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Sickle Cell Life Expectancy

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Last Updated: 04 October 2020

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Sickle Cell anemia is a disorder of blood caused by inherited abnormal hemoglobin. Abnormal hemoglobin causes distorted red blood cells. Sickled red blood cells are fragile and prone to rupture. When the number of red blood cells decreases from rupture, anemia result. This condition is referred to as Sickle Cell anemia. Irregular sickled cells can also block blood vessels, causing tissue and organ damage and pain. Sickle Cell anemia is one of the most common inherited blood anemias. The disease primarily affects Africans and African Americans. It is estimated that in the United States, some 90 000 to 100 000 Americans are afflicted with Sickle Cell anemia. Overall, current estimates are that one in 500 US African American births is affected by Sickle Cell anemia. Virtually all of the major symptoms of Sickle Cell anemia are direct result of abnormally shape, sickled red blood cells blocking the flow of blood that circulates through tissues of the body. Tissues with impaired circulation suffer damage from lack of oxygen. Damage to tissues and organs of the body can cause severe disability in patients with Sickle Cell anemia. Patients endure episodes of intermittent crises of variable frequency and severity, depending on degree of organ involvement. Major features and symptoms of Sickle Cell anemia include: fatigue and anemia, pain crises, Dactylitis and arthritis, Bacterial infections, sudden pooling of blood in spleen and liver congestion, lung and heart injury, leg ulcers, Aseptic necrosis and bone infarcts Eye damage some features of Sickle Cell anemia that can occur at any Age include: infants with Sickle Cell anemia do not develop symptoms in first few months of Life Because hemoglobin produce by developing fetus protects red Blood cells from sickling. This fetal hemoglobin is absent in red blood cells that are produced after birth, so that by 5 months of age, sickling of red blood cells is prominent and symptoms begin. Infants and younger children can suffer signs and symptoms of; fever, abdominal pain, pneumococcal Bacterial infections, painful swellings of hands and feet, and splenic sequestration. Adolescents and young adults more commonly develop: leg ulcers, Aseptic necrosis Eye damage symptoms in adults typically are intermittent pain episodes due to injury to bone, muscle, or internal organs. Sickling of red blood cells in patients with Sickle Cell anemia results in cells of abnormal shape and diminished flexibility. Sickling is promoted by conditions associated with low oxygen levels, increased acidity, or low volume of blood. These conditions can occur because of injury to the body's tissues, dehydration, or anesthesia. Certain organs are predisposed to lower oxygen levels or acidity, such as when blood moves slowly through spleen, liver, or kidney. In addition, organs with particularly high metabolism rates promote sickling by extracting more oxygen from blood. These conditions make these organs susceptible to injury from Sickle Cell anemia.

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* Please keep in mind that all text is machine-generated, we do not bear any responsibility, and you should always get advice from professionals before taking any actions

Life expectancy

Just a single point mutation in the beta globin gene leads to debilitating damage of Sickle Cell Disease. This inherited change drives complex, unrelenting conditions characterized by vaso - occlusion, chronic hemolysis, and chronic anemia. Hemoglobin S polymerization is the root cause of Sickle Cell Disease pathology and its long - term sequelae. In low oxygen environments, hemoglobin S molecules coalesce and begin to polymerize. Polymers coalesce into long fibers that distort red blood cells into characteristic Sickle shape. Hemoglobin S polymerization is a key event that leads to drastic changes in integrity and function of red blood cells. Polymers deform red Blood Cell membrane structure, making cells much more rigid and adhesive. This slows or obstructs blood flow, resulting in vaso - occlusion and diminishing oxygen delivery. Lower local oxygen levels induce further sickling, vaso - occlusion, reperfusion injuries, and inflammatory responses. Additionally, membrane changes caused by hemoglobin S polymers lead to cellular dehydration, chronic hemolysis, and early cell death. Sickled cells only survive for about 10 - 20 days versus 120 days for healthy red blood cells, which stress bone marrow and increase reticulocyte production. When red blood cells become fragile and lyse, they release hemoglobin and other cellular content that contribute to vasculopathy and further inflammation. Free hemoglobin is break down, decreasing the amount of active hemoglobin in circulation and leading to chronic anemia and its clinical complications. Left unchecked, pathologic effects of vaso - occlusion, chronic hemolysis, and chronic anemia can lead to progressive tissue damage and end - organ damage. Organs that may be affected by long - term chronic damage include: brain, eyes, lungs, heart, kidneys, and gallbladder. In summary, single nucleic acid substitution in beta globin gene causes hemoglobin S polymerization that initiates extensive pathological changes leading to vaso - occlusion, chronic hemolysis, and chronic anemia. There is an ongoing need for improved management of patients with Sickle Cell Disease and related end organ damage.

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* Please keep in mind that all text is machine-generated, we do not bear any responsibility, and you should always get advice from professionals before taking any actions

Risk factors

A recent publication aims to determine main risk factors associated with death in adults with Sickle Cell Anemia, after approval of Hydroxyurea in North America and Europe. The study followed 3 257 patients at the University of North Carolina, and also includes data from nine previous studies. Researchers identify significantly increased risk of death with each additional 10 years of age. Sickle Cell patients with Pulmonary hypertension, rare disease marked by high pressure in blood vessels of lungs, are known to be at higher risk of death. Other risk factors include high levels of enzymes creatinine or NT - proBNP, which can beA associated with kidney and heart failure, respectively. The study also found low levels of hemoglobin, fetal hemoglobin, and reticulocytes to be risk factors.

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Childhood sickle cell anemia prognosis

Sickle cell disease is a blood disorder that children are born with. It's passed down through parents ' genes. Children with SCD make abnormal types of hemoglobin. This is a protein in red blood cells that carries oxygen to all parts of the body. With SCD, body organs and tissues do get enough oxygen. Healthy red blood cells with normal hemoglobin are round and move easily through blood vessels. When a child has SCD, red blood cells are hard and sticky. They are shaped like letter C. These damage red blood cells clump together. They ca move easily through blood vessels. They get stuck in small blood vessels and block blood flow. This blockage can cause pain. It can also damage major organs. Sickle cells die sooner than healthy cells. Normally, spleen helps filter infections out of the blood. But sickle cells get stuck in this filter and die. Having fewer healthy red blood cells causes anemia. Sickle cells can also damage spleen. Without healthy spleen, children are more at risk for serious infections. There are several complex types of sickle cell gene. Some do cause symptoms or severe problems, but others do. Talk to your children's healthcare provider about the specific form of sickle cell your child has. Most children with SCD will start showing symptoms during the first year, often around 5 months. Sickle cells are present at birth. It is inherited when a child has 2 sickle cell genes, 1 from each parent. A child who has only one sickle cell gene is healthy. But he or she is carrier of disease. If two carriers have child, there is a greater chance their child will have sickle cell disease. Once parents have had a child with sickle cell disease, there is a 1 in 4 chance that another child will be born with sickle cell disease. There is also a 1 in 2 chance that child will be a carrier, like parents. Having a family history of SCD increases children's risk for disease. Scd mainly affects people whose families come from Africa, and Hispanics whose families are from the Caribbean. But genes have also been found in people whose families are from the Middle East, India, Latin America, and Mediterranean countries. It has also been found in American Indians. Most children with SCD will start to have symptoms during the first year of life, often around 5 months. Each childs symptoms may vary. They may be mild or severe. Symptoms can include: anemia. This is the most common symptom. Having fewer red blood cells causes anemia. Anemia can make children pale and tire. Yellowing of skin, eyes, and mouth. This is a common symptom. Sickle cells don't live as long as normal red blood cells. They die faster than the liver can filter them out. The yellow color is caused by a substance that is released when red blood cells die. Pain crisis, or sickle crisis.

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* Please keep in mind that all text is machine-generated, we do not bear any responsibility, and you should always get advice from professionals before taking any actions

The outlook

In JUNE OF 2012, 6 - year - old Gabby Carter reached a watershed moment in her young life. Having lived with Sickle Cell disease and its debilitating effects since the time of her first medical crisis at 10 months of age, she arrived at Transplant day. Gabby needs more than 30 blood transfusions and made 50 trips Louis Childrens Hospital from her Cape Girardeau, Mo., Home to manage her condition. Sickle Cell disease is marked by a host of symptoms, including sudden pain throughout the body. Although Gabby never suffered a stroke, many SCD patients do, and threat is always present. Transplant itself must have seemed eerily anticlimactic. Right there in her all - too - familiar hospital room, bag of what appeared to be oddly colored blood hung next to her, and, under the direction of Shalini Shenoy, MBBS, MD, its contents flowed into Gabby's veins. No operating room, nary scalpel, and the next day, Gabby was up and moving around. If all goes as plan, these donated cord blood stem cells would find their way to Gabbys Bone Marrow and begin producing normal, oxygen - carrying red blood cells, replacing her defective Sickle cells. Although the procedure was less than dramatic, Gabby pre - conditioning to receive it was finely tune. And 18 months post - transplant, have been closely monitoring tightrope walk, according to Shenoy. Today, Gabbys Sickle Cell disease is go cured. Now, her mom, Debbie, says Gabby is a vibrant 7 - year - old who plays in snow, goes swimming and attends second grade with her classmates. About 100 000 Americans live with SCD. It is the most common inherited blood disorder in the US. Sickle Cell affects those with African, Spanish, Mediterranean and Indian ancestry. In the US, about one in 500 African Americans and one in 1 200 Hispanic Americans are born with SCD. Scd occurs when a child inherits two Sickle hemoglobin genes, one from each parent. People are born with Sickle Cell;s It doesnt develop later in life and it is not contagious. Dysfunctional genes produce red blood cells with a distinctive Sickle shape, jag edges and brittle structure. These cells clump together, and intense pain results as blocked and damaged arteries starve organs and systems OF oxygen. Anemia is No. 1 symptom. Scd is a chronic illness, but with new therapies, many people are living MORE productive lives. Life expectancy has increased from 14 years to 50 and older. Gabby is among the earliest Sickle Cell patients in the country to be cured via umbilical cord blood transplant from an unrelated donor using a reduce - intensity conditioning procedure refined at Washington University School OF Medicine. The novel procedure is remarkable for two primary reasons: it is much gentler on patients than earlier procedures; and it dramatically expands the extremely limited donor pool. A Renowned leader in Pediatric stem Cell transplantation, Shenoy is Teresa J. Vietti, MD Scholar in Pediatrics and directs the Pediatric Bone Marrow Transplant Program at Louis Childrens Hospital and Alvin J. Siteman Cancer Center at Barnes - Jewish Hospital and Washington University School OF Medicine.

* Please keep in mind that all text is machine-generated, we do not bear any responsibility, and you should always get advice from professionals before taking any actions.

* Please keep in mind that all text is machine-generated, we do not bear any responsibility, and you should always get advice from professionals before taking any actions

What is sickle cell anemia?

Publish case study reports that patients with mildly symptomatic sickle cell disease can exceed the US median life expectancy of 47 years for patients with disease if it is managed properly. The Report published in Blood, Journal of American Society of Hematolog y, Case series of octogenarians with sickle cell disease, analyzed four women diagnosed with milder forms of SCD who have lived as long as 86 years. For those with mild forms of SCD, these women show that lifestyle modifications may improve disease outcomes, state Samir K. Ballas, MD, professor emeritus in the Department of Medicine at Sidney Kimmel Medical College at Thomas Jefferson University in Philadelphia, and principal author of Case study. Ballas goes on to explainA that strong and long - term familyA support are important factors for reporting long life expectancy and high quality of life of SCD patients. Moreover, strict adherence to medication and appointments has also been reported as being highly important for disease outcome. It is very likely that their healthy lifestyles were important contributors to their longevity, say Ballas. All of the women were non - smokers who consumed little to no alcohol and maintained a normal body mass index. This was a couple with strong compliance to their treatment regimens and excellent family support at home, he say. Four cases studied in this report were considered by Ballas to beA desirable disease states. These women never had stroke, never had recurrent acute chest syndrome, had relatively high fetal hemoglobin count, and had infrequent painful crises. Patients like this usually but not always experience relatively mild SCD, and they live longer with a better quality of life, Ballas say. Nevertheless, Ballas points out that this study included only four participants and all were women. Give so, more studies with broader groups of studies are still require. In summary, Ballas is hopeful that the stories of these four women can serve as examples for SCD patients. I would often come out to the waiting room and find these ladies talking with other SCD patients, and I could tell that they give others hope, that just because they have SCD does not mean that they are doomed to die in their 40s that if they take care of themselves, and live closely with those who can help keep them well, that there is hope for them to lead long, full lives, author conclude.

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* Please keep in mind that all text is machine-generated, we do not bear any responsibility, and you should always get advice from professionals before taking any actions

What affects someones prognosis?

Many things play a role in determining how long someone with SCA will live. But experts have identified a few concrete factors, particularly in children, that can contribute to poorer prognosis: having hand - foot syndrome, which is painful swelling in hands and feet, before the age of 1, having hemoglobin level thats less than 7 grams per deciliter, having high white blood cell count without any underlying infection access to nearby, affordable healthcare can also play role. Regularly following up with doctor helps identify any complications that might need treatment or concerning symptoms. But if you live in rural area or dont have health insurance, this is easier to say than do. Find low - cost health clinics in your area here. If you ca find one in your area, call the clinic that is closest to you, and ask them about any rural healthcare resources in your state. Sickle Cell Society and Sickle Cell Disease Coalition also offer helpful resources for learning more about conditions and finding medical care.

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* Please keep in mind that all text is machine-generated, we do not bear any responsibility, and you should always get advice from professionals before taking any actions

A Health Policy Approach

The shortage of long - term follow - up programs, registries, or Data Collection systems has limited understanding of SCD. To address this gap in knowledge, in 2015, CDC implemented the Sickle Cell Data Collection Program to address the need for this Public Health approach to improving health outcomes. Using state - base Surveillance systems, program provides important population - level data about disease course and impact of interventions, health care use, and premature death and identifies providers and sites of care. Understanding the onset and progression of complications helps when planning strategies for prevention, early detection, and intervention. Four main objectives of the Sickle Cell Data Collection Program are to 1 establish the health profile of the SCD population in the United States; 2 to track changes in SCD populations outcomes over time; 3 to ensure that the SCD community has credible, scientifically sound information to inform standards of care; and 4 inform policy and Health care changes. By achieving these goals, program could improve the quality of life, life expectancy, and health of people living with SCD. Without data and mechanisms to track and understand SCD care and outcomes, evidence of what works and where improvements could be made is limited. These two current efforts, Get connect and Sickle Cell Data Collection Program, along with adequate resources and support, have the potential to provide an evidence base to inform health care policies and improve the lives of people living with SCD.

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* Please keep in mind that all text is machine-generated, we do not bear any responsibility, and you should always get advice from professionals before taking any actions

A Community Approach

The rhythmic whoosh - whoosh - whoosh of blood coursing through Kaimora Smiths brain sounds bit like ocean on a Doppler scan. A Hillcrest Middle School student lay on a gurney as Dr. Alan R. Anderson placed probe first on the left side of her head, then right, checking the flow of blood in her arteries. Test is used to determine the likelihood of stroke, hardly a concern for most 13 - year - olds. But Kaimora has Sickle Cell Disease. And stroke is one of danger even in children. Fortunately, scan showed that everything looked good, and the plucky girl with flowing lemonade braids giggle. It makes me happy to learn new things, she told the Greenville News. Especially when it about my body. And it is a good thing. Other News: Urgent care Centers, Blue Cross Blue Shield of South Carolina wrangle over coverage Sickle Cell Disease is a disorder of red blood cells that disproportionately afflicts African - Americans, according to the US Centers for Disease Control and Prevention. It is marked by hard, sticky cells that die early and clog blood flow, causing intense pain, infections, organ damage, stroke and other serious conditions, agency say. It also causes constant shortage of red blood cells, often requiring transfusions. This potentially life - threatening disorder occurs in about 1 of every 365 African - American babies and about 1 in every 16 300 Hispanic - American births, CDC reports. Kaimora's test is part of care she receives through a comprehensive new program for patients with Sickle Cell Disease, or SCD, that follows them from childhood through adulthood. Typically, once patients become adults, they do get the same care, Anderson say. And sadly, that results in a decline in life expectancy at a time when life spans are improving for nearly all other conditions, he say. Other News: Belton boy with cerebral palsy Get a puppy with deformed leg who's just like me. 've put together a model of treatment that combines preventive therapy and consistent team approach and that leads to increases in survival that are seen in kids now, say Anderson, who is a pediatric hematologist and Medical director of program. So now if youre child with SCD, 96 percent plus of those children will live to the age of 18, where before that. We had high death rate among children, he add. That success story. But a 2013 study showed that life expectancy, which was 42 for men and 48 for women in 1994, had dropped to 38 for men and 42 for women by 2005, he say. For any other chronic illness we take care of in the US in adults, youll see that survival is going up with better treatments and better follow - up, he say. With SCD, once patients transition to adult care, survival starts to drop off. So Anderson, who was involved with several pediatric Sickle Cell Disease and cancer projects at hospitals in Africa, began working on a new program.

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* Please keep in mind that all text is machine-generated, we do not bear any responsibility, and you should always get advice from professionals before taking any actions

A Public Health Approach

Survival trends have improved with early diagnosis of SCD. Early diagnosis allows for introduction of an evidence base and wellestablished preventive measures including Penicillin prophylaxis, vaccinations against encapsulate bacteria, family education regarding quick and prompt medical management for fever, and recognition of splenic sequestration. These strategies when integrated in Public Health programs for Screening and followup care, as in North America, parts of Europe, Brazil and Jamaica, have been demonstrated to reduce early morbidity and mortality associated with disease. 16 - 18 With recognition that infants with SCA have a greatly increased risk of bacterial sepsis, particularly invasive pneumococcal, landmark multicenter doubleblinded - placebocontrolled PROPS trial proves that Penicillin prophylaxis significantly lowers the risk of bacteremia and death. 19 this provides justification and impetus need to identify affected infants soon after birth and to enable delivery of these simple and affordable lifesaving interventions. Newborn Screening has been attempted in pilot projects in a number of SSA countries like Benin, Ghana, DRC and Kenya. For example, in Benin, where neonatal screening and comprehensive health care management were practice, lower mortality rate of SCD was 15. 5 per 10 000, which is ten times lower than the overall underfive mortality rate. 20, 21, these results are comparable with those in highincome countries, demonstrating advantages of Newborn Screening and close followup of children in comprehensive care approach. 22 Despite overwhelming evidence of efficacy of Newborn Screening for SCD, no country in SSA With 75% of the World's Disease burden has a universal Newborn Screening Program in place. Unfortunately, late diagnosis is make, often and most commonly when children develop severe complication,sss and, not uncommonly, child dies without a diagnosis being make. Barriers to widespread implementation of Newborn Screening programs include lack of public awareness and knowledge regarding SCD and advantages of early diagnosis, costs of testing techniques and blood sampling kits, lack of laboratory infrastructure, challenge with tracking screenpositive babies, poor access to newborn babies delivered outside birthing institutions, weak public Health systems To initiate and monitor programs and competing healthcare priorities that pose challenges in placing SCD high on Public Health agenda. 23 To surmount some of these barriers, case has been made to develop pointofcare tests to facilitate diagnosis of SCD. 2 To meet this need, POC devices should have the following characteristics: low cost, portable, high sensitivity and specificity, and ability to generate rapid results. In addition, ideal POC device should be user - friendly, results easy to interpret and device operable in extreme temperatures and humidity. Current POC devices under development are based on methods including lateral flow immunoassay, microfluidic electrophoresis, and densitometry methods. One POC diagnostic tool, based on the direct lateral flow immunoassay method, has made it to market while another, based on competitive immunoassay method, is under development.

* Please keep in mind that all text is machine-generated, we do not bear any responsibility, and you should always get advice from professionals before taking any actions.

* Please keep in mind that all text is machine-generated, we do not bear any responsibility, and you should always get advice from professionals before taking any actions

Circumstances of Death

Because of the difficulty of ascertaining the exact cause of death, in most cases, we focus on the general clinical status of patients and circumstances in which they die. The result of classification is shown in Table 2. Thirty - seven percent of patients underwent autopsies, and approximately 60 percent died outside the study center. For 38 patients who died without clinical evidence of chronic organ failure, there was insufficient information to classify their immediate circumstances of death. A small proportion of patients die of causes not related to Sickle Cell Disease, including trauma and cancer. In these cases, it was impossible to determine the extent to which underlying hemoglobinopathy contributed to death. Relatively few patients die with clinical evidence of chronic organ failure. In some instances, organ failure was attributable to cause other than Sickle Cell Disease, but undoubtedly complicated by it. Because arterial - blood gas measurements and pulmonary - function studies were not required for all patients, there was no working definition of chronic pulmonary failure in this study. Large numbers of patients had no overt chronic organ - system failure and die relatively suddenly and unexpectedly, presumably as a complication of the Sickle Cell crisis. Death occurred in the course of painful episode in 45 patients, 20 of whom had simultaneous acute chest syndrome. Nine patients had acute chest syndrome alone. Acute stroke occurred before death in 15 patients, with documented hemorrhage in 11. Although 13 of patients without chronic organ failure die of infection, infections vary, in contrast to the almost universal occurrence of pneumococcal sepsis in children under five years of age who die of infections.

* Please keep in mind that all text is machine-generated, we do not bear any responsibility, and you should always get advice from professionals before taking any actions.

* Please keep in mind that all text is machine-generated, we do not bear any responsibility, and you should always get advice from professionals before taking any actions

Sources

* Please keep in mind that all text is machine-generated, we do not bear any responsibility, and you should always get advice from professionals before taking any actions.

* Please keep in mind that all text is machine-generated, we do not bear any responsibility, and you should always get advice from professionals before taking any actions

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