Get the Answers to These Frequently Asked Sickle Cell Disease Questions
Here’s how to handle reporting SCD crises. Sickle cell disease (SCD) “affects more than 100,000 people in the United States and 8 million people worldwide. In the United States, 9 of 10 people who have sickle cell disease are of African ancestry or identify as Black,” according to the National Heart, Lung, and Blood Institute (NHLBI). That’s reason enough to sharpen your SCD diagnosis reporting skills. But keep reading, as there’s more to SCD coding that meets the eye, especially when it comes to understating the condition’s complications. What Is Sickle Cell Disease? SCD, also known as sickle-cell disorder in ICD-10-CM, is a genetic disorder that affects red blood cells. The condition affects the cell shape, changing it from its usual disc shape to a crescent, or sickle, shape. This makes it harder for the cells to move around the blood vessels in the body, which in turn causes the cells to die off quickly, producing abnormal hemoglobin (Hb) levels; or the cells block the vessels completely, creating complications such as acute pain crises. How Is SCD Coded? SCD is divided into several types, which are named for the genetic mutations creating the condition type. The types, more or less, correspond to specific ICD-10-CM codes in the D57.- (Sickle-cell disorders) code subcategory. 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. Coded to: Either D57.0- (Hb-SS disease with crisis) or D57.1 (Sickle-cell disease without crisis) depending on the associated conditions. More on that in a minute. HbSC: This milder form of SCD occurs in people who inherit two abnormal hemoglobin genes — the “S” and the “C” genes — from different parents. Coded to: 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 zero thalassemia: In this form, the patient inherits the “S” gene from one parent, and another abnormal hemoglobin gene, beta thalassemia, from the other. Coded to: 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. Coded to: 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. Sickle cell trait: It is possible for a patient to have a copy of the sickle cell gene but not experience any of the symptoms of the condition. If patient undergoes screening for the disease, and the screening results in the patient testing positive for the trait, you should go ahead and use Z13.0 (Encounter for screening for diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism). This identifies the patient as a carrier of the sickle cell gene. What Is Sickle Cell Crisis? Also known as a vaso-occlusive crisis, this is one of the disease’s most serious complications. The pain, which can affect “the abdomen, chest, lower back, or arms and legs,” can be “triggered by high altitudes, dehydration, illness, stress, or temperature changes,” though “often a person does not know what triggers the crisis,” according to the NHLBI. Without crisis: If the patient is not undergoing an SCD crisis, your code choices are easy: With crisis: Coding choices in this situation are a little more complicated and require your provider to give you more information about the specific acute condition the patient is experiencing. You’ll begin by adding the 5th character “1” to indicate the patient is undergoing a crisis, then you’ll add the appropriate 6th character to specify the nature of the crisis: Once you have narrowed down the specific condition and added the appropriate 4th, 5th, and 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: Bruce Pegg, BA, MA, CPC, CFPC, Managing Editor, AAPC
