Hi all--Adam had his first visit to Johns Hopkins yesterday. It was a great appt. We were going for three main reasons: (1) CHOP said no CF; Children's Nat'l said CF. His mutations are supposed to non-symptomatic and his only sweat test was borderline. Zenpep has helped him tremendously. (2) If CF, we wanted another opinion on his treatment plan. Children's Natl docs have him doing no airway clearance; PFTs usually range from 79-82. (3) If not CF, then what?
We saw a resident and then Dr. Mogayzel. After reviewing Adam's history,recent test results, and an exam, he concluded that he does not know if he has CF. I truly appreciated his honesty and his willingness to help us find out. So, we will go back in a couple of weeks for a repeat sweat test, CT scan of sinuses and lungs, and an NPD. He will also do a bronchoscopy (sp?) if needed for a more definitive dx.
He did say that if a dx of CF is confirmed, he will add airway clearance to Adam's treatment plan. As for an alternative dx if not CF, he mentioned the possibility of idiopathic PI or Schwachmanns. Adam's BMI is at the 29th percentile so they want him to gain at least 2 lbs before his next visit.
In the end, I feel like we will get some answers and will be able to move forward without nagging doubts/questions.
---Chris
We saw a resident and then Dr. Mogayzel. After reviewing Adam's history,recent test results, and an exam, he concluded that he does not know if he has CF. I truly appreciated his honesty and his willingness to help us find out. So, we will go back in a couple of weeks for a repeat sweat test, CT scan of sinuses and lungs, and an NPD. He will also do a bronchoscopy (sp?) if needed for a more definitive dx.
He did say that if a dx of CF is confirmed, he will add airway clearance to Adam's treatment plan. As for an alternative dx if not CF, he mentioned the possibility of idiopathic PI or Schwachmanns. Adam's BMI is at the 29th percentile so they want him to gain at least 2 lbs before his next visit.
In the end, I feel like we will get some answers and will be able to move forward without nagging doubts/questions.
---Chris