The Number Game.

"Whenever you find yourself doubting how far you can go, just remember how far you've come.  Remember everything you have faced, all the battles you have won, and all the fears you have overcome"

This past week has been a challenging week for me, mentally and physically. Despite committing to working out daily for the last month, going to pulmonary rehab 3 times a week and focusing on trying to do everything right - my numbers continue to drop, my lungs continue to fail me.  Prior to my thyroid surgery, I sat at about 25% lung function.  This week, I was at 21%.  To most people this 4% change probably doesn't seem significant, but for me this is entirely disappointing and most of all frightening. My best number this week was 23%.  

PCD is relentless.  It simply doesn't care who you are, how old you are, if you have a family, if you're weak or how hard you work to stay strong, it fights back with a vengeance.  It doesn't care if you have plans, if you have dreams.  It's absolutely terrifying to know that no matter what you do, ultimately it will "win", and by "win" I mean it will progress and my only option is a life saving double lung transplant.  No matter how many times one is told this, no matter how long or how little you think about it, you NEVER become "comfortable" with the idea that your only option is a transplant, and that transplant certainly isn't a guarantee and most definitely doesn't come without it's own challenges.  It's unnerving on so many levels and the "what if's" are endless.  What if the transplant never happens? What if my lungs don't last me until transplant?  What if something goes wrong during the 12 hour surgery? What about all the potential complications? What if I get infection and/or rejection? What about that person that had to pass away in order for me to live, and their loved ones? What if?

I express my vulnerability here because it is my reality.  I'd like to think most of the time I remain upbeat, positive and look forward headstrong - but I am human and I have my good days and my bad days, and I am the first to admit it on my bad days...and, well, it's a little hard to hide when you're at pulmonary rehab and you start bawling that ugly, snot-nosed, red-faced cry in front of people you barely know. Yeh, that happened. 

People are always asking what they can do to help?  I'll tell you what will help.  Help an organization that's sole purpose is to make  the lives of those affected by PCD better.  An organization striving to gain speed for research and advancement of care for this awful disease. October is PCD awareness month.  Learn a fact or two about PCD and share it.  Did you know that less than 1,000 people have a confirmed diagnosis of PCD, but it is estimated that about 25,000 people, just in the US, are living with it?  The only way we can make progress is through funding and any amount of donation can help us. We can help find specific therapies and treatments for PCD, as currently there are none.  There is no cure.  If you are feeling like lending a helping hand please visit the PCDF website where you can get involved in any way you'd like, or make a donation.  And, in the meantime, please send some positive vibes to those of us who battle every single day, for each and every breath we take.

http://www.pcdfoundation.org/en/get-involved

And the results are in ...

It's been a CRAZY two months.  From being diagnosed with thyroid cancer one day, to meeting the surgeon the very next day, to having powerful IV therapy for three weeks and surgery one month later, then Thyrogen shots and radioactive iodine ablation a month after that (WHEW!) ... and finally a scan to determine if the cancer is gone and/or has spread, the results came back this last week on Friday.

It is with the biggest smile that I can report some GREAT ... no FANTASTIC news!!!  The small amount of thyroid tissue that was left over after surgery (which is normal, it's almost impossible to get all the tissue out at time of surgery.  What is leftover is scant in amount) will be fully ablated (gone) within 4 months ... and even better news is that the cancer cells did not spread anywhere else that we weren't aware of and that has been removed!!!!  What a huge relief this is.  I cried a huge sigh of relief when I got the news and it made for such a stress-free weekend, celebrated by my brother in-law's wedding in Minnesota surrounded by family and seeing some long-time friends over lunch.

I'll keep this blog short, but wanted to share the awesome news we received and thank everyone from near and far for the love, support an ongoing encouragement we've received. I truly believe everything happens for a reason ... I don't know exactly why this happened, but I do know that I am stronger for it, my relationship with my husband is even stronger, my confidence in my healthcare team has grown beyond that of just my Pulmonologist and I know that I/we can face challenges thrown our way with grace and a huge fighting spirit. Of course, I couldn't do any of this without the support of so many.

So, as you sit down to dinner with your loved ones or you're watching football .. or whatever you might be doing, know that I am thanking you for every little bit of love I received.  I am also thanking a bigger power than oneself, that I have been so carefully watched over the past 2 months. There were so many variables and it ultimately it turned out the best way it could. So, cheers my friends and remember whatever you might be going through, to "Fight On"!!!

Rebs

Fight On.

Yesterday marked 2 weeks post surgery.  On one hand I feel like it's been a month, and the other I can't believe it's already been 2 weeks.  The saying above is my moto these days, "Fight On".  We're so relieved to have surgery in the rearview mirror and I can't thank those of you near and far who reached out and sent well wishes, goodies, called, texted ... you name it, it got me through a few rough days/weeks. 

Surgery was a huge success.  They were able to complete the surgery without intubating me, and just giving me heavy sedation.  Let me tell you, sedated I was!  I was wheeled into the OR, hooked up to the machines, moved to the operating table and the anesthesiologist told me he was going to give me something to relax me.  I then had about 15 doctors, nurses, etc all standing around the table watching me... and waiting for me to get silly.  After about 15-30mins, I was then told that they were going to give me the Precedex (main sedating drug).  I looked at the clock and it was 8:19am.  I said a little prayer that I would wake up without complications, and see my family.  The next thing I knew, I woke up at 10:24am to everyone cheering, saying "We're all done, you did great"!!  With my face half numb and in a stupor, I tried to cry but couldn't and "mouthed" (because I had no voice), "Yes, let's get a picture!!".  With a generous laugh from everyone, they snapped a picture of me with these two wonderful doctors.  Don't mind me, this was literally about 2 minutes after waking up!  

Dr. Suliburk (surgeon, Left), Dr. Kim (anesthesioogist, Right)

They removed my entire thyroid and removed/tested a suspicious lymph node during surgery.  The rapid test of the lymph node showed it too was malignant, so I had a whole slew of lymph nodes on my left neck removed as well.  Pathology results came in from that and I did have a few more positive (malignant) lymph nodes.  The doctors didn't seem too concerned with those results, so I am trusting them that they got everything they needed to out!

Below are a few pics of my removed thyroid, beware: graphic.  You can see the picture labeled "LLT" looks different than that labeled "RLT".  The "LLT" was the side that contained the cancer.  Amazing, the difference!

Healthy Thyroid tissue - RIGHT

Malignant thyroid tissue-LEFT

After surgery, I was then taken to the ICU where I spent the next 48hrs.  I had no voice for about 24 hrs, which if you've never experience actually having absolutely NO voice, it's so frustrating! The doctors and nurses would come ask me how I was, if I needed anything ... and I couldn't communicate.  I was in a fair amount of pain as well, so they ended up giving me a pain pump so that I would have a continuous infusion to manage the pain better.  That helped tremendously, as I needed to cough but it hurt like hell, b/c I use those muscles in my neck to cough.  After a couple rough nights dealing with pain and no sleep (thank god for my angel of a husband and mother who stayed with me those rough nights) I was happy to be discharged home.  It's now been 2 weeks, I've been able to get back to the gym and my energy/strength are slowly returning.  My neck is loosening up, the incision is healing and the pain is minimal.

1 wk post op

2wks, 1 day post op

Here are a few pics of the incision at 1 week post op and 2 weeks, 1 day post op.

Now that the hard part is over hopefully, we proceed with further treatment.  I have started on thyroid medication, since I have no thyroid.  I started on what is called a T3 medication, but will switch to a T4 starting tomorrow. I'm hopeful that this is an easy transition with minimal side effects.  I will start what is called "Radioactive Iodine Ablation Therapy".  This will consist of getting a few shots in my bum to simulate a hormone called TSH (Thyroid Stimulating Hormone).  In order for the thyroid cells to uptake the radioactive iodine needed for ablation, I need to have an increased level of "TSH".  I will be given a dose of radiation in the form of a capsule or liquid and that dose of radiation will target and kill any remaining thyroid cells (cancerous or not).  I will then have a scan after the shots and radiation pill to see if the cancer is gone.  This scan will also show if the cancer is anywhere else in my body.  This process will take about a week.  Assuming all goes well with that, we will then do some bloodwork and scans in about 6 months to see if we are "all clear".  I will continue to have scans and bloodwork about every 6 months until the doc feels comfortable that I can go longer between scans.  She is being extra cautious because of the positive lymph nodes and my fragile lung situation.

So, all in all, this bump in the road is jumped and I can't begin to say how relieved I am.  Obviously we still have some hurdles to jump, and we will do so as gracefully as we can.  Thank you all for all of your love and support for me, my husband and my family!!  I will do my best to keep this blog updated as we move on.  As for now, "Fight On" my friends!!

Love - Rebs

What the hell just happened????

My life was changed, yet again on Wednesday, July 22nd.  I was diagnosed with thyroid cancer.  It all started with an MRI I did 6 months ago for back pain and it was noted then that the thyroid had a nodule on it.  From there I had an ultrasound, which was "suspicious", so on Monday last week I had a thyroid biopsy that came back malignant.  Only 5 % of thyroid nodules are malignant, leave it to my good luck to fall in that "rare" malignant category.  Within 24 hrs, I got a call from my doctor with the diagnosis, met with a surgeon and had a CT scan done to check for any abnormal lymph nodes in my neck.  CT was done Friday last week, to find out Monday I have one enlarged node they have a little concern over.  

So this brings me to yesterday, Tues, July 29th.  Went in for a lymph node biopsy at 8am.  The radiologist couldn't find the lymph node that was showing up on the CT scan - so no biopsy was able to be performed. Then, we were told we'd do some pre-op testing and meet with the anesthesiologist who will be with me day of surgery ... only to find out once we were there that he is out on a camping trip until next week ... really?? WTF???  We spent 6 hrs in the med center and walked out with a pre-op EKG and labs done.  We did speak with a nurse practitioner who works with the anesthesiologist, which to her credit was probably the only thing that kept me from falling apart and looking like a hot mess in the hospital.

Words I never imagined would be a part of my vocabulary in my patient chart - "cancer" and "possible metastatic disease" - but throw that in there along with those words of "double lung transplant" and "bilateral mastectomy".  Can't a girl get a "f-ing" break??  We still aren't sure how this will affect me as a lung transplant candidate, that is yet to be discussed with our team down here, but we're hopeful that once the thyroid/lymph node is removed, I will essentially be cured, and not a high risk patient for transplant.  Right now we're trying to focus on this thyroid/lymph node mess and then we'll readdress the transplant situation.  I also started IV antibiotics and steroids on Monday this week to try and get my lungs in as best condition possible for the surgery which is tentatively scheduled for 8/20.

I will forever remember that call from my pulmonologist, who so sensitively gave me the news.  I was driving to (what a surprise) another appointment.  I received an email from her and all it said was "can you call me".  Right then I new the news wasn't favorable.  I was literally driving through a roundabout when she told me.  My heart sank, I felt like I was the only person on the road, my hands started shaking, I almost threw up, I lost all the air my so depleted lungs ... and I think I went around the roundabout 3 times.   People probably thought I was on drugs, or just a terrible driver...not that I just found out I have this cancer growing in my body.

This has all been so overwhelming, scary and very emotional.  But, we've had a ton of support and positive vibes sent our way. We'll get through this as we do everything else ... and hopefully life will return to "our normal" soon.  From here, we continue with the IV therapy through surgery, we'll hopefully try again to meet the anesthesiologist next week to come up with a game plan on anesthesia, possibly try again on the lymph node biopsy and then get surgery over with.  I will be in the ICU after surgery as a precaution and will likely stay in the hospital for a couple days.

Thanks to everyone who has reached out, even those I haven't talked to in a long time ... those who had no clue what was going on but wanted to show support.  Those who've offered to travel thousands of miles to be with me.  One can't help but feel "alone" in this world during times like these, even though I know I'm not.  When it comes down to it, I am the one with the diagnosis, I am the one who has to undergo the knife and recovery ... but I know I have so many who will be by my side.

I also can't say this enough, but thank you to all who have shown support to those who support me most ... my husband who dropped everything to be with me at my lowest.  My parents who in a matter of only a couple hours packed their car and came over to Houston to be with me.  My brother and sister in law, who despite living in Boston, made me feel like they were right here with me.  People often forget how these things affect loved ones ... it's incredibly stressful for them as well. I need them and your support helps them, help me.

So - ending on a positive, we will move forward, one step, one day at a time and we will find the goodness in all of this mess somehow.  Much love to you all.  -Rebs

Mother's Day - A Day to Remember

Tomorrow marks a big day of celebration - one that so many gather together for, buy special gifts for, make the day for - Mother's Day!!  It's a day to acknowledge all the hard work Mom does, recognize the fact that she carried and nurtured you for 9 months in that belly of hers. She changed your poop filled diaper at 3am in a zombie like state, she cared for you when you were sick and basically helpless and crying all hours of the night.  She introduced you to new things,  hauled your butt to and from school, athletics, friends houses...she said "no" when she knew what was best for you, and reluctantly said "yes" when she knew you needed to learn on your own.  She's always got your back.  She put your life ahead of hers in so many ways, so many times and she shows you unconditional love that is irreplaceable.  She's your best friend...At least, I am lucky enough to say that about my Mom.  

My best friend :)

I celebrate with a smile, knowing that my Mom is my best friend.  I can call her anytime, anywhere and say anything and I know she will be there.  I can't imagine my life without her.  We've been through some pretty challenging and scary times.  She's battled breast cancer and I called her a "mouse" - because her bald head was so tiny and she looked like a cute little mouse, haha!! (Really I meant it as a compliment).  I remember caring for her after her chemo treatments, when she was extremely sick, weak and fragile.  She's been with me since day 1 of my health journey and has spent countless hours and days caring for me, at doctors appointments and in the hospital ... As we look ahead at the future, knowing a transplant is my only chance at possibly breathing like a normal human being one day, I would be terrified to go through it all without my Mom by my side, cheering me along.  I hate that she has to be a cheerleader in such a challenging game, but I know we aren't given anything we can't handle, especially together.  We share so many things, and the time I spend with my Mom is something I cherish any time I can get.  We laugh - we cry - and we are there for each other.  I try to celebrate my Mom everyday, but tomorrow is one day a year where, whoever or wherever your Mom is, whatever your relationship may be, take time to tell her you love her, or take a moment to remember her if she is no longer with you.  She brought you into this world, and there is nothing more wonderful that being able to bring another life into this big world.

Mother's Day is also a bold reminder for me and so many others, that I (we) will likely never get to be a mother...well at least to a human being that is ;)  This is something I always thought I would be one day, something I always planned and hoped for ... I'd love to be able to have the chance at being a Mom, to experience the feeling of carrying my unborn child, feel him or her kick in my belly, the pain and beauty of childbirth.  I would love so much to see (what I can only imagine would be) a beautiful boy or girl Bob and I would have.   I'd love to see even more how great of a Dad I know my husband would be.

So on this Mother's Day I hope for two things.  First and most importantly, I hope you celebrate your Mom and show her how much she means to you, tell her how much you love her.  Don't let it be just Mother's Day that you show your love and respect for your Mom, but try every day to do so.

Secondly, on this Mother's Day, remember all of those who are mommies in a different sense - Mom's like me that maybe don't have a human child, but have babies with four legs, fur and a tail!  I am proud to call myself a "doggie Mom" to my sweet Bella and Tarver.  They are my "kids" and I love them with my whole heart.  I just hope I am half the Mom to them, that my Mom is to me.

I love you Mom, today is your day and I hope you know how special and loved you are!!!  To all the mommies out there, Happy Mother's Day!!!

FUELED.

Wow!!! ... Is all I can say to start this blog.  I am completely overwhelmed with the amount of love, encouragement, well wishes and support I received after posting my last blog.  I (for lack of better words) "went public" with my health situation, something that has been something I've sheltered for so long. I heard from people near and far.  I received emails and messages from people whom I've never met, from people I haven't talked to in years, best of friends and friends of friends, other people in similar situations ... and I can honestly say, receiving those messages, calls, texts ... literally propelled me into my appointment last week, so thank you to every single one of you that took time out to read my blog and send me some positive vibes.  You have no idea how much it means to not only myself, but my husband, my family.

So now an update and how the appointment went.  Every time I see the transplant team, I start off by registering and course paying "my dues", b/c of course money comes first (Eh hem, smirk).  Then comes the part that I get so anxious about - my pulmonary stress test and spiro test.  These are the two tests that the docs look at and compare to my previous performance to determine my "stability" (along with other factors, but these are really important).  For the pulmonary stress test, I have to walk "laps" (up and down a bare, sterile and quite frankly boring, hallway from cone to cone). They want to know how far I can walk, how quickly, do I need oxygen and if so how many much.  They measure my blood pressure before, right after and 5 minutes after finishing.  They also constantly monitor my oxygen levels and my heart rate.  For my walk this time my husband was my cheerleader along with the respiratory therapist performing the test.  I was able to walk about the same distance (even a little further) than my last walk test, I required less oxygen and at a lower level than my last test!!!  Every time I turned the corner and saw my husband at the end of the hall, I pushed a little more and remembered why I fight for every single breath.  The spiro is basically a measurement of my lung function.  I hook up to a machine, plug my nose and in the end, have to take the biggest possible breath in and then I am told to "blast" air out initially and then slow and steady.  This big blast of air at the beginning is called the FEV1, which is the volume of air exhaled during the first second of a forced expiratory breath. It's converted to a percentage that tells you what your lung function percent is at compared to someone your height, weight and age.  My lung function is at 25%.  Normal healthy people will have lung functions between 90-100% generally, especially at 32 years old.  Sooooo...it's obvious why it's a little hard to breathe these days.

Good news is, my numbers were stable!!  I literally take these appointments 3 months at a time (if not sooner).  I have had days where I thought I felt great and my numbers decreased slightly (like last time, when they hinted that I might need to be listed at this appointment), and other times when I feel not so great and my numbers will not change, so I really have no idea how I'll be going into these tests.  

The docs are still saying I am too early to be listed as an "active" on the transplant list. For those of you who don't know what this means, as soon as you go "active", you can receive a call any minute, of any day saying that they need me to be at the hospital to be prepped for the surgery.  Imagine...always being "on call", but this call will potentially save your life.  That thought alone is both frightening and exciting.  Being an active potential transplant recipient comes with a lot of stress and anxiety as you can imagine, for everyone involved.  ANY time your phone rings, it could be "the call".  Then, just b/c you get "the call" doesn't mean the surgery is "a go".  The transplant team has to perform MANY, MANY checks to ensure that those donor lungs are a perfect match for the recipient.  Surgery can be called off at any point if they feel they aren't a perfect match, that is called a "dry run" and they aren't uncommon.  

There is a whole new blog that I can write detailing the pre-post transplant life and I will save that for another time but for now I wanted to share the good news that I am still stable which is everything we hoped for!! This however I take very cautiously b/c things can change in a matter of hours/days for me.  One's normal cold can land me in the hospital on a ventilator needing a transplant ASAP.  I don't have the normal immune system everyone else has b/c of my PCD.  This is why I am a bit OCD on washing hands and cleanliness.  I am thinking positive though and I hope I continue to be able to keep my lungs as long as possible, as they are the best for me, despite being so damaged.  For now we continue to try and keep me as healthy as possible and hope for the best between now and my next appointment. 

I want to thank each and everyone of you for reading my blog and sending me well wishes.  I can honestly say that amount of goodness y'all sent to me game me a little extra jolt in my step for this past appointment, so THANK YOU!!!!  Please continue to keep me, my husband and family in your thoughts as every day is a struggle and we are dealing with a very serious and stressful situation.  I won't lie and say this is easy, but it makes it a little easier knowing we have the support of so many awesome people out there!!!

"Team Rebs"

I LIVED.

                                                                   "Hoping you take that jump

But don't fear the fall

Hope when the water rises

You built a wall

Hoping the crowd screams out

Screaming your name

Hope if everybody runs

You choose to stay

Hope that you fall in love

And it hurts so bad

The only way you can know

Is give it all you have

And I hope that you don't suffer

But take the pain

Hope when the moment comes you say

I, I did it all

I, I did it all

I owned every second

That this world could give

I saw so many places

The things that I did

With every broken bone

I swear I lived"

In my lifetime, one thing I never want to do is look back and regret not "living" in the moment, for the moment and for the future.  This song encompasses many things.  It's music video actually features a Cystic Fibrosis patient (https://www.youtube.com/watch?v=z0rxydSolwU) I think many people with life threatening, chronic illnesses probably have similar feelings..or anyone who's really been challenged in life for that matter.  My "life expectancy" is not that of an average Joe, to put it bluntly.  Sure the average Joe could fall over from a heart attack at 50 years old, the average Joe could be in car accident at 20 years old.  But the average Joe ( who is not dealing with a lung function that is on the decline, with not much room to decline) would have what you might consider a normal life expectancy.  Of course, I want to defy the odds and I plan to.  Heck, I already have in my mind.  I was told 12 years ago that I would need a lung transplant 2 years ago, by the time I turned 30.  Screw you doc. I'm still kicking.  

I've just about finished a round of IV meds.  Yes, I feel so much better than before I started, but things are different now, than say 5 years ago.  Despite hardcore antibiotics, IV and oral steroids, rest, no work, much help from family and friends .... my body still hurts. It's fighting just to be.  I'm exhausted.  This disease is exhausting, that's one of the best descriptive words I can come up with to describe what it's like to deal with this daily.  Not only is it exhausting to just breathe and get through the day, but is a full time job keeping up with my medicine, my treatments, medical bills, doctor appointments ... I feel like I am fighting myself.  My mind wants to do normal things, be a normal 32 year old, but my body won't let me.  That is the tough and possibly the worst part.

The best part of all of this is that I realize how precious life is.  I appreciate those days/moments when I can take my dogs for a walk around the block.  I appreciate being able to go grocery shopping, being able to meet a friend for coffee or a glass of wine ... those "normal" things.  My perspective on life is mine, and it is something that cannot be taken away from me ... not this disease, not one person, nothing can change the thing that keeps me so positive despite, let's call it like it is, a shit show for a situation.  I am able to adapt to ever changing "norms".  Lots of people can't handle change.  I embrace it.  I value being able to adapt, I have to.  If my plan for today has to change b/c I am having a particularly tough day, so be it.  If I am hurting too much to go to the gym, okay fine.  If I can't make that trip we've been planning so long for, well crap, but it is what it is ... life goes on.  I can thank PCD for my ability to cope with change, appreciate life and value those things that the average person doesn't.  Life is much brighter, more enjoyable and a lot more fun when the smallest things bring the greatest pleasure.  I wouldn't trade that for anything. 

This week will be an interesting one.  I am due to finish IV's tomorrow (Monday). I have a transplant follow up appointment on Wednesday and we are traveling on Thursday.  I ask that whoever reads this, to send positive thoughts and vibes for me and my family for this Wednesday.  I already have anxiety about what the docs will say, where my numbers will be and what is to come.  One thing is for sure though, I will take it for what it is and walk away screaming "I Lived", b/c that is all one really can do.