Functionality, Scleroderma, Scleroderma (Limited and Diffuse), Scleroderma, Localized, Systemic Sclerosis (SSc)
Conditions
Keywords
Juvenile scleroderma, scleroderma, systemic sclerosis, physical performance, functionality
Brief summary
Physical functionality and performance are important for individuals diagnosed with scleroderma. There is a need to address children and adults diagnosed with scleroderma comprehensively and to evaluate them on a biopsychosocial basis to support their disease management. In light of all this literature, the aim was to evaluate the biopsychosocial characteristics of both pediatric and adult scleroderma patients and to examine their physical performance and functionality. Additionally, this study aims to identify effective tests that can be used in future research to assess physical performance and functionality in individuals diagnosed with scleroderma.
Interventions
OTHERQuestionnaire and functionality application for child and adult scleroderma patients
Scales and functionality tests will be applied to cases diagnosed with scleroderma
Sponsors
Hacettepe University
Study design
Observational model
COHORT
Time perspective
PROSPECTIVE
Eligibility
Sex/Gender
ALL
Age
7 Years to No maximum
Healthy volunteers
No
Inclusion criteria
\*For a child diagnosed with scleroderma\* Inclusion Criteria: * Cases diagnosed with scleroderma, * Cases aged 7-18 years will be included.
Exclusion criteria
* Advanced heart/lung/liver/kidney disease, neurological disease, and malignancies * Cases that are not willing to participate in the study will be excluded. * Having participated in a regular exercise program for the past 3 months * Individuals who are not willing to participate in the study \*For adults diagnosed with scleroderma\* Inclusion Criteria: * Individuals diagnosed with scleroderma, * Individuals over the age of 18 will be included.
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| 6-Minute Walk Test | Two weeks | Participants are asked to walk as quickly as they can along a 30-meter corridor. The total distance will be measured in meters. Fatigue, pain and dyspnea will be monitored using the Modified Borg Scale. |
| Timed Up and Go Test | Two weeks | The time it takes for a person to get up from a chair with back support, walk 3 meters at a normal pace, and return to the chair is measured in seconds to evaluate lower extremity balance. The test will be performed 3 times. |
| 10-Step Stair Climbing Test | Two weeks | To assess the subject's lower extremity strength and dynamic balance, the time taken to quickly climb and descend a staircase with 10 steps, each measuring 16-20 cm high and equipped with a handrail, is recorded in seconds. The test is repeated three times, and the times are recorded. |
| 10-meter walk test | Two weeks | The individual is on a 10-meter path. The assessment is performed in three repetitions and recorded in seconds. |
| 30-second sit-to-stand test | Two weeks | It is performed to evaluate lower extremity endurance by having the individual stand up from a chair with their arms crossed over their chest and then sit back down. The individual is instructed to repeat this as quickly as possible for 30 seconds. The score for this assessment is the number of times the person fully stands up from a sitting position with arms crossed within 30 seconds. |
| Glittre Activities of Daily Living Test | Two weeks | It consists of a cycle of standardized activities such as walking, carrying weights, climbing stairs, and moving objects on a shelf. The equipment required for the test includes a chair, steps, a shelf, a backpack, weights (0.5 kg-2.5 kg), and a stopwatch. The patient completes the 10-meter course created for the test for five laps, and the time is recorded. |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Scleroderma Health Assessment Questionnaire (SHAQ) | Two weeks | It was developed by adding five questions to the HAQ regarding Raynaud's phenomenon, digital ulcers, gastrointestinal, pulmonary, and general scleroderma symptoms, to be rated on a 15 cm line. The SHAQ total score ranges from 0 to 3. A high score indicates low functionality. |
| Cognitive Exercise Therapy Approach-Biopsychosocial Questionnaire (BETY-BQ) | Two weeks | It was standardized as a biopsychosocial assessment tool and is scored using a 5-point Likert scale, with each item rated from 0 to 4 points. A high total score on the 30-item scale indicates a high level of biopsychosocial impact. |
| Short Form-36 (SF-36) | Two weeks | It will be used to determine an individual's quality of life levels. Each sub-parameter is scored between 0 and 100 points, with a high score indicating good health status. The SF-36 scale has eight sub-parameters: general health perception, physical function, social function, pain, mental health, role difficulty due to physical reasons, role difficulty due to emotional reasons, and vitality. It contains 11 questions consisting of a total of 36 items. |
| Central Sensitization Scale (CSS) | Two weeks | It is a 40-item scale, scored on a 0-4 Likert scale, that assesses symptoms related to central sensitization. A score above 40 is interpreted as an indicator of central sensitization. |
| Hospital Anxiety and Depression Scale (HADS) | Two weeks | It consists of fourteen questions, with odd-numbered questions assessing anxiety and even-numbered questions assessing depression. The options are scored from 0 to 3, with higher scores indicating a worse emotional state. The Turkish cut-off values for the scale are 10 for the anxiety subscale and 7 for the depression subscale. |
| Modified Rodnan skin score (MRSS) | Two weeks | A pediatric and adult rheumatologist assessed it. The MRSS evaluates skin thickening in 17 body regions, graded from 0 (normal) to 3 (severe skin thickening). The total score ranges from 0 to 51, with increased scores indicating poor skin involvement. |
| Childhood Health Assessment Questionnaire (CHAQ) | Two weeks | It assesses the functional abilities of children diagnosed with rheumatic diseases in their daily living activities. It consists of eight subscales (dressing and personal care, standing up, eating, walking, body care, reaching, grasping, activities) (0-3) and also assesses pain and general well-being using a visual analog scale (0-100). A high score indicates low functionality. |
| Modified Hand Mobility in Scleroderma Test (mHAMIS) | Two weeks | It is a functional test developed specifically for scleroderma to assess an individual's hand function. It evaluates four specific hand movements (finger flexion, finger extension, finger abduction, and dorsal extension). Scores range from 0 (normal) to 3 (complete failure) for each movement, with a total score ranging from 0 to 12. |
| Pain Catastrophizing Scale-Children (PCS-C) | Two weeks | It consists of 13 items that describe the different thoughts and feelings children may experience when they feel pain. The questionnaire assesses three areas: rumination, magnification, and helplessness. All items are rated on a scale from 0 (never) to 4 (always). Higher scores reflect more catastrophic beliefs about pain. The total score can range from 0 to 48. |
| Transition Readiness Assessment Questionnaire (TRAQ) | Two weeks | It includes 20 items divided into five subscales. These subscales are related to Management of Treatment (the first four items), Scheduling Appointments (items 5-11), Monitoring Health Issues (items 12-15), Communication with Healthcare Personnel (items 16-17), and Management of Daily Activities (items 18-20). The scale uses a 5-point Likert-type scale and has a total score range of 20 to 100. The scale score reflects the level of readiness for transitioning to adult care. |
| Pain Catastrophizing Scale | Two weeks | It includes 13 items that describe different thoughts and feelings that may occur when pain is experienced. The questionnaire assesses three areas: rumination, magnification, and helplessness. All items are rated on a scale from 0 (never) to 4 (always). Higher scores indicate more catastrophic pain beliefs. The total score ranges from 0 to 52. |
| Clinical Frailty Scale | Two weeks | It assesses elements such as comorbidity, cognitive impairment, and disability. Frailty scores range from 1 to 9, with one meaning 'very fit' and nine meaning 'terminally ill'. A score of 5 or above is considered a sign of frailty. |
| Frailty Scale | Two weeks | FRAIL is a 5-item scale developed by Morley and colleagues to evaluate frailty. Each question is scored as 0 or 1 point. The total score ranges from 0 to 5 points (0 points = Fit, 1-2 points = Pre-frail, 3-5 points = Frail). |
| Localized Scleroderma Assessment Tool (LoSCAT) | Two weeks | LoSCAT consists of two sections: the modified Localized Skin Severity Index, which measures disease activity, and the Localized Scleroderma Damage Index, which assesses damage (usually scored between 0 and 3). The Localized Skin Severity Index evaluates the color of the lesion border for erythema, skin thickness, and the presence of new lesions or lesion extension. In the Localized Scleroderma Damage Index, three areas of cutaneous damage are combined to obtain a score. These areas evaluate hyperpigmentation or hypopigmentation, whichever is more prominent, as well as dermal atrophy, subcutaneous atrophy, and dyspigmentation. |
| Juvenile Arthritis Quality of Life Questionnaire (JAQQ) | Two weeks | It consists of 74 items covering various health domains, including physical functioning, emotional well-being, and general symptoms. The items are divided into four dimensions related to the child's quality of life: gross motor function (GMF), fine motor function (FMF), psychosocial function (PF), and systemic or general symptoms (SGS). Each domain is scored on a Likert-type scale from 1 to 7; higher scores indicate poorer health-related quality of life. There is also a not applicable to me option for the items. The scale also includes a pain measurement (10 cm Visual Analog Scale (VAS)) to assess pain, but this score is not included in the total score. |
| Juvenile Arthritis Biopsychosocial Scale-JAB-Q-Patient | Two weeks | It is a patient/parent-centered measurement method that helps assess the patient's biopsychosocial aspects, such as disease activity, posture, functional and psychosocial status, fatigue, and school performance. The results from the questions are used to assess the functional status of children, while their psychosocial status is recorded based on their answers to 21 questions (ranging from 0 to 358). A high score indicates a poor psychosocial status. |
| Juvenile Arthritis Biopsychosocial Scale-JAB-Q-Family | Two weeks | JAB-Q is a multidimensional questionnaire and a parent (family) form. Developed in Turkish by Ünal and colleagues, the questionnaire is completed by one of the child's parents. The family form evaluates the parents' biopsychosocial status from their own perspective and provides scores ranging from 0 to 102. Higher scores indicate a worse biopsychosocial status. |
| Pain Catastrophizing Scale-Parent (PCS-P) | Two weeks | It is assessed using a 5-point Likert scale ranging from 0 (never) to 4 (always) and consists of 13 items. Similar to the child questionnaire, it assesses 3 domains: rumination, magnification, and helplessness. A total score between 0 and 52 is obtained; higher scores reflect a higher level of catastrophizing in parents. |
| Child and Adolescent Scale of Participation (CASP) | Two weeks | It is a 20-question survey that evaluates the community participation of children and adolescents in school, neighborhood, and local settings. The survey is divided into four sections: 6 questions about home participation, four questions on neighborhood and community involvement, five questions regarding school participation, and five questions on home and community engagement. It is rated on a scale of expected for their age (full participation), somewhat limited, very limited, unable to participate, and inapplicable. The survey is completed by the child's family or primary caregiver. The person filling out the questionnaire is asked to select the answer that best describes the child's level of participation. The assessment is scored out of 100 points, with higher scores reflecting a greater level of participation. |
Countries
Turkey (Türkiye)
Contacts
Primary ContactOrkun Tüfekçi, PT, PhD(c)
Outcome results
None listed