Sickle Cell Anaemia

Abstract

Sickle cell anaemia is a genetic disease, whose indispensable feature is the presence of a mutation in the beta globin gene that specifies one of the chains of haemoglobin. This mutation endows the haemoglobin with a new property: the capacity of polymerizing when deoxygenated. This mutation has several untoward consequences: haemolytic anaemia, acute chest syndrome, stroke, renal insufficiency, leg ulcers, osteonecrosis, etc. Lifespan is compromised and the only accepted treatment is hydroxyurea, which is capable of ameliorating the phenotype and extending the life of the patient.

Sickle cell disease: causes, treatments and the patient experience

“…Whilst this intervention may not be universally available, continuing professional development (CPD) has been available for management in of SCD in the community (Brousse et al, 2014). Specific responsibilities of the primary care team include (Patel, 2016): Access to primary care has been highlighted as a barrier to effective treatment; continuity of care and follow-up after discharge from hospital (Al Juburi et al, 2012). Alternative models of care within the community, for instance with specialist service involvement in providing home or day case management have been described (Brousse et al, 2014;Lanzkron et al, 2015;Raphael et al, 2008;Wright et al, 2004).…”