Hey, this is Carina's Sickle Cell Anemia page! Sickle Cell Anemia is a serious disease in which your body makes sickle-shaped red blood cells. Sickle-shaped means the cells are shaped sort of like a crescent, instead of the regular disc shape. It takes two copies of the sickling gene, one from each parent, for someone to have Sickle Cell Anemia. But, if someone receives only one of the genes, they will have Sickle Cell trait. Sickle Cell trait is not a disease, but it can be passed down to offspring.[1] Other names for Sickle Cell Anemia:
Description Red blood cells contain the protein hemoglobin, which carries oxygen from your lungs to other parts of your body. Sickle cells contain abnormal hemoglobin which sticks together to form strands, making the cells sickle shaped. The cells are stiff and sticky and tend to form in clumps, which blocks blood vessels. Blocked blood vessels can cause pain, serious infections, and organ damage.[1]  This picture compares sickle cells with normal cells "Sickle Cell Anemia." Sickle Cell Anemia. August 2008. National Heart Lung and Blood Institute. 15 Dec 2008 <http://www.nhlbi.nih.gov/health/dci/Diseases/Sca/SCA_WhatIs.html> Bone marrow is constantly making new red blood cells to replace old ones. Normally, red blood cells last 120 days before they die. Sickle cells last only ten to twenty days. A deficiency of cells develops because the bone marrow can't make new ones fast enough to replace the dead ones.[1] The signs and symptoms vary for different people. Some get very serious symptoms and have to be hospitalized, while others get only mild symptoms. When blood vessels get blocked, a "sickle cell crisis" occurs. A sickle cell crisis is when you get a sudden pain throughout your body that ranges from very mild to very severe. The pain can last for weeks to months, or hours to days, depending on whether it's acute or chronic pain. Even though Sickle Cell Anemia is present at birth, children don't show any signs of it until four months of age.[1] Symptoms include:
Sickle Cell Anemia affects about 70,000 people in America. This disease affects mainly African Americans and Hispanic Americans, though the disease occurs in other ethnicities as well. About 1 in 500 African Americans and 1 in 36,000 Hispanic Americans are diagnosed with this disease at birth. About 2 million Americans, including 1 out of 12 African Americans, have Sickle Cell trait.[1] A person with Sickle Cell Anemia looks and acts just like a person without it, except for a few differences. Their skin may be paler, they might fatigue easily, they might suffer many pains related to this disease, and they might visit a doctor more often than most people.[1] There are no known or common environmental factors that contribute to Sickle Cell Anemia. Treatment There is no absolute cure for Sickle Cell Anemia, but there are a number of treatments that offer relief from pain and prevent complications. Mild pain is treated with over-the-counter medicine and heating pads, while severe pain is treated with hospitalization. The usual treatments for acute crises are pain killing medicines and fluids that are given through the mouth or a vein, but they came come in pill form too.[1]  These are some of the pain medications children with Sickle Cell Anemia use. Courtesy of Xechem, medicine company Common pain medicines:
Infections include:
There are different treatments for the many complications and problems Sickle Cell Anemia causes. Some complications include: strokes, acute chest syndrome, leg ulcers, gallbladder disease, and eye damage. Here are some preventions for these very serious complications a person with this disease might experience. [1]
Thanks to our modern day technology, people with Sickle Cell Anemia are now living into their forties and fifties and longer. Because of these and other preventives and treatments, people with Sickle Cell Anemia can live happy, normal lives, just as if they didn't have the disease.[1] Research Right now, scientists are discovering ways to predict how severe Sickle Cell Anemia is. They have been researching bone marrow transplants, gene therapy, stem cells, and new medicines. Hopefully, gene therapy will be able to replace the sickling gene with a normal gene, or possibly turn on/off the gene.[1]  This is a picture of the inside of a bone, the bone marrow. "Bone Marrow Transplant." Virtual Medical Center. Quality Health. 15 Jan 2009 <http://www.virtualcancercentre.com/uploads/VMC/DiseaseImages/605_Bone_Marrow.jpg&imgrefurl>. New medicines include:
Current Events There have been a couple of new developments published recently. One is about the St. Joseph's Hospital in Phoenix, Arizona, where children can now get transcranial Doppler ultrasounds. Here is the link. The other is about a breakthrough in stem cells curing mice of Sickle Cell Anemia. Here is the link.  Image from: www.scienceprogress.org/.../ Genetics   Punnent squares by Carina Salaiz The base adenine got switched to the base thynine, creating the amino acid valine, instead of glutamic acid. Original image from: library.thinkquest.org/17109/diseases.htm Edited by: Carina Salaiz  A Day in the Life This is a slide show of a day in the life of a woman named Sarah. She has the same general schedule as someone without Sickle Cell Anemia, except, she might experience pain and regular checkups with a doctor. Sometimes, Sarah feels upset or in pain because her condition limits her physical activities and she has to spend a lot of her time treating it. But mostly, she doesn't want her kids to go through the same pain she does. Most of the time, she is grateful of her supportive family and the improving technology that could possibly cure Sickle Cell Anemia. Caring for small children with Sickle Cell Anemia is a difficult job. Have your child tested immediately if there's a chance they might have this disease. The next step is to talk to your doctor about preventions. Regular checkups with him or her are vital because they can inform you about various procedures, like transcranial Doppler ultrasounds, that could save your child's life. Learn as much as possible about this condition so you will be able to tell when your child is in pain and how bad it is. A daily dose of penicillin is needed for most children up to five years of age.[1]  Transcranial Doppler ultrasounds are recommended to check the blood flow of the brain. Image from: www.ercim.org/.../Ercim_News/enw60/salvetti.html Vocabulary Anemia- a deficiency of red blood cells or hemoglobin Hemoglobin- the oxygen-carrying pigment of red blood cells Sickle-shape- crescent shape Sickle cell crisis- a sudden pain when blood vessels get blocked Chronic- having long duration Acute- brief and severe NSAIDs- medicines such as aspirin or ibuprofen Narcotics- an addictive drug that reduces pain Folate- a salt or ester of folic acid, which is a water soluble vitamin essential to the human body Bonus  Dr. Beth Pletcher Image courtesy of Health Link, the university hospital I interviewed Dr. Beth Pletcher on a career in genetics and pediatrics. Please skip the first 55 seconds of the interview because we had a hard time starting.....please. Bibliography American Academy of Pediatrics, Pediatrics. First. Elk Grove Village, Illinois: American Academy of Pediatrics, 1948. CPO Focus on Life Science. First. Nashua, New Hampshire: Delta Education LLC, 2007. Golonka, Debbie. "Sickle Cell Disease." North Shore. 19 January 2007. Health Wise. 19 Jan 2009 <http://www.northshore.org/healthresources/encyclopedia/encyclopedia.aspx?Version=&DocumentHwid=hw254173>. "Sickle Cell Anemia." Sickle Cell Anemia. August 2008. National Heart Lung and Blood Institute. 15 Dec 2008 <http://www.nhlbi.nih.gov/health/dci/Diseases/Sca/SCA_WhatIs.html>. Sickle Cell information Center. 25 September 2003. 19 Jan 2009 <http://www.scinfo.org/toc.htm>. References [1] -"Sickle Cell Anemia." Sickle Cell Anemia. August 2008. National Heart Lung and Blood Institute. 15 Dec 2008 <http://www.nhlbi.nih.gov/health/dci/Diseases/Sca/SCA_WhatIs.html>. [2] - CPO Focus on Life Science. First. Nashua, New Hampshire: Delta Education LLC, 2007 |
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