Hb-SS disease with dactylitis
ICD-10 D57.04 is a billable code used to indicate a diagnosis of hb-ss disease with dactylitis.
Hb-SS disease, commonly known as sickle cell disease, is a genetic blood disorder characterized by the production of abnormal hemoglobin, known as hemoglobin S (HbS). This condition leads to the distortion of red blood cells into a sickle or crescent shape, which can cause blockages in blood vessels, leading to pain and potential organ damage. Dactylitis, or hand-foot syndrome, is a common complication in infants and young children with Hb-SS disease, characterized by painful swelling of the fingers and toes due to vaso-occlusive crises. This condition is often the first clinical manifestation of sickle cell disease in infants. The underlying genetic factor is a mutation in the HBB gene on chromosome 11, which encodes the beta-globin subunit of hemoglobin. Patients with Hb-SS disease may experience recurrent pain episodes, anemia, increased risk of infections, and other complications such as acute chest syndrome and stroke. Management typically includes pain control, hydration, and preventive measures such as vaccinations and antibiotics to reduce infection risk. Regular monitoring and comprehensive care are essential to manage the chronic nature of this disease.
Detailed clinical history, lab results showing hemoglobin levels, and evidence of vaso-occlusive crises.
Management of pain crises, blood transfusions, and hydroxyurea therapy.
Ensure accurate documentation of all complications and treatments to support coding.
Growth and development assessments, vaccination history, and records of dactylitis episodes.
Routine check-ups, management of acute pain episodes, and preventive care.
Focus on early identification of complications and comprehensive care plans.
Used during hospitalization for severe anemia due to Hb-SS disease.
Document the reason for transfusion and patient response.
Hematology specialists should ensure compliance with transfusion protocols.
Dactylitis is often one of the first signs of sickle cell disease in infants and indicates vaso-occlusive crises, which can lead to further complications if not managed properly.
