Pathophysiology of Sickle Cell Anemia

Sickle cell disease (SCD) is health condition that often involves the continued production of abnormal red blood cells (Connes et al., 2018). The red blood cells produced in the body tend to have a sickled appearance hence giving rise to the name, sickle cell anemia. In addition to that, people with sickle cell anemia tend to have different chronic and acute health complications. The symptoms vary in severity and occurrence. In the acute phase of sickle cell anemia, individuals tend to experience pain that ranges from moderate to intense. The atypical red blood cells hinder the blood vessels in the body hence creating a situation referred to as a vaso-occlusive crisis. SCD is common in African American males. According to Renoux et al. (2016), 1 in every 400 live births among African Americans has SCD. Most of the patients diagnosed with this condition reside in impoverished areas. Therefore, this paper discusses the pathophysiology of sickle cell anemia and analyzes it in the discussion section, which identifies the need for further research on the disease.

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Pathophysiology of Sickle Cell Anemia among African American Male Adolescents

The deformed shape of the red blood cells in individuals with sickle cell anemia causes the hemoglobin to induce damage to the membrane of the red blood cells. Thus, this leads to an influx in calcium in the cell. As Adeyemo et al. (2018) argued, calcium influx often results in crosslinking of the proteins of the membranes and activates channels that allow for efflux of water and potassium from the cells. The result is the dehydration of the sickle-shaped red blood cells. A situation referred to as a vaso-occlusive crisis tends to result when sickle cells obstruct and reduce the flow of blood in the body. Primarily, this leads to necrosis, ischemia, and intense pain (Adeyemo et al., 2018). Repeated occurrences of the vaso-occlusive crisis could lead to bone infarction and necrosis. With time, the bone marrow of a patient could degenerate over time.

In most cases, long bones are frequently affected when an individual has sickle cell anemia. Besides, the structure of the bone marrow of the ribs and vertebral bodies are changed significantly. Some of the life-threatening conditions resulting from the infarction of the bone marrow are pulmonary fat embolism and Acute Chest Syndrome (ACS). In addition to that, hemoglobin released from the sickle-shaped red blood cells readily binds and depletes, leading to contraction of cell muscles and platelet aggregation. Vaso-occlusive crisis leads to hypoxia within the tissue, thus setting the right conditions for a vicious cycle that encourages further hypoxia and sickling.

Additionally, sickle cells are often weak; hence are prone to intravascular hemolysis (Renoux et al., 2016). Hemolysis is a term that refers to the obliteration of red blood cells. Intravascular hemolysis is a state when the red blood cells die due to the destruction of the cell membrane. With time, the red cells in the blood reduce in number leading to extravascular hemolysis. Although the bone marrow tries to recompense for the destruction by increasing the number of red blood cells produced, the production rate cannot match the extent of the destruction. It is worth noting that healthy red blood cells survive in the body for 90-120 days. A sickle-cell, on the other hand, persists for about 10-20days. Cholelithiasis is one of the complications that result from the excessive production of bilirubin. Bilirubin is a yellow compound often released by the liver and helps in giving color to feces. The production of bilirubin increases with the destruction of red blood cells.

Another common problem facing people with sickle cell anemia is splenic sequestration. According to Connes et al. (2018), splenic sequestration often happens when sickle-shaped red blood cells are trapped in the spleen. In consequence, the spleen enlarges and gets damaged, which inhibits its normal functioning. Splenic sequestration leads to congestion of the spleen. The overall immunity of a victim lowers. Notably, the spleen is essential for macrophage phagocytosis of bacteria. With this in mind, patients with SCD are prone to bacteremia with pathogens that lead to diseases such as pneumonia, influenza, and meningitis. The pathogens could cause life-threatening conditions to patients with sickle cell anemia. Asides from that, viruses affecting the respiratory system are frequent among individuals with SCD due to the damage of the bones and the poor functioning of the spleen. Continued dysfunction of the spleen leads to a condition referred to as autosplenectomy, a condition characterized by bone infarction and loss of the functional nature of the spleen. Such conditions create different complications among people with SCD.

Lastly, some neurological complications could result among people with SCD. Some complications include strokes (Ataga et al.,2012). Strokes emanate from the effects of acute chest syndrome, which is the appearance of pulmonary symptoms such as hypoxia, fever, and chest pains. Additionally, some cardiac complications, such as enlargement of the heart chambers, result due to chronic sickle cell anemia. Also, Ataga et al. (2012) indicated that myocardial infarction could occur from the vas-occlusion of the coronary heart arteries. In this section, one can see the progress of the disease among patients. When left untreated, patients with SCD could develop neurological and heart complications that may eventually result in death.

Discussion

Having discussed the pathophysiology of sickle cell anemia that is prevalent among African American male adolescents, one can see the need for urgent measures and solutions to this health issue. From the pathophysiology, it is articulate that adolescents with the condition cannot continue with their studies and have to concentrate on getting the desired treatment. Failure to treat the disease could result in further complications, as seen in the section above. According to Ballas (2015), people born with SCD tend to show signs of the disease during the first year of their lives, especially from 5months and above. In most cases, symptoms of the ailment vary from mild to severe, depending on an individual. SCD, however, worsens with time. Importantly, most of the male adolescents with the condition had it from birth, but symptoms developed later in life. Research is required to ensure that early diagnosis of the state is made, which will assist in early detection of SCD hence preventing any form of complications and infections that might result when the disease progresses.

Further, this study indicates that pain is one of the acute and intermittent complications of the ailment. The pain can be debilitating in most occasions. As Ballas (2015) argued, most adolescents with SCD are likely to associate pain with psychosocial influences. Most young people are unaware of the pathophysiology of SCD. With this mind, most people are likely to ignore some severe symptoms that could show the development of further complications and instead assume that they are routine stressors of SCD. Therefore, awareness of the disease is an essential strategy that would help to ensure smooth management of the drug among African American male adolescents.

Conclusion

To conclude, sickle cell anemia is a condition characterized by the production of atypical red blood cells. Mainly, this paper discusses the pathophysiology of SCD. SCD is prevalent among African American male adolescents. As the disease advances in one's body, different complications result, such as infection-causing bacteria, cardiac, and neurological complications, to mention a few. The lack of awareness of the symptoms and progress of the disease among male adolescents from the African American community are contributing factors that worsen the condition among the victims. Therefore, this paper proposes the need for awareness and further research on early diagnosis of the disease to minimize the extent of complications.

References

Adeyemo, T. A., Ojewunmi, O. O., Oyetunji, I. A., Rooks, H., Rees, D. C., Akinsulie, A. O., ... Menzel, S. (2018). A survey of genetic fetal-haemoglobin modifiers in Nigerian patients with sickle cell anaemia. PLoS ONE, 13(6), 1-10. https://doi.org/10.1371/journal.pone.0197927

Ataga, K. I., Brittain, J. E., Desai, P., May, R., Jones, S., Delaney, J., ... Key, N. S. (2012). Association of coagulation activation with clinical complications in sickle cell disease. PLoS ONE, 7(1), 1-9. https://doi.org/10.1371/journal.pone.0029786

Ballas, S. (2015). Emerging therapies targeting the pathophysiology of sickle cell disease, an issue of hematology/oncology clinics. European Journal of Haematology, 94(1), 92. https://doi.org/10.1111/ejh.12472

Connes, P., Renoux, C., Romana, M., Abkarian, M., Joly, P., Martin, C., ... Connes. (2018). Blood rheological abnormalities in sickle cell anemia. Clinical Hemorheology & Microcirculation, 68(2-3), 165-172. https://doi.org/10.3233/CH-189005

Renoux, C., Romana, M., Joly, P., Ferdinand, S., Faes, C., Lemonne, N., ... Martin, C. (2016). Effect of age on blood rheology in sickle cell anaemia and sickle cell haemoglobin c disease: a cross-sectional study. PLoS ONE, 11(6), 1-11. https://doi.org/10.1371/journal.pone.0158182