 Hello everyone. I'm Dr. Aineen Kinemi, pediatric pulmonologist at Manipal Hospital, Spangler. Today we want to talk about a disease called cystic fibrosis. Cystic fibrosis is an inherited progressive disorder. In India the prevalence is variable. Anything that is between 1 is to 20,000 to 1 is to 40,000 kids can be affected by this disease. This disease can be diagnosed by various methods. There is a newborn screening test called the IRT. There is a test called sweat chloride which measures the chloride in the sweat which is the gold standard. And of course the genetics test would be able to pick up all the different types of mutations associated with the disease. What is particularly important is this genetic defect affects the ionic transport in cells particularly related to the lung and to the GIT. So what happens in the lungs is a child with this type of defect unable to clear the secretions. A lot of mucus production which gets impacted in the airway, the child is unable to cough out or have frequent infections and this sputum gets impacted in the lung which is very thick, viscid and the child gets recurrent infections, inflammation. It is an endless cycle of respiratory infections and hospital admissions. So what affects this child particularly is the morbidity and also the long term problems associated with the lung damage. The other important factor is the gut. Because there is an enzyme deficiency related to this disease children are unable to digest the proteins, the carbohydrates and the fats. So eventually these children land up with malnutrition, unable to gain height, weight and many other nutritional deficiencies including that of vitamins and minerals. Hence these kits require not only the multivitamin and nutritional supplements apart from a high calorie, high protein, high carbohydrate diet they also require the most important enzymes supplement which is a pancreatic lipase. There are many other systems involved including the GI tract, it can also involve the sinuses and it can also involve the various different organs that are particularly we need to screen for endocrinology and because of recurrent infections antibiotic usage is also very high in these kits. What is important to understand is this disease it is very difficult to diagnose at times and challenging for most pediatricians. However with various diagnostic modalities newborn screening, sweat test as well as the genetics we can pick up these kits early, manage them well and provide them at least a minimal quality of life. It includes multidisciplinary teams including pediatricians, pediatric pulmonologists, endocrinologists, gastroenterologists, physiotherapists as well as nutritionists and dietitians. In India we still have a problem with accessing the drugs that are available. However this limited access and inability to get our children to these drugs should not be a limiting factor and at least provide them an equal or a better quality of life. Thank you.