Going Down the Road Feeling Bad

Tuesday day started off like any other day. I woke up, showered, did my treatments, surfed the internet, and got ready for my day. However, the day was most certainly NOT like any other day.  Who was I kidding? It was the day University of Pittsburgh Medical Center (UPMC) would be meeting to decide if they would take me as a candidate for lung transplant.  After being declined by Duke University in December, UPMC was the center that might take me when no one else would. Yes there were interviews with other centers (IU & UK) lined up, but honestly if UPMC didn’t take me who would?  UPMC is well known for taking the sickest of the sick, the riskiest of the risky, and doing it all with better than the national average outcomes. During my visit it seemed that everyone I talked to reassured me that “Oh our son/daughter/spouse (insert name of really sick person) was turned down by center (insert name of one or more transplant centers) and UPMC was willing to take him/her.

What makes my case complicated? Well I have a history of rectal cancer. Thankfully that has been cured for more than two years and no nodes were involved. Two years out is enough for most centers. Also, I have multi-antibiotic resistant pseudomonas aeruginosa (MARPA). If these resistant bugs grow in your lungs then it can be another reason to be turned down. Lastly, I have esophageal peristalsis and reflux. This means that my esophagus has little movement and also that stomach acid comes up my esophagus and damages it’s lining. It can also damage the inside of a new set of lungs if left untreated. This is the reason Duke declined me. They thought my case was too severe. However, UPMC was willing to evaluate me.  During my visit, the head of the program Dr. Pilewski assured me that he knew all my issues and wouldn’t have brought me there if he didn’t think I could be a good candidate. Of course that was with the reservation that hopefully none of my other tests during evaluation showed anything weird. Since most of these tests were repeats of ones I had at Duke, I was feeling good on the road home from UPMC last Sunday.

So back to my day on Tuesday… I left home on the way to pulmonary rehab. I was really glad to have rehab as I needed the distraction to keep my mind off the UPMC meeting. Rehab went fine but I did need four liters of oxygen for some of my exercise which is more than what I’m used to. I left and drove north to run a few errands. At my first stop I thought to check my oxygen tank. I’d forgotten to refill it at rehab and noticed I was running low. Of course my thought was that I could squeeze in a quick lab draw at IU North Hospital before running out. At the hospital I started walking to the door and did not get far before feeling pretty short of breath. The weather was wet and cold so I decided to abandon ship and head for home. I started heading south on very busy Meridian street in rush hour traffic.

Then I really was headed down the road feeling bad. I started feeling light headed. A quick check using my pulse oximeter revealed an oxygen saturation of 72. YIKES!!! Anything below 88 is not where you want to be without oxygen. Obviously my supply had run out. In a panic I dialed 911 and told them my location. But then I got so short of breath I thought just the act of talking would possibly make me pass out. Would the EMTs get there quick enough anyway if I pulled over? Would they find me unresponsive and blue in the face? A mind without oxygen starts to get a little weird and I was starting to really freak out. Imagine having someone suffocate you with a pillow while you are driving during rush hour. That was me Tuesday.

In a flash of brilliance, my mind did remember where I could get oxygen close by. My mom worked at a surgery center less than a mile away. They did surgery so I knew they would have oxygen for sure. I got in a left turn lane to head that way. The left turn light was read. In a flash of stupidity mixed with fear, I slowly cut across three lanes of oncoming traffic while beeping the horn constantly to avoid collision. All the while 911 kept redialing me constantly trying to locate me. Thankfully I made it to the surgery center and laid on the horn again. A nurse getting off duty helped me. I told her to get O2 and that my mom worked there. Minutes later nurses descended and hooked up to O2. Then the ambulance came. They had been driving up and down Meridian looking for me. I’m still not sure how they found me. It might be that the nurse answered my phone calls from them. Honestly, my mind was reeling so much some of the experience is a little foggy. My mom came out next and joined me in the ambulance. My blood pressure was 200+ over 100+. Normally it should be 120 over 80. My O2 saturations did return to normal. We went back to IU North Hospital to get checked out just in case. Maybe I should never have left there???

The rest of the evening I felt pretty ragged. It felt like I sprinted a marathon. By Wednesday morning my body was back to baseline and I kept busy trying to keep my mind off the UPMC decision. They are supposed to call by Thursday and let me know. So here I am awake at 4:00 on Thursday writing this blog. It’s way more therapeutic than tossing and turning in bed. Between the prednisone I’m taking and the heavy things on my mind sleep is not the easiest thing to come by these days.  Here’s to hoping for a day of good news and safe travels. IU Medical Center is my next stop.  Leave the light on for me and remind me to pack some extra O2.

The Road to Transplant

Many say the path to transplant is a long and winding road. Right now for me it feels like a roller-coaster. Just after Thanksgiving I was evaluated for a lung transplant at Duke Medical Center. It was a crazy and exhausting week of many tests. The surgeons felt I might be too early for transplant. The transplant pulmonologist said it might be time to get listed. I expected somewhere in between. My husband Randy & I returned to Indiana prepared that we might be leaving home again soon. We knew that when it was time to get listed that we would be OK with it even if our time on the transplant road was shorter than expected. Although in reality I’ve been on this road since my first evaluation in 1992. So I guess that’s a pretty good trip!

Right after we got home I noticed that my output (aka poop) in my colostomy had stopped. After trying many things at home, my physician told me to go to the emergency room so they could get a CT scan of my abdomen. This scared the heck out of me. I had surgery in May 2010 to resect a blocked small bowel portion. It resulted in a month long stay part of which was in ICU on BiPap assisted ventilation. I really wasn’t sure if I could do it again.

Just as we were getting ready to leave for the ER, Duke called. The transplant coordinator told me the news I least expected. Duke rejected me as a transplant candidate. I was shocked. I failed to meet their criteria due to esophageal aperistalsis (dismotility) and reflux. I felt like someone punched me in the stomach and drained all the blood from me simultaneously. I’ve worked so hard to stay on this journey and now a road block had suddenly appeared.

Luckily my bowel obstruction was only partial. I spend the next day curled up on the couch in some pain as the obstruction passed. My mom spent the day with me as we both dealt with the emotional turmoil over my rejection by Duke. Lots of tears were shed and by the end of the day we felt not exactly better but at least emotionally numb enough to deal with it.

By the next day I decided that curling up in a ball would not work as a life strategy long term. So I set about to find a solution to my problem. I put posts on nearly every CF and transplant forum and Facebook page I know. After checking far too frequently I finally got a good hit. A woman named Irene had been in a similar situation two years ago. She ended up at University of Pittsburgh Medical Center (UPMC) and suggested I message her so we could get connected. This meant the world to me and gave me hope again. A few others replied as well. It’s such an amazing thing to be able to find someone to talk to on an issue that is so narrowly focused (thank you internet!). Ilene and I talked a few days later. She answered so many of my questions and reassured me that UPMC might be a viable option. Her openness about her journey and willingness to help others along the transplant road make Ilene a great resource for others. We could use many more Ilenes in this world.

After talking to Ilene, I decided it was time to start contacting other centers. Hubby Randy said “Hey now you have another project to work on.” He knows me well enough to know that I need to be engaged in activity to keep sane. So for now I have been calling, faxing, and emailing various centers to find a good fit. I’m hopeful again and have faith something will work. I’m back on the road again and it feels good!

20 Years & Tasty Beers

It’s that time of year and it’s time to think beer. Since it's inception in 2000 I've been the chair of the Festiv-ale beer tasting event to benefit the Cystic Fibrosis Foundation. Each year is great but this year’s event is really special. This year I celebrate a milestone.

 

‎20 years ago this month I almost lost my life to CF. One morning I woke up and felt like there was an elephant on my chest. I had a serious pneumonia and was in right heart failure. At one point I was too weak to even walk from the car to the house and needed to be on oxygen 24/7. My only option was being listed for a double lung transplant. However, at that time a new drug called Pulmozyme was in clinical trials funded by the CF Foundation. It gave me hope. I exercised to gain strength for transplant but also in hoping I wouldn’t need one. I was lucky. It worked. Just two years later I started Pulmozyme, was off oxygen, and was taken off the transplant list.

 

Now twenty years later it feels like the movie Groundhog Day. Once again I’m about to be evaluated for transplant. Once again I’m using oxygen (at night and with exercise). Yet once again there is hope. The new hope is a new drug VX-809 from Vertex Pharmaceuticals. They are enrolling for stage 3 of a clinical trial funded by the CF Foundation. Vertex recently released a drug called Kalydeco. It is saving lives for people with the G551D genetic mutation. However, the vast majority of those with CF (including me) have the Delta508 mutation. The hope is real and it’s only a few years away.

 

Festiv-ale is about tasting beer, but it is also about raising a glass to help raise money to support this research. Join us on September 22^nd. We will be celebrating beer and we will also be celebrating life. Hopefully for another 20 years.

 

Tickets are on sale now for $45 and can be purchased by calling the office at 317-202-9210 or at http://indiana.cff.org/festivale. You can also make a donation at this link for those of you that cannot attend.

 

Thanks and Cheers,

Steph

Festiv-ale Chairperson

CF Adult

 

RSVP on Facebook: http://www.facebook.com/events/455436567823344/

Follow us on Twitter: @FestivAle_CFF

Eye of the Tiger

Cue the cheesy song “Eye of the Tiger” by Survivor from Rocky III:

“Risin’ up, back on the street
Did my time, took my chances
Went the distance, now I’m back on my feet
Just a man and his will to survive
So many times, it happens too fast
You trade your passion for glory
Don’t lose your grip on the dreams of the past
You must fight just to keep them alive

It’s the eye of the tiger, it’s
the thrill of the fight
Rising up to the challenge of our rival
And the last known survivor
stalks his prey in the night
And he’s watching us all with the eye of the tiger”

So many things in this song remind me of the ongoing battles we have with CF. We “go the distance” each and every time we have an exacerbation. For Rocky it was the “thrill of the fight” that kept him going. Like Rocky winning against CF is a motivator for us to carry on. Other times we have to reach deeper to keep up the fight. Maybe a loved one or upcoming life event keeps us going. Otherwise we rely simply on only our “will to survive”. Regardless of motive, the important thing is that we just keep trying.

Recently, I’ve been struggling with motivation. My workouts have been sporadic and not well thought out. Consequently, my energy and strength are down and my oxygen use is up. To be honest I’ve been lazy. This is not my norm. Usually I’m running at 110%. My first instinct is to blame CF and think maybe it’s time for antibiotics or other medical intervention. But a trip to my pulmonologist revealed that my lung function is stable and I’m doing relatively well. This trip brought me to the realization that it’s time for ME to make MYSELF feel good. It’s time to get back the “eye of the tiger” and get serious.

So I recently started a six day a week exercise program. My program includes yoga, pulmonary rehab, and weightlifting. I made several spreadsheets (yes I’m an accountant by trade) to chart my progress and keep me on track. It’s been over a week and already my energy level is up. I’m also sleeping more soundly. My muscles are tightening and my lungs are loosening.

Ask yourself if you’re doing all that you can. If not don’t feel bad. Recognize that maybe you need a mental holiday from it all once in a while. However, don’t stay on that holiday too long. Rocky didn’t quit and neither should you. Rise up to the challenge of our rival CF!

Living the dream

Considering it's been a year since my last post I suppose it's time to catch you up on what's up with me. Lately a lot has been up. But first let me digress to last year. As many of you may or may not know the beginning of 2011 was filled with several hospitalizations including another abdominal surgery. After all that I declared the rest of 2011 to be a year of recovery for both Randy & I. It was a time to stay close to home and rest. It also gave us time to finish projects on the house and get our lives back to some sense of normalcy.

Feeling rejuvenated I began making plans for 2012 or what is know known as "the year of the Rath's". My goal for this year is to see many of our friends far and wide. I do this also as a way of doing, seeing, going, as much as possible before my health gets worse. At that time we will most likely return to a staying close to home mindset. So far we've done our annual music cruise, attended Mardi Gras, taken a road trip (including dog beaches) with our pups Rocky & Rosie, camped at a music festival, and recently spent time at lakes in the southeast with family and friends.

All of this travel is not physically easy but it is good for the soul. In my retirement I try to keep busy while at home. However, getting away helps to break up the weekly routine and creates memories that last a hopefully long lifetime. During our most recent trip our friend Ed said "we're making memories here". So right he is.

The downside of travel is that it interferes with my exercise routine. In a perfect world I would get up early and get in workouts even when on the road. In reality that's much harder. I've been holding my own lately but don't feel at the top of my game physically. Today I saw my pulmonologist. The good news is my lung functions are the same as my last visit in April. My lungs sound reasonably good as well. This is all great news considering how rough last year was.

When I see someone they almost always ask how I'm doing. If being truthful, I would say "Well I'm more short of breath than I used to be, get tired easier, and am using oxygen more." But my new response is "I'm doing great. I'm living the dream."  "Living the dream" is how I feel we are living right now. Randy & I are lucky to have the friends to visit and the flexibility to do so. He can do his job from anywhere that has internet access. Since he's his own boss now he can work when he wants to. Of course I'm still not working which has been great for stabilizing my health.

Our next adventures include a transplant workup in November at Duke in North Carolina. Until then we will visit more friends and continue living the dream. I'm lucky to be alive and even luckier to have a life I love.

With gratitude,
Steph

Three Little Pigs

Yes it's been quite a while since my last post. Part of the delay is due to me mentally needing to distance myself from all that has happened. The other part of the delay is due to a need to get my life back in order after everything.

Here's a brief summary of what's happened since August of 2010:

August: Went to Duke University for a lung transplant evaluation which consists of almost a weeks worth of rigorous testing. After this testing I also elected to have a colonoscopy to investigate rectal bleeding. The result was a diagnosis of rectal cancer.

September: Began radiation & chemotherapy to treat cancer.

October: Hospitalized for a painful bowel obstruction (three days) and low potassium (one day). Began inhaled antibiotics to treat a respiratory infection.

November: Surgery performed to remove rectum and create permanent colostomy (ten days of hospitalization.

December: Began inhaled antibiotics to treat a respiratory infection.

January: Hospitalized for pancreatitis/bowel obstruction (11 days).

February: Hospitalized for respiratory infection (10 days).

April/May: Hospitalized for bowel obstruction, emergency surgery to remove nine inchese of small bowel, and post surgical respiratory infection (32 days).

Yes the last hospitalization was a doozy. Not only was it long, it was also painful and at times frightening. I was about ready to be discharged (after two weeks) when I started to feel very short of breath. The cause was a pneumonia and I had to have assisted ventilation via a BiPap machine. This was my first time ever having a machine help me breathe. At the time I was very thankful for the help of it. In retrospect I'm still thankful but I also recognize this is another indication that my disease progression is getting more serious. Hey at least I wasn't on a vent though.

After discharge I was very weak and tired both mentally & physically. Not only was I in the hospital for 32 days, it was my sixth hospitalization in seven months. Rebounding after all that was not easy. To be honest there were many mornings I woke up thinking "How am I going to get through the day?". Then one day I received a little glass flying pig named Penelope (thanks Amy!). This particular Penelope was the third I'd received since October. Obviously people know I have a fondness for flying pigs ;) Penelope the Piggie comes with the following message: With her head-strong determination and good fortune, Penelope is the perfect depiction of overcoming impossible odds! Her personal wager against anyone using the phrase "When pigs fly!" has brought much abundance and prosperity indeed.

Penelope had been trying to get my attention but maybe it took a third one "landing" to really sink in. I just needed her to remind me that I am "head-strong and determined", have "good fortune" and most certainly have been "overcoming impossible odds". From then on I got up every morning trying to think of Penelope and trying to get my life back.

So began a serious effort to workout, gain weight, work on projects to improve the neglected house/yard, see friends, etc etc. Once again I feel like myself again. Yes there are times it's still difficult. I still wonder when the next illness/hospitalization will come. Hopefully I can always remember to think of Penelope when the difficult times do come. All three Penelopes are on my mantle in case I need a reminder. Thanks Penelopes!!!

P.S. Please attend Festiv-ale a Celebration of Beer Tasting. The event is Saturday August 20th and benefits the CF Foundation. If you can't attend please consider making a donation. More information can be found here: http://indiana.cff.org/festivale

There's only so much you can control

Well here I sit again...in the hospital. It's an odd thing being in again. It's somewhat like the movie Groundhog Day. I come home get settled back into a routine and then whamo it's time to check in again. Yes maybe I'm being a bit melodramatic. But I've had five hospitalizations since the end of October. When you start hitting those numbers things just get a little surreal. Life has been such a blur that I even forgot one of the stays. I was thinking "oh this is just my fourth time in" but my mom reminded me of an overnighter and then it was "d'oh" headslap time.  I keep asking the hospital registrar for frequent flier miles but no such luck yet. I'm guessing I'd be platinum elite concierge level by now if they had them.

My past stays were due to cancer complications, surgery, pancreatitis. This time my lungs finally got pissed about being ignored for so long. It all started with night sweats which lead to fever spikes and then pain every time I inhaled. My lungs felt tight, gunky and generally pissed off at me.  I had the choice to try IV meds and steroids at home. But after talking to Randy & my mom decided it was time to "go big" and get admitted.

So today is day six of my stay and things are going very well. I get respiratory percussion treatments four times a day. My lungs are getting really cleaned out. I'm also on some really high powered antibiotics and some steroids. The steroids make me feel a bit manic in that I'm not sleeping well and have lots of crazy energy. Can you say lots of to do lists? It seems there are to do lists & projects all over this room right now. And of course I've only done some because I think of new to dos as fast as I do them (if not faster).

The antibiotics are my biggest cause for concern right now. Yes they are working. However, I'm running out of antibiotics that can effectively fight the bugs (psuedomonas aeriginosa "PA") in my lungs. PA is a very smart germ. Once it has encountered a new antibiotic it learns how to defend itself from future attacks by changing its structure. So as time goes on an antibiotic can become less effective against it and then basically is of no good at all. This is called resistance. Right not I have a strain of PA that is resistant to almost every antibiotic except one or so. This means a few things: 1) eventually this PA could grow out of control and damage my lungs beyond repair, 2) if I am transplanted there is somewhat of a risk of having this PA strain in my sinuses or trachea and transferring it to the new lungs. Either possibility is scary and not good. This realization hit really hard one night after talking to my infectious disease doc. I cried and struggled to come to terms with my own possible mortality.

So much has happened lately and the possibility of getting transplanted down the road has always been a hopeful goal to focus on. If the bad bugs exclude me from that it would be a hard thing to cope with. After a night of talks with Randy, my mom, and friends I felt much better the next day. What I've come to realize is that I'm doing everything possible to stay well and still enjoy life. If transplant happens it happens. If not I'll be prepared for that too. Either way I'm doing all I can and the rest (including this nasty PA bug) is beyond my control and I'm trying to find peace in that. Of course I'm not perfect and yes crying is allowed and frankly an honest expression of it all.

Of course it's not all gloom and doom dear reader. If you know me you know I can try to find a bright side on a dark rock in a dark cave in the pitch black night. Lately I've been in the cave too long but I'm emerging stronger than ever. So here's the upside:

• The PA in my lungs is a small population. So for now it may not be an issue for transplant purposes.
• Considering all the setbacks my body has taken related to the cancer "cure" it's amazing how well the lungs have held up. I mean this is the first time I've done IV antibiotics since December 2009. Thank you lungs!!!
• Duke, where I was evaluated for transplant in August, specializes in transplanting Cystic Fibrosis patients that have drug resistant organisms. They have a policy that I need to be cancer free for two years before transplant. Due to my radical cancer surgery and clean nodes I'm hoping an exception might be made if I need a transplant sooner than that. My local oncologist feels my treatment cured the cancer and no waiting period is necessary.  I plan to touch base with Duke soon to find out their current assessment of my situation.
• This wake up call is prompting Randy & I to evaluate doing some things that we've been wanting to do now. Why wait?

Thanks for listening. There's only so much I can control and the rest will be alright :)

P.S. Right now I'm hoping to get out of here by this Friday Feb 11 or Saturday Feb 12.