Martin D. Fried MD Pediatric Gastroenterology & Nutrition 3200 Sunset AVe Ocean NJ (732)682-3425 www.HealthyDays.info
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Male Speaker: Cystic Fibrosis, what is it? Dr. Martin Fried: Cystic Fibrosis is a condition in which there is a defect in the transport of sodium. And you get lot of mucus collections in the lungs and in the pancreas and the pancreas it prevents you from having your digestive enzymes into the intestinal track. So you don't absorb fat and carbohydrates, the stools are very big, sometimes they are orange because of the non absorption of fat, in the lungs you tend to get a lot of bacterial infections because of all the mucus collections and the main reason of death in cystic fibrosis is usually due to lung infections. Male Speaker: We now are screening newborns and now they look for little markers in circum to come back the kid could have cystic fibrosis. How do you handle that, is that mean you are going to have it or making work more further testing? Dr. Martin Fried: Further testing, the goal standard is still the sweat test and abnormal elevated sweat content will probably be the best screening test. Male Speaker: We also do know there are mild forms of cystic fibrosis sometimes you go the full life and not even know you have it or sometimes a kid has been labeled as asthmatic and sometimes a good doctor have found sweat test and to his amazement the kid really have cystic fibrosis, so it's only to present classical way, but as a GI Nutritional Expert you are more involved in the non absorption part, what is the hallmark of the non absorption you will be looking for? Dr. Martin Fried: Poor waking, you are not absorbing fat they don't have the enzyme lipase. Male Speaker: Something unique about this stools usually? Dr. Martin Fried: They are big, they are bulky, they usually are -- the color is usually orange because of the non absorption of fat. Male Speaker: And they can float sometime. Dr. Martin Fried: They float because of the grease, yeah. Male Speaker: Can a baby be born and it doesn't make still in the beginning and you have some concern? Dr. Martin Fried: Yes, sometimes babies are born with cystic fibrosis and they don't have bowel moments for a while. Male Speaker: If you do have that diagnose in the GI point of view what do they usually give the kid to keep the kid gaining and thriving what could you do? Dr. Martin Fried: They give them supplemental pancreatic enzymes and these enzymes contain the enzymes to digest fat and the enzymes to digest carbohydrates and proteins. Also you can give a child who with cystic fibrosis formula in which the protein is already digested and the fat is most medium chain triglycerides. Male Speaker: The example of that would be? Dr. Martin Fried: Progestimil, and progestimil probably is one of the highest and medium chain triglycerides with the fats already absorbed. Male Speaker: So a regular format would not be ideal form for triglycerides? Dr. Martin Fried: The regular formula would not be ideal because it doesn't have the fat profile. Male Speaker: Can kid have this is for GI component and of the lung component. Dr. Martin Fried: There are many, many different forms of cystic fibrosis. Male Speaker: Some get more GI component, some get more lung component, some have both things at a high level. Okay, if you are giving that diagnosis to a child, the life expectancy years ago it was very short what it is today? Dr. Martin Fried: The life expectancy today for the most severe form is in the 20s to 30s. Male Speaker: But a milder form with treatment. Dr. Martin Fried: 40 to 50. Male Speaker: Some maybe even longer when really well controlled. We really don't know between such what we are going to have 40 50 years from this treatment from today because we have to wait 40 to 50 years. Dr. Martin Fried: The main thing that's helping kids survive say the two main things are the good nutrition and good lung care that as soon as they get a bacterial infection you treat it aggressively because it's the bacterial infections that cause destruction of the lungs and then harder breathing and tha
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