Research Report

Effects of a 6-Week, Individualized,

Supervised Exercise Program for
People With Bleeding Disorders and R. Mulvany, PT, DPT, is Associate
Professor, Department of Physical
Therapy, College of Allied Health
Hemophilic Arthritis Sciences, University of Tennessee
Health Science Center, 930 Mad-
Ruth Mulvany, Audrey R. Zucker-Levin, Michael Jeng, Catherine Joyce, ison Ave, 6th Floor, Memphis, TN
Janet Tuller, Jonathan M. Rose, Marion Dugdale 38163 (USA). Address all corre-
spondence to Dr Mulvany at:
rmulvaney@uthsc.edu.
Background. People with bleeding disorders may develop severe arthritis due to A.R. Zucker-Levin, PT, PhD, MBA,
joint hemorrhages. Exercise is recommended for people with bleeding disorders, but GCS, is Associate Professor, De-
guidelines are vague and few studies document efficacy. In this study, 65% of people partment of Physical Therapy, Col-
lege of Allied Health Sciences,
with bleeding disorders surveyed reported participating in minimal exercise, and 50% University of Tennessee Health
indicated a fear of exercise-induced bleeding, pain, or physical impairment. Science Center.

M. Jeng, MD, is Associate Profes-

Objective. The purpose of this study was to examine the feasibility, safety, and sor of Pediatrics, Stanford Univer-
efficacy of a professionally designed, individualized, supervised exercise program for sity, Palo Alto, California.
people with bleeding disorders.
C. Joyce, MSW, is affiliated with the
Comprehensive Hemophilia Clinic,
Design. A single-group, pretest-posttest clinical design was used. Department of Hematology, Col-
lege of Medicine, University of Ten-
Methods. Thirty-three patients (3 female, 30 male; 7–57 years of age) with mild nessee Health Science Center.
to severe bleeding disorders were enrolled in the study. Twelve patients had co- J. Tuller, RN, MPH, is Clinical
existing illnesses, including HIV/AIDS, hepatitis, diabetes, fibromyalgia, neurofibro- Nurse Coordinator, Comprehen-
matosis, osteopenia, osteogenesis imperfecta, or cancer. Pre- and post-program mea- sive Hemophilia Clinic, Depart-
sures included upper- and lower-extremity strength (force-generating capacity), joint ment of Hematology, College of
range of motion, joint and extremity circumference, and distance walked in 6 Medicine, University of Tennessee
Health Science Center.
minutes. Each patient was prescribed a 6-week, twice-weekly, individualized, super-
vised exercise program. Twenty participants (61%) completed the program. J.M. Rose, PT, MS, ATC, Assistant
Professor, Department of Physical
Therapy, Health Science Center,
Results. Pre- and post-program data were analyzed by paired t tests for all par- College of Allied Health Sciences,
ticipants who completed the program. No exercise-induced injuries, pain, edema, or University of Tennessee.
bleeding episodes were reported. Significant improvements occurred in joint motion,
M. Dugdale, MD, is Medical Direc-
strength, and distance walked in 6 minutes, with no change in joint circumference. tor, Comprehensive Hemophilia
The greatest gains were among the individuals with the most severe joint damage and Clinic, Department of Hematology,
coexisting illness. College of Medicine, University of
Tennessee Health Science Center.
Limitations. Limitations included a small sample size with concomitant disease, [Mulvany R, Zucker-Levin AR, Jeng
which is common to the population, and a nonblinded examiner. M, et al. Effects of a 6-week, indi-
vidualized, supervised exercise
Conclusions. A professionally designed and supervised, individualized exercise program for people with bleeding
program is feasible, safe, and beneficial for people with bleeding disorders, even in disorders and hemophilic arthritis.
Phys Ther. 2010;90:509 –526.]
the presence of concomitant disease. A longitudinal study with a larger sample size,
a blinded examiner, and a control group is needed to confirm the results. © 2010 American Physical Therapy
Association

Post a Rapid Response or

find The Bottom Line:
www.ptjournal.org

April 2010 Volume 90 Number 4 Physical Therapy f 509

Supervised Exercise Program for People With Bleeding Disorders and Hemophilic Arthritis
B
leeding disorders and the mus-
culoskeletal pathologies that
accompany them offer numer-
ous challenges to health care provid-
ers. Hemophilia A, hemophilia B, and
von Willebrand disease are the most
common inherited bleeding disor-
ders. These disorders affect people
of all ages, with no ethnic or racial
predilection.1 Hemophilia A and B
are caused by an x-linked inherited
deficiency of clotting factors VIII and
IX, respectively, and are found al-
most exclusively in males, whereas
females are carriers of the trait.1–3
Von Willebrand disease is caused by
an inherited defect in or deficiency
Available With
This Article at
ptjournal.apta.org
• The Bottom Line clinical
summary
• The Bottom Line Podcast
• Audio Abstracts Podcast
This article was published ahead of
print on March 4, 2010, at
ptjournal.apta.org.
Figure 1.
Severe chronic synovitis in hemophilia, with invasive hypervascular synovial hypertrophy. Joint arthropathy led to total knee
replacement in this young man.
510 f Physical Therapy Volume 90
of von Willebrand factor and affects
both sexes equally.4 Bleeding disor-
ders can be classified as mild, mod-
erate, or severe, depending on the
amount or efficacy of circulating fac-
tor levels. In moderate or severe
bleeding disorders, minor trauma
can cause bleeding in joints (hemar-
throsis) or in muscles (hematoma).
This bleeding may initiate a cycle of
musculoskeletal degeneration lead-
ing to disabling arthritis.5,6
Hemarthrosis is the most common
and disabling manifestation of hemo-
philia. Approximately 80% of hemor-
rhages associated with hemophilia
are hemarthroses.5 Hemarthroses
usually begin around 12 to 24
months of age and persist through-
out life.5 Joints most frequently af-
fected are knees, followed by elbows
and ankles; less frequently, hips and
shoulders.5–7 Any joint that has 3 or
more bleeding episodes over a pe-
riod of 3 to 6 months is a target joint
and is much more susceptible to sub-
sequent bleeding and arthritic
changes.8,9
Number 4
Repeated bleeding into joints causes
hemophilic arthritis (hemarthropa-
thy), producing joint tissue destruc-
tion similar to that seen in rheuma-
toid arthritis. This destruction results
in invasive hypervascular synovial
hypertrophy, chronic synovitis, ar-
ticular cartilage damage, bony hyper-
trophy, and subchondral cysts.5–10
Hemophilic arthritis is manifested by
pain,11 joint instability, malalign-
ment, muscular atrophy, impaired
range of motion (ROM), and im-
paired function5–7,11–13 (Fig. 1). Ar-
nold and Hilgartner14 described 5 ra-
diographic stages in the progression
of hemophilic arthropathy (Tab. 1).
In stage I, joint integrity is main-
tained with no skeletal or cartilagi-
nous changes. In stage V, the articu-
lar cartilage is fibrotic and
deteriorated with a complete loss of
joint space.14 Many joints are ankylo-
sed at this stage, leaving joint re-
placement the only viable option for
functional movement (Fig. 2).
In addition to hemarthroses, bleed-
ing may occur directly into a muscle.
The most commonly affected mus-
cles are the iliopsoas, quadriceps,
April 2010
 Supervised Exercise Program for People With Bleeding Disorders and Hemophilic Arthritis
Table 1.
Arnold and Hilgartner14 Radiographic Staging of Hemophilic Arthropathy and Clinical Presentation
Stage Joint Integrity
I No change
II No change
III Disorganization of joint
IV Advanced
disorganization,
irreversible joint
changes
V Marked fibrosis,
substantial
disorganization of
structures, irreversible
changes
gastrocnemius-soleus, and muscles
of the forearm. Bleeding into mus-
cles can cause severe problems, in-
cluding compartment syndrome,
neurovascular compromise, fibrosis,
adhesions, contractures, hemato-
mas, and pseudotumors.15–18 (Fig. 3).
Less common sites for bleeding are
the gastrointestinal tract, vital or-
gans, spine, and within the cranium.
Although bleeding at these sites is
less common, it poses a much
greater risk and requires immediate
medical intervention.19,20
April 2010
Clinical Presentation:
Progression Along the
Skeleton Cartilage Synovium Continuum
No change No change Swelling of synovium Acute hemarthrosis: as
and soft tissues blood fills joint capsule,
joint becomes tense,
swollen, hard, hot, and
tender; often held in
flexion, with restricted
range of motion and
pain
Osteoporosis, especially No change Swollen, thickened, Subacute hemarthrosis:
of epiphyses; boggy; early after 2 or more
epiphyseal reaction similar to hemarthroses;
overgrowth rheumatoid thickened and boggy
arthritis synovium, moderate
restriction of range of
motion; pigmented
villonodular synovitis,
similar to rheumatoid
arthritis
Osteoporosis, No significant narrowing of Opacified, with Chronic hemarthrosis:
subchondral cysts, joint space; squaring of hemosiderin after subacute joint
progressive patella deposits; synovial involvement has been
overgrowth of hypertrophy and present for ⱖ6 mo
epiphyses, widening vascular
of intercondylar hyperplasia
notch of knee and
trochlear notch of
ulna
Progression of stages II Severe cartilage destruction, Opacified and fibrous Destructive progression to
and III narrowed joint space, end stage
osteochondral lesion,
fibrillation and erosion,
irreversible changes
Extensive enlargement Loss of joint space, absence Little or no Chronic, fibrotic,
of epiphyses, of cartilage recognizable contracted; totally
enburnated bone synovial tissue destroyed joint
ends
Overview of Management agement of von Willebrand disease
for Bleeding Disorders depends on the type and severity,
Currently, there are 3 major methods with medical options including tab-
of managing hemophilia: medications lets, liquids, nasal sprays, and factor
to promote clotting and to manage concentrate infusions.21,23
pain and coexisting diseases, surgery,
and rehabilitative exercise. Historically, the management of hemo-
philia and its secondary musculoskel-
Medications to Promote Clotting etal impairments was greatly compli-
Depending on severity, medical man- cated by factor replacements derived
agement options for hemophilia in- from contaminated blood products,
clude infusion of concentrated puri- leading to the spread of HIV, hepatitis,
fied factor replacement5,21,22 or a and other blood-borne pathogens.24
nasal spray to stimulate the release of Currently, the risk of secondary infec-
stored factor into the bloodstream tion is diminished by the availability of
for clotting control.21 Likewise, man- purified factor replacement. Some pa-
Volume 90 Number 4 Physical Therapy f 511
Supervised Exercise Program for People With Bleeding Disorders and Hemophilic Arthritis
Figure 2.
Bilateral stage IV and V elbows and knees due to multiple hemarthroses.
tients will use factor replacement as
needed (episodic), whereas others re-
ceive regularly scheduled replacement
(prophylactic). Improved safety and
prophylactic use have resulted in
less joint destruction and the ability
to lead a more active lifestyle.22,25
Surgery
When indicated, orthopedic surgical
interventions for hemarthropathy are
similar to those for rheumatoid ar-
thritis and osteoarthritis, with the
added complications of bleeding
and coexisting diseases acquired
from blood products.26 –32 Some of
the most common orthopedic proce-
dures are synovectomy, arthroscopy,
total joint arthroplasty, and arthrode-
sis.26,28 –30,32,33 To ensure an optimal
outcome, the medical team should
involve the primary care doctor, sur-
geon, anesthetist, hematologist,
nurse, physical therapist, occupa-
tional therapist, and social worker.
Because of the complexities of reha-
bilitation, physical therapy should be
initiated before and continued well
after surgery.27,28,31,33
512 f Physical Therapy Volume 90
Rehabilitation and Exercise
A major component of rehabilitation
for people with hemarthropathy and
muscle bleeds is therapeutic exer-
cise.27,33–36 Therapeutic exercise is the
selective application of stress to cause
beneficial physiologic adaptation and
to restore function. Because exercise
is a form of biomechanical stress, a
delicate balance exists between too
much stress, which may cause bleed-
ing and trauma, and not enough stress,
producing a subtherapeutic response.
This fine line between beneficial and
detrimental activity has led many peo-
ple with bleeding disorders to refrain
from exercise for fear of initiating a
bleeding episode.34,36 Paradoxically,
refraining from exercise leads people
with bleeding disorders to experience
decreased function due to weakness,
decreased ROM, and diminished qual-
ity of life. When surveyed by the Na-
tional Hemophilia Foundation’s Na-
tional Prevention Program, 60% of the
adolescents with hemophilia reported
that they limit or refrain from physical
activity.37 The survey supported the
findings that children with hemophilia
Number 4
generally have decreased strength
(force-generating capacity) and flex-
ibility, are less active, and have lower
aerobic working capacity than their
unaffected peers.33,34,36 – 43 These in-
adequate levels of strength and flex-
ibility leave people with bleeding
disorders even more vulnerable be-
cause strong, flexible muscles sup-
port joints, help attenuate stresses,
and diminish the risk of injury.37
Thus, people with bleeding disor-
ders who exercise appropriately may
improve their strength and flexibil-
ity, which, in turn, could diminish
the chances of developing recurrent
hemarthrosis, synovitis, and subse-
quent joint destruction.34 – 43 Further-
more, because obesity and heart dis-
ease are as prevalent in people with
bleeding disorders as they are in the
general population, cardiovascular
fitness should be included in any ex-
ercise program.44,45
Literature that examines the role of
exercise on function in people with
bleeding disorders is limited. Harris
and Boggio46 performed a descrip-
April 2010
 Supervised Exercise Program for People With Bleeding Disorders and Hemophilic Arthritis
Figure 3.
Massive pseudotumor and compartment syndrome due to repeated hemorrhages in the gastrocnemius muscle.
tive review of 46 people with bleed-
ing disorders and found that those
who reported regular exercise had
significantly greater ROM in the large
joints than those who did not exer-
cise. Likewise, Wittmeier and Mul-
der43,47 described the benefits of ex-
ercise and hypothesized that people
with bleeding disorders would ben-
efit from sports, fitness, and physical
activity. In addition to these reports,
experienced therapists have pro-
vided guidelines for exercise safety
in people with bleeding disorders in
publications by the World Federa-
tion of Hemophilia47,48 and the Na-
tional Hemophilia Foundation.49
However, experimental design was
not used when establishing these
guidelines.
Few studies that used experimental
designs have been reported. Hilberg
et al50 performed an experimental
April 2010
exercise protocol on 9 people with
bleeding disorders who participated
in a 6-month specialized training pro-
gram including gentle strength train-
ing with low resistance. Significant
improvements in isometric muscle
strength and proprioceptive perfor-
mance were found, and generic
guidelines for strengthening in peo-
ple with bleeding disorders were
provided, which include the use of
low-resistance, high-repetition exer-
cises over a 6-month period. Like-
wise, Pelletier et al,51 in a quasi-
experimental study, found strength
gains after a 3-week isometric exer-
cise program in a single 12-year-old
participant. These studies provide
limited guidance in decision making
when prescribing exercises to peo-
ple with bleeding disorders.
The literature implies that people
with bleeding disorders could bene-
Volume 90
fit from a structured, supervised ex-
ercise program. However, there is
little scientific evidence on which to
base specific exercise prescriptions
for people with bleeding disorders,
and continuous rehabilitation under
a physical therapist may not always
be economically feasible. Due to the
dearth of evidence-based literature,
therapists must rely on their best
judgment for developing exercise
prescriptions when treating people
with bleeding disorders. Therefore,
it was the goal of this study to deter-
mine whether people with bleeding
disorders can safely and effectively
exercise and achieve improved lev-
els of function using an individual-
ized exercise program that was de-
signed by a physical therapist and
supervised by a trained fitness in-
structor. In contrast to the studies by
Hilberg et al50 and Pelletier et al,51
our study offers a comprehensive ex-
Number 4 Physical Therapy f 513
Supervised Exercise Program for People With Bleeding Disorders and Hemophilic Arthritis
ercise program that challenges
strength, ROM, and cardiovascular
function in patients with severe
comorbidity.
Materials and Method
Study Design
A single-group, pretest-posttest clin-
ical design was used, with measure-
ments taken prior to initiation of the
supervised exercise program and im-
mediately following the 6-week
intervention.
Participants
Participant selection for this Institu-
tional Review Board–approved study
was based on a sample of conve-
nience from the patient population
of the Comprehensive Hemophilia
Clinic of the University of Tennessee
Health Science Center. Thirty-three
volunteers, (30 male, 3 female), met
the inclusion criteria. All participants
signed informed consent and liability
release forms. Additional consent
forms were signed by legally autho-
rized representatives for those volun-
teers under the age of 18 years. None
of the participants had participated
in an exercise program for a 1 year
prior to initiating the program. Our
population represents a typical co-
hort of people with bleeding disor-
ders. The study group had a range of
severity of hemophilic arthritis:
some had previous orthopedic sur-
gery, some had a coexisting illness
such as HIV or hepatitis, and some
were on a regimen of prophylactic
factor replacement.
Inclusion criteria were: (1) diagnosis
of mild, moderate, or severe hemo-
philia or von Willebrand disease; (2)
willingness to exercise twice a week
for 6 weeks and to complete the pre-
and post-program evaluations; (3)
ability to arrange transportation to
and from data collection and exer-
cise sessions; (4) approval by their
hematologist to participate in the ex-
ercise program; (5) aged 7 to 60
years; and (6) agreement of those
514 f Physical Therapy Volume 90
participants with severe hemophilia
to receive a prophylactic intrave-
nous dose of factor prior to exercise.
Exclusion criteria were: (1) the in-
ability to attend exercise sessions at
least twice a week for 6 consecutive
weeks; (2) nonadherence to instruc-
tion on proper exercise technique;
(3) surgical procedures performed 6
weeks prior to or during the exercise
program; (4) participation in any
other form of exercise, including re-
habilitation, during the study; (5)
changes in medication during the
study; and (6) a major bleeding epi-
sode that posed a risk or prevented
exercise.
Outcome Measures
At the pre-program session, one
physical therapist (R.M.) with 20
years of experience in treating peo-
ple with bleeding disorders was des-
ignated to complete the evaluation
and prescribe an exercise regimen
for each participant based on individ-
ual needs. The same therapist per-
formed the post-program evaluation,
following the same procedures as in
the pre-program evaluation, to en-
sure consistency in testing and exer-
cise prescription.
Pre- and post-program data included
in the musculoskeletal evaluation
were functional walking, ROM, mus-
cle strength, and circumferential
measurements.
Functional walking. The Six-
Minute Walk Test (6MWT) is used to
measure walking ability and baseline
cardiovascular function for people
with disease or low levels of fit-
ness.45 Due to pre-existing comor-
bidities in many of our participants,
we determined that the 6MWT was
the most appropriate test to measure
cardiovascular function. Participants
walked an 800-ft,* unobstructed,
rectangular pathway following the
* 1 ft⫽0.3048 m.
Number 4
guidelines of the American Thoracic
Society.52 To ensure safety and to
measure the exact distance walked
in 6 minutes, the physical therapist
followed closely with a stopwatch
and Rollatape meter.† The Rollatape
meter measures distances in 1-ft in-
crements. The 6MWT is a submaximal,
quantitative evaluation of functional
exercise capacity and is reflective of
ability to perform daily physical activ-
ities.52 A significant correlation (r⫽
.73) between the 6MWT and peak
oxygen uptake has been reported
for patients with end-stage lung dis-
eases. The short-term reproducibility
of 6MWT is excellent.52
ROM. Joint passive ROM of the
knees, hips, ankles, and elbows was
measured with a universal goniome-
ter according to the method de-
scribed by Norkin and White.53 Nor-
kin and White reported the reliability
of goniometric measurement as
good to excellent. In addition to hav-
ing 20 years of experience with our
population, the measuring therapist
helped develop the protocol and
train researchers for goniometric
measurement for people with bleed-
ing disorders for the Universal Data
Collection Study of the Centers for
Disease Control and Prevention.54
Thus, her measurements are consid-
ered highly consistent from patient
to patient and test to test for people
with bleeding disorders.
Muscle strength. Isometric mus-
cle strength of bilateral hip exten-
sion, flexion, and abduction; knee
flexion and extension; and elbow
flexion and extension were mea-
sured with a Nicholas handheld dy-
namometer‡ while the patient was
placed in standard testing positions,
as described by Hislop and Mont-
gomery.55 The Nicholas dynamome-
†
Rollatape, 255 W Fleming, Watseka, IL
60970.
‡
Lafayette Instrument Co, 3700 Sagamore
Pkwy N, Lafayette, IN 47402.
April 2010
 Supervised Exercise Program for People With Bleeding Disorders and Hemophilic Arthritis
ter provides specific, numeric results
that we believe are more valuable in
quantifying changes in ability to re-
sist force. Isometric muscle testing
was used because movement with
maximal resistance can be painful
for many individuals who have lim-
ited pain-free range. Testing isotonic
force production could have re-
sulted in erroneous results, as pain
could inhibit muscle function and in-
dividuals might avoid maximal con-
traction in the painful ROM. The
Nicholas handheld dynamometer
was used to enhance objectivity and
consistency and to measure small in-
crements of change that we pre-
dicted would be necessary for assess-
ing the population. It has been
proven valid and reliable for measur-
ing isometric muscle force.56 –58
Circumferential measurements.
Knees and elbows were measured at
the joint line, as well as 6 in§ above
and 4 in below the joint line, to de-
termine whether joint or muscle
swelling developed. Although we
did not find a study using circumfer-
ential measurement on the lower ex-
tremity, Taylor et al59 reported that
arm volume measured circumferen-
tially was highly reliable. We felt cir-
cumferential measurement would be
the most time-efficient method for
our population when considering
the number of measurements we
were performing. To ensure consis-
tency of measurement, the same
tape measure and landmarks were
used.
Individualized Exercise Program
The levels of intensity and guidelines
for exercise progression used in this
study were devised by the prescrib-
ing physical therapist for this unique
setting and for these participants.
The attempt was to provide a practi-
cal, flexible strategy for exercise that
addressed the varying joint condi-
tions from acute to chronic status
§
1 in⫽2.54 cm.
April 2010
and from stage I to stage V arthrop-
athy. Each participant’s individual-
ized exercise program was pre-
scribed by the evaluating physical
therapist and included exercises for
strength, flexibility, and cardiovascu-
lar function. Each program began
conservatively, with progression of
exercise intensity based on tolerance
of the previously performed exer-
cise. Participants were taught to self-
monitor heart rate and to report any
adverse reactions such as dizziness,
chest pain, muscle bleeds, joint
bleeds, increased pain, swelling, or
fatigue. All major muscle groups
were exercised. However, some par-
ticipants were not able to perform
isotonic strengthening exercises on
all joints due to either severe pain or
joint ankylosis. Likewise, flexibility
exercises were tailored to those
joints that were not ankylosed, yet
had limited motion.
Strengthening. Three intensity
levels were established based on the
available ROM, strength, history of
pain, hemarthroses, and perceived
fragility of each major joint (Appen-
dix 1). The structural integrity of
each joint and the biomechanical
stress of lifting weight also were con-
sidered. For example, maximal resis-
tance with heavy weight was
avoided to prevent excessive and
possibly deleterious compressive
forces. These factors plus the results
of the muscle strength measure were
considered by the therapist when es-
tablishing initial levels of exercise in-
tensity. Although the dynamometry
testing was isometric, exercise pre-
scription was isotonic to promote ac-
tive ROM, muscle endurance, joint
nutrition, proprioception, and motor
control that would not be as effec-
tively gained with isometric exer-
cise. Isotonic exercise at submaxi-
mal intensity was tolerable
throughout the painful ranges.
Level 1 intensity was for the most
fragile joints, identified target joints,
Volume 90
or previously injured muscles.8,9
Strengthening exercise at level 1 was
approximately 40% of the result de-
termined by the isometric Nicholas
dynamometry muscle test with 1 set
of 10 repetitions performed in the
pain-free range.
Level 2 intensity was prescribed for
joints and muscles that had a history
of bleeding and demonstrated mod-
erate hemarthropathy but had no his-
tory of bleeding in the previous 6
months. Strengthening exercise at
level 2 was approximately 50% of the
result determined by the isometric
Nicholas dynamometry muscle test
with 1 set of 10 repetitions in the
pain-free range.
Level 3 intensity was prescribed for
joints and muscles that demon-
strated minimal or no signs of impair-
ment. Level 3 intensity was approx-
imately 60% of the result determined
by the isometric Nicholas dynamom-
etry muscle test with 1 set of 10 to
20 repetitions in the pain-free range.
If no increased pain or swelling oc-
curred, the intensity could be in-
creased by 5% to 10% per week up to
a maximum of 75% of the isometric
Nicholas dynamometry muscle test.
A set of 10 to 20 repetitions was
added in the second week, and a
third set was added in the following
week. By the end of the third week,
a participant who had no adverse
effects could be performing 3 sets of
20 repetitions with up to 75% inten-
sity. Any increase in pain or swelling
required ceasing exercise for that
structure and consulting with the
physical therapist. Options for
strengthening exercise included free
weights, stationary resistance equip-
ment, Thera-Band exercise bands,㛳
and functional strengthening activi-
ties. Although the resistance of
Thera-Band exercise bands cannot
㛳
The Hygenic Corporation, 1245 Home Ave,
Akron, OH 44310.
Number 4 Physical Therapy f 515
Supervised Exercise Program for People With Bleeding Disorders and Hemophilic Arthritis
be quantified, some participants
were not able to safely exercise with
free weights or stationary resistance
equipment due to pain, limited
ROM, or muscle imbalance. Partici-
pants who used Thera-Band exercise
bands exercised in standard posi-
tions and were progressed through
the color hierarchy (Appendix 1).
Functional exercise included walk-
ing, sit-to-stand, and stair-climbing
tasks, with intensity graded based on
required use of upper-extremity sup-
port. Level 1 intensity corresponded
to using 2 hands, level 2 intensity
corresponded to using 1 hand, and
level 3 intensity required no hands to
perform the activity. If a participant
progressed past level 3, exercise pro-
gression was advanced in 1 of 2
ways: either increasing the number
of repetitions of the functional activ-
ity or performing the activity with
weights. Plyometrics were avoided
as the high impact, torsion, and load-
ing of joints were deemed too in-
tense and potentially damaging to
joint structures.
Flexibility and ROM. An individu-
alized exercise program including
soft tissue flexibility and joint ROM
was prescribed based on limitations
found during the evaluation. Due to
the fibrous nature of joint and mus-
cle limitations in people with bleed-
ing disorders, stretching was per-
formed after warming the tissue
with active exercise. Gentle, low-
load, prolonged stretch guidelines
within the pain-free range were
used.60 Each stretch was held for a
minimum of 2 minutes to a maxi-
mum of 20 minutes. For efficiency
with prolonged stretching longer
than 2 minutes, a body part would be
positioned and externally stabilized
with weights or straps in a stretched
position while other body parts were
exercised.
Cardiovascular exercise. Each
participant’s cardiovascular program
began conservatively and was ad-
516 f Physical Therapy Volume 90
justed according to any precautions
identified by the physician or any
difficulties reported by the partici-
pant. The participant’s resting and
post-walking heart rate and respira-
tory rate from the 6MWT were con-
sidered in the development of the
cardiovascular workout. Because all
participants were unaccustomed to
cardiovascular exercise and muscu-
loskeletal impairments prevented
the normal stress testing process, we
used the American College of Sports
Medicine’s formula for estimating
maximal heart rate (estimated HR-
max⫽220 ⫺ age).45 The sessions be-
gan at 50% of maximum heart rate
for the first 2 sessions and pro-
gressed by 5% to 10% increments up
to a maximum of 70% if no adverse
effects were reported. A final consid-
eration was the ability of the partic-
ipant to use equipment. For exam-
ple, none of the participants had
enough ROM in the lower extremi-
ties to exercise on a stationary bi-
cycle. Each participant chose one
cardiovascular activity from the fol-
lowing list: hydrotrack (aquatic
treadmill), therapeutic pool, land-
based treadmill, upper-extremity er-
gometer, cross-country skier, and
low-impact aerobic exercise. For par-
ticipants with severely limited ROM,
aquatic exercise was most effective.
Twenty minutes of cardiovascular
exercise was the goal.
Following the evaluation and individ-
ualized exercise prescription, each
participant was scheduled to begin a
twice-weekly supervised exercise
program with a minimum of 2 days
of rest between each session. Partic-
ipants were not provided with a
home exercise program but were in-
structed to continue with their nor-
mal daily activities. Participants with
severe hemophilia were instructed
to infuse with factor no later than 2
hours prior to each exercise session.
Participants with mild or moderate
hemophilia or von Willebrand dis-
ease were instructed to bring their
Number 4
appropriate coagulation medication
to all exercise sessions. In order to
ensure safety, consistency, and
proper technique, each participant’s
exercise session was supervised by a
fitness instructor who possessed a
master’s degree in fitness and exer-
cise. Every effort was made to edu-
cate the fitness instructor to ensure
safety of all participants. She re-
viewed the publications on bleeding
disorders from the National Hemo-
philia Foundation,61,62 received train-
ing from the physical therapist, and
indicated she understood the tenu-
ous nature of the pathology and the
importance of adherence to the pre-
scribed exercise program. She guided
the participant through the program
on a twice-weekly schedule and re-
corded the exercise performed at each
session. In the event of an adverse re-
action, she was asked to document it
and contact the physical therapist. The
physical therapist consulted with the
fitness instructor weekly to progress
the exercise program. Participants
who missed more than 2 exercise ses-
sions during the 6-week time period
were terminated from the study. At
the end of 6 weeks, each participant
was re-evaluated using the same test-
ing procedures as for the pre-program
evaluation. The physical therapist per-
formed post-program evaluations and
recorded the post-program data on
blank evaluation forms without re-
viewing the pre-program data.
Data Analysis
Analyses were carried out on a per-
sonal computer using a spreadsheet
(Microsoft Excel 2003#). Outcomes
were evaluated with paired t tests.
Data from both the left and right ex-
tremities were combined for analy-
ses. This was done because people
with bleeding disorders have drasti-
cally different patterns of joint de-
struction based on bleeding history
of each individual joint. For exam-
#
Microsoft Corp, One Microsoft Way, Red-
mond, WA 98052-6399.
April 2010
 Supervised Exercise Program for People With Bleeding Disorders and Hemophilic Arthritis

ple, a person with bleeding disorders Table 2.

may have one knee rated at stage V Demographic Characteristics of Study Participants (N⫽33)
on the Arnold and Hilgartner scale, Enrolled in
and the contralateral knee may show Characteristic Study Completed Study
minimal or no joint pathology. This Race
situation is very different from that Asian 1 1
of people with other forms of arthri-
African American 16 6
tis, who tend to have almost symmet-
rical destruction due to a systemic White 16 13

disease process. Sex

Male 30 18
Effect sizes were calculated to ac- Female 3 2
count for group variability. The effect Group
size indexes were calculated for all
Adult 20 12
outcome measures. The calculations
were performed by dividing the differ- Child 13 8

ence between the pre- and post- Age (y)

program means by the standard devi- Adult
ation of the pre-program scores.63 An X 40 40.5
effect size of 0.20 or less represents SD 12.1 12
a small change, 0.21 to 0.80 was con-
Range 19–57 26–27
sidered a medium change, above
0.80 was considered a large change. Child

X 14 15
Role of Funding Source SD 3.2 3.9
This work was supported by Baxter Range 7–18 7–18
Healthcare. Baxter Healthcare had Diagnoses
no involvement in the design, con-
Hemophilia 30 18
duct, or reporting of the study.
Mild 1 1

Results Moderate 3 3
Participants Severe 26 14
Thirty-three participants volun- Von Willebrand disease 3 2
teered for the study (Tab. 2). Twenty Mild 1 1
(61%) of the participants completed
Moderate 2 1
the program, attending a mean of
11.5 of the 12 scheduled sessions, HIV positive 11 9

and returned for post-program data

collection. Thirteen participants
(39%) did not complete the program
due to transportation problems, ill-
ness, or scheduling difficulty. None
of the 33 participants reported any
adverse reactions from the exercise
program.
Anthropometry
ROM. Significant improvement in
ROM was found in all joints when
comparing pre- and post-program
data (Tab. 3). A negative number in-
dicates a lack of range; for example,
the baseline range of knee extension
was ⫺38 to 14 degrees, indicating
one participant lacked 38 degrees of
knee extension and another partici-
pant had 14 degrees of knee
hyperextension.
Strength. Muscle strength was
tested in 17 of 20 participants. Three
participants were not tested at base-
line assessment due to pain. These 3
participants participated in the en-
tire exercise protocol, including
strength training as tolerated, but
were not tested for strength at the
final assessment. One additional par-
ticipant was not tested for hip flex-
ion and extension at post-program
due to low back pain. For the 17
participants analyzed, significant im-
provement was seen in all muscle
groups tested when comparing pre-
and post-program data (Tab. 4).
Circumferential measures. Cir-
cumferential measurements were
taken on all participants. Significant
differences were seen in all joints
and limb segments tested in the up-
per extremity when comparing pre-
and post-program data. However, no

April 2010 Volume 90 Number 4 Physical Therapy f 517

Supervised Exercise Program for People With Bleeding Disorders and Hemophilic Arthritis

Table 3.
Range of Motion (in Degrees) and Effect Sizea

Baseline Measurement Final Measurement 95%

Confidence Effect
Variable X SD Range X SD Range Interval Size

Knee flexionb 112.0 32.0 28 to 149 118.4 30.8 40 to 155 102.1 to 121.9 0.2

Knee extension c
⫺7.1 10.7 ⫺38 to 14 d
⫺2.1 6.9 ⫺18 to 12 d
⫺10.4 to ⫺3.8 0.46

Ankle dorsiflexionb ⫺2.6 10.3 ⫺20 to 20 1.2 10.4 ⫺20 to 26 ⫺5.8 to 0.6 0.36

Ankle plantar flexionb 39.7 10.4 15 to 60 47.0 9.5 30 to 70 36.5 to 42.9 0.7

Elbow flexionc 132.3 17.3 80 to 153 137.2 14.9 80 to 155 126.9 to 137.7 0.28

Elbow extension b
⫺12.4 20.9 ⫺87 to 20 d
⫺8.5 17.9 ⫺75 to 14 d
⫺18.9 to ⫺5.9 0.19

Elbow pronationc 59.0 23.0 5 to 85 64.5 20.1 20 to 92 51.9 to 66.1 0.24

Elbow supinationc 60.3 29.5 ⫺30 to 90 65.3 26.0 ⫺20 to 90 51.2 to 69.4 0.17
a
N⫽40 accounting for bilateral limbs of 20 participants. A negative number indicates a lack of range of motion; for example, the baseline range of knee
extension was ⫺38 to 14 degrees, indicating one participant lacked 38 degrees of knee extension and another participant had 14 degrees of knee
hyperextension.
b
Pⱕ.01.
c
Pⱕ.05.
d
Hyperextension.

differences were found in the lower
extremities (Tab. 5).
Functional walking. The 6MWT
was performed by 19 participants for
pre- and post-program evaluations.
Baseline and final assessments were
not performed on one participant
because of severe pain when walk-
ing. A significant (P⬍.01) improve-
ment with a large effect size (0.90)
was seen when comparing baseline
distance walked (X⫽1,145 ft, SD⫽
318, range⫽376 –1,617) with final
distance walked (X⫽1,431 ft, SD⫽
471, range⫽471–2,297).
Discussion
People with bleeding disorders may
have a wide range of orthopedic and
psychological sequellae of the dis-
ease, including weakness, joint de-
struction, pain, and fear, that may
limit participation in fitness programs.
Safety in exercise is a foremost con-
cern for this population, with scarce
guidance found in the literature.
Our participants represented a typi-
cal cross-section of patients followed
in a hemophilia clinic serving a large
metropolitan area. In addition to is-
sues related to bleeding disorders,
our population had a variety of chal-
lenging comorbidities, including HIV/
AIDS, hepatitis, hypertension, can-
cer, diabetes, and osteogenesis im-
perfecta. Of the 20 participants who
completed the study, 14 had at least
1 joint with severe stage IV or V
arthropathy, and 1 participant had 8
stage V joints.
Due to the severity of joint destruc-
tion, our findings of statistically sig-
nificant gains in ROM in all joints
tested were unexpected. The mean
arc of ROM increased as follows: an-
kle dorsiflexion and plantar flex-
ion⫽11 degrees, knee flexion and

Table 4.
Muscle Strength (in Newtons) and Effect Sizea

Baseline Measurements Final Measurements 95%

Confidence Effect
Variable X SD Range X SD Range Interval Size

Hip extensionb,c 15.6 4.4 0.0 to 26.6 22.9 6.1 4.3 to 35.2 14.1 to 17.1 1.66
b,c
Hip flexion 18.1 6.3 6.3 to 32.6 22.4 6.6 9.5 to 36.3 15.9 to 20.3 0.68

Hip abductionc 16.5 7.9 6.1 to 29.1 21.2 9.4 11.3 to 38.0 13.9 to 19.2 0.59
c
Knee flexion 12.8 4.8 0.8 to 27.2 16.3 5.7 4.6 to 29.0 11.2 to 14.4 0.73

Knee extensionc 15.0 7.8 1.0 to 29.7 20.5 9.9 3.8 to 40.6 12.4 to 17.6 0.71
c
Elbow flexion 14.4 7.4 0.0 to 30.1 15.0 7.8 0.8 to 31.6 11.9 to 16.7 0.08

Elbow extensionc 11.7 8.3 0.0 to 24.8 14.2 8.3 0.4 to 28.8 8.9 to 14.5 0.3
a
N⫽34 accounting for bilateral limbs of 17 participants; 3 participants were not tested before exercise due to pain.
b
n⫽32; at the post-program evaluation, one participant was experiencing low back pain, thus hip extension and flexion were not tested.
c
Pⱕ.01.

518 f Physical Therapy Volume 90 Number 4 April 2010

 Supervised Exercise Program for People With Bleeding Disorders and Hemophilic Arthritis
Table 5.
Circumferential Measurements (in Centimeters) and Effect Sizea
Baseline Measurements
Variable X SD
Knee 6 in above joint line 45.2 7.1
Knee at joint line 37.8 3.3
Knee 4 in below joint line 35.1 4.2
b
Elbow 6 in above joint line 29.8 5.3
Elbow at joint lineb 28.0 3.3
Elbow 4 in below joint linec 25.6 3.5
a
N⫽40 accounting for bilateral limbs of 20 participants.
b
Pⱖ.05.
c
Pⱕ.01.
extension⫽11.4 degrees, elbow flex-
ion and extension⫽8.8 degrees, and
pronation and supination⫽10.5 de-
grees. The overall change in mean
arc of ROM may not appear large.
However, when we realized that
some individuals had no improve-
ment due to ankylosis, the significant
findings can be attributed to large
gains in some joints. For example, in
elbow flexion, 17 of 40 joints
showed 3 degrees or less of improve-
ment in ROM. Three degrees may be
a function of instrument or examiner
error. Therefore, the significant find-
ings were due to large ROM gains in
23 of the 40 joints. Our findings are
consistent with the report of Harris
and Boggio,46 who described the
ROM of large joints in adults with
hemophilic arthritis. They deter-
mined that individuals who partici-
pated in thrice-weekly exercise had
significantly (P⫽.003) better ROM
than those who had not participated
in an exercise program. Our findings
indicate that initiation of an exercise
program in a group of patients with
hemophilic arthritis who had not
previously participated in a regular
exercise program can significantly
improve joint ROM. Clinical rele-
vance can be found in the increase of
function that accompanies increased
ROM. For example, increased ROM
of the elbow can promote the ability
of individuals to reach their head,
April 2010
Final Measurements
Range X SD
32.0 to 66.5 47.2 8.0
31.0 to 46.0 38.1 3.0
28.0 to 45.0 35.5 4.2
19.5 to 40.0 30.4 5.7
21.5 to 33.0 27.6 3.2
19.0 to 33.5 26.3 3.8
neck, and face, allowing them to
wash their face and hair, shave, ap-
ply makeup, brush and floss teeth,
feed themselves, button collars, and
don and doff jewelry or ties.
Strength significantly improved for
all muscle groups. Again, this finding
was unexpected for the following
reasons. In addition to severe ar-
thropathy, 9 (45%) of the partici-
pants in our study were HIV positive
or had AIDS, predisposing them to
sarcopenia.64 The prescribed exer-
cise program was intentionally con-
servative to avoid biomechanical
stress on joints, thus diminishing the
expected strength gains. Strength
gains were not expected in muscles
surrounding ankylosed joints, which
are prevalent in this population. For
example, 13 of the 34 joints tested
for elbow flexion showed gains of 2
N or less, indicating the significant
change in strength was due to large
gains in the remaining 21 joints.
These findings are in concert with
those of Hilberg et al,50 who tested
proprioceptive performance and iso-
metric muscle strength in 9 partici-
pants with hemophilia who took
part in a 6-month specialized training
program.
The specialized training program in-
cluded gentle strength training with
low resistance performed for 20 to
Volume 90
95%
Confidence
Range Interval Effect Size
33.0 to 69.0 43 to 47.4 0.28
32.5 to 44.0 36.8 to 38.8 0.09
28.0 to 45.0 33.8 to 36.4 0.10
19.8 to 41.0 28.2 to 31.4 0.11
22.0 to 32.0 27 to 29 ⫺0.12
19.5 to 31.5 24.5 to 26.7 0.2
25 repetitions. The 9 participants
showed significant (P⬍.05) improve-
ment in maximal isometric leg mus-
cle strength, as measured by leg
press. This low-intensity, high-
repetition program was performed
to apply minimal stress to the lower-
extremity joints. Likewise, Pelletier
et al51 tested the effect of a 3-week
isometric exercise program on a sin-
gle 12-year-old participant with se-
vere factor VIII deficiency and
chronic knee arthrosis. Their inter-
vention produced increased strength
in the right hamstring and quadri-
ceps muscle without adverse effect.
When comparing our study to those
of Hilberg et al50 and Pelletier et al,51
we find similarities in strength gain
with more intensive isotonic exer-
cise. Our participants were pre-
scribed an exercise program based
on their joint integrity, pain, bleed-
ing history, strength, and available
ROM. We allowed participants to
progressively increase intensity of
exercise up to 75% of the pre-
program strength measures. The
clinical relevance of this finding is
that it indicates a more intensive iso-
tonic strengthening program can be
both safe and effective.
Circumferential measurements were
taken at the joint line of the knee and
elbow to determine whether exer-
cise increased joint swelling. Addi-
Number 4 Physical Therapy f 519
Supervised Exercise Program for People With Bleeding Disorders and Hemophilic Arthritis
tional girth measurements were
taken at specific distances above and
below the knee and elbow to serve
as a baseline in the event of a muscle
bleed. We found a small decrease in
the circumferential measure at the
elbow joint line and a small, but sig-
nificant, increase in the upper-
extremity girth measurements. Lower-
extremity girth measurements did
not show significant change. This
result, in combination with clinical
examination and participant self-
report, implies that the exercise pro-
gram did not induce major muscle or
joint bleeding. To elaborate on the
safety of our program, at the conclu-
sion of the study, several participants
with severe hemophilia reported
that their adherence to factor infu-
sion prior to exercise was not 100%;
yet no bleeding episodes were re-
ported resulting from exercise.
These findings agree with those of
Pelletier et al,51 who did not find
adverse effects of exercise on cir-
cumferential measures. Due to the
low level of intensity and short dura-
tion of our program, it is unlikely
that the small increases were due to
muscle hypertrophy or significant
physiologic adaptation. These small
increases may have been due to mea-
surement error. We believe that the
improved performance in strength
measures was likely the result of neu-
romotor adaptation60 and that hyper-
trophy might have been more evi-
dent with a longer period and higher
intensity of exercise. However, hy-
pertrophy is of special interest due
to the sarcopenia related to HIV sta-
tus in several participants.
As previously stated, each limb was
examined as independent observa-
tions with up to 40 observations
made on 20 participants (ROM). This
was done because typically people
with bleeding disorders have asym-
metry in joint destruction. To ad-
dress any concerns about the validity
of pairing data collected from both
the right and left extremities, single-
520 f Physical Therapy Volume 90
limb analyses also were performed.
Similar results were found for ROM,
strength, and circumferential mea-
sures when comparing single and
paired data65,66 (Appendix 2).
Finally, we found significant gains in
distance walked on the 6MWT. This
gain was important for our partici-
pants because it reflects an improve-
ment in functional exercise level
such as walking, activities of daily
living, and self-care.52 Several factors
may have contributed to this func-
tional improvement: increased stride
length from improved ROM; im-
proved muscular endurance; im-
proved cardiopulmonary efficiency;
improved circulation; and improved
biomechanical loading on the joints
from gains in ROM and muscle
strength, resulting in a more com-
fortable and efficient gait.52 The im-
provement in functional walking
also could result from behavioral and
psychological factors such as in-
creased confidence, improved body
image, and decreased fear of move-
ment or injury.
Limitations
Our participants represented a typi-
cal population with bleeding disor-
ders managed at a metropolitan he-
mophilia clinic with a wide range of
ages (7–57 years) and functional abil-
ities ranging from independent to se-
verely limited. This variability allows
us to generalize our results, but at
the same time it creates a number of
limitations in applying the results
that should be recognized. We be-
lieve this trade-off was acceptable
because had stricter inclusion and
exclusion criteria been imposed, the
cohort of participants would have
been diminished. Limitations of our
study included nonrandomization of
participants who served as their own
controls. Safety issues were the high-
est concern; therefore, the indepen-
dent variable (exercise) was not one
specific protocol that could be ap-
plied to all participants, and the pre-
Number 4
scribed conservative strengthening
regimen may have failed to challenge
participants with higher functioning.
One physical therapist performed all
evaluations, developed individual-
ized exercise programs, and modi-
fied the programs, which may have
led to examiner bias. To gather as
much information from the study as
possible, many measurements were
collected and many comparisons
were made, which may have re-
sulted in an inflation of the type I
error rate. Finally, some participants
had pain, which precluded full par-
ticipation for evaluation and inter-
vention. Additional minor limitations
include confounding variables im-
posed upon the health of the partic-
ipants due to concomitant disease.
Long-term follow-up was not for-
mally performed; thus maintenance
of gains were not assessed. Due to
these limitations and limited sample
size of 20 without a control group,
further study is needed to confirm
these results in people with bleeding
disorders.
Conclusions
Hemophilia and bleeding disorders
are rare and may result in lifelong,
chronic musculoskeletal problems
and functional limitations. Physical
therapists are challenged with devel-
oping interventions to protect peo-
ple with bleeding disorders from ep-
isodes of bleeding and joint
destruction but also to find ways to
promote physical function and inde-
pendence. Although exercise is rec-
ommended for recovery from bleed-
ing episodes, as well as for health,
fitness, and enhanced quality of life,
there are few reported evidence-
based studies on the benefits and
safety of exercise in people with
bleeding disorders. Therefore, the
purposes of this study were to inves-
tigate the efficacy, safety, and feasi-
bility of an individually designed ex-
ercise program for people with
bleeding disorders and to initiate the
development of evidence-based
April 2010
 Supervised Exercise Program for People With Bleeding Disorders and Hemophilic Arthritis
guidelines for exercise in people
with bleeding disorders. It is our rec-
ommendation that people with
bleeding disorders should:
• have permission from their physi-
cian to exercise,
• have a thorough physical therapist
evaluation and individualized exer-
cise prescription prior to initiating
exercise,
• have coagulation medications avail-
able for mild and moderate bleed-
ing disorders,
• use prophylactic factor replace-
ment for severe bleeding disorders,
• start with a very conservative base-
line exercise program that accom-
modates weakened joints and
muscles,
• exercise within their pain-free range,
• avoid equipment or activities that
apply biomechanical stress in the
form of impact or torsion,
• expect a longer period of time to
achieve goals, and
• be supervised by a fitness instructor
who has been educated by and
works closely with a physical
therapist.
Following these guidelines, our par-
ticipants improved, with no adverse
events. Ten of the 20 participants
requested to repeat the 6-week exer-
cise program. The results of this
study suggest that the combined ef-
forts of physical therapists and
trained fitness instructors to provide
individually prescribed and super-
vised exercise programs for people
with bleeding disorders can succeed.
Consistent with the goals of Healthy
People 2010,67 physical therapists
can play an important role in the
promotion of health and the devel-
opment of exercise programs for un-
derserved populations and can re-
duce health care disparities for
individuals with disabilities. Our pro-
gram proved effective, safe, and fea-
sible for our population of people
with bleeding disorders receiving
care at a metropolitan hemophilia
April 2010
clinic to improve health, fitness, and
well-being through exercise.
Improvement of function through
exercise in people with bleeding dis-
orders is understudied. Further study
is needed to determine the benefits
of exercise on variables such as gait
parameters and pain and to promote
the development of evidence-based
exercise guidelines. Dividing the par-
ticipants into subgroups based on se-
verity of hemophilia to determine
which patients would gain the most
from an exercise program also
should be explored.
Dr Mulvany, Dr Zucker-Levin, Dr Jeng, and
Dr Dugdale provided concept/idea/research
design and writing. Dr Mulvany, Ms Joyce,
Ms Tuller, and Mr Rose provided data col-
lection. Dr Mulvany, Dr Zucker-Levin, and Dr
Jeng provided data analysis, project man-
agement, and clerical support. Dr Mulvany,
Ms Tuller, and Dr Dugdale provided fund
procurement. Dr Mulvany, Dr Jeng, Ms
Joyce, Ms Tuller, and Dr Dugdale provided
participants. Mr Rose provided facilities/
equipment and institutional liaisons. All au-
thors provided consultation (including re-
view of manuscript before submission). The
authors acknowledge the study participants
for their determination and persistence; An-
gela Redden, fitness instructor; Penny Head,
PT; and all student physical therapy re-
searchers for their significant contributions,
especially: Kellee Berry McBride, David
Grigsby, April Webb, Ed Moyer, and Kristi
Lott-Davidson.
This project was approved by the University
of Tennessee, Health Science Center, Institu-
tional Review Board.
Poster presentations of this work were given
at the World Federation of Hemophilia Meet-
ing; May 20, 2002; Seville, Spain, and the
Annual Conference of the American Physical
Therapy Association; June 18 –22, 2003;
Washington, DC. An oral presentation of this
work was given at the Global Nursing Sym-
posium; September 20 –22, 2005; Dublin,
Ireland.
This work was funded by a grant from Baxter
Healthcare.
This article was received June 26, 2008, and
was accepted December 15, 2009.
DOI: 10.2522/ptj.20080202
Volume 90
References
1 Prevention and control of haemophilia:
memorandum from a joint WHO/WFH
meeting. Bull World Health Organ. 1991;
69:17–26.
2 McDaniel P. Focus on factors. J Intraven
Nurs. 2000;23:282–289.
3 Susman-Shaw A, Harrington C. Haemophilia:
the facts. Nurs Stand. 1999;14:39 – 46.
4 Perry JJ, Alving BM. von Willebrand’s dis-
ease. Am Fam Physician 1990; 41:219 –
224.
5 Medical and Scientific Advisory Commit-
tee (MASAC) Recommendation #132:
Standards and Criteria for the Care of
Persons With Congenital Bleeding Disor-
ders. New York, NY: National Hemophilia
Foundation; approved March 24, 2002.
6 Rodriguez-Merchan EC. Effects of hemo-
philia on articulations of children and
adults. Clin Orthop Relat Res. 1996;328:
7–13.
7 Molho P, Verrier P, Stieltjes N, et al. A
retrospective study on chemical and radio-
active synovectomy in severe haemophilia
patients with recurrent haemarthrosis.
Haemophilia. 1999;5:115–123.
8 Jansen NW, Roosendaal G, Lafeber FP. Un-
derstanding haemophilic arthropathy: an
exploration of current open issues. Br J
Haematol. 2008;143:632– 640.
9 Kern M, Blanchette V, Stain AM, et al. Clin-
ical and cost implications of target joints in
Canadian boys with severe hemophilia A.
J Pediatr. 2004;145:628 – 634.
10 Roosendaal G, Jansen NW, Schutgens R,
Lafeber FP. Haemophilic arthropathy: the
importance of the earliest haemarthroses
and consequences for treatment. Haemo-
philia. 2008;14(suppl 6):4 –10.
11 Choiniere M, Melzack R. Acute and
chronic pain in hemophilia. Pain. 1987;
31:317–331.
12 Johnson RP, Babbitt DP. Five stages of
joint disintegration compared with range
of motion in hemophilia. Clin Orthop Re-
lat Res. 1985;201:36 – 42.
13 Rodriguez-Merchan EC. Pathogenesis,
early diagnosis, and prophylaxis for
chronic hemophilic synovitis. Clin Orthop
Relat Res. 1997;343:6 –11.
14 Arnold WD, Hilgartner MW. Hemophilic
arthropathy: current concepts of patho-
genesis and management. J Bone Joint
Surg Am. 1977;59:287–305.
15 Ashrani AA, Osip J, Christie B, Key NS.
Iliopsoas haemorrhage in patients with
bleeding disorders: experience from one
centre. Haemophilia. 2003;9:721–726.
16 Rodriguez-Merchan EC. Common ortho-
paedic problems in haemophilia. Haemo-
philia. 1999;5(suppl 1):53– 60.
17 Shaheen S, Alasha E. Hemophilic pseudo-
tumor of the distal parts of the radius and
ulna: a case report. J Bone Joint Surg Am.
2005;87:2546 –2549.
18 Valentino LA, Martinowitz U, Doolas A,
Murali P. Surgical excision of a giant pelvic
pseudotumour in a patient with haemo-
philia A. Haemophilia. 2006;12:541–544.
Number 4 Physical Therapy f 521
Supervised Exercise Program for People With Bleeding Disorders and Hemophilic Arthritis
19 Meena AK, Jayalakshmi S, Prasad VS, Mur-
thy JM. Spinal epidural haematoma in a
patient with haemophilia-B. Spinal Cord.
1998;36:658 – 660.
20 Medical and Scientific Advisory Commit-
tee (MASAC) Recommendation #175:
Guidelines for Emergency Department
Management of Individuals With Hemo-
philia. New York, NY: National Hemophilia
Foundation; approved October 15, 2006.
21 Medical and Scientific Advisory Commit-
tee (MASAC) Recommendation #190:
Recommendations Concerning the Treat-
ment of Hemophilia and Other Bleeding
Disorders. New York, NY: National Hemo-
philia Foundation; approved March 21,
2009.
22 Medical and Scientific Advisory Commit-
tee (MASAC) Recommendation #179:
Recommendation Concerning Prophy-
laxis (Regular Administration of Clotting
Factor Concentrate to Prevent Bleeding).
New York, NY: National Hemophilia Foun-
dation; approved November 4, 2007.
23 Medical and Scientific Advisory Commit-
tee (MASAC) Recommendation #185:
Recommendations Regarding Women
With Inherited Bleeding Disorders. New
York, NY: National Hemophilia Founda-
tion; approved November 15, 2008.
24 Arnold DM, Julian JA, Walker IR. Mortality
rates and causes of death among all HIV-
positive individuals with hemophilia in
Canada over 21 years of follow-up. Blood.
2006;108:460 – 464.
25 Manco-Johnson MJ, Abshire TC, Shapiro
AD, et al. Prophylaxis versus episodic
treatment to prevent joint disease in boys
with severe hemophilia. N Engl J Med.
2007;357:535–544.
26 Gilbert MS, Weidel JD. Treatment of He-
mophilia: Current Orthopedic Manage-
ment. New York, NY: National Hemo-
philia Foundation, 1995.
27 Gilbert MS, Radomisli TE. Therapeutic op-
tions in the management of hemophilic
synovitis. Clin Orthop Relat Res.
1997;343:88 –92.
28 Koch B, Cohen S, Luban NC, Eng G. He-
mophiliac knee: rehabilitation techniques.
Arch Phys Med Rehabil.
1982;63:379 –382.
29 Limbird TJ, Dennis SC. Synovectomy and con-
tinuous passive motion (CPM) in hemophiliac
patients. Arthroscopy. 1987;3:74–79.
30 Norian JM, Ries MD, Karp S, Hambleton J.
Total knee arthroplasty in hemophilic ar-
thropathy. J Bone Joint Surg Am. 2002;
84:1138 –1141.
31 Salomon O, Steinberg DM, Seligshon U.
Variable bleeding manifestations charac-
terize different types of surgery in patients
with severe factor XI deficiency enabling
parsimonious use of replacement therapy.
Haemophilia. 2006;12:490 – 493.
32 Silva M, Luck JV Jr. Long-term results of
primary total knee replacement in patients
with hemophilia. J Bone Joint Surg Am.
2005;87:85–91.
33 Dietrich SL. Rehabilitation and nonsurgical
management of musculoskeletal problems
in the hemophilic patient. Ann N Y Acad
Sci. 1975;240:328 –337.
522 f Physical Therapy Volume 90 Number 4
34 Gilbert MS. Should Older Children and
Adults With Hemophilia Participate in
Sports and Recreational Activities? New
York, NY: National Hemophilia Founda-
tion; 1996.
35 Heijnen L, Mauser-Bunschoten EP,
Roosendaal G. Participation in sports by
Dutch persons with haemophilia. Haemo-
philia. 2000;6:537–546.
36 Lane H, Audet M, Herman-Hilker S, Hough-
ton S. Physical Therapy in Bleeding Dis-
orders. New York, NY: National Hemo-
philia Foundation; 2004.
37 Nazzaro AM, Owens S, Hoots WK, Larson
KL. Knowledge, attitudes, and behaviors
of youths in the US hemophilia popula-
tion: results of a national survey. Am J
Public Health. 2006;96:1618 –1622.
38 Greene WB. Musculoskeletal aspects of
hemophilia. Mo Med. 1984;81:136 –140.
39 Buzzard BM. Sports and hemophilia: antag-
onist or protagonist. Clin Orthop Relat
Res. 1996;328:25–30.
40 Engelbert RH, Plantinga M, Van der Net J,
et al. Aerobic capacity in children with he-
mophilia. J Pediatr. 2008;152:833– 838,
838e1.
41 Falk B, Portal S, Tiktinsky R, et al. Anaero-
bic power and muscle strength in young
hemophilia patients. Med Sci Sports Exerc.
2000;32:52–57.
42 Koch B, Galioto FM Jr, Kelleher J, Gold-
stein D. Physical fitness in children with
hemophilia. Arch Phys Med Rehabil.
1984;65:324 –326.
43 Wittmeier K, Mulder K. Enhancing life-
style for individuals with haemophilia
through physical activity and exercise: the
role of physiotherapy. Haemophilia.
2007;13(suppl 2):31–37.
44 Hofstede FG, Fijnvandraat K, Plug I, et al.
Obesity: a new disaster for haemophilic
patients? A nationwide survey. Haemo-
philia. 2008;14:1035–1038.
45 Armstrong LE, Whaley MH, Brubaker PH,
Otto RM. ACSM’s Guidelines for Exercise
Testing and Prescription. 7th ed. Philadel-
phia, PA: Lippincott, Williams & Wilkins;
2006.
46 Harris S, Boggio LN. Exercise may de-
crease further destruction in the adult hae-
mophilic joint. Haemophilia. 2006;12:
237–240.
47 Mulder K. Exercise for People With Hemo-
philia. Montreal, Quebec, Canada: World
Federation of Hemophilia; 2006:44.
48 Jones P, Buzzard BM, Heijnen L. Go for It:
Guidance on Physical Activity and Sports
for People With Haemophilia and Re-
lated Disorders. Montreal, Quebec, Can-
ada: World Federation of Hemophilia;
1998.
49 Anderson A, Forsyth A. Playing It Safe,
Bleeding Disorders, Sports and Exercise.
New York, NY: National Hemophilia Foun-
dation; 2005:44.
50 Hilberg T, Herbsleb M, Puta C, et al. Phys-
ical training increases isometric muscular
strength and proprioceptive performance
in haemophilic subjects. Haemophilia.
2003;9:86 –93.
51 Pelletier JR, Findley TW, Gemma SA. Iso-
metric exercise for an individual with he-
mophilic arthropathy. Phys Ther. 1987;67:
1359 –1364.
52 ATS statement: guidelines for the Six-
Minute Walk Test. Am J Respir Crit Care
Med. 2002;166:111–117.
53 Norkin C, White D. Measurement of Joint
Motion: A Guide to Goniometry. Philadel-
phia, PA: FA Davis Co; 1995.
54 Universal Data Collection Joint Range of
Motion Reference Guide: Orientation
Manual for Physical Therapists. New
York, NY: National Hemophilia Founda-
tion/Centers for Disease Control and Pre-
vention; 1997.
55 Hislop H, Montgomery J. Daniels and
Worthingham’s Muscle Testing: Tech-
niques of Manual Examination. Philadel-
phia, PA: WB Saunders Co; 1995.
56 Byl NN, Richards S, Asturias J. Intrarater
and interrater reliability of strength mea-
surements of the biceps and deltoid using
a hand held dynamometer. J Orthop Sports
Phys Ther. 1988;9:395–398.
57 Surburg PR, Suomi R, Poppy WK. Validity
and reliability of a hand-held dynamome-
ter applied to adults with mental retarda-
tion. Arch Phys Med Rehabil. 1992;73:
535–539.
58 Wadsworth CT, Krishnan R, Sear M, et al.
Intrarater reliability of manual muscle test-
ing and hand-held dynametric muscle test-
ing. Phys Ther. 1987;67:1342–1347.
59 Taylor R, Jayasinghe UW, Koelmeyer L,
et al. Reliability and validity of arm volume
measurements for assessment of lymphed-
ema. Phys Ther. 2006;86:205–214.
60 Kisner C, Colby LA. Therapeutic Exercise
Foundations and Techniques. Philadel-
phia, PA: FA Davis Co; 2007:928.
61 Anderson A, Forsyth A. Playing It Safe.
Bleeding Disorders, Sports and Exercise.
New York, NY: National Hemophilia Foun-
dation; 2000:44.
62 Anderson A, Holtzman TS, Masley J. Phys-
ical Therapy in Bleeding Disorders. New
York, NY: National Hemophilia Founda-
tion; 2000:30.
63 Portney L, Watkins M. Foundations of
Clinical Research: Applications to Prac-
tice. Upper Saddle River, NJ: Pearson Pren-
tice Hall; 2009:892.
64 Capili B, Anastasi JK. Body mass index and
nutritional intake in patients with HIV and
chronic diarrhea: a secondary analysis.
J Am Acad Nurse Pract. 2008;20:
463– 470.
65 Derr J. Valid paired data designs: make full
use of the data without “double-dipping.”
J Orthop Sports Phys Ther. 2006;36:42–
44.
66 Menz HB. Analysis of paired data in phys-
ical therapy research: time to stop double-
dipping? J Orthop Sports Phys Ther. 2005;
35:477– 478.
67 Healthy People 2010. Office of Disease
Prevention and Health Promotion, US
Department of Health and Human
Services. Available at: http://www.healthy-
people.gov/. Accessed January 16, 2008.
April 2010
 Supervised Exercise Program for People With Bleeding Disorders and Hemophilic Arthritis

Appendix 1.
Strength Training Protocol and Progression

1. Progression to next level only if no adverse reaction to previous week of exercise.

2. Prophylaxis: Factor infusion recommended for people with severe hemophilia; people with mild and moderate
hemophilia to have medications available, if needed.

3. Intensity⫽percent of isometric Nicholas dynamometry muscle test (INDMT) to assess pounds of weight to use or
color of Thera-Band exercise band. The Hygenic Corporationa reports correspondence of colors to weight resistance
as the following:

yellow⫽2.5 lb, red⫽4.5 lb, green⫽5.0 lb, blue⫽7.5 lb, black⫽9.0 lb, and silver⫽15 lb

4. Repetition⫽to be done only in pain-free range.

5. Rate⫽5–10 seconds concentric with exhale; 5–10 seconds with inhale.

Level 1: Prescribed for the most fragile joints, target joints, previously injured muscle, and joints with painful active
range of motion, passive range of motion, or weight bearing. No acute swelling or bleeding within past 2 weeks.
No. of
Progression Intensity Repetitions No. of Sets

Week 1 40% 10 1

Week 2 45%–50% 10–20 2

Week 3 50%–60% 10–20 3

Week 4 55%–65% 10–20 3

Week 5 60%–70% 10–20 3

Week 6 65%–75% 10–20 3

Level 2: Prescribed for joints and muscles with history of bleeding and chronic, mild-to-moderate impairment. No
bleeding in past 6 months.
No. of
Progression Intensity Repetitions No. of Sets

Week 1 50% 10 1

Week 2 55%–60% 10–20 2

Week 3 60%–70% 10–20 3

Week 4 65%–75% 10–20 3

Week 5 70%–75% 10–20 3

Week 6 75% 10–20 3

Level 3: Prescribed for joints and muscles with minimal history of bleeding and no signs of impairment.
No. of
Progression Intensity Repetitions No. of Sets

Week 1 60% 10–20 1

Week 2 65%–70% 10–20 2

Week 3 70%–75% 10–20 3

Week 4 75% 10–20 3

Week 5 75% 10–20 3

Week 6 75% 10–20 3

(Continued)

April 2010 Volume 90 Number 4 Physical Therapy f 523

Supervised Exercise Program for People With Bleeding Disorders and Hemophilic Arthritis

Appendix 1.
Continued

Example: Participant 4, a 23-year-old man with severe hemophilia.

Right elbow⫽level 1: Target joints; had 6 episodes of bleeding over past 2 months. Active and passive range of
motion painful at end range of flexion and extension. No acute swelling, no bleeding within past 2 weeks. Right
biceps muscle isometric Nicholas dynamometry muscle test⫽5 lb.

Left elbow⫽level 3: Only 2 episodes of bleeding in past. Last episode of bleeding was 2 years previously. Pain-free
motion, no swelling or crepitus. Normal end-feel. Left biceps muscle isometric Nicholas dynamometry muscle test⫽
30 lb.

Week 1:

Right elbow flexion: 40% ⫻ 5 lb⫽lift 2 lb or use yellow Thera-Band for 1 set of 10 repetitions in pain-free range.

Left elbow flexion: 60% ⫻ 30 lb⫽lift 18 lb or double thickness of black Thera-Band for 1 set of 10 –20 repetitions in
pain-free range.

a
The Hygenic Corporation, 1245 Home Ave, Akron, OH 44310.

524 f Physical Therapy Volume 90 Number 4 April 2010

 Supervised Exercise Program for People With Bleeding Disorders and Hemophilic Arthritis

Appendix 2.
Statistical Results When Separating Out Left Versus Right Joint Results for Range of Motion, Manual Muscle Test, and
Circumferential Measures
Baseline Measurements Final Measurements
Range of Motion (°)
(nⴝ20) X SD Range X SD Range
a
Left knee flexion 115.9 29.0 28 to 149 122.5 28.3 43 to 155

Right knee flexiona 108.1 35.6 40 to 145 114.3 33.3 40 to 152

Left knee extension a

⫺8.2 12.7 ⫺38 to 14 b
⫺2.0 6.8 ⫺16 to 12b

Right knee extension c

⫺6.0 8.9 ⫺24 to 14 b
⫺2.2 7.4 ⫺18 to 10b

Left ankle dorsiflexiona ⫺4.3 9.5 ⫺20 to 10 1.3 10.2 ⫺20 to 20

Right ankle dorsiflexiond ⫺0.9 11.3 ⫺18 to 18 1.2 11.1 ⫺20 to 26

a
Left ankle plantar flexion 38.8 11.1 20 to 50 48.7 9.3 36 to 70

Right ankle plantar flexionc 40.6 10.2 15 to 60 45.4 10.0 30 to 65

c
Left elbow flexion 131.3 20.3 80 to 153 136.4 17.9 80 to 154

Right elbow flexionc 133.4 14.8 105 to 150 138.0 12.2 110 to 155

Left elbow extensiona ⫺12.4 22.6 ⫺87 to 10b ⫺9.5 20.0 ⫺75 to 10b

Right elbow extension c

⫺12.4 20.2 ⫺52 to 20 b
⫺7.5 16.5 ⫺40 to 14b

Left elbow pronationc 60.5 20.0 10 to 85 65.5 18.5 20 to 92

Right elbow pronationd 57.5 26.7 5 to 85 63.6 22.4 20 to 90

Left elbow supinationd 65.3 24.4 0 to 90 68.8 21.9 15 to 90

Right elbow supinationd 55.3 34.5 ⫺30 to 90 61.7 30.3 ⫺20 to 90

a
Pⱕ.01.
b
Hypertension.
c
Pⱕ.05.
d
Not significant.

Baseline Measurements Final Measurements

Muscle Strength (N)
(nⴝ17) X SD Range X SD Range
a,b
Left hip extension 15.9 6.8 4.3 to 26.6 22.9 8.5 7.1 to 39.0

Right hip extensiona,b 15.4 6.9 0.0 to 24.1 22.8 8.6 4.3 to 33.5
a,b
Left hip flexion 18.6 5.6 8.1 to 32.6 23.4 6.3 13.0 to 36.3

Right hip flexiona,b 17.7 6.5 6.3 to 30.2 21.3 7.0 9.5 to 30.7
b
Left hip abduction 16.2 5.6 7.2 to 29.1 21.6 7.8 11.4 to 38.0

Right hip abductionc 16.7 6.4 6.1 to 25.9 20.7 8.4 10.9 to 36.5
b
Left knee extension 14.4 7.2 1.0 to 23.8 19.9 9.2 4.3 to 28.5

Right knee extensionb 15.6 8.5 2.1 to 29.7 21.0 10.8 3.8 to 40.6
c
Left knee flexion 12.1 5.4 0.8 to 21.6 15.6 6.7 4.6 to 28.6

Right knee flexionb 13.5 6.8 4.0 to 27.2 17.0 6.3 6.6 to 29.0
c
Left elbow flexion 13.6 7.6 1.6 to 30.1 16.7 7.6 2.3 to 28.4

Right elbow flexionc 15.1 7.5 0.0 to 29.6 17.4 8.1 0.8 to 31.6
b
Left elbow extension 11.2 6.4 0.3 to 16.6 13.6 7.4 0.4 to 26.0

Right elbow extensionc 12.2 6.5 0.0 to 24.8 14.8 7.2 0.5 to 28.8
a
n⫽16.
b
Pⱕ.01.
c
Pⱕ.05.
(Continued)

April 2010 Volume 90 Number 4 Physical Therapy f 525

Supervised Exercise Program for People With Bleeding Disorders and Hemophilic Arthritis

Appendix 2.
Continued
Baseline Measurement Final Measurement
Circumferential Measure
(cm) (nⴝ20) X SD Range X SD Range

Left knee 6 in above joint linea 45.0 6.8 33.5 to 60.0 47.0 8.3 33.0 to 69.0

Right knee 6 in above joint linea 45.0 7.7 32.0 to 66.5 47.0 8.1 33.0 to 68.5

Left knee at joint lineb 37.9 3.6 31.0 to 46.0 38.3 3.2 32.5 to 44.0
b
Right knee at joint line 37.7 3.1 32.5 to 43.0 37.9 2.9 32.5 to 43.5

Left knee 4 in below joint lineb 28.6 4.4 28.0 to 43.5 32.9 4.4 28.0 to 42.5
b
Right knee 4 in below joint line 28.6 4.3 28.0 to 45.0 32.4 4.2 28.0 to 45.0

Left elbow 6 in above joint linec 29.0 5.4 21.0 to 38.0 30.0 5.8 19.8 to 37.5
b
Right elbow 6 in above joint line 30.0 5.5 19.5 to 40.0 31.0 5.8 20.0 to 42.0

Left elbow at joint lineb 27.7 3.3 21.5 to 33.0 27.3 3.2 22.0 to 31.5
b
Right elbow at joint line 28.2 3.4 22.5 to 33.0 27.8 3.3 22.0 to 32.0

Left elbow 4 in below joint linea 25.3 3.6 19.0 to 32.0 26.2 4.0 19.5 to 34.0
b
Right elbow 4 in below joint line 25.9 3.7 21.0 to 33.5 26.4 3.7 19.5 to 32.0
a
Pⱕ.01.
b
Not significant.
c
Pⱕ.05.

526 f Physical Therapy Volume 90 Number 4 April 2010

Copyright of Physical Therapy is the property of American Physical Therapy Association and its content may
not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written
permission. However, users may print, download, or email articles for individual use.