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Sickles Cell Anemia in Children

In 1904 a hospital intern at The Presbyterian Hospital in Chicago Illinois, Dr. Earnest Irons, who was the first physician to describe sickle cells, wrote a report on Walter Clement Noels blood. Also in 1904, Dr. James B. Herrick, a Chicago Physician, treated a twenty- year-old college student from the West Indian islands of Grenada. The young man went to Herrick complaining of shortness of breath, heart problems, abnormal pain, and aches and pains in his muscles. The patient explained to Herrick that he is tired al the time, he said he had headaches, he get dizzy sometimes and have had ulcers in his legs.

Dr. Herrick took notes and took a sample of the man blood and viewed it under a microscope. The man blood cell where long and curved instead of round. The blood shapes on the microscope where not blood cell of anemia. The Dr. studied the disease for six years after he view it. 1910, Dr. Herrick was the first person to publish a medical report on sickle cell anemia. As the report accumulated, a patter emerged. In 1926, Dr. Thomas P. Cooley and Dr. P. Lee described two forms of sickle cell disease: sickle cell anemia and sickle cell trait.

During 1945, Dr. Linus Pauling discovered that an abnormal form of hemoglobin was the reason for the sickness in sickle cell patients. These two took a train together and started talking about sickle cell anemia. Castle told Pauling about how the cells in sickle cell patients sickled when their oxygen level is low. Paling was amazed by this conversation and did further research on the disease. After that Pauling was able to tell which patient had sickle cell trait and which one had the disease. In 1956, Dr. Vernon Ingram made a distinction between normal and abnormal hemoglobin.

Dr. Ingram used Paulings test to find the key difference. Like other proteins, hemoglobin is made up of chains of smaller chemical building blocks and amino acids. Dr. Ingram used enzymes of other chemicals to break the bond in the hemoglobin molecule. He discovered two proteins had different amino acids in one spot on the chain. Linus Pauling was a pioneer in sickle cell disease research. In December 1968, Jessie Jackson led a protest against construction companies, trying to force them to hire African American workers. Jackson and his group were arrested for picketing outside one of the constructions sites.

When Jackson was about to be released he collapsed and was rushed to the hospital. Jackson was suffering from Mononucleosis, but as his blood was tested, doctors discovered that Jackson was carrying the sickle cell trait. During the seventies the federal government finally acknowledged sickle cell disease as a serious illness that need attention. Before sickle cell was known about it was referred to a black disease because it affected African American most although it was in other races. Nationwide sickle cell screening programs were started but lawmakers concentrated only on African Americans.

Many blacks felt they race was being targeted and instead of helping them the screening was used discrimination against the African American race. Congress passed the National Sickle Cell Anemia Control Act in 1972. This law established the first national program that provided finding to fight sickle cell anemia. In 1980 Dr. Martin Clin attempted a transfer of normal beta globin genes to two patients with beta- thalassemia During 1902 The University of Chicago Medical Center reported its first bone marrow transplant for sickle cell treatment.

A British research team reported the successful transfer to adult mice of the human gene that directs bet hemoglobin production. A Cancer drug, hydroxyura, was found to stimulate fetal hemoglobin production preventing effects of sickle cell disease in 1990. During 1993, a National Institutes of Health panel recommended universal newborn screening for sickle cell disease. Mia Xyloportas, the AHEPA Cooleys Anemia poster child, was born with beta- thalassemia. She received blood transfusion about once a month; when she was four year old chelation therapy was added.

Mia was a happy active child and paid no attention to the treatments. When she was five, a bone marrow transplant cured her and she no longer needs treatment. Miss Tionnie Watkins is the spokesperson for Sickel Cell, she is the first celebrity to have this disease. She is Tboz that sing with the female R&B group TLC and promises she will not let it bring her down and continue to be strong for her fans. In the United States, sickle cell anemia is mostly found in African Americans it appears among three in 1,000.

Even though sickle cell attacks he organs those who manage their medical attention has been said to extend their live span from less than twenty years to more than fifty years. There is no way to remove the sickle cells patients suffer dramatically. Each year about one in four hundred African American infants are born with the disease, after inheriting from both parents. People with one copy of the mutation are said to have the sickle cell trait. It is said that one in twelve African Americans have sickle cell trait. One in 10 children suffer strokes before adulthood.

Health experts estimates 2 million Americans carry the sickle cell trait, there is another 72,000 with full-blown sickle cell anemia. Sickle cell affects 1 of every 500 African- American children, and 1 of every 1,100 to 1,400 Hispanic Americans are born with sickle cell anemia. The disease can also occur among non African Americans. An abnormal type of oxygen carrying pigment called hemoglobin causes sickle cell anemia. Normal hemoglobin is round and shaped like a ball, it is a folded molecule made up of four protein subunits two alpha chains and two beta chains.

Sickles cell occurs in someone who inherits hemoglobin S from both parents. Someone who gets hemoglobin from one parent and normal hemoglobin A from the other parent will have sickle cell. These symptoms usually dont occur until after four months old. Sickles cell anemia may become life threatening if damaged blood cells breakdown or bone marrow stop producing blood cells. If red blood cells continue to break down repeatedly it can cause damage to the kidneys, lungs, bones, liver and central nervous system. If veins and arteries are blocked and organs are damaged the person will have sharp pains.

The hemoglobin in the red blood cells carries oxygen and takes to all other parts of the body. The hemoglobin molecules in each red blood cell carry oxygen from the lungs to the body organs and tissues, it brings carbon dioxide back to the lungs. The hemoglobin in the disease is defective. After the molecules give up their oxygen they cluster together and form long rod like structures. These structures cause the red blood cells to become stiff and to make a sickle shape. Normal red blood cells last about 120 days in the bloodstream, sickle red blood cells die after only about 10 to 20 days.

Because they cannot be replaced fast enough. The error in the hemoglobin gene results from a genetic mutation that occurred many thousands of years ago in people in part of Africa, the Mediterranean basin the Middle East, and India. A deadly form of Malaria was very common at the time. Malaria empidemics caused the death of people. Studies show that in the areas where Malaria was a problem, children who inherited one sickle hemoglobin gene, carried the sickle cell trait. There are several forms of sickles cell disease. One of the most common forms is SS, SS is when the child inherits two sickles cell genes.

SC is when one sickle cell gene is inherited, and one gene for another abnormal type is called C. S beta thalassemia is when the child has one sickle cell gene and one thalassemia another type anemia that is inherited also. A child that is born by two people with sickle cell has one in two chances of also having sickle cell trait. One chance out of four of having sickles cell anemia and one chance in four of inheriting neither. You inherit the abnormal hemoglobin from both parents that might be carrying the sickle cell trait or parents with the disease you cannot catch it you are born with the hemoglobin and it wont go away.

If a women is having a baby there is a twenty five percent chance that the baby will inherit two normal genes or not having the disease. There is also a fifty percent chance of being an unaffected carrier like the parents. Babies and young children with sickle cell disease have special problems. Infants and young children who are affected have sever bacterial infections. Infections are the leading cause of death in children whit sickle cell. Pneumococcal infections used to be the pricipl cause of death of young children with sickle cell.

When children with sickle cell anemia are infected the have a hard time fighting them off once they get started. The infections may result in damage to the spleen from the sickled cells. This will prevent the spleen from destroying bacteria in the blood. Infants and young children can be killed by the infections in little as nine hours from onset fever, blood vessels in the hands or feet are blocked, pain in swelling result to fever. The may be the first sign of sickle cell anemia in infants. Fatigue, paleness, and shortness of breath all are all symptoms of anemia. You will also have a shortage of red blood cells.

There are unpredictable pains in any body organ or joint whenever sickled blood cells are blocking oxygen flow to the tissue. The pains are frequent and the amount of pain varies. Sometimes the pains last a few hours and other times he pains last several weeks. If you experience severe on going pain, the patient should be hospitalized and treated with painkillers, intravenous fluids, antibiotics and other medicines. Pain is the principle symptom of sickle cell anemia in children and adults. Eye problems are also a form, when the retina does not get enough nourishment from circulating red blood cells, it can deteriorate.

If the retina is damages serious enough it may cause blindness. Sign of jaundice, yellowing of eyes and skin results in the rushed breakdown of red blood cells. Children may also experience delayed growth and puberty the slow growth is caused by little red blood cells. Strokes occur when the defective hemoglobin damages the walls of the red blood cells causing then to stick to the blood vessel wall. This can cause the development of narrowed or blocked, small blood vessels in the brain this can cause serous serious life threatening strokes. This type of stroke occurs primarily in children.

Acute chest syndrome is also a symptom of sickle cell anemia, this a life threatening complication similar to pneumonia that is caused by infection and trapped sickle cells in the lungs. Acute chest syndrome is chest pain, fever and cause abnormal chest x rays. Early diagnosis and treatment help reduce the risk of infection and death a lot. Newborn testing alerts the doctors of the sickle cell trait. Early diagnosis is critical so that children who have the disease can receive proper treatment. Over forty states perform a simple, inexpensive blood test for sickle cell anemia on all newborn infants.

This test is done during other routine newborn screening test. Hemoglobin electrophoresis is the most widely used diagnostic test, if the test is positive a second test is performed to confirm the diagnosis, these test also detects sickle cell trait. There is no cure for sickle cell anemia but doctors can just help. Blood transfusion can be used treat and prevent some of the complications. Blood transfusions can correct anemia by increasing the number of normal blood cells. Transfusion can also be used to treat spleen enlargement in children before the sickle cell becomes life threatening.

Regular transfusions therapy also can prevent recurring strokes before the child has nervous system complications. By giving young children with sickle cell oral penicillin twice a day beginning at two months until five years can prevent pneumococal infection and early death in children. A doctor may also prescribe folic acid, a vitamin supplement. Taking penicillin or vitamins does not cure sickle cell it just help the children not be so sick. A bone marrow transplant has been shown to provide a cure for severly-affected children with sickle cell.

But bone marrow transplants is not entirely ithout risk because a drug is given to kill patients marrow and this drug can be very dangerous to someone who has had a stroke. The marrow must come from a health match sibling donor and only eighteen percent are likely to have a matched sibling. A bone marrow transplant can be very difficult and dangerous, a very close match must be found before the transplant can be done. The ultimate cure for sickle cell anemia is said to be gene therapy, where you correct the gene and insert it into bone marrow of people with the disease and try to produce normal hemoglobin.

It is import for children with sickle cell to stay healthy as possible. The children should eat health foods, get plenty of rest and drink lots of water. Kid with sickle cell can play games and sports as long as they dont get too hot, too cold or too tired. Children with sickle cell dont have enough normal red blood cells so they will get tired easy. Children will sickle cell may not be as hungry as normal children and they use the restroom a lot. The lives of people with sickle cell have improved dramatically. Because doctors understand the disease better now there is more hope for people who are affected.

Understanding the disease has not brought on a cure yet but it has enable doctors to decrease its danger and pains. Timely medical care is a for sure essential for the well bring of sickle cell patients and so does home care. Home care should begin when the sickle cell affected child is an infant. The first thing you must do is find out is the child has sickle cell or will have it in the future; the disease can be diagnosed before birth. After the child is diagnosed treatment should begin immediately after. Taking the child for regular doctor visit is very important.

Sickle cell children may suffer from episodes, and sometimes the pain is much to severe to be relieved at home. Children with sickle cell have special needs that family, friend, and teachers, can meet once they know how. Table 7. 1 Some Dominant and Recessive Traits In Humans Recessive TraitsPhenotypesDominant TraitsPhenotypes Sickle-Cell AnemiaDefective hemoglobin that causes red blood cells to collapsePolydactylyExtra Fingers and toes Source: George B. Johnson, The Living World 2nd ed, pg. 166 Sickle cell disease was named in the early 1900s, but it has been a fact of life for African people.

Parents watched helplessly while some of their children who seemed to be perfectly fine die suddenly for no apparent reason. There were even many tribal names for the disease, like chwechweechwe,nuiduidui, and ahututuo. They were imitations of the moans of the sufferers. In one West African tribe when the children died soon after birth were called ogbanjes. Ogbanjes mean children that come and go, the tribe people believed that a evil sprit was trying to come in the ogbanies family and they believe the babies bravely died to save the family from the demon.

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