Whoa! August 1st already?!?

I've come out of my cave! I'm so sorry I haven't written an update in 4 months. I hope you can forgive me!

Let's see...

Since I've last posted, I've been keeping pretty busy and have definitely had my ups and downs with my health. On May 19th I got to lead a team at the Sacramento Great Strides walk! This was my second year leading a team, but this year was so much more fun! For starters I found a wonderful shirt printing business locally that donated their services to print team shirts for all of The Iron Lungs (our team name). This was the first time I've ever been able to provide shirts for my team, so it was so awesome to see all the people walking around wearing our team logo. We had 28 walkers this year on The Iron Lungs, 15 more than the last time we walked (2 yrs ago). I can't tell you how much it warms my heart to see my friends and family (and Benito's co-workers) gather for such a worthy cause. We had a goal set at $3,000 overall for the team, but we surpassed our goal and raised a whopping $4,070.00 making this our most successful year yet! I'm so grateful for all those on my team and can't wait to do it all over again next spring! (See above photo of MOST of our team! The family of my dear friend Kristina Love (whom passed away last fall) walked with us and made up shirts of their own saying "Love for Leah"!

On July 6th I did my last photopheresis treatment! The protocol is a total of 30 treatments and as of July 6th I'm free of the strict schedule of getting my blood tanned ;) My doctor had mentioned a few months ago the possibility of doing some sort of maintenance schedule once my 30 treatments were up, but he has yet to set that up. I'm so extremely grateful that he was willing to try photopheresis on me when nobody else was willing to treat my rejection. I'm now in "remission" and am happy to see my lung function stable again. To clarify, because I get asked a lot how much lung function I've gained back, once someone has chronic rejection (and especially for how long it went untreated in my case) the damaged tissue is no longer viable. I will not get that lost lung function back, no matter how hard I tried, but what photopheresis DID do was STOP the progression of damage done to my lungs by my immune system. I'm currently stable at 33% lung function (fev1) and 10% small airway function (fef25-75). I really couldn't ask for more of a response from the photo treatments.

Now, what most of you don't know is that Benito and I have been looking since late winter for our first home. Over the course of about 5 months we've seen countless houses and even put offers on 6, yes 6 homes! 5 of those offers were not accepted because of different reasons. We waited as patiently as we could and the right home finally came along. The 6th offer was accepted and we've since been doing all the inspections, paperwork, etc. to get into our first home! Our close of escrow is only 2 weeks away and we really couldn't be more excited. I've had the pleasure of meeting the seller and am even more confident that this is the right home for us now that I see how absolutely sweet this woman is. She's doing everything she can to make sure this home is ready for us to move into by fixing all the necessary issues before we even have to request them to be fixed. What a huge weight off our shoulders to know the home is in the best possible condition before we move into it!

So, the home is 1,299 square foot. 4 bedrooms, 2 bathrooms. GREAT covered patio in the backyard that is just begging for us to barbeque under! I just can't wait to move in and make this home OURS. I can't wait to give Roxy (our dog) a backyard to play in! The neighborhood is absolutely wonderful and I even had the opportunity to introduce myself to a few neighbors at a yard sale that was going on the same day I was there for an inspection! I can't wait to have neighbors that I can associate with and even borrow a cup of sugar from when needed ;)

My health is so-so. I'm still having problems with my breathing being too tight and wheezy, and my pain level fluctuates dramatically for no reason I can figure out. I'm just trying to ignore as much of the bad days as I can and focus on the good ones. I've had 3 bronchs since my last update. Although I felt like the brachytherapy treatment that my dr did on me in March made me feel better than I had in a very long time, it did not last as long as my dr had hoped, so he's skeptical to try it again just yet. He mentioned wanting to try and remove the existing stent I have in my right main bronchi that has fallen lower than my stenosis, but he and my CF doctors are hesitant to do that because of the risk of blood loss. That stent has been there for about 4-5 years now and scar tissue has grown all around it, so removing it is now quite a dangerous task that would require the operating room, general anesthesia, and a cardio-thoracic surgeon to perform. I'm not so sure removing the stent and replacing it with another will help my breathing, but I'm curious if it would help my pain because my pain is always in the exact same place, which also happens to be where you can feel the stent "buzzing" when I breathe. I don't think that is as coincidental as my doctors want to believe. I'm pretty sure the pain I feel has a lot to do with the stent and am considering having the stent removed purely for that reason. We'll see though... The possibility of having to have an emergency lobectomy because of tearing during removal scares me enough to really think about how necessary it is.

So that's that. Nothing else really new going on. I hope everyone is having a wonderful summer!!

Hugs & stuff :D

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

Eeeek! Has it really been over 5 months since I've posted on here?!? I'm SO sorry if I worried anyone. I'm doing okay... I've definitely had less ups than downs in the past 5 months and it's just made me feel like crawling into a cave and hibernating until everything works out on it's own, but of course I can't do that now, can I? ;)

Only 2 weeks after my last post I lost another very close friend to Cystic Fibrosis. Christen and I met at CF camp back in 1990 and have been pen-pals ever since. A few years ago she switched hospitals and started seeing the same doctor I go to, so when she was admitted into the hospital I was able to come visit and see her again after so many years just communicating via email, facebook, or telephone. I was "lucky" enough to be able to spend a few days with Christen prior to her passing away and had some long talks to her and her mom about Christen being ready to leave this world. Although talking about that with her was very difficult (especially after having said goodbye to Kristina only weeks prior), I'm glad I got to have that time with her and it helped me knowing that she was ready. I wish I could say the passing of my two closest CF friends wasn't completely devistating to me, but it was and I'm still grieving over the loss of two such beautiful and loving friends. It breaks my heart knowing that I will not see their smiling faces again until it's my own time to leave this world (which, for the record, is a very long time from now).

So as you can imagine, my holidays were difficult to get through, but they came and went and I turned another year older just before the New Year. I'm so extremely grateful to everyone who has helped me get to this age that I never expected to see. Life really is precious!! After tomorrow I drop down to every 6 weeks for the photopheresis treatments and I'll be completely finished by July! So far it looks like they are keeping the rejection from progressing any more and I haven't lost any significant lung function since last May!! I'm really so happy I finally found a doctor willing to treat me and not tell me retransplant was my only option. Yes, it's nice to know I can get another transplant later down the road when I really need one, but that time is not now, thank God. Besides the rejection at bay, my health has been really rough the past 5 months. The stenosis (narrowing) in my airway has been giving me a lot of problems so I've gone in twice now to have it lasered to open it up more and although I can feel them lasering the airway (burns and I can actually smell my lungs burning), and I feel pretty awful for a good week afterward, I feel like this is the longest periods of relief I've gotten after any intervention with the airway. I still don't have a long time of feeling able to breathe well, but it's better than not lasering. I was referred to a radiation oncology doctor in December and Benito and I both talked with her about a treatment called Brachytherapy. Aparently one doctor says they've done this treatment previously on at least one patient whom had "somewhat" similar circumstances as I, and another doctor is saying no, they've never done this before, but they are willing to try it (in theory it should work). So the brachytherapy machine was down back in December and they said they expect it to be back up and running late January. I haven't heard back from them about scheduling it, but whenever it gets scheduled I am to be scheduled for a bronch and lasering no more than 5 days prior because they want my airway to be at it's most open during the brachytherapy. Now, here is what brachytherapy is as I understand it: Brachytherapy is a form of topical radiation to a specific area, in my case the stenosis in my right main bronchi. They'll go down in my lung with a tube and figure out exactly how far down said tube needs to be in order to be "sitting" on the stenosis. Once it's placed they'll x-ray and double check it's in the correct place and tape it down to my face so it can not move. After the tube is secure they'll thread a wire which has a radioactive tip on it down the tube and have it come out just at the end of the tube so the radioactive tip will be on the stenosis. They will leave the radioactive wire there for however much time they've calculated is enough time to be beneficial, but not enough to do serious damage then pull the wire and tube back out. The idea is that the radiation will break down the cells of the scar tissue that is my stenosis and after a period of time it will have broken all the scar tissue cells down and I'll have an open airway again. The tough part is knowing how much is enough, but not too much to cause the good cells that are my actual airway to break down. Oh, and I have a much higher risk of lung cancer after 10yrs they said if I do this treatment. So, it's something to think about, and it does sound kind of desperate, but I AM desperate. I am so tired of feeling like I'm ready for another transplant when I still have 15% more lung function than I did at the time of my first transplant. This stenosis at it's worse (which is like 90% of the time) feels like an extremely SEVERE asthma attack in which medications do not help. So, I ask you, wouldn't you be desperate too?

I have day 2 of my photo treatments for this month tomorrow and I need to get to bed. They still take a lot out of me and I feel like I could sleep for a few days after each set of treatments. Again, I'm very sorry I took so long to get on here and update you all. I hope you can forgive me... It's just been a very hard year already ;)

This year I'll be leading a Cystic Fibrosis Great Strides walk team again, so if you would be so kind to visit my Great Strides page....



www.cff.org/Great_Strides/leahbailey92504

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

Finally, some hope.

Good afternoon all to all my blog readers!

I'm very sorry I have not updated my blog until now. I didn't have anything new to share (until now), so I didn't really know what to write for you all. I don't want to bore you, so I waited until I had something worth while to share.

Since I last updated, I did a 4 week course of antibiotics (4 weeks oral/ Levaquin, and 3 weeks IV/ Cefapime ). I was feeling pretty junky in my lungs and running fevers while waking up with my sheets drenched from cold sweats, so I had to give in and get some antibiotics on board to help the infections that were cookin'. The IV antibiotic was ordered for me to do 22hrs per day, giving me 2 hours a day free to shower and run errands. After about a week on the IV antibiotic I was feeling pretty sick from the med itself on top of feeling bad from being sick. I finished up the antibiotic still feeling pretty crummy, so I talked to my doctor and decided that it was probably time to get bronched and open up the stenosis I have in hopes to open it up enough to allow me to cough junk up so I can get rid of the infection easier.

I went in on Thursday the 14th for my bronch; the day after a set of photopheresis treatments. My doctor went in expecting to see my airways red and inflamed, with either 1 or both airways stenotic (narrow). What he saw was my airways almost back to the pale color they are supposed to be! Both main airways were stenotic, so he opened them back up via balloon dilation and took some pictures to show me once I woke up.

I'm attaching both a set of photos that were taken back in February of this year, before I started photopheresis treatments, and a set of photos that were taken last week. Note the very red, irritated airways in the first set. This started to appear on my bronchs around the time that my PFTs began to decline a few years ago. My CF doctors assumed the inflammation and redness is due to chronic rejection and we really didn't think it would get back to it's normal pale color.

The second set of pictures are from the bronch I had a week ago. Note how the redness is almost gone! The photos were taken about 10 weeks after the start of photopheresis treatments there at U.C. Davis. We're excited and very hopeful that this is a result of the photopheresis treatments working! Maybe, just maybe, the treatments are stopping the progression of rejection! I have not done PFTs in a while, so I don't know if I have any improvement with that yet, but my lungs sure look better! You'll see that the airways are still stenotic in the first few pictures, before they dilated, then much more open (and even a little more red) after they dilated and messed with them.

So that's my good news! I'm extremely hopeful that these treatments are actually working. I do not feel better, but we really didn't expect for me to feel better with the treatments. We were just hoping I wouldn't get worse, which from the look of my airways, may be the case!

All of you who are considering photopheresis,
Like all medical treatments, it's not a sure thing. It does not work for everyone, but we chose this treatment over taking medication that would knock my immune system out and I'm very lucky that we may be seeing positive results from it. I feel so grateful that I found a doctor who would not give up on me and decided to give photo a try on my rejection. If it were up to my transplant center I would be waiting on the transplant list right now for new lungs. A huge thanks to my post transplant friends (especially Christy) for showing me all the options there are for people in chronic rejection and not allowing me to give up on these lungs. I know I may be jumping the gun a little, but I'm thinking positive! ;)

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.

Photopheresis A-Z

What a roller coaster I've been on for the past week! I was scheduled to start my photopheresis treatments on Thurs 5/5, so Benito got the morning off of work and we headed to the hospital bright and early with butterflies tucked securely in my stomach. Once we got there the nurse comes up to me and asked if I heard that my insurance had revoked their approval for treatments. WHAT?!? No, I hadn't heard! I wouldn't be there if I had heard! So they had Benito and I sit there and wait for the charge nurse to show up to work and see if she were able to get in touch with my insurance company to get approval for them to start that day. After waiting an hour, we were told that we should go home and they'd be in touch. The woman who was responsible to get the insurance approval had requested it incorrectly, so the insurance company had approved the central line placement, but not the treatments. The following day I got a call from the apheresis team at UCD and was informed that they had gotten approval for the first treatment, but was still waiting for approval for the 30+ others that I will need down the road. So we scheduled my first set of treatments for Monday 5/9 and Tuesday 5/10.

Benito came to the Monday appointment with me. I've been a nervous wreck in anticipation for these treatments to start and not really knowing what to expect, so having Benito there to hold my hand and keep me smiling was a huge relief. I had asked him to take photos of the treatment being done along the way because I wanted to show others what the photopheresis treatment is like. It's been very difficult to find very much photos or patient experiences online, so I want to make pictures available for others to see to help with their nerves if they are going to be starting photopheresis too. Benito was so interested in every step of the whole treatment process that he took a Lot of pictures for you all! Once we get those pictures off of his iPhone and onto my laptop I'll post a link for you all to see them on my Picasa albums.

Let me start by explaining a little how photopheresis works...
The whole process takes between 3-4 1/2 hours from the time I walk into the room to the time I walk out. I'm given a nurse that works one on one with me during the whole process and she starts the first day off by taking labs through my accessed port-a-cath to see what my hematocrit is at that point. Hematocrit is the percentage of blood volume that is occupied by red blood cells. The goal HCT (hematocrit) for a woman my size is 38. If my HCT drops to 28 or less the apheresis team will require me to receive a blood transfusion before they will be willing to do a pheresis treatment. After the treatments go on, my hct level will probably drop because the nurse is unable to give back ALL of my blood cells at the end of each treatment and depending on how fast my body is able to make new cells, I may see a small drop in hct over time, but they do all they are able to do in terms of giving me back as much as my cells after each treatment so I can keep my levels high. Another reason why they check the hct is to determine whether to use a "small bowl" or a "large bowl" for the kit they use to pull blood from me. A large bowl will remove more blood at any given time from my body, so if my hct is low, I may feel woozy if they were to use a large bowl at that point, so they would choose a small bowl. The nurse said that for my size they will probably use a small bowl the entire course of the treatment because I just don't have that much blood in my body compared to a large person who would get away with using a large bowl. I hope that makes sense and you're able to keep up with me so far! (Please leave a comment with any questions you may have in this post if you would like something clarified!)
So after they get the blood results back, the nurse sets up the photopheresis machine with the kit including the large or small bowl (small bowl for me!). The machine does an automatic prime of saline mixed with heparin to keep the blood from coagulating while it's out of my body. After the line is primed I'm then connected to it via my port and with a press of a button my blood is pulled out at a pretty speedy rate (I'll be able to give you that actual speed once I can remember it). The blood is pulled into the bowl and the bowl spins very quickly to separate my red cells from my plasma (which includes my white cells). The process is making what they call a "buffy coat", which is composed of white blood cells and platelets that will sit above the leftover red blood cells. The machine can tell when the buffy coat is at it's peak, and then switches off to separate the buffy coat and send it to a collection bag and returns my red blood cells back to me. That is the first cycle. The machine will repeat the cycle 5-6 times depending on what my blood work suggests At the end and they have as much plasma as they can saved into the collection bag the machine beeps and lets the nurse know that it's time to "photo activate". The nurse infuses into the collection bag a chemical that is like what plants use to absorb UV from the sun. The nurse mixes the chemical with my plasma in the bag then switches on the tanning bed looking part of the machine and the machine will photo-activate my plasma for however long it thinks is necessary at the time (usually 30-50min). The blood will constantly be moving over the tanning bed into the collection bag and back through the tanning bed throughout the 30-50min "tanning" process. After the machine has decided it's photo-activated enough it will then switch over to send my photo-activated plasma to a different bag and then send it all back into me. I can tell when this is happening because I get a metallic taste in my mouth, which the nurse says is the chemical she infused into the plasma. A lot of patients can taste that chemical when the plasma is re infused back into the body. Once the plasma is re infused the machine switches off and the nurse then does a manual return to try and get whatever cells were left in the bowl and tubing back into me. Once that is complete she flushes my line and gives me a dose of heparin to keep the port from clotting in between treatments. After each cycle of the machine my nurse takes a blood pressure and it slowly goes down throughout the course of the treatment, but once everything is returned to me it goes back up to normal again.

The whole thing is very interesting the first time around, but I can see how it can get pretty mundane after a while and I'll be able to relax and sleep during each treatment. I'm able to drive myself to and from each treatment, so Benito only came to the 1st one on Monday and I went alone to my appt on Tuesday.

As far as side-effects go, I felt normal (with the slight exception of getting very cold) throughout the treatments, but started getting a headache during the first treatment on Monday and it didn't go away completely until last night (Tues night). The doctor overseeing the treatments thought the headache could be because of all the extra fluids they gave me, and wasn't too concerned about it. I came into my treatment yesterday with a low grade fever (99.8*) and am told to just keep an eye on my temps for a few days. I have to be extremely careful when going outside for 48 hours following each treatment because I'll be very sensitive to the sun and can burn very easily. So, I'm required to wear full coverage dark sunglasses, a hat, and sunscreen when going outside for 2 days after the treatments. After 48hrs the chemical that makes me photo-sensitive should be metabolized and out of my body and I should be back to my normal self.

I really hope all of this makes sense. It's a very long post and I commend you if you've been able to get through the whole thing! Like I said earlier, I'll post pictures of the entire process as soon as I can. Until then, here is a photo of me and my plasma ;)
This picture was taken just before the photo-activation began and what you see on that plate is my plasma ready to be ran through the tanning bed thingy lol.

Tomorrow is the day!

It's been almost a week since I've had my new central line placed. It ended up being another port-a-cath (much larger than the one I already have) placed instead of a broviac line. The pro is that it's all under my skin and I can shower and not worry so much about infection, but the con is that I'm not sure if the pheresis team is going to be happy when they see that the line I got is only single lumen. I'm really hoping they don't have me go back and get another line because the surgeon didn't do the one they wanted.

My first photopheresis treatment is finally scheduled for tomorrow morning (8:15am). Benito is taking the morning off of work to go with me. I'll have another appointment on Friday then probably twice again next week and so on for a 6-18 months depending on whether or not we feel it's working. I'm nervous and excited about getting it started. I'll be very sensitive to the sun for 24-48hrs after each treatment, so I've been told to get a good sunscreen and dark sunglasses to wear. I'm going to have to get new prescription sunglasses because the ones I have are not enough coverage.

On a slightly hopeful note, I had CF clinic this past Monday and my PFTs didn't show any decline since my last visit! That's the first time in a very long time since my PFTs have been the same instead of dropping between visits. It makes me think that switching my Azithromycin to 250mg every day instead of 500mg 3x a week has been helpful. I wish I would have known sooner that I was able to do that.

That's all for now. I'll try and post sometime this weekend about how the pheresis treatments went.

Here we gooooooooo

I got the call yesterday that my insurance has approved the photopheresis treatments!! I was so happy I couldn't stop crying. I'm being given another chance to live; I just hope these treatments work and I can keep these lungs of mine for many more years to come!
This morning I got another call saying I need to check into the hospital at 9am Thursday morning to go into surgery/interventional radiology and have my new central line placed. I'm under the impression that as soon as that's placed they'll start my first photopheresis treatment. Not sure yet if I'll have to stay overnight. Benito will be going into work early that morning so he can get a few hours of work in before having to take the rest of the day off to take me to the hospital and sit with me through the first treatment. We're both nervous, but very hopeful at the same time.

This couldn't have come at a better time (okay, if it were next week, then it would have been easier on us, but oh well!). We got the majority of our stuff moved over to our new apartment in Roseville this past Saturday and plan on going this evening after he gets off work to go load up the remainder of our things and begin cleaning. I'll go alone tomorrow back to Yuba City and pick up my sister and she and I will clean until it's finished. That way it will be done before I have to start treatments and who knows how useless I'll be after having that new line placed. I'd feel better knowing it's all done and we can turn in our keys asap.

So that's my good news! I could use your continued support and prayers that these treatments work. It may be months before we even see my PFTs stabalize (if they do at all), but I'll keep you updated along this journey of ours!

Ask enough and you shall receive

First of all I want to thank the readers of my blog who continually come back for updates even though I do not always have new ones to share. I also want to apologize for not updating more often. I've never been the kind of person who is able to write just a little at a time, so I seem to put off writing updates because I know it will take a huge chunk of time to do so and can never commit myself to sit down long enough with my laptop and write. I owe it to you all as friends who have offered their support and prayers to me and my family to write more often and give you the updates you come to read. So I'm going to do my best to write at LEAST once a month (a suggestion given by one of my readers) and update you something about what's going on with me. It may not be a long update, but I'll try to at least let you know how my health is holding up at the time. :)

Last I updated I was looking forward to my future sister in-law's wedding, which I was to be her maid of honor with. It turns out that four days before the wedding I got severe hives that covered about 90% of my body and no matter how much benadryl I would take, it would only get worse. After one last attempt of a cold shower to help with the hives and it failing miserably I broke down and had Benito take me to the ER close by. I chose the nearby ER rather than my CF center's ER because I thought it would be a quick in and out visit. They would give me an IV dose of Benadryl and I'd be all fixed! Wrong. I spent four hours in the ER waiting room sat next to someone who was certain he had H1N1 (yes, I wore a mask and didn't touch Anything, but it's still scary to be in a crowded ER waiting room being post transplant!). Once I got taken back to be seen the ER doctor was more concerned with the fact that I had stopped taking an antibiotic that I was [at the time] sure caused the hives and he wanted to find an alternate antibiotic to put me on at 1 in the morning, rather than treating my hives. He called my tx center, which is really not treating me anymore, so they were not sure why they were called. After a few hours he had the nurse push IV Benadryl and an IV form of steroid to help the hives. After she pushed the Benadryl, she immediately pushed the steroid and as soon as she did so, I got a very strange burning and tingling sensation throughout my body. By the time she flushed it through I was having trouble speaking and couldn't lift my arms or legs. I tried to tell her what was wrong, but it took forever to get a single word out. Poor Benito was so worried. The nurse asked Benito if my speech was always like that (even though she and I had been talking to each other perfectly normal minutes ago) and Benito said no, so she went and got the doctor. He didn't know what was going on, so he had me just wait and see if it got better or worse. After three hours my speech was beginning to get better and I was able to move my legs enough to walk with some help. So the doctor decided to discharge me and have me follow up with my doctor the next day. We got home at 5am and by noon my CF nurse called to follow up on the blood draw I had done the morning before (pre ER visit). Aparently my creatinine (kidney function) was at 2.8 (almost 3x my norm). She had spoken to my CF doctor before calling me and he wanted me admitted immediately for acute renal failure. I told her about the night I had in the ER and she said that severe hives is a symptom of renal failure and was disappointed the ER doctor never did any blood work before giving me that large dose of steroid (which causes more strain on the kidneys). So I was admitted into my CF center and hooked up to IV fluids, had a foley cath placed to drain my bladder and had my Prograf held for a few days until my creatinine came down. My Prograf (immunosuppressant medication) level in my blood was at a very toxic level of 30 (my doctors try and keep it from 8-9 at all times, so we figured out that the antibiotic I was put on a week prior had increased the toxicity of the Prograf I was taking (although I did decrease the dose of Prograf I was on as directed by my physician). My levels came down low enough that my doctors allowed me to be discharged the evening before the wedding, so although Benito, his father and I missed the rehearsal and dinner, we were able to be there for the entire day of the wedding! The wedding was beautiful and we all had a wonderful time. I got to meet a lot of Benito's family that I hadn't met yet, so that was a real treat.

Just before being discharged from the hospital, the pulmonologist that had did my eval for transplant came in and saw me and discussed options for me to consider regarding treatments for rejection. He said he personally had experience with both rATG and photopheresis and found them to both be not a sure fix, but he was more comfortable trying photopheresis on me. I have three lingering infections in my lungs that we just can't seem to take charge of, so he didn't think using rATG on me was safe as it would allow the infections to worsen when my immune system dropped. Photopheresis is a procedure in which I would have to get a new central line placed with 2 lines, one that would remove my blood and the other that would place it back into my body. while the blood is out, it will be shown a special kind of light that will kill T-cells and them place the blood back into my body sans T-cells. The treatments will all be out patient, starting twice a week for a while then tapering off slowly. The doctor said we will hopefully see a halt in lung function loss within a month or two and if it seems like it's working we'll continue the treatment for six months. He has only worked with three other patients with photopheresis and these are how they worked: Pt A: halt in lung function decline, but no improvement. Pt B: Pt actually gained 10% of their lung function back and the signs of rejection disappeared. Pt C: Pt continued to lose lung function / photopheresis showed no benefit for this patient. So I'll be his fourth patient he's tried this on and I'm hoping to at least halt the decline, if not get some improvement. Really though, I'd be happy to just not lose any more lung function! Once my insurance approves the treatments I'll have the new line placed and hopefully get started on the photopheresis as soon as possible!

Benito and I had been talking about moving closer to his work (as it is, he's commuting 2hrs a day round trip to work), and with the gas prices on the rise, it's gotten to be so expensive to live so far away from his work. Now with the photophesis treatments in my future, and all the driving I'll be having to do to and from my hospital our decision was made for us; we needed to move asap. We spent a few weeks looking for a place that we were allowed to bring our dog, Roxy and as of a week ago, we've found an apartment 10min away from Benito's work, 25min away from my hospital and they allow Roxy! We'll be getting the keys to the new apartment on the 23rd and it looks like we'll be spending Easter driving a U-Haul packed full of our stuff to our new home in Roseville!