Changes in joint health were evaluated1

In a post hoc study of patients ≥ 12 years of age treated with ELOCTATE prophylaxis

Study Design

Study Design1

  • This post hoc study assessed change in joint health among 47 adult and adolescent males (≥12 years of age) with severe hemophilia A who were treated with ELOCTATE prophylaxis
  • Subjects completed the A-LONG pivotal trial, enrolled in the ASPIRE extension study (data cutoff of December 8, 2014), and had available mHJHS data
  • Forty-seven subjects had mHJHS data at all 4 time points (A-LONG baseline, ASPIRE baseline, Year 1, Year 2)
  • Joint health assessments included the change in mHJHS from A-LONG baseline to follow-up visits and comparisons of individual mHJHS domains. In addition, factors such as target joints, weight-bearing and non–weight-bearing joints, and prestudy dosing regimen were assessed for impact on mHJHS. Negative values indicate improvement

Study Limitations1

  • Data are reported from a post hoc analysis that was not powered to show statistical significance. There was a small sample size, no control group, and potential for interobserver variability
  • The mHJHS is not yet a validated tool
  • Further studies are needed to confirm these findings as well as the effect of improvements in joint health on overall improvement in quality of life
mHJHS Scoring Table

Scoring range

Domain HJHS mHJHS
Duration 0-1 0-1
Swelling 0-3 0-3
Muscle atrophy 0-2 0-2
Flexion loss 0-3 0-3
Extension loss 0-3 0-3
Strength 0-4 0-4
Crepitus of motion 0-2 0-1
Joint pain 0-2 0-1
Instabilitya - 0-1
Global gaitb 0-4 0-2
Total scorec 0-124 0-116

aInstability was scored the same as in the old version of the HJHS: 0=none and 1=significant pathologic joint laxity.1

bGlobal gait was scored as follows: 0=no difficulty with walking or climbing up/down stairs; 1=no difficulty with walking, but difficulty with stairs; 2=difficulty with walking and with stairs.1

cJoint scoring was performed separately for 6 joints (left and right ankle, left and right elbow, left and right knee) with a score range of 0 to 19 plus the gait score (0–2). Score of 0=normal; score of 116=most severe disease.1

HJHS=Hemophilia Joint Health Score; mHJHS=modified Hemophilia Joint Health Score.

Published in Haemophilia

See the research:

Improved joint health in subjects with severe haemophilia A treated prophylactically with recombinant factor VIII Fc fusion protein

Oldenburg J, Kulkarni R, Srivastava A, et al. Haemophilia. 2018;24(1):77-84.

TAKE ME TO THE ARTICLE

See the research:

Improved joint health in subjects with severe haemophilia A treated prophylactically with recombinant factor VIII Fc fusion protein

Oldenburg J, Kulkarni R, Srivastava A, et al. Haemophilia. 2018;24(1):77-84.

TAKE ME TO THE ARTICLE

Swelling, range of motion, and strength components
contributed most to changes in joint health scores1

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MAT-US-2003075-v5.0-03/2022 Last Updated: March 2022