My annual clinic appointment was today, a month late for my 2011 annual, but that’s where it belongs in the timeline. Overall it was good news, but we had some very good dialogue with the doctor about quality of life vs the PFT numbers and what the picture is now and what it looks like for the future. Prepare yourself for a long post because I really don’t want to break this one up over the week.
Good news first: I’m not dying. I’m also not expected to be on the transplant list any time soon. The rest is a big bag of mixed nuts that will take us a while to sort through. I really hope that you find this informative as an “aging CFer” to know what is ahead for those younger than me and also encouraging and transparent for those in the same inning as I am – that’s 33 for those new to the site.
For one, my PFTs are down 3% from 39% to 36%. They put me in the box for PFTs this year for the annual check-up, which measures 2 additional things: diffusion of O2 into the blood and the amount of air that gets clear in and clear out. My diffusion is excellent, which means that my body performs at a high level of gas exchange so I can still get oxygen when my volume is low. The test also showed that I am (this is what we remember hearing and then Googled it) gas-trapped: I’m breathing in deeply, but then all of the air doesn’t get blown back out. This eventually leads to high concentrations of carbon dioxide because the air is depleted of O2 and then can’t get back out. Protocol: new broncho-dilators. We are going to try an Atrovent/Albuterol combination for 4 doses and then try Spiriva for a few days to see which one makes me feel better. He said that I am not to take both concurrently.
That brought up the nagging question about my quality of life and why they won’t put me on IVs to feel better. I was both happy and sad about the answer, you’ll soon know why, too. In the last 2 years of being healthier because of compliance and much higher body weight, I have new susceptibility to IV meds that were once resisted by my bugs. Back then was my worst: I was down to having 2 meds to push on an infection that required that much intervention. I have at least 3 new meds that could work so we asked why they don’t put me on two of them to feel better and push transplant back further into the future. This is where I just about choke up because we used the “t-word” again. I also asked about how they determine the give/take between keeping PFTs up with IVs and saving IVs to stay alive while listed for transplant.
/gulp!
I got a very long answer, which finally made me understand the big picture and the methodology they use in their practice, combining results with theirs, Duke, and Pittsburgh to evolve the best course of action as CFers progress to late-stages of the pulmonary disease. /gulp They don’t save the IVs to keep you alive for the long-haul awaiting transplant here because listings typically aren’t very long; they use them as a gigantic cocktail just before and after transplant to kill all of the bacteria from the diseased lungs that course through the bloodstream during surgery and can land all over the body, including the new lungs. They essentially go all-in as in poker. They want as many weapons as possible to kill everything in an anti-biotic sort of chemotherapy. The light bulb went off in my head then: they really are preparing me for transplant… just not any time in the near future. My team is very, very good at what they do, and now I’m putting my life into their hands even more because I trust their combined science and wisdom in treating so many of us each month. I’m trusting them that they are making the best long-term decision to deny me 2%-5% PFT points in exchange for saving me from a life-threatening infection post-transplant.
On the note about pushing that event horizon further away, we got onto the topic of the chest tightness. He listened to me wheezing and then spaz when he made me “blow out the candle.” The discussion switched to reflux at that point. Solving that has a lot to do with delaying transplant because I may be acid-washing my lungs every day now, so I’ll be having a swallow test with barium and a 24-hour pH test to see what the pH levels are just above my stomach. I really, really, really, really, really don’t want to do the pH test, but if it means going longer with these airbags, I’ll do it. Did I mention that I really don’t want to do that? If I do have reflux, we’ll do a Nissen fundoplication to prevent acid from entering the esophagus when the stomach churns and contracts.
But wait! There’s more!
My weight and appetite have been a bit of an issue, but they aren’t super concerned about it because I’m not diving off the deep end with my weight. I was 120 this morning, but a 23 BMI puts me at 134 +/- 5lbs to avoid the issues of being overweight with CF. I told them that my problem is simple, but I don’t have a fix for it, thus it’s complicated. Here it is:
- I don’t feel like going through the effort of making my 1,000 Calorie breakfast because I’m not hungry in the morning
- I’m not hungry because I’m not exercising
- I’m not exercising because I’m so darn tired every day and take the entire weekend off now to stay strong to fight infections
They worked it backwards and put me on mirtazapine (Remeron) to stimulate my appetite. It’s classified as an anti-depressant and I discussed it further with Sue, who told me that it has excellent results for those it works for (most) and no one really has any side-effects. I need to gain 15 pounds, and that is what this is good for. Anyone have experiences with Remeron that helped them gain weight?
Then…
After we’d been home for a while, Sue e-mailed me to call her. She said my liver enzymes were up to 100% elevated from my ER visit levels in November, so they are putting me on ursodiol to help my liver cope. It’s not because of excess drugs that we take as CFers, rather it’s the gunking up of the liver in the same way our pancreas gunks up. Yay! Another medicine! Of all of these changes today, I’m the least concerned about this one, but I think this is the one that caught Beautiful by surprise the most.
All in all, we were very happy leaving clinic knowing (finally really knowing) why they have been and still are refusing to put me on IVs when I feel like crap. Until my numbers show something else or I: have recurring fevers, go on IVs every few weeks just to keep me alive, my blood gas shows higher concentrations of carbon dioxide, or heavy hemoptysis, my life remains as-is in the aging CF status quo. To quote my doctor’s best line today: “You’re walking around with the lungs of an 80-year-old – you’re not supposed to feel good,” when I told him that I just don’t feel as good as I used to.
Keep your stick on the ice. We’re all in this together. Oh, and go read Beautiful’s account of today, too.
Wow! That’s a ton of info to digest! I’m glad that you’re good to go for another ten thousand or more miles! It does seem overwhelming to have to deal with being “old” as a CFer and while only in your 30s. I feel old too sometimes and it’s scary to think about the future sometimes and the inevitable transplant :-/ Thinking of you and wishing you the best!
Thanks. Just slap some new paint on me and I’ll get back in the race tomorrow. I first remember feeling old somewhere around 25 or 26 when my sats dropped enough to be on supplemental O2 for a few weeks. I guess my race wasn’t over yet.
PS I’ve had an extreme burst in motivation for fundraising for the CFF in an effort to get the damn cure here ASA-freaking-P!
Remeron worked well for appetite for me for only a little time. I think I got the effects for like a week or two before they disappeared. It’s great for sleep though, so you probably are gonna take it at night. Oddly lower dosages = more sedation so if you are already really tired you may want to think about that. I take it for the anti-depressant/sleep thing but when I’m sick it makes me sleep a huge amount. It’s not as bad as waking up after taking Lunesta or something, but it’s still something to think about.
What about other options for weight? Megace got me to pack on a good 20lbs in about a month recently so that could be a short term option (considering that it has some nasty side effects if used for too long). One 5ml dose of Megace ES in the morning and I am starving all day. Literally every time I see food I eat it. Finally we found something that made my appetite go back to the way it was before the G-Tube (hungry all the time and food tastes good again).
Another option would be Marinol. Marinol is also good, but I wasn’t a huge fan of the fact that it was a quick hunger and my satiety didn’t change (1 small-regular meal per dose essentially) rather than a whole-day ravenous hungry (with Megace it was like 5+ meals per dose of huge meals).
Hey, thanks for the comment. Interesting the differences meds make in different people. Yeah, I’ve had my own battle with Megace: https://cffatboy.com/2010/06/i-say-megase-you-say-megace-i-gain-weight/ up until https://cffatboy.com/2010/09/major-progress-regarding-fatboys-man-boobs/ so we dropped that so my testosterone levels could normalize. I suddenly needed 1/8th of that and my raging more or less ended, but so did my appetite. For me, it was a per-meal basis and didn’t make me hungry from nothing but only shut off the “I’m full” signal to my brain.
I’m looking forward to see how this one treats me because Sue said it’s had good results AND I do have something to compare it to if we decide the side-effects of one are more or less tolerable vs the weight gain with each. So long as I’m not in any danger zones with my stats, I’m more than willing to try new things to develop more data and I’ll be sure to keep everyone updated.
i like the way you digested your annual visit. All in all it sounds like you have a fantastic team and im so glad you trust their approach. :). Hope your appetite perks up!
Thanks – I’m glad you liked it all in one. I was afraid it’d be too much for one sitting. 😉 We forgot to ensure that my nightly muscle relaxant I take for TMJ won’t interfere with the anti-depressant, so we’re going to check that tomorrow and start once we’re sure.
Jesse,
Check-ups are quite the deal aren’t they! Thank you for your post and for the details. To me at age of 55 you are just a kid CF’er! Ha! I know you are one tough dude so don’t let the slightly lower PFT numbers get you down. Next visit they could easily be up again. They vary. Mine range from a lows into the mid 30’s to a high of 62 a year ago and are now at about 52%. Like you it has been about 3 years since I have had IV’s and I think my time is coming due. Have occasional bleeding and some lung swelling but no fever so like your team they keep putting mine off and have been using Prednisone instead. One thing I can say is that walking is a great exercise and I am now up to about 3.5 miles 4 times a week. I prefer outside trails and try to do it in under 50 minutes. I wanted to mention that I have been approved for the Vertex 508 study. I do not have the single delta 508 coupled with what is called a “nonsense-x” gene so this new study will use two Vertex drugs. There was no place close for the trials so I started calling the different CF research centers. I flew from Wichita, KS to a CF Research Clinic in Ohio. They are fantastic. They have over 500 patients with about 230 or so adult. Their oldest CF adult is approaching 70! Ha! As you know I as a test animal may get a placebo or the real drugs and will have a lot of tests run over the next few months until the trial ends in April. These genetic drugs are a real hope for us and hopefully will be out within a couple of years. The Vertex drug has the potential to fix the “clogging issue” not just in the lungs but throughout the body.
Are you going to Toledo? That’s my home clinic as a kid until I was 16. I got an e-mail from the Orlando team last week to see if I had one copy or two, and since I have both, I was out of consideration. I’ll just wait for it to come to market unless they make it no muss, no fuss now – I won’t be able to handle the business, foster care, and a medical trial all at once.
Jesse,
It is in Columbus at Childrens Mercy Hospital. The first visit which was yesterday was just a bunch of tests to establish baselines for the study. It was a tough trip. Didn’t arrive until 9 pm the night before and then to the clinic at 8 am. Then flew back to Wichita at noon after the tests. Will be going back every two weeks for the next 3-4 months. You do a great job with your posts and I pray you keep doing well.
Like you my main support and encouragement and strength comes from my wife Stephanie and my faith in Jesus Christ. I sincerely hope these new genetic treatments will do wonders for us who are aging and give us some relief and added strength to run the race.
Thanks for all the info. It’s great to hear what the medical staff is saying and their reasons why. I’d never heard of a Nissen fundoplication before. Hope all the new meds/changes are helpful.
I only mentioned it in passing in 2010 when I started Prilosec, but it wasn’t news to me. I just thought I’d avoided it with a med.
Wow, Jesse. What an overwhelming day, information wise. Thank you for being so candid and honest about your visits and where you are in regards to this disease. I’m terrified of the day when the “t” word starts being thrown around, but I know it will happen eventually. Reading your story, seeing how positive you are, and seeing how long you still have before they revisit that issue helps to provide me with courage. They really need to find this cure – I’m tired of the unpredictability of this disease.
I’m keeping you in my thoughts and prayers and I truly hope this new medicine works well for you.
What is your FEV1 at now? I first heard it around 25 or 26 as a scare tactic to do my meds better and again around 30 when my clinical trial put me into the 20s after 2 weeks of a placebo. I’m no longer freaked out by the idea but we’ll see how I feel the day they send me to transplant clinic instead of regular clinic.
I’m very blessed right now. My FEV1 last appointment was at 87% though I know it will be down in two weeks when I go back. They had me on Hyper-Sal which, I believe, was ultimately causing the hemoptysis so I’m not doing it anymore. I’m also feeling a very distinct tightness in my chest when I try to get a deep breath, so I’m expecting a decent drop in my numbers. Hopefully not though. I actually first heard the “t” word as a scare tactic at 17. I came in to the hospital, according to docs, about a day away from not being able to pull out of a severe lung infection. I wasn’t doing my treatments and was pulling typical teenage stuff. I had a doctor come in and tell me that I was headed right down the road for a double lung and heart transplant. It woke me up. That’s for sure. It’s bothersome how unpredictable it all is. You never know when you’ll have a flare-up that you can’t easily bounce back from.
I’m glad that you’ve come to terms with the idea of a transplant (at least for now), but I do hope that it’s awhile before you have to head to the transplant clinic.
Hyper-Sal is very irritating, I know, but I do encourage you to start as soon as you can. From my experience with my brand of hemoptysis, I get it when I am either a) really infected or b) getting the bottom layer of gunk cleared out, exposing irritated tissue that bleeds.
I couldn’t agree with you more about how bothersome the unpredictability is. I suppose that’s what makes us live life with more fervor and conviction.
John has been on Ursodiol for many years now. He had high alkaline phosphate levels. They weren’t quite sure if it had to do with gallbladder issues or CF. So, he’s been on that one for a while and no real change in his liver levels one way or the other. They stay elevated, but with no concern to the doctor.
Well, that’s good to know. I took my first dose last night with dinner and didn’t notice any side-effects.
I’m also on Ursodiol. Have been for about three years now. They put my on Megace a few months back cause I too had just hardly any appetite. I’m hovering around 130 right now, but at 5’11 there is still some weight to gain to give me that “sick cushion”. You sound pretty much like me in the PFT realm. Similar numbers, but so far we are puttin back transplant for now cause otherwise I’m doin ok. The thing that got to me the most was the “lungs of an 80 year old”. Couple years ago I got the “lungs of a 65 year old smoker” from my doctor. That hit me hard that day. No one had put it in that context for me before. Def puts ur lung function in perspective I guess. Congrats on a pretty good clinic overall – stay healthy!
Oh, you’re an 18 BMI then, eh? I can attest to how much better my lungs felt once I got up over 20 BMI, which is almost exactly where I am now. If I drop below this, I know exactly how I’ll start to feel.
It’s amazing what context does sometimes! I suddenly realized that I really should be feeling like and moving around like my grandpa, who just turned 81. That was amazing perspective to be able to say “could Grandpa do this?” and feel better about my limitations.
Thanks for sharing, Atrovent worked well with me, I noticed results almost immediately. The swallow test and 24-hour ph test indicated that I could be treated with omeprazole twice a day, and I was advised to elevate my bed 6 inches at the head. If found Remeron truly amazing…it worked well before my transplant (110lbs to 126lbs). Then post transplant (126lbs to well over 160lbs), I am no longer on Remeron and weighed in at my clinic visit this past Wed. at 157…I am at the high end of my BMI. My latest PFT were 176% over predicted. Our team at TGH is amazing! Again, thanks for sharing. If you ever have any questions from someone who has walked down the same path or just want to talk I will send you my cell.
Thanks for that. I’ll definitely want some post-perspective from you, Buck, and Brian when that time comes. It’s super comforting to know I’ve got such good results from our team living awesome lives so nearby. I think Buck lives 2 minutes from our place.
Re: GERD, since I’m already on Prilosec once a day, I’m not so sure I’d want to go through the pH test just to double my dosage… since NG tubes make me puke. How uncomfortable was eating, coughing, and sleeping with the tube for a day?
Those are some amazing numbers! You must have acquired teen lungs or an athlete.
The tube was about the size of a piece of speghetti. It felt like a constant tickle in the back of my throat and bothered me most in my sinuses. Re:PFT, I was told I would peak around the 6th month. I was around 80% predicted then, so you have to really work them 🙂
I’ve had an NG tube twice – once to give me GoLytely in the early 90s when I had a blockage that got me admitted and once for the enteroclysis test. Both times, I puked and I constantly swallow, so I don’t know how I’d handle having one for 24 hours.
Very interesting post, Jesse! I appreciate all the info, too. I find my doctors will answer any questions I can come up with, but they never volunteer any info. Sometimes it’s hard to know which questions to ask!
I was about to ask how they test pH but it sounds like a tube in the gut for a day? I’m on Protonix 2x day. No one’s ever tested my pH.
I’m an old-timer, too. At 57, I’ve braced myself for the “other shoe” to drop so many times I can’t count them. It does wear on you. But I’m so grateful to live this long and trying to live each day to the fullest, with God’s help.
Yes, the questions are often the hardest thing to come up with to get the most out of the visits. Beautiful says that I sometimes say my issues too jokingly, so they get glossed over, so this time I didn’t let it go by that I am not satisfied with feeling this way.
Yes, the pH test is an NG tube just above your stomach for 24-hours. A local post-txp friend said it’s irritating, so I know I’d hate it. Every NG tube feels like a cold, wet noodle stuck at the back of my throat, so I continually swallow. So exhausting.