Linda Kwakkenbos

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.

Psychological health and well-being in systemic sclerosis: State of the science and consensus research agenda.

Introduction Systemic sclerosis (SSc; scleroderma) is a multisystem disorder characterized by disturbance in fibroblast function, microvascular disease, and immune system activation, culminating in fibrosis of the skin and internal organs (1,2). SSc is associated with extensive morbidity, including disfiguring skin thickening, finger ulcers, joint contractures, pulmonary hypertension, interstitial lung disease, chronic diarrhea, and renal failure (1,2). The rate of disease onset is highest between 30 and 50 years of age, with the risk for women being 4 to 5 times higher than for men (3,4). Median survival time from diagnosis is 11 years, and patients are 3.7 times more likely to die within 10 years of diagnosis (44.9% mortality) than age-, sex-, and race-matched individuals without SSc (12.0% mortality) (3).

Disease-related and psychosocial factors associated with depressive symptoms in patients with systemic sclerosis, including fear of progression and appearance self-esteem

OBJECTIVE The prevalence of depressive symptoms is high in patients with systemic sclerosis (SSc, scleroderma). This study was conducted to determine which disease-related and psychosocial factors are associated with depressive symptoms, independent of sociodemographic factors. METHODS In total, 215 patients with SSc completed questionnaires on sociodemographics, physical functioning (HAQ-DI), pain (VAS), fatigue (CIS), psychosocial characteristics (CISS, ICQ, PRQ, ASE, FoP-Q-SF) and depressive symptoms (CES-D). Disease characteristics (disease duration, disease subtype, modified Rodnan Skin Score) were collected. Hierarchical linear regression analyses were conducted to assess associations with depressive symptoms. RESULTS The mean CES-D score was 12.9 (SD=9.7) and the prevalence of patients scoring>= 16 and>=19 were 32.1% and 25.1%, respectively. The variance explained by sociodemographics and disease characteristics was negligible (R(2)≤.09). Fatigue and pain were independently associated with depressive symptoms (R(2) change=.35). After adding psychological factors (R(2) change=.21), satisfaction with social support, emotion-focused coping and helplessness were also significantly associated with depressive symptoms. Higher fear of progression was associated with more depressive symptoms (P≤.01), and appearance self-esteem showed a marginally significant association (P=.08). CONCLUSION Depressive symptoms were common in the present sample of patients with SSc and were independently associated with pain, fatigue, social support, emotion-focused coping, helplessness and fear of progression. Results suggest that, in addition to assessment of disease characteristics, attention should be given also to psychosocial factors found to be associated with depressive symptoms. For the development and trialling of psychological interventions, fear of progression could be an important target.

Triple P-Positive Parenting programs: the folly of basing social policy on underpowered flawed studies

Wilson et al. provided a valuable systematic and meta-analytic review of the Triple P-Positive Parenting program in which they identified substantial problems in the quality of available evidence. Their review largely escaped unscathed after Sanders et al.s critical commentary. However, both of these sources overlook the most serious problem with the Triple P literature, namely, the over-reliance on positive but substantially underpowered trials. Such trials are particularly susceptible to risks of bias and investigator manipulation of apparent results. We offer a justification for the criterion of no fewer than 35 participants in either the intervention or control group. Applying this criterion, 19 of the 23 trials identified by Wilson et al. were eliminated. A number of these trials were so small that it would be statistically improbable that they would detect an effect even if it were present. We argued that clinicians and policymakers implementing Triple P programs incorporate evaluations to ensure that goals are being met and resources are not being squandered.Please see related articles http://www.biomedcentral.com/1741-7015/10/130 and http://www.biomedcentral.com/1741-7015/10/145

Reporting of positive results in randomized controlled trials of mindfulness-based mental health interventions

Background A large proportion of mindfulness-based therapy trials report statistically significant results, even in the context of very low statistical power. The objective of the present study was to characterize the reporting of “positive” results in randomized controlled trials of mindfulness-based therapy. We also assessed mindfulness-based therapy trial registrations for indications of possible reporting bias and reviewed recent systematic reviews and meta-analyses to determine whether reporting biases were identified. Methods CINAHL, Cochrane CENTRAL, EMBASE, ISI, MEDLINE, PsycInfo, and SCOPUS databases were searched for randomized controlled trials of mindfulness-based therapy. The number of positive trials was described and compared to the number that might be expected if mindfulness-based therapy were similarly effective compared to individual therapy for depression. Trial registries were searched for mindfulness-based therapy registrations. CINAHL, Cochrane CENTRAL, EMBASE, ISI, MEDLINE, PsycInfo, and SCOPUS were also searched for mindfulness-based therapy systematic reviews and meta-analyses. Results 108 (87%) of 124 published trials reported ≥1 positive outcome in the abstract, and 109 (88%) concluded that mindfulness-based therapy was effective, 1.6 times greater than the expected number of positive trials based on effect size d = 0.55 (expected number positive trials = 65.7). Of 21 trial registrations, 13 (62%) remained unpublished 30 months post-trial completion. No trial registrations adequately specified a single primary outcome measure with time of assessment. None of 36 systematic reviews and meta-analyses concluded that effect estimates were overestimated due to reporting biases. Conclusions The proportion of mindfulness-based therapy trials with statistically significant results may overstate what would occur in practice.

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).

Psychosocial Aspects of Scleroderma.

Patients with systemic sclerosis (SSc; also called scleroderma) have to cope with not only the physical impacts of the disease but also the emotional and social consequences of living with the condition. Because there is no cure for SSc, improving quality of life is a primary focus of treatment and an important clinical challenge. This article summarizes significant problems faced by patients with SSc, including depression, anxiety, fatigue, sleep disruption, pain, pruritus, body image dissatisfaction, and sexual dysfunction, and describes options to help patients cope with the consequences of the disease.

Attentional and behavioural responses of spider fearfuls to virtual spiders

This study employed an immersed virtual environment (IVE) in the Nijmegen RIVERlab to study spider fearfuls’ attentional and motor reactions to virtual spiders. The participants were exposed to virtual spiders while completing an unrelated task, walking freely through a virtual museum. Compared to non-fearful controls, spider fearfuls showed an increase in state anxiety, they spent more time looking at spiders, and they exhibited spontaneous behavioural avoidance of spiders and visually similar objects. The results extend, and to some degree contradict, those of earlier studies with static pictures, and they speak to the usefulness of state-of-the-art IVEs in fundamental anxiety research.

Validity of the Fear of Progression Questionnaire-Short Form in patients with systemic sclerosis.

To validate the Dutch translation of the Fear of Progression Questionnaire‐Short Form (FoP‐Q‐SF) for patients with systemic sclerosis (SSc). Although concerns about the future are often expressed by patients with SSc, there is no valid quantitative measure available to assess the extent to which patients with SSc are troubled by those concerns.

Spirituality and negative emotions in individuals with coronary heart disease

Many individuals with coronary heart disease (CHD) experience disease-related anxiety, depressive symptoms, and anger. Spirituality may be helpful to cope with these negative emotions. Research findings on the role of spirituality in dealing with negative emotions are inconsistent. In this study, we examined the associations between 7 dimensions of spirituality (ie, meaningfulness, trust, acceptance, caring for others, connectedness with nature, transcendent experiences, and spiritual activities) and negative emotions among individuals with CHD in Indonesia, controlling for perceived social support as well as demographic and clinical characteristics. In total, 293 individuals with CHD were recruited from the 3 largest hospitals in Bandung, Indonesia. They completed the Spiritual Attitude and Involvement List, the Beck Depression Inventory-II, the Trait Anxiety Scale of the State Trait Anxiety Inventory, the Multidimensional Anger Inventory, and the Multidimensional Scale of Perceived Social Support. Hierarchical linear regression analyses indicated that a higher overall level of spirituality was associated with lower levels of depressive symptoms, less anxiety, and less anger. Specifically, a higher level of trust was significantly associated with both less depressive symptoms and less anxiety. Higher levels of caring for others and spiritual activities were associated with less anxiety, and a higher level of connectedness with nature was associated with less anger. These findings underscore the importance of specific dimensions of spirituality as a potentially independent buffer against negative emotions in individuals with CHD.