Sickle Cell Disease

A fuzzy logic-based system has been applied to a number of cases in medicine especially in the area of the development of diagnostic systems and has been discovered to produce accurate results. In this paper, a fuzzy logic-based system is presented which is used to simulate a prediction model for determining the likelihood of Sickle Cell Anemia (SCA) in individuals given a 3-tuple record containing the level of fetal haemoglobin, genotype and the degree of Anemia. Knowledge was elicited from an expert at Federal Medical Centre, Owo, Ondo State, Nigeria and was used in developing the rule-base and simulated the prediction model using the MATLAB software. The results of the fuzzification and defuzzification of variables, inference engine definition and model testing was also presented and showed that the fuzzy logic based model will be very useful in the prediction of the likelihood of Sickle Cell Anemia (SCA) among Nigerian patients.

Results: We report data from the largest series so far of semen analyses in patients with sickle cell disease: 108 samples were analyzed, of which 76 were collected before treatment. We found that at least one sperm parameter was abnormal in 91% of the patients before treatment, ...

Morphine is frequently used to treat painful episodes associated with sickle cell disease (SCD) but may fail to provide adequate analgesia in many patients. This concise review focuses on unique disease related changes in physiologic variables associated with SCD that impacts pharmacokinetics and pharmacodynamics of morphine and may contribute to the variability in analgesia. Emerging evidence suggests that the allelic variants in the genes involving the opioid (UGT2B7, OPRM1, and ABCB1 genes) and nonopioid system (COMT gene) can alter the efficacy of morphine. Am. J. Hematol., 2008. © 2007 Wiley-Liss, Inc.

Why do racial and ethnic controversies become attached, as they often do, to discussions of modern genetics? How do theories about genetic difference become entangled with political debates about cultural and group differences in America? Such issues are a conspicuous part of the histories of three hereditary diseases: Tay-Sachs, commonly identified with Jewish Americans; cystic fibrosis, often labeled a "Caucasian" disease; and sickle cell disease, widely associated with African Americans.

In this captivating account, historians Keith Wailoo and Stephen Pemberton reveal how these diseases—fraught with ethnic and racial meanings for many Americans—became objects of biological fascination and crucibles of social debate. Peering behind the headlines of breakthrough treatments and coming cures, they tell a complex story: about different kinds of suffering and faith, about unequal access to the promises and perils of modern medicine, and about how Americans consume innovation and how they come to believe in, or resist, the notion of imminent medical breakthroughs.

With Tay-Sachs, cystic fibrosis, and sickle cell disease as a powerful backdrop, the authors provide a glimpse into a diverse America where racial ideologies, cultural politics, and conflicting beliefs about the power of genetics shape disparate health care expectations and experiences.

Sickle cell disorder (SCD) is a chronic illness that in England disproportionately affects marginalized ethnic groups, but has yet to feature extensively within educational or disability research. This review of existing literature makes the case for a sustained developmental research programme around SCD, disability and education. There are potentially life‐saving decisions that could be made by teachers in caring for

Sickle cell disease (SCD) is the most common genetic disorder of the blood. The disease produces significantly abnormal hemoglobin (Hgb) molecules in red blood cells (RBCs). The sickling of RBCs occurs when partially or totally deoxygenated Hgb molecules distort their normal disk shape, producing stiff, sticky, sickle-shaped cells that obstruct small blood vessels and produce vasoocclusion as well as the disruption of oxygen to body tissues. Because tissue damage can occur at multiple foci, patients with SCD are at risk for other medical complications including, but not limited to, delayed growth and sexual maturation; acute and chronic pulmonary dysfunction; stroke; aseptic necrosis of the hip, shoulders, or both; sickle cell retinopathy; dermal ulcers; and severe chronic pain. The chronicity of the illness combined with frequent hospitalizations for pain and other medical management can contribute significantly to impaired psychosocial functioning, altered intra- and interpersonal relationships, and reduced quality of life. Unlike previous qualitative reviews of SCD, this article describes the relevant clinical and research data on the relation between psychosocial functioning and SCD in adult and child populations. The authors discuss the significant role of psychosocial issues in the trajectory and management of the disease and conclude that understanding the pathophysiology of SCD without thoroughly understanding the equally important psychosocial influences is misunderstanding SCD.

The current study aims to compare positive and negative measures of psychosocial functioning among children with sickle cell disease (SCD) and their healthy siblings. Participants were 41 African-American children with SCD, 97 healthy siblings, and their primary caregivers. Primary caregivers completed self-report questionnaires assessing child behavioral problems, while children with SCD and siblings completed self-report questionnaires assessing coping, self-efficacy, and perceived social support. No significant differences were noted between children with SCD and their siblings on all measures. Both groups reported self-efficacy and perceived social support within the normative range, and endorsed significantly greater use of Positive/Approach coping. In general, both groups of children do not have clinically significant behavioral problems. However, secondary exploratory analyses identified that a greater percentage of children from both groups scored above the established clinical cutoff on the behavioral summary scores. Number of visits to the emergency room was related to behavioral problems in children with SCD. While previous reports have been mixed in their findings that children with SCD are at greater risk for psychosocial and other behavioral problems, the current report finds that children with SCD and their healthy siblings endorse positive psychosocial functioning and as a group do not have clinically significant behavioral problems. Nonetheless, ongoing psychosocial evaluation for children receiving treatment for SCD is vital.

Objective. Sickle cell disease is perhaps the most racialized condition in the history of modern medicine, yet very little research has focused on how racial perceptions influence social attitudes about the disease. Subsequently, the implications of these perceptions for public health prevention efforts and the provision of clinical care are not well known. Design. In this brief commentary, we posit that social cognitive and media framing theories provide useful approaches for assessing relations between race and social attitudes about sickle cell disease. Conclusion. Such inquiries might lead to more rigorous study of mechanisms that shape perceptions about sickle cell risk, interpersonal empathy toward patients, and public support for sickle cell-related policies.

Background:
Sickle cell disease (SCD) is one of the most prevalent genetic disorders among the African descent. SCD is associated with intermittent excruciating pain, increased morbidity and mortality yet has received less recognition in the public domain. There is growing evidence on the need to increase awareness to reduce the disease incidence. This study aimed to elicit student’s knowledge, beliefs and attitude of SCD.
Method:
A descriptive cross-sectional study design was employed. A total of 380 university students at all levels were invited to participate in the study. Of these, 350 successfully completed the study (response rate, 92.10%). A
semi structured questionnaire was used to collect information on participant’s demographic characteristics, general knowledge of SCD, beliefs and attitudes of students towards SCD.
Results:
Almost all the students were aware of SCD (98.6%) with the main source of information being school (84.6%) and the media (12.6%). Knowledge level of respondents on SCD based on scores revealed a mean score
of 9.8 ± 4.2 with 45.1%, 47.8%, and 7.1% for poor, moderate and excellent respectively. Most of the respondents strongly agreed that they feel worried (52.9%) and sympathetic (51.4%) for people affected with SCD. Participants had the belief that it is an inherited disease acquired from parents (48.3%) but not a punishment from God (76.3%). Higher level of education and knowing a relative with sickle cell trait (SCT) or SCD was significantly associated with high knowledge of SCD (p<0.05).
Conclusion:
In general, there was a limited understanding and inadequate knowledge of SCD among the students particularly on the pattern of inheritance. Results from the study highlight the need for effective public health education on SCT/SCD in trusted sources such as schools, media (radio/Television), health centers and churches. This is necessary to address misconceptions and increase knowledge level as well as understanding of the risks of having a child with SCD to influence personal reproductive options.

Sickle cell disease is an autosomal recessive disorder of blood with defective RBCs that assume sickle shape, have short lifespan leading to vascular occlusion that can affect any organ in the body. It can show a variety of oral manifestations affecting the soft tissues and teeth. Dental pulp could show aseptic necrosis without any trauma or caries or restoration of the involved tooth. In this case report, we present a case of dento-alveolar abscess formation of the left mandibular molar in a patient with sickle cell disease. It had a sudden onset, the involved molar had only enamel caries and the lesion subsided with a course of antibiotics. Our patient also had enamel hypomineralization of maxillary incisors.

Sickle cell nephropathy is a major complication of sickle cell disease. It manifests in different forms, including glomeru-lopathy, proteinuria, hematuria, and tubular defects, and frequently results in end-stage renal disease (ESRD). Different pathophysiologic mechanisms have been proposed to explain the development of nephropathy in SCD, where hemolysis and vascular occlusion are the main contributors in the manifestations of this disease. Markers of renal injury, such as proteinuria and tubular dysfunction, have been associated with outcomes among patients with sickle cell nephropathy and provide means for early detection of nephropathy and screening prior to progression to renal failure. In small-sized clinical trials, hydroxyurea has demonstrated to be effective in slowing the progression to ESRD. Dialysis and renal transplantation represent the last resort for patients with sickle cell nephropathy. Nevertheless, despite the availability of diagnostic and therapeutic strategies, sickle cell nephropathy remains a challenging and under-recognized complication for patients with sickle cell disease.

Sierra Leone is thought to be one of the West African countries most affected by sickle cell disorders. Estimates state that one in four people carry the gene for sickle cell and between 1 to 2% of births are of children with the condition. Despite this, there has been a general public health and social neglect of the condition, so that prevalence is poorly understood and policy non-existent. This participatory qualitative research project, conducted in 2018 in two districts in the country, sought to understand both women’s experiences caring for children with the condition, as well as what the needs were of women who had the condition. The findings illustrated that a historical memory of the condition and its effects exists in the intergenerational memories and practices of people. This is important to learn from to combat stigmatisation of women and people who have the condition. However, past expertise and medical knowledge currently co-exist in isolation from each other, with access to proper healthcare unavailable in most parts of the country. This has led to an inability to get a correct medical diagnosis, no advice about how to live with the condition and an inability to access specialized medical and rehabilitative services. These failures in care have led to early deaths and disablement, with the result that the general public fear the condition.  A neglect of reproductive justice and the relational implications for women with the condition was also apparent in the high number of deaths of women and infants with sickle cell. Lastly, due to the work of the voluntary and medical sector, there is increasing awareness in parents, schools and communities of how the total environment is crucial for holistic management of sickle cell conditions in a low- income country. However, it is women who are still mostly responsible for ensuring that their children access a better quality of life and women who still incur moral blame and shame for their ill-health and that of their children.

BEHAVIORAL HEALTH Behavioral: The diver&#39;s mental capacity and emotional makeup are important to safe diving. The student diver must have sufficient learning abilities to grasp information presented to him by his instructors, be able to safely plan and execute his own dives and react to changes about him in the underwater environment. The student&#39;s motivation to learn scuba and his ability to deal with potentially dangerous situations is also crucial to safe diving.

Coronaviruses are a series of viruses that constitutes the subfamily Orthocoronavirinae (scientific name). There are numerous strains of the Coronavirus, some severe and some mild. Such strains consist of  (HCoV-OC43), HKU1, (HCoV-NL63, New Haven coronavirus), HCoV-229E, MERS-CoV(aka HCoV-EMC), SARS-CoV or "SARS-classic, SARS-CoV-2(aka 2019-nCoV or COVID-19 Pandemic). The common cold is also a member of this family. The Coronavirus primarily affects the upper respiratory system & gastrointestinal tract in humans. Western conventional medicine has not found a viable vaccine for the Coronavirus, as western therapies have proven ineffective, however Asian sciences have long stood the test of time in being able to provide scientific backed research and studies on various natural medicines & clinical herbs that have both strong antibacterial & antiviral properties & of which have shown promising efficacy in combating global pandemics such as the Coronavirus when properly compounded.

It is my intent to demonstrate, via documented research of oriental medicinal herbs, the properties of Lo-FluCoron Rx® & its promising effectiveness, in conjunction with other protocols we have developed, as a complementary therapy, to both prevent & provide effective, life-saving support for infections of the upper respiratory & gastrointestinal tract, in order to combat & defeat this dangerous pandemic that has altered life as we know it around the world.

It has been 100 years since Herrick published the first medical case report of sickle cell disease. In 1949, Pauling discovered hemoglobin S (HbS). As early as the 1960-70s, emerged a coherent detailed molecular-level description of pathophysiology of sickle disease. It involved polymerization of deoxyhemoglobin S with formation of long fibers inside red blood cells (RBC) causing a distorted sickle shape and shortened lifespan. These changes constitute the basic disease process and account for hemolytic anemia and for obstructive events underlying vasoocclusive crises (VOC). However, they do not explain the mechanisms that trigger VOC. The purpose of this review is to present recent data on dehydration of sickle cell RBC, abnormalities in RBC adhesion to the vascular endothelium, the role of inflammatory events and of activation of all cells in the vessel, and abnormalities of vascular tone and carbon monoxide metabolism. These data provide new insight into the pathophysiology of the first molecular disease.

Dépistage néonatal de la Drépanocytose en France
Pertinence d'une généralisation du dépistage à l'ensemble des nouveaux-nés

A systematic newborn screening program for SCD has been established since 1985 in the French overseas territories (islands of Guadeloupe and Martinique in the French West Indies, French Guiana, and islands of La Réunion and Mayotte in the Indian Ocean). In 2000, this program was extended to continental France, but with the noticeable difference that it would be targeted to the newborns of couples "at risk”, i.e. originating from Sub-Saharan Africa, the Mediterranean Basin, the Middle East, and the Indian subcontinent or Caribbean and American couples of African descent. Large disparities are seen across the French continental territory as only 7% of the newborns are tested in Brittany but 66% in the Parisian metropolis.

Questioned on the issue of universal vs targeted screening by the Ministry of Health, the French High Health Authority (HAS) conducted a more than a year-long reflection “fed on the national and international literature, and on numerous experts' hearings (doctors, specialists of social sciences, patients associations…). On March 11, 2014, HAS issued a 130-page report the conclusion of which is: that “universal screening in continental France is not justified”.

Indeed, "ethnic” screening will remain a “French cultural exception” for still some time!

Abstract: The following is a case report of a patient suffering from sickle cell disease with ß Thalassemia. The patient has been under homeopathic treatment since 1984(30 years) during which she was followed up extensively and management of acute episodes of pains and infections was done with homeopathic approach along with the constitutional treatment. The pathogenesis of the clinical symptoms, the totality of the case, miasmatic aspect were all considered for finding out the indicated remedy at various phases of the illness. All the clinical details and investigations have been documented.