THE EFFECTS OF THE COVID-19 PANDEMIC ON PEOPLE WITH RARE DISEASES

Year 2021, Volume: 15 Issue: 4, 685 - 697, 20.12.2021

Merve Deniz Pak Güre

https://doi.org/10.21763/tjfmpc.918503

Abstract

Aim/Background: COVID-19 has negative effects on both healthcare and social service delivery systems. During the pandemic, some groups of society have found themselves in a position in which their disadvantage grew bigger. One of these groups include people with rare diseases. 30 million people with a rare disease in Europe and 300 million worldwide were among the most vulnerable population groups during the pandemic. Because, rare diseases that are chronic, complex, degenerative and cause serious sensation loss and create special care needs. This study aims to examine how people with rare diseases are affected by COVID-19. Method: Scopus, Web of Science, Networked Digital Library of Theses & Dissertations, ProQuest, and Google Scholar databases reviewed using the keywords 'rare diseases and COVID-19', 'rare diseases and coronavirus' and 'the impact of COVID-19 on rare diseases' in Turkish and English. As a result, 40 studies were found and 18 articles were determined to be in accordance with the inclusion criteria. Results: The findings of the study show that people with rare diseases experience problems in terms of access to healthcare systems, changing daily life dynamics, access to holistic care services, and psychosocial aspects. Severe problems such as restriction of diagnosis, treatment, and rehabilitation services in terms of access to healthcare services are mentioned. In terms of the change of daily life dynamics and access to holistic care, there are disruptions in care services, family members assuming the responsibility of providing care, and families having economic hardships in this process. Psychosocial difficulties include increased levels of anxiety, fear and depression, and limitations in accessing mental health services. Conclusion: It is recommended that service delivery systems should be improved to eliminate the biopsychosocial and economic difficulties of people with rare diseases.

Keywords

COVID-19, Rare Diseases, Disadvantaged groups

COVID-19 PANDEMİSİNİN NADİR HASTALIĞI OLAN BİREYLER ÜZERİNDEKİ ETKİSİ

Abstract

Amaç: COVID-19’un hem sağlık hem de sosyal hizmet sunum sistemleri üzerinde olumsuz etkileri bulunmaktadır. Pandemi sürecinde toplumun bazı kesimleri dezavantajlı olma konumunun daha da derinleştiği bir noktaya taşınmıştır. Bu gruplardan biri de nadir hastalığı bulunan kişileri kapsamaktadır. Nadir hastalığı bulunan Avrupa'da 30 milyon, dünya genelinde ise 300 milyon kişi, pandemi sırasında en savunmasız nüfus grupları arasında yer almıştır. Çünkü genellikle kronik, karmaşık, dejeneratif ve ciddi şekilde yeti yitimlerine ve engelliliğe sebep olan nadir hastalıklar, özel bakım ihtiyaçları yaratmaktadır. Bu çalışma, nadir hastalığı bulunan kişilerin COVID-19'dan nasıl etkilendiğini incelemeyi amaçlamaktadır. Yöntem: Scopus, Web of Science, Networked Digital Library of Theses & Dissertations, ProQuest ve Google Scholar veri tabanları ‘nadir hastalıklar ve COVID-19’, ‘nadir hastalıklar ve koronavirüs’ ve ‘COVID-19'un nadir hastalıklar üzerindeki etkisi’ anahtar kelimeleri kullanılarak Türkçe ve İngilizce olarak taranmıştır. Tarama sonucunda 40 çalışmaya ulaşılmış ve araştırma kapsamına alma kriterlerine uygun 18 makale belirlenmiştir. Bulgular: Çalışmanın bulguları, nadir hastalığı olan hastaların sağlık sistemlerine erişim, günlük yaşam dinamiklerinin değişmesi ve bütünsel bakım hizmetlerine erişim ve psikososyal yönden sorunlar yaşadıklarını göstermektedir. Sağlık hizmetlerine erişim açısından teşhis, tedavi ve rehabilitasyon hizmetlerinin kısıtlanması gibi ciddi sorunlar belirtilmektedir. Günlük yaşam dinamiklerinin değişimi ve bütüncül bakıma erişim açısından, bakım hizmetlerinin aksaması, bakım verme sorumluluğunu aile üyelerinin üstlenmesi ve bu süreçte ailelerin ekonomik olarak zorlanması bulunmaktadır. Psikososyal güçlükler arasında ise anksiyete, korku ve depresyon düzeylerinde artış ile ruh sağlığı hizmetlerine erişimde kısıtlılıkların yaşanması bulunmaktadır. Sonuç: Nadir hastalığı olan bireylerin biyo-psiko-sosyal ve ekonomik yönlerden yaşadığı zorlukların ortadan kaldırılmasında hizmet sunum sistemlerinin geliştirilmesi önerilmektedir.

Keywords

COVID-19, Nadir Hastalıklar, Dezavantajlı Gruplar

References

• 1. Dünya Sağlık Örgütü. Coronavirus disease (COVID-19) Weekly Epidemiological Update and Weekly Operational Update. 2020. https://www.who.int/emergencies/diseases/novel-coronavirus-2019/situation-reports (Erişim 01.04.2021)
• 2. T.C. Sağlık Bakanlığı. https://covid19.saglik.gov.tr/ (Erişim 01.04.2021)
• 3. EURORDIS. (2020). EURORDIS open letter to policy makers: Recommendations to protect people living with a rare disease during the COVID-19 pandemic. 2020. https://www.eurordis.org/covid19openletter (Erişim 01.04.2021)
• 4. Pak MD. Nadir hastalıklarda sosyal hizmetin rolü. Sağlık ve Toplum Dergisi 2017; 27(3):3-13.
• 5. Satman İ, Güdük Ö, Yemenici M, & Ertürk N. Nadir Hastalıklar Raporu. Türkiye Halk Sağlığı ve Kronik Hastalıklar Enstitüsü. Ankara: TÜSEB Yayınları; 2019.p.19-20.
• 6. Haendel M, Vasilevsky N, Unni D, Bologa C, Harris N, Rehm H, & Dawkins H. How many rare diseases are there?. Nature Reviews Drug Discovery 2019;19: 77-78.
• 7. Yavuz Çolak M. Nadir Hastalıklar Epidemiyolojisi. In:Özgür İnce, Merve Deniz Pak editor. Tüm Yönleriyle Nadir Hastalıklar. Ankara: Nobel Akademik Yayınları; 2019.p.16-39.
• 8. Anderson M, Elliott E, Zurynski Y. Australian families living with rare disease: experiences of diagnosis, health services use and needs for psychosocial support. Orphanet J Rare Dis 2013;8(1):22.
• 9. Groft S, Posada dela Paz M. Rare Diseases: Joining mainstream Research and Treatment Based on Reliable Epidemiologic Data. M. Posada de la Paz, D. Taruscio, S. Groft, M. Posada de la Paz, D. Taruscio, & S. Groft içinde, Rare Diseases Epidemiology: Update and Overview Cham: Springer, 2017.p.3-21.
• 10. Doğuç E. Yetim ilaçların (orphan drugs-nadir hastalıklarda kullanılan ilaçlar) Türkiye’de ve dünyadaki durumu. Sağlık Akademisyenleri Dergisi 2017;4(3): 196-201.
• 11. Borski K. Social and welfare aspects of rare diseases. Gen Int Med Clin Innov 2017; 2: 1-4.
• 12. Uluslararası Nadir Hastalıklar Birliği. To ‘Build Back Better’, do not leave behind people living with a rare disease in COVID-19 response and recovery. 2020. https://www.rarediseasesinternational.org/covid-19-resource-center/ (Erişim 01.04.2021)
• 13. NORD. COVID-19 Communıty Survey Report. 2020. https://rarediseases.org/wp-content/uploads/2020/05/NRD-2061-RareInsights-CV19-Report_v2-2.pdf (Erişim 01.04.2021)
• 14. Nadir Hastalık Organizasyonları Asya Pasifik İttifakı. Managing Rare Diseases in APAC during Covid-19. 2020. https://a41c398e-e11c-457f-a97a-cda2c87c58b0.filesusr.com/ugd/0a4f38_5f10964496bd44b4b8570887e58c5d9c.pdf (Erişim 01.04.2021)
• 15. TÜSEB. COVID-19 Pandemisi ve Nadir Hastalıklar. 2020. https://www.tuseb.gov.tr/tuhke/uploads/genel/files/haberler/Ophanet-TR-UDK_COVID-19%20bilgilendirme.pdf (Erişim 01.04.2021)
• 16. Nadir Hastalıklar Ağı. COVID-19’UN “Nadir” Yüzü Basın Açıklaması. 2020. https://www.nadirhastaliklaragi.org.tr/duyuru/57/covid-19-un-%EF%BF%BDnadir-yuzu-basin-aciklamasi (Erişim 01.04.2021)
• 17. Chung CC, Wong WH, Fung JL, Kong RDH, Chung BH. Impact of COVID-19 pandemic on patients with rare disease in Hong Kong. European Journal of Medical Genetics 2020; 63(12): 104062.
• 18. van Koningsbruggen-Rietschel S, Dunlevy F, Bulteel V, Downey DG, Dupont L. SARS-CoV-2 disrupts clinical research: the role of a rare disease-specific trial network. European Respiratory Journal 2020; 56(3):1-3.
• 19. Baynam GS, Wicking C, Bhattacharya K, Millis N. Protecting the rare during a rare pandemic. The Medical Journal of Australia 2020; 213(2): 94-94.
• 20. Talarico R, Marinello D, Cannizzo S, Gaglioti A, Ticciati S, Carta C, Turchetti G. Shaping the future of rare diseases after a global health emergency: organisational points to consider. International Journal of Environmental Research and Public Health 2020; 17(22): 8694.
• 21. Lampe C, Dionisi-Vici C, Bellettato CM, Paneghetti L, van Lingen C, Bond S, Scarpa M. The impact of COVID-19 on rare metabolic patients and healthcare providers: results from two MetabERN surveys. Orphanet journal of rare diseases 2020; 15(1): 1-14.
• 22. Corrons JL, De Sanctis V. Rare anaemias, sickle-cell disease and COVID-19. Acta Bio Medica: Atenei Parmensis 2020; 91(2): 216.
• 23. Brizola E, Adami G, Baroncelli GI, Bedeschi MF, Berardi P, Boero S, Sangiorgi L. Providing high-quality care remotely to patients with rare bone diseases during COVID-19 pandemic. Orphanet Journal of Rare Diseases 2020; 15(1): 1-10.
• 24. Fiumara A, Lanzafame G, Arena A, Sapuppo A, Raudino F, Praticò A, Barone R. COVID-19 Pandemic Outbreak and its Psychological Impact on Patients with Rare Lysosomal Diseases. Journal of clinical medicine 2020; 9(9): 2716.
• 25. Chowdhury SF, Sium SMA, Anwar S. Research and management of rare diseases in the COVID-19 pandemic era: challenges and countermeasures. Frontiers in Public Health 2021; 9, 346.
• 26. Talarico R, Aguilera S, Alexander T, Amoura Z, Antunes AM, Arnaud L, Mosca M. The impact of COVID-19 on rare and complex connective tissue diseases: the experience of ERN ReCONNET. Nature Reviews Rheumatology 2021; 17(3): 177-184.
• 27. Rubio-San-Simon A, Verdú-Amorós J, Hladun R, Juan-Ribelles A, Molero M, Guerra-García P, Bautista F. Challenges in early phase clinical trials for childhood cancer during the COVID-19 pandemic: a report from the new agents group of the Spanish Society of Paediatric Haematology and Oncology (SEHOP). Clinical and Translational Oncology 2021; 23: 183-189.
• 28. McMullan J, Crowe AL, Bailie C, McKnight AJ. Evaluating the impact of COVID-19 on rare disease support groups. BMC Research Notes 2021; 14(1): 1-3.
• 29. Chaplin C. Unmasked: an insight into three patients’ rare disease experiences during the COVID-19 pandemic. Orphanet Journal of Rare Diseases 2021; 16(1): 1-4.
• 30. Aktas P. Chronic and rare disease patients' access to healthcare services during a health crisis: The example of the COVID‐19 pandemic in Turkey. Health Expectations 2021; 1:1-9.
• 31. Pak Güre M, Karataş M, Duyan V. Examination of the effects of COVID-19 on the psychosocial life and the sources of social support of people with rare diseases in Turkey. Asia Pacific Journal of Social Work and Development 2021; 1: 1-15.
• 32. Limongelli G, Iucolano S, Monda E, Elefante P, De Stasio C, Lubrano I, Network CRD. Diagnostic issues faced by a rare disease healthcare network during COVID-19 outbreak: data from the Campania Rare Disease Registry. Journal of public health 2021; 1: 1-9.
• 33. Halley MC, Stanley T, Maturi J, Goldenberg AJ, Bernstein JA, Wheeler MT, Tabor HK. “It seems like COVID-19 now is the only disease present on Earth”: living with a rare or undiagnosed disease during the COVID-19 pandemic. Genetics in Medicine 2021; 23(5): 837-844.
• 34. Chung CCY, Ng YNC, Jain R, Chung BHY. A thematic study: impact of COVID-19 pandemic on rare disease organisations and patients across ten jurisdictions in the Asia Pacific region. Orphanet Journal of Rare Diseases 2021; 16(1): 1-12.
• 35. Mistry P, Balwani M, Barbouth D, Burrow TA, Ginns EI, Goker-Alpan O, & Lee CU. Gaucher disease and SARS-CoV-2 infection: Emerging management challenges. Molecular Genetics and Metabolism. 2020; 130(3): 164–169.
• 36. Courbierand S & Hernando I. The impact of COVID19 for people living with a rare disease. Webinar on the impact of COVID 19 for people living with a Rare Disease - Public Health - European Commission. (2020 https://ec.europa.eu/health/ern/events/ev_20200609_en (Erişim 01.04.2021)
• 37. Roy NB, Telfer P, Eleftheriou P, de la Fuente J, Drasar E, Shah F, Lugthart S. Protecting vulnerable patients with inherited anaemias from unnecessary death during the COVID‐19 pandemic. British Journal of Haematology 2020;189(4): 635-639.
• 38. EURORDIS. Rare disease community raises alert over discrimination in critical care guidelines during COVID-19 pandemic. 2020. http://download2.eurordis.org/pressreleases/EURORDISstatement_COVID19Triage.pdf (Erişim 01.04.2021)
• 39. EURORDIS. Call to action for policy makers: How to protect people living with a rare disease as confinement measures are being lifted. 2020. https://download2.eurordis.org/pressreleases/EURORDISstatement_How-to-protect-people-with-rare-disease.pdf (Erişim 01.04.2021)
• 40. Annemans L, Aymé S, Le Cam Y, Facey K, Gunther P, Nicod E, Tomino C. Recommendations from the European Working Group for value assessment and funding processes in rare diseases (ORPH-VAL). Orphanet journal of rare diseases 2017; 12(1): 50.
• 41. Rare Barometer Survey. 2020. from https://www.eurordis.org/rare-barometer-programme (Erişim 01.04.2021)
• 42. Rare Disease Hong Kong. COVID-19 Worsens Health Condition and Financial Status of Rare Disease Patients in Hong Kong Group Calls on Government to Respond Efficiently and Effectively. 2020. https://www.rarediseasesinternational.org/wp-content/uploads/2020/07/RDHK-COVID-19-Survey-Press-Release-ENG-Final.pdf (Erişim 01.04.2021)
• 43. Triantafyllidi I, Boretti S, Goretti J. The Impact of COVID-19 on Rare Diseases: A Social & Digital Media Analysis. 2020. https://www.kantar.com/-/media/project/kantar/global/articles/files/2020/impact-of-covid-19-on-rare-diseases.pdf (Erişim 01.04.2021)
• 44. NORD. The impact of COVID-19 on rare dısease clınıcal trials. 2020. https://rarediseases.org/wp-content/uploads/2020/07/NRD-2043-CV19_RareEDU_Impact-on-Clincal-Trials_v1.pdf (Erişim 01.04.2021)
• 45. Brunetti-Pierri N, Fecarotta S, Staiano A, Strisciuglio P, Parenti G. Ensuring continuity of care for children with inherited metabolic diseases at the time of COVID-19: the experience of a metabolic unit in Italy. Genetics in Medicine 2020: 1-3.
• 46. Güney Afrika Nadir Hastalıklar Derneği. African Alliance for Rare Diseases. 2020. https://www.rarediseases.co.za/covid19 (Erişim 01.04.2021)
• 47. Peteet JR. COVID-19 anxiety. Journal of Religion and Health 2020; 59: 2203-2204.
• 48. Sher L. COVID-19, anxiety, sleep disturbances and suicide. Sleep medicine 2020; 70: 124.
• 49. Wang R, Pan Z, Wan C. Immediate psychological responses and associated factors during the initial stage of the 2019 coronavirus disease (COVID-19) epidemic among the general population in China, Int. J. Environ. Res. Public Health 2020; 17:E1729
• 50. Fortuné C, Gietzen A, Guillot G, Lewis N, Nielsen K, Richard M, Gottesman K. Protocol for a partially nested randomised controlled trial to evaluate the effectiveness of the scleroderma patient-centered intervention network COVID-19 home-isolation activities together (SPIN-CHAT) program to reduce anxiety among at-risk scleroderma patients. Journal of Psychosomatic Research 2020: 110132.f