Doctor Appointments..........CF Clinic & PFT!

Monday was a doctor filled day in our household.  Maria had an appointment in the morning which meant I was going to stay home with Alayna.  Maria’s appointment went well! She did receive some news, some advice, and a going forward plan of what has been bothering her since her c section.  Yes that is right she still has not been the same since giving birth to Alayna.   I hope that will be changing in the near future.  But Maria has been nothing less than a real trooper these last few months.  When I think about some of the things that she has been through in the last year I am lost for words. I know things will improve soon for her. 

As soon as Maria came home from her doctor appointment it was my turn to leave and make the journey to my CF clinic.  I always go with my entourage to the clinic but this time I did it without Maria.  My mom went with me this time! Thanks for coming to my appointment mom! It is nice to have people that care about your health in your life. 

We left my house at 2 pm and returned at 6 pm.  Like I said above, it was a journey and it always is going to clinic. In fact, 2 hours of this journey are spent in the car!  I knew that it was going to be a longer clinic day because I received a call in the morning from a researcher at my clinic.  He was asking if I would like to volunteer in a research study.  I said sure run it by me.  The first study they wanted me to have an MRI performed on my lungs which was going to last 45 minutes to look at lung damage/disease.  Apparently, they would like to use MRI’s vs. CT scans when looking at lung disease/damage in CF because an MRI does not use any radiation.  This sounded very interesting but I was unable to participate on a short notice because they wanted to do it today.  The researcher asked me if I was interested in any other study going on.  I said sure as long as I don’t need any needles or blood draws.  I know there are very few of this type out there.  But he said I do in fact have a study that has no blood draws well it is an optional blood draw.  He explained the study over the phone and I said ok I am in.  So here is the details of the study.  The study is basically looking at lung function and how we feel and to see if we feel sick if there is a change in our lung function.  There are also a bunch of questionnaires you fill out every time you come to clinic.  They ask things about your compliance with treatment and your mental state dealing with CF. I will also be doing a PFT every visit at clinic now for the study woo hoo. I love me some PFT’s.  

Just like any study there are two groups. The first group does not have the little handheld spirometer (peak flow meter) or what I call the at home PFT machine.  The second group has the little PFT machine at home.  You blow in the little machine two days a week twice a day.  I wanted to be in the second group so I could monitor my PFT at home but I was randomly selected for the first group.  It is ok because this group is important to.  When I go to clinic now I will have to answer all the questionnaires and have a date with the big PFT machine. Also, during the study if I ever feel sick and call my doctor or nurse the researcher will want to see me so I can fill out the questionnaires and have a PFT performed.  This study will last a year so I will be seen by the researcher another four times this year.

After I met with the researcher I had my PFT performed.  As I have said on my own blog and other blogs,  this test is a real mental challenge for me.  I usually feel better than what my results tell me.  I was anticipating this happening again but wasn't 100% sure.  I have not had a PFT since August.  I usually do not have one every visit for whatever reason but that will be changing this year as I am part of the research study.  In August, I blew a FEV1 of 74 and an FVC of 96.  These numbers I was happy with because it was right after I started using hypertonic saline for about 2 months.  

My first attempt I blew a lovely FEV1 of 67. Uh oh was my first thought.  Of course I was disappointed and I continued to have a few more attempts.  I was given a score of 70 for my FEV1 and a 93 for FVC.  So basically, there was almost no change from my appointment in August.  Of course I would have liked to see these numbers higher but it will give me more motivation next time I am at clinic.  I honestly feel that this PFT test is a lot about technique.  I always have problems with this because the respiratory tech always says I need to cut back and use the "H" sound instead of me straining my vocal chords.  Any suggestions out there on PFT technique would be appreciated?

I then met with my doctor.  He is great. I always love to see him and I take full advantage of my appointment by asking many questions.  My lungs were given the usual saying.  John you sound wonderful no crackling or anything like that. As for my weight I was stable since August.  I have no issues in the weight department.  I continued to tell him how much I love hypertonic saline and he recalls the first time I was at clinic post HTS and how much I loved it.  

I asked what was in my culture the last appointment and it was still the same. I have not cultured psuedomonias since last April.  Almost a year. Woo hoo!!! I have no staph either in my lungs.  Woo hoo.  I do have two bacteria invaders in there though.  Acromobacter alasocksadens spelling on that and another bug.  But no meds for me.  It has been almost a year since inhaled meds for me another Woo hoo!!!   He left me with the continue to keep doing what you are doing because you sound great and your PFT is fine.  

We also talked about the status of Vertex and how they are coming along with the hopeful miracle drug for many of us out there.  I guess they are about to begin a phase 3 study enrolling people at my clinic in May and starting the trial in June.  I am not eligible for this as the study is only open to double delta F508. I do have one copy of the F508 gene so if this miracle pill is promising it will benefit me.  So the key here is stay as healthy as possible until this magical pill comes to the market.  

So I left my clinic feeling pretty good about myself. I volunteered in a research study so hopefully I will benefit from this or someone will when the study is all said and done.  My PFT results were pretty much unchanged which is great.  The biggest thing for me is I have been feeling great these past few months. I honestly do not know the last time I really felt CF sick which is AWESOME! I count my blessings every day on this front and I am so thankful for the health that I do have.  

Alayna smiling away! She is always happy!!

Me and the PFT machine!! 

This is my makeshift mask for the hospital. Avoiding the germs! 

The little cupcake sucking her thumb.