Dexter, D

Kapata, N

Kiyaga, C

Kock, R

Ntoumi, F

Simons, D

Zumla, A

English

UCL Discovery

  Since January 2020 Elsevier has created a COVID-19 resource centre with free information in English and Mandarin on the novel coronavirus COVID-19. The COVID-19 resource centre is hosted on Elsevier Connect, the company's public news and information website.  Elsevier hereby grants permission to make all its COVID-19-related research that is available on the COVID-19 resource centre - including this research content - immediately available in PubMed Central and other publicly funded repositories, such as the WHO COVID database with rights for unrestricted research re-use and analyses in any form or by any means with acknowledgement of the original source. These permissions are granted for free by Elsevier for as long as the COVID-19 resource centre remains active.  Commente430 www.thelancet.com/haematology   Vol 7   June 2020deferiprone discontinued therapy more often because of side effects than patients on deferasirox raises the concern that deferiprone and deferasirox are not equivalent from a patient perspective and additional long-term follow up is needed. Effects of iron chelation therapy on hepatic iron concentrations and in patients with sickle cell disease were also not adequately evaluated, and merit further consideration in future trials.An important finding is the high baseline iron burden (serum ferritin concentration ≥2500 ng/mL) in 38% of patients, despite the well established mortality benefit and cost-effectiveness of iron chelation therapy and clear guideline recommendations supporting its use.1,8 Because iron-associated cardiac and liver pathology lead to premature mortality and inferior outcomes with allogeneic haematopoietic stem-cell transplantation,6,8 this trial reinforces the need and potential to optimise access to and use of iron chelation therapy in paediatric patients with transfusion-dependent haemo globino-pathies. On the basis of results from DEEP-2 and the different adverse event profiles of the two drugs, substituting deferasirox for deferiprone could be effective to maintain patients on iron chelation therapy if tolerance issues arise, without sacrificing efficacy. This possibility also ensures that an increased number of children have access to effective iron chelation therapy, especially considering that available novel therapies are primarily being developed in adults.Although not reported by Maggio and colleagues,7 the effect of deferiprone and deferasirox on quality of life is of great interest because little is known about this issue in paediatric patients, and adherence to long-term medications fundamentally relies on a patient’s perception of improvements in quality of life.ATT has received consultancy fees from Novartis, Celgene, Vifor, Protagonist Therapeutics, and IONIS; and has received research funding from Novartis, Celgene, and Protagonist Therapeutics, outside of the submitted work. AMZ has received research funding (institutional) from Celgene/Bristol-Myers Squibb, AbbVie, Astex, Pfizer, Medimmune/AstraZeneca, Boehringer-Ingelheim, Trovagene, Incyte, Takeda, Novartis, Aprea, and ADC Therapeutics; has participated in advisory boards or had a consultancy with and received honoraria from AbbVie, Otsuka, Pfizer, Celgene/Bristol-Myers Squibb, Jazz, Incyte, Agios, Boehringer-Ingelheim, Novartis, Acceleron, Astellas, Daiichi Sankyo, Cardinal Health, Taiho, Seattle Genetics, BeyondSpring, Trovagene, Takeda, Ionis, Amgen, and Epizyme; has served on steering and independent data review committees for clinical trials for Novartis and Janssen; has received travel support for meetings from Pfizer, Novartis, and Trovagene; is a Leukemia and Lymphoma Society scholar in clinical research; and is supported by National Cancer Institute’s Cancer Clinical Investigator Team Leadership Award, all outside of the submitted work. JPB declares no competing interests. All authors wrote and critically reviewed all drafts of the manuscript. This work was partly supported by the National Cancer Institute of the National Institutes of Health under award number P30 CA016359. The content is solely the responsibility of the authors and does not necessarily represent the official views of the National Institutes of Health.Jan Philipp Bewersdorf, Ali T Taher, *Amer M Zeidanamer.zeidan@yale.eduSection of Hematology, Department of Internal Medicine, Yale University School of Medicine, New Haven, CT 06520-8028, USA (JPB, AMZ) and Department of Internal Medicine, American University of Beirut Medical Center, Beirut, Lebanon (ATT)1 Shah FT, Sayani F, Trompeter S, Drasar E, Piga A. Challenges of blood transfusions in β-thalassemia. Blood Rev 2019; 37: 100588.2 GBD 2017 Disease and Injury Incidence and Prevalence Collaborators. Global, regional, and national incidence, prevalence, and years lived with disability for 354 diseases and injuries for 195 countries and territories, 1990–2017: a systematic analysis for the Global Burden of Disease Study 2017. Lancet 2018; 392: 1789–858.3 Di Maggio R, Maggio A. The new era of chelation treatments: effectiveness and safety of 10 different regimens for controlling iron overloading in thalassaemia major. Br J Haematol 2017; 178: 676–88.4 Pennell DJ, Berdoukas V, Karagiorga M, et al. Randomized controlled trial of deferiprone or deferoxamine in beta-thalassemia major patients with asymptomatic myocardial siderosis. Blood 2006; 107: 3738–44.5 Pennell DJ, Porter JB, Piga A, et al. A 1-year randomized controlled trial of deferasirox vs deferoxamine for myocardial iron removal in β-thalassemia major (CORDELIA). Blood 2014; 123: 1447–54.6 Ladis V, Chouliaras G, Berdoukas V, et al. Survival in a large cohort of Greek patients with transfusion-dependent beta thalassaemia and mortality ratios compared to the general population. Eur J Haematol 2011; 86: 332–38.7 Maggio A, Kattamis A, Felisi M, et al. Evaluation of the efficacy and safety of deferiprone compared with deferasirox in paediatric patients with transfusion-dependent haemoglobinopathies (DEEP-2): a multicentre, randomised, open label, non-inferiority, phase 3 trial. Lancet Haematol 2020; 7: e469–78.8 Cappellini MD, Porter JB, Viprakasit V, Taher AT. A paradigm shift on beta-thalassaemia treatment: how will we manage this old disease with new therapies? Blood Rev 2018; 32: 300–11.Mitigating the effect of the COVID-19 pandemic on sickle cell disease services in African countriesAn estimated 5% of the world’s population are carriers for trait genes for haemoglobin disorders, mainly sickle cell disease. Over 75% of the global burden of sickle cell anaemia occurs in sub-Saharan Africa, and sickle cell disease is the most prevalent inherited genetic disease with 10–45% of the population carrying the sickle cell gene. Advances in sickle cell disease management, including screening of newborn babies, pneumococcal prophylaxis, and hydroxyurea therapy have transformed sickle cell disease from a fatal childhood disease to a Published Online April 23, 2020 https://doi.org/10.1016/ S2352-3026(20)30122-8Commentwww.thelancet.com/haematology   Vol 7   June 2020 e431chronic condition of adulthood. Sickle cell disease remains a neglected tropical disease in Africa, characterised by a scarcity of specialist services and resultant high mortality for children younger than 5 years.1 Furthermore, few African countries have epidemiological data on disease burden, which poses an additional barrier to effective health systems planning. This situation is compounded by the additional burden of the coronavirus disease 2019 (COVID-19) pandemic on existing resources.As of April 15, 2020, 1 914 916 cases of COVID-19 have been confirmed and 123 010 people have died globally.2 Increasingly, cases on the African continent are being observed; as of April 14, 2020, 10 759 COVID-19 cases have been confirmed in 45 countries with 520 deaths reported.3 Many people are concerned about the burden that the COVID-19 pandemic will place on health-care systems generally and on specialist services particularly, including those for sickle cell disease.Infection with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) has shown different clinical courses across age groups.4 Modelling of fatality in the early course of the outbreak from a preprint study showed case fatality ratios (CFR) increasing from 1·3% for people aged 50–59 years to 9·8% in those aged 70–79 years.5 The increased CFR with older age has been attributed to increased comorbidities in these age groups, including pre-existing immunosuppression, diabetes, HIV, chronic obstructive pulmonary disease, and cardiovascular, liver, and renal conditions.That a very different epidemic pattern for COVID-19 will be seen in Africa is possible. The demographics of many sub-Saharan countries are heavily skewed towards younger populations, suggesting potential for a milder disease course than countries with a high proportion of older age groups; however, this might not always be the case, especially for countries with high burdens of pre-existing health conditions such as sickle cell disease. The ability of these often fragile health-care systems to manage COVID-19 has been discussed.6 However, that many people younger than 50 years on the African continent live with potentially important comorbidities (eg, HIV, hypertension, and sickle cell disease), has not been discussed.7Acute respiratory illnesses are a major cause of morbidity and mortality in patients with sickle cell disease who have an increased risk of asthma, pneumonia, and pulmonary vaso-occlusive disease, including acute chest syndrome. COVID-19 pathology is associated with reduced cellular oxygenation, pneumonia, and acute respiratory distress syndrome in severe cases. This pathological process is closely linked to an increased risk of vaso-occlusive complications in sickle cell disease, including acute chest syndrome. Specific diagnostic, treatment, and logistical challenges exist in meeting the health-care needs of people with sickle cell disease during the COVID-19 pandemic.The influenza A H1N1 pandemic in 2009 highlighted increased susceptibility of children with sickle cell disease to severe complications of a respiratory virus, with an up to 50% increase in hospitalisation for this patient population.8 Vaccination of people with sickle cell disease for influenza is a key intervention to prevent severe morbidity.9 For SARS-CoV-2, people do not have previous immunity, and the development of a vaccine is many months away. An outbreak of COVID-19 in regions with a high prevalence of sickle cell disease will rapidly overwhelm existing resource-limited health-care services. These health-care systems should anticipate and prepare for a steep increase in young patients requiring high-intensity supportive care.The risk of sickle cell disease complications and associated mortality can be mitigated through behav-ioural and pharmacological interventions. High-risk populations, including people with sickle cell disease, must be considered in the development of local preventative and health-care strategies. A crucial first step is to identify people who are at risk through screening. Uganda has shown that identifying people with sickle cell disease can be done with targeted screening;10 however, these programmes need widespread adoption to identify the true numbers of people with sickle cell disease across the continent. Diagnostic and treatment resources should be rapidly scaled up in conjunction with investment in public health campaigns targeted to the at-risk sickle cell disease population. When an effective and affordable SARS-CoV-2 vaccine becomes available, people with sickle cell disease should be prioritised for immunisation because of their increased risk of mortality. This immunisation should be aligned with routine pneumococcal vaccination, which has proven to be effective.The current focus on the COVID-19 pandemic in Africa and resource mobilisation provides unique opportunities for optimising health services for people with sickle cell disease in Africa and improving surveillance. The overlap Arno Massee/Science Photo LibraryCommente432 www.thelancet.com/haematology   Vol 7   June 2020in the epidemiology and clinical association of sickle cell disease with malaria, and bacterial and viral infections (including SARS-CoV-2), suggests that sickle cell disease should be included in the Integrated Management of Childhood Illness programme to improve outcomes. Provision for sickle cell disease diagnosis and treatment should be incorporated into national health systems programming, with an emphasis on delivering sickle cell disease services in the primary care setting. COVID-19 is expected to herald a global economic recession that might result in a contraction of international funding for health systems development in Africa.The COVID-19 pandemic is currently overwhelming health systems in high-income countries and so could have an increased effect in low-resource settings in Africa where health services are already overstretched. The effect of COVID-19 on the global economy could cause recession both in Africa and around the world, posing a substantial threat to the delivery of health care in Africa. Every effort should be made to invest in primary health care, and integrate and align sickle cell disease diagnosis and treatment into existing health systems, rather than building new vertical programmes focusing only on sickle cell disease with interventions delivered separately from other health services. African governments should leverage further funding resources to accomplish this aim. The preparation and mitigation stages of the pandemic also represent important opportunities to focus international efforts at rapidly scaling up sickle cell disease-related health-care infrastructure.AZ, FN, RK, DS, and NK are members of the Pan-African Network on Emerging and Re-Emerging Infections funded by the European and Developing Countries Clinical Trials Partnership the EU Horizon 2020 Framework Programme for Research and Innovation. AZ is in receipt of a National Institutes of Health Research senior investigator award. We declare no competing interests. All authors contributed equally.For more on the Pan-African Network on Emerging and Re-Emerging Infections see https://www.pandora-id.net/Daniel Dexter, *David Simons, Charles Kiyaga, Nathan Kapata, Francine Ntoumi, Richard Kock, Alimuddin Zumladsimons19@rvc.ac.ukGreat Ormond Street Hospital for Children, London, UK (DD); The Royal Veterinary College, University of London, Hatfield, Hertfordshire, AL9 7TA, UK (DS, RK); Ministry of Health, Kampala, Uganda (CK); University Marien Ngouabi, Brazzaville, Congo (FN); Zambia National Public Health Institute, Minsitry of Health, Lusaka, Zambia (NK); Center for Clinical Microbiology, Division of Infection and Immunity, Royal Free Hospital Campus, University College London, London, United Kingdom (AZ); and Division of Infection and Immunity, University College London and National Institute for Health Research Biomedical Research Centre, University College London Hospitals National Health Service Foundation Trust, London, United Kingdom (AZ)1 Ware RE. Is sickle cell anemia a neglected tropical disease? PLoS Negl Trop Dis 2013; 7: e2120.2 WHO. Coronavirus disease 2019 (COVID-19) situation report–86. April 15, 2020. https://www.who.int/docs/default-source/coronaviruse/situation-reports/20200415-sitrep-86-covid-19.pdf?sfvrsn=c615ea20_6 (accessed April 16, 2020).3 WHO. COVID-19 situation update for the WHO African Region—Sitrep 07 15 April 2020. April 15, 2020. https://www.afro.who.int/publications/situation-reports-covid-19-outbreak-sitrep-07-15-april-2020 (accessed April 17, 2020).4 Wu Z, McGoogan JM. Characteristics of and important lessons from the coronavirus disease 2019 (COVID-19) outbreak in China: summary of a report of 72 314 cases from the Chinese Center for Disease Control and Prevention. JAMA 2020; published online Feb 24. DOI:10.1001/jama.2020.2648.5 Riou J, Hauser A, Counotte MJ, Althaus CL. Adjusted age-specific case fatality ratio during the COVID-19 epidemic in Hubei, China, January and February 2020. medRxiv 2020; published online March 30. DOI:2020.03.04.20031104 (preprint).6 Kapata N, Ihekweazu C, Ntoumi F, et al. Is Africa prepared for tackling the COVID-19 (SARS-CoV-2) epidemic. Lessons from past outbreaks, ongoing pan-African public health efforts, and implications for the future. Int J Infect Dis 2020; 93: 233–36.7 Stanaway JD, Afshin A, Gakidou E, et al. Global, regional, and national comparative risk assessment of 84 behavioural, environmental and occupational, and metabolic risks or clusters of risks for 195 countries and territories, 1990–2017: a systematic analysis for the Global Burden of Disease Study 2017. Lancet 2018; 392: 1923–94.8 Inusa B, Zuckerman M, Gadong N, et al. Pandemic influenza a (H1N1) virus infections in children with sickle cell disease. Blood 2010; 115: 2329–30.9 Strouse JJ, Reller ME, Bundy DG, et al. Severe pandemic H1N1 and seasonal influenza in children and young adults with sickle cell disease. Blood 2010; 116: 3431–34.10 Kiyaga C, Hernandez AG, Ssewanyana I, et al. Sickle cell screening in Uganda: high burden, human immunodeficiency virus comorbidity, and genetic modifiers. Pediatr Blood Cancer 2019; 66: e27807.Effect of the COVID-19 pandemic on cancer treatment and researchThe coronavirus disease 2019 (COVID-19) outbreak, caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), has rapidly escalated into a global pandemic. Patients with haematological and other cancers,1,2 and recipients of haematopoietic stem cell transplantation (HSCT), could be at particular risk from COVID-19, since they tend to be older, have multiple comorbidities, and are often immunosuppressed by their disease or therapy. A retrospective analysis of 355 patients who died of COVID-19 in Italy showed that 20% had active cancer,3 and a study from 2013–17 of 678 patients who had an HSCT found that 112 (17%) developed human coronavirus infection, of whom 34 had lower respiratory Published Online April 24, 2020 https://doi.org/10.1016/ S2352-3026(20)30123-X

Mitigating the effect of the COVID-19 pandemic on sickle cell disease services in African countries

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Mitigating the effect of the COVID-19 pandemic on sickle cell disease services in African countries

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