• 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)
    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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Reality Check

Feeling a bit solum today in regard to the immediate future of CF for Rachel.  (She is well and I am thankful for that.)  For months now it has been easy to be caught up in the excitement and hope of new drugs on the horizon.  The FDA’s release of the first drug in history to treat the underlying cause of CF has absolutely opened the gate to this hope.  It feels like it is right around the corner for Rachel’s mutation.  However a bit of a reality check  yesterday.  Despite being up to my eyeballs in fundraising, I took some time to read up on some of my CF mama’s blogs, research, and other things related to CF.  It is somehow therapeutic to know that we are not in this daily fight alone, and that a cure is fast approaching.  Connecting with others is much needed.  As much as I wish no one else knew the horrors of CF, somehow knowing that others understand the world that we are in is comforting.  We share a connection between the families of CFers that is amazing.  When I read the successes, I rejoice for the families, when there is hardship, it pulls at my heart.  This road of CF is so unpredictable!  When life is good, you cherish it!  When it is hard…you pray to endure it!

One of the things I love most about the community of CFers is the base of knowledge that they hold.  IT IS ASTOUNDING!  Over the last six years, I have learned so much from patients and other parents.  I don’t know if the wisdom is so great because CF is so individualized and dependent on active efforts (or because so many have hours of sitting attached to a machine and the computer is a good escape and source of information).   I continue to watch and read the stories of Kalydeco and they are truly incredible!  Four or five days after people start the “pill” and people are exhibiting remarkable clinical changes.  It is fun to hear the mama’s talk about life being so different.  Some have even compared it to best day of their lives!  Right up there with their wedding and birth of first child.  These stories represent true hope that life will someday be different for Rachel.

In reading yesterday I came across a blog of a mama that explained the complexity of Rachel’s mutation in a way that I had never seen before.  I have talked about why G551D was the first to be fixed because it was the least complex of  the mutation to fix.  However, I didn’t realize the full extent of why Rachel’s (Double Delta F 508 mutation) would so difficult to solve.   (A fellow CF mama who is also a chemistry explained some of this very well in her blog http://luckycfmom.blogspot.com/)

This explanation was very clear.  It was also a reminder of the fight that researchers have ahead.  They are to take something out of the cell that looks, and acts like “trash” and restore it!.  There is only ONE that I know that can take trash and make it into something beautiful.  While that brings a sense of joy to my heart, it also brings a sense of reality.  We STILL have a really long fight ahead of us.  Last month researchers gave us a timeline that by 2016 there would be hope to have a working cure, and by 2020 the hope was that 90% of all CF would have a working therapy.  That news gave me and many others in the CF huge hope.  However, in the back of my mind…I have realized that while we wait, that is more time for CF to attach Rachel’s body and destroy her lungs.

The medication 770/809 studies are proving to be effective, however there is another medication that we have been watching that was going to be a possible back up 661 that looks like it will be far more effective for treating 90% of the CF population.  While that is good news that it will work better, it also slows down the timeline of getting the drug to patients, since it is a step behind the 809. =(  It was confirmed in the last week that Vertex will be proceeding with the 661 studies.  The initial four to eight years forecast is still there, but I am speculating with this new development it will probably more to the later of that time line.  That feels like a REALLY long time from now!!  I am thankful for a finish line…however it still feels like a marathon, and we are only at the training part, the race hasn’t started.  I know it has, but knowing that this disease can change at any moment is tough.

Recently at the CFF conference there was a doctor from Australia that has research that indicates that the current methods of testing lung health and function is unreliable and faulty.  He goes on to describe how CF is a silent killing disease.   Our current testing will often give a symptomless person a clean bill of health based lung cultures and X-rays.  Even without symptoms, damage is occurring.  His research showed that clear, “normal flora” hugely missed some of the greatest organisms present!  While many pulmonoligist have subscribed to that line of thought for years, Dr. Stick has research to back this up.  By age 1  Bronchiectasis (which is defined as irreversible localized dilation/destruction of the airways and is “clearly visible as widening of the airway”)  has occurred in 20% of CF patients. By age 6, RACHEL’S Age, 80% show this progression of disease.  Rachel’s last three years of lung X-ray’s showed that she had several areas of bronchiectasis and air trapping.  Reading this was a clear reminder that we are still in a desperate fight!!  Several years ago when we first heard that she had bronchiectasis, the doctors were somewhat nonchalant about it.  Kinda, yes, this is what we expect from someone with CF.  Progression and damage occurring.  As a mama, it is heartbreaking.   This is a picture of localized bronchiectasis.  We know Rachel has some damage in her lower section of her left lung, (but I don’t know to what extent, we will have another X-ray in July, however after reading information from Dr. Stick, it seems that doing a X-ray is not the best way to assess damage and concerns me that damage has been seen there.  So I would drove to know what is really going on but not sure I want to expose her to a chest CT scan and bronchoalveolar lavage which would be much more invasive, but also much more informative.)

While the finish line may seem close, and her health “Seemingly” has been good through the winter…damage is still happening EVERY. SINGLE. DAY!  Colds and viruses only intensive that damage.  While I appreciate the ease of life without extra treatments and hospitalizations, the reality that CF is a symptomless killer is a beyond unnerving.

Reading blogs and articles like these is not easy.  It is however, very helpful.  It reminds me to pray!  To trust that God is in control!  It encourages me to work hard at maintaing Rachel’s health.  We have an acquaintance that is taking Kalydeco and while she is experiencing dramatic results, she also has so much damage from the years of antibiotic use that she is still struggling in having antibiotics that are effective against bacterias that she has acquired.  She also has lung damage is irreversible.  Hence the great importance of making sure we do all that we can to prevent what we can for Rachel.  We are not promised tomorrow!  These new therapies on the horizon present amazing hope, however we must keep the focus for today and work hard to keep Rachel healthy enough to benefit from them tomorrow.

Sorry this was a bit of a ramble.  Now, it is time to get back to raising money for my baby.  No time to waste in getting the cure to her.  As defined by the researchers, we know how to fix the problem, now it is just a matter of having enough money and time to complete the research.  Thank you all for every dollar that you have given, the time you have invested in telling others our story, the prayers on our behalf.  May God truly bless and encourage your hearts to know that you are impacting a life.  My child’s life!  THANK YOU~

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