Hearing the Right Thing at the Right Time

The Big Appointment

I wanted to talk a bit more about Thursday's "big appointment" with one of the head pulmonologists at Duke - big because this was the appointment which could have (and did) give me the final green light that I was ready for transplant. (Note: this is not the same thing as officially being listed yet.)

There were two really key things that happened for me there.

First of all, this nagging feeling I've had (and my mom even more so) that I'm not quite ready to be listed yet...how much I can do, how fit I am...was finally (I hope) abolished. The doctor said first off how it was strange to be arguing this side - usually people are anxious to be listed and Duke is telling them all the reasons to hold off!

He said that it was obvious by looking at my how proactive I've been over the years and what good care I've taken of myself (Aww, shucks!) A lot of people with CF get to transplant stage and they are in nowhere near as good of shape as I am. But don't be fooled, he told my mother, she's sicker than she looks.

He went on to explain. Everyone with CF will reach a tipping point - the point where you end up hospitalized, in ICU, your carbon dioxide retention goes way up, you need bi-pap or to be intubated... While getting lungs at that time is still possible, it becomes a crisis situation/race against the clock and recovery from surgery in your weakened state becomes much more difficult. I am personally hoping to avoid all of that if possible.

For me, the tipping point could be in three months or in three years, there's no way to tell for sure. But because of all I have going on, it has become too risky to wait around for that tipping point to come: I have started to retain a low level of carbon dioxide; my PFTs, while actually up 10%! have been on a consistent downward trend; I have increased oxygen needs; and I am on antibiotics a lot - now with the additional complication of needing to be desensitized to any drug I will ever need in the beta lactam family.

It suddenly all made perfect sense to me. And to my mom as well, I hope.

There's Just This One Other Thing...

There was just one more thing eating away at us. This week, the CF Foundation announced success from phase three studies of a gene therapy for my mutation. They hope to fast-track the medication through FDA approval and have it on the market by early 2015.

It's hard to hear there is something that could help you - or could have helped you - when you are getting ready to be listed for a lung transplant. It's even harder for my mother who has waited literally 32 years for something like this to come along.

I had to see what the doctor had to say about this. Could this help me? Could it mean putting off transplant a while longer?

The fact is, there are way too many unknowns still. We don't know when it would be approved for sure, we don't know how well it would work on people with severe lung disease (it's only studied in the more healthy population) or even how safe it would be. Upon closer examination, the trials did not even significantly improve FEV1 in the participants. With all that in consideration, the doctor said, I'm just too far along for this to help me.

Again, I knew that, but I just had to hear him say it. Before I put all of my eggs into this one crazy basket. I felt so much better.

And I really have continued to feel good about things since then. I am much more relieved, optimistic, even starting to look forward to what my new life could be like.

Look for more blogging very soon!

p.s. I will continue to keep my What's the Latest thread updated with any daily developments in the coming weeks.

What's the Latest? (Part One)

With transplant I trade a world of treatments and oxygen for a world of pills and unknowns.

To my friends, family, acquaintances and other people following my journey:

I have decided to create a page that I can link to with more specific information on what's happening now that I am listed for transplant. The newest information will always be at the top.
I love you guys!! 

**For information since I have been listed, please see Part Two of this thread**

Thursday, July 17 - listed
I am LISTED! This is both incredibly exciting and one of the most terrifying moments of my life!!

What can I expect? Half of Duke's listed patients receive lungs within two weeks of being listed. The lung allocation score (LAS) is the single most important thing that dictates priority on the list. However, blood type, blood antibodies, body size and whether or not you are willing to accept higher risk organs also play in. I'm sure a lot of you are wondering about the term "higher risk" organs - I will expand on that if I continue to have more time to wait.

Wednesday, July 16
I thought today would be the day I was listed! But it was not. 

Had my final appointment with one of the surgeons today - it went really well and also ran really long. I was almost an hour late to my infusion that was scheduled afterward. Anyway, I waited and waited and waited for my coordinator to call and say she'd listed me - or really anything! I tried calling her and figured something must have come up. Turns out the surgeon's notes from today were not yet entered into the computer so that my coordinator could see them and get his final "okay."

Five o'clock came and went... Todd and I met a friend out for dinner, and then went to a movie to distract ourselves. I am going to touch base with my coordinator in the morning and we will proceed with listing as long as I am not feeling any ill effects from the infusion I had today (it's to strengthen my bones.)

Monday, July 14
I thought I had jumped all of the hurdles and was going to be listed today but no! My coordinator came back in town (vacation last week) and said that I need to come to clinic on Wednesday for a final set of labs, an EKG and to have a final meeting with one of the surgeons.

So, we keep waiting! Patience is the name of this game I realized long ago. It also helps that I am not desperate for lungs or else I may be singing a different tune.

Had a great day at rehab today! Walked 31 laps (just over 1.5 miles) in 30 minutes! I was worried that not exercising most of last week would set me back but apparently not! It is SO SO SO nice to get out of rehab at a reasonable time, to be able to run an errand on the way home if I'm up for it and to NOT be sitting in rush hour traffic. :)

Thursday, July 10
My admission to the hospital was ultimately successful but not without a lot of frustration. I waited 20 hours after being admitted to find out that an admission may not have been necessary. Long story short, desensitization was probably neither necessary nor would it help my itching and mild outbreaks from the Meropenem. But ultimately having me admitted was the fastest, safest route to get Meropenem on board with me.

So there was that. Instead of a full desensitization they simply gave me a 1% strength test dose (while closely monitoring me) followed by a full strength dose (also under close monitoring). It went well so we now know that I can tolerate the medication without major issues.

I got one additional item checked off my never ending (it seems) pre-transplant to-do list: an ENT doctor came and got nasal cultures from me. I was thinking it would be a torture session because going to the ENT truly is one of the most awful things about having CF but really it went very well. Check.

Blood sugars: I had two really good meetings with a woman from their endocrinology team who works a lot with CF patients. I was dreading seeing her because my sugars have not been the best and I get to feeling very defensive about my numbers sometimes, especially if someone doesn't know me well enough to know what a good patient I am!

Overall, the last six months have seen so many changes with my diet, eating frequency, exercise amount and amount of stress that it will end up needing to have been a fairly major overhaul to my pump programming when this is all said and done. But endocrinology assured me that I was doing the right things and that my numbers would soon reflect that and felt they would soon be kept under control about as well as one can keep them under control considering the circumstances and variables right now.

So that's a quick run down. Oh, and a status update on being listed? As I was leaving the doctor poked his head in "I'm going to go ahead and list you" he said. Then thought for a second, "oh wait, we have to see how your blood sugars do over the next couple of days. Let's touch base on Monday and get you listed early next week."

So it looks like Monday or Tuesday - barring no more hurdles. Holy shit!

I would not have survived this hospital stay without the gracious assistance of an amazing woman who both kept me fed and supported my coffee habit.

Monday, July 7
Tomorrow I go into the hospital to start my desensitization. I hope to be out by Friday, or Thursday if I'm really lucky. The remainder of the loose ends should be tied up while I am there. I will have more to report then!

An absolutely gorgeous weekend in the mountains, mostly sunny with temps in the 70s. Great to be there.

Wednesday, July 2
I know, I know, aren't I listed yet? The answer is no. There are so many ducks to get in a row for transplant. It has felt very much like the bear who climbed over the mountain only to see another mountain in front of him.

Here is what I know:

• TODAY I graduate from pulmonary rehab!! This means I am officially done with the pre-transplant program and now move on to an equally difficult but self-paced routine that will have me NOT driving home in rush hour traffic every day - yay!
• I am meeting my coordinator TODAY to sign consent forms - yay!
• I am going to the mountains over the weekend to enjoy a relaxing 4th of July with Todd and the dogs - yay!
• I will be admitted to the hospital sometime next week (yet to be determined) for desensitization to the antibiotic Meropenem, to get a bone-strengthening infusion of Reclast, and for an updated nasal culture. That will take 3 days or so.
• Given all of this, it is likely I will not be officially listed until the week of July 14. 

It keeps getting moved around but - trust me - that is the nature of this game. Obviously I want to get listed but my health is also stable now so there is no big rush. Rest assured if anything did get bad I could be listed right away.

Thinking of Montana today...wishing my brother-in-law Brad a happy birthday today and dreaming of future trips with new lungs.

Thursday, June 26 - Todd's Birthday!
Today was a great day. Had a really good session with the transplant psychologist. Things are really coming together for me in my head - yay! My feelization for the day was, I can do this! What the hell have I been stewing about the last two months?? But of course, it's all been a process.

As far as my meeting with the pulmonologist, we now have a plan: as soon as the committee officially approves me (hopefully Tuesday) they want to start desensitizing me to an antibiotic I am allergic to that they want/need to use after transplant (and that I may need for a flare-up pre-transplant).

To desensitize, I would be in the hospital for a day or so, and once they know I can handle the drug without a reaction they will send me home and - wait for it - officially list me!

Because my expected wait time is 2-3 weeks (I think) I would be on the drug continuously through and then after transplant.

So it could be as soon as next week, although Todd and I requested another week so that we can go up to the mountains for the 4th of July weekend...assuming these fevers and fatigue I've been having off and on don't turn into anything worse in the next week. We haven't been up there for a really long time. AND we are having the house thoroughly cleaned and the carpets cleaned before we go - it will be a whole new house! And one in which I should be able to breathe a lot better.

That's all for now - I may add more later but it's time to join my husband watching a movie on his special day. :)

Wednesday, June 25
I am feeling very good after my appointment with the infectious disease doctor today. We talked about which antibiotics to use on me both now and after transplant with my recently developed drug allergies. 

Since I am in need of starting IVs again very soon (quite possibly tomorrow), it will be a good experiment to see if we can find a penicillin-based drug that I do not react negatively to.

So yes, I am starting on IVs again...after only 2 weeks. I have been having low-grade fevers and fatigue off and on since this weekend. Two weeks!

And one other thing, kind of big. Dr Wolfe and I decided today that now was the time for my CF care to officially change over to Duke. As much as I love all of my UNC people, I feel this is the best thing now. Heck, what's one more major life change, right?

Tuesday, June 24 - Endoscopy
Today I had an endoscopy - they stuck a scope down through my esophagus into my stomach and then the upper part of my small intestine. The procedure is usually done under anesthesia (i.e. totally knocked out) but whether due to one of the many many screw ups that happened with the scheduling of this appointment or not I only got lightly sedated (i.e. I felt every inch of that huge scope going down my throat and moving around for about 10 minutes.) Not the most fun thing I've ever been though. But the drugs were good indeed!

They took some samples to send off but there was nothing they were worried about which is great! They did take a sample from my small intestine to rule out what may be evidence of Celiac disease (gluten allergy) but hopefully that will be nothing.

This is a very important week, specifically Thursday is a very important day. I go to clinic that morning for labs, pulmonary function tests and an arterial blood gas and that afternoon I meet with one of the head pulmonologists who will potentially clear me for transplant.

What does this mean exactly? It does not mean that I will then be listed. It is simply the final hurdle that I have to cross.

What it does mean is that next Tuesday - July 1st - the transplant committee will discuss my case and likely decide to list me. Then I get entered into the computer, find out my allocation score and then the call could come at any time. Some people get called that very day. I keep thinking how insane that would be.

Have I mentioned that this is both super exciting and also terrifying? I think I may have once or twice...

So we are now waiting on the biopsies from today (won't be back until early next week), waiting to see how my numbers look on Thursday, waiting to talk to the doc on Thursday and then waiting to hear what the committee says on Tuesday. Oh yes, and we are keeping a watchful eye on some fevers that I've been having and hoping for them to go away. If not, they are likely due to my lungs acting up (one week off IVs!) and we will have to address how to handle it given that I am now allergic to at least two of the handful of drugs that now work for me.

Complain, Complain, Sunshine, Complain

I rarely complain - I dislike it so much it's almost a pet peeve. But for whatever reason I am feeling the need to complain today. So here goes. Oh, and I'm adding in a bunch of sunshine/solsticey pictures to make it more palatable.

1. Pulmonary Rehab Sucks Ass

Yes, I devoted an entire blog to the pleasures of pulmonary rehab last week. But, a 'complaining thread' wouldn't be complete without me reiterating the absolute domination of my life and energy depletion that rehab is.

This week, Tuesday almost killed me with the "long walk" (30 minutes) followed by "leg day" at the weight station, 20 minutes on the exercise bike and then a 40 minute floor class with more leg and arm exercises. (As an aside, let me just tell you what all is in involved in leg day: leg press, leg curl, leg lifts on the nautilus machines; ~ 20 standing leg lifts to each side and then back with 2 lb weights on my legs; heel toe raises standing on one leg at a time; 30 seconds of balancing each foot on an inflated disc thing; ~ 12 step-ups (think aerobics class) with a bent knee leg lift; and a couple of stretches.)

Wednesday was another long day - started with a 10:30 am transplant education class at the hospital before rehab (left the house at 9:30, got home at 6.)

Thursday was grueling. I had an appointment with the transplant psychologist at 11 am - time well spent - that left me feeling pensive and a little sad. But still, it's hard to show up as an Eeyore at rehab - I was able to put on a bit of a smiling face to get through it. I ended up physically having a really good day but was just (you guessed it) totally exhausted by the end of it.

Friday was...awesome...because there was no rehab! That's right. I skipped it (sorta). But I did have a pretty good reason. Our power went out Thursday night before I could finish my therapy. As I tried to fall asleep, I was coughing and hot and couldn't get comfortable. It ended up being a non-restful night. I had two diabetes appointments Friday morning that I really did not want to miss, so I went to them but skipped my afternoon duties in lieu of a very long nap.

2. I Possibly Have The Worst Oxygen Company in the World

I have had many problems with my oxygen company over the last year. I have a literal list of about 10 major fuck-ups. I will spare you the entire history but highlight the most recent frustrations.

• The last time I called to arrange for out-of-town oxygen, for Erica's wedding in Greensboro, I was told that they would set it all up and then give me a call back to confirm. I never heard back from them.
  
• On Monday I called to place my regular oxygen order. I had three things to communicate to them - I know that's kind of a lot but I was hoping with careful explanation to be successful: I needed a new regulator that would go to a higher pulse dose, I needed the time of my delivery to be changed from afternoon to morning and I needed to order my tanks. Luckily, a new, inexperienced person answered the phone.
  
  
  
  
  It was immediately obvious that the new person had no idea what I was talking about with the higher pulse dose regulator. She kept putting me on hold to ask someone, then coming back to me: "Yes, yes, we have concentrators that go above 5 liters" No, not concentrators, regulators. I finally convinced her to let me talk to the person she kept putting me on hold to talk to. It was none other than the very helpful Anita who had never gotten back to me about my last travel request.
  
  Anita informed me that if I wanted the new regulator I would have to pay for it out of pocket because my current oxygen prescription was not written to cover it. WHAT??? "The last script we have for you in the computer is from November and says you are only on 2 liters."
  
  
  
  
  This was just insane. I kept explaining to her that Lincare had recently brought out a new concentrator for my home and they would have had to have that higher dose prescription in order to dispense that concentrator to me! But it fell on deft ears. She didn't know what happened to that other prescription (and truly she wasn't even taking the time to listen to my argument) but she assured me that if the doc faxed a new one in she would personally make sure it got into the computer. Aww...I love a personal touch.
  
  After the phone call when the anger of her unhelpfulness started wearing off I realized that, similar to how no one there ever wants to take responsibility for their mistakes, she had taken something that was clearly their problem and made it my and my doctor's problem. It's a first class operation they run, what can I say?
  
  
  
  
  The story does not end here. The next day it became obvious by close to noon that they had not told the driver to switch my delivery to morning. When I called him this was indeed the case.
  
  Later that day when the new prescription was received from my doctor, Anita called to let me know (helpful woman that she is) that they would be sending it out the following day and, while they were here, would also be checking to make sure my home concentrator went up to the proper liter flow amount (again - obviously my argument the day before was not absorbed into her brain whatsoever). Gee, thanks.
  
  So why don't I fire them you may ask? I know...I can't wait for the day. The answer is because I am so close to transplant it just doesn't make sense. After transplant if I do have oxygen needs (hopefully not!) I plan on getting them filled elsewhere.
  
  
  

3. With All Of This Other Shit Going On I Have Absolutely No Time For Anything Else

Without harping on the rehab stuff, I will just say that between my appointments, rehab and phone calls to various medical personnel, doctors and my transplant coordinator I have no time for myself. To think. To do normal life things. The exception is perhaps my drive to and from Durham, itself, though, mandatory. I have no time - or energy - for any of life's other tasks right now. My friend texts in the morning to see if I need anything from Costco after work and I don't even have time to think about what I may or may not need at Costco until well into the evening. That kind of thing.

Of course I am scraping by, but this is thanks to a lot of extra help and understanding from people around me.

*   *   *

Suffice to say that I have not been in the best mental place this past week. However, even with all of b.s., I still feel like I am coming around more to being ready for transplant. It has really been a mental struggle for me. Part of me feels like transplant is "giving up," and that does not come easily to me. As I said to the shrink this week, "It's hard to lay down your armor when you've been fighting for so long." 

Of course anyone who has been through transplant would say that it is anything BUT giving up - there is an incredible amount of hard work involved both in recovering from the surgery and in the ups and downs of infection and dealing with rejection. But it is a different kind of fight than I am accustomed to. It is one in which many more different things - and scary, hard to treat things that aren't all completely understood - can happen than with my old CF wind bags. It requires a leap of faith, and a peace with the fact that everything may not work out as I want it to. 

You never know where you will draw inspiration from and what sorts of things will resonate with you when you are going through a difficult time. And so I was a bit surprised to find some very solid and relevant words of wisdom from Rob Lowe's new book Love Life that I just finished reading:

"You can't study the map forever. At some point it's time to start walking; there is only so much daylight."

I have studied, researched, sought out, met with, asked questions about and read just about everything one can to prepare themselves for transplant. Now it is time to turn off my brain, open my heart, and fly.

 

Photo credit (top to bottom): splash sun, celestialelf.wordpress.com; woman, pixels.com; summer solstice sun, judithtownsend.com; sun, naturefriendsla.org; tree, society6.com; sun, freegreatimages.com; surya namaskara, theblissblissbliss.com; sun, theindigowillow.wordpress.com.

The Comfort of Disease

It might sound strange to you that of all my CF clinic appointments over the years - and even as my health has taken a nose dive over the last six months - I almost always leave the clinic feeling positive. And it's only now that I'm realizing why.

Yes, I love my doctor - that cannot be understated in this equation. But I have now come to see my clinic routine as another way that CF "challenges" have, over the years, actually become comforts to me.

Say It Ain't So!

It's a strange realization. But it also makes perfect sense in an 'evolve or die' sort of way. I don't think it is humanly possible to go through life with a demanding chronic disease and not find some way to cope with it so that it doesn't break you. Let alone keeping a positive attitude.

Many of these things that I sort of loathe - boiling my nebs, vesting, filling my pill box every week, keeping on my antibiotic schedules, rinsing my sinuses morning and night, seeing all of my doctors at UNC, having to exercise to feel halfway normal, my nightly ritual of soothing my nasal passage with un-petroleum jelly - will go away with transplant. (Lord knows the pill box filling will not!) But that also means letting go of the comfort I have come to get from all of it.

Onward and Upward

And not only that, but if all goes well, new lungs will not only allow me to not live the life of a sick person but to become part of the world of normal people. ("Normies" as fellow cystic Josh Mogren would say.) I could work again! Return to my active social life (without worry of akward coughing or peeing moments!) Go for a bike ride on the greenway! Visit my family without five bags of medical stuff! And travel without oxygen, without the guilt of always leaving my vest behind, without having to think about how I'm going to sterilize my nebs in a hotel room. That. Would. Be. Awesome.

I think that you all can understand that while all of that change is all very exciting it is also incredibly terrifying. Living with CF lungs is all I have ever known - at least it feels that way now.

I have thought a lot about how transplant forces you to give up control - to relinquish your body to the transplant surgeons and staff in those first weeks before you are (god willing) able to go home and begin to care for yourself again. And how I must rely on Duke's guidance and teaching about this whole new disease I will acquire (the figurative "disease" of transplant.) But giving up control and giving up comforts are two different things, both of which I need to work on.

Maybe I will be well served now to try to start seeing my CF tasks as what they really are - chores, endless chores that only become more difficult and less productive as time goes on. Because let's face it. My life kind of sucks right now. In the words of my friend who came to visit yesterday, "This isn't you, sitting around, hooked up something." Now is the time when it's okay to get fed-up, because hope is on the horizon. Now is the time to let myself get excited about living again.

 

Photo credits: Sun, agniyoga.si; Evolution, munnsbunns.blogspot.com; Chore lady, theorganizedwife.wordpress.com; L, dailydropcap.com

Pulmonary Rehabilitaton (aka Purgatory)

Purgatory: (in Roman Catholic doctrine) a place or state of suffering (pulmonary rehab) inhabited by the souls of sinners (transplant patients) who are expiating their sins (trying to build up a seemingly impossible amount of strength despite their extremely compromised state of health) before going to heaven (getting new lungs.)

Since my life has literally been dominated by pulmonary rehab lately I thought it appropriate to dedicate a blog entry to it.

To say that pulmonary rehab, which I am required to do five days per week unless I have conflicting appointments, is difficult is quite an understatement. Each day starts out hard then gets harder and harder. It challenges you to your physical limit (if you let it), and then - since you have no more to give physically - becomes some sort of tortuous mental exercise. Today, for instance, the smallest thing made me cry...  and it had nothing to do with the thing at all, it was just my body's reaction to this extreme mental and physical state I am forcing it to be in.

At least this is how it has been for me. On a good day, I feel very tired and a good kind of worn out after my three hours of working out. On a bad day, like today when I was easily getting short of breath from the time I got up, it seems like a victory just getting there. And every lap, every pedal, every weight repetition is a chore. At the end I am utterly exhausted.

And then I come back the next day and do it all over again. And the next day, and the next day, and the next day, and then hopefully I have some appointments on Friday so I can skip out (like I do tomorrow - yay!)

The whole thing is totally insane. You should see these people! There is no "can't" in this crowd. It really would be difficult even for a healthy person to endure, let alone someone running on a fraction of the energy that they used to have.

A Typical Day 

Having said all that, let me tell you about a typical day at rehab.

• Arrive, get checked in, switch from my oxygen to their tanks that come in push carts that are easier to push around the track.
• Walking: Tuesday and Thursday are long walks (mine today was 30 minutes, but I don't think it will go higher than that); Monday, Wednesday and Friday are 20 minute walks. We always count our laps and I'm always surprised how much my speed varies from day to day, constantly up and down. For instance, yesterday I did almost 20 laps in 20 minutes (flying!!) and today I did 21 laps in 30 minutes (turtle pace.)
• Biking: Always 20 minutes, but resistance is increased every day.
• Weights: Rotate days working "arms" and "legs" - although there are so many leg exercises that some spill over onto arm day. This includes things like leg curls, leg press, bicep curls, tricep press, squats, stairs and various shoulder stretching poses.
• Floor class: Taught by a different teacher every day, the floor class uses ankle weights, hand weights and therapy bands to work the major muscle groups. There is also some stretching and breathing at the end. (And a lot of coughing by me and all of the other cystics.)
• Education class: Classes on topics related to transplant such as occupational/physical therapy, feeding tubes, osteoporosis. These are all topics that Todd and I will learn much more about in the hospital but it is a good introduction.

That's it! Crazy, huh?

And If That Isn't Fun Enough...

I am lucky to get out by 5pm (class starts at 1:30) which puts me perfectly in time for rush hour traffic that essentially doubles my commute home. Now that is a really fun thing to do after already tiring day. Sigh.

When I do get home, it's always a toss-up for me between nap and therapy. I am exhausted but 6pm is an awkward time for a nap. So I usually end up just moping around the house being exhausted for a couple of hours until I catch my second wind just in time to ensure that I can never get to bed before midnight. Thank god I do not have to think about dinner these days. (Thank you friends and take-out!!)

There are more annoying things about pulmonary rehab...it totally messes up my eating and therapy schedules during the day (both of which are very important things right now), I get sweaty at rehab but am forced to either take a sink bath or shower head rinse off since my port is accessed, and sometimes the satellite radio spends an awful lot of time on the 50s station catering to the center's large majority of older patients.

But hey, no one likes a complainer (I'm kidding, since this whole blog post is sort of one long complaint). I do have a brand new car to enjoy my trips back and forth to Durham - and I enjoy the time alone to think. And while rehab hasn't improved my lung functions any, it does keep me coughing up stuff all day long so my lungs stay pretty clear and it has definitely made me stronger - starting to see some definition in my arms! Sometimes someone will even put on 80s or 90s music which makes me very happy.

Now. If I could just start feeling like I was mentally ready for transplant, I'm pretty sure my body is all set.

Photo Credit: Purgatory, emptykingdomcom; Group exercisers, gettyimages.com; Stretching cat, acctomarley.blogspot.com.