This is what happens...

...when daddy is in charge of mealtime!

Thankful Thursday: Picnics & Playtime

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 picnics. Ronnie and I are so blessed to both work from home, which gives our family a lot of flexibility. Yesterday, for lunch, we enjoyed a picnic together in our backyard under the shade of a tree. We ate, Mckenna splashed around in a bin (yes, bin, not pool ha!)

I'm thankful for new words. Mckenna has a new sound she makes. She has been making the sound Dadada all day for the last month and a half, but her new sound is...wait for it...MAMAMAMAMA! WAHOO!

I'm thankful for a gym that's close by. On Tuesday, our plans for the day changed a bit last minute, but because our gym is so close, we were able to both make it to the gym independently for a quick workout.

Ronnie's List:

I'm thankful for playtime with Mckenna. Each and every day I get to play with Mckenna and watch her discover all types of new things. I'll be the first to say that at times, playing with a baby can get a bit boring, BUT I am thankful for this time in her life and try to remember that she'll only be this age once. Plus, any one-on-one time I get with my baby is a good thing!

I'm thankful for AIM. Mandi and I work in separate parts of the house and AIM makes it much easier to communicate. There are a few times when we can't get our point across via the interweb, but 9 times out of 10, it does just fine. Plus, we save our voice boxes from trying to shout our messages across the house :)

I'm thankful for the pest guy that just came. We had a "professional scorpion hunter" (my title, not his) named Jason come out to our place and dust/spray our yard plus black light for  scorpions. We ended up catching about 30 adults and 30 babies. Not bad for a night's work! Let's just hope that the dusting and spraying does a number on any that are left.

So, what are you thankful for today?

Hospital Noise Fractures Sleep, Slows Healing

I found this article/study very interesting as I am a big believer in hospital stays and feel that they contribute positively to my health. What about you? Read the article and then tell me what you think!!

Nighttime noise in hospitals adds up to poor sleep, which may hurt healing when patients need it most, researchers found. 

In a laboratory sleep study, recorded hospital sounds of overhead paging, IV alarms, squeaky carts, and the like disrupted sleep and raised heart rates, Orfeu M. Buxton, PhD, of Harvard and Brigham and Women's Hospital in Boston, and colleagues reported.
Electronic alert sounds like ringing phones and IV alarms were the most "potent" in arousing sleepers, disrupting normal sleep brain wave patterns more than half of the time, even when set at their quietest settings. 

Likewise, staff talking and voice paging at a level of 50 dB -- quieter than normal conversation -- disrupted sleep half of the time in the study, which appeared in the June 19 issue of the Annals of Internal Medicine. 

"Preservation of patients' sleep should be a priority for contributing to improved clinical outcomes for patients who are hospitalized," Buxton's group wrote. 

Those benefits might include lower sedation requirements and shorter hospital stays, and even lower incidence of delirium in the intensive care unit, they suggested. 

Many centers contacted by ABC News in collaboration with MedPage Today have started implementing various strategies to limit night noise, which is consistently a top complaint from inpatients. 

That's important to tackle not only for the sake of patients but also for the hospital's reputation, Vineet Arora, MD, of the University of Chicago, commented in an email.
"Noise is a publicly reported quality measure for U.S. hospitals in CMS Hospital Compare [part of the HCAPS survey] and only half of patients report their rooms are kept quiet at night," Arora wrote. 

Common strategies cited were putting posters or stoplight-style monitors in hallways to remind staff and visitors to keep voices down, a switch to visual paging systems, and setting up "quiet time" hours. 

The study included 12 healthy adults who slept in a sound-dampened sleep lab for polysomnography over three nights. 

After a run-in night, participants were exposed to 14 common sounds -- such as voices, IV alarm, phone, ice machine, toilet flush, laundry cart, outside traffic, and helicopter noise -- played at levels increasing from 40 to 70 dB during specific sleep stages to test the impact.
Sleep was easier to disrupt in the phase after the transition into sleep, called N2, that adults spend most of their time asleep in, compared with the next deeper, slow-wave sleep phase called N3 or the rapid eye movement phase (REM, both P<0.001). 

Overall, as expected, louder sounds were more likely to disturb brain wave patterns during sleep. 

Arousal occurred at lower sound levels on the third night than on the second night, suggesting some sensitization to nighttime noise in the hospital. 

Heart rate jumped about 10 bpm when a sound fully roused a sleeper. 

These results likely underestimated the impact of noise on sleep among inpatients due to the young population studied, whereas the typical hospitalized patient is older with medical and psychiatric conditions, as well as pain and medication use, all of which contribute to a harder time reaching the deep N3 stage of sleep, Buxton's group cautioned. 

Another limitation in that regard was that noise exposure was sequential, kept to 10 seconds or less, and halted if a participant started to wake up, which is not the case in a real hospital setting, the investigators added.

Original article: http://www.medpagetoday.com/article.cfm?tbid=33223#.T9i_EOwNECE.twitter

Can you relate? I sure can, but I still think the positives of a hospital stay outweigh the negative. You?

Childcare with Cystic Fibrosis

Ran onto this old question and I thought I'd share...

I am in a serioius relationship, and am starting to think kids might be a possibility for me. I have a couple questions - are you the primary care giver? Do you have help? I don't know how I would be able to take care of myself and a child too. I just would like some insight.

Right now, I'm not the primary caregiver as my wife is on maternity leave. When she goes back to work, I'll be the primary caregiver on the days she's working. We won't have any consistent help, but we both have family in the area that would be more then willing if needed.

As for us, it's just one of those things that comes down to priority. My family is my priority. For me, this means taking care of them as well as myself. Everything outside of those two things just simply isn't as important. This may mean less time for myself, my friends, my hobbies, ect, but I knew that and was willing to make that sacrifice going into this. It's hard work, but it's doable and rewarding.

With that said, if you feel that having a family would put an undo strain on your health, then I think it's really something you should think about. It could be very unfair to a child to bring him or her into a situation where you know that one parent will always be there, but not there, because of their health. Our children not only need us there physically, but mentally as well. Just something to think about.

Thank you for this question. Hopefully I was able to shed some light on our situation as well as my thoughts on this subject.

(Almost) Back in the Saddle

As I posted last week, I was struggling to get back into the workout groove after vacation, so I set some goals. I promised I'd report back, so here I sit...reporting.

My goal was to run 4 times, lift weights 3 times and evening walks. Well I'm happy to report I get a B+. I got the hard stuff in. I did 4 runs, and lifted 3 times. We did go for a couple family walks, but they weren't in the evening and they weren't daily. Time of day obviously doesn't matter, but number does. So This week I need to step that up.

It felt awesome getting back at it. I will say though, I made the mistake of lifting on my legs on Saturday morning, and it reminded me EXACTLY why I don't lift on my legs...ever. I tend to totally overdo it, and then my legs are incredibly sore for the next 3-4 days. Sore muscles are good. They show that whatever you're doing it working. The problem is that really sore leg muscles don't exactly bode well for running. So last night, I did a shorter run, to just get my run in, but only did 2.5 instead of 3. I ran like Frankenstein the whole time, but I was glad I at least got out and did it. Needless to say, I will be neglecting my legs in my lifting routine once more though!

This week my goals are similar but I'm going to up them a bit. I want to run 4 times, lift 4 times, and go for a family walk every day. I already got one walk in this morning, so far, so good! Ha!

Other than that, life is nothing new; work, child-rearing, working out, and occasionally cleaning the house (anyone else let the cleaning go a smidge with young kids? Yikes! It's always picked up, but clean, clean...not so much).

Oh, one exciting thing. I think we are going to go look at some bikes today. We got come cheapy ($80-$100) beach cruisers when we got married, but needless to say cheapy bikes don't quite hold up. So we are going to invest in some bikes that will actually last, and buy a kids seat, so family walk time can be family bike ride time some days! I'm pretty excited! We're dubbing them anniversary, mother's day, father's day gifts...because, no, we haven't gotten each other anything for any of those occasions, pathetic, I know.

That's about it! Hopefully next week I'll report an A+ and have some pictures of new bikes :)

Peanut Moving Inside Belly

I was finally able to piece together all of the different film I had of Mckenna moving inside of mommy...



Pretty crazy right?!?!?

Thankful Thursday: Cow's Milk & Blow Torches

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 grocery stores. We are so blessed to be able to jump in a car, and after a 5 minute drive, get to a central location that has any food or drink or toiletry, etc we want. That's a luxury I rarely give thanks for.

I'm thankful for breakfast for dinner. Last night we had breakfast quesadilla's for dinner. Why is it that breakfast tastes better at dinner time?

Speaking of breakfast quesadillas, I'm thankful for cow's milk products. Mckenna didn't do so well with cow's milk, we think, so I cut it out of my diet when she was 3 weeks old. I didn't go crazy and read labels of packaged foods, but I cut out all big sources, milk, yogurt, cheese, butter, ice cream, and the like. Well this last week I have tried adding a little cow's milk back here and there, and she's been totally fine! Man oh man have I missed cheese! There are so many tasty foods with cheese, I have discovered. Ha! I'm just so thankful that she's been ok with a little cow's milk, and I'm excited to expand my culinary horizons again!

Ronnie's List:

I'm thankful for homemade tortillas. We had a quick trip down to Mexico recently, and one of my favorite parts about being down there is picking up some piping hot, freshly made tortillas. It is also pretty cool that they're only $1 a dozen. We used some of them to make some "breakfast quesadillas" that were pretty darn awesome if I must say so myself!

I'm thankful for my home. After so much travel, it's just good to be home. It's nice to sit on my couch. It's nice to sit at my desk. It's nice to sleep in my bed. It's nice to be in my element. Don't get me wrong, I love being in different places once in awhile, but my heart will always be here at home...assuming of course that Mandi and Mckenna are here as well :)

I'm thankful for black lights and blow torches. At about this time every year, scorpion hunting season begins. We live in an area known as "scorpion alley" since all homes in this area are known to have a few scorpions running around. We do the whole pest control thing, but something that I've been told, and that I truly believe is that the only way to get rid of the problem, is to "seek and destroy". We went out last night and torched about 10 of our friends and I won't stop until every last one of them is dead.

So, what are you thankful for today?

Toddlers and Treatments

I recently received a great (and very common) question from a momma in the CF community. I thought that many of you could relate to her question and I hope that some of you get something from my answer :)

I was curious to know when u were a child how did you feel about doing treatments?**** understands she does them to stay healthy but every now and again she will get upset while doing them and say things like "I wanna b like everyone else!" "none of my friends have to do this!"She is only 5, I know it's going to be hard to relate all this to her but I wondered how your parents helped u to understand. And if understanding even helped with the anger. And how to help with the anger they feel surrounding cf.Thank u for always answering these questions you are such a valuable resource to us parents!

That's a tough age and a common problem...

First piece of advice, always, like 100% of the time, follow through with the "law" you lay down. Even at a young age, if my mom said it was going to happen, it happened. She made it very clear that first came treatments, and then came whatever I wanted to do. She never backed down - not even once. I knew that if I fought treatments, my life would suck, and she would make sure of it 

My mom also made it clear that no one is "normal" - what is that anyway??  She said that some people have braces, some brown hair, some are tall, some are short, some are fast, some have glasses and some have cystic fibrosis. She also encouraged me to be very open about it as it's not something to be ashamed of or to hide from anyone.

Also, have you ever connected with Lisa Greene and read through any of her stuff. My mom, unknowingly, used many of the techniques that Lisa (mom with 2 kids with CF) teaches in her courses as well as writes about in her book. CysticLife also co-authored many of our materials with her that I would highly recommend that you read: http://www.cysticlife.org/cystic-fibrosis-reading-materials.php

Finally, there are many parents who make treatment time a great family bonding experience. They may only watch TV together during treatments, or play games, or braid each others hair, etc. In fact, I find myself doing that as an adult - only allowing myself to go on websites that I like IF I'm doing my treatment!

Hope I answered your question. Just remember - it's easier to start now and feel some short-term pain and angst then start later. Teach good habits before bad ones are developed.

Shoot, last thing - I promise that she'll thank you for it in the future. I've never heard a fellow "older" CFer say "I wish my parents didn't make me do so many treatments". I have however heard many CFers say "I WISH my parents would have made me do treatments". She may hate you for it now, but she'll love you for it later.

Nothing Left in the Tank

Game Face

Harumph! I feel like a sluggish lard! Getting back into town after a vacation always means struggling to fall back into a good routine for me. As Ronnie posted, we often feel like we need a vacation after our vacations (especially with a little one who keeps mommy up a lot in the night while traveling). I'll be the first to admit, vacation doesn't bode well when it comes to exercise. I put it off. I'm on the go doing other things. I simply don't care enough to fit it in. I did run a bit while in Singapore, but I have yet to run since being home (yeah, bad, I know!)

But I'm putting my game face on. I'm gearing up. I'm getting psyched, and I'm telling myself from here on out, it's back into the exercise groove. Do you ever do that? Totally psych yourself up for getting into the groove? Part of it for me is that I am genuinely excited to get back into a groove. The other part of it I fake, so I can convince myself it won't be awful buckling down! Don't you just love being able to play mind games with yourself? Ha!

So here's my plan for getting back in the groove...

1. Get running - My goal is to run 3-4 miles 4 days this week. I don't care what pace, I just care about the distance. I'm hoping to get back into my 5AM run schedule that I was in last summer. It will luckily be a bit easier as it will be without my running buddy in my belly!

2. Get lifting - Ronnie and I will get to the gym to lift 3 times this week! This may be interesting for Mckenna. The last few weeks separation anxiety has started to set in...so it may mean some rough child care experience for Mckenna, but as my mom said, "if she cries when you're away, it just means you should go away more often." Ha!

3. Get walking - I want to get in some evening family strolls with Ronnie and Mckenna in the evenings. This is both for extra exercise, and family bonding time!

So there is it. I'll post next Monday on how I did. So here's to 4 runs, 3 gyms, 6 walks!

Best Proposal Ever

I happen to think that my proposal to Mandi was pretty awesome, but man, this makes me bow my head in shame. Just kidding, but seriously, this is the best one I've ever seen.

Thankful Thursday: Hubby & Helping Hands

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 a wonderful hubby that is also a phenomenal father. Ronnie has more patience in his little finger than I have in my whole body. Mckenna has been quite the handful since getting home and being jet lagged and Ronnie, the baby whisperer, has been all over it. So sexy to see a man be a daddy!

I'm thankful for a wonderful hubby who is also a great cook. Ronnie has made us dinners since we got home. He makes the best meals, throwing random spices together, that are delish!

I'm thankful for a wonderful hubby who is also a comedian. Life is just funnier with Ronnie around. Period.

Obviously this week I'm thankful for my husband!

Ronnie's List:

I'm thankful for for our successful trip and time with family. It was quite the adventure flying half-way around the world with a 7 month old in tow, but it was also one of the best trips ever. We saw stuff that is generally reserved for dreams and postcards, and got to experience two great cultures while we were at it. Mckenna was an absolute all-star the entire trip and exceeded our expectations big time.

I'm thankful for helping hands. My brothers looked after the house and garden while we were gone and I'm happy to report that nothing died and the house is still standing. It's always nice to have trusted family or friends to turn the keys of the fort over to. Definitely eases the mind while away.

I'm thankful for the chicken we just had for dinner. I marinated chicken breast for about 24 hours in soy sauce, garlic cloves and yellow mustard, and I must say, it turned out great!! We cooked it in tin foil to keep in all of the juices and the flavor absolutely exploded in my mouth with every bite. We also harvested squash from the garden which I over-cooked and over-seasoned. Whoops.

So, what are you thankful for today?

I Need a Vacation

So am I the only one who thinks vacations are a lot more work than day-to-day life? I know I'm not in the majority, but I can't be the only one. Now, please don't get me wrong, I feel truly and utterly blessed to be in a position to even take a vacation, so please don't mistake my question as a complaint. Even though vacations are so tiring there isn't a single part of me that would take back any time I've been able to fly the  coop and check out another place on the map - especially this last jaunt around the world.

Mandi, Mckenna and I recently got back from a trip to visit my in-laws who are currently living in Singapore. While there, we were also able to take a little side trip to Thailand. Both places were two of the most beautiful places I've ever been. Singapore is a cool, hip and happening city while Phuket, Thailand seems like a postcard at every turn. I just so very thankful that we had a camera to capture all of the breathtaking sights!

The purpose of this post isn't to talk about how great the vacation was or how beautiful the sights were. The purpose of this post is to talk about all of the reasons why I need a vacation following a vacation. Maybe some of you can relate, maybe none of you can, but regardless, thought it'd be fun to chat about.

So what was it about this vacation that was so energy-draining? Here are some ideas...

Man was not meant to be on a plane for 11 straight hours. Singapore is really, and I mean really, far away. The 11 hours spent in a plane was only the part in which we crossed the Pacific Ocean. That doesn't count the 2 hours to San Francisco and the 6 hours from Tokyo to Singapore. I'm not math major, but that's 19 hours of flying. Add in travel to and from airports plus lay-overs and now you're talking 27 hours!! Yeah, 27 hours of traveling. It's madness, absolute madness. In a perfect world, I'd be able to sleep like a baby on a plane, but that's made even more difficult since we had our own baby on the plane (and I wasn't even the one with her most of the time, Saint Mandi was). Then, you have the +15 hour time difference and now you're talking a formula that really throws your body for a loop. Ever had jet lag? They need a new name for this as those two words don't do this justice.

Singapore is very, very humid. You feel it right when you exit the plane. It's like Bruce Lee and Chuck Norris got together to give me some swift roundhouses to the chest. I admit that my lungs are a little "soft" due to the ever so dry climate that I'm used to, but seriously, Singapore is like walking in the biggest sauna in the world. I don't know about you, but I find it hard to breathe when there is a ton of humidity in the air. It's tough to get a real deep and giant breath in. We walked around a ton, which was awesome in keeping my lungs going, but I noticed that I didn't cough up nearly as much stuff as normal. What's really strange, is I've been coughing up stuff like crazy since being home. Could be the weather, could be the travel or could be pure coincidence. Either way, I know this much is true - humidity is not my friend and I'm so thankful for the hot and dry Arizona weather.

I'm a schedule/routine guy. I think I've mentioned this before when it comes to being away from home, but I think I'm even more uncomfortable than I realize when thrown off of my routine. The thing is, I thoroughly enjoy the spontaneity the vacations bring and all of the different activities, but subconsciously I think my body is screaming "what the heck is going on!?!?". When I'm at home, I can give you a pretty good idea + or - 15 minutes on how my day will go. I can tell you when breakfast, lunch and dinner will be. I can tell you when my treatments will be done. I can tell you when gym time is. I can tell you when I'll wake and I can tell you when I'll sleep. On vacation however, no matter how hard I do (or don't) try, everything must be a bit flexible. It's funny because that's what I like about vacations, I'm just not so sure my body does.

I'm sure the list could go on, but I'd like to hear about some of your experiences as well. They may jog my jet-lagged mind so I can add to my list. If you've made it through this blog, thank you. I don't want you to think for even one nano-second that I'm complaining. I'm simply observing and starting a conversation. If I had it all to do over again, I do it in a heartbeat. You could double the flight time and cut the vacation in half and I'd still be the first in line. I got to spend some amazing time with my family and see some stuff that I've only seen in the movies, literally. They call this "James Bond Island":

So tell me, am I the only one?

Travels Abroad

Surprise! We were totally holding out on you all!! As some of you have maybe have seen in Facebook photos posted the last day or so, we were in Asia...SURPRISE! We were holding out before posting because we didn't exactly want to let on to the fact that our house would be vacant and we'd be out of the country! So let me back up and give you the whole scoop, now they we're home, settled and we have pictures uploaded to share!

First things, first...why the heck did we go? My parents are living in Singapore now (they were in Shanghai as you may have seen previously). They've been there since February, so we decided it would be a great opportunity to get back to Asian and bring Mckenna for a world adventure.

We set off for Singapore with Mckenna in tow. We were really nervous about the 24+ hours of travel time (one way) and the possible jet lag with Mckenna, but decided to just go for it. Turns out, this kid can travel!! She was phenomenal both ways and jet lag wasn't a problem at all going. It has been a bit of an issue being back, but that's ok! She'll get there.

We had a total blast! We spent time in both Singapore and Thailand. We saw the sites, spend quality family time, and relaxed. We will post pictures and stories over the next few weeks, but I figured I'd put up a few photos and let you know the gist! Here are some pictures from the trip:

We'll post more of our 1,897,609,325 pictures later I'm sure!

Have a favorite of this group?

Hypertonic Not Better Than Isotonic Saline in Young Cystic Fibrosis Patients

SAN FRANCISCO – Inhaled hypertonic saline did not reduce the number of pulmonary exacerbations in infants and children with cystic fibrosis in a randomized trial.

The trial pitted 7% hypertonic saline in 158 pediatric patients against 0.9% isotonic saline as a control in 163 patients. The solutions were nebulized twice daily for 48 weeks, with both groups getting albuterol or levalbuterol beforehand. The patients ranged in age from 4 to 60 months. Adherence was at least 75% in each group, judging from returned study drug ampoules, reported lead investigator Dr. Margaret Rosenfeld at an international conference of the American Thoracic Society.

In the hypertonic saline group, the mean pulmonary exacerbation rate was 2.3 events/person-year (95% confidence interval [CI], 2.0-2.5), and the mean number of total antibiotic treatment days for pulmonary exacerbations was 60 (95% CI, 49-70). In the control group, the mean pulmonary exacerbation rate was 2.3 events/person-year (95% CI, 2.1-2.6), and the mean total number of antibiotic treatment days was 52 (95% CI, 43-61).

No significant differences were seen in secondary end points, including height, weight, respiratory rate, oxygen saturation, cough, or respiratory symptom scores. Adverse event profiles were similar, with cough the most common adverse event in about 40% of each group (JAMA 2012 May 20 [doi:10.1001/jama.2012.5214]).

"There is great interest in the CF [cystic fibrosis] community about developing early intervention strategies to delay or prevent CF lung disease before the bronchiectasis becomes irreversible. From our current evidence, hypertonic saline does not fulfill that role. Based on its inability to reduce the rate of pulmonary exacerbations, we would not recommend that it be used in this age range," said Dr. Rosenfeld, a pediatric pulmonologist and associate professor of pediatrics at the University of Washington in Seattle.

The finding was a surprise because hypertonic saline is known to prevent exacerbations in older children and adults, perhaps by helping the lungs cough out bacteria. There has been hope it would also help very young children, and its use in that population has increased substantially in recent years, Dr. Rosenfeld noted (N. Engl. J. Med. 2006;354:229-40).

"We’ve been scratching our heads about" why that hope didn’t pan out in the trial. "We have a number of hypotheses. The first one is that pulmonary exacerbations may be really different beasts in infants and young children. [Perhaps] they are mostly triggered by viral respiratory infections. Hypertonic saline can’t prevent people from getting respiratory viruses," she said at the conference.

Exacerbations might have been too blunt a primary outcome measure, according to an editorial that accompanied the published study in JAMA.

Very young children with CF have not yet developed the outright lung damage that makes older patients particularly susceptible to exacerbations. Perhaps more subtle markers of early disease onset and progression were needed in the trial, wrote Dr. Elliott Dasenbrook, associate director of the Adult Cystic Fibrosis Program at Case Western Reserve University, Cleveland, and Dr. Michael Konstan, director of the school’s Cystic Fibrosis Center and chairman of its pediatrics department.

"Although the results of the study suggest that inhaled hypertonic saline should not be used routinely in young children, the final verdict on its use for infants and young children has not been rendered. It would be disheartening if a viable therapeutic option was discarded because of negative study results when more sensitive end points might have detected benefit from the intervention. Testing therapeutic agents in infants and young children may require different end points capable of assessing onset and progression of disease," they wrote (JAMA 2012 May 20 [doi: 10.1001/jama.2012.5853]).

There was one "tantalizing" hint in the trial that hypertonic saline may delay structural damage, Dr. Rosenfeld said. Among the 22 children aged 4-16 months in the hypertonic saline group who had pulmonary function tests, forced expiratory volume in 0.5 seconds (FEV_0.5) was a mean of 38 mL greater (95% CI, 1-76) than among the 23 children tested in the control group, the only significant pulmonary function difference.

Perhaps that could be a marker in future trials, but "statistically significant difference does not necessarily imply clinical significance," Dr. Dasenbrook and Dr. Konstan noted. "Even though the results of infant pulmonary function testing appear encouraging, ... these exploratory end points should be viewed as hypothesis generating, and research exploring the clinical effects of these differences is needed."

That research is likely to happen. "We would like to study [hypertonic saline] further and see if we get a signal if we choose more physiologic end points," Dr. Rosenfeld said.

Dr. Rosenfeld disclosed that she is an adviser to Genentech and Vertex Pharmaceuticals, and receives research grants from Vertex. Dr. Dasenbrook is a consultant for Savara and Gilead. Dr. Konstan is an adviser to Aradigm and a consultant for Boehringer Ingelheim, Genentech, Novartis, PARI Respiratory Equipment, Vertex, and several other companies. He receives grants or has grants pending from several companies, and receives speaker’s fees from Genentech and Novartis.

**Original article can be found at http://www.familypracticenews.com/news/more-top-news/single-view/hypertonic-not-better-than-isotonic-saline-in-young-cystic-fibrosis-patients/07c72680a78e0268c8b8eea2b5cf97d2.html

Kalydeco: First Week In

Guest post by Angie

WARNING: To those of you that are not my fellow cysters and fibros or caretakers of aforementioned cysters and fibros, this post contains discussion about mucus… wait, if that would bother you why would you be reading a blog about CF? Never mind.

I took my first dose of Kalydeco on a Tuesday night.  I took it and went to bed.  It’s hard to know if Kalydeco was the cause or not, but I had a hacky cough that is usually brought on by drainage in my throat all night.  When I got up in the morning my throat felt really dry and scratchy.  The hacky cough continued throughout the day.  The next day (Thursday) during my morning treatment I noticed that the mucus I was coughing out was thinner and lighter in color.  Could this drug already be making a difference or was it just wishful thinking? In the short time I had to research the effects of Kalydeco, I don’t think I took any action to manage my expectations.  I think I was torn between expecting to see a difference right away and thinking that would not be possible it would be more of a marathon than a sprint.  After all, it’s taken 36 years for my lungs to get to their current state, so they aren’t going to improve overnight, right? 

On Saturday I was contemplating how my first few days on Kalydeco had gone.  I realized that I was coughing a lot less than I normally do.  This was a bit concerning because my family and friends have always been able to locate me by the sound of my cough.  If my cough had decreased, how would anyone find me when we were out shopping?  This is a problem I am more than glad to have!! I also noticed I had more energy.  Don’t get me wrong, I still need my weekend naps, but I think that is just because of my love for napping.

 

It’s funny, but I noticed the most changes during my first week on Kalydeco.  I’ve continued to feel this way, most of the time.  My cough still increases when the weather changes.  Since I live in the Midwest, the weather is known to change anywhere from 20 to 30 degrees in a 24 hour period.  This has always made my chest feel tight and my head feel like it was going to explode.  Since being on Kalydeco, I have noticed my cough increases with these weather changes, but I’m not having sinus headaches.  I have also had more big, old looking globs come out of my sinuses and lungs.  I take that as a good sign that some of the old stuff is breaking up.  The true test for me will be when I have my first PFT since starting Kalydeco.  I have a feeling we will be celebrating once again when that day comes!

Next Update: This truly is a wonder drug: I’ve started running (although I use the term “running” loosely)!

Note from Ronnie: If you missed Angie's first blog about discovering that she did in fact carry the G551D mutation after many years of thinking she didn't, click here (you can also find her bio). She also gives an update about her emotions after/during receiving her first bottle of "the hope diamond" here. 

Cruisin' in Her Walker

Thankful Thursday: Picnic Baskets and Baby Blues

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 talking picnic baskets. Mckenna's favorite toy these days is a talking picnic basket. She can't get enough of it, and I happen to kind of like the stupid songs it sings...win win

I'm thankful for sore muscles. I have been slacking on my running lately and have gone for a couple runs getting back into the groove the last couple days. Man are my legs SORE...and I love it. It means it's working!

I'm thankful for air conditioning. Summer is here. It's hot out. So coming inside and feeling a blast of cold air is fabulous!

Ronnie's List:

I'm thankful for my beautiful wife. We just celebrated our 2nd wedding anniversary, and although these last couple of years have flown by, I'm so thankful that I was along for the ride. She makes me a better man, husband, Christ follower, friend, son and father. With out her, I'd be the shell of a man.

I'm thankful for Mandi's support. I'm sure I've been thankful for this before, but I just got back from a run, so it's fresh on my mind. Just a reminder - I hate to run and wouldn't ever choose to do it on my own. There are times when all my self-motivation fails me and I need an extra push - My wife is that push. She is so supportive, loving and inspiring. I'm a lucky man.

I'm thankful for the baby blues. Mckenna's eyes look fake in many of the pictures we take. They could stay that way, or they may not, but either way, I'm thankful for them now. Her little bald head, big smile and blue eyes is enough to make my heart explode each and every day.

So, what are you thankful for today?

New Enzyme Approval

May 23, 2012 — The US Food and Drug Administration (FDA) has approved pancrealipase delayed-release capsules (Pertzye, Digestive Care Inc) for the treatment of children and adults with exocrine pancreatic insufficiency (EPI) caused by cystic fibrosis (CF) or other conditions.

The unique pancreatic enzyme product contains bicarbonate-buffered enteric-coated microspheres and is protected by several US and international patents. It has been marketed by the company under the trade namePancrecarb MS-16 since 2004.

Representing the sixth such product for patients with CF, Pertzye comes on the heels of 2 other newly FDA-approved similar products (Ultresa, Aptalis Pharma US Inc; and Viokace, Confab Laboratories Inc), as reported by Medscape Medical News.

Other previously FDA-approved pancrealipase pharmaceuticals include Creon (Abbot Labs), Zenpep (Eurand SPA), and Pancreaze (Ortho-McNeil-Janssen Pharmaceuticals Inc).

Small Study Leads to Big Results

FDA approval was based on data from a small, randomized, double-blind study of 21 patients aged 8 to 43 years (mean, 20 years) who were randomly assigned to receive the study drug at individually titrated doses or matching placebo for 6 to 8 days of treatment, followed by crossover to the alternate treatment for an additional 6 to 8 days.

Results showed that the mean difference in coefficient of fat absorption (CFA) was significantly higher for the study drug relative to placebo, as determined by a 72-hour stool collection during both treatments to measure both fat ingestion and excretion (83% vs 46%; 95% confidence interval [CI], 28% - 45%; P < .001).

The coefficient of nitrogen absorption was likewise significantly increased (79% vs 47%), as evaluated using the same stool samples, with the assumption that proteins contain 16% nitrogen.

No differences were found between children and adults with respect to the severity of pancreatic insufficiency or response to the study medication.

"The improvement in mean CFA observed in the controlled study represents a clinically meaningful treatment benefit.... Availability of this unique buffered formulation of pancreatic enzyme is an important addition to the therapeutic options for CF and other patients with EPI," stated Michael W. Konstan, MD, chairman of pediatrics, Rainbow Babies and Children's Hospital and Case Western Reserve University School of Medicine, Cleveland, Ohio, in a company news release.

The most commonly observed adverse events were diarrhea, dyspepsia, and cough (10% vs 4% for those receiving placebo, for each event).

Recommended Dosing

Pancrealipase delayed-release capsules will be available in 2 formulations:

• 8000 USP units of lipase with 28,750 USP units of protease and 30,250 USP units of amylase; and
• 16,000 USP units of lipase with 57,500 USP units of protease and 60,500 USP units of amylase.

Children aged from 1 to less than 4 years and weighing 8 kg or more should begin with a dose of 1000 lipase units/kg body weight per meal (maximum, 2500 units or ≤10,000 units/kg/day) or less than 4000 lipase units/g fat ingested daily.

Older children and adults should start with a dose of 500 lipase units/kg body weight (maximum, 2500 units/kg per meal or ≤4000 lipase units/g fat ingested daily).

Because ingestion of larger amounts is linked to a risk for fibrosing colonopathy, the FDA warns patients to contact their clinician immediately if they have unusual or severe abdominal pain, bloating, and trouble passing stool, nausea, vomiting, or diarrhea.

Capsules should be taken whole to avoid irritation of the oral mucosa, but contents may be mixed with applesauce for infants and others having difficulty swallowing.

Treatment is associated with a risk for hyperuricemia, so uric acid level monitoring should be considered in patients with gout or renal impairment. Caution is advised when prescribing pancrealipase delayed-release capsules for patients with a known allergy to proteins of porcine origin.

Go to digestivecare.com for more information.


Original article can be found at http://www.medscape.com/viewarticle/764351?src=nl_topic