Treatment

There are no specific drugs or routine interventions that cure sickle cell anaemia. People require monitoring and treatment throughout their lives to ameliorate the effects of crises and complications of the disease.

Treatment of the crisis

During a crisis the person requires adequate pain relief, to be well hydrated, to keep warm and to rest for a few days. Some will require strong opiates such as morphine or diamorphine for pain relief, and these drugs may need to be given by injection. Moderately potent analgesics such as tramadol, dihydrocodeine or co-codamol are also used. Intravenous fluids and drugs to control vomiting may be necessary. Painful crises usually last a few days, and as the acute symptoms abate, the person is encouraged to mobilise and return to normal activities. Resolution of symptoms is likely to take place over seven to ten days. There is a small group of people who are very severely affected and these may take longer to recover from crises, for example spending 3-4 weeks in hospital on each occasion.

Some people are able to manage their crisis at home with care from relatives and advice and/or home nursing from specialist sickle cell teams. Others may routinely be admitted to hospital for a few days. Indications for admission include severe pain, oxygen administration, intravenous fluids and treatment of infections such as pneumonia or septicaemia. Hospital admission is also needed for severe anaemia requiring blood transfusion.

Long-term treatments

A number of measures are undertaken to maintain health and to try to avoid crises -:

• Balanced diet, adequate fluid intake and avoidance of cold
• Regular folic acid supplements to replace loss by haemolysis
• Continuous oral penicillin ( twice daily) to reduce risk of infection
• Prompt treatment of infections
• Full courses of vaccinations and flu vaccination
• Prophylactic regular blood transfusions for people at risk of severe complications such as stroke

The following treatments may be prescribed for particular associated problems:

• Hydroxycarbamide (hydroxyurea), a drug that affects red cell production, has a role in reducing the incidence of crises.
• Analgesic drugs e.g. paracetamol, co- codamol, dihydrocodeine, tramadol and anti-inflammatory drugs are prescribed for bone pain and arthritis. Severely arthritic joints may be suitable for artificial joint replacement.
• Skin ulcers are treated with regular debridement and dressings. They may improve with bed rest but often relapse, when skin grafting is undertaken with variable success.
• Treatment of renal failure including renal transplantation may be necessary – click on the link for details of Kidney disorders guidance.
• Bone marrow transplantation has the potential to cure the condition. However it has a relatively high mortality rate of 5 – 10% and is only suitable for some people.

Amended May 2009