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.

A pancreas and a pig

First let me start by saying we made it on our annual music cruise!!! It was a MUCH needed voyage for both Randy & I. Since my cancer diagnosis in August our lives have seemed a constant stream of tests, illnesses, treatments, and hospitalizations. I’ve been worn out physically & mentally but so has Randy. When I’m sick he not only carries his usual load of housework, cooking, cleaning etc but he also tends to my needs. In addition he runs his own business from home designing printed circuit boards www.pcbdesignpro.com (shameless plug). Even though he’s “healthy” all of it takes a toll on him too. I realized how much he needed the trip when he woke up teary eyed the first morning at home and proclaimed “I’m just so happy”. Clearly the trip was just what he needed.

Since I’m allergic to morphine & dilaudid, fentanyl is my go to drug if IV pain meds are needed. Unfortunately the drug has a short half life. So I would get a shot from the nurse; it would wear off in 25 minutues, and I’d spend the next 35 minutes in pain waiting for the next shot. This went on all night with no sleep for my mom or I. Thankfully I got a pain pump the next morning which helped tremendously. The upside to pain meds is they ease the pain. The downside is they slow your GI system.


My lab results showed elevated amylase and lipase levels which indicate pancreatitis. Pancreatitis is an inflammation of the pancreas that is extremely painful. It is more common in people with CF. I’ve only had it one other time when I was around 19 or so. The treatment involves restricting food & liquids, inserting a naso-gastric (NG) tube down your nose to your stomach to drain off bile and fluids that accumulate, and pain meds. During this bout I went almost seven days without liquids and food. At first that was “easy”. My belly was so distended I didn’t feel anything else could possibly go in. Even though the NG tube was somewhat gagging it helped me to keep from vomiting constantly. Of course I looked and felt like an elephant with it in.


Speaking of animals where does the pig fit in? Of course I’d have to fit a pig into this adventure. This pig is a pillow pet given to me by my college friends Jennifer, Julia, and Amy. It helped support me during coughing post surgery and also is a great snuggle pal. So everytime things get bad I have him to snuggle with and help ease the pain or just relax. Of course my boston terriers Rocky & Rosie usually get that job. But until hospitals allow dogs overnight the pig will do just fine.


After the first few days I was able to wean off the pain meds. My bowels had shut down and I needed to start pooping and moving stuff through. The other way to get things started was to get moving. So I walked. And I walked. And I walked some more. At first the walks were slow and short using my IV pole as a walker of sorts. As things progressed my speed picked up. The hard work paid off. The fluid in my belly shifted to my legs and ankles then eventually out as my amount of urine picked up. I got so excited about having fluid coming out finally!!! Then my bowels woke up too. One of my favorite yogis Bryan Kest said that walking is the best exercise you can do. Clearly in this case it did the trick.


Once things had settled down I got cleared for having a limited clear liquid dinner. To be honest this was a bit torturous. “Limited” meant I could only have 30ml of liquid an hour. That is about the size of a small shot glass. So I ordered jello got to eat two bites and then stare at it for the next hour until it was time for two more bites. ARGH!!! Luckily the next day liquids were unrestricted and then I was advanced to a full diet.


After eleven days in the hospital I finally returned home. I’m about ten pounds too thin and pretty weak. But lots of Ensures and Randy’s good cooking should help. And I’ve been walking some too ;)


Peace,
Steph


P.S. Once again I’d like to thank Randy, my mom Robin, Uncle John, Aunt Mickie, Courtney, Heather, Jen, and Neelu for visiting and making sure someone was there 24/7 for the majority of my stay. Thanks to many others for texts, emails, calls etc. Your support helps me keep on keeping on.Just two short mornings later we were having a leisurely breakfast. Randy started cutting up a grapefruit for us. By the time he was done I was starting to have terrible abdominal pain and vomiting. Hours later we were in my surgeon’s office for a scheduled follow up visit. I figured I’d rather go to the appointment and have him help than suffer the long wait and insanity of the ER. An abdominal x-ray was ordered and in the meantime I took two percocets. When the drugs barely made a dent in the pain I returned to the doctor’s office. A while later a hospital room was ready for me.

SOAR ON PINK PIGG! :-)

The following is a guest blog by my soul sister and bestest friend Kim (on the right below). Her presence this last week has been a bright light in a grey post recovery winter. And yes things are getting brighter all the time. Here's to a sunny 2011. I love you Kim!

Visits with Stephanie have been a highlight of my "back home again in Indiana" holiday adventures for the fourteen years since I left Indiana and headed west to live in the San Francisco area, but this year was extra special given the valiant fight she's been waging against cancer for the past four months. While I love living on the left coast, it also can be hard being away from family and friends when I wish I could be there for them physically, so I cherished my time with Steph and Randy more than ever this year.

Stephanie's determination, strength, and positive attitude around her Cystic Fibrosis have always been a huge source of inspiration for me, but as I snuggled up with her on her couch this week and we talked about all that she's been through the past several months, her spirit touched me more deeply than ever. So much so that I offered to write this guest blog for her, so I could help take an item off of her to do list...and also so I could attempt to give words to my undying love and respect for her.

Not only was Steph's surgery a success.. (She's now cancer free!)... She's going to be boarding a cruise ship this coming Monday for she and Randy's annual Jam Cruise adventure. Just four short weeks after major surgery and our energizer piggy is fully embracing life as she flies off on another adventure. Amazing!

When I asked Stephanie if there's anything that she wanted me to share in this blog post, she asked me to let those of you who have been reaching out to her via comments on this blog and through her Facebook page, etc. that your support and energy mean a great deal to her. The support and love she's been receiving from her extended community are a huge piece in her ongoing healing process. Even though she isn't able to respond to each of us individually, she reads and appreciates everything that is sent to her, so keep em' coming.

She also shared that her bout with cancer has been way harder than she ever imagined that it would be. She said that for the first time that she can remember she feels like she lost touch with the strong, can-do part of herself that has always been her driving force through her various health challenges. While she's gotten to know the trials that come with CF intimately over her lifetime, cancer presented a whole new set of challenges that threw her for somewhat of a loop.

I definitely understand why Stephanie feels that way given all that she's been through. But I was sure to remind her that even if she she couldn't access those inner resources as easily as usual, that they were definitely right there with her. She's been completely off pain medication for well over a week now. She's adapted to the reality of her new colostomy bag in no time flat. She's regularly walking a mile on her treadmill to help keep her lungs working at their best capacity, and she's preparing to go on a cruise right on the heels of major surgery. If that's not inner strength I don't know what is! :-)


Thankfully, Stephanie's hasn't experienced any infections or complications from her CF throughout her cancer ordeal, which is a huge blessing. While I was there, she started feeling some tightness in her right lung and immediately started an antibiotic regiment with the hope that she could nip in the bud before the cruise adventures. When I last checked in with her, she said that is was feeling noticeably better.


Sounds to me like she's well on her way to some much needed and well deserved fun in the sun. So please join me in wishing bon voyage to Stephanie and Randy...and keep sending them your positive energy and prayers!


Soar on Pink Piggy! :-) You inspire me always and I love you with all of my heart!