Can One Email Save Two Lives??

It has come to my attention that two girls in the cystic fibrosis community are in desperate need of our help. In this case, “help" can come in the form of an email from YOU and others in the CF community.

Here's what I know:
A couple of years ago Arkansas State Medicaid denied coverage of Kalydeco for four girls in need of the medication. Through the hard work and dedication of Beth Sufian (lawyer with CF herself) and her law partner, two of the girls were able to receive Kalydeco recently. Unfortunately, that still leaves two of the girls in Arkansas who are in desperate need of the medication. Their health is in decline, and time is limited. Action needs to be taken now!

The doctors of both girls have petitioned Vertex pharmaceuticals for a compassionate use of Kalydeco. The response to these pleas has been disappointing. Vertex’s response to the pleas was that they would meet with Arkansas State Medicaid. A meeting is good, but the medication would be better. A meeting will not stop the rapid decline in the health of these two girls. 

Vertex has the ability, under the law, to provide the girls with Kalydeco as early as tomorrow! The FDA allows for on-label compassionate use of any approved drug of which both of these girls qualify.

One can only imagine what these girls and their families must be thinking. The same family who participated in fundraising efforts that provided the money to accelerate the research for this drug, is now being denied access to it. Does that sound at all fair to you?

What YOU can do:
You can copy and paste the following text into the body of an email and to send it Charles Johnson, VP of Global Medical Affairs at Vertex. His email address is charles_johnson@vrtx.com. Let him know that this is unacceptable. Feel free to use any or all of the email provided or write your own. Please copy ronniesharpe@cox.net and info@cff.org to any email sent. 

***

Mr. Johnson - 

It saddens me to hear that Vertex is refusing to provide Kalydeco to the two girls in Arkansas; despite having the ability to provide it under compassionate use. As a community we have worked very faithfully for many years to raise funds for this very drug; for you. I understand you can’t help everyone who is denied coverage, but it would be nice to see you try. The CF community stands up and helps one another. We work tirelessly to raise these funds with the expectation they will be used to improve the health and lives of those living with cystic fibrosis. My hope is that you will serve this community like we serve each other. Thank you in advance for doing what is always right, not what is always profitable.

Sincerely, 

Your Name

***

Are you tapped into social media? You can also help on Facebook and Twitter!

Facebook:

Vertex’s 2014 second quarter financial results stated Vertex had $1.22 Billion dollars in cash.  The only drug Vertex sells is #Kalydeco. Yet they refuse to provide the drug to two girls in Arkansas who have been denied access through Medicaid, even though they can, according to law. http://ow.ly/A5NCi

#Fighttheinjustice #supplydontdeny

Two girls in Arkansas whose health is declining are being denied coverage by Medicaid for #Kalydeco, a drug which could help them drastically. Legally, #Vertex can provide compassionate use of #Kalydeco while their legal case continues. Yet they are doing nothing to help. Help these girls. http://ow.ly/A5NCi

#Fighttheinjustice #supplydontdeny

It’s important for a company to do the right thing. #Vertex is not in the case of two girls denied coverage for #Kalydeco by Medicaid. Show your support for fellow Cysters and their families. http://ow.ly/A5NCi

#Fighttheinjustice #supplydontdeny

Doing what’s right is always more important than doing what’s profitable. http://ow.ly/A5NCi #Fighttheinjustice #supplydontdeny

Twitter:

@vertexpharma Doing what’s right is always more important than doing what’s profitable. http://ow.ly/A5NCi #Fighttheinjustice #supplydontdeny #Kalydeco

@vertexpharma How can a company with $1.22 billion dollars in cash not provide the compassionate use of #Kalydeco? #cysticfibrosis http://ow.ly/A5NCi

@vertexpharma The Arkansas cysters need #Kalydeco now, not a meeting! http://ow.ly/A5NCi #supplydontdeny #cysticfibrosis

I can't thank you enough for doing this and taking time out of your busy day to help these girls.

You can read more about the girl's case here: http://online.wsj.com/articles/costly-drug-vertex-is-denied-and-medicaid-patients-sue-1405564205

Thankful Thursday: 5 Star Chef & Tantrums

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 eggs and sweet potatoes. I found a recipes online to “eggs and sweet potato hash” that I modified a bit. We’ve had it for breakfast two days in a row, and I’m pretty sure there is no yummier breakfast. Seriously, It’s like heaven in your mouth.

I’m thankful for hard workouts. One trainer in particular runs a harder class than the rest of the trainers. I love being in her class. She knows what she’s doing, pushes you, and makes it fun. There’s nothing like a hard workout to make you feel accomplished for the day.

I’m thankful for tantrums. Ok, not really. Mckenna has been quite the pill the last few days, enough to make me feel crazy. But I am thankful for what tantrums symbolize. They symbolize a little girl that is growing up and has her own thoughts, feelings and opinions…I don’t like how she shows those thoughts, feelings and opinions at times, but I am thankful that she is becoming her own little person….now just to make her express those without being a monster :)

Ronnie's List:

I'm thankful for a wife that has been on quite the tear in the kitchen. Mandi has been on fire lately creating delicious breakfast, lunches and dinners for Mckenna and I. She makes some of the most well balanced and delicious meals on the planet. When I'm trying to eat more healthy, it's nice to have the support of a 5 star chef in the kitchen ;)

I'm thankful for family time. This past weekend my mom and brother came up from Tucson to hang out with Mandi, Mckenna and me. They also came up to watch Mckenna while Mandi and I attended my mother-in-laws birthday dinner. We're so seriously blessed by the amount of time we get to spend with our families. We realize that not everyone is able to do that.

I'm thankful for Apple TV. We use Apple TV to watch shows and to entertain Mckenna quite often. Sometimes, I even forget how much is packed into that little tiny black (or is it white??) box. I think I can speak for Mandi when I say that our favorite part of the day is after we put Mckenna down for the night and are able to hang out on the couch and watch a show together.

What are you thankful for today?

What We Ate Wednesday: Egg and Sweet Potato Hash

This week was a good one for new recipes. We are trying out "carb cycling" because I'm trying to learn it to help a friend who wants to lose weight, learn it...and well, Ronnie's always a good sport. So  this week we got extra creative with recipes, and it paid off.

Here was out favorite recipe for the week (and maybe my favorite breakfast of all time):

Egg and Sweet Potato Hash

This is a recipe I saw all over on Pinterest, and I just pulled from several recipes and made up my own:

1 Sweet potatoes
2 eggs
1/3 c. of Santa Fe Medley (frozen black beans, peppers, onions in corn from Bashas)
1/2 tsp of Mrs. Dash Original Seasoning

Preheat the oven to 400 degrees. Peel the sweet potatoes and cut them into 1 inch cubes. Place cut potatoes onto a baking sheet sprayed with Pam and put into the oven for 30 minutes (or until soft) - turning them occasionally. Cook the bag of Santa Fe Medley mix for 5-6 minutes in the microwave. Fry the eggs over easy (so the yokes are still runny) while everything else is cooking. Plate the potatoes and Santa Fe Medley mix first, sprinkle some of the Mrs. Dash Seasoning, and top with the 2 fried eggs.

Total Calories: 305
Grams of fat: 10; protein: 19; carbs: 31

And presto chango, you have breakfast. Everything, but the eggs, can be made the day before and/or you can use leftovers. It is totally delicious. Ronnie, Mckenna and I all devoured it. We loved it enough to have it the next day. I woke up looking forward to the tastiness!

Easy adds for extra calories would be cheese, olive oil on the potatoes before cooking them, and avocado. Ronnie ate his with 3 eggs, and Mckenna ate hers with 1 egg. Lots of easy add ons!

Things That Make My Lungs Feel Awesome: Exercise

PSA: Video games will not make your lungs feel awesome.

As an "old man" with CF, I've figured out over the years what things I can do to make my lungs feel awesome and what things I can do, or not do, to make them feel eh, not so much. I realize that, as with everything with CF, some of these things will work for others while some may not. Here's what I do know, nothing changes unless something changes. So, if you see something in the coming weeks that you may want to try, give it a shot. A real shot. A real honest shot. I used to try and take shortcuts with my lungs when I was younger and it took me many years, and lots of pain, to realize that there are no shortcuts when it comes to health. Being at our best health-wise comes down to two things - good decisions and hard work.

So with that, here is probably the #1 thing that I have found make my lungs feel awesome...

Exercise.

You probably saw me going there. We've all heard it and we probably hear it quite a bit. But what exercise exactly? Well, the short answer is to do any exercise that you'll commit to and do consistently. For me, that has been High Intensity Interval Training (HIIT). According to the ever so wise Wikipedia, HIIT is "a form of cardiovascular exercise. Usual HIIT sessions may vary from 4–30 minutes. These short, intense workouts provide improved athletic capacity and condition, improved glucose metabolism, and improved fat burning." Did you catch that? Improved athletic capacity (ie better shape, ie in general, better lungs) and improved glucose metabolism. For those of us in the CF community, that's a win-win. Now, some of you read "improved fat burning" and thought, "My doc says I cannot lose any weight!!" and therefore dismissed HIIT as an option for you. Can HIIT promote weight loss? Yup. Does it have to? Nope. If putting on weight is a struggle for you, you can safely do HIIT but you must increase your calories. You must eat before your workout, to give your body the energy to carry you through the workout (and not burn your fat in the process) and you must eat after your workout (to aid in the recovery process and not allow your body to use its fat stores). It absolutely can be done. And, if you want an even better shot at maintaining, or even gaining weight after including some HIIT into your routine, may I suggest that you add a weight lifting routine to your schedule as well.  Again, you must eat right to take full advantage of your hard work, but it can be done! 

And back to the lungs, HIIT (or other cardio exercise) is like doing an albutertol treatment at the gym. I was actually in a study that showed this was the case. Exercise opened up the airways, and kept them open for an extended period of time. Plus, doesn't it just come back to the old tried and true phrase "if you don't use it, you lose it"? If you don't use your lung (function), you'll lose your lung function. I like to think of my lungs as a muscle I'm working out just as I would workout my chest, biceps or legs. Instead of lifting weights to work out my lungs, I'm taking deep breaths. I'm coughing. I'm pulling for air (in a good way of course!).

The hardest thing about exercise is getting starting. Most people I talk to (CF or not) don't exercise for two main reasons: time and knowledge.

This just made me laugh.

I don't think I've ever met a person who thinks they have a lot of time to spare. It's in our human nature to always feel busy...even if we're not. The bottom line is we all make time for things that are important to us. I have not been writing in this blog consistently for much of 2014. Reason? I have felt like I've had no time to devote to writing. I've felt pulled in a million different directions and didn't think I had any time to spend on RSBR. The truth, I have been busy, but I've been devoting my time to things that I either felt were more important, have the potential to have a greater impact on the CF community or things that I enjoy more. So I've had plenty of time, it's just been focused on other things. (Note: One of those is exercise which I devote about 2 hours a day to). To make time for exercise, it must be important to you. You need to find some kind of external or internal motivation for doing it and doing it consistently. If I gave you 1 million dollars to exercise daily, would you do it? Of course! You'd make the time no matter what! Unfortunately, I can't pay you to exercise (I wish I could), but I would argue that better health is worth a lot more than 1 million dollars (to me anyhow!!).

Ask Unknown Cystic if he thinks it was worth making the sacrifice to exercise every day. He works A LOT and didn't think he'd have anytime to fit exercise into his life. What did he do? He got creative! I believe they call that "if there's a will there's a way". He decided that if he was going to be working, then he'd be walking too. Check out the results he's had after walking on a treadmill during his working hours for the past year: One Year of Using a Treadmill Desk. The proof is in the pudding.

I understand that many of us can't afford a treadmill in our home, but don't let that be your reason for dismissing his story. The point isn't that we all must walk while we're working, the point is that we all must just do something. Commit. To. ANYTHING. Active.

Forgive me if I'm making it sound too easy, but in reality, it can be. It's just a matter of making it a priority and doing it. Don't have 1 hour a day, how about 10 minutes? Is 10 minutes an optimal amount of time for exercise? Nope. Is it more optimal than 0 minutes? Absolutely!

How can you get started? I'm glad you asked. Pick a time today that you're going to do something active and commit to doing it. Reading this really late at night? Pick a time tomorrow. How much time? At least 10 minutes and no more than 45 minutes. What's active? Anything that makes it harder for you to breathe as a result of exerting effort. That could be from sitting up and down on your couch multiple times to running 5 miles. Whatever it is, if you did nothing so far today that could be considered active, you'll already be two-steps of today, tomorrow (or later today!). 

This is all about improving yourself. Not being better than anyone else, just a better version of yourself. You can do it, just take the first step...of many, many steps.

Need help? Just ask. You can leave a comment here or find me on CysticLife.

If you want to get involved in the conversation, use #awesomelungs on Twitter and tweet @ me yo!! I'd love to link to any blog you write about what you do to make your lungs feel awesome. Send me a link to your blog so I can share it!!

I won't guarantee that it will be fun, but it will be worth it!!

Mandi Monday: My Favorite Mckenna Things Right Now

Being the mommy of a toddler has it's ups and downs. I never knew a little human could be SO fulfilling and bring so much joy, while being so frustrating and confusing. Toddlers are funny creatures. Their emotions change constantly and their impulse control seems non-existent. This makes them happy and hilarious 50% of the time, and crazed and confusing 50% of the time! Ha! But  even still, this may be my favorite age yet. I love that I can see the person that God gave us. Here are my favorite things she's doing right now:

Morning Ritual - Our morning ritual makes me smile just thinking about it. As I've mentioned in another post, "magic" opens her door at 6AM, and that's when she can get out of her bed. When she gets out of her bed, she carries a majority of the things from her bed (baby, stuffed animals, blankets, ice pack...yes she likes sleeping with an ice pack, etc) into the entry way (right next to her room), and sits it all onto the area rug and lays there; yelling for me. I usually do some silly dance down the hall towards her and she pops up and silly dances/runs to me for a hug. We carry all her stuff to the couch, where we snuggle and watch cartoons (usually Peter Rabbit) while I feed her cereal and milk (she could feed herself, but I'm not willing to get milk all over and couch! Ha! So I hold the bowl and pass over a spoonful from time to time). We continue this fun-fest until Ronnie gets up, and we make us all eggs and get on with the day.

Random Kisses - Mckenna is such a little lover, and one of her favorite ways to show love lately is through random kisses, usually on my forehead or the top of my head as I'm sitting next to her. It makes my heart so full when I feel her little lips on me.

I Miss You - Mckenna lately says, "I miss you, Mommy," or "I miss you, Daddy," a lot...even when you've been with her all day long. We aren't totally sure, but we think it's actually her way of just saying "I love you" or showing love and affection, even if the statement doesn't quite work. It's so sweet.

Bedtime Chatter - Mckenna, like most toddlers, has started the old bedtime stalling routine. She needs everything just so, the nightlight color just right, the books read, the sheets snuggly, water sipped, etc. Her newest? Chatting. She wants to discuss all sorts of ponderings she's ever had...from bulls we saw 6 months ago, to my grandfather's funeral, and everything in between. She knows she can add at least 3-5 minutes, and so she goes into question and statement overdrive. It can get frustrating, so I've just rolled back the time we head to bed a few minutes because, well, I kind of enjoy that little extra window into her brain.

Tracing - Mckenna started school last week. They have started tracing letters. Day one, Mckenna and I sat to do her homework, and well, it was almost scary. She had the attention span to do about 1 mm worth of tracing, and them would just scribble all over. I thought to myself, "oh boy...this may be bad..." but sure enough, 2 days later it was a whole different story. I say writing books in the dollar bins at Target, so I bought them and figured some extra practice wouldn't hurt. That night, she sat tracing as I made dinner, and to my shock, she stuck with it until she had traced all the letters on the page. I don't know if I've ever felt so much pride. You could tell she had tried her very best, and was so proud of herself when she was done. My heart exploded when I looked at the determination in her pen strokes...it may not be "perfect" but it's one of the most beautiful things I've seen!!