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We really like the phrase HOPE STANDS STRONG. (We might use it somehow on our CF Shirts this year.)

Where is your Hope? My hope is in the Lord!

Psalms 31:24 Be strong and take heart, all you who hope in the LORD.  Psalms 33:18 But the eyes of the LORD are on those who fear him, on those whose hope is in his unfailing love,

At our family reunion this last summer. Some of the cousins were sweet enough to wait for Rachel to get done with her treatment before they went off to play. So thankful for a sweet family.

Today there was some exciting news in the research of Cystic Fibrosis.  I think the timing it is particularly interesting in light of my post earlier.  Live like we are dying.  This development has the potential to add decades to the lives of CF people!!!  That is HUGE!!

God is our hope and our light!  We know that the number of each of our days is number by Him before we take our first breath.  There is great hope in that fact as we battle this difficult disease…this knowledge means that He also He knows what is in our future -EVERY PART OF IT!  That is comforting beyond all believe.  We can not add or subtract a single day to our lives…but He knows how it will play out.

Now this VX-770 drug may not be a “cure”, but it is the first time in medical, cf history that they have seen a pharmacolgical agent change the base defect of the mutated gene that causes CF.  That is incredible!  My husband and I read, and reread all of the information that we could find on this new drug .  We have been following it closely for at least the last two years.  While this specific drug, VX-770 does little to nothing for Rachel’s mutation.  It does bring extreme hope!

This particular drug only works in about 4% of the CF community.  The most common mutation is the DDf508 (which Rachel has, one copy of Df508 from me and one from my husband, making her a double delta). While that sounds like it benefits so few people, the great news is that they but there is a second drug that is in the works to go along with this drug that WILL TREAT HER CF.  Now she will always have CF, but these drugs will have the ability to slow down the progressiveness of this disease.  The average CF person looses somewhere between 2-5% of their lung function each year (as told to me by our respiratory therapist at our last clinic).  When we take our pulmonary lung function test (PFT’s) they are constantly checking to see what these numbers are.  Rachel’s has been really low the last year that she has taken them.  She is still little, and from what we hear from other patience, the test has a pretty good learning curve.  However, with that being said, the Dr.’s are still uncertain why hers are SO LOW.  Our next appointment they are going to test her a different, special way to see if they can get tot the bottom of why these numbers are so low.  They have told me that they should be an inflated number because of her age (somewhere over a 100%-125%)) while her numbers have yet to even reach 80%.  And last year when she was sick so much she consistently blew numbers in the 50%.  Those low numbers are scary!  SO, all that to say that having a drug that could improve her lung function, or at the very least keep it from going down so quick, is THRILLING!!!

The fact that VX-770 has made it through trials and now is working to get FDA approval is HUGE!!!! The researchers are starting a trial with this first compound vx-770, and a second compound vx-809 this next month for Rachel’s mutation!! This first time in history that they know how to fix the defect and are seeing solid clinical research to support their findings! Now, trials take a long time (maybe 2-5 years), but at some point soon Rachel will be old enough to participate in these trials!!  Praise the Lord!!!

Oh and I almost forgot the most exciting part….these drugs come in pill form!!!  Many of you have watched the wonder of Rachel being able to take BIG PILLS from the time she was just under 18 months old.  However, we still have at least an hour a day of breathing, and vest (shaking the mucus out of lungs) treatments when she is well.  These treatments are a huge time consumer…and hard when you are five to have to stop doing what you love, which is usually playing to come and take care of your lungs.  Rachel pops somewhere in the neighborhood of 50 pills a day.  1 MORE would be such a blessing.  WE told her about this pill sometime ago, and she comments about the hope of taking a pill instead of treatments very often.  Even at her young age, she understands which one would be more freeing.

A fellow CF mama wrote a wonderful entry the other day.  Please check out her blog.  http://bennettgamel.blogspot.com/2011/02/this-is-big.html  Breck has a similar approach to faithfully fighting this disease as we do.  First of all we share the faith that God is in control, and that He lovingly walks with us through the challenges that are created from Cystic Fibrosis.  We also share the quest for knowledge.  For me, if I can understand and comprehend a little of what is going on in the disease… l somehow feel like I can better cope with the difficulties that come with it.

Well, hears to prayerfully hoping that someday in the near future our vacation pictures will look a bit different that the picture posted.=)  God is Hope, and HOPE STANDS STRONG!!

Psalms 119:74
May those who fear you rejoice when they see me, for I have put my hope in your word.


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