Monday, December 30, 2013

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!

Monday, November 18, 2013

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!

Sunday, September 22, 2013

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!

Sunday, August 18, 2013

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!

Monday, August 5, 2013

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.  

Sunday, August 4, 2013

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!

Wednesday, July 31, 2013

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.

Monday, July 1, 2013

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! 

Monday, June 17, 2013

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!

Saturday, May 18, 2013

Tulsa Great Strides


This weekend we had family walking for E in Tulsa since they couldn't be at the other walk with us!  Thanks so much for going and for sending pictures, Nana!  We love you guys!

Thursday, May 16, 2013

ONE Year Old!

My baby is ONE!
It's amazing how much they change in the first year.

I wasn't sure what life would be like with 3 kids.  Going from 1 to 2 was really hard for me.  But E has been such a good baby.  It's pretty crazy that with all of the meds she is on and the airway clearance we have to do, she still seems like my easiest baby!  No inconsolable crying, no pacing around the house for hours at a time trying to get her to calm down.  She's been sleeping through the night since she was about 2 months old, which is pretty much the best thing a mommy could ask for.  She is amazing.

I am so thankful for every day I get to spend with this precious little girl.
Happy Birthday!

Sunday, May 5, 2013

Great Strides 2013

Great Strides is the Cystic Fibrosis Foundation's largest national fundraising event.  The money that is raised helps to support life-saving research, care and education programs to help those with cystic fibrosis.

Several months ago, Ryan and I decided to form a team for the Great Strides walk in our area.  When I registered, I was asked to enter an amount for our team's fundraising goal.  I entered $1000 thinking that I could surely get that if I asked everyone I knew and really worked hard to get the word out.  I sent an email to our family letting them know about the walk and asked if anyone wanted to donate or join our team.  One by one they started signing up.  Our team grew and people started raising money.  Everyone was excited about it and we actually had people who had never even met E sign up to walk with us!  This was our first walk and we had 24 adults and 10 kids on our team!!!
On the day of the walk, the weather wasn't very nice at all.  It was cold and windy and miserable but that didn't stop everyone from coming! 

I am so proud of everyone for getting the word out and sharing E's story.  I don't have the official numbers yet but I know that we have raised at least $19,000 for the Cystic Fibrosis Foundation!  Yes, you read that right.  NINETEEN THOUSAND!  Never in a million years did I think we would raise that much money!
Every single person on our team did an amazing job and I cannot even begin to tell you all how much we appreciate it.  I do have to give a special thanks to Uncle Eric for raising $8,000 on his own!  Unbelievable!

***Update: Our final total was $20,100.00!  WOW!!***

 There were "hero awards" for all the kids who have CF and each one got to go up and receive their medal.

Then it was time to walk!















All that walking wore her out!

Love my little hero!

Wednesday, April 10, 2013

Clinic 04/10/13

E had a pretty good clinic visit today!  She's been feeling great for the last month and a half or so.  No more coughing or congestion.  Yay!

She weighed 18 lbs 10 oz and is still 27 in long. 

Her weight is good but the dietician wants us to try to get a few extra calories into her every day.  I really do try to give her an extra bottle most days but she very rarely takes it.  So we'll work on that.

Also, Dr. R mentioned that he wanted us to come in for a throat culture in between clinic visits.  Since she is being seen every two months right now and it will change to every three months after she turns a year old, he doesn't want to risk her getting a bug that we don't know about until it has already made a nice little home in her lungs!  (Those weren't his words but that's pretty much how I understood it!)  From what he told me, this is not something that all clinics do.  But I'm realizing more and more how proactive he is and I think it's great that he wants to know as soon as possible if she cultures pseudomonas so that we can treat it and keep it from causing too many problems.


Monday, March 18, 2013

SmartVest Airway Clearance System

For months I've been looking forward to the day we could get an airway clearance vest for E.  Doing manual chest PT has become quite the challenge lately since she doesn't sit still for long and if we try to do percussion on her chest she just swats our hands away or tries to chew on the percussor (or "bopper" as we've come to call it at our house)!

So at our last clinic visit we asked if she was big enough for a vest and they told us she was!  We went through a long few weeks of waiting for insurance to approve everything and finally got it ordered and shipped last week.

If you have no idea what I'm talking about, this page will help explain how it works.
Basically, she wears a vest that is attached to an air compressor that shakes her chest to loosen the thick mucus in her lungs.




Thursday, March 14, 2013

CF Can Be A Lonely Disease

When we were first learning about cystic fibrosis after E was diagnosed, a lot of people asked us if there were support groups where other parents of kids with CF could get together and talk, share stories and resources, etc.  It might be nice for E to have a friend one day who could relate to what she is going through.  Someone who has to do the same treatments and take the same types of medicine and someone who understands how she feels.

However, there are risks involved when two people with CF are in the same room.  There is a risk of one person with CF spreading destructive germs to another person with CF that can lead to severe or worsening lung disease.  Bacteria such as pseudomonas aeruginosa (pseudomonas) and Burkholderia cepacia (B. cepacia) are resistant to many antibiotics which makes them difficult to treat.

The Cystic Fibrosis Foundation released these new changes to their infection prevention and control policy today:

We are writing to let you know that the Cystic Fibrosis Foundation recently implemented a new infection prevention and control policy for all Foundation events, meetings and offices to protect the health of people with CF. We took this step based on increasing medical evidence that the risk of people with CF spreading destructive germs to one another is greater than was previously thought. Our new policy reflects the advice of leading medical experts on this subject.

The health and well-being of people with CF is our topmost concern — it is at the heart of all we do. We have long had infection control guidelines in place to promote the safety of people with CF. With this new policy, we are acting proactively and immediately to reduce the risk of cross-infection among people with CF. 

The key elements of the Foundation’s new policy are:
  • At any CF Foundation-sponsored indoor event or meeting, including gatherings like committee meetings, only one person with CF may be present and he or she will be designated in close consultation with event chairs and key event volunteers.
  • At Foundation-sponsored outdoor events or gatherings, people with CF need to maintain a distance of at least 6 feet from each other.
  • Under no circumstances shall individuals who have ever had a confirmed positive sputum culture for Burkholderia cepacia (B. cepacia) complex attend any CF Foundation events, meetings or offices. 
Just another reminder of how cautious we have to be about germs, not just at these events, but everywhere we go.  And another reminder about how socially isolating this rare disease can be.  

I feel badly for adults with CF that cannot attend some of the fundraising or advocacy events that they used to.  And I hate that we can't get together with other CF families and let our kids play together.  But I'm grateful for all of the people I have "met" over the past few months either through facebook, blogs, or online forums.  I'm glad we have a way to connect and ask questions and to support each other when we need it.  And I hope as E grows up she will have friends who take the time to really understand what she is going through so that she will have the support she needs as well.

Wednesday, February 20, 2013

Let's Try Something Else

Three weeks ago, E caught a cold or virus that gave her a stuffy nose and a cough.  Since it didn't go away after a week, the protocol is to start an antibiotic to treat her for an upper respiratory infection.  She took Keflex for 2 weeks and the cough is still lingering around.  It's not worse, but it's not gone.  After talking to her CF Clinic nurse today, we decided to start another oral antibiotic - Cipro.  She'll take this for two weeks and if the cough is still not gone, we'll go in for a bronchoscopy. 

So...
Why do we have to put her on all of these antibiotics if it's just a junky cough and some congestion?
Her sisters have the exact same thing and we're not treating them for anything.

Here's why...
Since we all passed around this respiratory virus, we all had coughs and congestion.  The good news for us is that we are able to get rid of the congestion on our own eventually.

E doesn't really get to just "wait and see" if it goes away.  The mucus in her lungs is so thick that it is the perfect place for bacteria to hide out and cause problems.  That's why if a cough lingers around like this, it is usually a sign of a CF flare up in her lungs.  Every time she has a CF flare up, there is the possibility of causing permanent damage/changes to her lungs.

So, if antibiotics and extra airway clearance (Chest PT) are what she needs, then that's what we'll do.
Let's hope this does the trick!

Wednesday, February 13, 2013

Clinic 02/13/13

CF Clinic....I look forward to it and dread it at the same time.  I like going because she gets checked out by so many different people who are making sure she is growing well and staying healthy.  But seeing all of those people takes a long time!  And it's not so easy to keep a baby entertained when you're sitting in a small room for two hours during her nap time!

But we survived!

I had a lot of questions to ask about giving enzymes before she eats.  E normally eats 3 meals a day (baby food and formula) and then has an extra bottle at some point.  There have been times lately when I wanted to give her a bite of a cracker or something else that I happened to be eating but I hesitated because she hadn't had any enzymes.  I asked about how that would affect her and if it was okay to do that.  They told me that having one bite of something isn't likely to cause any problems, but if she wants to keep eating she needs to take the enzymes or else it could cause her stomach to hurt.  So basically, I don't have to panic if she eats a bite of something without taking enzymes first!  Good to know!

She gained almost 2 lbs since her last visit and weighed about 17 lbs 14 oz today.  
Great weight gain!

Now for the not-so-fun part:
Right after I wrote my last post, E started to get sick.  She caught a cold and had a cough that lasted for about a week so I called and got her started on an antibiotic.  Today was day 9 on the antibiotic and we haven't seen any improvement. 

It's possible that she just has a cold that is lingering around for 2 weeks, but if she's coughing for that long it could be a sign of something else going on in her lungs.  Dr. R decided to keep her on this antibiotic for another week and if there's still no improvement we'll start Cipro (a different antibiotic) and inhaled Tobi.  

Hopefully it won't get to that point, but if so, at least we have a plan.  And that plan could always change once we get the results of her throat culture in a few days.  So I guess we'll just wait and see what happens.

Thursday, January 31, 2013

No News is Good News!

I haven't been posting lately because luckily, there is nothing to post!  E has been feeling great!  Somehow she has managed to stay healthy so far through this nasty flu season (knock on wood).

She is eating lots of new foods and gaining weight really well.  The one thing that is so different about feeding a CF baby is having to do the enzymes before she eats.  I remember giving my other girls a bite of whatever I was eating or throwing a few Puffs out to keep them quiet until I could get their food ready.  But with E, I can't just give her a random snack whenever I feel like it.  If she doesn't get the enzymes first, the food just goes right through her.  So I think that has slowed us down a little when it comes to trying new things.  I will get better at it, just takes some adjusting!

We go to Clinic on the 13th so I'll update again then!