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.

Wednesday, March 11, 2015

If you're new to the blog...

Here is a little bit about what cystic fibrosis is and how it affects the body.  (

What Is Cystic Fibrosis? 

Cystic fibrosis (CF) is a life-threatening genetic disease that primarily affects the lungs and digestive system. An estimated 30,000 children and adults in the United States (70,000 worldwide) have CF.

In people with CF, a defective gene and its protein product cause the body to produce unusually thick, sticky mucus that:

  • Clogs the lungs and leads to life-threatening lung infections.
  • Obstructs the pancreas and stops natural enzymes from helping the body break down food and absorb vital nutrients.
When mucus clogs the lungs, it can make breathing very difficult. The thick mucus also causes bacteria (or germs) to get stuck in the airways, which causes inflammation (or swelling) and infections that lead to lung damage.

Mucus also can block the digestive tract and pancreas. The mucus stops digestive enzymes from getting to the intestines. The body needs these enzymes to break down food, which provides important nutrients to help people grow and stay healthy. People with cystic fibrosis often need to replace these enzymes with medicine they take with their meals and snacks, which helps them digest food and get proper nutrition.

How do people get CF?

Cystic fibrosis is a genetic disease. People inherit CF from their parents through genes, which also determine many other characteristics, including height, hair color and eye color.
CF is caused by mutations in a gene that produces a protein, called CFTR. The CFTR protein controls the flow of salt and water in and out of the cells of organs like the lungs and pancreas.

To have cystic fibrosis, a person must inherit two copies of the defective CF gene — one copy from each parent. Both parents must have at least one copy of the defective gene.
People with only one copy of the defective CF gene are called carriers, but they do not have the disease themselves. Each time two CF carriers have a child, the chances are:
  • 25 percent (1 in 4) the child will have CF
  • 50 percent (1 in 2) the child will be a carrier but will not have CF
  • 25 percent (1 in 4) the child will not be a carrier and will not have CF
There are more than 1,800 known mutations of the CF gene. Because there are so many, most genetic tests only screen for the most common mutations.

How is CF treated?

Treating a complex disease like CF requires therapies that address problems in different parts of the body, especially the lungs and the digestive system.
Because the type and severity of CF symptoms can differ widely from person to person, there is no typical treatment plan for people with the disease. CF Foundation-accredited care centers work closely with people with CF and their families to create individualized treatment plans.
However, each day, most people with CF typically:
  • Do some form of airway clearance to help loosen and get rid of the thick mucus that can build up in the lungs. Some airway clearance techniques require help from family members, friends or respiratory therapists. Many people with CF use an inflatable vest that vibrates the chest at a high frequency to help loosen and thin mucus.
  • Take inhaled medicines — liquid medicines that are made into a mist or aerosol and then inhaled through a nebulizer. These medicines include antibiotics to fight lung infections and therapies to help keep the airways clear.
  • Take pancreatic enzyme supplement capsules with every meal and most snacks to improve absorption of vital nutrients. People with CF also usually take multivitamins.
The CF Foundation supports research to discover and develop new CF treatments, and maintains a pipeline of potential therapies that target the disease from every angle.
The most recent drug approved for CF, ivacaftor (Kalydeco™), treats the underlying cause of CF in a small number of people with specific mutations of the CF gene. All other CF therapies available today treat the symptoms of CF.

Work is ongoing to find additional CF therapies that could help improve key symptoms of the disease by targeting the disease at its root.

Monday, December 22, 2014

"As long as it's healthy!"

I was talking to someone the other day about whether or not they were planning to try to have a baby soon.  We talked about the timing and he said they were trying to plan around a vacation and a few other things.  But then he said, "honestly, it doesn't matter when we have a long as it's healthy!"

I used to say that too.

I never really thought about what that might sound like to the mother of someone whose baby was not born healthy.

Just so you know, it sounds or rather feels like a punch in the gut.

And I know he didn't mean it that way.  No one means it that way. It's just something we say.  We all want our babies to be healthy.

But what happens if they're not?

We all have this vision of what life will be like after the baby is born.  Dressing her up in cute little dresses and hair bows, showing her off to friends and family, watching her learn to sit up, crawl, talk and walk.  Nowhere in that vision do we see our babies taking medicine every single time they eat.  We don't envision ourselves pounding on our baby's chest and back for an hour a day to help break up the mucus in her airways.  We know there will be doctor appointments, but we never thought there would be THIS MANY appointments!

None of this was in our plan.

While I know I said the exact same thing when I was pregnant or thinking about having babies of my own, I now see things differently.  It's okay that my baby wasn't born healthy.  It's okay that things didn't go according to our perfect plan.  She still does all of the normal things that kids do.  She makes us laugh, drives us crazy, sings along to the radio in the car, throws her food on the floor at dinnertime, runs up and gives her daddy a big hug every day when he gets home.  She's just like every other 2 year old girl, she just has cystic fibrosis.  

CF has brought people into our lives that we otherwise would have never known.  It has made us appreciate things that we otherwise would have taken for granted.  It is part of our life now, whether we wanted it or not.  And we are okay with that.

So if you have your baby and it isn't perfectly healthy, I'm here to tell you that you will figure it out.  It will be hard.  There will be times when you have no idea what you're doing...but you just figure it out.  And even though things didn't turn out like you planned, you will still love that baby with all of your heart.  That's what being a parent is all about.