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Effectiveness of an Educational Physiotherapy and Therapeutic Exercise Program in Adult Patients With Hemophilia
Accepted Manuscript Effectiveness of an educational physiotherapy and therapeutical exercise program in adult patients with hemophilia. A randomized clinical trial Rubén Cuesta-Barriuso, PhD, Ana Torres-Ortuño, PhD, Joaquín Nieto-Munuera, PhD, José Antonio López-Pina, PhD PII:
S0003-9993(16)31235-7
DOI:
10.1016/j.apmr.2016.10.014
Reference:
YAPMR 56723
To appear in:
ARCHIVES OF PHYSICAL MEDICINE AND REHABILITATION
Received Date: 12 September 2016 Accepted Date: 4 October 2016
Please cite this article as: Cuesta-Barriuso R, Torres-Ortuño A, Nieto-Munuera J, López-Pina JA, Effectiveness of an educational physiotherapy and therapeutical exercise program in adult patients with hemophilia. A randomized clinical trial, ARCHIVES OF PHYSICAL MEDICINE AND REHABILITATION (2016), doi: 10.1016/j.apmr.2016.10.014. This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.
ACCEPTED MANUSCRIPT Running head: Educational physiotherapy in haemophilia
Effectiveness of an educational physiotherapy and therapeutical exercise program in adult patients with hemophilia. A randomized clinical trial. a,b
c
c
Rubén Cuesta-Barriuso PhD , Ana Torres-Ortuño PhD ; Joaquín Nieto-Munuera PhD , José
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Antonio López-Pina, PhD . a
Department of Physiotherapy, European University of Madrid, Spain;
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Real Fundación Victoria Eugenia, Madrid, Spain.
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Department of Psychiatry and Social Psychology. Faculty of Medicine. University of Murcia.
Spain.
Department of Basic Phychology and Methodology, University of Murcia, Spain.
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Victoria Eugenia, Madrid, Spain.
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Address for corresponding author: Rubén Cuesta-Barriuso PhD. Real Fundación
Address: Instituto de Salud Carlos III, Pabellón 16. Calle Sinesio Delgado, 4. 28029, Madrid, Spain. Telf. (34) 913146508 Fax. (34) 913146575
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E-mail: [email protected]
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Acknowledgement. The authors acknowledge Manuel Moreno, MD, and Jorge Martínez, PT, for assistance with patient treatment. They also thank the workers at the
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Asociación Regional Murciana de Hemofilia.
Disclosures. The authors state that they have no interests which might be perceived as posing a conflict or bias. Reprints are not available
The study was registered: ClinicalTrials.gov Identifier: NCT02198014.
ACCEPTED MANUSCRIPT Effectiveness of an educational physiotherapy and home exercise program in adult patients with hemophilia. A randomized clinical trial. Abstract Objective: To assess the efficacy of an educational physiotherapy home exercise intervention
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on physical improvement, pain perception, quality of life and illness behavior in patients with hemophilic arthropathy. Design: A single-blind randomized study. Setting: Home
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Participants: 20 patients with hemophilia were randomly allocated to an educational intervention group or to a control group.
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Intervention: The educational intervention was performed every two weeks over a 15-week period and home exercises were carried out once a day, 6 days a week, over the same period. Main Outcome Measures: The joint status was evaluated using the Gilbert Scale; pain was assessed with the Visual Analogue Scale; the illness behavior was evaluated using the Illness
questionnaire.
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Behavior Questionnaire; and the perceived quality of life with A36 Hemophilia-QoL
Results: We observed significant differences in the experimental group for both quality of life
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and illness behavior. There was no significant improvement in the joint status, however an improvement was noted in terms of perception of pain in the ankle.
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Conclusions: A physiotherapy program based on educational sessions and home exercises can improve the perception of pain in patients with hemophilic arthropathy of the ankle. It improves some variables in the perception of quality of life and illness behavior. Key words: Hemophilia; Educational Physiotherapy; Pain; Quality of life; Illness Behavior.Key words: Hemophilia; Educational Physiotherapy; Pain; Quality of life; Illness Behavior.
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ACCEPTED MANUSCRIPT Hemophilia is a congenital bleeding disorder whose main clinical signs are muscle
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bleeding (hematoma) and joint bleeding (hemarthrosis).1 Recurrent bleeding in the same
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joint (target joint) leads to a degenerative process known as hemophilic arthropathy.
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This joint degeneration process presents with chronic pain, limitation of motion,
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muscular atrophy and biomechanical and proprioceptive disorders.2
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Prophylaxis with FVIII and FIX concentrates has become the gold standard for the
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prevention of hemophilic arthropathy. However, for adult patients treated on-demand
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after a bleeding episode (before prophylaxis) or for those who live in countries with no
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access to medical treatment, hemophilic arthropathy continues to be a reality.3
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Some programs of physiotherapy combined with electrical therapy4, manual therapy5 or
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hydrokinesitherapy6 have proven to be effective in improving muscle strength as well as
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the range of motion in patients with hemophilic arthropathy. Using an individualized
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exercise program, Mulvany et al.7 observed improvements in muscle strength and upper
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and lower limb mobility in a group of 33 patients with congenital bleeding disorders
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and degenerative arthropathy. In a recent study,8 some improvement, although not
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significant, was observed in mobility and ankle pain in 10 adult hemophilic patients
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after a 12-week program of home-based exercises and biweekly educational sessions.
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Physical changes that occur as a result of hemophilic arthropathy influence the
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perception of the quality of life in these patients. 9 However, despite the fact that many
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studies have assessed the quality of life of patients with hemophilia, only two studies10,
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The illness behavior affects the symptomatology and the clinical course of patients.
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Similarly, it also affects the patient-doctor relationship, the relationship with their
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reference social group as well as their response to therapeutic recommendations.12
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Because of its association with the quality of life, this variable needs to be taken into
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have assessed the efficacy of a Physiotherapy considering this variable.
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ACCEPTED MANUSCRIPT consideration since all behaviors, beliefs and emotions resulting from the chronic
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condition modulate the impact on the quality of life.
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The aim of this study is to assess the efficacy of an educational physiotherapy
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intervention based on home exercises for physical improvement, an improved pain
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perception and quality of life as well as with regard to illness behavior in patients with
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hemophilic arthropathy.
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Methods
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Study Design
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A single-blind, randomized, controlled clinical trial with two parallel groups to assess
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the efficacy of an educational physiotherapy program and self-care home exercises
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taking into account physical and psychosocial variables of patients with hemophilic
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arthropathy.
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Patient Recruitment and Selection
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Patients included in this study were required to comply with the following inclusion
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criteria: patients diagnosed with hemophilia A or B; aged over 18 years; patients with
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hemophilic arthropathy with at least 1 joint involved (elbow, knee or ankle); and having
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signed the informed consent document. Patients were either on prophylaxis or on-
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demand treatment with FVIII or FIX concentrates. Exclusion criteria included patients
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diagnosed with other congenital bleeding disorders (i.e. von Willebrand disease);
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patients who developed antibodies to FVIII or FIX (inhibitors) and those not able to
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walk as a result of hemophilic arthropathy or having any other disability. Patients were
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recruited from the Asociación Regional Murciana de Hemofilia in February 2012.
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Ethical Considerations
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ACCEPTED MANUSCRIPT The study was approved by the Ethics Committee of the University of Murcia. Prior to
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initiating the study, the patients signed an informed consent form conforms to Helsinki
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Declaration. The study was registered in the International Registry of Clinical Trials
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(www.clinicaltrials.gov). ClinicalTrials.gov Identifier: NCT02825706.
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Randomization
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Using opaque envelopes, an independent person not involved in the study objectives
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allocated the patients meeting the inclusion criteria to each of the study groups.
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Blinding
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A physiotherapist and a psychologist, both experienced in hemophilia care, blinded to
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patient allocation and to study objectives, made the assessments. The same individuals
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assessed dependent variables under the same blind conditions before and after the
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intervention and after a 6-month follow-up period.
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Interventions
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During the treatment period, the patients in the experimental group received 60-minute
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educational sessions every two weeks on the pathophysiology of hemophilia, clinical
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manifestations, postural advice and prevention advice to avoid recurrent bleeding.
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Likewise, doubts on the clinical progress of hemophilic arthropathy, functional
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limitations and management of joint pain were resolved.
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Parallel to the educational sessions, patients followed a 15-week home exercise program
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to be performed once a day, 6 days a week. The program included muscle stretching
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exercises of upper and lower limbs (biceps, triceps, quadriceps, hamstrings and calves);
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isometric exercises of quadriceps, hamstrings, biceps, triceps and calves; proprioceptive
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exercises on one leg with visual support (later, they were performed without visual
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support); and a 20-minute walk. As in the study conducted by Hilberg et al.13 low-
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intensity exercises with 20-25 repetitions were included. The home exercise program
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ACCEPTED MANUSCRIPT followed by the patients had been used in a previous study on patients with hemophilic
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arthropathy of the ankle.8 Exercises were adapted for patients included in this study as
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shown in Table 1.
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The subjects in the experimental group kept a record of the exercises carried out at
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home and monitored their progress.
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The patients in the control group did not receive any educational sessions and did no
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exercise at all at home.
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Before the study, all subjects were told to continue with the same daily, professional
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and sporting routines that they had been following so far to avoid any potential bias as a
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result of a parallel activity.
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Measurement instruments
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All patients underwent a radiological assessment of joint deterioration using the
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Pettersson scale14. This scale, with scores ranging from 0 (a normal joint) to 13
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(maximum joint deterioration), is the most widely used to assess joint degeneration
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produced by hemophilic arthropathy.
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Various clinical variables (physical assessment and pain perception) and psychosocial
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variables (quality of life, illness behavior and anxiety) were assessed in the study.
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The physical condition of joints was assessed using the Gilbert scale15 which measures
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swelling, muscle atrophy, joint crepitus, range of motion, axial deformity and
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instability. Scores range from 0 to 12 (0 represents no joint injury and 12 represents
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maximum joint deterioration).
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Joint pain was assessed using the Visual Analog Scale (VAS), with scores ranging from
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0 (no pain at all) to 10 (the worst pain imaginable). The Haemophilia A-36
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questionnaire16 was used to assess the perception of the quality of life. This
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questionnaire consists of 36 items which assess 9 domains (physical health, daily
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ACCEPTED MANUSCRIPT activities, joint damage and pain, treatment satisfaction, treatment difficulties, emotional
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functioning, mental health and social relationships).
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To assess illness behavior, patients completed the Illness Behavior Questionnaire
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(IBQ).17 This scale consists of 62 items and 8 domains (hypochondriasis, disease
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conviction, psychological vs. somatic perception of illness, affective inhibition,
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affective disturbance, denial and irritability).
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These measurements were carried out at the beginning and at the end of the educational
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and self-care program (in June 2012) as well as after a 6-month follow-up period (in
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December 2012).
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Statistical Analysis
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Descriptive variables are presented by mean and standard deviation for the three
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assessments. A Repeated Measures Analysis of Variance (RMAV) showed differences
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between the groups based on the assessments carried out. Using the Student’s t-test for
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independent samples, we calculated the difference between the two study groups for the
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various assessments performed. All results were considered significant with p<.05.
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Results
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Before the randomization of the subjects to different groups, two patients who met the
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criteria for inclusion in the study opted out. The reasons given were lack of time due to
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work and the distance between the treatment center and home. Of the 22 patients who
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met the inclusion criteria, 20 took part in the study. After randomization, 10 patients
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were allocated to each group. No patient withdrew during the study period or during the
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follow-up period. Figure 1 shows the flowchart of the study.
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The mean age of patients in the study was 30.95 years (SD: 11.9). 16 patients had
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Hemophilia A (80%) and 10 (50%) had a severe phenotype. 7 patients (35%) were on
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ACCEPTED MANUSCRIPT prophylaxis at the beginning of the study. The mean number of joints with hemophilic
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arthropathy was 4.45 (SD: 0.99). The radiological assessment of joint deterioration,
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using the Pettersson scale, for ankle, knee and elbow joints was 8.23 (SD: 4.44), 6.58
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(SD: 5.11) and 5.08 (SD: 4.66) respectively. Compliance with home exercises,
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measuring the daily exercise given at baseline, in patients in the experimental group was
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93.3% (84/90 average). Table 2 shows the main clinical characteristics of patients in
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each study group. As shown, there were only significant differences in weight and
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radiological joint deterioration of the ankle between the experimental group and the
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control group at the beginning of the study, and both groups were very homogeneous.
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RMAV for the three assessments performed showed significant changes in the
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experimental group. The assessment of the quality of life improved in 6 domains:
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physical health (p=.003), daily activities (p=.006), lack joints (p=.004), joint pain
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(p=.005), emotional functioning (p=.045) and overall quality of life (p=.003). As
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regards illness behavior, differences were observed in terms of general hypochondriasis
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(p=.038) and psychological vs. somatic perception of illness (p=.007). On the contrary,
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regarding physical scales, no significant improvement was observed in terms of
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examination in the treatment group using the Gilbert scale. Despite the improved
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perception of joint pain in the quality of life, the results of the VAS scale were only
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significant for ankle joints (p=.007).
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On the contrary, no differences were observed incontrol group patients after the study -
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neither in the physical scales nor in the quality of life and Illness Behavior
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questionnaire. Table 3 shows the main statistical data of RMAV for both groups.
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When using the t-test for independent samples, significant differences were observed
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after the treatment period between the two groups in the quality of life variables,
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including joint damage perception and treatment difficulties (p=.021). Likewise,
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ACCEPTED MANUSCRIPT differences were observed in the variables of general hypochondriasis and affective
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disturbance (p=.028 and p=.038 respectively) of the Illness Behavior Questionnaire.
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The physical assessment using the Gilbert Scale showed differences only in ankle
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assessment (p=.043).
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Similarly, the analysis of the results after the follow-up period showed differences
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between the two study groups. In this way, differences were observed in the perception
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of physical health, joint damage and treatment satisfaction (p=.038, p=.010, and p=.007
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respectively).
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In turn, differences were observed between the two groups in the affective disturbance
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variable of the Illness Behavior Questionnaire (p=.032). With regard to physical
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assessment, post-treatment evaluation revealed that the differences in the Gilbert Scale
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scores for ankle joint were sustained. Table 4 shows the statistics of the assessment of
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the groups using the Student’s t-test for independent samples.
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Discussion
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Retrospective studies on the quality of life in Hemophilia show that aspects such as
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severity,18, 19 bleeding frequency,20, 21 type of treatment,21, 22 viral infections23, 24 and joint
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problems24, 25 have a negative impact on the quality of life, being worse in patients with
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inhibitors.
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More recent studies show contradictory results. Fischer et al.26 described a deterioration
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of the perception of the quality of life, particularly as a result of hemophilic arthropathy
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in 96 patients with a mean age of 28.6 years. Other authors such as Lindwall et al.27
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claim that the quality of life of persons with hemophilia is similar to that of the general
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population, with differences relating only to physical functioning. Beeton et al.28
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ACCEPTED MANUSCRIPT observed that patients with hemophilia have a good quality of life despite viral
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infections and several musculoskeletal dysfunctions.
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In the literature there is conflicting data on the quality of life in patients with
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hemophilia. However, there is a certain amount of consensus on how the consequences
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of hemophilia not only affect the quality of life from a physical standpoint but also from
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an emotional and social perspective.18, 28, 29 Pain and pain-related effects are important
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aspects in the life of patients with hemophilia, although not many studies have been
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conducted on this issue.30-32 Its influence on the quality of life is clear, both physically
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(due to limitations) and mentally.31, 33, 34
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Our study, revealed changes in each group based on the assessments carried out. We
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observed that, despite greater joint deterioration according to the Pettersson Scale, the
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baseline mean score of quality of life in both groups was quite acceptable (mean: 111.0;
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PR 70). Following intervention, a significant improvement was observed in the
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experimental group, particularly in terms of physical health perception, daily activities,
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joint damage, pain, emotional functioning and overall quality of life. Moreover, this
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improvement was maintained beyond the intervention although a slight decrease was
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noted after a 6-month follow-up period.
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We may say that the educational intervention has been satisfactory as regards patient
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perception of the quality of life although no significant improvement was observed in
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terms of joint impairment, except for the ankle joint. This is consistent with the views of
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other authors who claim that physiotherapy33,
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quality of life of patients with hemophilia.37 Similarly, a retrospective educational study
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carried out by this work group, still unpublished, observed that the quality of life of
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patients was significantly improved despite increased joint pain and deterioration.
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35, 36
and physical exercise improve the
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ACCEPTED MANUSCRIPT Illness behavior involves all thoughts, emotions and attitudes developed by patients to
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cope with their condition; they are associated with certain coping and adaptation
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behaviors. In connection with this variable, following intervention, patients significantly
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improved their level of hypochondriasis and improved their perception of the
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psychological aspects involved, which had a positive impact on their way of coping and
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adapting to the disease situation. The results are consistent with those found by
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Lewellyn,38 who observed how the perception of the condition (particularly the beliefs
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regarding identity, consequences and treatment needs), played a significant role in
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identifying those subjects who were more likely to adhere to treatment. Therefore, a
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better knowledge of the disease and its consequences allows for a better control and
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management of such disease.
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Comparing both groups (experimental and control) after the intervention, we observed
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differences in two domains of the quality of life -joint damage and treatment
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difficulties- which improved significantly in the experimental group. The same
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happened with respect to the Illness Behavior Questionnaire domains of general
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hypochondriasis and affective disturbance. Patients in the experimental group showed
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better adaptive behavior with less concerns about the disease and with a suitable
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emotional state that allows for better coping. Concerning physical assessment, no
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differences were observed using the VAS scale; the Gilbert scale showed differences
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only with regard to the ankle joint. Although joint impairment was higher in the
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experimental group, the same differences were observed after the intervention.
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The analysis performed at 6months follow-up showed significant improvements in the
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experimental group; the quality of life improved in terms of physical health and
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treatment satisfaction and it remained constant in terms of joint damage. Regarding
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illness behavior, there was an increased improvement in affective disturbance. Patients
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negatively impacting their well-being, and reported that their treatment satisfied their
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needs. Furthermore, it was observed that their emotional state was not influenced by the
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disease, possibly because the educational intervention provides them with resources to
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better cope with the difficulties, which allows for better management. The same was
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observed in other studies on hemophilia28 and other chronic diseases39-41 which
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demonstrate how patients can enjoy quality of life or see positive effects in the face of
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adversity.
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Those studies argue that patients should focus on their resources and potentialities
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rather than on their limitations, taking into account the advances of medical science.
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Therefore, it seems clear that physiotherapy treatment, together with an educational
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intervention, has a positive impact on the perception of patients with hemophilia both in
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terms of quality of life and of illness behavior. This allows for better management and
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control of the disease. Additionally, an improvement in the perception of pain was
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observed despite joint deterioration which may support the above.
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Limitations of the Study
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Despite the fact that hemophilia is a rare disease, 20 patients with hemophilic
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arthropathy were recruited for this study. However, this fact is also the main limitation
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of our study which affect the strength of the results. In an attempt to overcome this
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limitation, the authors have strived to improve the methodological quality by
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randomizing the sample to both study groups, blinding the assessors and implementing
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a follow-up period.
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Another important limitation of this study is that, despite use of the Gilbert scale, which
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is one of the most widely used to assess hemophilic arthropathy, minor joint changes
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regarding mobility and strength have not been specifically and independently measured.
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ACCEPTED MANUSCRIPT Relevance to Clinical Practice
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Functional deficit resulting from hemophilic arthropathy has been widely described.
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Despite the fact that many studies have analyzed the relationship between physical
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deterioration and the quality of life in patients with hemophilia, to date there are no
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conclusive studies in this regard. Our study demonstrated how even small and, in many
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cases, non-significant physical improvements may enhance the quality of life of these
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patients.
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The results show that it is possible to provide our patients with physiotherapy treatment
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as well as training on an individual basis. In this way, we may effectively address
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patient care in a comprehensive way beyond hematologic treatment or physical
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limitations.
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Conclusions
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Implementation of a physiotherapy program based on educational sessions and home
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exercises improves the perception of pain in patients with hemophilic arthropathy of the
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ankle.
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An educational intervention based on physiotherapy exercises improves the perception
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of the quality of life and the illness behavior in patients with hemophilic arthropathy.
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The improved perception of quality of life and illness behavior as a result of a
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physiotherapy intervention and educational sessions is maintained after 6 months at
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follow-up.
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Further clinical trials are required to confirm that by following a program based
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physiotherapy and educational sessions the quality of life and the illness behavior in
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patients with hemophilia can be improved.
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related quality-of-life in individuals with haemophilia. Haemophilia 1999; 5: 378–85.
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24. Wang T, Zhang L, Li H, Zhao H, Yang R. Assessing health-related quality-of-life
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in individuals with haemophilia in China. Haemophilia 2004; 10: 370–5. 25. Aznar JA, Magallo´nM, Querol F, Gorina E, Tusell JM. The orthopaedic status of
severe haemophiliacs in Spain. Haemophilia 2000; 6: 170–6.
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26. Fischer K, Van der Bom JG, Mauser-Bunschoten EP, Roosendal G, Van dem Berg
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HM. Effects of haemophilic arthropathy on health-related quality of life and socio-
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economic parameters. Haemophilia (2005), 11, 43–48.
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27. Lindwal K, Von Mackensen S, Berntorp E. Quality of life in adult patients with
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haemophilia – a single centre experience from Sweden. Haemophilia (2012), 18, 527–
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531.
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28. Beeton K, Neal D, Lee C. An exploration of health-related quality of life in adults
with haemophilia – a qualitative perspective. Haemophilia,2005,11:123-132.
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29. Scalone L, Mantovani LG, Mannucci PM, Gringery A, asn The COCIS Study
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investigators. Quality of life associated to the orthopaedic status in haemophilic
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patients with inhibitors. Haemophilia,2006,12:154-162.
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30. Young G, Tachdjian R, Baumann K,Panopoulos G. Comprehensive management of
chronic pain in Haemophilia. Haemophilia (2014), 20, e113–e120. 31. Elander J, Robinson G, Mitchell K, Morris J. An assessment of the relative
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influence of pain coping, negative thoughts about pain, and pain acceptance on health
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related quality of life among people with hemophilia. Pain 2009; 145: 169–75
353 354
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32. Teyssler P,Kolostova K, Bobek V. Assessment of pain threshold in haemophilic
patients. Haemophilia (2014), 20, 207–211
33. Witkop M, Lambing A, Divine G et al. A national study of pain in the bleeding
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disorders community: a description of haemophilia pain. Haemophilia 2012; 18: e115–
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9.
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patients with severe haemophilia. Haemophilia 2006; 12: 147–53. 35. Heijnen L, de Kleijn P. Physiotherapy for the treatment of articular contractures in
haemophilia. Haemophilia 1999; 5: 16–9.
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34. Van Genderen FR, Fischer K, Heijnen L et al. Pain and functional limitations in
36. Reynolds F. The Subjective Experience of Illness In Physiotherapy – A
Psychosocial Approach, 3rd edn. Oxford: Butterworth Heinemann, 2004 37. Von Mackensen S. Quality of life and sports activities in patients with haemophilia.
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Haemophilia. 2007; 14 (2): 38-43. 38. Llewellyn CD, Miners AH, Lee CA, Harrington C, Weinman J. The illness
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perceptions and treatment beliefs of individuals with severe haemophilia and their role
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in adherence to home treatment. Psychol Health. 2003; 18: 185-200.
369 370 371 372
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39. Swain J, French S. Towards an affirmation model of disability. Disabil Soc 2000;
15: 569–82. 40. Stensman R. Severely mobility-disabled people assess the quality of their lives.
Scand J Rehabil Med 1985; 17: 87–99.
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41. Williams S. Chronic illness as biographical disruption or biographical disruption as
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chronic illness? Reflections on a core concept. Sociol Health Illness 2000; 22: 4–67.
List of figures
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Figure 1. CONSORT diagram.
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Table 1. Characteristics of the training program in manual therapy and educational groups. MT, manual therapy group; E, educational group;
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5
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Intervention Theory: Introduction to hemophilia: clinic and treatment. Anatomy and biomechanics aspects Theory: exercises for the maintenance and improvement of ROM, in favor of gravity Practice: muscle stretching exercises of upper and lower limbs (biceps, triceps, quadriceps, hamstrings and calves) Resolution of questions and group discussion Theory: Anatomy of ankle musculature. Function of muscles and haematomas treatment Theory: exercise for maintaining and improving strength Practice: isometric exercises of quadriceps, hamstrings, biceps, triceps and calves Resolution of questions and group discussion Theory: haemarthrosis, synovitis and arthropathy: clinical manifestations and treatment Theory: treatment of pain and mobility Practice: active exercises for mobility and pain management Resolution of questions and group discussion Theory: Proprioception: definition and importance in hemophilia Theory: proprioception exercises Practice: proprioceptive exercises on one leg with visual support (later, they were made with no visual support) Resolution of questions and group discussion Theory: Physical activity and sport: risks and benefits Theory: recommended sports in hemophilia Practice: 20-minute walk Resolution of questions and group discussion Theory review Review of practical exercises and resolution of questions and group discussion
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Session
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Min, minute; cm, centimeter; W, watt.
Characteristics of the home exercises 20 repetitions of 20 seconds each, with 10 seconds breaks between repetitions. Twice a day 20 repetitions with 20 seconds of contraction, with 10 seconds of rest between each repetition. Twice a day 20 repetitions of 20 seconds each, with 10 seconds breaks between repetitions. Twice a day 20 repetitions with 30 seconds per repetition, with 10 seconds of rest between each repetition. Twice a day
Once a day.
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Age (years) Weight (Kg) Number of joints with arthropathy diagnosis Radiological joint deterioration ankle (0-13) Radiological joint deterioration knees (0-13) Radiological joint deterioration elbow (0-13)
Experimental group Mean SD 31.9 12.52 89.91 15.93 4.8 0.92 5.53 2.41 7.55 5.63 6.15 4.82 n % 7/3 70/30 8/0/2 80/0/20 5/5 50/50
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All sample Mean SD 30.95 11.90 83.60 13.62 4.45 0.999 8.23 4.44 6.58 5.11 5.08 4.66 n % 16/4 80/20 10/3/7 50/15/35 7/13 35/65
Variables
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Table 2. Clinical characteristics of patients in the study according to the intervention group or control group.
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Type of hemophilia (A / B) Severity of hemophilia (Severe/Moderate/Mild) Treatment (Prophylactic /On demand)
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SD: standard deviation; Sig.: signification; n: number of patients; %: percentage; *P<.05
Control group Mean SD 30 11.85 77.3 7.061 4.1 0.99 5.40 4.18 5.6 4.57 4 4.36 n % 9/1 90/10 2/3/5 20/30/50 2/8 20/80
Sig. .731 .034* .119 .045* .440 .367 .288 .027* .177
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Table 3. Means (and standard deviations) of the 3 assessments in the dependent variables evaluated with Gilbert Scale, Visual Analogue Scale,
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Sig. .795 .874 .316 .007* .510 .148 .003* .006* .004* .005* .604 .193 .045* .335 .605 .003* .038* .225 .007* .115 .343 .343 .058
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Experimental Group Post Follow 2.95 (1.75) 2.80 (2.28) 1.30 (1.90) 1.25 (2.37) 1.00 (1.08) .90 (1.14) 0.32 (0.79) .60 (1.45) 0.35 (1.10) .10 (0.31) 0.00 (0.00) .20 (0.39) 27.8 (2.44) 24.5 (3.83) 13.0 (2.74) 11.6 (4.14) 8.0 (1.05) 6.5 (2.50) 6.1 (1.85) 4.7 (1.05) 7.3 (0.94) 7.3 (1.16) 13.0 (3.26) 11.5 (4.24) 16.9 (3.57) 15.4 (4.22) 10.8 (2.48) 10.6 (3.16) 18.8 (2.30) 18.6 (2.67) 121.2 (16.33) 111.6 (16.31) 1.60 (1.26) .60 (1.26) 1.80 (1.39) 1.0 (1.33) 3.50 (0.52) 2.1 (0.73) 2.20 (0.91) 1.5 (0.97) 0.70 (0.94) .20 (0.63) 3.70 (0.67) 3.4 (1.07) 0.20 (0.63) .60 (0.96)
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Gilbert ankle Gilbert knee Gilbert elbow VAS ankle VAS knee VAS elbow Physical Health Daily activities Joints Pain Satisfaction with treatment Dificulties with treatment Emotional functioning Mental Health Social relationships Overall Quality of life Hypochondrial Disease conviction Psychological perception vs. somatica Affective inhibition Affective disturbance Negation Irritability
Pre 3.00 (1.94) 1.20 (1.82) 3.00 (1.60) 1.47 (2.07) 0.60 (1.89) 0.07 (0.23) 22.3 (4.71) 10.0 (4.32) 6.4 (2.01) 4.5 (2.59) 7.0 (1.15) 12.1 (5.97) 15.4 (4.45) 10.2 (3.12) 18.5 (3.56) 106.4 (24.95) 0.5 (1.08) 1.7 (1.70) 3.5 (0.70) 1.6 (0.69) 0.6 (0.69) 3.8 (0.63) 0.6 (0.96)
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Variable
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Scale
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and questionnaires A-36 Hemophilia and IBQ. Pre 1.35 (1.59) 0.35 (1.10) 0.35 (0.95) 0.90 (1.84) 0.25 (0.63) 0.15 (0.28) 25.8 (3.61) 13.1 (2.51) 10.1 (1.96) 6.0 (0.47) 6.2 (1.61) 11.7 (1.56) 15.8 (1.31) 9.4 (1.77) 18.3 (2.31) 116.4 (11.26) 0.2 (0.63) 1.0 (1.15) 3.6 (0.69) 1.7 (0.94) 2.1 (1.85) 2.5 (1.43) 1.0 (0.94)
Control Group Post Follow 1.25 (1.23) 0.75 (1.28) 0.30 (0.79) 0.25 (0.79) 0.30 (0.94) 0.40 (1.26) 0.30 (0.56) 0.25 (0.79) 0.75 (0.18) 1.00 (0.31) 0.75 (0.18) 0.50 (0.15) 26.3 (2.94) 27.6 (2.11) 12.2 (2.39) 12.7 (2.98) 9.4 (0.69) 9.2 (1.54) 7.00 (0.66) 5.5 (2.27) 6.1 (2.64) 5.1 (1.96) 7.3 (6.34) 11.5 (2.01) 15.9 (1.66) 14.9 (1.79) 9.5 (1.35) 10.2 (1.98) 19 (1.63) 18.6 (1.50) 112.7 (10.22) 115.3 (11.30) 0.4 (0.96) 1.9 (2.02) 1.1 (1.10) 0.7 (0.67) 3.5 (0.70) 2.2 (0.63) 1.5 (1.35) 1.6 (0.84) 2.1 (1.72) 1.4 (1.50) 3.2 (0.91) 3.4 (1.17) 0.7 (0.48) 0.9 (0.99)
VAS: Visual Analog Scale; A-36: Haemophilia A-36 questionnaire; IBQ: Illness Behavior Questionnaire; Pre: pretreatment assessment; Post: postreatment assessment; Follow: follow up assessment; Sig: signification; *: P<.05.
Sig. .333 .510 .090 .488 .370 .392 .649 .548 .626 .017* .362 .032* .385 .394 .214 .333 .122 .413 .009* .703 .025* .363 .322
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Table 4. Means and signification of the assessment between the groups.
IBQ
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Postreatment EG CG 2.95 1.25 1.30 0.30 1.00 0.30 0.32 0.30 0.35 0.75 0.00 0.75 27.8 26.3 13 12.2 8 9.4 6.1 7.00 7.3 6.1 13 7.3 16.9 15.9 10.8 9.5 18.8 19 121.2 112.7 1.6 0.4 1.8 1.1 3.5 3.5 2.2 1.5 0.7 2.1 3.7 3.2 102.0 0.7
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Baseline CG Sig 1.35 .111 0.35 .365 0.35 .254 0.90 .611 0.25 .627 0.15 .512 25.8 .079 13.1 .066 10.1 .001* 6.0 .089 6.2 .220 11.7 .840 15.8 .788 9.4 .490 18.3 .883 116.4 .263 0.2 .458 1.0 .296 3.6 .754 1.7 .791 2.1 .058 2.5 .071 1.0 .361
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VAS
Gilbert ankle Gilbert knee Gilbert elbow VAS ankle VAS knee VAS elbow Physical Health Daily activities Joints Pain Satisfaction with treatment Dificulties with treatment Emotional functioning Mental Health Social relationships Overall Quality of life Hypochondrial Disease conviction Psychological perception vs. somatica Affective inhibition Affective disturbance Negation Irritability
EG 3.00 1.20 3.00 1.47 0.60 0.07 22.3 10.0 6.4 4.5 7.0 12.1 15.4 10.2 18.5 106.4 0.5 1.7 3.5 1.6 0.6 3.8 0.6
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Gilbert
Variable
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Sig .043* .302 .284 .338 .511 .241 .231 .497 .003* .166 .193 .021* .433 .164 .825 .181 .028* .229 1.00 .193 .038* .182 .062
Follow-up EG CG 2.80 0.75 1.25 0.25 0.90 0.40 0.60 0.25 0.10 1.00 0.20 0.50 24.5 27.6 11.6 12.7 6.5 9.2 4.7 5.5 7.3 5.1 11.5 11.5 15.4 14.9 10.6 10.2 18.6 18.6 111.6 115.3 0.6 1.9 1.0 0.7 2.1 2.2 1.5 1.6 0.2 1.4 3.4 3.4 0.6 0.9
Sig .023* .282 .424 .514 1.00 .309 .038* .504 .010* .326 .007* 1.00 .734 .739 1.00 .563 .102 .534 .749 .809 .032* 1.00 .503
VAS: Visual Analog Scale; A-36: Haemophilia A-36 questionnaire; IBQ: Illness Behavior Questionnaire; Baseline: baseline assessment; Post: postreatment assessment; Follow-up: follow-up assessment; EG: experimental group; CG: control group; Sig: signification
Enrollment
Assessed for eligibility (n=34)
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Excluded (n=14) Not meeting inclusion criteria (n=7) Declined to participate (n=2) Other reasons (n=5)
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Randomized (n=20)
Allocation
Experimental group (n=10) Received allocated intervention (n=10) Did not receive allocated intervention (n=0)
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Control group (n=10) Received allocated intervention (n=0) Did not receive allocated intervention (control group with treatment) (n=10)
Follow-Up Lost to follow-up (give reasons) (n=0)
Discontinued intervention (n=0)
Discontinued intervention (n=0)
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Lost to follow-up (give reasons) (n=0)
Analysed (n=10) Excluded from analysis (n=0)
Analysis Analysed (n=10) Excluded from analysis (n=0)
S0003-9993(16)31235-7
DOI:
10.1016/j.apmr.2016.10.014
Reference:
YAPMR 56723
To appear in:
ARCHIVES OF PHYSICAL MEDICINE AND REHABILITATION
Received Date: 12 September 2016 Accepted Date: 4 October 2016
Please cite this article as: Cuesta-Barriuso R, Torres-Ortuño A, Nieto-Munuera J, López-Pina JA, Effectiveness of an educational physiotherapy and therapeutical exercise program in adult patients with hemophilia. A randomized clinical trial, ARCHIVES OF PHYSICAL MEDICINE AND REHABILITATION (2016), doi: 10.1016/j.apmr.2016.10.014. This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.
ACCEPTED MANUSCRIPT Running head: Educational physiotherapy in haemophilia
Effectiveness of an educational physiotherapy and therapeutical exercise program in adult patients with hemophilia. A randomized clinical trial. a,b
c
c
Rubén Cuesta-Barriuso PhD , Ana Torres-Ortuño PhD ; Joaquín Nieto-Munuera PhD , José
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d
Antonio López-Pina, PhD . a
Department of Physiotherapy, European University of Madrid, Spain;
b
Real Fundación Victoria Eugenia, Madrid, Spain.
c
Department of Psychiatry and Social Psychology. Faculty of Medicine. University of Murcia.
Spain.
Department of Basic Phychology and Methodology, University of Murcia, Spain.
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Victoria Eugenia, Madrid, Spain.
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Address for corresponding author: Rubén Cuesta-Barriuso PhD. Real Fundación
Address: Instituto de Salud Carlos III, Pabellón 16. Calle Sinesio Delgado, 4. 28029, Madrid, Spain. Telf. (34) 913146508 Fax. (34) 913146575
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E-mail: [email protected]
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Acknowledgement. The authors acknowledge Manuel Moreno, MD, and Jorge Martínez, PT, for assistance with patient treatment. They also thank the workers at the
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Asociación Regional Murciana de Hemofilia.
Disclosures. The authors state that they have no interests which might be perceived as posing a conflict or bias. Reprints are not available
The study was registered: ClinicalTrials.gov Identifier: NCT02198014.
ACCEPTED MANUSCRIPT Effectiveness of an educational physiotherapy and home exercise program in adult patients with hemophilia. A randomized clinical trial. Abstract Objective: To assess the efficacy of an educational physiotherapy home exercise intervention
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on physical improvement, pain perception, quality of life and illness behavior in patients with hemophilic arthropathy. Design: A single-blind randomized study. Setting: Home
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Participants: 20 patients with hemophilia were randomly allocated to an educational intervention group or to a control group.
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Intervention: The educational intervention was performed every two weeks over a 15-week period and home exercises were carried out once a day, 6 days a week, over the same period. Main Outcome Measures: The joint status was evaluated using the Gilbert Scale; pain was assessed with the Visual Analogue Scale; the illness behavior was evaluated using the Illness
questionnaire.
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Behavior Questionnaire; and the perceived quality of life with A36 Hemophilia-QoL
Results: We observed significant differences in the experimental group for both quality of life
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and illness behavior. There was no significant improvement in the joint status, however an improvement was noted in terms of perception of pain in the ankle.
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Conclusions: A physiotherapy program based on educational sessions and home exercises can improve the perception of pain in patients with hemophilic arthropathy of the ankle. It improves some variables in the perception of quality of life and illness behavior. Key words: Hemophilia; Educational Physiotherapy; Pain; Quality of life; Illness Behavior.Key words: Hemophilia; Educational Physiotherapy; Pain; Quality of life; Illness Behavior.
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ACCEPTED MANUSCRIPT Hemophilia is a congenital bleeding disorder whose main clinical signs are muscle
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bleeding (hematoma) and joint bleeding (hemarthrosis).1 Recurrent bleeding in the same
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joint (target joint) leads to a degenerative process known as hemophilic arthropathy.
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This joint degeneration process presents with chronic pain, limitation of motion,
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muscular atrophy and biomechanical and proprioceptive disorders.2
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Prophylaxis with FVIII and FIX concentrates has become the gold standard for the
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prevention of hemophilic arthropathy. However, for adult patients treated on-demand
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after a bleeding episode (before prophylaxis) or for those who live in countries with no
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access to medical treatment, hemophilic arthropathy continues to be a reality.3
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Some programs of physiotherapy combined with electrical therapy4, manual therapy5 or
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hydrokinesitherapy6 have proven to be effective in improving muscle strength as well as
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the range of motion in patients with hemophilic arthropathy. Using an individualized
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exercise program, Mulvany et al.7 observed improvements in muscle strength and upper
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and lower limb mobility in a group of 33 patients with congenital bleeding disorders
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and degenerative arthropathy. In a recent study,8 some improvement, although not
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significant, was observed in mobility and ankle pain in 10 adult hemophilic patients
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after a 12-week program of home-based exercises and biweekly educational sessions.
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Physical changes that occur as a result of hemophilic arthropathy influence the
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perception of the quality of life in these patients. 9 However, despite the fact that many
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studies have assessed the quality of life of patients with hemophilia, only two studies10,
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11
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The illness behavior affects the symptomatology and the clinical course of patients.
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Similarly, it also affects the patient-doctor relationship, the relationship with their
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reference social group as well as their response to therapeutic recommendations.12
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Because of its association with the quality of life, this variable needs to be taken into
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have assessed the efficacy of a Physiotherapy considering this variable.
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ACCEPTED MANUSCRIPT consideration since all behaviors, beliefs and emotions resulting from the chronic
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condition modulate the impact on the quality of life.
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The aim of this study is to assess the efficacy of an educational physiotherapy
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intervention based on home exercises for physical improvement, an improved pain
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perception and quality of life as well as with regard to illness behavior in patients with
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hemophilic arthropathy.
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Methods
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Study Design
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A single-blind, randomized, controlled clinical trial with two parallel groups to assess
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the efficacy of an educational physiotherapy program and self-care home exercises
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taking into account physical and psychosocial variables of patients with hemophilic
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arthropathy.
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Patient Recruitment and Selection
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Patients included in this study were required to comply with the following inclusion
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criteria: patients diagnosed with hemophilia A or B; aged over 18 years; patients with
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hemophilic arthropathy with at least 1 joint involved (elbow, knee or ankle); and having
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signed the informed consent document. Patients were either on prophylaxis or on-
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demand treatment with FVIII or FIX concentrates. Exclusion criteria included patients
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diagnosed with other congenital bleeding disorders (i.e. von Willebrand disease);
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patients who developed antibodies to FVIII or FIX (inhibitors) and those not able to
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walk as a result of hemophilic arthropathy or having any other disability. Patients were
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recruited from the Asociación Regional Murciana de Hemofilia in February 2012.
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Ethical Considerations
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ACCEPTED MANUSCRIPT The study was approved by the Ethics Committee of the University of Murcia. Prior to
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initiating the study, the patients signed an informed consent form conforms to Helsinki
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Declaration. The study was registered in the International Registry of Clinical Trials
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(www.clinicaltrials.gov). ClinicalTrials.gov Identifier: NCT02825706.
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Randomization
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Using opaque envelopes, an independent person not involved in the study objectives
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allocated the patients meeting the inclusion criteria to each of the study groups.
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Blinding
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A physiotherapist and a psychologist, both experienced in hemophilia care, blinded to
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patient allocation and to study objectives, made the assessments. The same individuals
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assessed dependent variables under the same blind conditions before and after the
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intervention and after a 6-month follow-up period.
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Interventions
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During the treatment period, the patients in the experimental group received 60-minute
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educational sessions every two weeks on the pathophysiology of hemophilia, clinical
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manifestations, postural advice and prevention advice to avoid recurrent bleeding.
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Likewise, doubts on the clinical progress of hemophilic arthropathy, functional
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limitations and management of joint pain were resolved.
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Parallel to the educational sessions, patients followed a 15-week home exercise program
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to be performed once a day, 6 days a week. The program included muscle stretching
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exercises of upper and lower limbs (biceps, triceps, quadriceps, hamstrings and calves);
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isometric exercises of quadriceps, hamstrings, biceps, triceps and calves; proprioceptive
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exercises on one leg with visual support (later, they were performed without visual
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support); and a 20-minute walk. As in the study conducted by Hilberg et al.13 low-
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intensity exercises with 20-25 repetitions were included. The home exercise program
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arthropathy of the ankle.8 Exercises were adapted for patients included in this study as
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shown in Table 1.
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The subjects in the experimental group kept a record of the exercises carried out at
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home and monitored their progress.
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The patients in the control group did not receive any educational sessions and did no
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exercise at all at home.
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Before the study, all subjects were told to continue with the same daily, professional
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and sporting routines that they had been following so far to avoid any potential bias as a
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result of a parallel activity.
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Measurement instruments
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All patients underwent a radiological assessment of joint deterioration using the
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Pettersson scale14. This scale, with scores ranging from 0 (a normal joint) to 13
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(maximum joint deterioration), is the most widely used to assess joint degeneration
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produced by hemophilic arthropathy.
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Various clinical variables (physical assessment and pain perception) and psychosocial
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variables (quality of life, illness behavior and anxiety) were assessed in the study.
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The physical condition of joints was assessed using the Gilbert scale15 which measures
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swelling, muscle atrophy, joint crepitus, range of motion, axial deformity and
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instability. Scores range from 0 to 12 (0 represents no joint injury and 12 represents
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maximum joint deterioration).
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Joint pain was assessed using the Visual Analog Scale (VAS), with scores ranging from
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0 (no pain at all) to 10 (the worst pain imaginable). The Haemophilia A-36
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questionnaire16 was used to assess the perception of the quality of life. This
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questionnaire consists of 36 items which assess 9 domains (physical health, daily
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ACCEPTED MANUSCRIPT activities, joint damage and pain, treatment satisfaction, treatment difficulties, emotional
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functioning, mental health and social relationships).
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To assess illness behavior, patients completed the Illness Behavior Questionnaire
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(IBQ).17 This scale consists of 62 items and 8 domains (hypochondriasis, disease
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conviction, psychological vs. somatic perception of illness, affective inhibition,
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affective disturbance, denial and irritability).
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These measurements were carried out at the beginning and at the end of the educational
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and self-care program (in June 2012) as well as after a 6-month follow-up period (in
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December 2012).
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Statistical Analysis
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Descriptive variables are presented by mean and standard deviation for the three
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assessments. A Repeated Measures Analysis of Variance (RMAV) showed differences
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between the groups based on the assessments carried out. Using the Student’s t-test for
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independent samples, we calculated the difference between the two study groups for the
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various assessments performed. All results were considered significant with p<.05.
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Results
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Before the randomization of the subjects to different groups, two patients who met the
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criteria for inclusion in the study opted out. The reasons given were lack of time due to
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work and the distance between the treatment center and home. Of the 22 patients who
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met the inclusion criteria, 20 took part in the study. After randomization, 10 patients
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were allocated to each group. No patient withdrew during the study period or during the
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follow-up period. Figure 1 shows the flowchart of the study.
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The mean age of patients in the study was 30.95 years (SD: 11.9). 16 patients had
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Hemophilia A (80%) and 10 (50%) had a severe phenotype. 7 patients (35%) were on
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arthropathy was 4.45 (SD: 0.99). The radiological assessment of joint deterioration,
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using the Pettersson scale, for ankle, knee and elbow joints was 8.23 (SD: 4.44), 6.58
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(SD: 5.11) and 5.08 (SD: 4.66) respectively. Compliance with home exercises,
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measuring the daily exercise given at baseline, in patients in the experimental group was
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93.3% (84/90 average). Table 2 shows the main clinical characteristics of patients in
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each study group. As shown, there were only significant differences in weight and
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radiological joint deterioration of the ankle between the experimental group and the
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control group at the beginning of the study, and both groups were very homogeneous.
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RMAV for the three assessments performed showed significant changes in the
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experimental group. The assessment of the quality of life improved in 6 domains:
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physical health (p=.003), daily activities (p=.006), lack joints (p=.004), joint pain
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(p=.005), emotional functioning (p=.045) and overall quality of life (p=.003). As
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regards illness behavior, differences were observed in terms of general hypochondriasis
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(p=.038) and psychological vs. somatic perception of illness (p=.007). On the contrary,
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regarding physical scales, no significant improvement was observed in terms of
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examination in the treatment group using the Gilbert scale. Despite the improved
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perception of joint pain in the quality of life, the results of the VAS scale were only
143
significant for ankle joints (p=.007).
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On the contrary, no differences were observed incontrol group patients after the study -
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neither in the physical scales nor in the quality of life and Illness Behavior
146
questionnaire. Table 3 shows the main statistical data of RMAV for both groups.
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When using the t-test for independent samples, significant differences were observed
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after the treatment period between the two groups in the quality of life variables,
149
including joint damage perception and treatment difficulties (p=.021). Likewise,
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disturbance (p=.028 and p=.038 respectively) of the Illness Behavior Questionnaire.
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The physical assessment using the Gilbert Scale showed differences only in ankle
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assessment (p=.043).
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Similarly, the analysis of the results after the follow-up period showed differences
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between the two study groups. In this way, differences were observed in the perception
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of physical health, joint damage and treatment satisfaction (p=.038, p=.010, and p=.007
157
respectively).
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In turn, differences were observed between the two groups in the affective disturbance
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variable of the Illness Behavior Questionnaire (p=.032). With regard to physical
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assessment, post-treatment evaluation revealed that the differences in the Gilbert Scale
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scores for ankle joint were sustained. Table 4 shows the statistics of the assessment of
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the groups using the Student’s t-test for independent samples.
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Discussion
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Retrospective studies on the quality of life in Hemophilia show that aspects such as
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severity,18, 19 bleeding frequency,20, 21 type of treatment,21, 22 viral infections23, 24 and joint
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problems24, 25 have a negative impact on the quality of life, being worse in patients with
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inhibitors.
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More recent studies show contradictory results. Fischer et al.26 described a deterioration
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of the perception of the quality of life, particularly as a result of hemophilic arthropathy
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in 96 patients with a mean age of 28.6 years. Other authors such as Lindwall et al.27
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claim that the quality of life of persons with hemophilia is similar to that of the general
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population, with differences relating only to physical functioning. Beeton et al.28
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infections and several musculoskeletal dysfunctions.
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In the literature there is conflicting data on the quality of life in patients with
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hemophilia. However, there is a certain amount of consensus on how the consequences
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of hemophilia not only affect the quality of life from a physical standpoint but also from
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an emotional and social perspective.18, 28, 29 Pain and pain-related effects are important
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aspects in the life of patients with hemophilia, although not many studies have been
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conducted on this issue.30-32 Its influence on the quality of life is clear, both physically
182
(due to limitations) and mentally.31, 33, 34
183
Our study, revealed changes in each group based on the assessments carried out. We
184
observed that, despite greater joint deterioration according to the Pettersson Scale, the
185
baseline mean score of quality of life in both groups was quite acceptable (mean: 111.0;
186
PR 70). Following intervention, a significant improvement was observed in the
187
experimental group, particularly in terms of physical health perception, daily activities,
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joint damage, pain, emotional functioning and overall quality of life. Moreover, this
189
improvement was maintained beyond the intervention although a slight decrease was
190
noted after a 6-month follow-up period.
191
We may say that the educational intervention has been satisfactory as regards patient
192
perception of the quality of life although no significant improvement was observed in
193
terms of joint impairment, except for the ankle joint. This is consistent with the views of
194
other authors who claim that physiotherapy33,
195
quality of life of patients with hemophilia.37 Similarly, a retrospective educational study
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carried out by this work group, still unpublished, observed that the quality of life of
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patients was significantly improved despite increased joint pain and deterioration.
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35, 36
and physical exercise improve the
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cope with their condition; they are associated with certain coping and adaptation
200
behaviors. In connection with this variable, following intervention, patients significantly
201
improved their level of hypochondriasis and improved their perception of the
202
psychological aspects involved, which had a positive impact on their way of coping and
203
adapting to the disease situation. The results are consistent with those found by
204
Lewellyn,38 who observed how the perception of the condition (particularly the beliefs
205
regarding identity, consequences and treatment needs), played a significant role in
206
identifying those subjects who were more likely to adhere to treatment. Therefore, a
207
better knowledge of the disease and its consequences allows for a better control and
208
management of such disease.
209
Comparing both groups (experimental and control) after the intervention, we observed
210
differences in two domains of the quality of life -joint damage and treatment
211
difficulties- which improved significantly in the experimental group. The same
212
happened with respect to the Illness Behavior Questionnaire domains of general
213
hypochondriasis and affective disturbance. Patients in the experimental group showed
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better adaptive behavior with less concerns about the disease and with a suitable
215
emotional state that allows for better coping. Concerning physical assessment, no
216
differences were observed using the VAS scale; the Gilbert scale showed differences
217
only with regard to the ankle joint. Although joint impairment was higher in the
218
experimental group, the same differences were observed after the intervention.
219
The analysis performed at 6months follow-up showed significant improvements in the
220
experimental group; the quality of life improved in terms of physical health and
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treatment satisfaction and it remained constant in terms of joint damage. Regarding
222
illness behavior, there was an increased improvement in affective disturbance. Patients
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224
negatively impacting their well-being, and reported that their treatment satisfied their
225
needs. Furthermore, it was observed that their emotional state was not influenced by the
226
disease, possibly because the educational intervention provides them with resources to
227
better cope with the difficulties, which allows for better management. The same was
228
observed in other studies on hemophilia28 and other chronic diseases39-41 which
229
demonstrate how patients can enjoy quality of life or see positive effects in the face of
230
adversity.
231
Those studies argue that patients should focus on their resources and potentialities
232
rather than on their limitations, taking into account the advances of medical science.
233
Therefore, it seems clear that physiotherapy treatment, together with an educational
234
intervention, has a positive impact on the perception of patients with hemophilia both in
235
terms of quality of life and of illness behavior. This allows for better management and
236
control of the disease. Additionally, an improvement in the perception of pain was
237
observed despite joint deterioration which may support the above.
238
Limitations of the Study
239
Despite the fact that hemophilia is a rare disease, 20 patients with hemophilic
240
arthropathy were recruited for this study. However, this fact is also the main limitation
241
of our study which affect the strength of the results. In an attempt to overcome this
242
limitation, the authors have strived to improve the methodological quality by
243
randomizing the sample to both study groups, blinding the assessors and implementing
244
a follow-up period.
245
Another important limitation of this study is that, despite use of the Gilbert scale, which
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is one of the most widely used to assess hemophilic arthropathy, minor joint changes
247
regarding mobility and strength have not been specifically and independently measured.
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Functional deficit resulting from hemophilic arthropathy has been widely described.
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Despite the fact that many studies have analyzed the relationship between physical
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deterioration and the quality of life in patients with hemophilia, to date there are no
252
conclusive studies in this regard. Our study demonstrated how even small and, in many
253
cases, non-significant physical improvements may enhance the quality of life of these
254
patients.
255
The results show that it is possible to provide our patients with physiotherapy treatment
256
as well as training on an individual basis. In this way, we may effectively address
257
patient care in a comprehensive way beyond hematologic treatment or physical
258
limitations.
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Conclusions
261
Implementation of a physiotherapy program based on educational sessions and home
262
exercises improves the perception of pain in patients with hemophilic arthropathy of the
263
ankle.
264
An educational intervention based on physiotherapy exercises improves the perception
265
of the quality of life and the illness behavior in patients with hemophilic arthropathy.
266
The improved perception of quality of life and illness behavior as a result of a
267
physiotherapy intervention and educational sessions is maintained after 6 months at
268
follow-up.
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Further clinical trials are required to confirm that by following a program based
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physiotherapy and educational sessions the quality of life and the illness behavior in
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patients with hemophilia can be improved.
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34. Van Genderen FR, Fischer K, Heijnen L et al. Pain and functional limitations in
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39. Swain J, French S. Towards an affirmation model of disability. Disabil Soc 2000;
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41. Williams S. Chronic illness as biographical disruption or biographical disruption as
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chronic illness? Reflections on a core concept. Sociol Health Illness 2000; 22: 4–67.
List of figures
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Figure 1. CONSORT diagram.
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Table 1. Characteristics of the training program in manual therapy and educational groups. MT, manual therapy group; E, educational group;
2
3
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Intervention Theory: Introduction to hemophilia: clinic and treatment. Anatomy and biomechanics aspects Theory: exercises for the maintenance and improvement of ROM, in favor of gravity Practice: muscle stretching exercises of upper and lower limbs (biceps, triceps, quadriceps, hamstrings and calves) Resolution of questions and group discussion Theory: Anatomy of ankle musculature. Function of muscles and haematomas treatment Theory: exercise for maintaining and improving strength Practice: isometric exercises of quadriceps, hamstrings, biceps, triceps and calves Resolution of questions and group discussion Theory: haemarthrosis, synovitis and arthropathy: clinical manifestations and treatment Theory: treatment of pain and mobility Practice: active exercises for mobility and pain management Resolution of questions and group discussion Theory: Proprioception: definition and importance in hemophilia Theory: proprioception exercises Practice: proprioceptive exercises on one leg with visual support (later, they were made with no visual support) Resolution of questions and group discussion Theory: Physical activity and sport: risks and benefits Theory: recommended sports in hemophilia Practice: 20-minute walk Resolution of questions and group discussion Theory review Review of practical exercises and resolution of questions and group discussion
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Session
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Min, minute; cm, centimeter; W, watt.
Characteristics of the home exercises 20 repetitions of 20 seconds each, with 10 seconds breaks between repetitions. Twice a day 20 repetitions with 20 seconds of contraction, with 10 seconds of rest between each repetition. Twice a day 20 repetitions of 20 seconds each, with 10 seconds breaks between repetitions. Twice a day 20 repetitions with 30 seconds per repetition, with 10 seconds of rest between each repetition. Twice a day
Once a day.
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Age (years) Weight (Kg) Number of joints with arthropathy diagnosis Radiological joint deterioration ankle (0-13) Radiological joint deterioration knees (0-13) Radiological joint deterioration elbow (0-13)
Experimental group Mean SD 31.9 12.52 89.91 15.93 4.8 0.92 5.53 2.41 7.55 5.63 6.15 4.82 n % 7/3 70/30 8/0/2 80/0/20 5/5 50/50
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All sample Mean SD 30.95 11.90 83.60 13.62 4.45 0.999 8.23 4.44 6.58 5.11 5.08 4.66 n % 16/4 80/20 10/3/7 50/15/35 7/13 35/65
Variables
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Table 2. Clinical characteristics of patients in the study according to the intervention group or control group.
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Type of hemophilia (A / B) Severity of hemophilia (Severe/Moderate/Mild) Treatment (Prophylactic /On demand)
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SD: standard deviation; Sig.: signification; n: number of patients; %: percentage; *P<.05
Control group Mean SD 30 11.85 77.3 7.061 4.1 0.99 5.40 4.18 5.6 4.57 4 4.36 n % 9/1 90/10 2/3/5 20/30/50 2/8 20/80
Sig. .731 .034* .119 .045* .440 .367 .288 .027* .177
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Table 3. Means (and standard deviations) of the 3 assessments in the dependent variables evaluated with Gilbert Scale, Visual Analogue Scale,
IBQ
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Sig. .795 .874 .316 .007* .510 .148 .003* .006* .004* .005* .604 .193 .045* .335 .605 .003* .038* .225 .007* .115 .343 .343 .058
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A-36
Experimental Group Post Follow 2.95 (1.75) 2.80 (2.28) 1.30 (1.90) 1.25 (2.37) 1.00 (1.08) .90 (1.14) 0.32 (0.79) .60 (1.45) 0.35 (1.10) .10 (0.31) 0.00 (0.00) .20 (0.39) 27.8 (2.44) 24.5 (3.83) 13.0 (2.74) 11.6 (4.14) 8.0 (1.05) 6.5 (2.50) 6.1 (1.85) 4.7 (1.05) 7.3 (0.94) 7.3 (1.16) 13.0 (3.26) 11.5 (4.24) 16.9 (3.57) 15.4 (4.22) 10.8 (2.48) 10.6 (3.16) 18.8 (2.30) 18.6 (2.67) 121.2 (16.33) 111.6 (16.31) 1.60 (1.26) .60 (1.26) 1.80 (1.39) 1.0 (1.33) 3.50 (0.52) 2.1 (0.73) 2.20 (0.91) 1.5 (0.97) 0.70 (0.94) .20 (0.63) 3.70 (0.67) 3.4 (1.07) 0.20 (0.63) .60 (0.96)
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Gilbert ankle Gilbert knee Gilbert elbow VAS ankle VAS knee VAS elbow Physical Health Daily activities Joints Pain Satisfaction with treatment Dificulties with treatment Emotional functioning Mental Health Social relationships Overall Quality of life Hypochondrial Disease conviction Psychological perception vs. somatica Affective inhibition Affective disturbance Negation Irritability
Pre 3.00 (1.94) 1.20 (1.82) 3.00 (1.60) 1.47 (2.07) 0.60 (1.89) 0.07 (0.23) 22.3 (4.71) 10.0 (4.32) 6.4 (2.01) 4.5 (2.59) 7.0 (1.15) 12.1 (5.97) 15.4 (4.45) 10.2 (3.12) 18.5 (3.56) 106.4 (24.95) 0.5 (1.08) 1.7 (1.70) 3.5 (0.70) 1.6 (0.69) 0.6 (0.69) 3.8 (0.63) 0.6 (0.96)
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Gilbert
Variable
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and questionnaires A-36 Hemophilia and IBQ. Pre 1.35 (1.59) 0.35 (1.10) 0.35 (0.95) 0.90 (1.84) 0.25 (0.63) 0.15 (0.28) 25.8 (3.61) 13.1 (2.51) 10.1 (1.96) 6.0 (0.47) 6.2 (1.61) 11.7 (1.56) 15.8 (1.31) 9.4 (1.77) 18.3 (2.31) 116.4 (11.26) 0.2 (0.63) 1.0 (1.15) 3.6 (0.69) 1.7 (0.94) 2.1 (1.85) 2.5 (1.43) 1.0 (0.94)
Control Group Post Follow 1.25 (1.23) 0.75 (1.28) 0.30 (0.79) 0.25 (0.79) 0.30 (0.94) 0.40 (1.26) 0.30 (0.56) 0.25 (0.79) 0.75 (0.18) 1.00 (0.31) 0.75 (0.18) 0.50 (0.15) 26.3 (2.94) 27.6 (2.11) 12.2 (2.39) 12.7 (2.98) 9.4 (0.69) 9.2 (1.54) 7.00 (0.66) 5.5 (2.27) 6.1 (2.64) 5.1 (1.96) 7.3 (6.34) 11.5 (2.01) 15.9 (1.66) 14.9 (1.79) 9.5 (1.35) 10.2 (1.98) 19 (1.63) 18.6 (1.50) 112.7 (10.22) 115.3 (11.30) 0.4 (0.96) 1.9 (2.02) 1.1 (1.10) 0.7 (0.67) 3.5 (0.70) 2.2 (0.63) 1.5 (1.35) 1.6 (0.84) 2.1 (1.72) 1.4 (1.50) 3.2 (0.91) 3.4 (1.17) 0.7 (0.48) 0.9 (0.99)
VAS: Visual Analog Scale; A-36: Haemophilia A-36 questionnaire; IBQ: Illness Behavior Questionnaire; Pre: pretreatment assessment; Post: postreatment assessment; Follow: follow up assessment; Sig: signification; *: P<.05.
Sig. .333 .510 .090 .488 .370 .392 .649 .548 .626 .017* .362 .032* .385 .394 .214 .333 .122 .413 .009* .703 .025* .363 .322
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Table 4. Means and signification of the assessment between the groups.
IBQ
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Postreatment EG CG 2.95 1.25 1.30 0.30 1.00 0.30 0.32 0.30 0.35 0.75 0.00 0.75 27.8 26.3 13 12.2 8 9.4 6.1 7.00 7.3 6.1 13 7.3 16.9 15.9 10.8 9.5 18.8 19 121.2 112.7 1.6 0.4 1.8 1.1 3.5 3.5 2.2 1.5 0.7 2.1 3.7 3.2 102.0 0.7
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Baseline CG Sig 1.35 .111 0.35 .365 0.35 .254 0.90 .611 0.25 .627 0.15 .512 25.8 .079 13.1 .066 10.1 .001* 6.0 .089 6.2 .220 11.7 .840 15.8 .788 9.4 .490 18.3 .883 116.4 .263 0.2 .458 1.0 .296 3.6 .754 1.7 .791 2.1 .058 2.5 .071 1.0 .361
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VAS
Gilbert ankle Gilbert knee Gilbert elbow VAS ankle VAS knee VAS elbow Physical Health Daily activities Joints Pain Satisfaction with treatment Dificulties with treatment Emotional functioning Mental Health Social relationships Overall Quality of life Hypochondrial Disease conviction Psychological perception vs. somatica Affective inhibition Affective disturbance Negation Irritability
EG 3.00 1.20 3.00 1.47 0.60 0.07 22.3 10.0 6.4 4.5 7.0 12.1 15.4 10.2 18.5 106.4 0.5 1.7 3.5 1.6 0.6 3.8 0.6
EP
Gilbert
Variable
AC C
Score
Sig .043* .302 .284 .338 .511 .241 .231 .497 .003* .166 .193 .021* .433 .164 .825 .181 .028* .229 1.00 .193 .038* .182 .062
Follow-up EG CG 2.80 0.75 1.25 0.25 0.90 0.40 0.60 0.25 0.10 1.00 0.20 0.50 24.5 27.6 11.6 12.7 6.5 9.2 4.7 5.5 7.3 5.1 11.5 11.5 15.4 14.9 10.6 10.2 18.6 18.6 111.6 115.3 0.6 1.9 1.0 0.7 2.1 2.2 1.5 1.6 0.2 1.4 3.4 3.4 0.6 0.9
Sig .023* .282 .424 .514 1.00 .309 .038* .504 .010* .326 .007* 1.00 .734 .739 1.00 .563 .102 .534 .749 .809 .032* 1.00 .503
VAS: Visual Analog Scale; A-36: Haemophilia A-36 questionnaire; IBQ: Illness Behavior Questionnaire; Baseline: baseline assessment; Post: postreatment assessment; Follow-up: follow-up assessment; EG: experimental group; CG: control group; Sig: signification
Enrollment
Assessed for eligibility (n=34)
RI PT
ACCEPTED MANUSCRIPT
SC
Excluded (n=14) Not meeting inclusion criteria (n=7) Declined to participate (n=2) Other reasons (n=5)
M AN U
Randomized (n=20)
Allocation
Experimental group (n=10) Received allocated intervention (n=10) Did not receive allocated intervention (n=0)
TE D
Control group (n=10) Received allocated intervention (n=0) Did not receive allocated intervention (control group with treatment) (n=10)
Follow-Up Lost to follow-up (give reasons) (n=0)
Discontinued intervention (n=0)
Discontinued intervention (n=0)
AC C
EP
Lost to follow-up (give reasons) (n=0)
Analysed (n=10) Excluded from analysis (n=0)
Analysis Analysed (n=10) Excluded from analysis (n=0)