Janet L. Poole

The Scleroderma Patient-centered Intervention Network (SPIN) Cohort: protocol for a cohort multiple randomised controlled trial (cmRCT) design to support trials of psychosocial and rehabilitation interventions in a rare disease context

Introduction Psychosocial and rehabilitation interventions are increasingly used to attenuate disability and improve health-related quality of life (HRQL) in chronic diseases, but are typically not available for patients with rare diseases. Conducting rigorous, adequately powered trials of these interventions for patients with rare diseases is difficult. The Scleroderma Patient-centered Intervention Network (SPIN) is an international collaboration of patient organisations, clinicians and researchers. The aim of SPIN is to develop a research infrastructure to test accessible, low-cost self-guided online interventions to reduce disability and improve HRQL for people living with the rare disease systemic sclerosis (SSc or scleroderma). Once tested, effective interventions will be made accessible through patient organisations partnering with SPIN. Methods and analysis SPIN will employ the cohort multiple randomised controlled trial (cmRCT) design, in which patients consent to participate in a cohort for ongoing data collection. The aim is to recruit 1500–2000 patients from centres across the world within a period of 5 years (2013–2018). Eligible participants are persons ≥18 years of age with a diagnosis of SSc. In addition to baseline medical data, participants will complete patient-reported outcome measures every 3 months. Upon enrolment in the cohort, patients will consent to be contacted in the future to participate in intervention research and to allow their data to be used for comparison purposes for interventions tested with other cohort participants. Once interventions are developed, patients from the cohort will be randomly selected and offered interventions as part of pragmatic RCTs. Outcomes from patients offered interventions will be compared with outcomes from trial-eligible patients who are not offered the interventions. Ethics and dissemination The use of the cmRCT design, the development of self-guided online interventions and partnerships with patient organisations will allow SPIN to develop, rigourously test and effectively disseminate psychosocial and rehabilitation interventions for people with SSc.

Measures of hand function: Arthritis Hand Function Test (AHFT), Australian Canadian Osteoarthritis Hand Index (AUSCAN), Cochin Hand Function Scale, Functional Index for Hand Osteoarthritis (FIHOA), Grip Ability Test (GAT), Jebsen Hand Function Test (JHFT), and Michigan Hand Outcomes Questionnaire (MHQ)

Many of the rheumatic diseases result in pain, deformities, weakness, and other impairments that affect the hands. However, measures of these impairments may not provide information about the ability to use the hands for self-care, work, and leisure activities. Many health status, quality of life, and functional ability assessments have several questions pertaining to hand function, but these are usually limited to a few items due to the large scope of the assessment. For some individuals, specific assessments of hand function may be warranted to measure hand function and document treatment effectiveness. This section will review self-report and performance-based hand function tests that have been used with persons with rheumatic diseases and have psychometric support.

The Effectiveness of Inflatable Pressure Splints on Motor Function in Stroke Patients

The effectiveness of inflatable pressure splints was assessed by comparing upper extremity function in 18 subjects with hemiplegia. Matched pairs of subjects were randomly assigned to a splint or non-splint treatment condition. The splinted group had the splint applied for 30 minutes, 5 days a week, for 3 weeks, while the non-splint group received no treatment. Analysis of variance with repeated measures showed no statistically significant differences in mean change in upper extremity sensation, pain, and motor function from week 0 to week 3 between the splint and non-splint groups. These results are consistent with a previous finding in which a shorter treatment time period of 1 week was used. Suggestions for further research are discussed.

The Effect of Paraffin and Exercise on Hand Function in Persons with Scleroderma: A Series of Single Case Studies

The purpose of this study was to investigate whether the use of paraffin and active hand exercises would improve activity and participation in persons with scleroderma. In this series of three single case studies, participants used paraffin and performed active hand exercises daily for eight weeks. To assess hand function, measures of body function/structure and activity/participation were taken at baseline, at one month and at two months after intervention. All participants experienced clinically significant improvements in both body function/structure measurements of hand function and in their ability to participate in activities. Significant improvements were found more frequently on body function/structure measures than activity/participation measures. This preliminary study lends support in favor of using paraffin and hand exercises as a treatment to improve hand function related to participation in daily activities in persons with scleroderma. Further research with a larger sample and increased variable control is needed.

Assessments of Motor Function Post Stroke

Objective: The purpose of this article is to review assessments of motor function after stroke. Method: Eleven assessments were reviewed for what aspects of motor function are measured; tools needed; evidence for reliability and validity of the assessment and advantages and disadvantages of the assessment. The assessments were the Fugl-Meyer Assessment Scale for Stroke Patients (FMA), the Motor Assessment Scale (MAS), the Rivermead Motor Assessment (RMA), the Chedoke-McMaster Stroke Assessment (CMSA), the Motor Club Assessment (MCA), the Motricity Index (MI), the Stroke Rehabilitation Assessment of Movement (STREAM), the Frenchay Arm Test (FAT), the Action Research Arm Test (ARAT), the Functional Test for the Hemiparetic Upper Extremity (FT), and the Arm Motor Ability Test (AMAT). Results: The FMA, MAS, RMA, CMSA, MCA, MI, and STREAM measure motor function in both the upper and lower extremities while the FAT, ARAT, FT, and AMAT are specific to the upper limb only. The majority of the assessments test at the impairment level rather than at the activity level. The FMA, CMSA, MCA, MI, and STREAM require the least equipment (for example, a mat/bed, chair, marble/block) while the RMA, the MAS, the FAT, ARAT, FT, and AMAT require the use of numerous small objects, some of which are very specific in dimensions. The instruments differ in their reliability and validity with the FMA and CMSA having more sound psychometric evidence than the other tests reviewed. Conclusion: Familiarity with assessments of motor function can be helpful in selecting the one most appropriate to ones setting and need. These assessments, in addition to assessing activities of daily living, can separate specific lost neurological function from compensatory responses to document the outcomes and the effectiveness of therapeutic techniques.

Comparison of a self-report and performance-based test of disability in people with systemic lupus erythematosus

Purpose. The purpose of this study was to examine functional ability in people with SLE by comparing a self-report and a performance-based test. Method. Fifteen women with SLE and 15 healthy controls participated in this study. Participants completed a self report of daily living skills, the Health Assessment Questionnaire (HAQ). Actual performance during activities of daily living was evaluated with the Assessment of Motor and Process Skills (AMPS). Measures of cognition and disease activity were also collected. Results. HAQ scores of the participants with SLE indicated only very mild disability while scores on the AMPS indicated ineffective skill performance and potential safety risks. In the participants with SLE, cognitive status correlated significantly with the processing component (rs = 0.62, p < 0.05) and disease severity correlated with the motor component of the AMPS (rs = 0.84, p < 0.001). The HAQ did not correlate with the AMPS. Conclusions. This study suggests that cognitive deficits were more related to scores on the performance-based test of functional ability rather than the self-report. The performance-based test appeared to provide information that was not gained through self-report and measures of disease activity and gross cognition.

Become your own advocate: Advice from women living with scleroderma

Purpose. Systemic sclerosis (SSC) affects 300,000 people in the USA and has a significant effect on an individuals functional ability. The purpose of this study was to outline the key components of living with the illness and to identify the information that those who are newly diagnosed would need to initiate a successful course of disease self-management. The results will provide the groundwork for development and testing of a self-paced education program for patients with SSC. Method. Focus groups were conducted with 11 women diagnosed with SSC. Results. Analysis of the transcripts yielded three themes, Secure Effective Medical Management, Live Your Life, and Learn Everything You Can. The thread Become Your Own Advocate wove these three themes together and illustrated that taking control of SSC is ultimately a function of self-advocacy. Conclusion. For patients with SSC, taking control of their illness was a necessary component of maintaining the highest quality of life possible. A positive attitude, a strong support system, a commitment to moving forward with life, and access to high-quality, timely information all provided the participants with the tools to develop and implement a strategy of self-advocacy in disease management.

A comparison of static and dynamic wrist splints using electromyography in individuals with rheumatoid arthritis

This study compared strength, dexterity, and the muscle activation of individuals with rheumatoid arthritis (RA) when different splints were worn. Five persons with RA were observed in four splint conditions (none, static, hinged, spiral) during grip, pinch, and dexterity tests by recording muscle activity of eight muscles in the upper extremity using electromyography (EMG). Statistically significant differences were found in clinical tests when hinged (p<0.001) and spiral splints (p=0.02) were worn. Grip strength decreased when hinged splints were worn, whereas two-point pinch increased and dexterity improved on the Nine-Hole Peg Test with the spiral splint. Although no significant EMG differences were found during different splint conditions, wrist muscles were recruited more for grip, and shoulder muscles were used most during pinch and dexterity tests. Individuals with RA who wore splints had decreased grip when wearing hinged splints, but improved pinch and dexterity were found when the spiral splint was worn. Increased use of proximal shoulder muscles was observed during pinch and dexterity tests with and without splints.

Effectiveness of Nonpharmacologic Interventions in Systemic Sclerosis: A Systematic Review

To systematically and comprehensively document the effectiveness of nonpharmacologic interventions on physical functioning and psychological well‐being in patients with systemic sclerosis (SSc).

Oral hygiene in scleroderma: The effectiveness of a multi-disciplinary intervention program

Purpose. To investigate whether oral hygiene improves after persons with scleroderma received structured oral hygiene instructions and facial and hand exercises. Methods. Seventeen persons with scleroderma received a baseline dental evaluation including an examination for decayed or missing teeth, calculus, sites that bleed upon probing, measures of oral aperture, and the Patient Hygiene Performance Index. Upper extremity functioning including strength, joint motion, and dexterity were also measured. Participants received a structured home program consisting of patient education on brushing and flossing techniques, hand and facial exercises, adapted dental appliances, and a 6-month supply of dental products. Results. At the end of the 6-month intervention, there was a significant decrease (improvement) in mean PHP scores and a significant decrease in the number of teeth that bled on probing and with subgingival calculus. There were no differences in any of the upper extremity measures or oral aperture. Correlations between the upper extremity and oral measures showed associations between oral aperture and two of the dexterity measures and number of teeth with caries. Conclusion. These findings suggest that oral exercises and education regarding proper dental care may be useful in managing oral hygiene in persons with scleroderma.