OUR WONDER BABY WITH CONGENITAL PULMONARY AIRWAY MALFORMATION

All About Amelia

This is Amelia Violet Lillian Church at 24 weeks

She has her Mom’s nose and her Dad’s forehead and feet.

She goes crazy for Led Zeppelin and 80’s pop.

Her second middle name is after her Great Great Grandmother.

She is a very feisty little girl (just ask the sonographers).

Also…

Amelia has a Congenital Pulmonary Airway Malformation (CPAM or CCAM).

She was diagnosed at 21 weeks 4 days.We went though 3 weeks of scans and

agonizing before we came to a definitive diagnosis.

Read on for more information about our brave sick little warrior.

SO YOU CAN GET A BETTER SENSE OF WHO WE ARE AS A FAMILY

Meet Amelia’s Parents

This is Amelia’s dad, Aaron. He Loves Star Wars

And Mickey MouseAnd David Bowie too...

This is Amelia’s Mom, Jenn. She’s a Librarian

And Pretty Geeky in Her Own Right

The Beach Dinosaur Place

And She Loves Making Aaron Go Places

Team Ravenclaw! Dr Seuss Park

And Pose with Things

Bowie Peabody & Gromit

Here are Amelia’s Sister and Brothers

Aaron and Jenn Just Got Married in January

All We Need Now to Complete Our Family is a Little Geeky Baby….

ALL ABOUT AMELIA’S CONDITION

The Diagnosis

The Initial Diagnosis

Despite being told getting it would be nearly impossible, Jenn found out she was pregnant.

The pregnancy hasn’t been an easy one.At 19 weeks, we went for the scheduled anatomy

scan.During the scan it was determined Amelia’s heart

had been pushed over to the other side.It looked as though the stomach was up in the chest

cavity and pushed the heart over.A congenital diaphragm hernia (CDH) would cause

this occurrence and is what the Doctors think she has.

The Next Scan – 20 weeks 6 days

2 weeks later we had another scan to get a better look at her heart.

The OB at Hartford told us the scan showed either a large mass or a worsened hernia.

A MRI would be needed to get a better look at the lungs and determine if this is a hernia or a lung mass.

No left lung tissue was found, making the situation much bleaker.

Second Opinion

Jenn was not about to “wait and see” any longer.

If Amelia does have a hernia, this is a very serious and potentially fatal diagnosis.

She contacted Boston Children’s Hospital for a second opinion.

They immediately got her in for a full day of tests: ultrasound, MRI, and Fetal Echocardiogram.

At the end of the day, we would meet with a pediatric surgeon and nurse coordinator and have a definitive diagnosis.

Testing in Boston

The Ultrasound- 21wks 4 days

Over an hour was spent assessing Amelia’s entire body.

She is about a week bigger in development.

She has an upturned nose, wide mouth, and wouldn’t stop moving!

It looks more like a mass but a hernia is still a possibility.

The Fetal Echo

A Fetal Echo is another ultrasound in which they just examine the heart and heart functions.

The echo was assessed by a cardio specialist.Amelia refused to cooperate and kept moving

so an accurate in-depth assessment was unable to be performed.

However, her heart looked and functioned pretty great from what the doctors could see.

The MRI

The MRI was expected to be the ultimate determination into what Amelia’s condition was.

It was also used to assess her lung tissue levels.

The Consultation and Diagnosis

The Consultation

We met with Dr. Smithers and the nurse coordinator Donna to discuss the findings.

Amelia doesn’t have a hernia!She has what is known as CPAM or CCAM.It’s a congenital pulmonary airway

malformation.Basically, Amelia’s left lung has a normal

upper node and a multitude of cysts taking over her lower node.

What is CCAM/CPAM?

“A CCAM is a cystic mass which forms in the lung tissue of a fetus. The mass is usually located in one lung, and it does not function as normal lung tissue. The cause of a CCAM is unknown, and it is not related to anything the mother did or did not do during the pregnancy.”*

 ”The CCAM may grow so rapidly so as to become life-threatening before birth.  This usually happens between 18 and 26 weeks gestation.  The large size of the CCAM causes compression of the heart and eventual heart failure. Compression of the lungs can also cause the lungs to be too small for survival.”*

This is What Amelia’s CPAM Looks Like

What We Know

Amelia has a type 2 CPAM, meaning that she has many macrocysts that take up her lower left lung.

The cysts have expanded enough to push her heart over and flatten her diaphragm.

The next 6-8 weeks will involve frequent monitoring of the CPAM because it will keep growing.

The trick is to make sure the baby grows faster than the CPAM.

We will be keeping track of the CVR. That is the Congenital Pulmonary Airway Malformation Volume Ratio.

Anything under a 1 is good for this. 1.6 and over is of serious concern for complications. Amelia is at a 1.3 right now.

What We Have to Look Out For

As the CPAM gets bigger, the cysts fill with more fluid and push the diaphragm down and the heart over.

This will compromise the other lung, as well as the heart.

As it grows, there is the chance that it might compress blood vessels and lead to something called hydrops.

Hydrops is when fluid builds up in the baby and causes the heart to start failing.

Hydrops would make not only Amelia critically ill (making her survival rate much lower) but can also make Jenn ill as well and cause pre-eclampsia.

Amelia’s type of CPAM rules out the ability to go in and drain fluid in utero.

Pre-Eclampsia

Jenn can get what is known as “mirror syndrome” if hydrops occur. This means her body will mimic the fluid buildup going on in Amelia.

The fluid can easily lead to dangerous pre-eclampsia symptoms of high blood pressure, edema (fluid retention) and protein in the urine.

Pre-eclampsia is life-threatening to the mother and requires the baby be delivered immediately.

Jenn is already high risk for pre-eclampsia because of her high blood pressure.

If Amelia is born prematurely, this makes the pressure on her underdeveloped lungs even more life-threatening.

What we are Doing Now

Jenn will have frequent ultrasounds at Hartford and Boston.If the CVR is higher than 1.6, we will be more aggressive.

Jenn would undergo steroid treatment to encourage the CPAM to stop growth and Amelia to outgrow it.

This is similar to the steroid treatment moms of preemies have to promote lung development before birth.

If things continue to progress at this level, Jenn can deliver at Hartford instead of Boston. If it gets worse, Jenn will deliver at Brigham & Women’s in Boston.

It is CRUCIAL Jenn’s stress level and blood pressure stay within normal range in order to not develop pre-eclampsia!

After She is Born

Regardless of what hospital she is born at, Amelia will need to be monitored closely for her breathing at birth.

If she has issues breathing, surgery to remove the CPAM will need to occur after birth. This will be over a month in NICU time.

If she can breathe ok after birth, and there are no other issues with her organs, she can come home much sooner.

If she can breathe normally at birth, Amelia will not need to have the CPAM removed until about 6 months old.

Amelia will need the CPAM removed before her first birthday NO MATTER WHAT!

End of the 2nd Trimester Update

Amelia’s CVR has remained stable at 1.1 for the past 3 scans.

The mass is growing but she is a big baby!We are not out of the woods yet – the next month is

critical in her growth to make sure hydrops still don’t develop.

Jenn can’t do anything to help the situation beyond doing what she can to keep her stress level down and take her blood pressure medication. Her Dr just upped her blood pressure medicine once again.

So we need to make sure to continue enjoying this pregnancy, the holidays, and to have a lot of laughs!

How You Can Help

These trips to Boston and Hartford are very expensive and exhausting. Each trip to Boston costs about $2000 out of pocket.

Our friend Niki set up a Give Forward fundraiser for us. https://www.giveforward.com/fundraiser/hcn6/baby-amelia

If you can’t give, you can still help by getting the word out and forwarding the link to others.

Wish us love and light, pray for us, and keep positive thoughts for us and our little girl.

For Updates on Amelia

Please bookmark our website “Geek Meets Baby” for all updates and information about the pregnancy and Amelia’s upcoming birth.

We post updates and photos regularly.http://baby.rocksolyd.com/