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. While free essays can be traced by Turnitin plagiarism detection program , our custom written essays will pass any plagiarism test.
Our writing service will save you time and grade. 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. When sickle-shaped cells block small blood vessels, less blood can get to that part of the body.
Tissue that does not get a normal blood flow eventually becomes damaged. This is what causes the problems of sickle cell disease. As to this day there is really no cure for sickle cell disease.
Red blood cells take oxygen from the air we breathe into our lungs to all parts of the body. It helps red blood cells carry oxygen from the air in our lungs to all parts of the body. Normal red blood cells contain hemoglobin A.
Hemoglobin S and hemoglobin C are abnormal types of hemoglobin. Oxygen is carried in red blood cells by a substance called hemoglobin. The main hemoglobin in normal red blood cells is hemoglobin A. Normal red blood cells are soft and round and can squeeze through tiny blood tubes vessels. Normally, red blood cells live for about days before new ones replace them.
People with sickle cell conditions make a different form of hemoglobin A called hemoglobin S S stands for sickle. Red blood cells containing mostly hemoglobin S do not live as long as normal red blood cells normally about 16 days. They also become stiff, distorted in shape and have difficulty passing through the body's small blood vessels. Tissue that does not receive a normal blood flow eventually becomes damaged. This is what causes the complications of sickle cell disease.
There are several types of sickle cell disease. The most common are: Sickle Cell trait AS is an inherited condition in which both hemoglobin A and S are made in the red blood cells, there are always more A than S.
Sickle cell trait is not a type of sickle cell disease. People with sickle cell trait are generally healthy. Sickle cell conditions are inherited from parents in much the same way as blood type, hair color and texture, eye color and other physical things.
The types of hemoglobin a person makes in the red blood cells depend upon what hemoglobin genes the person inherits from his or her parents. If one parent has Sickle Cell Anemia and the other is Normal, all of the children will have sickle cell trait. When you pass an electric charge through a solution of hemoglobin, distinct hemoglobins move different distances, depending on their composition. This technique differentiates between normal hemoglobin A , Sickle hemoglobin S , and other different kinds of hemoglobin such as C, D, E,.
Medical Problems Sickle cells are destroyed rapidly in the body of people with the disease causing anemia, jaundice and the formation of gallstones. The sickle cells also block the flow of blood through vessels resulting in lung tissue damage acute chest syndrome , pain episodes arms, legs, chest and abdomen , stroke and priapism painful prolonged erection.
It also causes damage to most organs including the spleen, kidneys and liver. Damage to the spleen makes sickle cell disease patients, especially young children, easily overwhelmed by certain bacterial infections. Symptoms the patient suffers are swelling of the hands, feet and various joints. The pain occurs in any joint or organ impulsively. The amount of pain can vary from person to person.
Patients may have painful episodes or 'crises', while for others; they may not be as painful. They can be as. The pain can last for a few hours or several weeks. When the pain and swelling becomes too severe, patients are hospitalized and given intravenous fluids and painkillers. The anemia causes pallor of the skin, fatigue, and shortness of breath. The rapid breakdown of red blood cells in the body during the disease can cause yellowing of the skin and eyes.
These are symptoms of Jaundice. If there is extensive damage to the spleen, it may leave patients struggling with infections. Their bodies will have a harder time fighting off infections that the spleen would destroy. Because of its incapability, the bacteria can be fatal in the body it affects in as little as nine hours.
Pneumococcal infections were the principal causes of death in young children with sickle cell anemia. Physicians then began to give penicillin to infants that were diagnosed with sickle cell to prevent infection, and further on, sudden death. Sickle cell anemia can also be associated with blindness. The retina can deteriorate because it does not receive the proper nutrients that it needs.
The eye then cannot process visual images. Since shortages of red blood cells are associated with this disease, it also causes slow growth rate in adolescents.
Adults also usually have a small slight build. Preteens will experience delayed puberty and growth. Patients that experience severe back pain associated with acute chest syndrome use an Incentive Spirometer.
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.