Acute chest syndrome in sickle cell disease

It can worsen quickly, so new chest pain, fever, cough, breathing difficulty, or unusual sleepiness in someone with sickle cell disease should not be managed at home while waiting to see whether it passes.

The syndrome occurs when part of the lung becomes inflamed or blocked and a new abnormal area appears on a chest image alongside breathing-related symptoms. Infection, trapped sickled blood cells, or fat released from bone marrow can contribute, and more than one process may occur together.

People with acute chest syndrome may develop fever, cough, chest pain, shortness of breath, fast breathing, wheezing, or lower oxygen levels. Children may initially have fever and cough, while adults may have more prominent chest pain or breathing difficulty.

Seek emergency assessment and tell the clinical team immediately that the person has sickle cell disease. Keep them resting, bring their medicine list and sickle cell care information if readily available, and do not delay travel to try home remedies.

In hospital, clinicians may check oxygen levels, order a chest X-ray and blood tests, and look for infection or worsening anaemia. Treatment can include oxygen, antibiotics, careful pain relief, breathing exercises with an incentive spirometer, and appropriately managed fluids. A simple or exchange blood transfusion may be considered when oxygenation or illness severity warrants it.

Call the UAE ambulance service on 998 for severe breathing difficulty, blue or grey lips, collapse, confusion, marked drowsiness, or inability to speak normally because of breathlessness. These warning signs require immediate help.

Go to an emergency department promptly for new chest pain, fever, cough, shortness of breath, rapid breathing, or falling oxygen readings in anyone with sickle cell disease, including during or soon after a pain crisis or hospital procedure.

Ask what the chest imaging and oxygen readings show, whether infection is suspected, and how pain will be controlled without suppressing breathing. You can also ask whether transfusion is being considered, how breathing exercises should be used, and what follow-up plan may reduce another episode.