Recommendations for the management of sickle cell disease in South Africa.

The spectrum of sickle cell disease (SCD) encompasses a heterogeneous group of disorders that include: (I) homozygous SCD (HbSS), also referred to as sickle cell anaemia; (ii) heterozygous SCD (HbAS), also referred to as sickle cell trait; and (iii) compound heterozygous states such as HbSC disease, HbSß thalassaemia, etc. Homozygous or compound heterozygous SCD patients manifest with clinical disease of varying severity that is influenced by biological and environmental factors, whereas subject with sickle cell trait are largely asymptomatic. SCD is characterized by vaso-occlusive episodes that result in tissue ischaemia and pain in the affected region. Repeated infarctive episodes cause organ damage and may eventually lead to organ failure. For effective management, regular follow-up with support from a multidisciplinary healthcare team is necessary. The chronic nature of the disease, the steady increase in patient numbers, and relapsing acute episodes have cost implications that are likely to impact on provincial and national health budgets. Limited resources mandate local management protocols for the purposes of consistency and standardisation, which could also facilitate sharing of resources between centres for maximal utility. These recommendations have been developed for the South African setting, and it is intended to update them regularly to meet new demands and challenges.

Skull bone infarctive crisis and deep vein thrombosis in homozygous sickle cell disease- case report and review of the literature.

Here we describe an 8-year old male child with homozygous sickle cell disease who presented with left parietal skull bone infarction and, during his stay in hospital, developed a right femoral deep vein thrombosis (DVT), both uncommon complications of the disease. He initially presented with severe headache and generalised tenderness of the calvarium, which did not respond to simple analgesics. Scalp swelling in and around the left frontal (including left orbit) and parietal regions developed 24 h after presentation. The differential diagnosis included incipient stroke, acute sickle bone crisis and osteomyelitis, with a possible complication of epidural haematoma, or orbital compression syndrome. An initial exchange blood transfusion did not lead to appreciable reduction in opiate requirements. Significant symptomatic relief was attained only after a second exchange transfusion. The DVT developed at the site of catheterisation (right femoral vein), and this was treated with maximal doses of enoxaparin followed by warfarin. The child is now well and off anti-coagulants. In this article we present a review of the literature and discuss possible mechanisms of these complications in our patient.