Overcoming Challenges – Sickle Cell Disease
Sickle cell anemia, a hereditary blood disorder, is caused by abnormal hemoglobin, which makes the red blood cells take the shape of a sickle. It is mostly found in certain ethnic groups and causes vaso-occlusive crises and organ damage, which significantly affects the patient’s quality of life. The difficulties are the unavailability of care and the inability to properly deal with the symptoms. Improving the knowledge of its pathophysiology and treatment strategies is the key to improving the outcome and the special needs of people suffering from sickle cell anemia (Elendu et al., 2023).
Treatment Strategies
Hydroxyurea Therapy
Hydroxyurea is a drug that can enhance the amount of fetal hemoglobin production, thus preventing painful crises and acute chest syndrome in patients with sickle cell disease. Besides, it also cuts down the necessity of blood transfusions and hospitalizations.
Blood Transfusions
Blood transfusions are usually performed to increase the number of normal red blood cells in circulation, which can thus prevent complications like stroke, acute chest syndrome, and organ damage. Chronic transfusions are also used to prevent recurrent strokes in patients who are at high risk (Mangla et al., 2023).
Bone Marrow Transplantation
For patients with severe sickle cell disease, especially those with a matched sibling donor, bone marrow transplantation is the cure. This method involves replacing of the patient’s bone marrow with the healthy donor marrow, which can generate normal red blood cells.
Challenges
Patient Adherence
The most common problem faced by patients with sickle cell disease is adhering to the set medication schedules, specifically to daily therapies such as hydroxyurea (Mayo Clinic, 2023). Treatment adherence can be a big challenge for a bigger population like adolescents and young adults. Discounting factors like pill dosage, side effects, and perceived advantages are some of the reasons why people tend not to comply with their medication.
Transfusion Complications
Even though transfusion therapy is life-saving, it still carries a number of risks that include transfusion reaction, iron overload, and alloimmunization (development of antibodies against the transfused blood components), which may be a problem in the event of subsequent transfusions.
Transplantation Risks
Bone marrow transplantation is a high-risk procedure that involves graft-versus-host disease, infections, and graft failure(Maakaron, 2019). On the other hand, the problem of finding a solvable donor can also be encountered since not every patient has a matched sibling donor, and unrelated donor transplantation comes with even higher risks.
References
Elendu, C., Amaechi, D. C., Alakwe-Ojimba, C. E., Elendu, T. C., Elendu, R. C., Ayabazu, C. P., Aina, T. O., Aborisade, O. B., & Adenikinju, J. (2023). Understanding Sickle cell disease: Causes, symptoms, and treatment options. Medicine, 102(38), e35237. https://doi.org/10.1097/md.0000000000035237
Maakaron, J. E. (2019, October 19). Sickle cell anemia clinical presentation: History, physical examination. Medscape.com. https://emedicine.medscape.com/article/205926-clinical
Mangla, A., Ehsan, M., Agarwal, N., & Maruvada, S. (2023, September 4). Sickle cell anemia. StatPearls – NCBI Bookshelf. https://www.ncbi.nlm.nih.gov/books/NBK482164/
Mayo Clinic. (2023, December 22). Sickle cell anemia – symptoms and causes. Mayo Clinic; Mayo Clinic Staff. https://www.mayoclinic.org/diseases-conditions/sickle-cell-anemia/symptoms-causes/syc-20355876
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Question 
Pick one hematological disorder covered in this module. For this disorder, identify and describe three treatment strategies for the disorder.
Overcoming Challenges – Sickle Cell Disease
What challenges could arise in carrying out these strategies and providing care to the patient?
AA
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