Tuesday, February 18, 2014

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 97th 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 25-50th 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 16th birthday will be coming in no time.  For now both mommy and I will cherish every single moment with you!

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!

Sunday, February 16, 2014

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!