Alayna 16 Month Post

Really? You are 16 months old today (2/17) Alayna.  I am just glad I am not saying you are 16 YEARS old yet………I know that day is going to be coming faster than I or your mommy could ever imagine.  We still have a lot of growing up to do in fact all of us.  Your mom and I both are still learning this parenting thing.  I have been told by fellow parents including your grandparents that you never stop learning how to be a parent.  Just like how we both will never stop worrying about you.  So get use to it Alayna! I think we could just say you are loved more than you ever would know. 
 
Since I didn’t update on your 15 month birthday we will get you all caught up with what is going on in your life.  To start, let’s go backwards and talk about your 15 month doctor appointment.  Oh I think we will leave out the fact that you have had off and on diarrhea for basically the past month.  The pediatrician believes you have toddler diarrhea which basically is unexplained diarrhea.  Just for the record I get to put whatever I want in these posts about you Alayna.  When you read them one day I am sure you will be fully embarrassed.  But I said I was going to get everyone caught up on what you were doing.  What is even better about all this lovely poo business is that you never have diarrhea when daddy is home so we can all laugh at mommy who gets to clean it.  It does seem that your digestive systems is returning back to normal, FINALLY.  Thank goodness!!!
 
Let’s get back to the 15 month doctor appointment.  So would you be surprised to read that you are still in the 97^th percentile for height? Yes, you are one tall lady 32 5/8 inches. You are know more than half of your mom’s height.  I think that you will be taller than mommy sooner than anyone thinks.  But remember this no matter how tall you are you still have to listen to BOTH your parents.  As for your weight last month you came in at 22 pounds and 7 ounces.  I believe that was in the 25-50^th percentile.  Mommy and I both feel you look way older than any 16 month old we have ever seen.  In fact, when we go to stores or appointments and we see another child who looks around your same height and build they are always older than you. 
 
Your vocabulary is changing quickly.  I guess we can officially say you have learned your first curse word. It is more indirectly being said by you and you have since removed it from your vocabulary! Mommy and I are both pleased you have removed this word.  So how did you start saying the word SHIT! Yep you said it.  I think it all started with when you learned the word shoes and socks.  Soon after that you just kept repeating shoes and socks but the words came out as SHIT! Honestly, it was really hard for us to not laugh.  I am just really glad you have not learned how to say truck.  I have heard that word sounds like fruck! 

You do love to say the word Hi and bye and a few others now.  Unfortunately, you have learned the word NOOOOOOOOOO!!!! Yes that is basically how you say it.  We ask you questions where you should be saying yes to.  For instance, Do you love mommy and daddy? NOOOOOOOOOO is what you say. Do you want to eat? NOOOOOOOO.  Do you want to go take a nap? NOOOOOOOOO.  I know you do not even understand what you are saying yet. 

There is something that I really love about this age you are at.  You love both mommy and me more than anyone else. The greatest feeling for me is when I come through the garage door and there you are waiting with the biggest smile on your face.  You look at me and say Hi and smile.  If I am not having the greatest day that pure 10 seconds of joy you have in your eyes basically wipes any awful 8 hour work day away. 

As for your eating habits.  They are still kind of at a minimum.  You do love grilled cheese or toast n cheese as mommy calls it.  What do you call it?  You love to eat basically any noodles and you still think eating baby food is good.  Currently, you are not that big of a fan of eating meat.  We do get you to eat a few pieces here in there but it has to be disguised with either a gravy, sour cream, or ketchup.  Right now your favorite meat is chicken from the chicken nuggets.  I think what you really do not like is how meat is tough to chew.  You would rather prefer to eat already ground up baby food from gerbers.  Oh well whatever works is fine with us. 

I can tell you that you are one smart little toddler already.  You know what the tv controller is used for.  When you get a hold of the tv remote you always end up dvring (recording) tv shows.  Your mom and I will ask who recorded the customer information channel, or who recorded ESPN?  At least you have yet to order any graphic tv shows or dialed 911.  Speaking of how smart you are you already know how to sing Twinkle Twinkle Little Star and Row Row Row Your Boat.  You even know what the stairs are for.  Not to mention how you basically climbed up the stairs since mommy and daddy forgot to close the gate.  Ooopss!! Luckily, you waited about half way up the stairs. That could have been a recipe for disaster. 
 
How could I forget to mention you love sitting on my lap as I do my airway clearance (vest and hypertonic saline).  I think you just love the fact that you get to go up the stairs and sit and watch the computer for 30 minutes.  When I say let’s go upstairs you race to the staircase and shake the gate to death.  As you climb the stairs all by yourself, you head into daddy’s office where all the lovely CF equipment is.  You wait as patiently as you can to sit in my office chair.  As soon as I turn on the computer and visit the youtube website you go crazy.  I think it’s crazy how you know that website is where you see all your songs and Barney videos.   But before we get the songs going you watch every single move I make putting on my vest getting my neb ready and even hooking myself up to the vest.  I am starting you out very young learning about all this cystic fibrosis stuff.  You will come to see it is just a part of daddy’s daily life.  CF is just along for the ride with us Alayna.  I can honestly say you are the best vest buddy I could ever ask for.  I know that one day you will not be sitting on my lap so I am going to cherish these moments while they last. 

Yesterday, you decided to show us how smart you are.  You found a paper towel and looked at and then spit in it.  Yes, but before you spit in it you cleared your throat. How in the world do you know from only sitting with me for less than a month that I spit my lovely mucus into a paper towel?  I truly think you are wiser beyond your 16 months.  Now we will ask you what do you do with a paper towel?  We hand the paper towel and you look at it and spit in it.  Then you fold it up just like daddy does.  I guess this is what happens when your daddy has CF. You seem to pick up on things very fast.  Pretty smart little kid already.

That is all I can think of right now. But as you can read you are changing and growing up way to fast.  I am just so thankful I am writing your 16 month post instead of your 16 year old birthday.  I have been told that your 16^th birthday will be coming in no time.  For now both mommy and I will cherish every single moment with you!

 

WE LOVE YOU Alayna!

 

 

 

Best smile ever!

 

Alayna you look older than 16 months

 

You decided you wanted to get in the trash can!

 

One of your favorite movies Happy Feet 1&2

 

Big smile after eating all the Valentine Day icing from the cake

 

 

Big girl sitting facing front now!

 

 

Random Update......Kalydeco, CF Meds, Valentine's Day & More

I have done it again, MIA from blogger.  A friend of mine who doesn’t really use Facebook but does read my blog said I have been way behind in blogging.  After looking at my last post it was dated 1/23/14.  It has been just about a month since I last put all my thoughts into words.  In all reality I have been gone from here not because I have nothing to say because trust me I have plenty to say and share with everyone (those that are my friends on Facebook would probably agree).  I have been SO BUSY living life!

Before I start talking about me which I know I do a lot of I want to give an update on my girls.  Here is an update on Alayna and Maria.  I didn’t get the chance to do a monthly update on Alayna for her 15 months.  In fact, tomorrow will be 16 months!!! Where does the time go…………She is growing up faster than any of us would have ever imagined.  Alayna is developing into her own little person as we speak.  She is saying a few words.  As for expressions she definitely can tell us when she is NOT happy.  But most of all she is a big time mommy and daddy’s girl.  She loves us both! One of my favorite aspects of being a dad is when I come home from work even if my day is terrible she can put a smile on my face as soon as I get in the door.  I think her favorite words is Hi! She will say Hi and give me a big kiss.  I will save the rest of the updates until I make her official 16 month post. 

Now for an update on my lovely wife!!  Maria is my rock and she keeps not just me going but this entire family on track. I also do not know how she puts up with me day in and day out.  One of these days I will write about how I am so far from a perfect person. Or wait maybe I should have her write that post!!

 If it wasn’t for Maria I would have a lot more difficult time doing basically EVERYTHING!  I just want to thank you for helping me with the neb sanitization and every other thing you do to keep me and this family on track! It’s just another part of our day, CF ;)  LOVE YOU

When you have a baby it’s a life changing event and even till this day we are still getting use to our new lifestyle.  I can tell you having a child flips your life around 180 degrees.  What makes it easier is having a fantastic wife who understands about your health and can basically change anything on the fly such as adapting to every day changes that occur.  

We did celebrate Valentine ’s Day over the weekend.  It was just Maria and I at lunch and then we went to few stores.  Those are the things you look forward to on Valentine ’s Day when you live a crazy life like this.  I guess I should say our life isn’t crazy it’s just NOT enough hours in a single day to get anything done.  I will also say for those of you who have toddlers or who recently had a child it’s good to take your spouse out.  It pays dividends in keeping you both sane!

Ok on to the boring John stuff……I don’t know where to start so I guess I will start what took place after my doctor appointment.  I mentioned how my clinic was going to look into getting Kalydeco for me.  I sent emails to my  doctor and my research coordinator to show them why I believed Kalydeco would work for me.  Apparently, I thought we were going to see if we could fight for off label use but in reality the research coordinator was checking to see if I would qualify for a clinical trial at my clinic.  She sent my gene mutations to Vertex and they said NO dice.  Oh well I am use to climbing a mountain for the things that are valuable to me.  It just kind of really SUCKS that I know and a few others know in this community know that Kalydeco will work for my gene combination.   

My culture results did come back with the usual suspects that I culture.  I have the love staph growing NOT MRSA and the lovely achromobacter xylosoxidans.  Since my PFT wasn’t in my baseline range I pushed for antibiotics. I have not been on a single antibiotic in over a year.  I know it’s fantastic and hoping I start another streak here of not needing an antibiotic for a while.  We usually do not treat me unless I have more symptoms or have complaints.  Well I guess my complaint is my PFT is lower.   I have now been on Doxycycline for my staph and inhaled meropenem for my achromobacter.  Meropenem is usually done via IV but my dr. believes we can avoid IV’s if we can inhale some meds.

I had a really good experience the first time I ever inhaled meropenem before as it killed my pseudo a year ago.  After that 30 day stint of inhaling this med I have not cultured pseudo in a year.  So I am all for inhaled mero and all for avoiding IV’s.  The only thing is I recalled from last go around at this mero made me feel like complete CRAP the first few days.  I honestly felt like I had influenza and this time around it was no different.  I know mero is a nasty drug so I think my body just had to get use to it the poison that is.  This time around it lasted for about 2 days.  I was a trooper and went to work feeling awful but it was tough. 

How could I forget this in between my dr. visit and starting these drugs I completed a major milestone.  I have been compliant for 365 days on 2/1/2014!  I am still compliant even with adding another 30 minutes of neb time with this meropenem. Eventually, I will make an entire blog post about compliance and what it means to me.  But until then this will do.  Why did I change all this compliance stuff in my life when in all reality I was basically very compliant?  It all boils down to one thing and that is why couldn’t I be 100% compliant.  Before this I was about 97-98% compliant with all my treatment which is still fantastic but for me it’s a no excuse attitude when it comes to treatment time in this household.  What really changed me was when Alayna was born.  It took me a few months to get this mindset that 97-98% wasn’t going to cut it.  If I want to put my best foot forward every day that starts with me and my health.  One thing that NO one can ever take from me or anyone is my dedication to my compliance.  I will never ever have to hear John well you weren’t compliant enough and that is why you are sick.  For me my eye is on the future.  I have big plans in my life to be here on this planet as long as I can to see Alayna go to school for the first time, to witness her get her driver’s license (it’s less than 15 years away YIKES!!), watch her graduate high school, go on to college or whatever it is that she chooses to do after high school, walk her down the wedding aisle, be the grandfather to her children one day, and last but not least be there right along Maria’s side to witness all of these milestones.  As you can see I am driven to see these days and if I do not put myself in the best position to do that I may only have myself to blame.  I will end this compliance paragraph with a quote from my CF mentor Anne ( I LOVE you to death Anne) “Treatments are like money in the bank! Put the time in today and it will pay you back ten fold in the future!” I think that is how the quote goes.  Correct me if I am wrong Anne.

As I love to talk about Kalydeco here is another update on that magic pill.  There is a clinical trial that I may be able to get into to try Kalydeco for 2 weeks (6 week study on the west coast and you are responsible for all travel there is no reimbursement).  Yes, I know 2 weeks that is really not enough time to see if this magical pill works as I have friends who basically were sick with cold like symptoms for up to a month after starting this.  I guess the bigger question is do I even qualify for this study.  The inclusion criteria are not to clear.  But I do fit based on my one gene mutation 3849 as it’s a residual functioning mutation. It states the trial would like a minimum sweat chloride level of 55. For me I do not fit that description.

If you have ever read my CF diagnosis you will remember that I passed sweat tests.  I wasn’t diagnosed with CF until they did the cheek swab and sent my DNA off to a lab where I was found to have DF508 and 3849 + 10 Kb C->t.  The 3849 gene is known to produce normal sweat tests and I fit that description to a T.   I called my CF clinic and also my genetic counselor.  I had two tests done within a year from each other back in 1995 and 1996.  The tests produced numbers of 31 and 28 respectively.  A sweat level below 40 is a normal sweat level. Sweat tests should NOT change through the years as your DNA is basically responsible for this sweat level!

 So why do I have cystic fibrosis???  Based on the sweat chloride I have I shouldn’t have this disease but I know for sure that I have CF.  Not to mention as a newborn a few months old I was tested for CF and again I PASSED my sweat test. My lungs and health have suffered from being diagnosed later in life. Sometimes I do wonder what my lung health would be if I had earlier diagnosis.  I’m thankful we now have newborn screening for CF. Sweat testing is the golden test for CF but in my book it’s basically not a true valid way to tell anyone they have CF.  If even one child slips through the cracks of not being identified with CF its WAY TO MANY! In fact I have some very good friends who have a child in my same boat passing sweats but the child has CF!! I will expand on this topic in a later post.   

Back to the Kalydeco study………Since I have low sweat chloride numbersthat means I have working CFTR at the cell surface at least in my sweat glands.  This should mean that there should be some good working CFTR in my lungs as well.   Kalydeco will help with the working CFTR at the cell surface! That is why I NEED Kalydeco to prevent further declines!  There is a lot for me to think about with this study as it will require lots of money for traveling but also will I even see a benefit in two weeks? If I try to submit these results to the insurance to get Kalydeco off label I know they will throw at me that you were only on this pill for 2 weeks so why should we cover a pill that costs $300,000 a year!!! Ughh decisions decisions.

Thanks for reading and the next post will be about Alayna’s 16 months. I PROMISE I will get that post up this coming week Alayna. Daddy is slacking!

CF Clinic Appointment Update

The word is out at least on other social media outlets that I haven’t been feeling like myself this past week.  This all started during the night when I was awoken by a coughing fit.  To all of my CF friends, you understand how ANNOYING and completely out of the blue these can be.  I haven’t had one of these coughing fits in who knows how long maybe a year!! I am thankful that this is not the normal for me.    

But I did forget what it was like to have one of these episodes. I could feel all the mucus laying in there and rattling around.  My first initial thought was what is all this about.  I am not sick and this is so UNLIKE me.  I did a bunch of huff coughing and the mucus was thinner and lighter in color than my usual sputum.  So does this mean something new is growing in there??? I have had this happen before and I think that Staph was the culprit at least what I can remember. Not only was I privileged to experience this for one night, but I had 3 of these lovely nights.

After a recommendation from my cyster friend Beth, I got out my albuterol puffer before I did my HTS treatment.  This seemed to allow my airways to open up more to cough out that thin mucus. I can say that I have not had a coughing fit since last Thursday night thanks again Beth!

I decided to make my CF clinic appointment due to all this coughing at night.  This past Monday Maria and I made the trip to clinic.  I wanted my doctor to see my thinned out mucus and get this cultured to see if any new bacteria is growing.  In the car, I did cough up some mucus and thanks to my CF MENTOR, Anne I did remember to bring a Ziploc bag to cough this into. Does anyone worry they will not be able to produce any mucus on demand??? This Ziploc bag or sputum cup idea will help us! Thanks Anne!!!

On a side note I suggest every one in our community find someone who you feel is your CF mentor.  I am beyond thankful to have my second cousin Anne as someone to look up to.  She has been through some wars herself including a double lung transplant. Soon many of you will finally get the chance to “meet” her on blogger.  This will be something new I plan to rollout. More on that in a later blog……………..

Back to the appointment…. My doctor said my lungs sounded good. There was no rattling or anything like that.  But again all the rattling was at night.  I usually get that same report no interesting findings which is fine with me.  My weight is always good in fact one of these days I am going to have to drop a few pounds!  Then it was time for my battle  with the PFT. 

I knew coming into clinic that my FEV1 wasn’t probably going to be the greatest but I still thought just maybe I could see the same FEV1 from last clinic.  Even at my last clinic my FEV1 was lower than usual 69.  Into the PFT chamber I went.  First blow is in and it’s a whopping low of 62. UHHHHMMMMM NO thank you PFT machine.  I hate seeing a number like that.  My baseline for the last 2 years has been a range of 68-74.  After a few crazy thoughts run through my head (more on those thoughts in another post) it was time for blow number 2.  My second attempt came in at 64.  What the heck is going on??? At least it’s moving up.  Third attempt came in at 66.  Well I am not thrilled with that number but I am also happy it’s not any lower.

After the PFT attempts I talked with my doctor.  He said well I think I will start you on some antibiotics once we receive the results of the culture.  John call me in 8 days!! What 8 days that’s a long time but here is why. I can recall that culture results are not final until close to 10 days after the mucus is collected.  He does not want to just give me antibiotics without knowing what I am culturing especially since my mucus seems to be different. In my last culture I had the usual staph and achromabacter. I don’t know if the 3% drop in PFTS warrants an antibiotic but I will see how I feel once the culture comes back.  The wonderful thing is that I have not been on an antibiotic since November 2012 which is a very long time.  I think if he prescribes me a breathable antibiotic I will not remember how to even administer the drug.  YIKEs!

 

 In other exciting news, I finally got my doctor to buy into looking into Kalydeco for my gene.  I have 3849 10kb C->t gene which does respond to Kalydeco ALONE!!! YES, I know this for a fact as some people with my gene are seeing improved health from Kalydeco.  As many of you know in the CF community, Kalydeco is not FDA approved for any gene but G551D.  For me, to actually have the insurance pay for this is going to be like a David vs. Goliath fight.  But my doctor said the research coordinator at my clinic will be calling Vertex about Kalydeco and my gene.  I am super excited but very cautious on this as my doctor still wants evidence that Kalydeco alone will work for my gene.  He wants proof on a larger scale its safe and effective.  So some of you that I know who are taking Kalydeco who have the 3849 gene I may be in touch with you soon.  Again I am being very cautious on this. 

Lastly, at clinic it was my last research study visit for the study I am in.  Basically, the study is a questionnaire format and monitoring FEV1.  A lot of questions about your mental health and such are in the questionnaires.  I honestly enjoyed talking with my nurse coordinator. He lives a very similar life like Maria and I minus the CF aspect.  We had a good chat about kids and the nuttiness that goes along with being a parent.  He almost did a play by play of our same day to day routine.  So no wonder why its challenging raising a child, working full time, doing family chores, being a husband, being a father, and throw the lovely CF on top of it.  My plate right now in life is very full.  So if any of you have any extra plates or room on your plate please let me know.

All in all, it wasn’t the worst clinic or the best.  I guess we will take the wait and see approach on the culture results.  I want to say THANK YOU to all of you who wished me luck on my clinic and everyone who was eager to hear my results.  I truly do LOVE this community as we are all in this WAR together!  Keep fighting my friends and always remember “It’s a GREAT day to be alive!!”

 

Alayna sitting in a pan!

It's Time For An Update!

I know I have been MIA from my blog lately and I do miss it. I guess I have been busy living my dreams spending time with my girls and working away! It is a busy life when you work full time, come home to two beautiful girls in Maria and Alayna, house chores to do, weekends all full of plans or running here or there, and oh yea I have this thing called cystic fibrosis. It does get very hard to be a superhero or lack there of every day.  Sometimes I catch myself looking at the clock and it’s already 9 pm.  Where has the night and day gone? It goes even quicker on the weekends.  I can tell you this, I wouldn’t have all these great opportunities in life if it wasn’t for Maria and Alayna.  They keep me fueled every day to give my best and hold me accountable for my health.  So here is a little update about everyone in this household.  I know that many of you do not just come here to read what is going on with me

Let’s start with Maria!

I can tell you she is doing well and is the glue to this family.  If it wasn’t for her I think my life and Alayna’s life would be beyond NUTS.  I have to say I love that Maria is a SAHM (Stay at home mom).  I know that was her dream in life to have a child and be SAHM.  For anyone out there who thinks being a SAHM is a cake walk, I will tell you it is beyond more work than most of us do in our own careers.  A SAHM is unpaid, there are no days off, there are no lunch breaks, etc. This sounds like a job many of us may not want unless I told you were going to be a MOM or DAD.  But the rewards of being at home watching Alayna develop, learn, and grow are PRICELESS.  There are many days I know Maria is working A LOT HARDER than I do in a single day at my office job! I just want to say thank you for all that you do Maria ; )

If you have read my blog before, you may recall Maria was having a battle with her liver enzymes.  This all started a few months after Alayna was born.  This was frighten for the both of us as there was no real explanation to have such high liver enzymes.  The doctor even requested a liver biopsy be performed which from what Maria told me was nothing I would want done.  I have said it before but she is more of a trooper than me when it comes to medical tests.  You would think having CF I would be used to all the needles and tests but me and blood tests definitely do not mix.  In December, she had her quarterly blood test.  We both are happy to report that the liver enzymes have basically returned to almost normal.  They are slightly elevated, maybe a point or two.  This is great news and we are hoping again in March that the liver enzymes continue to drop.   

So what’s new with the princess Alayna!

I am going to keep most of the updates until I complete her 15 month post in the next few days.  I still do not understand why time goes so incredibly fast with a toddler.  Oh wait, I do understand.  There is never a dull moment in our life.  There is always something to do or going on.  

I will share with you that she is the apple of our eye.  There is nothing like your own child running around giving you kiss after kiss for no reason.  I guess you could say she loves her mom and dad.  She is to the stage where she is really developing a personality.  I love witnessing this as she really is full of life.  Something we all need to learn from.   There is something that I look forward to every day and that is coming home from work to see and hear Alayna get all excited.  She runs up to me with her arms reached out for a hug and kiss from her dad.  I really need to cherish this because when she hits teenage years I don’t think that she will be nearly as excited to see me when I come home!

That leaves me with one last update on myself!

As you all know I love to blab on and on so I hope this will be sweet and to the point.  I have been very busy in all aspects of my life lately.  There really is never a dull moment.  As soon as that alarm goes off I hit the ground running (I wish I literally was a runner!) with my morning airway treatment, getting ready for work, off to work for my 8 hours, back home, dinner time, some play time with Alayna, a chore to do, then its time for the evening airway treatment.  This sounds like my life Monday – Friday.  When it comes to weekends they are packed with stuff to do but not as scheduled.  I live off a schedule.  Any of you CFers feel the same?

In the last few weeks, I have been more involved in the CF community  than ever before.  I thought I would share some of what has been going on and what I am doing for some of you out there.  If it wasn’t for CF and my determination to spread awareness and educate people about this disease I wouldn’t have these great opportunities below.

Reaching out to newly diagnosed parents or even parents to CFers in general  is something I really pride myself on. I have been doing this a lot more lately.  To me, this is my mission to educate and bring more awareness about what it is like living with CF. There is no better way to do that than to share my life with them. This way they can see that their child can and will have the opportunity to live a normal life.  That is what I want for all the parents out there to realize that everything is POSSIBLE.  I have been blessed to share my story with many of you.  

Some of you have heard from me touting the benefits I have received from my compliance streak.  I am almost to a full year of compliance on 2/1/14.  Through this compliance streak I have felt AMAZING! I want that for many of my friends as well.   Do not wait until you continue to go downhill to make a lifestyle change.  It all starts with maintaining the lung function you have now and keeping your lungs clear of mucus.  This past week I motivated my cyster to dust off her VEST and start using it again.  I am so proud of you for making the first step on being compliant with your airway treatment.  Keep it up!!  On a side note please visit her blog and provide any advice as she is experiencing some crazy itching from the VEST jacket.  

This blog that I have created is being read all over the world and I have actually talked to some of the people who read it.  I never thought that I would be reaching people with CF who live in Brazil, South Africa, Canada, and Europe.  It's great to know they read what I have to write and they even educate me on how CF is treated in their countries.

We are in a new era of CF where CFers are becoming pregnant and becoming mothers and fathers. I love talking to these people as they are all excited for their new journey.  A child will add another motivator for you to be as compliant as possible.  I know Alayna has done that for me.  

The last few days I have talked with a fibro who recently was FINALLY diagnosed with CF. He is in his late 30s and is struggling with the realization of this disease. He and his wife have a child just as we do. I just cannot imagine learning you have CF after you just had a child a few months ago.  All I can say is keep fighting as hard as possible for your wife and child.  That is my goal in life to be here as long as I possible can for my family!

We are all in this war together!  I love how people will message me after their CF clinic to tell me how the appointment went.   We all share our positive news and sometimes negative news.  When someone feels down about something regarding their CF, this community is a great place to get a boost back in their step.  We are all in this war together my friends.

I am looking forward to a something that I hope will help many parents to young CFers explain why they are doing treatments and taking all these pills. This opportunity came to me from someone who left a comment on my blog. I never thought I would be asked to do  something like this. Stay tuned as I will soon feature this on my blog.  

All in all I have been very busy to say the least in the CF community. I cannot wait for 2014 to get under way as I have a feeling it's going to be a great year for my family and the CF community.  I truly hope this is the year that many of my friends and myself included will benefit from Kalydeco and maybe another pill from our friends at Vertex!!

Always remember………”It’s a GREAT day to be alive!”

Daddy's girl 

She loves Minnie Mouse 

Alayna got herself into the juice box

Mommy & Alayna hanging window decals. 

Alayna 14.5 Months & Christmas Wrap Up

Well here I go again, not keeping up with your monthly posts Alayna.  Shame on your daddy!!! I have to admit that I have been a little behind on the blog front as your mommy and I have been beyond busy living our life with you.  As I have said in many of these posts you have really completed our dreams.  We are just living them with you now every day.  I promise to keep up with the monthly updates for you because I know when you are in your teenage years you will be glad I did this for you.  Well I guess that is my own opinion anyway.

You are now 14.5 months!  Uhm where has time gone………….STOP, you are growing up way to fast.  As for the monthly developments, you are now more talkative than ever.  I guess I should say you are spitting out words not developing sentences yet.  This past month, your new favorite word has been HO! Every time you see Santa Clause whether its in person, on an ornament, or even your grandpa’s beard you just say HO! It really is beyond adorable. I think you are a very wise girl already. 

You already love Barney but I think you know like another tv character.  Ever since mommy recorded Elmo’s Christmas you have been in love with Elmo.  I think you really enjoy this episode as it is full of dancing and music and we all know how much you love to dance.  You really know how to change your dance moves to the beat of the music which is hilarious.  The Elmo episode has also added another word to your vocabulary.  When you see Elmo on tv you will say “Melmo”!  Haha.  A smart kid you are again.  Not only are you saying HO and Melmo you now say dog and the dog’s name, Mia. 

The personality you are developing is fun to watch.  When you see noodles or certain foods you will say “MMMMMmmmmmm” It’s funny you recognize certain things and associate them with food and tasting good.  Then you will have tantrums where all of a sudden you get really angry while we hold you and you decide to use your fist and hit mommy and me in the face.  Oh, if we have our glasses on our face forget it.  You try to rip them off and smash them in your hands!!

Most of this tantrum stuff is explained though. The last few days you have been teething and those molars are poking through your gums.  I guess that would give you a reason for being cranky, angry, and mad.  Amen for Tylenol though.  That really seems to do the trick for you. 

Last week, we celebrated another Christmas with you. The magic of Christmas has been instilled in our family again.  We made a gingerbread house with your niece Kendal.  You are still a little to young to understand what to do but you did place a few pieces of candy on the house.  I think Kendal really enjoyed it! Next year you will get the hang of it more.  It was fun to see and will become a yearly tradition I am sure. 

Another tradition in this house is sugar cookies.  As you get older you will see how fantastic of a cook and baker your mom is.  Maybe you will pick up this skill.  I wouldn’t mind having 2 wonderful cooks in the house.  Daddy can cook some but not to the standard that mommy does.  Anyway, mommy made the homemade sugar cookies and we all decorated them.  You loved eating the icing off the cookie or our finger.  We kept hearing you say “MMMmmm”  When we were all done decorating them we gave you a few bites of the decorated snowman and you were on cloud 9!

Then came Christmas morning! You brought the magic of Christmas back into our house.  It was a lot of fun watching you get all excited with the presents and the wrapping paper.  In fact, one of your favorite gifts from Santa was Billy Beats.  Billy Beats is a piano that has blocks and dances when you press buttons.  But you found this gift a few days early as Santa didn’t hide the presents to well in the house.  Haha.  I guess santa needs to find a better place as you get older.  We all had a fantastic Christmas and I have to say you are beyond a little spoiled girl. 

Every day you continue to amaze us! You continue to learn more and more and show us how truly blessed we are as a family.  I will end this post on this note………Last night, you and daddy played hide and seek for the first time.  I should say it was more of daddy hiding and you trying to find me in the various bedrooms.  You loved it!! It’s the small things like this that I truly will cherish for years.  I never knew if I would be able to have all these joys in my life.  I cannot say it enough but Alayna you truly have motivated me to do anything I can to be here on this planet for as long as I can.  One day I will explain all of this to you. 

WE LOVE YOU!   

The PFT Battle! My Response To A Cyster........

We all have struggles when it comes to cystic fibrosis and they are all different from person to person.  Some of us suffer with GI issues, not able to gain weight, sinus issues with nasal polyps, chronic lung infections that constantly decrease our ability to breathe, fertility issues making it harder to create a family, the list goes on and on.  

But then there is the struggle with our numbers………….Yes, our numbers. When we attend CF clinic we always do the same routinely tests/measurements.  Some of us dread stepping on the scale as many of us could eat three or four times the amount of food than a normal person in a day and not even gain a single ounce.  

Then there is that lovely test called a pulmonary function test (PFT)!!!! All cfers are supposed to have a PFT at least every 3 months.  The PFT measures how well our lungs are functioning compared to a normal non CFer.  For those of you who are not familiar with a PFT, I sit in what looks like a telephone booth with a pipe in my mouth and nose clips on.  I am then told to breathe normal for a few breaths and then take a deep breath in and blast it out as fast as I can.So if you would like to try this at home here is how I suggest attempting a PFT.  Find a paper towel roll and put it in your mouth (don’t eat it or choke on it please) and plug your nose with your fingers.  Take a few breaths in and then a deep breath in and blast it out.  

The PFT measures our FEV1 score.  The FEV1 score is determined by how much volume of air is blown out at the end of the first second which is measured in a percentage.As CF is a progressive disease, our FEV1 will start to decline as the disease progresses.  When you hear someone say they only have half their lung function that means their FEV1 is 50% which is the equivalent of breathing with one lung! Doesn’t sound fun does it?

The PFT is my biggest battle when it comes to my clinic visit and I know I am not alone.  The main reason I think it’s my biggest battle is I put SO MUCH weight and pressure on that number. I really should be concerned with how I feel and other aspects.  I will get into all that later in this post. But when I/we see our FEV1 number declining there a zillion thoughts that start to play through my head.  Why is my number dropping from 3 months ago and I feel fine? Do I have a cold? Is something irritating my lungs or breathing? Is my PFT form correct? Did I do my hypertonic saline and vest to close to my time for my clinic visit? Are my allergies irritating me today? Is my disease progressing? Do I need to be on antibiotics or is it time for my FIRST ever hospital stay? Will I be on this planet long enough to see my daughter get married?  I don’t like this PFT tech as they do not motivate me? As you can see these are some of the various questions that go through my mind and there are a bunch more!  

But I wanted to share my response I left a mother who has a daughter with CF battling my same battle………THE PFT!

What things have helped raise your (or your child's) FEV1 the most? 
Despite all we are doing both natural (tons) and conventional, HER FEV1 was down 3% today from three months ago and she is so disappointed. It's extremely difficult for a 15 yr old (or anyone, for that matter) to stay motivated and keep up such a demanding regimen when she doesn't see the results on her PFTs. Could she have allergies in addition to CF that are keeping her constricted? Need more physio? Any ideas welcome as we need some positive vibes coming our way! Thanks

There were various comments left on Facebook for this awesome young CFer.  They included PFT techniques, exercising advice, supplement advice, airway clearance techniques, and many other suggestions.  

Here is my response……………

As many have mentioned I think we all hate the PFT chamber. At least I do. I honestly think this is one of the most questionable tests for any of us CFers because there are so many variables that are in play. For me, my nerves start to kick in when it comes to PFT tests. I think that's because I put a lot of weight on the numbers. When I start to get nervous it fuels stress which doesn't allow me to get the deep breaths in and I start to feel my chest get tight.

Also, I have a certain PFT tech that I don't care for and I felt always added pressure to my PFT. Then on top of that you may not be feeling to well that day because of many reasons.  You may be suffering from a minor cold that isn’t CF related or even allergies.  I can recall, at clinic beforethere was this strong smell of perfume and another time a strong smell of bleach.  Those smells definitely made it more challenging for me to breathe.  A cold and allergies will almost always push our numbers down as our lungs and throat are irritated.

I just feel that WE all place way to much emphasis on thisPFT number. I honestly wish they would get rid of the numbers on the PFT test and just come up with you are in your baseline range or you are slightly below baseline or above etc. I feel that way this 3% drop your daughter had would be a mute point. A 3% drop is relatively unchanged.In all honesty, who knows what it could have been a day ago or even today. To me the PFT test is just a number in that instance. It's always fluctuating through the day and we can even change that number based on treatments we are doing. I have read if you do a treatment or HTS an hour or so before PFT you might have inflammation or irritationfrom the neb. So you can see my point that there are so many factors in this lovely test.

Just remember this we do not just do a PFT at clinic and go home!!!! There is so much more involved in our clinic visits. The doctor listening to our chest, weight checks, pulse ox, questions about how we feel, how we have been doing with treatments etc. My doctor has told me the PFT test is one of the most "quirky" tests and it's just one piece of the puzzle.  

Cyster’s name, please do not lose sleep over this. This is coming from a 28 year old fibro who still does not know how to properly do a PFT.  I have been doing these tests for years and I feel half the battle is mastering the proper technique when it comes to PFTs. I find myself worrying if I have a good tight fit with my mouth on the pipe. Or maybe my tongue is in the way during the test. I then find myself questioning if I should cut back my blast out as maybe I am restricting air flow with that huge blast out.  As you can see a lot of this is a MENTAL battle!!!  

The most important questions we need to be asking ourselves are these…..Please , ask yourself how am I feeling? Are you able to do the things you always have with ease? Is your appetites good? Are you breathless more often? How is your mucus production? How is my compliance to airway treatment? These secondary endpoint measurements are just as important or are MORE important than that PFT score. Keep doing what you are doing cyster.You are already seeing the true impacts of said pill with those big changes in fev1 since you started. Keep this in mind, our battles are not just won on clinic days they are won every day we put forth are best effort outside of clinic!If you have any questions or want any more advice when it comes to PFTs let me know :)

I also went on to suggest adding hypertonic saline as many of you know I LOVE it! If you cannot handle the 7% solution try to add the 5% or 3% solution.  HTS works wonders for me when it comes to clearing out the wind bags.  

This is something I think we all need to ponder………….I mentioned how the PFT is just a measure at that singlepoint in time at clinic and who knows what our PFT was yesterday or even 3 hours before the test.  But why do we only do PFT’s 4 times a year (assuming you go to clinic every 3 months)???? I think we need to question whether we need to have more at home monitoring when it comes to our CF care case in point PFT’s.  I can recall as a kidinvolved in a study where we had these little machines that we blew in that measured our FEV1.  I do not remember how often we were required to measure our FEV1 but I think it was beyond valuable for me.  I recall my mom attaching this monitor to the phone and transmitting the data to the clinic.  Just think now we can do all that stuff online and with computer software so it would be much more useful to the patient and doctor.  I suggest that we either invest in machines or have this part of our care just like the vest is.  I would only suggest using these FEV1 monitors if they produce results close to the real machines at clinic ; )

The flipside to this is……I know there are people who will not buy a scale in their house as they will obsess over their weight every day and I can see this same aspect for a FEV1 monitor.  But what I am suggesting is that maybe we use the monitor once a week or whatever makes you feel more at ease.  This will also provide us with more knowledge of when maybe we are feeling sick and need to call clinic to get a tune up.  We can also use this monitor to see what happens to our FEV1 when we have a cold.  But another big aspect of this at home monitoring system is it will reduce the risk of cross infection!! Anyone else get all germaphobic when you enter the PFT room? I know I do!Maybe these at home monitors (if everyone has the same model) will replace/reduce the need to do PFT’s at clinic every 3 months.  This is all food for thought : )

Thanks for reading my LONG post!!  Please if you have any more suggestions on PFT’s for this young cyster in our community please leave a comment.  I know the two of them will be reading this post.