anyone still out there?

Testing...Testing...1,2,3....Testing... We are back! Look for updates coming from 2012 and into 2013!

Fight the good fight!

Cystic Fibrosis is a chronic life shortening disease that is relentless.  There is no break for Mason or us from this disease.  We spend a lot of time each day fighting this uphill fight.  There are many components to fighting: inhalers, nebulizers, pills, IV's, blood draws, weight checks, specially prepared food, multiple doctor appointments, tests, and so much more.  Please watch.

I don't apologize for the many posts "showing" this disease.  It is what Mason and 70,000 others in the world fight to breathe on a daily basis.  It is not for pity or attention.  It is for awareness.  It is for the hope for a cure.  It is for my son.  So that my son will be able to grow old without having to worry about dying before the median life expectancy of 37.

Infant Pulmonary Function Test

Mason's Infant PFT (pulmonary Function Test) was today.  We woke him up at 4am to make sure we could get some food in him before the 5am cut off.  Derek got up with him at that time and was able to get some in but not much...but who wants to wake up to eat that early anyway?!  So, I took over at 7am while Derek got a little shut eye.  All Mason could have from 5am to 9am was clear liquids, after that it was nothing.  Today was a day I was thankful for the nuk to help pacify his hunger.

 Mia was not excited that Mason was up so early to terrorize her.

 Soooo sleepy at 8:30am.  He crawled into his chair with his blanket and my scarf (he likes to carry around my scarfs lately) and watched Mickey Mouse Clubhouse. Normally we only watch that show during treatment time, but today he got extra attention.

The  car ride, which I thought was going to be tough to keep him awake, went great.  Only a few times we started to close those eyes, but I jumped into action to not let him drift into dreamland (although all 3 of us wanted to go there).

When we arrived to Children's we headed right to Radiology for another Chest X-ray.  The last one we had was the day he was admitted to the hospital in July, so we wanted to see what his new "baseline" was.  We knew right where to head (kinda hate that I have learned the layout of Children's so well) and had to wait.  I knew I should have called the day before because they had to call to the clinic due to the order not being in the system.  We had timed our arrival just so that Mason was not sitting to long because we knew he was not going to be a happy camper.  Within a few min we were back in the room.

 "welcome to the gun show ladies."

 no?, that joke doesn't work here?..too bad  we have to have some fun!

We got the Pulmonary Lab/ CF clinic at 11am and got checked in.Happy to report that Mason is weighing in at a whopping 24 pounds, 6 ounces.  That is more than a pound increase in a month!  AWESOME JOB buddy!  He is 31 1/2 inches tall.  These stats put him very close to the 50% in all three categories!  LOVE IT!!!!!

 "I want to go to S.L.E.E.P people!!!!"

The had warned us that the tough part was getting the Chloral Hydrate into him.  It is the medicine to help him go into a deep sleep for the test.  It reportedly tastes very bitter.  He only gagged once and took the rest of the 3 syringes like a champ!  The nurse was impressed but we knew he would rock! It took him about 30 min to finally get into a deep enough sleep that they could take him into the room.

All tuckered out, hooked up to a monitor, and ready to rock! 

Now, just to warn you, the next set of pictures look worse than it really is.  He got "hooked up" to all the machine at about noon.  They placed "silly putty" around his mouth to help keep a good seal on the breathing mask.  He was breathing in room air the whole time, but oxygen was available if needed (which he did not).  They placed a cloth wrap around his chest on top of a plastic bladder that would fill with air.  They monitored his heart rate, respiration's, O2 levels the whole time.  A blood pressure check was done every 5 min. The test took about 1.5 hours.

 All hooked up in the "box."  They only close the box twice during the test and it is only closed for about 5 min.  They seal it to get indications on how much his lungs can hold.

Sorry it is a little dark.  The door was closed for this part. This was the only video we could do before we were told we couldn't video according to hospital policy.  Feel free to watch the video on the previous post to get a better idea.

 The room isn't very big so they set up chairs for the parent's to sit and watch if we wanted.  I stood in the room for the majority.

There are always two people present during this test: the nurse to monitor vitals and is there in case needed and the respiratory therapist who is performing the test.

 The wrap he has on would quickly inflate and basically push all the air out of his lungs.  It simulates what we do when we blow out.  They also force air into the lungs for this test...so we warned he could be a little gassy for 24 hours...as yes he is.  Actually, during one of the inflation's he let out a very large and long fart.

 His electrodes to monitor before, during, and after the test.  He was loopy after the test and provided us with some laughs.  We were told to watch him and not allow him to walk alone once home for a few hours because he would be walking like he was drunk..and that he did!

Results:  Overall great!  The preliminary results for the PFT ranged from 100%-128% FEV.5.  Now I am not entirely sure what this means but I was told this is very good.  We wanted over 100%.  Dr. Q was to review the results and give me a call tomorrow or the next day.  

Also, while Mason was having the testing done, Dr. Q brought Derek and I into an exam room to review his Chest x-ray.  We looked at all 4 that he has had this year.  She stated that the lungs "look better" since his hospitalization, but did notice some CF related changes.  Of course that's where the tears come in. I hate to hear there are "changes" meaning mucus build up, inflammation, and air trapping in his 16 month old lungs, but this is our reality.

For the most part we try not to think real hard about what his lungs will look like down the road, but every time we head into the clinic you can't escape it.  It's hard to swallow the fact that his lungs will continue to detirerate over time and there is nothing we can do to stop it...slow it down yes, but stop it no.  Parent's are suppose to protect their children and this is something we can't protect him against and are reminded daily of that struggle. But he is so darn cute we decided to keep him ten times over.  :) 

For now we do what we can.  Hypertonic Saline, Pulmozyme, and the Vest are three big guns we are fighting back with.  Exercise will be the forth.  Look out world because Mason is going to be the star athlete in every sport! Dr. Q was happy to hear we got Mason a bounce house for Christmas...it will be great for his lungs!

 Headed home!  Dr. Quintero gave Mason a bear to head home with.  Not sure if it was because he was such a trooper, because she felt bad that I cried again while discussing Mason's lung status, or because it is Christmas and the CF clinic rocks.  I think it might be all three.

After we got home we all took a 4 hour nap.  Mason decided it was time to wake up at 7:30pm to watch some Mickey Mouse Clubhouse.

Now that this is over, we can enjoy the rest of the holidays!  Just hope everyone stays healthy so we can see everyone!

Merry Christmas!

Off to Children's for another test/procedure!

We have a loooonnnngggg day ahead of us tomorrow.  We head into Children's for Mason's first Infant Pulmonary Function Test.  Basically, it is a test for his lungs.  We should have a baseline for what his lungs can do.  This will be a test that we will do on a yearly basis to monitor his lung function.  The video below is a snippet of what will be happening tomorrow.

We have to wake Mason at 4am to give him something to eat.  He then can have only water until 9am.  After 9am he gets nothing.  The test is set for 11am.  Oh and they would like us to keep him awake from 4am until the test!  HA!  This will be fun. The test will take 3-4 hours so they want him to be good and tired so he won't wake up, even though they are giving him some medicine to help him sleep.

Oh did I mention he is also getting a chest x-ray 45 min prior to the test.  Those are "fun" even when he is rested!  

I will post more about Mason's experience and results tomorrow once we are home.  We plan on taking pics and video.

Well, off to bed...we are all going to be tired tomorrow!

Meal Time Storytelling

Most times when we feed Mason any sort of marinara pasta we know bath time will follow suit. This time was of no exception...He was "telling" us a story the Italian way...by using his hands a lot. And that means pasta in the hair, the clothes, the floor, and ALL over the face.

"Wanna hear a story?"

"I know I look a little like an oompa-loompa right now, but just listen up.""

"Hmm...where do I begin?"

"You see I went fishing and caught one this big!"

"Then the pole broke. I couldn't believe it."

"So what do you do? I thought the big one got away."

"So I Stuck my head in the water and grabbed the fish like this."

" Give me a high-five Dad...I caught a fish!"

"Sorry mom, Guess I got a little carried away with my story."

He ate well that night...so as long as he is stuffing his face with calories we don't mind...until it's time to take him to the tub and clean up the dinner area. :)

his laugh makes it easier

It has been a rough couple of months for the Cystic Fibrosis Community. So many have lost their battle, their lives to this awful disease. Not one of the 11 I knew from Facebook, were over the age of 23. One of those 11...an innocent one week old baby boy, Baden. This disease shows no mercy. Just last night a 20 year old woman got her angel's wings and can finally breathe easy. She and those others no longer need to fight to breathe, chained to an oxygen tank, drowning in their own lungs. Hannah's death has hit me a little harder tonight. I am not entirely sure why, but she was just starting her life, engaged to be married. She couldn't even go into a bar and order herself a drink yet. I desperately want to see Mason graduate High school, then college. I want to see Mason get married and have children. I want him to live a long happy life. I want him to outlive us. Some days it's just hard to dream past these 24 hours because I am too scared to. I don't want to be blind sided with CF. It is scary because we know someday it very well could be our child people are posting their sadness about. There is a statistic that states 40% of people with cystic fibrosis don't even live beyond 17...makes me cherish every moment that much more with Mason.

He is my very best medicine and Hero!

With sadness there also comes hope. Hope that there is a drug, Kalydeco, only 6 months away that could stop this disease in it's tracks. Without going into much detail..because I would be bring you back to high school science and then some, there is great things happening in science these days. There has been a drug that is going through the FDA process as you read this. It is a first of it's kind. It is a drug that corrects one of the DNA defects which causes CF. Now, there are 5 different ways the CF gene is defective (mutated) and this only target's one way. They are divided into "classes." This drug has been researched on the mutation G551D (only 4% of the CF population have his mutation), which is a class 3 mutation. Mason does not have this one, but one of his mutations is either a 2 or 3...it is rare so they are not certain yet. His Pulmonologist had said to us that she would like to possible have him try it out in a few years to see if it would help! My heart about stopped right there. You see this drug, has increased the CF patient's lung ability (FEV1 scores), lowered a person's sweat test to normal ranges, and has increased weight (which is hard to do with CF even though they are eating about 50% more calories a day as me and you). There are also two other drugs that possibly do similar things and are still in the trial process, but have promising results so far! She also thinks it is a possibility that these would help Mason as well. So, we need to keep Mason's lungs in as best of shape as we can so when he can try these new AMAZING drugs they will be of most benefit. They won't reverse any lung damage. What I just shared with you is a VERY VERY condensed can of info. Please don't hesitate to ask questions! This video is from the CBS nightly news. It does a good job of explaining what I attempted above.

We also learned another little tid bit of info. A question was asked why all these drugs are targeted at the lungs. I assumed it was because it is the bigger concern and what people with CF die from...respiratory failure. Why not target the pancreas as well? Our pulmonologist explained, because the mucus started thickening even before birth, the pancreas ducts are blocked and the pancreas is shot by the age of 6 months. That floored me. Hence, he take his digestive enzymes with every meal and will for the rest of his life.

Most people (and us initially) thought Cystic Fibrosis was a lung disease, but it affects sooo many more parts/organs/systems in the body. The picture below does a great job of explaining...click on it to enlarge.

November 2011 review in pictures

Can you believe that it is December already!?! Crazy to believe how time fly's when you're having fun Mason style! Here is a review of our November 2011 in pictures...

Helping with his chores! :)

Playing in the leaves...we were not too excited about this.

On day while Mason and I were in the kitchen, he got pretty quite. He was on the other side of the table while I was at the sink. I walked over to see what he had gotten himself into and realized he had grabbed the diaper bag off the table and proceeded to take every last wipe out of the brand new wipe container. After that he proceeded to take those same wipes and wipe things all around the kitchen...perhaps he has seen me do that more than a few times with the Clorox wipes. Hey, at least he is helping to keep the place clean!

He has also discovered that he fit UNDER our bed. I caught him going after his ball that rolled under. Then soon realized that I need to run the vacuum UNDER the bed...it's amazing what you can see with wood floors!

It took some time but the food did find his mouth :)

Here comes Santa Clause! As you can see his belly sure is jolly!

Always smiling, melting my heart a million times a day

He now prefers to carry Mr. Bear and Mr. Blanket to all destinations

Mason's patented nuk honing device is so strong, he doesn't even need to use his hands to get it to his mouth!

He prefers Apple's of all types not oranges (no really he does not like oranges)

Our new med we spend an additional 30 min on a day now. The solution is twice as salty as the ocean and he breathes it into his lungs.

What?! You don't eat Ramen noddles in you Christmas p.j's mid afternoon? Why not? :)

Uncle Dave came to visit from North Carolina during hunting season. I wore my camo to show my support, even though they didn't get anything this year.

Not related one bit!

We spent Thanksgiving in Union Grove this year. Grandma Pam came down too. I amazingly did not pull the camera out too much this year. But managed to capture some of the little man. Apparently, any blanket will do in the absence of his.

We don't travel light anymore. Treatments MUST be done no matter where we go. We gotta try and keep those lungs as healthy as possible.

How many people does it take to change a toddler in to pajamas? hehehe

Hanging with Grandpa before we headed home stuffed with turkey

playing in my new house Grandma Pam surprised me with! Thanks Grandma!

Mason pretty much gets into a daze while he watches Mickey Mouse during treatment time.

Not sure what happened with the camera with the picture...sorry.

Mason loves to "ride the horsey" now that grandma taught him. It's pretty comical actually since he now will come over to you and mimic the motion to get you to do it.

We bought this "head band" on ETSY about a month ago. It has been a very slow process to get Mason used to the mask for his three treatments a day. We have been having to hold it and sometimes he fights us...so this picture does not fully capture the excitment of him wearing it. It actually brought a tear to my eye because it is exciting to see him get used to it, but saddens me for that same reason.

night time bottle (gotta sneak those calories in where ever we can)! Mason still is not a fan of holding his own bottle or sippy cup, but I am pretty sure Grandma doesn't mind.

We headed over to The Healing Place Farm in Sussex with Great Auntie Natalie and Cousin Paulette. It's a pretty cool place. I picked up some essential oils and learned some reflexology to help Mason's lungs. Mason even got to pet a Turkey and horse...He didn't care for the horse too much...wouldn't stop crying until we got back to the car.

Trial run in his new sled...now where is that snow?

teaching Mason a valuable lesson on how to play with his food. Milk does produce some darn good bubbles!

"Dear Santa, I can explain."

Love snuggles with my little guy

One morning he decided he wanted to hold on to BOTH his bear and monkey...I see A LOT of stuffed animals in our future!

Playing with his new train before Grandma headed home.

This is how we eat chili in our household!

As for what Mason is up to this Month. His walking is very steady, he can walk pretty fast and quite! He will sneak up on the best of them! He is starting to point at things he wants. He babbles and babbles and babbles. No words yet, but they are coming! He has quite the personality...smiling a lot, laughing a lot, and when he does not get his way watch out! If we get this now, what happens when he turns 2? AHHH! He loves to give hugs, including Mia the cat. He also loves to throw things over the gate and down the stairs. Pretty sure a few toys will never be same again after that trip. When he hears the garage door he runs to the door with excitement because that means mommy or daddy are home. He does the same for when the phone rings...runs right to it. Mommyitis is in full swing. But how can you blame him? Hehehehe. He also has all 4 one year molars popping at the same time. FUN! He investigates everything and puts nothing back...makes it fun to find a few things in which you left a little too close to the edge of the table. Well, on to December!