OBJECTIVES Thalassemia is a common genetic disease in Iran, especially in the north and south of Iran. The present study sought to determine the survival rate of patients with thalassemia in highly endemic regions of Iran and its variation in patients born before and after 1971.
METHODS
The present historical cohort study extracted data from the health records of patients with beta-thalassemia major, beta-thalassemia intermedia, and sickle beta-thalassemia who had presented to thalassemia treatment centers in the past years. The collected data were analyzed using the Kaplan-Meier test, the log-rank test, and the chi-square test.
RESULTS
Of the total of 5,491 medical records (2,647 men and 2,634 women; mean age, 23.81±11.32 years), 3,936 belonged to patients with beta-thalassemia major, and 999 and 89 to patients with beta-thalassemia intermedia and sickle beta-thalassemia, respectively. In 467 cases, the type of thalassemia was not clear. The cumulative survival rate was calculated as 0.92, 0.83, 0.74, and 0.51 by ages 25, 35, 45, and 55, respectively. The hazard ratio of death was 4.22 (p<0.05) for beta-thalassemia major and 0.77 for beta-thalassemia intermedia (p=0.70). It was calculated as 1.45 for men patients and as 3.82 for single patients.
CONCLUSIONS
The present study showed relatively high survival rates in patients with thalassemia. The survival of patients was unfavorable in poorer regions (Zahedan and Iranshahr). Factors including women gender, a higher level of education, being married, and living in metropolises decreased the risk of death at younger ages and improved survival.
Summary
Citations
Citations to this article as recorded by
Beta thalassemia: Looking to the future, addressing unmet needs and challenges Michael Angastiniotis Annals of the New York Academy of Sciences.2024; 1532(1): 63. CrossRef
Predicting factors of survival rates among alpha- and beta-thalassemia patients: a retrospective 10-year data analysis Kunapa Iam-arunthai, Tawatchai Suwanban, Pravinwan Thungthong, Supat Chamnanchanunt, Suthat Fucharoen Frontiers in Hematology.2024;[Epub] CrossRef
Relationship between Serum Ferritin Levels and Sarcopenia in Transfusion-Dependent Thalassemia Patient Rendra Prasetya Saefudin, Siprianus Ugroseno Yudho Bintoro, Sony Wibisono Mudjanarko, Merlyna Savitri, Lukita Pradhevi, Agustinus Vincent, Michael Austin Pradipta Lusida, Kartika Afrida Fauzia Biomolecular and Health Science Journal.2024; 7(2): 125. CrossRef
Investigating the effectiveness of Case‐Based Technology‐Enhanced workshops (e‐Flipped vs. Online) among health worker nurses during the COVID‐19 pandemic Nahid Zarifsanaiey, Roghaye Sajjadian, Zahra Karimian, Laleh Khojasteh, Hadi Raeisi Shahraki Nursing Open.2023; 10(4): 2329. CrossRef
The Need for Translational Epidemiology in Beta Thalassemia Syndromes Soteris Soteriades, Michael Angastiniotis, Dimitrios Farmakis, Androulla Eleftheriou, Aurelio Maggio Hematology/Oncology Clinics of North America.2023; 37(2): 261. CrossRef
Desire for biological parenthood and patient counseling on the risk of infertility among adolescents and adults with hemoglobinopathies Anne‐Catherine Radauer‐Plank, Tamara Diesch‐Furlanetto, Monika Schneider, Greta Sommerhäuser, Lucía Alácan Friedrich, Vivienne Salow, Jill Dülberg, Miriam Diepold, Alicia Rovó, Linet Muthoni Njue, Beatrice Drexler, Laura Infanti, Sabine Kroiss, Ramona Mer Pediatric Blood & Cancer.2023;[Epub] CrossRef
Systematic Literature Review Shows Gaps in Data on Global Prevalence and Birth Prevalence of Sickle Cell Disease and Sickle Cell Trait: Call for Action to Scale Up and Harmonize Data Collection Raffaella Colombatti, Inga Hegemann, Morten Medici, Camilla Birkegård Journal of Clinical Medicine.2023; 12(17): 5538. CrossRef
Economic burden of adult patients with β-thalassaemia major in mainland China Xuemei Zhen, Jing Ming, Runqi Zhang, Shuo Zhang, Jing Xie, Baoguo Liu, Zijing Wang, Xiaojie Sun, Lizheng Shi Orphanet Journal of Rare Diseases.2023;[Epub] CrossRef
Accelerated atherosclerosis in beta-thalassemia Julian Hurtado, Hassan Sellak, Giji Joseph, Caitlin V. Lewis, Crystal R. Naudin, Sergio Garcia, James Robert Wodicka, David R. Archer, W. Robert Taylor American Journal of Physiology-Heart and Circulatory Physiology.2023; 325(5): H1133. CrossRef
Prevalence and risk factors of fractures in transfusion dependent thalassemia – A Hong Kong Chinese population cohort Samantha Lai Ka Lee, Raymond Siu Ming Wong, Chi Kong Li, Wing Kwan Leung Endocrinology, Diabetes & Metabolism.2022;[Epub] CrossRef
Estimation of the prevalence of Hemoglobinopathies in Erbil governorate, Kurdistan region of Iraq SarkarS Aziz, BahraK Hamad, HeroO Hamad, MuzhdaI Qader, EmanN Ali, RayanH Muhammed, MudhirSabir Shekha Iraqi Journal of Hematology.2022; 11(1): 19. CrossRef
Thalassaemia Antonis Kattamis, Janet L Kwiatkowski, Yesim Aydinok The Lancet.2022; 399(10343): 2310. CrossRef
Bone Mineral Density, Serum Calcium, and Vitamin D Levels in Adult Thalassemia Major Patients: Experience From a Single Center in Eastern India Soumya Santra, Kunal Sharma, Ipsita Dash, Shaikat Mondal, Himel Mondal Cureus.2022;[Epub] CrossRef
Thalassemia Intermedia: Chelator or Not? Yen-Chien Lee, Chi-Tai Yen, Yen-Ling Lee, Rong-Jane Chen International Journal of Molecular Sciences.2022; 23(17): 10189. CrossRef
The Outcomes of Patients with Haemoglobin Disorders in Cyprus: A Joined Report of the Thalassaemia International Federation and the Nicosia and Paphos Thalassaemia Centres (State Health Services Organisation) Michael Angastiniotis, Soteroula Christou, Annita Kolnakou, Evangelia Pangalou, Irene Savvidou, Dimitrios Farmakis, Androulla Eleftheriou Thalassemia Reports.2022; 12(4): 143. CrossRef
Abnormal diastolic function and Global longitudinal strain in patients with Thalassemia Major on long term chelation therapy Sunil K. Nadar, Shahina Daar, Wael A. Abdelmottaleb, Muhammad M. Shaikh, Hafsa Al Mahrouqi, Majida Al-Raiisi, Moez Hassan, Badar Al Rawahi, Sarah Al Rahbi The International Journal of Cardiovascular Imaging.2021; 37(2): 643. CrossRef
The effect of curcumin on serum copper, zinc, and zinc/copper ratio in patients with β-thalassemia intermedia: a randomized double-blind clinical trial Mohammadreza Saeidnia, Peyman Nowrouzi-Sohrabi, Mehran Erfani, Pooria Fazeli, Gholamhossein Tamaddon, Mehran Karimi Annals of Hematology.2021; 100(3): 627. CrossRef
The Prevention of Thalassemia Revisited: A Historical and Ethical Perspective by the Thalassemia International Federation Michael Angastiniotis, Mary Petrou, Dimitrios Loukopoulos, Bernadette Modell, Dimitrios Farmakis, Panos Englezos, Androulla Eleftheriou Hemoglobin.2021; 45(1): 5. CrossRef
Developing a databank for multiple transfusion patients: Rh antigen and phenotype distribution among 3000 regular blood donors in Iran Zahra Bakhshandeh, Naser Amirizadeh, Mahtab Maghsoodlu, Arezoo Oodi, Amirali Naghi, Ali Arab Khazaeli, Azita Azarkeivan Transfusion and Apheresis Science.2021; 60(3): 103124. CrossRef
Thalassemia-Beta major-Case report Ambarkova Vesna, Krmzova Tina, Nonkulovski Zoran Archives of Hematology Case Reports and Reviews.2021; : 021. CrossRef
An insight into Indonesian current thalassaemia care and challenges Pustika Amalia Wahidiyat, Teny Tjitra Sari, Ludi Dhyani Rahmartani, Iswari Setianingsih, Stephen Diah Iskandar, Anastasia Michelle Pratanata, Ivana Yapiy, Mikhael Yosia, Fernando Tricta ISBT Science Series.2020; 15(3): 334. CrossRef
OBJECTIVES A health-promoting lifestyle (HPL) is a factor that affects the quality of life (QoL) in patients with beta-thalassemia (β-thalassemia). Due to the lack of studies of this issue, this study aimed to determine the association between HPL and QoL among adults with β-thalassemia.
METHODS
This cross-sectional (descriptive-analytic) study was conducted among 389 adult patients with β-thalassemia in Tehran, Iran. The research instrument included a questionnaire consisting of three parts: demographic items, the Short-Form Health Survey and the Health-Promoting Lifestyle Profile. The data were analyzed using SPSS version 23.0. The results were considered significant at the conventional p<0.05 level.
RESULTS
The mean age of the participants was 30.2±8.3 years. The mean score of the HPL dimensions was 127.28±21.53, and the mean score of the QoL domains was 61.44±23.38. The highest and the lowest mean scores of the HPL dimensions were found for spiritual growth (23.96±5.74) and physical activity (11.32±3.95), respectively. The QoL scores in all three domains (total, physical component summary score, and mental component summary score) were moderate. Health responsibility, physical activity, spiritual growth, and interpersonal relations were significant predictive factors of QoL in adults with β-thalassemia; these four dimensions explained 37.9% of the variance in QoL.
CONCLUSIONS
QoL and HPL were not at acceptable levels among patients with thalassemia. Therefore, educational interventions emphasizing spiritual growth, physical activity, and interpersonal relations are necessary for patients with thalassemia.
Summary
Citations
Citations to this article as recorded by
Exploring the perceptions and experiences of female’s with ß-thalassemia major in a Tertiary Care Private Hospital in Pakistan Rawshan Jabeen, Iqra Ansari, Butool Durrani, Mubarak Jabeen Salman, Laraib Mazhar, Muhammad Usman Hussain Ansari, Ali Hussain Ansari, Saba Kabani, Saqib Husain Ansari Transfusion Clinique et Biologique.2024; 31(4): 244. CrossRef
Systematic literature review of the indirect costs and humanistic burden of β-thalassemia Yesim Aydinok, Sneha Purushotham, Aylin Yucel, Mrudula Glassberg, Sohan Deshpande, Barbara Potrata, Myrto Trapali, Farrukh Shah Therapeutic Advances in Hematology.2024;[Epub] CrossRef
Impaired physical ability in patients with transfusion‐dependent β‐thalassaemia: Can regular physical activity be a countermeasure? Mathilde Noguer, Phanélie Berthon, Caroline Makowski, Laurent A. Messonnier British Journal of Haematology.2024;[Epub] CrossRef
The Effect of Benson Relaxation Technique on Anxiety and Quality of Life in Patients with Thalassemia Major: A Clinical Trial Mahnaz Ghaljeh, Fatemeh Kord Salarzehi, Sajad Salehipour Indian Journal of Hematology and Blood Transfusion.2023; 39(4): 557. CrossRef
Aftermath of the COVID-19 Pandemic on Mental Health and Well-Being of Patients With Thalassemia Major in Pakistan: A Qualitative Study Muhammad Hammad , Rasikh Arif, Sehar Bano, Usman Ghani, Hima Bindu Reddy Basani, Vivek Sanker Cureus.2023;[Epub] CrossRef
Thalassaemia - part 1: a clinical update for the dental team Brasanyaa Raveendran, Mohammed Dungarwalla BDJ Team.2023; 10(8): 28. CrossRef
Cardiopulmonary testing in adult patients with β-thalassemia major in comparison to healthy subjects G. Piatti, M. Giuditta, D. Consonni, E. Cassinerio, M. D. Cappellini Annals of Hematology.2022; 101(11): 2445. CrossRef
Thalassaemia - part 1: a clinical update for the dental team Brasanyaa Raveendran, Mohammed Dungarwalla British Dental Journal.2022; 233(11): 931. CrossRef
Assessment of Health-promoting Lifestyle among Medical Students of Mashhad University of Medical Sciences in 2018-2019 Nafiseh Mousavi Bazaz, Masoumeh Haghighi, Roya Boujaran, Vida Vakili, Mojtaba Mousavi Bazzaz, Gholamreza Haghighi Jorjani Biomedicine Journal.2022; 10(1): 4. CrossRef
A multicenter cross-sectional study of the quality of life and iron chelation treatment satisfaction of patients with transfusion-dependent β-thalassemia, in routine care settings in Western Greece Vassilis Goulas, Alexandra Kouraklis-Symeonidis, Kyriaki Manousou, Vassileios Lazaris, George Pairas, Paraskevi Katsaouni, Eugenia Verigou, Vassiliki Labropoulou, Vassiliki Pesli, Panagiotis Kaiafas, Urania Papageorgiou, Argiris Symeonidis Quality of Life Research.2021; 30(2): 467. CrossRef
Health literacy and health-promoting behaviors among adults at risk for diabetes in a remote Filipino community Christian Sandor B. Ydirin Belitung Nursing Journal.2021; 7(2): 88. CrossRef
Effects of aquatic exercise on dimensions of quality of life and blood indicators in patients with beta-thalassemia major AliHasanpour Dehkordi, Toba Hasani, Kiavash Fekri, Fatemeh Deris, Shahram Etemadifar International Journal of Preventive Medicine.2020; 11(1): 128. CrossRef
Examining the Relationship Between Spiritual Coping and Spiritual Wellbeing in Patients with Beta-Thalassemia Major Narjes Khatoon Sadeghi, Sakine Miri, Farnaz Jahantigh, Reza Nikbakht Medical - Surgical Nursing Journal.2019;[Epub] CrossRef
Effect of different iron chelation regimens on bone mass in transfusion-dependent thalassemia patients Mohammadreza Bordbar, Sezaneh Haghpanah, Omid Reza Zekavat, Forough Saki, Asghar Bazrafshan, Haleh Bozorgi Expert Review of Hematology.2019; 12(11): 997. CrossRef
Effect of A Mobile-Phone Mediated Based Education on Self-Care Behaviors of Patients With Thalassemia Major Fatemeh Gharaati, Teamur Aghamolaei, Zahra Hosseini, Sayed Hossein Davoodi, laleh Hassani, Razieh Mohamadi, Shokroallah Mohsseni, Moussa Soleimani-Ahmadi Journal of Caring Sciences.2019; 8(3): 149. CrossRef
Comparative effectiveness of alendronate and zoledronic acid on bone mass improvement in transfusion-dependent thalassemia patients Omid Reza Zekavat, Mohamadreza Bordbar, Sezaneh Haghpanah, Forough Saki, Asghar Bazrafshan, Haleh Bozorgi Journal of Bone and Mineral Metabolism.2019; 37(6): 996. CrossRef
Depression, Anxiety, and Perceived Social Support among Adults with Beta-Thalassemia Major: Cross-Sectional Study Aghbabak Maheri, Roya Sadeghi, Davoud Shojaeizadeh, Azar Tol, Mehdi Yaseri, Alireza Rohban Korean Journal of Family Medicine.2018; 39(2): 101. CrossRef
The Relationship between Health-Promoting Lifestyle (HPL) and Academic Self-Efficacy among students S Saadat, M Kalantari, MB Kajbaf Research in Medical Education.2018; 9(4): 38. CrossRef
Quality of Life: Transfusion Dependent Thalassemia vs Non-Transfusion Dependent Thalassemia Mehran Karimi, Nader Cohan Thalassemia Reports.2018; 8(1): 7489. CrossRef
Poor quality of life as a predictor of survival among thalassemia patients in Iran Shahab Rezaeian Epidemiology and Health.2017; 39: e2017013. CrossRef
Determinants of health-related quality of life in Iranian adults: evidence from a cross-sectional study Satar Rezaei, Mohammad Hajizadeh, Ali Kazemi, Masoud Khosravipour, Farid Khosravi, Shahab Rezaeian Epidemiology and Health.2017; 39: e2017038. CrossRef