Acute Chest Syndrome (ACS) is a condition that can severely impact lung health in those with sickle cell disease. Both children and adults can have acute chest syndrome. This condition causes an illness like pneumonia, and it is considered a life-threatening emergency. ACS can mimic the symptoms of other conditions, resulting in patients waiting too long to go to the hospital for medical treatment.
The common symptoms of ACS are:
The cause of Acute Chest Syndrome is a vaso-occlusion within the pulmonary structures. This condition only happens to those who have sickle cell disease. This occlusion happens because of the clotting problems related to sickle cell disease. In most cases, red blood cells stick together and form a clot, which clogs small blood vessels. This leads to the body not getting enough oxygen.
The risk factors for ACS are:
Diagnosis is often based on chest X-rays and pulse oximetry, which measures oxygen saturation. Blood tests like a CBC or metabolic panel might also be taken. Bronchoscopy can rule out a lung infection, and a CTA might be done to look at the blood vessels in the body and the lungs. The D-Dimer blood test will help to identify any risk of pulmonary embolism.
Prevention of ACS is often a key part of recovering from surgery if you have sickle cell disease. If you have to be inactive due to another illness, you still need to consider the risk of ACS and make sure that you are moving around and breathing correctly to prevent this complication. Patients who are at risk for ACS will be directed to get out of bed and walk at least 2 to 3 times a day when they are in the hospital or are recovering from surgery. Patients should also breathe deeply, even when they are in pain.
Some patients will be given an incentive spirometer to take home from the hospital. The device helps to measure slow, deep breaths and fill the lungs with air.
Treatment of this condition is often done on an emergency basis. Patients are almost always admitted to the hospital and placed on antibiotics to help ward off lung infections. Oxygen might be given to those who have poor oxygen saturation. Blood transfusions might also be needed.