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Sickle Cell Disease In Children

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Last Updated: 15 October 2020

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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 two Sickle Cell genes, one 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. In addition to complete medical history and physical examination, diagnostic procedures for Sickle Cell may include blood tests, complete family history and results of newborn screening. Many States now perform hemoglobinopathy testing as part of newborn screening. Blood tests that are routinely done State newborn screening refer to test done on every baby born in every state of the country within the first few days of life, to detect serious, life - threatening diseases. State laws require that babies be tested between two and seven days of age, even if the baby seems healthy and has no symptoms of health problems. Early diagnosis is essential to providing proper preventive treatment for some of the devastating complications of disease. Hemoglobin electrophoresis is a blood test that can determine if a child is a carrier of a specific Sickle Cell Trait, or has any diseases associated with the Sickle Cell gene. Advances in preventive care and new medicines have reduced life - threatening problems of Sickle Cell.S

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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

Departments

It is not know if ADAKVEO is safe and effective in children under 16 years of age. What is the most important information I should know about ADAKVEO? Infusion reactions. Infusion reactions may happen within 24 hours of receiving infusion of ADAKVEO. Tell your health care provider right away if you get any of the following signs and symptoms of infusion reaction: your doctor or health care provider may monitor you for signs and symptoms of infusion reactions. Tell your health care provider that you are receiving ADAKVEO before having any blood tests. Adakveo may interfere with laboratory tests to measure your platelet counts. Before receiving ADAKVEO, tell your health care provider about all of your medical conditions, including if you tell your health care provider about all of the medicines you take, including prescription and over - counter medicines, vitamins, and herbal supplements. These are not all of the possible side effects of ADAKVEO. For more information, ask your health care provider or pharmacist. You are encouraged to report negative side effects of prescription drugs to the FDA. Visit www. Fda. Gov / medwatch, or call 1 - 800 - FDA - 1088. Please see full Prescribing Information, including Patient Information.

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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 is sickle cell disease?

Table

ComplicationCauseSymptoms
Acute Chest SyndromeSickle cells clog and block blood flow to the lungs Can occur if your child has pneumoniaCough Chest pain Fever of 100.4F (38C) or higher Shortness of breath or difficulty breathing
AnemiaDecreased red blood cells, which commonly occurs because red blood cells dont live as long in children with sickle cell diseaseShortness of breath Dizziness Weakness Fast or irregular heartbeat Pale or yellow skin
InfectionsDamage to the spleen which normally filters bacteria out of blood caused by sickle cells Risk is higher in infants and children with sickle cell diseaseFever of 100.4F (38C) or higher Chills or shaking
Hand-Foot SyndromeSickle cells clog and block blood flow to the hands and feet Risk is higher in infants and children with sickle cell diseaseSwelling in the hands and feet Fever of 100.4F (38C) or higher
Pain EpisodesSickle cells clog and stop blood flow in small blood vesselsMild to severe pain, usually in the abdomen, arms, chest, lower back, and legs
Splenic SequestrationSickle cells clog and block blood flow in the spleen, causing it to get bigSudden weakness Pale lips Fast breathing Pain in the left side of the belly Fast heartbeat
StrokeSickle cells clog and block blood flow to the brainLoss of balance Severe headache Difficulty talking, walking, or understanding Weakness in the face, arm, or leg on one side of the body
Vision lossSickle cells clog and block blood flow to the eyeBlurry vision, difficulty seeing, or complete blindness

Sickle Cell disease is the name of a group of inherited blood disorders that is characterized by chronic anemia, periodic episodes of pain and other complications. Disease affects red blood cells. Sickle Cell disease results when red blood cells make mostly hemoglobin S instead of normal hemoglobin. Normal red blood cells are smooth, round and soft; their shape and softness enable them to move easily through blood vessels to carry oxygen to all parts of the body. In Sickle Cell disease, abnormal hemoglobin S causes red blood cells to become hard, point and sticky and shape like crescents or sickles. When these hard and point red cells go through small blood vessels, they often get stuck and block vessels. They also stick to and damage the lining of larger blood vessels. Damage vessels become scarred, narrowed and sometimes completely closed. Blockage of blood flow through blood vessels can lead to pain, stroke and damage to many organs. Sickle cells also break up too easily and survive for only 10 to 20 days compared to 120 days for normal red blood cells. Rapid destruction of sickle cells leads to anemia shortage of red blood cells and hemoglobin. It has become difficult for the body to make new red blood cells fast enough to keep up with needed supply.

* 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

Gene therapy for sickle cell

Gene Therapy is fighting to enter mainstream medicine. With sickle Cell Disease, fight is heating up. Roughly two years ago, FDA made the historic decision to approve the first Gene Therapy in the US, finally realizing the therapeutic potential of hacking our biological base code after decades of cycles of hope and despair. Other approvals soon follow, including Luxturna to target inherited blindness and Zolgensma, single injection that could save children with degenerative disease from their muscles wasting away and dying before the age of two. Yet despite their transformative potential, Gene Therapy has only targeted relatively rareand often fataldisorders. That about to change. This year, handful of companies deployed Gene Therapy against sickle - Cell Anemia, condition that affects over 20 million people worldwide and 100 000 Americans. With over a dozen therapies in run, sickle - Cell Disease could be an indication that allows Gene Therapy to enter the mainstream. Yet because of its unique nature, sickle - Cell could also be an indication that shins unflinching spotlight on challenges to nascent breakthrough,s both ethically and technologically. You see, sickle - Cell Anemia, while being one of the world's best - known genetic diseases, and one of the best understood, also predominantly affects third - world countries and marginalized people of color in the US. So far, Gene Therapy has come with a hefty bill exceeding millions; few people afflicted by the condition can carry that amount. Potential treatments are enormously complex, further upping costs to include lengthy hospital stays, and increasing potential side effects. To muddy waters even more, disorder, though causing tremendous pain and risk of stroke, already has approved pharmaceutical treatments and isnt necessarily considered life - threatening. How we handle gene therapies for sickle - Cell could inform many other similar therapies to come. With nearly 400 clinical trials in making and two dozen nearing approval, there is no doubt that hacking our genes will become one of most transformative medical wonders of the new decade. The question is: will it ever BE available for everyone in need?

* 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?

Painful episodes are frequent in sickle cell anemia patients and they occur during childhood, but are less frequent in babies. They are caused mostly by sickle red blood cells getting trapped inside small blood vessels. This is know as vaso - occlusive crisis, which is often accompanied by swelling of hands, feet, arms, or legs. It is very distressing for parents to see their child in pain and it is very important that pain is relieved as fast as possible. To treat pain in the best possible way, children must learn to communicate the location and intensity of pain. A pain scale from 1 - 10 can be helpful in assessing the level of pain.

* 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

Our approach

Hb SS and Hb S 0 are more severe forms of SCD than Hb SC and Hb S +. However, even within individual genotypes, there is a broad range of disease severity. Concentration of Hb F is the main determinant of this variability. Hb F modulates disease severity by inhibiting polymerization of Hb S in a dose - dependent manner. Coinheritance of - thalassemia can modify the phenotype of Hb SS by decreasing hemolytic rate and risk of stroke. However, there is some evidence that - thalassemia may increase the frequency of painful episodes and AVN. Thus, - thalassemia can modify the phenotype of Hb SS in both favorable and unfavorable ways. Except for successful prediction and prevention of overt stroke using Screening TCD programs, it remains difficult to identify young children with Hb SS who are at highest risk of adverse outcomes before irreversible organ damage occurs. A promising predictive model was developed based on occurrence of dactylitis, severe anemia, and leukocytosis in very young children, 32 but it was not validated in independent cohort. 33 Ongoing research using broad genetic approaches and sophisticated statistical modeling might benefit future children.


What is sickle cell disease (SCD)?

Sickle cell disease is a group of inherited red blood cell disorders. Red blood cells are round and carry oxygen. They move throughout the body through blood vessels. Scd causes red blood cells to be hard, sticky and have a namesake sickle shape that looks like a C - shaped farm tool or crescent. Sickle - shape red blood cells die earlier than normal red blood cells, causing person with SCD to have a constant shortage of red blood cells. Because of their shape, sickle cells also stick together in small blood vessels. This can cause blockage in blood flow and oxygen to bodys organs. These blockages can cause issues throughout the entire body, including repeated episodes of severe bone pain; lung, heart, gastrointestinal and urogenital conditions; organ damage, serious infections, or even seizures and stroke.

* 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 are the treatment options?

Pain is the most common complication of SCD, and the number 1 reason that people with SCD go to the emergency room or hospital. When Sickle cells travel through small blood vessels, they can get stuck and clog blood flow. This causes pain that can start suddenly, be mild to severe, and can last for any length of time. There are simple steps that people with SCD can take to help prevent and reduce the number of pain crises, including the following: drink plenty of water. Try not to get too hot or too cold. Try to avoid places or situations that cause exposure to high altitudes. Try to avoid places or situations with exposure to low oxygen levels. Adults with severe SCD can take medicine called hydroxyurea to help reduce the number of pain crises. People taking hydroxyurea are checked often by doctors to prevent complications, including increased risk of infections. Research shows that babies and children with SCD can also benefit from hydroxyurea therapy. Most pain related to SCD can be treated with over - counter pain medications such as ibuprofen and aspirin. Some people who have severe pain are given opioid medications daily, along with additional pain medication. Some people may be admitted to hospital for intense treatment.


Infection

Washing your hands is one of the best ways to help prevent infection. People with SCD, their family, and other caretakers should wash their hands with soap and clean water many times each day. Because bacteria in some foods can be especially harmful to children with SCD, food should be prepared safely. Vaccines can protect against harmful infections. Children with SCD should get all regular childhood vaccines, plus a few extra. Adults should have flu vaccine every year, as well as pneumococcal vaccine and any others recommended by doctor. Take penicillin every day until at least 5 years of age. See tip sheet on how to help prevent infection


What Is Sickle Cell Disease?

Stem cell transplant is the only known cure for sickle cell disease. Transplants are complex and risky, and for now are an option only for some patients. Scientists are studying gene therapy for sickle cell anemia. One day, it's hopped that doctors could stop disease by changing or replacing abnormal genes that cause it. But even without a cure, kids with sickle cell disease can lead fairly normal lives if they follow their treatment plan. Immunizations and daily doses of penicillin to help prevent infection. Kids with sickle cell disease should get all recommended vaccinations, including pneumococcal, flu, and meningococcal vaccines. Folic acid supplements, which can help kids make new red blood cells. Hydroxyurea, medicine that makes cells less sticky. This helps decrease the frequency and intensity of painful episodes and other complications. Hydroxyurea is taken every day. L - glutamine, another medicine. It's used if hydroxyurea isn't working well or someone still has pain crises even with hydroxyurea. Medicines to help manage pain when it does happen. Sometimes, blood transfusions for severe anemia or some complications.

* 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

Diagnosis

There are several tests suitable for determining what type of hemoglobin is made by a person's red blood cells. These include: hemoglobin electrophoresis Isoelectric focusing Chromatography. These tests can determine whether a person has a type of sickle cell disease or sickle cell trait. Dna analysis is used to determine changes in genes for making hemoglobin. This test indirectly predicts the type of hemoglobin made in red cells. Tests that are not suitable for determining sickle cell disease or sickle cell trait include: solubility tests. Sickling test Positive results from these tests simply indicate the presence of hemoglobin S in a person's blood; this occurs in both sickle cell trait and all types of sickle cell disease. Other tests that are not useful by themselves for diagnosing sickle cell conditions include: blood count. This test may detect anemia, but there are many other causes of anemia in infants and others with mild types of sickle cell disease, and there may be little or no anemia. Blood smear. Sickled cells can be easily seen on blood smear examined under microscope. However, even in some people with severe sickle cell disease and particularly in young children and in others with milder types of sickle cell disease, there may be no sickled cells on regular blood smears.


Prenatal Genetic Tests

Our doctors offer prenatal genetic tests, such as amniocentesis or chorionic villus sampling, for parents who are known carriers of sickle cell gene mutation or have a family history of the condition. If you and your partner have a family history of sickle cell disease and youre unsure about whether or not you carry mutation, you may also consider genetic testing. Amniocentesis, usually performed in the second trimester of pregnancy, looks for abnormal genes in small sample of amniotic fluid that surrounds the baby while he or she is in the womb. Chorionic villus sampling can be performed during the first trimester of pregnancy. With this procedure, needle is used to remove a small amount of tissue from the placentaa, temporary organ that carries oxygen and nutrients from mother to baby. Tissue is analyzed for presence of abnormal gene.S Nyu Langone genetic counselors can help you understand the results of these tests.

* 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

Complications

Sickle Cell anemia can lead to a host of complications, including: stroke. Sickle cells can block blood flow to area of your brain. Signs of stroke include seizures, weakness or numbness of your arms and legs, sudden speech difficulties, and loss of consciousness. If your child has any of these signs and symptoms, seek medical treatment immediately. Strokes can be fatal. Acute chest syndrome. Lung infection or sickle cells blocking blood vessels in your lungs can cause this life - threatening complication, resulting in chest pain, fever and difficulty breathing. It might require emergency medical treatment. Pulmonary hypertension. People with Sickle Cell anemia can develop high blood pressure in their lungs. This complication usually affects adults. Shortness of breath and fatigue are common symptoms of this condition, which can be fatal. Organ damage. Sickle cells that block blood flow to organs deprive affected organs of blood and oxygen. In Sickle Cell anemia, blood is also chronically low in oxygen. This lack of oxygen - rich blood can damage nerves and organs, including your kidneys, liver and spleen, and can be fatal. Blindness. Sickle cells can block tiny blood vessels that supply your eyes. Over time, this can damage your eye and lead to blindness. Leg ulcers. Sickle Cell anemia can cause open sores on your legs. Gallstones. Breakdown of red blood cells produces a substance called bilirubin. A high level of bilirubin in your body can lead to gallstones. Priapism. In this condition, men with Sickle Cell anemia can have painful, long - lasting erections. Sickle cells can block blood vessels in the penis, which can lead to impotence over time. Pregnancy complications. Sickle Cell anemia can increase the risk of high blood pressure and blood clots during pregnancy. It can also increase the risk of miscarriage, premature birth and having low birth weight babies.

* 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

SCT and Athletes

The benefits of regular exercise are well described and span the life cycle from childhood throughout adulthood, ranging from physiological to psychological to social. Growing evidence suggests that exercise in all forms triggers epigenetic changes in several pathways that underlie physiological benefits of exercise. 1 - 3 A prevention strategy, exercise prescription in general healthy population aims to reduce future cardiovascular risk. There is now growing acceptance that exercise prescription among individuals with chronic illnesses is equally important both for preventing comorbid conditions and, in some cases, conferring disease - modifying effects. Thus, adoption of the mantra that exercise is medicine is essential for both healthy individuals as well as those with chronic medical conditions. The goal of this review is to discuss evidence underlying concerns for adverse events relating to high - intensity exercise in SCT and SCA and potential pathophysiologic basis for these concerns. To underscore the need to consider both sides of the risk - benefit balance, potential negative implications of limiting exercise in these populations, giving growing evidence for unique benefits that regular exercise and improved fitness may confer in SCT and SCA, will also be discuss. This review will be framed in the form of key questions, comparing the history of existing evidence over years and gaps that remain in both conditions.

* 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

Symptoms

Sickle cell anemia is usually diagnosed in infancy through newborn screening programs. If you or your child develop any of the following problems, see your doctor right away or seek emergency medical care: fever. People with sickle cell anemia have an increased risk of serious infection, and fever can be the first sign of infection. Unexplained episodes of severe pain, such as pain in the abdomen, chest, bones or joints. Swelling in hands or feet. Abdominal swelling, especially if the area is tender to touch. Pale skin or nail beds. Yellow tint to the skin or whites of the eyes. Signs or symptoms of stroke. If you notice one - side paralysis or weakness in your face, arms or legs; confusion; trouble walking or talking; sudden vision changes or unexplained numbness; or severe headache, call 911 or your local emergency number right away.

* 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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