January is EA/TEF Awareness Month

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Bottom Left: Baby James in NICU, Bottom Right: Happy 10 Months Old!

Each year, 1 of of every 4,500 babies is both with EA/TEF.  Even after their surgical repair, many of these children battle a long list of chronic issues like acid reflux, weak immune system, compromised trachea, barky cough, aspiration, and others.

 

January is our official awareness month.  It is a chance to spread the word about this unknown condition.  It is also a time to celebrate modern medicine gifting our children a life they could not have lived in another time or place.

 

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What is EA/TEF?

EA/TEF is common terminology in the US.  In the majority of the rest of the world, the terms OA or TOF are used.

Esophageal Atresia is a congenital anomaly, where babies are born with their esophagus ending in a blind pouch, not connected to their stomach.  Tracheoesophageal Fistula is a congenital anomaly where babies are born with a fistula, or an extra tube, extending from their trachea to their stomach.

 

There are several different types of EA/TEF.  

  • Type A (7.7% of cases): Esophageal atresia in which both segments of the esophagus end in blind pouches. Neither segment is attached to the trachea.
  • Type B (0.8%): Esophageal atresia with tracheoesophageal fistula in which the upper segment of the esophagus forms a fistula to the trachea. The lower segment of the esophagus ends in a blind pouch. This condition is very rare.
  • Type C (86.5%): Esophageal atresia with tracheoesophageal fistula, in which the upper segment of the esophagus ends in a blind pouch (EA) and the lower segment of the esophagus is attached to the trachea (TEF).
  • Type D (0.7%): Esophageal atresia with tracheoesophageal fistula, in which both segments of the esophagus are attached to the trachea. This is the rarest form of EA/TEF.
  • Type E (also called Type H) (4.2%): Tracheoesophageal fistula in which there is no esophageal atresia because the esophagus is continuous to the stomach. Fistula is present between the esophagus and the trachea.

Approximately 50% of babies born with EA/TEF are also born with additional congenital anomalies, usually part of the cluster called VACTERL. Source

 

James’ Story

Our son James was born at 39 weeks in February, 2016.  At first he seemed perfectly healthy.  After a few hours we noticed that he was ‘purring.’  We asked for a doctor to examine him.  She attributed the purring to his fast delivery, and said he just needed a good cry.  About 8 hours after his birth, the nurses prepared for shift change.  Our new nurse was uncomfortable with the ‘purring’ baby, and brought in the head respiratory NICU nurse.  The NICU nurse was in our room for approximately 2 minutes before she correctly guessed that James had esophageal atresia.  An x-ray quickly confirmed the diagnosis, and a new pediatrician came in to explain that our new baby would need to be life-flighted to a bigger hospital that night to undergo major surgery.

 

We are so thankful that James’ EA/TEF was discovered quickly.  The treating physicians told us it can often take up to 3 days to discover EA, during which time a baby is not receiving any nutrients, and they often aspirate fluids and develop pneumonia.

 

You can read our full story here.

 

We are now ten months, one major surgery, and seven dilation operations later.  James sounds like a barking seal when he laughs or coughs.  Respiratory infections are still frightening due to his underdeveloped trachea, but he has handled them well.   Feeding an EA/TEF baby is tricky.  But with the help of a speech pathologist, he has even started to eat solid foods.   Overall, he is doing wonderfully.  We feel immensely blessed and grateful for modern medicine.

 

Community Experience

There are several wonderful Facebook groups for families with EA/TEF (here, here, and here are a few).  They are a great way to connect with others going through similar circumstances.

 

January, official EA/TEF awareness month, has been amazing.  Each day, parents post photos of their EA/TEF children, along with a brief update of how they are doing.  It is incredible to see so many thriving!  It is also heartbreaking to see those who have lost precious children to this condition.  

 

Current Research and Advances

Since EA/TEF is so rare, there is a dearth of research on this condition.

This is why it is so exciting that the first worldwide EA/TEF study is being conducted right now.  Dr. Evan Dellon of University of North Carolina School of Medicine, Division of Gastroenterology and Hepatology is conducting a survey examining the characteristics and long-term outcomes of patients with esophageal atresia, with a goal of improving health outcomes for EA patients.  This study is IRB (ethics committee) approved.  As a clinical research professional, and an EA parent, I highly encourage those affected by EA to consider participation in this important study:

https://unc.az1.qualtrics.com/jfe/form/SV_3vM4egSyXYvjZNb

There is also a national registry available to citizens of France:

https://clinicaltrials.gov/ct2/show/NCT02883725?term=esophageal+atresia&rank=1
In the spirit of research and medical advances, Baby James says STOP esophageal atresia 😉img_1273

 

 

 

 

Baby James: 10 Months!

James is 10 months old today!

Life is full-speed and full of laughter these days.

To catch a glimpse of life with James, here is a snapshot of bedtime reading with the boys.  Cape courtesy of Auntie Tracie ;).

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Milestones:

James continues to eat FOOD!  He has added bananas, avocados, sweet potatoes, toast, and butternut squash to his favorite foods list.

We are working with a speech pathologist to make sure the introduction to food is safe with his EA/TEF.

He climbs on everything he possibly can, and crawls around the house at top-speed.

Likes:

Books.  Reading them, eating them, he’s not picky.

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Helping us wrap Christmas presents.  

Eating said wrapping paper.

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Firsts:

First Christmas!

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We had such a fun Christmas with these boys!  The phrase ‘like a kid on Christmas morning,’ took on a whole new meaning.  They had so much fun!

This year my favorite gift is this wonderful little family of ours, and I am especially grateful for how far this little man has come in 10 short months.

Every good and perfect gift is from above, coming down from the Father of Heavenly Lights.  ~James 1:17

 

CIM: What I plan to Carry for 26.2 Miles at California International Marathon

This year held big challenges and changes for our family.  Just 9 months ago, I wondered if anything could ever feel ‘normal’ again.  My favorite version of ‘normal’ involves running.  I am planning to run California International Marathon (CIM) on Sunday, Dec. 4, and I could not be more excited!

What I plan to carry for 26.2 miles at CIM:

Gratitude

After the year our family has had (which pales in comparison to many in this world), I feel beyond grateful to get to pursue a simple joy like running.  Nine months ago, I was sitting in a NICU, still swollen from pregnancy, pumping every couple of hours, and alternating between fear and sadness over the unexpected challenges our beautiful new baby was facing.  I am so grateful that my baby is healthy enough that I can even think about running.  In those early days, I didn’t have the luxury of time or health for even a short walk outside, let alone getting to run my heart out for 26.2 miles.

Some Tough

Marathons usually hurt.  At some point in those 26.2 miles, I will be pretty uncomfortable.  

Luckily, I have spent some time in the Pain Cave this year.  Natural childbirth?  Ouch. Putting your brand-new, beautiful baby on a life-flight?  Painful.  Sending him into an hours-long surgery with unknown outcomes?  Terrifying.  Getting pneumonia that very week?  Come on!  Months and months of not-enough sleep?  My own personal form of torment.

As my dad says, You have to be comfortable with being uncomfortable.

I have come to know discomfort like an old friend, and I will be ok to sit in his company for a few miles.  

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Photo Credit: @oiselle_sally (Sally Bergesen)

So Much Support

I couldn’t pursue this ridiculous form of fun without a whole lot of support.  My biggest support is from Chris and my boys.  They have been nothing but encouraging.  I think Thomas is going to miss “Boys Mornings” where the boys break all the rules and get pancakes while Mom is on her long run.  Support from my mom (Bammie) watching the boys so I could get a long run in while visiting (even if she did make me go at Mountain Lion Breakfast Hour ;)). Support from Amy coming up with this idea, and planning every long run route.  Support from Jen and Lynn on a tough 20 miler.  Support from Stephanie pacing me through some tough workouts, and never doubting that I could do it.  Literal support out on the course from Tracie and Dad and Carol.  

Miles

Long run miles trying my best to hang on to Amy.  Tempo miles that started out struggling for 8s, and settled (sort of) comfortably into 7s.  Intervals that always felt a little too difficult, but somehow managed to get done.  Easy miles along the river trail, or more often, on the treadmill during the kids’ naptime.  I fit in as many miles as my working-mom schedule allowed.  This is the fewest miles I’ve ever trained for a marathon on, but I like to think I made them count.

Folsom to Sacramento… 26.2 Miles… Let’s do this!

This post was inspired by Running Starfish’s similarly-named post a year ago.  Our goals are more than a little different, as she is a complete rock star.  http://www.runningstarfish.com/2015/12/california-internation-marathon-what-im.html

Baby James: 9 Months

Baby James is 9 months old today!

He is the best little baby.  He smiles nearly all the time.  He loves to laugh.  His pediatrician calls it the happy, easy going, younger sibling syndrome, but I think this baby takes it to a whole new level.  Considering the strong willed personalities of the rest of the family, it’s nice that we have one easy-going person around here 😉

Milestones:

He is a baby on the move!  He crawls fast, and gets into everything.  Big Brother has nicknamed him ‘Bumpers’ because he bumps into everything.

He can stand as long as he is holding onto something.  Such a big kid!

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He now has 3 giant teeth, and puts everything in his mouth.  A 4th tooth is coming in, and he is not thrilled about it.

This baby, the one born unable to swallow, is eating.  Real food!  He has starting eating little crackers, rice puffs, yogurt bites, and more.  

Likes:

His family

Every person he has ever met

Eating real food

Exploring

Dislikes:

Being cold

His carseat

Being left behind.  This kid wants to be where the action is!

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One of the big kids

Firsts:

He had his first trip to the snow!  Considering Dislike #1, he really did well.

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First Halloween.   Baby Yoda was adorable, albeit totally confused as to why we were all walking around in the cold.

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First Thanksgiving!  This kid was a champ for the annual family Turkey Trot in the morning, and then thoroughly enjoyed a holiday that was all about eating.  Our family has so much to be thankful for, and this miracle baby is at the top of the list.

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He had his baby dedication at church.  This is the verse we selected: Consider it pure joy, my brothers and sisters, when you face trials of many kinds.  For you know that the testing of your faith develops perseverance.  And let perseverance finish its work in you, so you may be mature and complete, lacking in nothing.  James 1:2-4

That TOF Cough

Navigating our baby’s EA/TEF is a major part of our lives right now. When James was first diagnosed, I couldn’t find many personal accounts of EA/TEF, so I wanted to write our story.  You can find previous parts here.  In much of the rest of the world, the abbreviations used are TOF/OA, since esophagus is spelled oesophagus.

Let’s talk about that TOF cough.

A quick anatomy refresher: your esophagus is the tube that carries food to your stomach. Your trachea is your airway.  The two tubes are right next to each other.  

In utero, James’ esophagus developed as a pouch instead of a tube.  That pouch took much of the space that his trachea should have had to grow.  So his trachea is underdeveloped and “floppy.”

 

This results in a cough that sounds like a barking seal.  Whenever he coughs, laughs, or cries, he makes a loud, barking sound.  It doesn’t hurt him at all, but it definitely alarms people who haven’t heard it before.

For the most part, it just makes people curious.  But I have had little old ladies stop me stores to tell me, your baby is really sick.  Maybe you shouldn’t have him out.  Sometimes I take the time to explain, and sometimes I just nod and smile.  

We have had doctors unfamiliar with his condition immediately (mis)diagnose him with croup.  We have to explain that most kids born with EA/TEF sound like they have croup all the time, and this is his ‘normal’.

At the library or playground, other moms have given us the side-eye when James bark-coughs.  I totally understand.  We all want to keep our kids healthy, and little kids are basically walking petrie dishes.  

While James’ TOF cough is not painful or dangerous, his TOF history actually makes any respiratory illness quite dangerous for him.  Common respiratory illnesses like RSV could land James in the hospital.  So not only am I used to getting the side-eye over my apparently-sick baby, I have to confess that I am always on the lookout for anyone who looks contagious that could infect my baby.

His doctors say that he may outgrow this someday.  In the meantime, we will continue to answer the questions and field the stares.  And continue to be grateful that this is the worst we have to deal with at this stage.  We are so thankful for this happy, mostly-healthy, wonderful baby of ours.

Fellow EA/TEF parents:

How do you handle comments about your kids’ cough?

Has the cough improved over time?

Here is a link to an interesting article from another EA/TEF mom that addresses the cough:

http://differentdream.com/2011/01/you-might-be-a-parent-of-an-eatef-child-if-part-1/

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Baby James: 8 Months Old

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8 Months Old!  Photo Credit: Heidi Thomas

It’s hard to believe that just 8 months ago James rushed into our world!  Those early days feel like a lifetime ago, but now I feel like time is flying.

 

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Just 8 Months ago?  Really?  Photo Credit: Danae Cobb

James is just the best baby.  He nearly always has a smile on his face.  He is so cuddly and sweet.  James is the reason people love babies!

 

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Happy Camper.  Literally Camping 🙂

He loves to laugh, and big brother Thomas is especially good at cracking him up.  Due to James’ TEF, his laugh and cough sound like a barking seal.  It scares other people, but doesn’t bother him in the least.

 

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These two have so much fun

Milestones:

James is a baby on the move!  He is curious about everything, and crawls all over the house looking for new interesting things.  Thomas invented a game that is basically fetch.  He throws something down the hall, and calls “get it baby James!”  James scoots down the hall as fast as he can to grab it.  Then they both laugh and laugh.

 

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Baby on the move

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James has started waving hello and goodbye, which is so cute.

 

He babbles nonstop.  Like everyone in our house, he has a lot to say.  And Chris is pretty positive that James has started saying “dada.”

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Health: 

James is doing really well right now.  His last dilation was 2 months ago.  Actually, everyone else in our house got really sick last week, and James miraculously stayed well.  We know there are likely more health challenges around the corner, but we are thankful for every good, healthy day with this little man.

 

He is 95th percentile for height, and 90th percentile for weight.  Pretty impressive for a baby born unable to eat!

 

James, we could not be more grateful for you.  You are the best baby in the world, and we just love you so much. 

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Happy 8 months baby James!

Feeding an EA/TEF Baby: What we’ve learned so far

Navigating our baby’s EA/TEF is a major part of our lives right now. When James was first diagnosed, I couldn’t find many personal accounts of EA/TEF, so I wanted to write our story.  You can find Parts 1 -7 here.

For the most part, James is such a sweet, happy baby.  He’s had this content personality all along.  Even when he was tiny, he almost never cried.  Unless, that is, it was feeding time.  Eating was very difficult for him for the first 6 months.

With Esophageal Atresia (EA/TEF), he doesn’t have a ‘real’ esophagus.  A normal esophagus has muscles to help move the food down.  Since his esophagus was surgically created from a tracheosophageal fistula (an extra tube out of his trachea), it doesn’t have the same muscles.

 

 

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You know, just giving the baby a bottle at our favorite lakeside cafe

 

Bottles

At first, when James tried to drink bottles, food would pool in his esophagus, unable to move down.  Since the food hadn’t yet reached his stomach, he was hungry.  But he couldn’t drink any more until it moved down.  It just made for a really frustrated baby. So sad!

We did find a couple of tricks that helped feedings go more easily:

Relax: James ate the very best when he was sleepy and relaxed.  So we often fed him right at the beginning or end of naptime, and he would basically sleep through the feeding.

Slow Down: On bottles, we used the slowest flow possible.  He’s 7 months old now, and still using the Size 1.  It takes him awhile to drink a bottle, but it all stays down.

Give it Time: At about 6 months old, James started to take bottles much more easily.  It makes for a much happier baby!  

 

Solids

Once James turned 6 months old, his doctors encouraged us to start solids, or purees.  I was really nervous to start James on solids.  But it has actually gone really well.


Here’s how we do it:

  • He can eat pretty much any food, as long as it is blended.  We do baby oatmeal, fruit and veggie pouches, and pureed veggies.  One tip that might be helpful if you make your own purees: prepare a large batch, and freeze in silicon ice cube trays.  The squares are perfect small serving sizes, and can easily be popped out and thawed as you need them.
  • We only feed him small amounts at a time.  And we feed him slowly.  And offer plenty of water.
  • Popsicles!!  When we notice that something seems stuck, we give him a popsicle.  As it melts, it turns to juice, and helps flush his esophagus.  Plus, let’s be honest, popsicles = pure baby joy!

 

A Note About Strictures

 

To add to the confusion, we were always on the lookout for signs of a stricture.  It is common for the surgical site (anastomosis) to start to close, which is called a stricture.  When that happens, a dilation is required.  A dilation is basically an operation where the surgeon uses a balloon to stretch the baby’s esophagus.

Every EA/TEF baby presents differently.  Some babies just stop eating when they develop a stricture.  Some babies start spitting up more.  Some babies just eat less sometimes, and normally other times.  

The hard thing for us was that even without a stricture, James had a really hard time eating, and often only ate small amounts at a time.  So we never really knew if he was starting to develop a stricture, or if he was just dealing with the reduced esophageal motility normal for these EA/TEF babies.

He has had several strictures and dilations, and I will do a separate post about those.

 

I imagine this post will really only apply to other EA/TEF or TOF families.  Please use the comment section below to share your experience.


What have you done that’s worked well for feeding your babies?  Anything that worked great (or really didn’t)?