Alfin G. Vicencio, MD., Attending in Pediatric Pulmonary Steven and Alexandra Cohen Childrens Med Center, explains the treatments for Cystic Fibrosis.
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Dr. Vicencio on Treatment for Cystic Fibrosis Male: If so we had cystic fibrosis you have probably three ways to look at it. Lung-GI combined, lung-no GI, GI-no lung, is that correct? Dr. Vicencio: More or less? Cystic fibrosis is something that really affects the newest portions of the body including the endocrine system and reproductive system as well. Male: I mean the GI can give what’s missing and they can’t eat and digest food. Dr. Vicencio: Yes. Male: Now we get into the lung. Dr. Viocencio: Yes sir. Male: If a kid has cystic fibrosis probably he’s prone to get pneumonia things like that. If we’re going to keep this kid living what would you do or you recommend you do if the kid has major lung presentation cystic fibrosis. Dr. Vicencio: Anybody -- like there be has really come a long way and in good therapy against infections that are typical for cystic fibrosis particularly in the lungs has really made a dramatic difference in order to survive. Male: But isn’t it also preventative into back therapy that you’re impulsive or something. Dr. Vicencio: There are several different motive therapy for cystic fibrosis for a more severe pulmonary exacerbation oftentimes children need IV antibiotics. There are prophylactic types of medications which are inhaled medications that children are asked to use on monthly basis. One month taking medications and one month off in the medication that can help to suppress the growth of bacteria -- Male: What happens to the lung in -- in general sense is. What happens to the lungs? Dr. Vicencio: The mucus secretions in the lungs are very, very thick and very, very difficult express and they tend to harbor a lot of bacteria as well. So in addition to antibiotic therapy, there are therapies that can help the mucus to be better expressed to keep it out of the airways but there are some medications, one of them is Dornase sulfa which helps secretions and helps to -- Male: Catching those -- Dr. Vicencio: Yeah and nebulizer. Male: And is it readily available and is it also very expensive? Dr. Vicencio: It’s very expensive but it’s something that most people with cystic fibrosis are on chronically and it’s a medication that’s used very, very frequently in cystic fibrosis. There are several other therapies these days, a lot of children are on hypertonic saline for example, by nebulizer as well as to help them to draw more water into the airway and helps the mucus to be expressed a little bit easier. Again, antibiotic therapy both prophylactic and treatment doses are very important in the long-term maintenance of cystic fibrosis. Male: With a kid with lung presentation life expectancy is decreased but we’ve got them to look a lot longer that it were since 30 years ago, is that correct? Dr. Vicencio: Yes absolutely. These days the average ages of survival goes to 40. Male: And we think we could get that up in another 10 to 15 years if kind of worse. Dr. Vicencio: Yes, that is what -- Male: Is there anyway we could fixed the genetics, is there any research in that area right now? Dr. Vicencio: There are. For the past 25 years, a lot people had been working on genetic cures associated with cystic fibrosis. A lot of groups had been working on them and there are a couple of very promising treatments to help increase the expression of protein where it’s supposed to be expressed or to help augment its function. Male: So down the road, the potential -- I want to use the word cure for a very good control of the situation. We get life expectancy to put it to normal if these things will work, is that true? Dr. Vicencio: That’s what we’re hoping. Male: Yeah, that’s our goal, is that right? Dr. Vicencio: Yes. That’s our goal and this will work.