Introduction

An inherited disorder of red blood cells (RBC). RBCs are essential for the transportation of oxygen around the body. Anaemia is the term for the reduction or reduced efficiency of RBCs. Normal RBCs are round and in most people they are able to bend to go through small vessels. They then “pop back” into their normal shape. In people with sickle cell disoder, the RBCs can form into a crescent, or sickle shape, but they are unable to return to a normal shape.

Symptoms

The symptoms of sickle cell disorder include;

• chronic pain
• pain crises, in which there are sudden attacks of pain, often in the abdomen, bones and joints
• increase in infections
• a swollen and tender spleen
• delayed growth in children
• problems in the long term with heart, kidneys, eyes
• priapism, or a prolonged painful erection, in men

Sickle Cell Disorder may lead to chest pain and Sudden Cardiac Arrest (SCA) due to the sickle cells causing blockages in the coronary arteries.

Treatment

People with sickle cell anaemia need a range of treatments throughout their lives. These treatments include:

• drinking plenty of fluids and staying warm to prevent episodes of pain
• taking pain relief medicines (for example, paracetamol or ibuprofen) to manage pain
• being fully vaccinated to prevent infection and sometimes taking daily antibiotics
• having regular blood transfusions (usually every 3–4 weeks) to treat the anaemia

Sometimes children who have significant symptoms from sickle cell anaemia may be offered a bone marrow transplant. Some researchers are now experimenting with gene therapy to treat the disorder.