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!!! 

 

 

 

 

 

 

 

 

 

 

 

 

 

Happy 1st Birthday Alayna

Our journey to create our family was like a suspenseful movie or novel and on this day it ended with the best ending we could have ever asked for. On this day a year ago, at 5:45 pm our life was forever changed.  We were no longer just John & Maria or husband & wife. We became a family of three and would forever be known as Dad & Mom! 

Honestly, I cannot even believe that it has been a year since you were born Alayna.  I will never forget what it was like to meet you for the first time.  I was only able to see you for less than a full minute in the operating room as mommy was completely knocked out for the c-section.  But those first few seconds that I got to lay eyes on you I knew right then and there that life would forever be complete with you in our life.  Before you were born I didn’t’ think I could love another person more than your mom but I found out you can love two people on this planet just as much! 

When you were finally all cleaned up the nurses brought you to me and I can remember the first thing out of the nurses mouth.  She said “Congratulations!! Your daughter is gorgeous! She could be a baby model.” Well I was speechless………I couldn’t even say a word as I was just full of emotion.  You kept smiling back and forth at me and your eyes were wide open the entire time.  In fact, till this day you hardly even blink. 

It was close to midnight when you finally met mommy for the first time.  After all mommy went through to get to this point where you two finally met really completed our journey.  The two of you couldn’t keep your eyes off each other.  I could see the love the instant you were in her arms.  There just is something really magical about a mommy meeting their child for the first time.  I can tell you this Alayna, you have an AMAZING mom.  One day when you read this I know you will echo the same sentiments! 

Well keeping with the trend from all the month updates here is what you have been up to this last month.  In fact, this month you learned something faster than daddy did and probably even mommy.  October 2^nd, you took your first ever steps!!  It took daddy till he was 16 months to walk his ever first steps. I guess you are way more advanced than daddy!!   

You would go two steps and either grad onto something or just completely fall on your butt.  As you started to understand this walking concept those two steps turned into 3 then 4 and then walking around the room.  You were so proud and full of smiles that you mastered this walking concept completely on your own.  I even tried weeks before to hold your hands and walk with you. But you decided you didn’t want a single thing to do with that.  

Alayna, you were born into a family that loves music.  I think to even be in our family it is a prerequisite.  Well the one night we turned the music on the tv and you were loving every second of it.  We kept flipping the music station to different channels and you would just walk around with a smile.  All of a sudden we turned on some love song channel and you started to feel the music.  You started dancing and luckily daddy had his phone and took a video.  I guess you got those AWESOME moves from me, daddy.  Well we all know that your mom didn’t marry me for my good looks!! Haha. 

In other developments, you are now eating more of what mommy and daddy eat.  Well I should say a few things.  Mainly you love wheat bread ripped up in little pieces. You like green beans that are chopped up really tiny. Grilled cheese or toast and cheese as your mommy calls it has been added to your menu.  In fact you can eat an entire grilled cheese yourself.  Rice in chicken noodle soup is also something you like.  I think you get that one from your mommy because she loves that.  

A few days ago you went to Boo at the Zoo with mommy, daddy, Aunt Angel, Uncle Andrew, and your cousin Kendal.  You had a great time dressing up as Minnie Mouse. Your cousin Kendal just loves you.  The two of you will be best friends forever.  You would reach out for Kendal and hold her hand.  I know the two of you will be creating all kinds of mischief one day. 

As we walked around the zoo we heard so many times how adorable and cute you looked.  I think that people really loved how interactive you are.  You smile at everyone and just love attention.  Mommy said you get that from me. I don’t know though!! 

I just still cannot believe our little miracle is celebrating her 1^st birthday today.  If this is a sign of how fast things will be going in life you will be going to kindergarten,  16 and driving, and then off to college.  When I reread that sentence I think that is exactly how fast it will go.  But just remember Alayna you will always be our little girl!! 

HAPPY 1^st BIRTHDAY ALAYNA!

WE LOVE YOU! 

A Year Ago Today, Changed Our Life!

A year ago today, Maria and I had a special Doctor’s appointment.  I still remember everything about that day.  It was almost the beginning of our new chapter in life and the end to at least one journey.  I even remember how beautiful of a day it was on that October 16, 2012.  Maria and I jumped in the car full of excitement as we headed to what we hoped was our last OB appointment before we were going to meet our miracle.  We weren’t really sure if the doctor was going to say we will keep baby in there for a few more days as her due date was October 23, 2012.   In our minds we wanted this baby out ASAP.  Well I should say Maria would have loved to be induced that morning as she was suffering from the PUPPS.  Basically, Maria was allergic to the placenta.  It can happen but not very often. What I remember is it looked like poison ivy all over her belly that itched like no other.  In fact, the itch was extremely worse at night.  So just imagine being the most uncomfortable you can ever imagine with poison ivy all over your stomach.    I honestly, would have done anything for her so she could have at least slept.  I think she was trying to function on this day without sleeping for over 3 days basically.  I couldn’t imagine how exhausted she felt.  This was going to be the biggest challenge her body has ever went through and in order to be at your best you need sleep.  All I can say is that Maria is truly an amazing woman.

As we sat there in the waiting room we just hoped that the doctor was going to say today is the inducing day.  So we went into the exam room and Maria had her blood pressure taken and then they listened to the baby heartbeat.  It was one of the last times we were going to hear that with a monitor at least we hoped.  The doctor came in the room and said it looks like today is going to be the day you will be induced.  Talk about a big sigh of relief.  Maria wouldn’t have to suffer anymore itching and we were going to be meeting our miracle very soon!!

We were told to arrive at 4 pm that day and the induction process was going to begin.  I think we were so excited, nervous, and anxious at the same time.  We headed home to pack for our stay in the hospital. All the baby stuff was ready to go including the car seat.  I made sure to bring all my lovely CF stuff too.  When we packed the car it looked like I was going to be the patient and not Maria. 

Once we arrived at the hospital that was the last time we were just going to be John and Maria.  The next time we were going to leave this hospital we would be a FAMILY! A family of 3!!! WE were so excited to meet our little miracle.  T

hat day was the end to our CRAZY IVF ride.  It was only going to be hours away from us putting the finishing touch on that journey.  WE had the deck of cards stacked against us to begin with but never once was this family going to back down from our dream of a family.  As a friend pointed out to me the other day he called Maria and I the BULLdozers.  I guess that would pretty much sum us up.  If you put something in front of us we are going to plow right through it.  

Still a year later I can remember this day so vividly.  I will never forget how we felt about starting the next chapter in our life.  In less than 36 hours we would be meeting our miracle who changed the rest of life!!!

WE LOVE YOU Alayna!

 

Click here to read our birth story.

Here is a post that explains our IVF process.

 

 

Ready to start the induction process!!!

 

Last picture before the hospital gown came on!!!

 

 

 

 

 

I'm Back...........Here's An Update!

I’m back!!!! Well at least for this blog post anyway.  When I do not update or write something on my blog every week or so I truly do miss coming here to blab.  I know there are others in the internet world who come here often enough to see what is going in my life, my family’s life, or to read what I have to say. I thank you all for showing an interest in what I have to write about.  I guess that means what I am doing is impacting at least one person! So I apologize that I haven’t been on here to give updates or to blab about what I have found relating to our cystic fibrosis community.  It’s not that I do not have anything to blog/update about.  What I do not have is free time to commit to this as often as I may like. 

 

The month of September was very busy and October looks like its going to turnout that way as well.  For me, I love to be busy and active as it keeps me going! The month of September started off with us discovering why Maria has been feeling the way she has for a long time now.  I blogged about it in this post.  It was another discovery that was added to my wife’s list of life changing events these last three years.  Sometimes in life, things just start to pile up on people all at once, or so it seems that way.  There is a famous quote that people like to reference “God gives us only what we can handle!”  I do believe in that quote but sometimes it just doesn’t feel like in life you will ever get a break.  From my perspective it hurts to see the woman I love more than anything in this world having many things thrown at her at once.  I truly will never know how it feels to be in her shoes let alone anyone else’s.  But through these past three years, Maria has taught me invaluable life long lessons that I will never forget.  Those last three years the deck of cards has felt like it has been stacked against her and us on numerous fronts but somehow someway we have overcome them.  She has shown what it means to be involved in your own health. Never once has she given up on trying to find out the reason why she wasn’t feeling right.  There were doctors who said nothing is wrong and she could have stopped there but she was determined to find out the reason she was not feeling 100%.  She pushed this hard because she wants one goal to be on this planet for her family.   For all of those who ask me how Maria is WE thank you for keeping our family in your thoughts!

 

Also, in September we went on our first family vacation with Alayna for two weeks.  It was a big adjustment for us all but more so for Alayna and me. When we go to the beach we usually do whatever and whenever we want.  For me that means fishing all day from the beach! For Maria that means falling asleep while listening to the ocean in her beach chair.  This vacation I hardly went fishing and Maria did not fall asleep for one single minute on the beach.  All the whatever and whenever stuff that we do at the beach went out the door.  But I was able to see what its like being a SAHM (stay at home mom).  Let me tell you it’s not easy at all but it is the most important title a mom or dad or even babysitter has.  As I work full time and basically only see my girls for a few hours a day during the week and then the full days of doing errands on the weekend it really opened up my eyes.  I guess I didn’t realize how active Alayna truly is.  As my mom says she is a busy body!!  I honestly cannot say enough what it means to have a dedicated SAHM!  

 

Once we did get into a routine and were adjusted after the first 5 or 6 days we didn’t’ want to come back home. As for Alayna, she loved the ocean and loved the pool. We really didn’t take her out until after 4 pm since she is so incredibly pale. The only thing that bothered her on the beach or going to the pool was sunscreen.  She hated having sunscreen rubbed on her. We did have a great trip and it was much needed to get away.  We made some great memories and took hundreds of photos.  It looks like vacations will no longer be about Maria and myself like they use to be.  Next time we know what to expect and it should be a lot easier once Alayna can walk on the beach!

 

October is a special month in this house.  Alayna will be turning 1 year old on October 17^th! Uhm really???? I cannot believe I am typing this.  Where has the time gone? If this is how life will be from here on out I need to get more timeouts in this game.  Alayna is growing faster than I could ever believe.  I will keep her latest advancements until her official 12 month post. She is getting more and more active every day though.

 

Last but not least, my CF update.  As many of you know that read and follow this blog, I have a boring CF life.  I prefer it this way. Not much is going on in that department minus the upcoming clinic visit close to the end of October.  I am anxiously awaiting to get back to clinic as the last time I didn’t feel 100%.  I was battling a cold and my PFT dipped.  As I just reread that post, I told myself that I wouldn’t let my lower PFT score bother me.  I actually can say I haven’t really thought of it that much since my visit. It must be because I was able to get over that cold with finally no antibiotics. I credit that to my HTS and my compliance streak. Today, marks 250 days of 100% compliance.  I know that compliance streak is paying big dividends for me!!! Anyway, I am hoping this next upcoming clinic visit will show that my numbers are going in the right direction. 

 

I did finally manage to get a new compressor for my aerosolized nebs. My old machine was a dinosaur coming in at well over 10 years of service.  The reason I was in the market for a new compressor is I noticed over the last month or two I wasn’t getting my full neb of hypertonic saline in my 20 minute airway clearance session.  I would look in the neb cup once I was through vesting to see that half the neb was still left. So I began researching what kind of compressor to get. I went on cystic life and found that people really love the 50 PSI compressors made by Mobilaire.  The 50 PSI units reduce the amount of treatment time it takes to nebulize aerosolized meds.  So I talked to the respiratory therapist at my clinic and she said the best compressores to get are the 50 PSI and good luck getting insurance to approve them.   I found a medical equipment company who would take my insurance and I was able to have them cover my 50 PSI unit.  I did not get the Mobilaire unit as the medical equipment company was not able to order the unit. But this Medquip compressor is really a fantastic compressor.  I can control the amount of PSI (pressure) that is going to the neb cup for my own comfort.  So far I really love the compressor. I have been using it about a week now and I would never go back to a compressor that doesn’t allow you to control the PSI setting.  It is like night and day with this unit.  Now my neb cup is completely gone in less than 15 minutes. Not only is my neb cup empty I am hacking out a bunch of mucus during airway treatment.  I LOVE HTS and my new compressor.  If your compressor is on its last leg I suggest looking into getting a 50 PSI compressor.  You will not be sorry!

 

I promise I will be back on blogger soon as I have much more to blog about.  My next post will be Alayna’s 12 months and then after that I will most likely talk about my CF clinic appointment at the end of the month.  I did find some cool stuff to blog about in the coming posts we will see if I find the time in my busy life.  Here are some of the upcoming topics I plan to hopefully blog about.

 

CF Patient Registry data: USA vs. UK what are we doing right and what needs to be improved in the USA CF wise.  PFT data, bacteria data, median age, antibiotics, clinic visits, airway clearance methods, etc. will be blogged about.

 

Vertex and Kalydeco:  How much did it cost Vertex to bring Kalydeco to market?  Why does Kalydeco cost so much? Can the US healthcare system afford Kalydeoc?

CF and Stem Cells: This topic is very interesting and thanks to a friend for emailing me some great articles on CF research in general.

Cross infection in Cystic Fibrosis: Is it real? What can we do about it? How will it change the CF landscape for years to come?

 

****I also made a list of things that I would blog about at the beginning of the year and I have yet to get to some of those. So I may not get to the above list like I may want to.  But if any of these topics interest you and you really want me to write about them let me know!***

Picture time...............