Effect of new modalities of treatment on physicians management plan for patients with spinal muscular atrophy.

OBJECTIVE: To determine physicians` attitudes and stated practice in the management of patients with spinal muscular atrophy (SMA). We also aimed to explore their knowledge about consensus statement for standard of care in SMA and the role of new treatment modalities in changing the method of practice in the management of these cases. METHODS: This is a quantitative observational cross-sectional study, conducted from February to May 2017 among physicians who manage SMA patients in Kingdom of Saudi Arabia. The study cohort included pediatric neurologists, adult neurologists, and physicians of other sub-specialties who manage SMA patients. We used online and paper-based questionnaires. RESULTS: Half of the 169 participants were aware of the consensus guidelines for the care of SMA patients. With regard to the newly released Nursinersen treatment protocol for SMA-diagnosed patients, half of the participants were uncertain, and the other half were hesitant about its outcomes. Junior physicians tended to be significantly more inclined to reverse the do-not-resuscitate (DNR) status of an SMA-diagnosed child than more senior physicians. CONCLUSION: Our results indicate the existence of wide differences in physician practice with children of SMA disease. Our data demonstrate a need for increased awareness of consensus guidelines and further awareness about the physician`s role in the variability of care for children with SMA.

Mendelian Susceptibility to Mycobacterial Disease Caused by a Novel Founder IL12B Mutation in Saudi Arabia.

PURPOSE: Mendelian susceptibility to mycobacterial disease (MSMD) is a rare primary immunodeficiency predisposing congenitally affected individuals to diseases caused by weakly virulent mycobacteria, such as Bacillus Calmette-Guérin (BCG) vaccine strains and environmental mycobacteria. IL-12p40 deficiency is a genetic etiology of MSMD resulting in impaired IL-12- and IL-23-dependent IFN-γ immunity. Most of the reported patients with IL-12p40 deficiency originate from Saudi Arabia (30 of 52) and carry the recurrent IL12B mutation c.315insA (27 of 30). METHODS: Whole-exome sequencing was performed on three patients from two unrelated kindreds from Saudi Arabia with disseminated disease caused by a BCG vaccine substrain. RESULTS: Genetic analysis revealed a homozygous mutation, p.W60X, in exon 3 of the IL12B gene, resulting in complete IL12p40 deficiency. This mutation is recurrent due to a new founder effect. CONCLUSIONS: This report provides evidence for a second founder effect for recurrent mutations of IL12B in Saudi Arabia.

Feasibility and efficacy of sepsis management guidelines in a pediatric intensive care unit in Saudi Arabia: a quality improvement initiative.

OBJECTIVES: Evaluation of feasibility and effectiveness of Surviving Sepsis Campaign (SSC) Guidelines implementation at a Pediatric Intensive Care Unit (PICU) in Saudi Arabia to reduce severe sepsis associated mortality. DESIGN: Retrospective data analysis for a prospective quality improvement (QI) initiative. SETTINGS: PICU at King Saud University Medical City, Saudi Arabia. PARTICIPANTS: Children ≤14 years of age admitted to the PICU from July 2010 to March 2011 with suspected or proven sepsis. Comparisons were made to a previously admitted group of patients with sepsis from October 2009 to June 2010. INTERVENTIONS: Adaptation and implementation of the Surviving Sepsis Campaign-Clinical Practice Guidelines (SSC-CPGs) through AGREE instrument and ADAPTE process. MAIN OUTCOME MEASURES: We reported pre- and post-implementation outcome of interest for this QI initiative, annual sepsis-related mortality rate. Furthermore, we reported follow-up of annual mortality rate until December 2016. RESULTS: Sixty-five patients was included in the study (42 in post-guidelines implementation group and 23 in pre-guidelines implementation group). Mortality was insignificantly lower in the post-implementation group (26.2% vs. 47.8%; P = 0.079). However, when adjusted for severity, identified by number of failing organs in the multivariate regression analysis, the mortality difference was favorable for the post-implementation group (P = 0.006). The lower sepsis-related mortality rate was also sustained, with an average mortality rate of 15.11% for the subsequent years (2012-16). CONCLUSIONS: Adaptation and implementation of SSC Guidelines in our setting support its feasibility and potential benefits. However, a larger study is recommended to explore detailed compliance rates.

Respiratory support attitudes among pediatric intensive care staff for spinal muscular atrophy patients in Saudi Arabia.

OBJECTIVE: To explore therapeutic attitude of healthcare providers practicing in pediatric critical care in Saudi Arabia toward patients with Spinal Muscular Atroph (SMA) Type I, and to explore their awareness about the International Consensus statement for SMA care. METHODS: A cross-sectional survey was conducted in April 2015 during 6th Saudi Critical Care Conference, targeting physicians and respiratory therapists practicing in Pediatric Critical Care. RESULTS: Sixty participants accepted to participate in this survey. Out of those who answered the questionnaire, 44 were included in the analysis. Majority (66%) of participants were unaware of the International Consensus guidelines for SMA. Endotracheal intubation was reported as an acceptable intervention in SMA patients with acute respiratory failure by 43% of participants. Similarly, chronic home ventilation was agreed by 41% of participants. CONCLUSION: A nationwide adaptation of the International SMA Consensus guidelines for children with SMA I is recommended, aiming to decrease variability and standardize their management across various healthcare facilities in Saudi Arabia.

Cytomegalovirus associated severe pneumonia, multi-organ failure and Ganciclovir associated arrhythmia in immunocompetent child.

Cytomegalovirus (CMV) can rarely cause severe manifestations in immunocompetent individuals. Hereby, we report a twelve-year-old boy who presented with tachycardia, tachypnea, fever and leukocytosis, which progressed to hypoxemic respiratory failure and severe acute respiratory distress syndrome (ARDS). Subsequently, he developed multi-organ failure despite the ongoing full supportive care and empiric broad spectrum antibiotics. Cytomegalovirus infection was diagnosed by Polymerase Chain Reaction (PCR) in blood and histopathological examination of lung biopsy. Immunological work up for the child was unremarkable. Ganciclovir therapy was introduced and showed significant improvement until full recovery. However, our patient developed transient heart block as a rare complication for Ganciclovir therapy throughout his course. We present this case with literature review for the CMV infection associated morbidity and mortality among immunocompetent children.

Adverse events experienced with intrahospital transfer of critically ill patients: A national survey.

ABSTRACT: Research that focuses on transfers to and from the intensive care unit (ICU) could highlight important patients' safety issues. This study aims to describe healthcare workers' (HCWs) practices involved in patient transfers to or from the ICU.This cross-sectional study was conducted among HCWs during the Saudi Critical Care Society's annual International Conference, April 2017. Responses were assessed using Likert scales and frequencies. Bivariate analysis was used to evaluate the significance of different indicators.Overall, 312 HCWs participated in this study. Regarding transfer to ICUs, the most frequently reported complications were deterioration in respiratory status (51.4%), followed by deterioration in hemodynamic status (46.5%), and missing clinical information (35.5%). Regarding transfers from ICUs to the general ward, the most commonly reported complications were changes in respiratory status (55.6%), followed by incomplete clinical information (37.9%), and change in hemodynamic conditions (29%). The most-used models for communicating transfers were written documents in electronic health records (69.3%) and verbal communication (62.8%). One-fourth of the respondents were not aware of the Situation, Background, Assessment, Recommendation (SBAR) method of patients' handover. Pearson's test of correlation showed that the HCW's perceived satisfaction with their hospital transfer guidelines showed significant negative correlation with their reported transfer-related complications (r = -0.27, P < .010).Hemodynamic and respiratory status deterioration is representing significant adverse events among patients transferred to or from the ICU. Factors controlling the perceived satisfaction of HCWs involved in patients, transfer to and from the ICU need to be addressed, focusing on their compliance to the hospital-wide transfer and handover policies. Quality improvement initiatives could improve patient safety to transfer patients to and from the ICU and minimize the associated adverse events.

COVID-19 Critical Care Simulations: An International Cross-Sectional Survey.

Objective: To describe the utility and patterns of COVID-19 simulation scenarios across different international healthcare centers. Methods: This is a cross-sectional, international survey for multiple simulation centers team members, including team-leaders and healthcare workers (HCWs), based on each center's debriefing reports from 30 countries in all WHO regions. The main outcome measures were the COVID-19 simulations characteristics, facilitators, obstacles, and challenges encountered during the simulation sessions. Results: Invitation was sent to 343 simulation team leaders and multidisciplinary HCWs who responded; 121 completed the survey. The frequency of simulation sessions was monthly (27.1%), weekly (24.8%), twice weekly (19.8%), or daily (21.5%). Regarding the themes of the simulation sessions, they were COVID-19 patient arrival to ER (69.4%), COVID-19 patient intubation due to respiratory failure (66.1%), COVID-19 patient requiring CPR (53.7%), COVID-19 transport inside the hospital (53.7%), COVID-19 elective intubation in OR (37.2%), or Delivery of COVID-19 mother and neonatal care (19%). Among participants, 55.6% reported the team's full engagement in the simulation sessions. The average session length was 30-60 min. The debriefing process was conducted by the ICU facilitator in (51%) of the sessions followed by simulation staff in 41% of the sessions. A total of 80% reported significant improvement in clinical preparedness after simulation sessions, and 70% were satisfied with the COVID-19 sessions. Most perceived issues reported were related to infection control measures, followed by team dynamics, logistics, and patient transport issues. Conclusion: Simulation centers team leaders and HCWs reported positive feedback on COVID-19 simulation sessions with multidisciplinary personnel involvement. These drills are a valuable tool for rehearsing safe dynamics on the frontline of COVID-19. More research on COVID-19 simulation outcomes is warranted; to explore variable factors for each country and healthcare system.

Changes in healthcare workers' knowledge, attitudes, practices, and stress during the COVID-19 pandemic.

ABSTRACT: Coronavirus disease 2019 (COVID-19) has caused an unprecedented health crisis around the world, not least because of its heterogeneous clinical presentation and course. The new information on the pandemic emerging daily has made it challenging for healthcare workers (HCWs) to stay current with the latest knowledge, which could influence their attitudes and practices during patient care.This study is a follow-up evaluation of changes in HCWs' knowledge, attitudes, and practices as well as anxiety levels regarding COVID-19 since the beginning of the pandemic. Data were collected through an anonymous, predesigned, self-administered questionnaire that was sent online to HCWs in Saudi Arabia.The questionnaire was sent to 1500 HCWs, with a 63.8% response rate (N = 957). The majority of respondents were female (83%), and the most common age group was 31 to 40 years (52.2%). Nurses constituted 86.3% of the respondents. HCWs reported higher anxiety during the COVID-19 pandemic which increased from 4.91 ±â€Š2.84 to 8.6 ±â€Š2.27 on an 11-point Likert scale compared to other viral outbreaks. HCWs believed that their own preparedness as well as that of their hospital's intensive care unit or emergency room was higher during the COVID-19 pandemic than during the Middle East respiratory syndrome coronavirus pandemic (2012-2015). About 58% of HCWs attended one or more simulations concerning the management of COVID-19 patients in their intensive care unit/emergency room, and nearly all had undergone N95 mask fit testing. The mean score of HCWs' knowledge of COVID-19 was 9.89/12. For most respondents (94.6%), the perception of being at increased risk of infection was the main cause of anxiety related to COVID-19; the mean score of anxiety over COVID-19 increased from 4.91 ±â€Š2.84 before to 8.6 ±â€Š2.27 during the pandemic in Saudi Arabia.HCWs' anxiety levels regarding COVID-19 have increased since a pandemic was declared. It is vital that healthcare facilities provide more emotional and psychological support for all HCWs.

The psychological impact of COVID-19 pandemic on health care workers in a MERS-CoV endemic country.

BACKGROUND: The global pandemic of coronavirus disease of 2019 (COVID-19) has led to unprecedented psychological stress on health workers (HCWs). We aimed to assess the psychological impact of COVID-19 on HCWs in comparison to the stress brought on by the Middle East respiratory syndrome coronavirus (MERS-CoV) epidemic in Saudi Arabia. METHOD: Between February 5th and 16th, 2020, 811 health-care workers (HCWs) of a tertiary care teaching hospital were invited to fill a questionnaire regarding concerns and worries about the novel coronavirus pandemic, along with Generalized Anxiety Disorder (GAD-7) Anxiety Severity screening tool. RESULTS: Out of 582 HCWs who completed the survey questionnaire (response rate of 71.8%), about 40% were exposed previously to MERS-CoV infected or suspected patients during a previous hospital outbreak. While there were no COVID-19 cases reported yet in Saudi Arabia at the time of data collection, still, the anxiety level from COVID-19 was significantly higher than that from MERS-CoV or seasonal influenza: 41.1% were more worried about COVID-19, 41.4% were similarly worried about both MERS-CoV and COVID-19, and 17.5% were more stressed by the previous MERS-CoV hospital outbreak. The most frequent concern was transmitting the infection to family and friends (2.71/5) than to themselves only (2.57/5). CONCLUSION: Pandemic and epidemic infectious diseases such as COVID-19 or MERS-CoV impose a significant level of anxiety and stress on healthcare workers who are caring of infected patients, with their main concern being the risk of transmitting the infection to their families or to acquire it themselves. Therefore, optimizing the compliance of healthcare workers with the proper infection prevention and control measures is paramount during the infectious disease outbreak, to ensure their safety, to decrease the likelihood of getting infected or transmitting the infection to others, and consequently to alleviate their psychological stress and anxiety.

Long-stay patients in pediatric intensive care units. Five-years, 2-points, cross-sectional study.

OBJECTIVES: To explore the changing patterns of long-stay patients (LSP) to improve the utilization of pediatric intensive care units (PICUs) resources. METHODS: This is a 2-points cross-sectional study (5 years apart; 2014-2019) conducted among PICUs and SCICUs in Riyadh, Saudi Arabia. Children who have stayed in PICU for more than 21 days were included. RESULTS: Out of the 11 units approached, 10 (90%) agreed to participate. The prevalence of LSP in all these hospitals decreased from 32% (48/150) in 2014 to 23.4% (35/149) in 2019. The length of stay ranged from 22 days to 13.5 years. The majority of LSP had a neuromuscular or cardiac disease and were admitted with respiratory compromise. Ventilator-associated pneumonia was the most prevalent complication (37.5%). The most commonly used resources were mechanical ventilation (93.8%), antibiotics (60.4%), and blood-products transfusions (35.4%). The most common reason for the extended stay was medical reasons (51.1%), followed by a lack of family resources (26.5%) or lack of referral to long-term care facilities (22.4%). CONCLUSION: A long-stay is associated with significant critical care bed occupancy, complications, and utilization of resources that could be otherwise utilized as surge capacity for critical care services. Decreasing occupancy in this multicenter study deserves further engagement of the healthcare leaders and families to maximize the utilization of resources.