If this doesn't get you in the Christmas spirit...

....nothing will :)

Moganko For Cystic Fibrosis Awareness

In the mood to not only be entertained today, but also educated? Good, you've come to the right place!! Allow me to introduce you to Moganko. Moganko is a buddy of Josh from Joshland and his job is to spread awareness of Cystic Fibrosis, as well as show kids (and adults) that living with CF can be full of fun and laughs. Please watch the following video and then head over to www.mogankoforcf.org to check out a TON of other cool videos that will entertain AND teach your kiddos some great lessons about life and CF.



Now following the following link to support this incredible project and give my fellow fibro Josh some love...and you can say hi to Moganko too :)

Moganko For Cystic Fibrosis Awareness: Welcome Everyone!

Thankful Thursday: Zoos & Home Depot

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 holidays. My parents just got back into town for Christmas so we get to spend time with them, the house is decorated for Christmas, I eat at least 5 mini candy canes a day, Starbucks has a special Carmel Brulee latte, and everyone is especially nice and filled with holiday cheer. I LOVE everything about the Christmas season :-)

I'm thankful for my sweet baby girl. I love her so much. Her every smile, coo, squeal, you name it, make me smile. She is such a doll - so sweet, happy, silly, and smiley. It's amazing to watch her grow. Each day I love her more and sometimes find myself just staring at her (generally while feeding her) and wondering how we got so blessed and why God chose us to be the parents to this little peanut. This may be weird, but sometimes I just want to squeeze her...but I refrain.

I'm thankful for girlfriends...and girlfriends with babies Mckenna's age. Yesterday we went to the zoo with my friend Angie, who has a little girl, Harlow, that is 4 months old. We bought a year membership, so we are going to start having our weekly get togethers there, so we can get a little fresh air and exercise while we chat. Angie and I get together weekly with the girls to hang out and swap baby stories (every notice once you have a baby you find it hard to find other things to talk about?!) I can't wait for Mckenna and Harlow to get a little older so they can start actually playing together instead of just looking at each other.

Ronnie's List:

I'm thankful for the Phoenix zoo. I've been living in Phoenix for almost 7 years now yet I had never been to the zoo until yesterday. I'm no zoo connoisseur, but I'd have to give the zoo high marks for it's walkability. A few of the animals were pretty cool to see, like the mountain lion, giraffe and baboon, but I mostly enjoyed the nice relaxing walk around the grounds.

I'm thankful to have a Home Depot so close to the house. It's so nice to be able to hop in the truck and be to HD in a matter of minutes. I'm not the most handy guy in the world, but having that place so close definitely serves as a security blanket during projects. If I don't know exactly what to do, which is generally the case, I can usually find someone at HD who does!

I'm thankful for bath time with Mckenna. One thing that I look forward to everyday is bath time with my little girl. Mckenna totally relaxes during the bath and is usually in the mood to interact and smile at her daddy. It's definitely a time with her that I greatly cherish. 

So, what are you thankful for today?

Ever Have One of Those Days?

You ever have one of those days? I can see all of you throwing up your hands right now saying “Uhh, YEAH!!!". And for those of you who don't know what I'm talking about - I'm talking about those days where you're running around like a chicken with its head cut off and just when you think the day is winding down something pops up. The days when you're at the post office and decide to use the self-service machine to “save time” and the woman in the front of the line decides to use said machine to get all of her postage and stamp each one of her Christmas cards individually. Yeah, that kind of day. The kind of a day where all you want to do when you get home is kick off your shoes, put up your feet and just hang out. The kind of the day that the thought of doing something active at the end of it makes you want to cringe. Do you guys know what kind of a day I'm talking about now?

Yesterday was that kind of day.

All day I knew that at some point I was going to have to get my butt to the gym. It was tough to get done everything else that I needed to get done, including treatments, but gym time felt like it was looming over my head. I made a commitment to myself and to my family that I would make my health my number one priority. A big contributor to my increased lung function and energy has been consistently getting to the gym. It's not that I love being there, I actually love leaving the gym, but I know it's what I have to do if I want to be the best version of myself. Still, there are days like yesterday. The kind of a day that even a priority in your life seems just too hard to accomplish.

When I finally got home from running around the city, the first thing I did was love up on my family. It was probably a little bit of a stall tactic on my end thinking that the further off I put the gym maybe it would go away. I asked Mandi what our plans were for last night (take note guys) and she responded with, “you're going to the gym”. I love that woman. I felt guilty about being gone a good part of the day and even when I was home not really “being” there, but Mandi made me feel at peace with it by saying those five words. It's so nice to have a supportive wife that is willing to recognize how important it is that my health needs to be our family's priority. Admittedly, it makes my life about 1000 times easier. And it's not something that I think we can expect out of our spouses either, it should be, but sadly it often times isn't. I think communication was key and is key for us in terms of setting priorities for our family. Mandi made it very clear that she wouldn't be a nag when it came to my health, but she also made it very clear that she would kill me if I didn't take care of myself (Duly noted). That made setting my priority pretty easy. Oh yeah, I did in fact make it to the gym and I had a very good workout.

I guess my main point of this blog though is to encourage you guys to not only put your health as your number one priority, but to be willing to do anything to be the healthiest version of yourself. This doesn't have to just be for CFers either. To you parents, start right now with instilling in your child that there's nothing more important than their health. Without good health, we don't have much. And we must make taking care of ourselves the most important thing in our life.

Don't want to start with health? That's fine. Here's my challenge for you. Do something today that you've been putting off. It could be cleaning the bathroom, running errand, doing an extra treatment or finishing a school paper, whatever it is, promise yourself that you'll get it done today. Because here's the thing -  both you and I know that once you do it, you won't regret it.

Landscaping Troubles and Cystic Fibrosis

Mandi and I had been talking about buying plants for quite some time. We finally brought ourselves to get to a nursery and walked around for about an hour with the worker picking out plants to go in our yard. Like most people, we were really trying to find luscious, green, drought tolerant, freeze tolerant, hardy, beautiful and pleasant on the eye plants to fill up our yard. That's not too much to ask for right? I'm quite green when it comes to all of this yard stuff as I grew up with nothing but typical Arizona landscape. My idea of a yard included a lot of dirt, some pea gravel and a few desert style plants. I had never lived anywhere that had grass I had to maintain nor am I so sure that I knew what a drip system was.

After hemming and hawing over a handful of plants we finally decided on: duranta, fortnight lily, gardenia and agapantha. We were assured that this winter wouldn't be as harsh as last and that all of these plants would be fine if planted right now. I've been putting off planting for quite some time mostly because I had yet to yank out all of the dead stuff from last winter, whoops. While my dad was in town for Thanksgiving he finally gave me the motivation, and help, that I needed to get the job done. Let me just say this right now– I'm so thankful that I don't dig up plants for living.

When it finally came time to plant I woke up nice and early in the morning so I could get a jumpstart on the day ahead. I decided to start in our courtyard as this was the place that was going to require the most digging. There were a couple of spots that I was basically just replanting and those were no big deal, but the places that I needed to dig fresh holes was harder than expected. It is seriously amazing how hard the ground can be in some spots. It's pretty disheartening after digging for 20 min. and getting the hole to a place that you think is deep enough only to put the plant in said hole and see four inches of the root sticking out. Fortunately, I had nothing much going on that day, so I kept digging!

I finally got done in the courtyard (we planted three gardenias and two agapanthas) and it was on to the planters in back. I started with the planter on our North wall. We decided to plant three duranta and try our hand at training them up a trellis. The other task that I was intent on completing was building the beds back up. Over the years they've lost some of their soil to various conditions and it was definitely time to address that situation. I was able to plant the plants fairly easily as there were roses bushes and shrubs there previously, so it was a just a matter of ripping out a few existing roots. After I got them planted I cleaned up the drip lines, threw on some topsoil and followed that by mulch. I must say, it looks pretty good.

Then it was on to the bed/planter on the Northeast side of our yard. There were a few more rose bushes planted there previously which we ripped out when my dad was here. All that was left was the Queen Palms. We decided to throw in some fortnight lilies and an agapantha. After having a fairly smooth and trouble-free time, I was really looking forward to knocking out this project. But as we all know things rarely continue to go smooth in this life, I was up for quite a big challenge. Little did I know how “crazy” and difficult it would get.

To be continued...

Mckenna Monday: Making the Butter

Have you noticed the trend yet? Mandi Monday's come so late that they practically get posted on Tuesday! I used to write my Mandi Monday's on Sunday and have them automatically post Sunday night/Monday morning (shhh don't tell). But days just come and go and then all of a sudden it's Monday morning and I realize I've been a slacker for another week. So since I'm terrible, I'll send in my replacement...Mckenna. Sooo without further ado: Mckenna Monday:

Things are going pretty well for me. I'm getting bigger by the second. Mom says I'm a tank, but they still call me Peanut, so I'm not sure how that all fits together...maybe I'm a small tank? I had my 2-month check-up last Monday. I came in at 10 pounds 2 ounces and 22 inches long. If you're keeping track, that's exactly 4lbs and 5 inches since I was born. I'm growing like a weed! At that appointment I also got some shots. OUCHY!! I let out a huge wail once I realized what happened and that it didn't feel the greatest. Mom looked sad for me and like she was going to be sick, dad smiled with delight and kept saying "oh my goodness your face is so cute when you cry like that."

I'm pretty sure my name is Mckenna because they say it all the time (although they don't know I know because I don't respond to my own name yet). However, they call me lots of other names too. They call me Peanut a lot, which I've heard all along...I didn't hear Mckenna until much later in my life. But they also call me Goosey, short for Silly Goosey, which they call me some.

If there's one thing I'm good at it's making the butter. "Huh?" you probably thought to yourself. Yeah that's what I wondered too at first. My parents just kept asking "are you making the butter baby?" I finally realized that they're talking about going poo poo. Because everytime I dirty my diaper they say, "oh my goodness, did you make the butter? You're so good at making the butter, baby!" I didn't get it at first until I heard my mom explaining it to someone. Apparently mom thinks my poo poo smells like movie theater popcorn butter (you'll never look at it the same again I'm sure)...and it's yellow in color (I guess theirs is something different?) And because I drink milk and it churns in my tummy and then there's butter. So there you have it, mom and dad think I'm a butter factory.

Hm what else? Oh yeah, I've started sleeping like a champ. Bedtime is fun. I know what's coming because we do the same thing every night. I eat, we play a bit, I take a bath, eat again and then it's straight to bed. I go to bed around 9:30-10:30 and I wake up at 7ish. I either eat once around 4 or I just sleep all the way through. On the nights I sleep all the way through I could swear my mom looks like a different person and man is she thrilled to see me. She comes in and sings about what a good sleeper I am. I mean she's happy to see me every morning, but those mornings you'd think I had been out of town for the last week and she missed me like crazy. So maybe I'll start sleeping through the night every night? I'll try my best.

So, that's it. Life is good!

Inhaled Anti-Pseudomonal Drugs Promising in Cystic Fibrosis Patients

NEW YORK (Reuters Health) Dec 12 - Fosfomycin/tobramycin for inhalation (FTI) is a promising treatment for cystic fibrosis patients with chronic Pseudomonas aeruginosa airway infection and reduced lung function, researchers say.

Their phase II safety/efficacy study tested 28 days of FTI against placebo, immediately following 28 days of inhalation therapy with aztreonam.

Such continuous therapy is not typical. As the authors point out, "a 28-days 'on' and 28-days 'off' dosing schedule is used for inhaled antibiotics that are currently approved in the U.S. for airway P. aeruginosa in cystic fibrosis patients.

But the rationale for the "off" period is being questioned because of changing microbiological profiles and the increasing use of two or more inhaled antibiotics in rotation, they wrote online November 17 in the American Journal of Respiratory and Critical Care Medicine.

Both FTI and the aztreonam inhalation solution are being developed by Gilead Sciences, which sponsored the current trial.

Altogether researchers enrolled 119 patients (average age, 32 years) with a mean forced expiratory volume in one second (FEV1) of 49% predicted at screening.

After the 28-day open-label course of inhaled aztreonam three times daily, patients were randomly allocated to twice-daily FTI (60/40 mg or 80/20 mg) or placebo, for another 28 days.

With aztreonam, the mean improvement in FEV1 predicted was 7.0%. This improvement was maintained with FTI, whereas lung function in placebo-treated patients declined toward pre-aztreonam levels. The treatment effect favoring FTI 160/40 mg was 6.2% (p=0.002) and 7.5% favoring FTI 80/20 mg (p<0.001).

In addition, the researchers found reductions in mean P. aeruginosa sputum density in the FTI 80/20 group versus placebo (p=0.01).

FTI "was also effective against methicillin-resistant Staph aureus, for which there are relative fewer drugs," first author Dr. Bruce Trapnell, of University of Cincinnati College of Medicine and Cincinnati Children's Hospital Medical Center, Ohio, told Reuters Health.

He and his colleagues found that in 13 of 63 patients co-infected with S. aureus and P. aeruginosa, S. aureus could not be cultured from the end of the FTI treatment period (day 28) through the end of follow-up on day 56.

"However, the study was not set up or powered to evaluate this outcome," Dr. Trapnell cautioned. "The data should be considered supportive, but further studies are needed."

Fosfomycin is a phosphonic acid antibiotic active against gram-positive, gram-negative, and anaerobic bacteria. Tobramycin is an aminoglycoside with gram-negative activity.

"Adverse events, primarily cough," were noted, the authors say. Respiratory events, including dyspnea and wheeze, were less common with FTI 80/20 than FTI 160/40. All respiratory events were mild or moderate in severity, and there were no clinically significant changes in laboratory values.

Along with sponsorship by Gilead Sciences, Inc., the study was supported by grants from the FDA and the NIH General Clinical Research Center.

SOURCE: http://bit.ly/vjcVCR

Original article: http://www.medscape.com/viewarticle/755291?src=nl_topic

The Power of Placebo

In a recent issue of the New Yorker, long-time staff writer Michael Specter explored the nature of the placebo effect in medicine, primarily by describing the work of Ted Kaptchuk, who directs Harvard University’s Program in Placebo Studies and the Therapeutic Encounter (PiPS). The program’s mission is “to examine the placebo response and the implications of medical ritual, the patient-provider relationship, cultural context, and the power of imagination and hope in the healing process. Through endeavors in the clinical, basic, and social sciences, bioethics, the history of science and philosophy, PiPS seeks to elucidate, quantify, optimize and reaffirm the humanistic and more intangible dimensions of health care.”

Kaptchuk, who studied Chinese medicine in Macau and practiced acupuncture for many years, is arguing for a shift in contemporary medicine away from high-tech and pure science to a greater emphasis on healing. Having recently received funding from the National Institutes of Health, he and his colleagues are undertaking neuroimaging and molecular studies to understand the biological underpinnings of the placebo response.

In one study, he and his group acknowledged that a problem in directly exploring the placebo effect in a clinical setting was the use of deception — not letting patients and providers know of they are receiving an active drug or not. So they designed a randomized study in which patients with irritable bowel syndrome were given pills they were told were “an inert substance, like sugar pills” or no treatment. After 21 days, patients given the placebo had significantly different changes on global improvement scores (5 versus 3.9, P=0.002) and symptom severity (P=0.03).

In another study, patients with asthma were randomized to treatment with an albuterol inhaler, a placebo inhaler, sham acupuncture, or no treatment. The albuterol group experienced 20% improvement in forced expiratory volume in one second (FEV₁) measured on spirometry, and the other three groups all improved by 7%. However, when patients were asked if they felt better, 50% on albuterol reported improvements, as did 45% of those using the placebo inhaler, and 46% of those who had sham acupuncture, compared with only 21% of those who had no treatment (P<0.001). The researchers concluded that, while objective measures such as FEV1 are important in asthma care, “other outcomes such as emergency room visits and quality-of-life metrics may be more clinically relevant to patients and physicians.”

Specter concludes his article with a personal anecdote. He had been experiencing chest pain and was worried, being an overstressed middle-aged man, so went to his doctor, who conducted a careful examination. The physician told him he was fine, but needed to relax — and the pain simply disappeared. This was an example of “exactly the type of ritual that, according to Kaptchuk, will have to play a critical role in the future of American health care.

So what do you think should be the role of placebo — if any — in tomorrow’s medicine?

Original article: The Power of Placebo http://bit.ly/t39hKo from @medpagetoday

Polar Express in Pictures!!

Thankful Thursday: White Noise & "Dates"

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 learning. Mckenna seems to be learning and growing every day and it's sooo incredible and fun to watch. She's now so smiley, chatty, and playful and I love it. I can't wait to watch how much she changes over the next few months.

I'm thankful for white noise. Mckenna loves white noise when she seems to sleep a lot better when it's on. We downloaded white noise tracks from iTunes and play them at night time and bed time...I don't see us getting rid of that method of sleep induction any time soon!

I'm thankful for the holiday season. My parents get back into town on Saturday for Christmas and new years and I cannot wait to see them again!! It's only been a month but that's felt far too long.

Ronnie's List:

I'm so thankful for a wife that pushes us to do things out of the ordinary. A couple of days ago we traveled up north to ride on the Polar Express (pictures will be posted tomorrow). Now, that's typically not something that I would choose to do, but I had a great time and look forward to going back for years to come!

...and on that note I'm thankful that Mandi encourages me to be social. Truth be told, I could stay at home and hang out with my family all day everyday, but Mandi takes it upon herself to set up "dates" with friends. I appreciate her taking the lead on it as it's generally something that I don't press, and I always have a good time with whatever she has set up!

I'm thankful that Mckenna is a fan of my Vest. It's pretty much a guarantee that as soon as my vest starts and Mckenna is in her swing, she'll be out for the count. You of course never quite know how babies will react, and I'm just thankful that she's not scared of it....yet :)

 

So, what are you thankful for today?

Sign Me Up!!!

Where do I sign-up? I think I'd be pretty good at THIS kind of hand-to-hand combat!!

Top Ten Tuesday: Why I Don't Like the Rain.

I think I finally discovered my kryptonite - Rain. Don't get me wrong, once in a while it's an okay thing, but generally speaking, I could do without it. Here the top 10 things that I don't like about the rain:

10.  Watering schedule. Whenever it rains like it did today I have to worry about the setting or altering the current watering schedule for my yard. Big deal? No, but it's still a nuisance.

9. Shoes. I guess I have to wear shoes when it rains but I'd much rather have dry feet than wet. The only problem with this is that I would much rather rock my sandals.

8. Blue skies. One of the reasons I love living in Arizona is that I can count on blue skies almost every single day of the year. When it rains, I'm stuck staring at a gray sky all day.

7. Sleep. I've never been one to take a nap, but when it's raining all day I suddenly have the urge to. It's tough to get going and get things done when you're yawning and feeling like you need to lay down for a while.

6. Lethargic. Not only do I feel like I need to take a nap, but every action I take feels 10 times lower than the same action that I took the day before. It's almost like I'm covered of molasses... including my brain.

5. Gym. Because of the previous 2 reasons I just stated, you could see how the rain messes with my gym time. Without Mandi calling me out yesterday, I'm not so sure I would've made it. I'm of course glad that I went, but it was tough.

4. Joints. As I'm sure is the case with many of you, my joints start to really ache when it rains. I'd say it's the worst in my ankles and knees, but the pin in my wrist doesn't feel that great either.

3. Jezzabel. Whenever it rains my sweet little dog turns into a scared little puppy. She's attached on our hip, which I really don't mind all that much, until she has to go to the bathroom.

2. Messy. Everything just seems to get a little more dirty or messy when it rains. Whether it's our clothes or the floors to our house, everything just seems to get a little dirtier.

1.  Circadian rhythms. I'm a big believer in circadian rhythms and there's no doubt that the rain throws mine for a loop. I've touched on some of the ways that rain affects me, but I just the best way to sum it up would be to say that it just makes me feel “off”.

Now I'm guessing that most of you out there probably like the rain. For the sake of this blog I of course lived it up a little in terms of stating all of the reasons I don't like the rain, and although I can name plenty of reasons that I like the rain, I still think this list would outnumber it.

So my question for you is: Do you like when it rains? Why or why not?

Learning through a Butt Dimple?

As you may or may not know, I'm a worrier. I'm pretty sure I've expressed that before, but if I haven't or you haven't read it, there you have it. I've outted myself :) Ronnie is the complete opposite when it comes to worry. I'll worry about just about anything. He could have his arm cut off and his worry would be about a 1 on a 1 to 10 scale. Whenever we have a potential issue, problem, dilemma, my mind wanders and I begin to worry about the what-ifs. I come up with all the possible what-ifs, and then begin to think of solutions and next steps for each of those possibilities. Ronnie finds this absolutely ridiculous. He is constantly telling me, "let's start to worry when we actually know what's going on." or "let's wait until we have an answer and then we can discuss all the options." And I always know he's right, but that doesn't stop a worrier from getting stuck in the worry-cycle when something arises.

However, this last month, I managed to break out of my typical pattern - and even while in new mom mode. Shortly after Mckenna was born, we noticed a little "dimple" on her bum - at the top of her crack. Many babies are born with a dimple (1 in 50), but hers was on the "bigger side of normal". And while they told us it was probably nothing and just a slightly bigger than normal dimple, each nurse took an extra peak at it while we were in the hospital...which can send a worrier like me into a tail-spin. At her first appointment with the pediatrician we asked about it. She told us that she too thought it was likely nothing, but that they would order an ultrasound to look and make sure all was ok with her spine/spinal cord. We were told that it would be correctable if it was something, but that it would be something on the spina bifida spectrum.

Typically something like this would make me a worried mess. But for some reason I felt ok about it. Maybe because if it was "something" it could be corrected with surgery, or maybe because I (gasp) am actually learning something from my steady-eddy husband. Regardless, I wasn't too worried.

Well wouldn't you know, Ronnie was right and not worrying until we had more info paid off and saved me a lot of mental anguish. We had her little dimple looked at last Friday, and all checked out fine - the dimple was nothing to worry about!

It's funny how God can grow you and teach you through experiences like this. Somehow He kept me at peace about the situation, and wouldn't you know, it was nothing. I learned that if I wait to worry, I can free up a lot of mental space!

Pics from Mckenna's Photo Shoot!!

You guys have a favorite??

Thankful Thursday: SwaddleMe & Yard

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 my brother. This sounds like a no-brainer, I'm sure. But it's worth mentioning! He called on his way home from work yesterday to chat and we talked about his day, work, etc. Man do I feel so blessed that I have such a great brother and that we have such a great relationship. I mean, we're practically neighbors (they live in our neighborhood) yet we still chat on the phone. He's one of the best guys I know, and I feel blessed to call him my big brother!!

I'm thankful for SwaddleMe swaddling blankets. They make it so easy to swaddle Mckenna. You just flip over the sides and it velcros on one side so it stays all nicely help together.

I'm thankful for a great hubby! Ronnie's been Mr. ChoreMaster the last several weeks. He's been taking care of all of the stuff around the house AND doing chores like planting bushes, decorating for xmas, etc....on top of being really helpful and doing 50% of the work with Mckenna! He's so amazing!!!!

Ronnie's List:

I'm so thankful for our yard. Even though I get super frustrated by some of the work required of it, not that I don't enjoy it, I'm just not good at it yet, I still love looking out to a green, lush yard. When looking for a house, having grass was really important to Mandi and not so important to me. I'm sure glad she stuck to her guns.

I'm so thankful for the children of our friends'. We had some friends over and they brought their little baby girl who is a few months older the Mckenna. It's nice to know people who have kids in the same age range as our little girl. Speaking of that, our neighbor had a baby about two weeks ago. Bonus!!!

I'm thankful for the area that we live in. It's so nice to have access to just about any type of store that you can think of within 2 miles of us. It was one of the main draws for this area, and now that we're living it, I see why. Need to make a quick grocery run? No problem, be back in a jiff! What's that, irrigation parts? They're on the way! Jezzabel needs a cut and color? You get the point :)

So, what are you thankful for today?

Genotype and Severity

This comment was actually left on a blog titled Question From Reader: More Severe Genes? from back in June, but I wanted to be sure to share it as a blog so that everyone can get a little more insight into this issue. The comment was left by Jessica, a cyster in the community that is about 150 times smarter than your average bear. In fact, I generally don't understand what in the heck she's talking about (I know, big shock). If you would like to see the original blog, be sure to click the link above. 


.........................................


There are actually six classes of mutations, and they don't directly range from 1-6 in "severity". Also, they don't indicate severity so much as the manner in which the CFTR protein and/or transport is faulty - a cellular issue that has not been proven to be affected by patient behaviors. There's a lot of great information with more detail and specifics in this article on the CFF website: 
http://www.cff.org/aboutCFFoundation/Publications... 

Taking care of yourself - treatments, exercise, nutrition, etc, are all still extremely important to put yourself in the best position for optimum health, but none currently available have any direct impact on the cellular-level mutation problem itself - that's exactly where these new treatments come in and why they're so exciting! 

Lastly, a quick but important distinction: DeltaF508 is the most commonly identified mutation, but *double* DeltaF508 is a genotype, *not* a mutation. Everyone has two mutations, which are often not the same; the genotype identifies the pair, and their interaction with one another (which is arguably equally if not more important than the mutations individually). DF508 is the most *commonly identified*mutation, but DDF508 is not necessarily the most common *genotype*, and if it is, it's by a slim margin - it's not like there is a vast majority of double deltas and other genotypes are sort of "odd type out" in CF statistics. 

Also, while that DF508 is the most commonly *identified* mutation , many scientists argue that may just as well be because it was the *first* identified mutation, and as more mutations are identified AND more individuals learn their genotype, that will likely change, as it doesn't make sense that DF508 would be the most common from an evolutionary biology standpoint. 

The most important reason to know your (or your child's) genotype is because the new wave of treatments are all going to be rather class specific, if not mutation specific - knowing BOTH of your mutations lets you know whether new trials apply to you (so you can participate and get them to market quicker!) and if they don't, whether the science might be in the same wheelhouse and therefore might eventually - or at least more quickly than a treatment targeting a mutation in a completely different class. 

Mckenna is a smiley baby...

...if we are willing to make absurd noises.

Reruns and a Lazy Mom

So not much is new. It seems like when you have a new baby I feel like every day is a rerun of the day before, plus or minus 5 percent. We got up, feed, change, play, sleep; feed, change, play, sleepy...and repeat over and over until bath and bedtime. Mckenna has fallen into a pretty good natural routine. Although she's still up 2-3 times in the night (8pm-7am ish) to eat (about every 3 hours). She teased me a bit with going a 6 hour stretch one night and a 7 hour stretch another night two nights in a row...but that didn't last. But boy did I get my hopes up. I've tried to recreate those days in the hopes of getting those nights, but no can do. That's ok though, she's a growing girl...momma can get up and feed her!

I've handled the no sleep aspect of mommy-hood just as I thought I would; not great. I love my sleep and need a lot of it. So being limited makes me pretty lazy. I don't feel that tired during the days, but I do feel lazy. It takes everything in me to do stuff around the house or make dinner (SORRY RONNIE...I get an F in housewife these days)! Hopefully I'll get more accustomed to the lack of sleep, or she'll start sleeping longer stretches...either way it would be awesome to be a little more productive and not a couch potato during the days.

Speaking of lazy, we bought plants at a nursery yesterday that we are going to plan in the yard, and I'm working to peel myself off the couch in order to participate in the planting! Ronnie has been awesome about getting projects around the house done. So today I'm going to try to join in on his awesomeness! We'll post pictures!

Anywhoo that's about it. Time for Bessy the Cow to go be milked ;-)

Wedding Through The Eyes of Dad & Denise

While my Dad and Denise were here, we downloaded some of the pictures they took from our May 2010 wedding. Man does time fly!! I thought it would be kinda cool to post how our wedding looked through their eyes...

Rehearsal

Rehearsal Dinner

Morning of wedding:

Wedding:

Reception:

Brunch the day after: