Our old CF Commercial that plays on MCTV. Still working on this years.

Wednesday, February 15, 2012

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!!!

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!!!!



Victoria's Mom said...

That does stink. And yeah cleaning nebs everyday just sucks. But cfservicespharmacy has a great page on it. https://www.cfservicespharmacy.com/CysticFibrosisCleaningYourNebulizer/

However, I would certainly ask the doctor about going with Hypertonic Saline rather than Pulmozyme. Similar effect with different methods of achieving it. HTS is cheaper, doesn't have to be refrigerated (at least in the single dose ampules), & more natural. Though I don't know if Pulmozyme has been studied for kids under 6 - though many take it anyway. I'm pretty sure HTS is still 'off label' for anyone under 6.

I've been happy with HTS since we started in July. It works for Victoria.

Adding the albuterol first, all the time is to open the airways so the pulmozyme or whatever will work better / get in deeper.

I hope his cough resolves soon.

Next time though, you could tell the nurse that 'Dr. X said he was coming back in after the PFTs. Would you let him know we're back? I'd like to speak to him'. Then you just take up the exam room until the doctor comes back to talk to you. ;-)

Lil' Chris' Mom said...

Thanks for the advice! I'll def be asking about the saline!

The Liberto Family said...

I know how you feel! First of all wow Chris is already doing PFT's? Melissa's doctor said once she turns 5 then she can start them.
Melissa started Pulmozyme back in September when she had that bad cough and ended up in the hospital. I thought once she was over her cough she would be done with Pulmozyme, but nope they said it something she would take for ever. She now does her xopenex and pulmozyme with vest and every other month tobi. I hate to see her on all of this, but I know it's for her lungs. Did they tell you about the copay program for the pulmozyme?
Wow Chris is tall I can tell in the video and pictures. Melissa has her check up in a week. She is getting taller also. We love you all.

Liberto Family