Sickle-cell thalassemia beta zero with crisis with other specified complication
ICD-10 D57.438 is a billable code used to indicate a diagnosis of sickle-cell thalassemia beta zero with crisis with other specified complication.
Sickle-cell thalassemia beta zero is a genetic blood disorder characterized by the presence of both sickle cell disease and beta-thalassemia. This condition leads to the production of abnormal hemoglobin, which can cause red blood cells to become rigid and sickle-shaped, leading to hemolytic anemia. Patients often experience painful crises due to vaso-occlusive events, where sickled cells obstruct blood flow in small vessels. The 'with crisis' designation indicates that the patient is currently experiencing an acute episode, which may be accompanied by other specified complications such as infections, acute chest syndrome, or splenic sequestration. Management typically involves pain control, hydration, and sometimes blood transfusions. Genetic factors play a significant role in the severity of the disease, as the presence of beta-thalassemia can modify the clinical course of sickle cell disease, potentially leading to a milder or more severe phenotype depending on the specific mutations present.
Detailed patient history, genetic testing results, and documentation of crisis events.
Management of acute sickle cell crises, monitoring for complications, and coordinating care with other specialties.
Ensure that all complications are documented clearly to support the use of this specific code.
Genetic testing results, family history of hemoglobinopathies, and detailed patient counseling notes.
Counseling patients on genetic risks, interpreting genetic tests, and managing hereditary conditions.
Documentation must clearly outline the genetic basis of the condition to support coding.
Used during acute crises requiring blood transfusions.
Document the need for transfusion due to anemia or crisis.
Hematology specialists should ensure that transfusion protocols are followed.
The 'with crisis' designation indicates that the patient is currently experiencing an acute episode related to their sickle-cell thalassemia, which can significantly impact treatment and management strategies.