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  6. 2025 ICD-10-CM Code D57

2025 ICD-10-CM Diagnosis Code D57

Sickle-cell disorders

ICD-10-CM Code:
D57
ICD-10 Code for:
Sickle-cell disorders
Is Billable?
Not Valid for Submission
Code Navigator:

D57 is a non-specific and non-billable diagnosis code code, consider using a code with a higher level of specificity for a diagnosis of sickle-cell disorders. The code is not specific and is NOT valid for the year 2025 for the submission of HIPAA-covered transactions. Category or Header define the heading of a category of codes that may be further subdivided by the use of 4th, 5th, 6th or 7th characters.

Specific Coding Applicable to Sickle-cell disorders

Non-specific codes like D57 require more digits to indicate the appropriate level of specificity. Consider using any of the following ICD-10-CM codes with a higher level of specificity when coding for sickle-cell disorders:

  • D57.0 for Hb-SS disease with crisis - NON-BILLABLE CODE

  • Use D57.00 for Hb-SS disease with crisis, unspecified - BILLABLE CODE

  • Use D57.01 for Hb-SS disease with acute chest syndrome - BILLABLE CODE

  • Use D57.02 for Hb-SS disease with splenic sequestration - BILLABLE CODE

  • Use D57.03 for Hb-SS disease with cerebral vascular involvement - BILLABLE CODE

  • Use D57.04 for Hb-SS disease with dactylitis - BILLABLE CODE

  • Use D57.09 for Hb-SS disease with crisis with other specified complication - BILLABLE CODE

  • Use D57.1 for Sickle-cell disease without crisis - BILLABLE CODE

  • D57.2 for Sickle-cell/Hb-C disease - NON-BILLABLE CODE

  • Use D57.20 for Sickle-cell/Hb-C disease without crisis - BILLABLE CODE

  • D57.21 for Sickle-cell/Hb-C disease with crisis - NON-BILLABLE CODE

  • Use D57.3 for Sickle-cell trait - BILLABLE CODE

  • D57.4 for Sickle-cell thalassemia - NON-BILLABLE CODE

  • Use D57.40 for Sickle-cell thalassemia without crisis - BILLABLE CODE

  • D57.41 for Sickle-cell thalassemia, unspecified, with crisis - NON-BILLABLE CODE

  • Use D57.42 for Sickle-cell thalassemia beta zero without crisis - BILLABLE CODE

  • D57.43 for Sickle-cell thalassemia beta zero with crisis - NON-BILLABLE CODE

  • Use D57.44 for Sickle-cell thalassemia beta plus without crisis - BILLABLE CODE

  • D57.45 for Sickle-cell thalassemia beta plus with crisis - NON-BILLABLE CODE

  • D57.8 for Other sickle-cell disorders - NON-BILLABLE CODE

  • Use D57.80 for Other sickle-cell disorders without crisis - BILLABLE CODE

  • D57.81 for Other sickle-cell disorders with crisis - NON-BILLABLE CODE

Code Classification

  • Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism
    D50–D89
    • Hemolytic anemias
      D55-D59
      • Sickle-cell disorders
        D57

Tabular List of Diseases and Injuries

The following annotation back-references are applicable to this diagnosis code. The Tabular List of Diseases and Injuries is a list of ICD-10-CM codes, organized "head to toe" into chapters and sections with coding notes and guidance for inclusions, exclusions, descriptions and more.


Use Additional Code

Use Additional Code
The “use additional code” indicates that a secondary code could be used to further specify the patient’s condition. This note is not mandatory and is only used if enough information is available to assign an additional code.
  • code for any associated fever R50.81

Type 1 Excludes

Type 1 Excludes
A type 1 excludes note is a pure excludes note. It means "NOT CODED HERE!" An Excludes1 note indicates that the code excluded should never be used at the same time as the code above the Excludes1 note. An Excludes1 is used when two conditions cannot occur together, such as a congenital form versus an acquired form of the same condition.
  • other hemoglobinopathies D58

Index to Diseases and Injuries References

The following annotation back-references for this diagnosis code are found in the injuries and diseases index. The Index to Diseases and Injuries is an alphabetical listing of medical terms, with each term mapped to one or more ICD-10-CM code(s).

Patient Education


Sickle Cell Disease

What is sickle cell disease (SCD)?

Sickle cell disease (SCD) is a group of inherited red blood cell disorders. If you have SCD, there is a problem with your hemoglobin. Hemoglobin is a protein in red blood cells that carries oxygen throughout the body. With SCD, the hemoglobin forms into stiff rods within the red blood cells. This changes the shape of the red blood cells. The cells are supposed to be disc-shaped, but instead they are crescent, or sickle, shaped.

The sickle-shaped cells are not flexible and cannot change shape easily. Many of them burst apart as they move through your blood vessels. The sickle cells usually only last 10 to 20 days, instead of the normal 90 to 120 days. Your body may have trouble making enough new cells to replace the ones that you lost. Because of this, you may not have enough red blood cells. This is a condition called anemia, and it can make you feel tired.

The sickle-shaped cells can also stick to vessel walls, causing a blockage that slows or stops the flow of blood. When this happens, oxygen can't reach nearby tissues. The lack of oxygen can cause attacks of sudden, severe pain, called pain crises. These attacks can occur without warning. If you get one, you might need to go to the hospital for treatment.

What causes sickle cell disease (SCD)?

SCD is caused by a variant (change) in a gene that has instructions for your body to make one part of the hemoglobin. This changed gene is sometimes called a sickle cell gene. People with SCD are born with two sickle cell genes, one from each parent.

If you are born with one sickle cell gene, it's called sickle cell trait. People with sickle cell trait are generally healthy, but they can pass the defective gene on to their children.

Who is more likely to have sickle cell disease (SCD)?

In the United States, most of the people with SCD are African Americans:

  • About 1 in 13 Black or African American babies are born with sickle cell trait
  • About 1 in every 365 Black or African American babies are born with sickle cell disease

SCD also affects some people who come from Hispanic, southern European, Middle Eastern, or Asian Indian backgrounds.

What are the symptoms of sickle cell disease (SCD)?

People with SCD start to have signs of the disease during the first year of life, usually around 5 or 6 months of age. Early symptoms of SCD may include:

  • Painful swelling of the hands and feet
  • Fatigue or fussiness from anemia
  • A yellowish color of the skin (jaundice) or the whites of the eyes (icterus)

The effects of SCD vary from person to person and can change over time. Most of the signs and symptoms of SCD are related to complications of the disease. They may include severe pain, anemia, organ damage, and infections.

How is sickle cell disease (SCD) diagnosed?

A blood test can show if you have SCD or sickle cell trait. Genetic tests can tell if you have one or two copies of the sickle cell gene. Genetic tests can help confirm an SCD diagnosis if the results from blood tests are not clear.

All states now test newborns for SCD (as well as many other treatable conditions) as part of their screening programs. These programs help find the conditions early, so treatment can be started right away.

Health care providers can also diagnose SCD before a baby is born. That test uses a sample of amniotic fluid (the liquid in the sac surrounding the baby) or tissue taken from the placenta (the organ that brings oxygen and nutrients to the baby).

People who are thinking about having children can have the test to find out how likely it is that their children will have SCD.

What are the treatments for sickle cell disease (SCD)?

There are many ways to manage sickle cell disease. Your medical team will probably include a hematologist, a doctor who specializes in blood diseases. You will work with your medical team to set up a treatment plan. Possible treatment options may include:

  • Treatments that can help relieve symptoms and lessen complications, including:
    • Hydroxyurea, a medicine to reduce sickling of red blood cells. This can help prevent serious symptoms of sickle cell disease. This medicine can be used in adults and in children as young as 9 months old. But this medicine is not safe during pregnancy.
    • Voxelotor, another medicine to prevent the sickling of red blood cells. It can be used in adults and children ages 4 years and older.
    • Pain relievers for acute or chronic pain.
    • Antibiotics to try to prevent infections in younger children.
    • Blood transfusions for severe anemia. If you have had some serious complications, such as a stroke, you may have transfusions to prevent more complications.
    • Other treatments for specific complications, such as medicines to lower blood pressure and vitamins to treat a vitamin deficiency.
  • Bone marrow or stem cell transplantation, which can cure SCD. Because these transplants are risky and can have serious side effects, they are usually only used in children with severe SCD. For the transplant to work, the bone marrow must be a close match. Usually, the best donor is a brother or sister.
  • Gene therapies to treat SCD in people who are 12 years and older and have had repeated sickle cell crises. These new therapies involve taking some of your blood stem cells and either adding new DNA to them or changing their existing DNA. Then these cells are given back to you, and they can make a type of hemoglobin that is healthy. This can reduce the complications of SCD, including the SCD crises.

Complementary and alternative medicine (CAM) seems to help some people deal with pain caused by SCD. These types of CAM may lower your pain, especially if it is not well managed with medicines::

  • Cognitive behavioral therapy (a type of counseling)
  • Acupuncture
  • Exercise or movement programs, such as yoga
  • Massage
  • Meditation and mindfulness practices
  • Virtual reality, a computer-generated 3D environment you can see with special goggles

It's also important to take steps to keep yourself as healthy as possible:

  • Get regular medical care
  • Get your routine vaccinations
  • Live a healthy lifestyle
  • Avoid situations that may set off a pain crisis

NIH: National Heart, Lung, and Blood Institute


[Learn More in MedlinePlus]

Sickle cell disease

Sickle cell disease is a group of disorders that affects hemoglobin, the molecule in red blood cells that delivers oxygen to cells throughout the body. People with this disease have atypical hemoglobin molecules called hemoglobin S, which can distort red blood cells into a sickle, or crescent, shape.

Signs and symptoms of sickle cell disease usually begin in early childhood. Characteristic features of this disorder include a low number of red blood cells (anemia), repeated infections, and periodic episodes of pain. The severity of symptoms varies from person to person. Some people have mild symptoms, while others are frequently hospitalized for more serious complications.

The signs and symptoms of sickle cell disease are caused by the sickling of red blood cells. When red blood cells sickle, they break down prematurely, which can lead to anemia. Anemia can cause shortness of breath, fatigue, and delayed growth and development in children. The rapid breakdown of red blood cells may also cause yellowing of the eyes and skin, which are signs of jaundice. Painful episodes can occur when sickled red blood cells, which are stiff and inflexible, get stuck in small blood vessels. These episodes deprive tissues and organs, such as the lungs, kidneys, spleen, and brain, of oxygen-rich blood and can lead to organ damage. A particularly serious complication of sickle cell disease is high blood pressure in the blood vessels that supply the lungs (pulmonary hypertension), which can lead to heart failure. Pulmonary hypertension occurs in about 10 percent of adults with sickle cell disease.


[Learn More in MedlinePlus]

Code History

  • FY 2024 - No Change, effective from 10/1/2023 through 9/30/2024
  • FY 2023 - No Change, effective from 10/1/2022 through 9/30/2023
  • FY 2022 - No Change, effective from 10/1/2021 through 9/30/2022
  • FY 2021 - No Change, effective from 10/1/2020 through 9/30/2021
  • FY 2020 - No Change, effective from 10/1/2019 through 9/30/2020
  • FY 2019 - No Change, effective from 10/1/2018 through 9/30/2019
  • FY 2018 - No Change, effective from 10/1/2017 through 9/30/2018
  • FY 2017 - No Change, effective from 10/1/2016 through 9/30/2017
  • FY 2016 - New Code, effective from 10/1/2015 through 9/30/2016. This was the first year ICD-10-CM was implemented into the HIPAA code set.