Managing sickle cell disease

Introduction

In 1973, the average life span of a patient with sickle cell disease was 14 years. Thirty years later, the development of comprehensive care models, day hospitals, and ongoing efforts in both basic sciences and clinical research has increased life expectancy to 50 years. Advances in the management of sickle cell disease have dramatically changed the outlook for patients (box 1).

Polymerisation of sickle haemoglobin is the catalyst in the development of vaso-occlusion. Additional factors are critical to the pathophysiology of sickling (the development of sickle cells in the blood), including abnormalities in coagulation, white cells, vascular endothelium, and damage to the membranes of red cells.1 The culmination of these factors results in red cells with reduced deformability that easily adhere to vascular endothelium, increasing the potential for decreased blood flow and vascular obstruction. As a result, patients develop both haemolytic anaemia and vasculopathy.2 Organ damage is also an ongoing problem. Although the course of the disease is punctuated by episodes of pain, the organ damage is often silent until far advanced. We discuss the advances in the clinical management of sickle cell disease and new therapies that are available.

Sources and selection criteria

Our review is based on information from published research studies from the Children's Hospital and Research Center at Oakland. Electronic searches were through Medline and PubMed, and we downloaded relevant articles. We also extracted information from reviews of current work and consultations with other experts and health providers in the specialty of sickle cell disease.

Optimal management

The optimal setting for the care of patients with sickle cell disease is …