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!!!

CF Clinic Visit / Pulmozyme / 3rd PFT

Lil Chris had his first quarterly visit of the year with the CF clinic today at 4 1/2 years old. His weight went up from 40 lbs to 41.2 lbs, 60th percentile. YEA!! His height was up from 42 inches to 44.5 inches, 90th percentile!! His BMI on the other hand went from 30th to 20th percentile. BOOOOO. His doctor asked me if we have a lot of tall people in our family. LOL! She said that his BMI is so low b/c he is getting very tall quickly. She didn't seemed too worried about it even though they like CFers to be above the 50th percentile.

I told her how he has been eating a few more things since last time....jelly sandwiches, and cheese & mayo sandwiches, and I told her how he likes chicken nuggets and he even ate 10 one day for lunch!!!

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She said that his lungs sounded clear and everything looked good and we wouldn't change any meds. I asked her if he should stop the Bactrim in a couple of days when it is done, or if he will need more? I told her he isn't coughing much, but when he does cough, it is a little wet sounding still. She said to stop it when its done. He didn't cough the whole time we were there until right at the end. She heard that little wet sound and said lets do a PFT test and then she would come back and make a plan of attack. She said she would like him to not have any cough at all and he shouldn't have a chronic cough yet.

He did great on his PFTs, but they were a little bit lower than last time. The girl said it was probably b/c he is just getting over a cough. Last time his FVC was 94 and his FEV1 was 94...this time it was 89 and 96. One went down and one went up.

FVC - Forced Vital Capacity - after the patient has taken in the deepest possible breath, this is the volume of air which can be forcibly and maximally exhaled out of the lungs until no more can be expired.

FEV1 - Forced Expiratory Volume in One Second - this is the volume of air which can be forcibly exhaled from the lungs in the first second of a forced expiratory manuever.

When we came back in our room, I thought we were going to get to talk with our dr about a plan of attack, but we'll call her "nurse P", the nurse was there waiting for us with a bag. My first thought was OH NO!! She said that our dr thinks we should start Pulmozyne daily before his vest in the morning(I think Nurse P had some influence on this decision). This is a mucus thinner. It will help thin his mucus so that he can cough it up easier and spit it out. This "may" get rid of his cough, but it also may not be right for him. We would have to try it and see. It is about a 10 min neb, so we would have to get a nebulizer machine. This would be his first nebulizer medicine!! It's supposed to be done before his vest, but he can do it during his vest she said if we are running late.

Nurse P suggested in the beginning of our visit that he should do his Albuterol and Acapella every day instead of only when he has a cough. That he should get used to doing it now while he is young, since he will have to do it every day when he is older. If you look at past posts, you'll see that Nurse P is the one and only nurse that has always said he should be on a neb. I'm sorry, but if our dr's never thought he needed it then.....

So I was trying to ask her some questions about this Pulmozyme and she was telling me about it and how it will have to be sterilized each day and everything....but she wasn't exactly saying how long he would have to do this Pulmozyme. I kinda knew deep down from knowing so many CFers, but then she said that this would be "FOR THE REST OF HIS LIFE!!"

Just hearing those words again was a shot through the heart. We first heard them on the day of his diagnosis..."he will have Cystic Fibrosis FOR THE REST OF HIS LIFE"...."he will do his Vest FOR THE REST OF HIS LIFE"....and now "he will do this Pulmozyme, Albuterol, and Acapella FOR THE REST OF HIS LIFE???"

So today's clinic visit was a little frustrating for many reasons to say the least...

1. When we got there, we had to wait in line....yes, I said wait in line with other CFers....just to check in. 3 or 4 other kids like Lil' Chris, all wearing masks and within a few feet from each other!!! I was FREAKING OUT inside!! I let the girl know when it was finally our turn!! She said something about it being busy, and I said "Yeah, it's making me VERY nervous all of these CFers so close!" She got the hint and moved us along quickly.
2. Nurse P suggested doing Albuterol and the Acapella EVERY DAY....sorry, but I'd rather a dr tell me something like that!
3. The dr said she was coming back in our room after PFT's and she never did!
4. The nurse has to tell me that he will have to do this nebulizer FOR THE REST OF HIS LIFE!! Again....I'd rather hear something like that from a DOCTOR!!! Just makes me mad!!
   

I don't mind doing this neb if it's really going to help him, but if he doesn't really need it, then I'd rather not start him on it until he needs it.We do have to worry about him becoming immune to certain meds, as he may need them more when he is older.

Some CF Questions...

1. Can Pulmozyme be used just for coughs or does it have to be used long term?
2. Is it something that he would have to be weaned off of once his cough goes away?
3. Can he become immune to Pulmozyme after a while? It seems like just about every CFer I know, young & old, do Pulmozyme every day. I need to do some research!
   

So, I was told to go home and talk it over with my husband and decide whether we want to go ahead and do it every day for the rest of his life, try it for 30 days, or not try it at all. It's up to us. GREAT!!! I talked to my husband and he said he doesn't think he needs it yet and to just wait and see how he is when his Bactrim is done. He said that his cough usually isn't all gone until right at the end of the antibiotic anyways.

I miss our old dr. He used to put Lil' Chris on an antibiotic for 30 days and his cough would be completely gone by the end of it. Ever since we got this dr, she only wants him on antibiotics for 15 days...his cough isn't gone in 15 days!!!! So frustrating, b/c I've tried telling her this, but she doesn't want him on it longer than 15 days. I guess b/c you run the risk of becoming immune to it sooner...idk??? It just seems like he hasn't completely gotten rid of his cough in a very long time. As a CF parent you just feel so defeated...have we made the right choices in the past...are we going to make the right choice now???

All I can think of is to "empty my hands" and leave it up to God. I'm going to pray about it and follow God's lead as to what to do. Please pray with us for God's guidance and strength. Deuteronomy 20:4 "For the Lord your God is the one who goes with you to fight for you against your enemies to give you victory." Cystic Fibrosis is our enemy....go get CF God!! :)

On a good note, Lil' Chris did such a GREAT JOB at clinic today!! He did so good during his throat culture that the nurse said that he should teach all the other kids to do it as good as him!! Nurse P asked if maybe we could get a video of him doing his Acapella and PFT's to show the other kids who don't even do it at this age!

In the 2 hours we were there, the kids were really good between watching TV and playing with the iPad, that I just had to take them to Chuck E Cheese! I didn't really feel like going after we got hit with the Pulmozyme news, but I knew I had to be strong for the kids and not let them see me get upset. So I let them play and I vented to my sister D on the phone:) Thanks D for always being there for me!! I'm trying not to talk negatively about it near Lil' Chris, b/c his lil' ears hear everything, if you know what I mean;)

I'll let you know what his throat culture results are when I call in a couple of weeks. Please pray that there are no new bad bacterias.

Sorry for venting on here, but sometimes I just gotta let it out!! I know Pulmozyme isn't the worst thing. It could definitely be worse!! Lil' Chris has been soooo blessed that he has made it 4 1/2 years with no nebulizer!!! It's almost unheard of in the CF world!! Some of my CF friends probably think I'm crazy complaining over just a neb, but it's just the "FOR THE REST OF HIS LIFE" thing that gets me. I'm sure every CFer and CF parent feels defeated every time they hear those words. WE NEED A CURE NOW!!!!!!! Time to start fundraising!! I need to clear my head of all of this and focus on fundraising, so that no CF family will feel this way ever again!!!!

Thanks,
M

SURPRISE!!!!!

Here it is, our big announcement to the kids...

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We surprised the kids with a trip to DISNEYWORLD!!!! WOOHOO!!! It was soooooo much fun!!! We ALL had a BLAST!!!! It truly is a magical place:)

Don't worry, the video above is not the only video I got! More pics and video coming soon:)

I couldn't believe how tall the kids have gotten since last May when we went!! Lil' Chris is 44 inches and Ayla is 38 inches!! They got to go on extra rides this year!! Lil' Chris got to go on his first really BIG roller coaster!! I thought he was going to be scared(I was), but he LOVED it!! He went 2 times with Daddy and 2 times with me:) I couldn't help but watch his face the whole time laughing and giggling, even though I was afraid of hurting my neck on the twist and turns;-)

So get this...I got the letter from the CF clinic like last time so that we wouldn't have to wait in the hot lines b/c he has a fatal lung disease....and wouldn't you know...when we got to the park the first day, I had forgotten it at our hotel room!!! UGHHH! So I went to talk to them to see if I could get that pass at any park and the girl said "What did you need?" At first I didn't know what she meant. Then I said, "Well, last year they gave us a pass for the rides and also one for our stroller." She said "How many are in your party?" I said 4. So she wrote them up and gave them to me!! I was SHOCKED!!! I didn't even have the paper or nothing!! I was soooo happy tho, b/c we ended up going on a lot of rides that night. We got to go on a lot of rides EVERYDAY thanks to that pass!!! We would just get right on!! We even got to skip the lines for the characters autographs too!!! Although, I usually felt bad doing those, so we would only do it on the really long line ones, like for Mickey:) It was AWESOME!! The one and ONLY good thing about Cystic Fibrosis! I'm just so happy he was healthy enough to go again!!

I was a little worried about what the weather was going to be like, but the Lord blessed us with an absolutely beautiful week!!! It was in the 80's the whole time and no rain until we were driving home, then it rained a little on our drive!! Usually when we go to Disney, it is soooo hot that I have to use a fan/water squirter, but I didn't have to use it at all this time!! We even went to Blizzard Beach Waterpark the last 2 days and didn't have to worry about burning our feet on the hot concrete!! Both kids went down water slides that I was even leery about going on at first;-) LOL

Unfortunately Lil' Chris developed a cough on the second day. I think it was b/c we went swimming in our All Star Sports resorts heated pool late at night and then when we got out, it was a little chilly. I guess too much for his lil' lungs, IDK. Anyway, that night he woke up at about 2 in the morning with that weird barking cough that he had once before. He coughed for a lil' while and wanted to lay with me. Luckily I brought his inhaler and Acappella. He eventually calmed down and fell back to sleep. His cough didn't sound as bad when he woke up, but he did have a lil' cough the rest of the week, and by the end it turned into a wet nasty cough. Monday 1/30, when we got back home, the first thing I did was call the CF clinic. They got him on an antibiotic right away, since he cultured a new bacteria last time. He cultured his usual Staph Areus(MSSA), and then Stenotrophomonas (Xanthomonas) maltophilia, which is new, but they said does not need treatment unless he is ill. They said that this is a common bacteria and may come and go in his cultures from time to time.

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The CF nurse doesn't think it was b/c of the pool, but who knows?? He could've caught something at the park or at a rest stop on the long drive. Going to Disney is always a risk we take. I was especially nervous this time, b/c we went in cold and flu season. I guess in a way we are lucky he only got a cough and not the flu.

So he is taking Bactrim 2 times a day, 12.5 ML each time...that's the most I think he has ever taken!! Please pray his cough goes away completely, it's already starting to get better! He has a CF clinic appt on the 15th, so I'm praying it will be gone by then and his new throat culture won't have any new bacterias. Stinks he will miss his preschool Valentine's party;-( I was so sad when I noticed that, b/c that was my favorite day of the year growing up and I got all excited he was going to have one this year. Oh well, it's not like he doesn't have 12 more years to go, right?

My sister D and bro-in-law were nice enough to let us stay at their house on the way to Disney and back to break up the looooong drive. It was soooo nice spending time with them!! I really miss my big sis! I had a lot of fun painting nails with my nieces too. They are getting so big!! Lil' Chris had fun playing new video games with his cousin:)

On Sat 1/28, we celebrated Ayla's 3rd birthday!!! YEA!!! It was so nice celebrating with my family:) Ayla loved all her princess presents, esp the castle that Aunt D got her;) Pics/video coming soon, I have to finish editing it:) The story of my life!! LOL I noticed I have a ton of video's that I uploaded but never posted b/c I never finished editing them. Hmmmm I wonder if I can edit them on my iPad??? I'll have to try it.

Oh yeah, not sure if I mentioned it on here...I won an iPad2 at work!!!! I still can't believe I won it, I never win anything!! I'm soooo very thankful for it tho, b/c Lil' Chris loves playing with it while he does his Vest....I love it b/c it keeps him quiet during his Vest;-) LOL Plus it's a good incentive for him not to complain about putting his Vest on..."If you put your Vest on, you can play the iPad!" He usually runs right over;-) Sooooo nice!

On another good note about work, I was accepted into the Advancement into Management training!! I'm already part of the management team, but on the low end. This will make me an Executive...an Assistant Store Manager:) I've already completed my training and am just waiting for a position to open up!! I'm REALLY hoping and praying that I get to stay at the same store!!! I really don't want to be too far away from Lil' Chris if anything were to happen with his breathing. Also, IF he were to ever get hospitalized, I may still be able to work since it's only like 5 min away. Please pray with me that I get to stay at my current store :)

Thanks,
M
PS. Ayla developed a stuffy nose last week, but so far no one else has caught it...maybe allergies or something b/c it's not bad at all???? Probably something to do with this crazy warm winter we are having! Although....it IS SNOWING NOW!!! YEA!!!