Thalassemia is a blood disorder that causes mild or severe anemia (Antonio, 1993).
This happens when people do not have enough hemoglobin and is caused by the inheritance of a defective gene (Nathan, 1998).
Thalassemia is passed on from a parent to children through their genes (Simon, 1999).
The name thalassemia comes from the Greek word “thalassa” meaning “the sea” because the condition was first described in populations living near the Mediterranean Sea (Nathan, 1998).
However, the disease is also prevalent in Africa, Middle East, South Asia, and pacific descent (Simon, 1999).
Moreover, thalassemia causes an immense disruption in the hemoglobin synthesis (Nathan, 1998).
Although, there are two different types of thalassemia, alpha, and beta thalassemia they both affect the body.
Alpha-thalassemia occurs most commonly in people from Southeast Asia and China (Ghosh, 1994).
Its is caused when the hemoglobin does not produce enough alpha protein (Ghosh, 1994).
The most severe form of alpha thalassemia causes (stillbirth, and death of fetus before delivery) (Ghosh, 1994).
Whereas, beta thalassemia, is caused by a mutation in the beta globin chain (Nathan, 1998).
Genes from both parents must be inherited to acquire the major form of this disease (Simon, 1999).
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In the popular science book, The Selfish Gene, Richard Dawkins explains his theory of evolution, and its occurrence for the good of the gene rather than the good of the species or individual. Dawkins believes the gene to be practically immortal, carrying on generation upon generation. By calling them selfish Dawkins describes a genes strategy of competition for survival, its tendency to behave in ...
In beta thalassemia major children are normal at birth, but develop anemia during the first year of life (Simon, 1999).
The alpha and beta thalassemia’s are distinct diseases that affect the body in different ways. Therefore, the symptoms of thalassemia, which are paleness, tiredness, dark urine, and spleen enlargement all, depend on the type and severity of the disease ( Simon, 1999).
Furthermore, Thalassemia may be suspected if an individual shows signs that are suggestive of the disease. In all cases, however, laboratory tests are important to confirm the exact diagnosis. In addition, the following tests are used to determine thalassemia: complete blood test (identifies level of hemoglobin, and red blood cells), family genetic studies (blood tests of family members), and prenatal testing, which can determine if an unborn baby has thalassemia (Antonio/ Shafer, 1993/2000).
Moreover, Hemoglobin electrophoresis is also an important test to diagnose thalassemia (Nathan, 1998).
Nevertheless, Individuals with beta thalassemia major receive regular blood transfusions, usually on a monthly basis (Simon, 1999).
This helps prevent severe anemia and allow for more normal growth and development (Simon, 1999).
Patients who receive significant numbers of blood transfusions require therapy to remove iron from the body, called chelation therapy (Nathan, 1998).
An additional medication called deferoxamine is administered, that binds to the excess iron, and helps eliminate iron through urine (Nathan, 1998).
On the other hand, there is another treatment known as bone marrow transplantation from identical siblings (Nathan, 1998).
This is a very dangerous and expensive treatment yet, if it is successful, it can cure beta thalassemia (Nathan, 1998).
Besides, bone-marrow transplantation, and blood transfusion, thalassemia (during pregnancy) can be cured by stem cell transplantation (Teresa, 2003).
Lastly, the prognosis of thalamessia includes survival, death or chances of complication. Therefore, in case of blood transfusion, most death between 12 and 24 years of age is because of iron overload (Maren, 2000).
Also, recent studies testify 90% of patients on the drug deferoxamine survived without heart disease (Antonio, 1993).
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Sickle-Cell Anemia is a genetic blood disorder caused by the presence of an abnormal form of hemoglobin. These hemoglobin molecules tend to aggregate after unloading oxygen forming long, rod-like structures that force the red cells to assume a sickle shape. Unlike normal red cells, which are usually smooth and malleable, the sickle red cells cannot squeeze through small blood vessels. When the ...
Moreover, from 18 patients that had deferoxamine for about 4. 5 years, 7 had risk of cardiac disease, and early death (Antonio, 1993).
In conclusion, bone marrow or stem cell transplantation are cure for thalassemia but not in all cases for this reason, scientists are working on better ways to remove excess iron from the body in order to prevent iron overload, and to cure thalassemia for everyone.
Works Cited
Cao, Antonio. (1993, Nov/Dec).
Preventing a genetic disease, P. 26. Retrieved september 24, 2006 from EBSCO data (general science) on the World Wide Web: http://search.ebscohost.com/login.aspx?direct=true&db=hxh&AN=9404062882&site=ehost-live
Dyson, Simon. (2005, May 27).
Thalassemia Screening, P. 17-20. Retrieved October 17th, 2006 from EBSCO data (General Science) on the World Wide Web:
http://search.ebscohost.com/login.aspx?direct=true&db=afh&AN=17388721&site=ehost-live
Ghosh, A. (1994, Aug. 10).
Ultrasound measurement of placental thickness to detect pregnancies affected by homozygous alpha thalassemia, P. 988. Retrieved October 17th, 2006 from EBSCO data (General Science) on the World Wide Web: http://search.ebscohost.com/login.aspx?direct=true&db=gsh&AN=9411032635&site=ehost-live
Khan, Maren. ( 2000, Oct. 6).
Survival in …-thalassaemia major in the UK: data from the UK Thalassaemia Register, P. 2051. Retrieved October 19th,2006 from EBSCO data (General Science) on the World Wide Web: http://search.ebscohost.com/login.aspx?direct=true&db=gsh&AN=3203602&site=ehost-live
Lawrence, David. (2002, June 15th).
Stabilising protein could alleviate blood disorders, say US researchers, P. 2093. Retrieved October 20th, 2006 from the EBSCO data (General Science) on the World Wide Web: http://search.ebscohost.com/login.aspx?direct=true&db=gsh&AN=6813434&site=ehost-live
Nathan, David G. (1998, July).
Prospective on thalassemia, P. 281. Retrieved Octrober 18th, 2006 from EBSCO data (General Science) on the World Wide Web: http://search.ebscohost.com/login.aspx?direct=true&db=hxh&AN=847457&site=ehost-live
Parker, Shafer. (2000, Jan. 24).
The deferiprone furor, P. 14. Retrieved October 18th, 2006 from EBSCO data (General Science) on the World Wide Web: http://search.ebscohost.com/login.aspx?direct=true&db=rch&AN=2719053&site=src-live
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Tuveri, Teresa. (2003, May 7).
Fetal HLA typing in Δ thalassaemia, P. 41. Retrieved October 1st, 2006 from EBSCO data (General Science Collection) on the World Wide Web: