Management

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:

there is some evidence for the beneficial effects in avoiding a crisis with the use of other treatments such as piracetam and zinc sulphate (6). These treatment options, along with malaria prophylaxis and hydroxyurea, were classified as "likely to be of beneficial" in the systematic review

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.