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.