Hasbro Houseparty Twister & Bejeweled Game Night Slumber Party

I got to host an AWESOME event this past weekend for Houseparty, a Hasbro Twister & Bejeweled Game Night Slumber party.

In our house we love playing games, any kind of games. So to be able to receive some fun games from Houseparty to play and have fun with was great! We received classic Twister, Bejeweled and Twister Ringz from Houseparty. We also received a coupon for Twister Dance Rave and a box of single serve bags (10) of Simply Natural White Cheddar Cheetos.

We invited a bunch of my daughters friends over to a game night slumber party on Saturday 10/19.

A few of the girls

 Now if you have daughters you know that slumber parties are INSANE to begin with.  There is loud music, and lots of food, and dancing and gossip (all girls have to gossip even at 10&11), and of course games.

The girls had a blast playing the classic Twister game (it's a game we like to play at any party). We all know that Twister is so much more fun when you have a lot of people. You set up the mat, and mom (of course) gets the fun of being the Twister guru, and then the real fun begins....becoming a tangled mess of arms and legs!

I think Twister is by far one of my kids, and all the kids who attending the slumber party, favorite games. You can't help but become a tangled mass of giggling bodies. I remember playing Twister when I was a kid. The game mat seemed a lot bigger then! We really enjoyed the option to get crazier with Twister on the back of the spinner (left hand on yellow and bark like a dog!) We even got creative and made up our own crazy options.

The Bejeweled board game is a great way to bring internet games to life. 

All the girls had to get there hands in there to set the board up. We had to read the instructions a couple of times until we understood exactly how to play the game. But once you got the hang of it it was a pretty fun and easy game to play. However, this group of girls decided that playing it by the rules was no fun so they just went at it how they wanted to and had a blast.

Dr. Scholl's® For Her High Heel Insoles

I have been know to avoid wearing heals because I can't stand they way the make my feet hurt. Thanks to Influenster for sending me these Dr. Scholls for Her High Heel Insoles to give a try (complimentary at that!).



So I broke out my favorite Madden summer heals....

 

I bought these last year when I absolutely fell in love with them! They are cute and funky. We planned a girls night out and on came my Maddens, equipped with my Dr. Scholl's For Her High Heel Insoles.

 



OMG! I love this insoles! They are soft and cushy and have a nice little bit of arch support which really helps keep my feet from hurting. They fit into any size or style shoe without showing. And off to the local pub we went...

 

Spent a few hours dancing to a cool live a band and had a few drinks and by the end of the night my feet were still happy....As was I

 

Anyone who wears heels on a daily basis or even for just a fun night out, should add Dr. Scholl's For Her High Heel Insoles to every pair of heels they own! Run on out to Walmart and pick up a pair for just $7.97. It's well worth it to keep your feet happy.

 



 



 

 

Olay Fresh Effects {BB Cream!}.

 

 

 

I am a makeup virgin...well sort of. I don't use a whole lot of makeup, really never have. I sort of cringe at all the time it takes to get all put together. But when I was sent this Olay FreshEffects BB cream to try out from Influenster I was both hesitant and ecstatic.

 

I have never used a BB cream before. I have heard a lot about them, but never bought one, or tried one. I knew it had to be good since when I opened my voxbox in front of my friend she about stole it right out of my hands! (and is still begging me to give it to her!) 

 

 



 
 



 



 
 



So here goes....This is my before BB cream picture. I hate my face. It has a lot of blemishes (and I feel it is just too round, which no BB cream can fix LOL).

 

 



And here is my after...Using the Olay FreshEffects BB cream and a some eye makeup (Covergirl of course).

 

I am loving this Olay BB Cream. It is so light and has just enough tint to it to cover all those pesky blemishes. I received the "fair to light" BB cream because that is how my skin is (unless I am in the sun a lot). I generally use a foundation, but with the heat it is just too much. So this was a great addition to my make up bag. The best part of the Olay FreshEffects BB cream is that is has SPF 15! So not only do I get a nice light coverage, but I get the added benefit of sun protection.

 

You can pick up your own Olay FreshEffects BB cream at Walmart for just $10.97. A great value since you are getting a light cover up and sun protection in one!

 

 



Goody Ouchless Ribbon Elastics

It has taken me years to finally let my hair grow out. I have always loved long hair but for many years just didn't want the hassle of having it. I now have a love/hate relationship with my long hair.  I love it because there is so much more I can do with it now that it's long. I hate it because when it is 100 degrees outside it makes me HOT.

 

 

 

 This is me with long hair. Now that summer has hit, 90 percent of the time my hair is up in some sort of mess bun or pony tail. I just can't tolerate it on my neck all the time. I, as I am sure most women, am always looking for a good hair tie that will not pull my hair out of my head. I have tried every time of hair tie there is on the market and none of them can accomplish keeping my hair up and not killing me when I pull it out.

 

Goody's new Ouchless Ribbon Elastics are great. They are flat, unlike many brands on the market and they don't have that little glue spot or metal bracket holding them together. As you can see that have a nice little knot in them. Now I know what your thinking "wont that knot pull apart?" I have worn these elastics for the last couple of weeks, pretty much every day, and have not had one problem with them. My daughter, who is know to break hair ties, is also wearing them and using them as bracelets and pulling on the constantly and they still haven't come apart.

 

(Good golly please ignore that awful ignore that awful picture of my lol) As you can see we both have a ton of hair to keep under control. I am constantly fighting with her to keep hers out of her face, but she hates hair ties because they hurt her. She has fallen in love with these Goody's Ouchless Ribbon Elastic. She can now tolerate having her hair pulled up. And she loves all the colors they come in.

 

These ribbon elastics are a must for every girls hair supply bucket! You can find that at most of your big box stores and they only cost around $3.97 (at Walmart).

 

 





My new summer fun from Influenster!

               WOO HOO look what I finally got the other day!!!

 

 

 

I was so excited when my hubby said that my Sunkissed VoxBox had arrived at our old address, I immediately made him send it up to me here in New York. This is my first VoxBox from Influenster so I am very, very excited about it. It has some great summer items in it for me to give a try.

 

Want a quick rundown of what was in the Sunkissed VoxBox??? Here goes....(I will be doing individual post on each item thru the next few weeks)

 

 

Sinfulshine With Gel Tech nail polish.

In my house we can never have enough or too much nail polish. My daughter is a nail polish FREAK (literally have to buy a new color ever time we shop!) I received this really awesome pink/fuscia color called ROYAL FLUSH. I will be changing my nail colors tonight to give it a try :)

 

Olay Fresheffects BB cream

I have never used a BB cream before so I am looking forward to giving this one a try. I must say my best friend was with me when I opened my Influenster box and she tried to steal this item away from me!

 

 

Dr. Scholl's For Her High Heel Insoles

I love heels. I love the way the look anyways. But I hate that my feet hurt so bad afterwards. So I am really excited to put these babies in my favorite pair of heels and head out for a night on the town.

 

 

Goody Ouchless hair ties

(My lovely daughter has already take 2 of these) My daughter and I both have pretty long hair so we are always looking for the next best hair tie. I wear my hair up about 90% of the time, and I hate nothing more than to have my hair tie pull my hair out of my head when I take it out. These hair ties are suppose to be "ouchless" so I am guessing they aren't going to be pulling my hair out. YAY

 

Watch for my future post on each of these products individually. I am looking forward to giving them each a try!

 

 

 

My Favorite vehicles. #ChallengeToWrite

I have been absent for a couple days. I have had a lot going on, and none of it good. But I decided to take a moment tonight to check out the prompt for today. And honestly, I am not feeling it. I can't write about my favorite vehicle right now. I just don't have it in my heart.

What I will write about is my Uncle's "toys". I will never forget the 1st car I ever remember envying. It was my Uncle's Porsche when I was probably only 8 or 9 years old. It was a beautiful car. It was this blue color and it was so sleek and small. I remember thinking it was so cool that my Uncle would have such and awesome car. It was the first time I had ever seen a car that didn't have a backseat.  I feel instantly in love with it. Maybe this is where my obsession with Porsche's started.

Then when I was a teenager he was the first person I knew to own a Hum-vee. Not the ones you see on the road now, but an honest to God military like hummer. It was so COOL. It was big. It was noisy. It was a rough ride. But it was so neat. No car out there can compare to that big monsterous thing.

My Uncle always had the coolest "toys". I think he liked accumulating things. He had speed boats, and cigarette boats and house boats, jet ski's and even a floating house....yup a house that floated on the water that was NOT a boat,. And cars....oh the cars that man had... We used to go visit him and go tubing, and boating. He had an amazing house on a mountain lake.

My Uncle was amazing. He was my first crush (even though I knew I could never marry him, but as a child I adored him), he was my favorite person on this earth (after my parents). My Uncle was young. He was cool. He was fun. He was creative. He was loving.

At my wedding, to my first husband, my oldest son was just an infant and he was so fussy during the ceremony. My Uncle, who never had a children of his own, carried my baby around for the whole ceremony calming him and soothing him. He was amazing with my son.

I love my Uncle. Unfortunately I will never be able to tell him that again. My Uncle has been gone a year, and we weren't informed. My heart is broken. I feel we have had the right to grieve stolen from us. But I will always remember the fun times we had with him and his "toys". I will always love him.

Who is one of your favorite Authors and why? #Challengetowrite

Who's my favorite author???? Well who's not is a more accurate question. I love to read. I read just about anything I can get my hands on. If I have to pick just one it would be John Grisham. I have read, and owned, every book he has written. I don't know why, but from the first time I picked up my first Grisham book, I was hooked.

I love everything about the way he writes. Grisham has a way of making you feel like you are right in the story.  He writes about law and justice. At one time I thought about becoming a lawyer just because of Grisham's books. Needless to say, that didn't happen.

Even the books that he as written that I didn't think I would like (A year with out Christmas, the Theodore Boone series) I really enjoyed.

My second favorite author....E.L James of course!

Mornings. Day 2 #Challengetowrite

Good morning....NOT

 

Inevitably my day is going to start with the alarm clock. Oh how I hate the sound of a ringing alarm clock. Monday thru Friday my day starts with this annoying sound. Oh how I long for days when my day would start with a nice slow wake up to the sound of birds singing and a small bit of sun shining thru the curtains. But nope. Now that I have school aged kids we have to be up at a set time each and every single stinking day...7am

 

 I am not a morning person. Honestly, I loath mornings. Morning is my arch enemy. If I could sleep until 10-11am and then start my day, I believe I would be very happy. I tried to get into the whole waking up 30 minutes before the kids have to be up so that I could enjoy a cup of coffee and some quiet time, but it didn't last very long. A) because coffee isn't my favorite drink (although I do drink it and I absolutely LOVE my keurig) and B) again....I hate mornings.

 

I have this very bad habit of hitting the snooze button....a lot....like so many times that if we didn't live 2 minutes from school (via car) my daughter would be tardy daily! But I do manage to get her up and ready for school and at school at HER requested time (she is at the age that she wants to be to school a little early to talk to her friends). Then I drag my butt home to wake the other monster and get him ready (much more of a battle). He goes to school an hour later than she does.

 

I would like to say that now I get to go back to laying in my bed for a while, but that seldom happens. The joys of a working mom. After I get them off to school, I take the husband to work and head off to my job for a few hours.

 

Now weekends....I live for weekends. I get to sleep in (haha if you call 8am sleeping in) and don't have the alarm clock witch beckoning my from my sweetest dreams. But, without fail, on most weekends I wake to the sound of my husband asking "can we go to the beach?"....I roll over and act like I didn't hear him and am still asleep...hehehe

What is your favorite thing about Summer? #ChallengeToWrite

There are so many things I LOVE about summer. I mean, really, what's not to love??? Sun, warmth, no snow, no bundling up in layer upon layer of clothing....did I mention WARMTH! LOL I know, I know you are thinking "hey wait a minute you live in Florida, it's warm all the time". Yes I live in Florida now, but until recently I lived in Upstate NY and up there you LIVE for the summer time.

Summer time in Upstate NY ment and end to the fight against snow-banks and finally getting your feet out of those big, hot nasty boots. It means warm days at the local lake and cool nights to sit around the bonfire. Summer in NY is cookouts and boating...and nice adult beverages with friends (big SMILEY face here).

I am quickly learning that summer in Florida means something entirely different. Summer in Florida means sand, sea and surf!

Yes that is my car loaded down with the kids and husbands surf boards
 

My husband grew up here in Florida so naturally him and my kids had to have surf boards. Now I am not much of an "ocean" person. Don't get me wrong I love the water. But the idea of being in the OCEAN in water that is at least to my waist (if not deeper) with things that can take my leg off (sharks duh!) or sting me (jellyfish) just really does nothing for me. But my kids had other ideas. And of course they took to the water like fish and have no fear of the relentless waves or things that may want to eat them (yes I know it's an irrational fear, but we are all allowed to have them), and yes I do sit and watch the water for the elusive fin to appear. 

 

 

 

 

 

So every weekend we load up the car and head to the beach. I love the beach. Not the sand that ends up everywhere, but I do love going to the beach and just sitting there doing nothing for a few hours. However, going to the beach with "surfers" can be boring for a non-surfer. My wonderful, attentive (said with a lot of sarcasm) husband gets the kids all set-up with their boards and then paddles himself out and we don't see him for HOURS! No I am not exaggerating. He will be gone for hours, leaving my with these two fish-kids. Now, my kids have yet to master the art of surfing. They are learning, but they still need a lot of hands on teaching. So when they come to me and want me to help them I have to laugh. I HAVE NO CLUE! I refuse to get on a board. 1. because I am the absolute biggest klutz on the face of the earth, and would either break the board or my neck(I am dead serious) 2. I DON'T GO IN ABOVE MY CALFS!(remember those sharks and jellyfish) 

 

 

 

 

Sitting at the beach can get boring even for kids. You have to make sure you bring something to do or they have a very good imagination. My kids can find just about anything to complain about. But when we are at the beach I seldom hear a complaint. They actually get along with each other! It's amazing. They can play in the water for hours, swimming, jumping waves or playing with their boards. They build sandcastles, look for shells, and bury each other in the sand. You'd be amazed at the places you can find sand once you leave the beach. Especially if you spend a few hours burying your sibling (I think they do this to each other as punishment).

So, now that I have just rambled on and on about the beach I guess you can say my favorite thing about summer is....the beach.

 

What's your favorite thing about summer?

Ivory 2in1 Hair & Body

 

I received a bottle of Ivory 2 in 1 bath and body from Influenster to try. I am a big Ivory fan so it was nothing for me to give this product a try. The first day I got it I handed it to my daughter when it was bath time and told her to use it on her whole body...including her hair. She looked at me like I was nuts! And then had to read the bottle to make sure it has conditioner in it (which it does), before she would agree to give it a try.

 

She came out of  the shower with a smile on her face. She said "I only used a little bit on my hand to wash my hair and it worked so good". So then we had to do the sniff test (all mother's know you  have to do the sniff test after baths to make sure they wash good ). She smelled so clean and fresh, from her head to her toes. (she is shy and would not allow me to take a picture of her soapy head).

 

We have used many body washes over the years, I am always looking for a fresher, cleaner smell. I have fallen in love with this Ivory 2 in 1 hair and body wash. I love how it makes bath time with my two pre-teens so much faster.

Give me NICOTINE!!!! No wait dont't....yes do....no don't...

This is off the topic of what I have been posting about lately but I feel the need to just throw it out there. Last Friday, at 7:30pm, I smoked my last cigarette. Deciding to quit right now was not a decision I made, more like I was forced to make it. Plain and simple, we are broke. It came down to getting milk for the kids or a pack of cigarettes for me. A no brainer, right? However, it was not an easy choice for a smoker of...oh at least 15 years.

I started stealing my mom's smokes when I was 13. Yeah I started young. At that point it wasn't an everyday, multiple times a day thing. It was a kid thing. A rebellious teenage thing. I guess I probably started because I saw my mom doing it. I had a few other friends who would smoke too, but the majority of them didn't.

By the time I was 18, old enough to buy my own cigarettes, I was hooked. I had a job, so I was supporting my own habit, no big deal right. I know all the arguments about cigarettes aren't addictive, blah, blah, blah. But I am here to tell you that they are. I don't know what it is about smoking, but once you start it is so hard to stop. I don't know if it's the nicotine, or what, but you crave that puff. You need it to function. You feel like crap if you don't get it. And it doesn't matter how many times people tell you you need to quit, you just can't.

I did manage to quit with each of my pregnancies. I did see my unborn children's life as more important than that puff of nicotine. But within days, or weeks of each of their births, I was smoking again.

 I also managed to not smoke in the house....most of the time.  And let me tell you New York in the winter...sucks to have to stand in the cold garage and smoke. I don't know how many times I stood out in the rain and cold; how many times I missed a song or a dance; how many times I just plain missed out on something just because I needed that smoke.

But then there were the times that smoking kept me sane. It helped calm me down when I was an utter wreck. When I my best friend died. When I was going thru my divorce. When I  my son was diagnosed with Cystic Fibrosis. When my husband was in Iraq, fighting for our country and I was a single parent. The first time my son was hospitalized. I was alone in Denver, CO and I was upset. I would go out to the smoking area at the hospital and there were other mom's there to talk to. Smoking brought us together, helped us help each other. Smoking gave us each time away from whatever was going on upstairs in that hospital that was too much to deal with, it gave us short term friendships with people who knew what we were going through.

I noticed that I did most of my smoking while driving. Yup, major stressor. As soon as I would crank the car, I would light up. It didn't matter what time of day it was, what the weather was like or who was in the car with me (yeah I didn't smoke in the house, but did all the time in the car). And because I am such an on-the-go, never-stopping, kid-hauling momma, I smoked A LOT. 

So while the battle is still not completely won, I am working on it. The true test comes tomorrow, payday, will I buy a pack or not? I would like to say that I am not going to be buying any cigarettes, but I know me. I want that nicotine so bad! I want to feel that cigarette between my fingers. Yes, that's right, it's not just the puff, it's the comfort of the feel of the cigarette in my hand.

If I could just find something to keep my hands and mouth busy.....maybe I will be buying a HUGE bag of tootsie pops instead.

Shake, shake, shake....shake, shake, shake......shake your lungs up

Now we come to the wonderful world of treatments. We spend about an hour of our day on treatments right now. I know that doesn't seem like much, but when you are tied down to machines shaking you up and blowing smoke in your face it seems like forever. And when Zach gets sick, treatment times go up.

Most kids do some sort of chest therapy, either with a vest, a pep, acapella, or via hands on chest percussion.

This is what the vest looks like. It is attached to an air compressor. When this machine (there are other types) is turned on you have to set the frequency to which it vibrates (slow for fast), the amount of pressure it vibrates with (lighter or harder) and how long it goes for (we do 20 minutes at a sitting). It is recommended that you start at a lower frequency and pressure and stop every 5 minutes to do huff coughs (will explain those later) and work your way up the recommended frequency and pressure.

Zach rocking his camo vest
 

As you can guess, it is not always easy to haul these things around. Our machine weighs about 20 pounds, it's heavy and bulky and a pain in the butt to carry on trip. So we opt for a simpler method of chest therapy when we travel or when Zach goes away to camp in the summer.

This little guy here is called an Acapella. It is small and light-weight and fairly easy to use. This is what Zach takes when he goes away. They breathe through this device and it causes vibration on the inside of the lungs. Zach breathes into this device for 10 minutes. It's still not any fun for him, but it is easier for him to deal with because he is not tied to one spot.



On top of the fun of the chest therapy we also spend a lot of time doing inhaled treatment via the nebulizer. The nebs take just about as much time as doing the vest. Most of the time you can do the nebulize treatment while doing the vest, which cuts down on treatment time. There are a number of different medications they take through nebulizers. Zach is currently on Pulmozyme and albuterol.  Pulmozyme is a very expensive CF medication that helps break up the mucous in the lungs. This is one of our most important medications. In the past we also had to take antibiotics (to fight off pseudomonas) via neb and a hypertonic saline solution. Zach hated the hypertonic saline nebulizer. He said it burned his lungs. I have heard this from a lot of people. We found a great way to get hypertonic treatments with out actually doing them....SURFING! Being in the ocean is like getting a natural saline treatment, and its fun! So now we use the ocean as a treatment too.

While treatment times are no fun, they are an important part of a CFer's life. Without the daily treatment regime the junk in their lungs would just sit there and bread bacteria. It would cause infection after infection after infection.  It is a daily battle in our house to get Zach to do these necessary treatments. He absolutely hates them. They take too much time, they hurt, they're boring.....and on and on. He has been lucky so far that he has only had to battle psuedomona's infections a couple times, but he does have a severe MRSA colonization in his lungs. We battle flares ups on a regular basis. If he would just learn to do the treatments he needs to do, we would most likely have less. It helps that he is in the ocean on a regular basis, but it's not enough to just break the junk up, it needs to be cleared out and that is what he is missing.

Children's Mucinex Multi-symptom Cold review

                                     http://smiley360.com/1259999.cfm   

Nothing is more annoying than that nasty cold that starts going around the school. You know it's gonna hit your house at some point because, lets face it, kids just don't know how to cover their mouths when coughing or sneezing, nor do they wash the greatest. (yeah yeah I know we all want to believe our kids do the right things, but let's face it, they don't).

I have been dreading the cold this year because it has been a nasty one. So when I got selected by smiley360  to receive a free Children's Mucinex  Multi-Symptom Cold to give  a try, I jumped right on the chance.  And of course, no one in my house got sick for months. Go figure, I finally have medicine on hand to treat the nasty cold and my kids are uncharacteristically healthy.

So there it sat, on my shelf, for weeks. Just waiting for the first cough, or stuffy nose. Finally, after weeks, my daughter got the sniffles and a little cough. So I finally cracked open the bottle of Mucinex. With it's wild berry flavor I thought she would like the taste, not that any medicine tastes good. Well she didn't care for the taste. However, the medicine worked like a charm. With just the first dose her cough was under control and her congestion had started clearing. By the end of day three, she was almost completely cough free and her congestion wasn't nearly as noticeable.

Make sure you read all the warnings on the bottle before you start using Mucinex on your kids.

                                                                                

 

He's on what???? A look at our home pharmacy

Medication therapy varies greatly among CF patients. Some CFer's take a ton of medications, while others takes few. Our household pharmacies contain a wide combination of antibiotics, allergy medications, vitamins, nasal sprays, pancreatic enzymes; among many other things. The medications to keep our family members healthy cost us a small fortune. One of Zach's medications cost $1,100 a month! At one point we figure out the cost of his monthly medications. I must say we floored when we reached over $8,000 a month. And these are only preventative medications. There is no cure, there is nothing out there, yet, that is going to guarantee that he doesn't get sick, that he doesn't culture a life-threatening bacteria (like Pseudomonas) that will hospitalize him for weeks. All these med's do is keep his symptoms at a minimum and hopefully keep him as healthy as possible.

 

The other thing about medications is that these people have to watch what kind of over-the-counter medications they take. If your kid catches a cold, gets the sniffles, is coughing up a storm you reach for the cough medicine, right?  We can't. Our CF patients can't take medication that has cough suppressants in them. We need our CFer's to cough, they have to cough up that crap that is in their lungs or it leads to massive infections.

 

In the 10 years since Zach's diagnosis he has been on enormous amount of medications. These have changed over the years, as he has grown, or medications have developed and changed. In general, he takes about 25 pills a day (when he is compliant). Here is a look at what he has been on through the years:

 

 

 

 

Currently taking:

 

Creon: this is a pancreatic enzyme. He has to take these to digest his food. He takes 15+ of these pills a day!

Ventolin: rescue inhaler

Singular: cuts down on allergy symptoms

Zyrtec:

Vitamin E

Vitamin D

Aquadek: CF specific multi-vitamin

Vitamin K

Albuterol Nebulized

Pulmozyme Nebulized (will go into these more in treatments)

Prevacid

 

 

Taken in the past:

 

Doxycycline: to help keep MRSA under control

Advair

Xopenex



Hyper-tonic saline solution nebulized

Periactin: to help gain weight

Inhaled Tobramycin: to fight off pseudomonas infection

Various IV antibiotics

 

 

 

(10 day IV antibiotic therapy at home)

 



There are so many times that I have to laugh when someone complains about there kids being on an antibiotic for an ear infection, or needing Benadryl for seasonal allergies. And I don't do this meanly. It just makes me giggle. And when asked why I just say "you should see our medicine cabinet....or more accurately our home pharmacy".

 

You'll notice that a lot of Zach's meds are meds you hear about every day. Multiviatmins, allergy meds, asthma meds. Like I said none of these are a cure for CF. All of these help keep Zach's lungs as clear as possible, or help his digestion.

 

Our hope is that one day, and hopefully soon, Zach will be down to only a couple of medications. And that those medications will be a cure, or as close to one as we can get to keep him alive. There are medications in the works right now that "fix" the gene defect, that show great promise. Yes, this will be a pill they have to take everyday, but it's the best we have to look forward to for now.

 

 

The genetics of CF

By now you have read our diagnosis story, but you may be asking how we didn't know that having a child with CF would be a possiblilty for us. Well it's easy to not know when no on in your family, that you have ever heard of, has been diagnosed with the disease. Zach was the first, and is still the only, known person (young or old) in our family with CF.

 

That just goes to show how rare of a disease this is. I suppose there could be other older family members with CF who were just never diagnosed. People who have been living with various other conditions or who died from respiratory complications and it was never thought that they could have CF.  I bet if we look in our family history we will find stories of babies who died of malnourishement or what was thought to be pneumonia when in fact it was CF. Until the 1950's there wasn't a name for this disease so it was not diagnosed. And even once they discovered what it was most patients with CF didn't live to attend elementary school (we will get to more statistics on another post).

 

So how prevalent is the CF gene?

  

 

That's approximately 1 in every 31 Americans!

 

Looking at my family: I have 2 siblings. Together we have 7 kids. 

 

My dad had 5 siblings, counting me and my siblings that makes (roughly) 17 cousins (I may be missing 1 or 2). Now most of us have kids. Now your looking at 45 second cousins. A couple of those have had  kids...I think there are 3 3rd cousins (that I know of). So that makes a total of about 71 just on my dads side of the family! 

 

You add to that my mother's side of the family. She has 3 brothers. My uncles have fathered 7 kids (that I know of). Of those cousins I only 1 has had 2 kids.

 

Now we are up to a total of 80 family members (and that's not going back further than my parents) Is it really possible I am the only carrier in my family??? Statistically speaking there should be at least 1 more. Yet, my boy is the only one with the disease and my daughter is the only carrier we know of.

 

Then looking at my husbands family: He has 2 siblings. Those 2 have a total of 3 (biological) kids.

His father has a sister. She has two children. They now have 3 kids. (husbands family is a lot easier to keep track of LOL)

His mother has no biological siblings.

Just going back this far it makes sense that he is the only known carrier (statistically speaking)

 

Now to get to the insane part. I mean how likely is it for a woman from NY and a man from FL, both not knowing they carry the CF gene, to meet, fall in love, get married and have kids??? I'm sure if I was better at math I could come up with some crazy equation to figure it out, but lets just say it's not a likely as you would think. Pure luck I suppose you could say. It gets better.

 

Once those 2 people meet, fall in love, get married and have kids there is only a 25% chance their kid will have CF. 25 PERCENT! A 1 in 4 chance. Talk about luck of the draw. Every time those parents get pregnant there will be a 1 in 4 chance they will end up with a CF kid.

 

Since Zach's diagnosis we have made sure that all our family members make sure to tell their doctors that there is a family history of CF when they are planning to have children. We still don't know of any other carriers in either of our families. It's crazy.

 

 

 

What is Cystic Fibrosis?

Cystic fibrosis is a life-threatening genetic disease that causes mucus to build up and clog some of the organs in the body, particularly the lungs and pancreas. 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 does CF affect the lungs?

 

Normally, the healthy CF gene makes a protein — known as CFTR (Cystic Fibrosis conductance Transmembrane Regulator) — that is found in the cells that line various organs, like the lungs and the pancreas. This protein controls the movement of electrically charged particles, like chloride and sodium (components of salt) in and out of these cells.

 

When the protein is defective, as in cystic fibrosis, the salt balance in the body is disturbed. Because there is too little salt and water on the outside of the cells, the thin layer of mucus that helps keep the lungs free of germs becomes very thick and difficult to move. And because it is so hard to cough out, this mucus clogs the airways and leads to infections that damage the lungs

 

 

CF doesn't just affect the lungs

While most of the discussion about CF revolves around the lung issues our CFer's have that is not all the problems they can face. (I must add quickly that I am having a hard time not saying "CF kids" as that is what I deal with a CF kid, but I want to include all CFer's in my posts not just kids). CF is a whole body issue. It affects so many organs due to the underlying gene mutation that causes it. (which I just realized I think I missed posting about that whole thing so I am going to go into that later). Here are a few of the possible other conditions that can affect CFer's. (our experiences will be under the pics in purple)

Due to all the lung problems Zach had as an infant and toddler he already has slight clubbing in his fingers and toes. It isn't to the point where it is extremely obvious to the untrained eyes, but will get worse as he gets older and has more problems.

 

 

 We have been dealing with "acid reflux" all of Zach's life. To help deal with it he has been on prevacid for years. When he was a baby he would only eat really bland foods (oatmeal was his favorite) and as he's grown up he has become a very picky eater and is very hesitant to try new food for fear that it will bother him.

 

 

 

Ah....malabsorption...this is the best part of CF (NOT!!!) This was my second clue that something was just not right with my son. This is why so many CF babies deal with Failure to Thrive. Our kiddos pancreatic ducts are clogged full of mucus and can't secret the enzymes needed to digest their food....which also causes some very, VERY disgusting foul smelling poops. This is also why my little man looked like an Ethiopian baby when he was little(and that's what we jokingly called him)

 

 

 

This has been one of our biggest battles. Zach has had 2 sinus surgeries so far to have polyps removed and to clear our his sinus cavities (it's like a rotor-ruter they put in there to break up the hardened mucus)I think the kid has only been able to smell good for a total of 6 months or so of his life. It seems we are always dealing with blockages in there. He loves it when they clean him out because he can smell all the wonderful smells...and some bad ones he wishes he couldn't smell!

 

Those are the related problems we deal with at the moment. With each annual set of blood work and chest xrays we worry about finding any of the following.

 

We watch very closely for this. Zach battles a lot of respiratory infections as he has MRSA in his lungs, so once the coughing starts we are on the look out for hemoptysis.

This is one that I dread having to deal with. Even at 12 Zach knows he wants to be a father. And he will be able to be, it may just take more work.

 

Zachery's liver enzymes are monitored very closely because he has had funky test results in the past.

These are a few of the complications that can occur with CF. Because the lungs are so greatly affected it also can cause heart problems (like pulmonary hypertension). A good portion of CFer's have to have lung, heart and liver transplants. As you can see, health care for these people is a HUGE issue. But when you are blessed with a child like our Zach, each day you are given is a gift and you wouldn't trade it for all the healthy kids in the world.

**I apologize for how small the graphics are in this post. For some reason I can't get them to resize.