Oncology & Hematology Coding Alert

Coding for sickle-cell disease (SCD) (or sickle-cell disorder as it is also referred to in ICD-10-CM) can be especially tricky. Not only do you have to document the different types of SCD correctly, but as the D57.- (Sickle-cell disorders) codes are combination codes, you have to know the specific complications that occur with it as well.

This means you have to keep a lot of clinical terminology straight. But we’ve got your back with the following comprehensive guide to coding this inherited red blood cell disorder.

First, though, it’s important that you understand what SCD is and how it affects the body, as that information will help you understand the condition’s complications and make the correct code choice.

1. Know What SCD Is

SCD affects “1 in 500 African American children and 1 in 36,000 Hispanic American children,” according to Mahesh B. Borhade and Noah P. Kondamudi in their article “Sickle Cell Crisis” (www.ncbi.nlm.nih.gov/books/NBK526064/). SCD patients suffer from abnormal hemoglobin (Hb), which changes the normally round shape of their red blood cells to a sickle, or C, shape. These unhealthy cells either die off quickly, creating a low Hb level in the blood, or they block the patient’s blood vessels, creating complications such as acute pain crises (www.cdc.gov/ncbddd/sicklecell/facts.html).

2. Know the SCD Types

As SCD is an inherited disease, SCD types are determined by a person’s genes as follows:

HbSS: The most common type, HbSS, also known as sickle cell anemia, occurs when the patient inherits two abnormal hemoglobin “S” genes, one from each parent, which are the genes that form the distinctive sickle shape of the patient’s red blood cells.

Depending on the associated conditions, you’ll code HbSS either to D57.0- (Hb-SS disease with crisis) or D57.1 (Sickle-cell disease without crisis).

HbSC: This milder form of SCD occurs in people who inherit two abnormal hemoglobin genes — the “S” and the “C” genes — from different parents.

In the ICD-10-CM taxonomy, you won’t see a separate code for HbSC, but you will see it, along with Hb-S/Hb-C, listed as an inclusion term for D57.2- (Sickle-cell/Hb-C disease).

HbS beta thalassemia: In this form, the patient inherits the “S” gene from one parent, and another abnormal hemoglobin gene, beta thalassemia, from the other.

Again, depending on the type of beta thalassemia and associated conditions, you’ll code this SCD variation to D57.4- (Sickle-cell thalassemia).

HbSD, HbSE, and HbSO: These much rarer conditions are caused by the patient inheriting the “S” gene from one parent, and either an abnormal “D,” “E,” or “O” hemoglobin gene from the other.

You’ll code HbSD and HbSE using D57.8- (Other sickle-cell disorders) per the inclusion terms listed under that code. And even though it is not listed with them, HbSO will also go under that code as it is a specified condition, which is always coded using an “other,” rather than an “unspecified,” code.

3. Know the SCD Complications

Once you have determined the correct D57.- code by type, you now have to narrow down your code choice according to the patient’s complications. That process begins with determining whether the patient has suffered an “acute painful crisis,” which is “the main clinical feature of sickle cell disease,” according to Borhade and Kondamudi.

Without crisis: If the patient is not undergoing a crisis, your code choices are easy:

• D57.1 (Sickle-cell disease without crisis)
• D57.20 (Sickle-cell/Hb-C disease without crisis)
• D57.40 (Sickle-cell thalassemia without crisis)
• D57.42 (Sickle-cell thalassemia beta zero without crisis)
• D57.44 (Sickle-cell thalassemia beta plus without crisis)
• D57.80 (Other sickle-cell disorders without crisis)

With crisis: If the patient is suffering a crisis, however, your code choices are a little more difficult. That’s because “the term ‘sickle cell crisis’ is used to describe several acute conditions such as the vaso-occlusive crisis (acute painful crisis), aplastic crisis, splenic sequestration crisis, hyperhemolytic crisis, hepatic crisis, dactylitis, and acute chest syndrome,” according to Borhade and Kondamudi.

This means you will need your provider to be more specific than simply documenting that the patient has undergone an SCD crisis. You’ll need enough information to follow the ICD-10-CM taxonomy, which subdivides the “with crisis” category into the following conditions:

• Acute chest syndrome
• Splenic sequestration
• Cerebral vascular involvement
• Other specified complications
• Unspecified

Once you have narrowed down the specific condition and added the appropriate 4th, 5th, or 6th character to the code, your final task is to follow the instructions that accompany several ICD-10-CM subdivisions:

Cerebral vascular involvement: Here, you’ll need to follow the Code also instruction, which tells you to add a code from the I63.- (Cerebral infarction) group if applicable.

Other specified complications: In these categories, you should pay attention to the Use additional code instruction, which tells you to identify complications, such as K80.- (Cholelithiasis) or N48.32 (Priapism due to disease classified elsewhere).

Unspecified: You also need to pay attention to the inclusion terms for D57.819 (Other sickle-cell disorders with crisis, unspecified), which tell you to code vaso-occlusive pain not otherwise specified to D57.819.

And don’t forget: There is an easy-to-overlook Use additional code instruction that accompanies all the D57.- codes, which tells you to use R50.81 (Fever presenting with conditions classified elsewhere) if the patient is experiencing fever associated with SCD.

Test Your Skills

Your provider sees a patient who has been hospitalized with gallstones due to type HbSS SCD. The patient is running a fever.

In this encounter, you’ll need three codes to fully describe the patient’s condition:

• D57.09 (Hb-SS disease with crisis with other specified complication) for the SCD;
• K80.- (Cholelithiasis) to describe the gallstones, per the D57.09 Use additional code instruction; and
• R50.81 (Fever presenting with conditions classified elsewhere) for the fever, per the D57.- Use additional code instruction.