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Deaths Among Children With Sickle Cell Disease

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Sickle cell disease (SCD) is a group of genetically inherited diseases that affect the red blood cells. This disease is caused by a base substitution – which is a type of mutation- where the sixth amino acid valine is substituted for glutamic acid. The difference in the solubility of the two amino acids causes a change in the shape of the haemoglobin, where the round shape of the Hb becomes sickled. The sickled haemoglobin carries oxygen less efficiently due to the blood cells blocking small vessels which restricts blood flow.

The restricted blood flow causes damage to tissue which causes a painful episode also known as a crisis. These crises occur when there has been a change in internal and external conditions such as dehydration, low oxygen levels and drastic change in temperature. This disease is also accompanied by degenerative organ damage, like renal dysfunction. This disease mainly affects people of Afro-Caribbean descent however, it also does affect other ethnic backgrounds. In the United Kingdom alone there is said to be about 12,000 to 15,500 people who are suffering with this disorder in 2015.

The most common types of sickle cell disease are Sickle Cell Anaemia, Sickle Haemoglobin C, Sickle Beta Thalassemia and Sickle Beta Zero Thalassemia. However, sickle cell Anaemia is the most severe. This genetic disease is mostly linked to type O blood and least linked to AB. Symptoms of sickle cell anaemia start to present themselves before a baby reaches the age of one. The symptoms that are watched for are jaundice and anaemia. Jaundice is the yellowing of the skin, eyes and mouth. This occurs when red blood cells die a release a chemical substance called bilirubin. Since sickle cell anaemia is the most severe type of SCD, that make anaemia more prevalence in children. Anaemia is caused by the loss of a few red blood cells, which causes children to appear very pale and be more tired.

Children with sickle cell disease have a lower morality rate due to complications that are correlated to the disease. They are more prone to developing acute chest syndrome (ACS). ACS follows a crisis, the syndrome is characterised by chest pains, a cough, fevers and extremely low oxygen levels (hypoxia). Due to the pain, inflammatory markers increase, so there is an increase in platelet activation, so platelet adhesion occurs. Platelet adhesion causes permanent damage to the lungs and to other major organs. Children suffering with SCD are very vulnerable to developing splenic sequestration. Splenic sequestration is the build-up of red blood cells in the spleen. The spleen becomes every painful and enlarged resulting in the removal of the spleen. The function of the spleen is to fight foreign bacteria in the blood, controls the level of blood cells and removes old and damage blood cells. The removal of the spleen increases the risk of infection which decreases the rate of mortality in young children (Brousse, Buffet and Rees, 2014). Children with SCD have the highest risk of getting strokes. Strokes occur when the blood vessels to the brain are blocked. Strokes occur after ACS and painful events occurs.

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In the United Kingdom, there are routine tests that check for abnormal haemoglobin in new-born babies who have a family history for sickle cell disease or present symptoms during their physical test. The screening is done by a blood spot test, where a nurse or midwife pricks a new-born on its heel and collecting four drops of blood. Data from the screening program shows that ‘most of the patients diagnosed with a sickle condition (78%) are Black African, Black Caribbean and any other black.’. After the test results show that the child has abnormal haemoglobin, another test-called haemoglobin electrophoresis- is done to identify whether the child is a carrier of the disease. The test can also determine whether the new-born has any other diseases linked to the sickle cell gene. Haemoglobin electrophoresis separate the different types of haemoglobin into bands. Once a child is diagnosed with sickle cell disease, a support system is formed to ensure that their disorder is managed. Diagnosis is important so they prevent problems linked to SCD. The child would be referred to a haematologist who create a care plan for the child and their family.

A haematologist would start to suggest the appropriate treatments for the patients age, symptoms and general health, they will also consider how severe the patient’s condition is. To help prevent and manage a patient’s pain during a crisis, they are prescribed pain killer and advised to ‘drink lots of water daily (8 to 10 glasses)’. Due to the pain the young patient’s is feeling, they are given non-steroidal anti-inflammatory drugs and opioids to give them relief. Non-steroidal anti-inflammatory drugs (NSAIDS) like aspirin and ibuprofen work by blocking the pain receptors therefore reducing pain (Han et al., 2017). Fluids are advised as people with SCD have issues with concentrating their urine since they urinate more frequently, they lose important minerals and proteins. To treat anaemia blood transfusions are done to add more normal red blood cells.

The addition of new red blood cells reduces the blockage in the vessel which will ease the symptoms and prevent further complications. Children with Sickle cell disease are more susceptible to infections therefore they receive vaccinations. Stem cell transplant is a form of treatment that has shown evidence to cure some children with SCD. Stem cell transplant works by removing the patient’s bone marrow cells by destroying them with drugs. Those bone marrow cells are replaced by bone marrow stem cells that have been donated most likely from a family member. The goal from the transplant would be that the donors bone marrow would begin to produce new red blood cells to replace the sickled blood cells from the patient. Stem cell transplants don’t always work as the recipient’s body doesn’t accept the donor’s cells. More recently, there has been progress in another cure- genetic therapy. Genetic therapy is used to fix faulty or missing genes in the patient’s stem cells so that their bone marrow can produce healthy red blood cells.

Overall, the complications that accompany sickle cell disease decrease morality in children. To help with their well-being, families are educated to ensure they can provide the right care to their child. Without the necessities like a care plan or medication, most children with sickle cell disease die before the age of 5.

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Deaths Among Children With Sickle Cell Disease. (2021, September 12). Edubirdie. Retrieved August 19, 2022, from
“Deaths Among Children With Sickle Cell Disease.” Edubirdie, 12 Sept. 2021,
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Deaths Among Children With Sickle Cell Disease [Internet]. Edubirdie. 2021 Sept 12 [cited 2022 Aug 19]. Available from:
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