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 : )

Our Dog JJ - We Will Always Miss You!!

This past Sunday, was one of the most difficult days of our life.  If you have ever owned an animal and you treat your animal like your own child you will understand how hard Sunday was for our family.  I introduced our four legged family last year and gave everyone the summary of what JJ and Mia mean to our family.  Here is the post if you are new to my blog. 


Our dogs are like our children and they will always be our children.  Before we became parents to Alayna we were parents to JJ and Mia.  We treated both JJ and Mia just like our own kids.  We would do anything for them.  As you recall, JJ our male dog had back surgery about 3 years ago for a slipped disc.  We found out during the surgery that he had degenerative disc disease.  Basically, his discs in his vertebrate would always be bothering him to some degree.  During the surgery, the surgeon tried to fix every disc he could but there were going to be a few discs near his head and lower tail that would not be fixed.  They didn’t want to mess with discs in these areas because they could cause a lot of trauma and permanent damage. 

I know we will never forget picking JJ up after that surgery 3 years ago.  He was so happy to be walking out that door of the vet office.  He was coming back home to his parents and his friend Mia. The orders that JJ received when we took him home was he basically had to be crated or put in a pen where he could have little movement to heal his back.   We converted our family room into our bedroom.  We basically lived in our family room for 2 months sleeping on the couch and a mattress on the floor. 

After the surgery, we also found out he lost complete bladder control because his disc pushed so much on his spinal cord that it almost became severed.  He basically was thankful to be walking. JJ was in a diaper from the day he had surgery as he would just leak urine out since there was no muscle control.  So that meant we had to physically drain his bladder.  I will not get into all the details but this was going to be done 4 times a day.  Mainly Maria was doing all this work and it was like taking care of a baby.  She eventually got into a routine with all this and it became a part of our daily life. 

Since JJ was diagnosed with degenerative disc disease he would always have back flare ups where his back would go out and he would have to be crated to a room for 6 weeks. We had to keep him as calm as possible which meant basically no one could come to our house, no one could ring the door bell, no vacuum cleaning,  anything that would excite him would be a no go.  This was beyond challenging as JJ is a dog that got very excited by just about anything.

A few weeks before Alayna’s due date JJ’s back went out.  We thought then that it was going to be the end for our little warrior.  But Maria and I both wanted him to meet Alayna.  We knew it was going to be so difficult to keep him confined and relaxed but we tried everything we could.  Luckily, within a week or two his back finally calmed down.  JJ got to meet our miracle Alayna and we were thrilled.  We were given two little warriors in our life in the both of them. 

Fast forward to this past Saturday at 1 am. Maria and I both knew JJ’s back was out again.  He got out of his bed and started shaking and his back was fully arched and he could barely walk straight.  Maria went to express his bladder and he couldn’t hold himself up on his back legs.  We knew then it was time.  That night he didn’t sleep at all due to all the pain he was in. 

The next morning we knew it was time to say good bye to our warrior.  I have to say it was the hardest thing to ever experience for both Maria and I.  This was the first time we had ever had to say good bye to an animal.  For us JJ was more than our dog he was our son. He will always be our son. 

Through JJ I learned a lot about my own life and what I have been blessed with.  That is something that no one could ever take away from me.  I always knew I was blessed to have Maria in my life but through JJ I found out I was blessed beyond words. Through JJ it has made me realize how lucky and blessed that Alayna and I are to have you in our life, Maria.  You would do anything for our family even going above and beyond what anyone would ever do.  I’m beyond thankful to call you my best friend, my soulmate, my wife, and best of all the mother to our daughter.   You will always be JJ’s mom and no one can ever take that away from you.   Thank you JJ for creating memories that will last a lifetime and most of all for showing me how BLESSED we are to have Maria in our life. 

We will always remember you buddy!

We miss you and LOVE you J.J.!!!

JJ next to his daddy.  We will always be family.  He made it to welcome Alayna into this world :)



Mommy and JJ.  Mother and Son. 

JJ in his diaper with his best friend forever Mia.  

WE WILL MISS YOU JJ! We hope you are running around and having fun!!! 

CF Clinic Update - Redemption!

Yesterday, we (Maria and I) made the trip to my CF clinic. It always feels like an actual trip.  Here is why…….When I left work it was 2:30 by the time Maria and I got home from the appointment it was close to 7:00. But this is typical. 

 

I was really looking forward to this appointment as I called it my redemption appointment.  If any of you remember my last visit to clinic, I was battling a cold and I knew my PFT test was going to suffer.  Well it did last time and I got credit for a FEV1 62 back in August.  But I knew why it was lower but still it doesn’t sit well with me.  Yesterday, I was looking for redemption on that PFT score because I have been feeling FANTASTIC after I kicked my cold WITHOUT a single antibiotic. Woo hoo for me!!

 

When we arrived at clinic, I was pleasantly surprised to see a couple things have changed from my last visit.  Maybe it was because of all the talk about cross contamination from the CFF.  The first thing that caught my eyes was a stand that had a bunch of medical maks!!! Woo hoo! I have never seen this at my clinic before.  So did I wear one???? NO, because as soon as I got in there and talked to the receptionist my vest lady came out aka respiratory therapist to ask me questions.  She asked me what I do for airway clearance and nebs and all that stuff.  I told her my love for HTS is still there.  I asked her about the Minnesota protocol that I mentioned in my last blog.  She said yes she has heard of it and the individual who made the claim to fame on this protocol was also the founder of the Vest.  I asked what she thought of this and she said yea it works but the key is to make sure you are taking those PAUSES that I mentioned in my CLAMP motto (Cough, Lubrication, Air, Mucus, and Pause).

 

After I was through talking with her it was time to move on to visit with my doctor. I always love to talk to him.  We talk not just about CF but all the other exciting stuff going on in my life. I started off talking about Alayna turning 1 year old.  I shared with him all the videos of Alayna dancing and taking her first steps. He really could not believe how she dances.  He was just astonished.  Then the conversation transitioned into him watching Miley Cyrus music video.  He even mentioned tweeking! Hahaha. For all of you who do not know my doctor he is in his 70s and is talking about Miley Cyrus and tweerking.  It was hilarious.  My appointments at the CF clinic never feel like doctor appointments.

 

As for some serious stuff………..My lungs sounded good no crackling or wheezing.  My weight is stable and I was able to cough up a little mucus for the culture.  Does anyone ever feel like when they go to clinic that if they cough up mucus a few minutes before clinic that they will not be able to when asked for a sample for the culture? I fit into that boat. In fact, I have actually held my cough in on occasion to wait to cough it out when I am at the appointment.  Ok sorry for the TMI but I know I am not the only one.   

 

My doctor told me that he quotes me very often.  I told him after I had been using hypertonic saline for a few months “I LOVE MY SALT!”  We then discussed when and what strength of HTS I use.  I told him I use 7% during my morning treatment and 3% in my evening treatment.  I did mention that my evening treatment has become 10 times as effective now since adding 3% HTS.  He asked me I use 3% instead of 7% in the evening.  I do 3% in the evening so I don’t end up coughing my head off all night and it really improves my mucus clearance.  He said interesting I think I will start recommending this to patients now that do not neb HTS in the evening or for those who cannot handle 7% in the evening to try 3%.  I guess it looks like I’m going to start a revolution for HTS in the evening treatment at my clinic.  If any of you cysters or fibros are not nebbing HTS in the evening ask your clinic if you can experiment with adding it to your evening treatment.  Especially, if you find your evening treatment to not be as effective as your morning treatment! 

 

After I was through with my doctor, I then met with my research coordinator to fill out a bunch of questionnaires.  We chatted for a little bit and then it was time for my redemption.   

 

As I walked to the PFT room I felt good about this upcoming test.  I knew I was feeling good and ready to prove that I was back to my baseline.  My baseline over the last 2 years is anywhere between an FEV1 of 68-74.  I sat in the phone booth box and put my nose clips on and blasted out my first test.  The first score came in at FEV1 68.  Woo hoo.  That was 6 percent higher than last visit. I blew three more times and I got credit for a FEV1 of 69!! I wanted to get back into the 70s but I was very happy with that score. Next time I will be back in the 70s that is my goal! As for my FVC it was exactly the same as my last visit, 93.

 

So all in all it was a great appointment.  I redeemed myself and was able to make it back to my FEV1 baseline.  This appointment also showed me that my dedication to compliance is really paying off as well.  If it wasn’t for being 100% compliant then who knows where my numbers would have been or more importantly how I feel.  I left clinic with a request for blood work as well.  If you know me you will know I hate even the thought of having a blood test! Remember my butter knife episode?  Me and blood equals passing out.

She is already a genius!!

 

 

Effective Airway Treatment & The CLAMP Motto

What does an effective airway treatment look like to you? I think we are all guilty of an ineffective airway treatment at least once in our life.  I know I am.  I have fallen into the ineffective airway treatment more than once!  But lately I have been paying close attention to my airway treatments because I want to get the most bang for my buck!  Who doesn’t want to get the most out of their airway treatment?  I think we all can answer that during the time we are hooked up to that machine that shakes us we want the most effective form of airway treatment.  There are many things we need to remember when we are tethered to that vest machine. Hopefully, this blog will help some of you get the most bang for your buck when it comes to treatment time.

 

When it comes to airway clearance how many of you have actually been told how to properly vest????  I wish I could see a show of hands : )  I am guessing that not many of us have been given an actual lesson in airway clearance. My hand would be down as well so don’t feel bad.   For me, I can remember that day I got my vest machine from hil rom (I use the hil rom 105 model).  Before ordering the vest, I sat down with the respiratory tech at my clinic and she just let me try out the machine.  She said you can change the pressure, the frequency and it has a timer.  I was told to just do 5 minute intervals for a total of 15 minutes.  In between 5 minute sets, she said just make sure to cough.  Uhm ok that was about all the training I ever received for using the vest. 

 

The other day, I was reading on Facebook about an actual airway clearance technique called the Minnesota protocol. I am not promoting this technique just making you all aware of it.  I honestly think this is the best way to use our vest but I suggest playing around with the settings that best fit your lungs! 

 

At first, I have never even heard of this protocol.  After reading the link, it was clear to me that I have been basically doing this protocol.  Here is how I have been vesting for the last 10 years of my life.  I do 5 min intervals of each while nebbing HTS (I started HTS about 1.5 years ago, and you all know I LOVE IT) and I always make sure to huff cough and get the mucus out during the PAUSE.  I start out at 8hz/7 pressure, 12hz/7 pressure, 16hz/7 pressure, and 18hz/7 pressure.  By the time I hit the 18 hz setting I am coughing a ton of mucus out.    I think the biggest thing in the protocol is LUBRICATION (nebulizing) and taking a PAUSE to cough.  It is so important to cough in between frequency changes while vesting even if we need to make our self cough.  That will help loosen up the mucus!!!

 

After I read the article on the protocol, I developed my own acronym CLAMP.   Here is what it means and not in any specific order.  But we should all focus on these while doing our airway treatments.  

Cough

Lubrication (nebulizing)

Air (breathing)

Mucus

Pause

LUBRICATION (nebulizing) – Without oil or LUBRICATION in any machine the parts get stuck together and they do not move very well. Think about your car or any other piece of equipment that is heavy duty.  Our lungs are the same way especially in CF.  The lungs of CFers are not sufficiently hydrated enough which causes mucus to just keep on building up.  So if we are not properly LUBRICATING our lungs then the mucus will just stick around and keep building on top of mucus thus making it more likely for us to have infections.   That is why it’s so important to be nebulizing hypertonic saline or another aerosol to help LUBRICATE the lungs.  The more LUBRICATION we have in our lungs the more likely we will be able to cough out the mucus.   So lube up those lungs!!

 

AIR (breathing) – I think we all suffer from the proper BREATHING technique during airway treatments.  How many of us get distracted with facebook, blogger, emailing, music, reading, or tv?  I know I fall into this boat.  I find myself not taking in deep breaths and holding or even slouching during treatment.   We should be focused on sitting up straight and inhaling and exhaling effectively.  If we are not doing this we will not allow the aerosolized neb to fully reach or lungs.

 

I noticed lately that I have been focusing a lot more on my BREATHING and have seen an increase in the effectiveness of my airway treatment. More mucus has been coughed out and I just feel cleaner lung wise.   So the next time you are vesting/nebbing make sure you are thinking about proper BREATHING and posture.  We don’t want to sell our self short during treatment because of a lazy BREATHING or posture habit.

 

PAUSE & COUGH – I know there are people out there who have programmed their hil rom vest to their current settings and do not take a PAUSE in between the next frequency change to COUGH.  In the Minnesota protocol the PAUSE and COUGH is really emphasized.  The reason we are all suppose to PAUSE in between the next frequency change is to COUGH.  In order to move that mucus out or around we need to physically make our lungs push out the mucus.  I know that some people just will cough when they have the feeling to cough during treatment.  I think for a fully effective airway treatment we need to focus on the PAUSE.  We should give our self a full minute to sit with no neb in our mouth or vest shaking us to death to just COUGH.  Try to use the huff cough, flutter, acapalla, or whatever it is that makes you COUGH.  

 

I have incorporated the PAUSE into my airway treatment since I began vesting and I can tell that I am moving mucus!!  I can remember when I had my gall bladder removed my doctor told me there is no point in doing airway clearance if you are not coughing.  So incorporate the PAUSE and the forced COUGH into your airway treatment even if it makes you uncomfortable.  I think you will be glad you did. 

 

MUCUS – When it comes to CF we all have MUCUS in our lungs. It’s just a given aspect of the disease.  Even when we feel 100% and are not suffering from a CF exacerbation we will always have MUCUS.  That statement is according to my doctor.  I asked him if I will never have mucus and that was his response. 

 

You may feel that if you are not coughing out any MUCUS during your airway treatment that you are feeling really healthy.  I agree to a certain extent but I honestly would rather cough out MUCUS in every single airway treatment.  That for me reassures me that this airway treatment stuff is really doing its job. If you are not coughing out any MUCUS maybe you need to focus on your breathing technique, posture, or even question if you should be doing an extra aerosolized medicine.    

 

In my evening treatment, I noticed that I was not as productive when it came to moving that MUCUS out.  So I questioned my doctor about adding HTS in the evening.  I was only taking HTS 7% in the morning and noticed that I was clearing mucus left and right from adding this neb.  For me, my lungs were not lubricated enough during my evening treatment. We added HTS 3% to my evening treatment and it has changed the effectiveness of my airway treatment.  I am now coughing more mucus out in the evening than I ever did before!!

 

The more mucus we are clearing out the less there is to deteriorate our lungs and grow infection.  So get to moving that MUCUS out!! 

 

I understand every CFer is different when it comes to an effective airway treatment.  Those are just a few things that I think we all can focus on during airway treatments.  Even if you do not do the Minnesota protocol you should still think of the CLAMP motto every time you CLAMP that vest on you!!

 

Also, for all the parents of CFers who read my blog I suggest getting your children in the habit of an effective airway treatment.  You need to reassure the youngsters to focus on their breathing/posture, take a pause to cough, encourage them to get that mucus out, and to effectively nebulize.   It’s easier to get into an effective routine when you are young!  At least that’s how I feel. 

 

I hope that this has helped at least one person out there get the most bang for their buck when it comes to airway treatments.  If you have other effective airway treatment methods please share them!  WE ARE ALL IN THIS TOGETHER!!!