Dr Indu Khosla, Senior Consultant, Paediatric Pulmonology at Narayana Hospital - SRCC, Mumbai, explains the critical aspects of ...

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Cystic Fibrosis in Children | Symptoms and Treatments of Cystic Fibrosis | Dr Indu Khosla

hello I'm Dr indu kosla I'm the pediatric pulmonologist here at the NH srcc hospital for children in Mumbai which is managed by Nar Health today I'm going to cover a very important topic about a disease called cystic fibrosis yes it's a uncommon disease more commonly seen in the western population and it was believed that it is not seen so commonly in our country un un fortunately this disease though rare I would not say rare uncommon but it is very often missed and therefore uh this video is being created to give awareness of cystic fibrosis and a little bit of knowledge and information about this disease to parents so what is cystic fibrosis it is a genetic disease where it comes because of a mutation from both the parents so if one parent is having a defective Gene and the other parent has a normal Gene the child cannot get this disease so it has to come as a mutation from both the parents this disease affects the lungs the pancreas the liver and the intestine what it does it makes the mucus very very thick so when the mucus is very thick the lungs are blocked clogged the pancreas is blocked and therefore does not release in release enzymes in the intestine and because it does not release enzymes in the intestine to digest food the child has frequent stools which are oily and sticky and also a child can have liver disease so children who have cystic fibrosis present very early in life but some can present even later but they typically come with cough which is persistent cough which is wet with lot of mucus and expectoration and they come with frequent stools which could be oily sticky uh they also will have stomach ache because of these frequent oily sticky stools these children can also have failure to thrive when I say failure to thrive they do not gain weight the way they are supposed to gain so these are the common manifestations in childhood as this child grows older one can get infertility in adults so how does this disease occur this is because of a defective protein called as the cfdr protein the cystic fibrosis transmembrane regulator protein which is a defective protein which controls normally controls the movement of sodium chloride and water through the cells of the Airways the intestines Etc when the protein is defective the movement of water is defective and therefore the mucus becomes thick it clogs the lungs clogs the pancreas resulting in all these symptoms how do we diagnose it there are tests to diagnose it in the newborn period we have a we have a test called as uh imuno reactive trypsinogen which is a screening test another test that can be done which is called the the sweat chloride test and then we have genetic tests how do we treat these children these children need lifelong treatment in the form of nutritional Rehabilitation because they lose a lot of fat protein Etc through their stools so they need nutritional Rehabilitation they need uh vitamins they need enzymes which are normally secreted by the pancreas since it's absent we have to give it externally so that they can digest the food then they need chest physiotherapy to clear the mucus from their lungs so that the lungs are free from infection so nutritional Rehabilitation vitamins pancreatic enzyme replacement and chest physiotherapy forms the Crux of treatment Beyond this when they have infection children will require antibiotics lung transplantation is also a treatment modality which can be offered to these children thank you

Dr.

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