Transcultural Multidisciplinary Evaluation of Patients With Hemophili (ibHEro)

Multidisciplinary Evaluation of Patients With Hemophilia of Spain, El Salvador and Bolivia. An Observational Study

Multidisciplinary evaluation of patients with hemophilia Spain, El Salvador and Bolivia. It aims to analyze and evaluate the skeletal muscle of patients affected with hemophilia Spain, El Salvador and Bolivia; describe deficit mobility and muscular strength, depending on the degree of injury, age, presence of inhibitors and the development of physical activity; identify the relationship between the functional deficit by clinical assessment performed with the use of validated measurement scales, the assessment of the degree of injury to specific scales of hemophilia; detect biomechanical alterations of the lower limbs and their impact on gait in patients with hemophilia; and evaluate the factors that influence the perception of illness and quality of life of patients with hemophilia.

Study Overview

• Status: Unknown
• Conditions: Patients With Haemophilia

Detailed Description

Descriptive study of joint involvement in patients with hemophilia Spain, El Salvador and Bolivia.

Descriptive study of abnormal joint mobility and muscle strength as a result of acute conditions or the development of chronic articular sequelae.

Descriptive study of biomechanical changes in lower limb joints and their impact on gait in patients with hemophilia.

Descriptive study of the overall articular involvement (crepitus, pain, biomechanical and functional changes) in patients with hemophilia.

Descriptive study of the relationship between skeletal muscle pathology in patients with hemophilia and their perception of it, and their quality of life.

• Study Type: Observational
• Enrollment (Anticipated): 100

Contacts and Locations

Study Locations

• Spain
  • Murcia, Spain, 30107
    • Recruiting
    • Universidad Católica San Antonio de Murcia
    • Contact: RUBÉN CUESTA-BARRIUSO, PhD; Phone Number: 968278806; Email: ruben.cuestab@gmail.com
    • Contact: ANA TORRES-ORTUÑO, PhD; Phone Number: 968278806; Email: atorres.ortuno@gmail.com
    • Principal Investigator: RUBÉN CUESTA-BARRIUSO, PhD
    • Principal Investigator: ANA TORRES-ORTUÑO, PhD
    • Sub-Investigator: JOAQUÍN NIETO-MUNUERA, PhD

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 6 years to 65 years (Child, Adult, Older Adult)
• Accepts Healthy Volunteers: No
• Genders Eligible for Study: Male

• Sampling Method: Non-Probability Sample

Study Population

Patients with haemophilia from Spain, El Salvador and Bolivia

Description

Inclusion Criteria:

• Patients with hemophilia A and B.
• Patients of all ages (pediatric, adolescents, youth and adults).
• With or without joint involvement clinically diagnosed, and with or without a previous history of hemarthrosis joint load legs.
• Patients with or without inhibitors.

Exclusion Criteria:

• Patients without prior ambulation capacity.
• Patients diagnosed with other congenital coagulopathy (von Willebrand disease, etc..).
• Patients with neurological or cognitive impairments that prevent understanding the questionnaires and physical tests.

Study Plan

How is the study designed?

Design Details

• Observational Models: Cohort
• Time Perspectives: Prospective

Number of groups / cohorts

3

Cohorts and Interventions

Group / Cohort
Spain; Group of patients with hemophilia recruited in the Region of Murcia (Spain) and evaluated in the Universidad Católica San Antonio between June and July 2014.
El Salvador; Group of patients with hemophilia recruited in the city of San Salvador (El Salvador) and evaluated in the Hospital Nacional Rosales and the Hospital Nacional de niños "Benjamín Bloom" from San Salvador in April 2014.
Bolivia; Group of patients with hemophilia recruited in the city of Santa Cruz (Bolivia) and evaluated at the Hospital Nacional de niños in Santa Cruz August 2014.

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Assessment of range of motion of elbow, knee and ankle	We will use a goniometric evaluation, along the lines indicated by Querol for assessing the range of motion in patients with hemophilia	Screening visit
Assessment of muscle strength of arm, thigh and leg	We will use a measure of muscular balance with the guidelines outlined by Daniels, with the adaptation of Querol for assessing muscle strength in patients with hemophilia	Screening visit
Assessment of perception of joint pain	We will use the visual analogue scale (VAS) for the assessment of joint pain in patients with hemophilia, with a scale from 0 (no pain) to 10 (maximum pain).	Screening visit
Assessment of the status joint	We will use the specific questionnaire haemophilia: Haemophilia Joint Healts Score (HJHS) for the assessment of joint status	Screening visit
Evaluation of the performance of activities of daily living	We will use the questionnaire Haemophilia Activities List (HAL), specific for patients with hemophilia	Screening visit
Assessment of physical performance of patients with hemophilia	We will use the Physical Functioning scale (HEP-Test-Q), specific for patients with hemophilia	Screening visit
Assessment of personality traits of children	We will use the questionnaire Eysenck Personality Questionnaire Junior (EPQJ)	Screening visit
Evaluation of perceived quality of life for patients with hemophilia	We will use the Hemophilia-QoL and the Child Health and Illness Profile (CHIP-CE) questionnaires for children; and the Hemophilia-QoL and A36 and 36 Health Survey (SF36) questionnairesfor adults .	Screening visit

Secondary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Clinical variables of patients with hemophilia	Age (years); weight (kg); Size (cm); type of haemophilia (A or B); severity of hemophilia (severe / moderate / mild); pharmacological treatment (prophylactic / on demand); presence of inhibitors; central venous catheter; family history of hemophilia; hemarthrosis in the last month; arthropathy diagnosed in some joint; use of orthoses	Screening visit
Socio-demographic variables of patients with hemophilia	Number of hospital visits per year (reason); Distance to hospital (km); Academic formation of the patient (university studies / vocational training / basic studies / no studies); academic formation of the parents (university studies / vocational training / basic studies / no studies); patient employment (self-employment / employment has as an employed person / unemployed / retirement); parents employment (self-employment / employment has as an employed person / unemployed / retirement);	Screening visit

Collaborators and Investigators

• Sponsor: Universidad Católica San Antonio de Murcia

Study record dates

Study Major Dates

• Study Start: March 1, 2014
• Primary Completion (Anticipated): August 1, 2015
• Study Completion (Anticipated): December 1, 2015

Study Registration Dates

• First Submitted: July 23, 2014
• First Submitted That Met QC Criteria: July 23, 2014
• First Posted (Estimate): July 24, 2014

Study Record Updates

• Last Update Posted (Estimate): April 30, 2015
• Last Update Submitted That Met QC Criteria: April 29, 2015
• Last Verified: March 1, 2014

More Information

Terms related to this study

• Keywords: Quality of Life; Gait; Haemophilia; Arthropathy; Range of Movement; Strength; Proprioception
• Additional Relevant MeSH Terms: Hematologic Diseases; Blood Coagulation Disorders, Inherited; Coagulation Protein Disorders; Hemorrhagic Disorders; Genetic Diseases, Inborn; Blood Coagulation Disorders; Hemophilia A
• Other Study ID Numbers: ibHEro

Drug and device information, study documents

• Studies a U.S. FDA-regulated drug product: No
• Studies a U.S. FDA-regulated device product: No
• product manufactured in and exported from the U.S.: No