Blog is relocating for a bit

Dear Readers,

My blog is moving to the following website for the time being:


www.cotaforstephr.com


Please check the blog section of this site for updates on my transplant journey. This site was created by my friends to raise money for the Children’s Organ Transplant Association (COTA) in my honor to help with transplant related expenses.

There is no donation needed to continue reading my blog. It is just moving so that all of my transplant related web activity is housed in one website. This website will also have a guestbook, photo album and some other features that will be cool.

I also have a Facebook page if you care to follow that:


https://www.facebook.com/PigsFlyAway


Thanks so much for being part of this amazing and exciting journey. I really couldn't do it without all your support, love, and laughter.

This pig has wings and hopefully lungs soon,
Steph

Home is where the heart I mean lungs are

Hello all,

Just ten days ago I was strapped to a gurney and being transported via ambulance from my home hospital IU North in Indy to my transplant center UPMC in PIttsburgh. My mom rode with me while Randy, our Boston Terriers Rocky & Rosie and all variety of our things rode ahead in our wagon. Our original plan (before the pneumonia) was to travel to Pitt on a housing search so we could find a short term apartment that was pet friendly, clean, and safe. Then we would return home, pack up our stuff and make the move in an orderly fashion.

Unfortunately having CF does not always make keeping plans possible. The weekend before we were supposed to leave I started spiking fevers including a whopper of 103 degrees. So the new plan was to admit me to IU North, get me stable, and then transfer me to UPMC in Pittsburgh. While all of this was going on, Randy & I decided on an apartment sight unseen relying only on photos and information from the internet. We also checked with the UPMC social worker and she said the apartments were nice.

So while at IU North Randy kept busy during the days packing all sorts of household goods, clothes etc. My mom stayed with me some at the hospital during the day and I had many friends visit. Then we transferred to UPMC. While Randy checked into the apartment, unpacked and set up our new home, my mom was by my side days and some nights making sure I was OK. Randy would come in the evenings and some during the day as well. Although this was far from easy or ideal we adapted as all those dealing with CF must.

The first few days at UPMC were a little rough. I backslid a little and was very short of breath and needed lots of oxygen. The simple act of walking to the bathroom was tiring. Thankfully they increased my steroids to open my lungs up and I felt much better.

The best part about being in the hospital at UPMC was getting to develop a relationship with my new transplant/CF doc Dr. Pilewski. He is a very bright and caring person. I'm lucky to have him in my corner. After a few more days we both decided to discharge me last Friday. I say both because that's how he operates. He knows that I know my own body better than anyone and trusts me to help him make the right decision about discharge. This is EXACTLY the type of doctor/patient relationship that I have with Dr. Goodman back home and I felt immediately comfortable with Dr. Pilewski because of it.

After discharge I finally got to see my new "home" for the next few months. The apartment is really spacious and bright with lots of windows. Our view is not the best as we look out on the street. But since we are not permanent residents I guess we get the short end of the stick on locations. The good news is Rocky and Rosie love all the activity and passers by. Also, the complex is right on one of the three rivers in Pitt and has a great walking path for the dogs. Having them with us really makes it feel like home. Now if I can just get some new lungs our new home will be complete :)

Peace,
Steph

Sorority Rush

The more transplant evaluations I do the more they remind me of sorority rush. You meet many people in an organization and get to know them and vice versa in a very short time frame. You do your best to look well groomed, be friendly, ask important yet appropriate questions, and divulge enough information so they know you but not so much that you annoy them. When I rushed in college the first time my heart was set on Delta Gamma. They were very focused on academics yet still seemed to have a reasonable amount of fun. They had a beautiful house. Their symbol was an anchor and it all seemed so nautically magical. I thought for sure that they loved me as much as I loved them. In fact I even bought a cute cardigan with an anchor on it before knowing their decision. That’s how confident I was the feeling was mutual.

During my visit at Duke I loved them so much that I bought scrubs and a t-shirt with Duke Med on them. It seemed like the perfect place with a beautiful campus and really smart people who worked there. I was willing to do whatever it took to be a “dukie”. In the end I was declined by Duke just as I had been declined by Delta Gamma many years ago. They had valid medical reasons for not taking me due to my esophagus issues.

After Delta Gamma declined me I rushed again. This time I was given an offer by Alpha Xi Delta. I gladly accepted. They had smart girls to and I felt instantly at ease there. Was it where I first thought I’d be? No. Was it the best place for me? Yes. There I made lifelong friends that have made my life richer and happier.  During this second round of transplant evaluations I’m looking for my Alpha Xi Delta.

At UPMC in Pittsburgh, I think I may have found it. They take the cases that Duke rejects due to esophagus motility. The head of their program Dr. Pilewski is also the co-director of their cystic fibrosis clinic. He’s smart and he really gets what those of us with CF go through. I felt instantly at ease with him. They have extensive experience dealing with my complex issues and take on the riskiest cases. Waiting for their decision was one of the hardest waits I’ve ever had to endure. I knew if they rejected me then probably no other center would take me. Finally on Friday I got their call. My coordinator Paul left me a voicemail saying I was accepted as a candidate for a double lung transplant.  I will never forget sitting in my car with my husband as tears streamed down our faces. A huge burden had just been lifted and I was so relieved, overjoyed, and grateful.

Later that day my local center Indiana University said no. They did not feel comfortable with the amount of experience they had dealing with my issues. I’m also still considering University of Kentucky in Lexington. We meet with them tomorrow. I’m ready for rush to be over to be honest. But the good news is I’ve got one offer from a place that feels right to me. Do I have their t-shirt yet? No. But I do have a set of Duke Med scrubs if anyone is interested. The cardigan sweater with the anchor is long gone though.

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!