Hemophilia, Hemophilia A, Severe, Hemophilia B
Conditions
Keywords
Functional independence, Activities of daily living, Hemophilia, Factor VIII, Factor IX
Brief summary
This study aimed to establish the validity and reliability of the Turkish version of the Pediatric Haemophilia Activities List (short - PedHALshort) questionnaire.
Detailed description
Hemophilia is a blood disease characterized by a bleeding disorder that develops due to the deficiency of clotting factor VIII (Hamophilia A) or coagulation factor IX (Hamophilia B) proteins that provide clotting in the blood. In the hereditary disease showing X-linked recessive inheritance, female individuals are carriers and male individuals show a clinical picture. Although the clinical picture changes depending on the duration and frequency of bleeding, the severity of the disease is classified as severe, moderate and mild. Musculoskeletal problems are the most common complications in patients with severe and moderate hemophilia. Hemarthroses due to bleeding in the joint (80%) and hematomas due to intramuscular bleeding (20%) cause joint degeneration and muscle atrophy. Recurrent hemarthroses occur when there is not enough clotting factor in the setting, often before the previous hemarthrosis has resolved. This creates a vicious cycle of bleeding-inflammation-rebleeding known as the target joint. The resulting vicious circle causes permanent damage to bone and cartilage structures and causes arthropathy. The knee is the joint region where hemarthrosis and arthropathy are most common, followed by the elbow and ankle joints. Since these joints in the musculoskeletal system contain more synovial tissue than the others, they are more exposed to trauma and load and are injured. As a result of all these, pain, joint limitations and muscle strength losses occur. All these clinical findings lead to a decrease in functional independence in activities of daily living with the restriction of physical activity in patients. In the literature, there are questionnaires and measurement methods in which many functional evaluations are made for hemophilia patients. However, most of them have not been validated and reliable in Turkish. For this purpose, our study aimed to establish the validity and reliability of the Turkish version of the Pediatric Haemophilia Activities List (PedHALshort) questionnaire.
Interventions
None listed
Sponsors
Hasan Kalyoncu University
Study design
Observational model
COHORT
Time perspective
PROSPECTIVE
Eligibility
Sex/Gender
MALE
Age
4 Years to 17 Years
Healthy volunteers
No
Inclusion criteria
* Having been diagnosed with Hemophilia by a physician (Factor VIII-IX) * Ages 4-17 years old * Receiving regular prophylactic treatment * Children who volunteered to participate in the study and whose family consent was given
Exclusion criteria
\- Children with neurological signs that limit activities of daily living
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Paediatric Haemophilia Activities List | through of the study, average 6 months | PedHAL contains 22 items in 7 areas: sitting/kneeling/standing, functions of legs, functions of arms, use of transportation, personal care, housework and leisure activities, and sports. It consists of a child version (8-17 years old) and a parent version (4-17 years old). Items are scored for each item on a 6-point Likert scale (impossible, always, usually, sometimes, almost never, never), not applicable (N/A). . The score is converted to a normalized score from 0 to 100, the higher score representing better functional status. If more than half of the items are missing or scored as not applicable (N/A), a valid total score will not be calculated. |
| FISH | through of the study, average 6 months | The scoring system, which is categorized according to the international classification of functionality, disability and health (ICF), has been prepared by considering the activities that hemophilia patients may be affected by in their daily lives. Being a performance-based scale allows it to be used in different languages. The scale consists of 8 questions in total, including the sub-parameters of self-care, transfer and locomotion. Self care subparameter; eating-hygiene, bathing and dressing, transfer sub-parameter; chair and squat, locomotion subparameter; It consists of walking, stair climbing (12-14 steps) and running activities. Scoring of the activities is between 1 and 4 according to the degree of independence. The minimum score that can be obtained from the scale is 8, and the maximum score is 32. A high score indicates the functional independence of the individual. |
Countries
Turkey (Türkiye)
Contacts
Primary ContactTuğba GÖNEN, Asst. Prof.
Outcome results
None listed