Medicinal Cannabis Use in Sickle Cell Anemia

Approximately 100,000 Americans suffer from sickle cell anemia (SCA), a severe hereditary form of anemia in which red blood cells can mutate into a sickled shape causing severe pain crises that can lead to ED visits, hospitalization, and negatively impact multiple organ systems. Pain crises greatly impact the quality of life for SCA patients. Living with SCA can be stressful and often affects patients’ mental health, causing anxiety or depression (National Heart, Lung, and Blood Institute, 2016). Opioids have been a treatment mainstay for the severe pain caused by SCA but the side effects of opioids, plus the risk of dependence, are issues that have led both patients and researchers to consider medicinal cannabis as a treatment option. While there is limited research addressing the treatment of sickle cell pain with cannabis some research does suggest that cannabis could have a beneficial effect on the management of both chronic pain and acute pain (Choo, Feldstein Ewing, & Lovejoy, 2016; Kroenke & Cheville, 2017). The aim of this study is to evaluate the association between medicinal cannabis use and quality of life for individuals with SCA. The primary goal of this pilot study is to gather a cohort of participants and administer a baseline survey that will be used in a larger study. The goal of the larger study is to assess the impact of medicinal cannabis available through Pennsylvania’s Department of Health-approved dispensaries in Philadelphia on the quality of life for individuals with sickle cell anemia (SCA)

Optimal allocation of defibrillator drones in mountainous regions

Responding to emergencies in Alpine terrain is quite challenging as air ambulances and mountain rescue services are often confronted with logistics challenges and adverse weather conditions that extend the response times required to provide life-saving support. Among other medical emergencies, sudden cardiac arrest (SCA) is the most time-sensitive event that requires the quick provision of medical treatment including cardiopulmonary resuscitation and electric shocks by automated external defibrillators (AED). An emerging technology called unmanned aerial vehicles (or drones) is regarded to support mountain rescuers in overcoming the time criticality of these emergencies by reducing the time span between SCA and early defibrillation. A drone that is equipped with a portable AED can fly from a base station to the patient's site where a bystander receives it and starts treatment. This paper considers such a response system and proposes an integer linear program to determine the optimal allocation of drone base stations in a given geographical region. In detail, the developed model follows the objectives to minimize the number of used drones and to minimize the average travel times of defibrillator drones responding to SCA patients. In an example of application, under consideration of historical helicopter response times, the authors test the developed model and demonstrate the capability of drones to speed up the delivery of AEDs to SCA patients. Results indicate that time spans between SCA and early defibrillation can be reduced by the optimal allocation of drone base stations in a given geographical region, thus increasing the survival rate of SCA patients

Direct microscopy versus sputum cytology analysis and bleach sedimentation for diagnosis of tuberculosis: a prospective diagnostic study.

ABSTRACT: BACKGROUND: Diagnostic options for pulmonary tuberculosis in resource-poor settings are commonly limited to smear microscopy. We investigated whether bleach concentration by sedimentation and sputum cytology analysis (SCA) increased the positivity rate of smear microscopy for smear-positive tuberculosis. METHODS: We did a prospective diagnostic study in a Medecins Sans Frontieres-supported hospital in Mindouli, Republic of Congo. Three sputum samples were obtained from 280 consecutive pulmonary tuberculosis suspects, and were processed according to WHO guidelines for direct smear microscopy. The remainder of each sputum sample was homogenised with 2.6% bleach, sedimented overnight, smeared, and examined blinded to the direct smear result for acid-fast bacilli (AFB). All direct smears were assessed for quality by SCA. If a patient produced fewer than three good-quality sputum samples, further samples were requested. Sediment smear examination was performed independently of SCA result on the corresponding direct smear. Positivity rates were compared using McNemar's test. RESULTS: Excluding SCA, 43.2% of all patients were diagnosed as positive on direct microscopy of up to three samples. 47.9% were diagnosed on sediment microscopy, with 48.2% being diagnosed on direct microscopy, sediment microscopy, or both. The positivity rate increased from 43.2% to 47.9% with a case definition of one positive smear ([greater than or equal to]1 AFB/100 high power fields) of three, and from 42.1% to 43.9% with two positive smears. SCA resulted in 87.9% of patients producing at least two good-quality sputum samples, with 75.7% producing three or more. Using a case definition of one positive smear, the incremental yield of bleach sedimentation was 14/121, or 11.6% (95% CI 6.5-18.6, p=0.001) and in combination with SCA was 15/121, or 12.4% (95% CI 7.1-19.6, p=0.002). Incremental yields with two positive smears were 5/118, or 4.2% (95% CI 1.4-9.6, p=0.062) and 7/118, or 5.9% (95% CI 2.4-11.8, p=0.016), respectively. CONCLUSIONS: The combination of bleach sedimentation and SCA resulted in significantly increased microscopy positivity rates with a case definition of either one or two positive smears. Implementation of bleach sedimentation led to a significant increase in the diagnosis of smear-positive patients. Implementation of SCA did not result in significantly increased diagnosis of tuberculosis, but did result in improved sample quality. Requesting extra sputum samples based on SCA results, combined with bleach sedimentation, could significantly increase the detection of smear-positive patients if routinely implemented in resource-limited settings where gold standard techniques are not available. We recommend that a pilot phase is undertaken before routine implementation to determine the impact in a particular context

Hematological Indices of Sickle Cell Anaemia Patients with Pulmonary Tuberculosis in Northern Nigeria.

Nigeria has the fourth highest prevalence of TB and the highest prevalence of Sickle cell anaemia (SCA) in the world. SCA patients have impaired immunity and are vulnerable to TB. Hence, we studied the haematological indices of SCA patients with TB in Nigeria. A total of 23 SCA patients with TB were studied in parallel with equal number of age and sex matched SCA patients without TB. SCA patients with TB had significantly lower haematocrit, higher level of circulating sickle cells (CSCs) and similar level of reticulocyte count in comparison to patients without TB. SCA patients with TB had significantly higher mean WBC count associated with higher frequency of neutrophilia in comparison to those without TB. Monocytosis and eosinopenia were exclusively found in SCA patients with TB at frequencies of 52% and 65% respectively. Lymphocyte and basophil counts were normal in all patients with and without TB. Mean platelet counts were high in both patient groups but the frequency of thrombocytosis was significantly higher in patients with TB. SCA patients with TB had significantly higher mean ESR than their counterparts without the infection. The findings of this study revealed that TB in SCA patients was associated with rising level of CSCs, falling level of haematocrit, sub-optimal reticulocytosis, neutrophilia, monocytosis, thrombocytosis, eosinopenia and rising level of ESR. Hence, SCA patients presenting with these haematological indices should be investigated for TB

Global Burden of Sickle Cell Anaemia in Children under Five, 2010-2050: Modelling Based on Demographics, Excess Mortality, and Interventions

The global burden of sickle cell anaemia (SCA) is set to rise as a consequence of improved survival in high-prevalence low- and middle-income countries and population migration to higher-income countries. The host of quantitative evidence documenting these changes has not been assembled at the global level. The purpose of this study is to estimate trends in the future number of newborns with SCA and the number of lives that could be saved in under-five children with SCA by the implementation of different levels of health interventions.First, we calculated projected numbers of newborns with SCA for each 5-y interval between 2010 and 2050 by combining estimates of national SCA frequencies with projected demographic data. We then accounted for under-five mortality (U5m) projections and tested different levels of excess mortality for children with SCA, reflecting the benefits of implementing specific health interventions for under-five patients in 2015, to assess the number of lives that could be saved with appropriate health care services. The estimated number of newborns with SCA globally will increase from 305,800 (confidence interval [CI]: 238,400-398,800) in 2010 to 404,200 (CI: 242,500-657,600) in 2050. It is likely that Nigeria (2010: 91,000 newborns with SCA [CI: 77,900-106,100]; 2050: 140,800 [CI: 95,500-200,600]) and the Democratic Republic of the Congo (2010: 39,700 [CI: 32,600-48,800]; 2050: 44,700 [CI: 27,100-70,500]) will remain the countries most in need of policies for the prevention and management of SCA. We predict a decrease in the annual number of newborns with SCA in India (2010: 44,400 [CI: 33,700-59,100]; 2050: 33,900 [CI: 15,900-64,700]). The implementation of basic health interventions (e.g., prenatal diagnosis, penicillin prophylaxis, and vaccination) for SCA in 2015, leading to significant reductions in excess mortality among under-five children with SCA, could, by 2050, prolong the lives of 5,302,900 [CI: 3,174,800-6,699,100] newborns with SCA. Similarly, large-scale universal screening could save the lives of up to 9,806,000 (CI: 6,745,800-14,232,700) newborns with SCA globally, 85% (CI: 81%-88%) of whom will be born in sub-Saharan Africa. The study findings are limited by the uncertainty in the estimates and the assumptions around mortality reductions associated with interventions.Our quantitative approach confirms that the global burden of SCA is increasing, and highlights the need to develop specific national policies for appropriate public health planning, particularly in low- and middle-income countries. Further empirical collaborative epidemiological studies are vital to assess current and future health care needs, especially in Nigeria, the Democratic Republic of the Congo, and India

Invasive bacterial infections in Gambians with sickle cell anaemia in an era of widespread Pneumococcal and Haemophilus influenzae type B vaccination

Background: There is relatively little data on the aetiology of bacterial infections in patients with sickle cell anaemia (SCA) in West Africa, and no data from countries that have implemented conjugate vaccines against both Streptococcus pneumoniae and Haemophilus influenzae type b (Hib). Methods: We conducted a retrospective analysis of SCA patients admitted to the Medical Research Council Unit, The Gambia during a five-year period when there was high coverage of Hib and Pneumococcal conjugate vaccination. We evaluated 161 admissions of 126 patients between April 2010 and April 2015. Results: Pathogenic bacteria were identified in blood cultures from 11 of the 131 admissions that had cultures taken (8.4%, 95% CI 4.5-14.1%). The most frequent isolate was Salmonella Typhimurium (6/11; 54.5%), followed by Staphylococcus aureus (2/11; 18.2%) and other enteric Gram-negative pathogens (2/11; 18.2%) and there was one case of H. influenzae non-type b bacteraemia (1/11; 9.1%). There were no episodes of bacteraemia caused by S. pneumoniae or Hib. Conclusions: The low prevalence of S. pneumoniae and Hib, and the predominance of non-typhoidal Salmonella as a cause of bacteraemia suggest the need to reconsider optimal antimicrobial prophylaxis and the empirical treatment regimens for patients with SCA

Survival Probability in Patients with Sickle Cell Anemia Using the Competitive Risk Statistical Model.

The clinical picture of patients with sickle cell anemia (SCA) is associated with several complications some of which could be fatal. The objective of this study is to analyze the causes of death and the effect of sex and age on survival of Brazilian patients with SCA. Data of patients with SCA who were seen and followed at HEMORIO for 15 years were retrospectively collected and analyzed. Statistical modeling was performed using survival analysis in the presence of competing risks estimating the covariate effects on a sub-distribution hazard function. Eight models were implemented, one for each cause of death. The cause-specific cumulative incidence function was also estimated. Males were most vulnerable for death from chronic organ damage (p = 0.0005) while females were most vulnerable for infection (p=0.03). Age was significantly associated (p ≤ 0.05) with death due to acute chest syndrome (ACS), infection, and death during crisis. The lower survival was related to death from infection, followed by death due to ACS. The independent variables age and sex were significantly associated with ACS, infection, chronic organ damage and death during crisis. These data could help Brazilian authorities strengthen public policies to protect this vulnerable population

Lésions coronaires chez le noir africain dans les syndromes coronariens aigus

Introduction: les syndromes coronariens aigus (SCA) sont en progression en Afrique sub-saharienne. Les données angio-coronarographiques sont rares. L'objectif de cette étude était d'analyser les lésions coronaires observées dans les syndromes coronariens aigus (SCA) à Abidjan. Méthodes: étude transversale, du 1er janvier 2010 au 31er décembre 2014. Tous les patients admis pour SCA et ayant bénéficié d'une coronarographie à l'Institut de cardiologie d'Abidjan pendant la période d'étude ont été inclus. Deux cent cinquante-six patients ont été sélectionnés. Nous avons analysé et comparé les lésions coronaires chez les patients ayant présenté un SCA avec sus décalage persistant du segment ST (SCA ST+) et ceux ayant présenté SCA sans sus décalage du segment ST (SCA ST-). Résultats: l'âge moyen des patients était de 53,2 ± 10,8 ans. Il existait une prédominance masculine avec un sex-ratio de 6,1. Le taux de coronarographies anormales était significativement plus élevé dans les SCA ST+ (95,4% et 64,2% respectivement, p < 0,001). Trois cent quatre lésions coronaires ont été observées dans les SCA ST+ contre 43 dans les SCA ST-. Les lésions sténosantes étaient majoritairement monotronculaires (45,3%) dans les SCA ST+ et bi ou tri tronculaires dans les SCA ST- (68,0%). Les lésions de type B1 étaient plus fréquentes dans les SCA ST- (62,8% versus 36,5%, p = 0,002). Les lésions les plus complexes de type C étaient plus fréquentes dans les SCA ST+ (17,8%), sans différence significative. La majorité des patients avait un score SYNTAX inférieur à 22 quel que soit le type de SCA (87,4% dans les SCA ST+ et 90,1% dans les SCA ST-). Conclusion: les lésions coronaires dans les SCA sont majoritairement monotronculaires dans les SCA ST+ et plus diffuses dans les SCA ST- mais avec une proportion plus importante de coronaires angiographiquement normales. La prise en charge de ces lésions relève dans la majorité des cas de l'angioplastie coronaire

Interplay between foetal haemoglobin, micronutrients and oxidative stress biomarkers in sickle cell anaemia children

Foetal haemoglobin (HbF) has been speculated to have an impact on the quantity of micronutrients and the latter also have a role to play in oxidative stress (OS) in sickle cell anaemia (SCA). No previous study in Ghana has examined the interplay of these factors together among SCA children. This study compared the levels of OS biomarkers (8-hydroxy-deoxyguanosine [8-OHdG] total antioxidant capacity [TAC]) and micronutrients (zinc and copper), and their relationship with HbF in SCA and sickle cell negative, apparently healthy children. This case-control study recruited 58 SCA (out-patients [n = 42] and in-patients [n = 16]) children aged 1–14 years as cases and 62 sickle cell negative children as controls from the Sickle Cell Unit at the Eastern Regional Hospital, Ghana. The micronutrients were measured using the atomic absorption spectrophotometer (AAS) whereas OS biomarkers and HbF were assayed using enzyme-linked immunosorbent assay (ELISA). SCA out-patients had a significantly higher level of HbF compared to HbA patients (p = 0.035). SCA in-patients had significantly increased levels of zinc, but a reduced 8-OHdG than SCA out-patients compared to control group (p \u3c 0.05). HbF correlated significantly (r = 0.318, p \u3c 0.038) with zinc in SCA out-patients. Micronutrients are essential in maintaining the redox status in SCA out-patients and HbF can influence some micronutrients

Occurrence of haemolysin antibodies among sickle cell anaemia patients within Calabar metropolis of Nigeria

The role of alpha () and beta () haemolysins in blood transfusion has been well documented. However, the occurrence of haemolysins and its attending problems in sickle cell anaemia (SCA) patients has limited appearance in the literatures especially in black Africa. This study was therefore designed to investigate the occurrence of and haemolysins in SCA patients within Calabar metropolis. A total of 50 normal controls and 54 SCA patients attending the sickle cell clinic of the University of Calabar Teaching Hospital, Cross River State of Nigeria, were screened for haemolysins and quantitated using standard techniques. The distribution of haemolysins in the SCA patients was (16.7), (11.1) and + (16.7%). Their ABO blood groups were normally distributed in this order O>>A>B>AB (61.1, 20.4, 11.1 and 7.5%, respectively). The occurrence of haemolysin among the Ogroup was 15.2%, while haemolysin was 12.1% and + haemolysin was 30.3%. Control samples gave non-significantly lower haemolysin values than in SCA patients (P>0.05), but in the same order (, 9.0, , 7.0, and +, 9.0%). The higher prevalence of haemolysins in SCA could be a major limiting factor in donation of blood, blood selection and transfusion into SCA patients. Consequently, greater care should be taken during cross match of blood for SCA patients especially in multiple transfusion procedures