Guideline summary
Sickle cell disease is a hereditary condition affecting the haemoglobin contained within red blood cells. It predominantly affects people of African or Afro-Caribbean origin, but can also affect people of Mediterranean, Middle Eastern and Asian origin. The red cells of patients with sickle cell disease are prone to assuming a permanently sickled shape when exposed to a variety of factors including hypoxia, cold or dehydration. These cells are prone to mechanical damage, hence the haemolytic anaemia in this group of patients, and to occluding the microvasculature leading to tissue hypoxia and pain or end organ damage.
These painful crises can result in damage to the patient’s lungs, kidneys, liver, bones and other organs and tissues. The recurrent nature of these acute episodes is the most disabling feature of sickle cell disease, and many chronic problems can result, including leg ulcers, blindness and stroke. Acute chest syndrome is the leading cause of death amongst sickle cell patients.
This guideline provides guidance for paramedics
1 for the assessment and management of patients suffering a sickle cell crisis.
1The principles will be applicable to all pre-hospital clinicians.
Updated sickle cell crisis guideline - issued 21st April 2009
2006 guideline - issued October 2006