In order to increase public knowledge and raise awareness about sickle cell disease (SCD) and the struggles patients and their families go through, June 19 is observed as “World Sickle Cell Disease (SCD) Day” across the globe. Sponsored by the United Nations, the campaign goal is to heighten the awareness of SCD among the general public, government policymakers, public authorities, industry representatives, scientists and healthcare professionals. Also known as sickle cell anemia, SCD is an inherited blood disorder – wherein there is not enough healthy red blood cells to carry adequate oxygen throughout the body. In normal cases, the flexible, round blood cells move easily through blood vessels. However, people with SCD will have abnormal levels of hemoglobin (the oxygen-carrying protein found in red blood cells) levels. In this type of disease, the red blood cells are shaped like sickles and appear rigid and sticky and get stuck in small blood vessels. This can slow or block blood flow and oxygen to parts of the body. There is no specific cure for sickle cell anemia. However, several treatment modalities can help reduce pain and prevent complications associated with the disease. For accurate clinical documentation of this inherited blood disorder, physicians can consider utilizing the services of professional medical billing and coding companies.
As per reports from the World Health Organization (WHO) (2019 statistics), about 300,000 babies are born with severe hemoglobin disorders every year. The Centers for Disease Control and Prevention (CDC) point out that SCD affects approximately 100,000 Americans. It is estimated that about 1 in 13 Black or African-American babies are born with sickle cell trait (SCT). A defective gene (called a sickle cell gene) is the primary cause associated with the condition.
The 2021 campaign aims to educate people about real-life patient stories in an effort to overcome stigmas and clear up misconceptions about SCD. It aims to spread awareness about the different signs and symptoms associated with the condition and highlight the different ways to provide the right support to the affected population. People with the disease are born with two sickle cell genes, one from each parent. For a baby to be born with sickle cell anemia, both parents must carry a sickle cell gene. Signs and symptoms of SCD vary from one person to another and change over time. 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. If left untreated or becomes severe, 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.
Initial 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. In case of adults, a blood sample is drawn from a vein in the arm. In young children and babies, the blood sample is usually collected from a finger or the heel. If a parent or child has sickle cell anemia, the physician may request additional tests to check for possible complications of the disease. People who are planning to have children can have the test to find out how likely the chances will be 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 SCD.
Treatment for sickle cell anemia aims at avoiding pain episodes, reducing symptoms and preventing complications. Treatment may include – medications and blood transfusions. Medications such as antibiotics, pain relieving medications and Hydroxyurea (Droxia, Hydrea) are prescribed as part of the treatment. Blood transfusion (to prevent complications such as a stroke) and vaccinations, (such as the pneumococcal vaccine and the annual flu shot) are also recommended as part of the treatment. Bone marrow transplant (also known as stem cell transplant) is another potential treatment used for curing SCD in children and teenagers. The treatment is usually reserved for people younger than 16 as the risks increase for patients above 16 years. The diagnostic procedures and other treatment modalities performed by physicians must be carefully documented using the correct medical codes. Medical coding services include assigning the correct medical codes on physicians’ medical claims. ICD-10 codes for diagnosing Sickle Cell Disease (SCD) include –
- D57 – Sickle-cell disorders
- D57.0 – Hb-SS disease with crisis
- D57.1 – Sickle-cell disease without crisis
- D57.2 – Sickle-cell/Hb-C disease
- D57.3 – Sickle-cell trait
- D57.4 – Sickle-cell thalassemia
- D57.8 – Other sickle-cell disorders
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. Therefore, 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.
As part of the campaign, a wide range of activities are organized across the world. Wearing “Red” is a great entryway for promoting conversations and raising awareness about sickle cell. The Sickle Cell Disease Association of America (SCDAA) has planned to organize several events to mark this year’s “Sickle Cell Disease Awareness Day”. “Lift Every Voice to Shine the Light on Sickle Cell” – a spoken word contest – is jointly sponsored in part by Global Blood Therapeutics (GBT) and the SCDAA. For the event – which seeks to elevate community voices – U.S. patients living with SCD and caregivers are invited to sign up to submit original spoken word pieces or real-life patient stories about their experiences with SCD – in an effort to overcome stigmas and clear up misconceptions about SCD. For each submission received, GBT has pledged it will donate $100 to the SCDAA, up to a total of $5,000. The contest winner and finalists will be featured in virtual events held on June 18–19 on the Sickle Cell Speaks Facebook and Instagram pages.
Also, the multi-state collaborative SiNERGē (Sickle Cell Improvement Across the Northeast Region Through Education) will team up with the SCDAA to hold a 24-hour-long event “Shine the Light on Sickle Cell” on June 19. As part of the event, community members across the world will host local gatherings over a 24-hour period to call attention to SCD and the needs of those who live with the disease. Participants are invited to post about their event on the “Shine the Light” Facebook page. In addition to these initiatives, the SCDAA is urging people to mark this annual event on June 19 by promoting diverse blood donations to support those with SCD. Some of these blood donation initiatives can be found on the organization’s website.