Patients with sickle cell disease (SCD) and thalassemia experience several complications across their lifespan that lead to impairment in different health-related quality of life (HRQOL) domains. There is increasing interest in curative therapies for patients with SCD and thalassemia, including hematopoietic stem cell transplant (HSCT) and gene therapy; however, the effect of these therapies on various HRQOL domains remains unclear. Our objective was to systematically evaluate the most recent evidence for the effect of HSCT and gene therapy on HRQOL in patients with SCD and thalassemia. A systematic search of medical literature databases was conducted. A total of 16 studies (thalassemia, n = 9; SCD, n = 6; both, n = 1) involving 517 participants met inclusion criteria (thalassemia, n = 416; SCD, n = 101). HSCT was associated with a small to large positive effects in most HRQOL domains (Cohen’s d; mean = 0.47; median = 0.37; range, 0.27-2.05). In thalassemia, HSCT was frequently associated with large positive effects in physical and emotional HRQOL domains (median d = 0.79 and d = 0.57, respectively). In SCD, HSCT was associated with large positive effects in all HRQOL domains. Emerging data suggest improvement in HRQOL outcomes across different domains following gene therapy in thalassemia and SCD. The quality of evidence was moderate in 13 studies (81%). HSCT has a positive impact on several HRQOL domains in patients with SCD and thalassemia; however, more longitudinal studies are warranted to assess the sustainability of these effects. Reporting HRQOL outcomes from ongoing gene therapy or gene-editing trials in SCD and thalassemia is key to better understand the benefits of such therapies.

Background Unintentional medication nonadherence is common and has been associated with poor health outcomes and increased health care costs. Earlier research demonstrated a relationship between habit strength and medication adherence. Previous research also examined a habit’s direct effect on adherence and how habit interacts with more conscious factors to influence or overrule them. However, the relationship between habit and adherence and the role of habit-based mobile health (mHealth) interventions remain unclear. Objective This review aimed to systematically evaluate the most recent evidence for habit strength, medication adherence, and habit-based mHealth interventions across chronic medical conditions. Methods A keyword search with combinations of the terms habit, habit strength, habit index, medication adherence, and medication compliance was conducted on the PubMed database. After duplicates were removed, two authors conducted independent abstract and full-text screening. The guidelines for the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) were followed when reporting evidence across the included and reviewed studies. Results Of the 687 records examined, 11 met the predefined inclusion criteria and were finalized for data extraction, grading, and synthesis. Most included studies (6/11, 55%) were cross-sectional and used a theoretical model (8/11, 73%). The majority of studies measured habit strength using the self-report habit index and self-report behavioral automaticity index (9/11, 82%). Habit strength was positively correlated with medication adherence in most studies (10/11, 91%). Habit mediated the effects of self-efficacy on medication adherence (1/11, 9%), and social norms moderated the effects of habit strength on medication adherence (1/11, 9%). Habit strength also moderated the effects of poor mental health symptoms and medication adherence (1/11, 9%). None of the included studies reported on using or proposing a habit-based mHealth behavioral intervention to promote medication adherence. Conclusions Habit strength was strongly correlated with medication adherence, and stronger habit was associated with higher medication adherence rates, regardless of the theoretical model and/or guiding framework. Habit-based interventions should be used to increase medication adherence, and these interventions could leverage widely available mobile technology tools such as mobile apps or text messaging, and existing routines.

BACKGROUND Cortico-amygdalohippocampectomy (CAH) is effective for mesial temporal lobe epilepsy (mTLE). Concerns regarding surgical morbidity have generated enthusiasm for more minimally invasive interventions. A careful analysis of current data is warranted before widespread adoption of these techniques. OBJECTIVE To systematically review the use of laser interstitial thermal therapy (LITT), stereotactic radiosurgery (SRS), radiofrequency thermocoagulation (RF-TC), and focused ultrasound for mTLE. METHODS Major online databases were searched for prospective observational studies, randomized clinical trials, and retrospective studies (>50 patients), including mTLE patients. Outcomes of interest were seizure freedom (Engel I), complications and re-operation rates, and neuropsychological and quality-of-life (QoL) data. RESULTS Nineteen publications were identified. At ≥6 mo postoperatively, LITT (9/19) Engel I outcomes ranged from 52% to 80%. SRS (3/19) has a latency period (52%-67%, 24-36 mo postoperatively) and the radiosurgery vs. open surgery for epilepsy (ROSE) trial reported inferiority of SRS compared to CAH. RF-TC (7/19) demonstrated variable seizure freedom rates (0%-79%) and high re-operation rates (0%-90%). Twelve studies reported neuropsychological outcomes but QoL (4/19) was not widely reported, and few studies (3/19) assessed both. Study quality ranged from fair to good. CONCLUSION Based on nonrandomized data, LITT has compelling evidence of efficacy; however, comparisons to surgical resection are lacking. SRS has a latency period and is inferior to CAH (ROSE trial). RF-TC is a less resource-intensive alternative to LITT; however, comparisons of efficacy are limited. Additional studies are needed before minimally invasive procedures can supplant standard surgery.

No abstract