Independence and Enzymes

E has to take pancreatic enzyme supplements, or "enzymes" every time she eats in order for her body to digest food properly.  When she was a baby, we used to open up the capsules and pour the beads out onto a spoonful of applesauce.  She was very good at taking them!  So when she was about 15 months old we decided to try putting the whole capsule in applesauce to see if she would take it.  And she did!  After awhile, she started trying to do it all by herself.  She would put the enzymes in her applesauce pouch, swallow them whole, and then say "I did it!"

Well, lately she is becoming a little more opinionated and wants to do things her own way.  She went on an enzyme strike for about 3 days last week.  There was no way to convince her to take her pills.  She would swat them out of my hand, throw them on the floor, and tell me she didn't want them.  It was a rough few days!  I called her CF Clinic to get some advice and the nurse told me to try giving them in something new.  We could put them in ranch, ketchup, yogurt, pudding...basically anything she would eat!  And it turns out, a little more variety is just what she needed.  It also helps if I just set everything down in front of her without saying anything.  She doesn't want us to TELL her to do it!  It has to be her idea.  What toddler wants to be told what to do?  

Clinic 12/12/13

Another wonderfully uneventful clinic visit!

E's weight was excellent.  She's up to 24 lbs now and had gained 2 lbs since the last time she was there.      She had been sick for about a week before the appointment, runny nose and coughing quite a bit, so they went ahead and started her on a 3-week round of Augmentin. Her bacterial cultures just grew normal flora, so nothing new there.  She had a blood draw to check her vitamin D level again but I don't have the results of that yet.  In 3 months we'll go back for what will hopefully be another uneventful visit!

Pulmozyme

E has been on Pulmozyme for 3 months now and I think it is making a huge difference!  Normally, she'll catch a cold or respiratory virus and it will take weeks for her to get over it.  But this last time, at the end of October, she was able to get rid of it faster than ever!

She's doing great!

Clinic 9/11/13

E had a great clinic visit this week!  This is the first time we've been to clinic since she's started walking so it was a whole new experience for her.  She wanted to check out everything in the room and see how it worked.  We had a pretty long wait once we got into our room so she kept peeking her head out the door and waving to the nurses making sure they didn't forget about us!

She gained some weight since her last visit and is almost 22 lbs now.  I have to say that I'm really happy weight gain hasn't been an issue for us so far.  I hear about so many other kids with CF that struggle with that and I can't even imagine how frustrating that would be.  But every child comes with their own set of struggles.  Out of curiosity, I asked how many rounds of antibiotics E had been on since she was born.  In her short 16 months of life she has had 7 rounds of antibiotics for various reasons and a two-week hospitalization for a pulmonary exacerbation where she received IV antibiotics through a PICC line.

By looking at her, you would never know it.  She is a cute little toddler always following her big sisters around the house.  She copies everything they do and everything they say.  She loves playing outside with sidewalk chalk and swinging or sliding on the play set.  She's a typical one year old...exploring and learning and taking it all in.  Sometimes I even forget she has CF, until I look at the clock and realize it's 8:00 pm and we still have to do her nightly treatments!  We have a pretty good system going right now, but as soon as we get used to a certain routine something changes and we add a new medicine or an extra treatment and we have to figure it out all over again.

Luckily we make a pretty good team!

Moraxella

We got the results of the bronchoscopy.  It showed that she had cultured a type of bacteria called moraxella, so we started treating it with an antibiotic (Suprax).  Moraxella isn't one of the scary ones in CF that cause a lot of problems, thank goodness.

She started Pulmozyme on Monday to help break up the thick mucus and at first she wasn't sure about this whole nebulizer thing, but the last few times we did it she decided to stop fighting us.  So that's progress!

She isn't coughing or wheezing anymore and is back to her happy little self again!

She has clinic on the 11th so I'll update again then!

Bronchoscopy

E had her bronch this morning and did so well.  She was super sweet and snugly afterwards as she was coming out of anesthesia.  

When he was finished, Dr. R came out and told us he saw a lot of thick mucus secretions in her lower airways.  It was more than he expected to see in someone her age.  While having thick and sticky mucus in the airways is a common CF problem, it is not common to have this much so soon.  So he suggested increasing her CPT and starting Pulmozyme .  The Pulmozyme is an inhaled medication that will help break up and thin the mucus, making it easier for her to get rid of.  So our new daily routine will consist of 3 vest treatments,1 neb treatment of Pulmozyme, and albuterol 1-2 times a day.  I'm going to have to get very creative to keep this kid still long enough to get it all done!  Too bad she doesn't like to watch TV yet.

I have mixed emotions after this morning.  I wasn't really expecting to hear that there was anything abnormal going on.  I figured he would get a sample, send it off to the lab and then tell us in a few days that there's nothing growing in her lungs and I need to quit overreacting to every little cough she has!  

I'm a worrier.  I can't help it.  And I never know if my worrying is warranted or if I'm just a crazy overprotective mom who needs to chill out and let her kid cough in peace!  But apparently this time it was good to be the crazy overprotective mom that calls the CF clinic way too much and asks a million questions.  Because we found out something with the bronchoscopy that we wouldn't have otherwise known.  Normally our clinic doesn't start Pulmozyme until 2 years old.  So now that we know she needs it, she can start it early and hopefully start feeling better.

We'll find out later this week if there is any bacteria in there that needs to be treated.  

Figuring Out The Inhaler

We recently added an albuterol inhaler into the mix.  If only she was this happy when we were actually using it!

So far, she's not a fan.  But I'm hoping it will get easier the more we do it.  I do think it has helped with her wheezing, though!

Frustrated

Right after we finished her last round of antibiotics, E started up with the runny nose, cough and congestion again.  We tried to keep her nose clear and added in extra vest time to see if we could get rid of it, but it wouldn't go away.  

After 10 days of this coughing and wheezing I took her in to get her throat swabbed to see if she cultured anything this time.  The rule is to put her on antibiotics after she has been coughing for 7 days.  But I don't like the fact that we just automatically put her on an antibiotic without knowing whether or not she actually has an infection.  So that's why we did the throat culture first before starting meds.

Well, now 6 days later she is only growing normal flora.  Nothing showing up in her upper airway.  But her cough is getting worse.  

So what is causing this to keep happening over and over?  I know kids get runny noses and all the junk makes them cough.  That's normal. But hers seems to last forever.  

Is this just a normal CF cough?  Is she always going to sound like this?  What if there is something bothering her that we can't see?

With all this thick, sticky mucus hanging out in there she is bound to attract some bacteria that will cause problems.  So after talking with her CF clinic, the next step will be to take her in for a bronchoscopy.  I'm not really sure how I feel about this.  I want to see if there is something in her lungs that she's not culturing in her upper airway.  But there is also a chance that we won't find anything and she will have gone through all of this for nothing.

So that's where we are.  They are calling me sometime in the next few days to schedule the bronch for Monday and we'll see what he finds (or doesn't find) in there.

Predictable

Somehow E has managed to stay on this schedule of getting sick every other month since she was 4 months old.  It always starts with a runny nose, then sometimes a little fever, then a cough.  She gets cranky and doesn't eat very well.  Then the nose clears up but the cough lingers.  And after a week or so of increased airway clearance and no change in the cough, I have to call and get her started on an antibiotic.
This time she's taking Septra.

Fortunately, her last several sputum cultures have only shown "normal flora" so hopefully we can keep that up! 

Clinic 06/12/13

This was E's last clinic visit as a part of the BONUS study.  I'm really glad we were able ot participate in the study and hopefully help researchers to understand how different babies with CF grow in their first year.

We had to do a few extra things this visit. She had a chest x-ray and blood draw that she was not happy about. :(  Her weight is up to almost 20 lbs and she grew an inch since her last visit.  Yay!

Her vitamin D level was pretty low so we'll be supplementing extra vitamin D on top of the AquADEKs she is already taking.  Since most people with CF do not absorb fats properly, they have a hard time absorbing fat soluble vitamins (A, D, E and K) so we have to give her those vitamins with enzymes every day.

She hasn't been on antibiotics since April, and her sputum culture only grew "normal flora" so everything is looking great!

I'm looking forward to a fun-filled summer with my healthy, happy girls!