PCD Awareness

Well, I unintentionally, kind of slacked on the PCD Awareness month ... the last day of it is technically today, as it runs the month of October.  While the official awareness month might be over, PCD is NEVER over for me.  It is a daily, minute to minute, hour to hour struggle for me, so I felt appropriate to write this blog.  

It's been 28 days since I was put on the transplant list.  In that time, I've spent the last 14 days being pumped full of IV medications to get my lungs back to their whopping 22% functioning.  I've spent about 8 hrs a day hooked up, which includes late nights, early mornings and middle of the night infusions, so sleep has not been plentiful.  I'm tired, I'm bruised.  Yes, I feel quite a bit better, but lets remember that my "better" is probably comparable to a healthy persons worst day of having the gnarliest head and chest cold in your life, the only difference is that you get better, I don't ... in fact, I only get worse.  I still spend most of my days coughing, struggling to do the everyday tasks that once were easy, like cooking, cleaning, working out, shopping, walking the dogs...and it is because of this rare disease, Primary Ciliary Dyskinesia.  It is because of this disease, that I need a double lung transplant to survive.  PCD doesn't care what your dreams are, what you want to do, who you want to spend your time with, if you're married and want kids or where you want to go in life.  It interjects itself, and one simply must manage.



PCD is severely understood and often misdiagnosed.  Heck, I went 29 years of my life misdiagnosed.  It is crucial that awareness for this disease, and funds for research are raised.  We have come a long ways since the beginning of the PCD Foundation was launched nearly 15 years ago.  Started from scratch some amazing people (Michelle Manion and Lynn Ehrne amongst many others) have grown the PCD Foundation to what it is today.  Today we have a Clinical Centers Network for research and PCD care,we have a diagnostic and treatment consensus statement, we have genetics panels, we have a registry on the horizon, we have scientific meetings to teach & train medical professionals, we have the first North American PCD DRUG STUDY(!!!) and we have CARE days for PCD families.  Today there are 29 clinical and research affiliate centers here in the US and Canada.  This is up by 6 centers compared to just one year ago!!  My point is that we are making progress, but we still have SOOOOOO much more to do. So, so much more to do.

So, I ask of you, if you are so willing, would you please make a contribution on this day to the PCD Foundation?  Your contribution is tax-exempt and will be used to accelerate programs designed to improve the lives and healthcare outcomes of those with primary ciliary dyskinesia (PCD), like myself.

On this Halloween day, could you spare $31, for October 31st?  Or, maybe a calendar to go in your stockings this year?  Or, maybe you want a cozy t-shirt or sweatshirt showcasing PCD for this winter?  Thank you in advance to anyone who can donate, it means so very much to me!!!  Below are the links to the calendar, the apparel (and other merchandise) and of course the PCD Foundation Donation page if you simply would like to donate.  All of these methods, funds will go to the PCDF!!

PCD DONATION PAGE - https://pcdfoundation.squarespace.com/donate-now/

PCD APPAREL - https://shop.spreadshirt.com/breatheforpcd/

PCD APPAREL - https://shop.spreadshirt.com/RunningOnAir

PCD CALENDAR - https://www.yearbox.com/calendarsforgood/breathe-for-pcd-calendar/

TRANSPLANTAMOON.

Waking up today was much calmer and better than waking up the past few days ... that is because I got GOOD, no GREAT news!!!  My PET scan gave every indication to the doctors that the nodule they're following in my lungs is NOT malignant (yay, scream, happy dance!!).  This is by far the best news I've received in a while.

There is a little kicker however ... the doctors want me to have a repeat PET scan in 3 months to re-evaluate this sucker.  If the nodule is the same size, or larger, I will have to get a biopsy done to further rule out malignancy.  If the nodule shrinks (or even better, vacates the premises), no biopsy will be needed.

So...what does this means for going "active" on the transplant list?  Well, I can't go active until the nodule situation is cleared up (i.e., they determine in 100% certainty that it is not cancer).  The doctors are trying to AVOID a biopsy at all costs b/c the procedure is risky, and even more so for me and my already fragile lungs.  So, I am in somewhat of a stall period right now for another three months.

I was (and still am) at a point of being mentally, and physically ready to go active on the transplant list, so in one sense it is a bit disappointing that we can't move forward just yet (never did I ever think I'd say I was disappointed to not be listed) ... but in all honesty, I am just so thankful that the scan was negative, that is the main consideration.

Thank you for all the calls, texts, emails, etc over the past few days.  Waiting for critical results like this can be torturous.  For now, we try to keep me as healthy as possible and enjoy a few more months of "pre-listing" life.  As soon as I go active, I will have some limitations I have to abide by (like no travel more than 3 hrs from Houston, no alcohol, etc), that will extend beyond transplant as well.  In all reality we could be Houston bound for at least 2 years once we go active, so my little brain is wanting to get one last mini-vacation in, if my health allows ... we shall call it the "TRANSPLANTAMOON".  People always go on "honeymoons and babymoons" before getting married and before baby's arrival, so I say we do a "transplantamoon" before I get my miracle of a double lung transplant!!

Cheers and much love. 

Rebs

Hurdle Journey

Another day, another hurdle. Sometimes it feels like that is all I do lately, attempt to handle and jump life's hurdles.  

Last week we met with the transplant team.  I completed all of my lung transplant evaluation testing finally, and we were to meet with the team to determine a plan going forward.  A plan which we had mapped out in our minds going into the appointment.  A path and plan which I was (and still am) ready to travel, but we were met with yet another hurdle in this transplant journey.  The team has been following a nodule in my lungs now for about 9-12 months, since my thyroid cancer diagnosis (and subsequent chest CT scans after surgery).  I had a repeat CT scan last Wednesday, and it again shows the nodule.  Good news is that it has not changed sizes, and due to the chronicity and nature of my lung illness, the doctors feel quite certain it is not cancer, but it is likely scaring or anything but a malignant tumor.  In order to be listed however, they have to know in full certainty that it is not the big C.  I try to find comfort in the doctors reassurance that it is likely nothing, however, I would be lying if I haven't thought about the "what if" scenario.  I was told my thyroid nodule was "likely nothing", and that turned out to be malignant.  

So, on Wednesday of this week, I will have a PET scan to determine the nature of this nodule.  Cancerous tumors/cells require and use a lot of energy compared to healthy cells.  The PET scan will give the doctors a very good indication that this nodule deciding to take up residency in my lungs, IS NOT of the malignant type, and that it too can go, when I get my miracle of new lungs ... at least, that is what I keep telling myself.  I tell myself this, b/c the alternative just can not be an option.  So those of you reading this get a perspective, I'll be frank with what this means.  If it were determined that this nodule is malignant, my chances of being eligible for transplant would be extremely minimal.  One would think that they could just take out the bad lungs (and malignant nodule) with a transplant, but that is not how it works.  Without getting into all the details, I'll just leave it at this ... we HAVE to have a good result on Wednesday's scan, for my eligibility, for my future. 

This too is a hurdle, and hopefully it is one that I can jump over gracefully as I continue around this racetrack, with the end goal of happy, healthy breathing days ahead.  After this scan is completed (and we get favorable results!!), my case will be presented to the medical review board for the final decision and clearance to go active on the transplant list.  If all goes well, I could be listed within the next week or two.  

Please keep myself and my family members in your thoughts this week and in the coming weeks as we (hopefully) make a the transition to going active on the transplant list.  I'm very hopeful and excited to think about a chance at better breathing, but I am also dealing with fear and anxiety as we go through these final tests and wait to hear whether I can be listed.  I know my incredible husband, my amazing parents and brother, and my in-laws are also experiencing some of these emotions, so please reach out to them.  Let them know you are here for them.  I've said this before, and I will say it again ... it's very hard as a patient, but it's also incredible hard on loved ones and they need as much, if not more support as I do. 

Laces are on and I am ready to jump this hurdle. 

Much love.

Rebs

Breathe Again.

Over the last few days, I've had a little bit of what some might call an "epiphany", or what I call, a nice kick in the butt.  You see, about 4 days ago, a friend of mine here in Houston (who also has PCD and is listed at the same transplant center I am being evaluated at), got her miracle.  She let me know she got "the call", and about 12 (long) hours later, they finally wheeled her into the OR after all things point to a go.  Her surgery went great, and her recovery has been relatively smooth.  She's actually doing FANTASTIC after going through what she went through, and she continues to improve her numbers every day.  She's gaining her strength back, her new lungs are opening up, so her lung capacity continues to rise, and she's no longer attached to oxygen.  The road ahead is still a very challenging one, as it is for anyone going through transplant, but she is just beginning a new life, with new healthy lungs and I couldn't be more happy and excited to see her flourish and explore life breathing easy!!

Over the weekend, I was fortunate to be able to go visit her in the hospital and, WOW, to stand before someone and know that they just went through a life saving operation...to see how good she looked (despite how she probably really felt)...there really are no words.  That day, I also met her brother, who is a TWO TIME, double lung transplant survivor!  He will be celebrating 6 years with his lungs this year.  We were able to visit a little before I saw Barbie, and the value I take from experiences like that are just priceless.  As I reached the ICU to see my friend, I was also flagged down in the hall by a woman I didn't recognize.  She didn't speak much English, so she signaled to me that she knew someone in a room I had just passed, and apparently that person knew me.  I looked in to find a young gal who I had been in pulmonary rehab with a few months back ... and she too, had gotten her miracle!!  This gal is 24 years old and has Cystic Fibrosis.  I've wondered about her since I ended my Pulmonary Rehab, and then here she was.  In one day, in less then 1 hour, I witnessed 3 miracles, 3 double lung transplant recipients.   Seeing how well these three amazing people are doing, knowing they faced such battles and are doing okay, really hit home for me.  It made me think, "I too, can do this".  Am I still scared shitless??  That is a BIG yes.  The transplant journey is extremely fragile, tough, ever changing ... but it is remarkable, and can truly change your life for the better.  To be able to do things I once used to, or things that I've never been able to do b/c of my hindered breathing, WILL be amazing.

No one transplant experience is exactly the same, and I am realistic but optimistic about my outcome from the transplant.  I have been able to gain a greater sense of hope that I will not just survive transplant, but thrive after it.  I have a renewed sense of bravery instilled in me and a new sense of drive and purpose that had been somewhat dimmed by fear and by uncertainty.  I HAVE to stay strong and healthy for transplant.  I've seen how important it is to stay as active as possible many times over.  Barbie is a great example, as she was very diligent about doing her exercise every morning.  This only makes me want to continue to do my best at working out, eating right and staying strong.

I've finally scheduled my last test for transplant evaluation, a right heart catheterization, and soon after I will see the transplant team.  They recommended at my last appointment that it was time to "go active", and I think I am just about ready to take that giant step, that giant leap of faith that I too will be OK.  When and if my time comes to receive the gift of life, I can look to these amazing people, and know that I can learn from their experiences, lean on them in times of fear and uncertainty and count on them to help support myself and my family through the ups and downs of transplant life.

This all would not be possible however, without the real heroes, the donors and their families who make that unbelievable decision in a time of great sadness.  Thanks to their decision, they've saved countless lives, given people who have been deathly ill, a new life.  There aren't enough, or the right words to express how much this means to so many people and their loved ones.  One day, my life might be changed by a donor, and I will forever be indebted to that person ... one day, two lungs, a new life.  Until then, I try my best to stay strong, stay positive and live life to it's fullest.   Please keep my friend in your thoughts as she continues her path to recovery, she's a rockstar!!

Barbie and I, 3 days post double lung transplant

Living, Not Just Surviving.

It's taken me about a week to come to terms somewhat with how we left the transplant team last week.  You see, for the past two years I have held steady with my pulmonary function tests that give the doctors an indication of how these airbags are holding up.  And, I am happy to say that they are still hanging on.  I'm still at a whopping 26% lung capacity and I walked all of 1350 feet, using 3 liters of oxygen ... all of which are very similar to the past few years.  So, what's different? Why then did they mutter those terrifying words, "We think it's time to go active"??  I'm not going to lie, I pretty much "black out" at these appointments, well, not literally of course, but they're talking, I hear what they're saying ... but it doesn't sink in.  This is why I am so glad I have the support I do.  This time, Bob and I sat in the cold, small, sterile exam room as we heard the words we have known would come, but are always so glad to NOT hear when we're there.  "Active".

And, it makes sense.  After I get over the extremem emotional rollercoaster of what is happening, I can think clearer and I slowly turn the corner emotionally with a situation I've been terrified of since it was first mentioned to me when I was about 20 years old, 13 years ago.  A double lung transplant.  Can you imagine, they basically cut you in half, take out your old lungs, one at a time, disconnect blood flow, nerves and reconnect and replace those cavities with another person's former lungs. A generous human soul who is giving the ultimate gift ... life.  A second chance.  It's mind boggling.  It makes you think crazy thoughts. It tears you up both mentally and physically. But it's also exciting, and fills one's heart with hope, and a sense of purpose that has slowly been stripped away.  It gives me a glimmer of hope that one day I MIGHT be able to actually live a life not attached to oxygen, in pain or so fatigued from just being awake.

I told my husband the other day when I was having a particularly emotional hour (yes, it changes hourly these days, call me crazy, lol!), that I feel so robbed.  PCD has taken so much from me.  It has stripped me of my ability to do so many things that I once enjoyed.  Sports, running, working, traveling, sleep ... just to name a few.  These days, EVERYTHING is so much harder than it's ever been.  The little things that I loved to do, aren't as much fun anymore because they're so damn hard.  For example, shopping.  Many people (especially Bob) can attest to my love of shopping ... (for anything really) ... Well, now I have to cart oxygen around and my energy has plummeted so, it's not as fun as it used to be.  I've had to "ration" or monitor my energy out like a diabetic does their sugar levels.  I feel like my energy is on a budget.  I have to prioritize now more than ever.  If I have a particularly busy day one day, the next day I am pretty worthless.  I say all of this not for pity, but because this is something that has changed, and leads the team to think I should go active.  They consider quality of life a big indicator of timing for transplant.  There is a term they use called the "transplant window".  This is the time frame of when you are sick enough for transplant, but not too sick where they're concerned you won't make it through surgery.  I am in that window, and the fragility of my situation is very real.  I've got to stay healthy and strong enough for transplant, and that my friends is my new goal in life.  I am fighting for a second chance at life, and if I am lucky enough to get that chance, I will be forever indebted to someone.  I want to live, not just survive.

So, the plan going forward at this point is as follows.  I have to update some tests for the transplant list.  I will have to have a right heart catheterization, a CT scan, some blood work and maybe a few other tests.  I plan to do this in February and then we will meet with the transplant team in March to review results, crossing fingers there is nothing wacky with my tests since the last round in 2014.  They will then present my case to the Board and a decision will be made as to whether or not they will consider me for transplant.  If accepted, they will give me what is called a "LAS" or "Lung Allocation Score", which basically determines how high or low (or critical) you are on the list.  The lower the number, the "less sick" you are.  If you are lower on the list your wait time is anticipated to  be longer.  They guesstimated I would have a LAS of about 35-38 and a wait time of 6-9 months.  We were told once you are at about 40, is when "the calls" start coming in and wait time is about 3-6 months.  We were told to anticipate "dry runs" ... meaning they call you for lungs and can go as far as prepping you for surgery, but last minute the lungs are deemed no good for transplantation.  Of course, once you go on the list, you can be called anytime, of any day, so one has to be prepared to go into surgery once you are active on the transplant list.

Imagine, every time your cell phone rings, wondering if it is a call that could save your life?

Thank you all for taking the time to read my story.  I wish that my health didn't put us in this situation, that it didn't cause so much worry to the ones I love the most.  Besides actually living with such a severe disease, seeing the hurt and worry that my husband, my parents, my brother have go through, is the hardest part of it all.  I am determined though to put up the strongest, hardest fought fight of my life in this entire process.  On we go .... !!!!

Rebecca