The "Cysters" hit again

Not sure if I wrote about having an article in the newspaper done about Piper, Ali, Kristy, Lyndsey, Kelley and I back in September 2012, incase I didn't - basically they just wanted to do a quick story on our bond as CF and transplant patients. After it was featured we were hoping to get some calls to do some appearances so that we could talk more and get the word out about both how devastating CF is and how amazing organ transplant is. Two years later, we get an email from a director at the organ donor network saying that ABC is interested in interviewing us on TV! We later found out it was actually for a segment on the Katie Couric show. Our original date got postponed because they said they wouldn't have time to give our story justice on the day we were supposed to tape. So instead we waited and finally taping day on June 10th had arrived. We were all pretty excited because the 6 of us hadn't seen each other all together in quite some time. Lyndsey had moved to florida last summer and Piper after having a second double lung transplant in North Carolina was now home in Colorado. The Katie show flew Lyndsey and Piper in for the show and provided cars for the rest of us. It was a really exciting day. We hung out in the green room for a while. While in the green room the producers went over questions that we were going to be asked so we knew in advance how to answer, we got miked up and our hair and make-up was freshened up. I think the segment went really well. In a short amount of time Katie was able to cover what CF was, what it was like growing up with it and how transplant has changed our lives. She focused on the group and each of us spoke about the same amount of time. At the end of the show she announced that they were sending us on a trip for the 6 of us to rest and relax in the British Virgin Islands!!! Can you even imagine? We are obviously so excited and cannot wait for this to happen. It's a little tricky with everyones schedule and my health but we are aiming for mid august. Hoping that I will be recovered from my kidney transplant and maybe even feel a little better so I can actually enjoy the trip! The actual episode will air on July 23rd - so set yout DVRs!!! Soooo the kidney transplant. That's another story. My testing and my sister Christine's was all done and we were set for surgery to happen on June 18th. I went in for pre-op the week before and the very next day was called and told surgery had to be postponed because I had yet another infection and spot on my XRay. IV antibiotics were restarted for two weeks and I am hoping that they can schedule surgery soon after before I develop anymore infections. So that's what's going on over here for now. Nothing too exciting. I am using oxygen a little more than I had been previously so my wishes to return to work, even once a week may not really happen until after I have this kidney transplant and get new lungs. It would be amazing if gaining a new, functioning kidney actually made me feel much better where I could actually live a little and do somethings independently without the need for oxygen. We shall see. There's always room for a miracle, right?

life is short .. do what you can .. when you can

Went to the doctor's yesterday for a check-up. I went into the hospital two weeks ago with RSV and pneumonia and was pretty sick. I am home now, feeling much better but still on IV medications. My lung function, even though I'm feeling better is continuing to decline. I am currently at 30%. My doctor's are concerned that my windows of opportunity to get a kidney transplant are closing in. The trick is to do the kidney before my lungs get any sicker. Both of my sisters ended up being a match. Christine is a little bit of a closer match so she is going ahead with more testing to make sure she is definitely a candidate to donate her kidney. Hopefully we will be able to schedule surgery in the very near future. As soon as I am recovered from my kidney transplant, they are going to finish the few tests I have left to be re-evaluated to be re-listed on the waiting list for lungs. Right now my lungs are "too healthy", but with time they will just get worse. Thankfully right now I have not been needing oxygen. My breathing is horrible, but not being dependent on oxygen has been a blessing. I am okay when I am sitting at home, or doing things really slowly, but the second I have to do anything strenuous, I get REALLY short of breath. I feel like there is no oxygen and I'm just gasping for air. I'm in such a weird place right now. I wish that I was just able to breathe and that this chronic rejection miraculously disappeared. I want to go back to work. I want to make money so that Joey and I can start saving for a house. I want to be able to plan vacations again. I feel like once again, my life is on hold. People might think I am crazy, but as long as I am not dependent on oxygen - I am actually going to try to go back to work. Not full-time, but maybe once a week. To keep myself active, to make some money and to feel productive. I seriously hate not being able to contribute money at all. The NICU is a really safe area and the people that I work with are always looking out for me. I honestly think I'm more at risk of catching infections and viruses when I'm out in public, at work I use protective barriers and am always super careful. I am really so blessed to have a job that I love, that I cannot wait to go back to. Most people would actually like being in my place and not having to work. Since I am little, I have always liked working and staying busy. The more that is on my plate, the more productive I am. I'm definitely more of an "all or nothing" type person. Sometimes it's not the best characteristic trait, but I am pretty proud of my work ethic and wouldn't change it for the world. I know that the next few months/year is going to be rough. Things unfortunately are going to have to get worse before they get better. I think the only thing I can really do, is live each day as fully as I possibly can. I do not want to ever look back and have regrets, wishing that I would have done more.

Can't sleep ...

It's almost 4am and here I am wide awake as usual. The past week I have been feeling really shitty. Not that I really have felt "good" recently, but it's definitely been getting worse everyday. My fatigue is so bad. If I wanted to, I could probably sleep for 24 hours straight. My appetite is non-existant. The few times I have tried to force myself to eat, I end up running to the bathroom and vomiting within my first spoonful. I don't know how much of this is strictly kidney related, or how much is kidney and chronic rejection related. I am just hoping that some of it gets relieved a little when I finally get a kidney transplant. I know I will still be in chronic rejection, so I am not expecting miracles - but just to be able to tolerate food would be nice. I am down to 83 pounds. This is the lowest that I have ever been. Even prior to my first transplant, I was skinny but not this bad. I've decided to give up on school for now. At this point in my life, a higher degree would be nice, but it isn't necessary. I have so little time when I feel good lately - that I would just rather spend that time doing something fun with Joey or my family - and not having to stress about what readings and what papers I have to do for school. I'm an RN already. I have a job that is willing to take me back whenever I am ready and feel well enough to work. School was something that I thought would be a good distraction from everything else that's going on - but it really has just been more of a stressor for me. My lungs are failing and my kidneys are shutting down. I am slowly dying and it's scary as hell. I am really hoping that come next year, this period of my life will just be a bad bump in the road that I had to get through to become healthy again. However, I am realistic and I know that I may not get anymore miracles. I may not make it. I'm going to be 30 in less than two weeks - and all I want is to feel good. I don't care about material things. Everyone keeps asking me what I want for my birthday, but what I want isn't something that can be bought. I want to be healthy, I want a long future with my husband. I want to grow old and have a baby and buy a house and do everything that I've ever dreamed of. NONE of that right now can happen unless I get a new kidney and new lungs.

Here we go again ...

I've decided that it's time to start being more consistent with my blog entries. Life has decided to throw me some more curve balls and I think it's good to keep a record of events and feelings. People always tell me that with everything that's happened and everything that is about to happen, I should write a book. I personally do not think I could write a book, but maybe in the future, my blogs can help someone else write a story or book about these adventures. Tuesday marked 28 months since my double lung transplant. The first 12 months post transplant were absolutely amazing. I couldn't have asked for anything better. I was able to return to work full-time, I ran a 10K in Central Park, Joey and I got engaged, I went on a road trip to Texas, life was good. When people say "health is wealth", it's really true. I felt like I could take on the world. In December of 2012 after having the parainfluenza virus I ended up in the hospital with a bout of acute rejection and ended up in respiratory and kidney failure. I was on life support for a week. My family was told to prepare for the worst. I experienced my second miracle. I came off ECMO and dialysis and recovered. My brain was not damaged at all and after a long recovery I was once again able to return to work full-time and get busy with planning my wedding. My lung function post ECMO only went up to 78%, but I felt amazing so the numbers didn't really bother me. My doctor had said that 78% might be my new baseline after all of the damage that my lungs went through in December. I was okay with this. If I felt good and was able to work and be productive I did't care what those numbers said. In the months before my wedding, my PFT's began to slowly decline. After a bronchoscopy culture, I was diagnosed with a bacteria called MAC that required a combination of three oral antibiotics to be taken over the course of a year. The bacteria was not contagious person to person, it is something that exists in the environment and people with weakened immune systems are susceptible to it. I was also diagnosed with severe reflux in which case I had a Nissen fundoplication done to stop the acid from possibly entering my lungs. I was secretly hoping that the combination of reflux and MAC were what caused my decline in lung function and not chronic rejection, which I told was another possible diagnosis. After having the surgery and being on medications for a month for MAC, my lung function was still decreasing. 28 months after my first transplant we are now discussing both a kidney transplant and a second lung transplant. I am definitely in chronic rejection. My lung function is at 44% right now. When someone is in chronic rejection they usually start off by giving them Zithromax a few times a week, followed by IVIg. I am already on Zithromax (part of my MAC protocol) and had received IVIg while on life support. My only two options left were photopheresis or campath. Both treatments are aimed at stabilizing your lung function from progressing any further. Neither were reported to help you gain back any lost lung function. My doctor and I decided that it was best to start photopheresis, since it was less extreme than campath. I had to have a port placed for Photopheresis. For the first 6 weeks I have gone into the hospital weekly for an overnight stay. On wednesday I would go in, they would access my port and do one photophereiss treatment and then the next morning I would do the second and go home. I just completed my 6th week and I will be going to the doctors next week to evaluate whether it has worked or not. I'm not sure what my lung function is, but my breathing has gotten worse over the past few weeks. It's to the point where I actually need oxygen again during exertion. I get so exhausted and short of breath, my oxygen levels fall and my heart rate skyrockets. If my lung function is lower next week, then we will do a bronchoscopy to culture and see if the MAC is still present. Campath would be the last treatment option to try but it comes with risks. It basically eliminates your immune system completely and makes you highly prone to infections. If your body already has an infection (I have MAC), that infection could actually become stronger since my immune system is being wiped out. In the next few weeks/months I will be starting the evaluation process for possible listing on the lung transplant list for a second time. Before all of this happens however I am having a feeding tube placed on 3/25. My weight has dropped and my BMI is 16 (normal is 18-22). Having a lower than normal BMI, I wouldn't even be eligible for a transplant. I need a feeding tube to help me bulk up so that I meet the requirements for listing. I also need a kidney transplant. My kidneys failed in 2012 when I was on ECMO and ever since then they have not really recovered. They are not to the point where I need dialysis yet, but my numbers to fit the criteria to be eligible for a transplant. You need normal kidney function to be listed for a lung transplant, which is why a kidney transplant needs to happen before a second lung transplant happens. My sisters Christine and Tricia are going to be tested to see if they are a match for a kidney transplant. If one of them is a match, then we can schedule the transplant whenever we want. It would be a live donor so I wouldn't have to go on a list and wait for a donor. If they are not a match, then I will ask any family or friends who would be willing to donate a kidney to get tested. It would be best if we could find a live donor so that we could eliminate having to go on the waiting list. That's just a brief little history of what has happened health wise over the past few years. It hasn't been all bad. I am now officially Mrs. Joseph Monte!! We went on an amazing honeymoon. We have been living in our apartment for a few months and it is slowly becoming like home. I love being married!! I am also enrolled in an online graduate program for my Masters in Nursing Education. I am not working full-time right now, but plan on working per diem maybe once a week depending on how I feel. My masters is something that I want regardless because eventually I would love to teach part-time, either a skills lab, clinical or even an online course. I think that being in school and having something to concentrate and focus on besides just being sick - is really beneficial for me mentally. As always, I will continue to fight and hope that I am able to eventually get new, healthy lungs. I am not ready to go yet. I have way too many things that I want to do and accomplish in life. Hopefully there are some more miracles in store for me. I want to grow old and gray. I want to obtain my masters and I want to have children with my amazing husband.

TWO years ..

It's been two years since my life was saved by a complete stranger. A family lost their daughter and decided that they wanted to donate her organs, two lungs which happened to be a perfect match for me patiently waiting on the list for a transplant. My life will never be the same. I am so incredibly thankful that I have had this amazing second chance at life. A LOT has happened in the past two years. Some good, some bad, some amazing and some really scary - but you know what? I wouldn't change it for the world. "Angela's" lungs have given me the chance to go on a road trip with Lyndsey and not worry who was going to do or when I was going to do chest physical therapy, I went back to my job, saving tiny premature babies, I've gone rock climbing, I ran a 10k and did a 5k in MUD! I got engaged and married to my best friend and the best man in the entire world!! I was on an episode of Say Yes to the Dress. I've been able to play and run after my nieces and nephews. None of this would have been possible without Angela and without organ donation. Last year at this time I became pretty sick from the para-influenza virus. I ended up on ECMO (life support) and on dialysis because my kidneys were failing. No one knew if I was going to make it - but I did! Miracles really do happen when you believe. Three months after that I went back to work - when doctors found out I was on ECMO they were shocked to see how well I was going. A few months ago my lung function randomly started to decrease. I had bronchoscopies and they were negative for acute rejection. My first bronch showed that I grew out Pseudomonas - so I did a month of nebulized colistin and then two weeks of IV Ceftaz. My second bronch showed that the pseudo was gone but now MAC grows. Treatment for MAC is intense. Triple antibitoics for 9-12 months and possibly longer. At my last appointment my doctor said she believed I am in the beginning of chronic rejection because they just can't understand why my lung function keeps dropping. They did a pH probe just to see if my GERD got any worse and since transplant it has. I now have severe acid reflux requiring surgery. I am hopeful that between the treatment for MAC and the surgery that my lung function will go back up and that its NOT actually chronic rejection. My doctor said this is a possibility. So please pray with me, do whatever you have to. I am not ready to say goodbye to Angela. I have so many more things I want to conquer and do and I need healthy lungs for all of them!!!

It's okay NOT to be okay ...

I honestly can't even remember the last time I blogged.  I've thought about it often but I guess life happened and it just became something that wasn't top priority to me anymore.  Receiving the blessing of a double lung transplant on 11/12/11 was the best day ever.  My engagement was also an awesome and absolutely perfect day - but the transplant is number one, only because if I hadn't gotten lungs - well - the engagement would have never been possible.  My lungs were too sick and I wouldn't have made it and still be here today.

The first year post-transplant went by really well.  I got engaged.  I went on a road-trip to Texas with one of my best friends, Lyndsey.  I went back to work.  I ran (well ran/walked) in a 10K to support Cystic Fibrosis.  I was able to go to Disney World in September with my family and keep up with and even RUN after my nephews.  I honestly felt like things could not possibly get any better.  I had energy, I could breathe - I even worked a LOT of over time hours.  Not because I NEEDED the money but because I could, and I loved working.

On my one year anniversary 11/11/12 - my immediate family had a dinner for me.  It was a last minute thing because people were still displaced from the horrific Hurricane Sandy, but they didn't want my anniversary, my first "breathday" to go by unnoticed.  I felt a little weird that day.  A little more sweaty, kind of like I was coming down with something - I honestly thought it was just a little cold.  I ended up in the ER a few days later.  I was in the hospital for 2 weeks.  I was diagnosed with parainfluenza virus.  They put me in the MICU so that they could watch me closer and my lungs needed help breathing so I was started on bi-pap.  I did go home without oxygen.  My PFTs had dropped at my next appointment and it was assumed that I just needed some time to get better, not that anything else was going on.  12/9/12 began the WORST hospitalization I have had and hope to EVER have in my entire life.  

It's honestly all a little blurry for me because I was having high fevers and just felt like complete shit.  I was again admitted through the ER.  I was put on bi-pap.  At some point I was transferred to the MICU again.  Bi-pap wasn't enough so they intubated me and put me on a conventional vent, but as time went on a regular vent was not enough so they put me on ECMO (extracorporeal membrane oxygenation (ECMO) is an extracorporeal technique of providing both cardiac and respiratory support oxygen to patients whose heart and lungs are so severely diseased or damaged that they can no longer serve their function).  Due to the ridiculously high doses of medication to treat the rejection my kidneys also began to fail and they started dialysis.  My doctor's prepared my family for the worst.  However through some sort of miracle and tons of prayers, I pulled through and was taken off of the ventilator, ECMO and dialysis.  It was a very long 8 questionable days for my family and I.  When I woke up (they had me completely sedated), I saw all of the prayers and love and support that my family and I had gotten and was completely overwhelmed.  I just cried for a few days straight.  It's now March and while I am much better, I am STILL recovering.  

Me on ECMO & dialysis - almost unrecognizable (December 2012) 

As if all that wasn't enough - other stuff was happening in my life and it just seemed to all snowball.  I felt like I had lost my fighting spirit.  At one point I honestly thought it would have been better if maybe I hadn't fought so hard.  Today, I know how stupid and idiotic that sounds.  I was given these lungs for a reason, and I'll be damned if I let some setbacks ruin my drive for life and the precious gift that I was given.  

Recovery from all of that has been hard.  I am still not 100%.  Everyday is getting better and better.  I am having a problem with my red blood cell production (the kidneys produce a substance that goes to the bone marrow to stimulate red blood cell production).  Since my kidney's are still recovering, they are not producing enough and I am really anemic.  I was just started on a weekly injection which is supposed to boost the production of red blood cells, I am hoping it kicks in FAST!

I also started a Graduate Nursing Education program which I was super excited about in January - the day after I got discharged from the hospital.  I ended up deciding to withdraw.  I am now on a medical leave and can enroll within a year and start again.  I just felt like with everything going on, my health still not being 100% and my mental state somewhat in question - school was just not a smart option.  PLUS - I needed time to plan my DREAM wedding!!

On February 5th, my family lost an amazing man.  My Uncle Tommy died suddenly and unexpectedly.  It has been a really rough time and I know this is just the beginning.  My Aunt and Uncle and cousin have lived two houses down from me my entire life!  They have always been my second set of parents.  A refuge for when I couldn't take my house.  My uncle was the most caring man in the world.  I went on so many vacations with them.  He always tried to make my cousin Erin and I happy.  From driving and getting lost for hours just to find us stuffed animals that we wanted in Disney, to moving all of the living room furniture out so that we could roller skate around because it was too cold to go outside.  He was such a good hearted man.  I am still kind of in denial, I don't even know how my cousin and Aunt are being so strong.  I wish with all my heart that there was something I could do to comfort them, I just hope they know how much I love them and that I would do ANYTHING for them. 

My friend Lyndsey was in Australia when she had a little blip medically.  She also has CF and received a double lung transplant in April 2011.  When she came home she had a procedure and found out she had cancer (PTLD).  PTLD is a type of lymphoma that can happen post-transplant in various areas of the body.  She had surgery to remove the section of her colon where the cancer was, but is still waiting on blood and bone marrow results to see if they need to do chemo or anything further to treat it.  Lyndsey and I became really close friends while she was waiting for her lungs.  She was transplanted one month before I was put on the list.  She has literally become my lifeline and I don't know what I would do if anything happened to her.

I feel like it's okay if I feel pain and I'm hurt but I feel horrible when I see the people I love hurt.  The day after my Uncle's passing - I started seeing a therapist.  I've been against it for a while because I went when I was younger and had a creepy and bad experience.  That particular lady just stared at me like I was insane and expected me to do all the talking.  Now, I am really happy with my therapist and actually look forward to going to our sessions.  It's really nice to get everything (and there's a LOT) in my head out and to a person who is not involved at all in my personal life.  I now highly recommend it if you ever seem to be in funk.  

I still have a LONG way to go.  My PFTs are still down, I have lost about 15 pounds and I am still lacking energy due to the anemia BUT I do finally see light at the end of the tunnel.  Whatever else life hands me, for right now I can take it.  After almost dying in December I think I'm even tougher than before.  I kind of wanted to write this blog also to let people know that bad things happen.  Sometimes it takes a while, but eventually the pieces do fall together.  Sometimes you need a reminder from other people, sometimes you need to hole yourself up and close off the world for a little while and sometimes you just need to give it time.  EVERYONE has fears and is scared of things that they won't admit to.  I can honestly say - I am scared as hell that I will end up in rejection again and in that same state - but I can't let that paralyze me anymore.  I am trying to live each day and be thankful for still being here.  I am going to consume myself by spending time with the people I love, working out to strengthen my lungs as much as possible, going back to my job which I love and planning my wedding and honeymoon.  

March 9th 2013!

 Kristy, Myself and Lyndsey this past weekend celebrating Lyndsey's 28th BDAY!

It has been quite a while since I have written and I feel like a slacker!! Although lately, my life is chock full of events that I don't think I can really call myself a slacker or lazy. I wish I had more time to devote to this blog .. but when I don't have time to write, it's a constant reminder that I am out doing things and living life because I am NOT sick anymore!

 A lot has happened since the last time I wrote. We have the set the date for our wedding. I got my dress and was taped to be on an episode of TLC's Say Yes to the Dress! I am now working TWO jobs! I applied to graduate school to get my Master's in Nursing Education, I am hoping to start either in the Fall or by January.

 Okay so the health front .. My 6 month bronchoscopy showed MINIMAL rejection. I was treated with a quick steroid burst. I was slightly crazy and irrational for a few days when I was on 50 and 60mg of Prednisone but I am not back to my maintenance dose of 10mg, which doesn't really seem to effect me that much. At the beginning of this week I had another bronch just to check and make sure the rejection was treated with the steroids (and it was - NO MORE rejection!). They actually say with acute mild rejection that treatment with steroids is 98% effective - which is pretty amazing. The word rejection is so scary. But to have it be completely gone with just a little steroid burst and taper is not so bad.

 I started working again per diem in March at my old job in the NICU. With health insurance and wedding plans developing it was really time to start working full time. Unfortunately there were no full time positions available so I sent out my resume. I ended up getting a full time job in a great hospital in the Neonatal ICU. So right now I am on orientation for my new job and still working per diem when I have a free night for my old job! I have some amazing friends at my old job that I really don't want to part with - so it's fun to pitch in and work here and there when they need me. Not to mention the extra money doesn't hurt when you are have your own wedding coming up and a bunch of friends getting married as well!

 On to the wedding. We booked for next year - October 11, 2013! We are having the ceremony at my local church and then the reception will be held in Long Island right on the water! I went to Kleinfeld's in the city (the most amazing bridal store ever) and fell in LOVE with my dress! The episode will be airing during Season 9 which I believe starts in September. I will have an exact date soon and keep everyone updated!!

 And last but not least, yesterday I actually completely a 10K! For anyone who doesn't know conversions, that's just a tad bit over 6 miles! Joey trained pretty hard for it. I feel a little guilty because I didn't train as hard as I said I was going to, because life just got in the way but I finished and crossed the finish line in 1:30 minutes. Considering this time last year I was in a wheelchair and couldn't breathe without oxygen, it's a pretty amazing feat. Besides Joey, I participated with a few of my amazing cysters; Lyndsey, Piper, Bobbi and Gabby. Lyndsey and Piper are also both post-transplant and Bobbi and Gabby have not been transplanted and still ROCKED it!

 All in all, life is pretty freaking amazing right now. I couldn't be happier, I have so many awesome things going on and to look forward to! All this thanks to my donor Angela. For the last 8 months, her and I have made a fabulous team. I can't wait to see what the future holds and to take us on more adventures!! I also think I am going to start working on my letter to her family. I know it's going to take a little while to perfect exactly what I want to get out and make them see how much I love and appreciate their generous gift of life. I still can't believe it's been over 8 months since I got that life changing phone call.