Every year, June 19 is observed as “World Sickle Cell Disease Day” across the globe. Sponsored by the United Nations, this one-day event aims to raise public knowledge and understanding about sickle cell disease (SCD) and its treatment methods. It aims to make people understand the challenges patients affected by this disease and their families and caregivers experience. Sickle cell disease is an inherited form of anemia – a condition wherein there is not enough healthy red blood cells to carry adequate oxygen throughout your body. Patients with SCD will have abnormal hemoglobin (the oxygen-carrying protein found in red blood cells) levels. With this disease, the red blood cells become sticky and are shaped like sickles. These sickle-shaped blood cells can get stuck in small blood vessels, which can slow or block blood flow and oxygen to parts of the body. The condition is caused by a mutation in the gene that tells your body to make the red, iron-rich compound that gives blood its red color (hemoglobin). 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. Signs and symptoms of SCD vary from person to person and change over time. There is no specific cure for SCD. However, complementary treatment modalities can help relieve pain and other symptoms associated with the disease. For accurate clinical documentation of this inherited anemic disorder, physicians can consider utilizing medical billing and coding services.

As per reports from the World Health Organization (WHO, 2018 statistics), about 300,000 babies are born yearly with severe hemoglobin disorders. It is estimated that about 90,000 to 100,000 people (African Americans) are affected by this disease in the United States. However, the condition is more common in sub-Saharan Africa, the Caribbean, Central and South America, Middle East and Mediterranean countries. For a baby to be born with sickle cell anemia, both parents must carry a sickle cell gene. The condition can lead to a host of complications such as – organ damage, pulmonary hypertension, gall stones, acute chest syndrome, stroke, leg ulcers and blindness.

The 2019 campaign aims to spread awareness about the common symptoms associated with the condition and highlight the different ways to provide the right support to the affected population. Common signs and symptoms of SCD include – anemia, periodic episodes of pain (called crises), frequent infections, delayed growth, painful swelling of the hands and feet, and vision problems.

Diagnosis of SCD will begin with a blood test to check for hemoglobin S (the defective form of hemoglobin that underlies sickle cell anemia). Generally, this blood test is part of the routine newborn screening done at the hospital (at the time of childbirth) in the United States. However, older children and adults can also undergo the test. People who are thinking about having children can have the test to find out how likely it is for their children to have SCD. Physicians in such cases can diagnose SCD before a baby is born. The 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) to look for the sickle cell gene.

Treatment for sickle cell disease is usually aimed at relieving symptoms and preventing complications. Bone marrow transplant (also known as stem cell transplant) offers the only potential cure for SCD. This treatment is usually reserved for people younger than 16 because the risks increase for people older than 16 years. However, finding a donor is quite difficult, and the procedure can cause serious complications, including death.  Medications such as antibiotics, pain relieving medications and Hydroxyurea (Droxia, Hydrea) are also used as part of the treatment. In addition, blood transfusion (to prevent complications such as a stroke) and vaccinations, (such as the pneumococcal vaccine and the annual flu shot) are also important to prevent the condition. The diagnostic procedures and other treatment modalities performed by physicians must be carefully documented using the correct medical codes. Medical billing and coding services offered by reputable medical billing and coding companies can help physicians use the correct medical codes for their billing purposes.

ICD-10 Codes for Diagnosing Sickle Cell Disease (SCD)

D57 – Sickle-cell disorders
D57.0 – Hb-SS disease with crisis

  • D57.00 – Hb-SS disease with crisis, unspecified
  • D57.01 – Hb-SS disease with acute chest syndrome
  • D57.02 – Hb-SS disease with splenic sequestration

D57.1 – Sickle-cell disease without crisis
D57.2 – Sickle-cell/Hb-C disease

  • D57.20 – Sickle-cell/Hb-C disease, without crisis
  • D57.21 – Sickle-cell/Hb-C disease with crisis
    • D57.211 -Sickle-cell/Hb-C disease with acute chest syndrome
    • D57.212 – Sickle-cell/Hb-C disease with splenic sequestration
    • D57.219 – Sickle-cell/Hb-C disease with crisis, Sickle-cell thalassemia unspecified

D57.3 – Sickle-cell trait
D57.4 – Sickle-cell thalassemia

  • D57.40 – Sickle-cell thalassemia, without crisis
  • D57.41 – Sickle-cell thalassemia with crisis
    • D57.411 – Sickle-cell thalassemia with acute chest syndrome
    • D57.412 – Sickle-cell thalassemia with splenic sequestration
    • D57.419 – Sickle-cell thalassemia with crisis, unspecified

D57.8 – Other sickle-cell disorders

  • D57.80 – Other sickle-cell disorders, without crisis
  • D57.81 – Other sickle-cell disorders with crisis
    • D57.811 – Other sickle-cell disorders with acute chest syndrome
    • D57.812 – Other sickle-cell disorders with splenic sequestration
    • D57.819 – Other sickle-cell disorders with crisis, unspecified

The General Assembly of the United Nations on December 22, 2008 created a resolution that recognized sickle cell disease (SCD) as a global public health concern and one of the world’s foremost genetic diseases. The resolution urged the member countries and the United Nations Organization to spread awareness about sickle-cell disease at both the national and international levels every year. Thus, June 19 of each year was officially designated as the “World Sickle-Cell Day”. The first World Sickle Cell Day was celebrated on June 19, 2009.

The 2019 one-day event marks the 10th anniversary of World Sickle Cell Day. As part of the campaign, a wide range of activities such as hosting and holding local gatherings with friends, family, neighbors and others in your community, and regular health checkups to assess risks or trait status for the condition are organized.

Join World Sickle-cell Day (SCD) Campaign on June 19! Spread awareness about this inherited red-blood cell disorder and its associated symptoms.