You’re safe.

2:30 AM

Little foot steps approach our bedroom door and I hear the tiny voice speak that has interrupted our sleep.

“Mama…will you read me a book?”

Kid.  It’s the middle of the night.

“Iz…I love you, Bug, but it’s the middle of the night.  It’s time for sleep not reading. Go back to bed.”

Pause.

“Mama…will you read me a book?” Her pleas suddenly coming with tears.

“Iz….honey…please….it’s not time for books.  I promise to read to you in the morning.  It’s time to sleep.  Go back to bed!”

Door slams.

Cries commence.

Drama ensues.

Craig woke up for a millisecond but I lay awake wondering why my best girl needed this small bit of reassurance from me at such an hour.

It was the second day I wasn’t home to tuck her in…but that’s not unusual since I head out for errands at night or to the gym after dinner a few nights a week.

Maybe it was the scene at dinner that had her heart troubled.

 

5:30 PM the day before

“UH OH – YA-YA!” Evan gabbed as he dropped food from his high chair onto the floor and looked at me with a bit of mischief and pride in his eye.

I smiled and playfully waved my finger at him, “No, no!  Not nice Evan.  Food is for eating, playing.”

“Uh oh!” another dog treat of psketti riddled the floor from his high chair.

This little guy is something else.  

Iz giggled from across the table and took on the role of big sister. “No! No! Not nice, Evan!!”  Doing her best imitation of a good scolding in this house.  

I looked at her face as she giggled and smiled and shoved food in her mouth from her Hello Kitty plate.  She sat on her knees…not quite big enough to reach the big table sitting normally.  Between bites she’d stop and make her best buddy laugh.  

Evan’s sweet mouth would release the sweetest sounds of laughter as he watched his big sister and best friend make silly faces.

I felt my stomach turn and then I spoke.

“Hey, Iz.  I want to remind you that tomorrow you’ll be going over to your Tatay and Nana’s house.  Tita Candice is coming down to watch you for a few days.  Daddy and I are taking Evan back to the hospital for another operation for his face.  The doctor hopes to fix his smile.”

“AW!  Yay!” She exclaimed.

Tears suddenly filled my eyes. My gaze shared between Iz and Evan as I tried to reassure them that all would be ok.

“We aren’t doing this surgery because we think Evan isn’t perfect just as he is.  We have to do this so it will make it easier for him to speak and to eat.  But, his smile is perfect.  He’s beautiful.  He’s perfect.”

I looked at my little dude.  Psketti all over his face, his hair, in his ear.  Grinning his lopsided grin.  

I found the one spot on his chubby cheek that wasn’t laden with food and rubbed his baby soft skin and said through tears, “Buddy…you’ll be alright.  It’s going to be long and painful.  But we’ll get you back to being you.”

Suddenly…I was sobbing.

I felt tiny hands on my face.  I looked up and there was my best girl now standing by my side.

“Don’t cry, Mama.  Don’t cry.”

 

2:43 AM

I lay awake and my heart didn’t feel right.

I climbed out of bed and walked into Iz’s room.

My best girl was still awake.  Tear stained.  Hugging her Ducky.

“Iz…it’s very late.  I’m not going to read to you but I will lay with you for a few minutes until you feel better.”

I found a spot on her bed, which is a feat in and of itself since she is a closet hoarder…every animal, book, toy, Barbie….has real estate on her twin bed.

I tucked her in, held her tight, and whispered, “I love you, Iz.  Go ‘night ‘night.  I’m here.  Daddy’s here.  Evan’s here.  You’re safe.”

Within minutes her eyes fluttered shut and her breath was deep as she fell asleep.

 

The power of a mother on a child to reassure that all is well.

It’s amazing.

My gut has turned inside out at the thought of tomorrow.

Another surgery.  More painful than the last.

And my reassurance and kisses of “You’re safe…” won’t be able to make the pain go away.

Fourth surgery in 14 months.

You’d think it’d be easier to hand him over…but it just gets harder.

Evan is older…he’s more aware.  He’s been almost as normal as they come these days…eating like a regular toddler, taking steps, babbling, getting into everything and anything he can put his pudgy hands on.

His heart is doing it’s job but there is still so much risk with general anesthesia.

Every fear….every worry has enveloped my heart and mind.

 

As we face surgery day again…I plead once more prayers for my boy.

1 – For a successful surgery.  Pray for the hands of his surgeon that he’ll be able to repair Evan’s lip and palate fully.

2 – For safety during surgery.  Pray that his heart, brain, kidneys, and other organs stay safe while under anesthesia.  Pray against infection and bleeding issues.

3 – For recovery.  Pray that his heart is able to recovery quickly from being under anesthesia.  Pray that pain is controlled.  Pray for his organ’s to “wake up” from being under anesthesia.  Pray against bleeding and infection.

4 – Pray that he stays safe from the moment we step into the hospital until we return home with him.

 

I will update as often as I can.  Please visit our public Facebook page dedicated to Evan’s prayer warriors.

https://www.facebook.com/AMillionHeartsForEvan

Thank you again, friends.

 

Scar tissue.

Evan’s surgeon told us before his open heart surgery in August, that a majority of the time in the beginning will be getting through and cutting through all the scar tissue that built up in his chest from his initial heart surgery months before.

The body has this amazing way of healing itself – almost protecting itself – from any more damage and pain.  It creates a strong barrier – that scar tissue – around the parts that were “damaged” aka cut into.  It’s almost like the body is saying, “Don’t hurt me anymore…if you do…you’ll have to get through the wall of protection I’m going to build up around the damage you’ve done.”

The tissue builds up around that part that was cut or hurt.  It also can invade other parts that neighbor that wound….just doing what it can to heal itself….not paying any mind to the parts around it that weren’t affected.

The difficulty in all of this is that scar tissue also causes damage.  It’s technically a foreign invader in that part of the body.  The extra tissue becomes hard…not like the tissue that originally called that place home.  

Surgeons say that sometimes the trickiest part of the surgery is not necessarily the surgery itself….but getting through all the scar tissue that has built up.  They have to be extremely delicate….precise…yet firm….to get to the root of the problem without causing more damage.

Every time the body is cut or damaged or hurt…that tissue builds up even further…harder…to protect itself.

As we approach the next two weeks…I am dealing with some old wounds that are opening up again.  Evan has an ECHO (short for Echocardiogram – a sonogram of the heart) of his heart next Wednesday.  This will be the first time we will “see” his heart since open heart surgery in August.  Over 6 months…

We’ll see if his heart has adjusted appropriately to what his surgeon did.  We will see if it’s pumping efficiently.  We will see if the arteries and veins going in and out of his heart are growing well.  We will see if the valves are holding up and not leaking.

Essentially….we will see if my boy’s heart is working.

We also have what we hope will be a final repair for his cleft lip and palate on March 14th.  Of course, he will have more surgeries in the future for his cleft palate and some aesthetic revisions but this big kahuna of a repair is looming.  It will be another 6-8 hour surgery.

All those emotions of fear, worry, anxiety have surfaced as the days approach.  All those things I thought resolved and healed are now being open again.  Fresh.

But, instead of the times before where my heart and soul healed…the scar tissue that has built around my being has caused damage to me that makes the wounds deeper…

In its path of healing…I’m realizing I put up and built up barriers to protect things that really didn’t need protecting.

My marriage.  My faith.  My friendships.  My family relationships.  My joy.

All those things that were safe from the path of the pain I’ve experienced in this journey…I’m realizing have been touched.  My way of healing.  My way of protecting.

This journey for my boy and the fears and worries and the anger and the frustration and the doubts….these things that are broken about this path….I thought were healed.  Yet…I see how they are seeping into the pores of everything and everyone around me.

The wounds are deeper that I expected.  More severe than once thought.

I continue to revisit places in my soul that have received healing and cut into them trying to fix them all over once more.  In the process…I’m causing  more harm than good.

Yet, it’s inevitable.

Four major surgeries in 14 months since my boy was 6 weeks old.

Heart surgery in March.

Open heart surgery in August.

Cleft lip, cleft palate, gastric tube, ear tube surgery in November.

Now….cleft lip and palate surgery again in March.

How can I not revisit the fears? The wounds have never had a chance to heal.  I’ve been putting a band aid over what I thought was a little scratch…but it’s actually a deep wound of fear, of doubts, of all those things that lurk in my heart and head…that have never truly healed and resolved.

I’m finding out the hard way that my inability to heal fully has made the issues I haven’t fully dealt with worse.

My faith is being test and I’m not sure I’m passing the test.

My marriage is being tested…and if it wasn’t for the steadfastness of Craig…I’m not sure we’d be where we are today.

My friendships, while not being tested, I feel are going to suffer again…as I start seclusion into myself….not able to be the friend to anyone in the state I’m in.

I thought I healed. I thought I was past this point of feeling and aching and questioning and wondering and doubting and fearing.

But, as we approach another unknown path in this journey…I realize I’m not healed.  I just put bandaids over wounds and allowed the scar tissue over my soul mask the true hurt and the fear.

Layer upon layer of protection…but no healing.

 

 

CHD Awareness month: That’s all folks.

I went to a funeral this weekend.

It was the mother of one of mine and Craig’s close friends.  The church was packed…over 740 people attended.

It was such a testimony to the woman that she was….family, friends, colleagues….all surrounded her to celebrate her life.

I became very emotional during all of it.

Not just in sympathy for my friends….but in fear and anger.

Death is different to me now.  I’m surrounded by it daily.  But, the death I witness is not of people who have lived long lives…have a spouse, children, and grandchildren to leave behind.

The death I’m witness to is one of children.  Babies.  The most innocent leaving this world.

I ached as I listened to our friend talk about watching his mother struggle during her last days.  I listened intently as he talked about the scene that will he witnessed that “he’ll never forget.”

The thought of him losing his mother is heartbreaking….the only comfort I could muster up in this dark time for him was that room full of people.  The long life she lived…the lives she changed….the fullness of her life surrounding her that day.

I had to leave early because my boy needed me.  No one other than Craig and I know how to care for him…so during the waking hours…it’s either Daddy or Mommy duty.

I drove off…and I suddenly felt anger and fear.

Anger because I know there have been many in the Heartland that have had to plan a funeral for their child.  Have had to find the words, the music, the right this’s and that’s to accurately portray the beautiful life their little one had.

And that’s not fair.

Fear because I’m scared that I may outlive my son….a harsh reality of the Heartland.

And that’s not fair.

What does it all mean?  This whole month?  This whole month I’ve been shoving stories on your computer screens about babies with heart defects, women who are trying to change the face of congenital heart disease.  

My hope was to not alienate you.  My hope is to motivate, encourage, stir something up in your heart….to educate others about CHD, to share stories of hope about CHD, to find a cure for CHD.

My anger and fear for the lives of the children dying from CHD have no purpose but to give my heart an outlet to cry out.  It doesn’t change anything.  Doesn’t make anything better.

Hear this…something good must come out of the tragedies in this world.

Whether it’s congenital heart disease, child hood cancer, rare diseases….any disease….something good must result from it.

We can’t allow the fear, the anger, the tears, the helplessness we all feel to be it.

It can’t end at the next medication, the next surgery, the next intervention.  These things are all grand…they give our children time….

But, I don’t want “time.”  I don’t want a few years with Evan.  I want a lifetime with him.

I want to witness from the Heaven’s his celebration of life….where he’s surrounded by his wife, his children, his grandchildren, a church full of lives he touched and changed….from his long, full life.

The Heartland is rallying now to educate hospitals, birthing centers, midwives on the use of pulse oximetry to screen newborns who were not prenatally diagnosed.

We are witnessing how new drugs are helping our little ones hearts beat more efficiently…although we cringe as we give each one.

We are sharing stories of the latest innovations in surgical techniques to hopefully allow our children to use their ‘broken’ hearts for many years before a transplant may be needed.

We are creating our own walks, our own fundraisers to raise money for research…because not enough is being given to our cause.

We. The Heartland.  And now..hopefully after this month…you.

Us.  All of us…trying to give our children the greatest of hopes…

A cure.

It must end at that.

A cure.

That’s all folks.

 

 

CHD awareness month: Let it have purpose.

When I found out about Evan’s heart…I knew that something good had to come out of it.

Yes…we prayed for the surgeries to be successful.  Yes…we prayed for him to have as normal of a life as possible.  Yes…we prayed that his life had meaning and purpose…no matter how long it would be.

I found out about Cora.  Sweet Cora. 

How her mother…a woman who is making it her mission to make sure that her Cora’s life…has purpose.

Kristine is a woman all the CHD community should thank…she is using a tragedy to change and save lives.

Cora’s Story

http://www.corasstory.com

Four years ago, February 7 to 14 wasn’t a special week. I was celebrating my first Valentine’s Day as an engaged woman and had never heard the phrase “congenital heart defect.”

 That November, I gave birth to my first child, a girl we named Cora. I’d had a normal pregnancy, normal delivery and we took her home oh so in love.

Then one early morning, I settled down to feed her. I looked up for a split second, looked back down and my baby wasn’t breathing. She was limp and grey. We rushed her to the emergency room, but it was too late.

Days later, I got a call from the coroner and for the first time in my life I heard the phrase “congenital heart disease.”

I had no idea what it meant. I didn’t know babies were born with hearts that weren’t anatomically normal. I took to the Internet to learn more, and soon was contacted by a mother working around the clock to push for a simple screening, pulse oximetry screening for CCHD. The mom was Annamarie Saarinen with 1in100.

I took up the call. I started yelling, screaming, whispering to anyone that would listen. And people listened. Soon mothers across the country were asking for their babies hearts to be checked with pulse oximetry. I started to get notes that doctors were hearing the mothers too, when the moms said they wanted their babies hearts checked because of Cora, OBs were saying they’d heard of Cora.

I realized that although I could reach hundreds of thousands of mothers through social and traditional media, I could reach them all. So I started in my state. I wrote my state senator, who introduced a bill and that bill was eventually “Cora’s Law.” My home state, Indiana, signed the first piece of CCHD screening into law in May 2011.

Meanwhile, Annamarie kept working too, and all of that work culminated with the addition to CCHD screening to the recommended uniform newborn screening panel nationally in September 2011, but while that’s a huge move forward, it’s still up to states to implement.

Now moms are working around the country on this cause. It’s been awe inspiring to watch new laws passed and the progress so far.

I learned through this experience that if we persistently ask, people will listen. Cora’s name is known around the world. Babies are screened because of her. Sometimes that screening saves their lives.

I couldn’t be a prouder mother.

Please tell every woman you know of childbearing age about CCHD screening.

 

To learn more about Cora, visit http://www.corasstory.com.

To learn more about pulse oximetry screening, visit http://www.pulseoxadvocacy.com.

Share the website http://www.babyheartscreening.com with every pregnant woman you know.

CHD awareness month: Moms on a mission part deux

 

Today’s blog is from a dear Heart Mama that is almost the “go to” gal for many mamas.

She not only has some great medical knowledge just from her experience as a mom of a daughter with Hypoplastic Left Heart Syndrome…she is a wealth of knowledge on many fronts…latest medical journal…top centers….

She is staunch Democrat. But, during the political race this year, she had the privilege to speak at the Democratic National Convention.

As many of you know, I am a Conservative….but I listened intently to her words.  Because on that night, although she was proudly supporting her political party, she raised awareness for Congenital Heart Disease on a platform that reached thousands.

Putting aside our political view points…

Looking at the problem as a whole for all the people instead of a problem that will help one party or another…

This is my wish for all items that matter in our government.

But, on that night, Stacey spoke from her heart for all the broken ones.

Here is her interview with Kristine McCormick (whom you will get to hear from tomorrow!) – Heart Mom to Angel Cora.

http://corashopesanddreams.org/chd-awareness-hits-the-white-house-the-lihn-family-tell-their-story/

CHD Awareness Hits the White House: The Lihn Family Tell Their Story

Submitted by  on March 25, 2012 – 6:53 pm6 Comments

Update: As a result of this video, Stacey Lihn, her husband Caleb and their two daughters too the stage at the Democratic National Convention. Her speech was live on CNN and many other stations. The reaction has been amazing to see. People are calling her speech the favorite of the convention so far. For most of her speech, the words “congenital heart defects” appeared below her name. Amazing! I’m so proud of her for advocating for what she believes in, and spreading awareness. She told the world about the one in 100 babies with a heart defect.

Here’s the video:

Earlier this month, a video was uploaded to YouTube that featured a congenital heart disease family. I suspect this happens a few times a week. This video was much different. This video was uploaded by the president, well, his staff, but still given how much in the limelight this video has been on the president’s campaign materials, there’s little doubt the president must be CHD Aware by this point. The story told the story of Zoe Lihn and her family, parents Stacey and Caleb and sister Emerson from Arizona, and why they support the Affordable Care Act.

It’s a political video. In the CHD world, we don’t all agree on politics. Some of us are Republicans. Others of us might be Democrats. And then some of us might lean to the Tea Party, or the Libertarians or any other party. That doesn’t matter. Why? Congenital heart disease research, awareness and all related issues are an issue that cross party lines. 

I could write an entire post about this, and started to before hitting the “Backspace” key. The President of the United States and First Lady wrote about the video on their social media outlets. For years, long before I got here, we as a community have wanted a celebrity to mention our cause. Love him or not like him so much, can we get a bigger celebrity than a sitting president?

I had to find out how this all happened so I asked Stacey to answer some questions for  me, and for you. I’m so glad I did because like everything involving the Lihn family, it’s super inspirational. Turns out this all started with a Facebook post!

Zoe Lihn: Born with Hypoplastic Left Heart Syndrome

Cora’s Story: How did the video come about? Did you reach out to someone in the campaign?
Stacey Lihn: Several months ago, I responded to a link posted on the President’s facebook page. The question: “how had the affordable care act affected you or your family?” I wrote a short paragraph about Zoe, her journey and added a link to our family blog (www.thelihns.blogspot.com). Months later, I received a phone call from a producer with the President’s re-election campaign asking me questions about Zoe and her lifetime caps – which were lifted shortly after her birth. After several phone calls over a week’s time, plans were made for them to fly from Chicago to Arizona to film our story.

CS: What was filming like? How long did it take?
SL: The “crew” included the producer and cameraman – two people who really took the time to get to know our family. They filmed our family on and off for 3 days – to capture our ‘real lives’ on a day-to-day basis. It truly felt like they were good friends and we were sad to see them leave. We continue to keep in touch and the producer was truly touched with our journey with CHDs and was astounded by the fact that they’re so prevalent, yet so masked and underfunded.

CS:  You got emotional at one point in the video, what does the Affordable Care Act mean to your family?
SL: Simply stated, life or death. Without the Affordable Care Act, Zoe could be refused insurance for her pre-existing condition. She could be uninsurable after reaching her lifetime cap. Zoe’s defect, HLHS, is extremely costly to palliate.http://pediatrics.aappublications.org/content/128/5/e1181.abstract – a 2001 study on the cost of inpatient and surgical treatment for HLHS). Without coverage, our family would be forced to file bankruptcy, lose our home and all assets (retirement funds, vehicles, etc.) for her to go on state-assistance. It would drive our family into extreme hardship (both financially and emotionally). Not supporting the ACA contributes to the further breakdown of our country.

CS: The video was tweeted by the president and shared widely, what do you think that means for CHD Awareness?
SL: I’m extremely proud for the opportunity provided to our family to spread awareness for Congenital Heart Defects. I’ve received several emails from strangers around the country who support the ACA and ask questions about CHDs and Zoe’s journey. Many have visited our blog and learned of the statistics themselves. CHD parents across the country, whom I’ve never met, shared their story with CHDs and how important this act is to their children and grandchildren. I felt the opportunity to spread awareness could not be passed up – not to mention my firm support of the ACA and the impact I feel it will have on our children’s lives (healthy or not).

CS: Any future plans to continue advocating for the Affordable Care Act?

SL: I will always advocate for the ACA. The outcome of the Supreme Court ruling, oral arguments taking place next week, will determine just how much advocating is necessary. I’m hopeful, just as I am for Zoe’s future, that the SCOTUS (Supreme Court of the United States) will rule in favor of our children and the millions of Americans living with pre-existing conditions, women who are discriminated against on a cost-basis and begin to regulate the private insurance companies who are making outrageous windfalls with in our current healthcare environment.

CS: How is Zoe? How did she like the filming?
SL: Zoe will be 2 years old in May. The fact that we’ve kept her fairly secluded in the past to avoid illness, she very much enjoyed the guests in our home! She was unimpressed with the lights and cameras, which is good because I have a strong feeling she’s not done and will be raising awareness for many years to come.

CS: Is there anything else we should know?
SL: Next week is an extremely important time in our country’s history. The Affordable Care Act will either stay in effect or be struck down (in part or wholly) by the Supreme Court of the United States. I’ve seen the rising care in healthcare costs and not only in Zoe’s care, but in my own. It’s driving our families into significant financial stress and I’m hoping that change is coming soon. Yes, change is scary but all of us know that without risk, there is no gain – we see it every day in the eyes of our heart warriors.

Thank you Stacey and the Lihn family! I have a feeling that this is just the first of great things for Zoe as well. Again, no matter your political leanings, the sitting president of the United States and his campaign staff know about CHD and it’s effects all because one mother reached out. Inspiring much? Go to it!

For every 100 letters, Facebook posts and phone calls, you might only hear back one time, but that one time might be something that opens up a huge opportunity to share your story, and to advocate for congenital heart defects.

And now, without further ado, the video featuring the Lihn family:

CHD awareness month: Moms on a mission

I wasn’t sure what I could do after I found out Evan’s diagnosis.

I mean…surely his special heart wasn’t just meant to be….it had greater purpose.

And, slowly, this blog emerged.  Little by little a few of you…my faithful readers…began reading our journey.

You began to learn about what heart defects looked like…not the medical part of it…but the how it changes lives – all of it – the good, the bad.

I hope to eventually pen something for Heart Mamas, mamas with medically special kids, or just mamas – on the experience of being a mom who has been called to care for these extra special, little souls.  To show these mamas…hope lives on and in these journeys.

The next couple of days, I feel privileged to be sharing the stories of a few great women in the Heartland who I wholeheartedly believe are changing what this diagnosis means for our little ones.

These women took the fear, the anger, the unknown of what CHD looks like and are creating HOPE.

 

Meet Amy and Sister’s By Heart

On October 1, 2009, my life changed forever. I sat in stunned silence while a very experienced perinatologist told me that my unborn child would be born with ½ of a functioning heart. This doctor, with years of experience and numerous credentials, looked me in the eyes and told me my unborn child wouldn’t have a good quality of life and that we should consider terminating. I was devastated. It was not until I left his office and went home to research the condition that I realized how little he really knew, that he truly had no idea how much HOPE there is for children born with complex heart conditions. Little did I know that God would use that experience to prepare me for the road ahead, for a bigger plan than I could have ever anticipated.

 14 months, 3 open-heart surgeries and a pacemaker later, it was December of 2010, my amazing son was thriving and I was beginning to connect with other families in the CHD community.  When I read a blog post from an expecting mom about how she broke down in the middle of Babies ‘R Us, unable to find anything her son could wear after his upcoming open-heart surgery, I realized I could help provide her hope. I emailed a handful of heart mom friends and asked if they wanted to help me, to send this mom a package with Babylegs and a set of side-snap onesies, both of which her son would be able to wear after his surgery; and to somehow give her HOPE.

 The moms I sent that initial email to are AMAZING, their response was beyond my wildest expectations when I sent that email. Within days, Sisters by Heart was born, complete with a logo, a website and a plan to give new moms hope. 2 weeks later, that mom, and 2 others just like her, had Sisters by Heart care packages on the way. Those packages included a handful of items useful in the hospital, a welcome letter from us and pictures of our thriving HLHSers. That was the start of an incredible journey of determination, hope and passion.

 In the past 2 years since our inception, Sisters by Heart has experienced tremendous growth. Now a registered 501(c)(3) nonprofit corporation, we’ve sent out over 400 care packages, have over 12,000 individuals following our Facebook page and website, and have connected hundreds of families through our unique Linked by Heart network of HLHS families nationwide.  We’ve added 12 “Linker” moms to manage our Linked by Heart regions and 3 new members to our Board.

 Sisters by Heart has been such a blessing for me to be a part of. I have met some incredible moms through my work there, and have been so inspired by the families we’ve been privileged to serve. It really, truly has been a perfect example of one of those things in life where everything just “comes together,” and we were in the right place at the right time.

 Each of our Board members has her own motivation behind joining Sisters by Heart and working so hard to help our families. But for me, it’s all about that moment with my Perinatologist, where he gave me no hope. I never want another family to receive a diagnosis and to be given NO hope. Our dream is that every single family diagnosed in the US will also be given a Sisters by Heart brochure and told “There is HOPE.”

 

“You never know how strong you are until being strong is the only choice you have.”

www.hopeforbabybennett.com – how the journey to mend our son’s broken heart changed our family forever…

www.sisters-by-heart.org – supporting newly-diagnosed HLHS families…one precious heart at a time…

 

CHD awareness month: She woke up with a zipper.

You hear stories in the news all the time about teenagers or even school aged kids suddenly collapsing during a sport or play.  Sometimes, that son or daughter, is spared, sometimes the story ends tragically.

You’ve read Evan’s story – the diagnosis in utero.

You’ve read Ava’s, Ellie’s and Finley’s – diagnosis soon after birth.

Please, meet, Rose.  Daughter to a dear Heart Mom, Mary, who one day had a healthy 8 year old….and then suddenly…

 

Cuddling with my husband on the couch while we watched a favorite movie the night of January 13th, the ambiance suddenly went from totally relaxed to totally tense as we heard footsteps across the tile and a little voice call out.

“Mommy?  Daddy? I feel weird again.”

Our eldest daughter, Rose, who was 8 years old, had been coming out almost every night saying that she was feeling “weird”. There was no other way she could describe it. It was beyond frustrating. We had taken her to all her well check-ups, and she had a clean bill of health. Other than a surgery for taking out her tonsils and adenoids when she was 4 yrs. old, there was nothing to alarm us. Except her repeat performance. Every night for the last month.

We knew there was something going on, but whether it was physical or a need for extra attention. We sent her back to bed with a promise to come check on her like we always did, and as her footsteps receded down the hall, we turned and looked at each other. My chest was so tight it literally hurt. Deep down, my “Mommy Alarm” was telling me that there was something that needed immediate attention- I just didn’t know from whom.

When she came out again, saying she felt weird- again- I took a very deep breath and said,” Can you tell me any other words to describe how you feel? ‘Weird’ doesn’t really tell me anything, Honey.” I suggested that we pray and ask for words. We did. About 2 minutes later, she had the words I will never forget.
                “It feels like butterflies are trying to fly out of a tornado in my chest.”

My heart froze, and I willed myself to be calm so I didn’t scare her. I knew it was something heart related, so I had her sit down on my lap in the other room and took her pulse. It was 45 beats per minute! I told her to go lay down and rest, and when she was out of sight, called the pediatric exchange. When I described what was going on, the nurse told me to hold the line while she got a hold of the dr. When she came back and asked how our daughter was doing, I was taking her pulse. It had lowered to 40 bpm. She told me to not wait for the dr. Just go to the local ER. Perhaps we were missing some beats- sometimes that happens.
                I woke Rose up, and told her we were going to the ER “just to make sure everything was okay.” My husband stayed home with our other three children, who were sleeping, oblivious that our whole world was about to turn upside down.

I had been so thrilled when we found out we were having a baby. More accurately that I was able to carry a baby after two miscarriages. I threatened to miscarry with her, so spent most of my first trimester in a recliner. She was born a strong, very pink baby. With APGAR scores of 8 and 9. Perfectly healthy. I noticed from the start that she snored loudly for a newborn, but was told I was “just a paranoid new parent”.  I couldn’t rest easy with that explaination. She was really hard to nurse. We had to use ice cubes on her tummy to keep her awake to nurse- and it was December! She would latch on, and then jerk and arch her back. She had circles under her eyes, but that was just her “unique coloring”. Still, I couldn’t let it go, and woke up multiple times a night to check her breathing. With the birth of our second daughter, I realized just how loud our first really was breathing. I kept talking to anyone who would listen, and finally a friend’s mother-in-law lent me a pulseoxometer (a machine that goes on the finger and measures heart rate and oxygen saturation) to see if anything was happening while she slept. She called me a couple days later to tell me something was definitely going on with our little girl. Her heart rate was dropping at night and her oxygen was going down to 60%! Within 3 months, we had been to the ENTs, had tests, and had her tonsils and adenoids out. Her breathing quieted and her color returned to a rosy pink. Rose was 4 years old, and NOW was totally healthy- again.

Now, 4 years later, as I drove at 10 pm through our mostly deserted town to the ER in my truck with Rose sitting quietly beside me, I was praying that I was wrong- that I was just being paranoid again.

We walked into the Er, and after giving our info, sat down for all of 2 minutes. The triage nurse called us in, and asked what was going on, I told her about the “butterflies”. She gave me a quick look of concern, and then smiled as my daughter looked at her. I appreciated that she was trying to keep my daughter calm. She put the pulseoxometer on her finger, and immediately looked at me, then led us straight to the room directly across from the nurses’ station. I have learned that it is never a good sign when you are put in that room! They took some blood from Rose and then a nurse rolled an EKG into the room. She put the stickers on Rose’s chest and abdomen, and started reading it. After 30 seconds she tore off the tape and left the room. I smiled at Rose, and suddenly the ER doctor was there- literally 30 seconds after the nurse walked out. He looked at me…

And shattered my world.

“Your daughter has a third degree heart block. We are calling the children’s hospital and seeing if they have a bed for her. She’ll be transferred by ambulance tonight. If it’s a local ambulance available, then you can go with her. If they have to send one, I’m sorry, but she will be transferred alone, and you will have to drive there yourself. It’s about 3 ½ hours away.”

The x-ray machine arrived for a chest x-ray right then, and I was asked to stand outside the room. As I stood there, trying to figure out what was happening, the ER dr. came over and asked if I was alright. I had no idea. I told him that I didn’t understand what was happening. In my mind, I was thinking that heart block was something that only happened to older people who ate too many hamburgers, not 8 yr. old girls! He explained that the disease that she had was an electrical disease. The top and the bottom of the heart didn’t “talk to each other”, so they beat at two different rates. She had two different heartbeats! My head was swimming as I called my husband and tried to explain what was happening. When I got back to her room, she was just sitting in her bed, not saying a word. I knew she was scared, but she was being so brave! She asked me to run home and get her favorite blanket and her Paddington Bear. I was surprised that she wanted me to leave her, but after making sure she was really alright with it, I drove home and grabbed her blanket and bear, and a few things for me, and hurried back, praying I hadn’t missed the ambulance b/c one nurse (who seemed to have a love for the dramatic) warned me that if the ambulance came before I did, they would leave without me. I flew back to the ER, and as I walked in, this same drama nurse stopped me and said, “It’s so good you are back. You almost missed the ambulance. It’s here and…..(as she paused, looking at me for 5 seconds- literally 5 seconds-I stifled the desire to yell at her and smack her at the same time) it’s one of ours so you can go with her.” Relief flooded through me. It was 3 am.


                As she was wheeled out of the hospital room, I snapped a picture of her on my phone. It is a haunting image- the first pictures of a childhood cruelly interrupted by a congenital heart disease we had never even heard of. It’s hard to even look at it without tears. She never cried, just kept searching my face in a silent wait- searching to see if it was something to be scared of or not.


                In the ambulance ride, the EMT was excited about her diagnosis, but trying to be sensitive to our traumatic circumstances. He kept looking at the monitor and finally leaned over and said, “I just have to print out a tape of her heart rhythm for my students. They probably keep telling you how rare this is, huh?” I said yes, though I realized later that I kept hearing it was rare, but didn’t know exactly how rare it was. Later I learned it was I in every 14,000.

            We arrived at the children’s hospital at 6 am and were taken straight to the pediatric intensive care unit (PICU). As we waited while the EMT talked to the admitting doctor, I saw him look at me sideways as he told him, “I was given orders to work on her if her heart rate went into the 30s consistently.” I just looked at him.  She had been dipping into the 30s every once in a while. I didn’t’ think much about it because he didn’t seem concerned, but I realized that when he was checking the monitors, he was also preparing to work on her if her heart rate didn’t come back up. A shiver ran through me. When we got to the PICU, I looked around at the other kids there. They were covered in wires, had tubes coming out of them, stuffed animals everywhere, but most of all, they were just lying there. They were so sick, and all I could do was look at my precious 8 yr. old and think, What is going on? We don’t belong here! She’s still the same child as yesterday, but…..This has to be a mistake. These kids are so sick, and she isn’t. But if she isn’t, why are we here? I just couldn’t get my mind to stop, and sat in the oh-so-comfy chair next to her bed, trying to stay awake as a myriad of doctors came parading through to see her.

Tests were ordered- echocardiograms, blood, I don’t even know what else. I gave family history over and over, and when rounds were made, I was told I had that all parents had to leave for the privacy of the patients. I had to tell her I would be right back, and turn and walk out the door, leaving her there. So small. So scared.  I called our family to let them know what was happening and that she seemed fine except for her heart beat was really slow- no, I didn’t understand exactly. I had family tracing down every heart issue anyone had and faxing any and every record to the hospital to see if there was any connection genetically. I answered so many questions- Yes, she is breathing on her own. No she wasn’t sick. I’d call when I knew more. I had to call a friend at 6:30 am and ask if she could bring some formula to my husband at home because since I was exclusively nursing our baby, there wasn’t any at home. I called and asked my mother-in-law if she could get the kids so my husband could try and get some sleep.

Things at work were rough, and he hadn’t slept for two nights before this happened. I was worried about him. Worried about my other kids who were waking up and wondering where Mommy and Rose were, and how there was no answer about when we would be home. I met with a social worker who explained to me that her job was to take care of me- did I have money for food? Did I need a change of clothes? I had come with only the clothes on my back and my purse. Later that day there was a lady who was from Family Services who came by to give me a green tote bag with a note pad and pen, a small bag of toiletries. I never thought a toothbrush and lotion could mean so much to me. I tried to grab some cat naps in the chair by Rose’s bed, but it was almost impossible, save for the fact that my body just couldn’t take any more. Every time I opened my eyes, there was a new group of doctors there, and they all kept printing out the EKG tape of Rose’s heartbeat. I began to think that they were calling their doctor-friends to come and see her (Hey! You will never guess what we have here! Come on by and see this kid with her amazingly rare heart!). The nurse told me later that we had been seen by over 60 doctors that first day alone.
         

We were moved to a step-down unit later that first day, and she went through more tests. I remember hearing that they were really excited that her heart was healthy- except for this one issues. She was a baffling case because it was so healthy. The next day, Thursday, we met our cardiologist/electrophysiologist, who drew a picture of a heart with a line through the middle of it- horizontally. She explained that Rose’s heart was strong, but the signal from the top of the heart that tells the bottom of the heart to beat right after it does was blocked. Since the bottom wasn’t getting the signal to beat, it was on a “back-up generator” so to speak. So, the top of her heart is beating away at the merry tempo of about 80 bpm, but the bottom is going, “Hello? Anyone there? When do I let this blood out?!?”, and since there is no answer, it decided to let it out at the rate of about 45 bpm in the daytime, 39 at night. So she has two heart beats, and that’s why she feels the butterflies in her chest. Rose was scheduled for a pacemaker implantation surgery the following Tuesday (we arrived there on Wed., so it would have been in 6 days) but because of her “healthy heart” we were discharged that Friday! Just like that, we were being sent home! I couldn’t believe it.
         

After we got home, we had to have our other three children tested. We were so grateful to God that they all came back clear! Two weeks later, we were back at the children’s hospital for a follow up. This was actually the first time I heard the doctor use the word “disease”. Our daughter has a heart disease. What she actually said is that the risk of this particular heart disease, although really small, was that one night she could go to sleep and never wake up. My heart froze. If she hadn’t been able to tell us what was going on, if she hadn’t had the right words to explain how she was feeling, I could have walked in one morning, and she would be gone….That scenario started running through my mind and I had to consciously make it stop. A new kind of fear moved into my life that moment, and didn’t leave for 6 months. We had to make a choice whether to have her paced right away, which was my first gut reaction, or to wait and try to give her the most “normal” life we could. After a lot of discussion, prayer, tears, and soul searching, we decided to try and at least let her finish out the school year.

The next 6 months were a blur of doctors’ visits, halter monitors, trips to the ER for chest pain to make sure she wasn’t having a heart attack (an 8 yr. old having a heart attack. I never thought of that), having her on oxygen b/ her sats. were dipping into the 80s, blood tests and more blood tests for her and for me to see if I had antibodies that may have caused her disease, constantly checking her pulse, her blood pressure (which I had to learn to do with a stethoscope), and checking her almost every hour of the night. Literally, almost every hour. It was like when she was a newborn all over again, except I KNEW what was wrong now. I would tiptoe into her room and lay my hand on her chest, counting the thud(1)….thud(2)….Thud(3)….that was her heart beat. You could literally feel it slamming against her chest.


             I honestly don’t remember much except how it relates to how Rose’s heart disease affected out family. I took our second daughter, who was in Kindergarten, out to brunch to try and explain what was going on with Rose’s heart. She looked up at me over her hot chocolate and said, “Mommy, can Rose die?” What an awful question for a child to have on their hearts, and I knew she had been wondering about it ever since she heard her sister’s heart was “broken”. After few moments and a lot of swallowing, all I could say was that we were doing everything we could to make her heart better.


                Her pacemaker placement was scheduled for late that August. As the spring wound down and summer neared, we were seeing signs that things were getting worse, and quickly. She couldn’t keep up with her class on a wildflower hike, She couldn’t keep up in PE. The ER staff knew us by first name. When I walked in the door with her, they’d immediately bring out a wheelchair and take her back.


                On June 9th, while we were sitting at the table making plans for summer, the phone rang. The voice on the other end of the phone said that all the doctors had been conferencing, and it was time for her pacemaker. They didn’t want to wait any longer. We frantically made arrangements for our other children to stay with Grandma and Grandpa, called family and friends, and packed our bags. It was going to be a three day trip- one day down and a pre-op apt., her surgery and an overnight stay, and discharged the next day.


                That night, as I made my routine heart check, Rose wasn’t asleep. She was trying to rest, but she was terrified. So was I. I held her and rocked her just like when she was a baby, and the torturous thoughts of whether or not I’d get to hold her began to strangle my heart with fear. I did my best to stay calm and not burst into tears because I didn’t want her to know how afraid I was. She finally dozed off, and I laid her down, kissing her face. I kissed the other 3 children as well, and suddenly realized just how much cooler her skin was than their skin.


                The next day we tried to make as light of the situation as we could. Three and a half hours of driving, with a few stops for fun, made us glad to get out of the car for her pre-op appointment. It went great. We headed back to the hotel, and ordered pizza for an early supper. She couldn’t eat after 7 pm but she could have apple juice, so we made a run to a store around the corner and bought some, along with a few games and a pair of heart socks that she fell in love with. Hearts had become somewhat special to us. After eating the pizza, we watched TV and then tried to get some sleep, knowing full well we wouldn’t get any. I laid down with her until I heard her breathing slow, and laid my hand on her chest. Thud(1)….Thud(2)….Thud(3)….

             We woke up early, and set off for the hospital, which was only about 10 mins, away. I can still remember vividly the dress she was wearing along with her pink socks, and how she clutched her bear as she tried to give a brave smile. We got to the hospital and walked into registration. In a short time, she was changed into her gown and ready to go. They called her early, and at about 10:30am we kissed her and told her we’d see her soon. I wrestled with the urge to take a picture of her chest- her perfect, unscarred chest. I didn’t do it. They wheeled her into the elevator, where she gave a brave smile as she clutched her Paddington Bear beside her. Then the elevator doors closed.

           We went to the family waiting lounge where we were issued a pager to notify us when the surgery was done. We went to the cafeteria, and then back to the family waiting lounge, where we ran into a family we knew from home- small world! My husband went outside and read magazines while I chatted with the mom. Our girls had been in class together in the 1st grade. Before we knew it they were telling us that Rose was coming out of the surgery and the cardiologist was coming to confer with us. The cardiologist said that things went well with one hitch- her chest wall was too thin to accommodate a two-lead pacemaker, so for the time being there was only one to make the bottom of her heart beat fast enough to keep her safe. Her heart would not stop in her sleep. She would have to have surgery again when she was a teenager to put the second lead in. It was really disappointing to hear this, but a small thing in the scheme of things. We went to recovery to be with her when she woke up. Watching her wake up was one of the hardest things to endure. I wanted so badly to hold her, but I couldn’t because of the IV lines and her arm was in a sling. A lady wheeled a large harp into the recovery room and began to play. The music was so beautiful, and it was having an obvious affect on the children recovering. Rose relaxed with music. I could have hugged the lady. I was so grateful for anything anyone did for her. My husband and I noticed her chest thudding, and mentioned it to the nurse practitioner, who was the specialist in pediatric pacemakers. She told us that because Rose’s heart had been working so strong and hard for a while, that it beat hard naturally. It was okay for the time being, but should subside.


After she was moved to the short stay floor, she was in tremendous pain. She was given morphine, which made her sick to her stomach. One of the nurses on the floor went toe-to-toe with a doctor about the meds. He was telling her morphine wasn’t making Rose sick, and she informed him that she didn’t’ care about hwo much schooling he had, she worked with kids in recovery every day and she knew that it DID, so order something else! I love that nurse. As the evening wore on, Rose began to have hiccups, but these were so forceful that the bed would shake with her. We were really uneasy, and had the NP called again. She was already on her way to check and make sure that the pacemaker was working. When we pointed out our concerns about the “hiccups”, which of course weren’t happening at that moment, she stayed until she saw them. She lowered the voltage of the pacemaker, and that seemed to work. She surmised that because Rose was leaner than the average child, it seemed that the voltage wasn’t blocked by layers of fat and so it was pacing the diaphragm as well as the heart. After seeing that all was now working fine, she went on her way, telling us to call if anything else didn’t seem right. NOTHING seemed right. Six months ago, everyone in our family was fine, and now our daughter had a machine making her heart work right! I just smiled. I left to pick up some prescriptions from the hosp. pharmacy, and my husband stayed with Rose. When I walked back into the unit, every nurse at the nurses’ station stopped working and looked at me. My heart stopped. That is never a good thing.  Our nurse looked around and slowly stood up. She told me that after I left, Rose was sick to her stomach again. My husband was holding her and helping her, and looked up at the monitor as she was vomiting. Her heart rate dropped from the 80s to the 50s, which is where it had been before the pacemaker placement. Deb, the NP came back immediately, and told me that a chest x-ray was being done. She told me that she was 99% sure the pacemaker lead had detached from Rose’s heart. That meant that she would have to have surgery again. Immediately. The x-ray confirmed it, and we were packing everything up and making phone calls to family to inform them that she was going back into surgery. When we told Rose that the lead came loose and she had to have surgery again, all she quietly said was, “Okay.” I didn’t handle it with quite that much grace. I wanted to crumple up and bawl, but she was being so brave that I swallowed all my tears and tried to look calm for her sake. The NP and others kept telling us how rare this was. They were nervous. I told the NP that we believe that God is in control of everything, and we knew this wasn’t their fault. All the nurses at the nurses’ station stopped and gave us encouraging smiles as we walked quickly by on our way to the cath lab for surgery #2. 


       Because it was after hours, we had to update a new team of anesthesiologists. We were able to walk with Rose all the way to the cath lab before we kissed her and said, “See you soon, Baby. We love you.” The surgeons told us that this time the surgery should only be about 1 1/2 -2 hrs. instead of 3-4 hrs. like before, and that we would see them come out of the same doors with her.  We could then walk back to the recovery with her. There was a family waiting lounge across the way, and we waited there. It was 8:45pm.

       We walked over to the other side of the hosp. and found a small cafe, where we bought a hot chocolate and some fruit knowing that we needed to eat something, although we weren’t hungry at all. We wandered back to the waiting lounge and sat at the end, where we made a few phone calls to update some friends. There were other people there now, and they were laughing and talking quietly amongst themselves. They were happy, but respectful, and I was glad someone could enjoy being there.


About 10:15 pm I hung up the phone after talking with a friend, and looked up. I was startled to see two surgeons walking quickly towards us- without our daughter. Our hearts froze. Hands down the worst moment in my life. I knew something was wrong. One was our cardiologist, and the other was a doctor we didn’t know. We nicknamed him “Dr. Jolly”, because he seemed so thrilled at the prospect of operating on our daughter. We later learned that they grabbed him as he was about to leave because they needed him to assist. He was dressed in a torn white t-shirt and jeans, and no looking the most professional at that particular moment. My husband was giving him dagger looks as the cardiologist told us that first of all, she was ok. I remember thinking, “If she was ok, why the h— are you out here talking to us instead of bring our daughter out?” She went on to say that in the two plus hours since the x-ray, the lead had moved. It had pierced Rose’s heart, pierced her diaphragm, and was up against the outer wall of her chest. They needed to open her abdomen, poss. Open (crack) her chest. They needed to move her to a bigger OR across the hospital for emergency surgery and needed our consent. We gave verbal consent, and they left quickly to move her. While the surgeons were talking to us, the lounge went silent. Everyone knew something was wrong with our child. Some of them had seen her go by on the hospital bed when we first walked by. Now they all sat silently as the surgeons walked away and we slowly turned, sank into the chairs we had been sitting in, and I began to cry. After a minute or two, I got up and went to call our family. While I was gone, the second surgeon (Dr. Jolly) came back out with the papers and my husband signed them. He said that he though our cardiologist must have chewed him out because he was much more subdued and respectful. My husband still gave him dagger looks as he handed back the paperwork.  Soon after, our NP came and took us to a waiting area outside the cardiovascular intensive care unit (CVICU). She was visibly upset and concerned, and told us this just doesn’t happen. She showed us the elevators where Rose would be brought up to the floor, and told us it would be about 3-4 hrs. before we could see our daughter. Then she left to help with the surgery.

       I cannot put into words just how much we learned the real meaning of “praying without ceasing” and pleading to God to be given our daughter back. Thoughts of what life would be like without her and how we would tell the other children what happened flooded my mind. I wanted to hold her so badly. So badly. Sleep was out of the question although we were absolutely exhausted in every way. My phone was low, so we turned it off. It is a strange feeling when although she is both of ours daughter, we had to deal with this on an individual level as well as together. I kept remembering when we dedicated her. She was 8 mths. old. We promised her to the Lord. Although I meant every word of the dedication, I never dreamed that I would have to face the reality of how sincere I was head on. I had to give her back to God, and let go. It was agonizing, and so eye-opening as I begged God and searched my soul.

        At 12:15am I looked at my watch, and then looked up to see her cardiologist standing in front of us. She had a smile on her face as she told us the surgery went “perfect”. They didn’t have to open the chest completely, although they did have to cut part of her sternum to stitch the heart and attach the new pacemaker, which was a dual-lead and was now in the abdomen instead of up by her shoulder. This was better b/c instead of a small screw into the heart, the leads were actually sutured onto the heart, and she would not have to have another surgery until it was time to replace the battery- about 10 yrs. This is the silver lining and the blessing in this 3rd surgery. We literally all let out a sigh together, and it took a few breaths to get my heart back to normal. She said it would be at least another hour and a half before we could see her. We decided that my husband should go back to the hotel, which wasn’t far, and rest because he had to drive back home later that day. It was our youngest daughter’s birthday. I called our families to tell them the news, and then sat back down to wait for Rose. At 1:30am they brought her up to the CVICU. The surgeon (still another one) and the anesthesiologists were all smiling as they briefed me on the surgery. The surgeon was so compassionate. He took me into the CVICU and let me to about 20 ft. from Rose’s room, just so I could see her and see she was “OK”. I couldn’t go nearer b/c she wasn’t cleaned up from surgery yet. He asked me (begged, really) to wait about 20 more min. outside the unit. Finally, one of the nurses brought me into see her. She was still sedated. She had a chest tube in, and was on a ventilator. I couldn’t see her incision sites. She was so beautiful lying there. There was no thudding of the chest, and her skin was starting to have a warm rosy glow despite all she had been through. I actually thought she was overheated because she was so pink. I didn’t realize until later when I was putting school pictures together just how pale she had become. It’s like the frog in the boiling water- She had become so pale so gradually that we didn’t realize what was happening.


        The nurses urged me to go and see where they had arranged for me to stay for the night since she would be kept sedated. I could come back and sit with her for a little bit after that. When I returned they were calling for the doctor because she had shocked them all and was waking up, trying to pull out her breathing tube! The nurse and I held her hands away from her mouth as we waited for the doc, and marveled at how strong a sedated 8 1/2 yr. old could be after 3 surgeries in less than 14 hrs. They took out the breathing tube, and I started to feel hot and sweaty, and without even looking over, the nurse asked if I had to sit down.  Apparently this wasn’t all that uncommon with parents. The nurse brought me some ice water, and urged me to go and sleep. I decided to listen to her. I finally fell into a fitful sleep about 2:30am. 


        I woke up and went down to be with Rose at 7am. She was in pain, and very weak, but otherwise doing well. My husband arrived shortly after I did. When the doctors made their rounds and went through her case with the residents and fellows, they all were amazed that there was no internal bleeding from the heart or diaphragm. They kept repeating, “No bleeding? No Bleeding,” and shaking their heads. Prayer is awesome. Mark left about 10 am to get me some brunch, and when he came back, he stayed with her while I ate. There was no food allowed on the floor since the children couldn’t eat. Later that morning they transferred her to the interim ICU (a step down from the CVICU). My husband had to leave that day. At least one of us would be there for our youngest daughter’s third birthday. (I called later, and found out that she had a wonderful birthday with her grandparents, brother, and sister, and an aunt and cousins who came up and brought supper. She still hasn’t forgotten that I wasn’t there for her birthday, though. It still hurts.) The first night, we shared the room with a 5 1/2 month old baby. The next day the crib was taken out and a beautiful little 9 yr. old girl was brought in. God had a delightful surprise for us, because the little girl and her mother became quick friends with us. 


        We were able to come home Tues. afternoon. What had been an overnight stay in the hospital turned into 5 days. It was so hard to say goodbye to our new friends. We had only known them for 4 days, but we know we’ll be life-long friends. Home had never looked so beautiful.

I went to my mother-in-law’s to get my children, and my 14 month old screamed when I held him. He wanted nothing to do with me. I had to call my husband to come and get him. I expected it, but it still broke my heart. He wouldn’t come near me for the first day or so, which I also expected, and then he wouldn’t let go of me. He still gets terribly upset when I leave to do an errand, so we’re working on separation anxiety all over again. Our second daughter, who was 6, had a fever before we came home. She had to be really brave, too, because she couldn’t come home from Grandma’s and Grandpa’s until Thurs. It was so good to finally have our whole family together. It had been 8 days.


Later that month, I read the surgeons’ reports about the surgery. It made me queasy with fear to realize again how close this was, and I read a few details that I hadn’t heard before- like how the lead missed a major artery by less than .5 cm. I have copies of the x-rays, and can see how it all went, but still can’t quite wrap my mind around it all sometimes.


                We go back for Rose’s clinic visits every 6 months, where they interrogate her pacemaker by putting a magnet over it. She is 11 now, and it still makes her body jump like the giant hiccups – just like it did after her surgery. Every time we walk up to the front of the hospital, we slow our steps just a bit. I can’t help but remember how life changed there, and have to fight off a surge of conflicting emotions. I am so grateful that we found her CHD before something tragic happened, like so many of the athletes that collapse while playing high school sports, but there is always a surge of grief for what we lost- what Rose lost- a normal childhood.


                The night before Rose’s ER diagnosis, I was on the phone with my mom and told her, “I don’t know how moms with special needs children do it. I don’t think I could. I am so grateful that all my children are healthy.” Those words are haunting now. I have learned the hard way that moms with special needs children are just moms who have learned to tap into that deepest of instincts to do whatever we have to do for our children. We don’t have some sort of different instinct than other moms. We have just learned to trust it more. For years I had that nagging “paranoid” feeling that turned out to be right.


                I recently had someone we have known for years say to me, “Aren’t you glad it’s all over? Like, aren’t you just glad to put it all behind you now?” I was speechless, not because I didn’t have anything to say to her, but because I felt like she had just kicked me in the gut and there was no wind left. All over? Rose will have a machine helping her heart beat for the rest of her life, barring a miracle. She will have to have surgeries to replace the battery, or any broken leads, for the rest of her life. She will have a cardiologist who knows her on a first name basis her whole life. Decisions will be made based upon how close we are to a children’s hospital or how close she is to a good heart center when she is older. There are certain things she can not do, certain sports she can’t play, certain toys that aren’t allowed in our house because they will interfere with her pacemaker. When we were in the midst of her late diagnosis, we didn’t know ANYONE who understood. We didn’t know children could have pacemakers. We didn’t know that a child could look so healthy and have a heart disease. Since then, we have found a whole community of heart moms and dads that do “get it”. Even in that community, though, we are a bit of an anomaly. We recently received a set of records that may suggest that Rose wasn’t born with complete heart block. Whether she had 1st of 2nd degree, we don’t know. We don’t know when she developed her 3rd degree heart block. What we do know, is that regardless, we are loved and accepted in our heart community.


                The hardest part of this whole journey is seeing how it affected Rose. After her diagnosis, she became a very nervous child. She was brave most of the time, but there were moments when she would cry and ask “why me?” After her 3 surgeries in one day, she became very clingy, and then was diagnosed with Post Traumatic Stress Disorder. As she got used to her new rhythm and how it felt, she had no idea that her heart was supposed to speed up when she was scared, or how the heart reacted to other emotions like ( anxiety, excitement, etc.) and so she would freak out about it. People didn’t understand why she would have meltdowns. She didn’t very often, truthfully. We lost quite a few friends because they didn’t know how to deal with her “condition”, even though there was nothing to “deal” with. All they had to do was accept her.  We were excluded from get-togethers, and our circle became very small after the novelty of her condition wore off. It seemed that it was too hard for people to be reminded that anything can happen to a child at any time. I had a hard time seeing anything except through the filter of how it related to the CHD that had so cruelly reared its ugly head in our family. After some counseling, it has been downgraded to generalized anxiety, and now pretty much gone,  but every year, twice a year, just before we go to her clinic visit, she has a really hard time. I do to. I haven’t been diagnosed with PTSD or anything like that, but certain smells, certain sounds, certain situations that evoke emotions I felt that June take me right back to that moment when the doctors came out of the OR without our daughter, and my whole world stopped. I can’t breathe, I can’t focus, and I have to take a few moments to remind myself that she is doing great now, and I can’t deal with the “what ifs”.  So I hold all my children more. I love and appreciate all the little moments we have. I take nothing for granted. I beg moms to get a pulseoxometer test on their newborns. I tell parents to take their child’s pulse randomly just to know what it is so they can tell if it changes dramatically. And I tell our story whenever I can to let people know that heart disease isn’t just for old people, and just because your child was “healthy” didn’t mean that nothing can be wrong, so don’t ignore your gut feelings.


        About 6 months before our life changed in the ER, I mentioned my daughter’s heart to the pediatrician because I thought something was wrong with our other daughter’s heart. Her heart rate seemed to slow and speed up randomly, while Rose’s was steady all the time. He listened to Rose and had her jump up and down, and then said, “Well, if it didn’t accelerate it would be heart block, but her’s does, so just watch it and see if she starts having symptoms.” I didn’t know enough to ask for an EKG. How many times I wish that I had asked! I didn’t know anything about CHD except that a cousin’s baby was born with half of a heart and had to have 3 surgeries to repair it. I had no idea how far reaching it was or that it affected older children, too.


       I can’t change anything for Rose. There is no cure- yet.  If you saw her today, and didn’t know our story, you would have no idea that anything was wrong. If I can help one family through our story, and there is one less child who collapses on a field or in a hallway from a CHD no one knew that they had, then Rose will tell you it is worth it. She will also tell you that although she is only 11 years old, it is her passion to educate people about Congenital Heart Defects/Diseases.

 

CHD awareness month: The Diagnosis

Sometimes I wonder if getting Evan’s diagnosis while I was still carrying him was a blessing or a curse.  I spent the last half my pregnancy in agony…worried about what may be…if he would make it.

But, reading about stories of those who were diagnosed after birth….the shock and heartbreak of thinking you had a “normal” child to now be a child with a lifelong disease…is agony in itself.

I have included 3 stories today….all three late diagnosis.

Finley’s Story

Finley was not diagnosed until 3 days old. He showed no symptoms except trouble keeping his temperature up. Thankfully his pediatrician heard a different toned murmur and requested an echo. Instead of taking our baby boy home we received the most devastating news of our lives! It was very shocking, I had at least 8 sonograms, they saw four chambers at our anatomy sonogram. Since he was diagnosed late, he had been allowed to eat. That is likely why he developed NEC (a serious infection of the intestines that causes the intestines to deteriorate).  Complications from NEC led to a emergency LADDS procedure (stomach and intestine surgery) only days after his first heart surgery and a four month stay in the NICU.

I am thankful everyday for our chosen pediatrician. Without her, Finley would have gone home and likely died from NEC. There is appointment thatgoes by with her that I don’t thank her for saving his life!



 Ellie's Story
 

Ellie was born, at home on January 30, 2012 sometime between 11:59pm and 12am. I got to pick her birthday since it was so close, and since I did all of the work on the 30th, her birthday was the 30th! We were told we had a healthy baby girl developing in my tummy so we went ahead with the home birth. I had a csection with my first daughter and was pretty mentally scarred from it. I wanted a VBAC so badly. And when I felt that I couldn’t get that with my OB, I found a very supportive midwife and birthed at home. It was an AMAZING experience. It was a LONG day. And it was the hardest thing I have done. I was in labor for 20 hours and I pushed for 3.5 hours. Ellie came out perfect. She was breathing, she nursed, I was mentally healed from my last birth and everything was just perfect.

Until we went to the pediatrician on Feb 3…

I was still on my birth high and was happy to show my new baby off. When the pedi came in and asked about her birth, I answered that she was born at home. He looked at me like I had done something wrong and asked if anyone was there with me. I explained that I had a Certified Nurse Midwife with me, so yes. He didn’t like the idea of it… AT. ALL. He asked me why I had a home birth and I told him because I didn’t want another csection. And he continued to try to make me feel like an irresponsible parent. He asked about her coloring while he was listening to her chest. I mentioned her race to him (white, Hawaiian, Portuguese, and Asian). He may have mentioned a murmur (which honestly didn’t really mean anything to me) and he said she looked blue. I didn’t see the blueness that he claimed and I thought he was just saying that because he didn’t trust my midwife and was mad we had a home birth.

He asked the nurse for their pulse ox machine and clipped it on Ellie’s hand. The readings were in the 80s and 90s. He clipped it on her foot and it was in the high 70s. He told us that that wasn’t good, but that his pulse ox was for a child, not a newborn. He wanted us to go to the ER just to get a pulse ox done on her. He said that it needed to be 93 and above. And I was like, “well, her hand was in the 80s, that’s close”. Seriously, I had no idea what it could mean. And he didn’t bother to tell us either. He just told us to go get the test, that it was no big deal.

It would have been nice to have a heads up… considering the fact that he later told us that he knew it was probably her heart. I’m still angry about it.

Since the pedi didn’t make the situation sound urgent, we packed Ellie up and went to get lunch at McDonalds. She nursed and was fine. We had to get my other daughter from my sister’s house as well. On the way to the hospital, I got a call from the pedi and they are YELLING at me, asking me WHY I wasn’t at the hospital yet. So I told them I had to get my other daughter and take her somewhere else (to a friend’s house), so she would be close to the hospital. They weren’t nice, but again… they didn’t mention ANYTHING about her being seriously sick.

I drop my daughter off and we go to the ER. The pedi had mentioned that we would just get this ONE test (the pulse ox) done and nothing else… we didn’t belong in the ER, but that is where they wanted us to go. We get back in a room and SIX people surround Ellie. They start trying to get an IV in her and I start crying. My husband spoke up. Nobody listened. The pedi at the hospital got there and wanted to see her on room air. The ER nurses weren’t listening and were trying to put an oxygen mask on my screaming daughter’s face. My 4 day old daughter. My perfectly healthy VBAC baby. Her sats were in the 70s and 80s. The hospital pedi told us it could be something with her heart or circulation. They did a chest Xray and called Children’s National Medical Center. She came back and told us that Children’s wanted to see her. Ok. And she mentioned that a helicopter was on its way. WHAT?!?!?! Ok, so I can ride with her… we will be ok…. Actually, no. You can’t ride with her.

WHAT?!?!

We were angry. My husband told them that we would just take Ellie and drive up to Children’s ourselves (it is a little over an hour from that hospital). It was ridiculous. They STILL didn’t mention how serious this could be. I still have a HUGE problem with that. They convinced us not too and said the helicopter was almost there anyways. And she would be receiving Prostoglandin. I didn’t even know why she was getting it. They probably explained, but I was preoccupied with the fact that they were going to take my baby away from me on a helicopter ride.

The transport team from Children’s got there and started to give her prostoglandin before they took her and FINALLY they explained that something serious could be wrong. We finally felt the urgency of the situation. My whole head was spinning. Everything had just been perfect…

The transport team leaves, with my brand new baby, and my husband and I carry an empty carseat out of the hospital. We went to get Abby (my other daughter) and we saw the helicopter take off. It was horrible.

We dropped my Abby off at home (my parents took care of her) and got a few things and booked it up to the hospital. I pumped in the car. I had no clue what I was doing, I had never pumped before.

We got a call from the transport team while we were driving and they reported that Ellie slept the whole ride and was fine. They were so sweet.

Then, as we were almost in DC, we got a call… I guess it was her cardiologist? She didn’t hesitate. She made sure I was Ellie’s mom and then said, “You daughter has a life threatening heart defect, Transposition of the Great Arteries, she will need surgery to fix it.”…………………………..

I felt like I couldn’t breathe, or hear, or speak, or see.

I had to have her repeat it… there was nothing wrong with my perfect VBAC baby.

She repeated it. I repeated it. And she proceeded to tell me that she had to have a cath procedure. They were prepping her for it now. And that they might start before we get there. We hung up and I lost it. LOST. IT. I could barely get the words out to my husband before I started sobbing. I couldn’t believe my little baby had to have heart surgery!

We got a call a few minutes later from someone else… I think the person who was going to do the cath. He explained the whole procedure to me. Every step. And I said, “ok” at the appropriate times, but I have no clue what he said. I didn’t even know what was wrong with her heart. I had never heard of that before. I didn’t know anything about heart defects.

We got to Children’s in enough time to see her before her procedure. It was terrible to see her little body lying on a tiny hospital bed hooked up to all kinds of wires. I was in shock.

We went to talk to someone during her procedure and he explained, with pictures, what was wrong with her heart. He answered our millions of questions. They were all so kind and patient. I’m sure they are used to parents in shock and crying.

Everything went ok from there. She had her open heart surgery, to perform the arterial switch on February 9th. We got to bring her back home on the 18th. There were only slight setbacks, like her taking a while to come off of oxygen, but we found out that she had fluid around her heart, so it kept us there a could of extra days. Other than that, nothing else has happened.

And now looking back, it all seems like a horrible nightmare. I still remember it, but it doesn’t feel real. My eyes have been opened to the fact that anything can happen to anyone. It is still painful to think about. I’m still emotional when I tell our story. But I realize how lucky we are. The pulse ox saved her life.

Ellie is now 1 and you would never know she was so close to being gone. I looked in the notes from the hospital and when she got to Children’s, her sats were in the 60s. A few more hours or 1 more day, and she would not have made it. We are SO glad she is still here with us. She is the happiest baby. And, I plan to have more home births. 

 

Ava’s story 

I have always been fascinated by birth. I’ve worked as a doula for over five years and attended births in multiple hospitals. I thought I knew just about everything there was to know about prenatal care, safe practices during labor and delivery as well as good postpartum care. But until my daughter was born in July 2011, I had never seen a newborn hooked up to a pulse/ox monitor. I had observed newborns weighed and measured. I had seen newborns receive their first bath, first blood draw and first shot. I’d seen nurses and pediatricians listen to breath and heart tones. But I had never seen a little bandaid-type strip wrapped around a tiny hand or foot to check the oxygen level in the new baby’s blood. In all my reading and research, it was never mentioned (at least to my recollection) that this was a screening that shouldn’t be skipped.

Thankfully, Ava was taken to the newborn nursery shortly after she was born to keep her warm while my OB finished up my c-section. Why am I thankful that my newborn was taken away from me? Why am I thankful that I missed out on critical bonding time? Because while she was in the nursery, her nurse decided to hook Ava up to a pulse/ox sensor. There wasn’t a “need” to. Ava’s APGARs were 8 and 9. She wasn’t showing signs of respiratory distress. But when that nurse, forever one of Ava’s guardian angels in my mind, hooked her up, Ava’s oxygen level was 54%. Definitely NOT enough oxygen, even in a newborn.

Why was Ava’s O2 level so low? Because she was born with a congenital heart defect.

It wasn’t caught during the level 2 ultrasound done at 23 weeks. In fact, it wasn’t caught during any of the 7, yes I said 7, ultrasounds done on Ava during my pregnancy. The screening that saved Ava’s life was having that little bandaid-type strip wrapped around her pink little foot.

There is a push from the CHD world, the Heartland as Czarina has so aptly named it, to have every newborn screened with pulse/oximetry. Too many babies are still being born with a congenital heart defect that wasn’t caught during pregnancy. Tragically, babies are still dying from undiagnosed, and undetected, CHDs. However the need for all babies to be screened with pulse/ox is not met with unanimous approval.

There are some birth proponents, especially in the homebirth world, that view most post-birth “routines” with a jaundiced eye. They feel as though those things can be harmful to the essential mother/baby bonding that occurs in those critical first hours. I get it. When I got pregnant with Ava, I explored the option of homebirth. I’ve always been an advocate for homebirth, especially if you have a low-risk pregnancy. I’m of the mindset that the majority of births can be safely attended either at home or a birthing center. I advocate for delayed first bath, newborn exams performed while baby is laying on mama and not getting weight and length right away.

So the first time I was met with resistance at my assertion that ALL babies receive the pulse/ox screen, I was taken aback. How could anyone think this was a bad idea? There’s nothing invasive about it. It doesn’t hurt the baby. The baby doesn’t need to be held in a certain position. Baby can stay snuggled up on mama’s chest while the sticky probe is put on a foot or toe or hand.

So why would anyone be against it?

Not every baby is going to have perfect O2 levels a few minutes after birth. Some are a little lazy and it takes time for them to get the hang of this breathing thing. Not every nurse or doctor is adept at getting the probe placed correctly on a wriggly foot. So the baby may present with a “false” positive. And here’s where the concern comes in. That this baby, who is perfectly fine, then gets taken away from it’s mama and sent to the NICU for further tests. For xrays, EKGs, ECHOs, etc. And the bonding window is slammed shut.

So how do we fix this problem?

How do we make sure that babies get to bond with their mamas but also get this crucial screen? Where’s the middle ground?

Frankly, I feel that if a homebirth midwife or a birthing center is willing to have a portable oxygen tank and vials of pitocin on hand at a delivery, just in case, then there should be little resistance to adding a pulse/oximetry machine to their equipment. Heart and breath tones are listened to with a stethoscope, so what is the harm in putting on a sensor to check O2 levels?

Will some babies be referred to their pediatrician needlessly? Maybe.

But how many babies will be saved?

I think that the biggest key to all of this, to satisfy both sides of this debate, is education.

Help birth attendants understand which pulse/oximetry machines are preferable for newborns. Explain proper placement of the probe.

One of the first things heart mamas learn is how to read a pulse/ox machine. Is the sensor picking up “junk”? Are the numbers good? Because for us, with babies that have lower O2 levels all the time, knowing if we can “trust” the number on the display is the difference between calling the on-call cardiologist and calling 911. So we can teach birth attendants the same thing.

Have a newborn giving funky O2 readings during the pulse/ox screen?

Ask the following questions:

-Is the probe placed well? Should it go on a toe or hand instead of a foot?

-Is the extremity warm? Should a sock go over the foot?

-Does the probe need to be changed?

-If the machine runs off battery power, is the battery charged?

Many times, if you are seeing “scary” numbers on the pulse/ox machine, one of the troubleshooting steps above fixes the problem and gets you a good reading. Determining a baseline for what’s considered a “bad” reading would also be helpful. Is an O2 level of less than 90% when measured 20-30 minutes after birth a problem? Or can it be as low as 85% during that time and still be ok? Should O2 levels be measured at predetermined times? For instance, 15 minutes, 1 hour and 24 hours after birth?

Pulse/oximetry screening for newborns doesn’t have to interrupt crucial bonding between mother and baby. If you are pregnant, or considering having a baby, I strongly advise that you speak to your care providers about pulse/oximetry screening. It is a simple, life-saving test.

CHD awareness week: The teen years!

The Teen Years With a Heart Warrior

It started nearly 15 years ago, before he was even born, that we learned first hand about CHD’s.   Pregnant with our second son, we learned at 22 weeks gestation that he would be born with a complex heart defect called Hypoplastic Left Heart Syndrome (HLHS).  We were told we had 3 options: 1) transplant; 2) 3 staged surgical repair or 3) comfort care.  Even though we were at 22 weeks, we were even offered and almost urged to terminate the pregnancy.  Neither comfort care or termination was ever an option. After the shock wore off, we threw ourselves deep into research and reaching out to others who had been down the same road.  Unfortunately, at that time, there was no FaceBook so support came in forms of e-mails back and forth with a handful of success stories online.  

After countless hours of research and second opinions, Jarod was born on 6/2/98.  He underwent the Norwood at 2 days old, the hemi-Fontan at 6 months old and the Fontan at 2 years old ( the second and third surgeries by Dr. Norwood himself). 

While these early years were rough and extremely stressful, we did have another healthy son, JW,  to raise and watch grow.  He was and has been such an amazing big brother and always there to help out when needed.  There were many times where I had feelings of sadness for the normalcy he was missing out on as well as the change in our family life.   While JW has never once complained, he has sacrificed a lot.  Through all 3 surgeries, there was only one complication, an infection, landing us in the hospital for 6 weeks on antibiotics and out of state no less.  I missed JW’s kindergarten orientation and his first lost tooth but had to be with Jarod during this critical time.  Oh how I wished I could be in two places at once but always looked forward to the weekends when they would come to visit in Delaware.

Fast forward 11 years and all of the sudden, Jarod is a teenager, YIKES. Not only did I have one teenager, but two teenagers, and boys at that!   In actuality, the teen years with boys have been much easier than the early years.  Honestly, after Jarod’s repair was “complete”, life in general became much easier.  In addition to his HLHS, Jarod also has cerebral palsy, asthma, and thyroiditis.  
As far as meds go, we used to just crush his meds up and put in applesauce, him none the wiser. It wouldn’t be long before we realized this was a mistake.  There was one particular hospital stay when the nurse was going over meds with me and Jarod said “I don’t take any meds!” and there it was… I got that “look” from the nurse, that look I am sure many of you heart mama’s can relate to, that look you get like you literally have 3 heads and you feel the urge to look behind you to see who said nurse is looking at, yeah…that’s the look.  It was at that time, I made the decision to make him his own advocate. It wasn’t long before he would learn what each med is for, when it is to be taken and what it looks like.  His current meds are Lisinopril 10 mg daily, Singulair 10 mg daily, Synthroid 112 mcg daily, baby aspirin daily, Flovent 110 mcg inhaler twice daily, multivitamin daily, ProAir inhaler as needed and DuoNeb nebs as needed.  This is quite the list and I only list them here because Jarod can recite this list correctly and explain why he takes each one.  This is actually very important when your child is old enough.  I know how hard it is to leave our kids even for a few minutes in a hospital but, if we raise them to advocate for themselves, I personally think it makes the road ahead a little less worrisome as they grow into adulthood.  Because Jarod knows what the pill should look like, he checks before taking it and has questioned many nurses if a pill looks different (usually because it is made by a different manufacturer) and then will ask me if it is okay to take.  There was one hospital stay when he was less than a year old where I caught a “different looking pill” and asked them what it was.  The nurse tried to tell me it was his aspirin (at the time he was on a 1/4 of an 81 mg tab) but I was not comfortable so I made her check with pharmacy.  It turns out, the pill she tried to give was a 325 mg aspirin!  Another instance was at the pharmacy: I had a refill and Jarod had a refill.  When I got home, I checked the meds and my migraine medicine was in his lisinopril bottle, his lisinopril in my bottle.  While I work in healthcare and know to check, I shudder at the thought of the families that are unable to advocate for themselves.  I am a firm believe that as soon as you are able to teach your child about his or her medications, the better advocate your child will be.

As far as living with a teen CHDer, and Jarod is only 14 1/2 so we are learning as we go, I really don’t see a lot of difference between my boys.  Jarod moans and groans about chores, doesn’t like homework and watches too much TV.  He talks back and rolls his eyes too!  From the beginning, as difficult a road as we have traveled, we have tried our best to maintain a normal family life.  I took off from work 3 months after Jarod was born and have remained full time throughout the years, taking time off with each surgery and hospitalization.  Jarod goes to regular school, is an active Boy Scout and loves to ride the Harley.  He is living and loving his life but does on occasion ask “why me?” or “why doesn’t so and so have a heart problem”?.   I have always responded to Jarod that he was made with a very special heart and because of his special heart, he is my brave and strong hero.  Here lately, as we prepare for a big surgery this summer at CHOP, he is becoming very anxious and full of questions.  We have always been as open and honest as we can when it comes to Jarod’s health and heart issues. It has been over 12 years since his last open heart surgery so he doesn’t remember them at all.  This time, we are headed into uncharted territory with a risky aortic valve/root replacement.  The main questions he asks are always “will I be asleep” and “will you be there with me?”.   Of course, these are easy questions and every time we answer, he is content knowing we will be there with him.  There was a particular evening, not very long ago, that he was talking about life in general.  We were sitting at the dinner table and he said “it’s okay, when it’s my time to go, it’s my time to go”.  Obviously, this was something I was not ready to hear and as tears streamed down my face, I had to excuse myself so I could regain my composure.  While I remember the early years being hard, the fear and anxiety, the surgeries and the what if’s, the teen years have been so much easier with the exception of the questions and serious discussions.  We have always lived this journey with a day by day mentality and are doing well.  We allow Jarod to do what he is comfortable with and he lets us know when he is tired or not feeling well.   

I hope I have been able to offer a little bit of insight and a lot more hope for you and your warriors.  Our journey is an open book and I am willing to answer any questions you might have.  I am here on Facebook to offer support but am usually sitting back, offering support where I can.  A lot of times I forget that when things are going good, I should share so that the good times help another parent feel that the hope is there.  Thank you for taking the time to learn a little about our heart hero’s journey with HLHS <3 

CHD week: The Ultimate Gift

We were warned when I was carrying Evan that eventually he may need a heart transplant.

We hope that is a road we don’t have to travel down and that Evan’s heart gives him the chance to get to adulthood.

It was clear…a transplant doesn’t “cure” anything.  It’s just changing one disease for another.

It’s the difficult choice you make as a parent…praying to God for the perfect heart for your little one…while praying to God to comfort the family that is going to give YOUR child the ultimate gift…the gift of life.

Meet Dawn – Heart Mama to Adelaide 

Visit Adelaid’s blog at : http://nearadelaide.blogspot.com/

Adelaide wearing her Heart Hero cape!

Today, I saw a YouTube video that someone posted on Facebook… it was a video of a nurse giving a newborn baby a bath.  It was a very sweet video, and though it initially made me smile, I soon found myself tearing up as I watched the cuddly, sleepy, tiny newborn baby be cradled and bathed… because I missed out on so much of the joyful newborn baby experience.  I don’t often feel sorry for myself, but today I did – as I watched that video of that newborn baby, I felt sorry for the fact that I didn’t get that experience.  I didn’t give Adelaide a bath until she was almost three months old, because she spent the first eleven weeks of her life at Childrens Memorial Hospital.

Adelaide was born with a condition called hypoplastic left heart syndrome – HLHS.  We were unaware of her diagnosis before she was born, and we spent the first few days of her life in what I can now call (in hindsight) blissful naivety.  Looking back, I am so glad we had those days of cuddles and snuggles and happy family time, because they helped keep me going in the months that were about to come.  Adelaide passed two APGAR tests with flying colors, was pink and happy, and breastfed like a champ – we had no idea anything was wrong.  It wasn’t until she was three days old that her condition was discovered.

The last night before we were supposed to take Adelaide home, I had a sharp pain in my right leg.  Because I had a C-section, the doctor ordered an ultrasound of my leg to make sure everything was okay.  We sent Adelaide to the nursery so my husband could come to the ultrasound with me; this was the first time she had been away from us aside from the daily weight checks, as she had roomed in with us for our whole hospital stay.

Thankfully, my leg was fine.  However, while Adelaide was in the nursery, she turned grayish and started breathing rapidly.  One of the nurses noticed this and called the on-call doctor, who happened to be a cardiologist with the local childrens’ hospital.  He quickly ordered an echocardiogram, which showed that Adelaide had HLHS.  Basically, the left side of her heart was very underdeveloped and non-functional.  (It’s at this point that I have to say, it was nothing short of divine intervention that things unfolded as they did – I was given that pain in my leg for a reason… to make sure that Adelaide was under the constant watchful eyes of the nurses in the nursery, instead of in our dark hospital room as we slept, so that the nurse would notice her change in color – call the doctor – order the test – discover her life-threatening condition.  I don’t want to even think about what would have happened had she not been exactly where she was at the exact moment she needed to be.)

Another cardiologist came into my room around 3:30 that morning to explain Adelaide’s condition to us.  She let us know that within her first years of life, Adelaide would require three corrective surgeries to reroute the “plumbing” of her heart.  One would need to take place within the next several days, one around 4-5 months of age, and one around 2-3 years old.  Adelaide was going to be transported directly to Childrens Memorial Hospital in Chicago.  Before she was taken there, my husband and I were able to visit her in the NICU.  We were relieved to see that aside from some IV lines and bandages, she still looked like our daughter, and she didn’t seem to be in any pain.


Later that day, I was discharged from the hospital and my husband and I went to see Adelaide at Childrens – the place that would become our home away from home over the next three months.  

The first few weeks of Adelaide’s hospital stay were a busy blur of nurses, doctors, medicines, infections, and medical terminology I never thought I’d have to learn.  She was originally scheduled to have the first corrective surgery (The Norwood Procedure) at 8 days old, but it was postponed due to an infection in her bloodstream.  Thankfully, the infection cleared quickly and she had her Norwood at 13 days old.  The day of Adelaide’s surgery, our friends and family waited with us in the waiting room – anxiously awaiting updates from the APN as the surgery progressed.  After almost eight hours, Adelaide’s surgery was complete.  Things had gone as well as possible, and her heart had started beating on its own after she came off of the bypass machine – which was a very good sign.

After a week or so of incredibly fast recovery, unfortunately, Adelaide took a few steps backward.  Though she had been successfully extubated a few days after The Norwood, she started having trouble breathing on her own and ultimately had to have the breathing tube put back in to help her breathe and take some of her body’s workload away from her heart.  (If her body was struggling too much to breathe, it put more strain on her heart, which is bad in general – but especially bad for a baby who only has half of a heart and is recovering from open-heart surgery.)  Her heart function had also decreased and was not improving despite the breathing tube’s assistance to help her body recover.

It was at this point that we first met with the heart failure/heart transplant team.  I can’t remember exactly how or when the conversation we had with them changed from, “We just want to introduce ourselves to you in case we need to start talking about transplant as an option,” to, “We need to seriously talk about the process of listing your daughter for a heart transplant,” but… that happened, and one month exactly after Adelaide had her first open-heart surgery, we listed her for a heart transplant.

Adelaide was listed as status 1A – the most critical status, because she was on a ventilator and required a constant drip of IV drugs to help her heart function.  After we listed her, my husband and I struggled to deal with the fact that the thing we were most anxiously waiting for – a new heart for our daughter – meant that another family was about to experience the greatest tragedy and our worst nightmare.  It didn’t seem fair, but it was our reality.

Five short days after listing Adelaide for transplant, we got “the call.”  They had found a suitable donor heart for Adelaide and though they still had to go physically see the heart to confirm, all preliminary tests were very promising.  “The call” came the day after we had had a prayer service at our church, where friends and family came together to pray for a miracle.  “The call” also came on the eve of my husband’s and my fourth wedding anniversary.  We were overwhelmed, scared, excited, nervous, and overall – hopeful.  The signs were all favorable – our miracle was happening.

Adelaide went into her transplant surgery just after midnight on our wedding anniversary.  She was seven weeks old.  Around 7:00 that morning, the surgeon came out to tell us her surgery had gone very well, and that her new heart had started beating on its own – again, a wonderful sign.  

Adelaide had a very smooth recovery from her transplant.  When she was extubated three days after her surgery, she gave us her first smile – and it was in that moment that we knew she was going to be okay.

That’s not to say that life post-transplant isn’t without risks, or possible complications.  Adelaide will be on anti-rejection medicines for the rest of her life, and those medicines carry a looooooong laundry list of possible negative side effects and complications.  If you really want to know what those are, you can Google them – I’m not going to go into it here.  Because of her anti-rejection medicines, Adelaide also has basically no immune system, so she’s much more susceptible to infection and disease than your average person.  So yes, there are a lot of things that could pop up down the road and that may challenge us.

But, as her transplant team so often says (and we love them for having this mantra) – they didn’t save her life to not let her live.  They encourage us to help her have as normal a life as possible.  While we use caution and try not to take her out in public more than is necessary during the height of flu season, we still go out to dinner as a family now and then, and she loves to go to Target with us.  When she’s a bit older, we will take her to kid-friendly activities at the library, or at the park-district – and from the way she moves, I think she’ll love taking dance lessons when she’s ready!  And, we fully intend to send her to preschool, kindergarten, middle school, high school… college.  We will always be cautious, but we will never hold her back.  Her life was saved so she can LIVE.

Adelaide is now almost 17 months old and is thriving.  Though she did have some slight physical and feeding delays due to spending the first three months of her life in the hospital, she quickly caught up and hit most of her baby milestones right on time.  She is currently a spunky, energetic, sweet, loving, talkative, smiley, adorable little girl who loves to dance, adores penguins, and gives the cutest little kisses.

I am so thankful to have this opportunity to share Adelaide’s story with those of you reading this, and I hope you have enjoyed learning about our little heart warrior.  I also hope that her story and journey can help spread even a little awareness about congenital heart defects and the impact they have on children’s and families’ lives every day.  Thank you for taking the time to read Adelaide’s story!

Norwood: Adelaide the day before her Norwood procedure, and Adelaide one year after her Norwood.

 

Adelaide on her first Heart-a-Versary in October 2012

 

Happy 1 year birthday!