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Hematologic Disorder
Name
University
MN653
Hematologic Disorder
Blood is the single-most life-threatening body fluid circulating from the heart through arteries, capillaries, and veins. It transports oxygen, heat, electrolytes, vitamins, hormones, antibodies, and waste products to the appropriate tissues and nourishes the body. Due to blood’s numerous and complex functions, most hemolytic disorders tend to be very complicated and thus require special care by a physician or any other healthcare professional. Some popular hemolytic disorders include anemia, sickle cell disease, genetic disorders, and complications from transfusions or chemotherapy. As a healthcare provider, I encountered a patient with hematologic disorders. The patient was complaining of periodic pain episodes, vision problems, and swelling of feet and hands. All imaging was noted unremarkable and lacking any DVT’s. A lab test of the patient’s complete blood count showed that his hemoglobin and platelet counts were 6.5 g/dl and 467,000 /µl respectively, while that of white blood cells was 9700/µl. Blood film showed many sickle-shaped cells with a positive solubility test. These tests revealed that the patient had sickle cell disease.
Sickle hemoglobin cell has a curved shape (Sundd, Gladwin & Novelli, 2019). This shape changes the cell properties, making cells less flexible and more rigid, thus increasing the likeliness of hemolyzing and causing blockages of the blood vessels which interfere with blood flow. Sickle hemoglobin may also result from a mutation whereby thymine gets substituted for adenine on the 6th genetic sequence codon (Sundd, Gladwin & Novelli, 2019). As a result, the valine becomes coded instead of glutamate of the hemoglobin beta chain’s 6th position (Sundd, Gladwin & Novelli, 2019). Such genetic mutation also changes the shape of the hemoglobin cells into a sickle form, and alters their stability and solubility. With oxygen insufficiency in the vascular system, the sickle hemoglobin gets more insoluble and increases blood’s overall viscosity and polymerization. After a while, the cells membranes experience permanent damages and the cells remain permanently bi-concave in shape.
The patient did not know much regarding sickle cell disorder. He had heard about sickle cell from his friends and family. Although he didn’t know the exact cause of the disease, he believed that it was inherited. He also didn’t know the clinical manifestations of the diseases and how they could be diagnosed. This lack of knowledge regarding sickle cell shows the dire need for addressing the family, the patient, and the entire society about sickle cell disease, the causes, symptoms, and management. Besides, most people tend to discriminate against those who they think have this disease (Brandow et al., 2020). The healthcare system should sensitize people against discrimination.
The only known remedy for sickle cell disorder is stem cell transplant. However, transplants are risky and complex, and only some patients can undergo them. According to Brandow et al. (2020), scientists are still looking at how to treat sickle cell anemia using gene therapy. One day, doctors will help treat this disease by replacing or changing the foreign gene that makes hemoglobin cells sickled. Nevertheless, without the cure, the present recommendations for the disorder are hydration, immunization and everyday penicillin doses to prevent further infections, folic acid supplements for manufacturing new red blood cells, hydroxyurea to make the cells less rigid L-glutamine, pain killers, and blood transfusion for some severe complications (Abraham & Tisdale (2021).
According to Abraham & Tisdale (2021), the degree to which physicians and other healthcare providers are comfortable managing patients with acute sickle cell disorders is not clear. I have tended to sickle cell patients in the past and understood the disease process as a critical care nurse. I felt confident enough in educating. However, I did feel I needed help from my preceptor for confirmation in treatment. Nevertheless, every healthcare provider should understand that sickle cell disease patients need comprehensive care such as pain management, blood transfusions, preventive measures, and hydroxyurea. Besides, managing this condition could result in further complications such as heart failure, cirrhosis, or even death. Therefore, as a healthcare provider, I need to develop my healthcare acumen further to ensure that my patient gets the proper ambulatory management to reduce the chances of getting hospitalized and to avoid acute pain. Also, I need to change my attitude towards this disease, show compassion to my patient and always encourage him to recover. Thankfully, my patient just required 2 units of PRBC, hydration, analgesics, antihistamine and education for future crisis prevention. All which was provided and on the road to recovery.
References
Abraham, A. A., & Tisdale, J. (2021). Gene Therapy for Sickle Cell Disease-Moving from the Bench to the Bedside. Blood.
Brandow, A. M., Carroll, C. P., Creary, S., Edwards-Elliott, R., Glassberg, J., Hurley, R. W., … & Lang, E. (2020). American Society of Hematology 2020 guidelines for sickle cell disease: management of acute and chronic pain. Blood advances, 4(12), 2656-2701. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7322963/
Sundd, P., Gladwin, M. T., & Novelli, E. M. (2019). Pathophysiology of sickle cell disease. Annual review of pathology: mechanisms of disease, 14, 263-292. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7053558/
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