Anaesthesia for patients with sickle cell disease and other haemoglobinopathies

Abstract

Sickle cell disease is an autosomal recessive multi-systemic blood condition, caused because of a mutation in chromosome 11 resulting in production of abnormal β 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 cardio-respiratory, 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. Careful planning and preparation with haematology input, good oxygenation, multi-modal analgesia, hydration, antibiotic prophylaxis, and maintenance of normothermia are the key preventative strategies utilized in this setting. Hydroxyurea, voxelotor, blood transfusion and haematopoietic bone marrow transplant are the available options for treatment. L-glutamine is not currently used in clinical practice in UK.