Sickle-cell thalassemia beta zero with acute chest syndrome
ICD-10 D57.431 is a billable code used to indicate a diagnosis of sickle-cell thalassemia beta zero with acute chest syndrome.
Sickle-cell thalassemia beta zero is a complex hemoglobinopathy resulting from the combination of sickle cell disease and beta-thalassemia. This condition is characterized by the presence of both sickle-shaped red blood cells and reduced production of beta-globin chains, leading to a unique clinical presentation. Patients often experience hemolytic anemia due to the destruction of sickle cells, which can lead to various complications, including acute chest syndrome (ACS). ACS is a severe complication characterized by chest pain, fever, and respiratory symptoms, often triggered by infection, vaso-occlusive crises, or pulmonary fat embolism. The management of this condition requires a multidisciplinary approach, including pain management, oxygen therapy, and possibly blood transfusions. Genetic factors play a significant role in the pathophysiology of this disorder, as it is inherited in an autosomal recessive manner. Understanding the interplay between sickle cell disease and thalassemia is crucial for effective treatment and management of patients with this condition.
Detailed history of hemolytic anemia, genetic testing results, and treatment plans.
Management of pain crises, blood transfusions, and monitoring for acute chest syndrome.
Ensure documentation reflects the complexity of the condition and any acute complications.
Documentation of respiratory symptoms, imaging studies, and treatment for acute chest syndrome.
Evaluation and management of patients presenting with respiratory distress due to ACS.
Accurate coding of respiratory complications and their relation to sickle-cell disease.
Used during management of severe anemia or acute chest syndrome.
Document the reason for transfusion and patient response.
Hematology specialists should ensure accurate coding of transfusion events.
Coding D57.431 accurately reflects the complexity of a patient's condition, ensuring appropriate treatment and management strategies are implemented.
