Nurse Talks Review Notes: Sickle Cell Disease
Sickle Cell Disease is an autosomal recessive disease that causes the red blood cells to sickle. Because it is a genetic disease, both parents must be carriers of the sickle cell trait. There is a 25% chance that a child born with parents carrying the sickle cell gene can get this disease. If only one trait of the disease is passed, then the child is a carrier (Rogers, 2019).
Red blood cells are normally shaped as biconcave disks (Hoffman, 2016) containing normal alpha and beta chain groups. Because of a mutation in the haemoglobin, the 6th position of the amino acids in its beta peptide chain is replaced with valine instead of glutamic acid in sickle cell disease (Information Center for Sickle Cell and Thalassemic Disorders, 2002). The presence of valine makes the beta chains sensitive to low oxygen levels in the body such as going to a higher altitude or dehydration, pain and infection making the cells change its shape, stiff, and sticky (RegisteredNurseRN, 2018). Haemoglobin electrophoresis is a test of identifying the type of haemoglobin a person has (Rogers, 2019).
RBC’s have a long life span of about 120 days while sickled RBC’s tend to breakdown in 20 days which can result to anaemia. Long term occurrence of this can lead to chronic hemolysis.
The sickle cell trait can occur in any ethnic group but it is mostly common in people of African origin (Bennett, 2005). Sickle cell is particularly endemic in areas with high malaria burden because of evolutionary (heterozygote) advantage (Osmosis, 2019).
Complications of Sickle Cell Disease
The most common complication is sickle cell crisis. This is a painful episode due to the sickling of RBC’s which is triggered by several factors such as low oxygenation, infection, and dehydration. The pain is due to vaso-occlusion which reduces the blood flow in the blood vessels depriving the affected organs with oxygen. Vaso-occlusion can affect any part if the body and such crises can increase the risk of developing cerebrovascular accident disease when the blood vessels of the brain are affected.
Other complications of sickle cell disease include severe anaemia, hand-foot syndrome, splenic sequestration, delayed growth, eye problems, skin ulcers, heart disease, sickle chest syndrome, lung disease, priapism and gallstones (Rogers, 2019)
Management of Sickle Cell Crisis
The distorted cells can unsickle when the RBCs are once again oxygenated that is why sickle cell crises are always treated as a medical emergency. The management for sickle cell crises involves proper oxygenation, pain management, and adequate fluid hydration and monitoring complications.
Disclaimer:
The content in this blog is for informational purposes only and should not be taken as medical advice. It is always best to consult your doctor for medical questions.
If you are a healthcare provider, the content here should not be used to make any diagnosis, give advice or prescribe treatment as this blog is for informational and educational purposes only. Healthcare is an everchanging field and each patient is unique. It is your responsibility as a healthcare provider to always refer to current care standards and practices.
References:
Hoffman, J. F. (2016) Biconcave shape of human red-blood-cell ghosts relies on density differences between the rim and dimple of the ghost’s plasma membrane, Proceedings of the National Academy of Sciences, 113 (51), pp. 14847–14851. DOI:10.1073/pnas.1615452113.
Rogers, G., 2019. Sickle Cell Anemia. [online] Healthline. Available at: <https://www.healthline.com/health/sickle-cell-anemia>
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