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THE SICKLE CELL DISEASE




(By Solomon Mahoi, [pseudonym, "Abck Obba"])


May 16, 2024


Sickle cell is perhaps one of the most deadly disorders known to the human life, with symptoms that not only inflicts internal pain to the bones, but it is a gradual silent killer due to some of the complications that are associated with it. And like any other disease that at first appeared to not have a cure, sickle cell too is known to not have a cure except for a series of treatment options. Nevertheless, there is still hope for a cure as scientists and researchers alike continues to make strides to see that the disease become history to the human existence. In the meantime, it is advisable to keep abreast on media updates on the disease, including unbias suggestions from medical professionals with years of experience in the medical field. But first let's take a look at what causes the disease, the symptoms associated with it, its complications, and its treatment options.




What Exactly Is Sickle Cell And What Causes It?

Also called sickle cell anemia, sickle cell is a genetically inherited disorder caused by a mutation in the gene. Or perhaps another way to put it is to imagine with me, a vehicle that uses petrol, and when water accidentally finds itself mixed with the petrol, the petrol's effectiveness then becomes interrupted because the petrol now has water in it that causes blockage to the valves that transports the petrol to the vehicle's engine. And as a result of this blockage to the engine, the engine now finds itself observing an unusual beating tension as the engine now starts to shake (or shiver) just to accommodate the flow of the petrol with water mixed in it, leaving the option for the vehicle's engine to continue to excessively shiver or beat (while the water still flows through the engine's valves and into the engine, which certainly would damage the engine, or completely shuts it down). Or, another option will be for the water to be drained out of the engine, or the rest of the petrol in the vehicle's tank be sucked out just to give space to fresh petrol with no trace of water (or strange particles) in it, so that the engine could go beating normal without any blockage-effects that could deter the vehicle from conveniently running the way its manufacturer originally intends it to run.


With this picture in mind, we could now imagine the petrol as the blood that runs through the veins that supply oxygen (or natural energy) to all the cells in the body, but through the lungs. However, the blood being red in colour, have genetical properties in it, one of those properties being hemoglobin and is iron fortified and must remain iron fortified. But when this iron or hemoglobin is reduced in the blood, the red blood cells which usually are round and feeble, will then start to deform or mutate from its round shape to a sickle-like shape, like the sickle tool that is used in farming to harvest vegetable, cereal crops, and to cut grasses and other unwanted vegetative objects that affects specific plant growth. This is precisely how sickle cell anemia got its name, including what causes it.


Now that we know what sickle cell anemia is, including what causes it, we could now move on to the symptoms that are associated with the disorder.




What Are The Symptoms Of Sickle Cell Anemia?

Generally, onset of the disorder begins around 5 to 6 months of age after birth, however, certain individuals do not necessarily exhibit symptoms until adulthood. What must also be understood is that because the disorder is genetically inherited, the trace or trait(s) can therefore be linked to either parent, or both parents, of the adult whose symptoms starts surfacing at adulthood. With that said, let's now jump right into what the symptoms of sickle cell anemia are.


Major symptoms of sickle cell range from iron deficiency (or anemia) to frequent infections to pain to fatigue, and this is because somewhere in the body there is blood-flow blockage and as a result of this infections then starts to buildup within areas of the body, and as infections form pain is then accompanied, and as pain persists the body then starts experiencing fatigue or tiredness, which then causes the individual who suffers from the disease to be laying down almost helplessly, each time the symptoms reappear.


Other symptoms include vision problems, as well as delayed growth or puberty. And this is all because the iron level in the blood is low.




What Are The Health Complications That Are Associated With Sickle Cell Anemia?

The human blood is life, which therefore makes the human blood an essential component of the human body, pretty much the same way petrol is an essential liquid component to a vehicle's engine to have the vehicle run smoothly. And so if the blood is not flowing well in the human body, there is tendency for stroke to occur to a person, including blindness, acute chest syndrome, organ damage, leg ulcers, gallstones, pulmonary hypertension, deep vein thrombosis, splenic sequestration, avascular necrosis, priapism, as well as pregnancy complications. This stands true too for a person with the sickle cell anemia trait, all because there is blood-flow blockage within the body due to red blood cells that have died in the body, leaving a shortage of healthy red blood cells. And as frightening as these symptoms might be, there is still hope for a cure even though any hasn't been confirmed yet. But in the meantime, let's take a look at what the current treatment options for the disease are.




What Are The Treatment Options For Sickle Cell Anemia?

Because sickle cell anemia affects the blood, therefore the first step to treatment options is to do blood test to determine the level of red blood cells in the body. And if the test show low levels of red blood cell an official sickle cell diagnosis will then follow before treatment options come in. At this point medication is prescribed, and it could be one of the following or a combination of them, depending on the severity of the surfacing symptoms.

They Include:


  • Hydroxyurea (Droxia Hydrea) which reduces pain

  • L-glutamine oral powder (Endari) which reduces pain

  • Crizanlizumab (oxybryta) which improves blood flow

  • Pain-relieving narcotics


Also, taking penicillin prevents infections. And for children with the disease, yearly flu shots are recommended, including childhood vaccinations against pneumonia, meningitis, and hepatitis B, especially when the disorder has the potential to complicate one's health.


Blood transfusion, stem cell transplant, stem cell gene addition therapy, and gene editing therapy/CRISPR, are other treatment options as well.


It is also worthy to note that self care is as equally important as the treatment options we've just looked at. Self care include taking folic acid supplements daily, eating healthy diets, drinking plenty of water, avoiding exposure to extreme humid temperatures and extreme cold temperatures, exercising regularly (but without overdoing it), using pain medications cautiously to avoid kidney damage, as well as avoiding smoking (because it increases the likelihood of pain crises).




Summary

Sickle cell is a genetically inherited disease that affects both children and adult, but whose symptoms usually starts 5 to 6 months after birth, and in some cases at adulthood. Its major symptoms are iron deficiency (or anemia), frequent infections, and as well as bodily pain. Currently there is no cure for the disease except treatment options that range from taking medications to surgical procedures to hormonal therapies and to self care, the latter which is as equally important as the other treatment options. And because it is a disease that causes health complications due to the number of dead red blood cells in the body that causes blood circulation blockage to the brain, heart, and joints, its risk factors therefore range from stroke to acute chest syndrome to blindness to leg ulcers to organ damage to pulmonary hypertension to avascular necrosis to splenic sequestration.




Conclusion

Managing sickle cell can be a lifelong process that can be painfully torturing each time its pain-related symptoms surfaces due to frequent infections to the body which the deficiency of iron in the blood causes. But there is still hope as scientists and researchers alike continue to search for cure. In the meantime, the current established treatment objective remained at alleviating pain as well as to prevent health complications that could arise as a result of the onset of the disease. Finally, it is advisable that self care be given importance equally to the importance of the current established treatment options that are available. This is because self care not only also helps tackle the disease's symptoms, but it does so cost effectively, and at the convenience of one's convenience.




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