Treatment of sickle cell

Sickle cell disease (SCD) refers to a group of inherited red blood cell disorders that affect haemoglobin – a protein found in red blood cells that carries oxygen to all parts of the body. It is a genetic condition that is present at birth and is “inherited when a child receives two sickle cell genes—one from each parent”.1 It is caused by mutations in a gene that helps to make haemoglobin and results in those with the disease not producing haemoglobin properly.

Those with sickle cell produce an unusual crescent or ‘sickle’ shaped red blood cell. The red blood cells take this shape after the haemoglobin has delivered oxygen to the cells in the body’s tissues.2 Healthy red blood cells, in contrast, are round and sufficiently flexible to move through blood vessels.

Sickle-shaped red blood cells can cause problems because they are far less flexible than round red blood cells and thus do not move easily, particularly through smaller blood vessels.3 Consequently, they can get stuck, inhibit blood flow, and thus oxygen delivery to the rest of the body. This can cause severe pain (known as a ‘sickle cell crisis’) and other serious, long-term problems/complications including eye problems, stroke, jaundice, acute chest syndrome and infections.

Sickle cell can affect anyone, though it is more common in people from African and Caribbean backgrounds. NICE estimates that there are currently around 12,500–15,000 people with sickle cell disease in England while data from NHS Digital shows that there were 24,573 hospital admissions in England in 2020/21 where the primary diagnosis was sickle cell disorders.4

There are treatments which can prevent complications and lengthen the lives of those with SCD. Treatment options, however, can be different for each person depending on their symptoms and the severity of those symptoms. The NHS notes that treatments in England are typically delivered in a specialist sickle cell centre (known as Haemoglobinopathies Coordinating Centres (HCCs)). A list of HCCs is provided by NHS England.

Treatments relate to:

• preventing painful episodes;
• addressing sickle cell pain;
• preventing infections;
• treating sickle cell-related anaemia 5

References:

1 US National Heart, Lung and Blood Institute, Sickle Cell Disease, 1 September 2021

2 US National Heart, Lung and Blood Institute, Sickle Cell Disease, 1 September 2021

3 NHS, Overview: Sickle cell disease, 16 April 2019

4 NICE, Sickle cell disease: How common is sickle cell disease?, July 2021

5 NHS, Treatment: Sickle cell disease, 16 April 2019