Anaesthetic management of children with sickle cell disease

Abstract

By reading this article, you should be able to: Describe the pathogenesis of sickle cell disease (SCD). Recognise the acute complications that occur in children with SCD. Illustrate the key issues in the perioperative management of a child with SCD. Explain the available strategies and rationale behind multimodal analgesia for children with SCD presenting with acute pain. Sickle cell disease (SCD) is an inherited disorder with multisystem complications, often presenting in childhood. 1 Anaesthetists are frequently involved throughout perioperative care, in the management of acute pain and acute complications. SCD results from inheriting genes coding for an abnormal b-globin chain from both parents, where at least one is the sickle cell gene. Sickle cell anaemia (SCA), the most severe and common form of SCD, results from inheriting sickle bglobin genes from both parents, leading to a homozygous HbSS state. 2,3 Other disease forms include heterozygous states involving other abnormal b-globin alleles such as b-thalassaemia (HbSß), C (HbSC), and D-punjabi (HbSD). Inheriting one sickle cell and one normal haemoglobin gene leads to sickle cell trait (SCT), which usually has no clinical consequences. SCD is a significant global public health issue. The WHO reports that the sickle cell gene is responsible for more than 80% of all inherited haemoglobinopathies, with 85% of SCD cases in Africa. 4 SCD also occurs in the Mediterranean, Arabia, South Asia, and elsewhere mainly as a result of migration. 3,4 The distribution of SCD closely matches the distribution of malaria, because the heterozygous states are protective against Plasmodium falciparum infection. 4 However, those with SCA are more susceptible, and more likely to develop lifethreatening malaria. There is a disparity between the availability of healthcare resources for the treatment of SCD relative to its geographical distribution. Resources are most limited in many African Sonia Akrimi FRCA MRCP PGCME is a speciality trainee in anaesthesia in the Health Education Kent, Surrey and Sussex Deanery. She has interests in medical education and global surgery.

Anaesthetic management of a patient with sickle cell disease: Case report and review of literature

“…Pain is associated with risk of atelectasis and subsequently ACS. 12 SCD is a hypercoagulable state due to haemostatic alterations such as increased activation of platelets and clotting cascade, intravascular haemolysis and impaired fibrinolysis. Therefore, hospitalized patients with SCD must receive venous thromboembolism prophylaxis if there is no contraindication since it is associated with increased mortality.…”