Video - Living With Cystic Fibrosis

This is the video we used to help raise money and awareness this year.

Great Strides 2016

There are times when the words "thank you" just don't seem like enough.  

This is one of those times.

I don't know what other words I can use to let people know just how much it means to us that they took the time to show up in support of Emery and our family to participate in Great Strides.

Even to those who couldn't come to the walk, if you made a donation, asked for donations, bought a shirt, or even just shared a Facebook post, it all makes a difference!

Last year we raised $33,729.

This year we actually topped that number raising $34,826!

How is that even possible??

The amount of love you all have shown us over the past 4 years since we found out Emery had cystic fibrosis is unbelievable.  THANK YOU from the bottom of our hearts!

T-Shirt Day on Social Media

Every year we sell Emery's Angels shirts as a fundraiser for the Cystic Fibrosis Foundation.  We like seeing everyone in their shirts so we encourage people to post a picture on social media with a link to their Great Strides page, our facebook page, Emery's video or something else CF related to help us raise awareness.

It makes my heart so happy to see all of these pictures fill up my news feed!

Pneumonia and Hospitalization

I always hear people talk about how CF kids can be doing really well and then all of a sudden out of nowhere it changes.  Well let me tell you, it's true!  One Friday afternoon we were eating ice cream and having fun at Chick-Fil-A and about 3 hours later Emery had a fever and was complaining that it hurt to breathe.  I normally wouldn't overreact about a fever and a little cough, but when my baby says it hurts to breathe that got my attention.

After talking with her pulmonologist and trying albuterol, we didn't see any change.  She was still complaining about pain so we took her to the ER to get checked out.  Her chest x-ray looked good, she was negative for flu and RSV, no signs of respiratory distress, so they said it was probably a virus and sent us home.  

The next day her fever was higher, she was still complaining about pain and things just didn't seem right.  We took her back to the ER and they found pneumonia in her upper right lobe.  The difference in her chest x-ray from Friday night to Saturday afternoon was pretty significant.  

She was admitted on Saturday afternoon for a CF exacerbation and started a round of IV antibiotics. 

 We also realized she was constipated and that was another reason she was complaining about pain.  After a couple of days on antibiotics, some miralax, and increased respiratory therapy, she was looking much better and we were told we could finish out her IVs at home.  

Sweet girl staying busy in her hospital room.

Her mood completely changed when visitors came in!

 They put a picc line in on Monday and we went home on Tuesday.  
She was ready to get out of there!

CF Clinic

E had a routine check up at the CF Clinic yesterday.  I stopped at the gift shop to grab some mints and we ended up leaving with this lovely overpriced baby doll that laughs when you squeeze her tummy.

It kept her busy for 2 hours so I guess it was worth it!

She is finishing up a round of antibiotics for a cough that she had a couple of weeks ago so she is feeling well right now.  The only concern they had was that she didn't gain any weight since her last appointment.  She has always done really well with weight gain so it's not TOO concerning right now.  We'll wait and see how it goes for the next 3 months and hopefully by her next clinic appointment she will put on some lbs!  

Nasal Endoscopy

I haven't posted much about E's health lately because there wasn't much to post, thankfully!  She saw the ENT in December and again in February and he used an endoscope to check for new polyp growth in her nose.  The first time she saw the scope she was afraid he was going to try to put the whole thing in her nose!  But after we explained it and she was able to look at the inside of her nose on the tv screen, she was fine.

She does have some new polyps but since they haven't been causing any problems, he decided to leave them alone for awhile.  

We'll go back again in May unless something comes up before then!

Ready For a New Year

2015 was a year full of ups and downs.  

At the beginning of the year, I was frustrated about E's health and the fact that we were doing the same things over and over and expecting different results...

Insanity?  Yes!  

None of it made any sense to me, but I'm not a doctor.  I'm not supposed to know how to handle complicated cystic fibrosis related problems.  So when they told me "kids just get runny noses" or "she's too young to have sinus problems" or "time to start another round of antibiotics" I just went along with it.  I was frustrated and confused and I felt like no one was listening to me.

At the same time, our family was ready to start our annual fundraising for the Cystic Fibrosis Foundation.  They have all been so supportive and wonderful and excited to get involved in raising money for CF, but I just wasn't into it.  I was completely ready to forego Great Strides last year and just take some time off from fundraising because the everyday stress of CF was more than enough for us to handle.  But luckily, I was talked back into it.  With the help of our family and friends and people we don't even know, Emery's Angels raised over $33,000 for the Cystic Fibrosis Foundation.  I look at that number now and I can't believe I thought about taking a year off!

One of the big highlights of the year in the CF community was the FDA approval of Orkambi for CF patients ages 12 and older.  It is the first drug to treat the underlying cause of cystic fibrosis in people with the F508del gene mutation (which is what E has).  This breakthrough would not have been possible without the help of the Cystic Fibrosis Foundation and all of the wonderful people who donate year after year.

In addition to the extraordinary amount of money we raised, we also figured out why E was getting sick so often.  Nasal polyps and blocked sinuses.  After 2 sinuses surgeries, one in June and one in September, she got some much needed relief.

We know there will be hurdles to jump in 2016 as well, but this little firecracker is ready for whatever the new year brings!

Sinus Surgery #2

E had her second endoscopic sinus surgery on Tuesday.  We got to the hospital at 8:45 am and the procedure was scheduled to start at 10:55, but it ended up being pushed back to 1:15.  So for 4 1/2 hours we tried to keep her entertained and keep her mind off the fact that she hadn't eaten anything all day long!

The procedure went well. It turns out that the maxillary sinus on her right side was completely blocked and unable to drain which was probably causing a lot of pressure and pain.  He said that in a lot of cases with kids who have cystic fibrosis, they don't complain about this kind of thing because they are so used to it and they also have other problems going on at the same time.  But if it was you or me, we would be complaining about it!  So I'm really glad we got her in when we did.

We'll go back for a follow up in a month and I'm hoping we don't see new polyps developing already by then, but more than likely they will be.  They sent some samples off to get cultures done and we will find out in a few days if there was an infection in her sinuses that needs to be treated.

Thanks again to everyone for your prayers and support through all of this!  It means so much to us!

Planning For Another Surgery

Since E had her sinus surgery in June she seems to have been feeling a lot better.  No snoring, no congestion, not even a runny nose until about 2 weeks ago.  So I was surprised when we went back for a check up with her ENT in July and found out that he already saw new nasal polyp growth.  It had only been a little over a month after she had surgery to clear out her nose and sinuses.  

He suggested getting another head CT done to see how her sinuses looked so we waited until the end of August (almost 3 months post-surgery) and got the CT scan.  I got a call that afternoon from the doctor and he told me that her maxillary and ethmoid sinuses are completely full again and he wants to go back in and clear them out.

When she had her first surgery, he told me there was no way of knowing how soon they would come back.  He's had patients that go 2 years in between surgeries, and others that only go 3 months.  Well, it looks like she's one of the 3 month ones right now.  She will go in for another endoscopic sinus surgery in a couple of weeks to get her sinuses cleared out again.

In the meantime she is getting used to doing sinus rinses,  which our clinic nurse referred to as "airway clearance for the sinuses."  Just like she does her vest everyday to keep her lower airways clear, she needs to do sinus rinses every day to keep her nasal passage and sinuses clear.

We also had her quarterly CF Clinic visit yesterday.  She is growing well and made it over the 30lb mark!  Her lungs sound clear, she's not coughing, no problems there.  But her lab results showed that her vitamin D level is low again so we need to add a vitamin D supplement.  Just one more thing!  No big deal!

I am constantly amazed at how she handles all of this.  As soon as we get used to a certain treatment schedule, things change and we have to adapt.  We add new medicines and schedule extra appointments and she just goes with the flow, because this is her life and that's just what we do!

Great Strides 2015

Our original Great Strides walk was rescheduled this year due to severe weather.  So on June 27th, we were finally able to get together to celebrate our fundraising efforts!  Thanks to all of Emery's Angels we were able to raise $33,729 for the Cystic Fibrosis Foundation!

Every year I am amazed at how generous people can be.  Since Emery was diagnosed 3 years ago we have raised over $70,000 for the Cystic Fibrosis Foundation.
SEVENTY THOUSAND DOLLARS!!

The Foundation is doing so much with that money.  Just recently the FDA approved Orkambi, a new drug that will help cystic fibrosis patients ages 12 and over who have two copies of the f508del gene mutation.  Orkambi is not something that is used to treat the symptoms of CF, it actually treats the underlying cause of the disease!  This would not have been possible without the support of the Cystic Fibrosis Foundation and the money that was raised by families and friends just like us.
It makes a difference!
Thank you!