Sickle-Cell Disorder P013

Current RMA Instruments

Changes from previous Instruments

ICD Coding

• ICD-9-CM Codes: 282.5, 282.6
• ICD-10-AM Codes: D57

Brief description

Sickle-cell disorder is a group of inherited disorders occurring predominantly in people of Afro-Carribean descent.  The disorders affect the haemoglobin of red blood cells.  There are benign forms (sickle-cell trait) and more serious forms (sickle-cell anaemia).  The latter may be associate with chronic ill health and pain and intermittent exacerbations (or crises).  

Confirming the diagnosis

The diagnosis is confirmed by laboratory testing.

The relevant medical specialist is a haematologist.

Additional diagnoses covered by SOP

• Sickle-cell disease
• Sickle-cell anaemia
• Sickle-cell trait
• Double heterozygous sickling disorders

Conditions not covered by SOP

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

Clinical onset

This is a genetic disorder. Clinical manifestations are not present at birth.  They usually begin to become apparent after the first few months of life.  Diagnostic testing in potentially affected individuals is usually undertaken prenatally, or at or soon after birth.  Clinical onset should predate military service in all cases.

Clinical worsening

Clinical worsening may be evidenced by a sickle-cell crisis or series of crises resulting in significant end-organ damage or death, or a substantial increase in the requirement for hospitalisation for management of the manifestations or complications of sickle-cell disorder.