CF Update-Cold and Cough:(

Last weekend, we all got a cold, and now we all have a cough:( Poor Lil' Chris coughed his head off all day yesterday. A wet/dry cough. We tried everything...Albuterol puffer, Mucinex, Pulmozyme, Vest, Acapella, manual CPT, Vicks Vapor rub on his chest and feet to help him sleep last night(thanks Nancy for the reminder, I totally forgot we had that:). I felt so bad, he just couldn't stop coughing. At one point I started counting in between coughs to see how high I could count...I only got to 17 seconds:(

Luckily, he finally fell asleep last night and was able to sleep through almost the whole night. Of course, he woke up coughing again though, not as often, but still a lot:( I know the CF clinic won't give him an antibiotic unless he's been coughing for a few days to a week to see if it will go away on it's own, b/c they don't want him to become immune to the antibiotics. So, if he's still coughing by Monday, I'm calling to get an antibiotic. Hopefully his cough will be gone or hopefully the clinic will be good and prescribe an antibiotic ;p

Normally when he gets a bad cough they tell us to up his Vest from 2 times a day to 3-4 times a day...kinda hard when he is in school from 9-3:30. I know some parents who take the Vest to school and do treatments there during lunch or something, but I don't know how he would respond to his class mates seeing him do his Vest. So we will just have to do Vest before and right after school and then right before bed. So that's 1 1/2 hours each day, poor kid. Luckily he has been in good spirits. He goes along playing and laughing like he's not even coughing! I don't know how he does it!! I was coughing a lot at work and it gave me a headache and I felt miserable:( Ayla was the first one with a bad cough and she was put on Azithromycin for 5 days.

Please pray that his cough goes away soon and doesn't do any permanent damage to his lungs. Just yesterday the FDA approved expanded use of Kalydeco for 8 more mutations!! None of them are his mutations, but...this means they are getting closer and closer:) WOOHOO!!! We just need to keep his lungs as healthy as possible until they get it approved for him!!! http://www.cff.org/aboutCFFoundation/NewsEvents/2-21-FDA-Approves-Expanded-Use-of-Kalydeco-for-CF.cfm

I also just got some GREAT news from a fellow CF mom which totally made my day!!! So, despite everything that's going on with Lil' Chris' lungs right now, I have so much HOPE for his future:)

Thanks everyone,
M

Pulmozyme Decision / New Prevacid Capsule

After much thought, research, and prayer, our decision is in about the Pulmozyme....kinda;) LOL

This past Monday, I had to call the CF clinic b/c they left me a voicemail that he could no longer get the Solutab Prevacid that he has been on for years. So, now he is on the capsule Prevacid. They are just like his enzymes except bigger and a different color. He takes one 15ml capsule each morning. He's such a trooper that he can take all 3 enzymes AND prevacid all at once with a drink!!!! If you look in the pic below, you can see the aqua color capsule next to his enzymes. I took this pic for his 2012 Great Strides Video(see at top of blog:) Won't you please donate today?? :)

Ok, back to that phone call... when I called the CF clinic, I asked for my fav nurse and I was sooooo glad she was there!!! After we got the Prevacid thing settled, I started asking her about the Pulmozyme. She told me all about it and gave me a website to look up with videos. Its just Pulmozyme.com. I looked it up and right away was very encouraged b/c I saw 3 fellow adult CFers that I knew...Ronnie and his wife Mandi Sharp from Run Sick Boy Run CF blog and who founded CysticLife.org...Emily Schaller the founder of Let's Rock CF and the "You Know" You Tube videos which are Ellen Degeneres-like shows about CF(they are AWESOME), and Isabel Stenzel Byrnes who has a twin sister with CF too and they wrote a book about CF called "The Power of Two" I can't wait to get my hands on it and read it, b/c those girls are Amazing!!! Also, Isabel told me that her movie should be coming to OH soon!!! YEA!!! So, needless to say, when I saw the CFers that I knew, I felt very comforted!! When I started watching the videos, I realized that I had already watched them before at some point! I watch lots of videos that people post, just in case I will need that info for Lil' Chris. I especially liked the "How Pulmozyme Works" video.

Alright, back to my call again...I WILL get to our decision eventually;-) LOL As I was talking to my fav nurse, she was telling me that Pulomozyme is more like a preventative medicine. It will help keep his mucus thinner so he can cough it up and out easier. She mentioned that Pulmozyme has been around for 15-16 years and it usually increases the FEV1. She went on to tell me that they usually start this around 5 years old and are on it for life. Now, I always knew that more treatments would eventually get added, b/c I know so many CFers around the world, esp adult CFers that do 3-4 hour treatments every morning and night...but I guess I was thinking it would happen later on since he is doing so well, not in just a few months!! YIKES!!! She said it's not very effective if it is used just when he has a cough. They have seen better results in long term use. This med may prevent a lung infection, a hospital stay or many, and will hopefully help his cough get better b/c it will make him cough more to get it out.

I asked her about HTS or Hypertonic Saline which is very similar and I know a lot of CFers on it, some even do both Pulmozyme and HTS! HTS has only been around for the last 5-6 years. It does not have to be refrigerated like Pulmozyme does, and it is more natural. HTS is also cheaper. It draws more water into the airways and makes it easier to cough out the mucus. Pulmozyme is approved my FDA and works to thin mucus so people can cough it out easier. Fortunately, it looks like our insurances will cover it...THANK YOU LORD, b/c Pulmozyme is $2,000 for a 30 day supply!!!

CFF.org has some great info on both, so click these links or any of the color links above to learn more...
Pulmozyme-http://www.cff.org/treatments/Therapies/Respiratory/Pulmozyme/
HTS-http://www.cff.org/treatments/Therapies/Respiratory/HypertonicSaline/

So, as I was talking to the nurse, I was telling her about my rough experience at my last visit. (BTW, thank to my fellow CF moms who helped me get through that rough time by sharing your stories with me:) She apologized and offered to get a second opinion from another doctor, who is the director of our CF clinic as well. He suggested one more week of Bactrim to see if his cough will clear up completely(he has one more day left and he isn't coughing, but when I ask him to cough, you can still hear that it is a little bit wet). He also didn't think that Lil' Chris needed to go on the Pulmozyme right away. He suggested that we do some research on it, and come back to our next visit in 3 months with all the questions we have about Pulmozyme and HTS and ask our doctor. His next visit is on May 16th and I've already got a list started...I LOVE the list app on my phone;)

Since talking to this nice nurse, it's seems as though if we want to keep Lil' Chris "healthy", then we are going to have to start one of these chronic medications soon. He will continue his Vest as usual and will do this new treatment either before or during his Vest either in the morning or at night. I can't believe he has been on his Vest for over 1,100 HOURS!!! My husband and I have a BIG decision as to which one to go with. I'm kinda thinking Pulmozyme, and Chris is kinda thinking HTS. Which one is better in the long run...that is the big question!?!?

Chris and I were trying to decide whether to start him on it in May, or wait til his next appointment in August, when he'll be 5 years old, since it has only been tested in 5 year olds and up. Actually, the CF Foundation recommends it in 6 year olds and up, but if we wait too long, he could possible get irreversible lung damage....we DEF don't want that!!! I say we start it in May, that way he will have a couple of months to get used to it and get into a routine before he starts Kindergarten in August....YIKES KINDERGARTEN!!!! That's a whole nother story;-) LOL

Although it is disheartening to add one more chronic treatment to his daily life at such a young age....this is NOT the end!!! There are new drugs in the pipeline that are tackling the basic defect of CF. I've talked about them before, but we are getting closer and closer now! Kalydeco (previously known as VX-770) is a new oral medication that was approved by the FDA on January 31, 2012 for people with CF ages 6 and older with the G551D mutation of CF. It is the first drug available that targets the underlying cause of CF – a faulty gene and its protein product, CFTR. Although this won't help Lil' Chris yet, b/c his CF genes are Double Delta F508, doesn't mean that there is no hope. They are doing test now on DDF508 patients to figure out a way to get it to work for them too:) They say possibly within the next 5-10 years!!! THIS COULD BE THE CURE THAT WE HAVE BEEN PRAYING FOR AND RAISING MONEY FOR!!!!!

So what does this mean?? We need to keep praying and keep raising money so they can do their research and GET US THAT CURE!!!! This past weekend was my birthday weekend and I decided that the best present possible, would be donations for A CURE for Lil' Chris and all with CF!! So, many many many thanks to Nancy & Al, my Aunt Mar, my Dad, Mandi W, and Melissa V for all donating in honor of my birthday and it truly was my BEST birthday EVER...not b/c we went skiing or went to see Monster Jam(that was pretty cool seeing the big trucks up close tho;)...it was the $440 donated by you guys that is going to help save my son's life some day!!!!!!

So PLEASE CONSIDER DONATING TODAY EVERY SECOND COUNTS...click this link http://www.cff.org/Great_Strides/LilChrisChris6765

Thanks for being so patient with me in our decision on Pulmozyme and keep praying for a CURE!
M
PS. His throat culture results was Staph Areus sensitive to Oxycillin(MSSA) his usual, so YEA FOR NO NEW BAD BACTERIAS!!!! PRAISE THE LORD!!!

900 Hours on Vest / Wet Cough / Clinical Study on DF508

When we were at Disney, Lil' Chris reached the 900 hour mark on his Vest!! Way to Go Lil' Buddy!!! Also....he hit a milestone thanks to his cousin R! He can now unbuckle his Vest all by himself!!! YEA!!! Now he doesn't have to undo the velcro anymore and make it so it doesn't stick. Anyone with this Vest would know what I mean;-) lol

When we came back from our Disney trip, of course we all got sick ;( It was actually the day after Memorial Day. Big Chris, Ayla, and myself all caught a cold and had runny noses. Ayla had a cough too. Lil' Chris woke up that morning at 6am throwing up until about 10:30am off and on. We don't think he had any soy protein, but it seemed like it. Hopefully he's not allergic to something else too.

Anyway, he had a slight fever that day and just hung out on the couch for a couple of days(he's sleeping on the couch in the pic above...which NEVER usually happens), but no more throwing up. He didn't eat much either, but he would drink so that was good. After those couple of days he was fine!! He still has not caught our colds which is GREAT, but of course he woke up on his birthday with a weird sounding cough. Then he had a bad wet cough the whole car trip to Cedar Point. We almost turned around. Once we got to Cedar Point and he started going on the rides, he didn't cough much anymore:) Since then, he has been coughing off and on... still a wet cough. The last 2 mornings he has woken up early coughing a lot. Once he got up and moved around, he stopped coughing so much...just off and on again. I left a message on MyChart with the CF clinic letting them know that I would keep an eye on him this weekend and if it gets worse I'll call them on Monday. Meantime, we'll do more Vest treatments and Albuterol if he needs it. Please pray it gets better so he doesn't need an antibiotic. I'll keep you updated.

On a good note, there has been some more promising results in some of the clinical studies that could help Lil' Chris since he is a double Delta F508, the most common CF gene!! This could be HUGE for CFers!! Please pray this is the CURE or more CONTROL of CF we have been waiting for!! Click here for the whole article or read below....

Phase 2 Study of Two Potential CF Therapies — VX-770 and VX-809 — Shows Promising Results in Patients with Most Common Mutation

June 9, 2011

Vertex Pharmaceuticals Incorporated and the Cystic Fibrosis Foundation today announced promising results from an ongoing Phase 2 study evaluating combinations of VX-770 and VX-809, potential medicines designed to treat the defective protein that causes cystic fibrosis.

The study enrolled 62 people with two copies of the most common CF mutation, known as Delta F508. The trial lasted three weeks. Participants took VX-809 for two weeks, and VX-809 and VX-770 together for a third week.

Patients who took the drug regimen showed a positive change in sweat chloride levels. Excessive sweat chloride is a key clinical indicator of cystic fibrosis. The findings suggest that VX-809 and VX-770 together improve function of the defective CF protein, known as CFTR.

“The results of this study represent a milestone in our efforts to expand the use of small molecules to attack the root cause of cystic fibrosis in those with the most common defect,” said Robert J. Beall, Ph.D., president and CEO of the CF Foundation. “These data, while early, provide important new information that validates our approach and supports continued study of a combination-therapy approach to treating the basic defect of cystic fibrosis.”

The CF Foundation worked with Vertex to discover VX-770 and VX-809, and has provided substantial scientific, financial and clinical support throughout the development process, including an approximately $75 million investment.

These positive results support further testing of VX-770 and VX-809 as a combination therapy. Vertex plans to initiate the second part of this Phase 2 study in the fourth quarter of 2011.

In people with the Delta F508 mutation, the defective protein does not move to its proper place at the cell surface. VX-809 is designed to help the protein reach the cell surface, while VX-770 aims to help the protein function more normally once it is at the cell surface.

The defective protein creates a cascade of symptoms, including a buildup of mucus in the airways and other complications that lead to lung damage and ultimately premature death.

The CF Foundation has fueled dramatic improvements in research and care that have significantly changed the prognosis for people with CF. In the 1950s, children with CF usually died before reaching elementary school. Today, people with CF live into their 30s and beyond.

CF Fundraiser Tonight at CiCi's Pizza 5-8p!!!

Last years "A Cure 4 Lil' Chris" team...Thank You Everyone who attended and showed your support for Lil' Chris!!!

Join us tonight at Wooster's CiCi's Pizza between 5-8pm for "A Cure 4 Lil' Chris" and ALL who suffer from Cystic Fibrosis!!

A percentage of your receipt will go towards a CURE for Cystic Fibrosis and there will be a raffle for a FREE 1 hour massage at Tips, Toes, & Beyond!!

Save the following dates for my upcoming Wooster CiCi's Fundraisers for Cystic Fibrosis:

April 4th 5-8p

April 21st 5-8p

May 5th 5-8p

May 26th 5-8p

As always, you will see 5 Kohl's associates volunteering their time to help find "A Cure 4 Lil' Chris" and help us get a grant of $500 each event for the Cystic Fibrosis Foundation:)

Special thank you to tonight's Kohl's volunteers:

Me:)

Barb

Kayla

Melissa M

Rebecca G

Thank You So Much Girls!! It means the world to not only us, but ALL CFers!!!

If you can't make our fundraiser tonight but would like to donate, click here to make an online donation for

"A CURE 4 Lil' Chris!"

See you tonight at 5pm:)

M

CF Update / Positive Results for VX-770 Study!

Lil' Chris finished his Bactrim last night for his cough, but of course....he is STILL coughing!!! Arggggg!! So I called the CF clinic today and left a message. This is the first time this has happened, so I'm curious as to what they are going to do...maybe refill the Bactrim, or wait a little to see how he does, or wait and then put him on something different??? IDK. Do they even HAVE to wait?? I guess I'll find out when they call me back. Hopefully they call today!! His cough isn't super bad or all the time, but when he does cough...it doesn't sound the greatest. It just makes you sink and wonder what's going on in those lungs of his. I just pray it's not "the bad bacteria"...Pseudomonas.

Oh, and he also had another bad night on the 19th where he was throwing up again. We don't think he had any soy protein, so not sure why he keeps doing this. That makes 3 times in the last couple months I think. I wonder if we need to adjust his enzyme intake. I'll be asking the dr on 3/16 when we go for clinic visit. The pic above is him in between throwing up. Notice the trash can right next to him?? lol! If it's right next to him, he never gets it on the floor. He's pretty good about knowing when it's coming and when to get to the trash can. He's had enough practice, that's for sure. Poor kid.

Our snow FINALLY almost all melted a few days ago when we had like 60 degree weather, but that didn't last long!! We got hit again on Monday! Of course I had to drive home from work at the worst time!! But...I lived to tell about thankfully!! It was about 4-5 inches on top of 1 inch of ice and coming down hard! Cars were sliding all over the road, including me! Unfortunately, any way I go to my house there is a hill going down and up;( This time the roads were so bad that I started sliding down one hill sideways. I thought I was going to slide into the cop car at the bottom that was blocking the way coming up the hill I guess b/c cars were just sliding down it. Crazy, Crazy, but I made it home:) The Angels guided me the whole way!! Thank you Lord!! The pic above is the next day after my hubby snow plowed:) Thanks Honey!!

Now the following is some really encouraging news from the Cystic Fibrosis Foundation(what your donations for Great Strides fund). Lil' Chris' mutations are Double Delta F508(most common), but it's still promising for him...(click here for the whole article)...

Phase 3 Study of VX-770 Shows Positive Results

February 23, 2011

The Cystic Fibrosis Foundation and Vertex Pharmaceuticals announced today that VX-770, an oral medicine in development that targets the defective protein that causes cystic fibrosis, showed promising results in a Phase 3 clinical trial.

The trial was designed to evaluate patients age 12 and up who carry at least one copy of a CF mutation called G551D. The study included 161 patients who received at least one dose of VX-770 or placebo.

Patients who took the drug, compared to those on placebo, showed a marked improvement in lung function at 24 weeks, which was sustained for the duration of the 48-week trial.

Patients also showed improvement across all key secondary endpoints in the study, including reduced likelihood of experiencing a pulmonary exacerbation, decreased respiratory symptoms and improved weight gain. Each of these areas is critically important to the health of people with CF.

In addition, average sweat chloride levels of patients on VX-770 dropped toward normal levels, while those on placebo did not change — indicating the drug is impacting the underlying defect in CF. Excessive sweat chloride (salt) is a key clinical indicator of CF.

VX-770 is being developed by Vertex, and was discovered in collaboration with the CF Foundation, which provided substantial support to Vertex throughout the development process, including an approximately $75 million investment.

About four percent of people with CF carry the G551D mutation. More studies are needed to determine whether other CF mutations may benefit from VX-770.

“These results are highly encouraging. They provide scientific evidence that support our long-standing belief that targeting the underlying defect of CF may have a profound effect on the disease,” said Robert J. Beall, Ph.D., president and CEO of the Cystic Fibrosis Foundation. “We have much more to do to end the suffering caused by this disease, but these data are extremely exciting, especially for people with the G551D mutation and their families. The results also offer significant hope that a similar approach to treatment may help the majority of patients living with CF.”

The Phase 3 data support Vertex’s plan to submit a New Drug Application for VX-770 to the U.S. Food and Drug Administration (FDA) in the second half of 2011. Generally, the FDA takes between 6 and 12 months to review and rule on a drug application.

“The Cystic Fibrosis Foundation has played an instrumental role in our more than 10-year effort to discover and develop potential new CF medicines such as VX-770,” said Matthew W. Emmens, chairman, president and CEO of Vertex. “The data announced today reflect a significant investment of time, dollars and scientific expertise from both Vertex and the CF Foundation, and we look forward to working closely with the Foundation as we seek to bring VX-770 to people with CF.”

Said Preston W. Campbell III, M.D., executive vice president for medical affairs of the Cystic Fibrosis Foundation: “As a physician who has treated CF patients for many years, the VX-770 results are more than just numbers — they represent hope.” He added, “It’s not surprising that patients felt better on the drug because of the magnitude of lung function improvement and weight gain. These are important clinical outcomes, and the fact they were maintained through 48 weeks is very encouraging.”

Fundraising Award / New Vest and Hoses

Poster at the awards ceremony above...

Close up of Lil' Chris' pic on the poster:)...

New Pipeline chart...

The award I was given at the award ceremony:) YEA!!...

A few nights ago, Chris and I went to an Appreciation Ceremony on behalf of the Cystic Fibrosis Foundation. I was awarded for all my fundraising efforts and for raising the most money for the Wooster walk this past year!! YEA!! It's all thanks to YOU all who donated and to Kohl's for all the $500 grants for most of my fundraiser!!

So THANK YOU to:

• ALL my friends and family who donated
• ALL my awesome neighbors who donated, did a bake sale, and came to the walk:)


• ALL my Kohl's associates who helped out with all my fundraisers


• My boss, Bob, for letting me do Jeans 4 Genes days at work for 4 months and to ALL the associates who participated:)


• EVERYONE from the motorcycle run
  
• CiCi's Pizza for letting me do most of my fundraisers there AND for the 12 or 13 different CiCi's Pizza that did Pin-Ups for CF for the month of May.
• all the other restaurants I did a fundraiser at...Tumbleweed, Applebee's, & Friendly's.


• my MIL for setting up a walk in NJ for Lil' Chris and doing other fundraisers as well


• my sister, D, for setting up a walk in NC and doing fundraisers at her kids school and at a bouncy house.


• and ALL that made the walk a success!!

I couldn't have done it without you ALL!!!! CF WILL STAND FOR CURE FOUND SOON!!!!

In other news....I had to order ANOTHER set of hoses for the Vest!! I think that's the 5th or 6th time since we got the new blue Vest and it hasn't even been a year yet!! YIKES!! Hopefully they improve the new Vests soon. I also told them that the velcro on the shoulder straps are not sticking very well anymore too. They said to just order a new one and when it gets too bad, we can just change it. So we got the 2 new hoses and new blue outer shell the other day. I haven't washed the new shell yet, so here is a pic of what the old one was doing...(we had to keep the straps in the back instead of the front to make it tight, b/c it stuck better back there but would still pop off sometimes)....

Thanks again everybody for all that you do for Lil' Chris!! We love you all!!

M

Clinic Visit

MY BIG, BRAVE BOY....

Today was Lil' Chris' clinic visit. He goes every 3 months just like most CFers. This was the first time Daddy and Ayla didn't come with us;( Although we missed them, we still had a GREAT visit!! He wore his hospital mask from the parking lot to our room, and then in the hallway when he got his height and weight check, and then from the room to the car! What a BIG 3 year old, huh? He makes me more proud every time we go to clinic:) He did such a good job and was such a good boy the whole time, that I took him to Chuck E Cheeses as a reward:) He had a BLAST!! I LOVE watching him have fun and seeing his BIG smiles and hearing his cute lil' laughs:)

Here are some pics from our day....

Make an on-line slideshow at www.OneTrueMedia.com

His height and weight were good I guess, this new doctor didn't really say much. She didn't even tell me his percentages or BMI! I wish they would at least put it on the paper that they send us home with! Anyway, his weight last time(3 months ago) was 33 lbs and 2 ounces, this time it was 34 lbs and 5 ounces. So he DD have a gain...YEA!! Not sure how, since he doesn't eat much and hasn't been taking to the Scandishakes ever since the last time he was on an antibiotic! Last time his height was 38 1/2 and this time it was 39 1/4. Can't believe he is getting closer and closer to that 42 inches so he can go on the bigger rides;-) LOL! Next year will be so much fun!! :)

The doctor said that his lungs were CLEAR...YEA!!! I was a little worried, b/c he has a bad cough right now, but she did put him on another antibiotic;( I guess this is going to be the norm now...an antibiotic every other month;( That STINKS!!! But...at least he doesn't have to do any nebulizors yet...PRAISE THE LORD!! So he has to take 3.5 ml of Augmentin again for the next 14 days. I tried convincing her of doing the 21 days instead, to make sure the cough is gone b/c last time he was still coughing a little after the 14 days. She wouldn't go for it. I was told different doctors do different things. Our old dr. always did 21 days and this dr. always does 14 days, b/c the less time they are on antibiotics the better she says. I was told that to call at day 12 if he is still coughing and then she would prescribe another week. I guess we'll see how it goes and pray that his cough is gone by the 12th and doesn't turn into anything serious.

He got a throat culture done like usual and Lil' Chris did a GREAT JOB sitting on the table all by himself and he opened wide, stuck out his tongue, and said "Ahhhhh!" I was so proud of him!! The nurse got a great culture too...it had some yucky brown gunk on it. I've never seen that on his cultures before. I hope that isn't a bad sign. They told me they would let me know on Monday what the results are. They are going to tell me sooner than usual, b/c I told them that we are going to be around Li' Chris' cousin, Nathan who has Leukemia, next week. They said that if he cultures something bad, then he shouldn't go near Nathan, but if he cultures what he has been culturing lately, then he should be fine to go around Nathan as long as Nathan's white blood count isn't low. I asked if I could have some extra lil' kid masks just in case, so that the boys can at least see each other for a little bit. Please pray that Lil' Chris doesn't culture anything bad and that Nathan's white blood count is good and that Lil' Chris' cough goes away quickly.

A few weeks ago Lil' Chris started not wanting to take his chewable Vitamax. Vitamax is his CF vitamin. It consists mainly of vitamins A, D, E, and K. CFers bodies don't absorb these as much in the foods that they eat, so that is why they take some sort of this kind of supplement. I'm not sure why he suddenly started not wanting to take it, but I called the clinic and my favorite nurse sent us a couple of different liquid ones. He seemed to take to the Source CF one, so I told the doctor today and she gave us a prescription. It kinda stinks to go back to a liquid, but he NEEDS it.

Lastly, on our way out, we stopped by Kendall's room:) She is a fellow CFer about 15 months old in our area. I met her dad on Facebook and then I met her grandma in person at my Friendly's fundraiser earlier this year:) Today we got to meet Kendall herself, well almost, we got to see her for a minute and then they closed the door so they wouldn't share bacterias even though Lil' Chris was wearing his mask;) LOL! We did get to talk to her mom, Heather! She is super nice, they all are really great!! I wish our kids could have play dates, but CFer's shouldn't be within 3 feet of each other for risk of cross-contamination;( Maybe someday us parents can get together to chat though:)

Thanks everyone for checking in on Lil' Chris and for all your prayers. They are definitely working, so please keep them coming!!!
M

PS. Lil' Chris' cold seems to be gone...YEA!! Ayla's nose is still a little bit runny:(

Cayston is Available/Bad Sanitizers/Cold Update

Remember this post where I mentioned a new inhaled antibiotic getting approved by the FDA called Cayston??? Wellllllllll....IT'S AVAILABLE NOW!!! The CF Services Pharmacy has it! That's where Lil' Chris' meds all come from, b/c a percentage of each purchase goes towards a cure for Cystic Fibrosis:) Click here to see the whole article on the CFF website. Cayston is administered with a new device called the Altera® Nebulizer System that allows patients to take the medicine in less than FIVE minutes, a fraction of the time required for other inhaled antibiotics. It is the first new inhaled antibiotic developed for CF in more than a decade. I love this paragraph:) If only they could start making ALL of them to be administered in just 5 minutes...one can dream;) Although, it looks like it could possibly be a reality now:) All I can do is keep raising money and keep praying!! Thank you to everyone who has donated or raised money in the past to make this new drug be available for our precious CFers:)

How’s this for irony – the U.S. Food and Drug Administration is warning consumers to stop using two brands of hand sanitizers because they contain … dangerous bacteria.

That’s right. “Bee-Shield Hand Sanitizer” and “MD Quality Hand Sanitizer” (both made with aloe vera) were found to have high levels of Burkholderia cepacia, which “can cause serious infections in humans,” according to the FDA alert. Click here for the whole article. Kinda ironic isn't it? Sad...but ironic!

As for a quick update on every one's colds:

-Mine is great and was gone in practically 3 days.

-Ayla's nose has been slightly running still and it has been over a week now.

-Lil' Chris' nose never really ran like Ayla's and mine...it just kinda stayed clogged in his nose. Not sure if this is b/c of the CF and all the thick, sticky mucus or what???? Anybody else have this happen with their lil' CFer? Thankfully Lil' Chris doesn't mind blowing it out when we ask him and then he EVEN lets us suction it out, which is GREAT!!! BTW, I have to say a big THANK YOU to my sister, D. She told me before Lil' Chris was born to hold onto the lil' suction bulb that the hospital gives you when you have a baby. I am SOOOOO glad I listened to her, b/c the ones you buy in the store are CRAP! The hospital ones works like a charm every time;-) I even kept Ayla's too:) Thanks D!!!

-Lil' Chris also has a cough still too;( I think it might be from the drainage he might be having down the back of his throat, b/c the mucus is so thick. He has a CF clinic appt on the 31st, so hopefully it will be gone by then, if not, then hopefully we can get some answers. I'm kinda worried about this appt., b/c I think his weight is down again. He hasn't been eating as much lately.

Please pray Lil' Chris and Ayla get well soon!
M
PS. Guess what Ayla is doing now;)

VX-809 Promising Results in Phase 2a Clinical Trials

I LOVE seeing good news about CF!! Today it has been posted all over facebook, email, other blogs, and news, that the VX-809 phase 2a clinical trials look very promising!! Click here to read about it on the CFF website.

Here is the email from Bob Beall:

Dear Friend,
This week, we gained important new ground in our work to advance the development of new cystic fibrosis treatments.

VX-809, a potential CF drug developed by Vertex Pharmaceuticals, showed encouraging results in a preliminary Phase 2a clinical trial. VX-809 is one of the first investigational drugs aimed at treating the underlying causes of CF.

The oral therapy was tested in patient volunteers who have the most common cystic fibrosis mutation, Delta F508. (This is what Lil' Chris has)

Results from the trial showed VX-809 was well-tolerated and, in a subset of CF patients, reduced sweat chloride levels — a key indicator of CF.

VX-809 is the direct result of one of the Foundation’s largest investments in drug development. This gives us confidence that our innovative approach to science leads to progress.

Without your support, this milestone would simply not be possible. Your contributions help us build hope for the future and continue to advance vital cystic fibrosis research.

Thank you for your commitment to the fight against CF. Together, we are making a difference!

Sincerely,
Robert J. Beall, Ph.D.
President and CEO
Cystic Fibrosis Foundation
800-FIGHT-CF
info@cff.org
www.cff.org

Click here if you want to see my first post about VX-809. I think I put in this post that the VX-809 was a pill, but I'm pretty sure it is inhaled 3 or 4 times a day. The VX-770 is a pill.

They are going to do another clinical trial in the second half of 2010 and that will be combining the VX-809 and the VX-770 in patients with Delta F508 (Lil' Chris' genes) I believe. Keep praying that this is the CURE!! Keep fundraising or donating, b/c these clinical trials are not cheap!! The Cystic Fibrosis Foundation needs our help to keep the research going to get to a CURE that is so close right now!! Click this link to donate today...http://www.cff.org/Great_Strides/dsp_DonationPage.cfm?walkid=6765&idUser=244059

Thank you all so much for all the money you have donated over the past 2 years!! As you can see, your money is being put to good use...we are getting closer and closer to a CURE!!!
Thanks again,
M

VX-770 and VX-809=Potential CURE for Cystic Fibrosis!!!

Last Thursday 11/12/09, Chris and I went to a Cystic Fibrosis Volunteer Appreciation Reception. The special guest speaker was Robert Beall, the President and CEO of the Cystic Fibrosis Foundation! Exciting, right?!?! I know! It was such an honor to meet him and shake his hand. It is thanks to his dedication and the doctors and scientist hard work that my son's life expectancy is 37 instead of 5!! After the reception, I went up to him and asked to get a picture with him, and then we talked for a minute and I told him about my blog and how I talk to CFers all over the world! He seemed impressed for me only having a 2 year old with CF and doing so much already:)

He went over lots of good info, mainly including 2 drugs that are soon to be approved by the FDA and available to patients. These 2 drugs take care of the root of the problem in people with Cystic Fibrosis. They are called the Potentiator VX-770 and the Corrector VX-809. These drugs have the potential to add 10-15 years of life to patients with CF!!!!

The Potentiator VX-770 is in Phase 3, the Definitive Trial, and the Corrector VX-809 is in Phase 2, the Human Safety and Efficacy Trial. Drugs have to go through 3 phases which costs millions of dollars to do. Thanks to the money we raise through Great Strides, the Cystic Fibrosis Foundation can fund these drugs. Lil' Chris wouldn't be as healthy as he is today if it weren't for the CFF. Currently there are 30 drugs in the pipeline. Only 2 up until today have been approved, and I personally know many CFers that use TOBI. There are 13 drugs to be approved by the FDA within the next 3 years!!! Good news!! 4 of those drugs are in the last stage, phase 3. Great news!!

What are so special about the VX-770 and VX-809 drugs?? They could stop the disease at the cellular level. The basic defect in those with CF is that the chloride can't get out of the cell, CFTR. In a normal cell, proteins are transported to the outside of the cell, allowing chloride to flow in and out of the cell(see pic above). In a CF cell, nonworking proteins are transported to the outside of the cell and don't allow chloride to flow. Instead a thick, sticky mucus is formed. Robert Beall said, "Can't we just find a pill that will open up these chloride channels?" Well, the VX- 770 and the VX-809 do!!!

The VX-770 increases the opening(gating) of the CFTR channels with just a pill!! Right now is it working for those with G551D mutations, but Beall believes that in time it can work for other mutations as well. In a CF cell successfully treated with VX-770, the drug binds to the nonworking proteins on the outside of the cell, allowing chloride to flow and prevent mucus from forming. The VX-770 increased CFTR activity in Preclinical studies, and after a 2 week clinical trial, they were at normal levels!! Amazing! Their lung function improved 12%!! They are doing this study on children as young as 6 years of age and up! I didn't know they did these kinds of studies on children so young!

The VX-809 increases the number and function of CFTR channels. It is for those with double delta F508 mutations, which is what Lil' Chris has. The VX-809 helps CFTR to get to the membrane and helps get chloride through. These clinical trials are for those who are 18 and older. This one is very similar to the VX-770. Instead of increasing the opening so the chloride can flow, it makes a NEW opening to let the chloride flow. They do the same thing, but just in 2 different ways.

Robert Beall told us that "CF patients loose 2-3% of lung function EVERY YEAR. There is an 80% chance of drugs working when in phase 3. These drugs could add decades to CF patients lives! This is the most exciting time in our history, and we are closer than ever to treating the basic defect in CF!"

We need to pray, pray, pray that we raise enough money to keep these drugs moving forward to find the CURE for Cystic Fibrosis. These drugs could potentially be the CURE for Lil' Chris and other young CFers. It may not be the cure for older CFers, b/c it can't undo any lung damage that has already happened. But it can help it from getting any worse, which could still increase their life expectancy. Also, a lot less pills and treatments which is a HUGE PLUS!!! This all equals to a better quality of life:)

Other exciting news that he shared with us, is that they are teaming up with Warner's Brothers and CVS. CVS will promote movies that benefit Cystic Fibrosis! This will be huge awareness for the public! The more awareness there is about CF, the faster we will get to a CURE:)

Click here to read more about these drugs and to read about a fellow blogger I know, Somer Love, who is 30 with CF. Click here to read her blog.

Click here and donate today and do your part to help these drugs move a lil' faster to becoming Lil' Chris' CURE!

Click here to join a CF Panel if you are a CFer or a caregiver of a CFer and want to help find a cure for CF and get PAID to do it! Don't forget to mention my name;) Thank you to Traci Liberto for being the last one to mention my name:) A few days ago they called to update my info and I got $25! YEA!!! So far, I have earned $475 for basically doing nothing!! It's easy money to raise for Great Strides for a CURE;-)

With your help, CF will stand for CURE FOUND!!
M

Encouraging Email from Robert Beall/Great Strides for 2010

Here is another encouraging email I got from the President and CEO of the Cystic Fibrosis Foundation(we were fortunate enough to meet him last week at our Volunteer Appreciation meeting. I will blog about what I learned from him soon;)....

Dear Friend,

Thank you for your generous past support of the Cystic Fibrosis Foundation. With your help, we are making remarkable strides toward a cure.

Despite the economic uncertainty of the past 12 months, 2009 was a banner year in terms of scientific progress for people with CF.

We achieved important advancements in:

1. Clinical testing of breakthrough new therapies designed to treat the basic defect in CF



2. Development of therapies aimed at treating the symptoms of CF, including new antibiotics and anti-inflammatory drugs



3. Quality improvement at our care centers nationwide, through the Foundation’s award-winning quality improvement program.
   

Thanks to your help, the median age of survival has risen to more than 37 years, more than double what it was 25 years ago.

Our progress has been remarkable, but we still lose precious lives to this disease every day. With so much promising research on the horizon, we need your support now -- more than ever before.

Your tax-deductible donation will fund the life-saving research that is adding tomorrows every day.

Thank you for all that you do.

Sincerely,

Robert J. Beall, Ph.D.

President and CEO

Cystic Fibrosis Foundation

800-FIGHT-CF

Thank you all who donated the past 2 years in honor of Lil' Chris to help find a CURE for CF! As you can see, your money is being put to good use, but a CURE has not been found yet (although they did get a lot closer this past year;). I just found out that our walk for a CURE for CF for 2010 is going to be on May 1st at 9am. Please click here to sign up to walk with us, or to donate to FIND A CURE FOR CYSTIC FIBROSIS for Lil' Chris and ALL people with CF! We raised $4,440 in 2009, so my team goal for 2010 is $5,000 and my own personal goal is $4,000. Now who is going to be my first donor for 2010???? ;-)

Thanks,

M

PS. If anybody has any fundraising ideas for me, please let me know!!

CFF's View on the H1N1 Vaccine

Click here to get the Cystic Fibrosis Foundation's recommendations on the Seasonal Flu and H1N1 flu vaccines.

Thank you to those who took my poll on the upper right hand side of my blog here. I greatly appreciate it. It's nice to see what other CF families are doing about such an important issue for CFers. So far it looks like everyone who took the poll says that everyone in the CF household will be getting the H1N1 flu vaccine. Sounds good to me! I know both Lil' Chris and Ayla are for sure getting both vaccines. I can't get the seasonal flu shot, b/c I am allergic to eggs. Apparently if you are allergic to eggs, they recommend you don't get it.
M