Sickle cell disease (SCD) or sickle cell anemia is the most common inherited blood disorder in the United States, according to the Centers for Disease Control and Prevention and the National Institutes of Health. SCD causes the red blood cells become sickle shaped and they lose their ability to move smoothly through the blood vessels. The condition causes a deficiency in hemoglobin, the protein that allows the red blood cells to carry oxygen to the body’s tissues. Proper clinical documentation is crucial to help medical coding service providers assign the correct codes for appropriate reimbursement.
Signs and Symptoms
People with SCD inherit the disease from their parents. HbSS, HbSC, HbS beta thalassemia, and HbAS are the most common types of SCD. Screening for HbS at birth is currently mandatory in the US. Although sickle cell anemia is present at birth, symptoms do not appear for at least four to five months due to the presence of elevated levels of Hb F. When they appear, signs and symptoms include:
- Acute and chronic pain
- Bone pain, typically in long bones of extremities
- Aplastic crisis: failure of the bone marrow to produce any red blood cells
- Splenic sequestration: enlargement of the spleen when red blood cells become trapped in it
- Growth retardation, delayed sexual maturation, being underweight
- Hand-foot syndrome
- Acute chest syndrome
- Pulmonary hypertension
- Avascular necrosis of the femoral or humeral head caused by interruption of blood supply to the bone
- Ptosis and other eye conditions
- Dilation of both ventricles and the left atrium
- Liver and kidney problems
- Chronic leg ulcers
Patients also experience shortness of breath, dizziness, headache, coldness in the hands and feet, pale skin, and chest pain. A painful and debilitating condition, sickle cell crisis can last for hours, days, or even weeks to months. A sickling episode can become irreversible due to infection, stress-emotional or exertional physical, surgery, dehydration, acidosis, cold, or anything that leads to hypoxia (WellCare).
In addition to mandatory screening for HbS at birth, a variety of lab tests may be used to diagnose SCD. These include blood tests to check for hemoglobin S, low red blood cell count (anemia), peripheral blood smear, pulmonary and renal function tests, blood cultures, etc. Imaging tests include radiography, MRI, CT scan, Echocardiography, bone scanning, abdominal ultrasonography, and Transcranial Doppler ultrasonography.
The goal of treatment for sickle cell anemia is to avoid crises, relieve symptoms and prevent complications. Treatments options include antibiotics, medications to reduce pain and prevent complications, oxygenation, and blood transfusions, as well as a bone marrow transplant. Children age 2 and younger should make frequent visits to the physician. The Centers for Disease Control and Prevention recommends that those older than 2 and adults with sickle cell anemia should visit a physician at least once a year. Many SCD patients are frequently hospitalized or need emergency treatment for pain. Gene therapy is emerging as a potential cure for severe SCD (emedicine.medscape.com).
ICD-10 Codes for Sickle Cell Disorders
|D57.00||Hb-SS disease w/ crisis, unspecified|
|D57.01||Hb-SS disease w/ acute chest syndrome|
|D57.02||Hb-SS disease w/ splenic sequestration|
|D57.1||Sickle cell disease w/o crisis|
|D57.20||Sickle cell/Hb-C disease w/o crisis|
|D57.21||Sickle cell/Hb-C disease w/ crisis|
|D57.211||Sickle cell/Hb-C disease w/ acute chest syndrome|
|D57.212||Sickle cell/Hb-C disease w/ splenic sequestration|
|D57.219||Sickle cell/Hb-C disease w/ crisis, unspecified|
|D57.3||Sickle cell trait|
|D57.40||Sickle cell thalassemia w/o crisis|
|D57.41||Sickle-cell thalassemia w/ crisis|
|D57.411||Sickle cell thalassemia w/ acute chest syndrome|
|D57.412||Sickle cell thalassemia w/ splenic sequestration|
|D57.419||Sickle cell thalassemia w/ crisis, unspecified|
|D57.80||Other sickle cell disorders w/o crisis|
|D57.811||Other sickle cell disorders w/ acute chest syndrome|
|D57.812||Other sickle cell disorders w/ splenic sequestration|
|D57.819||Other sickle cell disorders w/ crisis, unspecified|
When it comes to reimbursement, appropriate charting is the key to success. For SCD admissions, provider documentation should capture the severity of the illness by reporting secondary problems. The location of the pain (in the back, knees, legs, arms, chest or stomach) and type of pain (throbbing, sharp, dull or stabbing) should be documented. Episodes and severity of pain gets varies from person to person and from crisis to crisis. The documentation should specify the SCD type, trigger of the crisis, any end-organ dysfunction, complications of the treatment, and underlying chronic comorbid conditions.
- Type of disease: There are different types of sickle cell genes leading to different types of sickle cell disease. Common types of SCD include: Hemoglobin (Hb) SS or sickle cell anemia, Hemoglobin (Hb) SC disease, Hemoglobin (Hb) S beta thalassemia.
- Triggers: Triggers of sickle cell pain include:
- High altitudes (above 2,000 meters, or about 6,500 feet), where there is less oxygen in the air
- Low-oxygen activities, such as scuba diving
- Sudden changes in temperature
- Being in cold weather (without protective clothing and gear) for longer than a few minutes
- Dehydration or not drinking enough water
- Intense exercise and dehydration
- Stress: Stress can cause the blood vessels to narrow, limiting blood flow and causing pain
- End-organ dysfunction: Progressive organ damage and dysfunction is the most important trait of the disease. End-organ dysfunction could be end stage renal failure (glomerulosclerosis), sickle chronic lung disease, intracranial hemorrhage, retinopathy, disabling leg ulcers, and generalized osteonecrosis. Organ damage is progressive and irreversible.
- Underlying chronic comorbid conditions: SCD involves multiple co-morbidities that can affect virtually any organ system in the body and increase with age. Acute comorbidities include vaso-occlusive crisis, acute chest syndrome (ACS), stroke, acute renal failure, priapism, splenic sequestration, cholelithiasis, cholecystitis, and ophthalmologic issues such as sickle cell retinopathy. Chronic comorbidities include avascular necrosis (AVN), leg ulcers, pulmonary hypertension (PH), diastolic heart dysfunction, gout, end-stage renal disease (ESRD) and ophthalmologic complications.
With comprehensive documentation, an experienced medical billing and coding company can help providers report SCD for appropriate care and optimal reimbursement.