My 14 year old daughter has been diagnosed with pulmonary fibrosis

[dawnddy]

My daughter since she was 8 years old has been dealing with Mix connective tissue disorder.. she went through alot of work to be able to have a some what normal life. she been on medications for the last 5 years to help maintain her illness..  This year she had her usually pft test where she dropped to a very low level. She had a lung biospy done on march 4 where she was give a life sentences of they do not know how long she has to live.. She was put on cytoxan for the last 8 months and this drug just made it worst. Her diffusion level has dropped and the doctors are telling us they do not know what is next to do.. I need some kind of help. She just had a stress test and we are waiting for the results to come back in from that. He told us that that point her pft was at 61/53.  I need to know what is a good score for a pft test and what level should we be concerned about..

[National Jewish Health]

I regret that the PFTs result you list is not specific enough for interpretation.  I suspect that these numbers are ‘percent of predicted’ values for one or more lung measurements.  "Normal" predicted lung values are often expressed at 80 to 100%, and so 61/53 would be reduced.

I suggest the National Institutes of Health (NIH) http://www.nih.gov for information about research in mixed connective tissue disease.  Those conducting the study at this link http://clinicaltrials.gov/ct2/show/NCT00582881?term=Mixed+connective+tissue+disease&rank=1 may be able to direct you to a therapeutic trial.  You may also want to contact the nearest children’s hospital to inquire if any of their rheumatology doctors are engaged in clinical research on mixed connective tissue disease.  You may also want to consider a second opinion at such an institution or you might consider seeking a second opinion at a nationally recognized rheumatology center, such as the rheumatology division of National Jewish Health or the associated division at the Children’s Hospital in Denver, Colorado.

I also recommend that you and your daughter's doctors consult with a regional transplant center to determine if, at some point, she might be a candidate for lung transplantation.  Lung transplants are performed for a variety of diseases and hers may be one of them.

Good luck.

[dawnddy]

Thank you for your help. My husband and I switch my daughter to the Children Hospital of Philadelphia (CHOP). She has been diagnose with Pulmonary Hypertension and now she has been put on a lot of different medicines and is having so many side effects. I am convinced that they are missing something but I think this hospital will find it out.. She has mixed connective tissue disorder and now this year she been diagnose with Pulmonary Fibrosis and Pulmonary Hypertension. I think they are not seeing the whole picture.. Her blood work is coming back with every number coming back extremely low. My daughter is an amazing young woman, Stronger then I ever was..