Sickle Cell Anemia, Research Paper Example

Article 1

Framing the research agenda for sickle cell trait: building on the current understanding of clinical events and their potential implications by Jonathan C. Goldsmith, Vence L. Bonham and Martin H. Steinberg (2012).

Summary

These researchers realized that the ‘Sickle Cell Trait (HbAS), the heterozygous state for the sickle hemoglobin beta globin gene’ (Goldsmith, Bonham & Steinberg, 2012, p 340) was prevalent among approximately 100 million people. Quoting world health organization data researchers confirmed that this estimate represented 25% of the world’s population.It was also discovered during their studies that people with HbAS during their early childhood demonstrated falciparum malaria infection resistance. Consequently, world health organization has expressed further concerns that these individuals with HbAS within malarialendemic African regions have a greater survival rate over individuals with affected by HbAA (Goldsmith, 2012).

Further reports emerging from sources in the United States of America highlight possible health risks for persons with HbAS. These include malignancy, increased incidence; thromboembolism; renal failure; splenic infarction; sudden death associated with exertion and highaltitude complication.As such, a workshop was convened in Maryland during June 3–4, 2010 with the caption Framing the Research Agenda for Sickle Cell Trait, to review the clinical manifestations of HbAS.Exercise-related sudden death reports in HbAS were high on the agenda.the public health, ethical and societal implications were fully discussed with the aim of understanding relationships among the phenomenon. Precisely, researcher sought to identify potential research questions since numerous knowledge exist in the science regarding sickle cell anemia and malaria(Goldsmith, 2012).

Article 2

Vasculopathy in Sickle Cell Disease: Biology, Pathophysiology, Genetics, Translational Medicine and New Research Directions. by Kato, G. Hebbel, R., & Gladwin, M. (2009).

Researchers in this article advanced that the characteristics of sickle cell disease include a one amino acid molecular hemoglobin disorder. From this feature the pathological polymerization emerges. Researchers are still not sure of how the disease progresses and believe that with more money expended towards through investigations cures could be effects and patient’s quality of life improved. This encompasses consequent red cell rigidity. Subsequently, inadequate microvascular blood flow occurs accompanied by tissue ischemia and infarction. Recently, an independent spectrum of blood vessel pathophysiology function was discovered identifying the development of abnormal blood vessel vascular toneaccompanying adhesive endothelium activation (Kato, Hebbel & Gladwin, 2009).

Further, these abnormal vasculopathic developments were attributedto hemolysis- associated pathways defects which involved‘oxidative stress,nitric oxide bioavailability;hemostatic activation, ischemia-reperfusion injury and leukocytes and platelets dysfunction’ (Kato et.al, 2009, p. 618). Sickle cell disease vasculopathy has been also been linked to the stroke; pulmonary hypertension, priapism and ulcerationof the lower leg. Primarily it depends on the severity of hemolytic activity. These researchers have also associated vasculopathy in patients with sickle cell disease with chronic organ dysfunction(Kato et.al, 2009).

These perspectives were deliberations created by a panel of experts engaged in a National Institutes of Health in August 2008 workshop exploring sickle cell disease and its vasculopathy implications. The workshop sessions embraced numerous questions which can later b researched to enhance understanding of sickle cell disease progression. Arguments have been in the past that enough money has not been expended on sickle cell research for a cure to be realized. The vulnerable African American population and blacks within African diaspora have struggled with this disease for ages (Kato et.al, 2009).

References

Goldsmith, J. Bonham, V., & Steinberg, M (2012).Framing the research agenda for sickle cell trait: building on the current understanding of clinical events and their potential implications. American Journal of hematology. 87(3); 340-376

Kato, G. Hebbel, R., &Gladwin, M. (2009).Vasculopathy in Sickle Cell Disease: Biology, Pathophysiology, Genetics, Translational Medicine and New Research Directions. American Journal of hematology. 84(9) 618-625