Sickle Cell Disease ICD-10-CM codes

Category D57 distinguishes the genotype first and then whether the patient is in crisis. Hb-SS disease is D57.0- with crisis and D57.1 without crisis. Sickle-cell/Hb-C disease is D57.2- (D57.20 without crisis, D57.21- with crisis), sickle cell thalassemia is D57.4- (with separate subcategories for beta zero D57.42 and D57.43-, and beta plus D57.44 and D57.45-), and other sickle cell disorders are D57.8-. D57.3 is sickle cell trait, which is a carrier state, not a disease, and is not used for a patient with any form of sickle cell disease.

The crisis codes carry the complication in the final character: 0 or 9 crisis unspecified, 1 acute chest syndrome, 2 splenic sequestration, 3 cerebral vascular involvement, 4 dactylitis, and 8 or 9 other specified complication. A vaso-occlusive pain crisis with no other complication documented is D57.00 for Hb-SS disease. Pain is integral to the crisis and is not coded separately. When the crisis has a specified complication, the tabular instructs to use additional codes for the associated condition, such as I63.- for stroke with D57.03 or J18.9 for pneumonia with D57.01.

A patient with sickle cell disease seen for an unrelated problem or routine follow-up with no crisis is coded D57.1 (or the without-crisis code for their genotype). Documentation of sickle cell disease alone with no genotype defaults to Hb-SS (D57.1 or D57.0-). Thalassemia without sickle hemoglobin is coded to D56 (D56.1 beta thalassemia, D56.9 unspecified). Hydroxyurea therapy may be reported with Z79.899.