Research Report Effects of a 6-Week, Individualized, Supervised Exercise Program for People With Bleeding Disorders and Hemophilic Arthritis
R. Mulvany, PT, DPT, is Associate Professor, Department of Physical Therapy, College of Allied Health Sciences, University of Tennessee Health Science Center, 930 Madison Ave, 6th Floor, Memphis, TN 38163 (USA). Address all correspondence to Dr Mulvany at:
[email protected].
Ruth Mulvany, Audrey R. Zucker-Levin, Michael Jeng, Catherine Joyce, Janet Tuller, Jonathan M. Rose, Marion Dugdale
Background. People with bleeding disorders may develop severe arthritis due to joint hemorrhages. Exercise is recommended for people with bleeding disorders, but guidelines are vague and few studies document efficacy. In this study, 65% of people with bleeding disorders surveyed reported participating in minimal exercise, and 50% indicated a fear of exercise-induced bleeding, pain, or physical impairment.
A.R. Zucker-Levin, PT, PhD, MBA, GCS, is Associate Professor, Department of Physical Therapy, College of Allied Health Sciences, University of Tennessee Health Science Center.
Objective. The purpose of this study was to examine the feasibility, safety, and
M. Jeng, MD, is Associate Professor of Pediatrics, Stanford University, Palo Alto, California.
efficacy of a professionally designed, individualized, supervised exercise program for people with bleeding disorders.
Design. A single-group, pretest-posttest clinical design was used. Methods. Thirty-three patients (3 female, 30 male; 7–57 years of age) with mild to severe bleeding disorders were enrolled in the study. Twelve patients had coexisting illnesses, including HIV/AIDS, hepatitis, diabetes, fibromyalgia, neurofibromatosis, osteopenia, osteogenesis imperfecta, or cancer. Pre- and post-program measures included upper- and lower-extremity strength (force-generating capacity), joint range of motion, joint and extremity circumference, and distance walked in 6 minutes. Each patient was prescribed a 6-week, twice-weekly, individualized, supervised exercise program. Twenty participants (61%) completed the program.
Results. Pre- and post-program data were analyzed by paired t tests for all participants who completed the program. No exercise-induced injuries, pain, edema, or bleeding episodes were reported. Significant improvements occurred in joint motion, strength, and distance walked in 6 minutes, with no change in joint circumference. The greatest gains were among the individuals with the most severe joint damage and coexisting illness. Limitations. Limitations included a small sample size with concomitant disease, which is common to the population, and a nonblinded examiner.
Conclusions. A professionally designed and supervised, individualized exercise program is feasible, safe, and beneficial for people with bleeding disorders, even in 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.
C. Joyce, MSW, is affiliated with the Comprehensive Hemophilia Clinic, Department of Hematology, College of Medicine, University of Tennessee Health Science Center. J. Tuller, RN, MPH, is Clinical Nurse Coordinator, Comprehensive Hemophilia Clinic, Department of Hematology, College of Medicine, University of Tennessee Health Science Center. J.M. Rose, PT, MS, ATC, Assistant Professor, Department of Physical Therapy, Health Science Center, College of Allied Health Sciences, University of Tennessee. M. Dugdale, MD, is Medical Director, Comprehensive Hemophilia Clinic, Department of Hematology, College of Medicine, University of Tennessee Health Science Center. [Mulvany R, Zucker-Levin AR, Jeng M, et al. Effects of a 6-week, individualized, supervised exercise program for people with bleeding disorders and hemophilic arthritis. Phys Ther. 2010;90:509 –526.] © 2010 American Physical Therapy Association
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B
leeding disorders and the musculoskeletal pathologies that accompany them offer numerous challenges to health care providers. Hemophilia A, hemophilia B, and von Willebrand disease are the most common inherited bleeding disorders. 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 almost exclusively in males, whereas females are carriers of the trait.1–3 Von Willebrand disease is caused by an inherited defect in or deficiency
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of von Willebrand factor and affects both sexes equally.4 Bleeding disorders can be classified as mild, moderate, or severe, depending on the amount or efficacy of circulating factor levels. In moderate or severe bleeding disorders, minor trauma can cause bleeding in joints (hemarthrosis) or in muscles (hematoma). This bleeding may initiate a cycle of musculoskeletal degeneration leading to disabling arthritis.5,6 Hemarthrosis is the most common and disabling manifestation of hemophilia. Approximately 80% of hemorrhages associated with hemophilia are hemarthroses.5 Hemarthroses usually begin around 12 to 24 months of age and persist throughout life.5 Joints most frequently affected 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 period of 3 to 6 months is a target joint and is much more susceptible to subsequent bleeding and arthritic changes.8,9
Repeated bleeding into joints causes hemophilic arthritis (hemarthropathy), producing joint tissue destruction similar to that seen in rheumatoid arthritis. This destruction results in invasive hypervascular synovial hypertrophy, chronic synovitis, articular cartilage damage, bony hypertrophy, and subchondral cysts.5–10 Hemophilic arthritis is manifested by pain,11 joint instability, malalignment, muscular atrophy, impaired range of motion (ROM), and impaired function5–7,11–13 (Fig. 1). Arnold and Hilgartner14 described 5 radiographic stages in the progression of hemophilic arthropathy (Tab. 1). In stage I, joint integrity is maintained with no skeletal or cartilaginous changes. In stage V, the articular cartilage is fibrotic and deteriorated with a complete loss of joint space.14 Many joints are ankylosed at this stage, leaving joint replacement the only viable option for functional movement (Fig. 2). In addition to hemarthroses, bleeding may occur directly into a muscle. The most commonly affected muscles are the iliopsoas, quadriceps,
Figure 1. Severe chronic synovitis in hemophilia, with invasive hypervascular synovial hypertrophy. Joint arthropathy led to total knee replacement in this young man.
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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
Skeleton
Cartilage
Synovium
Clinical Presentation: Progression Along the Continuum
I
No change
No change
No change
Swelling of synovium and soft tissues
Acute hemarthrosis: as blood fills joint capsule, joint becomes tense, swollen, hard, hot, and tender; often held in flexion, with restricted range of motion and pain
II
No change
Osteoporosis, especially of epiphyses; epiphyseal overgrowth
No change
Swollen, thickened, boggy; early reaction similar to rheumatoid arthritis
Subacute hemarthrosis: after 2 or more hemarthroses; thickened and boggy synovium, moderate restriction of range of motion; pigmented villonodular synovitis, similar to rheumatoid arthritis
III
Disorganization of joint
Osteoporosis, subchondral cysts, progressive overgrowth of epiphyses, widening of intercondylar notch of knee and trochlear notch of ulna
No significant narrowing of joint space; squaring of patella
Opacified, with hemosiderin deposits; synovial hypertrophy and vascular hyperplasia
Chronic hemarthrosis: after subacute joint involvement has been present for ⱖ6 mo
IV
Advanced disorganization, irreversible joint changes
Progression of stages II and III
Severe cartilage destruction, narrowed joint space, osteochondral lesion, fibrillation and erosion, irreversible changes
Opacified and fibrous
Destructive progression to end stage
V
Marked fibrosis, substantial disorganization of structures, irreversible changes
Extensive enlargement of epiphyses, enburnated bone ends
Loss of joint space, absence of cartilage
Little or no recognizable synovial tissue
Chronic, fibrotic, contracted; totally destroyed joint
gastrocnemius-soleus, and muscles of the forearm. Bleeding into muscles can cause severe problems, including compartment syndrome, neurovascular compromise, fibrosis, adhesions, contractures, hematomas, and pseudotumors.15–18 (Fig. 3). Less common sites for bleeding are the gastrointestinal tract, vital organs, 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
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Overview of Management for Bleeding Disorders Currently, there are 3 major methods of managing hemophilia: medications to promote clotting and to manage pain and coexisting diseases, surgery, and rehabilitative exercise. Medications to Promote Clotting Depending on severity, medical management options for hemophilia include infusion of concentrated purified factor replacement5,21,22 or a nasal spray to stimulate the release of stored factor into the bloodstream for clotting control.21 Likewise, man-
agement of von Willebrand disease depends on the type and severity, with medical options including tablets, liquids, nasal sprays, and factor concentrate infusions.21,23 Historically, the management of hemophilia and its secondary musculoskeletal impairments was greatly complicated by factor replacements derived from contaminated blood products, leading to the spread of HIV, hepatitis, and other blood-borne pathogens.24 Currently, the risk of secondary infection is diminished by the availability of purified factor replacement. Some pa-
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Figure 2. Bilateral stage IV and V elbows and knees due to multiple hemarthroses.
tients will use factor replacement as needed (episodic), whereas others receive 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 arthritis and osteoarthritis, with the added complications of bleeding and coexisting diseases acquired from blood products.26 –32 Some of the most common orthopedic procedures are synovectomy, arthroscopy, total joint arthroplasty, and arthrodesis.26,28 –30,32,33 To ensure an optimal outcome, the medical team should involve the primary care doctor, surgeon, anesthetist, hematologist, nurse, physical therapist, occupational therapist, and social worker. Because of the complexities of rehabilitation, physical therapy should be initiated before and continued well after surgery.27,28,31,33 512
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Rehabilitation and Exercise A major component of rehabilitation for people with hemarthropathy and muscle bleeds is therapeutic exercise.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 bleeding and trauma, and not enough stress, producing a subtherapeutic response. This fine line between beneficial and detrimental activity has led many people 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 quality of life. When surveyed by the National Hemophilia Foundation’s National 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
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generally have decreased strength (force-generating capacity) and flexibility, are less active, and have lower aerobic working capacity than their unaffected peers.33,34,36 – 43 These inadequate levels of strength and flexibility leave people with bleeding disorders even more vulnerable because strong, flexible muscles support joints, help attenuate stresses, and diminish the risk of injury.37 Thus, people with bleeding disorders who exercise appropriately may improve their strength and flexibility, which, in turn, could diminish the chances of developing recurrent hemarthrosis, synovitis, and subsequent joint destruction.34 – 43 Furthermore, because obesity and heart disease are as prevalent in people with bleeding disorders as they are in the general population, cardiovascular fitness should be included in any exercise program.44,45 Literature that examines the role of exercise on function in people with bleeding disorders is limited. Harris and Boggio46 performed a descripApril 2010
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Figure 3. Massive pseudotumor and compartment syndrome due to repeated hemorrhages in the gastrocnemius muscle.
tive review of 46 people with bleeding disorders and found that those who reported regular exercise had significantly greater ROM in the large joints than those who did not exercise. Likewise, Wittmeier and Mulder43,47 described the benefits of exercise and hypothesized that people with bleeding disorders would benefit from sports, fitness, and physical activity. In addition to these reports, experienced therapists have provided guidelines for exercise safety in people with bleeding disorders in publications by the World Federation of Hemophilia47,48 and the National 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 program including gentle strength training with low resistance. Significant improvements in isometric muscle strength and proprioceptive performance were found, and generic guidelines for strengthening in people with bleeding disorders were provided, which include the use of low-resistance, high-repetition exercises over a 6-month period. Likewise, Pelletier et al,51 in a quasiexperimental study, found strength gains after a 3-week isometric exercise program in a single 12-year-old participant. These studies provide limited guidance in decision making when prescribing exercises to people with bleeding disorders. The literature implies that people with bleeding disorders could bene-
fit from a structured, supervised exercise 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 determine whether people with bleeding disorders can safely and effectively exercise and achieve improved levels of function using an individualized exercise program that was designed by a physical therapist and supervised by a trained fitness instructor. In contrast to the studies by Hilberg et al50 and Pelletier et al,51 our study offers a comprehensive ex-
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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 clinical design was used, with measurements taken prior to initiation of the supervised exercise program and immediately following the 6-week intervention. Participants Participant selection for this Institutional Review Board–approved study was based on a sample of convenience 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 authorized representatives for those volunteers 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 cohort of people with bleeding disorders. The study group had a range of severity of hemophilic arthritis: some had previous orthopedic surgery, 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 hemophilia or von Willebrand disease; (2) willingness to exercise twice a week for 6 weeks and to complete the preand post-program evaluations; (3) ability to arrange transportation to and from data collection and exercise sessions; (4) approval by their hematologist to participate in the exercise program; (5) aged 7 to 60 years; and (6) agreement of those 514
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participants with severe hemophilia to receive a prophylactic intravenous dose of factor prior to exercise. Exclusion criteria were: (1) the inability to attend exercise sessions at least twice a week for 6 consecutive weeks; (2) nonadherence to instruction 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 rehabilitation, during the study; (5) changes in medication during the study; and (6) a major bleeding episode 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 people with bleeding disorders was designated to complete the evaluation and prescribe an exercise regimen for each participant based on individual needs. The same therapist performed the post-program evaluation, following the same procedures as in the pre-program evaluation, to ensure consistency in testing and exercise prescription. Pre- and post-program data included in the musculoskeletal evaluation were functional walking, ROM, muscle strength, and circumferential measurements. Functional walking. The SixMinute Walk Test (6MWT) is used to measure walking ability and baseline cardiovascular function for people with disease or low levels of fitness.45 Due to pre-existing comorbidities 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.
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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 increments. The 6MWT is a submaximal, quantitative evaluation of functional exercise capacity and is reflective of ability to perform daily physical activities.52 A significant correlation (r⫽ .73) between the 6MWT and peak oxygen uptake has been reported for patients with end-stage lung diseases. 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 goniometer according to the method described by Norkin and White.53 Norkin and White reported the reliability of goniometric measurement as good to excellent. In addition to having 20 years of experience with our population, the measuring therapist helped develop the protocol and train researchers for goniometric measurement for people with bleeding disorders for the Universal Data Collection Study of the Centers for Disease Control and Prevention.54 Thus, her measurements are considered highly consistent from patient to patient and test to test for people with bleeding disorders. Muscle strength. Isometric muscle strength of bilateral hip extension, flexion, and abduction; knee flexion and extension; and elbow flexion and extension were measured with a Nicholas handheld dynamometer‡ while the patient was placed in standard testing positions, as described by Hislop and Montgomery.55 The Nicholas dynamome† Rollatape, 255 W Fleming, Watseka, IL 60970. ‡ Lafayette Instrument Co, 3700 Sagamore Pkwy N, Lafayette, IN 47402.
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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 resist force. Isometric muscle testing was used because movement with maximal resistance can be painful for many individuals who have limited pain-free range. Testing isotonic force production could have resulted in erroneous results, as pain could inhibit muscle function and individuals might avoid maximal contraction in the painful ROM. The Nicholas handheld dynamometer was used to enhance objectivity and consistency and to measure small increments of change that we predicted would be necessary for assessing the population. It has been proven valid and reliable for measuring 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 determine whether joint or muscle swelling developed. Although we did not find a study using circumferential measurement on the lower extremity, Taylor et al59 reported that arm volume measured circumferentially was highly reliable. We felt circumferential measurement would be the most time-efficient method for our population when considering the number of measurements we were performing. To ensure consistency 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 prescribing physical therapist for this unique setting and for these participants. The attempt was to provide a practical, flexible strategy for exercise that addressed the varying joint conditions from acute to chronic status §
1 in⫽2.54 cm.
April 2010
and from stage I to stage V arthropathy. Each participant’s individualized exercise program was prescribed by the evaluating physical therapist and included exercises for strength, flexibility, and cardiovascular function. Each program began conservatively, with progression of exercise intensity based on tolerance of the previously performed exercise. Participants were taught to selfmonitor 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 participants 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 (Appendix 1). The structural integrity of each joint and the biomechanical stress of lifting weight also were considered. For example, maximal resistance 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 establishing initial levels of exercise intensity. Although the dynamometry testing was isometric, exercise prescription was isotonic to promote active ROM, muscle endurance, joint nutrition, proprioception, and motor control that would not be as effectively gained with isometric exercise. Isotonic exercise at submaximal intensity was tolerable throughout the painful ranges. Level 1 intensity was for the most fragile joints, identified target joints,
or previously injured muscles.8,9 Strengthening exercise at level 1 was approximately 40% of the result determined 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 moderate hemarthropathy but had no history 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 demonstrated minimal or no signs of impairment. Level 3 intensity was approximately 60% of the result determined by the isometric Nicholas dynamometry muscle test with 1 set of 10 to 20 repetitions in the pain-free range. If no increased pain or swelling occurred, the intensity could be increased 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% intensity. 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 equipment, Thera-Band exercise bands,㛳 and functional strengthening activities. Although the resistance of Thera-Band exercise bands cannot 㛳
The Hygenic Corporation, 1245 Home Ave, Akron, OH 44310.
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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. Participants who used Thera-Band exercise bands exercised in standard positions and were progressed through the color hierarchy (Appendix 1). Functional exercise included walking, sit-to-stand, and stair-climbing tasks, with intensity graded based on required use of upper-extremity support. 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 progression was advanced in 1 of 2 ways: either increasing the number of repetitions of the functional activity or performing the activity with weights. Plyometrics were avoided as the high impact, torsion, and loading of joints were deemed too intense and potentially damaging to joint structures. Flexibility and ROM. An individualized 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 muscle limitations in people with bleeding disorders, stretching was performed after warming the tissue with active exercise. Gentle, lowload, prolonged stretch guidelines within the pain-free range were used.60 Each stretch was held for a minimum of 2 minutes to a maximum 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 ad516
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justed according to any precautions identified by the physician or any difficulties reported by the participant. The participant’s resting and post-walking heart rate and respiratory rate from the 6MWT were considered in the development of the cardiovascular workout. Because all participants were unaccustomed to cardiovascular exercise and musculoskeletal impairments prevented the normal stress testing process, we used the American College of Sports Medicine’s formula for estimating maximal heart rate (estimated HRmax⫽220 ⫺ age).45 The sessions began at 50% of maximum heart rate for the first 2 sessions and progressed by 5% to 10% increments up to a maximum of 70% if no adverse effects were reported. A final consideration was the ability of the participant to use equipment. For example, none of the participants had enough ROM in the lower extremities to exercise on a stationary bicycle. Each participant chose one cardiovascular activity from the following list: hydrotrack (aquatic treadmill), therapeutic pool, landbased treadmill, upper-extremity ergometer, cross-country skier, and low-impact aerobic exercise. For participants with severely limited ROM, aquatic exercise was most effective. Twenty minutes of cardiovascular exercise was the goal. Following the evaluation and individualized 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. Participants were not provided with a home exercise program but were instructed to continue with their normal 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 disease were instructed to bring their
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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 exercise. Every effort was made to educate the fitness instructor to ensure safety of all participants. She reviewed the publications on bleeding disorders from the National Hemophilia Foundation,61,62 received training from the physical therapist, and indicated she understood the tenuous nature of the pathology and the importance of adherence to the prescribed exercise program. She guided the participant through the program on a twice-weekly schedule and recorded the exercise performed at each session. In the event of an adverse reaction, 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 sessions 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 testing procedures as for the pre-program evaluation. The physical therapist performed post-program evaluations and recorded the post-program data on blank evaluation forms without reviewing the pre-program data. Data Analysis Analyses were carried out on a personal computer using a spreadsheet (Microsoft Excel 2003#). Outcomes were evaluated with paired t tests. Data from both the left and right extremities were combined for analyses. This was done because people with bleeding disorders have drastically different patterns of joint destruction based on bleeding history of each individual joint. For exam# Microsoft Corp, One Microsoft Way, Redmond, WA 98052-6399.
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Supervised Exercise Program for People With Bleeding Disorders and Hemophilic Arthritis ple, a person with bleeding disorders may have one knee rated at stage V on the Arnold and Hilgartner scale, and the contralateral knee may show minimal or no joint pathology. This situation is very different from that of people with other forms of arthritis, who tend to have almost symmetrical destruction due to a systemic disease process.
Table 2. Demographic Characteristics of Study Participants (N⫽33) Characteristic
Enrolled in Study
Race Asian
1 16
6
White
16
13
30
18
3
2
Adult
20
12
Child
13
8
Sex
Female Group
Age (y) Adult X
40
40.5
SD
12.1
12
Range
19–57
26–27
14
15
Child X
Role of Funding Source This work was supported by Baxter Healthcare. Baxter Healthcare had no involvement in the design, conduct, or reporting of the study.
SD
3.2
3.9
Range
7–18
7–18
Diagnoses Hemophilia Mild
Results
Moderate
Participants Thirty-three participants volunteered for the study (Tab. 2). Twenty (61%) of the participants completed the program, attending a mean of 11.5 of the 12 scheduled sessions, and returned for post-program data collection. Thirteen participants (39%) did not complete the program due to transportation problems, illness, or scheduling difficulty. None of the 33 participants reported any adverse reactions from the exercise program.
Severe
Anthropometry ROM. Significant improvement in ROM was found in all joints when comparing pre- and post-program data (Tab. 3). A negative number indicates a lack of range; for example, the baseline range of knee extension was ⫺38 to 14 degrees, indicating April 2010
1
African American
Male
Effect sizes were calculated to account for group variability. The effect size indexes were calculated for all outcome measures. The calculations were performed by dividing the difference between the pre- and postprogram means by the standard deviation of the pre-program scores.63 An effect size of 0.20 or less represents a small change, 0.21 to 0.80 was considered a medium change, above 0.80 was considered a large change.
Completed Study
Von Willebrand disease Mild Moderate HIV positive
one participant lacked 38 degrees of knee extension and another participant had 14 degrees of knee hyperextension. Strength. Muscle strength was tested in 17 of 20 participants. Three participants were not tested at baseline assessment due to pain. These 3 participants participated in the entire exercise protocol, including strength training as tolerated, but were not tested for strength at the final assessment. One additional participant was not tested for hip flex-
30
18
1
1
3
3
26
14
3
2
1
1
2
1
11
9
ion and extension at post-program due to low back pain. For the 17 participants analyzed, significant improvement was seen in all muscle groups tested when comparing preand post-program data (Tab. 4). Circumferential measures. Circumferential measurements were taken on all participants. Significant differences were seen in all joints and limb segments tested in the upper extremity when comparing preand post-program data. However, no
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Supervised Exercise Program for People With Bleeding Disorders and Hemophilic Arthritis Table 3. Range of Motion (in Degrees) and Effect Sizea Baseline Measurement Variable Knee flexionb Knee extension
c
Ankle dorsiflexionb Ankle plantar flexionb Elbow flexionc
X
SD
112.0
32.0
95% Confidence Interval
Final Measurement
Range 28 to 149
⫺7.1
10.7
⫺38 to 14
⫺2.6
10.3
⫺20 to 20
39.7
10.4
15 to 60
132.3
17.3
80 to 153
X
SD
118.4
30.8
Range 40 to 155
⫺2.1
6.9
⫺18 to 12
1.2
10.4
⫺20 to 26
47.0
9.5
137.2
14.9
d
102.1 to 121.9
Effect Size 0.2
⫺10.4 to ⫺3.8
0.46
⫺5.8 to 0.6
0.36
30 to 70
36.5 to 42.9
0.7
80 to 155
126.9 to 137.7
0.28
d
b
⫺12.4
20.9
⫺87 to 20
⫺8.5
17.9
⫺75 to 14
⫺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
Elbow extension
d
d
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 walking. A significant (P⬍.01) improvement 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
lenging comorbidities, including HIV/ AIDS, hepatitis, hypertension, cancer, diabetes, and osteogenesis imperfecta. 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.
People with bleeding disorders may have a wide range of orthopedic and psychological sequellae of the disease, including weakness, joint destruction, pain, and fear, that may limit participation in fitness programs. Safety in exercise is a foremost concern for this population, with scarce guidance found in the literature. Our participants represented a typical cross-section of patients followed in a hemophilia clinic serving a large metropolitan area. In addition to issues related to bleeding disorders, our population had a variety of chal-
Due to the severity of joint destruction, our findings of statistically significant gains in ROM in all joints tested were unexpected. The mean arc of ROM increased as follows: ankle dorsiflexion and plantar flexion⫽11 degrees, knee flexion and
Table 4. Muscle Strength (in Newtons) and Effect Sizea
X
SD
Range
X
SD
Range
95% Confidence Interval
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
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
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
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
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
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
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
Baseline Measurements Variable Hip extensionb,c Hip flexion
b,c
Hip abductionc Knee flexion
c
Knee extensionc Elbow flexion
c
Elbow extensionc a b c
Final Measurements
Effect Size
N⫽34 accounting for bilateral limbs of 17 participants; 3 participants were not tested before exercise due to pain. n⫽32; at the post-program evaluation, one participant was experiencing low back pain, thus hip extension and flexion were not tested. Pⱕ.01.
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Supervised Exercise Program for People With Bleeding Disorders and Hemophilic Arthritis Table 5. Circumferential Measurements (in Centimeters) and Effect Sizea Baseline Measurements Variable
95% Confidence Interval
Final Measurements
X
SD
Range
X
SD
Range
45.2
7.1
32.0 to 66.5
47.2
8.0
33.0 to 69.0
43 to 47.4
0.28
Knee at joint line
37.8
3.3
31.0 to 46.0
38.1
3.0
32.5 to 44.0
36.8 to 38.8
0.09
Knee 4 in below joint line
35.1
4.2
28.0 to 45.0
35.5
4.2
28.0 to 45.0
33.8 to 36.4
0.10
Knee 6 in above joint line
Elbow 6 in above joint line
b
Effect Size
29.8
5.3
19.5 to 40.0
30.4
5.7
19.8 to 41.0
28.2 to 31.4
Elbow at joint lineb
28.0
3.3
21.5 to 33.0
27.6
3.2
22.0 to 32.0
27 to 29
⫺0.12
0.11
Elbow 4 in below joint linec
25.6
3.5
19.0 to 33.5
26.3
3.8
19.5 to 31.5
24.5 to 26.7
0.2
a
N⫽40 accounting for bilateral limbs of 20 participants. b Pⱖ.05. c Pⱕ.01.
extension⫽11.4 degrees, elbow flexion and extension⫽8.8 degrees, and pronation and supination⫽10.5 degrees. The overall change in mean arc of ROM may not appear large. However, when we realized that some individuals had no improvement 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 improvement in ROM. Three degrees may be a function of instrument or examiner error. Therefore, the significant findings 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 determined that individuals who participated 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 relevance 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
neck, and face, allowing them to wash their face and hair, shave, apply 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 arthropathy, 9 (45%) of the participants in our study were HIV positive or had AIDS, predisposing them to sarcopenia.64 The prescribed exercise program was intentionally conservative 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 isometric muscle strength in 9 participants with hemophilia who took part in a 6-month specialized training program. The specialized training program included gentle strength training with low resistance performed for 20 to
25 repetitions. The 9 participants showed significant (P⬍.05) improvement in maximal isometric leg muscle strength, as measured by leg press. This low-intensity, highrepetition program was performed to apply minimal stress to the lowerextremity joints. Likewise, Pelletier et al51 tested the effect of a 3-week isometric exercise program on a single 12-year-old participant with severe factor VIII deficiency and chronic knee arthrosis. Their intervention produced increased strength in the right hamstring and quadriceps 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 exercise. Our participants were prescribed an exercise program based on their joint integrity, pain, bleeding history, strength, and available ROM. We allowed participants to progressively increase intensity of exercise up to 75% of the preprogram strength measures. The clinical relevance of this finding is that it indicates a more intensive isotonic strengthening program can be both safe and effective. Circumferential measurements were taken at the joint line of the knee and elbow to determine whether exercise increased joint swelling. Addi-
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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 significant, increase in the upperextremity girth measurements. Lowerextremity girth measurements did not show significant change. This result, in combination with clinical examination and participant selfreport, implies that the exercise program did not induce major muscle or joint bleeding. To elaborate on the safety of our program, at the conclusion of the study, several participants with severe hemophilia reported that their adherence to factor infusion prior to exercise was not 100%; yet no bleeding episodes were reported resulting from exercise. These findings agree with those of Pelletier et al,51 who did not find adverse effects of exercise on circumferential measures. Due to the low level of intensity and short duration 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 measurement error. We believe that the improved performance in strength measures was likely the result of neuromotor adaptation60 and that hypertrophy might have been more evident with a longer period and higher intensity of exercise. However, hypertrophy is of special interest due to the sarcopenia related to HIV status in several participants. As previously stated, each limb was examined as independent observations with up to 40 observations made on 20 participants (ROM). This was done because typically people with bleeding disorders have asymmetry in joint destruction. To address any concerns about the validity of pairing data collected from both the right and left extremities, single520
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limb analyses also were performed. Similar results were found for ROM, strength, and circumferential measures 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 participants because it reflects an improvement in functional exercise level such as walking, activities of daily living, and self-care.52 Several factors may have contributed to this functional improvement: increased stride length from improved ROM; improved muscular endurance; improved cardiopulmonary efficiency; improved circulation; and improved biomechanical loading on the joints from gains in ROM and muscle strength, resulting in a more comfortable and efficient gait.52 The improvement in functional walking also could result from behavioral and psychological factors such as increased confidence, improved body image, and decreased fear of movement or injury.
scribed conservative strengthening regimen may have failed to challenge participants with higher functioning. One physical therapist performed all evaluations, developed individualized exercise programs, and modified 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 resulted in an inflation of the type I error rate. Finally, some participants had pain, which precluded full participation for evaluation and intervention. Additional minor limitations include confounding variables imposed upon the health of the participants due to concomitant disease. Long-term follow-up was not formally 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 Limitations Our participants represented a typical population with bleeding disorders managed at a metropolitan hemophilia clinic with a wide range of ages (7–57 years) and functional abilities ranging from independent to severely 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 believe 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 highest concern; therefore, the independent variable (exercise) was not one specific protocol that could be applied to all participants, and the pre-
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Hemophilia and bleeding disorders are rare and may result in lifelong, chronic musculoskeletal problems and functional limitations. Physical therapists are challenged with developing interventions to protect people with bleeding disorders from episodes of bleeding and joint destruction but also to find ways to promote physical function and independence. Although exercise is recommended for recovery from bleeding episodes, as well as for health, fitness, and enhanced quality of life, there are few reported evidencebased studies on the benefits and safety of exercise in people with bleeding disorders. Therefore, the purposes of this study were to investigate the efficacy, safety, and feasibility of an individually designed exercise 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 recommendation that people with bleeding disorders should: • have permission from their physician to exercise, • have a thorough physical therapist evaluation and individualized exercise prescription prior to initiating exercise, • have coagulation medications available for mild and moderate bleeding disorders, • use prophylactic factor replacement for severe bleeding disorders, • start with a very conservative baseline exercise program that accommodates 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 participants improved, with no adverse events. Ten of the 20 participants requested to repeat the 6-week exercise program. The results of this study suggest that the combined efforts of physical therapists and trained fitness instructors to provide individually prescribed and supervised 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 development of exercise programs for underserved populations and can reduce health care disparities for individuals with disabilities. Our program proved effective, safe, and feasible 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 disorders 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 participants into subgroups based on severity 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 collection. Dr Mulvany, Dr Zucker-Levin, and Dr Jeng provided data analysis, project management, 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 authors provided consultation (including review of manuscript before submission). The authors acknowledge the study participants for their determination and persistence; Angela Redden, fitness instructor; Penny Head, PT; and all student physical therapy researchers 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, Institutional Review Board. Poster presentations of this work were given at the World Federation of Hemophilia Meeting; 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 Symposium; 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
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Supervised Exercise Program for People With Bleeding Disorders and Hemophilic Arthritis 19 Meena AK, Jayalakshmi S, Prasad VS, Murthy JM. Spinal epidural haematoma in a patient with haemophilia-B. Spinal Cord. 1998;36:658 – 660. 20 Medical and Scientific Advisory Committee (MASAC) Recommendation #175: Guidelines for Emergency Department Management of Individuals With Hemophilia. New York, NY: National Hemophilia Foundation; approved October 15, 2006. 21 Medical and Scientific Advisory Committee (MASAC) Recommendation #190: Recommendations Concerning the Treatment of Hemophilia and Other Bleeding Disorders. New York, NY: National Hemophilia Foundation; approved March 21, 2009. 22 Medical and Scientific Advisory Committee (MASAC) Recommendation #179: Recommendation Concerning Prophylaxis (Regular Administration of Clotting Factor Concentrate to Prevent Bleeding). New York, NY: National Hemophilia Foundation; approved November 4, 2007. 23 Medical and Scientific Advisory Committee (MASAC) Recommendation #185: Recommendations Regarding Women With Inherited Bleeding Disorders. New York, NY: National Hemophilia Foundation; approved November 15, 2008. 24 Arnold DM, Julian JA, Walker IR. Mortality rates and causes of death among all HIVpositive 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 Hemophilia: Current Orthopedic Management. New York, NY: National Hemophilia Foundation, 1995. 27 Gilbert MS, Radomisli TE. Therapeutic options in the management of hemophilic synovitis. Clin Orthop Relat Res. 1997;343:88 –92. 28 Koch B, Cohen S, Luban NC, Eng G. Hemophiliac knee: rehabilitation techniques. Arch Phys Med Rehabil. 1982;63:379 –382. 29 Limbird TJ, Dennis SC. Synovectomy and continuous 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 arthropathy. J Bone Joint Surg Am. 2002; 84:1138 –1141. 31 Salomon O, Steinberg DM, Seligshon U. Variable bleeding manifestations characterize 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.
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34 Gilbert MS. Should Older Children and Adults With Hemophilia Participate in Sports and Recreational Activities? New York, NY: National Hemophilia Foundation; 1996. 35 Heijnen L, Mauser-Bunschoten EP, Roosendaal G. Participation in sports by Dutch persons with haemophilia. Haemophilia. 2000;6:537–546. 36 Lane H, Audet M, Herman-Hilker S, Houghton S. Physical Therapy in Bleeding Disorders. New York, NY: National Hemophilia Foundation; 2004. 37 Nazzaro AM, Owens S, Hoots WK, Larson KL. Knowledge, attitudes, and behaviors of youths in the US hemophilia population: 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: antagonist 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 hemophilia. J Pediatr. 2008;152:833– 838, 838e1. 41 Falk B, Portal S, Tiktinsky R, et al. Anaerobic power and muscle strength in young hemophilia patients. Med Sci Sports Exerc. 2000;32:52–57. 42 Koch B, Galioto FM Jr, Kelleher J, Goldstein D. Physical fitness in children with hemophilia. Arch Phys Med Rehabil. 1984;65:324 –326. 43 Wittmeier K, Mulder K. Enhancing lifestyle 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. Haemophilia. 2008;14:1035–1038. 45 Armstrong LE, Whaley MH, Brubaker PH, Otto RM. ACSM’s Guidelines for Exercise Testing and Prescription. 7th ed. Philadelphia, PA: Lippincott, Williams & Wilkins; 2006. 46 Harris S, Boggio LN. Exercise may decrease further destruction in the adult haemophilic joint. Haemophilia. 2006;12: 237–240. 47 Mulder K. Exercise for People With Hemophilia. 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 Related Disorders. Montreal, Quebec, Canada: World Federation of Hemophilia; 1998. 49 Anderson A, Forsyth A. Playing It Safe, Bleeding Disorders, Sports and Exercise. New York, NY: National Hemophilia Foundation; 2005:44. 50 Hilberg T, Herbsleb M, Puta C, et al. Physical training increases isometric muscular strength and proprioceptive performance in haemophilic subjects. Haemophilia. 2003;9:86 –93.
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51 Pelletier JR, Findley TW, Gemma SA. Isometric exercise for an individual with hemophilic arthropathy. Phys Ther. 1987;67: 1359 –1364. 52 ATS statement: guidelines for the SixMinute 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. Philadelphia, 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 Foundation/Centers for Disease Control and Prevention; 1997. 55 Hislop H, Montgomery J. Daniels and Worthingham’s Muscle Testing: Techniques of Manual Examination. Philadelphia, PA: WB Saunders Co; 1995. 56 Byl NN, Richards S, Asturias J. Intrarater and interrater reliability of strength measurements 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 dynamometer applied to adults with mental retardation. Arch Phys Med Rehabil. 1992;73: 535–539. 58 Wadsworth CT, Krishnan R, Sear M, et al. Intrarater reliability of manual muscle testing and hand-held dynametric muscle testing. 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 lymphedema. Phys Ther. 2006;86:205–214. 60 Kisner C, Colby LA. Therapeutic Exercise Foundations and Techniques. Philadelphia, PA: FA Davis Co; 2007:928. 61 Anderson A, Forsyth A. Playing It Safe. Bleeding Disorders, Sports and Exercise. New York, NY: National Hemophilia Foundation; 2000:44. 62 Anderson A, Holtzman TS, Masley J. Physical Therapy in Bleeding Disorders. New York, NY: National Hemophilia Foundation; 2000:30. 63 Portney L, Watkins M. Foundations of Clinical Research: Applications to Practice. Upper Saddle River, NJ: Pearson Prentice 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 physical therapy research: time to stop doubledipping? 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.healthypeople.gov/. Accessed January 16, 2008.
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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. Intensity
No. of 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
Progression
Level 2: Prescribed for joints and muscles with history of bleeding and chronic, mild-to-moderate impairment. No bleeding in past 6 months. Progression
Intensity
No. of 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. Intensity
No. of 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
Progression
(Continued)
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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.
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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
Range of Motion (°) (nⴝ20) Left knee flexion
a
Right knee flexiona Left knee extension
X
SD
115.9
29.0
108.1
35.6
⫺8.2
a
Right knee extension
⫺6.0
c
Final Measurements
Range
X
SD
28 to 149
122.5
28.3
43 to 155
40 to 145
114.3
33.3
40 to 152
⫺2.0
6.8
⫺16 to 12b
⫺2.2
7.4
⫺18 to 10b
12.7
⫺38 to 14
b
8.9
⫺24 to 14
b
Range
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
38.8
11.1
20 to 50
48.7
9.3
36 to 70
40.6
10.2
15 to 60
45.4
10.0
30 to 65
131.3
20.3
80 to 153
136.4
17.9
80 to 154
133.4
14.8
105 to 150
138.0
12.2
110 to 155
⫺12.4
22.6
⫺87 to 10b
⫺9.5
20.0
⫺75 to 10b
⫺12.4
20.2
⫺52 to 20
⫺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
Left ankle plantar flexion
a
Right ankle plantar flexionc Left elbow flexion
c
Right elbow flexionc Left elbow extensiona Right elbow extension
c
b
a
Pⱕ.01. Hypertension. Pⱕ.05. d Not significant. b c
Muscle Strength (N) (nⴝ17) Left hip extension
a,b
Right hip extensiona,b Left hip flexion
a,b
Right hip flexiona,b Left hip abduction
b
X
SD
Range
15.9
6.8
4.3 to 26.6
22.9
8.5
7.1 to 39.0
15.4
6.9
0.0 to 24.1
22.8
8.6
4.3 to 33.5
18.6
5.6
8.1 to 32.6
23.4
6.3
13.0 to 36.3
17.7
6.5
6.3 to 30.2
21.3
7.0
9.5 to 30.7
5.6
7.2 to 29.1
21.6
7.8
11.4 to 38.0
16.7
6.4
6.1 to 25.9
20.7
8.4
10.9 to 36.5
b
14.4
7.2
1.0 to 23.8
19.9
9.2
4.3 to 28.5
15.6
8.5
2.1 to 29.7
21.0
10.8
3.8 to 40.6
c
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
13.6
7.6
1.6 to 30.1
16.7
7.6
2.3 to 28.4
15.1
7.5
0.0 to 29.6
17.4
8.1
0.8 to 31.6
11.2
6.4
0.3 to 16.6
13.6
7.4
0.4 to 26.0
12.2
6.5
0.0 to 24.8
14.8
7.2
0.5 to 28.8
Left knee flexion
Left elbow flexion
Right elbow flexionc Left elbow extension
b
Right elbow extensionc
c
Range
16.2
Right knee extensionb
b
Final Measurements
SD
Right hip abductionc Left knee extension
a
Baseline Measurements X
n⫽16. Pⱕ.01. 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
Circumferential Measure (cm) (nⴝ20)
Final Measurement
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
37.7
3.1
32.5 to 43.0
37.9
2.9
32.5 to 43.5
28.6
4.4
28.0 to 43.5
32.9
4.4
28.0 to 42.5
28.6
4.3
28.0 to 45.0
32.4
4.2
28.0 to 45.0
29.0
5.4
21.0 to 38.0
30.0
5.8
19.8 to 37.5
30.0
5.5
19.5 to 40.0
31.0
5.8
20.0 to 42.0
27.7
3.3
21.5 to 33.0
27.3
3.2
22.0 to 31.5
28.2
3.4
22.5 to 33.0
27.8
3.3
22.0 to 32.0
25.3
3.6
19.0 to 32.0
26.2
4.0
19.5 to 34.0
25.9
3.7
21.0 to 33.5
26.4
3.7
19.5 to 32.0
Right knee at joint line
b
Left knee 4 in below joint lineb Right knee 4 in below joint line
b
Left elbow 6 in above joint linec Right elbow 6 in above joint line
b
Left elbow at joint lineb Right elbow at joint line
b
Left elbow 4 in below joint linea Right elbow 4 in below joint line
b
a
Pⱕ.01. b Not significant. c Pⱕ.05.
526
f
Physical Therapy
Volume 90
Number 4
April 2010
