Treating sickle cell disease

At UCLH we have one of the biggest apheresis units in the country, here automated red blood cell exchanges are performed. During an exchange an individual will have a majority of their blood removed and replaced with donated red blood cells. This is done by a large machine that takes the blood from a person, spins it to separate the products in a centrifuge, returns some of the products i.e. platelets to the patient and removes the red blood cells that are then discarded. This happens simultaneously, at no point does a person have no blood in their body. The exchange removes the sickle cells and so reduces the sickling process, although it is not removed completely. Exchanges need to be performed every 4 -6 weeks in order to keep a persons sickle percentage as low as possible. This will hopefully reduce the occurrence of pain, the risk of stroke, promote healing in leg ulcers and delay or prevent organ damage. It has had a positive effect on many individuals. Exchanges can also be performed by hand (manual red cell exchange), however this is on a much smaller scale than when done by a machine.

Although simple blood transfusions are used, they are not performed as often as they were previously as this can cause iron overload, which can cause further complications i.e. organ damage. During a blood transfusion, an individual will have a specific amount of donated red blood cells administered to them intravenously (through a vein). Top up transfusions are sometimes performed when the main problem is worsening anaemia. This will raise a persons haemoglobin and reduced some of the side effects that are induced through anaemia i.e. tiredness and breathlessness.

Hydroxyurea is a drug given to individuals with sickle cell to encourage production of young red blood cells (foetal haemoglobin). The foetal haemoglobin that is produced is not affected by the sickle mutation and so reduces the percentage of sickle cells in the body. It is often recommended if a person has frequent sickle cell crises and complications or suffers from severe anaemia. Hydroxyurea has been known to reduce the occurrence of sickle cell pain/ crises by up to 50% in some individuals.

As already discussed, these treatments are all given for preventative measures and to avoid certain complications that people with SCD are more susceptible to.

People with SCD are recommended regular follow up or review by a specialist sickle cell center, such as UCLH. We aim to monitor individuals regularly and start treatment before a complication is unmanageable or untreatable.