Cedars-Sinai consult update

First of all, I wanted to let you know that I'm trying to figure out a way to upload the Bronch video to show those of you who are interested in watching it. I tried uploading it to YouTube, but it told me after the 20min long upload that the video couldn't be published. If anyone has a suggestion for a better place to upload a 5 minute long iOS video I would be forever in your debt. Thanks! Now, on to our previously scheduled program:

I owe a much anticipated update about my Cedars Sinai appointment to you all... This is going to be long and may be hard to understand, so please let me know if you have questions after you've read through it.

My amazing friend Michael Adams, whom I met 11-12 years ago on a CF forum, has been speaking on my behalf to the lung transplant team at Cedars Sinai (where he got his double lung transplant 12 years ago). When I found out that UCLA was not willing to meet with me after seeing my bronch video which shows the extent of the stenosis issue I am struggling with, I was devastated. I had already been told by all the other lung transplant hospitals in CA that they were not willing to see me for different reasons, but "you're too high risk" was becoming a very familiar answer. The one hospital that didn't dismiss me immediately was Cedars, but when their financial department called me to discuss insurance coverage before scheduling a consult appointment I was told they do not accept Medi-Cal (a government assistance insurance coverage that I've had all my life). At that point I didn't see how I would be able to even see what Cedars' transplant doctors thought about my high risk issue because I couldn't be seen simply because of my insurance. Insert my friend Mike and his ever insistent love for Cedars and his friends. He began speaking to his transplant coordinator, doctor, and even surgeon there at Cedars whenever he saw them, or emailed them on a regular basis telling them that he had this friend who has been turned down everywhere else and he just KNEW Cedars would do whatever they could to help me, only my insurance was an issue. So one day Mike sends me a text message and says that Cedars wants to see the bronch video I'd sent to UCLA. I could send the video and have them review it without actually having to be seen there first. Then I got sick with the CMV infection and ended up in the hospital before Christmas...

   After I was discharged, Christmas and New Years passed, and things began to settle down, Benito copied my bronch disc and I put it in the mail to one of Cedars' lung transplant coordinators. I got a call from the coordinator only a few days later! He said that the whole team watched my bronch video and they feel like they can help me and that they've found a way for me to come in for a consult via pulmonary consult rather than a transplant consult because basically all of their departments contract with MediCal except their lung transplant program. So the plan was that I'd gather the list of medical records they want me to bring in with me, when I had all the records with me, I would call to schedule an appointment. 

I was able to get scheduled quickly for the full set of pulmonary function tests and 6 minute walk that they required I bring recent results of, then after those tests I walked over to my doctor's offices there in the hospital and was able to pick up a manila envelope full of all the other records Cedars had requested. That afternoon I called and told the transplant coordinator at Cedars that I had all the records they requested and I was ready to schedule the appointment! So they put me on the schedule for March 19th. I let my friend Mike know the date of my appointment because he had told me a few times already that he really wanted to be there at the clinic to support me when I got to meet with them.

So Benito and I left early in the morning (this last Thursday, March 19th) for our 7 hour drive to Beverly Hills, full of anxiety and excitement for this very important appointment that could quite possibly change our lives. We got to the clinic with maybe a half hour to spare (we gave ourselves 8hrs for a 6hr trip, but stopping for food and gas took quite a bit chunk of time!). My friend Mike met us once we got out of the elevator there at the clinic and he looked just as nervous and excited as we felt. So Mike points me in the right direction to sign in, introduces us to a friend of his that works there that had an amazing story about her father becoming a famed artist. Honestly, being able to listen to her story was a nice opportunity to breathe and take my mind off of my nerves for a few minutes. Before long my name was called and they took Benito and I back to get my vitals and put us in a room. A doctor (fellow) came in and talked to us about the history of treatment and intervention that's been done to my stenosis issue. He listened to my heart and lungs, then we discussed a little about the hurdles I've had with trying to look for a center for retransplant. Then he took my folder full of records and said he'd be back with Dr Chaux (the director of the lung transplant program there at Cedars). After a few minutes Mike walked in and sat with us until Dr. Chaux came in.

Dr Chaux introduced himself and said that he was able to look through my records as well as seeing the bronch video weeks prior. After reading the bronch notes that my doctor at UCD had written, Dr Chaux does not think that there is anything that Cedars can do as far as helping with my stenosis issue. He said that my doctor is already doing everything that he would do, so there's no point in coming all the way to LA to get bronched every three weeks when my doctor at UCD is able to keep doing them for me. So I asked him about retransplant... Did he think there was a way to get a second double lung transplant? Like I'd been told plenty of times before, my stenosis has gotten so bad that it does not leave enough space at all on my native airway to attach a right lung. (I'll attempt to attach a photo or a link to a video of the bronch so those of you who are curious and not grossed out by blood can see exactly what I'm talking about when I say there is no room.) I asked him if a single left lung transplant would be possible and he said that yes, I could get the left lung, but I would still have the problem with my right airway shutting down, causing the right lung to collapse and get pneumonia. That would put my immune system into overdrive, which could very well cause me to reject the left lung very quickly. So really it's not an option. As far as I knew, those were the only options out there and after he explained how each one of those options won't work, Benito and I were sure we had made the trip for nothing...

Then Dr. Chaux threw us a curveball and said that there is one more option for me and he would be willing to try and make it work... He wants to try and list me for a double lung and domino heart transplant. Basically, in order to attach a new set of lungs to me, it needs to still be attached to the heart, giving the surgeon more room to attach the heart and lungs in one piece onto my trachea, rather than attaching one lung at a time to the right and left main bronchi like a typical double lung transplant is performed. My heart is healthy (as far as we know anyway), so when I get a call for the heart/double lung transplant I'll be able to donate my own healthy heart to someone who is on the waiting list for a heart. Crazy, right?!? To be honest, as soon as Dr Chaux said I could be a donor at the same time I'm receiving my own transplant, I just knew that I wanted to do it. Being able to donate my heart to someone in need takes a lot of the scary out of a retransplant. Given the chance to give back and giving someone the chance to live again like I was given (and will be given again), I'm just so happy to be able to do it. This domino heart transplant bit is rare, but has been done before at a few hospitals throughout the country (per the research I've been doing on my own online). Dr Chaux admitted that he has not yet done this type of transplant at Cedars, but assured me that it HAS been done successfully at other transplant centers and he would be happy to give me a chance at it, especially since he can't offer me any other options there Cedars. 

So, whenever the heart is involved in a multiple organ transplant, say someone needs a liver or lungs very badly, and their heart is only beginning to fail, the patient would be listed as on the heart transplant waiting list, their second organ not taking priority because the heart Always takes priority. That being said, even though my heart is healthy, I will be listed on the heart waiting list and the fact that my heart is healthy puts me as a tier 2, very low priority. There is no other way to list me with my lungs being priority. Dr Chaux said my small size gives me an advantage because there are not as many smaller stature patients waiting for hearts as their are larger stature people. I also have a very common blood type, so that's in my favor. Dr Chaux expects me to be on the waiting list for "a year, give or take". Of course that does not mean that I could not get a call for transplant the day after I'm officially listed, if everything lined up just perfectly, or, I could be waiting much longer than a year... It's very hard to tell. What is very important to understand is that my lungs could ultimately fail me while I'm waiting for a call saying they have a heart and double lung donor for me. That's something I have to come to accept, but fight hard to stay as healthy as I can so I am still here to receive this amazing transplant and donate my own heart when the time comes. A very interesting fact about all of this is that how I understood it, the recipient of my own heart will be a patient of Cedars, who is waiting for their call for a heart while I'm doing the same (but mine will be coming with lungs!). This is so when the right organs are available for me, they will be able to have the heart only recipient on call and ready to receive my heart as soon as it's removed from my chest. This also means that there's a good chance my family will be sharing a surgical waiting room with the recipient of my heart... They not knowing their loved one's heart is coming from a living donor, but my family of course knowing. There are strict rules set up to make sure a recipient does not have personal details of his/her donor, and vice-versa. So, it will be quite amazing to see someone recovering from their heart transplant next door to me in the ICU, knowing my heart is beating in their chest. I know that at that moment, all the pain, struggle to breathe, stress and anxiety I've ever had in my entire life had been worth it because I gave someone else a second chance to live again. How many people get to say they were able to give someone their heart- literally? 

So our first step is to figure out financing. I've been assigned a very sweet and helpful financial counselor that is working with me to find an insurance that I can switch to that will be accepted at both Cedars-Sinai AND UC Davis Med Center. I will definitely need to continue the every three week bronch and laser at UCD to keep my right lung from closing up completely and causing my lung to collapse.

Some of you may already know this, but some of you may have probably been wondering WHY on earth Benito and I have been engaged for 4 1/2 years and haven't gotten married already?!? Well, the truth is that we were afraid to lose the insurance I currently have now (which covers All my hospital stays, procedures, tests, and medication) once my insurance began to figure in Benito's salary as my husband. So, we've been content on staying engaged and not fixing something that isn't broken. Now that I'm in need of a new insurance that Cedars accepts there is a very good chance that Benito and I will finally be able to marry and I'll transfer on to his work insurance. So that's what we're looking at for the time being... We're trying to get a list from Benito's health insurance rep that lists all the different insurance options his work gives him, then I'll have Cedars and UCD take a look at that list and tell me which ones they accept and then it should be as easy as picking out of the final list of insurance options that Both hospitals accept! Fingers crossed it's that easy... We all know how difficult dealing with insurance can be!

Once insurance is no longer an issue, Dr Chaux will refer me to Cedars' heart transplant doctor and that doctor will begin ordering tests to see if my heart really IS as healthy as we are hoping it is. If it is, they'll have me spend 3-4 days down there for a bunch of eval testing that if all goes well, will ultimately be my ticket to being listed there for their [and my] first double lung & domino heart transplant. Dr Chaux said that I'm welcome to stay living at my own home while I wait for my call (6-8hrs away from Cedars, depending on traffic), then after I'm discharged I'm given the choice to go back to my home or rent a place to stay for a couple months  that's close to the hospital. He said that as long as I made it to my once weekly transplant clinic I can live wherever I wanted. He even has patients who live in Colorado and Utah that fly in the night before, go to clinic early in the morning, then fly back home that afternoon. My only worry is how much I'll be able to stand the sternum pain while I travel back and forth. The 2mi drive from UCSF to the hotel my mom and I stayed at post transplant in San Francisco (10 1/2 years ago!) felt like the longest car ride with all the bumps on the road. It was so painful and squeezing a pillow only gave so much relief. For anyone that doesn't know, bone pain is Horrible. I wouldn't wish it on my worst enemy.

Part of the reason why I have taken so long to get this blog update posted is because it was important for me to talk to my immediate family in person about it before they had to read it online. This is a very scary, but hopeful turn of events for us and I wanted to be able to see their faces and cry with them instead of hearing later that they cried alone while reading my blog. So I apologize for the long overdue update, but I also thank you for your patience and allowing me to spread this news the way I needed to.

There has been a lot of tears and hugs between Benito and I this last week, but I can assure you they are tears of relief and amazement. We have been so happy together all these years, but the years have also been filled with a lot of grief, worry, and pain. I can not wait to start a new chapter to our story and it be all about how much we get out and do things instead of spending our time watching every tv series Netflix has to offer, while eating dinner in bed because I'm not feeling good enough to do much of anything else. 

So to those of you who pray, please pray that my heart is healthy so I can be a heart donor, pray that we can find an insurance that is accepted at both hospitals and doesn't require huge copays that will put us in debt, pray for the donor who is right now living their life, completely oblivious to the fact that he/she will be the second hero to save my life. To those of you who do not pray, please send me your positive energy, and love. I've said it plenty before, but I'll continue to say it: I owe my life to you, my family, friends, nurses, doctors, respiratory therapists, and of course my fellow CF/transplant friends. Your love keeps me going and I promise I will never Ever give up on this miracle of life I was given twice so far. I love you all SO much!!!

I'll update again once I know more... In the meantime, please talk to your family and friends about organ and tissue donation and sign up to be a donor on the national organ donor registry if you have not done so already. Thank You!!! <3 div="">

www.donatelife.net

Bronch Photos

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

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

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

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

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

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

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

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

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

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

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

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

Here we go again.

Forgive me because I don't know where to begin this blog entry that I've put off for as long as I possibly can.

The last update left off just after I was discharged from the hospital with another week of IV Meropenem to finish up at home. 5 days after I was done with the IV antibiotic I had a CF clinic appointment in which I brought Benito with me and was preparing to have a serious talk with my doctors about how much I want to be more aggressive toward finding out what's going on with my liver and the trouble I've been having with my stenosis getting worse. What I wasn't expecting was the talk my doctor was planning on having with me.

I was happy to see the younger CF doctor that day because I've been butting heads with my other doctor lately and there was so much that needed to be said, by both sides, to get caught up in disagreements.

As always, I did my PFTs first and was shocked to see that they were down from my baseline of 34% to 26%.  When my doctor made is way in to see me, he acknowledged that the large drop in my PFTs was very worrisome and that he was also concerned with the amount of pain I was in with pretty much no relief with my pain medication. He then went on to tell me that he knew I wasn't stupid, and we all know that my disease is progressing and that now is the time to get in touch with UCSF again if I intended to get back on the transplant list for a second lung transplant. He decided that the best option was to go ahead and admit me that day and while I was in the hospital we could have the Palliative Care team consult and see if they can come up with a way to control my pain. I'll also get started back on IV meropenem and once I've been on the antibiotic for a few days then I'd get a bronch to open up my stenosis again (even though the last bronch was only 2 weeks prior). We were all hoping that we could blame at least some of the lost lung function on my stenosis being closed off again.

I had my bronch 3 days later and was very surprised to find out that my stenosis was wide open, in fact it looked as well as it did when they finished the last bronch. So what that told us is that I'm definitely losing lung function to rejection again. I have a feeling the complete closure of my stenosis and my right lung not getting any air to it for a good week a few weeks prior is what shocked my immune system into rejecting again. What's done is done though. 9 days after my PFTs in clinic I did another set in the hospital and I was down to 22%. I was discharged the next day with plans to get over to UCSF as soon as I can.

As far as the pain management goes, the Palliative Care team in the hospital was very nice and tried very hard to help my pain. We attempted a nerve block first with them injecting lidocaine on and around the area that I have the chronic lung pain, but the injections really only caused more pain.  They put me on a PCA (pain med delivered directly through my IV each time I press a button) after my bronch - the day before discharge in hopes of it bringing my pain to a level I can live with before relying only on oral medication. The PCA and oral med combo helped, but I was so sleepy and out of it. They sent me home on my old pain med at a 3x dose along with Oxycontin to give me more of a long term relief so I wouldn't have to take my other med as often. Unfortunately my insurance refused to cover the Oxycontin at discharge, so the discharge planner managed to get me 3 days worth that "should" last until the insurance issue got smoothed out. Three days later we heard back from my insurance and they declined coverage, so I was back to only my old med by that Tuesday (1 week ago now). It took me about 4-5 days, but I've been weaning myself back to my old dose of my old pain med because it really doesn't help, no matter the dose, so I'd rather take less (just to keep from withdrawl, really). I can't believe how sleepy and foggy I was for the past 2 weeks on the Oxycontin. I'm still finding out things I've seen (tv shows), said, or did while I was so dopey and can't remember doing them! I am grateful for the time my pain was lessened, but I REALLY don't like living in such a fog and would rather not. I [think] I was supposed to get in touch with the pain management team once I was discharged, but I can't remember who exactly I was supposed to contact. I'm hoping they can eventually find a medication that will both help my pain and Not make me feel like I'm drugged up and forgetful. The pain I have is in my right lung, so God willing, this pain will not be an issue once I get new lungs.

I got a phone call the Monday following discharge from the Pre Lung Transplant Coordinator checking in with me and letting me know that they are waiting for some test results to be sent over from UCD before they schedule a clinic visit/consult with one of their doctors. Yesterday I got a phone call scheduling the consult visit for March 6th @9am with one of the doctors I'm familiar with already. Today I got a chest CT with contrast done that UCSF is requiring me to bring a copy of to my visit. I went ahead and purchased a personal pulse oximeter that tells me my oxygen saturation whenever I need to know (it's super small and I can keep it handy in my purse for whenever I need to check my o2 sats). Today while I was walking from the hospital's parking garage to the radiology department I was getting especially short of breath and dizzy so I put the sat monitor on and was surprised to see my oxygen saturation down to 85%. Once I sat down and rested for even 30 seconds my sats came back up to 97-98% on their own, so that's good news, but the desatting is definitely upsetting. I'm not ready to admit to myself that I am as sick as I really am right now. I prefer denial to be honest because every time I allow myself to think about it, I panic and get very scared that I either won't be accepted as a lung transplant candidate again, or I will just run out of time waiting for the eval and listing to be done. I'm trying my best to stay positive, but it's not always easy when I feel so helpless just waiting around doing nothing to speed things along.

So Benito and I wanted to let our family know about my need for another transplant before I made it public on here or Facebook and they found out via social media. Now that our families know what's going on I wanted to share the news with you all and ask for your positive thoughts, prayers, and whatever else you feel can help me get the lungs I need before it's too late. My first transplant was scary, but this need for a second puts a whole new level to "scary". This decline is so much more sudden and it has shaken myself and my family to the core.

I'll update again as I hear more. I'm not sure I'll have any news to share until after my appointment on the 6th, so don't get worried if you don't hear from me before then. I'll update after that consult though... I promise!

Lots of love to you all. And a big THANK YOU for all the support and love you've shown me and continue to give. I couldn't do any of this without the strength I get through your love!

XoXoXo

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

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

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

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

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

What we know now:

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

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

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

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

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

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

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

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

Until next time...

Brachytherapy- Take 2

Hello friends!

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

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

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

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

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

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

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

Brachytherapy tomorrow

Just a quick update:

I'm scheduled for that first brachytherapy treatment tomorrow afternoon. After talking again with the radiation oncology doctor yesterday I feel much better about the actual procedure. This is still new territory and the risks do scare me, but I'm hopeful that I may be able to breathe better after all this is done. Please, if you will, say an extra prayer or keep me in your good thoughts tomorrow that everything goes smoothly and I get to go home that night as planned. And of course, pray that this treatment does what we hope it will do, which is break down the scar tissue that's blocking my airway, so I can start taking full advantage of what's left of my lungs. If I could open that airway I won't feel so darn sick all the time and I won't feel like retransplant is just around the corner.

Thanks everyone! I'll try and let you know how it all went as soon as I can, but please don't be worried if you haven't heard back from me in a week. A normal bronch usually knocks me on my bum for a few days, so I expect this will be not much easier.

-Leah

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

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!

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! :)