Official publication of Rawalpindi Medical University
Adult Hemophiliacs in Pakistan: Health-Related Quality of Life and Psychological Aspects

Supplementary Files

PDF

Keywords

Hemophilia, psychological factors, Pain

How to Cite

1.
Yaqoob E, Mushahid Z, Asif MA, Ishfaq M, Sabah M, Waseem M. Adult Hemophiliacs in Pakistan: Health-Related Quality of Life and Psychological Aspects. JRMC [Internet]. 2023 Apr. 1 [cited 2024 Apr. 20];27(1). Available from: https://journalrmc.com/index.php/JRMC/article/view/2043

Abstract

Introduction: Coagulation factor VIII (FVIII) (in hemophilia A) or IX (FIX) (in hemophilia B) deficiency associated with the X chromosome is the primary cause of hemophilia. Bleeding carried on by hemophilia can have an impact on a patient's mental health and quality of life. Recurrent bleeds in their muscles and joints cause hemophilia patients to experience constant pain. Certain variables, such as depression brought on by low self-esteem in comparison to "normal" siblings and peers, may have an impact on hemophilia patients. 

Objective: The goal of this investigation is to assess how psychological factors and pain affect adult hemophiliac patients' well-being.

Materials and Methods: On 102 hemophiliacs, a cross-sectional study was done. Data were gathered using visual analogue scales for pain, depression anxiety stress assessments, and quality of life questionnaires specific to hemophilia. Regression analysis, analysis of covariance, and correlation coefficients were used to analyze the data. Results: According to the findings, 70.8% of hemophiliac patients felt pain on the research day. Patients with hemophilia had an average HRQoL score of 50.08 (standard deviation: 18.49). Additionally, stress, worry, and mild to serious depression were present in 45.7 %, 63.5% and 58.3% of the patients, respectively. Additionally, a substantial correlation between HRQoL and sadness, anxiety, and pain intensity were found. The study variables were able to explain 47% of changes in HRQOL in the regression model, with depression and pain intensity serving as important predictors. Conclusion: To enhance the psychosocial health and quality of life of patients, emphasis should be given to these factors.

 

https://doi.org/10.37939/jrmc.v27i1.2043

References

White GC, Rosendaal F, Aledort LM, Lusher JM, Rothschild C, Ingerslev J. Definitions in hemophilia. Thrombosis and haemostasis. 2001;85(03):560

.

Fischer K, De Kleijn P, Negrier C, Mauser‐Bunschoten EP, van der Valk PR, van Galen KP, Willemze A, Schutgens R. The association of haemophilic arthropathy with Health‐Related Quality of Life: a post hoc analysis. Haemophilia. 2016 Nov;22(6):833-40.

Witkop M, Neff A, Buckner TW, Wang M, Batt K, Kessler CM, Quon D, Boggio L, Recht M, Baumann K, Gut RZ. Self‐reported prevalence, description and management of pain in adults with haemophilia: methods, demographics and results from the Pain, Functional Impairment, and Quality of life (P‐FiQ) study. Haemophilia. 2017 Jul;23(4):556-65.

Roosendaal G, Jansen NW, Schutgens R, Lafeber FP. Haemophilic arthropathy: the importance of the earliest haemarthroses and consequences for treatment. Haemophilia. 2008 Nov;14:4-10.

Saulyte Trakymiene S, Clausen N, Poulsen LH, Ingerslev J, Rageliene L. Progression of haemophilic arthropathy in children: a Lithuanian–Danish comparative study. Haemophilia. 2013 Mar;19(2):212-8.

Bullinger M, von Mackensen S. Psycho‐social determinants of quality of life in children and adolescents with haemophilia—a cross‐cultural approach. Clinical psychology & psychotherapy. 2008 May;15(3):164-72.

Hartl HK, Reitter S, Eidher U, Ramschak H, Ay C, Pabinger I. The impact of severe haemophilia on the social status and quality of life among Austrian haemophiliacs. Haemophilia. 2008 Jul;14(4):703-8.

Solovieva S, Santavirta N, Santavirta S, Konttinen YT. Assessing quality of life in individuals with hereditary blood coagulation disorders. Quality of Life Research. 2004 Jun;13(5):987-1000.

Talaulikar D, Shadbolt B, McDonald A, Pidcock M. Health‐related quality of life in chronic coagulation disorders. Haemophilia. 2006 Nov;12(6):633-42.

Poon JL, Zhou ZY, Doctor JN, Wu J, Ullman MM, Ross C, Riske B, Parish KL, Lou M, Koerper MA, GWADRY‐SRIDHAR F. Quality of life in haemophilia A: hemophilia utilization Group Study Va (HUGS‐Va). Haemophilia. 2012 Sep;18(5):699-707.

Bastani P, Pourmohamadi K, Karimi M. Quality of life in hemophilia complicated by inhibitors. Iranian Red Crescent Medical Journal. 2012 Apr;14(4):250.

Handin RI, Lux SE, Stossel TP, editors. Blood: principles and practice of hematology. Lippincott Williams & Wilkins; 2003.

Barr RD, Saleh M, Furlong W, Horsman J, Sek J, Pai M, Walker I. Health status and health‐related quality of life associated with hemophilia. American journal of hematology. 2002 Nov;71(3):152-60.

Hassan TH, Badr MA, Fattah NR, Badawy SM. Assessment of musculoskeletal function and mood in haemophilia A adolescents: a cross‐sectional study. Haemophilia. 2011 Jul;17(4):683-8.

.

Siboni SM, Mannucci PM, Gringeri A, Franchini M, Tagliaferri A, Ferretti M, Tradati FC, Santagostino E, Von Mackensen S, Italian Association of Haemophilia Centres (AICE). Health status and quality of life of elderly persons with severe hemophilia born before the advent of modern replacement therapy. Journal of Thrombosis and Haemostasis. 2009 May;7(5):780-6.

Witkop M, Guelcher C, Forsyth A, Hawk S, Curtis R, Kelley L, Frick N, Rice M, Rosu G, Cooper DL. Treatment outcomes, quality of life, and impact of hemophilia on young adults (aged 18–30 years) with hemophilia. American journal of hematology. 2015 Dec;90:S3-10.

Kodra Y, Cavazza M, Schieppati A, De Santis M, Armeni P, Arcieri R, Calizzani G, Fattore G, Manzoli L, Mantovani L, Taruscio D. The social burden and quality of life of patients with haemophilia in Italy. Blood Transfusion. 2014;12(S3):s567-75.

Canclini M, Zanon E, Girolami A. Factors which may influence coping with disease in haemophilia patients. Haemophilia. 2004 Sep;10(5):675-.

Rambod M, Sharif F, Molazem Z, Khair K, von Mackensen S. Health-related quality of life and psychological aspects of adults with hemophilia in Iran. Clinical and Applied Thrombosis/Hemostasis. 2018 Oct;24(7):1073-81.

Forsyth AL, Witkop M, Lambing A, Garrido C, Dunn S, Cooper DL, Nugent DJ. Associations of quality of life, pain, and self-reported arthritis with age, employment, bleed rate, and utilization of hemophilia treatment center and health care provider services: results in adults with hemophilia in the HERO study. Patient preference and adherence. 2015;9:1549.

Rambod M, Sharif F, Molazem Z, Khair K. Pain: the voiceless scream in every haemophilia patient’s life. The Journal of Haemophilia Practice. 2016 Jan 25;3(1):8-13.

Rambod M, Sharif F, Molazem Z, Khair K. Pain experience in hemophilia patients: a hermeneutic phenomenological study. International journal of community based nursing and midwifery. 2016 Oct;4(4):309.

Dorgalaleh A, Dadashizadeh G, Bamedi T. Hemophilia in Iran. Hematology. 2016 May 27;21(5):300-10.

Dolatkhah R, Fakhari A, Pezeshki MZ, Shabanlouei R, Tavassoli N, Gholchin M. Social determinants and health‐related dimensions of quality of life in adult patients with haemophilia. Haemophilia. 2014 May;20(3):376-81.

Ghanizadeh A, Baligh‐Jahromi P. Depression, anxiety and suicidal behaviour in children and adolescents with Haemophilia. Haemophilia. 2009 Mar;15(2):528-32.

Holstein K, Klamroth R, Richards M, Carvalho M, PÉREZ‐GARRIDO R, Gringeri A, European Haemophilia Therapy Standardization Board. Pain management in patients with haemophilia: a European survey. Haemophilia. 2012 Sep;18(5):743-52.

Curtis R, Baker J, Riske B, Ullman M, Niu X, Norton K, Lou M, Nichol MB. Young adults with hemophilia in the US: demographics, comorbidities, and health status. American journal of hematology. 2015 Dec;90:S11-6.

Rambod M, Forsyth K, Sharif F, Khair K. Assessment and management of pain in children and adolescents with bleeding disorders: a cross‐sectional study from three haemophilia centres. Haemophilia. 2016 Jan;22(1):65-71.

Auerswald G, Dolan G, Duffy A, Hermans C, Jiménez-Yuste V, Ljung R, Morfini M, Lambert T, Šalek SZ. Pain and pain management in haemophilia. Blood Coagulation & Fibrinolysis. 2016 Dec;27(8):845.

Williams LJ, Pasco JA, Jacka FN, Dodd S, Berk M. Pain and the relationship with mood and anxiety disorders and psychological symptoms. Journal of psychosomatic research. 2012 Jun 1;72(6):452-6.

Dersh J, Polatin PB, Gatchel RJ. Chronic pain and psychopathology: research findings and theoretical considerations. Psychosomatic medicine. 2002 Sep 1;64(5):773-86.

Curtis R, Baker J, Riske B, Ullman M, Niu X, Norton K, Lou M, Nichol MB. Young adults with hemophilia in the US: demographics, comorbidities, and health status. American journal of hematology. 2015 Dec;90:S11-6.

Mercan A, Sarper N, Inanır M, Mercan HI, Zengin E, Kılıç SC, Gökalp AS. Hemophilia-Specific Quality of Life Index (Haemo-QoL and Haem-A-QoL questionnaires) of children and adults: result of a single center from Turkey. Pediatric hematology and oncology. 2010 Aug 27;27(6):449-61.

Ferreira AA, Leite IC, Bustamante-Teixeira MT, Corrêa CS, Cruz DT, Rodrigues DD, Ferreira MC. Health-related quality of life in hemophilia: results of the Hemophilia-Specific Quality of Life Index (Haem-a-Qol) at a Brazilian blood center. Revista Brasileira de Hematologia e Hemoterapia. 2013;35:314-8.

Varaklioti A, Kontodimopoulos N, Katsarou O, Niakas D. Psychometric properties of the Greek Haem-A-QoL for measuring quality of life in Greek haemophilia patients. BioMed Research International. 2014 Jan 1;2014.

Von Mackensen S, Eldar‐Lissai A, Auguste P, Krishnan S, Von Maltzahn R, Yu R, Wyrwich KW. Measurement properties of the Haem‐A‐QoL in haemophilia clinical trials. Haemophilia. 2017 May;23(3):383-91.

Varaklioti A, Kontodimopoulos N, Niakas D, Kouramba A, Katsarou O. Health-related quality of life and association with arthropathy in Greek patients with hemophilia. Clinical and Applied Thrombosis/Hemostasis. 2018 Jul;24(5):815-21.

Weigel N, Carlson BR. Physical activity and the hemophiliac: yes or no?. American corrective therapy journal. 1975;29(6):197-205.

Von Mackensen S. Quality of life and sports activities in patients with haemophilia. Haemophilia. 2007 Sep;13:38-43.

Buzzard BM. Physiotherapy, rehabilitation and sports in countries with limited replacement coagulation factor supply. Haemophilia. 2007 Sep;13:44-6.

Logan FA, Maclean A, Howie CA, Gibson B, Hann IM, Parry-Jones WL. Psychological disturbance in children with haemophilia. British medical journal. 1990 Dec 1;301(6763):1253-6.

Khatwa SA, Abdou MH. Adult depression in Alexandria, Egypt, 1998. The Journal of the Egyptian Public Health Association. 1999 Jan 1;74(3-4):333-52.

Elander J, Morris J, Robinson G. Pain coping and acceptance as longitudinal predictors of health‐related quality of life among people with haemophilia‐related joint pain. European Journal of Pain. 2013 Jul;17(6):929-38.

Al-Gamal E. Quality of life, anxiety and depression among patients with chronic obstructive pulmonary disease and their spouses. Issues in mental health nursing. 2014 Oct 1;35(10):761-7

Creative Commons License

This work is licensed under a Creative Commons Attribution-ShareAlike 4.0 International License.

Copyright (c) 2023 Eesha Yaqoob, Zonaira Mushahid, Muhammad Asad Asif, Muhammad Ishfaq, Maheen Sabah, Mishal Waseem