Sickle-cell disorders
Chapter 3:Diseases of the blood and blood-forming organs
ICD-10 D57 is a billable code used to indicate a diagnosis of sickle-cell disorders.
Sickle-cell disorders encompass a group of inherited blood disorders characterized by the presence of abnormal hemoglobin, specifically hemoglobin S. This condition leads to the distortion of red blood cells into a sickle or crescent shape, particularly under low oxygen conditions. The sickle-shaped cells are less flexible and can obstruct blood flow in small vessels, leading to episodes of pain, known as sickle cell crises, and increased risk of infections due to spleen dysfunction. Hemolytic anemia is a common complication, resulting from the premature destruction of these abnormal red blood cells. Sickle-cell disease (SCD) is the most severe form, often requiring comprehensive management strategies, including pain management, blood transfusions, and hydroxyurea therapy. Genetic factors play a crucial role, as the disorder is inherited in an autosomal recessive pattern. Other related conditions include thalassemias and enzyme deficiencies that can exacerbate the clinical picture. Accurate coding is essential for appropriate treatment and management of these patients, as they often present with a variety of complications that require multidisciplinary care.
Detailed clinical history, lab results, and treatment plans must be documented.
Management of pain crises, blood transfusions, and hydroxyurea therapy.
Documentation should specify the type of sickle cell disorder and any complications.
Growth and development assessments, vaccination status, and family history.
Routine check-ups, management of infections, and monitoring for complications.
Consideration of developmental milestones and psychosocial factors in children with SCD.
Used when a patient with SCD requires a blood transfusion due to severe anemia.
Document the indication for transfusion and patient’s hemoglobin levels.
Hematology specialists should ensure compliance with transfusion protocols.
Sickle cell trait indicates that a person carries one sickle cell gene and typically does not exhibit symptoms, while sickle cell disease occurs when an individual inherits two sickle cell genes, leading to various health complications.
