Evaluating the Patient-Reported Outcomes Measurement Information System scales in acute intermittent porphyria.

PurposeAcute intermittent porphyria (AIP) is a rare inborn error of heme biosynthesis characterized by life-threatening acute attacks. Few studies have assessed quality of life (QoL) in AIP and those that have had small sample sizes and used tools that may not have captured important domains.MethodsBaseline data from the Porphyrias Consortium's Longitudinal Study were obtained for 259 patients, including detailed disease and medical history data, and the following Patient-Reported Outcomes Measurement Information System (PROMIS) scales: anxiety, depression, pain interference, fatigue, sleep disturbance, physical function, and satisfaction with social roles. Relationships between PROMIS scores and clinical and biochemical AIP features were explored.ResultsPROMIS scores were significantly worse than the general population across all domains, except depression. Each domain discriminated well between asymptomatic and symptomatic patients with symptomatic patients having worse scores. Many important clinical variables like symptom frequency were significantly associated with domain scores in univariate analyses, showing responsiveness of the scales, specifically pain interference and fatigue. However, most regression models only explained ~20% of the variability observed in domain scores.ConclusionPain interference and fatigue were the most responsive scales in measuring QoL in this AIP cohort. Future studies should assess whether these scales capture longitudinal disease progression and treatment response

Therapeutic alliance in telephone-administered cognitive–behavioral therapy for hematopoietic stem cell transplant survivors.

A strong therapeutic alliance has been found to predict psychotherapeutic treatment success across a variety of therapeutic modalities and patient populations. However, only a few studies have examined therapeutic alliance as a predictor of psychotherapy outcome among cancer survivors, and none have examined this relation in telephone administered cognitive behavioral therapy (T-CBT). This study evaluated the extent to which therapeutic alliance affected psychotherapy outcomes in survivors of hematopoietic stem cell transplantation (HSCT), a treatment for some cancers

Self-Efficacy Beliefs Mediate the Relationship between Subjective Cognitive Functioning and Physical and Mental Well-Being after Hematopoietic Stem Cell Transplant

Cognitive problems are commonly reported by hematopoietic stem cell transplant (HSCT) survivors, and are associated with poorer physical and mental well-being. It was hypothesized that adverse effects of subjective cognitive impairment occur because cognitive difficulties reduce survivors’ confidence that they can manage HSCT-related symptoms—that is, self-efficacy for symptom management

Mapping genomic loci implicates genes and synaptic biology in schizophrenia

Schizophrenia has a heritability of 60-80%1, much of which is attributable to common risk alleles. Here, in a two-stage genome-wide association study of up to 76,755 individuals with schizophrenia and 243,649 control individuals, we report common variant associations at 287 distinct genomic loci. Associations were concentrated in genes that are expressed in excitatory and inhibitory neurons of the central nervous system, but not in other tissues or cell types. Using fine-mapping and functional genomic data, we identify 120 genes (106 protein-coding) that are likely to underpin associations at some of these loci, including 16 genes with credible causal non-synonymous or untranslated region variation. We also implicate fundamental processes related to neuronal function, including synaptic organization, differentiation and transmission. Fine-mapped candidates were enriched for genes associated with rare disruptive coding variants in people with schizophrenia, including the glutamate receptor subunit GRIN2A and transcription factor SP4, and were also enriched for genes implicated by such variants in neurodevelopmental disorders. We identify biological processes relevant to schizophrenia pathophysiology; show convergence of common and rare variant associations in schizophrenia and neurodevelopmental disorders; and provide a resource of prioritized genes and variants to advance mechanistic studies

Therapeutic alliance in telephone-administered cognitive–behavioral therapy for hematopoietic stem cell transplant survivors.

OBJECTIVE: A strong therapeutic alliance has been found to predict psychotherapeutic treatment success across a variety of therapeutic modalities and patient populations. However, only a few studies have examined therapeutic alliance as a predictor of psychotherapy outcome among cancer survivors, and none have examined this relation in telephone administered cognitive behavioral therapy (T-CBT). This study evaluated the extent to which therapeutic alliance affected psychotherapy outcomes in survivors of hematopoietic stem cell transplantation (HSCT), a treatment for some cancers. METHODS: Forty-six patients enrolled in a randomized clinical trial of T-CBT for posttraumatic stress disorder (PTSD) completed a baseline assessment (including self-report measures of PTSD symptoms, depression, and general distress), 10 individual T-CBT sessions, and follow-up assessments at 6, 9, and 12 months post-baseline. Therapeutic alliance was assessed after the third T-CBT session with the Working Alliance Inventory, which yields overall and subscale (task, bond, and goal) scores. RESULTS: Analyses revealed that higher total therapeutic alliance scores prospectively predicted decreased depressive symptomatology; higher task scores predicted decreased overall distress, depressive symptomatology, symptoms of re-experiencing, and avoidance; and higher bond scores predicted decreased depressive symptomatology and symptoms of re-experiencing. CONCLUSIONS: These results suggest that assessments of therapeutic alliance should be incorporated into routine clinical care, and therapeutic alliance should be specifically cultivated in interventions to maximize psychotherapeutic benefits involving vulnerable populations such as cancer survivors

The Effect of Systematic Light Exposure on Sleep in a Mixed Group of Fatigued Cancer Survivors.

STUDY OBJECTIVES:Sleep disturbances are commonly reported by cancer survivors. Systematic light exposure using bright light has been used to improve sleep in other populations. In this secondary data analysis, the effect of morning administration of bright light on sleep and sleep quality was examined in a mixed group of fatigued cancer survivors. METHODS:Forty-four cancer survivors screened for cancer-related fatigue were randomized to either a bright white light or a comparison dim red light condition. Participants were instructed to use a light box every morning for 30 minutes for 4 weeks. Wrist actigraphy and the Pittsburgh Sleep Quality Index were administered at 4 time points: prior to light treatment (baseline), 2 weeks into the intervention, during the last week of the intervention, and 3 weeks postintervention. Thirty-seven participants completed the end-of-intervention assessment. RESULTS:Repeated-measures linear mixed models indicated a statistically significant time × treatment group interaction effect with sleep efficiency improving more in the bright light condition over time compared with the dim light condition (F3,42 = 5.55; P = .003) with a large effect size (partial η2 = 0.28). By the end of the intervention and 3 weeks postintervention, mean sleep efficiency in the bright light group was in the normal range. Medium to large effect sizes were also seen in sleep quality, total sleep time, and wake after sleep onset for participants favoring the bright light condition. CONCLUSIONS:The results suggest that systematic bright light exposure in the morning may have beneficial effects on sleep in fatigued cancer survivors. Larger scale efficacy trials are warranted. CLINICAL TRIAL REGISTRATION:Registry: ClinicalTrials.gov, Title: Treating Cancer-Related Fatigue Through Systematic Light Exposure, Identifier: NCT01873794, URL: https://clinicaltrials.gov/ct2/show/NCT01873794

Is all pain is treated equally? A multicenter evaluation of acute pain care by age

Pain is highly prevalent in health care settings; however, disparities continue to exist in pain care treatment. Few studies have investigated if differences exist based on patient-related characteristics associated with aging. The objective of this study was to determine if there are differences in acute pain care for older vs younger patients. This was a multicenter, retrospective, cross-sectional observation study of 5 emergency departments across the United States evaluating the 2 most commonly presenting pain conditions for older adults, abdominal and fracture pain. Multivariable adjusted hierarchical modeling was completed. A total of 6,948 visits were reviewed. Older (⩾ 65 years) and oldest (⩾ 85 years) were less likely to receive analgesics compared to younger patients (<65 years), yet older patients had greater reductions in final pain scores. When evaluating pain treatment and final pain scores, differences appeared to be based on type of pain. Older patients with abdominal pain were less likely to receive pain medications, while older patients with fracture were more likely to receive analgesics and opioids compared to younger patients. Differences in pain care for older patients appear to be driven by the type of presenting pain