How to Bill and Code for Sickle Cell Disease

by | Published on Sep 15, 2023 | Specialty Billing, Specialty Coding

Sickle Cell Disease
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Sickle cell anemia belongs to a group of inherited disorders known as sickle cell disease.It is marked by flawed hemoglobin. Red blood cells that are typically round and flexible can move easily through the blood vessels. Sickle cell anemia is characterized by some red blood cells that are crescent-shaped like a sickle. In a patient with this disease, the delivery of oxygen to the tissues is disrupted.

Causes and Symptoms

When two faulty hemoblobin genes are inherited by a person – one from the mother and another from the father – he/she is born with sickle cell disease. A person who inherits just one gene is healthy and said to be a “carrier” of the disease. If you are a carrier, you have an increased chance of having a child with sickle cell disease if you parent a child with another carrier.

Signs and Symptoms Can Include

  • Anemia. Sickle cells break apart easily and die, often within 10 or 20 days. This causes anemia and resultant fatigue.
  • Episodes of pain. Sickle cell anemia is characterized by periodic episodes of extreme pain or pain crises. This pain arises when the sickle-shaped red blood cells block the flow of blood through tiny blood vessels to the abdomen, chest and joints.
  • Swelling of hands and feet. When the sickle-shaped red blood cells block blood circulation in the hands and feet, swelling is caused in these parts of the body.

Accurate Medical Documentation Is Crucial

Just as any other health condition, sickle cell disease diagnosis and the treatments provided have to be accurately documented in the patient’s medical records.

Accurate and detailed documentation of the patient’s medical history, physical examination, diagnostic tests, and treatment is crucial. Error-free billing and coding for medical services related to sickle cell disease (SCD) is essential for healthcare providers to ensure both proper patient care and reimbursement. Professional medical billing and coding support can make a significant difference when it comes to your revenue cycle management. It is important to understand the latest coding guidelines to assign the correct codes for billing and reimbursement purposes. Healthcare practices can rely on the services of medical billing companies that have professional teams to help ensure that the medical diagnosis and services are billed using the correct codes.

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Steps in the Billing and Coding of Sickle Cell Disease

  • Use the Correct Diagnosis Codes:
  • Use the appropriate ICD-10 diagnosis codes to represent Sickle Cell Disease on your medical claims. The primary code for SCD is D57 (Sickle-Cell Disorders), followed by additional codes to specify the type and severity of the disease.

    D57.00 – Sickle-cell anemia, unspecified
    D57.01 – Sickle-cell disease without crisis
    D57.219 – Sickle-cell disease with crisis
    D57.219 – Sickle-cell/Hb-C disease with crisis
    D57.42 – Sickle-cell thalassemia beta zero without crisis

  • Include the Relevant Procedure Codes:
  • When billing for specific medical services, use Current Procedural Terminology (CPT) codes to describe the procedures, tests, or treatments provided to the patient.

    • Document All Evaluation and Management (E/M) Services: Use the appropriate E/M codes (e.g.99202-99215) to bill for office visits, consultations, and follow-up appointments.
    • Laboratory Tests: Use accurate CPT codes to bill for laboratory tests such as complete blood count (e.g. 85027), hemoblobin electrophoresis (e.g. 83020), and other relevant tests.
    • Transfusions: Assign CPT codes for blood transfusions, if applicable. These may include codes such as 36430 for transfusion of packed red blood cells.
    • Pain Management Services: Use appropriate codes for pain management services, such as nerve blocks or medication management (e.g. 64450, 99605-99607).
    • Hospitalization: If the patient requires hospitalization for a sickle cell crisis or other complications, use the relevant inpatient or outpatient hospital visit codes (e.g. 99221-99233).
    • Modifiers: When necessary, use modifiers to provide additional information about the service provided. For example, you may use modifiers to indicate if a service was provided in the emergency department or during a surgical procedure.

Other Important Medical Billing and Coding Considerations

Verify Patient Insurance

Ensure that the patient’s insurance coverage is active and that you understand the specific requirements such as pre-authorization and other guidelines from the patient’s insurance provider.

Gather Patient Information

Collect all relevant patient information, including their medical history, current symptoms, and any previous treatments or procedures related to SCD.

Billing Forms and Claims

Complete the appropriate billing forms, such as the CMS-1500 for professional services or the UB-04 for hospital services. Ensure that all required information, including patient demographics, insurance information, and codes, are accurately entered.

Follow up on Claims

Monitor the status of your claims and follow up on any denials or rejections promptly. Be prepared to provide additional documentation if requested by the insurance company.

Education and Training

Ensure that your billing and coding staff are properly trained and up-to-date with the latest coding guidelines and regulations related to Sickle Cell Disease.


Adhere to all healthcare compliance and privacy regulations, such as the Health Insurance Portability and Accountability Act (HIPAA), when handling patient information and billing.

Consult with Coding Specialists

If you have complex cases or are unsure about proper coding and billing, consider consulting with certified medical coding companies or billing companies that have expertise in Sickle Cell Disease coding.

Medical Necessity

Ensure that the services provided are medically necessary and that you can support this with proper documentation. Insurance companies may request additional information to justify the medical necessity of certain procedures.

Submit Claims

Submit claims for the services provided using the appropriate diagnosis and procedure codes, along with any necessary supporting documentation, to the patient’s insurance company. Follow the insurance company’s guidelines for claims submission.

It’s important to stay updated with changes in coding guidelines and regulations as they may evolve over time. Accurate coding and billing not only ensure proper reimbursement but also contribute to improved patient care and data accuracy in healthcare records.

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Loralee Kapp

Since joining our RCM Division in October 2021, Loralee, who is HIT Certified (Health Information Technology/Health Information Management), brings her extensive expertise in medical coding and Health Information Management practices to OSI.

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