Hemophilia without prophylaxis: Assessment of joint range of motion and factor activity

Michael Wang, Michael Recht, Neeraj N. Iyer, David L. Cooper, J. Michael Soucie

Research output: Contribution to journalArticlepeer-review

Abstract

Background: Recurrent joint bleeding in hemophilia results in arthropathy and functional impairment. The relationship of arthropathy development and factor activity (FA) has not been reported in patients with FA levels <15%-20%. Methods: During the Centers for Disease Control and Prevention Universal Data Collection, joint range-of-motion (ROM) measurements were taken at each comprehensive visit. Data were extracted from male patients with hemophilia (PWH) age ≥2 years with baseline factor activity levels ≤40%, excluding those prescribed prophylaxis, and used to calculate a proportion of normal ROM (PN-ROM) measure. Data were analyzed using regression models. Results: There were 6703 eligible PWH with 30 102 visits. PN-ROM declined with increasing age, and was associated with hemophilia severity, race/ethnicity, obesity, and viral illnesses. PWH ≥30 years old with fFA ≤2% and those ≥50 years old with FA ≤5% had mean PN-ROM values >10% less than controls; those ≥40 years old with FA <1% had values >20% less than controls. In the multivariable analysis, subjects with <1% FA had a 0.43% greater decrease (−0.49 to −0.37, 95% confidence interval) in PN-ROM each year relative to those with 16%-40% factor activity. A less pronounced effect was seen with 1%-5% or 6%-9% FA. Conclusion: The effect of FA on ROM loss is far greater than that of any of the other characteristics, especially with FA <10%. This emphasizes the need to maintain a high index of suspicion for arthropathy in individuals with moderate and low-mild hemophilia.

Original languageEnglish (US)
Pages (from-to)1035-1045
Number of pages11
JournalResearch and Practice in Thrombosis and Haemostasis
Volume4
Issue number6
DOIs
StatePublished - Aug 1 2020

Keywords

  • arthropathy
  • clotting factor
  • hemophilia
  • joint disease
  • range of motion

ASJC Scopus subject areas

  • Hematology

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