management

Last reviewed 10/2021

This is aimed at avoiding factors that may precipitate a crisis and maintenance of the steady state:

• educating patients, parents and carers on
• how to manage uncomplicated problems at home
• how to recognise the onset of serious complications so that the patient could be admitted to a hospital immediately (1,2)  
• good nutrition with regular supplements of folic acid especially during pregnancy
• all women with sickle cell disease should be advised to take a supplement of 5 mg folic acid per day (3)
• antibiotic prophylaxis and vaccination
• prompt treatment of any infection
• management of pain at home (2)
• maintain adequate fluid intake
• avoidance situation which might precipitate sickling e.g. – dehydration, acidosis, cold
• prevention of vascular stasis, e.g. tight clothing
• malaria prophylaxis
• Falciparum malaria is believed to precipitate sickle cell crisis and to increase the risk of death in children with sickle cell anaemia; therefore, regular chemoprophylaxis with antimalarial drugs is advocated by consensus (4)
• monitoring the of frequency of acute complication
• early recognition and prevention of chronic complications e.g. – cerebrovascular disease, pulmonary hypertension (5)
• providing psychological therapy to patients  (2)

Specific therapy used in sickle cell disease includes (2):

• hydroxyurea - there is evidence that hydroxyurea therapy can ameliorate the course of sickle cell anaemia in some adults
• mortality and morbidity rates in sickle cell disease (SCD) have been considerably reduced since the introduction of hydroxyurea (HU) in the 1990s
• an update of the Multicenter Study of Hydroxyurea (MSH) showed that at 9 years' follow-up, the mortality rate among patients who take HU is reduced 40% compared with the rate among patients who do not take the drug (6)
• transfusion therapy –
• is an essential and life-saving therapy for some acute complications of SCD
• has been shown to reduce the risk of chronic progressive organ damage in the case of ischaemic stroke
• bone marrow/stem-cell transplantation – is the only potentially curative treatment method (2)

Treatment is required when crises occur for the control of pain, the maintenance of hydration and correction of anaemia.

Notes:

Reference:

• (1) Sickle Cell Society 2005. Sickle cell: a guide for GPs, nurses and other health professionals  
• (2) NHS Antenatal and Newborn Screening Programmes 2010. Sickle cell disease in childhood: standards and guidelines for clinical care
• (3) Oteng-Ntim et al. Sickle cell disease in pregnancy. Curr. Obs. & Gynae.2003;13(6): 362-368.
• (4) Clinical Evidence (March 2006). Sickle Cell Disease
• (5) Rees DC, Williams TN, Gladwin MT. Sickle-cell disease Lancet. 2010 11;376(9757):2018-31
• (6) Steinberg MH, Barton F, Castro O, et al. Effect of hydroxyurea on mortality and morbidity in adult sickle cell anemia: risks and benefits up to 9 years of treatment. JAMA. 2003;289: 1645-1651.