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.

November Thankfulness Post Thanksgiving!

It’s been a while since I have been back on the blogging band wagon.  That’s because life has been beyond busy in our household.  Well I am jumping back on the wagon now.  As the month of November flew by I never got to post my monthly thankfulness.  I have not always been posting a monthly thankfulness but I never once go through a day without feeling grateful.  So without my further babble here is my list.  I narrowed it down to 7 because I know not everyone would want to read a book.

#1 & #2 are a tie. Any guesses????????????

 

1.)    Our miracle Alayna

What can I say…….Alayna just is an amazing human being.  Before we had Alayna in our life I always wondered what it would be like to be a dad.  I honestly didn’t know if that day would ever come but I refused to let that detour my thinking of making my dream come true.  I can tell you this that there is nothing in this entire world that compares to being a dad.  There will be no other title like the one “dad”.  I love coming home from work and seeing Alayna run to me with the biggest smile.  In her eyes, I can do no wrong to her. Well I know that will change in the years ahead when I tell her there will be no dating until she is eighteen. 

I just love the fact that both Maria and I get to watch a part of us grow up.  Alayna enriches our life more than any item or amount of money ever could.  She provides us with non stop entertainment and non stop activity.  Another thing that I love about Alayna is how Maria tells me often that Alayna is a spitting image of me.  When I see pictures of myself as a baby she is my double. I am of course extremely proud of that. 

Alayna has provided me another reason to get out of bed in the morning to continue my fight every day.  I am more determined than ever before to see a FUTURE with her and to see every one of her milestones.  The determination that you provide me Alayna, is beyond PRICELESS.  

2.)    My wife Maria

As I said about Alayna……..Maria you are another amazing human being.  My wife is my best friend and my soul mate.  There is no one else I would want by my side every day to motivate me to be a better person.  She understands me, she thinks like me, and more than anything in this world she cares about me. 

Just like Alayna, Maria has provided me another reason to get out of bed every morning and stay compliant with my health.  Maria has shown me what it means to take personal responsibility for your own health.  This past year was another bumpy road in her health with battling elevated liver enzymes.  I am so proud you took it upon yourself to never give up and continue fighting for an answer.  You said numerous times that you were not going to give up fighting for an answers because you want nothing more to be here for Alayna and me.  This goes to show that if you are willing to fight that hard that I need to just as well because we are in this together. 

 

Maria, as I have told you many times before I am beyond blessed and thankful to have you in my life.  I LOVE YOU!

 

3.)    Compliance

If you have not been reading or following my posts on facebook or blogger this may come as a shock but I have been compliant with all my airway treatments, nebs, and pills for over 302 days now.  I started keeping track on 2/1/2013! I am almost to that 12 month mark and I really have my eyes on that 2/1/2014 date.   The other day I was asked what have I done different to make myself so compliant these past 300 days. Well for me I was always about 97-98% compliant when it came to my CF regimen.  But I knew that wasn’t going to cut it if I want to be on this planet to see many more of my dreams come true.  The two individuals in the previous #1 & #2 spot on my list are why I am so beyond nuts when it comes to compliance.  I will be here on this planet to witness Alayna attend her first day at school, graduate high school, get married, and even be a grandfather one day right along Maria’s side. Now do you see why I am so compliant???

 

4.)    Family

Maria and I both value our bonds with our family. We live very close to all of our family which makes it easier to get together for holidays and birthdays. The holidays are meant to be spent with the ones you love and we love our family. They have been there for us through everything. We are very blessed and thankful to have them in our life.  I’m glad we made it to both Thanksgiving’s this year and Alayna was a trooper : )

 

5.)    Cystic Fibrosis Foundation

Last year the CFF made it on my list as well.  As I have said before, if it truly wasn’t for the CFF and people who care enough to donate to finding a cure for this disease I wouldn’t be living the awesome life I am today.  I know soon in my head I will have that magical little blue lighting pill.  It may happen this coming year.  I will see what I can do to make that happen.  But I want to thank everyone who has ever donated to the CFF because you are not only helping me but thousands of others in this country who are battling day in and day out with CF.

 

6.)    Organ donation

 I am beyond thankful that there are people willing to give the greatest gift by being an organ donor.  That is one of the most unselfish acts anyone can do. Even though I have a genetic disease I am thankful to be an organ donor. You can donate just about any part of your body.  In the CF community there are way to many people waiting for a second shot at life.  It’s a reality that many of us will have to face someday to be placed on the waiting list.  I am just thankful that there is a way to extend our life if we need that gift.  If you are not an organ donor what are you waiting for?

 

7.)    Social Media

Before I ever had my blog site I truly wondered how others were dealing with this disease.  I am so thankful that we all have a way to communicate with one another.  One thing that I truly love is blogging about my life and other CF related topics. For all of you out there who read my blogs and enjoy reading my life I commend you! : ) If it wasn’t for you I guess there really would be no point in me blogging. 

 

I love this CF social media community and it has really opened up my eyes of how my fellow CFers live their life. Having this disease isn’t much fun but when you read and follow other people’s journey you know you are not alone. I have met through social media some of the nicest people I have ever met who are similar to me in one way or another

 

I know this post is post Thanksgiving so I hope you have had an enjoyable holiday with your friends and family.  Just remember that being thankful is just not something we should do once a year it should be a daily event : )

 

Making stuffing with mommy!

Alayna 13 Months.........A little late : )

Alayna, I have not forgotten about you!!! I know this is a little late but I still want to continue your monthly updates.  One day, when you get a chance to read all these monthly updates, I think you will be glad I took the time to write these.  I know I wish I had something like this when I was growing up.  We all can take 1,000,000 photos of our child but NOT very many have an actual status update every month that includes what is going on right then in their child’s life.  For that I am thankful I started doing this for you, Alayna.

Alayna you are now 13 months and a little over a week old today.  WE honestly do not know where the time goes but it does go somewhere : ) You had your 12 month checkup a week or so after your actual birthday and you are now 21 lbs. 7 ounces and 31 1/8 inches. Your weight puts in between the 25-50^th percentile and your height is still 97^th percentile  We still cannot believe how tall you are!! Who knows maybe you will play volleyball, basketball, softball, or dance when you get older.  Daddy doesn’t mind which one you choose : )

As for your monthly developments, not much is new.  I think you only walked for about a week or two until you mastered the art of running.  No one can stop you from going to any room in the house now.  Well I guess the fact that we are now closing bedroom doors. But it’s like we are playing defense against you.  I think maybe you really like to hear the word NO because that is basically what we say 75% of the day.  It’s more like “NO Alayna stop climbing the stair!!!”, “NO Alayna stop trying to pull out the plugs!!”, “NO Alayna you cannot eat the dog food!!”, etc etc. 

I think though you really enjoy reading books with mommy and daddy.  You are funny because you have a few books you really love more than others.  One of your favorites is The Five Little Pumpkins.  When you see that book on the floor you run over and grab it and say pum pum. That is how you say pumpkin.  Some of your other favorites is Maisey’s ABC’s which you have destroyed every single flap you can possibly open.  Oh well it’s your books so feel free to do what you want with them.  Other favorites include the Biscuit storybook series.  Biscuit is a dog who you love.  I think you like the books because you get to touch and feel the hair on a dog, hair on a cat, and various other touch and feel pages. Another one of your favorites is the body book. This book has photos of toes, fingers, knees, mouth, teeth, ear, and other photos of kids running, painting, standing etc. I think you are turning into a baby genius.  We asked you where your knee was and you pointed right to your little knee.  You had the biggest smile on your face! When you do not want to sit still and eat, we like to give you these books. That sitting still and eating thing is something you have yet to master.

Not only do you enjoy having books read to you, you love music more than anything.  Both mommy and daddy are thrilled about that as we enjoy music just as much.  We can turn on any radio station or even have the tv on and you pick up the beat.  One of our favorite things to see you do is feel the beat of the music when you are in the kitchen.  You walk over to the oven and look at yourself and dance.  Sometimes you will even bring a prop in with you.  It’s not that you dance only way you actually change up your dance moves based on the song.  That is something that truly amazes us.  Right now one of your favorite songs is sung by Lorde called Royals.  I think you just love the beat of this song more than anything.  You truly are an entertaining little girl already!

As for your diet, you still like to eat baby food. But we have added some additional foods to your menu.  I guess you are a noodle lover because you really like lasagna and butter noodles.  But when you eat the lasagna you spit out the meat. There are times when you will eat something once and the next day or so we give it to you its like the most awful thing created.  For instance Nutra Grain bars.  You love them one day and hate them the next day. 

As for your liquid diet, you still love breast milk. One day when you read that statement you will be embarrassed. (Don’t worry daddy will embarrass you in front of all your boyfriends one day!) We are in the process of weaning you but it’s really hard.  I don’t think you like bottles of any kind as we have probably bought almost every brand possible. But you do like the old school Tupperware cups that have the lids.  It’s funny because mommy and daddy both used these when we were growing up.  I am happy to report that you do like water.  There are some people out there who hate water.  Water is so good for you so let’s hope when you’re older you still enjoy it. 

Something else we are still struggling with is bedtime. I wonder when you will ever sleep in your own bed for a full night???? You end up sleeping in our bed every single night.  Oh well, your daddy did the same thing as a child.  I guess it’s time for a payback to see how my parents felt about me sleeping in their bed.  But one day I guess we will miss having you scream at 2 am begging to get in our bed. You make sure to sleep al kinds of different ways by kicking us in the face, gut, or laying basically on top of us.

Alayna, you continue to be a little social baby.  Anywhere we go you always are smiling at strangers.  I think you enjoy getting attention as you know you are adorable.  But I have to say you are very well behaved when it comes to going out in public.  You will just sit in your stroller and be so content.  We are wondering how much longer that will last!!

 

Right now, that is all that I can think of.  You continue to get older and smarter by the day.  It’s almost scary how fast you are learning about your environment.  I cannot wait to see what you learn in the next month Alayna!

 

We Love You!

 

 

 

Why Did I Start Blogging? 100th Post!!

This post marks my 100th blog post!!!! Honestly, I didn’t realize I have blogged this much in the past 15 months. I guess I have a lot to say : )  Not only that, if someone asked me 2 years ago if I would have a blog about my life and have 100 posts I would have said no way.  But I LOVE blogging.  Maybe that is an understatement….  

I never really expressed why or how I even started blogging about my life. For me, I have never met anyone with CF.  I can tell you this that I have always been interested in how others live and cope with this disease.  When it comes to meeting people with this disease most of the people in the community have met others through hospital stays or what we call tune ups.  For me, I am beyond lucky to never havehad a single hospital stay or tune up.  Basically, a tune up is where you go into the CF clinic and stay a minimum of 2 weeks receiving heavy doses of i.v. antibiotics and visits from respiratory therapists to pound the mucus of out your lungs.  It’s NOT a two week vacation from school, work, or anything else at least that is what I am told and have read. But anyway I was always curious how others battling my same battle battled every day.

So here is how it all started…………….One day I was google searching running and cystic fibrosis and stumbled upon a link that opened up my eyes to a new world that I didn’t really know ever existed.  I know that sounds very very strange. But the link I clicked on was my fibro’s blog site.  All I can say is thank you Ronnie!!! If it wasn’t for clicking on your blog site I would not have learned that this blogger world ever existed.   I started reading many of the blog posts on here and read the comments that were left on posts.  I would click on the profile names of individuals who left comments to be directed to their very own blogger page.  Through all this, I found MY community of warriors who are battling much of the same things I do every day.  I know we all have different obstacles when it comes to CF but it is nice to find people who may have gone through some similar things that you have or will in the near future.  

After reading and following other CF blogs in the community for close to a year, I decided it was a good idea for me to take my CF life public.  One of the biggest reasons why I decided to blog was during OUR fertility journey we couldn’t really find anyone with our similar story.  There was no one that we could find who had a CF spouse (ME) and that their spouse was also a CF carrier going through pre genetic diagnosis (PGD) and IVF.  Once we were given our miracle news that we were pregnant, I decided to put OUR life out there for the public eye.Graciously, Maria approved of that decision : ) I wanted to put it all out there because I know WE were not the only people who would/will be going through this process.

Apparently, I was correct in this assumption! One day, I decided to Google search CF and IVF.  If you google CF and IVF you will get close to 1,200,000 results.  My blog post about OUR IVF journey is #3 on that list.  I guess that means people are reading OUR story and Google thinks at least that post has some value to it : ) To me that is pretty neat!!

Before I began this blog, I wanted to find a name for my blog that fit my life.   I decided to name my blog “Living My Dreams With CF” because that is honestly how I feel about my life.   I have been blessed with a fantastic life beyond words could ever describe. Through my blog, you have seen how blessed I am to have a FANTASTIC wife in Maria who motivates me every single day to put my best foot forward in every single thing I do.  In fact, she has seen more ups and downs in her health than I have in the last 2 years.  But never once has she ever said I am done or I am giving up.  When Maria found out she was a CF carrier and then soon after that she found out her infertility  she could have said there is no way I am going to go through anymore.  But never once did she say I quit. She has shown me that I truly am blessed to have the life that I do have. I cannot thank you ever enough to have you right by my side every day!!

Another dream that has come true in my life is that I am a father.  This is something that many people may take for granted outside the CF world. In the CF community many of us were told we wouldn’t see life beyond a certain age.  For most of us in my CF generation that was anywhere from teenage years to mid 30s.  So that meant going to college, having a career, getting married and having a family was I guess you could say not realistic. But never once did I take those words to heart and I believed that I would defy the odds.  Well for all those who read my blog you can see that I have accomplished every single one of those. Most recently, with the birth of Alayna I defied the odds again.  

Alayna is another reason that fuels my motivation and determination every day I get out of bed.  I am more determined than ever before to be as compliant and healthyas I possibly can.  I want to see that day Alayna starts kindergarten, graduates from high school, and I want to be there to walk her down that wedding aisle, and not only that to become a grandfather to her future children.  

Through my blog and other various forms of social media, I found a passion that I never knew existed.  That passion is raising awareness about CF and putting a positive spin on this disease to my readers, my fellow cysters and fibros, and even parents to CFers.  I honestly LOVE talking to all of you whether it’s through blogging, facebook, or forums.  I have been so blessed to be as healthy as I am with this disease and I feel that helping others overcome their challenges with this disease is my mission.  I have had the opportunity to interact with many of you and have made lifetime friendships.  I never once thought before I got my feet wet in this community that I would be talking to people all around this world.  I have been able to help a mom living in South Africa to a CFer, others who live in Europe and even Australia.  That just blows my mind.  I will continue to raise awareness and stay as positive as I can when it comes to this disease.   I hope you all continue to read my journey as I create more awareness for everyone who is impacted by this disease.

But most of all…………………………………………………………………..

THANK YOU for reading and being a part of my blog.  I appreciate anyone who takes the time out of their busy day to read what I have to blab about : )