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

Review question

Can inhaled, short-acting bronchodilators for acute chest syndrome reduce illness and mortality in people with sickle cell disease and does this treatment cause adverse effects? 

Background

Sickle cell disease is an inherited blood disorder. People with sickle cell disease often suffer from acute chest syndrome. 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. Wheezing suggests that airways are narrowed, as with asthma. Bronchodilators are drugs which relax the muscles in the airways, so they open up to make breathing easier. They are used in this way for asthma, so may be of similar use in acute chest syndrome. This is an update of a previously published Cochrane Review.

Search date

The evidence is current to: 25 July 2022.

Study characteristics

We found no trials to show the effects of these drugs for this condition.

Key results

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. This is an update of a previously published Cochrane Review.

Objectives: 

The aim of the review is to determine whether the use of inhaled, short-acting bronchodilators for acute chest syndrome reduces morbidity and mortality in people with sickle cell disease and to assess whether this treatment causes adverse effects.

Search strategy: 

We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group's 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 2004) and Embase (1981 to 2004) and ongoing trial registries (28 September 2022).

Date of the most recent search of the Group's Haemoglobinopathies Trials Register: 25 July 2022.

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.