1. King Edward Medical University, Lahore, Pakistan; dua90zahra@gmail.com
2. Sunsari Technical College, Nepal; parshuram@gmail.com

*             Correspondence: parshuram@gmail.com

Keywords: psychological impacts, thalassemia, parents, patients

1. INTRODUCTION

An autosomal recessive condition, thalassemia is highly prevalent throughout the world, especially in the Mediterranean, Central Asia, and the Middle East [1, 2]. Pakistan has a 5-8% carrier rate for thalassemia, with 9.8 million carriers and 5,000 serious thalassemia newborns annually [3]. The psychological and social functioning of thalassemia major patients is as crucial for their well-being, even though recent advancements in healthcare have improved patients’ life expectancy and survival rate [4, 5]. Patients with thalassemia major require frequent blood transfusions and iron chelating therapy, which can be upsetting for both the patient and the patient’s parents [6]. Furthermore, up to 80% of children with thalassemia have been documented to experience psychosocial issues, such as anxiety and depression, which might interfere with their ability to perform typical developmental tasks [7]. Individuals caring for patients and their carers have a lot on their plates, including financial and psychological demands. The prevalence of thalassemia is rising globally, and this has made it a significant contributing factor to psychosocial problems such as anxiety, dejection, animosity, social disengagement, strained relationships, and poor academic performance [8]. About 3% of people worldwide carry the beta thalassemia gene, and treating the condition puts a significant financial and psychological strain on both sufferers and their parents [9, 10]. Patients with thalassemia and their parents may have a direct reduction in quality of life due to the physical, emotional, and cognitive effects [11]. Psychological strain on patients and their parents is increased by worries about future issues and a low quality of life; hospitalization, worsening medical ailments, and not being able to pay for treatment can all add to the psychological strain [12]. There is currently no comprehensive review that focuses on the mental health concerns that patients and their parents are dealing with, despite the fact that many studies have looked at the psychosocial elements of thalassemia major in patients. The goal of this systematic review is to provide an overview of the body of research in order to pinpoint psychological issues and develop initiatives that help patients with thalassemia receive early psychosocial care.

2. MATERIALS AND METHODS

In the current systematic review, articles published in the English language between 2002 and 2022 that described the psychological challenges faced by parents of thalassemia patients were sourced using the PRISMA approach. Searches were conducted using Mesh-based terms “thalassemia” and “psychosocial” on PubMed, Science Direct, and Google Scholar. They accessed central databases from September 3rd to September 6th, 2022. Duplicate studies were removed after the identified studies were uploaded to EndNote. The full-text articles were carefully reviewed after the articles were first filtered based just on their titles and abstracts. After reviewing the abstracts, one author (SS) selected a subset of the full articles for data collection. We included papers from 2002 to 2022 that looked at the psychological effects of thalassemia in patients that were published in peer-reviewed English-language publications. The articles that contained the terms “Thalassemia” and “Psychosocial” in their titles and had the highest percentage of positive replies (≥50%) on JBI checklists were included. On the other hand, we eliminated studies that assessed people with illnesses other than thalassemia, those that fell outside the purview of our investigation (e.g., evaluating the psychosocial implications of thalassemia screening methods or the creation of bio-psychosocial scales for patients with the disease), those that lacked full-text accessibility, and abstracts that were published in proceedings from conferences, seminars, or congresses; letters to the editor; case studies; short reports; and reviews. Every abstract and title was evaluated by two reviewers. The full text was requested if the abstract did not contain sufficient information to assess the inclusion or exclusion criteria. Following the independent evaluation of each chosen paper, decisions were debated in consensus sessions. Finally, a third author was consulted in case of dispute. Data extraction and analysis were conducted on a subset of the identified publications. The quality of each publication was assessed using JBI critical appraisal checklists, with distinct checklists for qualitative, cross-sectional, analytical, and case-control studies [13]. There were eight to ten items on each checklist, and the possible answers were yes, no, unclear, and not applicable. The study includes the publications with a high number of affirmative responses (≥50%). Seven studies were rated as moderately quality and fifteen as high quality based on the scores.

3. RESULTS AND DISCUSSION

The study’s results are presented in accordance with PRISMA criteria. A comprehensive search was carried out to find pertinent publications, yielding a total of 7540 articles from various sites such as PubMed, Google Scholar, and Science Direct. A total of 7424 papers were screened using their titles and abstracts after 116 duplicate articles were eliminated. The selected papers discussed the psychosocial issues that parents and patients with thalasseemia faced. Thirty-five articles (out of 49) were obtained in full text. Ultimately, 22 [14–35] articles were taken into consideration for the study after certain criteria were applied to include or eliminate articles. Of the 22, we discovered 6 case-control, 1 qualitative, and 15 cross-sectional studies. Of the 22, eight publications focus only on the emotional issues that parents face, eleven articles address similar issues in patients, and three research address the psychosocial issues that both patients and their parents deal with. Psychosocial problems are significantly more common in thalassemia patients [14, 15]. In addition to behaviour issues (anxiety, aggression, temper tantrums, and overactivity) [23–26], mood disorders (somatization, depression, loneliness, panic, free-floating anxiety, worrying, sleep disturbances, obsessive-compulsive traits) [15–27], tic disorder, and nocturnal enuresis [25,27] were among the conditions that affected the patients. The 20–25 age group was known for having psychotic difficulties (delusion, hallucination, mental disorder, lack of insight) and sexual problems (lowered libido, fear of impotence, decreased interest in the other sex) [24].

Figure 1: Psychosocial Problems Faced by Thalassemia Patients and their Parents

Studies have demonstrated the detrimental impacts of illness on learning [18–25] and personal athletics [18, 19]. According to the majority of patients, their illness interfered with their social life [15–18] [21–23]. There is a significant possibility that children with long-term medical conditions will experience psychological problems. Such risks were elevated by variables such as frequent hospitalization, dread of dying, overprotection, and others [25]. Just 25% of parents take antidepressants, despite the fact that over 80% of them acknowledged suffering depression [29–31]. A study found that the majority of the parents experienced psychosomatic ailments like body aches, diabetes, and heart disease [28,32] and felt despondent [31–35]. The parents’ connection is significantly impacted by the child’s illness [30–33]. According to a study, parents’ educational attainment positively correlated with their level of depression [31]. A study found that the main problems that the majority of parents faced were losing interest in their work and being unable to focus on their everyday tasks [28–32]. Seventy percent of parents struggle to make ends meet for their children who are thalassaemic [28–33]. The psychological and social well-being of parents affected by thalassemia major is greatly impacted by the following factors: stigmatisation [28–33], consanguineous marriages [30–34], frequency of marriage breakdowns [33] brought on by the disease, and insufficient awareness of the disease [31–34]. A few parents acknowledged that their child’s condition had an impact on the size of the family [31, 33]. 72% of the parents would have had an abortion if the disease had been discovered during pregnancy, per a study [31]. Based on the review’s findings, patients with Thalassemia and their parents experience a high prevalence of psychosocial issues, such as behavioural issues (Vijaya Raman MD et al., 2023), mood disorders (Manoj Jain et al., 24), tics, and psychosis (which are likely caused by patients’ low self-esteem, emotional instability, and self-doubt). A sign of this is poor performance in academic and athletic pursuits, which, in the words of Kumaravel KS et al. [26], can create a vicious cycle of underachievement and stress by further eroding self-respect, confidence, and emotional stability.  According to Nabavian M. et al. [28], the disease causes the parents of the affected person to experience long-term effects of ongoing stress and financial strain, which can lead to chronic illnesses like diabetes and heart disease. These illnesses can also make financial strain worse when the affected person’s earning capacity is reduced. Six case-control studies are included in this analysis; these studies are essential for understanding how these issues have changed over a long period of time and determining how persistent most of the issues have remained. These issues are indicative of a structural difficulty in the distribution of resources and the establishment of suitable infrastructure for the treatment and management of thalassemia patients. The studies that are included show that there was insufficient effort made to effectively treat the recurrence of certain problems, such as mood disorders [17, 26], spanning a range of time periods from 2002 to 2022. The absence of previous study on this topic has resulted in an inadequate assessment of the psychosocial requirements of patients and their parents, which is concerning given the extent of the problem. The fact that this systematic review is the first of its kind to be published on the issues of people with thalassemia is a good indicator of the dearth of pertinent literature. A significant contributing reason to the lack of progress in implementing appropriate measures to enhance the lives of patients with chronic conditions is the general society’s trivialization of the emotional anguish these people endure. Hopefully, contextualising the extent of these problems is the first step towards solving them on a large scale.

4. CONCLUSION

According to the research, thalassemia children face serious psychosocial difficulties, including low self-esteem, mood disorders, and behavioural problems like worry, anxiety, loneliness, insomnia, and obsessive-compulsive behaviours. Adolescents often struggle with sexual and psychiatric disorders. Parenting presents challenges, primarily in the form of mounting debt and broken interpersonal bonds, such as separation or divorce. Thus, understanding the psychological difficulties faced by people with beta-thalassemia major can help medical professionals and administrators create efficient treatments that improve patients’ and their families’ coping mechanisms, resulting in improved handling of such difficulties.

REFERENCES

1. Brousse V, Kossorotoff M, de Montalembert M. Beta Thalassemia. Br J Haematol 2015; 170 (5):615-25.
2. Origa R. β-Thalassemia. Official journal of the American College of Medical Genetics 2017;19(6):609-619
3. Ahmed S, Saleem M, Modell B, Petrou M. Screening extending Families for genetic hemoglobin disorders in Pakistan. N Engl J Med 2010;347(15):1162-68.
4. Vitrano A, Calvaruso G, Lai E, Colletta G, Quota A, Gerardi C, et al. The era of comparable life expectancy between thalassemia major and intermedia: Is it time to revisit the major-intermedia dichotomy? Br J Haematol 2017;176(1):124-30.
5. Ruggeri K, Garcia-Garzon E, Maguire Á, Matz S, Huppert FA. Well-being is more than happiness and life satisfaction: A multidimensional analysis of 21 countries. Health Qual Life Outcomes 2020; 18(1): 116.
6. Ratip S, Modell B. Psychological and sociological aspects of the thalassemia. Semin Hematol 1996; 33(1): 53-65.
7. Beratis S. Psychosocial status in pre-adolescent children with β-thalassemia. J Psychosom Res. 1993;37(3):271-9.
8. Klein N, Sen A, Rusby J, Ratip S, Modell B, Olivieri NF. The psychosocial burden of Cooley’s anemia in affected children and their parents. Ann N Y Acad Sci 1998;850(1):512-13.
9. Ansari SH, Shamsi TS. Thalassaemia prevention programme. Haematology Updates 2010;14(3):23-28.
10. Maryam Majeed, Rubab Ali, Umm-e-Aimen & Aqsa Jabeen. Medical Staff, Workplace Bullying and Its Effects on the Performance. Dinkum Journal of Medical Innovations, 2(04):120-125.
11. Marie Diack. Factors Influencing Relational Violence in Nepali Married Relationships. Dinkum Journal of Medical Innovations, 2(04):126-133.
12. Gul Rukh Malik, Rubab Zahra & Tahir Rana. Autism Spectrum Disorders Frequency in Asian Countries. Dinkum Journal of Medical Innovations, 2(04):134-139.
13. Parshu Ram Chaudhary, Asbin Bandhari & Parshu Kirby. Aeroallergens and Significant Environmental Pollutants: Aeroallergen Sensitivity Symptoms. Dinkum Journal of Medical Innovations, 2(04):140-149.
14. Imran Mehfooz Khan, Aron Kumar & Pradeep Sahejpal. Extensive Management Medical Incidents Provided to Gallbladder Cancer Using Cholecystectomy Specimen. Dinkum Journal of Medical Innovations, 2(04):150-156.
15. Elzaree FA, Shehata MA, El Wakeel MA, El Alameey IR, Abu Shady MM, Helal SI. Adaptive Functioning and Psychosocial problems in Children with Beta Thalassemia Major. Open Access Maced J Med Sci. 2018;6 (12):2337-2341.
16. Messina G, Colombo E, Cassinerio E, Ferri F, Curti R, Altamura C, et al. Psychosocial aspects and psychiatric disorders in young adult with thalassemia major. Intern Emerg Med. 2008;3 (4):339-43.
17. Batool I, Ishfaq K, Bajwa R. Psychosocial burden among thalassemia major patients: An exploratory investigation of south Punjab, Pakistan. Pak Pediatr J. 2017; 41(5): 158-162.
18. Khurana A, Katyal S, Marwaha RK. Psychosocial burden in thalassemia. Indian J Pediatr. 2006; 73(10): 877-880.
19. Inamdar S, Gangrade A. Psychosocial Problems of Thalassemics in A Tertiary Care Centre, Indore. Ntl J Community Med 2016; 7(5): 391-394.
20. Ishfaq K, Diah N, Ali J, Fayyaz B, Batool I. Psychosocial problems faced by thalassemia major patients of district Multan, Pakistan. Pak Pediatr J. 2018;42(2): 23-27.
21. Canatan D, Ratip S, Kaptan S, Cosan R. Psychosocial burden of beta-thalassemia major in Antalya, south Turkey. Soc Sci Med. 2003;56 (4): 815-819.
22. Raman V, Prakash A, D’Souza F. Psychosocial Issues in Children with Thalassemia: From Identification to a Model for Management in a Developing Country. J Pediatr Hematol Oncol. 2019;41(3):218-221.
23. Bagul A, Porwal A, Jain M. Psychosocial problems in thalassemic adolescents and young adults. Chronicles of Young Scientists. 2013;4 (1):21.
24. Saini A, Chandra J, Goswami U, Singh V, Dutta AK. Case-control study of psychosocial morbidity in beta-thalassemia major. J Pediatr. 2007;150(5):516-520.
25. Kumaravel K, Jagannathan S, Balaji J, Karthick N, Pugalendhiraja K. Psychosocial Problems Associated with Transfusion Dependent Thalassemia in a Tribal Population. Pediatr On call J. 2016;13(1):99-102.
26. Aydinok Y, Erermis S, Bukusoglu N, Yilmaz D, Solak U. Psychosocial implications of Thalas-semia Major. Pediatr Int. 2005;47(1):84-89.
27. Nabavian M, Cheraghi F, Shamsaei F, Tapak L, Tamaddoni A. The psychosocial challenges of mothers of children with thalassemia: A qualitative study. J Edu Health Promot 2022;11 (2):37.
28. Kaur N. Psychosocial and financial burden on parents of children with thalassemia. Int J Community Res. 2018;10(12):76268-76272.
29. Shafi N, Ahmed S, Siddique AR. The Psychosocial and Financial Ramifications of Thalassemia on Parents of Thalassemic Children Presented at Tertiary Care Hospitals. Open J Pediatr. 2021;11(3):379-387.
30. Aziz K, Sadaf B, Kanwal S. Psychosocial problems of Pakistani parents of Thalassemic children: a cross-sectional study done in Bahawalpur , Pakistan. Biopsychosoc Med. 2012;6(1):15.
31. Deepak SS, Jay SP, Subhash SS, Tukaram PH. Assessment of Psychosocial Impact on Parents of Thalassemic Children. SAS Journal of Medicine. 2017;3(3):57-60.
32. Rashid MAH, Abbasi SS, Manzoor MM. Socio-religious Prognosticators of Psychosocial Burden of Beta Thalassemia Major. J Relig Health. 2020; 59(6): 2866-2881.
33. Rashid MAH, Khan AM, Abbas Z, Ramzan S, Aziz L. Social epidemiological analysis of risk factors and psychosocial burden of beta thalassemia major (BTM) in Pakistan. EEO. 2021; 20(4): 2682-2687
34. Ishfaq K, Naeem SB, Ahmad T, Zainab S. Psycho-social and Economic Impact of Thalassemia Major on Patients’ Families. Isra Medical Journal 2016;8(1): 24-27