Skip to main navigation menu Skip to main content Skip to site footer

Admin menu

Volume 9 (2022): Issue 1

New challenges for an expanding generation of older persons with haemophilia

Abstract

Abstract Background

Increasing survival among people with haemophilia means that more individuals are at risk of developing age-related morbidity. Little is known about morbidity and health-related quality of life (HRQoL) in different age groups within a single large population of people with haemophilia.

Aim

This study aimed to explore the association between increasing age and comorbidity among people with haemophilia and to compare their HRQoL with that of a sample of the general population in England.

Methods

The prevalence of comorbidity recorded in medical records and HRQoL assessed by EQ-5D were compared by age group in participants in the Cost of Haemophilia in Europe: A Socioeconomic Survey study (CHESS) in Europe. HRQoL was compared with that of a sample of the general population taken from the 2012 Health Survey for England (HSE).

Results

Younger adults in CHESS were more likely to have received prophylaxis from an early age. The mean number of affected joints in younger adults was 1.0; participants aged 41–50 (1.25) and 51–60 years (1.41) had the highest mean number of affected joints. The prevalence of comorbidity was 36% in patients aged 18–30, 61% in 31–60-year-olds and 68% in those aged 61+. HRQoL impairment in young adults with haemophilia was comparable with that in the HSE population aged over 60.

Conclusions

Older people with haemophilia have impaired quality of life compared with younger adults and an increasing prevalence of several age-related disorders affecting mental health and cardiovascular and bone health. Young adults with haemophilia report impaired HRQoL comparable with that in a general population aged 61+.

Article

View Full Article

References

  • 1. Blanchette VS, Key NS, Ljung LR, et al.; Subcommittee on Factor VIII, Factor IX and Rare Coagulation Disorders of the Scientific and Standardization Committee of the International Society on Thrombosis and Hemostasis. Definitions in hemophilia: communication from the SSC of the ISTH. J Thromb Haemost 2014; 12: 1935–9. doi: https://doi.org/10.1111/jth.12672.
  • 2. Siboni SM, Mannucci PM, Gringeri A, et al.; 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. J Thromb Haemost 2009; 7: 780–786. doi: https://doi.org/10.1111/j.1538-7836.2009.03318.x.
  • 3. Oladapo AO, Epstein JD, Williams E, et al. Health-related quality of life assessment in haemophilia patients on prophylaxis therapy: a systematic review of results from prospective clinical trials. Haemophilia 2015; 21: e344–e358. doi: https://doi.org/10.1111/hae.12759.
  • 4. Escobar M, Sallah S. Hemophilia A and hemophilia B: focus on arthropathy and variables affecting bleeding severity and prophylaxis. J Thromb Haemost 2013; 11: 1449–1453. doi: https://doi.org/10.1111/jth.12317.
  • 5. Mannucci PM, Iacobelli M. Progress in the contemporary management of hemophilia: The new issue of patient aging. Eur J Intern Med 2017; 43: 16–21. doi: https://doi.org/10.1016/j.ejim.2017.05.012
  • 6. Chu WM, Ho HE, Wang JD, et al. Risk of major comorbidities among workers with hemophilia: A 14-year population-based study. Medicine (Baltimore) 2018; 97(6): e9803. doi: https://doi.org/10.1097/MD.0000000000009803.
  • 7. Wang JD, Chan WC, Fu YC, et al. Prevalence and risk factors of atherothrombotic events among 1054 hemophilia patients: a population-based analysis. Thromb Res 2015; 135: 502–7. doi: https://doi.org/10.1016/j.thromres.2014.12.027
  • 8. O’Hara J, Hughes D, Camp C, et al. The cost of severe haemophilia in Europe: the CHESS study. Orphanet J Rare Dis 2017; 12: 1–8. doi: https://doi.org/10.1186/s13023-017-0660-y.
  • 9. NatCen Social Research, University College London. Department of Epidemiology and Public Health. (2014). Health Survey for England, 2012. [data collection]. UK Data Service. SN: 7480. doi: https://doi.org/10.5255/UKDA-SN-7480-1.
  • 10. EuroQol Group. EuroQol-a new facility for the measurement of health-related quality of life. Health Policy (New York) 1990; 16(3): 199–208. doi: https://doi.org/10.1016/0168-8510(90)90421-9
  • 11. Kind P, Hardman G, Macran S. UK Population Norms for EQ-5D. Discussion paper 172. York: Centre for Health Economics. 1999. Available from https://www.york.ac.uk/media/che/documents/papers/discussionpapers/CHE%20Discussion%20Paper%20172.pdf (accessed 9 October 9 2019).
  • 12. R Core Team. The R Project for Statistical Computing. 2017. Available from https://www.r-project.org.
  • 13. Stonebraker JS, Bolton-Maggs PHB, Michael Soucie J, Walker I, Brooker M. A study of variations in the reported haemophilia A prevalence around the world. Haemophilia 2010; 16: 20–32. doi: https://doi.org/10.1111/j.1365-2516.2009.02127.x
  • 14. Stonebraker JS, Bolton-Maggs PHB, Michael Soucie J, Walker I, Brooker M. A study of variations in the reported haemophilia B prevalence around the world. Haemophilia 2012; 18: e91–e94. doi: https://doi.org/10.1111/j.1365-2516.2011.02588.x
  • 15. Berntorp E, Dolan G, Hay C, et al. European retrospective study of real-life haemophilia treatment. Haemophilia 2017; 23: 105–114. doi: https://doi.org/10.1111/hae.13111
  • 16. Konkle BA. Clinical challenges within the aging hemophilia population. Thromb Res 2011; 127: S10–S13. doi: https://doi.org/10.1016/j.thromres.2010.10.004.
  • 17. Philipp C. The aging patient with hemophilia: complications, comorbidities, and management issues. Hematology 2010; 191–196. doi: https://doi.org/10.1182/asheducation-2010.1.191

PDF Download

Download PDF

Open in full-page viewer

Authors

  • Jamie O’Hara

    ORCID iD
    jamie@hcdeconomics.com
    University of Chester ; HCD Economics, UK .
  • Declan Noone

    ORCID iD
  • Persefoni Kritikou

    Swedish Orphan Biovitrum AB, Sweden
  • Karl-Johan Myren

    ORCID iD
    Swedish Orphan Biovitrum AB, Sweden
  • Steve Chaplin

    ORCID iD
    HCD Economics, UK
  • Daniel P Hart

    ORCID iD
    The Royal London Hospital Haemophilia Centre, Barts and the London School of Medicine and Dentistry, QMUL, UK