Nov 7th – today’s the day! I saw Dr. L last week and he is basically waiting to see what Dr. G has to say. He seems to be quite a bit more concerned about me, but the last two visits I’ve been more concerned about myself. I think because I usually greet him with I’m ok when he asked how I’m doing and that hasn’t been the case the last two times. But all of the weight I lost earlier in the year I have gained back in just a few week period from the pulmonary hypertension and subsequent mild heart dysfunction so even though he says that I’m in the window for needing a transplant I’m now not a candidate because my BMI is too high. It’s just a vicious circle.
I have another echocardiogram and unfortunately the results are back when I meet with Dr. G. She seems pleased with the results of the Tyvaso and diuretics so far but without the echo she can’t really give me any real prognosis, just stay the course and she will message me when she has the results.
My benefit dinner is later that same day. WOW! Can I just say that people are amazing. We had such a great turn out. There wasn’t a parking space or a seat left in the place. People from all aspects of our lives, even a few people we didn’t even know. I can’t even express the amount of love and support I felt that night What I can say that between the GoFundMe and the money raised by this dinner has met almost 2/3 of the amount that the social worker at Cleveland Clinic told us to have on hand for transplant. I think I cried several times that night. Thank you again to anyone reading this that played any role in these at all. (I’ve tried several times to upload pictures from that night and it isn’t working, sorry)
And the night ended with the best news! Dr G sent me a message that the echocardiogram showed improvement!
The Tyvaso came towards the beginning of the month and I had to have a nurse come show me how to use it, apparently it’s mandatory. It came the week before my siblings and I went to Tennessee. I was bound and determined that if they went again, I would join this time. I’m a little concerned that I won’t be able to do a lot of things and getting ready for a trip with all of my medications and oxygen now requires a lot of additional planning. My siblings and their spouses were so considerate of my limitations. They pretty much did whatever it was I felt I could do. Our first day was in Pigeon Forge, we went with no plans or expectations. We played putt-putt golf, which ate up a lot of our time when you think about 12 people playing. We ate at Margaritaville. I really wanted to do the Alcatraz crime museum but between the putt-putt and the walking I had enough for one day. I have several days ahead of me and don’t want to be a party pooper by exhausting myself the first day. The second day was Gatlinburg, we started at Skypark, they have a Skybridge that I wanted to mark off of my bucket list. It’s 680 ft across (the longest bridge of it’s type in North America) and 140 ft off the ground. We took the walkway to the one side and walked the Skybridge back. I did ok until about the last 200 ft or so when it becomes an incline. Inclines are almost impossible for me now and my concentrator just didn’t keep up with my needs. By the time I got to the other side I was so out of breath that it took a long time to recoup. It didn’t help that I had a mental breakdown. I was feeling so many emotions, I was proud of myself for doing it but also sad that I just can’t be “normal”. I was trying to regain my breath when my brother, G (Alpha) came to my side, he didn’t say anything, he didn’t have to, just his presence and support was all it took and I began to cry like a baby. Then others joined in to let me know that I wasn’t alone and I want to believe that they felt what I was feeling. I finally regain my composure after making a fool out of myself in public but Hey, I got a t-shirt out of it from G to remind me that “I did it”. As hard as it was, “I did it”. We have lunch at Dick’s Last Resort and I think we all had a great time. I would not suggest you go there unless you have a good sense of humor as the service is intentionally rude for entertainment purposes. By this time I am ready to return to our AirBandB but most of the others stayed and tried some Tennessee whiskey and did a haunted house. It’s a good thing I did return when I did because by evening my leg muscles were angry with me. I think I actually may have gone into a small case of rhabdomylosis because this wasn’t normal muscle aches, my leg muscles were rock hard and I could barely move them. Luckily the room that B and I got was the only one with a jet hot-tub. B helped me get in and out of it and it seemed to help some and then I downed 800 mg of Motrin. By morning I felt better but today is a day of rest. We do visit a small local store that sells Amish goods, did the Smoky Mountain Alpine coaster (secretly, I may have enjoyed this the most), ate at Boss Hogg’s BBQ shack and then went to a small antique shop. Most evenings are spent playing games or sitting on the porch. I don’t know about anyone else but I’m having a good time. Our last day we make the long trip to Cade’s Cove Scenic Loop, it’s part of the Great Smoky Mountains National Park. I have to say I was quite disappointed in my choice to do this. There was really only one part of it where I could actually get out a view more than one building of the old settlements but at least the driving views were cool, AND I got to see a bear down in the creek just as we were living thanks to B’s quick eyes or I would have missed it. We ate a Mexican food truck called Taqueria Macho Taco on the way back to our place. The evening brought a heart to heart with my big sis, B. She lives in Washington state and she’s the one that also has autoimmune disorders. I remember during our conversation I told her that “I’m not afraid of dying, it’s living my family behind. I don’t want to be the reason they feel grief and pain”. And it’s the truth. Dying doesn’t scare me, I know where I’m going. But when I think about them living life without me, that’s what brings the emotions.
When we return it’s not long and my father needs to be admitted to the hospital. He’s very anemic and needs blood. They keep him for several days, do several tests and give him blood a few times. They really don’t have many answers and the only way to make a final diagnosis would require a bone marrow biopsy to see if his body isn’t producing any blood cells. He declines the test. The only other thing they are thinking is that his mechanical heart valves are basically shredding the cells when the blood goes thru them. The only treatment for that is surgery and he states he won’t do that either. Our family’s annual FallFest is coming up and we (the family) don’t want Dad to miss it as we know this will be his last. Luckily, things get arranged in time for him to be discharged and he is able to go. Another blessing from above.
The next weekend brings about a change that I have been putting off for two years. Back when I was diagnosed B wanted to move me into a smaller place because our home and yard are just too much and getting me moved “in to town”, will get me closer to my girls and closer to the Cleveland Clinic. Well, I didn’t want to give up my home, so our son stayed behind and we rented a lower level apartment in the same building as my middle daughter, K. This was also a blessing in timing as K’s building stays pretty full as it only has 12 apartments and they go fast. Well right after my appointments in August she said that the bottom apartment was going to be available. This apartment just happens to be the closest one to the laundry area too! I’m telling you, I can’t make this stuff up. I believe this is the hand of God. Always moving things in to path just when I need them. On October 4th, I said goodbye to my home, tears streamed down my eyes. Looking back at it, that was silly because we still own it. I can still go sit on my back patio any time I want, but that day it felt like I was closing a large chapter of my life. But, I’m happy to say that B was right. The apartment is much more manageable for me, so now I don’t feel overwhelmed, I feel accomplished!
Next up is my next round of appointments and a benefit dinner for me and my family to help alleviate some of the expenses we are about to incur. My middle daughter had already started a GoFundMe and her best friend is organizing the dinner. The dinner is planned for the evening of Nov. 7th, I will see the pulmonary hypertension specialist that morning. I hope I don’t get any bad news to damper the evening. Guess you will have to read the next post to find out.
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.
