Sickle cell disease (SCD) is a complex genetic disorder. Unfortunately, there are a number of misconceptions surrounding this disease. Dispelling this misinformation is essential to making informed decisions about treatment.
Sickle cell disease is a group of inherited red blood cell disorders characterized by the presence of abnormal hemoglobin, called hemoglobin S. When a person has sickle cell disease, their blood cells become stiff, sticky, and sickle-shaped. These sickled cells can block blood flow, leading to pain and possible complications. Symptoms of sickle cell disease can vary from person to person. Some of them include anemia, swelling, vision problems, frequent infections, and poor growth.
Sickle cell disease (SCD) is a complex genetic disorder. Unfortunately, there are several misconceptions surrounding SCD. Dispelling this misinformation is essential to make informed decisions about treatment. Dr Vinay Munikoty Venkatesh, Consultant, Paediatric Haematology, Oncology and Bone Marrow Transplant, Manipal Yeshwanthpur Hospital spoke to India.com and shared prevailing myths and facts about SCD.
Common myths about sickle cell disease
Myth 1Sickle cell disease is a unique disease with distinct signs and symptoms.
Made: Sickle cell disease is not a single entity. It is a group of disorders that includes sickle cell anemia (HbSS), sickle hemoglobin C disease (HbSC), and sickle beta thalassemia (HbSβ-thal); sickle cell anemia is the most severe form. Each type has different genetic variations and symptoms.
Myth 2: There is no difference between sickle cell trait and sickle cell disease.
Made: No. Sickle cell trait and sickle cell disease are not the same thing. In sickle cell trait (SCT), a person inherits only one sickle cell gene, while the other gene is normal. SCT usually causes no symptoms of the disease. In sickle cell disease (SCD), on the other hand, a person inherits two sickle cell genes, one from each parent, leading to the formation of sickle-shaped red blood cells that can cause a variety of health complications, some of which can be critical.
Myth 3: Blood transfusions are the only treatment for sickle cell anemia.
Made: Blood transfusion is one of the treatments for severe form of sickle cell disease. However, the availability of better treatments has significantly reduced the need for blood transfusions. Hydroxyurea is now an integral part of sickle cell disease treatment. For severe cases of sickle cell disease, recent innovations such as bone marrow transplantation and gene therapy have revolutionized treatment. For pain management, we have several painkillers that ensure almost pain-free episodes in sickle cell disease patients. There are several ongoing researches, including gene therapy and newer medications, that bring new hope and promise in managing the disease.
Myth 4: Sickle cell disease only causes pain
Made: Pain is a major symptom of sickle cell disease, but the disease affects many organs and systems in the body. It can cause complications such as anemia, stroke, acute chest syndrome, infections, organ damage, and growth retardation in children. The impact of sickle cell disease goes beyond pain and requires a comprehensive approach to manage its various manifestations.
Myth 5: Sickle cell disease significantly shortens life expectancy
Made: While sickle cell disease can affect life expectancy, advances in medical care have significantly improved outcomes. With proper treatment, many people with sickle cell disease can live to age 50 or beyond. Comprehensive care, regular checkups, and new treatments have improved the quality of life and longevity of those affected.