Hi,
I have a 5 year old daughter who was born with a severe bowel obstruction. She had malrotation, volvulus, and apple peel jejunal atresia. It was an unexpected nightmare. We were told that she wouldn't make it and if she did she would be short bowel and on feeding tubes, etc. She pulled through and went home six weeks later. She was tested for CF twice while we were in the NICU in Oklahoma, both times coming back negative(even though her mutations were on the test panel). Anyway--When she left, we were told that she would probably have loose, foul stools, and may have some trouble absorbing nutrients. She had all of this for the first 2-1/2 years and then at that age, I noticed protruding rectum during bowel movements and very greasy stools--also had only gained about a pound in a year. We moved to Kansas and she was tested for CF and celiac disease. I didn't think she had CF because was tested twice at birth. CF was confirmed genetically and through a sweat test. Her mutations are DF508 and 711+1G>T. She never had any colds, cough, runny nose, nothing as an infant, all the way through the first four years. She started having a little cough around age 4, but that's it. She has trouble digesting food, takes Zenpep 8 w/meals and 2-3 with snacks and still has about 4-6 loose, foul stools a day. I have to wonder if some of the malabsorption is from the lack of intestine (she lost a ton and alot was gone when they did the surgery to begin with) or if it's from the CF. The point is, it doesn't matter either way and now I'm certain that the CF caused the obstruction. But, I also have to wonder how she has been so fortunate with her lungs so far being 5 years old and no lung infections. I do know that the 711+1G>T is a class 5 mutation. Is that what may help keep her from being as sick? Or-should I expect her lung infections to be beginning. We do all her treatments and treat her like you would treat any other child/person with CF. She is starting school in the fall and I am very worried that these infections may start. Anyone else with no or little CF symptoms, even though they carry a Df508 mutation?
I have a 5 year old daughter who was born with a severe bowel obstruction. She had malrotation, volvulus, and apple peel jejunal atresia. It was an unexpected nightmare. We were told that she wouldn't make it and if she did she would be short bowel and on feeding tubes, etc. She pulled through and went home six weeks later. She was tested for CF twice while we were in the NICU in Oklahoma, both times coming back negative(even though her mutations were on the test panel). Anyway--When she left, we were told that she would probably have loose, foul stools, and may have some trouble absorbing nutrients. She had all of this for the first 2-1/2 years and then at that age, I noticed protruding rectum during bowel movements and very greasy stools--also had only gained about a pound in a year. We moved to Kansas and she was tested for CF and celiac disease. I didn't think she had CF because was tested twice at birth. CF was confirmed genetically and through a sweat test. Her mutations are DF508 and 711+1G>T. She never had any colds, cough, runny nose, nothing as an infant, all the way through the first four years. She started having a little cough around age 4, but that's it. She has trouble digesting food, takes Zenpep 8 w/meals and 2-3 with snacks and still has about 4-6 loose, foul stools a day. I have to wonder if some of the malabsorption is from the lack of intestine (she lost a ton and alot was gone when they did the surgery to begin with) or if it's from the CF. The point is, it doesn't matter either way and now I'm certain that the CF caused the obstruction. But, I also have to wonder how she has been so fortunate with her lungs so far being 5 years old and no lung infections. I do know that the 711+1G>T is a class 5 mutation. Is that what may help keep her from being as sick? Or-should I expect her lung infections to be beginning. We do all her treatments and treat her like you would treat any other child/person with CF. She is starting school in the fall and I am very worried that these infections may start. Anyone else with no or little CF symptoms, even though they carry a Df508 mutation?