Tuesday, November 20, 2012

Almost a Cure

I've had to explain what cystic fibrosis is to a lot of friends, family, and strangers over the years.  Since Brady started taking Kalydeco, I've found myself struggling with my spiel (a great problem to have!).  Let's examine the facts:

1) Brady still has cystic fibrosis.  He still has the genetic mutations he was born with (G551D, DF508). 

2) He still takes pancreatic enzymes, special vitamins, and several other prescription meds as a result of being born with CF.  His pancreas has suffered "permanent" damage that Kalydeco hasn't corrected.

3) According to the gold standard diagnostic test for CF--the sweat test, Brady no longer diagnoses positive for cystic fibrosis (last test was 17 mmol/L, 17!!!  Well within the normal range). 

4) Kalydeco has corrected the basic defect of cystic fibrosis in Brady's body (dysfunctional CFTR has now become functional).  Functional CFTR leads to thinner mucus, improved mucociliary clearance, and restoration of proper pH in the lungs and digestive tract in the body (CFers typically have an overly acidic environment in their lungs and digestive tract, promoting infection and impairing digestion.  Kalydeco restores the body's correct pH, giving the body innate bacterial killing power.).

5) In CT scan, Brady's lungs currently show "No visible damage" and appear to be functioning completely normally.

6) Today, Brady has absolutely no lung or sinus symptoms of CF :)

7) Kalydeco is anticipated to add decades and immeasurable quality to Brady's life.

8) If Brady stops taking Kalydeco, the symptoms of cystic fibrosis will return almost immediately.

So what does that all mean?  It means my explanation of Brady's condition just got a lot more complicated...I have caught myself using the following phrase recently because I don't know what else to say:
 
"It is almost like a cure for him." 
 
I hesitate to include that word at all--cure--because I am really not sure what that means, and because that word holds so much power.  I know that unless you are a part of the CF world, or perhaps have seen a news piece about Kalydeco, most people won't understand why Kalydeco is such a big deal for us.  They won't understand that it has changed everything we do and think about every minute of the day.  I mean, it isn't a real cure, because Brady still has CF and has to take that magic blue pill twice a day or he is back to old-fashioned CF...BUT, as long as he is taking Kalydeco, his body doesn't realize it has CF.  Almost a cure is good enough for me. 
 
With all this in mind, my husband, Brady's Dr., and I have some decisions to make about how to move forward with treatment.  Brady is currently still doing once daily Pulmozyme and 15 minutes twice daily on the Vest.  We have been very slow and deliberate in removing treatments from his regimen because his safety is ALWAYS our #1 priority.  We don't want to remove any treatment that could potentially still be beneficial to him. Theoretically, and from everything we have clinically observed in Brady--he shouldn't really need ANY lung treatments or lung clearance anymore.  Not even the experts at the NACFC seemed to have a real answer for us on the best path forward with treatment...the ball is in our court.  
 
The Scenario:
 
We are planning a big family trip to Spain over the holidays and I have had a goal in my mind of being able to make that trip without Brady's Vest machine. We have been training Brady on the Acapella device as a more travel friendly option for airway clearance, and would still be able to do manual chest PT in the event that he caught a bad virus and was in need of a good beating ;)  We are taking a portable nebulizer and will be continuing his Pulmozyme treatments as usual.  I am anxious to discuss this again with Brady's Dr. at his next appt. (end of Nov.), and see what he thinks!  Feel free to comment your two cents on the topic!  Take the Vest...or not?
 
The full-length Plenary Sessions from this year's NACFC are now available online and I encourage everyone to watch!   https://wiww.nacfconference.org/plen.archive.html
 
 



12 comments:

  1. Great post! I think you are ready to go vest free for the trip! I know it's a hard decision. If you don't feel totally comfortable without a device, maybe you could consider taking advantage of the Med Systems Percussor 5000 free 30 day trial. Insurance would not pay for us, but we enjoyed the device during the trial period. Have a great trip!

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  2. Hi! my name is Iñigo, and I have two kids with CF, we found out a year and a half ago and this blog has been very very helpful for us to understand what we are facing and how are the medication development going on. I just readed that you and your family plan to visit Spain, thats where we are from. If you need any information or any help for your trip (about anything, nice places to visit or enzymes because somebody lost your medication´s bag, anything), please let me know, email me for anything you may need. It is the least I can do to pay you back all the help and hope that you have given to us.

    Regards.

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    Replies
    1. Thank you so much Inigo! We are really looking forward to our trip and it gives me comfort to know that I have someone to call on if we need help! So glad you have found the blog informative and helpful! That makes it all worth it for me! Best to you and your family!

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    2. Wow, I almost cant believe that the lucky cf mom is thanking me for something!! now seriously, whenever you want send me an email and Ill give you my phone number, just in case once you are in Spain you need local advice.

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  3. By the way, almost forgot, my email is inigo.moja@gmail.com

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  4. Rebecca, I just came across your blog. It's fantastic! My daughter is a mirror of your son. Her name is Lu - 9 year old. She is also on Kalydeco and her pancreas is spent. I would love to bend your ear on how you are handling GI doctors. We move a lot and I struggle with getting any help in focusing on her digestive health. If you get a chance please drop me a note.

    Thanks,

    Lora Godfrey
    lora.godfrey@sbcglobal.net

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  5. Rebecca, I really appreciate your blog and I am so happy to hear you son is doing well. I am 25 years old my boyfriend is 28 years old with cf- he has a heterozygous mutation with: delta f508 and W1282x - I have a limited support system because it is hard for my friends to relate to what having a loved one with CF is really like and I don't want to become a burden to anyone. Since once of his mutations is a nonsense mutation I know that he could potentially benefit from atalueren and maybe vx809 and kayldeco . I am hoping that the combo of kayldeco and vx809 starts recruiting for phase 3. Or that ataluren gets approved soon. It feels like the clock is ticking; and sometimes I dont know if I am strong enough to get through this. My boyfriend has been lucky thus far; but I know his time is limited and that anything could happen at any point. Please Please let me know if you have any suggestions- I have educated myself as much as I possibly can- I just think it would really help to talk to someone who I maybe able to learn something from. May you have a happy and healthy holiday season.

    Thanks so much,
    Sara

    weins106@mail.chapman.edu

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  6. My Mother is 71 and has COPD, also lung cancer survivor with tissue damage. She contracted Psuedomonus in the hospital in August. For 4 months and 5 or six different antibiotics, some pills, Cipro intravenously, in and out of hospitals , back and forth to doctors. They could not even touch it. I thought for sure she is going to die. Heavy wet cough, horrible crackling sound when she breathes. Full blown phenmonia, bacterial, Psuedomonus. Just when I was at my wits end, I was watching
    Know the Cause, which is a medical program primarily dealing with fungus. Main focus is that doctors misdiagnose many diseases and illnesses and automatically give antibiotics, which are fungus themselves. Anyway, long story short, they said Psuedomonus and caught my full attention. Turns out
    Olive Leaf Extract is a natural killer of bacteria , molds and fungus. They specifically said psuedomonus. So I went to the one and only store here that carries the Seagate brand of Olive Leaf Extract. Took it to my mom and told her to take 3 capsules 3 times a day for a few days and lets see if it works. Why not. They said it would. To my surprise she was feeling much better within about 12 hours. I had no idea it would work that fast. Same with my sister, she has a different bacterial lung infection as well, and she started feeling better within 2 hours. This stuff is unbelievable. It is a gift from God. Mom is much , much better. She is able to get around now and we even went shopping after her doctors visit on Wednesday. She was active for 9 hours straight and hasn't even come close to that for 5 months. It is truely a miracle. The doctor said her lungs sounded good. I asked him to do culture on sputem and the results came back positive for the bacteria. However, she is feeling so much better that I believe if she keeps on the OLE for another couple weeks or maybe a month, however long it takes. I really think it will be eradicated once and for all.
    Even if it doesn't go away completely, at least she can enjoy her life a little now. It has made a world of difference. Also it will protect her from getting any other colds or infections as it is a bacteria killer, as well as fungus and molds. Do your own research please. OLE is very beneficial. It saved my Mom when everything else failed.Seagate is the best brand because it is cold pressed and not added ingredients. No China ingredients either. That is the only one I can vouch for. We ran out and had to get GNC while we are waiting on my order. It does not seem to be near as good. The program everyone should watch is called "Know the Cause" 30 minute program comes on every day several times a day on different channels. you will have to look up. Good Luck everyone. Antibiotics are Killers. They are fungus. Even if they did work on what you have, they cause another problem. Peace

    ReplyDelete
  7. My Mother is 71 and has COPD, also lung cancer survivor with tissue damage. She contracted Psuedomonus in the hospital in August. For 4 months and 5 or six different antibiotics, some pills, Cipro intravenously, in and out of hospitals , back and forth to doctors. They could not even touch it. I thought for sure she is going to die. Heavy wet cough, horrible crackling sound when she breathes. Full blown phenmonia, bacterial, Psuedomonus. Just when I was at my wits end, I was watching
    Know the Cause, which is a medical program primarily dealing with fungus. Main focus is that doctors misdiagnose many diseases and illnesses and automatically give antibiotics, which are fungus themselves. Anyway, long story short, they said Psuedomonus and caught my full attention. Turns out
    Olive Leaf Extract is a natural killer of bacteria , molds and fungus. They specifically said psuedomonus. So I went to the one and only store here that carries the Seagate brand of Olive Leaf Extract. Took it to my mom and told her to take 3 capsules 3 times a day for a few days and lets see if it works. Why not. They said it would. To my surprise she was feeling much better within about 12 hours. I had no idea it would work that fast. Same with my sister, she has a different bacterial lung infection as well, and she started feeling better within 2 hours. This stuff is unbelievable. It is a gift from God. Mom is much , much better. She is able to get around now and we even went shopping after her doctors visit on Wednesday. She was active for 9 hours straight and hasn't even come close to that for 5 months. It is truely a miracle. The doctor said her lungs sounded good. I asked him to do culture on sputem and the results came back positive for the bacteria. However, she is feeling so much better that I believe if she keeps on the OLE for another couple weeks or maybe a month, however long it takes. I really think it will be eradicated once and for all.
    Even if it doesn't go away completely, at least she can enjoy her life a little now. It has made a world of difference. Also it will protect her from getting any other colds or infections as it is a bacteria killer, as well as fungus and molds. Do your own research please. OLE is very beneficial. It saved my Mom when everything else failed.Seagate is the best brand because it is cold pressed and not added ingredients. No China ingredients either. That is the only one I can vouch for. We ran out and had to get GNC while we are waiting on my order. It does not seem to be near as good. The program everyone should watch is called "Know the Cause" 30 minute program comes on every day several times a day on different channels. you will have to look up. Good Luck everyone. Antibiotics are Killers. They are fungus. Even if they did work on what you have, they cause another problem. Peace

    ReplyDelete
  8. I've been friends with a family for a few months and their two wonderful boys have CF. They and I are so hopeful for the kalydeco combo to come out. I pray it works! I've been following your blog with joy. An update would be wonderful! Thank you!

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    Replies
    1. Thanks for reading the blog Hazel! I am traveling with my family in Spain right now, but am going to try hard to write an update very soon! I am so optimistic about the Kalydeco combo also--I know it can't come soon enough!

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    2. Awesome! Hope you're having a great trip, and I will look forward to the update!

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