Thalassemia and its management – A review article

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Year : 2024 | Volume : | : | Page : –
By
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Neha Kansal,

  1. Nursing Tutor, College of Nursing, Government Medical College and Hospital, Sector 32, Chandigarh, India

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Thalassemia is a hemoglobinopathy burdening India making it earn title of Thalassemia capital of world. Studying last decade, the overall prevalence worldwide and incidence rates have declined but prevalence rates were high in east and Southeast Asian countries. Screening for carriers and affected individuals and genetic counselling are promising approaches for reducing soaring cases and reducing the further spread. Thalassemia affects various organs due to iron overload and haemolysis leading to cascade of complications ranging from physical deformities to fatal outcomes like heart failure. Continuous monitoring of is requires with multidisciplinary approach to handle the adverse reactions of treatment modalities and deal with complications. The treatment of Thalassemia ranges from conventional blood transfusions and chelation therapy to curative i.e. bone marrow transplant and gene editing and newer drugs targeting ineffective erythropoiesis and production of foetal haemoglobin. Chronic nature of the disease affecting quality of life of children makes nurses teaching them self-care management techniques more essential. Role of nursing strategies for managing thalassaemic children is through holistic approach which is addressing their developmental needs as per their age. Various governmental and NGO initiatives and programmes have made a significant impact but multisectoral approach is needed to tackle the emerging cases.

Keywords: Thalassemia, Self-care, Quality of life, Genetic Screening, Bone Marrow Transplant.

How to cite this article:
Neha Kansal. Thalassemia and its management – A review article. International Journal of Tropical Medicines. 2024; ():-.
How to cite this URL:
Neha Kansal. Thalassemia and its management – A review article. International Journal of Tropical Medicines. 2024; ():-. Available from: https://journals.stmjournals.com/ijtm/article=2024/view=0

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References
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  1. National Health Mission (NHM), Punjab. Guidelines. Available from: https://nhm.punjab.gov.in
  2. National Center for Biotechnology Information (US). Information (US) NC for B. Thalassemia. Available from: https://www.ncbi.nlm.nih.gov
  3. Tuo Y, Li Y, Li Y, Ma J, Yang X, Wu S, et al. Global, regional, and national burden of thalassemia, 1990–2021: a systematic analysis for the global burden of disease study 2021. EClinicalMedicine. 2024;72:102619.
  4. Desk ITN, News IT. Latest study states why India is the thalassemia capital of world. Available from: https://www.indiatvnews.com
  5. Singh P, Shaikh S, Parmar S, Gupta R. Current status of β-thalassemic burden in India. Hemoglobin. 2023 Sep 3;47(5):181–90.
  6. Ghai OP, Gupta P, Paul VK. Ghai’s Essential Pediatrics. 2008;341–44.
  7. Marlow DR, Redding BA. Textbook of Pediatric Nursing. 6th ed. Philadelphia: Saunders; 1998. p. 530–33.
  8. Hockenberry MJ. Wong’s Nursing Care of Infants and Children. Elsevier; 2023. p. 1529–34.
  9. Textbook of Pediatrics. 19th ed. 2nd vol. Elsevier Health Science; 2013. p. 1674–77.
  10. Bhattacharya S, Thiyagarajan A, Sharma N, Srivastava A, Dhar D. Need for a universal thalassemia screening programme in India? A public health perspective. J Fam Med Prim Care. 2019;8(5):1528.
  11. Bhavsar DK. What is the purpose of genetic counseling for thalassemia prevention? Available from: https://www.icliniq.com
  12. Petrou M. Genetic counselling. Thalassaemia International Federation. Available from: https://www.ncbi.nlm.nih.gov
  13. Barr JA, Tsai LP, Welch A, Faradz SMH, Lane-Krebs K, Howie V, et al. Current practice for genetic counselling by nurses: an integrative review. Int J Nurs Pract. 2018 Feb 20;24(2)

 

  1. Thakur S, Singh S. Genetic counseling to prevent thalassemia and hemoglobinopathy in the Indian population.
  2. Thalassemia: Treatment cost in India.
  3. Sudhakar A. Essentials of Pediatric Nursing. 2017;1164–87.
  4. Hoffbrand AV, Moss PAH. Hoffbrand’s Essential Haematology. 6th ed. Chichester: John Wiley & Sons; 2008. p. 97–107.
  5. Vichinsky EP. Clinical manifestations of β-thalassemia. Cold Spring Harb Perspect Med. 2013 Mar 29;3(5)

 

  1. Thalassemia – Treatment. NHLBI, NIH. Available from: https://www.nhlbi.nih.gov
  2. Tubman VN, Fung EB, Vogiatzi M, Thompson AA, Rogers ZR, Neufeld EJ, et al. Guidelines for the standard monitoring of patients with thalassemia. J Pediatr Hematol Oncol. 2015 Apr;37(3)

 

  1. Yaish HM. Pediatric thalassemia medication: Antipyretics, analgesics, antihistamines, chelating agents, corticosteroids, antibacterial combinations, vitamins, vaccines, antineoplastic agent, growth hormone, antiplatelet agents, hematologic, erythroid maturation agents, gene therapies, hematologics. Medscape. Available from: https://www.medscape.com
  2. Lokeshwar MR. Pediatriconcall.com. 2013.
  3. Cappellini MD, Cohen A, Eleftheriou A, Piga A, Porter J, Taher A. Psychological support in thalassemia. Thalassaemia International Federation. Available from: https://www.ncbi.nlm.nih.gov
  4. Galanello R, Origa R. Beta-thalassemia. Orphanet J Rare Dis. 2010 May 21;5(1).
  5. Joyce MB, et al. A Textbook of Medical Surgical Nursing Management for Positive Outcomes. 8th ed. 2nd vol. Philadelphia: Saunders Elsevier; 2009. p. 2018–19.
  6. Firkin F, Chesterman C, Rush B, Pennigton D. De Gruchy’s Clinical Haematology in Medical Practice. 6th ed. Chichester: John Wiley & Sons; 2013. p. 134–42.
  7. Bajwa H, Basit H. Thalassemia. StatPearls Publishing; 2023. Available from: https://www.ncbi.nlm.nih.gov
  8. Nienhuis AW, Nathan DG. Pathophysiology and clinical manifestations of β-thalassemias. Cold Spring Harb Perspect Med. 2012 Dec 1;2(12)

 

  1. Khan A, Rehman AU. Laboratory evaluation of beta thalassemia. StatPearls Publishing; 2023.
  2. Children with chronic health conditions – Pediatrics. MSD Manual Professional Edition. Available from: https://www.msdmanuals.com
  3. Mazzone L, Battaglia L, Andreozzi F, Romeo M, Mazzone D. Emotional impact in β-thalassemia major children following cognitive-behavioral family therapy and QoL of caregiving mothers. Clin Pract Epidemiol Ment Health. 2009;5:5.
  4. Early childhood development. Available from: https://www.unicef.org
  5. Dubois A, Hall C, Courtois-Communier E, Brasseur A, Cacioppo M, Brochard S. Incidence and risk factors for care-related pain in children with physical disabilities. Eur J Phys Rehabil Med. 2023 Jun 1;59(3).
  6. Shah D, Choudhury P, Dubey AP. Current trends in management of the beta thalassemias. Indian Pediatr. 1999 Dec 1;36(12):1229–42.
  7. Saliba AN, Atoui A, Labban M, Hamade H, Bou-Fakhredin R, Mufarrij A, et al. Thalassemia in the emergency department: special considerations for a rare disease. Ann Hematol. 2020 Jul 3;99(9):1967–77.
  8. Borgna-Pignatti C, Gamberini MR. Complications of thalassemia major and their treatment. Expert Rev Hematol. 2011;4(3):353–66.
  9. Salem A, Desai P, Elgebaly A. Efficacy and safety of combined deferiprone and deferasirox in iron-overloaded patients: a systematic review. Cureus. 2023 Nov 4;15(11).
  10. Wood JC. Use of magnetic resonance imaging to monitor iron overload. Hematol Oncol Clin North Am. 2014 Aug 1;28(4):747–64.
  11. El-Beshlawy A, El-Ghamrawy M. Recent trends in treatment of thalassemia. Blood Cells Mol Dis. 2019 May;76:53–8.
  12. Office E. Beta thalassemia: new developments in treatment. The Waiting Room. 2023.
  13. Musallam KM, Taher AT, Cappellini MD, Sankaran VG. Clinical experience with fetal hemoglobin induction therapy in patients with β-thalassemia. Blood. 2013 Mar 21;121(12):2199–212.
  14. Amer J, Dana M, Fibach E. The antioxidant effect of erythropoietin on thalassemic blood cells. Anemia. 2010;2010:1–11.
  15. Mutha AS, Shah KU, Kinikar AA, Ghongane BB. Efficacy and safety of wheat grass in thalassemic children on regular blood transfusion. Cureus. 2018 Mar 11.
  16. Sears R, Carpenter R, Whitlock C. A general synthesis of tris-indole derivatives as potential iron chelators. Molecules. 2005 Feb 28;10(2):488–91.
  17. Karponi G, Zogas N. Gene therapy for beta-thalassemia: updated perspectives. The Application of Clinical Genetics. 2019 Sep;12:167–80.
  18. Kavitha K, Padmaja A. Strategies for enhancing quality of life in thalassemic children. BLDE Univ J Health Sci. 2017;2(2):69.
  19. Belleza M, RN. Thalassemia nursing care planning and management study guide. Nurseslabs. 2018.
  20. Mardhiyah A, Panduragan SL, Mediani HS, Yosep I. Nursing interventions to improve quality of life among children and adolescents with thalassemia: A scoping review. 2023 Jun;16:1749–62.
  21. Eleftheriou A. Health education. Thalassaemia International Federation. Available from: https://www.ncbi.nlm.nih.gov
  22. Everything you need to know about thalassemia. Healthline. 2017. Available from: https://www.healthline.com
  23. Jajhara I, Choudhary G, Singh J, Chachan V, Kumar A. A study on quality of life among thalassemic children aged 8 to 18 years. Int J Contemp Pediatr. 2021 Sep 23;8(10):1667.
  24. Sharma S, Seth B, Jawade P, Ingale M, Setia MS. Quality of life in children with thalassemia and their caregivers in India. Indian J Pediatr. 2016 Dec 7;84(3):188–94.
  25. Pouraboli B, Abedi HA, Abbaszadeh A, Kazemi M. Self-care in patients with major thalassemia: A grounded theory. J Caring Sci. 2017 Jun;6(2):127.
  26. Masinaienejad N, Mohammad AA, Jahantigh F, Afshar MZ, Allahyari J. The impact of self-care education based on Orem’s model on self-care behaviors of patients with β-thalassemia major: A clinical trial. Med-Surg Nurs J. 2019 Aug 31;8(3).
  27. National Organization for Rare Disorders. Beta thalassemia. NORD (National Organization for Rare Disorders). Available from: https://www.rarediseases.org
  28. Iam-arunthai K, Suwanban T, Thungthong P, Chamnanchanunt S, Fucharoen S. Predicting factors of survival rates among alpha- and beta-thalassemia patients: A retrospective 10-year data analysis. Front Hematol. 2024;3.
  29. Thalassemia India. Available from: https://www.thalassemiaindia.org
  30. DiLorenzo P. Thalassemia Support Foundation. Available from: https://www.helpthals.org
  31. Acts | Department of Empowerment of Persons with Disabilities (DEPwD), India. Available from: https://www.depwd.gov.in
  32. Thalassemics India. Available from: https://www.thalassemicsindia.org
  33. Thalassemia Patients Advocacy Group (TPAG). Available from: https://www.thalassemiapag.org
  34. TSCS INDIA – Best Thalassemia Treatment in Hyderabad. Available from: https://www.tscsindia.com
  35. JAI VIGYAN MISSION MODE PROJECT COMMUNITY CONTROL OF RHEUMATIC FEVER/RHEUMATIC HEART DISEASE IN INDIA COMPREHENSIVE PROJECT REPORT [2000-2010]. Indian Council of Medical Research. Available from: https://www.icmr.gov.in
  36. Mathew A, Sobti PC. The burden of thalassemia in Punjab: A roadmap forward. Pediatr Hematol Oncol J. 2017 Dec;2(4):85–7.
  37. Thalassemia Bal Sewa Yojana (TBSY). Available from: https://www.coalindia.in

 

Ahead of Print Subscription Review Article
Volume
Received 19/10/2024
Accepted 16/11/2024
Published 20/11/2024
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