Without a cure for the disease, there is much doctors can still do to help sickle-cell patients, and treatment is constantly being improved. Basic treatment relies heavily upon pain killers and oral or intravenous fluids to reduce pain. Other treatments include blood transfusion to reduce the amount of infected red blood cells, and increase the concentration of healthy red blood cells.
Doctors believe gene therapy to be the future of sickle-cell treatment. This is where a patient can go see a gene therapist and can actually stop the reproduction of infected red blood cells in their body.
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The Sickle Cell Disease Association of America offers a seven week sickle cell disease community health worker certification. The next training will begin on January 29, If you are interested you may download the application here https: For any additional questions, please contact Jessica Suggs at jsuggs sicklecelldisease.
Among them, the Society has launched an effort to develop clinical practice guidelines on the management of SCD. An inherited, lifelong chronic disorder affecting nearly , Americans, SCD is characterized by rigid, sickle-shaped red blood cells that stick to blood vessels, blocking blood flow.
Pain — both chronic and acute — is common and results in tremendous suffering and difficulty continuing daily activities like work or school.
Brain injury, organ damage, stroke, and death are all devastating complications associated with SCD. The guidelines will be based on a systematic review of available evidence and developed according to standards recommended by the National Academy of Medicine formerly the Institute of Medicine. ASH has contracted with the Mayo Clinic Evidence-Based Practice Research Center to conduct the systematic evidence reviews and to support the guideline development process. Publication of the guidelines is expected in These state-of-the-art guidelines will be produced and maintained by five panels of hematologists, other clinicians, people living with SCD, and experts in evidence-based guideline production.
The panels will address the care and management of acute and chronic complications of SCD, including pain caused by the disease, stroke prevention and treatment, and prevention and treatment of cardiopulmonary and kidney diseases. One panel has been called to address the use of blood transfusions to improve red blood cell count and mitigate complications of SCD, while another has been convened to improve the understanding of stem cell transplantation, currently the only cure for SCD.
Expert Panel Report, provides useful guidance and information for primary care, emergency, and other clinicians who may be unfamiliar with this rare disease. The ASH guidelines will provide clinical recommendations for hematologists and other specialists. The ASH guidelines will also take into account new evidence available since the publication of the NHLBI guidelines and will be continually updated to present the latest in optimal care strategies.
Last fall, ASH launched a call to action on SCD by publishing a report card that identified the areas of care most in need of improvement and announced the formation of a new Sickle Cell Disease Coalition.
The Society is in the midst of developing a consortium of sub-Saharan African countries to address newborn screening and early intervention. The consortium would introduce standard-of-care practices for screening and early intervention therapies at participating institutions, with the goal of decreasing childhood mortality rates for SCD. Additionally, ASH continues to work with partners to support legislation that will improve access to SCD care, treatments, and cures.
This month, the Society co-hosted a briefing on Capitol Hill to educate policymakers on advancements in SCD research, and has advocated for federal funding to expand surveillance of this population that will help us better understand health outcomes and health care system utilization patterns of people living with SCD.
ASH continues to increase its educational resources through the development of webinars and in-person training opportunities and will also host a broad array of sessions about SCD at the 59th ASH Annual Meeting in Atlanta. ASH is ready to tackle this effort on all fronts. For the thousands of individuals who suffer from the effects of sickle cell anemia, pain is a common part of their lives.
The inherited disease causes red blood cells of mostly people of African descent to deform into a sickle shape. This inhibits the red blood cells from carrying oxygen throughout the body, which can cause severe pain and organic damage. So, many of those suffering from sickle cell anemia are prescribed a variety of powerful pain killer derivatives.
Their desire to receive relief may be viewed through the biased lens of racism and criminalization as a result of the opioid epidemic, and deter them from receiving care for their pain. Many of these painkillers are equal in strength to oxycontin and oxycodone, which many people with sickle cell anemia have been taking since they were children. Thus, federal and state governments are seeking to pressure doctors to limit their prescriptions of such drugs to patients to curb the deadly trend.
Int J Adolesc Med Health. Methods Self-efficacy, trust and SCD knowledge were measured prospectively at baseline and months 3, 6, 9, and Adherence to clinic appointments and healthcare utilization were measured retrospectively from medical records. A repeated measures linear mixed-effect model with compound symmetry covariance structure was used to fit the data. There were no significant changes in self-efficacy, trust, hospital admissions, or blood transfusion adherence.
Lastly, SIRT1 did not change the proliferation of human erythroid progenitor cells or the expression of differentiation marker CDa. How I manage sickle cell patients with high transcranial doppler results.
Stroke is one of the most severe complications to affect children with sickle cell anaemia SCA. Transcranial doppler TCD is an accurate and non-invasive method to determine stroke risk. Randomised controlled trials have demonstrated the efficacy of chronic transfusion therapy in stroke prevention based on risk stratification determined by TCD velocities.
Significant resource allocation is necessary to facilitate training, quality assurance and failsafe arrangements for non-attenders. In a subgroup of patients, chronic transfusions for primary stroke prevention can be replaced by hydroxycarbamide therapy, provided careful monitoring is undertaken; including repeat TCD studies at frequent intervals.
The authors propose an evidence-based algorithm for the management of abnormal TCD velocities and discuss the role of this test in other clinical contexts, such as in Haemoglobin SC disease. The effect of hypnosis on pain and peripheral blood flow in sickle-cell disease: Vaso-occlusive pain crises VOCs are the "hallmark" of sickle-cell disease SCD and can lead to sympathetic nervous system dysfunction.
Hypnosis is a neuromodulatory intervention that may attenuate vascular and pain responsiveness. Due to the lack of laboratory-controlled pain studies in patients with SCD and healthy controls, the specific effects of hypnosis on acute pain-associated vascular responses are unknown. The current study assessed the effects of hypnosis on peripheral blood flow, pain threshold, tolerance, and intensity in adults with and without SCD.
Fourteen patients with SCD and 14 healthy controls were included. Participants underwent three laboratory pain tasks before and during a minute hypnosis session. Peripheral blood flow, pain threshold, tolerance, and intensity before and during hypnosis were examined. A single minute hypnosis session decreased pain intensity by a moderate amount in patients with SCD. Pain threshold and tolerance increased following hypnosis in the control group, but not in patients with SCD.
Patients with SCD exhibited lower baseline peripheral blood flow and a greater increase in blood flow following hypnosis than controls. Given that peripheral vasoconstriction plays a role in the development of VOC, current findings provide support for further laboratory and clinical investigations of the effects of cognitive-behavioral neuromodulatory interventions on pain responses and peripheral vascular flow in patients with SCD.
Current results suggest that hypnosis may increase peripheral vasodilation during both the anticipation and experience of pain in patients with SCD. These findings indicate a need for further examination of the effects of hypnosis on pain and vascular responses utilizing a randomized controlled trial design. Further evidence may help determine unique effects of hypnosis and potential benefits of integrating cognitive-behavioral neuromodulatory interventions into SCD treatment.
Disclosure The abstract of this paper was presented at the American Society of Hematology meeting as a poster with interim findings. The authors report no conflicts of interest in this work. Stigma and illness uncertainty: Persons with sickle cell disease SCD experience multiple medical and physical complications; the disease also has numerous effects on their social and emotional well-being.
We hypothesized that adults with SCD in Jamaica experience moderate levels of stigma and illness uncertainty and that these experiences may be associated with socio-demographic factors, such as gender, educational status and economic status. We surveyed adults with SCD The mean stigma score was Illness uncertainty was greater in females than in males, though not statistically significant, Stigma and uncertainty had a significant positive correlation r: In an age and sex controlled model, stigma scores were lower with higher numbers of household items coef: Illness uncertainty was higher in females coef: The study highlights socioeconomic factors to be significant to the stigma and illness uncertainty experiences in SCD.
Efforts by healthcare workers to reduce patient illness uncertainty may have additional impact, reducing their stigma. Interventions for treating intrahepatic cholestasis in people with sickle cell disease. Sickle cell disease is the most common hemoglobinopathy occurring worldwide and sickle cell intrahepatic cholestasis is a complication long recognized in this population.
Cholestatic liver diseases are characterized by impaired formation or excretion or both of bile from the liver. There is a need to assess the clinical benefits and harms of the interventions used to treat intrahepatic cholestasis in people with sickle cell disease.
This is an update of a previously published Cochrane Review. To assess the benefits and harms of the interventions for treating intrahepatic cholestasis in people with sickle cell disease. We searched the Cystic Fibrosis and Genetic Disorders Group's Haemoglobinopathies Trials Register, which comprises references identified from comprehensive electronic database searches and handsearching of relevant journals and abstract books of conference proceedings.
Each author intended to independently extract data and assess the risk of bias of the trials by standard Cochrane methodologies; however, no trials were included in the review. This updated Cochrane Review did not identify any randomised controlled trials assessing interventions for treating intrahepatic cholestasis in people with sickle cell disease.
Randomised controlled trials are needed to establish the optimum treatment for this condition. Fluid replacement therapy for acute episodes of pain in people with sickle cell disease. Okomo U1 , Meremikwu MM. Treating vaso-occlusive painful crises in people with sickle cell disease is complex and requires multiple interventions. Extra fluids are routinely given as adjunct treatment, regardless of the individual's state of hydration with the aim of slowing or stopping the sickling process and thereby alleviating pain.
To determine the optimal route, quantity and type of fluid replacement for people with sickle cell disease with acute painful crises. We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Trials Register which comprises of references identified from comprehensive electronic database searches and handsearches of relevant journals and abstract books of conference proceedings.
Date of most recent search of the Group's Haemoglobinopathies Trials Register: Randomised and quasi-randomised controlled trials that compared the administration of supplemental fluids adjunctive to analgesics by any route in people with any type of sickle cell disease during an acute painful episode, under medical supervision inpatient, day care or community.
Sixteen trials were identified by the searches, all of which were not eligible for inclusion in the review. Treating vaso-occlusive crises is complex and requires multiple interventions.
Extra fluids, generally oral or intravenous, are routinely administered during acute painful episodes to people with sickle cell disease regardless of the individual's state of hydration. Reports of their use during these acute painful episodes do not state the efficacy of any single route, type or quantity of fluid compared to another. However, there are no randomised controlled trials that have assessed the safety and efficacy of different routes, types or quantities of fluid.
This systematic review identifies the need for a multicentre randomised controlled trial assessing the efficacy and possible adverse effects of different routes, types and quantities of fluid administered to people with sickle cell disease during acute painful episodes.
Successful utilization of an electronic pain diary in a multinational phase 3 interventional study of pediatric sickle cell anemia. Patients with sickle cell anemia can experience recurrent pain episodes, which affect quality of life.
The reported prevalence of pain is higher in studies using patient diaries than in healthcare facility utilization data. Determining Effects of Platelet Inhibition on Vaso-Occlusive Events was a multinational study that assessed the efficacy and safety of prasugrel in reducing the rate of vaso-occlusive events in children with sickle cell anemia NCT and included an electronic patient-reported outcome diary to record pain occurrence.
We aimed to capture diary completion rates and compliance in children who used the electronic patient-reported outcome diary during the Determining Effects of Platelet Inhibition on Vaso-Occlusive Events study and examine factors contributing to diary completion rates and compliance.
Pain intensity was measured using the Faces Pain Scale-Revised. Data were entered by participants or caregivers and transferred wirelessly each day to a central database. Diary completion rates were the number of daily diary entries divided by the total number of expected daily diary entries.
A total of participants received a diary; provided diary data through Month 9. Diary completion rates and compliance were high throughout the collection period and across all groups and regions, despite no games being included on the device.
For subjective data, the overall completion rate was For objective data, the overall completion rate was With appropriate design, participant training, and sufficient monitoring, an electronic patient-reported outcome diary can capture daily sickle cell-related pain data in large multinational studies.
Sickle retinopathy reflects disease-related vascular injury of the eye, which can potentially result in visual loss from vitreous hemorrhage or retinal detachment. Here we review sickle retinopathy among children with sickle cell disease, describe the epidemiology, pediatric risk factors, pathophysiology, ocular findings, and treatment. Newer, more sensitive ophthalmological imaging modalities are available for retinal imaging, including ultra-widefield fluorescein angiography, spectral-domain optical coherence tomography, and optical coherence tomography angiography.
Optical coherence tomography angiography provides a noninvasive view of retinal vascular layers that could previously not be imaged and can be quantified for comparative or prospective analyses.
Ultra-widefield fluorescein angiography provides a more comprehensive view of the peripheral retina than traditional imaging techniques. In contrast, these more sensitive methods allow for more sensitive examination that includes the retina perimeter where sickle retinopathy is often first detectable. Use of these new imaging modalities may detect a higher prevalence of early sickle pathology among children than has previously been reported. Earlier detection may help in better understanding the pathogenesis of sickle retinopathy and guide future screening and treatment paradigms.
Fetal Hemoglobin in Sickle Cell Anemia: Fetal hemoglobin HbF has well-known tempering effects on the symptoms of sickle cell disease and its levels vary among patients with different haplotypes of the sickle hemoglobin gene. Compared with sickle cell anemia haplotypes found in patients of African descent, HbF levels in Saudi and Indian patients with the Arab-Indian AI haplotype exceed that in any other haplotype by nearly two-fold.
Genetic association studies have identified some loci associated with high HbF in the AI haplotype but these observations require functional confirmation. Saudi patients with the Benin haplotype have HbF levels almost twice as high as African patients with this haplotype but this difference is unexplained. We review the genetic basis of HbF regulation focusing on sickle cell anemia in Saudi Arabia and discuss new drugs that can induce increased levels of HbF.
Acute hepatitis and insidious onset were the commonest presentations. Patients with AILD were treated with standard immunosuppression. After a median follow-up of 3.
Ulcerative colitis, present in 4 patients 2 male patients, 3 with ASC was diagnosed in 2 patients before and in 2 patients after the diagnosis of AILD. AILD is not uncommon in patients with SCD, affecting mainly female patients and responding satisfactorily to immunosuppressive treatment. Liver biopsy is helpful in confirming the diagnosis and can be safely performed in the absence of acute vaso-occlusive sickling episodes.
Ulcerative colitis is common in the presence of ASC. Preclinical studies for a phase 1 clinical trial of autologous hematopoietic stem cell gene therapy for sickle cell disease. Optimization of the manufacture, characterization and testing of the transduced hematopoietic stem cell final cell product FCP , as well as an in depth in vivo toxicology study, are critical for advancing this approach to clinical trials.
In vitro and in vivo characterization of the FCP was performed, showing that all the release criteria were successfully met. Primary and secondary transplantation did not reveal any toxicity from the lentiviral vector. We present here a complete protocol, thoroughly optimized to manufacture, characterize and establish safety of a FCP for gene therapy of SCD. Published by Elsevier Inc. The meeting serves as a yearly forum for investigators and health care providers to discuss new developments in scientific and clinical aspects of SCD in an informal setting.
For more information and to register, please click here. The 6th Annual Sickle Cell Disease Conference, a forum to discover the latest advancements and future trends for sickle cell disease and drug development is scheduled for September 14, , in New York, NY. Conference attendees will hear from innovative industry leaders, patients, physicians, and clinical-stage companies.
To register for the conference, please visit www. The Annual Scientific Conference on Sickle Cell and Thalassemia is a three-day conference aimed at all those with a common interest in sickle cell disease and thalassemia. It will be held in London on October , There will also be sessions on genetics and genomic progress, curative therapies and emerging services, as well as abstract and poster presentations. For more information and to register click here.
This day conference will feature a myriad of topics designed to engage patients, families and the at-large community and to build broader awareness about the challenges of sickle cell disease and how patients and families may be able to get beyond those challenges.
Howard University President, Wayne A. For more information, visit our website www. The conference will benefit pediatricians, family practice physicians, advanced practice providers NP's, PA's , nurses, fellows, and residents. Other healthcare professionals involved in the care of pediatric patients with sickle cell disease mayfind the information useful and are welcome to attend. The purpose of this symposium is to update pediatricians and family practitioners on the most current research and clinical guidelines related to pediatric sickle cell disease, particularly in the school-aged child, and to discuss key considerations when caring for these patients.
For more information, contact ashley. This year the event will be held in Atlanta, Georgia, a city near and dear to the sickle cell community! SCiF is a two-day, intensive, educational update on sickle cell disease.
This two-day intensive educational conferences includes both clinical and scientific lectures, aimed at clinicians, academics, and other healthcare professionals involved in sickle cell disease around the world.
Sickle Cell News for June — To join or leave the listserv visit http: If approved, Endari would be the first FDA-approved treatment for pediatric patients with sickle cell disease, and the first new treatment for adult patients in almost 2 decades.
The FDA documented that while the difference was statistically different, the efficacy data was complicated by differences in discontinuation rates observed during the course of the study. While that was a concern, the FDA documented that their own exploration of the data favored L-glutamine over placebo in reducing the rates of crises.
New York Times - Patient Voices: Sickle Cell Anemia https: Here, six men and women speak about the impact sickle cell anemia has had on their lives and families. Mixing Music and Medicine: Grammy-nominated songwriter and music producer, Nana Kwabena has helped create hits for many notable artists including John Legend, Rick Ross and fellow Wondaland label mates Janelle Monae and Jidenna.
However, his musical genius and path to the entertainment industry began in one of the most unlikely places—a hospital. The year-old was diagnosed with sickle cell disease at a young age and says that he spent so much of his childhood in and out of medical facilities; it often felt like he was raised in the hospital. As a teenager, complications from the disease often meant that Kwabena was in the hospital for up to two months at a time.
He taught himself how to use the music production program Fruity Loops and started to create his own songs. After that experience Kwabena says he knew he wanted to pursue music and at the same time shed light on sickle cell disease. Biol Blood Marrow Transplant. Results of a Phase I Trial. A calcineurin inhibitor and mycophenolate mofetil were used for graft-versus-host-disease GVHD prophylaxis.
With median follow up of 2. Thus, this RIC regimen was able to achieve donor engraftment in the majority. Future efforts will focus on further reducing acute GVHD and viral infection rates. Sickle cell trait SCT is usually benign. However, there are some conditions that may lead to SCT-related problems and put athletes with the trait at particular risk.
Athletic trainers and team physicians play key roles in the policy implementation and we examined their perceptions and practices.
We used an interview guide with open-ended questions covering knowledge of SCT, historical screening and education practices, current implementation, and policy benefits and challenges. Participants were knowledgeable about SCT and thought the policy was beneficial in providing SCT health information to and for student-athletes.
Schools varied in provision of genetic counseling, offering the waiver, SCT tests administered, and other aspects. Athletic staff found the policy valuable, but felt it needs clarity and standardization.
Sensitivity of alternative measures of functioning and wellbeing for adults with sickle cell disease: Demand is growing for valid and reliable measures to systematically document these effects, particularly in adults. Statistical analyses, including analysis of variance and multiple linear regression, were conducted to determine the sensitivity of measures to SCD severity.
SCD severity was assessed via a checklist of associated treatments and conditions. The clinical implications of these results require further investigation. Increased complications of chronic erythrocytapheresis compared with manual exchange transfusions in children and adolescents with sickle cell disease.
Children and adolescents with sickle cell disease SCD are at high risk of strokes and are frequently treated with red blood cell RBC transfusions. The goal is to suppress hemoglobin Hb S while minimizing transfusion-induced iron overload. Chronic transfusion practices vary among institutions. This single-institution, retrospective cohort study compares Hb S control and therapy complication rates between MET and aRBCX in a cohort of children and adolescents with SCD and stroke during a 5-year period from through Duration and mode of transfusion therapy, achievement of Hb S suppression goal, iron burden by ferritin levels, and catheter complications were evaluated.
Thirty-seven children were included in analysis. There was no significant difference between modalities in achieving Hb S suppression or ferritin goals, but those receiving aRBCX had a greater likelihood of discontinuing chelation therapy. Transfusion therapy modalities should be compared in prospective studies for stroke prevention in children with SCD. Neuropsychological deficits, including difficulties with attention, are well described in children with sickle cell disease SCD.
This prospective, cross-sectional study included patients age, 4 to 18 y with SCD and completion of a neuropsychological evaluation between December and March ADHD medication usage rate was obtained by medical record review. Patients with sickle cell disease SCD are at risk of fatal sepsis with encapsulated bacteria, such as Streptococcus pneumoniae, because of the inherent autosplenectomy that occurs in SCD.
This risk is thwarted with oral penicillin prophylaxis during the first 5 years of life, and with stringent vaccination against S. But compared with the general African American pediatric population, the rate of invasive pneumococcal disease IPD in patients with SCD still remains high, resulting in hospitalization and fatality. Descriptive analysis of presence of risk factors for IPD, type of SCD, pneumococcal vaccination and prophylaxis status, clinical presentation, microbiological data, and the outcome of IPD was performed.
Three of the 8 isolates underwent serotype analysis 15 C in 2 and 15A in 1 , none covered with the current vaccination program. One patient had fatal outcome 15A. Breakthrough cases of IPD may involve nonvaccine isolates, and seem to occur after 5 years of age when oral penicillin prophylaxis has been terminated. A feasibility randomized controlled trial. Sickle cell disease SCD is a childhood and adult disease that primarily affects African Americans, characterized by life threatening sequelae mitigated by medications.
One-way and two-way short message service SMS medication reminders have differing efficacy in chronic diseases. Participants were randomly allocated to standard care or reminders. Participants were enrolled 28 to 60 days with a common termination date. Among the 47 study participants enrolled, Medication adherence scores improved significantly in the intervention group 3. Childhood-ACT scores improved in the intervention group Adult-ACT scores within the intervention arm were unchanged ACT scores did not improve significantly.
This study demonstrated the feasibility for two-way SMS medication reminders to improve medication adherence in a high-risk population where daily medication adherence is critical to health outcomes and quality of life.
Sickle cell disease SCD is a complex illness with many social-behavioral co-morbidities. The aim of this project was to describe unmet social-behavioral health needs for adults with SCD who presented to the emergency department for treatment of vaso-occlusive episodes VOEs.
A descriptive study using 1: We conducted interviews over 14 months. Four major themes emerged: Many patients with SCD who are treated in the emergency department have social or behavioral health risk factors. Emergency departments have an opportunity to screen and refer patients for follow-up. Future research should investigate referral outcomes and their effect on ED and hospital use. Interventions for preventing silent cerebral infarcts in people with sickle cell disease.
Sickle cell disease SCD is one of the commonest severe monogenic disorders in the world, due to the inheritance of two abnormal hemoglobin beta globin genes. SCD can cause severe pain, significant end-organ damage, pulmonary complications, and premature death.
Silent cerebral infarcts are the commonest neurological complication in children and probably adults with SCD. Silent cerebral infarcts also affect academic performance, increase cognitive deficits and may lower intelligence quotient. To assess the effectiveness of interventions to reduce or prevent silent cerebral infarcts in people with SCD. Randomised controlled trials comparing interventions to prevent silent cerebral infarcts in people with SCD. There were no restrictions by outcomes examined, language or publication status.
We included five trials children or adolescents published between and Four of the five trials were terminated early. One trial focused on preventing silent cerebral infarcts or stroke; three trials were for primary stroke prevention and one trial dealt with secondary stroke prevention. Three trials compared the use of regular long-term red blood cell transfusions to standard care. Two of these trials included children with no previous long-term transfusions: The third trial included children and adolescents on long-term transfusion.
Two trials compared the drug hydroxyurea and phlebotomy to long-term transfusions and iron chelation therapy: The quality of the evidence was moderate to very low across different outcomes according to GRADE methodology.
This was due to trials being at high risk of bias because they were unblinded; indirectness available evidence was only for children with HbSS ; and imprecise outcome estimates. Long-term red blood cell transfusions versus standard care Children with no previous long-term transfusions and higher risk of stroke abnormal TCD velocities or previous history of silent cerebral infarcts Long-term red blood cell transfusions may reduce the incidence of silent cerebral infarcts in children with abnormal TCD velocities, risk ratio RR 0.
No deaths were reported in either trial. Long-term red blood cell transfusions may reduce the incidence of: Long-term red blood cell transfusions may improve quality of life in children with previous silent cerebral infarcts difference estimate Transfusions continued versus transfusions halted: We are very uncertain whether continuing red blood cell transfusions has any effect on all-cause mortality, Peto odds ratio OR 8.
The trial did not report: Hydroxyurea and phlebotomy versus transfusions and chelation Primary prevention, children participants; one trial We are very uncertain whether switching to hydroxyurea and phlebotomy has any effect on: Secondary prevention, children and adolescents with a history of stroke participants; one trial We are very uncertain whether switching to hydroxyurea and phlebotomy has any effect on: Neither trial reported on quality of life or cognitive function.
Long-term red blood cell transfusions may reduce the incidence of silent cerebral infarcts in children with abnormal TCD velocities, but may have little or no effect on children with normal TCD velocities. In children who are at higher risk of stroke and have not had previous long-term transfusions, long-term red blood cell transfusions probably reduce the risk of stroke, and other SCD-related complications acute chest syndrome and painful crises.
In children and adolescents at high risk of stroke whose TCD velocities have normalised, continuing red blood cell transfusions may reduce the risk of silent cerebral infarcts. No treatment duration threshold has been established for stopping transfusions.
Switching to hydroxyurea with phlebotomy may increase the risk of silent cerebral infarcts and SCD-related serious adverse events in secondary stroke prevention. All other evidence in this review is of very low-quality. Potential therapeutic action of nitrite in sickle cell disease. Sickle cell disease is caused by a mutant form of hemoglobin that polymerizes under hypoxic conditions, increasing rigidity, fragility, calcium influx-mediated dehydration, and adhesivity of red blood cells.
Increased red cell fragility results in hemolysis, which reduces nitric oxide NO bioavailability, and induces platelet activation and inflammation leading to adhesion of circulating blood cells. Nitric Oxide inhibits adhesion and platelet activation.
Nitrite has emerged as an attractive therapeutic agent that targets delivery of NO activity to areas of hypoxia through bioactivation by deoxygenated red blood cell hemoglobin. In this study, we demonstrate anti-platelet activity of nitrite at doses achievable through dietary interventions with comparison to similar doses with other NO donating agents.
Unlike other NO donating agents, nitrite activity is shown to be potentiated in the presence of red blood cells in hypoxic conditions. We also show that nitrite reduces calcium associated loss of phospholipid asymmetry that is associated with increased red cell adhesion, and that red cell deformability is also improved.
We show that nitrite inhibits red cell adhesion in a microfluidic flow-channel assay after endothelial cell activation. In further investigations, we show that leukocyte and platelet adhesion is blunted in nitrite-fed wild type mice compared to control after either lipopolysaccharide- or hemolysis-induced inflammation.
Moreover, we demonstrate that nitrite treatment results in a reduction in adhesion of circulating blood cells and reduced red blood cell hemolysis in humanized transgenic sickle cell mice subjected to local hypoxia.
These data suggest that nitrite is an effective anti-platelet and anti-adhesion agent that is activated by red blood cells, with enhanced potency under physiological hypoxia and in venous blood that may be useful therapeutically. For more information- go to: Pediatric Sickle Cell Mini Symposium: Contact Rusinel Amarante rusinel. Sickle Cell News for March —April There was a lack of awareness of sickle cell and lots of stigma around the condition particularly in the high risk African and Caribbean communities most at risk of inheriting sickle cell so it was important to educate about sickle cell, testing and associated myths.
These resources now published include a good practice guide for people who commission, fund, deliver and evaluate outreach programmes, an overview of the work delivered and the research underpinning it. The resources capture the learning from years of outreach work. They include a detailed guide that explains the learning, video clips from public events and interviews with service users and people who delivered the outreach.
Resources can be accessed from: Stem cell transplant resource page: Local mom starts new career to help sons fight sickle cell disease http: Years ago, Tiffany was a school teacher. But after she became a mom, her 9 to 5 changed. The boys are now 12 and 8, trying to live normal lives with a tough disease. Tiffany also has a new career: There are so many side effects—one of them being stroke—even for kids.
But another major part of the job at Virginia Blood Services is done in labs. Researchers are always looking for rare blood that will go only to patients who desperately need it. Beth Johnson, of Virginia Blood Services, says they handle rare blood in a special way. Aside from working at Virginia Blood Services, Tiffany Dews is also continuing her work outside of the labs, within local communities.
Her mission is to get more African Americans to donate blood that could help sickle cell patients—like her two sons. NFL player Santonio Holmes knows the pain of sickle cell disease. He sees it in his year-old son, T. Holmes wants to change that. Sickle cell in the Medical Literature. Epub Feb 9. Sickle cell disease and venous thromboembolism in pregnancy and the puerperium. Noubouossie D 1 , Key NS 2.
Recent data strongly suggest an increased risk of venous thromboembolism in subjects with sickle cell disease and to a lesser extent, sickle cell trait. However, most studies have been retrospective, case-control or cross-sectional based on data obtained from administrative databases. More data from adequately powered prospective studies that include matched controls are needed to definitely establish the link between venous thromboembolism during pregnancy and sickle hemoglobin disorders.
Similarly, there remains a need for properly designed randomized control trials to establish the safety of various hormonal contraceptive methods in women with sickle cell disorders. Red blood cell transfusions are associated with HLA class I but not H-Y alloantibodies in children with sickle cell disease. Blood transfusions can induce alloantibodies to antigens on red blood cells RBCs , white blood cells and platelets, with these alloantibodies affecting transfusion and transplantation.
While transfusion-related alloimmunization against RBC antigens and human leucocyte antigens HLA have been studied, transfusion-related alloimmunization to minor histocompatibility antigens mHA , such as H-Y antigens, has not been clinically characterized. These results are consistent with immunological responder and non-responder phenotypes, wherein a subset of patients with SCD may be at higher risk for transfusion-related alloimmunization.
Adv Skin Wound Care. Case studies evaluating transdermal continuous oxygen for the treatment of chronic sickle cell ulcers. Refractory leg ulcerations are common in homozygous sickle cell anemia.
In this case series, patients were treated with transdermal continuous oxygen therapy TCOT , based on the hypothesis that oxygen deprivation caused by arteriovenous shunting may be remedied by providing oxygen directly to the wound bed.
The authors believe this is the first attempt to treat sickle cell ulcers with TCOT. The patients had recurring nonhealing wounds for 30, 21, 20, 20, and 15 years, respectively. All 5 patients healed or showed substantial improvement in the treatment periods of 3 to 36 weeks.
The authors conclude that TCOT may be a novel, effective, and inexpensive modality in treating patients with sickle cell disease ulcers.
Improvement was typically noticeable within 2 weeks. Further clinical trials may be considered to evaluate the efficacy of TCOT in sickle cell ulcers.
Depression and quality of life in children with sickle cell disease: The majority of available studies have shown that children with sickle cell disease SCD have a higher risk of depressive symptoms than those without. The present study aimed to: A total of children were included in the study, 60 group I with SCD and 60 matched, healthy control children group II. A higher level of parent support was a significantly associated with decreased depressive symptoms, demonstrated by lower CDI scores.
Better quality of life was shown by the associated higher total PedsQL 4. The present study demonstrates that higher levels of parent support were significantly associated with decreased depressive symptoms and better quality of life in children with SCD. Interventions focused on increasing parent support may be an important part of treatment for depression in children with SCD.
Pak J Med Sci. The purpose of this study was to determine the impact of mean platelet volume MPV on the frequency and severity of vaso-occlusive and cerebrovascular events in patients with sickle cell anemia SCA. The cases diagnosed with SCA were evaluated retrospectively with respect to the occurrence of painful crisis for the previous year. The incidence, severity and type of the vaso-occlusive crises of the patients with SCA between March and March were recorded.
The last MPV values in patients who were free of erythrocyte transfusion for the last three months and who had no current vaso-occlusive crises were evaluated. All the patients were grouped according to the frequency of the crises for the previous year preceding the data collection. In accordance with the results obtained during the evaluation of the cases diagnosed with sickle-cell anemia, MPV value was found to be significantly higher in patients with cerebrovascular events.
One of the contributing factors for this clinical heterogeneity may be related to the MPV values in patients with sickle cell anemia. The higher MPV values may be an early predictor of future cerebrovascular events in patients with sickle cell anemia and may require close follow-up and additional measures.
West Indian Med J. To determine the clinical factors associated with the length of hospitalization and mortality in patients with sickle cell disease SCD. Data were extracted from hospital charts and comprised demographic and clinical information, investigations, interventions, duration of stay, pathological data and outcomes.
Overall mean age was The mean length of hospitalization was The main admission diagnoses were painful crisis, acute chest syndrome, severe anaemia, sepsis, hepatic sequestration, congestive cardiac failure and renal failure. The mean value for the following laboratory investigations were: There were 40 deaths with four autopsies done. There were repeat SCD admissions. Sickle cell disease still carries a high morbidity and mortality in patients admitted to hospital.
Recurrent admissions are a concern, as they impact on patient's morbidity and quality of life. Estimated pulmonary artery systolic pressure and sickle cell disease: Many studies report estimated pulmonary artery systolic pressure ePASP in patients with sickle cell disease SCD screened by echocardiography.
To better understand the prevalence and outcomes of elevated ePASP in clinically stable SCD patients, we conducted a random-effects meta-analysis. A total of 45 studies, representing 15 countries and including individuals, met our inclusion criteria. Few studies reported 6-min walk tests or mortality outcomes, and estimates were highly heterogeneous. Reduced fitness and abnormal cardiopulmonary responses to maximal exercise testing in children and young adults with sickle cell anemia.
Physiologic contributors to reduced exercise capacity in individuals with sickle cell anemia SCA are not well understood. The objective of this study was to characterize the cardiopulmonary response to maximal cardiopulmonary exercise testing CPET and determine factors associated with reduced exercise capacity among children and young adults with SCA.
A cross-sectional cohort of 60 children and young adults mean In the largest study to date using maximal CPET in SCA, we demonstrate that children and young adults with SCA have reduced exercise capacity attributable to factors independent of anemia.
Complex derangements in gas exchange and oxygen uptake during maximal exercise are common in this population. Physiological Reports published by Wiley Periodicals, Inc.
Cochrane Database Syst Rev. Phytomedicines medicines derived from plants for sickle cell disease. Oniyangi O 1 , Cohall DH. Sickle cell disease, a common recessively inherited haemoglobin disorder, affects people from sub-Saharan Africa, the Middle East, Mediterranean basin, Indian subcontinent, Caribbean and South America. It is associated with complications and a reduced life expectancy. Phytomedicines medicine derived from plants in their original state encompass many of the plant remedies from traditional healers which the populations most affected would encounter.
There has been little systematic appraisal of their benefits. This is an update of a Cochrane Review first published in and updated in To assess the benefits and risks of phytomedicines in people with sickle cell disease of all types, of any age, in any setting.
Dates of most recent searches: Randomized or quasi-randomized trials with participants of all ages with sickle cell disease, in all settings, comparing the administration of phytomedicines, by any mode to placebo or conventional treatment, including blood transfusion and hydroxyurea.
Both authors independently assessed trial quality and extracted data. It did not affect the risk of severe complications or the level of anaemia. No serious adverse effects were reported. This needs to be further validated in future trials. More trials are required on the safety and efficacy of phytomedicines used in managing sickle cell disease.
Cognitive behavioral therapy in patients with sickle cell disease. Sickle cell disease SCD is an inherited autosomal recessive disorder. SCD is a lifelong disorder with no known cure.
SCD causes anemia, frequent painful episodes, and reduced life expectancy. The most disturbing clinical problem associated with SCD is severe pain episodes, the most common reason for hospitalization.
Pharmacological interventions have been the mainstream for treatment; however, psychological interventions such as cognitive behavioral therapy CBT may complement current medical treatment, leading to better coping and overall improved quality of life.
In a quasi-experimental one-group pretest-posttest study, 9 African American individuals with SCD completed 3 weekly educational sessions learning CBT methods. Participants demonstrated increased frequency of use of CBT methods post-intervention, including diverting attention, coping self-statements, and behavioral activities, leading to better pain control.
However, quality of life and role limitation did not show significant improvement. CBT may be beneficial to those suffering from SCD when combined with conventional treatment options; however, there are still barriers to incorporating psychological interventions into practice. CBT shows promise for individuals with chronic conditions such as SCD, but more investigation into its efficacy is needed with larger sample sizes over longer periods of time.
This analysis examined the influence of quantifiable parameters of daily sleep continuity, primarily sleep duration and sleep fragmentation, on daily pain in adults with Sickle Cell Disease SCD.
Seventy-five adults with SCD completed baseline psychosocial measures and daily morning sleep and evening pain diaries over a three-month period. Mixed-effect modeling was used to examine daily between- and within-subjects effects of sleep continuity parameters on pain, as well as the synergistic effect of sleep fragmentation and sleep duration on pain.
Results revealed nights of shorter sleep duration and time in bed, increased fragmentation, and less efficient sleep relative to one's own mean were followed by days of greater pain severity.
Further, the analgesic benefit of longer sleep duration was attenuated when sleep fragmentation was elevated. These results suggest that both the separate and combined effects of sleep duration and fragmentation should be considered in evaluating pain in adults with SCD. Subjective parameters of sleep continuity e. Additionally, sleep duration should not be considered in isolation and its association with pain may be qualified by sleep fragmentation.
Research and practice should include assessments of both when addressing pain severity. The aim of this study was to identify the factors associated with delays in treatment of sickle pain crisis in the pediatric emergency department with the goal of discerning whether earlier pain management is correlated with better clinical outcome.
This retrospective study examined data collected from clinical records of patients, aged 21 years or younger, who was treated for sickle cell pain crisis between January and June Demographic and clinical characteristics were extracted from electronic records, as well as time of registration, triage, initial pain assessment, analgesic administration, and pain reassessment. A total of sickle cell pain crises visits by 67 unique patients were identified.
Sickle Cell Anemia Research Papers Sickle cell anemia research papers examine the disease that is an inherited blood disorder. Medical health writers research the disease and give the most recent information in a custom written paper.
- Sickle Cell Anemia Sickle cell anemia is caused by a defect in the gene that controls the production of normal hemoglobin, which is an iron-containing protein in red blood cells that transports oxygen from the lungs to body tissues. The defective gene results in the production of .
Sickle Cell Anemia is a hereditary disease that changes the smallest and most important components of the body. A gene causes the bone marrow in the body to make sickled shapes, when this happens; it causes the red blood cell to die faster.4/4(1). Internet Research Paper For Sickle Cell Anemia The definition for sickle cell anemia is an abnormal, rigid, sickle shape. Sickling decreases the cells flexibility and results in a risk of various complications.
Essay The sickle cell disease is an inherited blood disorder that affects red blood cells. People with sickle cell have red blood cells that have mostly hemoglobin's, Sometimes these red blood cells become sickle-shaped or crescent shaped and have trouble going through small blood vessels. Sickle Cell Sickle Cell Disorder Research Paper Sickle cell anemia is a disease passed down through families in which red blood cells form an abnormal sickle or crescent shape.