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!