Sickle-cell thalassemia beta zero with crisis, unspecified
ICD-10 D57.439 is a billable code used to indicate a diagnosis of sickle-cell thalassemia beta zero with crisis, unspecified.
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 mixed phenotype of symptoms. Patients often experience episodes of vaso-occlusive crises, which can result in severe pain, organ damage, and increased risk of infections. The severity of symptoms can vary widely depending on the genetic makeup and environmental factors. In this unspecified crisis state, the patient may present with acute pain episodes, anemia, and other complications related to hemolytic anemia, such as jaundice and splenomegaly. Management typically involves pain control, hydration, and sometimes blood transfusions. Genetic counseling is also recommended for affected individuals and their families due to the hereditary nature of the disease.
Detailed clinical notes on hemoglobin levels, crisis management, and treatment plans.
Management of pain crises, blood transfusions, and monitoring for complications.
Ensure documentation reflects the complexity of the condition and any genetic testing performed.
Family history, genetic testing results, and counseling notes.
Genetic counseling for families, prenatal testing discussions.
Accurate documentation of genetic factors and inheritance patterns is crucial.
Used during hospitalization for severe anemia or crisis management.
Document the reason for transfusion and patient response.
Hematology specialists should ensure transfusion protocols are followed.
The beta zero designation indicates that there is a complete absence of beta-globin chain production, leading to more severe symptoms compared to other forms of thalassemia.
