Transplant Video

Thank you to the The Transplant Forum for putting this video together!

Mutations and Mom

--> Today I have an appointment at the cystic fibrosis clinic in the hospital where I had my lung transplant. I haven’t been to a CF clinic in at least ten years. And previous to transplant I only had one CF doctor my entire life, from my diagnosis to his death. Dr. Thomas Doland at Yale. He died shortly after my transplant. And I never saw a need to find anyone else. I belonged to my transplant team at that point. The interesting thing about having a lung transplant is that, for me at least, you sort of leave the CF camp and move to the transplant camp. Of course, I still have CF, and of course it still affects so many things, but my lungs no longer have CF, so it kind of eliminates the need to be followed continuously by a CF doctor I guess. Instead of seeing a CF doctor, I see my transplant doctor and a million other specialists—sinus ENT, ear ENT, cardiologist, several endocrinologist, kidney specialist, gastroenterologist, infectious disease and many more I can’t even think about. The roster is long—each doctor taking care of their specific organ.

But now my ear has really brought CF to the forefront again. There are several reasons I can’t get rid of this infection, but there is only one reason I got the infection to begin with, and that is cystic fibrosis. The pesky dumb aggressive little bacteria that is in my ear is pretty much a bacteria you can only grow if you have CF, a healthy person could lather in it; it wouldn’t do any harm. It’s the same bacteria that ate away at my CF lungs time and time again. And it’s the same bacteria that like to wreak havoc in my sinuses, and then found its way into my ear. But the point being CF has again found a way to rule my world, even though transplant is supposed to somewhat alleviate that from being the case, and trust me it did, I’ve been on IV medications, a rotation of three different antibiotics and one antifungal since December 19^th. I’m over it. And like all my ENTs like to say: we can’t transplant your _____ fill in the blank with ENT things. We have all done everything we can but we need a forever solution.

Since my transplant there have been a lot of advancement in medications that target the actual problem with CF—we can’t transport fluids and sodium properly in and out of our cells. This is something your body needs to do to be a normal functioning healthy person. There are two medications available now that try and correct the actual defect in the protein that causes the problem. My lungs don’t have CF so in essence I wouldn’t really benefit from a cure for CF, I now need a cure for transplant rejection—this is now my terminal disease. I hope this makes sense. So back to the ear problem that has led me here! These two medications Kalydeco and Orkambi COULD help my ear! So a few weeks ago my genius sinus surgeon told me he has seen big improvements in the sinuses of CF patients on these medications. I told him I didn’t even know what my mutations were.

So here I am a mixed jumble of emotions. I know I have a genetic disease. But it’s a different thing to actually find out specifically what mutations I have in my gene. I am entering back into a world that I haven’t been to in so long, even though I suppose it’s been there in the background the entire time. There are a lot of mutations and a big chance I don't have the right one, of course I’m scared of disappointment here. But this is the way medicine is headed and it’s thrilling to be honest, even if it doesn’t exactly mean a cure for my own lungs and me. The drug pipeline is exciting (https://www.cff.org/trials/pipeline)

There is more to it though and I’ve been trying to understand why I am SO emotional about this experience. And it’s because I got these CF mutations from my parents—neither of them alive anymore, neither of them with me. My mom should be with me for this. She should be sitting with me in the exam room like she always was before she died. I’ve never even seen a CF doctor without her and this is bringing it all back, everything we went through together my entire life. And it’s a visceral punch to my stomach. At the end of the day this loss is always circling me. The progression of life, these sorts of milestones, will always make the big losses slowly burn again, the ache roaring back. But I have to keep going and I have to keep facing these things and luckily I don't do it alone.  

Dear Mom

Dear Mom,

Mother’s Day is the hardest day of the year for me. It’s the biggest reminder of how desperately I wish you were here. I miss you every day but I guess today is different because I get to glimpse purposefully inside so many other daughters being with their moms and I don’t get to do that anymore.

I remember so vividly the gift I gave you on your last Mother’s Day—it was a picture of me as a kid, maybe 3, in a bathing suit and laughing by our pool. I was so happy in the photo, that was why I picked it—just a kid being happy, not a kid being sick. And on the photo in black sharpie I wrote I love you. You kept it on your nightstand.

I wish I had gotten to know you as an adult. I wish we had been able to grow together as I became me. I’m a different person than when you died. Your death made me a different person—kind, generous, more selfless and less selfish. It also made me more timid and more fearful. It changed completely who I was. I wish I were this person when you were alive. I was in my early 20’s, self-absorbed, and maybe even bitter because of my illness. I’m sure I didn’t listen to anything you said or treat you the best. I’m sure I said things I didn’t mean. I'm sorry. But no matter what I couldn’t function normally without you—you were my first call of the day and my last call of the night and several calls in between. And I still can’t functional normally without you. So much so that I mostly stopped talking on the phone after you died. I can’t stand that the other person on the end of the line is never going to be you again. I still scroll through the last text messages you sent me—the last communication we ever had over technology.  

I can’t believe the things I’m experiencing without you. It hurts my heart that I went to South Africa without you. Or that I spent the day on the Shark Tank set without you, because Robert would have loved you. As everyone did. He would have made a deal with you for something! You were dynamic, intoxicating, strong, powerful, smart and beautiful. You never took no for an answer and you were easy to admire. You lit up any room you were in. You were everything I hoped I would turn out to be.

Saying that I miss you just doesn’t seem right because it is so much more than that. I feel so incomplete without you. My heartbreaks for the things I never saw you accomplish, I’m always thinking how food has taken off so much now and you would be running circles around todays best restaurateurs.

Since I was little our thing always was saying to one another "I love you to the moon and back". And mom today I love you to heaven and back. 

 

loss and adapting

Prince William inspired me to write on this blog again. Please read this quote for context:

The prince, 34, who was 15 when his mother Princess Diana died in Paris, said: “The shock is the biggest thing, and I still feel it 20 years later about my mother.
“People think shock can’t last that long, but it does. It’s such an unbelievably big moment in your life and it never leaves you, you just learn to deal with it.”

I’ve written a few posts in the past few weeks but they are bone stabbing kind of deep. I don’t think they are ready for the Internet yet. But I think this is medium deep, haha! So it seems more appropriate. I watched this interview today with Prince William talking about the death of his mother—I bet we all remember this day. It made me realize more people experience the same losses as our own than we probably care to understand. It’s easy to think we are the only one that may have gone through a particular thing. But loss is universal, and the loss of a parent at a young age does actually happen to lots of other people. And it also does happen to other chronically ill people. I’m not the only person with CF to lose their mom to cancer. The thing is it’s all tragic of course, but it’s a punch in the gut on top of 100 other punches. What else can you do besides take it?

But the loss of a parent, especially a mother, is unlike anything else. It’s not like losing anyone else in your life. I came into this world attached to only one person, by an umbilical cord, by months together, by experiences in the past and promises of the future, and I felt it all violently ripped away from me when she wasn’t here anymore. (I am not diminishing people raised by someone other than their birth mom.)   

I think people assume I lost my mom so many years ago it must be behind me. And it's not. I don't talk about it with too many people but I'm still gutted by it every day. There are so many things that happen every day that compound her loss, from tiny to monumental, from crisis to joy. I've just learned to live my life with this gaping hole. And it's okay. You learn to adapt. That's what life is mostly about—adapting to anything that comes at you. The only real way to survive in almost any capacity is to adapt. And adapting doesn’t mean that things can go back to the way they used to be. It just means learning to move forward in a new way, in a new vehicle, or on a new path. And if you are lucky enough you have people that will follow along side of you.

Glamour Magazine

An essay I wrote was featured in the digital edition of Glamour Magazine! Please take a minute to read it and let me know what you think! Thanks!

oh, rejection always hurts!

I wanted to update you guys on my chest/back pain and consequent lung biopsy last week. I have some airway rejection going on inside my lungs. And I thank my body for sending off an alert sign to me so that we had a reason to go in and do a biopsy. I am starting treatment today of high dose prednisone (a medication I take everyday and will forever, but at a much much much higher dose than usual.) This should take care of the inflammation/rejection. Yes, of course this freaks me out. I haven't had an episode of rejection with these lungs yet and was hoping for a long long course of none of this, considering chronic rejection destroyed my last lungs. This is not chronic, but yes every case of any rejection makes chronic more plausible. However, this is a very very low grade and should be just a page in a very long book. 

So here is to my first day on 60 mg of prednisone, which will make me a crazy person for the rest of the month!

ny weekend visit part 1 - the lungs part

I had a few things happening in Manhattan Saturday to Monday - spending time with Aly, a paint bridal event for Laurie, and my second annual lung transplant testing for my new lungs! This weekend included lots of eating too, that I will blog about later. I wanted to first write about lungs, since I actually don't share THAT much about that aspect of my life...

First I can't believe it's been two years since my second double lung transplant. Time flies! Monday I had to do the following tests: bone dentisy scan, chest CT scan, full PFT (pulmonary function test), the normal xray and blood work, a 6 minute walk, an EKG and an echo. It was a full day! 

My CT scan looked really good. This always makes me nervous because in my 5th year of my first lungs all of a sudden I had spots all of my lungs in a CT scan, and then sort of started my downward spiral. But this one looked really good!  I wish I had a picture to show you guys. I forgot to capture this.

My 6 minute walk seemed good. It's much easier to do this without the aid of oxygen. I hadn't done this test yet with these lungs so there really is no base line - this is my base line. I can't tell you if I did better or worse than last year. But it did kill my leg muscles. Eleven years of all the medication I take and my muscles are pretty sad. 

My EKG looked good! The heart is still pumping! We are trying to figure out why I'm having serious palpitations at night even with the aid of two heart medications. We'll figure it out eventually!

My PFTs are pretty normal.  My lungs are at around 80% function - which is not where my first lungs were, but it's still great and I've learned to be okay with this after much internal conflict and anxiety. My lungs feel pretty good most of the time and this is what matters.  

The only test that came back that was really concerning to my team was my bone density scan. Just a quick explanation that I've had "bad" bones forever, this is a complication of CF and transplant and transplant medications, but since the last time I had a scan - likely 4 years ago, my hip bones in particular have drastically taken a turn for the worst. I am being set up to see the bone team there. I always have really bad lower back pain, but to be honest I just assumed it was a side effect of my life (i.e CF, meds, transplant) and never complain or say anything about it. It can get really severe too. I probably should have mentioned this years ago but alas we will figure this out too. 

So all in all the lungs look good. There is always something happening. This is transplant life. Besides the bones we are still trying to figure out if my stomach wrap has come undone and I'll go back for another test in a few months, but seriously I am breathing. Transplant is trading one disease for another. They don't give you lungs and set you free and everything is like a normal person. I still have CF, I still have a lot of problems, but the most important thing is they are all manageable. And so far I've come up against nothing I could not handle. But I hope this is a message for those of you that are healthy - literally something is always going on in the body of someone who has had a transplant and rarely will you hear one of us complain about it (like many other illnesses). Please think about that when you evaluate your own life and how lucky you probably are and how thankful you should be for your healthy specifically. I'm so very thankful for all of you that have followed and supported me along the way! I wouldn't be able to do it without you guys!

82% lung function - looking good!

They are filming at Columbia! Duck for cover!

A sweet message from Dylan's Candy Bar - my favorite

       

A little secret news...

I wasn't sure if I was going to write about this, because it's not that big of a deal, but I've only told one person so far! But I almost got a call for lungs already.  Crazy I know.  The average wait time is 6 months - 9 months, but last week the two people ahead of me that are my same size and blood type, well one was "not available" and one turned down the lungs, so that left little me! But it was an offer for a single lung only, and well I didn't even know I could get just a single lung (the first time I could only get double, cystic fibrosis only gets double lung transplants) but I guess in the case of chronic rejection you can get a single, but anyway, my doctor knew I wouldn't accept a single so they didn't call me.  But I still find it amazing after like 2 weeks on the transplant last my name even came up! I'd have to be really really sick to take an offer for just one lung (and I mean at that point I would be high on the list I would think - the sicker you are the higher you are - so....) but yeah! Exciting and scary! In other news I think my abscess is actually getting smaller today, and the pain is minimal so I think the antibiotics are finally working! Hooray!

I hope you are all having fun in the snow :)

Me and my buddy

Today I took my little mini oxygen out with me for the first time in public. It brings about a feeling of relief and anxiety. The relief comes from the fact that passerbys see that I am really sick as opposed to the anorexic thoughts that I know float through peoples heads when they see me, or the snide looks I get when I park in a handicapped spot - well trust me those stop when a 27 year old steps out of a car with oxygen on. All of that equals a lot of relief in what other people think. Why should I care? Who knows but I guess I do. There is some anxiety that comes along with it because EVERYONE looks! It's like you know that old saying: take a picture it lasts longer!

Well this is the start of me and my new buddy...

Would you want to know?

I'm so sorry about my lack of posting! I've been feeling a little under the weather with a crazy sinus infection....UGH! And my lungs are declining I can feel it, so things are taking more effort, I am more tired, sleeping more etc.  I have a visit next Tuesday and I would not be surprised if I was back on the list within the next two months.  Last time I was there my doctor asked me a question that I wanted to ask you all - we were talking about certain scenarious and things that could happen and what would I want in these cases and one of the questions that came up was: if you were dying would you want to know? In other words if I was in multi organ failure and dying and pretty much sedated and not really with it should she wake me up to tell me I am dying.  Would you want to know? At first I was like Oh God No! Because when my mom was dying the last thing I would have EVER done was wake her up to tell her she was dying.  Never would I ever have done that.  But the more I thought about it, the more I wasn't really sure.  What do you think you would want?

Lung Update

I am going to try and post every day this week, I know I have been MIA.  I had a check up last week and I wanted to update you all! My weight is stable - hooray for that! My lung function is down to 28% my oxygen is level is getting a little low, but not enough to need oxygen full time yet...So as usual things are steadily declining.  I am having crazy night sweats lately so we are trying to figure out what that is coming from.  They tested me for a few different viruses which all came back negative so now we need to try and figure out what else it might be.  Night sweats always scare me because they make me think of lymphoma but it seems pretty obvious it has to be coming from some sort of infection.  I'll keep you updated! Thanks for checking in :)

Lung Update

So we've finally had some movement in my lung function - I've been pretty stable at 33% for the past few months I think, or somewhere around there.  I was at the doctors last week with one of my bffs Lindsey (who comes with me a lot so I want to give her a little shout out!) and my lung function has dropped between 29% and 31% (they used two different machines but the worst would be used if they listed me.) That means that it makes sense that I feel a little crappier and I am slowly moving closer and closer to getting on the list.  We calculated what my score would be right now if I were listed and it was 39, and most people on the list are in the high 40's (the higher the number the closer to the top you are.)  In really good new my weight has stayed at 83 pounds for months now and I am working really hard to ensure I don't drop any or I know I will be strapped down and forced to get a feeding tube - this is something a foodie does not desire! This is the update, inching closer and closer to breathing again...

This is what 3000 calories looks like

And it's not even that much, I slacked on my snacking usually I snack way MORE!

Is that like Facebook?

A friend of mine is on a diet and using this website SparkPeople to add up the calories she eats everyday.  Well we had this idea that it would be good for me to track what I eat to make sure I am eating enough.  I should aim for 3000 calories a day at least.  Well I did it today for the first time and moral of the story is: I am NOT eating enough! I thought I was consuming so many more calories than I am!  I need to kick this eating plan up a notch and get higher calorie foods and fattier foods.  Hopefully I stick with the tracking, it's a little tedious.  But when I saw my friend doing it I said "is that like facebook or something?" having no idea what the website was really for.

Do you guys track your calories (those I am sure trying to lose weight) it's kind of eye opening! I thought I was eating so much more!

Sometimes family is absurd

As my friends know my mom passed away three years ago, new followers I need to let you know that before I start to have my first real bitch ranting post.  So for my first transplant my mom was my everything.  Now that I don't have her I have a small circle of family friends and my best friends that are supporting me and helping me as I go through this process again.  Now my family on the other hand, I never knew how in denial most of them are.  I've seen it over the past few months that they don't really absorb the fact that I need new lungs and chronic rejection doesn't go away.  But I had someone say today that a key member of my immediate family "thinks I am just going to get better" I'm sorry. WHAT THE FUCK is what I have to say to that.  I am NOT getting better.  The only thing that will save my life is another set of lungs.  Chronic rejection does not stop, it does not heal itself, it does not reverse itself - once it starts it just keeps going.  I am looking at probably being put on the transplant list between the next 3-6 months at the rate things have been going.  Like I have people say things to me that insinuates I might be years away from needing a transplant or I am not really that sick and I just look at them like "you're a fucking moron" I'm sorry it's not only frustrating but it's insulting.  The people that care about me and love me should ask me, or go educate themselves, and my family has already been through this once, so they have no excuse.  I don't know how to deal with it, but it's getting to a point where it's beyond frustrating and it ends up upsetting me.   It's time to get on board and support me and realize I am NOT getting better! What would you guys do? How would you deal with it in a tactful way?

Lung Update

I completely forgot to update all of you on my list visit.  I am steadily declining in the same way, losing about 1% of my lung function every 3 weeks.  But the difference is at 33% you feel it much much more than at 50%.  So even though I am not losing a lot of function, I can really feel even just 1%.  I am at 33% now, and I lost 5 lbs which is NOT okay.  I got read the riot act a little bit that if I lose anymore weight they won't list me.  It's not my fault I am losing weight clearly, the more lung function I lose the more weight I will lose, that is just the nature of it when my lungs are working so hard, but I need to just be eating like a cow - all day long.  I have been eating so much since my last visit, I don't want any reason for them not to give me new lungs.  I think yesterday I ate 20 munchkins, haha.  And you will see lots more high fat cooking of things I don't usually cook (i.e. I don't really bake.) because I really need to at least maintain my weight.  I am back on the 21st.  And the journey continues...

For myself

I am writing this post basically to myself.  I want to remind myself how hard it is to breathe today, and how short of breathe I am and how crappy I feel and how much I hate it and how much I can't wait to just breathe and be normal and do everyday things again.  I want to be able to read this when I am up at night scared of having another transplant, scared of the process, scared of the waiting, the unbearable pain, and everything else.  I just need to remind myself how hard today is because it makes it harder to be scared. 

Who am I anyway?

I just want to post a little blurb about myself for those of you that are going to be following this and don't know me (yet.) The reason for the breathing part in the title of this blog, is that I have Cystic Fibrosis and had a lung transplant 6 years ago. I am currently undergoing the process of preparing for another transplant.....and I will chronicle that in this blog....The second part of the title of this blog is because I use butter in practically everything I cook and I think it makes everything delicious! It just makes life better. And I do a lot of cooking, which I will also chronicle in this blog! (Along with other things) but breathing and butter are two of my favorite!