Anaesthesia for patients with sickle cell and other haemoglobinopathies

Publication date: Available online 22 February 2019

Source: Anaesthesia & Intensive Care Medicine

Author(s): Lorna CJ. Sissons, Kailash Bhatia

Abstract

Sickle cell disease is an autosomal recessive multi-systemic condition, caused as a result of a mutation in chromosome 11 resulting in production of abnormal beta globin of haemoglobin or HbS. The abnormality under specific conditions results in polymerization of the globin chain resulting in deformed sickle-shaped red cells. These haemolyse under adverse conditions resulting in occlusion within the circulatory system resulting in cardiorespiratory, neurological, renal, musculoskeletal and bone marrow dysfunction, along with increased susceptibility to infections leading to significant morbidity and mortality. The perioperative period provides the perfect milieu for exacerbations and careful attention to the fundamentals of oxygenation, analgesia, hydration, antibiotic prophylaxis and temperature control are the key preventative strategies utilized in this setting. Hydroxyurea, blood transfusion and haematopoietic bone marrow transplant are the available options for treatment.

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