Today is World Sickle Cell Day. Sickle cell disease is a genetically inherited condition that affects millions worldwide and is chronic, painful and often leads to mortality. In SCD, the normally flexible red blood cells (RBCs) become stiff and sticky and acquire a sickle or crescent shape. These sickle-shaped RBCs cluster together and stick to walls of blood vessels thereby cutting off blood supply and oxygen to different body parts or organs. This can cause organ damage or even stroke. The disease is caused by the mutation of a gene that controls RBC production. Sickle cells carry a type of haemoglobin that contributes to the characteristic sickling. Dr Vishal Sehgal, Country Head, Portea Intensive & Specialty Homecare shares important pointers about Sickle Cell Disease.
The symptoms of this condition are as follows:
Anaemia: While normal RBCs have a life span of 120 days, sickle cells only live for about 10-20 days, leaving the patient severely anaemic. Typical signs of anaemia include lack of energy and constant fatigue.
Pain: Patients have episodic pain in different parts of the body where blood circulation has been affected. Intense pain, called crises, can last for anywhere from a few days to weeks. Excruciating pain, ulcers, abnormal swelling of face, etc., and bone damage may require immediate hospitalization.
Slow growth: Children affected by SCD often fail to grow to their full potential with teenagers lagging behind in height and onset of puberty.
Problems with vision: Blood supply to the optic nerves and muscles may be affected in many cases, leading to blurred vision and other problems.
Low immunity: Patients suffer from frequent infections. Due to low immunity, even common infections can prove to be life-threatening in people with SCD.
Recurrent venous thromboembolism (VTE): SCD puts patients at risk of developing venous thromboembolism or a clotting disorder in which clots are formed in deep veins, which can travel to critical organs, such as lungs, and lead to organ damage. Higher rate of internal bleeding is also seen in patients with this condition, leading to life-threatening complications.