• My Family

    Blessed by the Lord for the Joy of family!!

  • Rachel’s Daily Cystic Fibrosis Medications

    Albuterol (2-4 x)
    Hypertonic (2x)
    Pulmozyme (1x)
    Glutathione Inhaled (2x)
    Advair (2x)
    Prevacid (2X)
    Ursodial (2x)
    Glutathione oral (3x)
    L-Arginine (1x)
    Acidophilus (3x)
    Vitamin (2x)
    Periactin (2x)
    vitamin d3 (1x)
    Cal/Magnesium blend (1x)
    ADEK (1x M,W,F)
    Creon 12 (6 with meals and snacks)
    Vest (2-3x) 30 minutes
    Percussion (30 minutes)
    alpha lipoid acid (1x)
    singulair (1x)
    fish oil (1x)
    nasonex (1x)
    zyrtec (1x)
    azithromyicin (M, W, F)

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First stop, weight, height, blood pressure and o2 sats.

Love how the clinic (and in particular, some little old ladies from a club) blessed us sometimes.  Rachel received a “fun bag” today.  It was specifically for pulmonary patients. This was a huge blessing on this particular visit!!

Well, Rachel and I made it through another appointment.  It was one of the hardest ones so far, but for different reasons than would be expected.  It was a good reminder that we never know what to expect.  Going in, I was a bit concerned about several different aspects of her health.  Most of which, were actually alleviated half way through the appointment.  The sore throat, is a common side effect of one her med’s that coats the vocal cords.  Dr. said that side effect can pop up at any time so changed delivery of that med.  Rachel’s weight was…okay, down a bit their scale, though not too much was made of it.  Still waiting for cultures.  But…her pft’s were the highest that they have ever been!  Whoohoot. (I think the dr.s let the weight slide for the greater good of having good numbers on her lungs.)

PFT’s were awesome!!

The doctor was thrilled were her lung function test especially since she is having some symptoms of allergy/inflammation somewhere.  Her FEV1 was 91% which is almost 5 points up from last visit.  I was amazed.  I was really expecting this number to be much lower.  However,  the one that was really surprising was her FVC the number that tells the strength of the lungs.  Still working on understanding all of these numbers, FEV’1 is currently the one the doctors look at the most  because it shows how much air can move in and out of the lungs, and the speed and strength of the chest wall in 1 second, as well as the level of obstruction and restrictiveness in the small airways.  However, in years past the FVC (forced expiratory vital capacity, the total volume in liters that the lungs are capable of producing)  was the only one that they used to look at, so dr’s have changed the “importance” of each in these numbers recent years, even though they both tell a story.  Her FVC number went up over 11 points!!  It was at 99 and it came back at 110!!!  The doctor was very surprised to see this number move.  When I told him that we have been running, he smiled and said, “Keep doing it!”  As a family we have been trying to run at least 3-4 times a week for about the last 6 weeks.  We have made Rachel run at least 400-800 m. but she has gone as far as to run/walk a mile and a lap!!  He was very excited to hear this.  He reiterated that exercise is one of the key factors to keeping her lungs health and strong.  Guess we will keep running! =)

So, after the weight check, the pft’s and the actual check up, I talked to my doctor about the new medication, Kalydeco.  I have been reading up on the fact that there was a small subset of DDF508’s that actually did respond very well to this medication (have a medical journal of 217 pages that I have been slowly perusing to gain this info).  DDF508’s number was small, but it was there.  So in talking to the doctor, he actually said, “Well, maybe you could be the test case in our clinic!  However, the trick will be figuring out how to get the insurance to pay for it.  It’s worth a try though!”  I was super excited to hear him say this!!  I think when you have a child that has a deadly diseases it is like a ticking time bomb day after day, the possibility and chance to change that future is beyond imaginable.  Just the thought of it was overwhelming.  I do understand that trying this drug would be a long, loooong shot, and there are actually a ton of things to continue to research and figure out before we would continue, but still the thought was so hopeful that there might someday be a hope for Rachel’s future with freedom from some of the weight of CF.

What I remember.

Well, everything was going great right up till this point!!!  After we discussed the “off-label” drug use, the dr. said the first step was to check the sodium chloride levels.  We would need a pre and post numbers to compare the effectiveness.  Then he started looking throughout her chart and realized that Rachel had never had a sweat test done.  She was diagnosed through a DNA testing due to her meconium illius at birth.  The doctor couldn’t believe that we had never had her tested.  Clearly she has CF, and the DNA test proves conclusively, so there was never really a reason to have the sweat test done.  (The sweat level is the way that the researchers are tracking the effectiveness of the new drugs coming down the pipeline.)  I, of course clearly remember having the boys tested the day after we received the news about Rachel’s CF.  I wanted/needed to know for them.  They both came back clear, and it was relatively pain free, or I may have been in a state of shock still?

We were greatly blessed with Rachel because the newborn screening was not approved until she was 10 days old and she may have been one of those kids that would have been missed.  (I have heard the horrors of parents that struggle through the first year(s) without a diagnosis or treatment with a CF kid.)  I very specifically remember this date because it was one of the longest days of my life.  Rachel was just 10 days old.  It was the day that her lung collapsed and her heart failed and we were not sure that she was going to make it throughout the night.  We watched her CO2 numbers every 15 minutes throughout the night to see if those numbers had begun to stabilize and start to move in the right direction.  We were literally up all night praying and watching!  When the morning paper came around about 5am the headline was, “Cystic Fibrosis Testing Approved in Nebraska”.  I remembered thinking this was the first time I had every seen the words Cystic Fibrosis in writing.  Despite the excruciatingly long night, Rachel made it through, and there was hope that although her fight was huge before her, she had lived to see another day!  Anyway, all of that flashed through my mind when the doctor suggested getting a sweat test.  I was all for it.

The very antiquated box for testing of CF.

Preparing the straps for the test.

As they brought out the archaic machinery, that should have been my first clue.  The box looked like it was from the 1950’s and then the respiratory therapist said it was older than she was.   Pretty sure that it was!!!  The RT hooked Rachel up and we proceeded to have the next 20 minutes of sheer torture!!  It was among the worst appointments that I have ever been to.  I had to hold and attempt to comfort Rachel while they shot the electricity just under the surface of her skin in order to elicit a response of the cellular level and obtain a sodium response.  The RT said some people feel like it is just mildly irritation, and others feel like it is painful.  Rachel was among the far latter!!  It was miserable.  By the time we were finished with her right arm, I was done!!  THEN WE HAD TO DO THE OTHER ARM!  Rachel was beside herself, and so was mom!  You can only tell her it is almost done so long.  My muscles were tense from holding her and keeping her from ripping the panels off, not to mention seeing her scream in pain for minutes on end.  It was heart wrenching!!  By the time the test was over, she was a hot, sweaty, sad mess!  I was literally shaking by the time we left the room.  It was crazy and all I could think about was the fact that she would need to have a sweat test to enter in the clinical trials as well as have one when the study was finished…made me rethink.  Although, if they let her get in a good, viable study, I will sit on her to make it happen kicking and screaming if I must.

Rachel was sure her arm was going to fall off…or at least she sounded that way for the ENTIRE TIME OF THE TEST. This picture was actually taken as the RT was preparing to take it all off. Should have seen her during the test! Whew!

Still mad, hot sweaty and ready to be done. Notice the plate size and her gripping her arm. There was a plate on both sides of her arm so the total surface area was pretty significant compared to the one at Children’s. WE WILL GO THERE NEXT TIME TO GET THIS DONE!!!!! =)

When we finished, I looked at the RT and said, that I didn’t remember this being that bad on the boys.  She then informed me that the ones that were at Children’s was much easier and significantly smaller.  Like the difference of a dime and an orange for surface connected to her arm!!  INSANE!  After the electricity push through her arm, we then had to wait for another 30 minutes to gather the sweat.

Still not happy.  But much, much better.  Her arm wrapped up in plastic wrap and sealed to allow for no occlusion of the salt. Her arms were bright red for about 3 hours afterward where the panels touched her. =(

Praise the Lord for a booksale in the lobby of the hospital, I was more than thrilled to let Rachel go pick something from the sale.  I felt so awful.  I work really hard to tell Rachel the truth on every appointment and procedure that she must endure.  When she asked me about this one, I honesty didn’t remember it being this awful.  I have had to hold her through some difficult things, but this one was definitely up there with one of the top and one of the worst!  Now to wait for a good culture!

The upside of this all, Rachel has blood draws and other labs coming up at her next appointment and she thought they sounded like a piece of cake after this whole ordeal!!! =)  Seriously, this is so NOTHING compared to what so many CF people deal with.  I wrote this to remember and give perspective for myself.  There are so many that have feeding tubes, and pic lines, and oxygen running through the day and night, and all sorts of other yucky stuff going on.  This was just an unexpected part of an appointment.  I have said it before, but each and every appointment is a reminder that we are blessed, but that we are in a fight for Rachel’s life and breath.  While this WAS actually a really good appointment, it was also a reminder that there are so many kiddos (and mama’s watching) with CF that have had to endure so much.  It is hard to watch your baby suffer!  No matter if it is a skinned knee or a test to check CF levels.  It is truly hard to complain, even a little.  We have been blessed!  This little hiccup… just caught me off guard and was a reminder why it is good to have Dad coming with to appointments just in case!!!  Daily I pray for a cure and for strength for those that battle this disease and those caretakers that must walk beside!!  It is tough, but God is able to bring comfort.  Praise the Lord for His goodness and mercy.  Will let you know about her cultures when we hear.

One last note, thank you again for all of you that have given to CF.  Our walk is next Saturday.  My heart is overflowing with gratitude to know that we could have an appointment that even discussed the possibility of a drug that changes lives!  It is because of your kindness and generosity that we have that hope!  THANK YOU!



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