Inhaled drugs for opening up the airways in cases of acute chest syndrome in people with sickle cell disease

Sickle cell disease is an inherited blood disorder. People with sickle cell disease often suffer from acute chest syndrome, although it is not known why. Acute chest syndrome can cause fever, coughing, chest pain and shortness of breath and can be life-threatening. Often, people with sickle cell disease and acute chest syndrome also wheeze. This suggests that airways are narrowed, as with asthma. Bronchodilators are drugs which relax the muscles in the airways, thus opening them up to make breathing easier. They are used in this way for asthma, so may be of similar use in acute chest syndrome. However, to March 2014, we found no trials to show the effects of these drugs for this condition. Research needs to assess the benefits and risks of using inhaled bronchodilators for acute chest syndrome in people with sickle cell disease.

Authors' conclusions: 

If bronchial hyper-responsiveness is an important component of some episodes of acute chest syndrome in people with sickle cell disease, the use of inhaled bronchodilators may be indicated. There is need for a well-designed, adequately-powered randomised controlled trial to assess the benefits and risks of the addition of inhaled bronchodilators to established therapies for acute chest syndrome in people with sickle cell disease.

Read the full abstract...
Background: 

Bronchodilators are used to treat bronchial hyper-responsiveness in asthma. Bronchial hyper-responsiveness may be a component of acute chest syndrome in people with sickle cell disease. Therefore, bronchodilators may be useful in the treatment of acute chest syndrome.

Objectives: 

To assess the benefits and risks associated with the use of bronchodilators in people with acute chest syndrome.

Search strategy: 

We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Trials Register comprising references identified from comprehensive electronic database searches, handsearches of relevant journals and abstract books of conference proceedings. Additional searches were carried out on MEDLINE (1966 to 2002) and Embase (1981 to 2002).

Date of the most recent search of the Group's Haemoglobinopathies Trials Register: 17 March 2014.

Selection criteria: 

Randomised or quasi-randomised controlled trials. Trials using quasi-randomisation methods will be included in future updates of this review if there is sufficient evidence that the treatment and control groups are similar at baseline.

Data collection and analysis: 

We found no trials investigating the use of bronchodilators for acute chest syndrome in people with sickle cell disease.

Main results: 

We found no trials investigating the use of bronchodilators for acute chest syndrome in people with sickle cell disease.