• My 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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Busy Week, But Pressing ON

This has certainly been a busy month in our home. Actually a pretty busy last couple of months trying to figure out health, family life, school, sports and just trying to keep up.

Our set up for the Eclipse ball of the Tobramycin in the form of iv infusions.

So thankful for advances in medical delivery. Instead of pumps and hanging bags of iv medications, this was the delivery system. A ball filled with liquid that didn’t have to hang, but could be sat beside her and easily carried around while infusing. Small but grateful blessings.

It seems like it has been months since we were in the hospital and even several weeks since we were doing the iv infusions at home.  Reality, it was just 5 days ago! CRAZY!!! While we are extremely worn out (all of us in ways that somehow don’t even seem normal), we are pressing on. Such reminder that life goes on.  Thankfully~

Monday we went to the hospital to get midline taken out. We were smiles going in. Rachel wore her Never EVER give up shirt, and I wore my grateful shirt. Fitting an appropriate.

Waiting to see the doctor.

We had no idea what to expect from the appointment, in that we thought they would get some pft’s, then pull the line out and we would be on our merry way.  However, it was not quite like that.

The pft’s were pretty low to start out with. Upper 60’s and low 70’s.  Finally she squeaked out an 81%.  Unfortunately, we had an issue with style of the test.  We were at Children’s instead of UNMC where we have been since she left the NICU. The Respiratory Therapist is new, the nurses are new, even the dr. is a new one to us.  That being said, we were informed that she has been doing the pft’s incorrectly for the last…6 years!! As long as I can remember Rachel has stood to do her pft’s.  We were told that is the INCORRECT way to do them and that they alter the numbers making it a bit higher than average. BUMMER!  Rachel has never had high numbers.  Decent, but not great. After 11 weeks of oral, inhaled and iv antibiotics…we would have expected them to go up a little.  However, they did not. Also, as another kind of yucky part, the doctor said that she heard “crackles” in Rachel’s lungs.  I am pretty sure that NO ONE has ever heard crackles in her lungs.  I am not sure what to say about that, other than prayers for God’s grace and direction.

The doctor and I discussed what might be the issues affecting these numbers. To give you a little background information on these numbers.  There are many people Rachel’s age and even older that have numbers in the upper 90’s and even OVER 100%.  So to see the upper 60’s…that kind of freaked me out.  81% can be dealt with, but the average person with cf slides 3-5% every year with this lung function.  So any slide in those numbers or the inability to get them up despite iv’s and antibiotics is a bit frustrating.

Picture of Rachel after she had 6 puffs of albuterol that made her feel super jittery and like she was going to throw up! Add to that the pain of the line being removed. The tape and the movement of the line trying to get the tape off without moving it was too much for her. She ended up getting clammy, lightheaded, and sweaty. It could have also been a blood sugar issue as it was after 1:30. She was fine after a few moments reclining and getting a bit of a snack in her.

One thought was that her asthma component was more significant that first thought. We did a pre, and post broncodialator a few years ago and noticed about a 10% increase with more albuterol.  So we added a long acting steroid to her regiment and that was helpful.  When tested this time, it did provide a 3% increase, which is not significant, but interesting.  We also talked about the fact that Azithromyicin was pulled from her regiment last year when she cultured MAC after her bronchoscopy.  Azithromyicin feeds MAC, so it was unsafe to keep that one as an inflammatory agent with her cf care. HOWEVER, maybe it was doing more than we thought that it did. She has been on that regiment for about the last 8 years or so.  Anyway… all that to say, we still don’t know WHAT to do to get her function up.  She doesn’t currently have any issues, which we are thankful for, but on the flip side, when she does, it affects that function pretty significantly.  Those with cf can fluctuate lung function of 20% or more pretty easily with a cold or exacerbation. That’s where the concern for the future comes in.  We need these numbers as high as we can get them for as long as we can.

Happy as a clam to be home AND WITHOUT a line in her arm.

The doctor was not comfortable with keeping the midline in any longer due to the toxicity concerns which the medication is known for (affecting liver, kidney’s and hearing to name of few), but still thought Rachel could benefit from another month of the inhaled version of the iv’s she had been on for the last week. So while that kind of felt like a slap in the face to me and ANOTHER BURDEN too heavy to carry…my sweet girl smiled broad and bold and replied with, “At least the line will be out of my arm!” Bless her heart and perspective.  LOVE THAT I GET TO LEARN FROM HER STRENGTH, INSIGHT & PERSEVERANCE!

The girls had a blast dressing up for a Hallelujah party at church with friends. They designed and helped with their outfits. Rachel was a Warrior Princess, she picked it. But I couldn’t think of anything SO FITTING for the last couple of months. Shiloh was a gum ball machine. She did an AWESOME job on making this! And Megan, loved the idea of being a baby bumble bee. It was a fun evening of “normal” life for our family after the crazy last couple of weeks. THANKFUL!! Oh, and they each won a prize for their costumes. Bonus!

So the current plan is to do the next month of inhaled antibiotics, and retest in 6 weeks to see if she has any improvement.  Hard thing with this is that she will have been on antibiotics for 15 weeks since August.  We are doing all we can to support her immune system and tummy, but those are issues that she is contending with as well. =( BUT…WE PRESS ON!  Because of Jesus, and THROUGH JESUS!! ALL THINGS ARE POSSIBLE!!

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