Breathing difficulties or chest pain in Sickle Cell

You must attend the Emergency Department (ED) without delay if you are having difficulty in breathing, chest pain, or cough and fever.Acute Chest Syndrome (ACS) is a special form of acute Sickle Cell “crisis” and is serious because it can rapidly become life threatening. It refers to a collection of respiratory problems, such as chest or upper back pain, cough, breathing difficulty, low oxygen levels, and changes on a chest xray. If it is not treated quickly, the lower oxygen intake through the lungs worsens the sickling of red blood cells which in turn causes lower oxygen levels and a vicious cycle of deterioration.

Acute Chest Syndrome can be caused by: a severe pain crisis, chest infection/pneumonia, a blood clot in the lungs (pulmonary embolus,PE), or after an operation when you may be unable to breath as well as normally.


The ED will give you all the care you would normally receive for a pain crisis, and in addition the medical team will usually suggest you have a blood transfusion, as this has been shown to improve recovery from the episode. You will also be prescribed antibiotics, perhaps blood thinning medication, and a breathing device called an incentive spirometer. This machine helps you to get the oxygen you breath in deep into the lungs. If you have asthma you will also be asked to use inhalers.

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