Sickle cell disease and pathophysiology (II)

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Tola Dehinde

Last week, I started a topic on the gloomy anatomy of sickle cell. I know it is rather depressing, but this is essential for caregivers to know, as well as the person living with it to take better care of the patient or themselves. Sickle cell is known to affect any part of the body and poses diagnostic and therapeutic dilemmas to the treating medical team. I also talked about the many clinical manifestations of sickle cell. The consequences are numerous and affect most of the organs in the body. Let’s continue:

(E) Progressive ischaemic atrophy of the spleen from ischaemia, increases the risk of sepsis (particularly pneumococcal). The function of the spleen is to clear the blood cells. Sickle cell is one of the common diseases that affect red blood cells. It can affect any part of the body and one of the most common and early organ to be affected is the spleen. One of the other functions of the spleen is to defend the immune system. A major risk of spleen dysfunction is susceptibility to infections, disease expressions, and vascular risks.

(F) Haemolysis resulting in gall stones and related diseases. Haemolysis is the rupture and destruction of red blood cells. Haemolysis, therefore, causes chronic anaemia and pigment gallstones. While most gallbladder stones are asymptomatic, some of them can result in infection and inflammation of the gallbladder stones. Recurrent ischemia, (a restriction in blood supply to the tissue, muscle groups, or organs), and infraction can cause chronic dysfunction of multiple different organ systems.

In addition, there are complications of therapy and prophylaxis for sickle cell disease, namely:

. Blood transfusion causes iron overload and sensitisation to blood group antigens.

. Blood-borne infections in the transfusions, for example, Hepatitis B & C viruses, and HIV.

. Opioids and their potential complications include dependency, nausea, vomiting, constipation, and respiratory depression.

. Hydroxycarbamide (previously termed hydroxyurea) therapy can cause neutropenia.

. Other complications include Ischemic stroke. Stroke is a common and potentially devastating manifestation of sickle cell that can affect children and adults. Individuals with sickle cell are at risk of ischemic and haemorrhagic stroke. Seizures occur in people living with sickle cell, two to three times more than the general public. Usually, seizures occur within the first 24 hours of a stroke; there is also a high likelihood of developing epilepsy within the next six months for those who had seizures within the first 24 hours after a stroke presentation.

. Avascular necrosis of the renal concentrating defects; renal dysfunction is among the most common complications of sickle cell, from hyposthenuria in children to progression in young adults and it becomes chronic kidney disease. Emerging evidence is now suggesting that this extends to those individuals living with sickle cell trait too. Renal papillary necrosis is a type of damage that occurs in one’s kidneys. It decreases kidney function and can lead to kidney failure.

. Renal papillary necrosis is the death of cells and tissue in your medulla and renal papillae. These areas of dead tissue reduce kidney function and can thereby lead to kidney failure. Chronic kidney disease arises because the kidneys are especially sensitive to sickled red blood cells. People living with sickle cell can develop chronic kidney disease because of reduced blood flow to the kidney. This can lead to end-stage renal disease and is a common cause of death for people with sickle cell.

. Heart failure occurs because long-term anaemia in people living with sickle cell lowers the ability of blood to carry oxygen. This makes the heart work harder to make up for the lower oxygen levels in the blood. This raises stress and pressure on the heart, which increases the risk of heart failure.

. Pulmonary hypertension happens when there is a growth in the breakdown of red blood cells within the blood vessels causing inflammation.

To conclude this column on the pathophysiology of sickle cell, I want to talk about the various crises that people living with sickle cell experience.

  • The most common is a vaso-occlusive crisis, (pain crisis). It is caused by tissue hypoxia, (hypoxia is low levels of oxygen in your body tissues) and leads to ischemia and infarction, usually in the bones, but also in the spleen, lungs or kidneys.
  • Another type of crisis is an aplastic crisis and this happens when bone marrow erythropoiesis (the production of red blood cells), slows during acute infection due to human parvovirus infection; during which an acute erythroblastopenia (deficiency in bone marrow) may occur.
  • An additional kind of sickle cell crisis is acute chest syndrome which results from pulmonary microvascular occlusion and is a common cause of death, with mortality rates of up to 10 per cent. Repeated episodes prompt chronic pulmonary hypertension as discussed earlier.
  • A further type is the sequestration crisis, which frequently occurs in children. Acute sequestration of sickled cells in the spleen exacerbates anaemia. Hepatic sequestration may transpire in children and adults, causing right upper pain, behind the lower ribs and this could be accompanied by renal failure.

The last type of crisis is priapism which only affects men living with sickle cell. This is a prolonged erection of the penis that occurs as a result of normal blood flow in muscles, tissues, or nerves.

If you would like to get in touch with me about Sickle cell, do so, via email: [email protected] And do checkout my blog: My book on sickle cell – How To Live With Sickle Cell and my other books are available for purchase on www.amazon.com

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