There are several treatments available to help manage sickle cell (HbSS) and prevent or treat its symptoms. Your GP, hospital doctor or specialist nurse will recommend appropriate treatments for you or your child. Hydration, warmth and excellent nutrition can contribute to overall health quality.
Penicillin (or erythromycin in the case of allergy) is prescribed to all infants from the age of 3 months to prevent overwhelming pneumococcal sepsis.
Other infections, for example, chest infection or osteomyelitis (infection of the bone) are treated with the appropriate antibiotic.
Mild to moderate painful episodes may be managed with over-the-counter painkillers like ibuprofen and paracetamol which should be taken regularly until the episode is over. It is sometimes necessary to go to hospital for severe pain as stronger painkillers such as opiates may be needed.
Blood transfusions are used in the management of sickle cell disease in different ways. In the case of a sudden drop in haemoglobin, such as acute splenic sequestration or aplasia due to parvovirus infection, a simple top-up of non-sickle cell blood is given. In acute emergencies, such as a stroke or acute chest syndrome, an exchange blood transfusion is needed to reduce the amount of sickle cells in the blood. Regular, usually monthly, blood transfusions are indicated in the long-term management of stroke or if there is no response to other medications such as hydroxycarbamide.
Also known as hydroxyurea, hydroxycarbamide increases the amount of foetal haemoglobin (HbF) in the blood and reduces the frequency of painful episodes and acute chest syndrome. Hydroxycarbamide must be taken every day for it to work well and regular blood tests are necessary. It is now recommended that hydroxycarbamide should be discussed with parents of all children with sickle cell disease for the age of 9 months in order to prevent the onset of long-term complications, improve quality of life and maintain healthy growth.
Crizanlizumab (Adakveo) was recommended as a new disease modifying treatment for sickle cell anaemia by National Institute of Clinical Excellence (NICE) and NHS England on a Managed Access Agreement.
On the 26th May 2023, the European Medicines Agency's (EMA) Committee for Medicinal Products of Human Use (CHMP) made a recommendation to revoke the conditional marketing authorization for Crizanlizumab in sickle cell disease patients aged 16 years and older.
Findings from a recent study did not confirm that there was a clinical benefit of the drug in successfully reducing the number of painful crises requiring a healthcare visit or treatment at home in patients with sickle cell disease and so the benefits do not outweigh the risks.
Patients who are currently taking Crizanlizumab (Adakveo) as part of the Managed Access scheme, are reassured that it is safe. However, if you have any questions, we recommend that you speak to your Consultant Haemotologist who will determine the best options for each patient depending on their individual circumstances.
The CHMP's recommendation will be provided to the European Commission (EC) and a final decision will be made by the EC in the next two months.
Bone Marrow/ Stem Cell Transplant
Stem cell or bone marrow transplants are the only cure for sickle cell disease, but they're not done very often because of the significant risks involved. Stem cells are special cells produced by bone marrow, a spongy tissue found in the centre of some bones. They can turn into different types of blood cells.
A stem cell transplant is an intensive treatment that carries a number of risks. Stem cell transplants are generally only considered in children with sickle cell disease who have severe symptoms that have not responded to other treatments when the long-term benefits of a transplant are thought to outweigh the possible risks.
This information about sickle cell treatments is from the Sickle Cell Society and is available to download as a poster.
More information on sickle cell treatments can be found on their website- www.sicklecellsociety.org