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Severe ACS can be life threatening
Severe ACS can be life threatening

Acute chest syndrome in sickle cell disease

How different is this from pneumonia?

Worried Dad.

Dear Worried dad, Acute chest syndrome (ACS) is a problem that sometimes happens in people who have sickle cell disease.

People with sickle cell disease sometimes get pains in certain parts of the body due to oxygen deprivation to that part of the body.

This phenomenon is called vaso-occlusive crisis. Vaso-occlusive crisis occurring in the lungs is called Acute Chest Syndrome.

For us medics, we diagnose ACS through clinical manifestations as well as some evidence seen on x-rays of the lungs called pulmonary or lung infiltrate.

Acute chest syndrome is often precipitated by a lung infection, and the resulting inflammation and loss of oxygen saturation leads to further sickling of red cells, thus exacerbating lung and systemic oxygen deprivation, sickling, and vaso-occlusion.

The sickled red blood cells block the blood vessels in the lungs further worsening the situation. Also, fat which usually comes from damaged bones get to the lungs and damage the lungs.

These problems can cause sudden damage to the lung tissues leading to body fluid buildup in the area, much like the swelling that occurs from an injury.

This fluid buildup makes breathing difficult because the lungs become stiff from the accumulated extra fluid in the lungs. This extra fluid also impairs oxygen exchange in the lungs, worsening the already poor oxygen availability in the body.

Acute chest syndrome is often precipitated by a lung infection

About half of all patients with sickle cell disease have ACS at least once during their life. Once a child has ACS, the child is more likely to have it again. Repeated episodes of ACS can cause permanent damage to the lungs.

Severe ACS can be life threatening underscoring the need for patients and parents/caretakers to understand, identify early and seek early intervention.

Children with concurrent sickle cell disease and asthma or other types of lung problems are at a higher risk of having ACS. Patients with ACS present with fever, cough, chest pain, fast breathing and productive cough.

There is the need to seek urgent help in a hospital where they will be examined. Blood tests and x-ray will be taken and the oxygen saturation measured.

The key to prevent any crisis is regular follow ups, early recognition of signs and symptoms and taking religiously all the prescribed medications.

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A member of Paediatric Society of Ghana

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