Tuesday, June 19, 2012

Eye of the Storm

I can still feel CF swirling around me--like a devastating tornado.  I turn on my computer every morning and survey the damage.  Almost everyone in my CF circle does NOT have Kalydeco or any type of "miracle drug."  They are dealing with shitty old fashioned CF, that is a relentless and degenerative disease.  The more time that passes, the more perspective I'm able to gather on what is happening for Brady and our whole family right now.  Brady saw his CF specialist last Thursday.  It has been two months since our last check-up and 4 months total since he started Kalydeco.  I want to continue summarizing his progress.  You can check out his April visit here: http://luckycfmom.blogspot.com/2012/04/new-brand-of-cystic-fibrosis.html

Cough: None

Weight: 48 lbs. Same as last visit.  I wish he had gained at least a little weight, but I feel pretty good about his growth in general.  Brady has a tendency to grow either up our out...but not both at once.  Plus, we live in Northern Idaho, and the weather has been bad for a long time.  We are finally getting some sunny days and Brady's activity level has gone WAY up.  Sometimes I swear that those Kalydeco tabs are laced with a tiny bit of crack cocaine because there has been such a noticable change in his energy level.  We also did some experimenting with his enzyme dosing, which is further explained below.  

Height: 44 1/2 inches.  He grew an inch and a half in the last two months!

Pancreatic Function: At the April clinic visit, we had decided to play around with his enzyme dosage to see if we were giving him the appropriate amount.  After a small reduction, Brady began to lose a bit of weight, so we decided to keep him at his original enzyme dosage (3 Zenpep with meals, 2 with snacks).  He hasn't been complaining of any stomach aches and his poo has been very "normal."  It doesn't appear that Kalydeco is going to do much for Brady in terms of reducing his enzyme needs.  His pancreatic insufficiency has always been severe.  His pancreas just stopped working one day, when Brady was a little over 2 weeks old.  He may still need enzymes, but I can say without a doubt that Kalydeco has offered him many other digestive benefits. 

Breathing Treatments and Vest: When Brady began Kalydeco on Feb. 10th, his schedule looked like this:

Baseline Morning: Albuterol and Hypersal via nebulizer followed by 20 minutes on the VEST.  Total time spent was about 45 min.
Baseline Evening: Albuterol, Pulmozyme, and Hypersal via nebulizer followed by 20 min on the VEST.  Total time spent was about 50 min.

After 4 months on Kalydeco, his treatment schedule is now:
Morning: Twice a week (Monday and Thurs) we do Albuterol and Hypersal in the morning via neb. We are in the process of weaning Brady completely off Hypersal.  It is incredible to me that we used to do this treatment 14 times every week and now we are down to 2 times a week.  Our plan is to continue twice a week Hypersal for a month, then drop to once a week Hypersal for another month...then DONE!  We will continue to use Hypersal if Brady is sick, but we no longer feel like he needs this for maintance.  Brady continues to use his VEST every morning, but the time has been reduced from 20 to 15 minutes.  So now, there are 5 days every week where we wake up and do NO nebulizer treatments.  15 minutes on the VEST and he is done!  It is barely enough time for me to cook his breakfast!  It feels like we have so much extra time in the morning!
Evening: Another change that happened at the last clinic visit is that we have started using "The Vortex" (a holding chamber with a mask attachment) device with a regular Albuterol inhaler, rather than nebulizing Albuterol.  http://www.pari.com/products/holding_chambers/product/detail/info/smarttouch_pediatric_vortex_masks.html
When Brady wakes up from his afternoon nap, I give him a couple of puffs on the Vortex (just takes a minute).  He eats a snack while he wakes up and then does his Pulmozyme treatment via nebulizer followed by 15 minutes on the VEST.  Total treatment time in the evening is about 22 min. vs. the 50 min. we used to spend each night.

Other Oral Meds: Holding steady.  We haven't dropped any of his other meds. 

Sinuses: Kalydeco seems to have completely resolved Brady's previously serious sinus issues. 
Baseline: Brady had undergone 1 sinus surgery and did a round of oral Prednisone every 6-8 weeks to manage his polyp growth.  He also did steroid rinses for his sinuses 3 times every day using a device called the Nasatouchhttp://www.sinusdynamics.com/
Today: Brady's sinuses are clear and he is breathing freely.  His sense of smell has obviously returned, as he smells everything around him!  "Mom, smell this rock."  He NEVER used to comment on smells.  He hasn't taken any oral steroids in over 4 months now.  We also decreased and then eventually stopped all the steroid rinses also.  Today, we do NOTHING for his sinuses.  He stopped snoring after 3 days on Kalydeco and we haven't heard it since.  Brady's sinus issues had a real negative impact on his quality of life and the side-effects of Prednisone are less than pleasant.  To be completely free from this issue feels like such a miracle.  It is something we used to battle on a daily basis and now the issue is GONE.  We visited Brady's Ear/Nose/Throat Dr. a few weeks ago to document how well he is doing and the Dr. remarked that Brady's sinus tissues actually "look different" than before.  By the end of the visit, he was asking about Vertex stock as an investment opportunity.  I'd say he was pretty impressed! 

Mental Outlook: Before Kalydeco, I felt like I was swirling around in the dangerous winds of that tornado.   You feel so out of control and every once in a while you get flung into the side of a brick wall.  Going to clinic paralyzed me with fear. What new meds are they going to add?  Will I be able to get Brady to actually swallow/inhale/sit still through it?  What will his throat culture show?  Will his lungs be clear?  CF never rests and that means that you can never rest.  You have to fight CF CONSTANTLY.  It is so damn exhausting and wild rides make me sick to my stomach...

Today, we still have treatments to do and Brady still takes lots of pills, but it is eerily calm right here.  I can see the winds raging violently all around me...but it feels like Kalydeco has led us to the eye of the storm.  Life feels oddly light-hearted and non-eventful.  I have an enormous amount of appreciation for the little chunks of time we have been getting back over the last 4 months. I've replaced some of our morning treatment time with back massages (I've been trained in Massage Therapy and Brady has finally gotten wise to it).  In the evenings now, we spent more time playing outside because treatment time is so short.  An evolution of "normal" has taken place in our home. Kalydeco has been such a gift for all of us.  I calculated that Brady has ingested $105,264 (258 pills @$408/pill) worth of Kalydeco so far.  I'm so thankful that our insurance has decided that Brady is "worth it."  I feel like my experiences with CF and Kalydeco have allowed me to place my priorities firmly in the correct order and live my life accordingly.  And while I greatly appreciate the calm that the eye of the storm provides...I think we would all prefer that the storm would just GO AWAY ALEADY! 


  1. Keep fighting Brady! We are all so proud of your progress!

  2. Hi Rebecca, I just came across your blog and my head is spinning. My wife and I had a baby girl just one month ago today, and she was diagnosed with CF (mutations G551D and Delta F508) through the newborn screen. It was devastating news, and we've been on an emotional roller coaster. Seeing your blog, though, definitely puts me at one of the high points on that coaster. I plan to read all your blog posts from the beginning, but is there any chance you might be open to talking to me and my wife at some point as well? We're still just trying to get our minds wrapped around all this, but seeing that another child with the same mutations our daughter has is doing so well with Kalydeco is immensely exciting! Thanks so much -Rob

    1. Rob, please give me an email address so I can send you my number. I have so much positive news and hope to share with you and your wife. I am traveling now, but will be back home Sunday afternoon. Can't wait to talk to you both!

    2. Thanks so much, Rebecca! I'd rather not post my email in a public forum, but I'm the only Rob Samsot on Facebook, if you want to reach me on there. Really looking forward to catching up - thanks again!


  3. HI Rob there is a FB site re Kalydeco. My children have the same mutations as your daughter. My son has been on Kalydeco for 2.5 years (he has been on it during the trial). He is 32. He is doing amazingly well and I can tell you that you no longer have to fear cf because your daughter has the G551D mutation. If you are in the US you should be able to access the drug some time soon (Im not sure of the ages). If you're in another country you may have a bit longer to wait, but it's on it's way so rejoice!

    1. Great to know, Jo! Thanks for alerting me. And that's fantastic news about your son. I really do feel incredibly lucky that Kalydeco seems to be getting such great results, and that it seems our daughter will be able to take advantage of it.

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  5. Hi Rebecca,

    My son is 2 months old and was diagnosed DF508 and G551D through newborn screening. Your blog is so inspirational, and I love to read Brady's story. Many times I've felt that I'm reading my own story. Before our sweat test, I asked our pediatrician to tell me if two mutations were detected (since we live in Illinois and they DNA test if IRT is elevated). My world stopped as he read "Delta F508 and G551D..." At that seemingly tragic moment, I didn't know that we were actually given a gift. I feel so fortunate that Trevor is G551D and will be eligible for Kalydeco, all thanks to the CFF. We found out at clinic that trials will soon begin for kids ages 2-5, how exciting!

    Like Rob, I would really love hear all the awesome POSSIBILITIES and promise of treating the basic defect. Honestly, it's what keeps me going. Can we connect on Facebook (Kelly McGuire Larson) or exchange emails...how can we exchange privately? Please posting about Brady...love to hear about his progress!


    1. Hi Kelly,
      That G551D mutation is like a golden ticket! Brady is so healthy and doing fantastic. Your son will get Kalydeco much younger than he did-- I'm sure it will be FDA approved for 2 year olds by the time he reaches his second birthday. I think I found you on Facebook and sent a friend request. I'm more than happy to chat or answer any questions you might have! I promise to keep the good news coming!

  6. You have done a brilliant job making sure that people understand where you are coming from. And let me tell you, I get it. Please post more updates to cure.yaldoeyecenter.com