Discuss how vaccines such as a meningococcal vaccine, influenza vaccines, and other recommended children and adults scheduled vaccine work in Sickle cell disease.

Sickle cell disease Sickle cell disease is a group of inherited genetic disorders that affect red blood cells. It is as result of mutations on the hemoglobin molecule. Usually, the red blood cells are discoid shaped. However, in sickle cell disease the shape of the red blood cells is distorted to form a crescent-like shape referred to as sickled. The affected red blood cells are usually prone to hemolysis, which leads to anemia. Sickled shaped red blood cells also tend to stick on the walls of the blood vessels, causing a blockage. Blockage often results in tissue damage and pain. Other associated symptoms include hypertension, organ damage, neurological complications, recurring infections, arthritis, chest pains, and coughs. Sickle cell disease exists in various forms. The most common variant is the HbSS, which results from the inheritance of sickle cell gene (“S”) from either parent. This form is typically the most severe form of the disease. It usually results in chronic anemia. (Rees, Williams, & Gladwin, 2010). Drugs used in the treatment and management of sickle cell disease and associated symptoms Sickle cell disease is a condition that worsens with age. Treatment options such as disease modifiers and supportive agents can prevent complications and prolong the lives of those living with the disease. Treatment for sickle cell disease and its associated symptoms include: 1. Hydroxyurea. It works by inducing fetal protein hemoglobin, reduces red blood cells adhesion and decreases the number of red blood cells. 2. Opioid analgesics to ease pain such as aspirin, methadone, and morphine sulfate, acetaminophen, and fentanyl. 3. Nonsteroidal anti-inflammatory drugs such as ibuprofen, acetaminophen, and aspirin. 4. Antibiotics such as amoxicillin, azithromycin, cefuroxime, and penicillin VK to treat associated infections. 5. Vaccines such as a meningococcal vaccine, influenza vaccines, and other recommended children and adults scheduled vaccines. 6. Endothelin -1 receptor antagonists such as bosentan and ambrisentan, which treat hypertension. 7. Tricyclic antidepressants such as amitriptyline. 8. Phosphodiesterase inhibitors, such as sildenafil or tadalafil. 9. Vitamins such as folic acid for neuronal development. 10. L-glutamine powder called Endari that is given orally to prevent sickle cell crisis in adults and children above the age of five. 11. Anti-nausea such as promethazine. (Yawn et al. 2014) Impact of genetics on the efficacy of the drugs used in sickle cell disease Although sickle cell is a monogenetic disease, it expresses a variety of phenotypic variation. Response to drugs given to manage sickle cell disease varies in patients depending on the genetic polymorphism. For instance, some patients exhibit intolerance or no response to hydroxyurea. This has been attributed to the genetic variation in Hbf induction. Researchers have found specific genes markers such as KLF 10, ARG 1, ARG 2, and CAR that can be used to predict the response of patients to hydroxyurea. The response to opioids is also variable in different patients. Occasionally nurses tend to associate this variability to nonadherence to treatment. However, recent research shows that the perception of pain and response to opioids varies widely as a result of genetic polymorphism in the COMT, ABCB 1, and OPRM 1 genes. (Husain, Hartman, & Desai, 2017). Measures to reduce side effects associated with drugs used in managing sickle cell disease. Most patients using hydroxyurea rarely exhibit any side effects. However, a few may complain of nausea, irritation, and headache. Management of these side effects can be done using, such as antiemetic and painkillers, which ease nausea and headache, respectively. Side effects associated with chronic use of opioids and other analgesics can be managed by alternating one opioid with another, changing the route of administration, and reducing the dosage given. Sickle cell disease is a chronic condition that needs to be managed keenly throughout a patient’s life. Nurses have a role in providing quality healthcare to these patients to improve quality and prolong their lives. Healthcare givers should reiterate on adherence to improve pharmacotherapy intervention response. References Husain, M., Hartman, A. D., & Desai, P. (2017). Pharmacogenomics of sickle cell disease: steps toward personalized medicine. Pharmacogenomics and personalized medicine, 10, 261–265. doi:10.2147/PGPM.S123427 Rees, D. C., Williams, T. N., & Gladwin, M. T. (2010). Sickle-cell disease. The Lancet, 376(9757), 2018-2031. Yawn, B. P., Buchanan, G. R., Afenyi-Annan, A. N., Ballas, S. K., Hassell, K. L., James, A. H., … & Tanabe, P. J. (2014). Management of sickle cell disease: summary of the 2014 evidence-based report by expert panel members. Jama, 312(10), 1033-1048.

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