• 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)

  • Recent Posts

  • Monthly Archives

Interesting 1st Day of School Field Trip

IMG_1741

                            Fun morning!

IMG_1733IMG_1723

Well today kicked off the official start of our school year.  We did our normal activities.  Back to school pictures, math, writing, history, english, Bible, and enjoyed a time of just connecting with one another.   However, this year was a bit different.  Late morning we had our first field trip of the year.   The older kiddos went with Rachel and me to the hospital to perform some tests.

IMG_1705IMG_1717IMG_1703

IMG_1750

Let me back up a bit. About three months agoRachel was approached about entering a clinical trial.  It came on the tails of a pretty crummy appointment.  God’s perfect timing!!  (This drug had just been approved for older patients with cystic fibrosis, but with the steep price tag of the drug…the odds of getting insurance to approve it “off label” (meaning anyone that does not fit the exact parameters of the recent study.  And since Rachel is 2 years from the minimum age, it seems highly impossible for her to access the drug!)  So this study would be a plausible way for her to get the drug earlier than the two years.  And with cystic fibrosis, time is certainly of the essence.

IMG_1388 copy

Rachel’s first question about this test, “Do you mean I can’t smile?” CRACKED me up. Only she would say that. Her lips had to be sealed around the mouthpiece allowing no air to get in or out. The machine would detect every little bit. As well as a swallow (they were not to move their tongue, mouth or even swallow saliva). Top it off that those nose clips were SUPER clips. She uses some like that at every appointment when she does pft’s, however these allowed NO air. Every kid had red marks on their nose when they were finished. They said it hurt a bit. Rachel’s test took about 45 minutes compared to everyone else’s of around 10 minutes. Her nose was pretty red. =)

The emotional angst of our whole family in prayerfully considering how to prepare and proceed with this trial has been a growing and stretching experience.  Rachel was given 1 of 2 guaranteed spots for this trial!!!  Talk about AMAZING!!!  Incredible!! GOD ORCHESTRATED for sure!!!  So we have sat on and prayed over this news for the last couple of months. There is great anticipation that this drug has the potential to seriously CHANGE RACHEL’S LIFE!!  While it is not a cure, and there will still be many difficulties to overcome with cf…it will be a HUGE STEP FORWARD for her and those with CF.   However, when Rachel was first approached they begin telling her about what was expected and she literally started bawling in my sleeve as they talked about all of the blood draws, the sweat test (remember three years ago she had a REALLY, REALLY rough experience with an antiquated, adult sweat test machine) the long days at the hospital.  It broke this mama’s heart.  However, through the amazing power of prayer (us and close friends) by the next day, Rachel was starting to wrap her mind around it all.  Her prayer the next morning in our devotion time was, “Jesus, please help me to be brave and do what I need to do to help others with cf!”  Talk about a prayer that brought a tear to her mama’s eyes. One of my favorite things about Rachel’s young mind processing this trial is that a big deciding factor on participating in it was knowing that it would help other kiddos with cf!!!  I LOVE THAT!!

So on to today.  Our clinic researchers are still in the process of calibrating the machines and seeking the final approval to get the study started.  (I think they said today that the drug is already on site!!)  They needed 6 healthy volunteers, and 6 with cf to calibrate and ready the lung wash machine (basically calculating how long it takes for your lungs to get all of the nitrogen out of it by a flow of 100% o2 being forced in through an airtight mouth piece).  When I spoke to them several weeks ago, I volunteered knowing that we could help fill several of those spots.  Not to mention the fact that Rachel would get an opportunity to interact with the researchers and a bit of a look at one of the procedures that she will be required to perform.  Little did I know when we volunteered the AWESOME blessing of having siblings walking alongside Rachel.  None of them really wanted to go, but all conceded knowing that this would expedite the beginning of the trial.  IMG_1391 copy

Today the 5 of us (Grandma graciously stayed home with sweet Megan) crammed in a 10 by 10 room with two researchers, a huge machine, o2 tanks and watched as each of the children performed the lung washing procedure. Rachel went first.   She had the hardest time.  It took her more than double and nearly triple the time that it did the other kiddos.  As I watched her work so hard to do what the researchers wanted her to do trying her best, it was so clear that her body was betraying her desire.  The goal was to take the nitrogen in the lungs which is normally about 78% (with the other 20% being oxygen) to less than 2% nitrogen and 98% o2.  It was timed and the machine counted the respirations along the way.   My heart was aching a bit for her because she would get so close (about 8% or even 5%) and then watch breath after breath those numbers not budging.  I could see the frustrating in her eyes.  For me it was again the reality that while she looks and acts like her siblings…she is having to work twice as hard to simply breathe!  The researchers were AMAZING, and encouraged her alongside me.   Praise the Lord for gentle spirited gals.  Obviously, cystic fibrosis (and asthma) played the major role in why it took Rachel so long to clear her lungs.  But it was still somehow a stark reminder why we were there.  But it also had a great sense of  HOPE that life will soon be a bit easier for her.

When Rachel’s turn was over, there was great relieve and a grin from ear to ear that her little sister was next and brothers to follow. =)  Thank you, Jesus!  Thank you for working out the details.

IMG_1392 copy

A quick picture and snack while the researchers were recalibrating the machines for the bigger boys. =)

IMG_1394 copy

IMG_1399 copy

Caleb did great. He was last up, but pretty excited to show what he could do.

Unknown-1
While the day was long, (we were at the hospital for over 3 1/2 hours with about a 15 minute break to grab a quick snack) it was definitely a memory maker for our first day of school, as well as a team building experience for four of my precious kids.  Memories that these kiddos are not soon to forget.  Not to mention…they learned a little science along the way!!  Seriously, who gets to go learn about “actual” room air, nitrogen, and how a lung washing machine works on their first day of school.  Bonus! IMG_1806

IMG_1744IMG_1799********************************

BTW I did ask researchers what I could share.  They said as long as I don’t share clinical results, numbers or how she is actually feeling as she starts the trial, I am free to document.  So I am not sure how I will proceed.  It may be a hidden blog post until after she is on open label section (she will be assured to be getting the “real” drug then) which will be in six months??  I will need to seriously process a lot of emotions as we proceed.  =)  This is a double blind placebo study meaning that she will not be certain of taking the actual drug vs. a placebo.  The range of side effects have been all over the place.  But praying that she GETS the real drug for the trial (could make winter and cold season a lot easier on her), and that she deals with the side effects well!!  My God is ABLE!!  Appreciate the prayers along this journey.  Excited!  Prayerful! And leaning into Jesus…

Advertisements

Leave a Reply

Fill in your details below or click an icon to log in:

WordPress.com Logo

You are commenting using your WordPress.com account. Log Out / Change )

Twitter picture

You are commenting using your Twitter account. Log Out / Change )

Facebook photo

You are commenting using your Facebook account. Log Out / Change )

Google+ photo

You are commenting using your Google+ account. Log Out / Change )

Connecting to %s

%d bloggers like this: