Another Great Positivity Blog

This is a blog by a fellow cyster, Lauren. It's actually in response to an amazing blog written by Piper just a few days ago. I highly encourage you guys to read through the start of this post and continue to read it by clicking over to Lauren's blog.

A CFer who I is listed for transplant and who I respect and admire so much, Piper, recently posted a blog which you can read HERE . She discusses positivity and questions how CFers, at different stages of the disease, can understand it. She got me thinking about how all CFers as well as non-cfers can universally understand each other's take on being positive. Before I begin my discussion I want to make a Disclaimer that this blog is in no way meant to offend anyone but rather to bring together the CF community in discussion.

First let me make a couple points:

1. CF is a confusing disease in the sense that it affects everyone differently. There are many factors contributing to this difference including environmental factors, upbringing, bacteria, access to healthcare and technology, age, biology, genetics (both with the CF gene and others), compliance, lifestyle, and just plain luck. Some people with CF may appear perfectly healthy, some run marathons, some have never been in the hospital, some have received transplants and are living healthy lifestyles, some live on oxygen every day, some spend half of their year or more in the hospital, some are preparing for transplants, and unfortunately still some don't make it past the age of 16, or 20, or 30 (and we pray for the families and lives of those people every day). The fact is, it affects everyone differently and although there are some people who are non-compliant, there are also people who try their best to be healthy and still end up sick.

2. It has been my own personal experience that if I do everything I am supposed to as far as compliance and exercise, that I am one of the "lucky" ones. But this is taking into account that I go to clinic and have been admitted to a world renown hospital in Boston, I was diagnosed very young (although not at birth), I have been lucky enough to always have good insurance that enables me to have the best treatments possible, I live a lifestyle that is not detrimental to my health (i.e. I have not yet begun to work full time), and I am young. I have had medicines like pulmozyme and tobi for the majority of my life, I have had access to the vest since I was 7. Yet, before I started to be religious about doing my treatments and exercising, I was in the hospital two or more times a year for 2 or 3 weeks at a time (and not just for routine things, I've had severe hymoptisis, i've been on oxygen, been to the ICU, been so sick I could barely talk, etc.) I also had my fair share of skipping treatments (sometimes for a week at a time) of cheating (dumping out my nebs, shortening my vest, not taking my pills) of not exercising, of being lazy. And I saw, as a direct result of that, a lot more time spent feeling sick and being in the hospital.

3. Although it is difficult to compare someone with CF who is virtually symptomless and someone who is getting new lungs, there should still be respect for the lives that we all live. There is still a baseline of numerous amounts of treatments and therapies that all CFers must do to keep themselves healthy. The disease is still progressive and just because someone may be healthy now, it doesn't mean their disease might not take a turn for the worst at any moment. (And vice versa, it doesn't always mean that just because someone is unhealthy now they can't get better)

I think that as a CF community we all need to respect the fact that our disease affects each of us differently, sometimes we don't have control over it, but that doesn't mean that we should ever stop trying to make ourselves better, like Piper said "here's to trying." We can still be inspired by each other's fights even when one of us is sicker than the other if we respect and understand how differently CF can affect some people. I stress compliance and exercise a lot in my blog because ...

To read the rest of Lauren's great blog, please click here.

How to Raise a Child with CF

The following issue is a very common one amongst the CF community. I just wanted to post the question and my response to see what you guys think. What would you have said?

Post:

Let me start by asking that no one give me the pat answer of "treat your CF kid the same as the non-CF kids" I know that in an ideal world we could pretend the CF doesn't exist and place the child rearing ahead of the CF, but the reality is that my first priority for my daughter is making sure she gets all of her CF treatments.

I'm really running into a problem with my three year old and discipline. Yeah, some of it is the age, but some of it is due to the fact that she is pretty spoiled in several areas. There are lots of things that I don't say "no" about for her. Its not about purposefully spoiling her -- but rather about fighting the important battles first. Alyssa HATES to do her vest and its a huge production every night. The only thing that works to keep her quiet and not ripping the tubes out or shutting off the machine is to let her pick the tv show we are watching. This means that at treatment time, whatever her brothers are watching turns off for the sake of keeping her treatment complete.

Another example is food. Alyssa has to eat like a CF kiddo, and that means that I allow her to have a wider choice of foods. If she doesn't like what I've made I try to accommodate her, if she doesn't eat dinner, she can still have ice cream afterwards. Different rules apply to her and the boys know it and deal with it.

But the problem is that she is beginning to see that different rules apply to her and they are spilling into all the other areas of life. She instigates issues with her brothers (they walk by, she pushes them or she purposefully pulls a toy out of their hand, or she throws a fit when the tv show is not "hers"). All of these not CF things get handled promptly by me. She gets a warning (unless she hits) and then a time out, but it doesn't change her behavior at all. Some days she'll get 10 different time outs (in her room). I talk to her all the time about good behavior and give lots of positive reinforcement for when she does share. But you can't exactly sing the praises of a kid who didn't hit their brother this time.

I'm going nuts here. And I know that a lot of this is because of the CF and how it changes the rules, but I just don't know what to do about it. I'd love some insight from those who have been there.

My response:
I have no CF children. I however have CF and think that my mom did quite possibly the best job ever. So of course with my advice, you can take it or leave it...

I would never ever ever say "because of CF". If you think you have problems now, wait until she's interacting in the real world where nobody gives you special privileges "because of CF". My mom treated me no different than my brothers. CF wasn't a central focus in my house. Yeah, I was a sick kid at times and had to go in the hospital and do my treatments 3 times a day, ok, so what? I was still a kid. I still got disciplined the way my brothers got disciplined (except a lot more cause I was a brat).

She's not eating the ice cream "because she has CF". She's eating it because she's 3 and likes ice cream. Don't finish your plate, no ice cream. If you're concerned about your daughter getting enough calories, there are powders you can put in milk that will add nutrition and calories. High fat and high sugar foods aren't the only way to pack on the calories. You can do it many other ways without her even knowing.

How do you handle your other kids when they disobey? Do the same thing for her. We are who we are; CF doesn't make us who we are. If she's this way with CF, she'd be this way without.
Schedule treatment time for the same time every night, that way, the boys can pick the show before it, and then when it's treatment time it's her turn. Simply allowing her more options and priority "because of CF" is going to create a monster.

I also agree that taking away something important to a child is usually the best option for discipline. At least, I know that's what worked for me, and I was a crazy kid.

Bottom line: I would treat a CFer no differently than his/her brothers or sisters. Life isn't going to be fair her whole life and that's not changing. Life isn't fair for all non-CFers as well. A lot of it is her age, but I really think you need to nip it in the bud before her whole mentality is shaped into "I have CF, therefore, my way or the highway".

With all of that said...I have no kids and I talk a big game now, but who knows how I'll respond until I'm in the situation. Just know that I get that and I'm just trying to help.

Ronnie

Was I too harsh? What would your advice be?

What Does Being "Positive" Mean to You?

I had something else planned for today, but I couldn't pass up posting another cyster's blog because it is just that good. I won't post the entire thing, but I HIGHLY encourage you to click here and visit Piper's blog. It's raw. It's real. It's thought provoking.

What does "positivity" mean to you in the context of CF, or life in general?

I feel like "positive attitude," "positivity," and other words get thrown around a lot by really amazing and well-meaning people, but I'm not sure everyone's version of what it means to be "be positive" is really the same. I mean, I highly doubt many people see themselves as having a "negative attitude" for example -- they may see themselves as being "realistic" or even "honest," and in turn they may think that others are unduly cheerful or even "in denial." And I guess this all got me thinking, because is one person's positive thinking another person's denial? Or is my realism your negativity?

And even more vexing, is our "hope" or "positivity" threatened by stories that don't fit into our paradigm? Or is our sense of self and worth degraded when others do better than we have with the same disease?

Please click here to continue to read this fantastic blog post!

I plan to respond to this blog sooner rather than later, but I know that it's going to take me a while. I tend to either offend people or put my foot in my mouth when it comes to this topic. I will try to craft a well thought out and sincere response soon though as I feel that this is a very important topic.

Wash Sprints

This past week has been pretty terrible for weather here in "sunny" Arizona. It seems like every time I get ready for a run, the skies open up and it starts to pour. I have to admit, it's really throwing me off of my game. I still start each morning out with a walk however by bundling up, throwing my hoodie on and just "toughing" it out. It's actually pretty peaceful to walk in the rain. I can tell you that I enjoy it far more than Jezzabel does. She still acts like she's going to melt every time she even thinks about rain. Anyway, getting that morning walk in for me is crucial. If I don't get that walk in, the "ick" feeling in my lungs will usually stay with me throughout the day.

Although the afternoon runs haven't been going as planned, Mandi and I have discovered another way to exercise. The wash behind her parent's house. Now, for those of you not from the desert. A wash is basically a river bed that rarely sees a drop of water and most of the time is "over grown" with some vegetation and random cacti. Lucky for us, her parent's house backs directly up to one. This has proven to be a great way to get in a quick workout and something the JBell thoroughly enjoys. See, Jezzy isn't the most disciplined walkee in the world and often tugs on her leash. The beauty of the wash, we're the only ones down there and she is leash free. I can't begin to tell you how much she loves it. It's nice for Mandi and I also as we can focus more on each other than constantly yanking back J Beezy.

We started a new game with Jezzabel that has proven to be quite the workout. While Jezzy is busy sniffing around and not paying attention, Mandi and I sneak off. Then just as we're getting out of eye site from her, we call her name and sprint the other direction. We run until J catches us. I'm not sure how many wind sprints we did today, but I know it was a lot and I was FOR SURE winded! We also managed to play a game of hide and seek which Jezzabel proved to be way too good at.

Point is, something is better than nothing. The weather doesn't always cooperate with what we want to get done and 5 minutes of sprints are better than sitting on the couch watching Judge Judy (although I did that also). Hopefully the weather holds up tomorrow, I push through this head cold I'm experiencing and maybe, just maybe I'll actually be able to run after putting on my shoes!

Smog in the City

I was going through some old video from my trip to China last year and ran into this...I almost forgot how much smog there was in Shanghai. Not sure how I ever made it out alive.

When Tune-Ups Don't "Fit"

Today at lunch Ronnie and I were just discussing the busy couple of months we have coming up in our lives. My brother, Josh, is getting married, Ronnie goes out of town to speak at an event in Albany, Ronnie needs to get in for a tune up, we have wedding prep, our wedding, and our honeymoon. We had tentatively thought through timing of it all, but we hadn't talked about it all since Ronnie's trip to Albany was scheduled. As we started chatting, we began to realize it was going to be a TIGHT squeeze to get Ronnie's tune up in. Josh's wedding is March 28th, and he is standing in it, so he can't go in until after their wedding. Then, Albany is April 16-17th, which means he can't go between Josh's wedding and Albany, because that's only 2 weeks. We get married May 22, which means if he goes in after he gets back from Albany, and stays 26 days (4 days under his typical stay). That would get Ronnie out 1 week before the wedding, just enough time to help with last minute plans and moving all of our stuff into his condo or into a new house (details on the house hunt possibly next week). Talk about a TIGHT squeeze. If anything goes slightly wrong while he's in, it could get interesting for the wedding day. But, we need to do it that way. The hospital stay needs to happen, so we're going to make it happen, and trust that everything will go smoothly.

All this to say, we truly believe that hospital stays are IMPORTANT. It is CRUCIAL to be proactive and not reactive when it comes to taking care of CF. Could we push off the stay? Yes. Could that result is him getting sick and causing irreversible damage? Yes. Is it worth that? NO. A little frustration and a little uneasiness about timing and scheduling is well worth preventing potential, long term damage. I would love Ronnie to be around the month before the wedding to help with last minute planning, errands, moving, etc. I will be forced to do a lot more work, and potentially all of the work, but one month of more work on my end and missing my fiance is well worth maintaining his good health and lung function. So I'll take it. Ronnie is being proactive and not waiting until he's really sick to go in, so it's likely that his stay will be nice and quick, and he'll be out and feeling awesome for the wedding and honeymoon. So we're going to work really hard this next month so he goes in as healthy as possible.

Red Stripe Ad

...probably my fondest memory in Jamaica

Exciting Article About Drug Delivery

Beating the cystic fibrosis barrier

Biodegradable nanoparticles capable of penetrating the mucus barrier in the lungs and gut of cystic fibrosis (CF) sufferers have been developed by researchers at John Hopkins University, led by Justin Hanes, professor of chemical and biomolecular engineering.

“Cystic fibrosis mucus is notoriously thick and sticky, and represents a huge barrier to aerosolised drug delivery,” said Pamela Zeitlin, CF expert and professor of paediatrics, who collaborated on the study. “Nanoparticles were engineered to travel through cystic fibrosis mucus at a much greater velocity than ever before, thereby improving drug delivery,” she said.

The nanoparticles comprise of two parts made of molecules regularly used in existing medications. An inner core of polysebacic acid (PSA) traps therapeutic agents inside while a dense outer coating of polyethylene glycol (PEG) molecules allows the particle to move through the mucus by preventing it from reacting with proteins.

Hanes previously demonstrated that latex coated with PEG could slip past mucus coatings, but because the body could not break it down into harmless components it was impractical. “The major advance here is that we were able to make biodegradable nanoparticles that can rapidly penetrate thick and sticky mucus secretions and that these particles can transport a wide range of therapeutic molecules,” said Hanes.

The nanoparticles have potential applications treating lung and cervical cancers, and inflammation of the sinuses, eyes, lungs and gastrointestinal tract where mucus is produced to protect sensitive areas, said Benjamin C Tang, lead author and postdoctoral fellow in the department of chemical and biomolecular engineering. Zeitlin concludes: “This work is critically important to moving forward with the next generation of small molecule and gene-based therapies.”

Read the full article here: http://www.labnews.co.uk/laboratory_article.php/5285/2/beating-the-cystic-fibrosis-barrier

My First Experience with Hemoptysis

**Reposted from August 2009**

I present to you the first edition of First Friday. I believe this idea came from Casey who wrote a blog about his CF "firsts"(click here to view) a couple weeks ago. Here are some examples of other's "firsts" blogs (that sure was a lot of the letter "s" at the end, how far off was I grammatically?) from Piper, CysticGal, Chrissy and Megan (if I missed yours and you'd like to be included, please shoot me an email and I'll add it).

What I've decided to do is highlight ONE of my CF firsts every Friday and try to capture exactly what happened and what I was feeling in that moment. It can be very scary experiencing something for the first time, so hopefully you guys can use this and other CFers' blogs as resources when those times occur.

My first bout with hemoptysis reared it's ugly head at about age 12. I distinctly remember coughing up so much junk that I ran to the toilet to spit it out. Now, for me, that was a big deal. Back then it didn't bother me at all to swallow mucus that I had coughed up from out of my lungs. Today, it's a different story, and I try to spit it out into a tissue (or on my floor). But this particular coughing festival produced much thinner mucus than I was used to and it did taste a little different than normal, plus, it was filling up my mouth. So I ran to the toilet to spit it out. Low and behold, my mucus was red. First I thought, "man what did I eat that made my mucus so red". Remember, I was a kid, a healthy kid, and hemoptysis wasn't even in my vocabulary.

As I leaned over the toilet and continued to cough up that lovely warm red stuff, it hit me, "Wait a second, it looks like blood and now it tastes like blood, hmmmmm, it must be blood!" Here's the deal though, I didn't panic. I don't know why and I still can't fully explain why I don't panic now 17 years later when I'm coughing up CUPS of blood (although I admit, coughing up multiple cups of blood while in CHINA, didn't produce the calmest of emotions). Back then, I remember thinking "this is kind of cool". I don't even think I told my mom or grandma (I was at her house when this happened) about the episode. I'm not a big fan of having people (especially my mom) worry about me. I do however know that people (especially my mom) worry about me already without bringing anything else to the table.

What's strange, is I didn't have another case of hemoptysis until well into high school or maybe even until college. After the age of 20 though it was pretty common for me to have a bout or two in between hospital stays. I would usually cough up around 1-4 tablespoons per bout. In recent history, I've been embolized twice for the hemoptysis, in November 2005 and February 2009. Both procedures were considered a success and I had very little bleeding between the two embolizations. Since February's procedure I have coughed up blood twice, but it was very little and during a run.

So there it is, my first experience with hemoptysis...wanna tell me about yours????

PS- I'm hoping to get no responses because none of you have ever experienced coughing up blood :) I can hope right?

Rondi Sings "Girls Just Wanna Have Fun"

This is long overdue and we want to thank Phoenix for his patience. Keep rocking it little fibro!! This is for you!!!

The Ebbs and Flows of Running

We can't always predict how our workouts are going to go from week to week, but what's important is that we keep moving forward. Sometimes we can let what should be a small setback turn into a large one. It's so important to set your goal and do absolutely whatever it takes to accomplish it.

Top Ten Favorite "Reality" Shows

So I just got done watching the season finale of The Bachelor with Mandi and I've got to say, I just don't get it. I, like Jake, thought that Tinsley would be the (2nd) most perfect wife out there. I never thought in a million years that he would pick Vienna. With that said, I TOTALLY get his reasoning. If you have to force yourself to be someone different or feel like you're walking on eggshells around that person, pretty sure it's not a good match. It's so important to find that person that you can be yourself around and not worry about the consequences.

Anywhoo, it got me thinking about reality tv in general and how much I love it. I know that I've done a blog about favorite tv shows, so there will be some overlap, but this is my Top Ten "Reality" Shows of all time...well, at least of the last year :)

10. Undercover Boss - This is a new one that airs on Sundays. It takes a CEO of a company and has him/her go undercover in their own company to "learn the ropes" as a new trainee. It has basically the same story line every episode, but I have really enjoyed it thus far.

9. The Sing Off - I got into this show my last hospital stay. It had acapella groups perform against each other and I was absolutely fascinated by the sounds they could make.

8. So You Think You Can Dance - I have nothing but respect for people that can dance the way that they do.

7. Survivor - I don't watch every season, but when I do, I always enjoy it. This season is very enjoyable as it matches past "Heroes" vs "Villains". All of the character development has been done and now you can sit and enjoy the back stabbing.

6. The Bachelor - I don't understand the choices always, but I do always have quite a reaction.

5. Dating in the Dark - I'm hoping they bring this show back. The premise was simple. Have two people "date" without being able to see each other. Then, once they have picked a person on conversation alone, that person is revealed. Once revealed, they decide if they would like to continue with a relationship.

4. The Amazing Race - My mom got me into this show. I like being able to see other cultures and all of the beautiful scenery, plus, it's awesome to see hoe people navigate through the stress that the show brings.

3. Shark Tank - I'm pretty sure it's my goal to one day be on this show. You simply pitch an idea to 4 investors and if they like it, they give you money. Some of the ideas are a waste of time, but many of them are really cool.

2. The Biggest Loser - Probably the most positive and impactful (is that a word?) out there. It warms my heart every season to see the transformations.

1. American Idol - Best. Show. Ever.

Alright, let me have it! What are your guilty pleasure reality shows?

Engagement Photos Slideshow

As I shared two weeks ago, Ronnie and I had our engagement photos taken Valentine's Day weekend. We finally got them back and, as promised, we wanted to share them with you.

Do you have a favorite? We're trying to figure out which one to use for our "Save the Date".

You can see all of the photos here if you're on Facebook :)

One Happy Couple

Smart Pseudomonas

Adapting To Clogged Airways Makes Common Pathogen Resist Powerful Antibiotics

People with cystic fibrosis frequently have lung infections that defy treatment. Cystic fibrosis is an inherited disease that clogs airways with thick mucous. While the life expectancy for children with cystic fibrosis has increased over the past few decades, many lives are still shortened in young adulthood by the ravages of lung infections.

These chronic infections are often caused by common, environmental microbes that mutate in ways that let them live and thrive in viscous lung secretions. The same adaptations also make the pathogens less likely to be killed off by powerful antibiotics, according to a recent study led by Dr. Lucas “Luke” Hoffman, University of Washington assistant professor of pediatrics.

Surprisingly, he added, the pathogens don’t need any previous exposure to the antibiotics to resist their effects. The results were published in the latest edition of PLoS Pathogen.

The researchers looked at Pseudomonas aeruginosa, a microbe that can infect a cystic fibrosis patient early in life and then undergo various changes as it establishes a chronic lung infection. Pseudomonas aeruginosa with specific alterations tend to give patients a poor outcome. Some of those alterations diminish the chances of eradicating the infection with antibiotics.

It’s believed that these adaptive alterations in Pseudomonas, all of which are caused by genetic changes, could be selected for by the environment inside a patient’s airways, the researchers noted. Characteristics that facilitate microbial survival begin to emerge.

The specific airway conditions that select for these genetic changes, Hoffman said, remain unclear. “But,” he added, “we have some clues from what is known about airway mucus.”

From the point of view of Pseudomonas, the physical properties of cystic fibrosis mucus, Hoffman said, “make it a great place for the stuff people routinely breathe in to set up shop.” Cystic fibrosis secretions contain a lot of nitrates and amino acids, which Pseudomonas can use to grow.

Inside mucus plugs oxygen levels are low. Some Pseudomonas strains can live in this oxygen-poor, nutrient-rich environment. Hoffman and his team found that a mutation that occurs commonly in Pseudomonas from cystic fibrosis patients allows the pathogen to grow better in the nutrient environment in cystic fibrosis secretions. This particular mutation inactivates a gene named lasR. Pseudomonas with this mutation apparently undergo a metabolic shift: consuming less oxygen while utilizing nitrate more efficiently. lasR mutant bacteria also can handle oxidative stress resulting from an imbalance of damaging substances called free radicals forming faster than they can be detoxified.

One source of oxidative stress encountered by Pseudomonas is the antibiotic treatment that is frequently given to people who have cystic fibrosis. Antibiotics like ciprofloxacin and tobramycin kill bacteria partly by inducing the overproduction of free radicals and causing oxidative stress. Hoffman and his team found that, because these mutant microbes are resistant to oxidative stress, they were relatively resistant to these antibiotics when grown in conditions that were like cystic fibrosis mucus.

“We learned that simply by adapting to the conditions inside the airways of cystic fibrosis patients, mutated Pseudomonas can withstand the effects of ciprofloxacin and tobramycin,” Hoffman said. They did not need any previous exposure to these antibiotics to reduce their susceptibility.

Hoffman and his team suspect that Pseudomonas is not the only microbe that can do this. Some of the characteristics conferred by the mutation in Pseudomonas are also exhibited in other microbes found in chronic lung infections, such as tuberculosis or the fungal pathogen, Cryptococcus neoformans, Hoffman noted. Metabolic shifts may be a way many microbes get the upper hand over their hosts — and over antibiotics.

This report, Hoffman said, may point to new ideas for treating chronic lung infections. Luckily, colonies of Pseudomonas with the lasR mutation are relatively easy to identify in hospital laboratories by their distinctive iridescent sheen. Because lasR mutant Pseudomonas has been associated with worse outcomes in cystic fibrosis patients, indentifying Pseudomonas with the lasR mutation may be of prognostic value and may indicate the need for treatment with specific antibiotics like monobactams, tetracyclines, or polymyxin, whose mode of action differs from ciprofloxacin and tobramycin. Other treatment methods may be targeted at preventing adaptive changes, such as the lasR mutation, in Pseudomonas, the researchers said.

View the full article here: http://www.bmedreport.com/archives/10015

First Time Finding the Baby in a Cake

As some of you know a couple of weeks ago a super sweet cyster out in the community decided to send me a King Cake so I could be a part of their Mardi Gras celebration. You can find the post about it here. Well, video has finally surfaced of us finding the famous baby in that cake. Let me just tell you, a bit creepy!!!

Thankful for New Drugs!!!

This Thankful Thursday is a pretty easy one! I'm so thankful for the new drug just approved by our FDA :) I've heard nothing but great things about this drug from fellow cysters and fibros and I really can't wait to try it out myself. Make sure to call your clinic ASAP to see if this is a drug that can help you and one that you can get on sooner rather than later.

The drug by the way is Cayston, also known as AZLI. Think of it as another TOBI type of drug that can be done during your off month of TOBI or can be done instead of TOBI all together. It has to be taken three times a day, but the great part is, the treatment takes 2 to 3 minutes!!!

Bring it on!

Gilead Sciences, Inc. today announced that the U.S. Food and Drug Administration (FDA) has granted marketing approval for Cayston(R) (aztreonam for inhalation solution) as a treatment to improve respiratory symptoms in cystic fibrosis (CF) patients with Pseudomonas aeruginosa (P. aeruginosa). Cayston's safety and efficacy have not been established in pediatric patients below the age of 7, patients with forced expiratory volume in one second (FEV1) of less than 25 percent or greater than 75 percent predicted, or patients colonized with Burkholderia cepacia.

Cayston is administered at a dose of 75 mg three times daily over a 28-day period and is delivered via the Altera(R) Nebulizer System, a portable, drug-specific delivery device using the eFlow(R) Technology Platform, developed by PARI Pharma GmbH. PARI Pharma also contributed to the development of Cayston's drug formulation for delivery with the Altera Nebulizer System. Cayston will be available in the United States by the end of next week through certain specialty pharmacies.

"All of us at Gilead extend our thanks to the investigators and to the people with cystic fibrosis who took part in the Cayston clinical trials," said Norbert Bischofberger, PhD, Gilead's Executive Vice President, Research and Development and Chief Scientific Officer. "We look forward to making Cayston available to the cystic fibrosis community as soon as possible."

CF is a chronic, debilitating genetic condition that affects the respiratory and digestive systems of approximately 70,000 people worldwide, including 30,000 people in the United States. Chronic respiratory tract infection with P. aeruginosa contributes to the decline in pulmonary function, which is often associated with morbidity and mortality among CF patients.

"Since its founding in the 1950s, the Cystic Fibrosis Foundation has worked to advance the care and treatment of cystic fibrosis and we are pleased with the progress to date," said Robert J. Beall, PhD, President and Chief Executive Officer, Cystic Fibrosis Foundation. "However, a significant need for new treatments remains for people with cystic fibrosis, particularly for those with chronic pseudomonal infection. As the first new inhaled antibiotic approved for use in cystic fibrosis in more than a decade, Cayston therefore represents an important therapeutic option in the care of patients with cystic fibrosis."

Cayston received conditional marketing authorizations in the European Union and Canada in September 2009 and was approved in Australia in January 2010. Applications for marketing approval of Cayston are currently pending in Switzerland and Turkey.

Reimbursement and Access to Care

Gilead also announced today the establishment of a program designed to minimize barriers to access for Cayston for uninsured, privately insured and government-insured people with cystic fibrosis.

Additionally, Gilead is launching the Cayston(R) Access Program, a call center developed with Cystic Fibrosis Foundation Pharmacy, LLC, a wholly owned subsidiary of the Cystic Fibrosis Foundation. The program will assist people with cystic fibrosis and members of their care team with insurance verification, referral to participating specialty pharmacies, claims support and co-pay assistance. For information about the Cayston Access Program, call 1-877-7CAYSTON (877-722-9786) or visit www.cayston.com.

About Cayston

Cayston (aztreonam for inhalation solution) 75 mg is an inhaled antibiotic for patients with cystic fibrosis who have P. aeruginosa. Aztreonam has potent in vitro activity against gram-negative aerobic pathogens including P. aeruginosa. Cayston contains aztreonam formulated with lysine, a proprietary formulation of aztreonam developed specifically for inhalation. Aztreonam formulated with arginine has previously been approved by FDA for intravenous administration.

Cayston is administered three times a day for a 28-day course, followed by 28 days off of Cayston therapy. Cayston is administered by inhalation and should only be used with an Altera Nebulizer System. Patients should use a bronchodilator before administration of Cayston.

Article from Business Wire. February 22, 2010. To read the full article please click here.

Top Ten Toys I Never Grew Out Of

(**Written by Mandi)

Sunday night Ronnie and I went to listen to live music with friends of ours to celebrate one of our good friend's birthdays. As a little birthday gift, Ronnie and I bought her a Giant Googly Ball (see the picture to the left) that lights up when you push a button inside. It was a huge hit. Even people that weren't there with us took their turn playing with it. That got us talking on the car ride home about classic toys we still loved as adults. Here's our list in no particular order:

10. Etch a Sketch - You all know this one well. Two knobs on the lower left and right hand sides under the screen. The toy that takes massive amounts of concentration to make good images on even at an old age.

9. Pin Art - What other toy can you spent hours with making your hand and face into metal art? I used to love seeing that I could create, and would find myself fascinated with just the mere feel of it.

8. Slinky - Who walks the stair without a care. It shoots so high in the sky. Bounce up and down just like a clown. Everyone knows its Slinky. The best present yet to give or get. The kids will all want to try. The hit of the day when you're ready to play. Everyone knows it's Slinky. It's Slinky, It’s Slinky for fun it's the best of the toys It's Slinky, It’s Slinky the favorite of girls and boys.

7. Yo Yo - Now, I never was good enough to compete in any competitions, and never attended any conventions. But I did enjoy them and mastered the art of walking the dog and around the world.

6. Lite Bright - Ah there's nothing like making images out of little neon light bulbs. There's no wonder this is a toy that's been around since the 60s!

5. Slap Bracelet - It's metal covered in fabric, it's a bracelet, it's dangerous and removed from the market, it's CLASSIC!

4. Magnadoodle - I think my love for this toy stems from a fascination that it actually works!

3. View Master - Like your own personal slide show, no electricity needed.

2. Rubiks Cube - I've never once been able to actually make all the sides the same color, so I've never actually been GOOD at this toy. But I can waste hours playing with it.

1. Magic 8 ball - Will I marry him? Will my hair look good blonde? Will I get an A on my math test? The answer to all life's questions can be found in the infinite wisdom of the Magic 8 ball.

What are your favorite toys that you just never grew out of. We all have them!

Pre-marriage Counseling by Rondi

Drug Improved Survival in Mice With Cystic Fibrosis

It may only be mice, but it's a start....

In the search for new treatments for cystic fibrosis, U.S. researchers have identified a defective signaling pathway that contributes to the severity of the inherited lung disease.

Cystic fibrosis causes thick, sticky mucus to build up in the lungs and digestive tract, and is one of the most common potentially lethal genetic diseases in children and young adults.

In the new study, the researchers found that correcting the defective signaling pathway for a protein called peroxisome proliferator-activated receptor-y (PPAR-y) reduced cystic fibrosis symptoms in mice.

"Cystic fibrosis results from a genetic mutation in a channel, or membrane pore, that facilitates the transport of chloride and bicarbonate electrolytes from inside the cell to the spaces outside the cell," lead investigator Dr. Gregory Harmon, of the University of California, San Diego School of Medicine, said in a news release from the school.

"Loss of the cystic fibrosis pore channel results in inflammation and mucus accumulation. It also results in dehydration of the cell surfaces that make up the lining spaces inside the lungs and other affected organs, such as the intestinal tract," he explained.

Working with cells from mice and human cell lines from cystic fibrosis patients, Harmon and his colleagues determined that multiple genes affected by PPAR-y were reduced in cystic fibrosis.

The researchers then treated mice with cystic fibrosis with the drug rosiglitazone (a drug that binds and activates PPAR-y) and found that gene expression was largely normalized and survival improved. Among the other findings:

• Drug treatment also corrected part of the inflammatory process associated with cystic fibrosis.
• Deleting PPAR-y in the intestine of mice worsened cystic fibrosis.
• Activating PPAR-y can increase bicarbonate production in intestinal tissue by increasing the activity of bicarbonate-producing enzymes called carbonic anhydrases.

"For the first time, we are able to use a drug that activates bicarbonate transport without affecting chloride transport, and see improvement in the disease," Harmon said.

The findings, published in the Feb. 14 issue of Nature Medicine, may lead to new treatments for cystic fibrosis.

Find original article at http://www.palmbeachpost.com/health/drug-improved-survival-in-mice-with-cystic-fibrosis-253691.html