Volume 1 (2014): Issue 3

Joint assessment in haemophilia – current physiotherapist practice in the UK

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Paul McLaughlin
Melanie Bladen
Mike Holland
Kate Khair

Abstract

Abstract Aim

UK guidelines recommend regular assessment of joint status in children with haemophilia using a standardised tool performed by haemophilia physiotherapists. We surveyed UK physiotherapists working in haemophilia care regarding their current practice with respect to joint scoring.

Methods

A survey was posted on SurveyMonkey and all haemophilia physiotherapists practising in the UK were invited to respond. Responses were analysed and discussed at a roundtable meeting attended by invited physiotherapists and specialist haemophilia nurses.

Results

In all, responses were received from 29 of the estimated 37 physiotherapists in the UK who see haemophilia patients. Both the survey and subsequent discussion reflected agreement that joint scoring offers a valuable tool to clinicians, but that better ways of assessing joint health were needed. There was enthusiasm for combining joint scoring with systematic and validated patient-reported outcome measures.

Conclusion

Greater understanding is needed of the relationship between joint scores and measures of physical function and quality of life.

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References

  • 1. Poonnoose PM, Manigandan C, Thomas R, et al. Functional Independence Score in Haemophilia: a new performance-based instrument to measure disability. Haemophilia 2005; 11: 598-602.
  • 2. van Genderen FR, Fischer K, Heijnen L, et al. Pain and functional limitations in patients with severe haemophilia. Haemophilia 2006; 12: 147-53.
  • 3. Roosendaal G, Tekoppele JM, Vianen ME, et al. Articular cartilage is more susceptible to blood induced damage at young than at old age. J Rheumatol 2000; 27: 1740-4.
  • 4. Manco-Johnson MJ, Abshire TC, Shapiro AD, et al. Prophylaxis versus episodic treatment to prevent joint disease in boys with severe hemophilia. N Engl J Med 2007; 357: 535-44.
  • 5. Liesner RJ, Khair K, Hann IM. The impact of prophyactic treatment on children with severe haemophilia. Br J Haematol 1996; 92: 973-8.
  • 6. Srivastava A, Brewer AK, Mauser-Bunschoten EP, et al. Guidelines for the management of hemophilia. Haemophilia 2013; 19(1): e1-47.
  • 7. Richards M, Williams M, Chalmers E, et al. A United Kingdom Haemophilia Centre Doctors' Organization guideline approved by the British Committee for Standards in Haematology: guideline on the use of prophylactic factor VIII concentrate in children and adults with severe haemophilia A. Br J Haematol 2010; 149: 498-507.
  • 8. Feldman BM, Funk S, Bergstrom B-M, et al. Validation of a new pediatric joint scoring system from the International Hemophilia Prophylaxis Study Group: Validity of the Hemophilia Joint Health Score (HJHS). Arthritis Care & Research 2011; 63: 223-30.
  • 9. Bladen M, Main E, Hubert N, et al. Factors affecting the Haemophilia Joint Health Score in children with severe haemophilia. Haemophilia 2013; 19: 626-31.
  • 10. Hilliard P, Funk S, Zourikian N, et al. Hemophilia joint health score reliability study. Haemophilia 2006; 12: 518-25.
  • 11. Fischer K, de Kleijn P. Using the Haemophilia Joint Health Score for assessment of teenagers and young adults: exploring reliability and validity. Haemophilia 2013; 19: 944-50.
  • 12. Chen L, Sun J, Wu R, et al. Joint health status of Chinese hemophilia children: a pilot study using the Hemophilia Joint Health Assessment Scale (HJHS). Haemophilia 2008; 14 Suppl 2: 79.
  • 13. Den Uijl IE, Biesma D, Grobbee D, Fischer K. Outcome in moderate haemophilia. Blood Transfus 2012; 1-7.
  • 14. Den Uijl IE, de Schepper AM, Camerlinck M, et al. Magnetic resonance imaging in teenagers and young adults with limited haemophilic arthropathy: baseline results from a prospective study. Haemophilia 2011; 17: 926-30.
  • 15. Khawaji M, Astermark J, Berntorp E. Lifelong prophylaxis in a large cohort of adult patients with severe haemophilia: a beneficial effect on orthopaedic outcome and quality of life. Eur J Haematol 2012; 88: 329-35.
  • 16. Saulyte Trakymiene S, Ingerslev J, Rageliene L. Utility of the Haemophilia Joint Health Score in study of episodically treated boys with severe haemophilia A and B in Lithuania. Haemophilia 2010;16: 479-86.
  • 17. van der Net J, Vos RC, Engelbert RH, et al. Physical fitness, functional ability and quality of life in children with severe haemophilia: a pilot study. Haemophilia. 2006;12: 494-9.
  • 18. Groen WG, van der Net J, Helders PJ, Fischer K. Development and preliminary testing of a Paediatric Version of the Haemophilia Activities List (Pedhal). Haemophilia 2010; 16: 281-9.
  • 19. Groen W, Van Der Net J, Lacatusu AM, et al. Functional limitations in Romanian children with haemophilia; further testing of psychometric properties of the Paediatric Haemophilia Activities List. Haemophilia 2103; 19: e116-125
  • 20. van Genderen FR, Van Meeteren NL, Van Der Bom JG et al. Functional consequences of haemophilia in adults: the development of the Haemophilia Activities List. Haemophilia 2004; 10: 565–71.
  • 21. van Genderen FR, Westers P, Heijnen L, et al. Measuring patients’ perceptions on their functional abilities: validation of the Haemophilia Activities List. Haemophilia 2006; 12: 36–46.
  • 22. von Mackensen S, Bullinger M. Development and testing of an instrument to assess the quality of life of children with haemophilia in Europe (Haemo-QoL). Haemophilia 2004; 10 Suppl 1: 17-25.
  • 23. Pollak E, Mühlan H, von Mackensen S, et al. The Haemo-QoL Index: developing a short measure for health-related quality of life assessment in children and adolescents with haemophilia. Haemophilia 2006; 12: 384-92.
  • 24. von Mackensen S, Czepa D, Herbsleb M, Hilberg T. Development and validation of a new questionnaire for the assessment of subjective physical performance in adult patients with haemophilia--the HEP-Test-Q. Haemophilia 2010; 16: 170-8.
  • 25. Haemophilia Alliance. A National Service Specification for Haemophilia and other Inherited Bleeding Disorders (2nd edition). 2006. Available from http://www.ukhcdo.org/docs/HaemAlliance-NatSvsSpec2006.pdf
  • 26. McLaughlin P, Chowdary P. Correlation of Haemophilia Activities List (HAL) and musculoskeletal physical assessment in a cohort of 95 severe hemophilia patients. 13th International Musculoskeletal Congress. Chicago, April 2013. Abstract B4.6
  • 27. Doward LC, McKenna SP, Defining Patient-Reported Outcomes. Value in Health 2004; 7(S1): S4-S8.
  • 28. Khair K, Bladen M, Holland M. Physical function and quality of life in adolescents with haemophilia (SO-FIT study). The Journal of Haemophilia Practice 2014;1(2):1114.

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Authors

  • Paul McLaughlin

    p.mclaughlin@nhs.net
    Clinical Specialist Physiotherapist in Haemophilia, Katharine Dormandy Haemophilia Centre and Thrombosis unit, Royal Free Hospital NHS Foundation Trust, Pond St, London, UK NW3 2QG.

  • Melanie Bladen

    Physiotherapist, Great Ormond Street Hospital for Children NHS Foundation Trust, London WC1N 3JH UK

  • Mike Holland

  • Kate Khair

    Consultant Nurse, Great Ormond Street Hospital for Children NHS Foundation Trust, London WC1N 3JH; Visiting Professor of Health and Social Care, London South Bank University, UK