Rehabilitation Program in Patients With Scleroderma

Physical Rehabilitation in Women With Scleroderma: Effects on Pulmonary Function, Lung Ultrasound, Muscle Function, Hand Functional, Functional Capacity, and Quality of Life

Scleroderma (or Systemic Sclerosis - SSc) is one of the most neglected diseases worldwide, according to the World Health Organization. In the adult population with SS, the systemic effects of the disease, such as respiratory and peripheral muscle dysfunction, cause a decrease in quality of life. As a consequence, there is a concern about functional rehabilitation, since the aging of this population is already a reality. Thus, the objective of this project is to evaluate the effects of functional rehabilitation on functional capacity and quality of life in women over 18 years of SS. In this longitudinal intervention study, patients will be submitted to a three-month rehabilitation program. Before and after the intervention, patients will be submitted to the following assessments: Cochin Hand Functional Scale (CHFS), Short-Form 36 Health Survey (SF-36); Scleroderma Health Assessment Questionnaire Disability Index (SHAQ-DI); lung function; lung ultrasound; handgrip; Glittre Activities of Daily Living test. Thus, it is expected that patients with SS will benefit significantly, with a consequent improvement in musculoskeletal function and , functional capacity and health-related quality of life.

In the current epidemiological context, chronic diseases affect very specific and neglected populations. In addition to their rarity, these diseases are characterized by a progressive increase in the life expectancy of their patients and important functional limitations due to their multisystemic character. In this context, there is Systemic Sclerosis - SSc (or Scleroderma), which presents peculiar clinical and functional aspects that limit the activities of daily living (ADL) of patients. Although the focus of SSc treatment is the clinical control of the disease, a new perspective must be given to the aspect of functional rehabilitation. Therefore, this study proposes a therapist-oriented home rehabilitation program (TOHR) for these patients. Considering that in SSc the hands are predominantly affected, and that functional incapacity of the hands is the main component of global incapacity in this population, the ADL-Glittre test has been used as a submaximal functional capacity test as it involves lower and upper limbs, as well as diversified tasks, coming closer to the activities that the individual does in its usual environment. Thus, the present study aims to evaluate the effects of a TOHR program on the functional capacity of patients with SSc, through the ADL-Glittre test, considering the impact of hand function, peripheral muscle strength, pulmonary function and quality of life. For this, women with SSc will be evaluated, submitted to the following tests: Cochin Hand Functional Scale, Short-Form 36 Health Survey; Scleroderma Health Assessment Questionnaire Disability Index; handgrip dynamometry; impulse oscillometry technique; spirometry; lung ultrasound and ADL-Glittre test. Additionally, patients will undergo a 12-week TOHR protocol. At the end of this period, all evaluations will be repeated. This will be a prospective, longitudinal study, with quantitative assessment of sample data. The aim of the study is to support the applicability of a TOHR program in women with SSc.

Women with Scleroderma will use a booklet-guided rehabilitation program that lasts three months (3 times per week with a duration of 60 minutes per session). Consisting of stretching, warm-up and cool-down exercises, muscle strengthening (flexion, extension, adduction, and abduction movements in exercises involving open and closed kinetic chains), endurance exercises, aerobic training, balance training and proprioception. The patient will be evaluated at 2 different timepoints (baseline and after 12 weeks of training). The physiotherapist contacts the patient by phone weekly to follow the progression of the treatment. Before and after the intervention, patients will be submitted to the following assessments: Cochin Hand Functional Scale (CHFS), Short-Form 36 Health Survey (SF-36); Scleroderma Health Assessment Questionnaire Disability Index (SHAQ-DI); lung function; lung ultrasound; handgrip; Glittre Activities of Daily Living test.

The program comprises the practice of warm-up, muscle strengthening with free weights and with their own body weight against the action of gravity for the main muscular groups, balance control training, aerobic training, and relaxation exercises. The proposal consists of 3 weekly sessions, for 12 consecutive weeks.

After a physical therapy evaluation, the patient underwent a booklet-guided physical exercise program that lasted three months (3 times per week with a duration of 60 minutes per session). Activities included overall stretching and strengthening (flexion, extension, adduction and abduction movements) and muscular endurance exercises (exercises involving open and closed kinetic chains), along with aerobic conditioning using a functional circuit. The patient was evaluated at 2 different timepoints (baseline and after treatment). The physiotherapist contacted the patient by phone weekly to follow the progression of the treatment.

Functional capacity through the Glittre Activities of Daily Living test (ADL-Glittre test) before and after the execution of the treatment plan

Performed as proposed by Skumlien et al. (2006), the participant must complete 5 laps in the shortest possible time. The test consists of carrying a backpack containing a 2.5 kg weight completing a circuit with the following activities. The participant rises from a seated position and walks on a 10-m flat course, interposed halfway by a stairway with 2 steps to ascend and 2 to descend. After completing the course, the individual approaches a 2-teir shelf containing 3 objects weighing 1 kg each, placed on the highest shelf (shoulder height), that must be moved 1 by 1 to the bottom shelf (waist height) and then down to the floor. Then, the objects are placed on the bottom shelf again and finally on the top shelf. Then, the individual turns and walks back over the course; immediately after completion of 1 lap, another lap is started, completing the same circuit. The instructions are standardized, and there is no incentive during the test. Two tests are performed with a minim.

Isometric handgrip strength (IHGS) was measured using a hydraulic isometric dynamometer (SH5001, Saehan Corporation, Korea) with the hand on the dominant side of the body. Participants were comfortably seated in a chair with no arm rest, with the feet flat on the floor and hips and knees flexed at 90°. The shoulder was adducted, the elbow was flexed at 90º, and the forearm was in a neutral position; the wrist position could vary between 0-30º of extension and 0-15º of ulnar deviation. The participants were then instructed to perform 3 maximum voluntary contractions with a 60-s interval in between. The tests followed the standards established by the American Society of Hand Therapists. The highest recorded value among the 3 measurements was considered.

The CHFS was used to assess the level of hand ability and functionality in the performance of daily activities. The CHFS contains 18 items concerning ADLs that require manual skills, such as cooking, dressing and personal hygiene. This scale takes approximately 3 min to complete and considers only the experiences of the individual during the past month. Each item has 6 possible answers, as follows: without difficulty (0); with very little difficulty (1); with some difficulty (2); with much difficulty (3); nearly impossible to do (4); and impossible to do (5). The score is the sum of all items and ranges from 0 to 90; the higher the score is, the greater the hand dysfunction.

Quality of life related to activities of daily living through the Medical Outcomes Study 36-Item Short-Form Health Survey (SF-36) before and after the execution of the treatment plan.

The 36-Item Short Form Survey (SF-36) is an oft-used, well-researched, self-reported measure of health. It stems from a study called the Medical Outcomes Study. Patients or individuals are asked to fill out the questionnaire (tick boxes) by themselves and then it is scored by a researcher. It contains the 36 questions and answers in the bodily pain and general health domains.Scores for the different domains are converted and pooled using a scoring key, for a total score indicating a range of low to high QOL. Two component scores can also be tallied; a physical component summary and a mental component summary.

Impulse oscillometry (IOS) will use an impulse oscillometer (Quark i2m, Cosmed, Rome, Italy). IOS measures the respiratory system impedance (Zrs) composed of the respiratory system resistance and the respiratory system reactance. The minimum acceptable coherence values will be those ≥ 0.9 Hz. (RIBEIRO et al, 2018) Participants will also perform spirometry (VT 130 SL, Codax Ltda, Rio de Janeiro, Brazil), using standardization made by the American Thoracic Society/European Respiratory Society. The predicted values of FVC, expiratory volume in one second (FEV1) and forced expiratory flow between 25-75% of FVC (FEF25-75%) will be calculated according to the equations of Pereira et al (2007) and the results will be expressed as percentages of the predicted values. Obstructive disorder will be defined by an FEV1/FVC ratio <70%, while a restrictive disorder will be inferred by a FVC <80% predicted in the absence of reduced expiratory flows.

Using an Aplio XG Aplio XG equipment (Toshiba Medical Systems, Tokyo, Japan), with a 7.5-10 MHz multifrequency linear transducer or a 3.5-5 MHz convex transducer in B mode, the USP exams will be performed on the same day as the TFP. Following a 12-zone protocol and with participants in a seated position, the USP exams will be performed in 6 areas of each hemithorax (two anterior, two lateral and two posterior). USP images will be examined for the evaluation of the following signs: B lines > 2, coalescent B lines and subpleural consolidations. In order to classify the lung injury, in each of these 6 areas, weights ranging from 1 to 3 will be assigned for each finding in the USP, as follows: 1 = lines B > 2; 2 = coalescent B lines; and 3 = subpleural consolidations). The sum of all 6 areas evaluated at USP will represent the aeration score, which may vary between 0-18 points. (SOUMMER et al., 2012; TUNG-CHEN et al., 2020)

Disability assessment using the Scleroderma Health Assessment Questionnaire Disability Index (SHAQ-DI) before and after the execution of the treatment plan

The SHAQ-DI will be used to assess the participants' level of physical function. This questionnaire includes questions related to fine movements of the upper extremities and motor activities of the lower limbs (BRUCE & FRIES, 2003). There are 20 questions divided into 8 categories that represent a comprehensive set of functional activities performed in the last 7 days, including the ability to dress, stand up, eat, walk, perform personal hygiene, reach or grab something, and perform usual activities . To quantify the values found, the SHAQ-DI has a 26 score ranging from 0 (no disability) to 3 (maximum disability). The total score is the average of the scores of the 8 categories.

Inclusion Criteria: Women with Scleroderma, older than 18 years Clinical stability Signature of the Informed Consent Term (TCLE) Exclusion Criteria: Patients with inability to perform the Glittre Activities of Daily Living test Patients who have cognitive impairment by mini mental state examination (MEEN). Abandonment of treatment of scleroderma during the application of the protocol. Uncontrolled hypertension (> 180/100 mmHg with medication use) Use of psychotropic drugs Any significant limitations due to osteoarthropathy History of surgery in the previous six months with exercise restriction IPAQ with very active classification

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Translation, cultural adaptation and reproducibility of the Cochin Hand Functional Scale questionnaire for Brazil

http://bases.bireme.br/cgi-bin/wxislind.exe/iah/online/?IsisScript=iah/iah.xis&src=google&base=LILACS&lang=p&nextAction=lnk&exprSearch=296502&indexSearch=ID

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