Monday, January 4, 2016

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!

Friday, September 18, 2015

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!

Thursday, September 3, 2015

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!

Wednesday, July 22, 2015

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.

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!

Tuesday, June 16, 2015

Sinus Surgery

On June 4th, Emery went in for endoscopic sinus surgery to have nasal polyps removed and to get her sinuses cleaned out.  It ended up taking about 2 hours to do the procedure and afterwards her doctor said it was a really good thing that we brought her in.  He showed us a picture of one of her nasal passages which I will spare you in case you are eating!  But it was very interesting to see!

Now that we know what to look for, hopefully we can get her in before it gets too bad next time.  And yes, there will be a next time.  This is a recurring thing with many people who have CF, so her doctor said to expect to be back at some point. 

 We can tell that it has really helped.  No more snoring, she's trying new foods, we can hear that she's breathing more easily.  It was a great thing.

Wednesday, May 13, 2015

Sinus Problems

I've had concerns for awhile about E's constant runny/stuffy nose and snoring.  It got to the point where we knew it was time to dig further and figure out what the problem was, so we made an appointment with a pediatric ENT.

Right away he noticed several nasal polyps and sent us down to do a CT scan.  He told us we could either try to do it downstairs, or go to Children's and have her sedated if I thought she wouldn't lay still for it.  Luckily she was very cooperative and we were able to get some good pictures!  She's so easy going when it comes to doctor visits.

The CT scan confirmed that she has extensive sinus blockage.  

The picture below shows what the sinuses should look like in a healthy person.  All of the black areas at the top are aerated.  The sinuses and nasal passages should be black like this, showing that air is able to move in and out.

This is a picture from E's CT scan.  The grey color inside her nasal passage and sinuses shows that air is not moving through there.  They are filled with mucus and polyps.  So this is why she can't breathe through her nose!

We scheduled for her to have endoscopic sinus surgery in a few weeks to get rid of this mess.  I think she'll feel like a new person afterwards when she can finally breathe through her nose again!

Now...this is not something that we found during a routine check up.  I had to push for it.  I had to persist because I knew something wasn't right and I didn't feel like anyone was really hearing my concerns.  We had tried over the counter medicines, nasal sprays, decongestant drops, and nothing was helping.  It was time to look deeper.  So for any other CF parents that might be reading this, don't forget that you know your child best and if something seems off, keep pushing until you figure it out!

I'll update again after the surgery.  Please keep E in your prayers!

Wednesday, May 6, 2015

Trying Some New Meds

I keep a journal about E's health where I record when she starts to develop new symptoms or when she starts new medications.  I was looking back at it one day and noticed that she almost always has a runny/stuffy nose.  And then I started paying closer attention and realized I can't even remember the last time she breathed through her nose.  Over the counter meds have not been helping so I talked to her clinic to see if they had any other ideas.  We started a nasal decongestant drop and a nasal steroid spray to see if that will help.  We also talked about her snoring and decided it would be a good idea to see an ENT to find out if there is anything else going on in there.  So she has an appointment on Monday.

She started an antibiotic last week as well for a cough that she couldn't kick.  So we're hoping that with all of these added meds she will get some relief!  I will update again next week after her appointment.

Friday, April 24, 2015

The "Newby" Award

Our local chapter of the Cystic Fibrosis Foundation awarded our team, Emery's Angels, with the "Newby" Award for fearlessly playing a significant role in the chapter though new to the world of cystic fibrosis!

We are honored to be a part of this organization!

Thursday, April 16, 2015

Our CF Awareness/Fundraising Video

We decided to try making a video this year to help with our fundraising.  So many people know that E has CF, but they don't really know what it is or what she goes through.  This was a great way to share her story and help them understand why we are raising money.  
The response to the video has been great.  People keep telling us that all they usually see is a healthy, happy girl.  They didn't know about what all she has to do to stay that way.

Thursday, April 9, 2015

Emery's Angels T-Shirts!

Here are the t-shirts for our Great Strides walk this year.  But you don't have to be walking to buy one!  On May 1st there will be an official #emerysangels t-shirt day where you can show your support from wherever you are!

If you haven't bought yours yet, you can get it here:

We have also created a facebook page to keep you updated on our fundraisers and on Emery's health.