Can a white man get sickle cell
Sickle cell disease is a condition in which red blood cells are not shaped as they should be. Red blood cells usually look like round discs. But in sickle cell disease, they're shaped like crescent moons, or an old farm tool known as a sickle. These sickle shaped cells get stuck together easily, and block off small blood vessels.SEE VIDEO BY TOPIC: Sickle cell anemia - causes, symptoms, diagnosis, treatment & pathology
SEE VIDEO BY TOPIC: Sickle Cell Anemia: A Patient's JourneyContent:
- Data & Statistics on Sickle Cell Disease
- 10 Things You Should Know About Sickle Cell Disease
- Sickle Cell Trait
- Can biracial children get sickle cell disease?
- Sickle cell disease
- One Disease Hits Mostly People of Color. One Mostly Whites. Which One Gets Billions In Funding?
- About Sickle Cell
- Sickle Cell Disease
Data & Statistics on Sickle Cell Disease
Sickle cell disease SCD is a group of blood disorders typically inherited from a person's parents. The care of people with sickle cell disease may include infection prevention with vaccination and antibiotics , high fluid intake, folic acid supplementation, and pain medication. As of , about 4. Herrick in Beet and J. Signs of sickle cell disease usually begin in early childhood. The severity of symptoms can vary from person to person.
The terms "sickle cell crisis" or "sickling crisis" may be used to describe several independent acute conditions occurring in patients with SCD, which results in anaemia and crises that could be of many types, including the vaso-occlusive crisis , aplastic crisis , sequestration crisis , haemolytic crisis , and others. Most episodes of sickle cell crises last between five and seven days.
The vaso-occlusive crisis is caused by sickle-shaped red blood cells that obstruct capillaries and restrict blood flow to an organ, resulting in ischaemia , pain , necrosis , and often organ damage. The frequency, severity, and duration of these crises vary considerably. Painful crises are treated with hydration, analgesics , and blood transfusion ; pain management requires opioid drug administration at regular intervals until the crisis has settled.
For milder crises, a subgroup of patients manages on nonsteroidal anti-inflammatory drugs such as diclofenac or naproxen. For more severe crises, most patients require inpatient management for intravenous opioids; patient-controlled analgesia devices are commonly used in this setting. Vaso-occlusive crisis involving organs such as the penis  or lungs are considered an emergency and treated with red blood cell transfusions.
Incentive spirometry , a technique to encourage deep breathing to minimise the development of atelectasis , is recommended. Because of its narrow vessels and function in clearing defective red blood cells, the spleen is frequently affected.
This spleen damage increases the risk of infection from encapsulated organisms ;   preventive antibiotics and vaccinations are recommended for those lacking proper spleen function. Splenic sequestration crises are acute, painful enlargements of the spleen, caused by intrasplenic trapping of red cells and resulting in a precipitous fall in haemoglobin levels with the potential for hypovolemic shock. Sequestration crises are considered an emergency. If not treated, patients may die within 1—2 hours due to circulatory failure.
Management is supportive, sometimes with blood transfusion. These crises are transient; they continue for 3—4 hours and may last for one day. Acute chest syndrome is defined by at least two of these signs or symptoms: chest pain, fever, pulmonary infiltrate or focal abnormality, respiratory symptoms, or hypoxemia. Most cases present with vaso-occlusive crises, and then develop acute chest syndrome. Aplastic crises are acute worsenings of the patient's baseline anaemia, producing pale appearance , fast heart rate , and fatigue.
This crisis is normally triggered by parvovirus B19 , which directly affects production of red blood cells by invading the red cell precursors and multiplying in and destroying them. In normal individuals, this is of little consequence, but the shortened red cell life of SCD patients results in an abrupt, life-threatening situation.
Reticulocyte counts drop dramatically during the disease causing reticulocytopenia , and the rapid turnover of red cells leads to the drop in haemoglobin. This crisis takes 4 to 7 days to disappear. Most patients can be managed supportively; some need blood transfusion. Haemolytic crises are acute accelerated drops in haemoglobin level.
The red blood cells break down at a faster rate. This is particularly common in people with coexistent G6PD deficiency. One of the earliest clinical manifestations is dactylitis , presenting as early as six months of age, and may occur in children with sickle cell trait. Normally, humans have haemoglobin A , which consists of two alpha and two beta chains, haemoglobin A2 , which consists of two alpha and two delta chains, and haemoglobin F , consisting of two alpha and two gamma chains in their bodies.
Of these three types, haemoglobin F dominates until about 6 weeks of age. Afterwards, haemoglobin A dominates throughout life. Sickle cell conditions have an autosomal recessive pattern of inheritance from parents. Sickle cell gene mutation probably arose spontaneously in different geographic areas, as suggested by restriction endonuclease analysis.
Their clinical importance is because some are associated with higher HbF levels, e. This is normally a benign mutation, causing no apparent effects on the secondary , tertiary , or quaternary structures of haemoglobin in conditions of normal oxygen concentration.
However, under low oxygen concentration, HbS polymerizes and forms fibrous precipitates because the deoxy form of haemoglobin exposes a hydrophobic patch on the protein between the E and F helices Phe 85, Leu In people heterozygous for HbS carriers of sickling haemoglobin , the polymerisation problems are minor because the normal allele is able to produce half of the haemoglobin.
In people homozygous for HbS, the presence of long-chain polymers of HbS distort the shape of the red blood cell from a smooth, doughnut -like shape to ragged and full of spikes, making it fragile and susceptible to breaking within capillaries. Carriers have symptoms only if they are deprived of oxygen for example, while climbing a mountain or while severely dehydrated.
The allele responsible for sickle cell anaemia can be found on the short arm of chromosome 11 , more specifically 11p A person who receives the defective gene from both father and mother develops the disease; a person who receives one defective and one healthy allele remains healthy, but can pass on the disease and is known as a carrier or heterozygote. Heterozygotes are still able to contract malaria, but their symptoms are generally less severe.
Due to the adaptive advantage of the heterozygote, the disease is still prevalent, especially among people with recent ancestry in malaria-stricken areas, such as Africa , the Mediterranean , India , and the Middle East. The malaria parasite has a complex lifecycle and spends part of it in red blood cells.
In a carrier, the presence of the malaria parasite causes the red blood cells with defective haemoglobin to rupture prematurely, making the Plasmodium parasite unable to reproduce. Further, the polymerization of Hb affects the ability of the parasite to digest Hb in the first place. Therefore, in areas where malaria is a problem, people's chances of survival actually increase if they carry sickle cell trait selection for the heterozygote. In the United States, with no endemic malaria, the prevalence of sickle cell anaemia among people of African ancestry is lower about 0.
Without endemic malaria, the sickle cell mutation is purely disadvantageous and tends to decline in the affected population by natural selection , and now artificially through prenatal genetic screening.
However, the African American community descends from a significant admixture of several African and non-African ethnic groups and also represents the descendants of survivors of slavery and the slave trade. Thus, a degree of genetic dilution via crossbreeding with non-African people and high health-selective pressure through slavery especially the slave trade and the frequently deadly Middle Passage may be the most plausible explanations for the lower prevalence of sickle cell anaemia and, possibly, other genetic diseases among African Americans compared to West Africans.
Another factor that limits the spread of sickle cell genes in North America is the relative absence of polygamy. In polygamous societies, affected males may father many children with multiple partners.
The loss of red blood cell elasticity is central to the pathophysiology of sickle cell disease. Normal red blood cells are quite elastic and have a biconcave disc shape, which allows the cells to deform to pass through capillaries.
These cells fail to return to normal shape when normal oxygen tension is restored. As a consequence, these rigid blood cells are unable to deform as they pass through narrow capillaries, leading to vessel occlusion and ischaemia. The actual anaemia of the illness is caused by haemolysis , the destruction of the red cells, because of their shape.
Although the bone marrow attempts to compensate by creating new red cells, it does not match the rate of destruction. In other forms of sickle cell disease, Hb levels tend to be higher. A blood film may show features of hyposplenism target cells and Howell-Jolly bodies. Sickling of the red blood cells, on a blood film, can be induced by the addition of sodium metabisulfite. The presence of sickle haemoglobin can also be demonstrated with the "sickle solubility test".
A mixture of haemoglobin S HbS in a reducing solution such as sodium dithionite gives a turbid appearance, whereas normal Hb gives a clear solution. Abnormal haemoglobin forms can be detected on haemoglobin electrophoresis , a form of gel electrophoresis on which the various types of haemoglobin move at varying speeds. Sickle cell haemoglobin HgbS and haemoglobin C with sickling HgbSC —the two most common forms—can be identified from there. The diagnosis can be confirmed with high-performance liquid chromatography.
Genetic testing is rarely performed, as other investigations are highly specific for HbS and HbC. An acute sickle cell crisis is often precipitated by infection. Therefore, a urinalysis to detect an occult urinary tract infection, and chest X-ray to look for occult pneumonia should be routinely performed. People who are known carriers of the disease often undergo genetic counseling before they have children.
A test to see if an unborn child has the disease takes either a blood sample from the fetus or a sample of amniotic fluid. Since taking a blood sample from a fetus has greater risks, the latter test is usually used. Neonatal screening provides not only a method of early detection for individuals with sickle cell disease, but also allows for identification of the groups of people who carry the sickle cell trait. Treatment involves a number of measures.
While it has been historically recommended that people with sickle cell disease avoid exercise, regular exercise may benefit people. From birth to five years of age, penicillin daily, due to the immature immune system that makes them more prone to early childhood illnesses, is recommended. The protective effect of sickle cell trait does not apply to people with sickle cell disease; in fact, they are more vulnerable to malaria, since the most common cause of painful crises in malarial countries is infection with malaria.
People with sickle cell disease living in malarial countries should receive lifelong medication for prevention.
Most people with sickle cell disease have intensely painful episodes called vaso-occlusive crises. However, the frequency, severity, and duration of these crises vary tremendously. Painful crises are treated symptomatically with pain medications ; pain management requires opioid drug administration at regular intervals until the crisis has settled.
Diphenhydramine is also an effective agent that doctors frequently prescribe to help control itching associated with the use of opioids. Extra fluids, administered either orally or intravenously, are a routine part of treatment of vaso-occlusive crises but the evidence about the most effective route, amount and type of fluid replacement remains uncertain.
Crizanlizumab , a monoclonal antibody target towards p-selectin was approved in in the United States to reduce the frequency of vaso-occlusive crisis in those 16 years and older. Management is similar to vaso-occlusive crisis, with the addition of antibiotics usually a quinolone or macrolide, since cell wall-deficient ["atypical"] bacteria are thought to contribute to the syndrome ,  oxygen supplementation for hypoxia , and close observation.
In the absence of high quality evidence regarding the effectiveness of antibiotics for acute chest syndrome in people with sickle cell disease, there is no standard antibiotic treatment as of Should the pulmonary infiltrate worsen or the oxygen requirements increase, simple blood transfusion or exchange transfusion is indicated.
The latter involves the exchange of a significant portion of the person's red cell mass for normal red cells, which decreases the level of haemoglobin S in the patient's blood.
However, there is currently uncertain evidence about the possible benefits or harms of blood transfusion for acute chest syndrome in people with sickle cell disease. Hydroxyurea probably reduces the frequency of painful episodes and the risk of life-threatening illness or death but there is currently insufficient evidence regarding the risk of adverse effects.
It was the first approved drug for the treatment of sickle cell anaemia, and was shown to decrease the number and severity of attacks in  and shown to possibly increase survival time in a study in
10 Things You Should Know About Sickle Cell Disease
Sickle cell disease SCD is a group of blood disorders typically inherited from a person's parents. The care of people with sickle cell disease may include infection prevention with vaccination and antibiotics , high fluid intake, folic acid supplementation, and pain medication. As of , about 4. Herrick in Beet and J.
Sickle cell disease SCD is a group of inherited red blood cell disorders. If you have SCD, there is a problem with your hemoglobin. Hemoglobin is a protein in red blood cells that carries oxygen throughout the body. With SCD, the hemoglobin forms into stiff rods within the red blood cells. This changes the shape of the red blood cells.
Sickle Cell Trait
A person with sickle cell trait inherits one gene to make normal hemoglobin A and another gene to make some sickle cell hemoglobin S. Hemoglobin is the part of the red blood cell that carries oxygen to different parts of the body. Sickle cell trait AS usually does not cause any health problems. Sickle cell trait is found in 1 out of every 11 African Americans, but it can occur in people of any race or background. Our genes determine the color of our eyes, our blood type and many other features, including our hemoglobin type. We inherit one gene for hemoglobin from each of our parents. Each of our parents has two genes for hemoglobin, but they pass only one of these genes on to each child. To inherit sickle cell trait, a child must get the sickle S gene from one parent and a normal gene A from the other parent Picture 1.
Can biracial children get sickle cell disease?
Yes, they can. Sickle cell disease can affect people of ANY race or ethnicity. Sickle cell disease, an inherited disorder of the red blood cells, is more common in African Americans in the U. However, there are other groups of people who also may inherit sickle cell disease. In all those regions, both dark and light skin people can carry copies of the sickle cell genes.
Increasing numbers of white babies are born each year carrying the sickle cell anaemia trait because of hidden black ancestry, according to a leading geneticist. Extrapolating from 16th century census data, Dr Steve Jones, of University College London, has calculated that one in five Britons, 11 million people, have a black ancestor. People who think of themselves as white but have the sickle cell trait almost certainly had a black ancestor, he said. Sickle cell anaemia is an inherited blood disorder that causes anaemia and chronic pain.
Sickle cell disease
Haemoglobin is the substance in red blood cells that is responsible for the colour of the cell and for carrying oxygen around the body. People with sickle cell disorder are born with the condition, it is not contagious. It can only be inherited from both parents each having passed on the gene for sickle cell. The main symptoms of sickle cell disorder are anaemia and episodes of severe pain.
From the Cleveland Clinic. Sickle cell anemia until recently has been thought to be limited to the Negro race. Even in this race it is relatively uncommon, although about 7 per cent of Negroes show sickling of the red blood cells when a film of fresh blood is sealed under a cover slip for several hours latent sickling. The anemia characteristic of meniscocytosis is hemolytic anemia with good response of the marrow, since the bilirubin content of the blood plasma is increased and the. Arch Intern Med Chic. Coronavirus Resource Center.
One Disease Hits Mostly People of Color. One Mostly Whites. Which One Gets Billions In Funding?
Sickle cell disease is an inherited genetic abnormality of hemoglobin the oxygen-carrying protein found in red blood cells characterized by sickle crescent -shaped red blood cells and chronic anemia caused by excessive destruction of the abnormal red blood cells. Worsening anemia, fever, and shortness of breath with pain in the long bones, abdomen, and chest can indicate sickle cell crisis. A special blood test called electrophoresis can be used to determine whether people have sickle cell disease. Avoiding activities that may cause crises and treating infections and other disorders quickly can help prevent crises. See also Overview of Anemia.
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About Sickle Cell
Back to Sickle cell disease. If you're a carrier of sickle cell, it means you carry one of the genes that causes sickle cell disease, but you do not have the condition yourself. People who carry sickle cell will not develop sickle cell disease, but may be at risk of having a child with sickle cell disease and may occasionally need to take precautions to stop them becoming unwell.
Sickle Cell Disease
Sickle cell disease is an inherited form of anemia where red blood cells become abnormally long and pointed, similar to the shape of a banana. It affects approximately , people in the United States and millions worldwide. Although sickle cell disease is not an extremely rare condition, there are some lesser-known facts and misconceptions everyone should know.
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