Awareness – the best form of Prevention

World Sickle Cell Day is observed annually on 19th of June to increase public knowledge and awareness on sickle cell disease and the associated challenges that results to patients and their families. It was established in 2008 at the United Nations General Assembly. On this day, individuals and groups across the globe recognize sickle cell anemia as one of the contemporary public health concerns. Education too is promoted on this day to promote global awareness of sickle cell disease as one of the most important genetically inherited disease conditions condition.

Background

Definition

Sickle Cell Disease (SCD) consists of genetically inherited blood disorders that make the red blood cells develop a rigid sickle-shaped appearances. The change in shape can be triggered by different conditions such stress, cold or dehydration. The most significant disorder of SCD is sickle cell anemia where the oxygen carrying capacity is impaired. The condition also disposes the patient to infections and pain.
Signs and Symptoms
Anemia which is defined as reduced hemoglobin levels is a major symptom of sickle cell disease. It is characterized by shortness of breath and easy fatiguability. The sickle cell shaped red blood cells also block small vessels that results in organ damage and pain due to reduced blood flow to the organs. Furthermore, the spleen is damaged and its role in fighting diseases is impaired making the patient susceptible to infections. Red blood cells in sickle cell disease also break down at faster rates than is normal [1]. This further worsens the anemia and may result in yellowing of the eyes and below the tongue.
Sickle Cell Disease Burden
Globally, approximately 300,000 babies are born with SCD annually; this number is expected to increase to up to 400,000 individuals by 2050 [2]. Nearly 90 percent of the world’s SCD population lives in three countries: Nigeria, India, and the Democratic Republic of Congo where the disease affects up to 2 percent of the population, and the carrier prevalence rate (sickle cell trait) is as high as 10% to 30% [2].The heterozygosity trait is also prevalent in malaria-affected regions across the globe and is observed to be protective in sickle cell disease. Sickle cell disease (SCD) is the most common hemoglobinopathy encountered in Kenya. In the western part of the country, about 4.5% of children are born with SCD, and 18% of children are born with sickle cell trait [3].
When left untreated, patients remain vulnerable to chronic conditions like stroke, acute chest syndrome, organ damage, blindness, leg ulcers, deep vein thrombosis, gallstones, pulmonary hypertension, and pregnancy adversaries. The social impacts of SCD include increased mortality and morbidity and the increased financial burden on low socioeconomic demographics.
In Kenya, engagement programs with patients have been held to share experiences of families and victims with SCD to show support and concern to the affected. The Sickle Cell Anemia Foundation – Kenya is a not-for-profit non-governmental organization that was founded in 2008 with a primarily focus on the provision of education, understanding and awareness for persons living with Sickle Cell Disease in Kenya. There are many more organizations within Kenya that are leading national campaigns for sickle cell disease awareness. Global awareness has also been enhanced through the celebration of the World Sickle Cell Day on June 19th which are championed by teams such as the Global Sickle Cell Disease Network, Center for Disease Control, and the World Health Organization.
Debunking Common Myths about SCD
Popular myths on SCD claim that only people of African descent can get the SCD, but anyone can get it regardless of their race or ethnicity. Victims with SCD have been believed to be immune to malaria. The sickle cell disorder is believed to have been an evolutionary response to Malaria, however sickle cell patients can still contract Malaria and should seek treatment as it is still a significant contributing factor to mortality in sickle cell patients[4]. Furthermore, there is a belief that sickle cell disease is transmissible via blood or bodily fluids. This is false as SCD is can only be inherited from the parents who are genetically predisposed to the disease.
Prevention and treatment of Sickle Cell Disease
Patient education is the best form of prevention because it creates awareness of the genetic condition. Through patient education, genetic counseling and testing of individuals can help plan and anticipate SCD in their children. Individuals with sickle cell can also avoid the triggers such as low oxygen in high altitudes, stressful events, or dehydration.
Pharmacotherapy and non-pharmacologic therapy help in managing and controlling SCD and its symptoms. Management of SCD is symptomatic and is dependent on severity. Patients with anemia are transfused with the appropriate volume of blood and painkillers such as morphine and a drug called hydroxyurea are used for acute chest syndrome and painful crises. Oxygen therapy is used to supplement the low hemoglobin and to prevent the red blood cells from more rigidity and bending into the sickled shape. The only known cure for sickle cell disease is a bone marrow transplant from a compatible donor[5].
Financing Options and Policy
Socio-economic disparities between populations determine the patient’s ability to receive treatment, care, and education on SCD. In remote areas and poverty-stricken regions, individuals rely heavily on global organizations and nations to receive financial assistance meant to fight SCD conditions. Out of pocket payments and social health insurance are also used to meet the costs of treatment. However, access to sickle cell specialists is limited in Sub Saharan Africa.

Various countries have instituted guidelines and policies that can be used by healthcare workers to manage sickle cell patients. The guideline is made available by the government to facilities and are also available online for free download. For example, Kenya has a national guideline for the control and management of sickle cell disease. There are National Health policies against sickle cell disease aim at organizing screening and care to reach populations at risk and concentrate the efforts upon them. In Tanzania, the government has incorporated SCD into its non-communicable diseases program in its national campaign to reduce mortality in children under 5 years[6].

Health Strategies in fighting SCD
The Sickle Cell Disease Control and Management strategy in Kenya correlates with the UHC framework because the aim of both is to improve the quality and affordability of healthcare to people living with SCD. In both, the agenda is to fight disparities that increase cases of morbidity and mortality from the condition.
In Kisumu, Kenya the county government plans to establish a comprehensive Sickle Cell Disease (SCD) Center at the Jaramogi Oginga Odinga Teaching and Referral Hospital (JOOTRH) to respond to the high burden of the condition in the region. The ministry of health in Kenya aims to increase its healthcare workforce by offering professional training programs meant to improve health outcomes in the nation. However, the lack of enough skilled personnel in health facilities remains a major challenge when controlling and managing SCD.
Global and national partnerships in combating SCD have been effective because they pre-dispose vulnerable populations to funding sources. The collaborations between experts across the globe lead to new discoveries about the condition, thus, enhancing how knowledge is shared and integrated into the clinical settings.
Calls to Action and Recommendations
It is important for governments, organizations, and stakeholders in the healthcare industry to develop common goals that enhance the provision of high-quality and affordable care across populations, especially those with SCD. One way to enhance the provision of quality care is through the increase in training of the existing and incoming healthcare workforce to expand the administration of accessible care to citizens. Sensitization campaigns on the danger signs and triggers of sickle ought to be initiated in endemic regions. There should be establishment of sickle cell disease payment options within the national health insurance schemes. This will ensure financial protection of families that are burdened by the disease.

References

[1] “Is Jaundice Caused by Sickle Cell Disease?” https://sickle-cell.com/symptoms/jaundice (accessed Jun. 08, 2022).

[2] F. B. Piel, S. I. Hay, S. Gupta, D. J. Weatherall, and T. N. Williams, “Global burden of sickle cell anaemia in children under five, 2010-2050: modelling based on demographics, excess mortality, and interventions,” PLoS Med., vol. 10, no. 7, Jul. 2013, doi:10.1371/JOURNAL.PMED.1001484.

[3] “Establishing care for sickle cell disease in western Kenya: achievements and challenges | Blood Advances | American Society of Hematology.” https://ashpublications.org/bloodadvances/article/3/Supplement 1/8/429226/Establishing-care-for-sickle-cell-disease-in (accessed Jun. 08, 2022).

[4] N. M. Archer, N. Petersen, M. A. Clark, C. O. Buckee, L. M. Childs, and M. T. Duraisingh, “Resistance to Plasmodium falciparum in sickle cell trait erythrocytes is driven by oxygen-dependent growth inhibition,” Proc. Natl. Acad. Sci. U. S. A., vol. 115, no. 28, pp. 7350–7355, Jul. 2018, doi: 10.1073/PNAS.1804388115/SUPPL_FILE/PNAS.1804388115.SAPP.PDF.

[5] “Sickle Cell Disease Clinical Guidelines | CDC.” https://www.cdc.gov/ncbddd/sicklecell/recommendations.html (accessed Jun. 08, 2022).

[6] J. Makani et al., “Health policy for sickle cell disease in Africa: experience from Tanzania on interventions to reduce under-five mortality,” Trop. Med. Int. Heal., vol. 20, no. 2, pp. 184–187, Feb. 2015, doi:10.1111/TMI.12428.

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