CF child with liver disease has normal pfts but has chronic shortness of breath

[Mom of CF]

My ten year old daughter has Cystic Fibrosis with Cirrhosis of the Liver, Esophageal Varices, Portal Hypertension, microscopic colitis, CF sinus disease, CF diabetes, and autism spectrum disorder. She grows aspergilus.
She is currently on the liver transplant list. She is on a low protein diet because she has high ammonia levels. She has had many tune ups for pulmonary issues in the last year, but has not needed one in the last eight weeks. Her latest lung x-ray is great, her PFT's are wonderful, we have done exercise pft tests, IV bubble study echo, and arterial blood draws. hepatopulmonary syndrome and pulmonary hypertension has been ruled out. She has no or very little acites.  The PFTS, and echo is great. The only thing that showed up was her PaO2 was in the 60s ( not too bad ) So far there is no pathological reason for her to have the feeling of shortness of breath. This has been going on for three months, and seams to be slowing getting worse. This is something she is complaining about often and worsens as the day goes on. Any ideas???

[National Jewish]

Please check with a specialist at your local children's hospital that has a cystic fibrosis (CF) clinic.

[Mom of CF]

Sunny;

Thank you so much for your comments and thougts.  I will definatley bring that up next time I see one of her Docs. Thanks again!!!

Mom

[Sunny602]

Hello CF mom...firstly, I am sorry your daughter is going through so much at such a young age. I also have CF, diagnosed later in life (I was diagnosed at 33). I am not a doctor, but maybe her advanced liver disease is fatiguing her to the point of feeling short of breath with any exertion. Her Pa02 is on the low side, not horrible but still a bit low...maybe something there too to consider as to why her PaO2 is on the low side. Another thing to think about is if she is getting enough O2 during sleep, perhaps maybe an overnight oximeter test might help. Did the docs test her SpO2 levels while she was walking etc....? Some people have good O2 levels at rest, but when they do anything, their oxygen levels fall. I can't think of anything else to say except God's speed regarding her getting a new liver. Hugs, Sunny