The psychosocial dimension in the treatment of thalassemia: a review of the literature

Journal title PSICOLOGIA DELLA SALUTE
Author/s Marco Bani, Francesca Barile, Umberto Mazza
Publishing Year 2013 Issue 2013/1
Language Italian Pages 22 P. 5-26 File size 325 KB
DOI 10.3280/PDS2013-001001
DOI is like a bar code for intellectual property: to have more infomation click here

Below, you can see the article first page

If you want to buy this article in PDF format, you can do it, following the instructions to buy download credits

Article preview

FrancoAngeli is member of Publishers International Linking Association, Inc (PILA), a not-for-profit association which run the CrossRef service enabling links to and from online scholarly content.

Thalassemia major is a genetic disorder that requires very frequent blood transfusion and an iron chelation treatment as well as a multidisciplinary management. Like any chronic disease causes a great impact on quality of life, psychological well-being and family background of these subjects. The medical advances of the last 30 years have improved the life expectancy of these patients who now may reach adulthood, have a work, have children, but they need a constant compliance to treatment. The aim of this work it to review the results of the last 10 years of research in relation to psychosocial difficulties of patients and families, the quality of life and the presence of psychopathology in thalassemic patients, also across different cultural contexts. The data emerging from the review show a greater level of psychopathology compared with control subjects and a lower quality of life for both patients with thalassemia and their family members, suggesting a greater effort towards the spread of psychosocial interventions that already exist in some situations.

Keywords: Thalassemia, psychopathology, caregiver, coping, quality of life

  1. AA. VV. (2003). Guidelines for the Clinical Management of Thalassemia, Thalassemia International Federation
  2. AA. VV. (2007). Guidelines for the Clinical Management of Thalassemia – 2° edition, Thalassemia International Federation
  3. Aydin B., Yaprak I., Akarsu D., Ökten N. and Ülgen M. (1997). Psychosocial aspects and psychiatric disorders in children with thalassemia major. Pediatrics International, 39 (3): 354-357. DOI: 10.1111/j.1442-200X.1997.tb03752.x
  4. Aydinok Y., Erermis S., Bukusoglu N., Yilmaz D. and Solak U. (2005). Psychosocial implications of Thalassemia Major. Pediatrics International, 47 (1): 84-89. DOI: 10.1111/j.1442-200x.2004.02009.x
  5. Aydinok Y., Ulger Z., Nart D., Terzi A., Cetiner N., Ellis G., Zimmermann A. and Manz C. (2007). A randomized controlled 1-year study of daily deferiprone plus twice weekly desferrioxamine compared with daily deferiprone monotherapy in patients with thalassemia major. Haematologica, 92 (12): 1599-1606. DOI: 10.3324/haematol.11414
  6. Ammad S.A., Mubeen S.M., Ul Hassan Shah S.F. and Mansoor S. (2011). Parents’ opinion of quality of life (QOL) in Pakistani thalassaemic children. Journal of Pakistan Medical Association, 61: 470-473
  7. Angastiniotis M. (2002). The adolescent thalassemic. The complicant rebel. Minerva Pediatrica, 54 (6): 511-515.
  8. Anie A.K. (2005). Psychological complications in sickle cell disease. British Journal of Haematology, 129: 723-729. DOI: 10.1111/j.1365-2141.2005.05500.x
  9. Anie K.A. and Green J. (2007). Psychological therapies for sickle cell disease and pain (Cochrane review). Cochrane Database of Systematic Reviews, Issue 3. DOI: 10.1002/14651858.CD001916.pub2
  10. Anie K.A. and Massaglia P. (2001). Psychological therapies for thalassaemia. Cochrane Database of Systematic Reviews, 3. Art. No.: CD002890 (update 2008). DOI: 10.1002/14651858.CD002890
  11. Atkin K. and Ahmad W.I.U. (2000). Family caregiving and chronic illness: How parents cope with a child with sickle cell disorder or thalassaemia major. Health and Social Care in the Community, 8 (1): 57-69. DOI: 10.1046/j.1365-2524.2000.00211.x
  12. Atkin K. and Ahmad W.I.U. (2001). Living a ‘normal’ life: young people coping with thalassaemia major or sickle cell disorder. Social Science & Medicine, 53: 615-626. DOI: 10.1016/S0277-9536(00)00364-6
  13. Aydin B., Yaprak I., Akarsu D., Okten N. and Ulgen M. (1997). Psychosocial aspects and psychiatric disorders in children with thalassemia major. Acta Paediatrica Japonica, 39 (3): 354-357.
  14. Azarkeivan A., Hajibeigia B., Alavianb S.M., Lankaranic M.M. and Assaric S. (2009). Associates of poor physical and mental health-related quality of life in beta thalassemiamajor/ intermedia. Journal of Research in Medical Sciences, 14 (6): 349-355.
  15. Beratis S. (1993) Psychosocial status in preadolescent children with β-thalassaemia. Journal of Psychosomatic Research, 37: 271-279. DOI: 10.1016/0022-3999(93)90036-F
  16. Bocchetta A. (2005). Heterozygous beta-thalassaemia as a susceptibility factor in mood disorders: excessive prevalence in bipolar patients. Clinical Practice and Epidemiology in Mental Health, 1: 6. DOI: 10.1186/1745-0179-1-6
  17. Bush S., Mandel F.S. and Giardina P.J. (1998). Future Orientation and Life Expectations of Adolescents and Young Adults with Thalassemia Major. Annals New York Academy of Sciences, 850: 361-369. DOI: 10.1111/j.1749-6632.1998.tb10494.x
  18. Cakaloz B., Cakaloz I., Polat A., Inan M. and Kalkan Oguzhanoglu N. (2009). Psychopathology in thalassemia major, Pediatrics International, 51: 825-828. DOI: 10.1111/j.1442-200X.2009.02865.x
  19. Canatan D., Ratip S., Kaptan S. and Cosan R. (2003). Psychosocial burden of s-thalassaemia major in Antalya, south Turkey. Social Science & Medicine, 56 (4): 815-819. DOI: 10.1016/S0277-9536(02)00080-1
  20. Cappellini M.D. and Pattoneri P. (2009). Oral Iron Chelators. Annual Review of Medicine, 60: 25-38. DOI: 10.1146/annurev.med.60.041807.123243
  21. Clarke S.A., Skinner R., Guest J., Darbyshire P., Cooper J., Shah F., Roberts I. and Eiser C. (2009). Health-related quality of life and financial impact of caring for a child with Thalassaemia Major in the UK. Child: care, health and development, 36 (1): 118-122. DOI: 10.1111/j.1365-2214.2009.01043.x
  22. Clemente C., Tsiantis J. and Sadowski H. (2002). Psychopathology in children from families with blood disorders: a cross-national study. European Child & Adolescent Psychiatry, 11 (4): 151-161. DOI: 10.1007/s00787-002-0257-3
  23. Dahlui M., Hishamshah M.I., Rahman A.J.A. and Aljunid S.M. (2009). Quality of life in transfusiondependent thalassaemia patients on desferrioxamine treatment. Singapore Medical Journal, 50 (8): 794-799.
  24. Di Palma A., Vullo C., Zani B. and Facchini A. (1998). Psychosocial integration of adolescents and young adults with thalassemia major. Annals of the New York Academy of Sciences, 850: 355-360. DOI: 10.1111/j.1749-6632.1998.tb10493.x
  25. Gafari Saravi V., Zarghami M., Tirgari A. and Ebrahimi E. (2007). Relationship between thalassemia and depression. Research Journal of Biological Science, 2 (3): 280-284.
  26. Ghanizadeh A., Sirin K. and Hamid A. (2006). Prevalence of psychiatric disorders, depression and suicidal behavior in child and adolescent with thalassemia major. Journal of Pediatric Hematology/Oncology, 28: 781-784. DOI: 10.1097/01.mph.0000243665.79303.9e
  27. Gharaibeh H.F. and Gharaibeh M.K. (2012). Factors influencing health-related quality of life of thalassaemic Jordanian children. Child: care, health and development, 38 (2): 211-218 DOI: 10.1111/j.1365-2214.2011.01224.x
  28. Gharaibeh H., Amarneh B.H. and Zamzam S.Z. (2009). The Psychological Burden of Patients with Beta thalassemia Major in Syria. Pediatrics International, 51 (5): 630-636. DOI: 10.1111/j.1442-200X.2009.02833.x
  29. Hajibeigi B., Azarkeyvan A., Alavian S.M., Lankarani M.M. and Assari S. (2009). Anxiety and depression affects life and sleep quality in adults with beta-thalassemia. Indian Journal of Hematology and Blood Transfusion, 25 (2): 59-65. DOI: 10.1007/s12288-009-0015-5
  30. Kattamis C. (1989). The child with thalassaemia. Bulletin International Paediatric Association, 4: 19-29.
  31. Ismail A. (2010). Measuring the Health Related Quality of Life of Malaysian Children with Thalassaemia: Reliability and Validity of PedsQL 4.0 Generic Score and SF36v2. Journal of Statistical Modeling and Analytics, 1 (1): 1-28.
  32. Ismail A., Campbell M., Ibrahim H. and Jones G. (2006). Health-related quality of life in Malaysian children with thalassaemia. Health and Quality of Life Outcomes, 4: 39. DOI: 10.1186/1477-7525-4-39
  33. Laurice M. (2005). Caring for Adults with Thalassemia in a Pediatric World. Annals of the New York Academy of Sciences, 1054: 266-272. DOI: 10.1196/annals.1345.034
  34. Louthrenoo O., Sittipreechacharn S., Thanarattanakorn P. and Sanguansermsri T. (2002). Psychosocial problems in children with thalassemia and their siblings. Journal of the Medical Association of Thailand, 5: 881-885.
  35. Marvasti V.E., Dastoori P. and Karimi M. (2006). Is b-thalassemia trait a risk factor for developing depression in young adults? Annals of Hematology, 85: 873-874. DOI: 10.1007/s00277-006-0154-9
  36. Masera G., Monguzzi W., Piga A., Massaglia M.P., Vania A., De Pascale A., Magnano C. e Di Palma A. (1996). Raccomandazioni per l’intervento psicosociale nella talassemia. [Recommendations for psychosocial intervention in thalassemia]. Rivista Italiana di Pediatria, 22: 110-112.
  37. Mazzone L., Battaglia L., Andreozzi F., Romeo M.A. and Mazzone D. (2009). Emotional impact in β-thalassaemia major children following cognitive-behavioural family therapy and quality of life of caregiving mothers. Clinical Practice and Epidemiology in Mental Health, 5: 5. DOI: 10.1186/1745-0179-5-5
  38. Mednick L., Yu L., Trachtenberg F., Xu Y., Kleinert D.A., Giardina P.J., Kwiatkowski J.L., Foote D., Thayalasuthan V., Porter J.B., Thompson A.A., Schilling L., Quinn C.T., Neufeld E.J. and Yamashita R. (2010). Symptoms of depression and anxiety in patients with thalassemia: Prevalence and correlates in the thalassemia longitudinal cohort. American Journal of Hematology, 85 (10): 802-805. DOI: 10.1002/ajh.21826.
  39. Messina G., Colombo E., Cassinerio E., Ferri F., Curti R., Altamura C. and Cappellini M.D. (2008). Psychosocial aspects and psychiatric disorders in young adult with thalassemia major. Internal and Emergency Medicine, 3 (4): 339-343. DOI: 10.1007/s11739-008-0166-7
  40. Mikelli A. and Tsiantis J. (2004). Brief report: Depressive symptoms and quality of life in adolescents with b-thalassaemia. Journal of Adolescent Health, 27: 213-216. DOI: 10.1016/j.adolescence.2003.11.011
  41. Monastero R., Monastero G., Ciaccio C., Padovani A. and Camarda R. (2000). Cognitive defcits in beta-thalassemia major. Acta Neurologica Scandinavica, 102: 162-168. DOI: 10.1034/j.1600-0404.2000.102003162.x
  42. Moorjani J.D. and Issac C. (2006). Neurotic Manifestations in Adolescents With Thalassemia Major, Indian journal of pediatrics, 73 (7): 603-607.
  43. Musallam K., Cappellini M.D. and Taher A. (2008). Challenges Associated With Prolonged Survival of patients With Thalassemia: Transitioning From Childhood to Adulthood. Pediatrics, 121 (5): 1426-1429. DOI: 10.1542/peds.2007-1944
  44. Musallam K.M., Khoury B., Abi-Habib R., Bazzi L., Succar J., Halawi R., Hankir A., Koussa S. and Taher A.T. (2011). Health-related quality of life in adults with transfusionindependent thalassaemia intermedia compared to regularly transfused thalassaemia major: new insights. European Journal of Haematology, 87: 73-79. DOI: 10.1111/j.1600-0609.2011.01623.x
  45. Osborne R.H., De Abreu Lourenço R., Dalton A., Houltram J., Dowton D., Joshua D.E., Lindeman R. and Ho P.J. (2007). Quality of Life Related to Oral versus Subcutaneous Iron Chelation: A Time Trade-off Study. Value in Health, 10 (6): 451-456. DOI: 10.1111/j.1524-4733.2007.00200.x
  46. Kuo H.T., Peng C.T. and Tsai M.Y. (2006). Pilot Study on Parental Stress and Behavioral Adjustment to the Thalassemia Major Disease Process in Children Undergoing Iron-Chelation in Western Taiwan. Hematology, 30 (2): 301-309. DOI: 10.1080/03630260600642658
  47. Pakbaz Z., Treadwell M., Kim H.-Y., Trachtenberg F., Parmar N., Kwiatkowski J.L., Cunningham M.J., Martin M., Sweeters N., Neufeld E.J., Giardina P.J., Olivieri N., Yamashita R.C. and Vichinsky E. (2010). Education and employment status of children and adults with thalassemia in North America. Pediatric Blood & Cancer, 55: 678-683. DOI: 10.1002/pbc.22565
  48. Pakbaz Z. (2005). Quality of life in patients with thalassemia intermedia compared to thalassemia major. Annals of the New York Academy of Sciences, 1054: 457-461. DOI: 10.1196/annals.1345.059
  49. Pless I.B. (1984). Symposium on chronic disease in children. Clinical Assessment: Physical and psychological functioning. Pediatric Clinical of North America, 31: 33-35.
  50. Politis C., Di Palma A., Fisfis M., Giasanti A., Richardson S.C., Vullo C. and Masera G. (1990). Social integration of the older thalassaemic patient. Archives of Disease in Childhood, 65 (9): 984-986. DOI: 10.1136/adc.65.9.984
  51. Pradhan P.V., Shah E., Rao P., Ashturkar D. and Ghaisas P. (2003). Psychopathology and Self-esteem in Chronic Illness. Indian Journal of Pediatrics, 70 (2): 135-138. DOI: 10.1007/BF02723739
  52. Rao P., Pradhan P.V. and Shah H. (2004). Psychopathology and Coping in Parents of Chronically Ill Children. Indian Journal of Pediatrics, 71 (8): 695-699. DOI: 10.1007/BF02730656
  53. Ratip S., Skuse D., Porter J., Wonke B., Yardumian A. and Modell B. (1995). Psychosocial and clinical burden of thalassaemia intermedia and its implications for prenatal diagnosis. Archives of Disease in Childhood, 72 (5): 408-412. DOI: 10.1136/adc.72.5.408
  54. Ratip S. and Modell B. (1996) Psychosocial and sociological aspects of the thalassemias. Seminars in Hematology, 33: 53-65
  55. Sobota A., Yamashita R., Xu Y., Trachtenberg F., Kohlbry P., Kleinert D.A., Giardina P.A., Kwiatkowski J.L., Foote D., Thayalasuthan V., Porter J.B., Thompson A.A., Schilling L., Quinn C.T. and Neufeld E.J. (2010). Quality of life in Thalassemia: A comparison of SF-36 results from the Thalassemia longitudinal cohort to reported literature and the US norms. American Journal of Hematology, 86 (1): 92-95. DOI: 10.1002/ajh.21896
  56. Sabry N. and Salama K.H. (2009). Cognitive Abilities, Mood Changes and Adaptive Functioning in Children with β Thalassaemia. Current Psychiatry, 16 (3): 244-254
  57. Sadowski H., Kolvin I., Clemente C., Tsiantis J., Baharaki S., Ba G., Lee C., and Taylor B. (2002). Psychopathology in children from families with blood disorders: a cross national study. European Child and Adolescent Psychiatry, 11 (4): 151-161. DOI: 10.1007/s00787-002-0257-3
  58. Sapountzi-Krepia D., Roupa Z., Gourni M., Mastorakou F., Vojiatzi E., Kouyoumtzi A. and Van Shell S. (2006). A qualitative study on the experiences of mothers caring for their children with thalassemia in Athens, Greece. Journal of Pediatric Nursing, 21: 142-152. DOI: 10.1016/j.pedn.2005.06.017
  59. Shaligram D., Girimaji S.C. and Chaturvedi S.K. (2007). Psychological Problems and Quality of Life in Children with Thalassemia. Indian journal of pediatrics, 74 (8): 727-730
  60. Sharghi A., Karbakhsh M., Nabaei B., Meysamie A. and Farrokhi A. (2006). Depression in mothers of children with thalassemia or blood malignancies: A study from Iran. Clinical Practice of Epidemiology in Mental Health, 2: 27. DOI: 10.1186/1745-0179-2-27
  61. Songul Yalcın S., Durmusoglu-Sendogdu M., Gumruk F., Unal S., Kargıw E. and Tugrul B. (2007). Evaluation of the Children With b-Thalassemia in Terms of Their Self-concept, Behavioral, and Parental Attitudes. Journal of Pediatric Hematology/Oncology, 29: 523- 528. DOI: 10.1097/MPH.0b013e3180f61b56
  62. Surapolchai P., Satayasai W., Sinlapamongkolkul P. and Udomsubpayakul U. (2010). Biopsychosocial Predictors of Health-Related Quality of Life in Children with Thalassemia in Thammasat University Hospital. Journal of Medical Association in Thailand, 93 (7): 65-75.
  63. Thavorncharoensap M., Torcharus K., Nuchprayoon I., Riewpaiboon A., Indaratna K. and Ubol B. (2010) Factors affecting health-related quality of life in Thai children with thalassemia. BMC Blood Disorders, 10: 12-15. DOI: 10.1186/1471-2326-10-1
  64. Tsiantis J. (1990). Family reactions and relationships in Thalassaemia. Annals of the New York Academy of Sciences, 612: 451-61. DOI: 10.1111/j.1749-6632.1990.tb24332.x
  65. Tsiantis J., Dragonas Th., Richardson C., Anastasopoulos D., Masera G. and Spinetta J. (1996). Psychosocial problems and adjustment of children with beta-thalassemia and their families. European Child & Adolescent Psychiatry, 5 (4): 193-203. DOI: 10.1007/BF00538846
  66. van den Tweel X.W., Hatzmann J., Ensink E., van der Lee J.H., Peters M., Fijnvandraat K. and Grootenhuis M. (2008). Quality of life of female caregivers of children with sickle cell disease: a survey. Haematologica, 93 (4): 588-593. DOI: 10.3324/haematol.11610
  67. Vichinsky E., Pakbaz Z., Onyekwere O., Porter J., Swerdlow P., Coates T., Lane P., Files B., Mueller B.U., Coic L., Forni G.L., Fischer R., Marks P., Rofail D., Abetz L. and Baladi J.F. (2008). Patient-Reported Outcomes of Deferasirox versus Deferoxamine in Sickle Cell Disease Patients with Transfusional Hemosiderosis. Acta Haematologica, 119 (3): 133-141. DOI: 10.1159/000125550
  68. Woo R., Giardino P. and Hilpartner M. (1984). A psychosocial needs assessment of patients with homozygous B-thalassaemia. Annals of New York Academy of Science, 445: 316- 322. DOI: 10.1111/j.1749-6632.1985.tb17201.x
  69. Zani B., Di Palma A. and Vullo C. (1995). Psychosocial aspect of chronic illness in adolescents with thalassaemia major. Journal of Adolescente Health, 18: 387-402. DOI: 10.1006/jado.1995.1029

Marco Bani, Francesca Barile, Umberto Mazza, La dimensione psicosociale nella cura della talassemia: una rassegna della letteratura in "PSICOLOGIA DELLA SALUTE" 1/2013, pp 5-26, DOI: 10.3280/PDS2013-001001