Marriages among kin, a major cause for sickle cell disease
India is home to about 1,50,000 sickle cell patients and around 88% of cases occur in Asia.
Bengaluru: The incidence of Sickle Cell Anaemia (SCA) is on the rise in communities where consanguineous marriages are prevalent, said Dr Sunil Bhat, Senior Consultant & Head Paediatric Haematology, Oncology and Blood & Marrow Transplantation, Mazumdar-Shaw Cancer Centre, Narayana Health City. This World Sickle Cell Day, marked on June 19, aims at safe blood transfusion and creating awareness. Around 50% of the world sickle cell population resides in India and highest prevalence is noted in Gujarat, MP, Maharashtra, Chhattisgarh, Odisha, TN, Kerala and Karnataka.
“The chance of both mother and father carrying the abnormal genes increases significantly if they have descendants of a common ancestor. India is home to about 1,50,000 sickle cell patients and around 88% of cases occur in Asia,” he said.
SCD is a chronic genetic blood disorder that is caused by abnormal production of haemoglobin molecule, the oxygen-carrying substance in red blood cells (RBCs). This abnormal condition distorts the shape and properties of RBCs which affects their normal functions. The disease affects all major organs of the body mainly because blood vessels are blocked and supply of oxygen is affected to major tissues and organs.
Dr Girish Navasundi, Consultant Cardiologist, Apollo Hospitals, said, “"Cardiac complications are common among SCD patients and are felt to be an important cause of morbidity and mortality associated with the disease. SCD affects the heart as well as surrounding structures in patients due to the abnormal haemoglobin caused by the disease.”
Approximately 3.2 million of the population belongs to this high-risk group. About 120 million sickle cell traits and 500,000 sickle cell traits in newborns have been reported annually, worldwide. Swollen hands, pain, frequent infections, delayed growth, vision problems and pooling of blood in spleen are some of the leading symptoms of sickle cell anaemia. SCD is also a major reason for premature child deaths in the country.According to an Indian Council of Medical Research survey, around 20 per cent of children with SCD die by the age of two and 30 per cent from tribal communities die before reaching adulthood.
Dr Sunil Bhat said sickle cell anaemia is also associated with complications like stroke, acute chest syndrome characterised by sudden onset of pain and breathing difficulty, pulmonary hypertension, organ damage, especially liver and kidney, blindness, gall stones, leg ulcers, bone and joint damage and painful penile erection leading to impotence.
“Awareness is the key to this genetic disorder which mandates extensive counselling, premarital screening, antenatal diagnosis and newborn screening. These efforts will help in effectively managing the disease,” he said.