Summer ’24

I’m due to go back to Cleveland Clinic at the end of June, in the meantime, I’ve done all of my homework. I saw a gastroenterologist and had my colonoscopy but I also had an upper endoscopy and a stretching done as we discussed my issues with food sticking. During the endoscopy they found esophagitis with esophageal stricture – biopsy results came back with tissue consistent with Barrett’s esophagus. Both have been caused by severe chronic gastro-esophageal reflux disease (GERD). Because of this I have several tests related to GI issues scheduled this time around to see if I will need a feeding tube whenever I need my transplant, because if you remember my lung biopsy results, I’m aspirating – most likely at night from severe GERD. All of this is related to my auto-immune disorder – the one that I learned in school was basically dry eyes/dry mouth – yeah, maybe more teaching/research needs to be done on that!

I have a follow up with my lung dr in April. I forgot to mention that in my last post. I’ve been having issues with the antifibrotic medicine, OFEV. I vomit almost every day with it, even if I take the protein they recommend, so we decrease the dose to half. We also trialed coming off of Prednisone since I have been on the CellCept and prednisone now for a year. The lung dr and I agree to try to eliminate the steroid. Then he tells me that in July he will be leaving the practice. My world seems to be crashing….the Dr. I’ve come to trust with my life is leaving the practice but he asks me which dr in the practice I would like and makes arrangements for that physician to take over my case. As much as I hate to see him leave, he’s young, intelligent and caring and the nurse tells me that he has a wonderful opportunity set before him – I don’t ask what that is but I know he well do great things for the next patient he cares for

As far as the prednisone, Let me tell you, the withdrawal from that if awful. I was only on a small maintenance dose so we don’t wean off – I just stopped taking it. Three days without it and I’m already aching all over, weak, nauseated. By day 10, I’m an emotional mess, I’m crying at work, I’m crying at home, I feel very emotionally unstable. By the end of the second week, I’m also starting to have issues with my oxygen saturations and my ability to function without oxygen is declining. I’m finding myself needing it for things I haven’t needed it for in almost a year. I get in touch with my lung dr and he puts me back on the steroids, within a day I’m starting to feel more “normal”.

My Cleveland Clinic appointments goes fairly well. The worst part was the GI tests, June 24th. There are two of them, an esophageal manometry test and a GI probe. The manometry test measures muscle pressure and coordination of the esophagus. A GI nurse placed a thin catheter thru my nose into my esophagus and then gives directions on when to swallow sips of water, not bad until she gets to the part of the test where I can’t swallow for like 15 or 30 seconds, I can’t remember the exact length of time now. You would think that isn’t hard to do, just don’t swallow for 15 – 30 seconds right – wrong, not so easy for someone who coughs like 200 times a day and it’s an automatic reflex for me when I feel a cough coming on. The nurse gets extremely upset with me and says “It’s just a mind over matter kind of test”! No kidding! I’m seriously trying lady. It isn’t until I throw up all over myself and almost on her that I get any sympathy from her. That’s when she realized I really was trying and she aborts trying any more “episodes” and says she will just send what she has. Next comes the pH probe, this will monitor the acid levels in my esophagus for the next 18 hours or so. Again, the nurse placed another thin catheter in my nose and down my esophagus. This one will stay in until the morning when I remove it myself. The probe is attached to a monitor and I am given instructions to press a button every time I have reflux symptoms and I am to write down when/what I eat and drink. All of this information will be turned in to the GI department in the morning. This part isn’t too awful except for when I eat. I think that the end of the probe is either IN my stomach or very close because every time I eat it feels like my stomach is pulling the tube further in – it’s quite annoying and I keep pulling the tube back a little bit all night.

The next morning, I return everything to the GI department and complete my normal transplant eval tests that I will complete every time I come, lab work, chest xray, blood gases, lung functions test, walk test and meet with the dr and nurse coordinator. Dr. L reviews the manometry test, I do have SOME issues with swallowing but he says it shouldn’t be a contraindication to getting a transplant but could affect post-op management, especially feeding strategy. Overall, I am stable and not reading for listing. I will come back in about 4 months and I will see a GI specialist (to discuss post op feeding needs) and an infectious disease dr then (this is just routine).

July 20th, my family and I go to Pittsburg for the Pulmonary Fibrosis Foundation walk. I have been able to walk the distance so I am able to participate (I think my last post I wrote that it was 2 miles, it was only one – there was a 2 mile option but I wasn’t sure I could do that distance, it takes me half an hour just to do one! Having my family with me is just incredible, their love and support make me speechless. I would love to be able to do this again next year. There are many families there, some have lost loved ones to PF already, some are there because they had PF and now they have new lungs, some are like me and currently have PF. One lady sought me out though and asked me about my situation. She had just lost her husband to idiopathic PF a few months before the walk, she was there with her granddaughters and walking in his honor. She was such a sweet lady – she showed me such compassion all while expressing her own grief. Some humans just amaze me, and she doesn’t know it, but she left such a mark on my heart, I will never forget her.