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. Their recommendations may include:
Penicillin (or erythromycin in the case of allergy) is prescribed to all infants from the age of 3 months to prevent overwhelming pneumococcal sepsis. [1,2] Other infections, for example chest infection or osteomyelitis (infection of the bone) are treated with the appropriate antibiotic.
All infants should receive immunisations according to the universal schedule. In addition, those with sickle cell disease should receive extra vaccinations against Meningitis (Men ACWY) and Pneumococcus (PPV). The latter is given 5 yearly throughout life.
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 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 (see below). Blood transfusions come from donated blood, which is collected, carefully tested for infections such as hepatitis and HIV, and then closely matched to the recipient to prevent reactions.
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. It is important to remember that hydroxycarbamide must be taken every day for it to work well and regular blood tests are necessary both to monitor benefits and possible side effects. It is now recommended that hydroxycarbamide should be discussed with parents of all children with sickle cell disease from the age of 9 months in order to prevent the onset of long-term complications., improve quality of life and maintain healthy growth. However, while conversations should start from the age of 9 months, hydroxycarbamide is only licensed for use in children over the age of 2 years.
Early Recognition and Follow Up
The mortality in childhood sickle cell disease has fallen from 10% in the 1980’s to <1% now. This is almost entirely due to new-born screening, early registration into care and penicillin prophylaxis. Advice and support to parents is also crucial in insuring early recognition of symptoms and good access to medical care. The morbidity from stroke has reduced significantly with the introduction of routine transcranial Doppler screening from the age of 2 years. The adoption of the British Society of Haematology guideline to discuss with parents the early use of hydroxycarbamide and starting treatment from the age of 2 years, should improve quality of life further and prevent long- term complications before they develop.