Sickle cell ailment (SCD), an inherited genetic disorder, is a collection of hereditary red blood cell maladies that affect hemoglobin, the protein that transports oxygen to the body. During her budget speech, Finance Minister Nirmala Sitharaman declared that the government aims to eliminate sickle cell anemia by 2047. Sickle cell anemia is among the sickle cell disorders where the shape of certain red blood cells, typically round and pliable, becomes sickle-shaped and inflexible. This can obstruct blood flow, leading to various complications. International Sickle Cell Awareness Day is commemorated annually on June 19. The day is observed to raise greater awareness about sickle cell ailment and the difficulties experienced by patients, their families, and their caregivers. (Also read: International Sickle Cell Awareness Day 2023: Date, history, significance, and celebration)
In an interview with HT Digital, Dr. Vijay Ramanan, Senior Consultant Clinical Hematologist, Bone Marrow & Stem Cell Transplant at Ruby Hall Clinic in Pune, discusses the correlation between sickle cell ailment and the elevated risk of ischemic and hemorrhagic strokes.
“Sickle cell ailment (SCD) heightens the risk of both ischemic and hemorrhagic stroke. Cerebral infarction is a typical complication of sickle cell ailment and may present as obvious stroke or cognitive impairment associated with ‘silent’ cerebral infarction or SCI on magnetic resonance imaging. By the age of 21, the majority of sickle cell ailment patients have SCI,” states Dr. Ramanan.
The risk factors for cognitive impairment, overt ischemic stroke, silent cerebral infarction, overt hemorrhagic stroke, and vasculopathy in sickle cell ailment coincide with severe acute and chronic anemia. The majority also reported chronic anemia, acute chest crises, reticulocytosis, and poor oxygen saturation.
“The precise mechanism by which SCD causes hemorrhagic stroke is not fully comprehended. It could be due to weaker blood vessels called Moya-Moya in individuals with SCD, or it may also be connected to enlarged arteries known as aneurysms.
People with more severe forms of SCD, such as sickle cell anemia and sickle beta zero thalassemia, face a higher risk of experiencing an ischemic stroke,” explains Dr. Ramanan.
“Regular blood transfusions reduce, but do not eliminate, the chance of neurological issues in children with sickle cell ailment who have a history of overt stroke, silent cerebral infarction, or abnormal transcranial Doppler results. There is limited information on the use of hydroxyurea or alternative therapeutic approaches. Maintaining hydration, oxygen therapy, and alkaline pH (using sodium bicarbonate) will aid in the long term. Early detection of neurocognitive problems is expected to become increasingly crucial in the treatment of sickle cell ailment,” advises the expert.