Valid for Submission
D57.1 is a billable diagnosis code used to specify a medical diagnosis of sickle-cell disease without crisis. The code D57.1 is valid during the fiscal year 2022 from October 01, 2021 through September 30, 2022 for the submission of HIPAA-covered transactions.
The ICD-10-CM code D57.1 might also be used to specify conditions or terms like arthropathy associated with a hematological disorder, arthropathy secondary to hemoglobinopathy, disorder of myocardium due to sickle cell hemoglobinopathy, double heterozygous sickling disorder, focal segmental glomerulosclerosis , focal segmental glomerulosclerosis due to sickle cell disease, etc.
Tabular List of Diseases and Injuries
The Tabular List of Diseases and Injuries is a list of ICD-10 codes, organized "head to toe" into chapters and sections with coding notes and guidance for inclusions, exclusions, descriptions and more. The following references are applicable to the code D57.1:
Inclusion TermsInclusion Terms
These terms are the conditions for which that code is to be used. The terms may be synonyms of the code title, or, in the case of "other specified" codes, the terms are a list of the various conditions assigned to that code. The inclusion terms are not necessarily exhaustive. Additional terms found only in the Alphabetic Index may also be assigned to a code.
- Hb-SS disease without crisis
- Sickle-cell anemia NOS
- Sickle-cell disease NOS
- Sickle-cell disorder NOS
Index to Diseases and Injuries
The Index to Diseases and Injuries is an alphabetical listing of medical terms, with each term mapped to one or more ICD-10 code(s). The following references for the code D57.1 are found in the index:
- - Anemia (essential) (general) (hemoglobin deficiency) (infantile) (primary) (profound) - D64.9
- - Herrick's - D57.1
- - Disease, diseased - See Also: Syndrome;
The following clinical terms are approximate synonyms or lay terms that might be used to identify the correct diagnosis code:
- Arthropathy associated with a hematological disorder
- Arthropathy secondary to hemoglobinopathy
- Disorder of myocardium due to sickle cell hemoglobinopathy
- Double heterozygous sickling disorder
- Focal segmental glomerulosclerosis
- Focal segmental glomerulosclerosis due to sickle cell disease
- Hemoglobin S sickling disorder without crisis
- Hemoglobin SS disease without crisis
- Hereditary persistence of fetal hemoglobin
- Hereditary persistence of fetal hemoglobin with sickle cell disease syndrome
- Homozygous hemoglobinopathy
- Homozygous hemoglobinopathy
- Nondiabetic proliferative retinopathy
- Papillary necrosis
- Priapism due to sickle cell disease
- Proliferative retinopathy due to sickle cell disease
- Renal papillary necrosis due to sickle cell disease
- Sickle cell anemia in mother complicating childbirth
- Sickle cell anemia with coexistent alpha-thalassemia
- Sickle cell arthropathy
- Sickle cell hepatopathy
- Sickle cell nephropathy
- Sickle cell retinopathy
- Sickle cell-hemoglobin SS disease
- Sickle cell-hemoglobin SS disease
- Sickle cell-thalassemia disease
- Sickling disorder due to hemoglobin S
- Thalassemia with other hemoglobinopathy
Diagnostic Related Groups - MS-DRG Mapping
|MS-DRG||MS-DRG Title||MCD||Relative Weight|
|811||RED BLOOD CELL DISORDERS WITH MCC||16||1.3776|
|812||RED BLOOD CELL DISORDERS WITHOUT MCC||16||0.8797|
The relative weight of a diagnostic related group determines the reimbursement rate based on the severity of a patient's illness and the associated cost of care during hospitalization.
Convert D57.1 to ICD-9 Code
The General Equivalency Mapping (GEM) crosswalk indicates an approximate mapping between the ICD-10 code D57.1 its ICD-9 equivalent. The approximate mapping means there is not an exact match between the ICD-10 code and the ICD-9 code and the mapped code is not a precise representation of the original code.
Information for Patients
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 this changes them into a crescent, or sickle, shape.
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)?
The cause of SCD is a defective gene, called a sickle cell gene. People with the disease 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 at risk for sickle cell disease (SCD)?
In the United States, most of the people with SCD are African Americans:
- About 1 in 13 African American babies is born with sickle cell trait
- About 1 in every 365 black children is 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 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. All states now test newborns as part of their screening programs, so treatment can begin early.
People who are thinking about having children can have the test to find out how likely it is that their children will have SCD.
Doctors 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).
What are the treatments for sickle cell disease (SCD)?
The only cure for SCD is bone marrow or stem cell transplantation. 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.
There are treatments that can help relieve symptoms, lessen complications, and prolong life:
- Antibiotics to try to prevent infections in younger children
- Pain relievers for acute or chronic pain
- Hydroxyurea, a medicine that has been shown to reduce or prevent several SCD complications. It increases the amount of fetal hemoglobin in the blood. This medicine is not right for everyone; talk to your health care provider about whether you should take it. This medicine is not safe during pregnancy.
- Childhood vaccinations to prevent infections
- Blood transfusions for severe anemia. If you have had some serious complications, such as a stroke, you may have transfusions to prevent more complications.
There are other treatments for specific complications.
To stay as healthy as possible, make sure that you get regular medical care, live a healthy lifestyle, and 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]