Hemoglobin disorders in Europe: a systematic effort of identifying and addressing unmet needs and challenges by the Thalassemia International Federation
Hemoglobin disorders (thalassemia and sickle cell disease) are a group of hereditary anemias that today occur across the world. The recent population movement has led to a steady increase of carriers and patients in all countries of the European Union. Requiring complex monitoring and treatment and, as a consequence, well-organized and nationally coordinated, supported and funded services, these lifelong conditions are now visible to healthcare services in the EU. The purpose of this study is to provide an overview of the current situation pertaining to these disorders, as perceived by the patient/parent community that the Thalassemia International Federation (TIF) represents. The aim is to establish a comprehensive understanding of the situation and unmet needs faced by migrants with thalassemia. The implementation of activities by TIF in 2018-2020 to identify and address these challenges, paves the way to increased awareness, education and policy changes building on international expertise and knowledge that will enable the provision of state-of-art clinical management services thus guaranteeing an improved quality of life. A bird’s eye view of the prevalence of these disorders is presented contributing to the further understanding of challenges met by both patients and healthcare professionals in the receipt and provision of quality healthcare respectively.
Piel FB, Patil AP, Howes RE, et al. Global distribution of the sickle cell gene and geographical confirmation of the malaria hypothesis. Nat Commun 2010;1:104.
Plato CC, Rucknagel DL, Gershowitz H. Studies on the distribution of glucose-6-phosphate dehydrogenase deficiency, thalassemia, and other genetic traits in the coastal and mountain villages of Cyprus. Am J Hum Genet 1964;16:267-83.
Modell B, Darlison M, Birgens H, et al. Epidemiology of hemoglobin disorders in Europe: an overview. Scand J Clin Lab Invest 2007;67:39-69.
Aguilar Martinez P, Angastiniotis M, Eleftheriou A, et al. Haemoglobinopathies in Europe: health & migration policy perspectives. Orphanet J Rare Dis 2014;9:97.
Grosse R, Lukacs Z, Cobos PN, et al. The prevalence of sickle cell disease and its implication for newborn screening in Germany (Hamburg Metropolitan Area). Pediatr Blood Cancer 2016;63:168-70.
Hunter P. The refugee crisis challenges national health care systems: Countries accepting large numbers of refugees are struggling to meet their health care needs, which range from infectious to chronic diseases to mental illnesses. EMBO Rep 2016;17:492-5.
International Organization for Migration (IOM). Migration data - Europe. Available from: https://migration.iom.int/europe?type=arrivals
Thuret I, Pondarré C, Loundou A, et al. Complications and treatment of patients with β-thalassemia in France: results of the National Registry. Haematologica 2010;95:724-9.
Agouti I, Thuret I, Bernit E, et al. Data from the French registry for beta-thalassemia patients. EHA Learning Center. Badens C 2019;266587.
Global Blood Therapeutics. Overview of the sickle cell disease environment in select European countries; October 2020. Available from: https://ir.gbt.com/static-files/29a0775c-ed27-4778-8c10-90dbb9ae1f09
Kunz JB, Lobitz S, Grosse R, et al.; German Sickle Cell Disease Registry. Sickle cell disease in Germany: Results from a national registry. Pediatr Blood Cancer 2020;67:e28130.
Cela E, Bellón JM, de la Cruz M, et al.; SEHOP-Hemoglobinopathies Study Group (Sociedad Española de Hematología y Oncología Pediátricas). National registry of hemoglobinopathies in Spain (REPHem). Pediatr Blood Cancer 2017;64:26322.
Bardón Cancho EJ, García-Morín M, Beléndez C, et al. Update of the Spanish registry of haemoglobinopathies in children and adults. Med Clin (Barc) 2020;155:95-103.
National Health Service. NHS Haemoglobinopathy Annual report for 2018-9. Available from: https://nhr.mdsas.com/wp-content/uploads/2019/06/NHR_AnnualReport201819.pdf
Frontex. Migratory map - Europe. Available from: https://frontex.europa.eu/along-eu-borders/migratory-map/
Nelson EC, Dixon-Woods M, Batalden PB, et al. Patient focused registries can improve health, care, and science. BMJ. 2016;354:i3319.
Taher AT, Weatherall DJ, Cappellini MD. Thalassemia. Lancet 2018;391:155-67.
Cappellini MD, Cohen A, Porter J, et al., eds. Guidelines for the management of transfusion dependent thalassemia (TDT) [Internet]. 3rd ed. Nicosia (CY): Thalassemia International Federation; 2014.
Farmakis D, Giakoumis A, Angastiniotis M, Eleftheriou A. The changing epidemiology of the ageing thalassemia populations: A position statement of the Thalassemia International Federation. Eur J Haematol 2020;105:16-23.
Aymé S, Rodwell C. The European Union Committee of Experts on Rare Diseases: three productive years at the service of the rare disease community. Orphanet J Rare Dis 2014;9:30.
EuroBloodNet. Available from: https://eurobloodnet.eu/
Modell B, Khan M, Darlison M, et al. Improved survival of thalassemia major in the UK and relation to T2* cardiovascular magnetic resonance. J Cardiovasc Magn Reson 2008;10:42.
Noronha SA, Sadreameli SC, Strouse JJ. Management of sickle cell disease in children. South Med J 2016;109:495-502.
Powell RE, Lovett PB, Crawford A, et al. A multidisciplinary approach to impact acute care utilization in sickle cell disease. Am J Med Qual 2018;33:127-31.
European Centre for Disease Prevention and Control (ECDC). Assessing the burden of key infectious diseases affecting migrant populations in the EU/EEA. Stockholm: ECDC; 2014.
Baldovino S, Moliner AM, Taruscio D, et al. Rare diseases in Europe: from a wide to a local perspective. Isr Med Assoc J 2016;18:359-63.
Moliner AM, Waligora J. The European Union Policy in the field of rare diseases. Adv Exp Med Biol 2017;1031:561-87.
Iolascon A, De Franceschi L, Muckenthaler M, et al. EHA research roadmap on hemoglobinopathies and thalassemia: an update. Hemasphere 2019;3:e208.
Weil LG, Charlton MR, Coppinger C, et al. Sickle cell disease and thalassemia antenatal screening programme in England over 10 years: a review from 2007/2008 to 2016/2017. J Clin Pathol 2020;73:183-90.
Longo F, Corrieri P, Origa R, et al. Changing patterns of thalassemia in Italy: a WebThal perspective. Blood Transfus 2020. [Epup ahead of print].
- Abstract views: 319
- PDF: 94
Copyright (c) 2021 the Author(s)
This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.