Accepting That It Is Time

I always told myself I would be uncertain about whether or not I would get a transplant until the time came for me to need one. Now here I am, hanging out in transplant land, and I am having a hard time accepting the reality.

I feel like I can still do a lot, like I have a good quality of life. I go out, I see friends, heck, I went to the beach for a week last week! I can take deep breaths in and out (sort of.) I can take walks around the neighborhood. All of these things make me feel normal-ish and happy.

From what I've gathered both this week and elsewhere, the consensus seems to be that I am either in or very close to my transplant window. I will know more about that on Tuesday when Duke conferences about me and lets me know my status with them.

Will I ever feel ready? If Duke was ready to list me on Tuesday (which is not going to happen just yet, I know that much), would I be okay with that? Frankly, it scares the hell out of me. How do people do this??

On one hand I know I can do it. But on the other hand it still feels like something that belongs in the future.

What a difference a year makes

When I think back to how my health was a year ago - even six months ago, the differences are shocking. I was working, we could go up to the mountains, I didn't have this insane amount of coughing to deal with, I had the energy to go out for more than just short bursts.

But I have realized the flaw in my thinking: as much of a rock star as I may think I am dealing with all of this, I really would not be able to get by right now without an incredible amount of help from Todd, friends and family who have stepped up (thank you!!!) Yes I have a good quality of life, but no I do not go grocery shopping or cook much or run errands anymore. My life has become much more limited than it used to be.

So this is what I have been thinking about this week and working on - acceptance. It's a hard thing to come to terms with, and I hope I'll be able to do so without suffering a big health decline that leaves me desperate.

Header photo from tariqmcom.com

Friends & Family

Hanging out with Simon at Myrtle Beach

My week at the beach was awesome. Got to relax, spend time with family, eat good seafood, sampled plenty of key lime pie, possibly had some of my last days of sunbathing (can't be out in the sun after transplant) and discovered the joys of non-alcoholic beer! But the best part was getting to spend so much time with my almost 3-year old nephew Simon.

Simon and my brother Nathan playing mini-golf

My mom (grandma!) reads The Nose Book to Simon while he holds his purple elephant

Simon and his mom JoEllen bundled up after a swim at the pool

I am glad to have a couple of down days now before my last two days of transplant evaluation on Tuesday and Wednesday. Tuesday is a day of more or less regular pulmonary clinic testing (x-ray, blood tests, arterial blood gas, pulmonary function tests) and important consultations (cardiologist, pulmonologist and surgeon.) Wednesday might or might not be a long day of heart tests depending on whether or not they want to do the left heart catheterization. I will also be getting an ECHO cardiogram and an EKG.

And then, I wait. For a week. Until the following Tuesday (June 3rd) to find out their recommendation(s). Possible recommendations include: 

1. I am ready to be listed (not likely)
2. I am looking good as a candidate but need to do X, Y or Z before they can make a final decision
3. I am looking good as a candidate but am not yet ready to be listed - come back in three months
4. Something cropped up during the evaluation that makes me not a good candidate and they will not be listing me (very unlikely)

My personal guess is that their response will be a combination of #2 and #3. And in the meantime, I continue going to the daily rehab in Durham.

My Best Friend's Wedding

Fortunately there will be a lot going on to distract me. My oldest best friend from Michigan, Erica, is getting married on June 7 in Greensboro. I am the one and only bridesmaid. I think it's going to be fantastic.

However, being in end-stage lung disease is far from the ideal time to have this honor bestowed upon me. So far, I managed to throw an excellent bachelorette party in the mountains in February...barely! I went wedding dress shopping and bridesmaid dress shopping with her. I went to the engagement party and there is a wedding brunch I hope to make it to next weekend.

But a lot of that has been just me showing up. Aside from making the wedding favors (60 small bars of 808 soap!) I haven't been able to do nearly as much as I would have wanted for this wedding. When Todd and I had our commitment ceremony five years ago, Erica was my right hand lady and she did so much to ensure the big day ran smoothly and that I was happy and able to enjoy things. I had total confidence in her. And now I wish that I could return the favor. Aside from showing up with my bars of soap and making a toast, I'm not really going to be able to do much of anything. Honestly, just showing up and making it through both the rehearsal dinner and wedding day is going to be a major accomplishment.

Me, enjoying newly discovered Coors non-alcoholic beer, while my friend Erica sips on a margarita

I know this is how it is - these are my limitations right now and fortunately I have a friend - friends - who understand and who are fine with picking up extra slack. But that doesn't mean it still doesn't make me a little bit sad.

So while it's a once-in-a-lifetime event for my friend, to me it will also be another weekend of making sure I plan for enough portable oxygen, trying to keep up with my insulin pump and an irregular eating schedule, doing enough chest PT, getting extra sleep, taking the pills that make me cough less/keep my back from hurting/give me extra salt in the heat and trying to not cough so hard that I pee myself in front of a bunch of people in an expensive dress. In other words, it's hard to give someone else the attention they deserve then you are so busy trying to take care of yourself. What did I tell you? My health is a full time job! With no weekends off.

A Mix of Emotions, But Mostly Really Really Happy

This week I both completed four days of transplant evaluation appointments at Duke and bought a fabulous new car. I am feeling relieved to have made it to the end of the week. I have two more days of testing when I return from the beach, but I have 10 whole days to just relax until then and play with my 2 year old nephew. **big smile**

Reflection

I drove to Durham for the first time by myself in my new car today - it was just to have a nose catheter thing for my 24-hour esophageal pH study pulled. As I was driving home, it was my first real chance to think alone after everything that's happened this week.

I started to get a little emotional. I felt overwhelmed by the amount of love and support I have gotten as I've started this journey. People have reached out to me and offered to help, some I didn't even think were that close of friends. My close friends have been amazing - incredibly understanding and supportive. And, importantly, they allow me to keep some semblance of normal in what I jokingly refer to as my convalescent stage. My family has continued to be some of my biggest cheer leaders. And Todd has - as always - been my rock, my lovable Buddha, my most trusted confidant.

As hard as it was to start asking for help, people have been so happy to do it! They run errands for me, bring me delicious home cooked food, come clean my house. Later on in this process they will be carting me back and forth to Durham.

Seriously, How Jealous Are You of My Life??

A natural filtering process has removed all but the very best, most awesome, supportive, loving, kind, considerate, authentic people in my life. How lucky am I? If only I could have this AND my health life would truly be unbeatably amazing! But of course it is because of my health that I have gotten this clarity.

Similarly, all of my worries, concerns, things I tried to control that I couldn't, and to some extent my fears about the future have dissolved away. I will not let one single ounce of negative use up my valuable health and energy. I feel free.

Of course, as a part of this process there are people and things that could not come along. And for some of this, I am still sifting through sadness. I am grieving disappointment. I am feeling the loss of all things left behind.

But other than that, my gosh, I've accomplished this huge thing! Going from being unsure about transplant, scared, my health on a downward trend to being here and now, and dealing with everything (at least trying my best!) including transplant one day at a time. Coincidence or not, my health also seems to have hit a plateau for the time being.

So if you're one of the amazing people mentioned above - thank you! And if by chance you are someone in one of the other categories, well, I hope you understand that I have to do what's best for me right now. Preparing for death and one's (hopefully) impending rebirth makes one have to be a little selfish.

I want to end this blog on a positive note: I am so so happy right now!! And really, now that I have my brand new Jetta, there's nothing else in the world I can think to ask for. :)

Busy Bee

This is what the inside of my new car will look like!! First VW, first diesel, first BRAND NEW CAR!!

Two weeks ago we got married, this week we bought a car, next week I start transplant testing and the following week I go to the beach - how crazy is my life right now? And this is while also juggling my bridesmaids duties for my best friend's wedding in early June, continuing with the arduous process of applying for social security disability insurance (SSDI), getting our wills done, starting the new pulmonary rehab program in Durham and on and on.

My car! I got a 2014 Jetta TDI (diesel) manual transmission car - very fun to drive! Very cold AC! Light color outside and exterior to help it stay cooler in the summer! (good for my breathing) Plus, lumbar support in the seats!! (good for my back) It also gets great gas mileage especially on the highway. And it's a sweet ride...nice and new...I never thought I would have a new car EVER.

The timing may seem strange, but we have been thinking about it for a long time and I have done a ton of research. Now we finally know what our income will look like with me not working and I am in need of reliable wheels with all of these trips to Durham. I HAVE A NEW CAR - AWESOME AWESOME AWESOME!!! (I won't pick it up until early this week, hence the stock photo above.)

The good news is that I have managed, for the most part, to not totally wear myself out with all that we have done. And it has been so awesome! The getting married and the car I am super excited about. I am also actually a little bit excited to start my evaluation.

I thought I might get nervous, but so far not. I am taking it one day at a time. Here is a run down.

Patient is positioned, laying down, into the circular part. Then a die is injected into you making your whole body feel warm while it takes pictures.

Tomorrow I have only an orientation class, a scan and a meeting with the nutritionist - and believe me, there will be a lot to talk to her about with all of the weight I have recently lost. The worst part of it is that we have to be there AT 8 am, which means leaving here at 7 am, which means getting up at 5:30!

The next day is also short: a CT scan of my lungs followed an imaging of my upper GI tract.

This shows someone's intestinal tract in the Barium study

Wednesday is a bit longer: I have an ultrasound on my liver (people with CF an also have liver issues - hopefully it looks good or else they may want to do a biopsy!) Next is meeting with the infectious disease doctors. We will be discussing my bacterial resistance and how to best deal with that during and after surgery.

Then I meet with the social worker who meets my mom and Todd (primary and secondary caregivers), makes sure we are capable of getting through post transplant at home, makes sure nobody smokes, and just basically inquires about your support system. Lastly we meet with the financial councilor. I hope for this to be a fairly uneventful meeting with lots of good news. Meaning, that Aetna has agreed to cover my transplant 100%.

Thursday starts out with what I have heard is the worst of the tests. It's called a manometry pH test. I have renamed it "the barf test." Here's why. The first part they put a small tube into your stomach through your nose and check the pH levels there. Then they have you swallow sips of water while they watch how the esophagus behaves. It's supposed to make people really nauseous and if you barf you have to swallow it again. The good news is it's only 10 minutes or so. The second part is that either with the small tube already in your nose or another one they measure the pH in your stomach over a 24-hour period. So you go home, eat and drink "normally," record what you eat, and then come back the next day for them to take it out.

Next Week: Beach! Beach! Beach!!

The next week, we are off to the beach for the week with my mom, brother, sister-in-law and nephew. Actually, Todd can only go for the first part sadly. I am super excited if you can't tell.

Right now I am feeling good about my energy level. I hope this holds up. I am going to be very diligent about rest this week. My lung function numbers are still really low, but I have been less short of breath this past week, I am coughing a little less and my energy is better. Yay!

When I get back from the beach, I will have Sunday and Monday (Memorial Day) to recover, and then two more days of transplant testing. Tuesday is super busy with labs, PFTs, xray, and a cardiac consult (for Wednesday's test) and meetings with both the surgeon and pulmonary doctor.

For this test, a catheter is threaded up through your groin to your heart (only on one side, not both). They look around and make sure everything's working alright. Fortunately you get conscious sedation.

Wednesday is the other day tied with manometry for most fun: the cardiac cath. I don't actually think the procedure will be a deal at all, I will be twilight sedated so I won't know what's going on. The hard part is that you have to lie flat (problem!) afterward and avoid coughing (not even sure how that's possible) so that a blood clot doesn't break off into your leg. Ugh! I will definitely talk to them about all of this beforehand. And surely I am not the first cystic to have coughing issues when I lie flat...or the first one to not have to cough for two hours. I probably cough every two hours in my sleep!

After that, my life becomes all about the 1:30-5:30 daily rehab sessions in Durham and preparing for my best friend's wedding on June 7. It really wasn't bad, the one I went to last week. Very comparable to what I was doing before. Then that next Tuesday (June 3rd) the committee meets to discuss all of my tests and decide about me being ready to be listed or not. I am putting the odds at 70% they won't list me, 30% they will. That is assuming nothing has cropped up in the testing.

So yes, that's what all is going on. Wanted to fill you in about the transplant stuff in case I don't get a chance to blog for the next couple of weeks.

Thank you everyone!!

My Amazing Week (Last Week) In Fuzzy iPhone Pictures

On the fifth anniversary of our commitment ceremony, May 2, Todd and I got legally married!!

We didn't want to make too big of a deal about it - we just went down to the court house with my mom and my longest best friend Erica. Everything was smooth and fast and then I was a Mrs.! This is going to make our legal and financial lives so much easier - as well as the the transplant process.

Quick Update About Other Things

I am feeling pretty good these days. I actually feel fairly stable since coming off the IVs two weeks ago - yay! Don't get me wrong, I still have limited energy and frequent coughing fits but that is just a part of life for me now.

I am officially starting lung transplant evaluation at Duke on Monday!! I may blog more about this over the weekend, but just wanted you all to know. And I officially started Duke's pulmonary rehab program on Wednesday, although today will be my first full day of exercise.

Some Things I Liked About Duke

I had my initial Duke appointment for lung transplant evaluation this week. The day consisted of an introductory meeting with the pulmonary rehabilitation folks, some basic tests (lung functions, x-ray, a blood draw and a 6-minute walk) and a meeting with the head pulmonologist. Mom, Todd and I were there for about seven hours total. Long day!

So many things were going through my head as I sat in the back seat with my big oxygen tank on the ride home. I felt numb and discouraged...  I could not wrap my brain around how this was all going to work out, and all of the pictures of transplant I'd had in my head suddenly seemed like looking back at old black and white photographs. Was I sure this was really for me?

Probably some of that was fatigue. And the fact that one should feel overwhelmed after a day of going from clinic to clinic, seeing all new faces crowding into a waiting rooms filled with the ghosts of Christmas past and future. It was like all of the progress I've made toward warming up to transplant was stripped away.

The Next Day

It's a thing with me that I hit rock bottom before a big upward swing. And fortunately I woke up the next day with less doubt, fear and feeling less overwhelmed. Let's do this!

I emailed my transplant coordinator some bullet points I wanted to be sure the pulmonologist understood about my current state of decline, went to pulmonary rehab and continued talks with Todd and everyone about what all of this means, what it might mean and what all that means about what we should do now.

Looking super dorky with the pulse-ox around my forehead before my walk test

The Outcome

Ultimately, we and the pulmonologist agreed that it was the best thing for him to recommend me to go ahead with a full evaluation. There is a committee made up of the doctors, transplant coordinators, social workers, pulmonary rehab folks, et al. that meets every Tuesday which makes decisions about who to accept (and, after evaluation, who to officially list) for transplant.

They will discuss me on Tuesday - I should be a pretty clear case since there are no "deal breakers" with me at the outset, I am clearly either very near or at the start of the window for transplant evaluation and (I hope) because they have realized my awesomeness and what a good candidate I am in their short time knowing me. I should hear either way from my transplant coordinator by the end of next week I hope.

Assuming that I get the green light, scheduling will then begin for evaluation stuff in the coming weeks and (at some point) they will want me to start their treacherous month-long pre-transplant pulmonary rehab program. I am confident after meeting everyone that they will not let me  deteriorate waiting for the four weeks to be up if I continue to have these (pretty much) recurring lung infections causing more decline. Physically, I am fit enough by their standards to undergo transplant; anything else is icing on the cake as far as I am concerned.

Some Things I Liked About Duke

And now for promised things I liked or was impressed with on Wednesday:

• Everyone was super duper good about gloves, sanitizing, washing hands - of course that should be expected but I was impressed nonetheless.
• For every test I had, results were printed out for and given to me to keep without me asking! That was like a dream come true for me - I am always trying to scribble things down in my notebook otherwise and then later can only half-interpret some things.
• With all the tests I had and places I was shuffled around to, I felt like they were really well organized...they never lost track of me.
• The doctor was very thorough, and I have since seen the same thing from my transplant coordinator although I have yet to meet her.
• I came across some really nice, helpful and caring people there who were also really good at their jobs.

Some Things I Didn't Like So Much About Duke

• Long walk to the transplant clinic with end-stage lung disease, no nearby parking, valet parking with a huge waiting line.
  
• Crowded waiting room filled with people who were not wearing masks - what?!?
• My appointment with the pulmonologist was kind of strange...it was as if he had his own way the appointment was going to go in his head but that he wanted to keep that a secret from us. I wasn't sure when to talk, when to let him type, and he did not give me any clues. He was thorough, but I also left feeling like he missed the big picture of why things have become so serious so fast with me - part of that was my jumbled mind at the end of a day's worth of appointments, but part of it was him, too. I do not want to form an opinion of him based only on this one meeting though.
• Arterial blood gas draws at every appointment - yikes! For those who do not know what that is, click here (note, I will spare you an informational page with photos for those who are squeamish of needle photos like I am. Suffice to say, not fun.)

Overall, we all left feeling like we had come at the right time, and that we were in good hands. And while they told me there was nothing more I could be doing to be a better candidate, I have decided to try and start doing pulmonary rehab 3 instead of 2 days a week in Raleigh. And I am making a better effort to get my blood sugars under better control - things have just been wacky lately and it doesn't help that my sugar intake is higher than it should be.

Other than that, continuing to talk about and process things with my friends and family, doing IVs every 8 hours, living life as normally as possible these days and still holding onto some hope that things may improve or at least stop declining so that this won't all feel like it's happening so fast.

Springtime in Raleigh

Although I continue to struggle with low energy, poor lung function and also had some reactions to my increased antibiotic dose this week, overall things are continuing to go pretty well.

Not to be under-stated is the importance of me continuing to have and do fun things in my life. I try to have a very deliberate balance of blue (appointments) and pink (friends/family) on my calendar. This week I Skyped with both my dad and my little nephew; I visited with my mom and her new cat! I had dinner out with friends; we had friends over for dinner and played a board game; and this weekend I have plans to make soap with my friend Elaine.

Sully the cat tired out after playing with Aunt Laura

By far the most difficult thing this week has been pulmonary rehab and - by extension - any days I was not breathing well but had to walk around and or do stuff anyway. Overall I have been better since starting this course of IVs (Tuesday will be two weeks on them - I will probably go for one more week after that), coughing less, less congested.

But as has been my pattern with IVs, my numbers are not getting better and I'm still honestly sometimes having a really difficult time just doing everyday things (showering, getting dressed, talking after walking from one side of the house to the other). My PFTs have gotten to an all-time low. I really feel like my lungs have a mind of their own and it doesn't seem to matter how much airway clearance, exercise and rest I give myself. My only reward for all this hard work is knowing that things might be worse if I weren't doing it!

My appointment for Duke has been set for Wednesday and I could not be more excited. I continue to feel like if I can just get to Duke (without a major problem beforehand) that things will be okay...  I just need to get under their wing and things will be alright. It's crazy how much I've declined just since I started this process with them a month ago.

What I mean about a major problem is that with all I know about end-stage lung disease complications - lung collapse, pleurisy, retaining carbon dioxide - part of me feels like I am waiting for the other shoe to drop.

One scary thing did happen this week, but fortunately went away as quickly as it had come. I often wake up at night coughing or from coughing, and sometimes I end up sitting up in bed to get the gunk out. Also, it's not all that unheard of for me to cough up blood from time to time - it happens about once a month or so, but I'm lucky that although the severity varies, my bleeds have always subsided fairly quickly. There is almost always a discernible trigger (having been around smoke, quick change in blood pressure from laying down or bending over, etc.)

Tuesday night I woke up coughing, sat up in bed and then realized it wasn't the usual stuff I was coughing up - it was blood. It freaked me out because it was one of my larger bleeds and because there had been absolutely no trigger. I had just been laying there asleep! The only thing I could think is that since the dose of my antibiotic had recently been adjusted, maybe a patch of infection broke free and exposed some raw, inflamed lung tissue. Who knows. But waking up to coughing up blood like that is definitely not cool. *Please other shoe, stay on foot.*

Anyway, I am trying to taper my expectations for Wednesday's appointment at Duke - after all, I'm not supposed to get any official answers about transplant eligibility or time frame then. (That won't happen until after the committee meets and discusses me the following week.) Although I am hoping that since I am meeting with the head of the program I will get more information and answers than I otherwise might. But seriously, I don't just think I'm a good candidate. I also think that I am getting close to the window of opportunity for being transplanted.

So...here's to another week of sunshine and thunderstorms, pollen, cool sunny days and humidity that's just a taste of what's to come...it's springtime in Raleigh.

Pictures: Owls in tree, clipartbest.com; Sully, me; Owl in hat, cutepics.org; Owls on branches, behance.net.