Sickle-Cell Disorder P013

Current RMA Instruments

Changes from previous Instruments

ICD Coding

• ICD-10-AM Codes: D57

Brief description

Sickle-cell disorder is a genetic disorder resulting in the production of haemoglobin S (HbS), an abnormal form of haemoglobin affecting red blood cells. Clinical severity varies, ranging from largely asymptomatic forms to severe disease associated with chronic anaemia, vaso-occlusive complications, end-organ damage, pain crises and recurrent hospitalisation.

Sickle-cell disorder may involve vaso-occlusive, anaemic or infectious complications resulting from deformation of red blood cells. 

Confirming the diagnosis

The diagnosis is confirmed by laboratory or genetic testing.

Diagnostic assessment may include haemoglobin electrophoresis, genetic testing, or other laboratory evaluation demonstrating the presence of haemoglobin S (HbS).

The diagnosis may also be established in asymptomatic individuals through genetic testing. Management and confirmation are usually undertaken by a haematologist.

Diagnoses covered by SOP

• Sickle-cell disease (HbSS)
• Sickle-cell anaemia
• Sickle-cell trait (HbSA)  
• Double heterozygous sickling disorders involving one HbS gene and another β-globin gene variant. 

Examples of double heterozygous sickling disorders include:

• Sickle-cell/Hb-C disease; and
• Sickle-cell beta thalassaemia.

Conditions not covered by SOP

• Other hereditary haemolytic anaemias, such as hereditary spherocytosis, hereditary elliptocytosis and other haemoglobinopathies

# non-SOP condition

Clinical onset

Sickle-cell disorder is a genetic condition present from birth, although clinical manifestations may not become apparent until later infancy or childhood.

Clinical onset generally corresponds to the time when manifestations or complications attributable to sickle-cell disorder first become clinically evident, or when the diagnosis is established through laboratory or genetic testing.

Clinical worsening

Clinical worsening may be evidenced by increasing frequency or severity of vaso-occlusive, anaemic or infectious complications, progressive end-organ damage, or increasing requirement for hospitalisation or specialist management.

Clinical worsening may also include progression of chronic complications associated with sickle-cell disorder. Specialist advice would be important when evaluating whether there has been clinical worsening.