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. 

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!!

Two and a half weeks later...

Hola!

So I know I said I would update after the brachytherapy, but I was expecting to be doing this sooner than 2 and a half weeks after the fact... Sorry about that! I'm sure you can tell that I'm awful about getting on here and updating, so I'm sure you are not too surprised to see that I wasn't on here updating as soon as the treatment ended.

Well, the brachytherapy procedure itself went really well. I was there for about 7 hours, but most of that time was recovery time and visiting with the EXCELLENT nursing staff in between CT scans and threading attempts. They ended up using a "good sized" amount of radiation in a total of 3 whole minutes. Kind of a blast of radiation in a short amount of time. The idea is that it would be enough to not need to have to go back and do it again at a later date... Hopefully all the stenosis will be radiated and break down. So far so good! Don't get me wrong, I had a very rough 10 days or so after the brachytherapy. I don't know if it was coincidental that I caught some awful stomach bug the evening of the treatment, or my body had some strange reaction to the radiation, but I spent nearly 12 hours being horribly nauseous and vomiting what seemed like continuously. I was discharged from the recovery with instructions to take my oral pain med and my anti-nausea med (Phenergan) once I got home; yeah, that didn't happen. I couldn't keep anything down, so 3 hours of continuous vomiting later Benito drove me back to the hospital so I can be seen in the ER in hopes of them starting an IV and giving me something through that to stop the vomiting and control my pain. Everyone knows that being post transplant you expect to be high priority when visiting the ER and should be taken straight back to avoid all the sickies. We post transplantees avoid the ER at all costs, so it's a big deal if we are seen in one. Well, after 4 hours sitting in the ER waiting room being told "you're next" and watching my blood pressure rise by the hour (surely because of my pain increasing w/out being treated) we decided to just go home and hope that the nausea lets up enough to where I can deal with it myself. It ended up letting up enough later to where I could hold down the Phenergan and later on I attempted and succeeded to hold down my pain medicine. It's really too bad it got that bad though and without the help I needed. (It is NOT fun to vomit continuously for almost 12 hours! Talk about a migraine!!)

So here I am about 2 and a half weeks later and I THINK so far my airway is staying pretty much open. I still get wheezy, but my breathing is overall staying okay. When I go outside in the cold air I can still feel my airway tightening, but I started taking an allergy medication that may help with the asthma as well, so fingers crossed that helps that problem.

I had a full set of PFTs done on Wednesday of last week and my fev1 (lung capacity) is up to 35%, with my FEF 25-75 (small airways) at 10%!! That's about a 2% increase, so yay!! My PFTs have honestly not been this high in over a year, so this is really good news.

On Thursday of last week I had a Dermatology skin screening. Since I'm on immunosuppressant’s every day of my life and now that I'm doing the Photopheresis treatments my risk for skin cancers are much higher than a "normie" (non-transplantees). I've had a few suspicious spots on my hands and arm that a Dr froze with a liquid nitrogen gun a few months ago, but it was definitely time to get a full body scan done by a "professional". They went ahead and froze a spot on top of my right hand that has been problematic for a while, a spot on my chest that showed up about 3 months ago, and a spot on my right cheek (sideburn area if I were a man) that just popped up about 2 weeks ago and has since doubled in size. They called these Pre-cancerous Squamous Cell spots. If the spots come back I'm supposed to let them know and we'll try freezing AGAIN. Otherwise they saw a dark freckle on the bottom of my left foot (in the arch area, so luckily I'm not putting pressure on it) and decided to go ahead and biopsy and remove it right there in clinic, no warning whatsoever. To say I was nervous would be an understatement. As it turned out the only part that hurt was the lidocaine shot they gave me to numb the area first. Then they scraped away the freckle and put it into a little specimen cup and followed up by cauterizing the hole in my foot. I should get the results to the biopsy within the next week or so. Attached is the rad photo of what it looked like the next day when we put a fresh band aid on it. Cool, huh? ;)

On a side note, the excellent nurse who was in charge of my care at the Radiation Oncology dept during my brachytherapy treatment called me two days after the treatment to follow up and see how I was doing. He said that he saw that I had been waiting since September for a referral to go through and have an appointment made for the pain management clinic. So after about 6 months waiting (and 3 referrals put in by 2 different doctors) this nurse who barely knew me was able to call on my behalf and get them to set me up with an appointment! I got a phone call the following day from the pain management clinic ready to set up an appointment! The soonest they can get me in is mid-April, but I'm still so happy to have an appointment! Jean (my rad-onc nurse) is going to get a giant Thank You card when I go back in for a follow-up appointment! He did the impossible for me and I'm so extremely grateful. I'm so so SO tired of being on pain medication that only works about half the time; I'm really hoping that these doctors can help come up with an idea on how to treat my chronic pain w/out medication. My goal is to eventually wean off of the medication and NOT need it, so wish me luck! I'm kinda interested in accupuncture, so we'll see what these drs have to say about it.

That's about it! I'll update when / if anything new comes up!


Meanwhile, be nice and don't do anything I would do! :D

Friends are Forever

Hi friends!

It's been about 2 months since I've posted my hopeful news about how great my lungs are looking since starting the photopheresis treatments. Now, 2 months later I feel about the same as I did in the last post. The relief I got from opening up my airway via bronch and balloon was short lived. I've been struggling with my breathing feeling really tight again and went to clinic this past Monday (9/12) and spoke to my doctor about going back in and re-opening it. Dr. Morrissey said he has been doing some thinking and discussing with other pulmonary doctors there at UCD about how he could give me longer relief in between these dilations. He suggested freezing away (as you would a wart) one half side of the scar tissue which is the narrowing in the airway and going back in a few weeks later and seeing if it's staying open. If it seems like it's working, he'll go ahead and freeze the other half, but he doesn't want to do the whole thing in case it causes a lot of inflammation and closes off the airway completely. So, I have an appointment for 1pm on Friday for a bronch and to see if they can cryo (freeze) the airway. My doctor did say however that if the airway looks as open as it was after they dilated it back in July (which I highly doubt it will), he will skip the procedure and wait until it's necessary again.

I have a set of photopheresis treatments coming up tomorrow and Wednesday mornings and am looking forward to having lunch with a close friend from the hospital on one of those days. Her name is Kathy Lorenzato and she runs the music therapy program at UC Davis Med Center. I had the pleasure of meeting Kathy on one of my very first admissions to the hospital back when I was 5 years old (23 years ago, wow!). Since then, I've stayed very close to Kathy and have always looked up to her and her big heart. Recently I was emailed by a reporter from the Davis Enterprise about an article they were wanting to write about Kathy, so I got to speak to this reporter about what Kathy has done for me and what a great person she is. The article ran in the Davis Enterprise yesterday and I'm very happy with how it turned out. The link to that article is:

http://www.davisenterprise.com/home-page/featured-stories/music-therapist-brings-comfort-and-joy-to-children-who-need-it-most/

(You may need to copy and paste the above link into your browser in order to go to the site.)

August was a tough month. I lost my closest, longest known CF friend on August 22nd. Kristina was 3 years older than me and had always been a role model to me. We met at the hospital when we were young and instantly became friends. Being a few years older than me, Kristina always went through the scary CF stuff before me and was there to hold my hand when I went through it myself. She had her double lung transplant at the same hospital I had mine, only 1 year earlier. At the time Kristina had her transplant is when I was told I would need to decide whether or not I was willing to be put on the transplant list myself. So Kristina was there to answer all my questions, relieve my fears, and remind me that I can do this too. Her strength gave me strength, her courage gave me courage, and her laugh would without a doubt make me laugh too. I had a very hard time with the passing of Kristina because I no longer have her to hold my hand anymore. I feel so much more alone now, but I'm sure she's up in heaven still rooting me on. I wasn't emotionally "okay" to make it to Kristina's rosary / viewing, but I (along with Benito and my mom) went to the Funeral and I'm very glad I did. Her service was absolutely beautiful, just like her. Her mother sang Amazing Grace and although I was in tears listening, I had to smile because I know Kristina was there in spirit smiling at how beautifully her mom sang the song. At the reception after the service I had the pleasure of finally meeting Kristina's father and her best friend Shannon, whom she used to speak of Every time I talked to her. Just before the reception was over I got the courage up and stood and spoke about Kristina as I knew her. About how strong I know she was and what a wonderful friend she was to me. I've lost more friends to this horrible disease than I care to count, but the loss of Kristina has left me questioning my own fate. You see, Kristina and I were the last two [living] CFers out of our group who grew up together, roaming the halls of the adolescent ward at UCD. Losing Kristina has made me even more determined to fight this disease and not let it win though.

Just a week after Kristina passed away was Benito's birthday and knowing how much we needed some cheering up, I surprised Benito with a trip to Santa Cruz and Monterey. I wanted to keep the destination of the trip a secret, all he knew was we would be gone 1 night and to pack for weather a little cooler than he's used to here. He was determined to do the driving, saying it would be fun to drive somewhere that he doesn't know where he's going, so I let him drive, giving him step by step directions until we pulled up at the Santa Cruz Beach Boardwalk. We had a great time in Santa Cruz. The day after the boardwalk, we drove the 45 extra minutes to Monterey; somewhere I've always wanted to go (after reading all about Cannery Row in Steinbeck's Cannery Row and Sweet Thursday books). We didn't have much time to spend in Monterey because we had such a long drive home (Roxy was staying with grandma, so we had to pick her up on the way home, which added even more time), but we walked around Cannery Row and found a great place where Benito did some wine tasting. We plan on going back to Monterey when we can spend more time seeing everything because what we saw, we loved!

So that's about it. I'll write again when I have more to share.
Thanks to everyone who checks in on me from time to time!

Love & Hugs,

Leah

P.S.
In only 6 days I'll be celebrating my 7th Breathday (transplant anniversary)! Crazy how fast time flies!! :)

Kristina and I back before either of us had our transplants, May 2002.

Benito and I got our portrait done at the Beach Boardwalk, does it look like us? :D

Pictures!

Finally! Here are the pictures I promised of the photopheresis treatment. I tried to add captions to all the photos so you know what you're looking at. I just hope these pictures are able to help someone else feel more comfortable about starting photopheresis treatments.

https://picasaweb.google.com/leah92504/Photopheresis#

You may need to copy and paste the above link into your browser in order to view.