A Brief Description of Sickle Cell Anemia

Dwayne Adams Instructor: Croshaw Medical Terminology 1 18, April 2013 Sickle Cell Anemia Sickle-cell Anemia is a genetic blood disorder caused by the presence of an abnormal form of hemoglobin molecules in which the red blood cells loose their disc-shape and become crescent shaped. The shape also known as “hemoglobin S”. unlike normal red cells which are usually smooth and malleable, tend to collect after releasing oxygen, and cannot squeeze through small blood vessels. The organs are then deprived of blood and oxygen.

The basic life-span of an affected cell is generally from 1. 5 to about 3 weeks, which represents approximately 10% to 20% of a normal cell’s life. Because they cannot be replaced fast enough, the blood is chronically short of red cells. This results in abnormal functioning and breakdown, causing episodes of re-occurring pain, discomfort, and damage to vital organs, causing Anemia. It is not contagious, nor can it be transmitted through contact. It can only be caught through birth, and must be inherited from both parents for the illness to occur in children.

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A child with only one copy of the gene may have sickle-cell traits but no symptoms of illness. Historically, Sickle Cell Anemia has only affected people who originate from areas of Africa, parts of India, the Mediterranean, South and Central America, the Caribbean, or descendants of these groups. About 2,000 babies are born with sickle cell disease each year in the United States. Symptoms of this condition include, severe pain, anemia, chest pain and difficulty breathing, strokes, joint or arthritis pain, bone infarctions, and blockage of blood flow in the spleen or liver.

These are sometimes followed by severe infections, fatigue, paleness, rapid heart rate, shortness Adams 2 of breath, yellowing of the eyes and skin. Younger children with sickle cell anemia have attacks of abdominal pain. Other symptoms may occur like painful and prolonged erections (priapism), poor eyesight or blindness, problems with thinking or confusion caused by small strokes, and ulcers on the lower legs (in adolescents and adults). Over time, the spleen no longer works. As a result, people with sickle cell anemia may have symptoms of infections such as: one infection (Osteomylitis), Gallbladder infection (Cholecystitis), lung infection (Pneumonia), and Urinary tract infection. Other symptoms include, delayed growth and puberty, painful joints caused by arthritisPatients with sickle cell disease develop severe pain in the chest, back, arms, legs, and abdomen. Pain can occur anywhere in the body. Sickle red blood cells in the lungs can cause severe illness with chest pain, fever, and difficulty breathing. Sickle cell disease can also cause permanent damage to the brain, heart, kidneys, liver, spleen, and bones.

The severity and symptoms vary greatly from person to person, even within the same family. Patients with sickle cell disease need ongoing treatment, even when they are not having a painful crisis. It is best to receive care from health care providers and clinics that take care of many patients with sickle cell anemia. Treatments have vastly improved over the years, with new donations increasing slightly over the course of the past twenty years. Many healthcare providers have found specific measures to be rather effective, for example, Folic acid supplements should be taken because it s needed to make new red blood cells. Treatments may include blood transfusions (may also be given regularly to prevent stroke), pain medicines, and plenty of fluids. Other treatments for sickle cell anemia may include Hydroxyurea (Hydrea), a medicine that may help reduce the number of pain Adams 3 episodes (including chest pain and difficulty breathing) in some people, or antibiotics to prevent bacterial infections, which are common in children with sickle cell disease.

There are additional treatments that may be needed to manage complications of sickle cell anemia, like dialysis or a kidney transplant for kidney disease, counseling for psychological complications, Gallbladder removal in people with gallstone disease, and hip replacement for avascular necrosis of the hip. In certain severe cases, surgery for eye problems, treatment for overuse or abuse of narcotic pain medicines, and wound care for leg ulcers have been necessary. At other times depending on severity, bone marrow or stem cell transplants have been performed to cure sickle cell anemia, but are normally not options for most patients.

Sickle cell anemia patients are often unable to find well-matched stem cell donors. Although there are a wide range of medical generalists and specialists, clinics, and agencies that can treat the various areas regarding Sickle-cell disease, personal education concerning its ramifications are advised. People with sickle cell disease must reduce their risk of infections. This includes receiving certain vaccinations, among which are Haemophilus influenzae vaccine (Hib), Pneumococcal conjugate, and polysaccharide vaccine.

At different times other procedures may be found to be beneficial including common lab tests such as CBC and retic counts, blood chemistry values, urinalysis and urine protein, Ferritin, and hemoglobin electrophoresis. After any general battery of test may come the necessity for different therapies to further combat any progression. Some effective are fluid therapy consisting of glucose, and or water, Transfusion therapy, Chelation therapy for iron removal, ultrasound, echo-cardiograms,pulmonary function tests. Also , hearing tests, TCD’s, CT and MRI scans, x-rays, and bone scans. Adams 4

There is no cure for Sickle-cell Disease and adults with the trait are wise to thoroughly consider the possible effects of procreating with another person with the disease. As in other areas of modern healthcare, advances in technology have facilitated its early detection. Prenatal tests include Amniocentesis, and chorionic villus sampling. Fortunately some countries require newborn screening for hemoglobin problems, including Sickle-cell Anemia making the early detection and preventive measures possible There exists much research and numerous clinical trials to examine possible causes.

There unfortunately seems to exist somewhat of a hesitancy by the generally affected groups to participate in any abative efforts. The general consensus is one of feelings of mistrust of government and science in general. Furthering the knowledge of, and finding funding are at present the focus of several major organizations. Further information can be found in literature, and on the websites of organizations such as the National Institutes of Health (NIH), the American Sickle Cell Anemia Association (ASCAA), the American Society of Hematology, the National Heart, Lung, and Blood Institute (NHLB), and many other groups.

Adams 5 Works Cited National Institutes of Health, National Heart, Lung, and Blood Institute, Division of Blood Disease and Resources, The Management of Sickle Cell Disease, No. 02-2117, 1984, 1989, 1995, 1999, June 2002 (fourth Ed. ) Platt, Allan F. Jr. P. A. -C, M. M. Sc. , et al. J. E. , L. H. Hope and Destiny; The patient Guide to Sickle-cell Disease and Sickle-cell Trait, Revised Third Edition. Hilton, 2011. Print. www. medterms. com/script/main/art. asp? article key=9368, n. d. Web. 20 Apr. 2013. www. nim. nih. gov/medlineplus/ency/article/000527. htm, n. d. Web. 20 Apr. 2013.

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