Saturday, May 26, 2012

Kalydeco: First Week In

Guest post by Angie

WARNING: To those of you that are not my fellow cysters and fibros or caretakers of aforementioned cysters and fibros, this post contains discussion about mucus… wait, if that would bother you why would you be reading a blog about CF? Never mind.

I took my first dose of Kalydeco on a Tuesday night.  I took it and went to bed.  It’s hard to know if Kalydeco was the cause or not, but I had a hacky cough that is usually brought on by drainage in my throat all night.  When I got up in the morning my throat felt really dry and scratchy.  The hacky cough continued throughout the day.  The next day (Thursday) during my morning treatment I noticed that the mucus I was coughing out was thinner and lighter in color.  Could this drug already be making a difference or was it just wishful thinking? In the short time I had to research the effects of Kalydeco, I don’t think I took any action to manage my expectations.  I think I was torn between expecting to see a difference right away and thinking that would not be possible it would be more of a marathon than a sprint.  After all, it’s taken 36 years for my lungs to get to their current state, so they aren’t going to improve overnight, right? 

On Saturday I was contemplating how my first few days on Kalydeco had gone.  I realized that I was coughing a lot less than I normally do.  This was a bit concerning because my family and friends have always been able to locate me by the sound of my cough.  If my cough had decreased, how would anyone find me when we were out shopping?  This is a problem I am more than glad to have!! I also noticed I had more energy.  Don’t get me wrong, I still need my weekend naps, but I think that is just because of my love for napping.
It’s funny, but I noticed the most changes during my first week on Kalydeco.  I’ve continued to feel this way, most of the time.  My cough still increases when the weather changes.  Since I live in the Midwest, the weather is known to change anywhere from 20 to 30 degrees in a 24 hour period.  This has always made my chest feel tight and my head feel like it was going to explode.  Since being on Kalydeco, I have noticed my cough increases with these weather changes, but I’m not having sinus headaches.  I have also had more big, old looking globs come out of my sinuses and lungs.  I take that as a good sign that some of the old stuff is breaking up.  The true test for me will be when I have my first PFT since starting Kalydeco.  I have a feeling we will be celebrating once again when that day comes!

Next Update: This truly is a wonder drug: I’ve started running (although I use the term “running” loosely)!

Note from Ronnie: If you missed Angie's first blog about discovering that she did in fact carry the G551D mutation after many years of thinking she didn't, click here (you can also find her bio). She also gives an update about her emotions after/during receiving her first bottle of "the hope diamond" here

Friday, May 25, 2012

Cruisin' in Her Walker

Thursday, May 24, 2012

Thankful Thursday: Picnic Baskets and Baby Blues

It's thankful Thursday time! We all have so much to be thankful for and we love to take this opportunity just to write down each and everything that comes to mind. Please take this time to share with us what you're thankful for as well. If you have a blog expressing your thankfulness, please share the link! Without further ado, here's what we're thankful for:

Mandi's List:

I'm thankful for talking picnic baskets. Mckenna's favorite toy these days is a talking picnic basket. She can't get enough of it, and I happen to kind of like the stupid songs it win

I'm thankful for sore muscles. I have been slacking on my running lately and have gone for a couple runs getting back into the groove the last couple days. Man are my legs SORE...and I love it. It means it's working!

I'm thankful for air conditioning. Summer is here. It's hot out. So coming inside and feeling a blast of cold air is fabulous!

Ronnie's List:

I'm thankful for my beautiful wife. We just celebrated our 2nd wedding anniversary, and although these last couple of years have flown by, I'm so thankful that I was along for the ride. She makes me a better man, husband, Christ follower, friend, son and father. With out her, I'd be the shell of a man.

I'm thankful for Mandi's support. I'm sure I've been thankful for this before, but I just got back from a run, so it's fresh on my mind. Just a reminder - I hate to run and wouldn't ever choose to do it on my own. There are times when all my self-motivation fails me and I need an extra push - My wife is that push. She is so supportive, loving and inspiring. I'm a lucky man.

I'm thankful for the baby blues. Mckenna's eyes look fake in many of the pictures we take. They could stay that way, or they may not, but either way, I'm thankful for them now. Her little bald head, big smile and blue eyes is enough to make my heart explode each and every day.

So, what are you thankful for today?

Wednesday, May 23, 2012

New Enzyme Approval

May 23, 2012 — The US Food and Drug Administration (FDA) has approved pancrealipase delayed-release capsules (Pertzye, Digestive Care Inc) for the treatment of children and adults with exocrine pancreatic insufficiency (EPI) caused by cystic fibrosis (CF) or other conditions.
The unique pancreatic enzyme product contains bicarbonate-buffered enteric-coated microspheres and is protected by several US and international patents. It has been marketed by the company under the trade namePancrecarb MS-16 since 2004.
Representing the sixth such product for patients with CF, Pertzye comes on the heels of 2 other newly FDA-approved similar products (Ultresa, Aptalis Pharma US Inc; and Viokace, Confab Laboratories Inc), as reported by Medscape Medical News.
Other previously FDA-approved pancrealipase pharmaceuticals include Creon (Abbot Labs), Zenpep (Eurand SPA), and Pancreaze (Ortho-McNeil-Janssen Pharmaceuticals Inc).
Small Study Leads to Big Results
FDA approval was based on data from a small, randomized, double-blind study of 21 patients aged 8 to 43 years (mean, 20 years) who were randomly assigned to receive the study drug at individually titrated doses or matching placebo for 6 to 8 days of treatment, followed by crossover to the alternate treatment for an additional 6 to 8 days.
Results showed that the mean difference in coefficient of fat absorption (CFA) was significantly higher for the study drug relative to placebo, as determined by a 72-hour stool collection during both treatments to measure both fat ingestion and excretion (83% vs 46%; 95% confidence interval [CI], 28% - 45%; P < .001).
The coefficient of nitrogen absorption was likewise significantly increased (79% vs 47%), as evaluated using the same stool samples, with the assumption that proteins contain 16% nitrogen.
No differences were found between children and adults with respect to the severity of pancreatic insufficiency or response to the study medication.
"The improvement in mean CFA observed in the controlled study represents a clinically meaningful treatment benefit.... Availability of this unique buffered formulation of pancreatic enzyme is an important addition to the therapeutic options for CF and other patients with EPI," stated Michael W. Konstan, MD, chairman of pediatrics, Rainbow Babies and Children's Hospital and Case Western Reserve University School of Medicine, Cleveland, Ohio, in a company news release.
The most commonly observed adverse events were diarrhea, dyspepsia, and cough (10% vs 4% for those receiving placebo, for each event).
Recommended Dosing
Pancrealipase delayed-release capsules will be available in 2 formulations:
  • 8000 USP units of lipase with 28,750 USP units of protease and 30,250 USP units of amylase; and
  • 16,000 USP units of lipase with 57,500 USP units of protease and 60,500 USP units of amylase.
Children aged from 1 to less than 4 years and weighing 8 kg or more should begin with a dose of 1000 lipase units/kg body weight per meal (maximum, 2500 units or ≤10,000 units/kg/day) or less than 4000 lipase units/g fat ingested daily.
Older children and adults should start with a dose of 500 lipase units/kg body weight (maximum, 2500 units/kg per meal or ≤4000 lipase units/g fat ingested daily).
Because ingestion of larger amounts is linked to a risk for fibrosing colonopathy, the FDA warns patients to contact their clinician immediately if they have unusual or severe abdominal pain, bloating, and trouble passing stool, nausea, vomiting, or diarrhea.
Capsules should be taken whole to avoid irritation of the oral mucosa, but contents may be mixed with applesauce for infants and others having difficulty swallowing.
Treatment is associated with a risk for hyperuricemia, so uric acid level monitoring should be considered in patients with gout or renal impairment. Caution is advised when prescribing pancrealipase delayed-release capsules for patients with a known allergy to proteins of porcine origin.
Go to for more information.

Original article can be found at

Tuesday, May 22, 2012

Two Year Anniversary

To my beautiful wife - Happy anniversary! Two years huh? I know we talked about this earlier, but seriously, where has the time gone? Thank you for being you and for loving me unconditionally. Thank you for carrying the love of our lives in your belly for 9 months. Thank you for taking every single shot that you had to take to get us there. Thank you for having the confidence in me to not only be the man of your dreams, but the man of your reality. I love you so much and I can't wait to add on to this "cotton" day!

I hope the vows that I said to you on our wedding day are backed up by my actions every single hour that we share together...

My vow to you - May 22nd, 2010:

Mandi, I know that you have been dreaming of your wedding day since you were a little girl. And although I can’t claim that I ever dreamed about this day as a little boy, I now know that it was you I was dreaming of when I became a man. I could stand here and tell you all of the reasons that I love you, but we’d miss our honeymoon.

I just want you to know that you’re my inspiration. You have given me a zest for life that I’ve never felt before. Your smile, love, laughter, winks, hugs, kisses, drive, trust, spark, words, glances, encouragement and your support, fuel me each day and give me the energy to be who I am. Before you, I didn’t quite know what my purpose was. I now know that it was to be your best friend and your husband. No matter what lies ahead for us I will see it as a journey; one that can only be completed by the two of us together, with God by our side.

I promise to you today before God, our family and friends to love you unconditionally. I believe that God has been preparing us for this moment and for our future together since the day we were born. Before this world was even created, I believe that God chose you as a perfect compliment to me.

Through the good and bad, I want you to always know that I will be by your side. I promise that you will never again face life alone.

I promise to dance with you in the sunlight and prepare us for the storms. When the storms do come, I promise to hold you and to protect you. I promise to never keep tallies of wrongs, instead give you praise for all that you do which is right. I promise to never let the sun go down on our anger, and to treat each morning as a new day to love you. Our love may be like the ebb and tide of the ocean, but it will always flow.

In times of want, I will do all that I can to make our love rich. And in times of plenty, I will never let our love grow poor. In sickness, I will nurse you back to health. In health, I will do everything I can to watch you fulfill your dreams. In sadness, I will give you a shoulder to cry on. In happiness, I will be there to create memories with you.

When you need someone to encourage you, I want it to be me. When you need a helping hand, I want it to be mine. When you long for someone to smile at, look to me. When you want to tell a story, turn to me. When you feel weak, take my strength.

When my strength isn’t enough, I promise that I will call out to our Lord in Heaven. I promise to lead by example in our relationship with Christ and remain faithful to Him all of our days. I promise to lead us in a life of worship and to always put the Will of the Father before our own.

I will not forsake you or these vows that we have made, but rather strive to show you my love for the rest of our lives. This is my promise to you: From this moment, everything I am and everything I have is yours and for eternity my love for you will remain.

Monday, May 21, 2012

This Kid Can Eat!

Our little love definitely takes after her parents when it comes to eating. She eats everything...and loves it. She loves everything about the process of eating. From the time you sit her in her bumbo and attach the tray, to when you pull out the baby food and spoon, until you start feeding her, she flails her arms and squeals. When you go to give her the first bite, it's like she can't get it into her mouth fast enough - as if we haven't fed her in weeks. She will try anything you put in front of her. She'll eat any fruit or veggie. She'll try licks of any food or sauce we're eating. If it's edible, she'll eat it.

So far she has some some preferences. Her favorites so far are avocado, apples and squash. She also totally dug licks of peppered beef sauce, curry, you name it! We don't give her more than a lick of these things, yet, to avoid tummy upsets, but we have her try pretty much everything.

We make some of her food and use jarred baby food. It just depends on the food. I would make all of her food, but sometimes laziness gets the best of me. The thing I like about making my own food is that I can vary the texture (she likes some chunk in her food) and there's something nice about it being made fresh. However, it is a bit annoying trying to make small portions (I'd prefer not to freeze and thaw) and sometimes I'm just plain LAZY!

Mckenna doesn't only love the food, but the means by which she eats as well. She LOVES spoons. She's constantly trying to "help" us feed her by holding the spoon while we feed her. And then after each meal she likes to "clean the spoon" AKA sitting and sucking on the spoon while mom throws away/cleans the bowls or jars.

So all that said, I'll share a video of our little eater eating avocado...her favorite.

....Ok so as you can see, something was amiss! We thought maybe it was the garlic strainer we resorted to to get it "mashed"(We were struggling - normally it will just mash with a fork). But upon tasting it, we realized that it was SO hard to mash because it was NOT AT ALL RIPE (we thought our veggie drawer froze it a bit). WHOOPS!! Apparently avocado that's not ripe tastes AWFUL!