The beta thalassaemia trait in Jamaica.

The objective of this study was to review the prevalence and features of the beta thalassaemia trait in Jamaican populations. Screening of 221,306 newborns over the last 46 years has given an indication of the distribution and prevalence of beta thalassaemia genes, and screening of 16,612 senior school students in Manchester parish, central Jamaica, has provided their haematological features. The prevalence of the beta thalassaemia trait predicted from double heterozygotes was 0.8% of 100,000 babies in Kingston, 0.9% of 121,306 newborns in southwest Jamaica, and 0.9% of school students in Manchester. Mild beta+ thalassaemia variants (-88 C>T, -29 A>G, -90 C>T, polyA T>C) accounted for 75% of Kingston newborns, 76% of newborns in southwest Jamaica, and 89% of Manchester students. Severe beta+ thalassaemia variants were uncommon. Betao thalassaemia variants occurred in 43 patients and resulted from 11 different variants of which the IVSII-849 A>G accounted for 25 (58%) subjects. Red cell indices in IVSII-781 C>G did not differ significantly from HbAA, and this is probably a harmless polymorphism rather than a form of beta+ thalassaemia; the removal of 6 cases in school screening had a minimal effect on the frequency of the beta thalassaemia trait. Red cell indices in the beta+ and betao thalassaemia traits followed established patterns, although both were associated with increased HbF levels. The benign nature of beta+ thalassaemia genes in Jamaica means that cases of sickle cell-beta+ thalassaemia are likely to be overlooked, and important clinical questions such as the role of pneumococcal prophylaxis remain to be answered.

The haematology of Jamaicans: red cell indices in HbAA, HbAS, HbAC, and HbA-HPFH genotypes.

Based in the parish of Manchester in central Jamaica, the Manchester Project offered free detection of haemoglobin genotype to senior classes in 15 secondary schools between 2008 and 2013. Restricting the database to 15,103 students aged 15.0-19.9 years provided an opportunity to examine the red cell characteristics of the different haemoglobin genotypes, including normal (HbAA) in 85.0%, the sickle cell trait (HbAS) in 9.7%, HbC trait (HbAC) in 3.5% and hereditary persistence of foetal haemoglobin (HbA-HPFH) in 0.4%. Compared to the normal HbAA phenotype, HbAS had significantly increased mean cell haemoglobin concentration (MCHC), red cell count (RBC), and red cell distribution width (RDW) and decreased mean cell volume (MCV) and mean cell haemoglobin (MCH), these differences being even more marked in HbAC. Compared to HbAA, the HbA-HPFH had significantly increased RDW, but there were no consistent differences in other red cell indices, and there were no significant differences in haematological indices between the two common deletion HPFH variants, HPFH-1 and HPFH-2. Although these changes are unlikely to be clinically significant, they contribute to an understanding of the haematological spectrum of the common haemoglobin genotypes in peoples of African origin.

An international multidisciplinary consensus statement on fasting before procedural sedation in adults and children.

The multidisciplinary International Committee for the Advancement of Procedural Sedation presents the first fasting and aspiration prevention recommendations specific to procedural sedation, based on an extensive review of the literature. These were developed using Delphi methodology and assessment of the robustness of the available evidence. The literature evidence is clear that fasting, as currently practiced, often substantially exceeds recommended time thresholds and has known adverse consequences, for example, irritability, dehydration and hypoglycaemia. Fasting does not guarantee an empty stomach, and there is no observed association between aspiration and compliance with common fasting guidelines. The probability of clinically important aspiration during procedural sedation is negligible. In the post-1984 literature there are no published reports of aspiration-associated mortality in children, no reports of death in healthy adults (ASA physical status 1 or 2) and just nine reported deaths in adults of ASA physical status 3 or above. Current concerns about aspiration are out of proportion to the actual risk. Given the lower observed frequency of aspiration and mortality than during general anaesthesia, and the theoretical basis for assuming a lesser risk, fasting strategies in procedural sedation can reasonably be less restrictive. We present a consensus-derived algorithm in which each patient is first risk-stratified during their pre-sedation assessment, using evidence-based factors relating to patient characteristics, comorbidities, the nature of the procedure and the nature of the anticipated sedation technique. Graded fasting precautions for liquids and solids are then recommended for elective procedures based upon this categorisation of negligible, mild or moderate aspiration risk. This consensus statement can serve as a resource to practitioners and policymakers who perform and oversee procedural sedation in patients of all ages, worldwide.

Tracking and Reporting Outcomes Of Procedural Sedation (TROOPS): Standardized Quality Improvement and Research Tools from the International Committee for the Advancement of Procedural Sedation.

Many hospitals, and medical and dental clinics and offices, routinely monitor their procedural-sedation practices-tracking adverse events, outcomes, and efficacy in order to optimize the sedation delivery and practice. Currently, there exist substantial differences between settings in the content, collection, definition, and interpretation of such sedation outcomes, with resulting widespread reporting variation. With the objective of reducing such disparities, the International Committee for the Advancement of Procedural Sedation has herein developed a multidisciplinary, consensus-based, standardized tool intended to be applicable for all types of sedation providers in all locations worldwide. This tool is amenable for inclusion in either a paper or an electronic medical record. An additional, parallel research tool is presented to promote consistency and standardized data collection for procedural-sedation investigations.

Voluntary premarital screening to prevent sickle cell disease in Jamaica: does it work?

To determine whether identifying haemoglobin genotype, and providing education and counselling to senior school students will influence their choice of partner and reduce the frequency of births with sickle cell disease. The Manchester Project provided free voluntary blood tests to determine haemoglobin genotype to the fifth and sixth forms (grades 11-13), median age of 16.7 years, of all 15 secondary schools in the parish of Manchester in south central Jamaica. A total of 16,636 students complied, and counselling was offered to carriers of abnormal genes over 6 years (2008-2013). The genotypes of their offspring were determined by newborn screening of 66,892 deliveries in 12 regional hospitals over 8 years (2008-2015). The study focused on the genotypes of live deliveries to female students with the four most common haemoglobin genotypes: 7905 with an AA genotype, 898 with the sickle cell trait, 326 with the HbC trait and 78 with the beta thalassaemia trait. A total of 2442 live deliveries were identified by the end of 2015 in mothers screened at school. Eleven babies had clinically significant genotypes, and the prevalence of SS and SC disease did not differ from that predicted by random mating. First pregnancy was not delayed in AS or AC mothers. There was no evidence that knowledge of maternal haemoglobin genotype influenced choice of partner. On an interview, mothers of affected babies correctly recalled their genotype, but either did not discuss this with their partners or the latter refused to be tested. Subjects delaying child bearing for tertiary education would be largely excluded from the present study of first pregnancies and may make greater use of this information. Future options are a greater role for prenatal diagnosis.

Pulmonary aspiration during procedural sedation: a comprehensive systematic review.

Background: Although pulmonary aspiration complicating operative general anaesthesia has been extensively studied, little is known regarding aspiration during procedural sedation. Methods: We performed a comprehensive, systematic review to identify and catalogue published instances of aspiration involving procedural sedation in patients of all ages. We sought to report descriptively the circumstances, nature, and outcomes of these events. Results: Of 1249 records identified by our search, we found 35 articles describing one or more occurrences of pulmonary aspiration during procedural sedation. Of the 292 occurrences during gastrointestinal endoscopy, there were eight deaths. Of the 34 unique occurrences for procedures other than endoscopy, there was a single death in a moribund patient, full recovery in 31, and unknown recovery status in two. We found no occurrences of aspiration in non-fasted patients receiving procedures other than endoscopy. Conclusions: This first systematic review of pulmonary aspiration during procedural sedation identified few occurrences outside of gastrointestinal endoscopy, with full recovery typical. Although diligent caution remains warranted, our data indicate that aspiration during procedural sedation appears rare, idiosyncratic, and typically benign.

Paediatric emergence delirium: a comprehensive review and interpretation of the literature.

There remain unanswered questions and implications related to emergence delirium in children. Although we know that there are some predisposing factors to emergence delirium, we still are unable to predict accurately those who are at greatest risk. Emergence delirium should be considered as a 'vital sign', which should be followed and documented in every child in the postanaesthesia recovery period. Standardized screening tools should be adopted for paediatric emergence delirium.

Feasibility of measuring memory response to increasing dexmedetomidine sedation in children.

BACKGROUND: The memory effect of dexmedetomidine has not been prospectively evaluated in children. We evaluated the feasibility of measuring memory and sedation responses in children during dexmedetomidine sedation for non-painful radiological imaging studies. Secondarily, we quantified changes in memory in relation to the onset of sedation. METHODS: A 10 min bolus of dexmedetomidine (2 mcg kg-1) was given to children as they named simple line drawings every five s. The absence of sedation was identified as any verbal response, regardless of correctness. After recovery, recognition memory was tested with correct Yes/No recognitions (50% novel pictures) and was matched to sedation responses during the bolus period (subsequent memory paradigm). RESULTS: Of 64 accruals, 30 children (mean [SD]6.1 (1.2) yr, eight male) received dexmedetomidine and completed all study tasks. Individual responses were able to be modelled successfully in the 30 children completing all the study tasks, demonstrating feasibility of this approach. Children had 50% probability of verbal response at five min 40 s after infusion start, whereas 50% probability of subsequent recognition memory occurred sooner at four min five s. CONCLUSIONS: Quantifying memory and sedation effects during dexmedetomidine infusion in verbal children was possible and demonstrated that memory function was present until shortly before verbal unresponsiveness occurred. This is the first study to investigate the effect of dexmedetomidine on memory in children. CLINICAL TRIAL REGISTRATION: NCT 02354378.

Newborn screening for sickle cell disease in Jamaica: logistics and experience with umbilical cord samples.

The study aims to describe the logistics and results of a programme for newborn screening for sickle cell disease based on samples from the umbilical cord. Samples were dried on Guthrie cards and analysed by high pressure liquid chromatography. All suspected clinically significant abnormal genotypes were confirmed by age 4-6 weeks with family studies and then recruited to local sickle cell clinics. The programme has screened 66,833 samples with the sickle cell trait in 9.8 % and the HbC trait in 3.8 %. Sickle cell syndromes occurred in 407 babies (204 SS, 148 SC, 35 Sbeta+ thalassaemia, 6 Sbetao thalassaemia, 6 sickle cell-variants, 8 sickle cell-hereditary persistence of fetal haemoglobin) and HbC syndromes in 42 (22 CC, 14 Cbeta+ thalassaemia, 1 Cbetao thalassaemia, 5 HbC- hereditary persistence of fetal haemoglobin). Focusing on the year 2015, screening was performed in 15,408, compliance with sample collection was 98.1 %, and maternal contamination occurred in 335 (2.6 %) but in only 0.05 % did diagnostic confusion require patient recall and further tests. This model of newborn screening for sickle cell disease is accurate, robust and economic. It is hoped that it may be helpful for other societies with high prevalence of abnormal haemoglobins and limited resources, who are planning to embark on newborn screening for sickle cell disease.

Dexmedetomidine: review, update, and future considerations of paediatric perioperative and periprocedural applications and limitations.

Despite lack of paediatric labelling, contributions to the literature on paediatric applications of dexmedetomidine have increased over recent years. Dexmedetomidine possesses many properties that are advantageous for a sedative and anaesthetic; it has been reported to provide sedation that parallels natural sleep, anxiolysis, analgesia, sympatholysis, and an anaesthetic-sparing effect with minimal respiratory depression. In addition, there is increasing evidence supporting its organ-protective effects against ischaemic and hypoxic injury. These favourable physiological effects combined with a limited adverse effect profile make dexmedetomidine an attractive adjunct to anaesthesia (general and regional) for a variety of procedures in paediatric operating rooms. A comprehensive understanding of the pharmacological, pharmacokinetic, and pharmacodynamic effects of dexmedetomidine is critical to maximize its safe, efficacious, and efficient paediatric perioperative applications. This review focuses on the current paediatric perioperative and periprocedural applications of dexmedetomidine and its limitations, with a consideration for the future.

Challenges in paediatric procedural sedation: political, economic, and clinical aspects.

Paediatric sedation has expanded in volume and demand over the past decade. In parallel with the increasing demand for and delivery of sedation by multi-specialty providers, conflicting political agendas have surfaced. With a limited selection of sedatives and few new sedatives to market over the past decade, some providers utilize agents that formerly were considered exclusive for administration by anaesthesiologists. This review highlights the important contributions to paediatric sedation over the past century. Considerations include the barriers and politics that impede progress and also future advances and contributions that may lie ahead.

Recent advances and contributions to procedural sedation with considerations for the future.

As the demand for sedation services have increased, the delivery of sedation has shifted from the operating room setting to areas distant to the operating room, both within and outside the hospital setting. Sedation delivery is not monopolized by any one specialty, but rather is being delivered by anesthesiologists as well as non-anesthesiologists. As the field of sedation burgeons and multi-specialists enter the realm of meeting the demands, so also have the politics increased. Unfortunately, the choice of sedative agents has not increased in parallel with the demand for sedation. Over the past decade, there has been a paucity of new introductions for procedural sedation. Rather, new formulations of approved agents are being reexamined. The safe, efficient and predictable delivery of sedation remains at the forefront of discussion and review worldwide. This review will explore the recent, significant and noteworthy contributions to the field of sedation.

Adverse event reporting tool to standardize the reporting and tracking of adverse events during procedural sedation: a consensus document from the World SIVA International Sedation Task Force.

Currently, there are no established definitions or terminology for sedation-related adverse events (AEs). With clear terminology and definitions, sedation events may be accurately identified and tracked, providing a benchmark for defining the occurrence of AEs, ranging from minimal to severe. This terminology could apply to sedation performed in any location and by any provider. We present a consensus document from the International Sedation Task Force (ISTF) of the World Society of Intravenous Anaesthesia (World SIVA). The ISTF is composed of adult and paediatric sedation practitioners from multiple disciplines throughout the world.

The changing face of homozygous sickle cell disease: 102 patients over 60 years.

Earlier reports on homozygous sickle cell (SS) disease have been biased by severely affected cases. The Jamaican clinic which seeks to avoid such bias has 102 patients surviving beyond 60 years. The objective of this study was to examine the features of elderly cases and assess factors determining survival and the behaviour of this disease with advancing age. A retrospective review of all cases and prospective assessment in survivors was conducted at The Sickle Cell Clinic at the University of the West Indies, Kingston, Jamaica previously operated by the MRC Laboratories. All patients with SS disease born prior to December 31, 1943 who would, by January 2004, have passed their 60th birthday were traced and their current status ascertained. The molecular and clinical features were assessed and observations on the clinical behaviour of the disease and of haematology and biochemistry are presented. Of the 102 patients, 58 had died, four had emigrated and 40 were alive, resident in Jamaica and aged 60-87 years. Survival was associated with female gender and higher foetal haemoglobin but not with alpha-thalassaemia or beta-globin haplotype. A tendency to familial clustering among elderly survivors did not reach statistical significance. Painful crises ameliorated with age and there was a benign course in pregnancy. Mean haemoglobin levels fell with age and were generally associated with rising creatinine levels indicating the importance of renal failure. Elderly survivors present some features of intrinsic mildness but also manifest age-related amelioration of painful crises and falling haemoglobin levels from progressive renal damage.

Sickle haemoglobin and haemoglobin Stanleyville II: possible confusion with sickle cell-haemoglobin C disease.

OBJECTIVES: To bring to the attention of East African practitioners, the characteristics of Hb Stanleyville II, its interaction with HbS, and the resemblance of the double heterozygote to sickle cell-haemoglobin C (SC) disease. DATA SOURCES: A prospective study of 100 patients with Sickle Cell (SS) disease in the steady state attending the sickle cell Clinic at Mulago Hospital, Kampala, Uganda. STUDY SELECTION: Out of 100 patients with SS disease, two were also heterozygous for an alpha chain variant identified as Hb Stanleyville II. CONCLUSIONS: In association with HbS, Hb Stanleyville II produces a hybrid haemoglobin band which on alkaline haemoglobin electrophoresis, travels in the position of HbC. Such cases may cause confusion with sickle cell-haemoglobin C (SC) disease. The index cases in both families had associated alpha thalassaemia but from this small group, no conclusions may be drawn on the haematological or clinical significance of the interaction of Hb Stanleyville II with SS disease.

Coagulation abnormalities in pediatric and adult patients after sclerotherapy or embolization of vascular anomalies.

OBJECTIVE: The purpose of our study was to examine the coagulation status in patients with vascular anomalies who had undergone sclerotherapy or embolization. SUBJECTS AND METHODS: Ours was a prospective pilot study of 29 patients who had undergone sclerotherapy or embolization of large vascular anomalies. Fibrinogen, platelet, and d-dimer levels and prothrombin time were obtained before, immediately after, and on the day after the procedure. RESULTS: Five patients with venous malformations had positive d-dimer levels before the procedure. A subgroup analysis revealed a relationship between the type of agent used and the change in coagulation status. Specifically, a positive relationship was found between the use of dehydrated alcohol or sodium tetradecyl sulfate and a disruption in coagulation profiles as evidenced by a decrease in platelets and fibrinogen, an increase in prothrombin time, and a conversion from negative to positive d-dimers. In contrast, sclerotherapy or embolization with cyanoacrylic, polyvinyl alcohol foam particles, or platinum microcoils was not associated with coagulation disturbances. CONCLUSION: The coagulation disturbances that occur in response to dehydrated alcohol or sodium tetradecyl sulfate sclerotherapy or embolization could compromise the patient's clotting ability. Patients who receive dehydrated alcohol or sodium tetradecyl sulfate during a preoperative sclerotherapy or embolization may experience coagulation disturbances that could increase the risk of bleeding, thrombosis, or hematoma. This patient population may benefit from the use of glue, foam, or coils as a substitute for dehydrated alcohol or sodium tetradecyl sulfate.

National Public Health Performance Standards: workforce development and agency effectiveness in Florida.

The Florida Department of Health (FDOH) was the first state to pilot test both the Centers for Disease Control and Prevention (CDC) state agency and local Public Health System Performance Standards. The standards were found to be complementary and supportive of the FDOH quality performance improvement system, which had been in place for a decade, and the new Quality Management initiative. The pilot test found uneven performance across the state's county health departments and identified several areas, especially workforce development, that required additional efforts. The FDOH, in collaboration with the Center for Leadership in Public Health Practice at the College of Public Health in the University of South Florida, have collaborated and will continue to collaborate to design and deliver training in critical workforce development areas.