Assignment: Hereditary Spherocytosis

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Assignment: Hereditary Spherocytosis

Assignment: Hereditary Spherocytosis


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Hereditary Spherocytosis

Anabella Caradonna

West Coast University

The long term outlook of people with hereditary spherocytosis is excellent in terms of treatment. The management and treatment of the condition rely on the severity of the disease. At the mild stage, a person may not have any symptoms unless the environment triggers such signs. In most cases, there is no therapy provided other than monitoring and managing symptoms related to anemia and other signs. Adequate data related to life expectancy about the condition seems to be limited. According to Manciu, Matei, & Trandafir (2017)” In all people who undergo splenectomy, there is a lifelong, increased risk of developing a life-threatening infection (sepsis). Although most septic episodes have been observed in children whose spleens were removed in the first years of life, older children and adults also are susceptible. Fortunately, taking certain precautions can reduce this risk and can prevent minor infections from becoming life-threatening” (p. 110-116)

Hereditary spherocytosis is an illness that adversely affects red blood cells. Individuals suffering from hereditary spherocytosis usually experience a shortage of red blood cells that results in the condition known as anemia. According to Manciu, Matei and Trandafir (2017) “Hereditary spherocytosis occur in 1 in 2,000 individuals of Northern European ancestry. This condition is the most common cause of inherited anemia in that population. The prevalence of hereditary spherocytosis in people of other ethnic backgrounds is unknown, but it is much less common” (p. 110-116). Infants are adversely affected by this condition; however, at the age of 1, the condition begins fading away. Some of the signs related to the state include yellowish of an eye and even skin. The patient also experiences an enlarged spleen a condition that is commonly referred to as splenomegaly. While some symptoms fade away after the newborn baby is born, some states such as splenomegaly persist even in adulthood.

More than half of individuals affected by the disease develop hard deposits in the gallbladder known as the gallstones which present from late childhood to adulthood. According to the Manciu, Matei, & Trandafir (2017) “There are four forms of hereditary spherocytosis, which are distinguished by the severity of signs and symptoms. They are known as the mild form, the moderate form, the moderate/severe form, and the severe form” (p. 110-116). The study also holds that 20 to 30 per cent of individuals have what is known as the mild form of hereditary addition it is also estimated that 60 to 70 per cent of people are suffering from the moderate form of genetic spherocytosis.10% of patients are also suffering from moderate/severe form of hereditary spherocytosis and only three to five per cent of people are suffering from severe form of hereditary spherocytosis.

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