Friday, December 13, 2013

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.  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.

Wednesday, December 4, 2013

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!