Lung Condition: Acute Chest Syndrome

Acute chest syndrome (ACS) is a complication of sickle cell disease (SCD). Sickle cell disease is a genetic disorder that affects a protein found in red blood cells, known as hemoglobin. With sickle cell disease, your red blood cells are shaped like crescents instead of their typical round shape. This causes difficulty for the sickle red blood cells to pass through blood vessels, meaning that the hemoglobin is unable to carry an adequate amount of oxygen throughout the body.

Acute chest syndrome is a severe lung-related complication of this disease that can affect both children and adults, creating symptoms similar to pneumonia. This condition occurs when sickle cells block the blood vessels in the lungs, and is considered one of the leading causes of hospitalizations and death in people with sickle cell disease.

There are many factors that may be related to the development of acute chest syndrome, including other medical conditions such as pulmonary embolism, fat embolism, bone marrow necrosis, infections like pneumonia, vaso-occlusive crisis (VOC), and asthma. Other possible causes of acute chest syndrome can include low oxygen levels, low fetal hemoglobin, recent trauma or surgery, smoking or secondhand smoke exposure, age, and genotype.

Acute chest syndrome can present itself differently in children and adults, with children typically showing signs of infection. Their symptoms include cough, fever, low concentration of oxygen in the blood (hypoxemia), rapid breathing, and wheezing. Adults may have all these symptoms, as well as chest pain, difficulty breathing, fluid buildup in the lungs and chest, and acute pain in the arms, legs, and back that occurs due to blocked blood flow.

People with SCD who develop acute chest syndrome require immediate treatment to prevent complications such as altered mental status, kidney injury, liver dysfunction, multi-organ failure, respiratory failure, severe pain, acute respiratory distress syndrome (ARDS), which is a life-threatening lung injury, and even death.

If you have been diagnosed with sickle cell disease, your doctor can give you possible methods of preventing the development of acute chest syndrome. These methods include exercising regularly to strengthen your lungs, receiving frequent blood transfusions, blowing into a bottle often to clear your airways and reduce fluid buildup around your lungs, and taking hydroxyurea or L-glutamine to reduce pain and prevent hospitalization.

If you believe that you may have acute chest syndrome, it is important to seek medical attention immediately so you can be diagnosed and begin treatment. To determine if you have this condition, your doctor will begin with a physical exam and ask questions about your medical history. Your doctor will then listen to your breathing with a stethoscope, order a chest X-ray, and check your oxygen saturation levels with pulse oximetry.

Your doctor may also conduct other tests such as blood tests, including a complete blood count, metabolic panel, and cultures, as well as bronchoscopy, computed tomographic angiography (CTA), ultrasound of the legs to measure blood flow speed, electrocardiogram, or an MRI.

Diagnosing acute chest syndrome early is extremely important, as early treatment can help prevent a variety of complications. Treatment for this condition may include blood transfusions, medications for pain relief, supplemental oxygen therapy, antibiotics and antiviral medications, and IV fluids to prevent dehydration, as this can worsen sickle cell disease. Your doctor may also give you an incentive spirometer, which is a device that encourages you to take deep breaths and helps keep your lungs clear.

A new gene therapy, known as LentiGlobin, is also currently being studied for long-term results that may provide a potential cure for sickle cell disease. Although this form of treatment is still in its early stages, researchers are optimistic about the possible benefits it may provide.

At Gwinnett Pulmonary and Sleep, we offer services to help determine if you are dealing with lung conditions such as acute chest syndrome, and treatment to help if you are. These services include pulse oximetry and pulmonary function tests, such as spirometry, lung volume, and diffusion capacity; as well as pulmonary rehabilitation.

If you suspect that you may have acute chest syndrome, it’s important to get tested as soon as possible so that you can begin receiving treatment. Learn more about our services here, or schedule an appointment to talk to our doctors.