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Integrated Health Sciences Relationship between Quality of life, functional independence and Joint functions of adult hemophiliacs-a cross sectional study

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Integrated Health Sciences Relationship between Quality of life, functional independence and Joint functions of adult hemophiliacs-a cross sectional study

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Background: QOL is a wide based subject and multifaceted topic dependent on culture, beliefs, and social and environmental factors etc., it is expected that a comprehensive evaluation with available tools in an Indian population would give more insight and awareness to their problems and limitations.
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*Correspondence:
E-mail: atramalingam@gmail.com
JIHS
The Journal of
Integrated Health Sciences
Available online at www.jihs.in
Relationship between Quality of life, functional independence and Joint functions of adult
hemophiliacs- a cross sectional study
Thangamani R1*, Chaudhari I2, Jariwala K2, Patel J2, Patel N2, Purani S, Kumar S3, Patel M3
1Lecturer, 2Physiotherapist; Sarvajanik College of Physiotherapy, Surat, Gujarat, India
3 Lead Clinical Support Therapist, Ann Physiocare Ltd, UK
4Consultant Physiotherapist, Sparsh Physiotherapy Clinic, Surat, Gujarat, India
ABSTRACT
Background: QOL is a wide based subject and multifaceted topic dependent on culture, beliefs, and social and
environmental factors etc., it is expected that a comprehensive evaluation with available tools in an Indian
population would give more insight and awareness to their problems and limitations.
Objective: The main aim of the current study was to asses QOL, Functional independence and functional
limitations related to joints of adult Hemophilia patients.
Methods: Adult male patients age equal or greater than 18 years with factor viii or ix deficiency and who were
willing to participate and given consent were included in the study. The data were collected by getting the self-
reported outcome measures filled, the WHOBREF QOL, and the objective functional instruments Functional
independence score of hemophiliacs (FISH) and GILBERT joint score administered by the researcher. The data
were analyzed with Pearson correlation analysis, non -parametric test and Regression analysis to find out the
variability.
Results: The result showed that the functional components like transfer and locomotion were mainly determined
by joint bleeds and total joint score respectively. And the moderate bleed groups were different in terms of pain,
total joint score, transfer and total functional independence.
Conclusion: More the bleed in the joints leads to reduced joint function and functional independence of
hemophiliac patients, affects their health related quality of life.
Keywords: Hemophilia, QOL, Functional Independence, Joint function
INTRODUCTION
Hemophilia is a genetically transmitted disorder of
deficiency of circulating blood clotting factor.
Hemophilia A and B are rare disorders which result
from a deficiency of clotting factor VIII&IX.1,2 The
deficits of hemophilia individuals lead to repeated
spontaneous and post-traumatic internal bleeding
episodes and the consequent musculoskeletal
implications of haemophilia and immobilization
may result in muscle atrophy, synovitis, chronic
hemophilic arthropathy, joint and muscle
contractures, Limb length inequality and loss of
proprioception.3-5
Quality of Life (QoL) is a phrase used to refer to an
     
emotional, social, and physical aspects of the
    
wellbeing may be impacted over time by a disease, a
disability, or a disorder.6 Hemophilia may have a
significant impact on the quality of life (QoL). And
older children were reported to have higher
impairments on social adaptation to conditions.7,8 As
patients with haemophilia age, A need for support
around employment is necessary for individuals and
they are less involved in full time work and suffer
more from occupational disability.9 For example, a
Original Article
Journal of Integrated Health Sciences | Vol IV | Issue I |June 2016
18
Polish study, found that 38% were unemployed and
received some form of social subvention [10].
Adults with haemophilia have financial
consequences for themselves and their families as
well as feelings of dependency and more likely to
take early retirement.1 One study stressed the
importance of career counseling regarding training
and work for young adults. 11
To explain the higher reported quality of life in some
studies
used to describe the correlation between poor quality
health and the development of adaptive coping
strategies, which leads to good quality of life.12
Hence a constant measuring of QOL in changing life
situations can help to determine the relationships
between QOL and risk factors. Interpretation of
these data can help identify the needs for health
policies and legislation. 13 Significant differences in
quality of life between countries were found. The
paper attempts to describe conceptual,
methodological and practical foundations
of QoL research in adults and children at an
international level14 Use of factor prophylaxis and its
cost in both children and adults require objective
measures of health, such as joint status and quality
of life. There was a highly significant negative
correlation between functional independence and
quality of life scores. Quality of life in the Turkish
patients were more impaired in the subscales of
physical health, feeling as well as more impaired
joint scores and authors recommended primary
factor prophylaxis and encouraging the patients to
learn home treatment to improve joint scores and
quality of life.15-17 The development of functional
scores such as the Functional Independence Score
and quality-of-life measurement tools, have helped
to provide a more comprehensive assessment and
management of health in hemophilia. 18
Purpose of the study
Increasingly more emphasis is placed on issues
related to QoL in disorder, disease and disability.
Since QoL is a wide based subject and multifaceted
topic dependant on culture, beliefs, social and
environmental factors etc., it is expected that a
comprehensive evaluation with available tools in an
Indian population would give more insights into the
policies and treatment plan related to haemophilia
patients. The main aim behind the current study was
to asses and find out the relationships between QOL,
Functional independence and functional limitations
related to affect in adult Hemophilia patients of
Indian population in a small centre like Surat.
METHODOLOGY
Subjects
Adult male patients aged equal or greater than 18
years with factor viii or ix deficiency and who were
willing to participate and willing to give consent
from Surat chapter of hemophiliacs were included in
the study. Patients with disabilities other than
hemophilia were excluded from the study. A Non
probability sampling method was used.
Procedure
The data were collected from the eligible individuals
with hemophilia, who gave consent to participate in
the study, by getting the self-reported outcome
measures filled and the objective functional
instrument administered by the researcher.
Outcome measures
WHOBREF Questionnaire (QoL) The
WHOQOL-BREF consists of 24 facets and provides
a profile of scores on four dimensions of quality of
life: physical health, psychological, social
relationships, and the environment. WHOQOL-
BREF is available in both self-administered and
interviewer-administered and assisted forms.19 For
patients who did not understand English language
were helped with interviewer assisted forms.
Function independence score - The Functional
Independence Score in Hemophilia (FISH) was
developed as a performance-based assessment tool
to objectively measure an indiv 
ability (self-care, transfer and locomotion) for
individuals suffering with Haemophilia. It is
intended to measure what the person with disability
Journal of Integrated Health Sciences | Vol IV | Issue I |June 2016
19
actually does, not what he ought to be able to do, or
might be able to do if circumstances were different,
or thinks he can do. It can also be used to evaluate
change in functional independence over time, or
after a therapeutic intervention. The current version
of FISH includes the assessment of eight activities:
eating, grooming, dressing, chair transfer, squatting,
walking, step climbing, and running. Each activity
is graded according to the amount of assistance
required to perform it.18,21
Gilbert Score - This tool measures joint health, in
the domain of body structure and function (i.e.
impairment), of the joints most commonly affected
by bleeding in hemophilia knees, ankles, elbows. It
is primarily designed for adults and children with
established arthropathy to measure pain, bleeding
and joint score (Swelling, Muscle atrophy, Axial
deformity, Crepitus on motion, Range of motion,
Flexion contracture, Instability). It may be used
when there is a need for orthopedic intervention, or
as an outcome measure to study the effect of
physiotherapy interventions.22,23
Statistical analyses
Data collected were cleaned for paucity before the
analysis and the categorical variables presented in
percentages and numerical variables in terms of
mean and standard deviations for the demographical
and clinical outcomes. A Pearson correlation
analysis was done among QOL, functional
independence and joint scores. Significant outcome
variables entered in regression analysis to find out
the variance after determining the dependent and
independent variables theoretically. A mean
comparison between mild and moderate bleeding
groups was done using non parametric Mann
Whitney U test. A Logistic regression analysis was
done to find out the significant personal
demographical factors for bleeding groups.Data
were analyzed using IBM SPSS Statistics for
Windows, Version 20.0. Armonk, NY: IBM Corp.
Statistical significance was set at p<0.05 (Two
tailed).
RESULTS
The present study collected data from 59 adult male
hemophiliacs with mean age 30.89±9.10, among
them 57 with factor VIII defect and 2 with factor IX
defect. The personal demographical and clinical data
of the subjects are given in Table 1 and 2.
Table 1. Personal demographical data of the
subjects
Subject
characteristics
Level
Percent
(%)
Education
Illiterate
School
Higher study
3.4
47.5
49.2
Occupation
Unemployed
Employed
Business
27.1
28.8
44.1
HTC use
(hemophilia
treatment center)
Frequent
Infrequent
Rare
20.3
22.0
57.6
Factor level
<1
>1
86.4
13.6
Inhibitor
No
Yes
81.4
18.6
Mode of treatment
Minimal transfusion
Episodic transfusion
E/H
3.4
94.9
1.7
Pain
No pain
Mild pain
Moderate pain
Severe pain
8.5
27.1
62.7
1.7
Bleeding
Nobleeding
Mild bleeding
Moderate bleeding
6.8
66.1
27.1
Total joint score
Mild
Moderate
Severe
16.9
44.1
39.0
Table 3, 4 & 5 show the correlation between the
quality of life components, Functional independence
measure components and the total joint limitation
score of Gilbert joint evaluation of adult
haemophiliac individuals. Significantly correlated
outcomes were used in regression analysis to find
out the proportion of variance between outcomes.
Table 6 shows the regression analysis of the
significantly related out comes of QOL, functional
Journal of Integrated Health Sciences | Vol IV | Issue I |June 2016
20
independence and gilbert joint score.
Table 2. Clinical outcome data of the subjects
Clinical outcome*
Mean
Std. Dev
WHO BREF QOL
Physical health (0-100)
Psychological(0-100)
Social relationship (0-100)
Environment(0-100)
63.23
66.15
69.42
73.15
9.30
12.34
23.55
13.62
FUNCTIONAL INDEPENDENCE (FISH)
Self-care (3-12)
Transfer (2-8)
Locomotion (3-12)
Total functional
independence (8-32)
11.74
5.55
8.69
25.98
0.658
1.27
1.92
2.93
JOINT SCORE(GILBERT)
Pain (0-3)
Bleeding (0-3)
Total joint score(0-12)
1.58
1.20
7.85
0.674
0.550
3.22
(*The minimum and maximum scores are mentioned in
bracket, for QoL 0 indicates poor function and 100
indicates excellent function; for functional independence
more score indicates independence ; for joint score more
score indicates joint limitation, more pain and bleeding.)
DISCUSSION
Despite long term improvement in life expectancy of
patients with Haemophilia over the years, QoL
measures are a subject of intense discussion and
every attempt in research is a part to constantly
improve issues related to it. As mentioned
previously, since QOL is a multifaceted topic which
varies with culture, beliefs, etc., we aimed to study
this function comprehensively in small sample of
Haemophiliacs Indian population. Based on the
results from the baseline indicators, the sample
population that we assessed were predominantly of
Haemophilia A (Factor level < 1, 86.4%), the most
common type in general population. And at the time
of study, most of the patients in the sample
population studied were of mild bleed around 66%
in the past 1 year, who reported of mild and
moderate pain in the range of around 27% and 63%
respectively. 95% of the patients reported of
episodic transfusion implying that at the time of
assessment of this study most of the patients would
have preventative measures and coping measures in
place for the disease and did not have an active
bleed. The percentages for the total joint score were
moderate and severe of 44% and 39% respectively
indicated that the subjects in the sample population
have predominantly established deformities from
episodes.
Overall, in terms of QOL, the sample population
assessed for their Physical, Psychological, Social
and Environmental domains espoused fair results (>
65%) as showed from the mean values indicating
they exhibited fairly good adjustment in these
domains in their daily life, however need to study in
depth in relation to functional independence and
joint status which is mentioned below.
Analyzing from the results, Psychological health
domain positively correlated with pain (p < 0.003) in
Gilbert joint score and locomotion (p < 0.01) in
Functional independent measure, indicating that
Psychometric values of emotions are clearly related
to pain and inability to move among hemophiliac
patients. And issues related to pain dominate the
Psychological health of a patient compared to
locomotion. The Environmental domain of QOL
showed positive relation to pain, locomotion and
total joint score. As observed, pain in major joints
(Elbow, Knee and, Ankle) seemed to dominate the
mobility and feeling of self satisfaction among the
sample population. Compared to other factors
commonly affecting hemophilic patients, transfer
ability dominated the Social relationships domain
and this could be due to improper usage of
appropriate walking aids for mobility and it is
expected that a proper evaluation of walking aid is
warranted.
The social relationships domain was positively
related (p = 0.006) only to transfer ability of the
hemophiliacs meaning that most of the patients
believed that their mobility about a chair and ability
to squat was helpful in maintaining relationships.
And importantly the functional independence of
hemophiliacs was dependent upon the severity of
Journal of Integrated Health Sciences | Vol IV | Issue I |June 2016
21
pain, bleeding of joints and tota l joint score which
were common and evident from other studies done
on hemophiliac patients. The functional components
like transfer and locomotion were mainly determined
by joint bleeds and total joint score respectively
which were observed by the positive correlation
between the respective components. Hence more the
bleed and joint score, higher the dependence for
transfer and locomotion.
Table 3. Pearson correlation among Qol and, Functional independence score
Physical
health
Psycho-
logical
Social
relationship
Environ-
ment
Self-
care
Transfer
Locomotion
Total
Functional
independence
Physical health
1
0.406**
0.172
0.501**
0.117
0.073
0.165
0.163
0.001
0.193
0.000
0.378
0.584
0.212
0.219
Psychological
1
0.430**
0.475**
0.204
-0.031
0.377**
0.281*
0.001
0.000
0.121
0.818
0.003
0.031
Social
relationship
1
0.346**
0.171
-0.353**
0.095
-0.050
0.007
0.194
0.006
0.476
0.708
Environment
1
-0.036
0.068
0.269*
0.195
0.787
0.607
0.039
0.140
Self-care
1
-0.053
0.183
0.319
0.687
0.166
0.014
Transfer
1
0.534**
0.762**
0.000
0.000
Locomotion
1
0.922
0.000
Total
Functional
independence
1
** Correlation is significant at the 0.01 level (2-tailed).** ; * Correlation is significant at the 0.05 level (2-tailed).*
Table 4. Pearson correlation among Qol and Gilbert Joint score
Physical
health
Psycho-
logical
Social
relationship
Environment
Pain
Bleeding
Total joints
core
Physical health
1
0.406**
0.172
0.501**
-0.253
0.041
-0.160
0.001
0.193
0.000
0.053
0.758
0.227
Psychological
1
0.430**
0.475**
-0.327*
-0.005
-0.240
0.001
0.000
0.011
0.972
0.067
Social relationship
1
0.346**
-0.123
0.231
-0.017
0.007
0.353
0.078
0.896
Environment
1
-0.495**
-0.078
-0.364**
0.000
0.558
0.005
Pain
1
0.329*
0.701**
0.011
0.000
Bleeding
1
0.671**
0.000
Total joint score
1
** Correlation is significant at the 0.01 level (2-tailed).** ; * Correlation is significant at the 0.05 level (2-tailed).*
Journal of Integrated Health Sciences | Vol IV | Issue I |June 2016
22
Table 5. Pearson correlation among Functional independence and Gilbert Joint score
Pain
Bleeding
Total joint
score
Self-care
Transfer
Locomotion
Total functional
independence
Pain
1
0.329*
0.701**
-0.014
-0.361**
-0.208
-0.300*
0.011
0.000
0.917
0.005
0.115
0.021
Bleeding
1
0.671**
0.193
-0.607**
-0.234
-0.372**
0.000
0.144
0.000
0.075
0.004
Total joint score
1
0.087
-0.575**
-0.384**
-0.487**
0.512
0.000
0.003
0.000
Self care
1
-0.053
0.183
0.319*
0.687
0.166
0.014
Transfer
1
0.534**
0.762**
0.000
0.000
Locomotion
1
0.922**
0.000
Total functional
independence
1
** Correlation is significant at the 0.01 level (2-tailed).**
* Correlation is significant at the 0.05 level (2-tailed).*
Table 6. Regression Analysis of outcomes
Dependent
Variable
Independent
variable
Adjusted
R square %
Constant
Un standardized
B
‘P’
value
Physical health
-
-
-
-
-
Psychological health
Pain
9.2
75.975
-5.590
0.011
Locomotion
12.7
45.126
2.418
0.003
Social health
Transfer
10.9
105.645
-6.515
0.006
Environmental health
Pain
Total joint score
21.9
89.281
-9.543
-0.139
0.005
0.841
Locomotion
5.6
56.584
1.905
0.039
Self-care
-
-
-
-
-
Transfer
Pain
Bleeding
Total joint score
38.8
7.633
-0.056
-0.948
-0.111
0.843
0.006
0.146
Locomotion
Total Joint score
13.3
10.498
-0.230
0.003
Total functional
independence
Pain
Bleeding
Total Joint score
20.1
29.49
0.271
-0.354
-0.443
0.716
0.687
0.030
Journal of Integrated Health Sciences | Vol IV | Issue I |June 2016
23
The mean comparison of mild and moderate bleed
groups of hemophiliacs showed that the moderate
bleed groups were different in terms of pain, total
joint score, transfer and total functional
independence. More the bleed in the joints and
involvement in more number of joints could be the
factor which leads to reduced joint function and
functional independence of hemophiliac patients
which in turn affects their health related quality of
life. The result of the present study was similar to
many authors who pointed out that the psychosocial
dimensions are of great importance in determining
and to maximize quality of life [1][11]. Repeated
joint bleeds, pain, arthritis and arthropathy may
reduce the QOL and lack of independence due to
reduced mobility [1] [24]. Nowadays living a better
life with better QOL is possible for hemophiliacs
due to advancement in medical treatments and
psychosocial support [25] [26]. The present study
also reported moderately good score in all the
dimensions of QOL and the personal demographical
factors did not contribute to bleeding of joints. This
study is a reinforcement of the existing studies done
on QOL on haemophiliacs with respect to outcomes.
The limitation of the study was patients were a
small sample size taken predominantly from a single
center Surat. And the present study used only
generic QOL tool and the result may have less
generalizability.
CONCLUSION
The present study shows that the adult hemophiliac
patients of Surat chapter reported reasonably good
quality of life. The moderate bleed group
hemophiliac patients have more of pain and lack of
independence. A more concerted, timely and
aggressive Physiotherapy principles in the
prevention of pain and bleeding for hemophiliac
patients and improving their independence may be
of more importance and advantage for leading a
good quality life.
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Journal of Integrated Health Sciences | Vol IV | Issue I |June 2016
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Article
Full-text available
The aim of this study is to describe the health status, health care received, and their impact on the quality of life in patients with hemophilia. Patients with severe factor VIII or IX deficiency without inhibitors or other chronic disease were enrolled. Turkish version of the Hemophilia-Specific Quality of Life Index (Haemo-QoL) questionnaire was administered to the pediatric patients aged 4 to 16 years and Haem-A-QoL to the adult patients. Joints were evaluated according to the World Federation of Hemophilia (WFH) orthopedic joint scores.Thirty-nine children/adolescents and 31 adult patients were enrolled. Mean Haemo-QoL scores were 39.6 +/- 15.0 for the children and mean Haem-A-QoL 47.4 +/- 14.1 for the adult patients, respectively. Internal consistency reliability was generally sufficient. Total Cronbach's alpha coefficient was >.70 (range .77-.96) in all the age groups. Mean total WFH orthopedic joint scores were 1.83 +/- 2.7, 4.9 +/- 4.96, and 6.94 +/- 6.15 in 4-7, 8-12, and 13-16-year-old groups, respectively. They were more impaired in the adult patients (16.23 +/-14.12). These results show that the Turkish version of the Haemo-QoL and Haem-A-QoL are reliable instruments to measure the quality of life in the pediatric and adult patients with severe hemophilia. When compared to the Haemo-QoL scores of an international multicenter West European study of children, quality of life in the Turkish patients were more impaired in the subscales of physical health, feeling, view, school and sport, and treatment as well as more impaired WFH joint scores. The authors recommend primary factor prophylaxis and encouraging the patients to learn home treatment to improve joint scores and quality of life.
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Hemophilia A and B are the most common of the severe bleeding disorders. The present article focuses on the practical aspects of the management of neonates and children diagnosed with hemophilia, and is based on questions frequently posed to paediatric hematologists. It highlights the importance of early diagnosis, the principle of early intervention and the role of comprehensive care hemophilia treatment centres.
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A multicentre, international, cross-sectional study was carried out in the frame of field testing of the first haemophilia-specific quality-of-life (QoL) questionnaire (Haemo-QoL). The aim of this paper is to describe health status and health care and their impact on QoL in haemophilic children in Western Europe. Children aged 4-16 years with severe haemophilia without inhibitors were enrolled by 20 centres in France, Germany, Italy, the Netherlands, Spain and the United Kingdom. Clinical information was collected by the physicians with a medical documentation form. Health-related QoL (HRQoL) of children was assessed with Haemo-QoL, available for three age groups. Clinical data were available in 318 patients, 85.5% with haemophilia A. The mean age at first bleeding was 11 months, at first joint bleed 25 months. Functional joint impairments were found in 11.3%. Prophylaxis treatment was given to 66.7% of children in whom breakthrough bleeds occurred 0.4 times a month compared to 1.1 bleeds in children receiving on-demand treatment. A significantly higher factor consumption was found only in the two younger age groups of prophylaxis patients compared to on-demand patients. HRQoL was satisfactory in this cohort: young children were impaired mainly in the dimension 'family' and 'treatment', whereas older children had higher impairments in the so-called 'social' dimensions, such as 'perceived support' and 'friends'. Health care of children in Western Europe is progressively improving with a large diffusion of home treatment and prophylaxis. This provides a high level of health status and HRQoL, being better in haemophilic adolescents on prophylaxis.
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The very high cost of haemophilia care, including the increase in use of factor prophylaxis in both children and adults requires that funders of clotting factor concentrates require objective measures of health, such as joint status and quality of life (QOL). Many clinical trials, especially those for licensing of new products, are including QOL instruments in their protocols to evaluate the patients' perspective of wellbeing before and during therapy. This article gives a perspective on QOL the importance of QOL measurement in the field of haemophilia and its impact on patient outcome.
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The functional independence score in haemophilia (FISH) is a performance-based instrument used to assess musculoskeletal function in patients with haemophilia. We aimed to evaluate the functional independence of haemophilia A adolescents and its correlation to radiological joint scores. A cross-sectional study was carried out on 50 adolescent haemophilia A patients. Musculoskeletal function was assessed using the FISH and individual joints were assessed radiologically using the Pettersson score and MRI scale. The mean age of our patients was 16 ± 1.1 with a mean FISH of 23.32 ± 4.69 (range 13-28) and a mean Pettersson score of 2.32 ± 3.09 (range 0-13) for the knees, 1.86 ± 2.67 (range 0-11) for ankles and 1.42 ± 2.17 (range 0-10) for elbows. The mean MRI score for the knees was 3.92 ± 2.74 (range 0-10) while that for ankles was 3.16 ± 2.64 (range 0-10) and for elbows was 2.34 ± 2.63 (range 0-10). There was highly significant correlation between both radiological joint scores and FISH and between degree of factor VIII deficiency and each of FISH, Pettersson score and MRI score. MRI was superior to conventional radiography in detection of subchondral cyst formation and erosions at joint margins. Given the highly significant correlation with both radiological joint scores, FISH appears to be a reliable tool for assessment of functional independence in adolescents with haemophilia A. MRI is more sensitive than conventional radiography in detection of early joint abnormalities.
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Treatment of haemophilia patients with inhibitors against factor VIII/IX (FVIII/IX) is still challenging and recurrent haemarthroses cause arthropathy with associated restrictions on participation in physical activities and sports. Rehabilitation is a multidisciplinary approach which includes physiotherapy, occupational therapy, psychology, social work and technical applications like prostheses, orthoses (splints and braces), shoe adaptations, walking aids and adaptations in the house and work situation, but also education. The theoretical principles and practical advice regarding rehabilitation and physiotherapy for both children and adults with haemophilia without inhibitors are highly applicable for patients with inhibitors. Hydrotherapy is useful in the treatment of painful or stiff joints and/or muscles after an acute haemarthrosis, muscle bleeds and chronic arthropathy. In addition, it is of use in cases of chronic synovitis and to start mobilization after long periods of bed rest or during the weaning of a splint. In cases of bleeding and arthropathy, adequate treatment of pain is very important, as are functional exercises. Everyone should be physically active for 30-60 min day(-1). Participation in sports is recommended for people with haemophilia, the best sport being swimming. Children should participate in sports appropriate to their size and physical characteristics.