3):e2020035.
17. Mathew HR, Choi MY, Parkins MD, Fritzler MJ. Systematic review: cystic fibrosis in the SARS-CoV-2/COVID-19 pandemic. BMC pulmonary medicine. 2021;21(1):1-11.
18. Colombo C, Burgel PR, Gartner S, van Koningsbruggen-Rietschel S, Naehrlich L, Sermet-Gaudelus I, et al. Impact of COVID-19 on people with cystic fibrosis. Lancet Respir Med. 2020;8(5):e35-e6.
19. Mondejar-Lopez P, Quintana-Gallego E, Giron-Moreno RM, Cortell- Aznar I, Ruiz de Valbuena-Maiz M, Diab-Caceres L, et al. Impact of SARS-CoV-2 infection in patients with cystic fibrosis in Spain: Incidence and results of the national CF-COVID19-Spain survey. Respir Med. 2020;170:106062.
20. Chapman KD, Moffett KS. Cystic Fibrosis and COVID-19. South Med J. 2020;113(9):422.
21. Bezzerri V, Lucca F, Volpi S, Cipolli M. Does cystic fibrosis constitute an advantage in COVID-19 infection? Italian Journal of Pediatrics. 2020;46(1):1-3.
22. Çocuk Solunum Yolu Hastalıkları ve Kistik Fibrozis Derneği. Hekimler için Kistik Fibrozis Tanı ve Tedavi Rehberi. Ankara; 2020. Available from: https://www.kistikfibrozisturkiye.org/wp-content/uploads/2020/01/ Hekim-KF-kitapcik-son-basilan.pdf
23. Turck D, Braegger CP, Colombo C, Declercq D, Morton A, Pancheva R, et al. ESPEN-ESPGHAN-ECFS guidelines on nutrition care for infants, children, and adults with cystic fibrosis. Clin Nutr. 2016;35(3):557-77.
24. Somaraju URR, Solis-Moya A. Pancreatic enzyme replacement therapy for people with cystic fibrosis. Cochrane Database Syst Rev. 2020;8:CD008227.
25. Brown PS, Durham D, Tivis RD, Stamper S, Waldren C, Toevs SE, et al. Evaluation of Food Insecurity in Adults and Children With Cystic Fibrosis: Community Case Study. Front Public Health. 2018;6:348.
26. Feeding America. The impact of the coronavirus on food insecurity in 2020 & 2021; 2021 [cited 2022 Aug 9]. Avaible from: https:// www.feedingamerica.org/sites/default/files/2021-03/National%20 Projections%20Brief_3.9.2021_0.pdf.
27. Lim JT, Ly NP, Willen SM, Iwanaga K, Gibb ER, Chan M, et al. Food insecurity and mental health during the COVID-19 pandemic in cystic fibrosis households. Pediatr Pulmonol. 2022;57(5):1238-44.
28. Academy of Nutrition and Dietetics Evidence Analysis Center. Cystic Fibrosis Nutrition Care during the Novel Coronavirus Covid-19 Pandemic. [cited 2022 Aug 9]. Available from: https://www.andeal. org/files/files/Cystic%20Fibrosis%202020/CF_nutrition-care-during-covid19.pdf