Introduction
Sickle cell disease (SCD) is an inherited pathology of erythrocytes that are most met among African American and Hispanic American individuals. In the United States, these ethnic groups are minor, and the chances of receiving help from the state for the treatment of SCD are currently unperceptive. The government has issues with financing orphan disorders such as SCD, the pathologies that are affecting less than 200.000 people nationwide. In the U.S., SCD affects circa 100.000 individuals; however, millions of humans worldwide (Snudd et al., 2018). Still, such a disorder as cystic fibrosis addresses a smaller number of patients yearly and receives 3.5 times more funding from the National Institutes of Health (Lee et al., 2019). Additionally, SCD leads to a variety of conditions worsening the life quality of patients suffering from strokes, infections, kidney failure, and other comorbidities. Inadequate life quality means losses not only in healthcare but also in the economic outcomes of the country. Developing treatment programs for patients with SCD and correcting health inequities in the United States can improve the condition of these patients and facilitate the burden of pathology.
Background of the Disorder
Sickle cell disease can be called a misnomer as it refers to various disorders, as different mutations can occur in the beta-globin chain of hemoglobin. The latter is a protein in erythrocytes that is responsible for their special form, also captures oxygen, and carries it to the tissues of a human body. The proper structure of hemoglobin chains is essential for the flexibility of red blood cells and their proper function of carrying oxygen via blood. Depending on the mutation in the HBB gene and its inheritance from one or both parents, SCD has different variants. If the mutation is inherited from both parents, a person has homozygous hemoglobin S.S. with the majority of crescent erythrocytes and severe clinical symptoms (Ware et al., 2017). Heterozygous conditions include hemoglobin C with hemoglobin S (HbSC), hemoglobin S with β0-thalassemia, hemoglobin S with β+-thalassaemia, hemoglobin S.E., hemoglobin S.D., and others (Ware et al., 2017). The migration of people from South-Eastern Asia made HbSE disorder more spread in the U.S. and Europe.
The pathology is oftentimes met among people from African countries, Hispanic origin, and people of Mediterranean, Middle Eastern, and Indian descent. The lifespan of such patients has not enhanced further than five decades. In sub-Saharan Africa, the prognosis on life duration is, unfortunately, sadder due to a lack of resources and treatment facilities. In some African tribes, one can hear the names referred to the disorder, such as kibeka or malari ya mifupa ina pasuka translated as big spleen or broken bones addressing the bright signs of the pathology (Ware et al., 2017). The other African tribes can describe a person with SCD as ako kufa lobi meaning “he will die tomorrow.” Still, with the progress of current medicine, teaching the endemic population disease complications, the disorder stopped being unknown worldwide. In endemic regions, the survival of children with SCD has considerably improved due to newborn broadcasting screening and early pneumococcal immunizations.
The Current Issue of the Disease
The usual form of erythrocytes is biconcave, which gives them flexibility when going through the capillaries. The such shape also makes red blood cells smooth preventing them from being stuck on vessel walls. With SCD, erythrocytes cannot go through small capillaries and block normal blood flow. The latter leads to ischemia and lack of oxygen in tissues and organs, and a constant feeling of fatigue. Increased death of red blood cells leads to going out of hemoglobin, and it is breaking down through biliverdin. The latter cannot be utilized fast enough by the liver, and it storages in blood, skin, and mucosa coloring everything yellow. Impact on blood flow leads to various symptoms such as acute chest pain, chronic pains, delayed growth and puberty, retinopathy, coronary heart disease, pulmonary hypertension, gallstones, aseptic necrosis in joints, kidney failure, leg ulcers, and stroke. These are some of the many complications that can lead to the worsening of a patient’s condition. To economize costs on treating all of them, patients with SCD should be able to get access to early strategies simplifying their symptoms and sufferings.
Effect of the Disease on Public Health Community
Sickle cell disease is passed down through generations, impacting many people. Kato et al. (2018) claim that sickle cell disorder can lead to life-long disability leading to low life expectancy. In addition, the treatment cost of this disorder is very high for people with this condition and also for the health units. The cost of hospitalization with sickle cell disease complications is estimated to be high, involving high-cost facilities such as transfusions, transplantation, cytostatic treatment, and gene therapy (Ware et al., 2017). Individuals suffering from sickle cell disorder are at risk of high morbidity and mortality from infections, pain, acute chest syndrome, and other complications. Public health society spends substantial amounts of capital in the process of preventing and curing people living with sickle cell disease. This has a significant side effect on society representatives that does not possess the needed amounts of cash to afford treatment. Consequently, the patient ends up suffering in health facilities. When the health sector worker does not attend to patients due to a lack of capital, the mortality rate also increases. The state should consider developing programs for such patients to get easier access to high-cost treatments.
Interventions
The Centre for Disease Control and Prevention, the National Center on Congenital Disabilities and Developmental Disabilities, and the Division of Blood Disorders consider sickle cell disease a significant pathology. These organizations play a major role in creating public awareness of this disorder. They educate the public on genetic health to spread knowledge about sickle cell disease. These authorities also raise awareness of this disorder in the general public to equip health workers who care for sickle cell disease patients. Additional studies have been conducted to search for a better treatment direction. In a recent study, bone marrow or stem cell transplants were proved to be effective methods for SCD patients (Kato et al., 2018). The process involves taking healthy donor body cells and transplanting them to an individual matching with human leukocyte antigen. Although the process cures the disease, it can also have adverse effects on the patients.
Each person receives different treatment options depending on the disease symptom. Possible treatments may include consistent blood transfusions as well as hydration recommended water intake of 8-10 glasses per day. For severe SCD, hydroxyurea medicine is recommended, which is a ribonucleotide reductase inhibitor decreasing leukocyte count. Preventive research conducted by Morrison-Levy et al. (2018) suggests that this medicine can reduce pain and recurrence of the acute chest. It also reduces the number of vaso-occlusive complications, SCD complications, and mortality rates in high-income states (Kato et al., 2018). Medicine providers in public health programs are not entirely responsible for improved survival for children with sickle cell disorder (Ware et al., 2017). The programs remain important components in preventing sickle cell currently, and hydroxyurea is a widely accepted and used treatment for sickle cell disorder. However, this medicine has several side effects that negatively impact patients, such as blood clotting.
Spiritual and Family Concepts
Religion may play a role in the treatment of SCD, influencing patients’ decision-making about their health. Some cases may rely on spiritual and alternative medicine rather than traditional scientifically proven methods. This can be dangerous due to the rapidly deteriorating effect the condition has. Since the disease is hereditary, there may be changes in reproductive lifestyles, either voluntarily or encouraged by a religious leader. Family lifestyle will likely dramatically shift for patients with SCD, requiring significant rest, monitoring of health, and expenses for highly specialized treatments. However, it is possible to live a potentially prolonged life if the patient is compliant with treatment and receives support from state organizations and family members. Close relatives can be tested on HLA type and become a matching donors for hematopoietic stem cell transportation. It is a big step for a close relative as the procedure is attached with various risks. However, stem cell transplantation shows promising results among patients with SCD and allows being cured.
Conclusion
Sickle cell anemia is a serious condition characterized by changes to red blood cells impacting their form and ability to carry oxygen. The pathology is inherited and impacts mostly people of African and Hispanic origin. The latter means that minor groups of individuals inhabiting the United States are suffering from pain, fatigue, and various organ dysfunctions. Patients with the condition have to shift lifestyles and care to protect physiological and psychological needs. Healthcare providers can offer basic treatments for symptoms; however, more advanced methods should become more accessible for patients in the U.S. Currently, stem cell transplantation shows encouraging outcomes. To prevent severe complications of SCD, the state has to develop strategies and programs for people seeking help in healthcare regarding their disease. By implementing such help, the U.S. can prolong the life of these patients, improve their life quality, and save the working population for the benefit of the economy.
References
Kato, G. J., Piel, F. B., Reid, C. D., Gaston, M. H., Ohene-Frempong, K., Krishnamurti, L., Smith, W. R., Panepinto, J. A., Weatherall, D. J., Costa, F. F., & Vichinsky, E. P. (2018). Sickle cell disease. Nature Reviews Disease Primers, 4, 18010. Web.
Lee, L., Smith-Whitley, K., Banks, S., & Puckrein, G. (2019). Reducing health care disparities in Sickle Cell Disease: A review. Public Health Reports, 134(6), 599-607. Web.
Morrison‐Levy, N., Knight‐Madden, J., Royal‐Thomas, T., King, L., & Asnani, M. (2018). Improving disease knowledge in 6‐to 10‐year‐olds with sickle cell disease: A quasi‐experimental study. Child: Care, Health, and Development, 44(3), 501-506. Web.
Sundd, P., Gladwin, M. T., & Novelli, E. M. (2018). Pathophysiology of Sickle Cell Disease. Annual Review of Pathology: Mechanisms of Disease, 14(1). Web.
Ware, R. E., de Montalembert, M., Tshilolo, L., & Abboud, M. R. (2017). Sickle cell disease.
The Lancet, 390(10091), 311-323. Web.