Due to a decline in my pulmonary function and a horrible 6 Minute Walk test my scleroderma specialist referred me to a new pulmonologist. She was hoping I could be put on a new drug for Idiopathic Pulmonary Hypertension, Perfinidone, but that is currently in trial for scleroderma and won't be prescribed. I am hopeful that this new doctor will be the right fit; I liked her and she is knowledgeable in ILD, pulmonary fibrosis, scleroderma and transplants. She is going to review all of my CT scans and my tests. Although I made arrangements for her to have everything by my appointment of course she didn't get them (they went into archives instead of my file but I had everything with me). I have an appointment with my PH specialist in a couple of weeks, followed by a right heart cath, and her thoughts are that another medication will need to be added to the Adcirca I currently take for PH. I now have to use O2 while sleeping as well as exertion. At the end of my appointment the doctor said she was excited about working with me, that she hates it when they send her someone who only has asthma. She said the fact that I am on anti-rejection medications that are also known to help fibrosis along with my complicated medical conditions makes me a challenge. She would like to get her other patients on the anti-rejection meds but they won't be approved. My final judgement is still out because I have felt that way about doctors before only to be let down.
My CT scan was about the same as the last time when my lungs started to honeycomb. That is when I started the process for lung transplantation but discovered I am not a candidate. The major difference is that for the first time there is no ground glass on the scan. I questioned that and was told ground glass is how inflammation turns up on film so my lungs are no longer inflamed. This might be due to the anti-rejection meds. I still have end stage lung disease.
My 6 Minute Walk Test was horrible. I desaturated quickly and never felt like I was getting any oxygen. I can always tell when the O2 is turned on and just didn't feel it. I walked 200 feet less than last time. I started out at 97% on room air. After 43 seconds I went down to 88%. According to the results they gave me 2 liters of O2 and I went down to 87%. They increased to 4L and I went up to 89% and then they increased to 6L and I went down to 87%. When I finally saw the numbers I truly believed that the O2 was not connected correctly so I am having a retest in June. This just doesn't seem right to me as I always come up with O2.
Sleeping with oxygen seems to be helping and isn't too bad. I have started exercising again. I will keep you posted as to what happens with the PH Specialist and further testing.