Provide recommendations for alternative drug treatments and patient education strategies for treatment and management of Thalassemia fgolden.

Thalassemia is an inherited autosomal recessive blood disorder that is passed to children via mutated hemoglobin genes. This is most common in people who are African-American, of either Mediterranean and/or Southeast Asian ancestry. (Arcangelo, Peterson, Wilbur, & Reinhold, 2017) This causes clinically severe anemia due to an in an imbalance of either the a- or b- globin chains. In simple terms, there are fewer red blood cells in a person’s body therefore there is less hemoglobin which restricts the amount of oxygen can be carried throughout your body causing anemia leaving one very fatigued. (Taher, Weatherall, & Cappellini, 2018) There are four genes that are responsible for the production of a-globin chains and mutations of these can result in a-thalassemia and depending on the number of mutations a person will either be a silent carrier, have mild or even severe anemia. (Arcangelo et al., 2017) Any patient with even a more mild form of Thalassaemia will need to be monitored closely for iron deficiency. (ARC) Chronic transfusion is considered in patients when the hemoglobin concentration is consistently below 6–7 g/dL. (Chonat & Quinn, 2017) Unfortunately with chronic blood transfusions comes the high potential of iron overload to a person’s vital organs greatly increasing the chances of that person to fall into organ failure. (Taher et al., 2018) In order to avoid iron overload iron chelation therapy should be considered which is accomplished with deferoxamine, deferasirox, and deferiprone. First course of action should be determining a serum ferritin level baseline to check for effectiveness of the iron chelation therapy. First stage is to use Deferoxamine subcutaneous infused for up to 24 hours. This is followed up with Deferiprone 3 times per day. Deferasirox can be substituted for subcutaneous Deferoxamine used as a first-choice treatment as it comes in an oral liquid suspension. (Chonat & Quinn, 2017) Children who have Thalassaemia with require regular blood transfusions during their first year of life in order to achieve normal development. When this happens iron chelation therapy is not done in the normal time frame which would be within one year of transfusion. In the case of infants, the therapy is delayed beyond one year due to the toxicity of the iron chelation. (Carson & Martin, 2014) Much care and thought my be utilized to allow these infants to develop and grow into healthy adolescence and achieve puberty without unnecessary delay. References Arcangelo, V. P., Peterson, A. M., Wilbur, V., & Reinhold, J. A. (2017). Pharmacotherapeutics for Advanced Practice (4th ed.). Philadelphia, PA: Wolters Kluwer. Carson, S. M., & Martin, M. B. (2014). Effective iron chelation practice for patients with ß-thalassemia major. Clinical Journal of Oncology Nursing, 18(1), 102-111. http://dx.doi.org/10.1188/14.CJON Chonat, S., & Quinn, C. T. (2017). Current Standards of Care and Long Term Outcomes for Thalassemia and Sickle Cell Disease. Advances in experimental medicine and biology, 1013, 59-87. http://dx.doi.org/10.1007/978-1-4939-7299-9_3 Taher, A. T., Weatherall, D. J., & Cappellini, M. D. (2018). Thalassaemia. The Lancet, 391(10116), 155-167. http://dx.doi.org/10.1016/S0140-6736(17)31822-6


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