Sickle-cell/Hb-C disease with acute chest syndrome

This code identifies a specific type of sickle cell disease, Sickle-cell/Hb-C disease, when it is complicated by acute chest syndrome. Sickle-cell/Hb-C disease is a compound heterozygous hemoglobinopathy where an individual inherits one sickle cell gene and one hemoglobin C gene. Acute chest syndrome is a severe pulmonary complication characterized by fever, respiratory symptoms, and new pulmonary infiltrates on chest X-ray.

This code is used when a patient with a confirmed diagnosis of Sickle-cell/Hb-C disease presents with or is admitted for acute chest syndrome. Documentation should clearly state the diagnosis of Sickle-cell/Hb-C disease and describe the signs, symptoms, and diagnostic findings consistent with acute chest syndrome, such as new lung infiltrates.

• Ensure documentation specifies "Hb-C" and not another hemoglobinopathy type.
• Do not use this code for other forms of sickle cell disease (e.g., sickle cell anemia, sickle cell trait) with acute chest syndrome.
• Code any associated manifestations or complications of acute chest syndrome, such as pneumonia or respiratory failure, as secondary diagnoses.