Replacing fluids to treat acute episodes of pain in people with sickle cell disease

Sickle cell disease is a common genetic disorder characterised by periodic episodes of pain which usually happen again and again throughout life. These episodes occur when sickled cells obstruct blood vessels. The degree of pain may range from a mild discomfort to a severe disabling pain where the person needs treatment in hospital. A drop in body fluid levels promotes and sustains the sickling process. Routine treatment includes the use of drugs to relieve pain and the maintenance of adequate fluid levels. Fluid levels will fall if insufficient fluids are taken in compared to the amount of fluid lost. The kidneys of people with sickle cell disease do not concentrate urine properly, which results in poor control of bodily fluids. Additional fluids are given to try and slow or stop the sickling process which should reduce the amount and duration of pain. Fluids may be given in many ways, but orally or intravenously are most common. Fluids should be given with care so as to prevent fluid overload which may in turn cause adverse events such as heart failure or fluid building up in the lungs. We looked for randomised controlled trials to show the best way to replace fluids, which type of fluids and how much fluid to replace to treat acute episodes of pain in people with sickle cell disease. We did not find any such trials. We conclude that there is a need for large multi-centre trials to examine these questions.

Authors' conclusions: 

Treating vaso-occlusive crises is complex and requires multiple interventions. Extra fluids, generally oral or intravenous, are routinely administered during acute painful episodes to people with sickle cell disease regardless of the individual's state of hydration. Reports of their use during these acute painful episodes do not state the efficacy of any single route, type or quantity of fluid compared to another. However, there are no randomised controlled trials that have assessed the safety and efficacy of different routes, types or quantities of fluid. This systematic review identifies the need for a multicentre randomised controlled trial assessing the efficacy and possible adverse effects of different routes, types and quantities of fluid administered to people with sickle cell disease during acute painful episodes.

Read the full abstract...

Treating vaso-occlusive painful crises in people with sickle cell disease is complex and requires multiple interventions. Extra fluids are routinely given as adjunct treatment, regardless of the individual's state of hydration with the aim of slowing or stopping the sickling process and thereby alleviating pain. This is an update of a previously published Cochrane Review.


To determine the optimal route, quantity and type of fluid replacement for people with sickle cell disease with acute painful crises.

Search strategy: 

We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Trials Register which comprises of references identified from comprehensive electronic database searches and handsearches of relevant journals and abstract books of conference proceedings.

We also conducted searches of Embase (November 2007), LILACS and (05 January 2010).

Date of most recent search of the Group's Haemoglobinopathies Trials Register: 24 September 2014.

Selection criteria: 

Randomised and quasi-randomised controlled trials that compared the administration of supplemental fluids adjunctive to analgesics by any route in people with any type of sickle cell disease during an acute painful episode, under medical supervision (inpatient, day care or community).

Data collection and analysis: 

No relevant trials have yet been identified.

Main results: 

Sixteen trials were identified by the searches, all of which were not eligible for inclusion in the review.