Hb-SS disease with dactylitis

This code represents a specific manifestation of sickle cell anemia, homozygous (Hb-SS disease), characterized by dactylitis. Dactylitis, often referred to as "hand-foot syndrome," is an early and painful complication of sickle cell disease, involving inflammation and swelling of the digits (fingers and/or toes). It is caused by vaso-occlusion in the small bones of the hands and feet.

Use this code when documentation clearly indicates a patient with confirmed homozygous sickle cell disease (Hb-SS) is experiencing dactylitis. This typically presents in infants and young children with painful swelling of the hands and/or feet. Supporting documentation would include physician notes detailing the diagnosis of Hb-SS and the clinical presentation of dactylitis.

• Always ensure the documentation specifies "Hb-SS disease" and not another sickle cell variant, as dactylitis can occur in other forms but would be coded differently.
• This code inherently includes the sickle cell disease; therefore, a separate code for sickle cell anemia (e.g., D57.00) is not typically needed unless further specificity is required for an unrelated manifestation.
• Consider sequencing this code as the primary diagnosis if the dactylitis is the reason for the encounter, or as a secondary diagnosis if the patient is being treated for another complication of their sickle cell disease.