Introduction
Sickle cell anemia is a complex disease that is characterized by many factors that include the creation of new approaches that impact health in many ways. Sickle cell anemia must be better understood in order to accomplish the desired objectives and to recognize the importance of the disease and its limiting factors in addressing patient outcomes. The challenging nature of the disease impacts the health of its patients and requires further evaluation of its pathophysiology and other factors in order to treat the disease with the current treatments that are available. There are important determining factors associated with sickle cell anemia that require further evaluation; therefore, these considerations must be made in order to accomplish the desired treatment goals. The following discussion will address its pathology, current treatments, and potential future directions in treatment for this unique disease.
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Body
Sickle cell anemia is most commonly identified as a homozygous HbS disease (HbSS), and it includes chronic pain and regular vaso-occlusive crises that impact organs and systems . The disease is observed in patients with a genetic mutation that includes thymine for adenine, thereby coding valine rather than glutamate and polymer formation, accompanied by limited oxygen that contributes to the sickle cell formations that are common in this disease . Therefore, when the cells continue this formation, they become irreversibly sickled, even when oxygenation occurs, thereby gaining Na+ and losing K+, along with an increase in membrane permeability to Ca++ . This process begins as early as ten weeks, but the first sign of changes are typically evident at 6-12 months, which requires further investigation and diagnosis at this stage .
With sickle cell disease, antioxidant presence is markedly reduced, and in particular, SOD, GPX, catalase, vitamin C, vitamin E, and glutathione . Under these conditions, it is necessary to recognize the importance of restoring some of these antioxidant properties as best as possible, including the use of A-HOOH and dehydroascorbic acid supplements, along with vitamin C supplements and alpha-lipoic acid to reduce RBC sickling, in some cases up to one-half . However, other treatment alternatives must also be considered because antioxidant therapies on their own are not always successful, thereby requiring other types of treatment to be considered as key factors in supporting a long-term approach to the disease that will positively impact patients . To be specific, this treatment may require a combination of antioxidant therapies rather than a single therapy in order to support treatment needs more effectively (Chirico & Pialoux, 2012).
Other possible treatment alternatives for sickle cell disease often emphasize the importance of ROS production, including Hydroxyurea (HU) to reduce vaso-occlusive crises and respiratory complications through the formation of fetal hemoglobin (Chirico & Pialoux, 2012). In addition, HU may also promote a NO response to decrease adhesion and cell injury in some cases, while treatment with arginine may also promote a positive response to vasodilation and the prevention of hemolysis that is caused by oxidative stress in cells (Chirico & Pialoux, 2012). Furthermore, desferoxamine, a catalase mimetic, and an NF-kB inhibitor may play a role in improving outcomes through the alleviation of oxidative stress, inflammation, and adhesion (Chirico & Pialoux, 2012).
Most importantly, treatment for sickle cell disease must be continuous in order to prevent symptom alleviation and control the pain that is associated with the disease, along with antibiotics such as penicillin to prevent infections and other vaccinations for pneumococcus, haemophilus influenza type B, meningococcus, hepatitis B, and influenza, as well as careful monitoring of patients who show any possible signs of infection that could impact their overall health . Other possible treatment alternatives that are commonly used include the use of blood transfusions in patients to address acute complications and symptoms, along with the prevention of stroke for many of these patients . Furthermore, the pain of sickle cell disease requires further investigation and evaluation because it contributes to poor quality of life in many patients; therefore, pain is often treated with acetaminophen, NSAIDs, and opioids, with the latter carefully monitored under specific conditions because of their addictive properties .
Most importantly, patients must be monitored closely and regularly for any possible symptoms that might occur, including routine doctor visits, blood tests that are used to evaluate blood counts, antibiotic prophylaxis, particularly in very young children up until the age of five, the use of folic acid as a regular supplement, an annual Transcranial Doppler ultrasound in patients over the age of two, and retinal examinations annually to evaluate any possible signs of retinal disease in patients (Philadelphia, 2014). These strategies are commonly used to manage and treat the condition.
Conclusion
Although the biological and physiological properties of sickle cell disease are now much better understood, many factors remain a challenge for researchers and clinicians alike. Therefore, this condition is far from a cure, yet new possible treatment discoveries are on the horizon. Considerable factors must be addressed in order to accomplish the objectives of treating sickle cell disease effectively and proactively in order to alleviate common symptoms, such as chronic pain, which may be debilitating in some patients, thereby reducing quality of life and overall health and wellbeing.
- Chirico, E. N. & Pialoux, V., 2012. Role of Oxidative Stress in the Pathogenesis of Sickle Cell
Disease. IUBMB Life, 64(1), pp. 72-80. - Maakaron, J. E., 2013. Sickle Cell Anemia: Medscape. [Online]
Available at: http://emedicine.medscape.com/article/205926-overview#aw2aab6b2b2
[Accessed 7 May 2014]. - Philadelphia, T. C. H. o., 2014. Overview of Sickle Cell Disease. [Online]
Available at: http://www.chop.edu/service/hematology/our-programs/sickle-cell-center/diagnosing-and-treating-sickle-cell-disease.html#how_treated
[Accessed 7 May 2014].
