Saturday, March 23, 2013

My Journey to Compliance

Guest blog by Aubrey Bean

I want to start by first thanking Ronnie for this amazing and inspiring blog, and also for the opportunity to share my story.

My name is Aubrey, and I am a 24 year old from a little town in NH. I was diagnosed with Cystic Fibrosis when I was six days old, after I had surgery for meconium ileus. I was a healthy and (very) lucky kid, always having high PFTs and never having to "work" for them. I started enzymes when I was born, but didn't start any nebs or the vest until middle school. I had IVs when I was 8, 16, and 19, and those PICCs weren't because I was sick, but because my numbers happened to slip slightly below my 100% baseline. I spent my first 20 or so years of life being blissfully unaware of the possible repercussions of this disease. However, throughout my 4 years away at college, when treatments happened when it was convenient, and exercise happened never, I slowly saw my lung function decline to 85%. Every appointment I repeatedly promised myself that I would get it back up “eventually.”

Finally “eventually” had to happen now, when in my last semester of college the noncompliance and hatred for any kind of exercise finally caught up to me. In May 2011 when I was a college senior, 2 weeks before graduation, I saw numbers that stunned me, and even caused me to shed a few tears. I blew a 61%. I was sent into the hospital shortly after I graduated college, and was even more stunned by the fact that after 4 weeks of IV antibiotics my numbers improved only slightly. To make a long and pretty complicated story short, I spent the next 7 months in and out of the hospital, including close to a week in the ICU with pneumonia, unable to get out of bed. I spent those months battling a few different medication allergies, new bacteria, and even a fungal infection in my lungs. Finally in January, after my third case of pneumonia in less than 6 months, at 34% lung function, I made the difficult but necessary decision to quit my job, one I had been offered in October. I hated that I had to quit my job. I hated the fact that I lost 50% of my lung function in just one year. But more than that, I was scared that it would never come up again.
During my hospital stay in January 2012, I constantly heard the voices of multiple doctors telling me that pneumonia causes permanent damage and they weren’t sure how much lung function I would be able to recover. I even had one of them tell me I should shoot for 50% lung function, but not much higher. I left the hospital hearing those words over and over in my head. Their voices became an echo, at first scaring me, but suddenly I turned it around, and those words became my motivation. I realized that all I could do was try. I thought even if these doctors are right, I at least want to make a strong effort to prove to them and most importantly to myself, that no matter how much CF knocked me down in the past year, I was going to stand up and try my best to come back. So that’s what I did. I started to exercise (with the help of RSBR and other awesome CF blogs!) and made a vow to never skip a treatment, unless it was absolutely necessary.

I started slowly, walking on the treadmill until my lungs started to ache. My walks started getting longer, and feeling a little better. After a few weeks of just walking, I started to run, (slowly) and soon I was running very small lengths at a time. It was hard and extremely challenging. A couple times I almost made myself sick I coughed so hard, and other times I spent my ride home from the gym crying, wondering why I couldn't do more than walk. I was frustrated more than satisfied, and I was in pain more than comfortable, but I wasn't giving up! I continued to run more, and exercising became a lot easier and more pleasant within a few months. I kept at it about 5 times per week, and it was all worth it when in August of 2012 my hard work paid off and I ran a mile for the first time. Ever. Today, it is still worth it.

Sitting here writing this today, I'm reliving my 2011-2012 journeys and I realize just how different my life is, and how different my lungs are! I got another job in November, and have not been sick once. Also, last week I went to clinic, and I blew FVC 80 FEV1 65 (And I’m shooting for higher next time!), over 30% higher than last February. My PFTs increased by that thirty percent because of hard work, patience, a lot of faith, and a lot of treatment compliance. In the last year, I have missed one treatment (for my sister's wedding!!) and last week I ran 2.5 miles on the treadmill.

My last hospitalization was January 2012. Coincidence? I don’t think so!

In the last few years, CF has taught me so much about myself and life in general. I've learned life lessons I never would have learned otherwise. I have also learned that as much as I am being pulled in so many directions every day, I know that treatment compliance and exercise are the most important aspects of my life to never let go of, and I am very grateful for that lesson. I've also learned that although CF is tough, and may knock me down sometimes, I have proved to myself that I am tougher and I will always try my hardest to fight back.

Bio: My name is Aubrey and I am 24 years old. I live in southern New Hampshire, and have been seen by doctors in Boston, MA my whole life. I graduated from Merrimack College in North Andover, MA in 2011, with a degree in Spanish. I am currently employed at a bank in NH and I really like it! In my free time I like hanging out with friends, family, and my dog, singing, dancing, shopping, and of course working out and trying to stay as healthy as possible!!

Note from Ronnie: I can't thank Aubrey enough for sharing her story with us. We're seeing this type of story happen more and more, so I hope that it's a huge encouragement for the community. Maybe it can also serve as a wake-up call to those in college who have put their health on the back burner. People ask me all the time where they can find motivation - well, here it is!! The proof is in the pudding so they say. Thanks again cyster!!

Interested in contributing a guest blog about you and the fact that you kick some CF booty?!? Email me your blog idea at

Friday, March 22, 2013

Fun at the Playground

I love to watch Mckenna make new friends :)

Thursday, March 21, 2013

Thankful Thursday: Daddy's Girl & March

It's thankful Thursday time! We all have so much to be thankful for and we love to take this opportunity just to write down each and everything that comes to mind. Please take this time to share with us what you're thankful for as well. If you have a blog expressing your thankfulness, please share the link! Without further ado, here's what we're thankful for:

Mandi's List:

I'm thankful for Ronnie. He helps me so much around the house and is always willing to take over on Mckenna duty. I am really blessed to be married to someone who is always so helpful.

I'm thankful for my little daddy's girl. Mckenna is a daddy's girl through and through, especially right now. When she gets hurt, only daddy can comfort her. When she's looking to snuggle, daddy is who she wants. I absolutely love that she has turned into a total daddy's girl. It's really wonderful for both of them.

I'm thankful for water. Sometimes nothing tastes better than a HUGE glass of water. I know this one is kind of odd, but we live in the desert...what do you expect?

Ronnie's List:

I'm thankful for March. March is one of my favorite months of the year here in Arizona. The weather is perfect. Everything is greening up and growing. The birds are chirping. And of course, March Madness is upon us. I just hope that my Cats can play well today and get a crack at New Mexico.  

I'm thankful for a good girl for the doctor. Mckenna has something going on with her arm, so we took a trip to see the doctor. The doctor thought it would be best to get x-rays, and so after leaving the doctor's office, it was straight to the radiologist. Mckenna was such a good girl while getting her "special pictures" taken.

I'm thankful for TV. Now that my little brother is without TV service, I see how fortunate we are to have it. This also means he's around more though which is always a good thing!!

So, what are you thankful for today?

Wednesday, March 20, 2013

How was your day?

Me: God, can I ask you a question?
God: Sure.
Me: Promise you won't get mad?
God: I promise.
Me: Why did you let so much stuff happen to me today?
God: What do you mean?
Me: Well, I woke up late.

God: Yes.
Me: My car took forever to start.

God: Okay.
Me: At lunch they made my sandwich wrong and I had to wait.

God: Hmmm.
Me: On the way home my phone went dead just as I picked up a call.

God: Okay.
Me: And on top of all that, when I got home I just wanted to soak my feet in my new foot massager and relax, but it wouldn't work!!! Nothing went right today! Why did you do that?

God: Well, let me see. The Death Angel was at your bed this morning and I had to send one of the other angels to battle him for your life. I let you sleep through that.

Me: (humbled): OH...

GOD: I didn't let your car start because there was a drunk driver on your route that would have hit you if you were on the road.

Me: (ashamed)

God: The person who made your first sandwich today was sick and I didn't want you to catch what he has.

I knew you couldn't afford to miss work.

Me: (embarrassed): Ok...
God: Your phone went dead because the person who was calling was going to give false witness about what you said during that call. I didn't even let you talk to them so that you would be covered.

Me: (softly) I see, God.

God: Oh, and that foot massager had a short that was going to throw out all of the power in your house tonight. I didn't think you wanted to be in the dark.

Me: I'm sorry God.

God: Don't be sorry, just learn to trust me in all things, the good and the bad.

Me: I will trust you.

God: And don't doubt that MY plan for your day is always better than your plan.

Me: I won't, God. And let me just tell you God, thank you for everything today.

God: You're welcome, child. It was just another day being your God, and I love looking after my children.

- Author Unknown

Tuesday, March 19, 2013

FDA Sends Cystic Fibrosis Treatment Back for More Study

The US Food and Drug Administration (FDA) has completed its review of Pharmaxis' New Drug Application (NDA) for its dry powder formulation of mannitol (Bronchitol, Pharmaxis) and has recommended that the company conduct an additional study to obtain marketing approval in the US, Pharmaxis announced today.
"The submitted data do not provide a favorable benefit-risk balance to support the use of inhaled mannitol in patients with cystic fibrosis 6 years of age and older," the FDA said in a complete response letter to the company.
"The determination of efficacy based on the 2 clinical trials conducted to date are not adequate because of the treatment-related frequent early dropouts in trial 301 for which the primary statistical analyses did not account and the lack of statistical significance in trial 302 for the primary endpoint," the agency stated.
In relation to safety, Pharmaxis said the FDA expressed concerns over the occurrence of hemoptysis, particularly in patients younger than 18 years.
The complete response letter follows a unanimous decision January 30 by the FDA's Pulmonary-Allergy Drugs Advisory Committee against approval of Bronchitol for the management of cystic fibrosis in patients aged 6 years and older to improve pulmonary function.Pharmaxis CEO Gary Phillips said the company is "clearly disappointed" in the FDA's decision, but noted that the regulator has provided guidance on the necessary measures to gain approval and that Pharmaxis will meet with the FDA soon to determine appropriate parameters of an additional clinical trial.
The FDA previously granted Bronchitol orphan drug designation for the treatment of patients with cystic fibrosis.
Bronchitol, which helps facilitate mucus clearance in the lungs of patients with cystic fibrosis, is approved for marketing for patients aged 6 years and older in Australia and for patients aged 18 years and older throughout the European Union.

Monday, March 18, 2013

Weekend Videos

We had a fun weekend just hanging out as a family. Ronnie's brothers were around a lot this weekend (Grant came up and stayed for the weekend and Andrew came by most days). We also hung out with Josh and Chrissy quite a bit. It was awesome.

In hanging around the house just playing, I caught some funny moments with Mckenna on video. ENJOY!!

The first video is a total mommy fail. Mckenna saw Uncle Grant have a lollipop the day before this, so I let her have one. Well the next day (when this video happened) Mckenna was playing in the pantry while I made dinner. She came out of the pantry with a lollipop, unwrapped, in hand, with a big smile on her face. I tried hard to be stern so she'd know she couldn't just eat them whenever she pleased, but I was too impressed that she got it out and unwrapped, that I let her have it. Not too mention I couldn't stop laughing!

Mckenna talks on the "phone" to people all the time...she loves talking to her uncles and aunts and grandparents...All at the same time!

This one band camp...