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!

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!

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!

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!

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.

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.

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.

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!

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.

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!