Hematologic Disorders: Sickle Cell Disease

Sickle cell disease (SCD) is defined as a genetic hematologic disease resulting from the production of hemoglobin S, resulting in the formation of rigid and elongated red blood cells that are sickle-shaped. The pathophysiology of SCD starts with the mutation in the hemoglobin beta gene that generates hemoglobin S, which, under hypoxic conditions, forms polymers that cause the red blood cells to rigid and assume a sickle shape. These sickle-shaped cells have a reduced life span and, therefore, cause recurrent chronic hemolytic anemia: Hematologic Disorders: Sickle Cell Disease.

The tendency of these sickle cells to adhere to the endothelial lining of blood vessels leads to vessel occlusion, tissue ischemia, and gradual organ damage. The patient may develop fatigue, jaundice, recurrent pain, and increased infection susceptibility in clinical manifestation. Diagnosis is based on techniques such as hemoglobin electrophoresis, while the treatment involves symptom management, the use of hydroxyurea to prevent the formation of sickle cells, blood transfusion, and, in severe cases, bone marrow transplantation (Ehsan & Maruvada, 2023).

Individualized Considerations

Genetic factors are crucial in SCD, as it is inherited in an autosomal recessive pattern. Infection, dehydration, and hypoxia are environmental conditions that may precipitate a crisis or worsen the disease. Awareness of these factors assists in patient care, particularly when an individual is developing a management plan that best suits the specific patient’s needs. Possible preventive measures include staying hydrated, getting regular vaccinations, and avoiding high-altitude areas to decrease the number of vaso-occlusive crises and hospitalizations (Obeagu & Obeagu, 2024).

Social Determinants of Health and SCD

Social determinants of health, including access to quality healthcare, socioeconomic status, and education, significantly impact patient outcomes. Lack of quality healthcare and adequate preventive measures lead to higher morbidity and mortality rates among individuals in underserved areas. Education of patients, prevention, and elimination of barriers to quality health are needed to enhance the lives of SCD patients. Policies should ensure equal access to health facilities so that patients can receive necessary treatments (Khan et al., 2022).

Policies and Evidence-Based Practice

Facility policies and evidence-based guidelines shape the management of SCD by promoting standardized care practices and reducing complications. Guidelines, including timely assessment, administration of pain control measures, and prevention of infections, aim to improve the patient’s condition. Policies implemented in healthcare organizations ensure that best practices of transfusion services and multidisciplinary support are provided to relevant clients (Smeltzer et al., 2021).

References

Ehsan, M., & Maruvada, S. (2023). Sickle cell anemia. Nih.gov; StatPearls Publishing. https://www.ncbi.nlm.nih.gov/books/NBK482164/

Khan, H., Krull, M., Hankins, J. S., Wang, W. C., & Porter, J. S. (2022). Sickle cell disease and social determinants of health: A scoping review. Pediatric Blood & Cancer, 70(2). https://doi.org/10.1002/pbc.30089

Obeagu, E. I., & Obeagu, G. U. (2024). Implications of climatic change on sickle cell anemia: A review. Medicine, 103(6), e37127. https://doi.org/10.1097/md.0000000000037127

Smeltzer, M. P., Howell, K. E., Treadwell, M., Preiss, L., King, A. A., Glassberg, J. A., Tanabe, P., Badawy, S. M., DiMartino, L., Gibson, R., Kanter, J., Klesges, L. M., & Hankins, J. S. (2021). Identifying barriers to evidence-based care for sickle cell disease: Results from the sickle cell disease implementation consortium cross-sectional survey of healthcare providers in the USA. BMJ Open, 11(11), e050880. https://doi.org/10.1136/bmjopen-2021-050880

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Hematologic Disorders
Choose a hematologic disorder from the text.

• Define the condition.
• Address key points of pathophysiology, clinical manifestation and evaluation toward diagnosis and treatment. Use appropriate physiological terms in your response.
• Apply individualized considerations, such as genetic/genomic and environmental factors regarding hematologic disorders
• Would social determinants of health have an impact on outcomes for hematological conditions? How so?
• Considering the pathophysiology of this hematologic disease. How might evidence-based guidelines and also facility policies guide practice?
• Provide evidence based references to support your content.
  

  Hematologic Disorders: Sickle Cell Disease