Skip to main content

Effect of joint range of motion on health-related quality of life in children with hemophilia



Assessment of quality of life (QOL) in patients with hemophilia is important for the disease outcome. In patients with hemophilia, repeated occurrences of hemarthrosis lead to limitations in range of motion (ROM) of major joints.


The aim of this work is to assess joint ROM and detect the presence of limitation of joint ROM in patients with hemophilia and their effect on the QOL in these patients.

Patients and methods

The study included 25 children with hemophilia recruited from Pediatric Hematology Unit, Fayoum University Hospital, during the period from June 2016 to December 2016. Their ages ranged between 4 and 16 years. Large joint examination and measurement of joint ROM were done at rheumatology clinic using goniometry by rheumatologist. QOL was assessed by using the hemophilia QOL questionnaire.


The most impaired dimensions of QOL were family, treatment, and physical domains. Total health-related quality of life (HRQOL) score, physical health, view of self, and sport and school scores were found worse with increasing age. Patients who had target joints had poor QOL score. Limitation of movement (LOM) of the knee and ankle joints mainly impaired the physical and social aspects and the overall HRQOL, and LOM of shoulder joint impaired the treatment domain.


Age of the patient was found a factor affecting the total HRQOL score, in addition to physical, view of self, and sport and school dimensions. LOM of the knee and ankle joints mainly impaired physical and social aspects and the overall HRQOL.


  1. Franchini M, Mannucci PM. Past, present and future of hemophilia: a narrative review. Orphanet J Rare Dis 2012; 7:24.

    Article  Google Scholar 

  2. Lobet S, Hermans C, Lambert C. Optimal management of hemophilic arthropathy and hematomas. J Blood Med 2014; 5:207–218.

    Article  Google Scholar 

  3. Roosendaal G, Lafeber FP. Blood induced joint damage in hemophilia. Semin Thromb Hemost 2003; 29:37–42.

    Article  Google Scholar 

  4. Soucie JM, Cianfrini C, Janco RL, Kulkarni R, Hambleton J, Evatt B, et al. Joint range of motion limitations among young males with hemophilia. Blood 2004; 103:2467–2473.

    Article  CAS  Google Scholar 

  5. Bullinger M, Von Mackensen S. Quality of life assessment in hemophilia. Hemophilia 2004; 10:26–33.

    Article  Google Scholar 

  6. TahaM, Hassan M. Health related quality of life in children and adolescents with hemophilia in Basra,Southern Iraq. J Pediatr Hematol Oncol 2014; 36:179–184.

    Article  Google Scholar 

  7. Pollak E, Mu Hlan H, Von Mackensen S, Bullinger M. The Haemo Qol Index: developing a short measure for health related quality of assessment in children and adolescents with hemophilia. Hemophilia 2006; 12:384–392.

    Article  CAS  Google Scholar 

  8. Chen CM, Huang KC, Chen CC, Huang SU, Huang CE, Chen YY, et al. The impact of joint range of motion limitations on health-related quality of life in patients with haemophilia A. Haemophilia 2015; 21:176–184.

    Article  Google Scholar 

  9. Paul-Scott J, Robert R. Montgomery hereditary clotting factor deficiencies (bleeding disorders). In Kliegman RM, Behrman RE, Jenson HB, Stanton BF, eds. Nelson textbook of pediatrics. 19th ed. Philadelphia, PA: Saunders Elsevier; 2011. 470:1699–1701.

    Chapter  Google Scholar 

  10. White GC II, Rosendaal F, Aledort LM, Lusher JM, Rothschild C, Ingerslev J, Factor VIII and Factor IX Subcommittee. Definitions in hemophilia. Recommendation of the scientific subcommittee on factor VIII and factor IX of the scientific and standardization committee of the International Society on Thrombosis and Haemostasis. Thromb Haemost 2001; 85:560.

    CAS  PubMed  Google Scholar 

  11. Gringeri A, Von Mackensen S, Auerswald G, Bullinger M, Garrido R, Kllermanne E, et al. For the haemo-qol study, health status and health-related quality of life of children with haemophilia from six West European countries. Haemophilia 2004; 10:26–33.

    Article  Google Scholar 

  12. Von Mackensen S, Bullinger M, and the Haemo-QoL Group. Development and testing of an instrument to assess the quality of life of children with haemophilia in Europe (Haemo-QoL). Haemophilia 2004; 10:17–25.

    Article  Google Scholar 

  13. Norkin CC, White DJ. Measurement of joint motion: a guide to goniometry. 4th ed. Philadelphia, PA: F.A. Davis; 2009.

    Google Scholar 

  14. Cocchiarella L, Andersson GBJ. American Medical Association: guide to the evaluation of permanent impairment. 5th ed. Chicago, IL: AMA; 2001.

    Google Scholar 

  15. Karim MA, Siddique R, Jamal CY, Islam A. Clinical profile of haemophilia in children in atertiarycare hospital. Bangladesh J Child Health 2013; 37:90–96.

    Article  Google Scholar 

  16. Payal V, Sharma P, Janu Y. Joint health status of hemophilia patients in Jodhpur region. Indian J Hematol Blood Transfus 2015; 31:362–366.

    Article  Google Scholar 

  17. Abdel Ghany HM, Hassab HM, El-Noueam KI. Hemophilic arthropathy: clinical, radiologic, and functional evaluation: a single-center experience in a limited resource country. Egypt 2016; 43:35–40.

    Google Scholar 

  18. Tantawy A, Mackensen S, El-Laboudy M, Labib JH, Moftah F, El-Telbany MA, et al. Health-related quality of life in Egyptian children and adolescents with hemophilia A. Pediatr Hematol Oncol 2011; 28:222–229.

    Article  Google Scholar 

  19. Alhaosawi MM. Target joint ‘new concept of identification’. J Appl Hematol 2015; 6:35–38.

    Article  Google Scholar 

  20. Prejs R, deKleijn P, Grobbee DE, vandenBerg M. The effects of postponing prophylactic treatment on long-term outcome in patient with severe hemophilia. Blood 2002; 99:2337–2341.

    Article  Google Scholar 

  21. Bullinger M, von Mackensen S. Psycho-social determinants of quality of life in children and adolescents with hemophilia a cross cultural approach. Clini Psychol Psychother 2008; 15:164–172.

    Article  Google Scholar 

  22. Scalone L, Mantovani LG, Mannucci PM, Gringeri A; COCIS Study Investigators. Quality of life is associated to the orthopedic status in haemophilic patients with inhibitors. Haemophilia 2006; 12:154–162.

    Article  CAS  Google Scholar 

  23. Remor E, Young NL, Von Mackensen S, Lopatina EG. Disease specific quality of life measurement tools for hemophilia patients. Hemophilia 2004; 10:30–40.

    Article  Google Scholar 

  24. Karin K, Berntorp E. Haemophilia and joint disease: pathophysiology, evaluation and management. J Comorb 2011; 1:51–59.

    Article  Google Scholar 

  25. Royal S, Schramm E, Berntorp P, Giangrande M. Quality-of-life differences between prophylactic and on-demand factor replacement therapy in European haemophilia patients. Haemophilia 2002; 8:44–50.

    Article  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations


Corresponding author

Correspondence to Nermeen Ahmed Foaud.

Additional information

This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms.

Rights and permissions

This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

El Hawary, M.A., EL Dash, H.H., Foaud, N.A. et al. Effect of joint range of motion on health-related quality of life in children with hemophilia. Egypt Rheumatol Rehabil 46, 237–243 (2019).

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: