Haemophilia (2006), 12, 518525

DOI: 10.1111/j.1365-2516.2006.01312.x

Hemophilia joint health score reliability study

P. HIL LIARD,* S. FUNK, N. ZOURIKIAN, B.-M. BERGSTROM, C. S. BRADLEY,*
M. MCLIMONT, M. MANCO-JOHNSON, P. PETRINI, M. VAN DEN BERG** and
B. M. FELDMAN*
Departments of *Rehabilitation Service, Population Health Sciences, and the Division of Rheumatology, Department of
Pediatrics, The Hospital for Sick Children, Toronto, Canada; University of Toronto, Canada; Mountain States Regional
Hemophilia and Thrombosis Center, University of Colorado and Denver Health Sciences Center, Denver, CO, USA;
Department of Physiotherapy, Hopital Sainte Justine, Montreal, Canada; Departments of Physiotherapy and the
Division of Blood Coagulation Disorders, Pediatrics, Karolinska University Hospital, Stockholm, Sweden; and
**Van Crevald Clinic, Department of Hematology, University of Utrecht and Wilhelmina Kinderziekenhuis
(WKZ)/University Medical Center Utrecht (UMCU), The Netherlands

Summary. Measurement of joint health is critically

important when assessing children with haemophilia. Few measures exist; they lack sensitivity to
small changes, dont account for normal development and were never formally validated. To
address these concerns, the Hemophilia Joint
Health Score (HJHS) was developed by modifying
existing scores. Objective: To test the interobserver and testretest reliability of the HJHS.
Methods: Using a fully factorial design, four
physiotherapists (from Canada, the United States
and Sweden) examined eight boys with severe
haemophilia A on two consecutive days using the
HJHS. The boys ranged in age from 412 years

Introduction
Recurrent joint bleeding in persons with haemophilia
is known to lead to joint damage associated with
pain, loss of range of motion and function and longterm physical and psychosocial impairments [1,2].
Almost 80% of recurrent haemarthroses in persons
with haemophilia have been found to occur in the
elbows, knees and ankles [3]. Consequently, a key
factor in preserving these joints and improving
overall outcomes in haemophilia is to reduce the
frequency and severity of joint bleeds [4].
The use of prophylactic factor replacement reduces
the number and/or severity of bleeding episodes in
Correspondence: Pamela Hilliard, Department of Rehabilitation
Services, The Hospital for Sick Children, 555 University Ave.,
Toronto, ON M5G 1X8, Canada. Tel.: 416 813 6755 ; fax: 416
813 8557; e-mail: pamela.hilliard@sickkids.ca
Accepted after revision 16 May 2006

518

and presented with variable joint damage. Six

index joints (elbows, knees and ankles) were
assessed on 11 impairment items including swelling, flexion and extension loss and gait. Concordance was measured by the intra-class correlation
co-efficient. Results: Reliability of the HJHS was
excellent with an inter-observer co-efficient of 0.83
and a testretest of 0.89. Conclusion: This study is
the first in a series to assess the psychometric
properties of the HJHS, a promising new measure
of joint health in boys with haemophilia.
Keywords haemophilia, joint health, outcome measure, physiotherapy, reliability, scoring instrument

haemophilia, ultimately resulting in less joint damage

[5]. In a 25-year survey of prophylactic treatment of 65
patients with severe haemophilia A, Nilsson et al. [6]
concluded that fewer joint bleeds, and lower mean
orthopaedic and radiological scores correlated with
earlier, more intensive prophylactic treatment. In
addition, Heijnen et al. [7] reported that patients with
severe haemophilia who did not have the benefit of
replacement therapy from an early age had five or six
arthropathic joints by the age of 20 years. A 6-year,
multi-centre study involving 477 patients with severe
haemophilia A from the USA, Europe and Japan
demonstrated that patients on prophylaxis, defined as
the regular infusion of factor concentrates for
>46 weeks per year, had fewer bleeding episodes,
better baseline and final examination scores, significantly less progression of X-ray changes, fewer days
lost from school/work, and less time hospitalized [4].
Moreover, in a comparison of outcomes over 22 years
in two countries, Fischer [8] concluded that Dutch
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HEMOPHILIA JOINT HEALTH SCORE RELIABILITY STUDY

patients treated primarily with prophylaxis presented

with fewer joint bleeds per year, had better clinical
joint scores and less arthropathy than French patients
primarily treated on demand. Accordingly, the current
trend in treating young boys with severe haemophilia
is to use prophylactic treatment where available.
Evaluation of this treatment approach, with its
associated benefits to overall joint health, requires
measures that are capable of detecting minor changes
in joint health and function [9]. One measure
currently in existence is the World Federation of
Hemophilia (WFH) evaluation system for the haemophilic joint. This system, described by Gilbert [10]
is comprised of four parameters: pain, bleeding,
physical examination and X-ray examination. However, while this system has been the most widely used
joint assessment instrument to date [9] including the
above-mentioned studies, one of its primary shortcomings according to De Kleijn is its lack of
established reliability, validity and sensitivity to
change. The deficit of functional ability measures is
also considered significant by De Kleijn [11] and
needs to be dealt with separately.
In an attempt to address these shortcomings,
modified versions of the WFH scoring system have
been suggested the Colorado [12] and the Stockholm versions [13]. However, while these versions
are promising, haemophilia treaters worldwide are
keen to have one mutually agreed upon international
scoring instrument of joint health. In 2001, a group
of international collaborators formed the International Prophylaxis Study Group (IPSG). Their initial
focus was to explore the possibility of harmonizing
existing musculoskeletal and magnetic resonance
imaging scoring systems. The initial meeting of the
IPSG was in November 2002 in Toronto, Canada.
A subgroup of the IPSG, the Physiotherapy (PT)
Working Group, composed of physiotherapists and
physicians from Canada, the United States, Sweden
and the Netherlands first met at this time. While the
PT group identified that pain, impairment, function
and quality of life should be components of an
outcome evaluation system, it was agreed that
developing a psychometrically sound measure of
physical joint health would be its first priority. The
group agreed that the measure could be achieved by
harmonizing the existing scoring systems (WFH,
Colorado and Stockholm instruments); the initial
effort addresses assessment of children ages 4
18 years in order to focus the tool. Each of the
existing measures was reviewed in detail. Through
e-mails and conference calls, group consensus on the
elements to include in the new measure was

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519

achieved. The resulting measure is called the Hemophilia Joint Health Score (HJHS).
The HJHS is an 11-item scoring tool for assessing
joint impairment in children ages 418 years. The
most significant similarities and adaptations to the
previous scales are as follows.
From the original WFH measure [10]
Joint instability was retained (but reduced to two
points) in order to capture more significant joint
damage as opposed to variations within normal
limits in children.
From the Colorado versions [12]
The 3-point scale for swelling, gait and strength
categories that had been added to the Colorado PE-1
and Colorado PE-0.5 to increase the tools sensitivity
to evaluating mild joint change was retained; the
more age appropriate tasks (e.g. gait) for the developing child, reflected in the Child PE instrument were
maintained.
From the Stockholm instrument [13]
Duration of swelling (to capture chronic synovitis)
and degrees lost (rather than percentage loss) for
range of motion were retained.
PT working group consensus
The axial alignment category (vs. axial deformity on
other scales) was decreased to two points (within or
outside normal limits) in order to detect changes
because of joint arthropathy rather than normal
developmental variation; the moderate category for
the muscle atrophy and crepitus items was deleted in
order to reduce the likelihood of scoring error; the
range of motion and flexion contracture categories
were replaced by flexion and extension loss, recorded
in degrees (this notation better delineates loss of
hyperextension and end range flexion); a more
objective measure joint pain on over-pressure
was chosen instead of the subjective items joint pain
with and without activity.
Once completed, the expert working group postulated that the HJHS would have improved psychometric properties over its predecessors. Subsequently,
the purpose of this study was to assess the inter-rater
and testretest reliability of the HJHS. The validity
and the sensitivity of the HJHS will be addressed in
future research.

Haemophilia (2006), 12, 518525

520

P. HILLIARD et al.

Materials and methods

The Reliability Study included four physiotherapists
from three different countries and eight children with
severe haemophilia A, and was conducted over
2 days in September 2003 in Toronto, Canada.
Study participants
Subjects were chosen to reflect a range of both age and
severity of joint disease. A list of boys with severe
factor VIII or IX deficiency registered with the
Paediatric Comprehensive Care hemophilia program
at the Hospital for Sick Children in Toronto was
compiled. This list was subdivided into those 8 years
of age and those >8 years. The clinic physiotherapist
identified which index joints (elbows, knees, ankles)
for these listed patients had clinical signs of damage on
previous musculoskeletal clinical examination and
denoted them as mild/moderate (clinical findings on
13 items e.g. swelling, range of motion) or severe
(clinical findings on four or more items). From this list,
eight boys were selected to participate in the study.
One extra patient was selected for each age group in
case of illness or family emergency.
Assessors
Three of the four participating physiotherapists were
members of the Physiotherapy Expert Working
Group of the IPSG; one from Stockholm, Sweden
(13 years experience in haemophilia care), one from
Denver, USA (27 years experience in haemophilia
care), and one from Montreal, Canada (11 years
experience in haemophilia care). The fourth physiotherapist from Toronto, Canada had 5 years experience in paediatric orthopaedics and rheumatology.
Outcome measure (HJHS)
The HJHS is an 11-item scoring tool for assessing joint
impairment of the six key index joints (elbows, knees
and ankles) in children ages 418 years with haemophilia. The HJHS is shown in Appendix 1. Data for
each assessed item are scored on an ordinal categorical
scale. Each of the six index joints is assessed individually; knees and ankles are scored using all 11 items,
with a total joint score range from 0 to 26. Elbows are
scored using nine of the 11 items (gait and axial
alignment are not assessed at the elbow) for a total
joint score range from 0 to 20. A global gait score is
separately assessed from 0 indicating that all skills are
within normal limits, to 4 indicating that no skills are
within normal limits. All joint scores and the global

Haemophilia (2006), 12, 518525

gait score can then be combined to provide a total

score ranging from 0 to 148.
Explanation sheets that detail the examination
techniques and scoring criteria for the HJHS were
modified from the Mountain States Regional Hemophilia Centers explanation document. Worksheets
were developed prior to the study, for the purpose of
recording raw data during patient examination that
would be converted into a score at the end of each
evaluation. Explanation documents and worksheets
are available from the corresponding author.
Study design
A half-day training session for the study physiotherapists was held one day prior to the study. Each of the
11 items of the HJHS was reviewed, along with the
accompanying explanation document and worksheets, to communicate instructions for examining
and scoring the joints. The study physiotherapists, in
order to clarify positioning and techniques for examination assessed two boys with severe haemophilia.
Minor modifications were subsequently made to the
explanation and worksheets to improve clarity.
During the two study days, the boys were divided
into two groups by age: four boys 8 years and four
boys >8 years. The younger boys were scheduled in
session 1 (early morning) and session 3 (early
afternoon), whereas the older boys were scheduled
in session 2 and session 4 (later in the morning and
afternoon). Each physiotherapist examined two boys
in each session in a randomly assigned order. Each
boy was then examined by each physiotherapist in
the same order on day 2 using a fully factorial study
design. The Hospital for Sick Children hemophilia
clinic physiotherapist monitored the flow of patients,
time allotment per patient, proper completion of
worksheets and timely completion of scoring sheets.
To assure patient safety, the haemophilia clinic nurse
coordinator was present during the study; she
collected baseline patient data (including height,
weight, prophylaxis regimen), answered questions
about acute bleeds and organized extra treatments
for patients as required. A study physician was also
on-site during the study period.
Data collection
The study physiotherapists completed raw data
worksheets for each patient during the examination.
Following the examination, the HJHS scoring sheet
was completed. A database was subsequently created
to record the scores for each patient and the data was
double entered. Missing data were identified and the
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HEMOPHILIA JOINT HEALTH SCORE RELIABILITY STUDY

individual physiotherapist contacted to clarify/complete these data.

Analysis
Inter-rater reliability was calculated by comparing
the concordance of scores between the different
physiotherapists examining the same patients using
the intraclass correlation coefficient (ICC) calculated according to Cohens Generalizability Theory
[14]. The ICC is a measure of concordance adjusted
for chance agreement; a score of 0 suggests agreement due to chance alone, while a score of 1
suggests perfect agreement. Intra-rater reliability
was assessed by calculating the concordance between day 1 and day 2 scores for each physiotherapist examining the same patient twice again
using the ICC. The internal consistency (reliability)

521

of the different items of the HJHS was assessed

using Cronbachs alpha.
In addition, the BlandAltman limits of agreement
were determined for the repeated assessment by
subtracting the day 2 scores from the day 1 scores
and determining the mean difference and 2 SD from
the mean [15].

Results
The eight subjects represented a variety of ages, joint
damage, treatment regimens and inhibitor status.
Their demographics are outlined in Table 1.
The overall inter-rater reliability was very good
(ICC 0.83). Some variations were seen in the
range of scores for each joint; for example, patient
4 had a range of 013 recorded for his left ankle.
Table 2 denotes the median score and range for

Table 1. Sample demographics.

Subjects for Reliability Study
Joint change (none, mild/moderate, severe)*
Study no.

Age

RA

LA

RE

LE

RK

LK

Prophylaxis (frequency)

Inhibitor (yes/no/tolerized)

1
2
3
4
5
6
7
8

12.5
11.1
4.1
6.2
11.6
7.4
7
9.7

mi/mod
mi/mod
none
mi/mod
mi/mod
none
none
mi/mod

mi/mod
mi/mod
none
mi/mod
none
mi/mod
none
severe

mi/mod
none
none
none
none
none
none
mi/mod

none
none
none
none
none
none
mi/mod
none

none
none
none
none
none
none
none
none

severe
severe
none
none
none
none
none
none

3/week
3/week
1/week
2/week
3/week
2/week
3/week
daily

Tolerized
Tolerized
no
no
no
no
no
yes

Yes means inhibitor still present; Tolerized means inhibitor suppressed.

mi/mod, mild/moderate; LA, left ankle; RA, right ankle; LE, left elbow; RE, right elbow; LK, left knee; RK, right knee.
*see text for description

Table 2. Hemophilia Joint Health Score Reliability Study Scores, median score (range).
Patient no.
1
Day 1
LA score (max 28)
RA score (max 28)
LE score (max 24)
RE score (max 24)
LK score (max 28)
RK score (max 28)
Day 2
LA score (max 28)
RA score (max 28)
LE score (max 24)
RE score (max 24)
LK score (max 28)
RK score (max 28)

4.5
4
1
1
11.5
2

(28)
(07)
(03)
(02)
(1015)
(05)

6
6
1
0
12
2

(69)
(07)
(01)
(00)
(1115)
(02)

1.5
1.5
0
0
0
0

(02)
(02)
(02)
(01)
(03)
(05)

4
0
0.5
0
0.5
0

(013)
(01)
(01)
(01)
(01)
(02)

1
3
0
0
0.5
0

(03)
(06)
(01)
(01)
(04)
(01)

3
2
0.5
0
0.5
3.5

(23)
(06)
(01)
(03)
(02)
(26)

4.5
2.5
2
0
1.5
1.5

(07)
(24)
(02)
(01)
(03)
(04)

10.5
8
1
9
2
4.5

(413)
(211)
(01)
(611)
(07)
(16)

8.5
4.5
0
1
8.5
2.5

(49)
(37)
(01)
(07)
(512)
(27)

6
2
1
0.5
15
2

(59)
(23)
(01)
(02)
(1316)
(02)

1.5
0
0
0
0.5
0

(02)
(02)
(00)
(02)
(02)
(04)

5.5
3
0.5
0
1
1

(013)
(04)
(01)
(00)
(04)
(03)

2
4
0
0
1
0

(23)
(06)
(01)
(01)
(04)
(03)

3
2
0
0
1
1

(25)
(26)
(00)
(00)
(05)
(06)

4
2
0
0
2
1

(05)
(03)
(04)
(00)
(02)
(02)

12
3
0.5
6.5
2
1

(1113)
(33)
(05)
(49)
(22)
(02)

LA, left ankle; RA, right ankle; LE, left elbow; RE, right elbow; LK, left knee; RK, right knee; max, maximum score.
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P. HILLIARD et al.

Table 3. Reliability (ICC) of the HJHS scores by item.

Item

Table 4. Reliability (ICC) of the HJHS scores by joint.

Interobserver

Testretest

0.64
0.72
0.16
0.78
0.03
0.45
0.17
0.06
0.14
0.73
0.38

0.84
0.90
0.45
0.71
0.71
0.43
0.54
0.13
0.25
0.81
0.63

Swelling
Duration of swelling
Axial alignment
Muscle atrophy
Crepitus
Flexion loss
Extension loss
Joint pain
Instability
Gait
Strength

ICC, intraclass correlation coefficient; HJHS, Hemophilia Joint

Health Score.

Change in HJHS score (day 2 day 1)

30.0
25.0
20.0
15.0
10.0
5.0
0.0
5.0
10.0
15.0

Joint
Left ankle
Right ankle
Left elbow
Right elbow
Left knee
Right knee

Interobserver

Testretest

0.37
0.24
0.20
0.80
0.79
0.24

0.87
0.47
0.19
0.78
0.71
0.36

ICC, intraclass correlation coefficient; HJHS, Hemophilia Joint

Health Score.

inter-observer), whereas crepitus was scored with

much higher consistency on testretest. Overall, the
second day scores were slightly higher (mean difference 0.56) with the BlandAltman limits of agreement (2 SD of the mean difference) of )12.3 to 13.5
(Fig. 1).
The overall internal consistency of the items was
very good (Cronbachs alpha 0.86). However, the
internal consistency varied between joints. These
values are presented in Table 4.
Of note, there were some changes in patient status
from day 1 to day 2; for example, patient 6 had knee
and shin abrasions on day 1, and patient 8 had an
ankle haemarthrosis on day 2 most likely related to
poststudy activities.

20.0
25.0

Discussion

30.0
0

10

20
30
Total HJHS score on Day 1

40

Fig. 1. Change in Hemophilia Joint Health Score (HJHS) total

score vs. total HJHS score. BlandAltmann plot: Scatterplot of
change in HJHS total score (day 2day 1) on the y-axis and total
HJHS score (day 1) on the x-axis. The horizontal lines represent
the mean change score and 2 SD (the 95% limits of agreement).
The symbols are different for each of the four study physiotherapists.

each patient per joint, recorded by the four physiotherapists on day 1 and day 2. Overall, the ankle
joint was not scored as well (inter-observer) as the
knee or elbow joints. Discrepancies were also noted
in the scoring of the individual items. Instability,
joint pain, crepitus and axial alignment were
scored with poorer inter-observer consistency than
swelling, muscle atrophy and gait (see Table 3).
The overall intra-rater reliability was also excellent
(ICC 0.89). However, some variation was noted
from day 1 to day 2. For example, patient 8 had a
median score of 8 (range 211) for his right ankle on
day 1, compared with a median score of 3 (range 3
3) on day 2 (see Table 2). Items such as joint pain
and instability were less consistently scored by the
same physiotherapist on day 1 and day 2 (similar to

Haemophilia (2006), 12, 518525

The Hemophilia Joint Health Score is a newly

developed 11-item scoring tool for assessing joint
impairment in boys with haemophilia from 4 to
18 years of age. The HJHS is based on expert review
of previously existing measures and consensus of
most relevant items. The purpose of this study was to
assess the inter-rater and testretest reliability of the
HJHS. Overall, we found that the reliability of the
HJHS instrument was very good (inter-rater, intrarater and internal consistency of the items).
The HJHS was developed to be sensitive to minor
changes in joint health for use in many countries,
especially countries where prophylactic treatment is
used and less joint damage is evident. As such, future
work must be performed to establish the responsiveness of the tool in these settings.
A high inter-rater reliability between physiotherapists of differing backgrounds and nationalities
indicated that different physiotherapists in different
haemophilia treatment centres and different countries could use the HJHS instrument and produce
very similar results. Additionally, the high intra-rater
reliability suggests that the tool can be used at
different points in time and yield very comparable
results. These properties are particularly important
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HEMOPHILIA JOINT HEALTH SCORE RELIABILITY STUDY

in multi-centre clinical studies and in research

analysing musculoskeletal outcomes of various treatment approaches.
There are a few possible limitations associated
with the HJHS and the assessment of its reliability.
With respect to its reliability, all four physiotherapists were highly trained in musculoskeletal examination (three have worked with haemophilia
programs for a total of 51 years, range 1127 years;
the fourth has worked in rheumatology and orthopaedic programs for 5 years). It is possible that if a
less experienced physiotherapist were to use this tool,
the reliability may be compromised. This requires
further evaluation.
The testretest examinations were performed over
two consecutive days, which may have introduced a
recall or re-training effect. However, patient scores
were not exactly consistent between day 1 and day 2,
suggesting that scores were not solely dependent on
physiotherapist memory of the previous days presentation. As well, some of the scoring inconsistencies
between day 1 and day 2 may relate to changes in
patient condition. As a result, the intra-rater reliability may actually be higher than assessed in this
study.
Additionally, despite the very good overall reliability, there were some specific variations noted in the
scoring of the HJHS. In order to reduce some of the
observed inconsistencies, the Physiotherapy Working
Group is continuing to identify areas of the HJHS
instrument and explanation document that need
further clarification and make improvements. The
worksheets have been updated to make them more
user friendly.
With respect to limitations of the HJHS, the
instrument was designed to detect milder joint
changes and therefore, may not be as relevant for
use in developing countries where factor treatment
and prophylaxis are not always available, and the
burden of haemophilic arthropathy is correspondingly greater. This should be studied at a later time.
As well, the initial age group focus of the tool is for
children 418-years old. Its use in evaluating younger
children and adults will require further study.
Finally, the instrument was developed in English
and will require translation for broader use.

Conclusion
This study is the first in a series to test the
psychometric properties of the HJHS. With a very
high inter-rater and testretest reliability, the HJHS
is a promising new measure of joint health for boys
with haemophilia.
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523

Acknowledgements
This work was supported by external funding for the
IPSG from Bayer HealthCare LLC, Biological Products Division, Baxter BioScience, Novo Nordisk,
Wyeth Pharmaceuticals, and ZLB Behring administered through the Hospital for Sick Children Foundation, Toronto, Canada. We would like to express our
recognition to the International Prophylaxis Study
Group Steering Committee: Dr Victor S. Blanchette
(Chair), Dr Louis M. Aledort (Co-Chair), Dr Rolf
Ljung (Co-Chair), Dr Brian M. Feldman, Dr Georges
E. Rivard, Dr Marilyn Manco-Johnson, Dr Pia Petrini,
Dr Marijke van den Berg, Dr Wolfgang Schramm,
Dr Alessandro Gringeri for their continuous scientific incentive and advice. We would also like to
acknowledge Ms Ann Marie Stain RN, and Ms
Sawsan Bakhit for their assistance with the study,
and Raoul Engelbert PT, PhD and JanJaap vanderNet
PT, PhD for their valuable input.

References
1 Beeton K. Evaluation of outcome of care in patients
with haemophilia. Haemophilia 2002; 8: 42834.
2 Panicker J, Warrier I, Thomas R, Lusher JM. The
overall effectiveness of prophylaxis in severe haemophilia. Haemophilia 2003; 9: 2728.
3 Rodriguez-Merchan EC. Effects of hemophilia on
articulations of children and adults. Clin Orthop 1996;
328: 713.
4 Aledort LM, Haschmeyer RH, Pettersson H. A longitudinal study of orthopaedic outcomes for severe factor-VIII-deficient haemophiliacs. The Orthopaedic
Outcome Study Group. J Intern Med 1994; 236: 391
9.
5 Fischer K, Astermark J, van der Bom JG, et al. Prophylactic treatment for severe haemophilia: comparison of an intermediate-dose to a high-dose regimen.
Haemophilia 2002; 8: 75360.
6 Nilsson IM, Berntorp E, Lofquist T, Pettersson H.
Twenty-five years experience of prophylactic treatment in severe haemophilia A and B. J Intern Med
1992; 232: 2532.
7 Heijnen L, De Kleijn P. Physiotherapy for the treatment of articular contractions in haemophilia. Haemophilia 1999; 5 (Suppl.1): 169.
8 Fischer K, van der Bom JG, Molho P, et al. Prophylactic versus on-demand treatment strategies for severe
haemophilia: a comparison of costs and long-term
outcome. Haemophilia 2002; 8: 74552.
9 Rodriguez-Merchan EC. Orthopaedic assessment in
haemophilia. Haemophilia 2003; 9 (Suppl. 1): 6574;
discussion 74.
10 Gilbert MS. Prophylaxis: musculoskeletal evaluation.
Semin Hematol 1993; 30(3 Suppl. 2): 36.

Haemophilia (2006), 12, 518525

524

P. HILLIARD et al.

11 De Kleijn P, Heijnen L, VanMeeteren NL. Clinimetric

instruments to assess functional health status in patients with haemophilia: a literature review. Haemophilia 2002; 8: 41927.
12 Manco-Johnson MJ, Nuss R, Funk S, Murphy J. Joint
evaluation instruments for children and adults with
haemophilia. Haemophilia 2000; 6: 64957.
13 Hill FGH, Ljung R. Third and fourth workshops
of the European Paediatric Network for haemo-

philia management. Haemophilia 2003; 9: 223

8.
14 Streiner D, Norman G. Health Measurement Scales. A
Practical Guide to their Development and Use. 2nd
edn. Oxford: Oxford University Press, 1995.
15 Bland JM, Altman DG. Measuring agreement in
method comparison studies. Stat Methods Med Res
1999; 8: 13560.

Appendix 1
Hemophilia Joint Health Score
LA

RA

LE

RE

LK

RK

Other

Swelling
Duration (swelling)
Muscle atrophy
Axial alignment
Crepitus on motion
Flexion loss
Extension loss
Instability
Joint pain
Strength
Gait
Joint total
Global gait score
Total score (sum of joint totals + global gait score).
LA, left ankle; RA, right ankle; LE, left elbow; RE, right elbow; LK, left knee; RK, right knee.

Swelling
0 no swelling
1 mild
2 moderate
3 severe
Duration
0 no swelling or <6 months
1 >6 months
Muscle atrophy
0 none
1 mild
2 severe
Axial alignment
Measured only at knee and ankle
0 within normal limits
2 outside normal limits (see guidelines in explanation document)
Flexion loss
0 <5
1 510
2 1120
3 >20
Extension loss
0 <5
1 510
2 1120
3 >20

Haemophilia (2006), 12, 518525

 2006 The Authors

Journal compilation  2006 Blackwell Publishing Ltd

HEMOPHILIA JOINT HEALTH SCORE RELIABILITY STUDY

525

Instability
0 none
1 significant pathologic joint laxity
Joint pain
0 no pain either through range or at end range of motion
1 present (observed grimace, withdrawal or resistance)
Strength (using Daniels and Worthinghams scale)
Within available ROM
0 holds test position against gravity with maximum resistance (gr. 5)
1 holds test position against gravity with moderate resistance (but breaks with maximal resistance) (gr. 4)
2 holds test position with minimal resistance (gr. 3+), or holds test position against gravity(gr. 3)
3 able to partially complete ROM against gravity (gr. 3)/2+), or able to move through ROM gravity eliminated (gr. 2), or through
partial ROM gravity eliminated
4 trace (gr. 1) or no muscle contraction (gr.0)
Gait
0 all skills are within normal limits
1 one skill is not within normal limits
2 two skills are not within normal limits
3 three skills are not within normal limits
4 no skills are within normal limits (walking, stairs, running, skipping)

 2006 The Authors

Journal compilation  2006 Blackwell Publishing Ltd

Haemophilia (2006), 12, 518525