Bronch Photos

Attached is the printout of images that I was given yesterday after my bronchoscopy. If you get grossed out with seeing graphic photos of someone's lungs then do Not scroll down.
I thought it might help to send this so everyone gets an idea just how bad I am when I say that my breathing is tight. 
My right lung was completely closed off so I wasn't getting Any air to it for about a week. When it's closed off like this I'm literally only breathing with approx 12% lung function and with no supplemental oxygen, which I really need (the test to determine whether I need oxygen was done 3 days post bronch, when my airways were open, so they won't order oxygen for home). The scary part is that this complete closure is happening much more frequent and is becoming much harder to open back up. I still do not have an answer from UCSF whether or not they'll be willing to retransplant me.
I'm spending the day in bed sleeping or watching Netflix because my lungs hurt too much for me to move around. 

A very long day, but my fight is still Stronger than this disease.

I started this as being just a Facebook post, but once I figured how long it was getting I figured I'd just include everything and post the same update on FB and my blog. I'm sorry for the multiple posts to those of you who follow me on here and Facebook.

As promised, here is a brief update on today's adventures at UCSF.

As we were told I had an appointment with one of the pulmonary tx doctors, I was also told that if time permits I may be able to see a few others from the lung transplant team. Admittedly, I know about 80% of the staff already from my past visits with my first transplant, but there are a few unfamiliar faces that I was hopeful to meet and put a name to. 

The single new face I got to meet today was one of their new social workers. She, Benito, and I had a nice long visit and got a lot of worries and questions out of the way. Yay! I am so extremely happy with this social worker... Unlike the previous ones I'd had, she really wants to be available and help however she possibly can. She may even be able to figure out how Benito and I can finally get married without me losing my insurance! SCORE!!! More on that at another time... The real reason for this update was to tell you about the retransplant option, not talk weddings (but still... YAY!!!). 

So the transplant pulmonologist sat with us for quite a while going over every possible health concern she or we had. Our biggest concern is whether or not I have enough room on my native airway to attach a new lung because my stenosis issue has made its way that high up my airway. So I go back in 2 weeks for a bronch and they'll take a look at that airway and see if there is enough space. If there is then we can move forward with the prep and testing to see if I'm eligible for retransplant. If there's not enough room, well, I don't know yet. I'm just hoping and praying that my stenosis hasn't left me with nothing to work with.

If all goes well after that, then our next step is to figure out what's going on with my liver. My dr wanting to ignore the problem is going to have to go ahead and order diagnostic testing, whether he thinks it's necessary or not. I Have to have a diagnosis before they'll consider me for retransplant.

Lastly, we need to figure out a better way of treating my acid reflux. For those that don't already know, acid reflux can lead to chronic rejection. It can be aspirated without even knowing it's happening and burn/damage lung tissue, causing chronic rejection to flare up. I'm almost convinced that this is the reason why I'm rejecting right now. While in the hospital they don't carry my usual acid blockers and I had some of the worst heartburn of my life during the past two hospital stays. It got to the point where I was having Benito sneak in my two acid blockers from home because they wouldn't let me give them my own bottles to dose me with and I was miserable.

So once these three obstacles are behind me I can begin the testing for the retransplant evaluation. Like I said, I have a Lot of stuff that needs to be done and out of the way before I'll know whether or not UCSF thinks I'm a good candidate for retransplant and will list me.

I'll try to keep you all up to date on any new developments, but until you see me post in all caps that I'm on the list, know I'm still working Hard to get to that point.

I appreciate the outpouring love and prayers I've been getting the past few weeks, especially today! Please keep it coming. Like I always say- You give me strength through the love you share with me. I may be on the verge of fighting for my life again, but I don't doubt for an instant that I am Blessed to still be here to share this incredible, crazy, Amazing journey of my life with all of you. I am So grateful for every single one of you. Love and Hugs!!

The end of a rough year leads to a rough new year.

I've had a pretty tough few months, but the last few weeks have been a doozie. I've been complaining to my doctors via phone about having more and more trouble breathing, fevers rising, and my pain getting worse. Finally, just before my birthday I got a phone call back saying that they'd like to admit me because they thought I may have a virus (although my symptoms had been going strong for Weeks). I told them that I had a 4 night "getaway" planned for my birthday - New Years that I really needed to go to, even though I wasn't planning on anything more than laying around and just spending some time away from home with Benito. So we cancelled our plans because my doctor was supposed to call me on Saturday to discuss admit, but after not hearing from him, we decided to check and see if our reservation had been rebooked by someone else yet. Luckily the place was still open so we went ahead and spent 3 nights in the East Bay. We literally spent the entire time watching rented DVDs, sleeping, or just laying around talking, but we did it together and had a great time away from home for those few days.
Once we got home I called my clinic to see if I can go ahead and be admitted, but my nurse was out of the office until the following Monday (Jan 6th). So we spent the next few days preparing for an admit and Benito getting a lot of work done at his office.
I wasn't allowed to say anything before, but Benito was offered a management position at his office and his first day at Operations Manager of the entire production part of the company was Jan 2nd! I'm just so darned proud of him for all the heard work he's put in over the past 5 years. He's such a hard worker and just LOVES this company, so when the previous VP of the company decided to buy out the company from the family who started it 20+ years ago, he knew he wanted Benito to run production. It's all so very exciting now that we can actually tell people! We've had to keep this secret for nearly 3 months before the big announcement on Jan 2nd!

So I called my nurse on Monday morning (Jan 6th) saying that I really need to be admitted. CF clinic is on Mondays, so I knew it would be a while before I would get a call back about getting a bed. I spent the day making sure I had my ducks in a row... Bills paid, laundry done, library books renewed or ready to return, etc. Toward the late afternoon I took a quick shower and by the time I got toweled off I was Bright RED, shaking, and super weak. I pulled out all my equipment and took my vitals... My temp, blood pressure, and heart rate were high, while my oxygen saturation was at an all time low since transplant. I called my nurse again and left a voicemail letting her know the update. Within a few hours my dr called me saying that the hospital was full because of how hard the flu has hit everyone this year, but he's trying to get me a bed. If a bed didn't open up then he'd look into getting me in to a different hospital until I could be transferred. The ideal ward I prefer to be put at my hospital is the transplant ward... The nursing staff is extremely kind and so knowledgeable about all of my medications and needs while I'm in the hospital.

Fortunately by 6:30pm I received a call saying they had a bed for me at my hospital, unfortunately for me, that only open bed was on the orthopedic floor, not the transplant or even the CF floor. So Benito accessed my port at home before we left because the nurses on the Ortho floor are so unfamiliar with central lines and I don't like having to coach someone through something while I'm feeling so awful to begin with.

So we got to the hospital at around 8pm and ended up getting put on supplemental oxygen right away because my oxygen saturation was 91% on room air. It took a long time before my nurse was allowed to do anything beyond that because nobody knew who my doctor/hospitalist was and who to ask. So no orders got written for quite a few hours. Eventually they drew blood, got an xray, started me on IV Meropenem, and respiratory treatments every 4 hours.

What we know now:

My xray showed that my lower right lobe of my lung is shrunken up, but they can not hear ANY air moving throughout my entire right lung. We believe that the stenosis has completely closed off and has caused my lung to "collapse". Because donor lungs have a strong tendency to "glue" themselves to the recipient's chest wall with scar tissue, my lungs won't look like they've collapsed via an xray. They can't physically collapse while they're stuck to the chest wall, but it's giving me so much more pain than my usual lung pain. Attempting to take a deep breath is Very painful and I get a feeling not unlike trying to inhale through an empty balloon. Not comfortable.

Once my oxygen sats came up enough to where I could be taken off the supplemental oxygen there was no longer anything they were doing in the hospital that I couldn't be doing at home. I needed to come home. Most of the nurses on the orthopedic floor were very kind, but every night and day was a struggle with my medications. It got to the point that they were just having me skip the meds that they weren't sure of, so I ended up having some Awful acid reflux every night, which for a transplant recipient, it could mean rejection (yes, reflux CAN contribute to rejecting lungs). I had Benito sneak in my own acid blocker med that I normally take at home (by prescription, so no funny stuff) so I could get my reflux a little more under control.

So they got one last xray done and set me up with IV Meropenem to continue for another 2 weeks at home. I was told that my CF dr was going to speak to my other pulmonologist that does my bronchs and get a bronch scheduled for early the next week (this week). So on Monday I called my CF nurse to check in to see how that bronch was coming along... My dr did talk to my other doc, but he didn't tell him that I needed a bronch asap. :-/

So my nurse got in touch with my bronch pulmonologist and I got a call today saying that the soonest I could get scheduled was next Tuesday the 21st. I broke down and cried... There's no way I could wait another week in this amount of pain. She said my doctor is going out of town tomorrow and won't be back until Tuesday, but she'd call him and see if there was Anything we can work out. A little while later she said my doctor agreed to get me in for an early bronch at 8am tomorrow morning. Basically, his flight leaves at 10am and he's swinging by the hospital on his way to the airport so he can help me out before he leaves town. I can't tell you how much of a relief that is! I can't wait to be able to feel again what pain less than an 8 or 9 out of 10 feels like!

For over a month now I've been experiencing some strange new symptoms and they're continuing to get worse. I've had a Lot of itchiness that Benadryl doesn't help, ammonia smelling sweat (yes, very Gross!!), pain in my right upper abdomen, and some others that are a little too personal to share with you all ;-)

So I got blood work done and found out that my liver enzymes have been rising for a few months. It took a LONG time for me to get my doctor to agree to do any further testing, but he agreed to an abdominal ultrasound and a bunch of other blood tests. Everything is coming back negative, so we don't know what's causing my liver to act up, but during this past hospital stay I asked to be referred to a GI specialist so they can help me figure out what's going on (because my CF doctor told me he didn't want to "dwell" on it any longer). It's hard to just ignore all that stomach pain and constantly paranoid that you smell like ammonia... I'm grateful to be seeing someone soon who is familiar with liver issues and won't just ignore me.

I'm totally worn out, but I will try to get on here soon to tell you about what we've been discussing regarding retransplant. Basically the docs and I are worried that one of these bronchs with laser could go from routine, to life threatening because my doc can't see what he's burning once it all goes black with char and he could very easily hit an artery... Scary stuff, but something to think about.

I do hope you all are having a good year so far.

Until next time...

Brachytherapy- Take 2

Hello friends!

It has been about 6 months since I've posted an update, but there hasn't been a whole lot to share in that time.

I've gotten into a somewhat routine of going every 4 weeks to be bronched and having my right main bronchi opened. Due to an overgrowth of scar tissue my right main bronchi gets so small that my doctor can barely thread a tiny wire through it, so you can imagine how difficult it is to breathe through. When it gets that small my dr has to basically guess where the center is and makes a small slit so he can get the bronchoscope through the hole and then balloons it open as much as possible. Most times he'll then use a laser to burn away the scar tissue, which leaves me feeling pretty awful for about a week, then able to really BREATHE for a good two weeks before it begins to tighten back up again. It's a pretty frustrating roller coaster, but it's the only thing we've found that "works". We've tried putting in stents to hold the airway open, but because of the shape of the stenosis it doesn't want to stay put. Having a stent able to move from where it's intended to be can cause a whole lot of unwanted problems, including an increase of scar tissue and choking if it lodged itself sideways. So we've given up on trying to make a stent work. We tried freezing, but it actually made my airways even more inflamed, so we never tried that again. About 18 months ago my bronch / end stage pulmonology doctor got in touch with the radiation oncology team and we tried a brachytherapy treatment. Basically they did a 3 full minute blast of strong radiation directly on the stenosis hoping that the radiation would break down the scar tissue w/out hurting too much of my healthy tissue. It was a tough recovery, but I felt like it gave me a little more time before needing another bronch. My pulm doc wasn't convinced that it worked well enough to try it again, given that it's such a heavy dose of radiation to one specific spot, which increases my risk of lung cancer dramatically. So we never entertained that idea of brachytherapy again. In the last 18 months the time between my need for bronchs have gradually decreased from every 8, to 6, to now needing a bronch no later than every 4 weeks. By week 3 I'm usually struggling pretty bad, so this window of feeling "good" is shrinking.
On Friday I had an appointment with my bronch doctor. I wanted him to see me at my "best" because he only sees me awake for a few minutes prior to each bronch and I'm never feeling well at those times. I wanted him to see how "good" I can feel, and to discuss options to widen the gap between bronchs. After going back and forth about our lack of options, I brought up brachytherapy up again. Because we have zero other options at this point, he's willing to give brachy ONE more try, but he's not willing to do more than that because the risk of cancer I'd be bringing to myself. So he's going to get in touch with the radiation oncology team again and set up a two part treatment this time around. He wants to bronch me one day, then the following day do the first brachytherapy treatment, then no more than 7 days after that, a repeat brachytherapy treatment. He's hoping that a two part treatment will have better results and will continue to break down the scar tissue for a longer period of time. This is our Last ditch effort before we have to agree that the bronchs are our ongoing future of treating the stenosis. If you're a praying person, I'd really appreciate all the prayers that this method works so I don't have to continue this frustrating and scary roller coaster. I've begun having anxiety attacks when my airway is super narrow and I feel like I can't ever get enough air. Sleeping on 5-6 pillows then waking up with an anxiety attack is not my idea of a good night's rest, so I'm really counting on this brachytherapy helping me.

About a month ago, one week after a bronch I was feeling the BEST I have felt in as long as I can remember. I felt like my airway was completely open and I got to feel for the first time what 34% lung function feels like, which isn't all that bad! I have been feeling like I've been suffocating for a few years, but I realized last month that that suffocating feeling is mostly because of the stenosis, not my low lung function. I had NO idea I could still breathe that well to be honest. I thought I lost that feeling forever, but it just took a really good bronch to open things up well enough for me to fully breathe to my capacity. I took full advantage of those couple of weeks of feeling great and exercised on our elliptical every day, painted our dining room and kitchen, and got a lot of small jobs done around the house that have been put off since moving in a year ago. I had high hopes of feeling that great again after this last bronch I had about 10 days ago, but due to a sinus infection and feeling overall pretty blah, I haven't felt too hot this time around. I'm on a 10 day course of an oral antibiotic for the sinus infection, so I'm hoping that the fevers, headaches, and overall body aches go away soon enough so I can have a few days of feeling really good before my airway gets too tight again.

Benito turned 30 at the end of August, so we rented a car and drove to San Diego where one of this friends and his wife now live. They were wonderful hosts and we had such a blast during the 4 days we were there. We saw a Padres/Giants baseball game, saw a movie at the ritziest theater I have EVER seen let alone been in, enjoyed a beautiful sunset in La Jolla, and got to be tourists with some of the best tour guides! It was a trip we'll never forget and we're both hopeful to go visit again sometime soon. On our long drive home, we were lucky enough to meet up with a very long time friend of mine that I met on a CF forum about 10 years ago, but had yet to meet in person. The three of us had lunch and a very nice visit together. Mike is just as awesome in real life as he is over the internet.

That's it for now. I hope you're all doing well and enjoying the beautiful colors of fall! I just LOVE this time of year :-)

Michael Adams and I - meeting for the first time after 10 years!

Life happens in between blog posts

Howdy! I can just imagine your eyes have bugged out in complete SHOCK to see that I have posted an update to my blog. Please blink a few times and continue reading...

6 months since my last update... Wow!

First off, I have to tell the world that I am in LOVE with our new home! I'm still in shock every time I wake up in this beautiful home. Being a homeowner had always been a dream, but I had no idea I would love it this much! The flowers that I planted in January are now starting to bloom and have made our home even more beautiful. I walk around outside just about every day to admire the blooming flowers that I planted or that the previous owner had planted and are just now showing their beauty. Once we got the essential living things unpacked, organized, and decorated, we kind of stopped cold with painting and doing all the things we had planned on doing once we moved in. It's just so nice to be able to relax in your own home and not be rushed to paint the spare bathroom, ya know?!? Another huge plus to our move is that we have made some amazing friends that just so happen to be neighbors. These two couples are so caring and generous that there have been a few times where after a bronch or when I'm just not feeling well, these couples would bring over a home cooked dinner for Benito and I. At one dinner, the couple went as far as bringing paper plates and plastic cups so we wouldn't even have to worry about clean-up afterward. We feel so blessed to have found such amazing people to call friends.

The last time I updated I had talked to my doctor about planning on getting things in order for when the need to be re-listed for transplant came to a head. Since then I have been on a roller coaster of whether or not the drop in lung function is due to chronic rejection coming back or my stenosis causing more problems than usual. Just this last Monday I had CF clinic and blew a 36% fev1 with my PFTs! So we came to the exciting conclusion that my 10% drop in lung function is purely because the stenosis gets SO closed up (literally pin sized). What a relief it was to see my PFTs back up to my previous baseline! So now as far as retransplant goes, I'm in no rush to get re-evaluated and put back on the list for a second transplant. We still need to be hyper aware of any changes, so if/when the times comes where I need to be listed quickly, I'll be ready. In the meantime, the ongoing issues with the stenosis is my main struggle. I have already had 4 bronchs this year to open up my airway and it seems that each time I go in to have it opened, it's closed off even more than the time before. I wish there was a long term solution to the problem, but for now the only thing we can do is continue to go have the airway lasered open once I feel like it's gotten too narrow.

Now, on to the latest news that I wanted to share:

A week ago I had my annual dermatology appointment where they check me head to toe for anything suspicious that could potentially turn into skin cancer. Being on the immunosuppressive drugs for 8 1/2 years now, plus the 15 months of photopheresis treatments I did has made me much more prone to cancers, especially skin cancers, so I have to be very aware of any changes in my skin. I have had about 6 or 7 pre-cancerous spots frozen off, but nothing serious has ever come up. During the visit last week I told the dermatologist that the spot they have previously frozen 3 times just comes right back every time, so she took a biopsy of it just to be on the safe side. Five days after the biopsy I got a call from the dermatologist herself saying that the biopsy tested positive for squamous cell carcinoma, the second most common forms of skin cancer. She gave me two choices, I could: A) have the skin surrounding the carcinoma cut away, then stitched up to close the large opening. This will most likely cause a large scar and because of the stitches, has a larger potential of infection. Or, I could: B) do a scrape and burn procedure where they would scrape scrape scrape, then burn the site with a 1,200* cauterizing tool, and repeat these steps over and over with the last round of scrapes being sent to pathology to make sure it was cancer cell free. I couldn't care less about the scar, but the increased risk of infection with stitches made me choose the scrape and burn method. I have the removal scheduled for next Friday, April 19th. My father and his wife had a little girl that was diagnosed with cancer at 1 year and later passed away at 5 years old, so the big C word is terrifying in my family. I made sure to try and break the news of this skin cancer gently to my family before making it public, so I could explain the best I could about the risks and treatments before they could think the worse. I'm very optimistic that I won't have to worry about this specific spot again after the removal next week. I will however be much more aware of any spots that come up and will insist that my dermatologist biopsy anything that continues to come back after two freezings.

Lastly, the most exciting news to share is that since my last update I celebrated a very important birthday... I turned 30!! Growing up with this nasty disease and watching so many of my friends pass away because of it, I never imagined I would still be here to see 30 years old, but here I am!! Happier now than I ever have been, too! :)
Here's to another 30 years!!!

Here are a few photos of the skin cancer on my arm. Everyone needs to be super diligent with their sunscreen when they are out and about! The majority of my spots I get are on my forearms and hands and I can only imagine it's because that's where I'm most exposed while driving. I'm going to be much better about applying sunscreen before driving!

Picture 1) pre-biopsy

 

 Picture 2) five days post biopsy. My dermatologist thinks that it's healing so much slower than even my norm because the cancer cells won't allow it. Hopefully once they remove ALL the cancer cells it will heal quickly.

Before I end this update, I want to add that I'm very excited to be leading a team at the Great Strides walk for the third time on May 18th. If you're interested in joining my team, or donating toward my walk then PLEASE visit my Great Strides page! You can find my page by clicking on the Great Strides banner link on the upper right hand side of my blog. Thank you!!