Sickle Cell Anemia: Effects of the disease and potential treatment

human blood cells

This study found that a daily hydroxyurea pill may finally bring some relief for young children living with sickle cell anemia. This could be a big game changer for resource-challenged sub-Saharan Africa where the disease is prevalent and health care is suboptimal (Ware et al, 2018). Few studies had been done where the burden is greatest. The study published in the New England Journal of Medicine found treatment was feasible and safe in children living in the area of Africa most affected. It proved to reduce vaso-occlusive events, infections, malaria, transfusions, and death. These results illustrate a need for more access to treatments. In the US and Europe early identification of children with sickle cell by means of neonatal screening. This allows these children to be given simple effective, and lifesaving interventions (Tshilolo et al, 2018).

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What is this disease?
Sickle cell disease autosomal recessive blood disease having a molecular disorder of hemoglobin structure and form/shape of red blood cells. Children with sickle cell disease are at risk for serious morbidities related to vascular occlusion, hemolysis (break down of red blood cells before their time of 120 days), and infection that can impair their quality of life and lead to early death. The effects of sickle cell disease can be seen in mineralization of connective tissue impairing mobility. This disease is very common in Saudi Arabia. And while the prevalence is decreasing in every region of Saudi Arabia it continues to be endemic in southern and eastern Saudi Arabia. The red blood cell change from nice round shape to a sickle shape and this change lead to a condition called sickle cell crisis. Complications include pain in long bones (where blood is manufactured) and joints, anemia, organ damage, infections and lung involvement (Alamri et al, 2018).

Areas commonly affected by Sickle Cell
This study included children from four regions: Africa (Ghana, Kenya), the Americas (Brazil, Canada, US), Europe (Belgium, Italy, UK), and the Middle East (Egypt, Lebanon, Oman, Saudi Arabia, Turkey, United Arab Emirates). This study compared reported pain rates, pain intensity, or home opioid use in different geographical regions. Rates of pain and analgesic use were calculated for each participant. Pain intensity was determined by use of the Faces Pain Scale. And although similar rates were found in all areas, those from the Americas and Europe reported significantly higher pain intensity than those from Africa and the Middle East. It has been suggested that transition from acute to chronic daily pain begins in pre-adolescence. Highest level reported was the Americas and lowest was in Africa. And it is felt that availability of opioid medications may also contribute to perception of pain (Kanter et al, 2018).

Health Issues from Sickle Cell Disease
This study provided evidence for a noted relationship between processing speed and parameters in sickle cell anemia and model risk factors. Patients showed processing speed deficits with sickle cell regardless of a silent cerebral infarct being present. This result suggests that the degree of slower processing speed is related to loss of white matter integrity in the brain. These results are significant because they suggest that while there is variability within the sickle cell population, this group is at risk for significant cognitive difficulties. The results were able to link cognitive impairment in patients with sickle cell with white matter integrity when looked at with an MRI. This study enforces previous reports of white matter injury in sickle cell leading to possible functional consequence of this kind of injury. All agreed further work is required to determine if the damage done to the white matter from sickle cell to see if it was preventable or reversible (Stotesburg et al, 2018).

Usual treatments for anemia are harmful for some
Anemia prevalence in children under five years is highest in sub-Saharan Africa and south-east Asia where rates of naturally occurring infection is also high. Iron is suggested to help with the problem of anemia however this study found the failure of resolving this problem has been connected to problems with financing, supply, distribution or adherence to instructions.

The problem with this giving iron to treat the anemia is it can make the children prone to infection. Studies done in Malawi and Tanzania showed children with low iron were less apt to contract malaria. The study also showed a cruel paradox. Children with iron deficiency anemia are most likely to benefit from iron intervention. Ye the responses of the body to the increase in iron makes these same children more susceptible to malaria. And that isn’t the only pathogen they were found with after iron increase. In Kenya infants were found with shingella, Escherichia and clostridium (Pasricha et al, 2018).

Malaria is the most important parasitic disease of man. In endemic areas it is the major cause of anemia and in some areas the most common reason for blood transfusion. As children in these areas are bitten multiple times they acquire a disease controlling immunity. However repeated malarial infections can result in splenomegaly. In areas of high malaria transmission supplementing iron and/or folate has little if no effect on childhood mortality.
Mortality associated with severe malarial anemia depends on access to and quality of health services. In one study a series of children admitted with severe malaria had mortalities ranging from 2.6% to 10.3%. those patients who are admitted with these anemia’s do not survive they pass quickly with almost half of the deaths occurring within 12 hours of admission (White, 2018).

Sickle cell disease can have sinister results
Prevalence of children with sickle cell disease having strokes in sub-Saharan Africa is disturbing. This disease is one of the most commonly inherited disorders in this area. It has been the primary cause of both overt stroke and silent strokes leading to neurological and cognitive issues. These strokes continue to accumulate during childhood leading to long-term impaired function and increased mortality. Prior to the institution of standardized stroke prevention strategies in the US there was a 2-3 fold prevalence of pediatric stroke from sickle cell disease. Further study of sickle cell disease needs to be conducted to help stop the incidences of stroke from this disease. But there is data limitations for imaging of sensitive and specific stroke diagnosis from this study that also needs to be addressed (Marks et al, 2018).

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Work Cited
Alamri, M. H. et al. (2018). Oral health status and treatment needs of children with sickle cell disease in Abha and Khamis Mushait cities of Southern Saudi Arabia. International Journal of Research in Medical Sciences, 6(2).

Kantor, J. et al. (2018). Novel findings from multinational DOVE study on geographic and age-related differences in pain perception and analgesic usage in children with sickle cell anemia. British Journal of Hæmatology.

Marks, L.J. et al. (2018). Stroke prevalence in children with sickle cell disease in Sub-Saharan Africa: A systematic review and meta-analysis. Global Pediatric Health.

Pasricha, S.R. et al. (2018). Reducing anæmic in low income countries: Control of infection essential. BMJ Clinical Research Edition.

Stotesbury, H. et al. (2018). White matter integrity and processing speed in sickle cell anemia. Neurology.

Toshilolo, L. et al. (2018). Hydroxyurea for children with sickle cell Anemia in sub-Saharan Africa. The New England Journal of Medicine.

Ware, R. et al. (2018). Sickle cell anemia treatment safely lowers disease burden in African Children. Cincinnati Children Hospital Center.

White, N.J. (2018). Anaemia and Malaria. BioMedCentral-Malaria Journal.

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Comments

In the years I have been a nurse I have cared for multiple children in sickle cell crisis. The reports of unimaginable pain are real and I know that with further study more treatments for this hideous disease is possible