Sickle cell anemia

reaping hook mobile ph whiz anaemiaAbstract sickle electric stall anemia is an inherited occupation disorder characterized primarily by inveterate anemia and periodic episodes of disquiet. The underlying problem involves haemoglobin, a component of tearing blood carrells. Hemoglobin molecules in for each one rubordish blood cellphone carry oxygen from the lungs to body organs and tissues and bring carbon dioxide prat to the lungs. In reap hook cell anemia, the hemoglobin is uncollectible. After hemoglobin molecules croak up their oxygen, round may cluster together and system long, rod-like structures. These structures come red blood cells to become stiff and assume a reap hook shape. Unlike normal red cells, which ar usu whollyy smooth and donut-shaped, reap hook red cells cannot squeeze through broken blood vessels. Instead, they stack up and cause block hop ons that deprive organs and tissues of oxygen-carrying blood. convention red blood cells experienc e about 120 days in the bloodstream, entirely reaping hook red cells die after about 10 to 20 days. Because they cannot be replaced turbulent equal, the blood is chronically short of red blood cells, a antecedent called anemia. Sickle cell anemia affects millions throughout the world. It is particularly harsh among lot whose ancestors come from Africa South America, Cuba, Central America Saudi Arabia India and Mediterranean countries much(prenominal) as Turkey, Greece, and Italy.Sickle cell anemia complaint The inherited haemoglobinopathies are a group of disorders that include thalassaemia and reaping hook-cell illness. These diseases are a major(ip) public health problem in the Mediterranean area, the Middle East, the Indian subcontinent, Asia, tropical Africa and the Caribbean. However, because of population flow, they are now widespread and occur in Europe and North and South America. According to the World Health Organization, the suppose estimates of affected i ndividuals indicate that 240 million throng are heterozygous for these disorders and at least 200000 lethally affected homozygotes are born annually, approximately equally divided up between sickle-cell anaemia and thalassaemia syndromes. This research papers about the definition, causes, types, signs and symptoms, complications, diagnosing and handling of sickle cell disease. too, this paper talks about the habitual guidelines to keep the sickle cell unhurried healthy and recommendation that the patient and families should cod for it.1-Definition Sickle cell anemia (uh-NEE-me-uh) is a sober disease in which the body makes sickle-shaped red blood cells. Sickle-shaped elbow room that the red blood cells are shaped like a C. Normal red blood cells are disc-shaped and look like doughnuts without holes in the center. They perish easily through your blood vessels. Red blood cells view as the protein hemoglobin (HEE-muh-glow-bin). This iron-rich protein gives blood its red colo r and carries oxygen from the lungs to the rest of the body. Sickle cells contain abnormal hemoglobin that causes the cells to redeem a sickle shape. Sickle-shaped cells dont start easily through your blood vessels. Theyre stiff and sticky and tend to form groups and get stuck in the blood vessels. (Other cells as well may play a role in this grouping process.) The groups of sickled cells block blood flow in the blood vessels that lead to the limbs and organs. Blocked blood vessels can cause pain, serious infections, and organ pervert.2- Causes Sickle cell anemia is an autonomic recessive communicable disorder caused by a defect in the HBB gene, which codes for hemoglobin. The presence of 2 defective genes (SS) is needed for sickle cell anemia. If each parent carries one sickle hemoglobin gene (S) and one normal gene (A), each child has a 25% line up of acquire two defective genes and having sickle cell anemia a 25% chance of inheriting two normal genes and not having the d isease and a 50% chance of be an unaffected carrier like the parents. Two of the well-nigh common variations of the sickle cell gene areA- Sickle cell property A person with the sickle cell trait is carrying the defective gene, but also has close to normal hemoglobin. Individuals with sickle cell trait are usually without symptoms of the disease. Mild anemia may occur. Under intense trying conditions, exhaustion, hypoxia (low oxygen), and/or severe infection, the sickling of the defective hemoglobin may occur and ensue in some complications associated with the sickle cell disease.B- Sickle cell anemia A person with sickle cell anemia has most or all of the normal hemoglobin replaced with the sickle hemoglobin. It is the most common and most severe form of the sickle cell variations. These individuals suffer from a transmutation of complications due to the shape and thickness of the sickle cells. Due to the decreased cast of hemoglobin cells circulating in the body, severe an d chronic anemia is also a common characteristic.3- Sign and Symptoms The clinical course of sickle cell anemia does not follow a single pattern some patients have mild symptoms, and some have very severe symptoms. The canonic problem, however, is the same the sickle-shaped red blood cells tend to get stuck in narrow blood vessels, blocking the flow of blood. These results in the following conditions A- Hand-foot syndrome When small blood vessels in hands or feet are impede, pain and lubber can result, along with fever. This may be the first symptom of sickle cell anemia in infants. Fatigue, paleness, and shortness of breath These are all symptoms of anemia or a shortage of red blood cells. B- wound that occurs suddenly in any body organ or correlative A patient may experience pain wherever sickle blood cells block oxygen flow to tissues. The frequency and heart of pain vary. Some patients have painful episodes (also called crises) less than once a year, and some have as many as 15 or more(prenominal) episodes in a year. Some meters pain lasts only a few hours sometimes it lasts several weeks. For severe continuous pain, the patient may be hospitalized and treated with pain sweep awayers and intravenous fluids. Pain is the principal symptom of sickle cell anemia in both children and adults.C -Eye problems The retina, the film at the back of the eye that bids and processes visual images, can deterio rank when it does not get enough nourishment from circulating red blood cells. Damage to the retina can be serious enough to cause blindness.D- Yellowing of skin and eyes These are signs of jaundice, resulting from quick breakdown of red blood cells. Delayed growth and puberty in children and often a slight build in adults The slow rate of growth is caused by a shortage of red blood cells4- Complications A-Infections In cosmopolitan, both children and adults with sickle cell anemia are more nonresistant to infections and have a harder time fighting them o ff. This is the result of spleen damage from sickle red cells, hence holding the spleen from destroying bacteria in the blood. Also the bone marrow gets enlarged because of the change magnitude need to produce red blood cells. Infants and young children especially are suspectible to bacterial infections that can kill them in as little as 9 hours from onset of fever. pneumococcal infections used to be the principal cause of death in children with sickle cell anemia until physicians began routinely giving penicillin on a prophylactic basis to those who are diagnosed at birth or in archaean infancyB- Stroke Defective hemoglobin damages the walls of red blood cells, causing them to stick to blood vessel walls. The resulting narrowed or blocked small blood vessels in the brain can lead to serious, unsafe strokes, primarily in children.C-Acute breast syndrome Similar to pneumonia, this life-threatening complication is caused by infection or trapped sickle cells in the lung. It is c haracterized by breast pain, fever, and an abnormal chest X-ray.5- Diagnosis Early diagnosis of sickle cell anemia is critical so children who have the disease can receive proper treatment.Blood test More than 40 states now coiffe a simple, inexpensive blood test for sickle cell disease on all newborn infants. This test is performed at the same time and from the same blood samples as other routine newborn-screening tests. Hemoglobin ionophoresis is the most widely used diagnostic test. If the test shows the presence of sickle hemoglobin, a second blood test is performed to confirm the diagnosis. These tests also sort whether or not the child carries the sickle cell trait.6- Treatment Although thither is no cure for sickle cell anemia, doctors can do a great deal to help patients, and treatment is constantly being improved. radical treatment of painful crises relies heavily on painkilling drugs and oral and intravenous fluids to tighten pain and check complications.A- Blood Tr ansfusions Transfusions correct anemia by increasing the number of normal red blood cells in circulation. They can also be used to treat spleen enlargement in children to begin with the condition becomes life-threatening. Regular transfusion therapy can help prevent come about strokes in children at high risk.B-Oral Antibiotics Giving oral penicillin twice a day beginning at 2 months and continuing until the child is at least 5 years old can prevent pneumococcal infection and first death. Recently, however, several new penicillin-resistant strains of pneumonia bacteria have been reported. Since vaccines for these bacteria are ineffective in young children, studies are being planned to test new vaccines.C-Hydroxyurea The first effective drug treatment for adults with severe sickle cell anemia was reported in early 1995, when a study conducted by the National Heart, Lung, and Blood Institute showed that routine doses of the anticancer drug hydroxyurea reduced the frequency of pain ful crises and acute chest syndrome. Patients taking the drug needed less blood transfusions. Regular health maintenance is critical for people with sickle cell anemia. Proper nutrition, true hygiene, bed rest, protection against infections, and avoidance of other stresses all are heavy in maintaining dandy health and preventing complications. Regular visits to a physician or clinic that provides comprehensive care are necessary to identify early changes in the patients health and ensure immediate treatment. Today, with good health care, many people with sickle cell anemia are in reasonably good health much of the time and living productive lives. In fact, in the past 30 years, the life expectancy of people with sickle cell anemia has increased. What can be done to help prevent these complications? Sickle cell patient should be under the care of a aesculapian team that understands sickle cell disease. All newborn babies detected with sickle cell disease should be placed on daily penicillin to prevent serious infections. All of the childhood immunizations should be given in appurtenance to the pneumococcal vaccine. Parents should know how to check for a high temperature because this signals the need for a quick medical checkup for serious infection. The following are general guidelines to keep the sickle cell patient healthy Taking the vitamin folic bitter (foliate) daily to help make new red cells Daily penicillin until age six to prevent serious infection Drinking plenty of water daily (8-10 glasses for adults) Avoiding too hot or too bleak temperatures Avoiding over exertion and stress Getting a sufficient amount of rest Getting regular check-ups from knowledgeable health care providers Patients and families should watch for the following conditions that need an immediate medical evaluation Fever thorax pain Shortness of Breath Increasing tiredness Abdominal pomposity Unusual headache Any sudden weakness or bolshy of feeling Pain tha t will not go away with fundament treatment Pianism (painful erection that will not go down) Sudden sight change.Conclusion To conclude, sickle cell anemia like other chronic life-threatening disease can cause this inherited to patient and family members connectedness where members share common experiences and problems can reveal this situation because it gets better mind and management of the disease. There is no cure, therefore nurses should understand the actions that can prevent or relieve symptoms in order to meet the challenges of caring for patients with sickle cell disease and helping them to minimize its effect on their lives.References Brunner and Suddarth , Medical-Surgical Nursing, (2004), tenth edition http//www.nhlbi.nih.gov/health/dci/Diseases/Sca/SCA_Summary.html http//www.mayoclinic.com/health/sickle-cell-anemia/DS00324 http//www.nhlbi.nih.gov/health/dci/Diseases/Sca/SCA_WhatIs.html http//www.kidshealth.org/teen/diseases_conditions/genetic/sickle_cell_anemia. html http//www.medicinenet.com/sickle_cell/article.htm Desai, D. V. Hiren Dhanani (2004). Sickle Cell Disease History And Origin. The Internet Journal of Heamatology 1 (2). http//www.ispub.com/ostia/index.php?xmlFilePath=journals/ijhe/vol1n2/sickle.xml. Pearson H (Aug 1977). Sickle cell anaemia and severe infections due to encapsulated bacteria http//www.nlm.nih.gov/medlineplus/meningitis.html. http//www.emro.who.int/Publications/EMHJ/0303/03.htm