DD is 7. She has had stuff going on since birth. She spent most of the first two years on TPN since she was unable to absorb anything through her gut. She gets pnuemonia a few tims a year. She cultured staph when she was bronched. She has had recurrent ear infections - on her 6th set of tubes, recurrent sinus infections, and has had sinus and ear polyps.
She is pancreatic insufficient. She takes creon12 - 5 with meals, 4 with snacks. She weighs 42 pounds.
I can't remember her fecal elastase level. I think I remember them saying she was putting out 29% fat. Would that make sense?
She has had 3 negative sweat tests - 56, 59, and 39.
She has the S1235R mutation.
Her most recent chest x-ray shows mild scarring in her right lung.
DS is 5. He was a preemie, but has also had symptoms since birth. He gets pnuemonia 4 or 5 times a year, dx through chest x-ray. He had a collapssed lung in January. He has cronic sinus infections, and a cough 90% of the time.
He is also pancreatic insufficient. He is on creon 6 - 3 with meals, 2 with snacks. He weighs 35 pounds.
I don't remember his fecal elastase results either, but I want to say they were normal, or close to normal.
He has ahd 2 negative sweat tests - 36 and 59.
His mutation is also S1235R.
His most recent chest x-ray shows moderate scarring in both lungs.
Our clinic is saying since they have both had numerous negative sweat tests and only show one mutation from the ambry test that is was atypical cf (now CRMS)so they are not doing chest PT. They are on contact precautions, both take Creon, AquaADEKS, and singulair. She takes previcid to help the enzymes. He also takes flovent. And both of them use simply saline. Both kids get nebs pretty much all winter since they are so junky.
