Management Of Sickle Cell Disorder Research Articles

Individualized Homeopathic and Organopathic Supportive Management of Sickle Cell Disorder: A Case Series of Six Patients from a Particularly Vulnerable Tribal Group in India.

Sickle cell disorder (SCD) is a hereditary blood disease characterized by an abnormality in the oxygen-carrying protein hemoglobin present in red blood cells. Genetic abnormality causes these cells to become sickle-shaped, with shorter lifespan. Vaso-occlusive crisis is a major symptom of SCD: it is a sudden and severe episode of pain, and occurs when sickle-shaped cells block blood flow. This blockage can lead to tissue damage, inflammation and pain. This case series aims to observe the clinical outcomes from prescribing individualized homeopathic medicines along with organopathic supportive medicine in the management of SCD through the analysis of case studies of six patients from a particularly vulnerable tribal group (PVTG) in India that manifests genetic predisposition for the disease. The patients were administered individualized homeopathic and organopathic supportive medicines, after a comprehensive door-to-door survey and subsequent screening, conducted between October 2020 and May 2023 in the Dindori and Mandla districts of the central Indian state, Madhya Pradesh. Clinical symptoms, laboratory parameters including hemoglobin, along with scores from a visual analogue scale (VAS) for pain and from the World Health Organization Quality of Life (WHOQoL) Questionnaire, were determined. Individualized homeopathic and organopathic supportive management led to improvements in clinical symptoms for all six patients. Laboratory test results showed a statistically significant increase in hemoglobin level associated with treatment. The VAS for pain indicated decreased pain frequency and severity. WHOQoL scores also improved, indicating enhanced well-being for each patient. No adverse effects were reported during treatment. This study suggests that individualized homeopathic medicine and organopathic supportive management have a beneficial role in managing SCD and may be valuable in the context of PVTGs in India. To establish a more comprehensive understanding of its efficacy, further studies should involve larger cohorts to allow for a thorough evaluation, including comparative analyses with standard therapies.

Acute and sub-acute toxicity of antisickling polyherbal extract in Wistar albino rats

Piper guineense, Gongronema latifolium and Cymbopogon citratus (PGC) serve as an effective polyherbal antisickling extract used in the management of sickle cell disorder. This present study assessed the toxicity effect of the ethanol leaf extract of PGC in rats. The acute oral toxicity test of the polyherbal was evaluated in albino rats using a single-dose based on behavioral changes and mortality. Sub-acute toxicological evaluation of PGC was examined using biochemical, hematological and histopathological methods. Biochemical analysis was carried out using liver markers enzymes, kidney markers enzymes, and lipid profiles. The hematological measurement includes white blood cell counts (WBC), Lymphocytes (LYM), monocytes (MON), granulocytes (GRAN), Red blood cell count (RBC). The organs (liver, kidney, and heart) were collected and prepared using standard protocols with hematoxylin and eosin for histopathological evaluation. The acute toxicity study of ethanol leaf extract of PGC up to the limit dose of 2000 mg/kg body weight of the animals used did not produce any signs and symptoms of toxic effects or mortality after 48 hrs. of oral administration. There were no significant differences (p < 0.001) in the observed values between the control and the treated groups for all the biochemical and hematological parameters analyzed. Histopathological evaluation of the organs demonstrated mild degeneration in the kidney and liver while the heart revealed no pathological changes in the treated group of rats. The result of acute toxicity indicates that the combined antisickling polyherbal PGC extract appeared to be safe and non- toxic. Our findings for the 28 days daily oral administration of PGC extract was dose dependent and well tolerated by the animals. Although, slight changes were observed in some biochemical parameters and histology of the kidney and liver at high doses when compared with control rats. Therefore, the consumption of the antisickling polyherbal PGC extract orally should be used encouraged at lower doses and high doses should be avoided for the management of sickle cell disorder until subjected to further cytotoxicity evaluation. 
 Keywords: Polyherbal combination, acute toxicity, subacute toxicity, biochemical parameter, hematological parameter, histopathological parameters.

Studies on the effect of a nutritious vegetable, Telfairia occidentalis, on HbSS blood

Medicinal plants have been used traditionally in Africa, especially Nigeria, in the management of sickle cell disorder (SCD) whose treatment has been mainly palliative. The antisickling properties of ethanol extract of Telfairia occidentalis Hook, F. (TO) (Family Cucurbitaceae) leaf was tested in vitro at concentrations 1, 2, 4, 8 and 16 mg/mL using inhibitory and reversal models. Nitrogen gas was used to induce hypoxia for 1 h. The effect of TO on red cell density and cell membrane were also determined. The methanol sub fraction of TO extract was subjected to GC/MS to identify some of the active compounds. The TO gave antisickling activities of 78.84 ± 1.34% inhibition and 95.4 ± 0.81% reversal, which are significantly (p < 0.05) higher than that of Ciklavit®. The TO extract gave a change in density of 17.83 ± 0.77% and a dose dependent activity on RBC membrane. Methyl 9-cis 11- trans-octadecadienoate; 1, 4- benzenedicarboxylic acid; 9, 12-octadecadienoic acid (Linoleic acid); and hexadecanoic acid methyl ester (palmitic acid); were identified from TO ethanol leaf extract for the first time using GC/MS. This study authenticated the ethnomedicinal claims of the use of T. occidentalis in the management of sickle cell disorder.

Anti-sickling activities of the stem bark of three &lt;i&gt;Khaya&lt;/i&gt; species found in Nigeria: &lt;i&gt;K. senegalensis&lt;/i&gt; A. Juss., &lt;i&gt;K. grandifoliola&lt;/i&gt;, (Welw) CDC., and &lt;i&gt;K. ivorensis&lt;/i&gt; A. Chev.

According to the World Health Organization (WHO), at least 100,000 babies die from sickle cell disorder (SCD) in Nigeria every year, making it the highest sickle-cell endemic country in Africa. WHO also stated that over 500,000 babies with severe forms of sickle cell disorder are born worldwide, with majority in low and middle income countries. There has been no cure for this disease, even after over a century of research into this disorder. Sickle cell disease is managed by the traditional medical practitioner using medicinal plants or herbs especially in the developing countries among the lower economic strata of the population and one of such herbs used is Khaya species which are generally called Mahogany in English and Oganwo by the Yoruba speaking people of Nigeria. Three species are found along the Western African region and they are Khaya senegalensis (KS), Khaya grandifoliola (KG) and Khaya ivorensis (KI). Therefore, in this study the in vitro activities of the cold and hot extracts of the stem barks of the three Khaya species used in the management of SCD were evaluated using standard anti-sickling methods of inhibitory and reversal of sickled red blood cells. Sodium metabisulphite (2%) was used to induce sickling while vanillic acid and p-hydroxyl benzoic acid were used as positive controls. The three species of Khaya (KS, KG and KI) stem barks showed antisickling activities for inhibition against sickling of red blood cell (RBC) 51.77% ± 1.47, 65.55% ± 1.65, 80.71% ± 2.93 and reversal of sickled red blood cell 44.95% ± 2.00, 43.28% ± 2.80, 41.61% ± 1.22 respectively and could be recommended for further development into herbal remedy in the management of sickle cell disease.

Anti-sickling activities of the stem bark of three &lt;i&gt;Khaya&lt;/i&gt; species found in Nigeria: &lt;i&gt;K. senegalensis&lt;/i&gt; A. Juss., &lt;i&gt;K. grandifoliola&lt;/i&gt;, (Welw) CDC., and &lt;i&gt;K. ivorensis&lt;/i&gt; A. Chev.

According to the World Health Organization (WHO), at least 100,000 babies die from sickle cell disorder (SCD) in Nigeria every year, making it the highest sickle-cell endemic country in Africa. WHO also stated that over 500,000 babies with severe forms of sickle cell disorder are born worldwide, with majority in low and middle income countries. There has been no cure for this disease, even after over a century of research into this disorder. Sickle cell disease is managed by the traditional medical practitioner using medicinal plants or herbs especially in the developing countries among the lower economic strata of the population and one of such herbs used is Khaya species which are generally called Mahogany in English and Oganwo by the Yoruba speaking people of Nigeria. Three species are found along the Western African region and they are Khaya senegalensis (KS), Khaya grandifoliola (KG) and Khaya ivorensis (KI). Therefore, in this study the in vitro activities of the cold and hot extracts of the stem barks of the three Khaya species used in the management of SCD were evaluated using standard anti-sickling methods of inhibitory and reversal of sickled red blood cells. Sodium metabisulphite (2%) was used to induce sickling while vanillic acid and p-hydroxyl benzoic acid were used as positive controls. The three species of Khaya ( KS, KG and KI) stem barks showed antisickling activities for inhibition against sickling of red blood cell (RBC) 51.77% ± 1.47, 65.55% ± 1.65, 80.71% ± 2.93 and reversal of sickled red blood cell 44.95% ± 2.00, 43.28% ± 2.80, 41.61% ± 1.22 respectively and could be recommended for further development into herbal remedy in the management of sickle cell disease.

&lt;i&gt;In vitro&lt;/i&gt; antisickling and antioxidant properties of aqueous and ethanol extracts of fifty selected plants used in the management of sickle cell disorder in southern Nigeria

Sickle Cell Disease (SCD) is an ailment with enormous social and economic burden for patients and care givers. The study evaluated the in vitro antisickling and antioxidant properties of aqueous and ethanol extracts of fifty selected plants used in the management of sickle cell disease (SCD) with the aim of justifying their use in the management of the disease in southwestern Nigeria. Aqueous and 70% ethanol extracts of fifty (50) surveyed plants were subjected to in vitro antisickling activities and forty plants with above 50% activity levels in both inhibitory and reversal models were later tested for their antioxidant assay involving four tests namely DPPH, FRAP, Fe-chelating and total antioxidant content using standard methods. Significant mean values were separated using the Least Significant Difference at 0.05 % level of probability. Among all the plants with above 50% activity levels in both inhibitory and reversal models, three plants which are Gongronema latifolium, Cymbopogon citratus and Piper guineense had the highest value of 89.81, 89.72 and 84.48 % respectively in ethanol extracts. The least activity for both aqueous and ethanol extracts was found in Amaranthus spinosus and Amaranthus viridis. It can be inferred from the result of the study that 80 % the plants evaluated possessed high antisickling and antioxidant activities and may thus justified their use for the management of SCD in the South – West, Nigeria.Keywords: Sickle cell disease, antisickling, inhibition, reversal, antioxidant

In vitro antisickling and antioxidant properties of aqueous and ethanol extracts of fifty selected plants used in the management of sickle cell disorder in southern Nigeria

Sickle Cell Disease (SCD) is an ailment with enormous social and economic burden for patients and care givers. The study evaluated the in vitro antisickling and antioxidant properties of aqueous and ethanol extracts of fifty selected plants used in the management of sickle cell disease (SCD) with the aim of justifying their use in the management of the disease in southwestern Nigeria. Aqueous and 70% ethanol extracts of fifty (50) surveyed plants were subjected to in vitro antisickling activities and forty plants with above 50% activity levels in both inhibitory and reversal models were later tested for their antioxidant assay involving four tests namely DPPH, FRAP, Fe-chelating and total antioxidant content using standard methods. Significant mean values were separated using the Least Significant Difference at 0.05 % level of probability. Among all the plants with above 50% activity levels in both inhibitory and reversal models, three plants which are Gongronema latifolium, Cymbopogon citratus and Piper guineense had the highest value of 89.81, 89.72 and 84.48 % respectively in ethanol extracts. The least activity for both aqueous and ethanol extracts was found in Amaranthus spinosus and Amaranthus viridis. It can be inferred from the result of the study that 80 % the plants evaluated possessed high antisickling and antioxidant activities and may thus justified their use for the management of SCD in the South – West, Nigeria.Keywords: Sickle cell disease, antisickling, inhibition, reversal, antioxidant

Ethnomedicinal survey of medicinal plants used in the management of sickle cell disorder in Southern Nigeria

Ethnopharmacological relevanceThe present study entails the medicinal plant species used to manage sickle cell disorder in Southern States of Nigeria. Materials and methodsThe ethnomedicinal information was gathered through multistage approach from three geopolitical zones of Southern Nigeria, which were purposively selected. Semi-structured questionnaires were administered on 500 respondents in 125 locations. The ethnomedicinal data collected were analyzed using quantitative value indices such as fidelity level (percentage) and use value. The information got was cross checked using literature search and other related materials. ResultFive hundred respondents comprising 53.12% females and 46.88% males were observed. It was noted that 26.70% were illiterate while 73.30% had formal education. Seventy-nine percent is traditional healers, 27% herb traders and the other 4% are those who have awareness of sickle cell disease . One hundred and seventy five plant species belonging to 70 families, of which Fabaceae made up 26.76% and Euphorbiaceae 16.90% forming the highest occurrence. It was observed that leaves were the most common plant part used (69.10%) followed by root (15%) and stem bark (14%) in the preparation for sickle cell management. Majority (48.57%) of these plants were harvested from wild with 38.86% being trees. Citrus aurantifolia and Newbouldia laevis had highest use values of 0.69 and 0.64 respectively. Plants with the least use value (0.001) include Abrus canescens, Acacia xanthophloea, Aerva lanata and Axonopus compressus. The result of fidelity level values of the plant species for the management of Sickle Cell Disorder (SCD) revealed that Citrus aurantifolia had the highest value of 70.2% while Angraecum distichum and Axonopus compressus had the lowest Fidelity Level value of 0.18%. ConclusionThe study revealed that people in the studied areas were well grounded in the medicinal plants used to manage sickle cell disease. This study reported for the first time 102 plant species having anti-sickling potentials with Fabaceae and Euphorbiaceae as the most dominant plant families. Many of the claimed plants were harvested from the wild showing threat thus providing needs for conservation of plants. The documented plants had high use value and fidelity level that provided quantitative and qualitative ethnomedicinal evaluation within and across the plant families. These give room for further scientific investigations in pharmacological profiles.

Antisickling and toxicological evaluation of the leaves of Scoparia dulcis Linn (Scrophulariaceae).

BackgroundScoparia dulcis Linn (Scrophulariaceae) together with other medicinal plants serve as antisickling remedies in Africa. This study was aimed at investigating the antisickling activity of the leaves of the plant as well as establishing the toxicological profile.MethodChemical tests were employed in phytochemical investigations. Evaluation of the antisickling activity involved the inhibition of sodium metabisulphite-induced sickling of the HbSS red blood cells obtained from confirmed sickle cell patients who were not in crises. Concentrations of the crude extract and its fractions were tested with normal saline and p-hydroxybenzoic acid serving as controls. Acute toxicological evaluation was carried out in mice while 30-day assessment was done in rats.ResultsPhytochemical screening revealed the presence of alkaloids, tannins, flavonoids and saponins. Percentage sickling inhibitions of the aqueous methanol extracts of S. dulcis were significant all through the period of assay p < 0. 05 compared to normal saline, but not significant with PHBA. The fractions had less activity compared to the crude extracts. The LD 50 of the extract in mice was above 8000 mg/kg body weight when administered orally. Toxicological evaluations at 250 and 500 mg/kg showed mild congestion in virtually all the target organs.ConclusionThe antisickling results confirmed traditional usage of Scoparia dulcis in the management of Sickle cell disorders and a candidate for further investigations.

English

Securinega virosa (Euphorbiaceae) together with condiments from natural sources serve as antisickling remedies in Nigeria. This study was aimed at establishing the pharmacognostic profile as well as the antisickling activity of the leaves of S. virosa Roxb. ex Willd (Euphorbiaceae). Evaluation of the fresh, powdered and anatomical sections of the leaves were carried out to determine the macromorphological, micromorphological and chemomicroscopic characters. Chemical tests were employed in phytochemical investigations. Evaluation of the antisickling activity involved the inhibition of sodium metabisulphite-induced sickling of the HbSS red blood cells obtained from confirmed sickle cell patients who were not in crises. Concentrations of the crude extract and its fractions were tested with normal saline and p-hydroxybenzoic acid serving as controls. Microscopical studies showed anomocytic stomata arrangement and glandular trichomes. Phytochemical evaluation revealed the presence of tannins, flavonoids, alkaloids, saponins and cardiac glycosides. Percentage sickling inhibitions of the aqueous methanol extract of S. virosa as well as all the fractions, except the petroleum ether fractions were significant all through the period of assay p &lt; 0. 05 compared to normal saline. These results are suggestive of a potential role for S. virosa in the management of sickle cell disorders and a candidate for further investigations. &nbsp; Key words: Securinega virosa, euphorbiaceae, pharmacognostic standardization, sickle cell disorders.&nbsp

The in vitro antisickling and antioxidant effects of aqueous extracts Zanthoxyllum heitzii on sickle cell disorder.

BackgroundSeveral plant extracts from Rutaceae family are currently used to the management of sickle cell disorder (SCD) in the African. Few reports have shown that extracts from Zanthoxyllum or Fagara genus demonstrated anti-sickling property. This study investigates the in vitro antisickling and antioxidant properties of extracts from Zanthoxyllum heitzii.MethodsThe sickling of red blood cells (RBCs) was induced using sodium metabisulfite (2%) followed by treatment with extracts at different concentrations. The osmotic fragility tests permits to explore the effect of Z. heitzii extracts on haemoglobin S solubility and sickle cells membrane stability. For each extract, qualitative phytochemical tests were used to identify the presence of alkaloids, tannins, saponins, flavonoids, glycosides and phenols, while some quantitative methods such as Folin, Ferric Reducing Antioxidant Power (FRAP) and diphenyl 1, 2 picryl hydrazyl (DPPH) were used to determine the antioxidant potential of these extracts.ResultsSodium metabisulphite increased the sickling of RBCs from 29.62 to 55.46% during 2 h. Treatment of sickling cells with extracts at different concentrations showed that a decrease of the percentage of sickling cells was found in both induced and non induced sickling cells. The fruits extract of Z. heitzii demonstrated the best anti-sickling property. The same extract at 250 μg/mL showed the best membrane cell stability compared to others. All the extracts revealed an antioxidant and anti-radical activities although lesser compared to the standard.ConclusionThe fruit extract of Z. Heitzii demonstrated the most significant antisickling effect with a potential for use in the clinical management of SCD.

Anticoagulants, Antiplatelets and Thrombolytics, 2nd Edition

Anticoagulants, Antiplatelets and Thrombolytics, 2nd Edition . Edited by Shaker A. Mousa . Published by Humana Press, a part of Springer Science+Business Media (http://www.spinger.com) , New York, Dordrecht, Heidelberg, London , 2010 . 316 pp, price £99, ISBN : 978-1-60761-802-7 This volume is number 663 of the Methods in Molecular Biology series published by Humana Press. It is largely the work of Shaker A. Mousa who edited the volume, wrote nine of the 13 chapters and was a co-author of three of the four remaining chapters. The book is 316 pages long. The book is principally about drugs that act as anticoagulants and antiplatelet agents which are sometimes referred to as antithrombotics. Actually, there is very little on thrombolytic agents. Some chapters provide information on laboratory and other approaches to obtain information on the antithrombotic actions of anticoagulants and antiplatelet agents. Others focus on clinical trials in which the effects of the agents have been tested in man. Others look to the possible future use of such agents in cancer and sickle cell disorders. The first chapter describes a range of in vitro methods for evaluating such drugs. The main focus is on approaches using blood from animals after drug administration. There is a brief resumé of mechanisms involved in platelet adhesion to subendothelial surfaces, platelet aggregation and coagulation. This is followed by a description of coagulation and platelet aggregation testing and also something on erythrocyte deformability and rigidity. The chapter concludes with an assessment of the use of a cone-and-plate viscometer and a flow chamber. Chapter 2 is the longest chapter in the book occupying some 90 pages. It describes animal models for drug evaluation and is quite comprehensive. It contains a wealth of technical detail regarding stasis models, vascular injury models for testing antithrombotics and also knock-outs for examination of the relevance of specific pathways relevant to haemostasis and thrombosis. Chapter 3 provides specific information on the clinical benefits of the anticoagulants, heparin and low-molecular weight heparins. Heparin use is not without risk and Chapter 4 focuses on heparin-induced thrombocytopenia, laboratory tests for its diagnosis, and subsequent clinical management through use of alternative therapies. Chapter 5 describes anticoagulant therapies other than heparin and moves the focus to the use of oral agents and those that do not require monitoring or dose adjustment. Following this chapter 6 focuses specifically on the oral direct Factor Xa inhibitors with special emphasis on rivaroxaban. Chapter 7 turns to antiplatelet therapies, focusing mainly on use of the P2Y12 antagonist clopidogrel and the outcomes of clinical trials in which this drug has been used with aspirin. There is brief mention of the emergence of AZD6140, a reversible P2Y12 antagonist, which is now known as ticagrelor. Chapter 8 is about clinical trials involving prasugrel, a novel P2Y12 antagonist that appears to be more effective than clopidogrel. Chapter 9 is about naturally derived products with an impact on coagulation and platelet function. Chapter 10 speculates on the potential value of anticoagulant and antiplatelet agents as assessed using animal models of experimental lung metastasis. Chapter 11 is about adhesion molecules on various cell types and approaches to interfere with their involvement in physiological and pathological processes. Chapter 12 describes the value of genetic information in improving the safety of pharmacotherapy. Chapter 13 is on the diagnosis and management of sickle cell disorders. The book concludes with a subject index. This volume is largely written by the head of a team of experimentalists and researchers in the antithrombotics area for other experimentalists and researchers. The broad subject matter provides useful information for non-clinical laboratory researchers and also clinical scientists. It is quite comprehensive as far as anticoagulants and antiplatelet agents are concerned, but, despite the title, I would be disappointed if I were hoping to obtain information on where we are in the investigation and use of thrombolytics in 2010. There are no competing interests to declare.

The lives of adults over 30 living with sickle cell disorder

The study investigated the lived experiences of adults over 30 years old living with sickle cell disorder (SCD) and sought to develop a model understanding participants' ability to function. Over a period of 10 months, in-depth individual interviews were held with nine participants and three focus groups were held with six further participants. Grounded theory, a qualitative methodology, was chosen to explore functioning across domains of experience in order to build an explanatory model. Physical, psychological, and social functioning were adversely effected by SCD. Access to and quality of resources including friends, family, and finances formed the context in which participants attempted to manage the disorder. Management of SCD improved over time as participants moved to acceptance of the condition. This allowed participants to strengthen their resilience by creating meaning, developing their identity, and actively coping with the effects of SCD, thus improving their ability to function and to appreciate life. The study demonstrates the considerable resilience of people over 30 with SCD, providing a positive and hopeful model which can be utilized clinically to support the functioning of people with SCD.

Guest Editorial&lt;BR&gt;&lt;BR&gt;From plants to medicine for management of sickle cell disorder

Guest Editorial&lt;BR&gt;&lt;BR&gt;From plants to medicine for management of sickle cell disorder

Indigenous Nigerian medicinal plants may be useful in the management of sickle‐cell disorder

Indigenous Nigerian medicinal plants may be useful in the management of sickle‐cell disorder

Anti-sickling Potential of Terminalia catappa Leaf Extract

The ethanol extract of the leaves of Terminalia catappa L. (Combretaceae) has been found to inhibit osmotically-induced hemolysis of human erythrocytes in a dose-dependent manner. It also prolonged the clotting time of uncoagulated blood. A 1.0 mg/ml solution of the extract was effective in preventing and reversing the sickling of human ‘SS’ erythrocythes induced by 2% sodium metabisulphite solution. These promising initial results indicate Terminalia catappa leaves may indeed have potential in the management of sickle cell disorders, as claimed previously.

Management of Sickle Cell Disorders

Management of Sickle Cell Disorders