A natural history study was conducted in 142 Thalassemic (Thal), 199 transfused Sickle Cell Disease (Tx-SCD, n 5 199), and 64 non-Tx-SCD subjects to describe the frequency of ironrelated morbidity and mortality. Subjects recruited from 31 centers in the US, Canada or the UK were similar with respect to age (overall: 25 ± 11 years, mean ± SD) and gender (52% female). We found that Tx-SCD subjects were hospitalized more frequently compared with Thal or non-Tx-SCD (P < 0.001). Among those hospitalized, Tx-SCD adult subjects were more likely to be unemployed compared with Thal (RR 5 1.6, 95% CI 1.0-2.5) or nonTx-SCD (RR 5 3.1, 95% CI 1.3-7.3). There was a positive relationship between the severity of iron overload, assessed by serum ferritin, and the frequency of hospitalizations (r 5 0.20; P 5 0.009). Twenty-three deaths were reported (6 Thal, 17 Tx-SCD) in 23.5 ± 10 months of follow-up. Within the Tx-SCD group, those who died began transfusion (25.3 vs. 12.4 years, P < 0.001) and chelation therapy later (26.8 vs. 14.2 years, P 5 0.01) compared with those who survived. The unadjusted death rate in Thal was lower (2.2/100 person years) compared with that in Tx-SCD (7.0/100 person years; RR 5 0.38: 95% CI 0.12-0.99). However, no difference was observed when age at death was considered. Despite improvements in therapy, death rate in this contemporary sample of transfused adult subjects with Thal or SCD is 3 times greater than the general US population. Long term follow-up of this unique cohort of subjects will be helpful in further defining the relationship of chronic, heavy iron overload to morbidity and mortality. Am. J. Hematol. 82:255-265, 2007. V V C 2006 Wiley-Liss, Inc. Contract

We conducted a multicenter pilot investigation of the safety and feasibility of bone marrow transplantation (BMT) in adults with severe sickle cell disease (SCD) (NCT 01565616) using a reduced toxicity preparative regimen of Busulfan (13.2 mg/kg), Fludarabine (175 mg/m2) and Thymoglobulin (6 mg/kg) and cyclosporine or tacrolimus and methotrexate for graft-versus-host disease (GVHD) prophylaxis. Twenty-two patients (median age 22 years; range 17–36) were enrolled at 8 centers. Seventeen patients received marrow from an HLA-identical sibling donor and 5 patients received marrow from an 8/8 HLA-allele matched unrelated donor. Before BMT, patients had stroke, acute chest syndrome, recurrent pain events, were receiving regular red blood cell transfusions, or had an elevated tricuspid regurgitant jet (TRJ) velocity, which fulfilled eligibility criteria. Four patients developed grade II-III acute GVHD (18%) and 6 developed chronic GVHD (27%) that was moderate in two and severe in one patient. One patient died of intracranial hemorrhage and one of GVHD. Nineteen patients had stable donor chimerism, 1-year post-transplant. One patient who developed secondary graft failure survives disease-free after a second BMT. The one-year overall survival and event-free survival (EFS) are 91% (95% CI 68–98%) and 86% (95% CI, 63–95%), respectively, and 3-year EFS is 82%. Statistically significant improvements in the pain interference and physical function domains of health-related quality of life were observed. The study satisfied the primary endpoint of 1-year EFS ≥70%. This regimen is being studied in a prospective clinical trial comparing HLA-matched donor BMT with standard of care in adults with severe SCD (NCT02766465).

Patients with sickle cell disease frequently experience severe pain events that lead to unplanned healthcare utilization. Mobile health tools (mHealth) may help prevent these events by providing remote monitoring and self-management support. This article describes the feasibility of the Sickle cell disease Mobile Application to Record symptoms via Technology (SMART), an mHealth app developed to help sickle cell disease patients monitor and manage their day-to-day symptoms. Fifteen patients recorded their pain intensity using a paper visual analog scale (VAS) and then repeated this measurement using an electronic VAS pain measure on SMART. Patients continued using SMART to record clinical symptoms, pain intensity, location and perceived severity, and treatment strategies for at least 28 days. Patient median age was 29 years (range 16-54); 60.0% were male. There was a high intraclass correlation between pain measurements entered on the paper VAS and SMART on the iPhone and the iPad We found a strong association between patient perceived pain severity and pain intensity entries using SMART (b = 1.71; p < 0.01). Daily compliance with SMART entries was a mean 75.0%, with a high of 85.7% in week 1 and low of 57.9% in week 4; however, one-third (n = 5) of the patients were 100.0% compliant even in week 4. Patients who were over age 35 or used an iPad for the study had the highest compliance rates. This study showed that SMART is a useable and feasible method for monitoring daily pain symptoms among adolescents and adults with sickle cell disease-related pain.

The prevalence of objective insomnia assessed by polysomnography was higher than the prevalence of subjective insomnia according to DSM-IV-validated questionnaires. Clinical trials.gov ID: NCT00596713.

The widespread use of mobile phones among patients provides a unique opportunity for the development of mobile health intervention designed specifically for sickle cell disease, which will improve self-management as well as health care delivery. Our objective was to determine the receptiveness of patients with sickle cell disease to technology and a mobile application (app) designed for sickle cell disease. Phase I included 100 patients who completed a survey inquiring about receptiveness to technology and use of mobile devices to self-manage and communicate with providers. Phase II surveyed 17 additional patients who tested a newly developed sickle cell disease app, to report its usability and utility. In Phase I, on a 0-10 Likert scale where 0 is not comfortable, and 10 is extremely comfortable, 87.0% of participants reported >5 comfort level using a mobile device for health care management. Participants were comfortable with texting (81.0%) and emailing (77.0%) but not with social media (40.0%). Most participants (84.0%) owned computer devices (desktops, laptops, tablets, or iPads), and 92.0% owned mobile devices. In Phase II, participants reported that the app tested was useful to track pain (88.0%), and 94.0% reported that it was easy to use, practical, and useful for health self-management. All reported that the app was useful to help one communicate with providers. Following the use of our app, patients found it an extremely valuable tool for tracking pain, health management, and communicating with providers. We conclude that mobile technology might provide an appropriate venue for sickle cell disease healthcare management.

Carbapenemase-producing bacteria cause difficult-to-treat infections related to increased mortality in health care settings. Their occurrence has been reported in raw sewage, sewage-impacted rivers, and polluted coastal waters, which may indicate their spread to the community. We assessed the variety and concentration of carbapenemase producers in coastal waters with distinct pollution levels for 1 year. We describe various bacterial species producing distinct carbapenemases not only in unsuitable waters but also in waters considered suitable for primary contact.

The year 2020 has generated profound changes in personal and working relations, and in dreams of millions of people worldwide. The aim of this study was to investigate the frequency and content of nightmares during the COVID-19 pandemic in Brazil, evaluating its associations with sociodemographic, occupational, and clinical factors. Cross-sectional exploratory study, including 1,057 participants who responded to an online survey about mental violence and nightmares during the pandemic, between May 25 and June 1, 2020. A descriptive analysis of the results was done to obtain frequency tables. McNemar's non-parametric test was used to compare the frequency of nightmares before and after the pandemic, and logistic regression models, to identify factors most strongly associated with the pandemic nightmares. Participants were from 21 Brazilian states, with a mean age of 38 ± 14 years, and 78% women. Half of them (n = 529) reported at least one nightmare episode during the pandemic, and 32.9% (n = 348) described a pandemic content. There was nearly a 3-fold increase in the occurrence of nightmares "once a week or more" during the pandemic, 9% before vs. 25% after. Prior psychiatric care, suicidal ideation, sleep medication, increased pandemic alcohol consumption, perceiving high risk of contamination, being woman, and of younger age were factors associated with having nightmares during the pandemic. Prior psychiatric care, sleep medication, and age remained significant after excluding participants without nightmares and comparing between individuals with and without a pandemic content. We conclude the COVID-19 pandemic has affected people's dreams. The increase in the frequency of nightmares, their pandemic content, and association with previous conditions are a concerning public mental health issue and should be taken into consideration by authorities and policy makers.

To evaluate the effect of smoking habits on sleep, data from 1492 adults referred to the Sleep Institute were accessed and divided into 3 categories of smoking status: current, former and non-smokers. Categories of pack-years (<15 and ≥15) defined smoking severity. The association of smoking status and smoking severity with sleep was analyzed for sleep parameters, especially apnea and hypopnea index (AHI) ≥5, more than 5% of total sleep time (TST) spent with oxyhemoglobin saturation (SaO 2 ) <90%, and arousal index. The arousal index was higher among current (21 ± 17) and former smokers (20 ± 17) than non-smokers (17 ± 15; P < 0.04). Former smokers had a higher percent of TST at SaO 2 <90% than non-smokers (9 ± 18 vs 6 ± 13; P < 0.04). Former smokers with pack-years ≥15 compared to <15 exhibited higher AHI (22 ± 24 vs 16 ± 21; P < 0.05) and arousal index (22 ± 19 vs 18 ± 15; P < 0.05). Current smokers with pack-years ≥15 compared to <15 exhibited higher arousal index (23 ± 18 vs 18 ± 16; P < 0.05) and percent of TST at SaO 2 <90% (11 ± 17 vs 6 ± 13; P < 0.05). Smoking status and pack-years were not associated with AHI ≥5 on logistic regression analysis, but current smokers with pack-years ≥15 were 1.9 times more likely to spend more than 5% of TST at SaO 2 <90% than non-smokers (95%CI = 1.21-2.97; P = 0.005). The variability of arousal index was influenced by gender, AHI and current smokers with pack-years ≥15 (all P < 0.01). Smoking habits seem to be associated with arousal and oxyhemoglobin desaturation during sleep, but not with AHI. The effect was more pronounced in current than former smokers.