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!!

Just a real quick update to let you all know that I got the biopsy results back from the dermatologist. The freckle they removed was benign and nothing to worry about! It had a few days where it was getting infected, but it's starting to look a lot better and doesn't hurt so much to put pressure on it. We're huge SilverSol fans in this house... It's better than Neosporin!

I'll post again when something new and/or exciting happens!

Take care :)

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

Ray Lamontagne soothes the soul

It's been over a month since I've been told there is nothing left to do but wait to get sicker. A lot has happened since I've last updated, so I'll try to cover everything.

I'm sitting in my bed with my laptop listening to Ray Lamontagne; letting his raspy voice and beautiful lyrics soothe my soul while I recall some of the not so fun things that have happened so far this year.

My last post went over what happened at my last CF clinic appointment. Just a quick recap: After Stanford declined taking me on as a patient, I was relying on my CF doctors to help stop the decline in lung function. Why not have my transplant center (UCSF) take care of me and my possible chronic rejection you ask? Well, my transplant center is one of what seems like a growing group of transplant centers that are very apathetic toward their patients (I should include there are a Few people I've talked to who have not had this same experience with UCSF) and do not have a protocol toward treating chronic rejection. My CF doctors have exhausted all their resources and are not familiar enough with the transplant field to know what to do for the possible chronic rejection(CR). So, I've been left with absolutely no idea what I'm to do about my increasingly fast decline of lung function. In the last month alone Benito and I both can tell a huge difference in my breathing. My last fev1 (lung function lingo) was at 33% prediction, which is at the point where people would be listed for transplant. I was at 17% fev1 at the point of my first transplant, but since the damage CF did to my lungs was so gradual, I was able to adapt to the low lung function. The decline I have now seems to be happening to fast that I'm not able to adapt and it feels like it takes forever to be able to catch my breath after walking just a few feet. Imagine trying to inhale through an empty balloon... That's exactly how my chest feels when I'm out of breath. My sound of my breathing is getting to be so noisy that it keeps me up at night (I sleep with a fan going even if it's freezing, just to try and cover the noise of my breathing).

So after the last clinic appointment at my CF center I had yet another balloon dilatation done on my right narrow airway and after that procedure was over, my doctor came to the recovery room and talked with Benito and I about where we need to go from here. I had brought a printed out copy of a conversation between a very good CF/Transplant friend of mine who goes to Duke hospital. In these conversations she was helping me with what treatments she knew of that hers and others have used to treat chronic rejection. I gave this copy to my CF doctor to read over before my procedure that day and by the time I saw him after the procedure he had read it over a few times and had spent a half hour speaking with the pulmonologist there at UCD that worked my up for my transplant evaluation six years ago. This pulmonologist used to work with the lung transplant team at UCD years ago when there actually was one, so he knows a little more about transplants than my CF doctor. Basically all they came up with, was that I needed a transplant center to administer these treatments because they have never prescribed them and didn't feel comfortable having me be their guinea pig. So, this left me to first go back to UCSF and see what they could do for me, then if I get nowhere with them, I can look into possibly going to UCLA, but it's eight hours from where we live and it would be both physically and financially draining to have to relocate.

Fast forward to February 10-11th. Benito and I went to San Francisco to visit my transplant doctor for the first time in a year hoping they will give us some hope, although I wasn't expecting much. I had my PFT's (which showed my fev1 down to 33%), CT scan and transplant clinic on the 10th and bronch with biopsies the following morning. My doctor basically told me he wasn't convinced what I'm going through is chronic rejection. He's STILL convinced the stenosis I have in my right main bronchi is causing all this decline in lung function. I was willing to believe him for a while, but after 2 years of my lung function getting worse and worse by the month even though I was getting balloon dilatation's to open the airway every month. So I brought up IF what's going on is chronic rejection, what does he usually do for his patients in CR? He basically said that after making sure the patient is on Azithromycin (which I've been on since my transplant), he doesn't do anything. He believes there is no proven treatment for CR. In fact after I brought up a list of treatments that I know other hospitals are using for their patients in CR, he denied that more than one center is using one of the treatments (which I know is untrue), and said the other treatments don't work. *Huge sigh* At that point he said "there is one thing you haven't mentioned yet" and I knew what he meant... Re-transplant. He said he would re-transplant me in a heartbeat because I'm "such a great patient". I can't tell you how upset that made me. I feel like this hospital treats me like a customer and not a patient they need to care for. They are so eager to re-transplant me (where they'll make $200k just for the surgery alone), rather than do whatever it takes to try and treat what's wrong now. It's very unnerving to not have faith that your doctor is doing what's best for you.

The bronch the following morning was an absolute nightmare. I have chronic side pain, which is assumed to associated with a chest tube and nerve problems. This pain gets a lot worse when I deep breathe and it's forced me to take pain medication every day in order to just get out of bed every day. Well the fellow DR that was in charge of my bronch for some reason was so afraid to over medicate me that I was awake during the bronch, given only a child's dose of Versed (medication which is supposed to help you relax). So during the bronch, the guy who calls himself a doctor was only able to biopsy my left lung because my right airway wasn't open enough to get the tube down through it. Once I got to the recovery area I was in 12 out of 10 pain in my side and stayed that way for 3 HOURS because the doctor was scared to give me any pain medication because somewhere in his tiny brain he thought I might get over medicated. Mind you, my O2 sats were 97%, my heart rate was around 100bpm and my blood pressure was around 160/80, which is too high, a sign of a person in pain and not in danger of being over medicated. After about 30min of crying, the nurse closed my curtain around me so he and everyone else could try and ignore me. 2 1/2hrs later while still in 12 out of 10 pain and still crying, the nurse came in and said "good news! the doctor is allowing you to take half of one of your pain pills!" I was like "You've got to be kidding me! Why can't I take a whole pill? I take a whole pill at home every 8 hours!" The nurse took my bottle and cut one of the pills in half and handed me the half along with a glass of water. I asked him for the bottle and dumped his half back into it and took a full pill. That nurse flipped out! He opened my curtain and told me I'll never be able to take my own medication again (Fine with me! The hospital shouldn't force their patients to treat their own pain! THEY shouldn't allow their patients to be in excruciating pain for 3 hours without trying to help them!). He then demanded that I give him back my pill bottle. Ummm HELL NO! I told him that once he brought my fiance back into the room I'll give the bottle to him. He didn't need to be worried that I'd take more. I've been on this medication for years and I know not to over medicate myself. So about 45min later when my pain was beginning to get better, the "doctor" came in and said that since I took a whole pill against medical advice, he was going to put me in the ER to be watched and made sure I didn't desat and stop breathing because of my excess medication dosing. WHAT?! First of all, it wasn't excess. I had less medication so far that day than I usually do on a daily basis. Second, no doctor who knows anything about transplant recipients would send their patients to the ER where there are God knows what kinds of germs floating around and risk getting me sick. A few minutes after he left, a different nurse started his shift and found out what had been going on and called one of my REAL doctors. Within a half hour my tx doctor had come in and wrote discharge papers saying to hurry up and go before that other "doctor" followed through with his crazy ER plan. It's sad, but events like these are not all that uncommon for me at UCSF. Most of the nursing staff is great, but I can't say the same for their doctors.

Two weeks later I was back to UCSF because my tx doctor wanted me to come in and go under general anesthesia while they go down to open up my airway and take a better look around. Thankfully once I got to the recovery room that time, the doctors and nurses gave me adequate pain control and were very kind. My main tx doctor came in to talk to Benito and I after the dilation with a huge grin on his face saying "I think we did a great job opening that airway". Unfortunately the week following that procedure my breathing got really bad and for the first time I think even scared Benito. It's been almost 2 weeks since the dilation and I'm beginning to get back to my baseline. I have CF clinic on Monday and I'm not sure what to expect.

Onto better news, Benito's sister will be getting married this next Sat 3/12 and I'm her maid of honor! Benito and I haven't really done any major wedding planning for us yet. I want to enjoy my wedding and as of now, my health won't let me. So, I'm hoping to start wedding plans the day after I am feeling better! :)

Biopsy

Had my biopsy yesterday. 24hrs later and the anesthesia has finally worn off enough for me to post an update. My lungs showed a lot of inflammation and suprising enough, my nerves have re-attached themselves in my donor lungs. Every time they would "snip" a little sample off my lungs, I would wake up in pain. For those of you who don't know, donor lungs usually do not have working nerve endings because of the transplant process and not being able to reattach them. I'm unique though! yay me! This explains why my right side has been hurting so bad the past 2 years, it's probably my stent causing the pain.
Anyway, waiting back on both the biopsys and cultures they took. Due to the inflammation and low lung funtion, my docs say that if an infection doesn't show it's ugly head, then it's most likely chronic rejection I've been dealing with all this time. Pretty scary, but not exactly a surprise. I should get results in a week, so prayers would be wonderful. Thanks friends!