Sickle-cell thalassemia beta zero with crisis
ICD-10 D57.43 is a billable code used to indicate a diagnosis of sickle-cell thalassemia beta zero with crisis.
Sickle-cell thalassemia beta zero with crisis is a complex hemolytic anemia resulting from the combination of sickle cell disease and beta-thalassemia. This condition arises due to mutations in the HBB gene, leading to the production of abnormal hemoglobin (HbS) and reduced production of beta-globin chains. Patients with this condition experience episodes of vaso-occlusive crises, which can lead to severe pain, organ damage, and increased risk of infections. The clinical presentation may include symptoms such as fatigue, pallor, jaundice, and episodes of pain due to sickle-shaped red blood cells obstructing blood flow. Management often involves pain control, hydration, and sometimes blood transfusions or hydroxyurea therapy to reduce the frequency of crises. Genetic counseling is also important for affected individuals and their families, as this condition is inherited in an autosomal recessive pattern. Regular monitoring and comprehensive care are essential to manage complications and improve quality of life.
Detailed patient history, genetic testing results, and treatment plans.
Management of pain crises, blood transfusions, and monitoring for complications.
Ensure documentation reflects the complexity of the condition and any associated comorbidities.
Family history, genetic counseling notes, and results of genetic tests.
Counseling families about inheritance patterns and risks for future offspring.
Accurate documentation of genetic testing and counseling is crucial for coding.
Used during hospitalization for severe anemia due to sickle-cell thalassemia beta zero.
Document the indication for transfusion and patient response.
Hematology specialists should ensure accurate coding of transfusion episodes.
The beta zero designation indicates that there is a complete absence of beta-globin production, which significantly impacts the severity of the disease and management strategies.
