Essay on Management of Acute Chest Syndrome

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Treatment of acute chest syndrome
Acute chest syndrome occurs when there is a vaso-occlusive crisis in the pulmonary vessels. A combined treatment of oxygen, bronchodilators, blood transfusion, incentive spirometry, analgesics and antibiotics are usually required. The main goal of the treatment is to decrease the HbS concentration to below 30% and if the haematocrit is 30% or higher then an exchange transfusion is necessary. Transfusions given early can potentially halt progressive respiratory deterioration and can prevent further attacks when given long term. Chronic transfusion together with hydroxyurea can decrease the rate of acute chest syndrome by about 50%. In cases of severe hypoxia, diffuse pulmonary involvement and quick
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Although blood transfusion is on the whole beneficial, it is important that Hb does exceed 10g/dL as there is a risk of expulsion of trapped cells from the spleen, leading to polycythaemia and high blood viscosity. (13) Prevention and treatment of infections
Studies show that infection is the main leading cause of death in SCD, especially in less developed countries. Managing infections can have a great positive impact on health outcome and therefore quality of life.

Between the ages of 6 months to 3 years, individuals with SCD often develop hypo- or asplenism due to congestion and engorgement of the sinusoids with sickled cells. This is a result of high oxygen extraction rates, sluggish circulation and local acidosis which all encourage red cell sickling. Blood may therefore bypass the filtering systems and macrophage phagocytosis is usually impaired. With continuous sickling and ischaemic damage, the spleen may undergo multiple infarcts creating irreversible scarring and atrophy. Ultimately, this causes ‘autosplenectomy’ and the spleen becomes a remnant, lacking in IgM memory B cells and is therefore functionless.

SCD sufferers are therefore at high risk of local infections, which can easily become systemic or permit sepsis to occur. The main concerning pathogen is S. pneumoniae, however H. influenzae, Neisseria

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