Sample size calculation in cross-sectional studies

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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.

Opiates were the most common initial pain medication prescribed and administered. The mean time to initial analgesic administration and pain reassessment was 89 and 60 minutes, respectively.

Patients with orders for imaging studies experienced significant delays in time to initial analgesic medication and pain reassessment. In addition, higher triage pain score correlated with shorter time to first dose of pain medication. However, age, sex, and final disposition did not affect time to administration of analgesic medications.

Earlier pain management resulted in shorter ED length of stay for all patients regardless of disposition. However, earlier pain management did not affect the total length of hospitalization for patients admitted to the inpatient services.

Pediatric patients with sickle cell pain crises experienced significant delays to initial analgesic medication. The lifespan of patients with sickle-cell disease SCD continues to increase, and most affected individuals in high-resource countries now live into adulthood. This necessitates a successful transition from pediatric to adult health care.

Care for transitioning patients with SCD often falls to primary care providers who may not be fully aware of the many challenges and issues faced by patients and the current management strategies for SCD. In this review, we aim to close the knowledge gap between primary care providers and specialists who treat transitioning patients with SCD.

We describe the challenges and issues encountered by these patients, and we propose a biopsychosocial multidisciplinary approach to the management of the identified issues.

Examples of this approach, such as transition-focused integrated care models and quality improvement collaboratives, with the potential to improve health outcomes in adulthood are also described. Patients with sickle cell disease frequently experience severe pain events that lead to unplanned healthcare utilization. Mobile health tools mHealth may help prevent these events by providing remote monitoring and self-management support.

This article describes the feasibility of the Sickle cell disease Mobile Application to Record symptoms via Technology SMART , an mHealth app developed to help sickle cell disease patients monitor and manage their day-to-day symptoms. Patients continued using SMART to record clinical symptoms, pain intensity, location and perceived severity, and treatment strategies for at least 28 days.

Patient median age was 29 years range ; Patients who were over age 35 or used an iPad for the study had the highest compliance rates. This study showed that SMART is a useable and feasible method for monitoring daily pain symptoms among adolescents and adults with sickle cell disease-related pain.

How I treat acute strokes and long-term management in sickle cell disease. Neurological complications are a major cause of morbidity and mortality in sickle cell disease. Limited evidence is available to guide acute and chronic management of individuals with sickle cell disease and strokes.

Current management strategies are based primarily on single arm clinical trials and observational studies, coupled with principles of neurology and hematology. Standard therapy for secondary prevention of strokes and silent cerebral infarcts includes regular blood transfusion therapy and in selected cases, hematopoietic stem cell transplantation. A critical component of the medical care following an infarct is cognitive and physical rehabilitation.

We will discuss our strategy of acute and long-term management of strokes in sickle cell disease. Guideline on the management of acute chest syndrome in sickle cell disease.

How I treat priapism. Priapism is a disorder of persistent penile erection unrelated to sexual interest or desire. This pathologic condition, specifically the ischemic variant, is often associated with devastating complications, notably erectile dysfunction.

Because priapism demonstrates high prevalence in patients with hematological disorders, most commonly sickle cell disease SCD , there is significant concern for its sequelae in this affected population. Thus, timely diagnosis and management are critical for the prevention or at least reduction of cavernosal tissue ischemia and potential damage consequent to each episode.

Current guidelines and management strategies focus primarily on reactive treatments. However, an increasing understanding of the molecular pathophysiology of SCD-associated priapism has led to the identification of new potential therapeutic targets.

Future agents are being developed and explored for use in the prevention of priapism. Allogeneic donor availability for hematopoietic stem cell transplantation in children with sickle cell disease. Hematopoietic stem cell transplant is curative of sickle cell disease SCD but limited by donor availability. Sickle Cell Conferences and Events. Monday 15 — Tuesday 16 June The 9th Sickle Cell in Focus returns to London in This year, in addition to updating on the emerging and current clinical and management issues related to sickle cell disease, there will be a focus on new drug developments, clinical trials and new genetics.

Sickle Cell in Focus has become a internationally renowned educational update for sickle cell disease. It attracts a wide audience of clinicians, academics and other healthcare professionals involved in the disease from around the world. Online booking will be open soon. September 23 - 26, Hilton - Baltimore, MD.

While innovations in drug development improve patient health, care management plans in hospitals and academic health institutes are evolving simultaneously to allow for better patient care at reduced costs. SCD, a commonly inherited blood disorder resulting from abnormal hemoglobin, is associated with lifelong disabilities and can reduce life expectancy. As you may have heard from Dr. Swee Lay Thein, M. Over the course of her career she has made major contributions to our understanding of sickle cell phenotypes as well as pioneered new treatment strategies in clinical trials.

He received his M. Also joining this newly created Branch will be Drs. Stopping kids' silent strokes http: About , Americans have sickle cell disease -- a genetic condition where the body's red blood cells are deformed, clogging up arteries, and causing pain, disability or major stroke, even in kids.

Patients who suffer strokes often have regular blood transfusions to prevent a repeat attack. Researchers now say those transfusions can be crucial for many more young sickle cell patients, even those who are showing no outward signs of brain injury.

Alexis Haynes, 12, has come a long way. At age 6, a sudden stroke put her in a coma for a full month. Every six weeks, Alexis spends hours getting her blood transfused. New red blood cells replace her sickle-shaped ones. While Alexis' stroke was apparent, experts say one in three children with sickle cell suffer silent strokes. These kids have a higher risk of memory problems. Many have trouble at school. They're also at much higher risk for having a major stroke. Noetzel studied children age 5 and older, who had brain scans that showed evidence of silent strokes.

For three years, 99 received monthly transfusions, the rest did not. Researchers found the transfusions reduced the risk of strokes of any kind by 58 percent. Identifying kids at risk before any damage is done. Risks from transfusions include infections, reactions to donated blood and buildup of iron in the bloodstream. Researchers are planning longer-term studies to see whether transfusions, in combination with other sickle cell treatment options -- like stem cell transplantation -- can help prevent kids from losing cognitive function.

Sickle cell patient, 51, advocates screening for intending couples before marriage - See more at: If a person is born with it, steps should be taken to reduce complications resulting from. But it can be prevented as well if intending couples should go for genotype screening and counseling in order to know their genotypes before getting married to avoid having a child with sickle cell disease. This would go a long way in curbing the prevalence of sickle cell anaemia in our society.

Olajide who revealed that he was diagnosed with sickle cell anaemia in , at the age of two disclosed that Africa Sickle Cell News and World Report will make every edition of sickle cell news available online for free starting from January to strengthen sickle cell awareness in Africa and throughout the world and help reduce its spread.

During my school days I started conducting research on sickle cell, read different books to know more about the disease and that is what helped me to live up to this stage of life.

People who are at high risk of having a child with sickle cell anemia planning to have children should consider genetic counseling. A counselor can explain the risk of likelihood of having a child who has the disease. He or she also can help explain the choices that are available. Sickle erythrocytes and platelets augment lung leukotriene synthesis with downregulation of anti-inflammatory proteins: Abstract Initiation, progression, and resolution of vaso-occlusive pain episodes in sickle cell disease SCD have been recognized as reperfusion injury, which provokes an inflammatory response in the pulmonary circulation.

Some 5-lipoxygenase 5-lox metabolites are potent vasoconstrictors in the pulmonary circulation. We studied stimulation of production of the inflammatory eicosanoids leukotrienes LTs and prostaglandin E2 PGE2 by isolated rat lungs perfused with sickle HbSS erythrocytes.

Lung weight gain and blood gas data were not different among the groups. Inclusion of autologous platelets platelet-rich plasma elevated LTC4 production to The data suggest that HbSS erythrocytes and activated platelets in patient's pulmonary microcirculation will enhance the synthesis and release of the proinflammatory mediators LTC4 and PGE2, both of which may contribute to onset of the acute chest syndrome in SCD.

Sleep and Asthma Cohort. Abstract Previous studies have shown that the highest incidence of acute chest syndrome ACS in sickle cell disease SCD occurs in children less than 4 years old, and a history of ACS at this age is a risk factor for future ACS episodes. However, the interval associated with the highest risk of subsequent ACS or severe pain is not known. A total of Abstract Intravenous immunoglobulin IVIG decreases neutrophil adhesion to endothelium and red blood cell-neutrophil interactions in sickle cell mice undergoing vaso-occlusion.

There were no significant increases in neutrophil and leukocyte counts, suggesting that IVIG may more selectively inhibit Mac-1 function as opposed to neutrophil adhesion.

This study provides the first in-human validation of pre-clinical murine studies that IVIG can decrease Mac-1 function. Abstract Limited data exists regarding health care utilization HCU in patients receiving allogeneic hematopoietic cell transplantation alloHCT for sickle cell disease. Abstract Serial phlebotomy was performed on sixty children with sickle cell anaemia, stroke and transfusional iron overload randomized to hydroxycarbamide in the Stroke With Transfusions Changing to Hydroxyurea trial.

There were phlebotomy procedures with only 33 adverse events, all of which were grade 2. Abstract We developed a microfluidics-based model to quantify cell-level processes modulating the pathophysiology of sickle cell disease SCD.

This in vitro model enabled quantitative investigations of the kinetics of cell sickling, unsickling, and cell rheology. We created short-term and long-term hypoxic conditions to simulate normal and retarded transit scenarios in microvasculature.

From these measurements, we identified two severe cases of SCD that were also independently validated as severe from a genotype-based disease severity classification. These results point to the potential of this method as a diagnostic indicator of disease severity. In addition, we investigated the role of cell density in the kinetics of cell sickling. We observed an effect of HU therapy mainly in relatively dense cell populations, and that the sickled fraction increased with cell density.

These results lend support to the possibility that the microfluidic platform developed here offers a unique and quantitative approach to assess the kinetic, rheological, and hematological factors involved in vasoocclusive events associated with SCD and to develop alternative diagnostic tools for disease severity to supplement other methods.

Such insights may also lead to a better understanding of the pathogenic basis and mechanism of drug response in SCD. Abstract Hemoglobin SC disease is a very prevalent hemoglobinopathy, however very little is known specifically about this condition. There appears to be an increased risk of thromboembolic events in hemoglobin SC disease, but studies evaluating the hemostatic alterations are lacking.

We describe a cross-sectional observational study evaluating coagulation activation markers in adult hemoglobin SC patients, in comparison with sickle cell anemia patients and healthy controls. A total of 56 hemoglobin SC and 39 sickle cell anemia patients were included in the study, all in steady state, and 27 healthy controls. None of the patients were in use of hydroxyurea. Endothelial activation soluble thrombomodulin and soluble vascular cell adhesion molecule-1 , and inflammation tumor necrosis factor-alpha markers were both significantly elevated in hemoglobin SC patients when compared to controls, being as high as the levels seen in sickle cell anemia.

Overall, in hemoglobin SC patients, higher hemolytic activity and inflammation were associated with a more intense activation of coagulation, and hemostatic activation was associated with two very prevalent chronic complications seen in hemoglobin SC disease: In summary, our results demonstrate that hemoglobin SC patients present a hypercoagulable state, although this manifestation was not as intense as that seen in sickle cell anemia.

J Pediatr Hematol Oncol. Abstract Given the availability of various pain severity scales, greater understanding of the agreement between pain scales is warranted. The agreement between the 2 measures improved at increasing levels of pain severity. Blinder MA 1, Russel S 2. Cystectomy entails removal of the bladder and the prostate and diverting the urinary stream using parts of the intestine.

What is surveillance for bladder cancer? Patients diagnosed and treated for superficial bladder cancer need regular follow-up to detect recurrences and treat them effectively.

The following is a typical follow-up protocol:. Patients with bladder cancer are more likely to get upper urinary tract kidney and ureter tumors that arise from the inner lining of these organs and share a common origin with bladder tumors. The risk of upper urinary tract recurrence depends on the stage and grade of the initial disease and the response of the tumor to BCG.

Individuals with recurrent high-grade bladder tumors can have a risk of developing a tumor in the upper tracts. Commercially available tumor markers that test urine samples for evidence of bladder tumor recurrence are also being used in follow-up protocols. However, their exact role is undefined as of now, and they are not an adequate substitute for cystoscopy and cytology. What is the treatment for muscle-invasive bladder cancer? Muscle invasive bladder cancer generally requires a more aggressive treatment plan than superficial bladder cancer.

The standard and most effective treatment is the surgical removal of the bladder and diversion of the urinary stream using intestinal segments. This procedure, known as radical cystectomy, is a major operation; the doctor and patient should have a thorough discussion about the risks, complications, and benefits prior to this surgical procedure.

In short, the procedure entails removal of the bladder, prostate, seminal vesicles, and the fatty tissue around the bladder through an incision made in the abdomen. The surgery also includes removal of lymph nodes in the pelvis on both sides of the bladder to detect their involvement with the cancer. This helps in deciding further management after surgery, including the need for chemotherapy.

Patients who undergo a thorough lymph node dissection have a better chance of cure as compared to patients who either do not receive one or undergo a less extensive dissection. Radical cystectomy can be performed via open surgery, laparoscopy, or with robotic assistance. The outcomes in terms of cancer control and cure rates do not differ between these different approaches. However, the use of laparoscopic and robotic approaches significantly decreases blood loss during the surgery, decreases the need for blood transfusions, and may help in early recovery by decreasing postoperative pain at the surgical site.

An additional advantage of the robot is that it permits an enhanced magnification of the surgical field with three-dimensional vision, which helps to enhance surgical precision. It is very important to note that all these approaches can achieve comparable results in terms of cancer control in the hands of surgeons skilled and experienced in a particular modality.

So, the comfort factor and experience of an individual surgeon in a particular approach should play a major role in the patient's decision regarding selection of the approach for surgery.

The physician must divert the urine once the bladder has been removed. There are three popular ways of doing that. All of them require the use of segments of the intestine that are still connected to their blood supply but have been disconnected from the gastrointestinal tract. Radical cystectomy open, laparoscopic, or robot assisted combined with one of the three urinary diversion methods is the gold standard for the treatment of muscle invasive and selected cases of high-grade superficial bladder cancer.

Certain patients, however, may be unfit or unwilling to undergo this surgery. Segmental, or partial cystectomy is rarely done. Bladder cancer is so often multifocal in the bladder that such an approach is rarely effective. These patients can often undergo a combination of extensive TURBT, chemotherapy, and radiation in an attempt to cure or control the disease without the need to remove the urinary bladder surgically.

Radiation to the bladder can, however, lead to its own set of problems and complications, including radiation damage to the bladder and rectum that give rise to bleeding and irritative symptoms "radiation cystitis" and "radiation proctitis". What is chemotherapy for bladder cancer? Patients who are diagnosed with metastatic bladder cancer M stage - M1; cancer which has spread to other parts of the body are usually treated with chemotherapy.

This is " adjuvant chemotherapy. Chemotherapy has the potential to control metastatic bladder cancer and increase the chances of cure when used in a neoadjuvant or adjuvant setting along with surgery. However, chemotherapy has its own set of side effects that some individuals find intolerable. The time-honored chemotherapy regimen for bladder cancer is the MVAC. It is a combination of four medications given in cyclical form. Oncologists currently prescribe MVAC in a "dose dense" fashion.

This means the patient takes the drugs more frequently than was previously done in the accepted treatment schedule, as well as taking growth factors to help the blood counts to recover faster from the effects of the chemotherapy drugs. An alternative regimen is a combination of gemcitabine Gemzar and cisplatin.

Physicians use this more often nowadays since some studies have shown that it is equally effective as the MVAC regime with fewer side effects. Cisplatin, which is the main medication in all these regimens, is not given to patients who have an abnormal kidney function. In this case, doctors may substitute it with carboplatin Paraplatin , which, however, is not as effective as cisplatin-based chemotherapy.

Chemotherapy is an ever-changing method to reduce or eliminate cancer cells; it is best for patients to discuss this therapy with their doctors.

In addition, health care professionals may introduce newer compounds at any time that may be advantageous to use instead of conventional chemotherapy agents. The following is a list of compounds that some clinicians use to treat various stages of bladder cancer, usually in combination with other anti-cancer cell compounds:. A few cancer treatment centers use, in addition to chemotherapy and endoscopic resection, external radiation beam therapy to treat patients.

However, the protocol is complex with toxicity and high pretreatment mortality death rates mainly due to sepsis from the chemotherapy. External beam radiation therapy is mainly used in other countries; it is infrequently used in the United States as a primary treatment.

Its use to reduce pain from metastases of bladder cancer, especially to the bones, is still of value. Immunotherapy drugs, such as atzolizumab Tecentriq and durvalumab Imfinzi , also treat bladder cancer. These drugs block a molecule known as PD-L1 that leads to increased immune system T-cell activation and decreased tumor size; you should discuss with your doctors what individual treatments are best for your current condition.

What is the prognosis for bladder cancer? The most important factors that affect the prognosis or the chances of control and cure of bladder cancer are the stage and grade of the tumor.

The lower the stage and grade, the better the outlook. Other factors such as number, size, pattern of recurrence if any , response to initial treatment like BCG, coexistent carcinoma in situ, and certain genetic mutations also play a role.

The table below is based on the National Cancer Institute's database:. This necessitates regular follow-up, even in these low-risk tumors. However, unlike the more aggressive variants of bladder cancer, the chances of progression for example, chances of the tumor invading into the deeper layers of the bladder are minimal.

Typically, these tumors, even when they recur, do so in the same stage and grade as the original tumor and do not compromise the life expectancy of the patient. High-risk superficial tumors are those that are high-grade, T1 tumors and are associated with extensive areas of carcinoma in situ. Multiple tumors, large tumors, and those that recur despite BCG treatment are also at an increased risk for recurrence and progression. They are also much likely to invade into the deeper layers. Doctors need to manage these tumors more aggressively since they have a potential to invade and spread to other parts of the body thereby shortening the life expectancy of the patient.

After radical cystectomy, survival depends mostly on the stage of the disease. The five-year disease specific survival rate for various stages after a radical cystectomy is as follows:. Is it possible to prevent bladder cancer? The best way to prevent bladder cancer is to avoid exposure to agents that cause the disease.

People who don't smoke are three to four times less likely to get bladder cancer as compared to smokers. Continuing to smoke after the diagnosis of bladder cancer portends a poorer outcome and increases the chance of the disease coming back after treatment. Avoidance of occupational exposure to cancer-causing chemicals such as aniline dyes may also be important. Despite research in this area no medication or dietary supplement has been conclusively demonstrated to decrease the risk of bladder cancer in normal individuals.

However, recent studies of patients taking atorvastatin Lipitor to lower cholesterol have suggested the drug may lower the risk of prostatic cancer and by inference, bladder cancer, but this needs further study. Where can people find more information on bladder cancer?

A number of online resources are available for bladder cancer patients to gain more insight into this disease and its management. Bladder Cancer Advocacy Network http: What are topics of bladder cancer research? Bladder cancer is a topic of intense scientific research currently. Basic science research is focused on finding and studying the genetic alterations or changes in the human DNA that predispose to bladder cancer in the hopes to discover new medications and treatments for curing the disease.

Other areas of research include the following:. This field is likely to see significant advances in the years to come and hopefully would provide effective treatment strategies and hope for the millions of bladder cancer patients worldwide. Prostate cancer is the most common cancer in men. Learn the signs and symptoms of prostate cancer, along with causes and treatments.

Know the stages, survival rates and lower your risk of prostate cancer. Blood in the urine is termed hematuria. Hematuria, whether it be gross or microscopic, is abnormal and should be further investigated. Bladder Cancer - Symptoms Patient Comments: Bladder Cancer - Diagnosis Patient Comments: Blood in Urine Blood in the urine may or may not be accompanied by pain, but it is always abnormal and should be further investigated by a healthcare professional.

Painful blood in the urine can be caused by a number of disorders, including infections and stones in the urinary tract. Painless blood in the urine can also be due to many causes, including cancer. Blood in the urine is also referred to as hematuria. Visible blood in the urine is referred to as gross hematuria, while blood in the urine that is not visible to the naked eye is referred to as microscopic hematuria.

Readers Comments 8 Share Your Story. Prostate Cancer Slideshow Pictures. Cervical Cancer Slideshow Pictures. Readers Comments 2 Share Your Story. Readers Comments 5 Share Your Story. Readers Comments 25 Share Your Story. Readers Comments 1 Share Your Story. Campbell-Walsh Urology , 9th Edition. Post Bladder Cancer - Symptoms What symptoms did you experience with your bladder cancer? Please describe what it was like. Post View 1 Comment. Find more information about bladder cancer, the stages of bladder cancer, and available treatment options.

Bladder infection is an infection of the bladder, usually caused by bacteria or, rarely, by Candida. Certain people, including females, the elderly, men with enlarged prostates, and those with chronic medical conditions are at increased risk for bladder infection.

Bladder infections are treated with antibiotics, but cranberry products and adequate hydration may help prevent bladder infections. The urinary bladder is a muscular sac in the pelvis, just above and behind the pubic bone. See a picture of the Bladder and learn more about the health topic. Cancer is a disease caused by an abnormal growth of cells, also called malignancy. It is a group of different diseases, and is not contagious.

Cancer can be treated through chemotherapy, a treatment of drugs that destroy cancer cells. Learn the basics about cancer including types, causes, how it spreads, symptoms and signs, stages and treatment options. Read about the common type of cancers.

Experts have praised certain foods for their ability to reduce cancer risks. Learn which foods and eating strategies may help reduce your risk of developing cancer. A CT scan is an X-ray procedure that combines many X-ray images with the aid of a computer to generate cross-sectional and three-dimensional images of internal organs and structures of the body.

A CT scan is a low-risk procedure. Contrast material may be injected into a vein or the spinal fluid to enhance the scan. Chemotherapy is the treatment of cancer with drugs that can destroy cancer cells. These drugs often are called "anticancer" drugs. Chemotherapy is often used with other treatments.

Coping with side effects fatigue, nausea, vomiting, pain, hair loss, infection, diarrhea, constipation, fluid retention, mouth and throat problems are important to understand when undergoing chemotherapy treatment. It is important to eat well during chemotherapy, and get the support you need both during and after treatment. Internal bleeding occurs when an artery or vein is damaged and blood to escapes the circulatory system and collects inside the body.

Internal bleeding can be caused by a variety of situations such as blunt trauma, deceleration trauma, medications, fractures, and spontaneous bleeding. Treatment of internal bleeding depends on the cause of the bleeding. MRI or magnetic resonance imaging scan is a radiology technique which uses magnetism, radio waves, and a computer to produce images of body structures. MRI scanning is painless and does not involve X-ray radiation. Patients with heart pacemakers, metal implants, or metal chips or clips in or around the eyes cannot be scanned with MRI because of the effect of the magnet.

Lymph nodes help the body's immune system fight infections. Causes of swollen lymph nodes glands may include infection viral, bacterial, fungal, parasites. Symptoms of swollen lymph nodes vary greatly, but may include fever, night sweats, toothache, sore throat, or weight loss.

Causes of swollen lymph nodes also vary, but may include cancer, the common cold, mono, chickenox, HIV, and herpes. The treatment of swollen lymph nodes depends upon the cause. Ultrasound and ultrasonography is imaging of the body used in the medical diagnosis and screening of diseases and conditions such as: TIAs, stroke, aneurysm's, heart valve irregularities, carotid artery disease, heart disease, gallstones, kidney stones, liver disease, diseases of the female reproductive, and diseases of the male reproductive organs.

Urinalysis urine test, drug test is a test performed on a patient's urine sample to diagnose conditions and diseases such as urinary tract infection, kidney infection, kidney stones, inflammation of the kidneys, or screen for progression of conditions such as diabetes and high blood pressure.

Cancer symptoms can surprise women if they don't know what to watch out for. Learn possible clues to finding and detecting cancer early.

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