Enlightening Conversation About Vertex Results

I thought it would be good to share a conversation I had today with a momma in the community that wasn't exactly pleased with my thoughts/comments/commentary on the Vertex announcement yesterday. Many of you may feel the same way...

"I am upset at the comments you made yesterday and the one today."

What specifically upset you? (see paragraph towards the end of this post for her response to this question)

"I am surprised that you would post things that could upset parents and other CF'ERS that have new hope. I understand that you have strong feelings and emotions about this combo and they are rightfully yours to have."

Right. And I'll always be honest, even if that means a few feathers are ruffled or I have some push back. And if posting something accurate and truthful on FB takes away the hope of some, I am sorry. With that said, I also included parts in my statement that would support the hope felt like "There are also people who blew the doors off of old PFT results!!!!!" or "I'm not saying that this is not good news." or "And I've always been consistent in saying that we do not need to see positive numbers for it to be a success. Remaining steady would be a BIG WIN for the CF community."

"However, the post yesterday was used by others to continue to be hateful to many in the community."

I cannot control what others do, and I will never censor myself based upon the possibility of others using my words out of context. That would not be authentic and I certainly won't put on a false facade - with what I say or what I don't.

"It may not be what you intended but it came across as you being mad that you were not able to get them at a time when you were younger."

Honestly, I have no clue how that came across to you in what I said. Do you think I would volunteer 1000's of hours a year in the CF community, if I were mad or bitter in any way at the opportunities afforded to the younger generation? Does that sound like a guy who thinks "life isn't fair"? I thought that was pretty clear when I said, "My biggest goal, and frankly why I got so involved with the CF community, is to make the life experience of the next generation of CFers, and the generation after that, better than my own. I don't want to see the CF patients of the 90's, 00's and 10's experience the same struggles as I did, a patient of the 80's."

"I have always respected you and seen you as such a godly man. (I even spoke of how you were such to my mother yesterday when everyone else was posting hateful comments)."

I'm certainly happy to hear that you think (or thought?) of me in that way. I do my best to show God's love through my approach and interaction with others, and unfortunately, I'm pretty sure I fail everyday. I can assure you if I served a God that wasn't gracious, I'd be screwed.

"To see your post after saying these things was disappointing. I hope that you see that your posts can be hurtful and truly upsetting to some."

Again, I am sorry if anyone was hurt by my commentary yesterday. This was in direct response to seeing statements like "I'll finally be able to breathe again" or "This may be my last hospital stay ever!!" or "We'll basically be cured." or "I can't wait to take those pills and for this to all be over!". We still have a long way to go. As it stands right now, the combo drugs will not support the statements above for the majority of people with CF. Less exacerbations? Probably. Less frequent hospital stays? Probably. Maintain lung function for longer? Probably. Cure? No. No hospital stays ever again? Probably not. No more treatments? Probably not.

"You have been given a wonderful platform to be such a strong witness for the Lord. I am always so thankful for your standing up for what is right and true."

I will continue to do so.

"Please know that this is not intended to be mean, just expressing how this has made one Christian woman feel."

Not a problem. I didn't think it was mean at all. It's hard to articulate feelings through an online medium such as FB. Expressing oneself, and having different feelings towards the same object/result/person is what personal expression is all about.

"I want this combo to give you decades more to work for the Lord and be with your family as much as I want it for my little girl."

Amen. I want the combo to work for your little girl with every fiber of my being. I would gladly give up any potential decades I have left to see that happen.

"On a side note, the lawsuit against Vertex being brought forward by the state of MA makes me question the validity considering their health care system. Just a thought."

The lawsuit is about people purchasing the stock based on "grossly overstated" results by said company and then losing 100's of thousands of dollars when that information was redacted...all the while executives of said company potentially made 100's of thousands of dollars by selling a portion of their shares before the results were corrected.

"I think the most upsetting part was saying I hate to be that guy type of comment. It kind of starts you off with a negative attitude. I did not see the comments from people that you mentioned. I do not think this is the end of treatments or anything for my daughter. But I do think it could keep her where she is until something better comes along which is more than I've had before. I know that most parents feel hope but know this is not a cure. For those of us who have a realistic view of the combo, the post was like someone coming into the first glimmer of hope we've had and screaming "just kidding!". Because we look up to you so much....I don't think you are bitter and that's why I even said something because it seemed out of character. I know you can't control what others do; however, if you had read what some were saying you might have held off on.that comment for a few days. It's ugly out there...and it just gets worse. It's hard for a parent of a child with CF that deals so much to see the negativity we've seen from this announcement. There are so many that have been so hateful about this not helping them that they don't want anyone else to have hope. It seems like such a miserable life for them and it's hard to understand. Your posts may not have upset me on another day but after dealing with all the negativity it was hard to see yours. I do not look at you differently now. I just wanted to let you know how it is from a mommy's view right now. There was such excitement for Kalydeco and we can't have the same joy that's all we want. I know you want that too. Thanks for responding! And I want address the other comments on the lost because I don't feel it's okay to stir the pot. But feel free to share this without my name." 

Totally understand where you are coming from [withheld]. I can see why this may have felt like I was piling on. It was never intended to be that way. I was trying to provide some history and context to a community that has felt let down many times in the past based on announcements like this only to discover it's not what they thought it would be. I'm sure I could have worded it better or, as you suggested, wait for the flames to die down a bit before throwing some gasoline on them.

On a side note: Did you know that I am currently on the drug and I think it helps? Maybe that would have been beneficial for me to say?

"I had no idea. I am thankful you are and are doing well on it! I hope it keeps you healthy and at home with your family more so than not! It would probably put out the fire if you said that ;)"

Haha, maybe I'll mention it then. I didn't see PFT improvement, but I think it gave me the ability to fight off infection quicker and I have remained stable for longer periods of time.

Proceed with Caution - Vertex's Big News

For those of you who haven't heard, Vertex announced the results of it's big Phase III combo drug that may cover roughly 50% of the community. You can read the press release here.

I'm so pumped to see the joy and hope exhibited by so many in the CF community today. I hate to "be that guy", but I did want to throw out a bit of context and history to be sure that we are all managing our expectations:

1. The Phase 3 study showed an average increase in lung function of 3% Context: Cayston showed a 2% increase in it's Phase 3 head-to-head study against TIS.

2. The results have not been completely vetted by those outside of Vertex. Context: This is the same company that is now being sued for "grossly overstated" results in the Phase 2 trial of the combo drugs - http://ow.ly/yp7L2

3. About 46 people dropped out of the Phase 3 study because of complications. Context: Extrapolate this to the potential users of this combo, and you'd have around 1400 people with DDF508 who still wouldn't benefit from this drug. You'd also have to include those who saw no benefit but elected to stay in the trial. (NOTE: There are also people who blew the doors off of old PFT results!!!!!)

I could go on, but I'm feeling like Captain Buzzkill. I just see a lot of celebrating without facts (or misinformation) and want peeps out there to understand that we have a long way to go for the next generation of CFers. With that said, if this drug has the potential to in essence halt the decline in lung function at an early age, I'd cut off my left arm if that meant that everyone had access to it.

#captainbuzzkill

Added after reading feedback: Yes, celebrate!! Any move towards a better life experience while managing cystic fibrosis is a win. I'm not saying that this is not good news. Selfishly, anything that could possibly extend my life even a minute with my wife and daughter, is something worth being excited about. My biggest goal, and frankly why I got so involved with the CF community, is to make the life experience of the next generation of CFers, and the generation after that, better than my own. I don't want to see the CF patients of the 90's, 00's and 10's experience the same struggles as I did, a patient of the 80's. Progress doesn't only come in the form of a pill, but I'll sure as heck take one if/when it does!

And I've always been consistent in saying that we do not need to see positive numbers for it to be a success. Remaining steady would be a BIG WIN for the CF community. Shoot, not having to work so hard to maintain what I currently have is something I'd pay good money for :)

(I took this from my FB page as I know many of you are not friends of mine on there yet you read this blog. I'd love to hear your thoughts)

Thankful Thursday: Thankful for the Gift and New Adventures

Having plenty of options is usually a good thing in life...especially when it comes growing your family. It's no secret that adding another child to our cherished family of 3 hasn't been easy. Up to this point (in the last 18 months) we have filled you in on our 2 egg retrievals, 1 fresh cycle, 3 cancelled cycles, 1 miscarriage, and 2 failed cycles...as of last week, we unfortunately added the chemical pregnancy (early miscarriage) of two donated embryos to the list.

After our last failed cycle using our last two frozen embryos, we decided that perhaps using our own genetic babies wasn't going to result in holding baby number 2 for us. We found out after discussing our openness and desire to use donor embryos with our doctor that they actually had 2 donated embryos that they would give us, even though we weren't at the top of their waiting list. So within a week of our last failed cycle I was back onto our 8.5 week med protocol. On June 4th we put those little loves in and prayed for a positive outcome. We had an initial blood draw 6 days post transfer and it showed HCG (pregnancy hormone) in my system. We were cautiously excited! Our second blood draw (the official test at 9 days post transfer) still showed HCG, but the numbers hadn't risen properly...a sign that the pregnancy wouldn't continue. Three days later we had another draw and my numbers had dropped...showing the pregnancy for sure would was ending. They call it a chemical pregnancy, which is just a really, really early miscarriage.

Disappointment. Grief.

This one was a major disappointment, they all are. But this one stung even more. In fact it was downright heartbreaking. The way it all came together made it feel so right; so perfect. I fell in love with those little frozen babies from the time I heard about them. I was so ready to be their mommy and so grateful for this gift from someone we had never met. I cherished every minute they were with me, and just as with all our other losses, they will always have a little piece of my heart.

So now what? Onward and upward, friends. All we can do is choose to rejoice in our current circumstances and trust that God has a plan. Our attitude will not change our present situation, so we are choosing joy and moving forward with anticipation. There are so many incredible ways to grow a family, and we will continue to press on until we feel our family is complete...although I will say with every passing failure, I do become more and more comfortable with the idea of staying our sweet little family of three. We sure are blessed to be such a wonderful trio!!

SO while we are officially taking a little bit of time away from IVF medications (oh boy am I ready for that!!), we are pursuing another dream. We are pursuing our dream of becoming foster parents. We have always dreamed of fostering, we just planned to do it "once our family was complete." However, it's becoming more and more apparent that perhaps God's vision of complete and ours is different, and so we are no longer pushing off the call in our hearts to help little loves in need of a loving place to go. We have started the process to get certified and are excited to see where it takes us, and who it brings into our lives. Right now we are mentally preparing ourselves to foster for the sake of fostering, but are open (and excited!!) to foster to adopt if God presents that opportunity for us. All we know as of now is that we have a call in our hearts and a lot of love to give, so we are ready for whatever God has planned through the process.

We will keep you posted on this new journey in case it can help others who are looking at options to grow their family or looking to help other families in need.

And as a last note...I heard this song on Monday for the first time, right before we got the confirmation that the pregnancy was ending. It brought my heart a lot of joy and peace. I'm so thankful for songs that perfectly encourage me in times of heartache!

Two verses are my favorite are:

In my wrestling and in my doubts
In my failures You won't walk out
Your great love will lead me through
You are the peace in my troubled sea
You are the peace in my troubled sea

AND

I won't fear what tomorrow brings
With each morning I'll rise and sing
My God's love will lead me through
You are the peace in my troubled sea
You are the peace in my troubled sea

Started a New Routine: StrongLifts 5x5

Are you looking for a new routine to breakup the monotony of you current one? Or looking for something that is good for beginners? How about something that only has you lift weights for 3 days a week at 45 minutes a day? Something super easy and straightforward that even has an app that you can use to track your progress? Than I have the workout for you!! (Man, I definitely think they should use me to pitch their free product ;) )

I recently started the StrongLifts 5x5 program and I absolutely love have organized, easy and quick it is. The last thing I want to do in the gym is think, and this take all of the thinking out of it! I downloaded the app to track my progress and to tell me what to do every single workout and even tell me what weight to use. Couldn't be easier.

It's called the 5x5 program because there are only 5 different lifts that you have to "master" and each lift will be done at 5 sets of 5 reps (except for deadlift which is only 1 set of 5). You will only do 3 of those lifts on each day of your workout. That's it.

The five lifts are...

Squat


Deadlift


Bench Press


Overhead Press


Barbell Row



I'll keep you updated on my progress and would love to hear if any of you start this program as well!

Mandi Monday: Happy Father's Day

Happy (belated) Father's Day to all the dads out there! Us girls and moms would struggle to achieve our full potential without a wonderful dad in the picture.

The first father in my life I'd like to thank is my dad. He is a man among men. He was the kind of dad I thank God for every day, as I realized he helped shape me into the bold, hopeful, resilient and happy woman I am today. He showed me that being of high moral character can take you farther in life than any other character trait. He showed me what it means to work hard. He showed me how a husband should treat a wife. He showed me how to take life's punches in stride, head on. He also taught me that love can be best shown through humor and a back rub. I wasn't always the easiest daughter, but he showed me that unconditional love can be sweet and caring, and that it can also be tough love! He always has stood by my side, even when he hasn't agreed with decisions I've made. He's cried with me when I've suffered losses and he's rejoiced with me in my triumphs. A perfect illustration for his support for me was running by my side through marathon training and a marathon, despite his own injuries and discomfort because he told his little girl he would run with her.

The second, and now most important father in my life is Ronnie. When you marry a man, you pray he will be the daddy you always dreamed of for your kids. Ronnie has far surpassed my hopes and dreams as a partner in parenting. Ronnie is the parent by which other parents could be measured. I've never met a parent so patient. Ronnie is the perfect mix of fun, but with clear limits. Mckenna could not adore her daddy any more than she does. When she gets hurt, she wants a daddy snuggle. When she wants to rough house, she looks for him. When she wants a good scare, she looks for the "tickle monster" that is her daddy hiding in random spots in the house.

Happy Father's Day!

Thankful Thursday: Mexico & Kayaking

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 family time in Mexico. Every summer we do a long weekend with Ronnie’s whole extended family in Rocky Point, Mexico. This year was my favorite yet. Mckenna is a stellar traveler these days, so that helped immensely, and she was just a doll all trip. She had such a blast with all the “cousins” (I think they’re all second cousins technically). It was great spending time with family and watching Mckenna enjoy the trip.

I’m thankful for healthy eating. After a vacation, I realize how much I love eating well. Sure I LOVE junk…probably more than most. I have a sweet tooth that never ends and I can eat more fried food than a grown man. But after eating junk for a long weekend straight, I realize how much I love putting good food into my body. It feels good to be home and eating normally again.

I’m thankful for sleep. I’ve been falling asleep on the couch the last few nights really early (maybe catching up from vacation fun?) and it has been amazing. There’s nothing better than a good night’s sleep!

Ronnie's List:

I'm thankful for a successful trip to Rocky Point. My family has been going down to Mexico for a family reunion of sorts for the past 35+ years. This year, there was 33 of us and I think it's safe to say that we all had a good time. We run like a well oiled machine down there and not an hour goes by that you feel hungry or bored ;)

I'm thankful for summer softball league. We just had our first two games tonight (1-1) and it was nice to get back onto the field and give it all that I got. I am probably the least talented guy on the team, so I always focus on attitude, effort and hustle because that's about the only three things I can control out there!

I'm thankful for kayak rides. Quite possibly my favorite part of the Mexico trip this year was throwing a life jacket on Mckenna and jumping into the kayak. We paddled far out into the ocean with Mckenna leaning back on me the whole time and even giving me a hand with paddling. It was a very awesome way to spend some one-on-one time with her.

What are you thankful for today?

Thankful Thursday: Voice & Honey

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 workout buddies. I’m blessed to have a hubby who’s always game for a trip to the gym, but I’m also blessed with active friends. As we’ve posted about on the blog, Mckenna took swim lessons recently. I adored her swim teacher, who is a sweetheart and VERY active. So I turned her into a workout buddy. She runs with some friends most days a week (which I’m not bold enough to weasel my way into), so we meet 1-2 times a week at the gym on the days she’s not running. It’s perfect because it means that most days I still am working out with Ronnie, with a few sessions a week with a friend!

I’m thankful for Mckenna’s voice. Mckenna loves music and LOVES to sing songs. I really adore hearing her little voice. She carries a tune about as well as Ronnie and I (which isn’t impressive), but it is so endearing to hear her belting out a song.

I’m thankful for a Grammy who I enjoy. This one sounds silly, but I’m thankful for a Grammy (my mom’s mom) who I can talk to like a friend. I call to chat with my Grammy about once a week, usually in the car on my way to or from work. I know for some calling a grandparent can be a task you check off a to do list. But I am so thankful that I have a Grammy that I simply adore and truly enjoy talking to. We usually joke around and laugh a majority of the call, and I always hang up with a huge smile on my face! She’s one fun lady!!!

Ronnie's List:

I'm thankful for honey. Since we've been trying to eat cleaner lately, yet my sweet tooth still exists, I've been eating honey to get my fix. I've particularly been fond of honey drizzled over frozen fruit. It's quite the treat after dinner!

I'm thankful for organized files. I've gotten much more organized over the years and it sure makes everything much easier. I've recently had to access old files and information and having it all labeled within file folders by date made everything a breeze. Now if only I could keep my desktop clean!

I'm thankful for teeny, tiny cantaloupe. We picked out first melon of the season from the garden and it was a cantaloupe. I had to pick it a bit early because it was starting to burn, and to my surprise, it was still incredibly sweet. I'm looking forward to the bigger one being ready soon now!!

What are you thankful for today?

Top Ten Tuesday: Rocky Point

We have our annual family trip to Rocky Point, MX coming up and I couldn't be more excited to head down south of the border! My family has been going for 30+ years and now bringing a family of my own is a dream come true. Here are the top ten things I love about the Rocky Point trip.

The drive down - We usually have 20-25 or so people caravan down at the same time to Mexico and stop at the same places every year. Since half of us are coming from Phoenix and the other half Tucson, we meet at a town called Why before heading down into Mexico. We usually stop at an ice cream shop right on the border before crossing and the taste of that ice cream signals to me the trip has officially begun!

The changes in Sonora - It's been amazing to see the first border town we drive through in Mexico change over the years. They are constantly building and are looking more and more like a "city" instead of a town in a third-world country. Don't get me wrong, they still have mainly dirt roads, plenty of buildings in shambles and are behind about 30 years, but progress is happening.

The first peek at the ocean - On the drive down, you always get the first peek at the ocean at the same spot. I can visualize that scene in my head right now because I have seen it so many times. The desert landscape changes to sand dunes, the dunes seem to roll for miles, and then all of the sudden, a bright blue ocean.

The first breath - I love taking in that sea air after we park the car at the house. The crispness of the air. The humid feeling on the skin. The sounds, the smell; it all brings back so many memories.

The assembly line - After traveling down as a large family for 30+ years, you learn some things. My family has learned to unpack and set up with the best of them. You should see how quickly we can unpack 6-8 cars! People are coming and going in all directions, taking stuff out of the nearest vehicle and then putting it where it needs to go. I'm telling you, we're a machine.

Sand beneath my feet - The first walk on the beach is always nice. We've been going to the same beach house now for about 8 years or so and the beach is literally steps away from the backdoor. Taking that first walk down the beach and dipping my toes into the ocean is always such a treat.

The meals - In Rocky Point, each family is assigned a day in which they take care of preparing and cleaning up each meal. We have many of the same meals each year and I always look forward to them. The dinner nights are usually hamburger/hot dogs, BBQ chicken, Shredded Beef Nachos, Steak and chicken soup. My family is in charge on steak night, so I really have to hone in on my grill skills!

The conversations - I don't get to see my extended family as much as I would like (mostly only on holidays), so it's nice to be able to catch up with everyone while we're down there. We have fives days to get all of the updates on what's going on with everything and there is always a surprise or two each year that I had no clue of regarding someone in the fam.

The bread/tortillas - I love the fresh tortillas and bread that we get each year. I know bakeries are here in AZ as well, but there is just something about the bakeries in MX that get me every time. The smell and the variety is always so awesome to take in. Plus, it always seems so cheap...and yummy.

The joy - Everyone has so much fun on this trip. And now, I especially love to see Mckenna running around with her cousins with a gigantic grin on her face. She loves the ocean, sand, sun and everything else that comes along with this trip.

Writing this blog post has me even more excited than before! We are so blessed to be able to do this each year and the fact that I'm taking my own young family is a dream come true :)

Mandi Monday: My Favorite Foods

Ronnie and I have been trying to eat pretty healthy since he's been out of the hospital. We always eat pretty well, but candy and desserts are our downfall. We have cut out any of those kinds of sweets, stuff out of boxes, sugar, etc...you know, anything that makes your mouth water. I kid. The thing I like best about eating well is that it forces you to get creative. Here are some of my favorite foods/recipes right now:

Sweet Treats
Since we like sweets..A LOT...we have a couple things we've been eating lately to get our fix.

Peanut butter and honey on toast. Really peanut butter and honey on good on anything! But on toast and apples are the go to right now. We use natural honey and natural peanut butter. I have a major sweet tooth and this does just the trick!

"Sorbet". We've been making our own "sorbet" of sorts in our Vitamix. The key is to add a ton of frozen fruit in relation to something not frozen (just enough to make the blender turn). One of our favorite combinations is greek yogurt with frozen mango, frozen pineapple and frozen pomegranate arils. The other favorite right now is fresh mango with frozen pineapple and frozen cherries (sometimes with a little honey for even more sweetness). It look just like sorbet and tastes delicious!!!

Caffeine Fix
It's heating up here in Arizona (in the 100s already), so a nice ice cold coffee is AMAZING. Lately we have been doing cold brew Toddy for our coffee and adding a splash of whole milk or soy milk. We like to drink it out of a mason jar and fill it to the brim with ice. There's something about watching the milk mix with the coffee in the mason jar that makes it feel like a treat.

Veggie-filled Dinner
We have been using a wonderful alternative to pasta (not that pasta is bad for you, but we enjoy this low calorie alternative), broccoli slaw. You just add a tiny bit of water, cover it on medium heat, and stir it occasionally until it's the softness you want. For the sauce, we've been using Full Circle Organic sauce, with ground turkey or sliced chicken breast, some tomatoes and basil from the garden, and some olives.

If you have any yummy recipes to share, send them along. We are always looking for fun, YUMMY, new ideas.

Limited Exercise Research

I thought this was a very interesting article highlighting the limited research on exercise. I've highlighted the biggest take-aways if you don't have the time to read the whole thing.

“A lot of physiologists come into the discipline because they fundamentally like exercise,” Martin Gibala, an exercise physiologist at McMaster University in Ontario, told me. “But you learn very quickly that there’s not a lot of research money out there to fund applied studies.” On matters as simple as how many sets and reps best promote muscle growth, Mr. Gibala explained, “We can’t nail down the answer.”
Even if the funding were there, Mr. Gibala says, “That’s not state-of-the-art research that you’re going to publish in the best journals and advance your career.” Instead, he says, physiologists study questions of basic science, “like the molecular signaling proteins that regulate skeletal muscle adaptation.”

I also thought of the CF community and what we're trying to do in promoting patient-centered or patient-driven research when I read this...

The human body is an adaptation machine. If you force it to do something a little harder than it has had to do recently, it will respond — afterward, while you rest — by changing enough to be able to do that new hard task more comfortably next time. This is known as the progressive overload principle. All athletic training involves manipulating that principle through small, steady increases in weight, speed, distance or whatever.
So if your own exercise routine hasn’t brought the changes you’d like, and if you share my vulnerability to anything that sounds like science, remember: If you pay too much attention to stories about exercise research, you’ll stay bewildered; but if you trust the practical knowledge of established athletic cultures, and keep your eye on the progressive overload principle, you will reach a state of clarity.

I think we all see people in the CF community that are doing the things and making the choices that work for them. In some cases, there is a large cohort of the CF community that has bought into a certain lifestyle or treatment or "culture" that seems to be having positive effects. I look forward to identifying those a doing what we can to promote research around those areas.

If you have any comments, I'd love to hear them.

http://www.nytimes.com/2014/05/25/opinion/sunday/fitness-crazed.html?_r=1

Mandi Monday...on Tuesday

Well apparently this weekend got away from me. We had a great holiday weekend filled with time with family and house projects. But the highlight was receiving the following picture via text message from Ronnie's brother, Andrew and his (now) FIANCEE:

Andrew is an amazing guy. He's funny, loving, and genuine. A real class act. We are so blessed that he lives close by and thats we are able to see each other a lot. He's forever saying yes to last minute babysitting requests and helping with our garden (which is actually Ronnie and his garden). When we found out he was ready to propose to his girlfriend, Kirsten (or Aunt Kiki as we call her around here), we were thrilled. He could not have found a better life mate. She is smart, funny, loving and gorgeous (I know, looks may fade but they are sure awesome while they last, right? Hehe). I am so excited to watch these two plan their wedding and start a life together. I am also thrilled to have another sister!! 

We love you guys and are excited for you both! Here's to the beginning of a new chapter!

Thankful Thursday: Wedded Bliss & Fans

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 4 years of wedded bliss :) Today Ronnie and I celebrate 4 years together as husband and wife and I’m so thankful for every day, month and year together. I couldn’t ask for a better partner in this life.

I’m thankful for having Ronnie home. Ronnie got home Monday night from his 2.5 week stay, and it has been amazing having him home!! Life is so much better with him under the same roof.

I’m thankful for a wonderful babysitter. Mckenna has had a babysitter come hang out with her on Thursday afternoons for the last couple months so that Ronnie and I can get some work done together. The last couple of weeks Mckenna has been especially clingy (I think Ronnie being gone may have driven this), and has been out of character when the sitter comes (full of tears, fits, sadness and fear)…Mckenna has insisted on starting the time in bed, reading by herself or “sleeping”. Our sweet sitter just waits her out and loves her anyways. Within 30 minutes, Mckenna is usually ready to play. I am so thankful for a sitter who understands a child’s heart, loves unconditionally, and is a lot of fun.


Ronnie's List:

I'm thankful for 4 years of marriage! I can't believe I was able to trick another human being to spend 4 years with me (6 including our dating years). God has truly blessed me with an incredible partner in crime that I absolutely love doing life with. Here's to another 40...that is crazy...and sounds very tiring...but with Mandi, I am willing ;)

I'm thankful for vanilla soy milk. I don't think I've ever tried the stuff before last week, and now I can't get enough of it! Now, I don't drink it by itself, but in iced coffee, although I don't mind the taste of it as a standalone. I also have to give a shout-out to my wifey for pumping out the iced coffees for me!! 

I'm thankful for fans. This time of year it starts to get incredibly hot back in my office (it was an addition to the house and doesn't have the best airflow). I picked up a standing fan at Costco that also came with a little desk fan. Now I have air blasting my face, body and top of my head (from the room fan above) to try and combat the heat exhaustion that I feel while I'm working ;)

What are you thankful for today?

Top Ten Tuesday: My Favorite Things About Hospital Stays Ending

Ronnie is home...WAHOO!! He got home last night. I'll let him give the specifics in his next post, but I figured it would be a good time to do a top 10 list of what I love about him coming home (some will be very obvious!)

1. FAMILY TIME - I love being back together as a family. It is so amazing to have us all under the same roof again. Both practically, for the extra help, but also for the emotional aspect of just having daddy and hubby home.

2. Starting Anew - Ronnie and I use hospital stays to reboot our lives...this may be one of my favorite things about the end of a hospital stay. We usually revamp our diet, our exercise routines, our chores, you name it. This time out Ronnie and I have decided to clean up our eating (as in, eat clean for awhile), rework some of our workout routine, reschedule some of our daily routine, and reorganize our chore list. It's a nice opportunity to hit restart!

3. Increased Energy - Ronnie usually has a lot more energy when he gets out (vs when he goes in). I love having him back at 100%. He doesn't complain before a stay, and he tries not to have it affect our lives, but I can always tell he's forcing it before he goes in.

4. Appreciating the Under-appreciated - I always love and adore Ronnie, but I appreciate him SO much more the first few days he's home from the hospital. His presence I felt more than usual. Hearing his goofiness resound from the playroom or finding laundry folded waiting for me make me truly appreciate him.

5. Daddy's Girl Back with Daddy- Mckenna struggled this stay more than usual with Ronnie being gone. I love seeing daddy's little girl back with her daddy. She smiles a little bigger, laughs a little louder, and snuggles a little tighter when she's with her daddy.

6. Evening Company - I don't very often think about evenings when Ronnie is around. We veg out. We watch TV. We sleep. It's all low key. But when he is gone for 3 weeks, evenings start to feel lonely. It's quiet. I'm alone. The house feels emptier. Having him home is truly felt in the evening. Even watching TV with someone else feels remarkably more full, even if no one is talking.

7. Help - It's exhausting to be a single mom for weeks on end. Help is amazing. Getting to pee alone sometimes during the day. Someone else to be on potty duty. Someone else to grab something out of the fridge. Someone else to answer all of the "why" questions. Everything. Help is amazing!!!

8. Gym Buddy - I love working out because I love all of the benefits...but one thing I love the most is doing it with my gym buddy. It's our time together. We can talk, joke, check each other out in the mirrors...It is a really fun time together.

9. A Warm Body - Our bed feels empty when Ronnie is gone. I stack pillows on his side of the bed, just so it feels less empty, but it's really nice to have a real person back in bed. I would say it's nice to have snuggles during the night, but usually he says I'm too hot to snuggle with, so he snuggles pillows ;-)

10. FAMILY TIME - This one is too good not to do twice! It's nice to have our threesome together again!

Thankful Friday: Family

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 friends that are like family. Mckenna and I flew to SC to visit some family friends, the Giblins. My parents have been here a week and Ronnie, Mckenna and I were set to meet them for a long weekend. Ronnie ended up in the hole, so he didn’t join us, but Mckenna and I came anyways. It is such a blessing to have family friends that really are like part of the family. They are like another set of parents to Ronnie and I and we couldn’t be more thankful for their friendship.

I’m thankful for a change of scenery. Mckenna had a rough few days at the beginning of the week. I think she may feel Ronnie being gone and just how long he’s been gone this stay (I’ll write another whole blog about that). Anyways, she was a PILL! Thankfully, I think heading to SC was exactly what she needed to get out of her funk. A change of pace has been good for both of us.

I’m thankful for friends who are always there. As I mentioned, Mckenna was having a rough few days. She was clingy, nervous about me leaving her sight, but a pill when with me. On Tuesday, I had committed to take my brother to his follow up appointment for his leg, but Mckenna had to go to swimming, so her sitter (who she usually LOVES) came to watch her. Well it was not pretty. Mckenna sobbed and insisted on just “sleeping” in her bed alone until I got home. I called my friend, and someone who feels like a second mommy to Mckenna and let her know she may get a call from the sitter. Next thing I knew, I got a text that they were all meeting for gelato, followed by a picture of a perfectly happy Mckenna eating with her bestie. My friend proceeded to take Mckenna home, feed her a yummy dinner, and love on her (and played) until I got there. I am so thankful to have a friend who is always going out of her way to make my life easier, and loved so much by Mckenna.


Ronnie's List:

I'm thankful for a great response in my first week in the Hole. I can't remember the last time my lungs responded so positively after just my first week in the Hole. My FEV1 actually went up 18% and more importantly, I can take a much deeper breath with little to no pain.

I'm thankful for family help. When I'm in the Hole, there are many family members who take on extra responsibilities or tasks to make my life easier. Mandi of course steps up BIG TIME and becomes a single mama while I'm gone which is quite the feat. My mom makes dinner for me almost every night. My brother Grant brings me food and hangs out almost every day. My brother Andrew is housesitting and dog-sitting while Mandi and Mckenna are out of town. CF can definitely be a team sport!!

I'm thankful that I can do almost all of my work from a computer. It takes a lot of pressure off of me getting out of the Hole quickly when I can still take care of my work tasks from my hospital room. Conference calls via the web are easy and email of course works from practically anywhere. I'm also thankful for those I work with be more flexible with me while I'm in here!

What are you thankful for today?

The Choices We Make

When I made the decision to come to the hospital, it was not an easy one. The way I was feeling, my energy level, my increased cough, my increased mucus production, my lung pain and my lung function test results all pointed to an easy decision, but it was certainly not so.

Mandi and I had been planning on a trip to South Carolina with my father-in-law and mother-in-law and family friends for months. The tickets had been purchased the schedules rearranged and we were ready to go. That "we" is now just Mandi and Mckenna. They left on a plane for South Carolina this morning.

It's funny as it seems every time there is a decision whether or not to come into the hospital, there is always a balancing act. I have to decide how to weigh the perceived sacrifices with the hopeful
outcomes. And really, the hopeful outcome is the only reason I come into the hospital. If I didn't have that hope that I could and would get better there be no reason for me to be in here. Thankfully I still have that hope, and have never lost it. When I come in for a tune-up, I do get better. I do become the best version of myself. It make take 2 weeks, 3 weeks or more, but it does happen.

So what led to this decision? The easy answer is that my lungs felt beat up. I was unable to exercise like I'm used to. I found myself getting winded after a flight of stairs. It was hard to take a deep breath because of the pain that I experienced each time. With me, it becomes a vicious cycle. Less than effective workouts contribute to more mucus buildup which contributes to more inflammation which contributes to a harder time breathing. It's up to me to break that cycle. Sometimes I can do it through increased treatments or increased exercise, but other times it takes the decision to come in for a tuneup.

When I went to clinic and talked it over with the team they thought it was a pretty good idea that I come in. They knew about my looming trip to South Carolina and told me that I would have their full support if I decided to try to hang on and make the trip. We all knew that the decision to come into the hospital would mean that I was choosing not to go to South Carolina but to go into the Hole. It was not an easy decision, but there is no doubt that there was a right one. I had to put my health first.

I don't have many regrets in life, but as it relates to CF, I have one big one. There was a time in my life when health certainly did not come first. My social life came first. My work came first. My girlfriend was my priority. My friends won out over my treatments. Delaying hospital stays to go on vacation was the norm.

And after years and years of making those decisions and putting everything else in front of my health, I am now paying for it.

I have to work so hard now to stay healthy. I have to put in so much time each day to keep my energy at a level that my wife my daughter deserve. So much time is spent doing my treatments or at the gym that I wish it could be spent at the park with my daughter. I now have to miss family vacations to come into the hospital.

If I would've kept my health first, as the first priority in my life, would I have to make the sacrifices now? Of course I do not know the answer for sure, but I'd be willing to bet that I wouldn't have to work so hard now. I wouldn't have to take so much time away from my family when life is really important. I probably wouldn't be missing this trip to South Carolina.

That's the bed I made and now I lay down in it.

So what's the point? If you're reading this and you're struggling with putting your health first, I beg of you, don't make the same mistakes that I did. There will come a time in your life that you will wish you didn't have to work so hard because of choices in the past. When we were younger many of us thought that we were just “living life”. Let me tell you as a man with a wife and a daughter - This is life. I'm now in a life that matters. I have two beautiful girls who count on me to be at my best. That doesn't mean that if you don't have a family in the future you won't be living “the life”. But I promise you that there is something down the road worth sacrificing for. The truth of the matter is you're going to have to work hard now or work hard later, but hard work and sacrifice will happen.

I encourage you to make that sacrifice now, and to work hard now, at the chance that you won't have to work so hard later.

Mandi Monday: Mother's Day Recap

Ronnie is still in the hospital, so Mckenna and I flew solo for Mother's Day...what better way to celebrate being a mom, than, well, spending the day with the little lady that made me a mommy. Here's our day in pictures:

We started the day with breakfast and coffee outside...I ate while Mckenna played with chalk.

Mckenna played and goofed around while I got ready for church.

We went to Fatburger...Mckenna loves hot dogs, and I like not waiting for 35 minutes with everyone else and their moms!

After nap, Mckenna and I went to a peach festival at a local farm to pick peaches. We got water ice there...nothing says peach picking like watermelon water ice...I guess?!

We have a lot of interesting-looking peaches because I just let Mckenna decide what was good...which usually was whatever she could reach.

9lbs of peaches is heavy. Most the peaches at the bottom of the bag are bruised because, well, a toddler drops a 9 lb bag of peaches...every step.

Mommy-Mckenna selfie. Note who's carrying (and eating) the water ice...I really only got it for her for myself. I know I'm not the only mom that does that!

Hayrides too and from where you pick peaches! It was awesome, except all the dust and hay that scratched our corneas the whole time we rode.

"Will you help me? I only two and a half. I can't reach this." Truth.

Some passerby took pity on me as we took a selfie. Thank you, stranger, for your photo-taking.

We ate a lot of peaches.

We also ate a lot of cinnamon roasted cashews...hmm we ate a lot.

We took lots of pictures for Daddy. I love my little ham.

And I finally got a "smile". I'll spare you the other 34839248023 outtakes.

Oh and then Mckenna became a grumpy bus driver. She actually had fun, until she got into the role of a bus driver.

Happy Mother's Day!

Happy Mother's Day to all the moms out there! I am SO thankful that this passionate, affectionate, smart, fun-loving, outrageous, loving little spark plug made me a mommy!!

My Torn Pleural Adhesion

Mandi mentioned some of the things that may have contributed to my current hospital stay on a blog a few blogs ago, so I figured I would talk about one of them.

It was the night of April 3rd and somewhere around 3 AM I felt a slight tug and some pain in my left lung. Now, I have had similar feelings before so I wasn't too worried and I took some deep breaths thinking it would pass. The pain did not pass but it didn't really get worse so I figured I would just sleep it off and address it in the morning. About 3 hours later the pain had intensified to a pain I had not felt up until that point in my life (in my lungs).

When I rolled out of bed I had to walk with a hunch as it was the kind of pain that almost brought me to my knees. I made it to our couch in the living room and I just sat there rubbing the area trying to take deep breaths thinking again that hopefully it was something I could breathe through. The pain only got worse. I was unable to take very deep breaths and when I did the air seemed to get forced out of my lungs. I was unable to speak properly and I would make more jumbled moans than actual words. It was the first time this kind of pain with these kind of symptoms had ever occurred.

Mandi took one look at me sitting on the couch writhing in pain and said "we're going to the ER”. Usually I would fight her on something like this, thinking that I could fight it on my own or that it would pass shortly–I put up no such fight. She grabbed me some clothes got Mckenna together and out the door we went.

We made our way to the biggest hospital within striking distance and Mandi dropped me off in front of the ER entrance. There was no receptionist present as I clung on to a sign which was assisting me in keeping me up right. A security guard noticed this and rushed to get me a wheelchair to sit in. He then went back into a different room to grab some assistance. Just then Mandi and Mckenna came through the doors, which was good timing, because I was having trouble having enough air to answer the nurses questions (I was later told they figured I had a massive kidney stone). I of course told them about cystic fibrosis and that I've had similar pain before but not to this level. I shared with them that if I had to guess what a pneumothorax or a popped lung felt like, this would be it.

They brought me back to a bed and immediately administered an albuterol treatment and oxygen. They then took me back to x-ray and I also had a CAT scan performed. This was my first time at this hospital so they had no record of me or any x-ray images or CAT scan results from past. They reached out to one of my pulmonologists in Tucson to compare notes and results of the different tests. Fortunately, as time started to pass the pain started to subside.

They concluded that I must of torn a pleural adhesion. Which in layman's terms, which of course I asked for, in my case means that they felt I ripped some scar tissue in my lung. I had never even thought of this being a possibility. I mean it intuitively makes sense, and I understand that you can of course rip scar tissue, I just never thought of it actually happening. I know that I have scar tissue all over both lungs and I'm surprised I guess that it took this long to happen. Being as active as I am and using as my lungs as much as I do I feel blessed that I've avoided it up until this point. I'm also very thankful that it did last, that is the pain, “only” 5 hours or so. I mean I had some residual pain for the days that followed but nowhere near the initial pain that I felt.

It was suggested that I should do some exercises that would stretch the cavity wall and that I didn't do anything too aggressive for at least a week or so. They also suggested that I get these “lung stretches” into my daily or weekly routine to try and avoid another torn pleural adhesion in the future. I did have a cold the week before this happened and am wondering if that contributed to this event as well.

I also think this contributed to my downward trend over the course of the following month. A downward trend which ultimately led to me being where I am today, the Hole. It wasn't the whole story however--there were other factors that led to me being in here which I will get into in later blogs.

Has anybody else out there ever been told they tore a pleural adhesion? I'd like to hear your story!

Top Ten Tuesday: Favorite Things During a Tune-Up

As you maybe read, Ronnie is in for a tune-up. He got a bed on Saturday afternoon, so we are in full swing. I will let him write a post about what sent him into the hole, but my guess is it will be a few days...he's still at the point in his stay where is feels worse before he feels better.

I figured it would a good chance to read some of my favorite things to do with Mckenna while he is getting tuned-up. The name of the game is to pass time and have fun! They are in no particular order!

1. Take walks - Mckenna and I do this when Ronnie isn't in the hospital too, but we have more time on our hands while he's in, so we do more of it. Sometimes she rides in the stroller, while other times she walks also. We like to go "hunting for busses" in the mornings.

2. Go go the park - Daddy is usually the park-player. Mckenna and Daddy go to the park while I make dinner many evenings. But while he's in, the two of us go and she "teaches" me to do all the fun things she's learned with her daddy.

3. Adventures - I use the extra time to take Mckenna to places we haven't been in our area. It's fun to explore your own town.

4. Playdates - Mckenna and I have lots of playdates usually, but we have that many more when Ronnie is gone. It's nice to spend time with friends...for both of us.

5. Gym time - One of Mckenna's favorite things to do is play at the gym. One of my favorite things to do is to get a workout in and have a little "me" time. During a hospital stay, we both love it that much more!!

6. Eat together - We eat most meals as a family, but usually Mckenna is in her high chair. But when Ronnie's gone, Mckenna and I sit side by side at the kitchen island to eat meals, and it's fun to have meal dates together!

7. Bubble baths - Mckenna loves bubble baths. When Ronnie is gone, Mckenna and I kill some time by taking bubble baths together. We fill the tub with suds, put a show on the iPad, and play with toys until we are both prunes!

8. Snuggles in bed - Some mornings it's hard to spring right out of bed, so Mckenna and I snuggle in my bed for 30 minutes to an hour snacking on cereal, watching shows and playing games.

9. Time with family - We spend a lot of time with my parents and Ronnie's mom while he's in. It's a fun opportunity for all of us to see more of each other.

10. VISITING DADDY - This is the best of them all. Mckenna and I LOVE visiting Ronnie at the hospital. Time with a toddler in a hospital room passes slowly, but we all have fun spending time together in a new setting. Mckenna loves seeing daddy, but she also loves visiting the children's library at the hospital, as well as playing with the fun toys and "attractions" they have around the hospital.