Review Articles
Vol. 18 No. 1 (2026): Mediterranean Journal of Hematology and Infectious Diseases
END-OF-LIFE CARE IN SICKLE CELL DISEASE AND TRASFUSION DEPENDEND-β-THALASSEMIA: CLINICAL, PSYCHOLOGICAL, AND ETHICAL CONSIDERATION
Challenges in Supportive Care, Equity, and Compassion at End of Life
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All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article or claim that may be made by its manufacturer is not guaranteed or endorsed by the publisher.
Received: August 17, 2025
Accepted: December 21, 2025
Accepted: December 21, 2025
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Sickle cell disease (SCD) and transfusion-dependent β-thalassemia have transitioned from being mostly pediatric conditions to complicated, chronic disorders characterized by advancing multi-organ complications. The offering of end-of-life care for these patients is still poorly developed and applied sporadically even in the face of apparent needs. This review examines EOL treatment for hemoglobinopathies, focusing on symptom management, ethical difficulties, and psychosocial-spiritual support. The study utilized an analytical narrative technique, analyzing peer-reviewed literature from 2025, international norms, and European policy frameworks. Findings: The final stages of both SCD and β-thalassemia are characterized by persistent pain, permanent organ impairment, and significant psychological distress. Regardless of the variations in disease progression, both necessitate prompt and cohesive palliative care measures. Disparities in access to symptom management—particularly within marginalized communities—continue to endure. Cultural influences often play a significant role in shaping decision-making, especially in ethically sensitive contexts like transfusion withdrawal or gene therapy for advanced diseases. The psychosocial and spiritual aspects of suffering become more pronounced as one approaches the end of life, highlighting the need for interdisciplinary teamwork and care that is sensitive to cultural differences. In conclusion, it is essential to provide multidisciplinary, anticipatory, and culturally competent end-of-life care in the context of hemoglobinopathies. Health systems ought to implement referral criteria tailored to specific diseases and encourage adaptable hospice models, ensuring access to transfusions when clinically indicated. Access to opioids, enrolment in hospice care, and psychosocial support must be prioritized, especially in areas with a high prevalence of hemoglobinopathy.
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References
Weatherall DJ. The challenge of haemoglobinopathies in resource-poor countries. Br J Haematol. 2021;193(5):845–854.
Angastiniotis M, Eleftheriou A, Aessopos A. Thalassaemia International Federation Guidelines for the Management of Transfusion Dependent Thalassaemia. TIF; 2021.
Makani, J., Cox, S. E., Soka, D., Komba, A. N., Oruo, J., Mwamtemi, H., ... & Newton, C. R. (2011). Mortality in sickle cell anemia in Africa: a prospective cohort study in Tanzania. PloS one, 6(2), e14699.
Nwogu-Onyemkpa E, Dongarwar D, Salihu HM. Inpatient palliative care utilization in sickle cell disease: temporal trends and disparities. BMJ Open. 2022;12(8):e057361.
Konstantis A, Paraskeva M, Avgoustidi M, et al. The implementation of palliative care in Greece: a position paper by the Palliative Care Working Group of the Hellenic society of Medical Oncology (HeSMO). Forum Clin Oncol. 2024. doi:10.2478/fco-2023-0038
Ballas SK, Kesen MR, Goldberg MF, Lutty GA, Dampier C, Osunkwo I, Wang WC, Hoppe C, Hagar W, Darbari DS, Malik P. Beyond the definitions of the phenotypic complications of sickle cell disease: an update on management. The Scientific World Journal. 2012;2012(1):949535
Piel FB, Steinberg MH, Rees DC. Sickle cell disease. New England Journal of Medicine. 2017 Apr 20;376(16):1561-73.
Yawn BP, Buchanan GR, Afenyi-Annan AN, Ballas SK, Hassell KL, James AH, Jordan L, Lanzkron SM, Lottenberg R, Savage WJ, Tanabe PJ. Management of sickle cell disease: summary of the 2014 evidence-based report by expert panel members. Jama. 2014 Sep 10;312(10):1033-48.
Lanzkron S, Carroll CP, Haywood C Jr. Mortality rates and age at death from sickle cell disease: U.S., 1979–2005. Public Health Rep. 2021;136(2):179–187.
Taher AT, Musallam KM, Cappellini MD. β-Thalassemias. New England Journal of Medicine. 2021 Feb 25;384(8):727-43.
Ganz T, Nemeth E. Pathogenic mechanisms in thalassemia II: iron overload. Hematology/Oncology Clinics. 2023 Apr 1;37(2):353-63.
Carsote M, Vasiliu C, Trandafir AI, Albu SE, Dumitrascu MC, Popa A, Mehedintu C, Petca RC, Petca A, Sandru F. New entity—thalassemic endocrine disease: major beta-thalassemia and endocrine involvement. Diagnostics. 2022 Aug 9;12(8):1921.
Chiew JY, Thiruchelvam J, Rahmat MB, William SP, Shafien ZB, Banerjee KG. The key complications of beta thalassemia major: a review and update. Int J Res Med Sci. 2021 Jun;9:1846-52.
Delicou S, Manganas K, Diamantidis MD, Venou TM, Delaporta P, Pantelidou D, Spachiou E, Tsagia S, Pappi V, Petropoulou F, Kapsali E. Comparative analysis of mortality patterns and treatment strategies in thalassaemia and sickle cell disease patients: A 12‐year study. British Journal of Haematology. 2025 May;206(5):1466-78.
Essien EA, Winter-Eteng BF, Onukogu CU, Nkangha DD, Daniel FM. Psychosocial challenges of persons with sickle cell anemia: A narrative review. Medicine. 2023 Nov 24;102(47):e36147.
Coco M, Henderson WA, Park CL, Starkweather AR. Growing beyond sickle cell disease: A metasynthesis of children, adolescents, and young adult experiences living with sickle cell disease. Research in Nursing & Health. 2023 Jun;46(3):299-312.
Treadwell MJ, Anie KA. Quality of life in sickle cell disease: what matters. Hematology/Oncology Clinics. 2022 Dec 1;36(6):1137-49.
Forni GL, Grazzini G, Boudreaux J, Agostini V, Omert L. Global burden and unmet needs in the treatment of transfusion-dependent β-thalassemia. Frontiers in Hematology. 2023 Jun 20;2:1187681.
Bizri M, Koleilat R, Akiki N, Dergham R, Mihailescu AM, Bou-Fakhredin R, Musallam KM, Taher AT. Quality of life, mood disorders, and cognitive impairment in adults with β-thalassemia. Blood Reviews. 2024 May 1;65:101181.
Papadopoulos NA. ‘Active Ageing’under International Law: The Localization of a Human Rights-Based Approach. Diritti umani e diritto internazionale. 2024(1):5-34.
Patel RV, Nwogu-Onyemkpa E, Kanter J, Osunkwo I. Palliative Care for Adults With Sickle Cell Disease: An Unrecognized Opportunity. The Hematologist. 2024 Sep 1;21(5).
22.Allen A, Kiser SB, Nwogu-Onyemkpa E. Palliative Care in Sickle Cell Disease: Development of an International Working Group. Journal of Pain and Symptom Management. 2024 May 1;67(5):e633.
23.Schatz AA, Oliver TK, Swarm RA, Paice JA, Darbari DS, Dowell D, Meghani SH, Winckworth-Prejsnar K, Bruera E, Plovnick RM, Richardson L. Bridging the gap among clinical practice guidelines for pain management in cancer and sickle cell disease. Journal of the National Comprehensive Cancer Network. 2020 Apr 1;18(4):392-9.
Ahmadi M, Rassouli M, Gheibizadeh M, Ebadi A, Asadizaker M. Experiences of Iranian Patients with Thalassemia Major Regarding Their Palliative and Supportive Care Needs: A Qualitative Content Analysis. International Journal of Community Based Nursing & Midwifery. 2025 Jan 15.
Erondu MU, Boateng S. Equity in Palliative Care: Interviews with Leaders of a Sickle Cell Community-Facing Organization. Journal of Pain and Symptom Management. 2024 May 1;67(5):e644-5.
Anie KA. The intersection of sickle cell disease, stigma, and pain in Africa. Hematology. 2024 Dec 6;2024(1):240-5.
Berghs M, Ebenso B, Ola B. Social Determinants of Severity in Sickle Cell Disease. InSickle Cell Disease in Sub-Saharan Africa 2024 Apr 30 (pp. 17-30). Routledge.
van Hooff LC, Merz EM, Kidane Gebremeskel AS, de Jong JA, Burchell GL, Lunshof JE. Balancing benefits and burdens: a systematic review on ethical and social dimensions of gene and cell therapies for hereditary blood diseases. BMC medical ethics. 2025 Mar 14;26(1):36.
Wright KY. Sickle Cell Specific Holistic Management Framework Design (Doctoral dissertation, Capella University).
Darby JE, Akpotu IC, Wi D, Ahmed S, Doorenbos AZ, Lofton S. A scoping review of social determinants of health and pain outcomes in sickle cell disease. Pain Management Nursing. 2025 Feb 1;26(1):e1-9.
Cáceres-Titos MJ, Porras-Santana JM, Cabillas-Romero MR, Begoña García-Navarro EE. Managing cultural diversity in end-of-life care: a qualitative study. BMC palliative care. 2025 May 4;24(1):124.
Asamoah EH. Perceptions and Experiences of Sickle Cell Disease Patients and Parents/Caretakers on Alternative Treatment Options for Pain Management (Doctoral dissertation, Walden University).
Benjamin L. Pain management in sickle cell disease: palliative care begins at birth?. ASH Education Program Book. 2008 Jan 1;2008(1):466-74.
Kayastha N, LeBlanc TW. Palliative care for patients with hematologic malignancies: are we meeting patients’ needs early enough?. Expert Review of Hematology. 2022 Sep 2;15(9):813-20.
Shaw, PA A. Hospice Criteria: Determining That Time Is Limited. InThe Arc of Conversation: A How-to Guide for Goals of Care Conversations 2025 Feb 14 (pp. 91-157). Cham: Springer Nature Switzerland.
Reyes J, Lin J. Bridging Pediatric and Adult Palliative Care to Amplify Interdisciplinary Home Hospice Support. Journal of Pain and Symptom Management. 2025 May 1;69(5):e585-6.
Thomas K, Russell S. Advance Care Planning in the United Kingdom–A snapshot from the four UK nations. Zeitschrift für Evidenz, Fortbildung und Qualität im Gesundheitswesen. 2023 Aug 1;180:150-62.
Office for National Statistics (UK). Deaths in hospice: data from 2022. [Accessed July 2025].
Eleftheriou A, Forni GL, Sayani F. Multidisciplinary Care and Reference Centres in addressing haemoglobin disorders. Guidelines for the Management of Transfusion-Dependent β-Thalassaemia (TDT)[Internet]. 5th edition. 2025.
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“END-OF-LIFE CARE IN SICKLE CELL DISEASE AND TRASFUSION DEPENDEND-β-THALASSEMIA: CLINICAL, PSYCHOLOGICAL, AND ETHICAL CONSIDERATION: Challenges in Supportive Care, Equity, and Compassion at End of Life” (2026) Mediterranean Journal of Hematology and Infectious Diseases, 18(1), p. e2026015. doi:10.4084/MJHID.2026.015.
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