• My Family

    Blessed by the Lord for the Joy of family!!

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    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)
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    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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Game Changer

This week one of the drug studies showed some great promise while not a “cure” per say, it has the potential to be a MAJOR GAME CHANGER!!.  The drug VX 880 is being used with the already approved Kalydeco (VX 770) in the Delta mutations, which Rachel has (she actually has a double copy of that mutation so she is a DDf508).  Several weeks ago there was a lot of chatter that this particular study had not met sevearl end point for statistically improving CF patients lungs.  The company had even warned the market analysis of this possible negative outcome.  However, yesterday despite the lack of one of the endpoints, the drug study actually revealed an improved lung function.  LUNG FUNCTION IMPROVED FOR 76% of CF people!!!  The stock market also went up 54%!!!  The initial marker that researchers were looking at was the sodium chloride levels and in Kalydeco, the NaCl levels went down and in some cases the turn upward in lung function followed.  But, overall in Kalydeco the lung function had risen in that study by about 10%.  The researchers were looking for those same markers.  However, the sodium levels did not drop.  Amazingly, the lung function in 46% of the patients went up by 5% in one month and 30% of patients went up as much as 10%.  Unbelievable!!!  This says that there is a high possibility that this combination drug will have much of the same results as the Kalydeco.  That is our prayer!  Even a small increase in lung function, translates to the fact that the CF person is not “loosing” the function.  With 2-5% loss each year, by the time a patient is 25 there is potentially little function left of the lungs. This study if translates throughout the next study will be HUGE!

Now, there are still many unknowns and I questions about what this means for future studies (Rachel is quite possibly at the age that she would be eligible to get into some of the phase 3 studies!!!!  WHOOOHOOT!!!).  We will have to wait and see when the end results of the study are released as to what the collective plan is as far as the next stage in research.  I do have to say that I was just about jumping up and down knowing that there is so much hope for a “game changer” in Rachel’s life.  We  know that Rachel will always have CF, and she will still have much to contend with for the rest of her life, but it will be similar to insulin for a diabetic.  People still have diabetes, but insulin greatly improves their quality of life and longevity of life.  The possibilities that life could be drastically different and drastically LONGER is hard to comprehend.  I have not let myself “go there” for more than a moment.  Knowing that for now…we must keep the path.  Her lungs have to be well enough to benefit!  So we will continue to work on her health for TODAY!  But…just for a moment yesterday, I dreamed!  It brought tears to my eyes and a joyful song to my lips in praise of the Lord most high.  He hears our prayers!  If and when that “game changing” drug comes…we will have the biggest celebration ever known to our family!

CELEBRATE

hope,

life,

breath,

goodness,

faithfulness,

and health!!

In the meantime, we have a walk coming up this weekend.  Although the walk is a great time each year, there is still a sobering aspect to this day that is so very difficult to explain to others that have not walked this road.

 

The first youtube is a little hard to watch.  To see Eva struggling to breathe…is a charge to keep fighting.  (I posted the second one, although it has much of the same footage as the first one, to show you that it was on Oprah’s t.v channel this week.)  Pretty cool.  May is CF awareness month.  Please continued to share, give, fight and work to make this disease change forever!

The name of this documentary on Oprah’s channel is called 65 Red Roses.  (I have yet to watch it, but hope to shortly.)  It is about a young girl, Eva, that walked the road of CF valiantly and with great courage and spirit despite the many hardships and ultimately loosing her life to this disease.  I did not know her other than through the blogs/internet, but watched the struggle of her final days here on earth.  Heart wrenching to say the least that this is the plight of most every CF persons.  I can not imagine what it is to not be able to breathe.  Or worse yet, to watch my child struggle for breath.  My heart breaks at the thought that we have had some tough times, but without new treatments and therapies this road that Eva traveled could be Rachel’s.  By the grace of God, I pray that it is not!!  On the eve of the great research news, I have hope that Rachel’s life will be different when she is in her early 20’s!  But, I must also be real, there is still that small fear in the back of my mind that CF has had many great breakthroughs over the years and maybe it will still take a decade or two to get a “game changer” for her.  In ten years, what will Rachel’s life be like?  Will she still be relatively healthy, or will she need a lung transplant, will she be full of life, or struggling?  Those thoughts are in the back of my mind constantly.  As much as I push back on them…they are there.

So, as I contemplate the walk this year, I choose to focus on the hope that soon the lives of young people will be forever changed.  I continue to work at spreading awareness, and raising money to keep the research going!  I do this, because I must.  I do it because I have a hope that there will one day soon be freedom from the fear of CF.  Hope that CFers will have freedom  to breath easy with less tubes whether they be nebulizer tubes, or feeding tubes, or oxygen tubes, or iv tubes.    That they would experience freedom from the great burden of medications, and treatments that are just a part of CF life.  Freedom for Rachel to be a kid…no rather, for her to be able to dream about growing healthy and very old!  Just that thought brings a lump to my throat.  Eva did not have that chance to grow old.  She, like so many others with CF,  lost her young life to the silent grip of this disease.  Take a moment to watch the videos.  It is impactful.  The thing that struck me the most, was how mostly normal she looked even as she got the call for the transplant.  She ran?  She still looked so good on the outside, although her lungs were done!  It was a reminder that CF is a somewhat silent, hidden disease and yet it grips people’s lives.  But it doesn’t have to be that way!

 YOU CAN MAKE A DIFFERENCE Please come out Saturday and support our efforts for a “game changer” in Rachel’s life.  The Great Strides walk brings that hope to families.  I am not sure how, but somehow it provides hope and strength to know that we are not fighting this awful disease on our own.  Having a team of people fighting for my child’s life and breath is one of the most amazing blessings.  It is a reminder that God walks alongside us and that He sends friends and family to share in that journey with us!  Thank you for your love and care for our family!!!  It really does mean the world to us…game changer or not…we are blessed!

If you are able to make it~please come.  We would love to see you!!!

 

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One Response

  1. Just found your blog through another CF one. I’m looking to connect more with other CF moms, esp ones who believe in our Lord Jesus Christ. I have a 9 year old boy with CF. I would love it if you emailed me to perhaps chat and if you could share some of your community sites that you go to.

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