CF Patient Registry

Click here to read an article that explains the "registry" of CF patients. I don't think I have ever talked about this before. I can remember the social worker telling us about this registry that they keep of all CF patients on our second visit to the CF clinic, right after he was diagnosed. I thought it was pretty cool that they keep track of everything since everyone is different and certain meds effect people differently. Plus it's good for knowing about possible clinical trials you are eligible for. They explain it much better, so just click the link above;) lol

We are so excited about Christmas this year!!! Only 2 more days!!! We gave Lil' Chris a gift early and he LOVED it!! Then he wanted more gifts! He kept saying..."More presents?? More presents??" It should be a lot of fun this year:) I bet he is going to tear into all of them in no time! This kid just puts a smile on my face EVERYDAY!!! With all that he goes thru, I don't know how he can be such a happy kid. I just love him SOOOOOO MUCH!!!

Praying for Phenny and Reilly who had to go to the hospital. Glad they can be home for Christmas:)

This is from Aidan's(a CFer in Kindergarten) mom on facebook.....Aidan's comment tonight totally out of the blue....."Christmas time is just so magical, mom. Wouldn't it be great if the magic took my CF away?" The one present he really wants and the one I can't give....yet....

If you want to get me or Lil' Chris a present for Christmas, you can donate to help find a CURE! Click here to donate:) Thank you sooooo much!!


Click here to see Santa's message to Lil' Chris:)
M
PS. My cold wasn't so bad and didn't last long, so thank you for all your prayers;-) Thankfully the kids never caught it:) YEA!! Gotta love hospital masks;)

400 Hours on the Vest/Important CF Stuff

Lil' Chris has reached 400 hours on his Vest! Crazy when you think that in the past 19 months, my son has sat still(or somewhat still;) for 400 hours while a vibrating vest shakes the mucus out of his lungs. Poor kid is only 2 1/2!!!! Looking at the pics, it's hard to believe that Lil' Chris was Ayla's age now when he first got his Vest!! I should dig up some of those pics on of these days:) This video was taken on Tues 12/15 when he reached 400 hours. At the end are a couple of pics and a video of him playing a balloon guitar he got from an elf at the mall...he LOVES guitars!!

Make photo slide shows at www.OneTrueMedia.com

Lil' Chris has a CF clinic appt on the 30th, so we are trying to get him to eat as much as possible to get his weight up, but it's hard when he refuses to eat anything;-( I can't wait until he grows out of this stage! I'll take any advice you may have for a very picky eater.

Both Lil' Chris and Ayla got their H1N1 booster shot last Wed. Ayla also got her Season flu booster shot too, then she had her regular check up...she weighs 21 lbs and 6 oz., and is 30 inches tall!!! My lil' piglet;-) She is in the 75th percentile for her weight and in the 90th percentile for her height!!! Our dr always laughs at me when my jaw drops every time I hear 90...I'm just not used to hearing 90, I don't think Lil' Chris was ever in the 90's for anything...I'll have to look at my records;-) I WISH I would hear 90 for Lil' Chris:) Someday, someday;)

Please say a prayer for Wesley's family. He passed away from Cystic Fibrosis yesterday and he was only 17. He got his wish...to know what it was like to breathe...he had new lungs for a year before he passed. Just breaks my heart. A double lung transplant isn't a cure. Click here to read Wesley's page.

Click here to read a very interesting article about how a CFer in the UK was saved by receiving "salvaged" lungs. Lungs that would have been unsuitable. This could be HUGE for CFers if this comes to the states!!!

Lastly, check out this new drug that could really help CFers as well...click here to read about aztreonam lysine for inhalation. The drug would offer a much-needed antibiotic alternative for cystic fibrosis patients who battle recurrent lung infections and often develop resistance to existing antibiotics. It is manufactured by Gilead and will fight against Pseudomonas Aeruginosa infections in the airways of Cystic Fibrosis patients. It has already been approved in both Europe & Australia under the name Cayston(R) and the US has been lagging behind in its approval. Our Cystic Fibrosis patients with Pseudomonas Aeruginosa are running out of antibiotic options to help fight infections in their lungs. Please email druginfo@fda.hhs.gov to help get this drug approved if you are a CFer or a caretaker of a CFer. Just send a short emial stating how much this could effect your life or child's life. Also copy the Commissioner of the FDA too margaret.hamburg@fda.hhs.gov and then bcc the CFF at publicpolicy@cff.org. Thanks so much for helping get this important drug approved as about 60% of people with Cystic Fibrosis have a chronic respiratory infection caused by the bacteria called Pseudomonas Aeruginosa that settles into the thick mucus trapped in the airways. Once it sets up house in the respiratory tract, Pseudomonas Aeruginosa is almost impossible to get rid of. Respiratory failure caused by this infection is often the ultimate cause of death in many people suffering from Cystic Fibrosis.

Until there is a CURE...
M
PS. I have to wear my hospital mask for the next few days, since I got a cold last night;-( Ughhhh!!! Please pray that the kids don't get sick too, esp Lil' Chris.

Updates on the Kiddos:)

Lil' Chris has been doing ok without his binky so far. We had a few rough times getting him to go down for a nap, but hopefully that will get better soon. What am I saying....it's been a NIGHTMARE!!!! Just kidding:) LOL Sometimes it can seem like that, but then other times he goes right down! He'll ask for his binky once, and then that's it!! He even did a GREAT job for the babysitter yesterday! He took a nap AND went to bed for her with hardly any problems! When he doesn't do good, he likes to play a game with us..."Potty! Potty!" Take him in there... "No potty." Put him back in bed..."Potty! Potty!" Take him in there..."No potty." He just keeps doing it over and over. He knows we are trying to potty train him, so I am hesitant to not put him on the potty when he says he has to go. This kid is just too smart!!! Any ideas for me before I go completely grey???? Or bald??? You know, before I pull all my hair out;-) LOL

In other news....he continues to do a GREAT job with potty training. We took the little potty out, so now he only uses the big potty. He still is not potty trained for nap and night time, but we'll get there eventually:)

I've been worried lately that Lil' Chris hasn't been eating enough. We have a CF clinic appt on the 30th and I don't want his weight to be down. We usually try to give him real food and then if he doesn't eat a lot, we give him some baby food. Well...I took him off of baby food for a few weeks and he lost THREE lbs!!! Food has always been a touchy subject when it came to Lil' Chris. We never tried to push or make him eat, b/c he will just gag and throw it up or we would be afraid of making eating time an unpleasant time. Eating is sooooo important for him that we didn't want to do that, but.....it has come to the point that nothing else is working, so we have no other choice but to be harder on him to eat the food that is put in front of him. If he won't eat it, we say "take 3 bites than you can have your yogurt." He always wants his Danimals or Go-Gurts. So far, we have been shocked that he will actually eat the 3 bites after a little bit of fighting. I figure that getting the food in his mouth will get him used to the taste and used to eating those kinds of foods. I'll keep you updated on how he progresses. Click here to see some weight gaining strategies if you need them too. Let me know if you have any ideas for me:)

Ayla has been crawling around EVERYWHERE!! She can even go side to side now and turn around:) She also can pull herself up on the couch so she is standing! Pretty soon she will be scooting herself around the couches:) Then she'll be walking and running ALL over!! She's so cute:) Oh yeah, she's been trying to climb the stairs!!! Won't be long now;)

She now says "night night" when she gets sleepy, claps when people clap on TV, claps when anyone says "Yea!", shakes her head "no" when someone says "no", shakes her head "yes" when someone says "yes", and is always standing up in her crib when we go in to get her;) She also has been eating so much more! She eats all the stage 3 foods and eats ALL the snacks that Lil' Chris eats! If I try to feed Lil' Chris something, she crawls over and starts whining until I give her some;-) Too funny! She eats it FASTER than him too! Even Chris and I can't eat something without her wanting some;-)

Ayla also likes to dance to music too;) Her brother definitely rubbed off on her;-)
M
PS. Do you like the new header pic??? More to come this month;-)

Miley Cyrus' tattoo says "Just Breathe"

Miley Cyrus got a tattoo that says..."Just Breathe" Yea! Finally some media about Cystic Fibrosis!! You go, Miley!!! Click these two links to read some articles about her and her new tattoo:)

http://www.examiner.com/x-11363-Dallas-TV-Examiner~y2009m12d6-Miley-Cyrus-tattoo-has-positive-meaning

http://www.buddytv.com/articles/hannah-montana/hannah-montana-why-miley-cyrus-33105.aspx

NO MORE BINKY!! YEA!!

Lil' Chris hit a HUGE milestone yesterday and last night! Yesterday, when I was putting him down for a nap I noticed that his binky was split down the middle! I thought..."YES! Perfect time to get rid of the binky... FINALLY!" So I explained to him that his binky is broken and he can't use it anymore. So we threw it in the trash and I told him that he is a big boy and can sleep without it. To my surprise....HE FELL RIGHT TO SLEEP!! No fussing or crying! WOW!!! Then he had a really good long nap too!!

When I put him down for the night, I thought he was NEVER going to go to sleep without his binky. He has relied on a binky from day 1 practically(see his special ducky binky above;). I think it was when he turned 1 when we only let him have it at night time and nap time... oh yeah and he gets it in the car too(keeps him quiet and helps him sleep;)

So when I put him down for bed last night, I was expecting it to take a REALLY long time, esp when he asked for his binky right as I laid him down. He cried for a little, but after 15-20 minutes of playing games with me...HE FELL ASLEEP! I couldn't believe it!! I thought for sure that he was going to wake up in the middle of the night crying for his binky like he used to when it would fall out of the crib, but NOPE! HE SLEPT THE WHOLE NIGHT THRU!! What a good boy;-)

When we went to go get our Santa pics today, I forgot to get the binky out of the car. As soon as he saw it, he popped it in his mouth and grabbed the top of his shirt like he always does;) He got me that time, but it soon will magically disappear or get a slit in it;-) ;-)

M

PS. Did you notice my new header pic? I'll change it to the Santa pic soon:) Plus I have lots more I'm going to change it to, so keep a look out;-)

Ayla Saying "Uh oh" and Standing Up Now:)

Photo and video editing at www.OneTrueMedia.com

On Thanksgiving, I was feeding Ayla and tried to get her to say "Uh oh" and she said it right away practically!! I couldn't believe it!! So now she can say "Uh oh", "Dadda", and "Momma"!!! YEA!!!

I just had to add a few pics of my handsome lil' man imitating his Daddy by putting his hands in his pockets...too cute:)

We had the best Thanksgiving with my sister-in-law, her hubby, and their 3 kiddos. We celebrated our Christmas too, since we won't see each other at that time. The kids had a blast opening their gifts and playing with them all weekend:) Thanks Steph and Greg and kids for a great Thanksgiving!!!

Here are some pics of our Thanksgiving Christmas;) and one of Ayla pulling herself up to a standing position!! This morning when my hubby went to get her out of her crib, he said she was standing up holding on!! When I went to get her from her nap, there she was...STANDING!! Wow! She catches on pretty quick:) Wasn't it like last week when she started sitting up by herself and crawling?!? She started crawling only 5 days ago, to be exact;-) Before we know it, she is going to be walking!!

Make sure you watch Ayla in these video's...you can see her eye up the presents and her brothers toys;) Oh yeah, and you can see the first video of her CRAWLING!!! YEA!! Hey Tyler...Ayla says "Thank you for her first Christmas present EVER!!" :)

Make an on-line slide show at www.OneTrueMedia.com

Lil' Chris caught a lil' bug the night of Thanksgiving and was up in the middle of the night with a lil' fever and threw up twice. By Saturday he was fine though. Right now he just has a lil' stuffy nose, but it isn't running. Pray nothing worse comes of it!

I hope everyone had a GREAT Thanksgiving like us!!!
M
PS. We got our Christmas pics taken, so be watching for a few new blog header pics this month:)

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!!