CF Clinic Appointment Update

The word is out at least on other social media outlets that I haven’t been feeling like myself this past week.  This all started during the night when I was awoken by a coughing fit.  To all of my CF friends, you understand how ANNOYING and completely out of the blue these can be.  I haven’t had one of these coughing fits in who knows how long maybe a year!! I am thankful that this is not the normal for me.    

But I did forget what it was like to have one of these episodes. I could feel all the mucus laying in there and rattling around.  My first initial thought was what is all this about.  I am not sick and this is so UNLIKE me.  I did a bunch of huff coughing and the mucus was thinner and lighter in color than my usual sputum.  So does this mean something new is growing in there??? I have had this happen before and I think that Staph was the culprit at least what I can remember. Not only was I privileged to experience this for one night, but I had 3 of these lovely nights.

After a recommendation from my cyster friend Beth, I got out my albuterol puffer before I did my HTS treatment.  This seemed to allow my airways to open up more to cough out that thin mucus. I can say that I have not had a coughing fit since last Thursday night thanks again Beth!

I decided to make my CF clinic appointment due to all this coughing at night.  This past Monday Maria and I made the trip to clinic.  I wanted my doctor to see my thinned out mucus and get this cultured to see if any new bacteria is growing.  In the car, I did cough up some mucus and thanks to my CF MENTOR, Anne I did remember to bring a Ziploc bag to cough this into. Does anyone worry they will not be able to produce any mucus on demand??? This Ziploc bag or sputum cup idea will help us! Thanks Anne!!!

On a side note I suggest every one in our community find someone who you feel is your CF mentor.  I am beyond thankful to have my second cousin Anne as someone to look up to.  She has been through some wars herself including a double lung transplant. Soon many of you will finally get the chance to “meet” her on blogger.  This will be something new I plan to rollout. More on that in a later blog……………..

Back to the appointment…. My doctor said my lungs sounded good. There was no rattling or anything like that.  But again all the rattling was at night.  I usually get that same report no interesting findings which is fine with me.  My weight is always good in fact one of these days I am going to have to drop a few pounds!  Then it was time for my battle  with the PFT. 

I knew coming into clinic that my FEV1 wasn’t probably going to be the greatest but I still thought just maybe I could see the same FEV1 from last clinic.  Even at my last clinic my FEV1 was lower than usual 69.  Into the PFT chamber I went.  First blow is in and it’s a whopping low of 62. UHHHHMMMMM NO thank you PFT machine.  I hate seeing a number like that.  My baseline for the last 2 years has been a range of 68-74.  After a few crazy thoughts run through my head (more on those thoughts in another post) it was time for blow number 2.  My second attempt came in at 64.  What the heck is going on??? At least it’s moving up.  Third attempt came in at 66.  Well I am not thrilled with that number but I am also happy it’s not any lower.

After the PFT attempts I talked with my doctor.  He said well I think I will start you on some antibiotics once we receive the results of the culture.  John call me in 8 days!! What 8 days that’s a long time but here is why. I can recall that culture results are not final until close to 10 days after the mucus is collected.  He does not want to just give me antibiotics without knowing what I am culturing especially since my mucus seems to be different. In my last culture I had the usual staph and achromabacter. I don’t know if the 3% drop in PFTS warrants an antibiotic but I will see how I feel once the culture comes back.  The wonderful thing is that I have not been on an antibiotic since November 2012 which is a very long time.  I think if he prescribes me a breathable antibiotic I will not remember how to even administer the drug.  YIKEs!

 

 In other exciting news, I finally got my doctor to buy into looking into Kalydeco for my gene.  I have 3849 10kb C->t gene which does respond to Kalydeco ALONE!!! YES, I know this for a fact as some people with my gene are seeing improved health from Kalydeco.  As many of you know in the CF community, Kalydeco is not FDA approved for any gene but G551D.  For me, to actually have the insurance pay for this is going to be like a David vs. Goliath fight.  But my doctor said the research coordinator at my clinic will be calling Vertex about Kalydeco and my gene.  I am super excited but very cautious on this as my doctor still wants evidence that Kalydeco alone will work for my gene.  He wants proof on a larger scale its safe and effective.  So some of you that I know who are taking Kalydeco who have the 3849 gene I may be in touch with you soon.  Again I am being very cautious on this. 

Lastly, at clinic it was my last research study visit for the study I am in.  Basically, the study is a questionnaire format and monitoring FEV1.  A lot of questions about your mental health and such are in the questionnaires.  I honestly enjoyed talking with my nurse coordinator. He lives a very similar life like Maria and I minus the CF aspect.  We had a good chat about kids and the nuttiness that goes along with being a parent.  He almost did a play by play of our same day to day routine.  So no wonder why its challenging raising a child, working full time, doing family chores, being a husband, being a father, and throw the lovely CF on top of it.  My plate right now in life is very full.  So if any of you have any extra plates or room on your plate please let me know.

All in all, it wasn’t the worst clinic or the best.  I guess we will take the wait and see approach on the culture results.  I want to say THANK YOU to all of you who wished me luck on my clinic and everyone who was eager to hear my results.  I truly do LOVE this community as we are all in this WAR together!  Keep fighting my friends and always remember “It’s a GREAT day to be alive!!”

 

Alayna sitting in a pan!