Sunday, October 12, 2014

A Year has Passed

Dear Evan,

One year ago today, we received the phone call that changed our lives forever.  It was the Wednesday after the October General Conference 2013.  The day that I was told my happy, carefree, perfect five week old baby boy had Cystic Fibrosis.  Cystic Fibrosis?  We had so many questions.  Dr Larson and the team at Primary’s were so great to patiently and calmly guide us through the all the information and arm us with the medications and training necessary to begin your treatment immediately.  Then came the dreaded question, “What does this mean long term for Evan?”  And the dreaded response that I’ll never forget.  “There have been many advances in the last several years, but this disease will most likely shorten your son’s life.”   My world crumbled.

How could I ever see beyond the dark blur that consumed my every action?  How could I function normally knowing that even with recent medical advances there will most likely come a day when I will have to burry my son?  I cried every day for months until my face was swollen and my soul hurt.  But, through the tears you were there smiling, growing, living. 

You.  You, my son, are what saved me. 

For the past year, your care has been our primary concern.  To gain weight, capsule after capsule of pancreatic enzymes have been cracked open onto the end of a baby spoon with applesauce and fed to you before every meal or snack.  We have watched as your weight as steadily decreased in percentage from the healthy 7 lb 15 oz baby you were born as.  We have shoved every kind of high fat and high calorie food at you we could think of just to have you gain any extra ounce possible.  We have been medically diligent, never missing a day of inhalation treatments and chest percussion therapy.  We have cried as you have cried, your tears begging us to stop.  But, together we push through the tears, ever determined to keep your lungs open to give you the precious breath that you need to survive. 

Although I know this disease will slowly take over your body more and more as you get older, I can promise you this.  No matter how bad the pain, how long the day, or what the statistics say, we will never give up.  You have been blessed with wisdom, strength, courage and determination already far beyond your years.  Your strength as a one-year old has given me strength.  Your love for life has given me a purpose beyond that of my own.  One year later and you are still lighting up the room with your gentle nature and loving spirit. 

There is still no cure, but for the first time there are new drugs that have begun treating the underlying cause of CF.  So we hope.  We wait.  We will not give up until you can breathe without pain.  I will be here right beside you fighting every breath with you. 

Today, we celebrate you.  We celebrate the life you have lived and the life you have yet to live. 

Love you,

Monday, June 30, 2014


 Dear Evan,

We pray, we plead every day for a cure.  We aren't there yet, but today we are one step closer.

Evan 9 Months
Today, we received an email that VX809 has been successful in the clinical studies and is now awaiting FDA approval. During our very first appointment with Dr Asfour, we asked if there were any new medications or advances in the pipeline for your mutation.  He mentioned VX809 and that if everything proved successful, you would be right on target to begin treatment with it at the age of two.  For that moment and again today after reading the email, all I could do was cry because my heart felt different. I felt different. I felt hope.

Celebrating with bubbles

Hope is what drives me to be perfect in every way possible when it comes to your care.  Some days it's overwhelming between the breathing treatments, CPT, Creon, blessed Creon, keeping the bills straight, appointments made, prescriptions ordered, weekly calls to insurance, the trips up to Primary's, trying to prevent your crawling, teething self from putting every disgusting thing you can find from the floor in your mouth, sanitizing the house, getting you to eat anything, nursing, wondering if I'll have to nurse forever because you refuse to do anything else, oh and somewhere in there having fun and creating memories.

Swimming at Nana and Bampa's June 2014
But, I know that you were meant to come to our family and this earth at this time for a reason.  The medical advances made towards finding a cure and improving lung function are historical.  What seemed like a distant glimmer of hope for a drug that literally improves lung function, something that has never been achieved before, is becoming a reality.  I hope there will come a day when we can look back and say remember when you had to do all of those things!  I believe we will get to experience that day.  Hope. It is what drives me.

A typical look into mealtime
To look too far into the future is too overwhelming and scary and frankly impossible.  Instead, we focus on small, attainable goals.  Right now we are working on getting you to eat three meals of solid food a day.  You refuse to be spoon fed anything. You also refuse to take a bottle or sippy cup.  Only nursing and feeding yourself will do.  Ideally, we would be giving you calorie-filled baby food, and power packing your formula, but we don't live in an ideal world and you are far to stubborn for that.

Let the battle begin
Ultimately, I hope that through your journey and life, you will know that your dad and I did the very best that we could to provide you with the best medications, treatments, home and life we could.  There isn't anything we wouldn't do for you.  Before you and your brother, I always felt like there was some great mission or something bigger I was supposed to be doing with my life.  Now I know it was you.  You are my purpose, you are my mission.  I no longer yearn for some grand worldly status.  Instead, I hope for more tomorrows.  I hope for more memories.  I hope for a long and happy life for you.  I hope.  I love you son.


Sunday, June 29, 2014

VX809 Breakthrough

Letter to the Community on Exciting Vertex Clinical Trials Results
June 24, 2014


This morning, Vertex Pharmaceuticals announced the results from the Phase 3 clinical trials of ivacaftor (Kalydeco™) and lumacaftor (VX-809) in people with two copies of the F508del mutation.
The studies showed that, compared with those on placebo, participants who took the combination treatment showed significant and consistent improvement in lung function and in other important health measures, including weight gain, and a reduction in the rate of pulmonary exacerbations.
These positive results represent an important milestone in the history of CF — and a significant step forward in our effort to bring new treatments targeting the underlying cause of the disease to all people with CF.

Based on these results, Vertex plans to submit a New Drug Application to the U.S. Food and Drug Administration (FDA) by the end of this year. While we cannot predict the outcome of the FDA’s review, we believe the Phase 3 results present a compelling case for the approval of this much-needed potential new treatment.

We congratulate an extraordinary community of people who contributed to the discovery and development of this combination treatment and helped bring us to this incredible day: the scientists who laid the foundation for our understanding of the genetic defect in CF, the researchers at Vertex and the clinical research teams who helped move these critical studies forward in record time and, especially, the more than 1,100 people with CF around the world who took part in the trials.  
Equally important, we want to thank the tens of thousands of families, volunteers, donors, friends and staff who contributed their time, talent and dollars to help support the early discovery and development of this potential treatment.

The combination therapy ultimately aims to treat those with two copies of the F508del mutation or nearly 50 percent of the CF population. If it is approved, and with Kalydeco already available as a single therapy to a small segment of the CF population, the CF community could have effective drugs aimed at the root cause of CF for more than half of those living with the disease.

Many of you have asked whether the drug combination might be made available before its potential FDA approval to people who are seriously ill, including those on lung transplant lists. We are pleased that Vertex is exploring options for an expanded access (sometimes called compassionate use) program for those with two copies of F508del who could safely benefit from the combination therapy during the FDA review period.

These results further validate that we are on the right track to finding new treatments targeting the root cause of the disease. Even as we celebrate today’s announcement, we are determined to press ahead speedily in our pursuit of new therapies for all people with CF, including those with rare mutations, and we are making steady progress on a number of fronts.

We are encouraged that Vertex plans to conduct studies testing ivacaftor with another potential compound, called VX-661, in people with one copy of the F508del mutation.

We are also actively funding Vertex and other leading pharmaceutical and biotech companies — including Pfizer and Genzyme — to discover the next generation of CF therapies that can provide even more robust benefits to the health and quality of life for those with the disease. We look forward to sharing more about the progress of these and other research initiatives.

We hope in the meantime you will take a moment to reflect on today’s exciting news and the important milestone it represents in our shared work to end cystic fibrosis. We know that with your dedication, passion and support, we will continue to find new ways to help advance new treatments for all people with the disease.

Robert J. Beall, Ph.D.Preston W. Campbell, III, M.D.
President and CEO Executive Vice President for Medical Affairs

Evan Primary's 8 Month

MAY 5TH, 2014

Weight 18 lbs 12 oz
Height 26.5 in
Creon: 17.5 (6000 mg) pills a day

Growth Status
Green: Low or No Risk
Weight:  Gained 8.6 ounces (expected weight gain is 10-15 ounces per month)
Height: Grew 2.17 inches (expected height gain is 1.5-5.6 inches)

Medications per day
Salt               1/2 tsp
Pulmozyme  1 Vial nebulized daily
Creon            14 (6000 mg) capsules a day
Albuterol        2 Puffs morning and night
AquDEKs      1 mL 
Synagis         1 mL once a day every 30 days


  • Evan should have gained at least 10 ounces between this visit and our last.  He gained 8.6.  For Evan, every ounce counts.  We discussed ways to continue to increase Evan's caloric intake including new recipes for toddler muffins, different ways to add fats to his food, and healthy fats.  
  • Promising results so far for VX809.  VX809 coupled with kalydeco is showing improvements in lung function so far in people with Evan's mutation.
  • Learned that it doesn't matter the order of the CPT as long as hit all the spots. 

Sunday, April 27, 2014

2014 Great Strides Walk

2014 Great Strides Walk

Hope is the only thing stronger than fear.  
We hope and pray everyday for a cure for this little guy.  
Until that day comes, we will fight to keep him as healthy and strong as we can.  
His strength makes us stronger. 
 His courage makes us face everyday head on.  
His smile makes all of our troubles go away.  
Our future, our hopes, our dreams,  our everything is wrapped up into this one tiny being. 
We will beat Cystic Fibrosis!
People count life time by days, I count mine by every breath.

Introducing Evan's Extreme Team!

We had such a fun time raising awareness and walking for the Cystic Fibrosis Foundation!  Thank you to all of our family and friends near and far for your support.  We love you! 

 Mama and Evan
 Uncle Jay and Aunt Heather
 Grandma & Grandpa Allen
 Evan fell asleep on Grandpa during the walk
 Big brother Jaxson with his raffle tickets
 The Allens
 We love our boys!

Wednesday, April 9, 2014

6 Months ago today

Dear Evan,

We did it!  We made it to 6 months!  The first milestone of many.

It's been 6 months of "heartbeats", therapy, breaking open capsule after capsule of Creon and trying to fit every last bead on the end of of a baby spoon.  It's been 6 months of applesauce, salt, daily sanitation cleaning, doctors galore, shots galore.  It's been 6 months of limited church and public activities.  It's been 6 months of monthly trips to Primary's and holding our breath every time you sneeze, cough, or have even the slightest runny nose.

But, it's been six months of snuggles, giggles, smiles, milestones and love.  And I wouldn't give up one day of any of the hard stuff for one less day with you.  You have been the biggest blessing to our family.  You have given us a purpose beyond our own.

I have learned more about love, sacrifice, charity, and eternity in these past 6 months, then I could have every learned in a lifetime and for that I am  eternally grateful.

The day you were diagnosed with Cystic Fibrosis was the hardest day of my life.  I cried for months, not for the task at hand, but for the burden that will be yours for life.  But, I will be there with you every step of the way.  Cystic Fibrosis does not define you - you define it.  We will provide you with a home where you feel safe and loved.  We will laugh daily.  We will cherish the good days.  We will build memories.

I don't have all the answers.  There will be difficult and sad moments.  But, I can promise you this - no matter how difficult the task, we will do EVERYTHING we can to keep you healthy for as long as we can.  We will fight everyday until they find a cure.  I love you son!


6 Months Later...Reflections

Six months ago today we received news that changed our family forever. It was a Wednesday afternoon, I was at work. A week prior we had received word that Evan's newborn screening test (which all babies born since 2011 must undergo) came back with Cystic Fibrosis 'indeterminate'. Our doctor told us this was nothing to worry about. 90% or more of 'indeterminate' results come back negative once more in-depth testing genetic testing is done.

We weren't worried.

At 3:30 in the afternoon on Wednesday October 9th my phone started ringing. I picked it up and heard Stacy, frantic, on the other end of the phone. I stepped out of my office and asked her to slow down. She said the doctor's office had just called. Evan's doctor wanted to meet with both of us at 6:00 that night. It was about Evan's test results. We both needed to be there.

I rushed home. We tried to think of a possible way it could be good news.  Maybe the tests had shown he had some crazy athletic gene or genius gene. Maybe he would be 7 feet tall and an NBA star. We were grasping at straws.

We got in the car and went on a drive. There was no way I could go back to work, and sitting at home wasn't an option. It was a rainy afternoon in the desert. We drove clear to Pine Valley (45 minutes from St. George). On that drive we promised each other that we would stick together and that, regardless of the trials ahead, we would take care of Evan.

We left Jaxson with some friends in our ward (thanks Bennions and Woolleys) and then went to the Doctor's office. They took us into an exam room.  I noticed that the nurses wouldn't look us in the eye as they took us back. Eventually the doctor came in and said, "We do a variety of tests to look for Cystic Fibrosis, and this time it appears we found it." Genetic tests done in Salt Lake had confirmed Evan had CF.

A wave of shock rushed over us both. And then the questions started pouring out. How long would he live? What would his quality of life be? What the heck is Cystic Fibrosis? What does this mean for our family? Would he have a normal childhood? Could he play sports? Could he serve an LDS mission? Could he have children of his own?

The doctor did his best answering our questions. He then said we needed to be at Primary Childrens' in Salt Lake at 11:00 the next morning. They could, he said, answer all of our questions.

We staggered out to the parking lot.

Once we got into the car, we both lost it. We sobbed for a solid 15 minutes and just held each other. We went back to our neighborhood, picked up Jaxson and went to our house.

Then we called both of our parents. Those were tough phone calls.

I didn't sleep that night. I spent most of the night online researching Cystic Fibrosis and the rest of the night tossing and turning and praying. We left for Salt Lake early. Along the way we met my parents who took Jaxson and followed us to Salt Lake. Seeing them was very emotional, but we were grateful Jaxson would be with them so we could focus on Evan.

The next two days were a blur. We spent a total of 15 or 16 hours at Primary Children's over the next two
days. We met Evan's CF team--a pediatric pulmonologist, 2 CF nurses, a respiratory therapist, a nutritionist, a social worker--and other support staff. One of the hardest things about that trip was when Jaxson had to be tested for CF. Because the disease is genetic, there was a 25% chance Jaxson had it too. The test is not fun. They put Jaxson in a chair, cover his arms in pads, and then basically shock his arms until he sweats a lot. Based on the consistency of the sweat they can tell if he has CF or not. The sweat collects in the pads so they can analyze it. It is a painful test and was hard for a 2 year old boy to understand. We told him that they were testing to see if he was a superhero or not. They wanted to see if he was Spiderman so they had to test his arms to see if they could shoot out spiderwebs. He was a trooper during the 30 minute test. It was tough to watch him be scared and he was, at times, in pain. We were relieved when the test came back negative. Having Jaxson test positive as well would have been crushing.

So...since then we've gotten used to our new realities: 98 pills (and growing) each week, 90 minutes of lung therapy each day, nebulized medications every day, germophobia, monthly visits to Salt Lake, and the realization that there is a higher statistical likelihood that Evan will die before he's 18 then there is that he will have normal lung function when he's 18.  And that a common cold or flu for you or I could, simply, kill him. The most difficult reality is that there is no 'we'll get through this tough trial' mentality. There is nothing to 'get through.' He won't get better. He will only deteriorate. Our goal, simply, is to slow that deterioration.

But we are also encouraged by the medical progress being made. Average lifespan is now around 40 years old--it used to be under 5 years. New drugs are in the pipeline to be released in Q3 2014 that have the potential to make that lifespan substantially longer. We hope and pray every day for a cure.

The nurses at Primary Children's were so kind. They cried with us, they hugged us, and gave us support. Our ward and our friends were incredible. They have been extraordinary. Thank you. My company has been awesome too. They've been very understanding and supportive. Our families, though,  have been our rock. My parents have been totally willing to take Jaxson overnight while we take Evan to see his doctors. We simply couldn't have done all of these appointments without them. Stacy's mom flew down immediately after we found out. Those were a tough few days and we couldn't have made it through without their help.

So, anyway, 6 months later we've made some changes. We realize that worrying about the future isn't the most important thing. The most important thing is having a high quality of life right now (that's the worst financial advice ever, I know). We simply don't know how long 'the future' will be for our family so we're trying to enjoy every day with our boys.

We are 6 months into our new reality and we're doing well. Thanks for your support and love.