Still Standing

In October of 2013, we took Alexandra on what we call her “Ivy League” tour, having a liver transplant evaluation at Yale in Connecticut and receiving a second cardiothoracic opinion on her by Harvard, or Boston Children’s. We then in April of 2014 did a combined heart/liver transplant evaluation at Boston Children’s, and in October of 2014 did a combined evaluation at Stanford Lucille Packard – all three ended in denials. This is the story of why we are still standing.

I remember the look on Dr. Miloh’s face implicitly. His lips were drawn, pursed almost. He looked like someone had stabbed him in the heart right in front of us. “She needs a liver transplant, the only way to help her is to get her a new liver.” My heart sank. I always thought maybe she’d need a heart one day, but never did I think another organ. I also didn’t think that any of this would have taken us on the journey we have been on. Never in my wildest dreams (or nightmares) could I have imagined the lengths we have gone to in search of an answer. In search of someone, anyone who would help her.
  

I seem to have all these memories of doctors faces over the years. Like frozen moments in time. The face I like to call her local cardiologists “oh s**t face”; Dr. Miloh’s face every time her ammonia was over 100; the look on Dr. Hellenbrand’s face (the chief of cardiology at Yale) when he told me there was no way she could have a stand-alone liver transplant; the look on her Boston cardiologists face when he had to tell us her heart wasn’t in the same shape we were being told it was at home. I will live with these doctors' faces etched in my memory forever.

I remember the last denial for transplant clearly. Dr. Kaufmann called me and discussed why Alexandra would not be a candidate for transplant. I didn’t say much other than “Okay, I understand.” What can you say? “She’s only got 1 SVC; her body is just not compatible with a four chambered heart.” What else could I say?

Three no’s from the most prestigious centers who see the most complex kids in the country, and the world. No one will touch her.

I felt raw. Alone. I had a breakdown. I alienated people who I felt were some of my closest friends because I couldn’t figure out why. I couldn’t figure out where to go or what to do. I fell apart.

Three no’s. I essentially spent the rest of 2014 and the first part of 2015 in a rage. I was so angry. The world, the entire world was against me and my child. I was lost in a storm of tears, and heartbroken from the fact that there would be no saving my first born gal.

But let me tell you where we are today – we are good. We are great. We are learning to live in the reality of this life. We are truly living.  No one will give her a transplant, and that’s okay. She told me herself that if I forced the transplant on her she would die. I think our kiddos know so much more then we think they do.

I’m going to bury my daughter. I say it all the time. People look at me like I’m crazy. I think it’s the least crazy thing I have ever said. I laugh wholeheartedly, and I wear everything out for the world to see. Yes, someday I will stand above her grave and my arms will be empty from the loss. But that day is not today.

What we have now is true freedom. I live in reality. I bet you’re thinking I’ve given up hope, but I am far from it. I have so much hope. Because I put the power and control into places that they needed to be in. I learned to be vulnerable, and not to be afraid of the pain or the craziness inside. We’ve been given a life. A life full of pain and heartbreak. Filled with open heart surgeries, and drugs, and therapies, and tons of crap to fill up every single day with no minute to spare. But it’s glorious. It’s messy. I’m messy – she is messy, and beautiful.

It’s a life. And even with three no’s to transplant, a left lung that’s just doing nothing but recycling unoxygenated blood, and worsening scoliosis – we live.  We live because it’s what we have to do. Yes, it took me to have a breakdown, to feel like I was coming apart at the seams (and sometimes I still feel that way), to question every move I’ve made since the day she was born – but I realized that this was our life. It isn’t going to be pretty, not everyone is going to want to be my friend, and sometimes I’m going to look horrendous – but it will be glorious.

Live gloriously. Live vulnerably. Just live. And if that fear of standing over your child’s grave hits you from time to time, remember today. Remember what’s right in front of you.

Remember and live gloriously. 

Danielle Edges (Mom to heterotaxy hero, Alexandra)

We Chose CHD!

Instead of Congenital Heart Disease (CHD) choosing us, I guess you can say that we chose CHD!  A common question I got asked by doctors who met Lily Grace for the first time was, "Did you know how complex her heart was when you adopted her?"  Imagine their shock when I tell them that we chose Lily specifically based upon how gloom and doom her medical prognosis was.  The very thing that caused other adoptive families to run away in fear had our family racing to bring our sweet girl home.

Lily was born in China with Hypoplastic Left Heart Syndrome (HLHS).  Simply put, Lily was born with only half of a heart.  She also has Heterotaxy, which means that all of her organs are on the wrong side of her body.  Lily’s heart is further complicated by being upside down, backward, and in the right hand side of her chest.  She had two open-heart surgeries in China, and then the surgeons deemed her “terminal” and inoperable.  At that point her orphanage had her adoption paperwork completed.  We came to find out on Gotcha Day, the day we met Lily for the very first time, that her orphanage truly believed a family would never ‘chose’ her. 

Children with HLHS typically have a series of three palliative open-heart surgeries.  It wasn’t until Lily was home from China, and she had her third open heart surgery that we understood why the Chinese surgeons deemed her inoperable.  The very first surgery that Lily ever had was to insert a shunt into her heart to increase pulmonary blood flow.  A textbook BT Shunt is placed on the right hand side of the heart, near the Superior Vena Cava.  Lily’s Superior Vena Cava is located on the left hand side of her heart due to Heterotaxy.  Unfortunately, the surgeon placed Lily’s BT Shunt on the right hand side of her heart, far away from her Superior Vena Cava.  This mistake began a series of critical issues for Lily Grace.  Shortly after her third open heart surgery, called the Fontan Procedure, Lily’s right pulmonary artery (RPA) became completely discontinuous from her heart.  In 2013 Lily’s surgeon attempted to reattach her RPA to her heart with a donor adult femoral vein, but unfortunately that surgery failed.  Lily is currently living with only one functioning lung. 

The US cardiologist who reviewed Lily's medical file while she was still in China was worried that he may have given us false hope about Lily's congenital heart defect.  The truth of the matter is that before we even knew if Lily was operable we submitted a letter of intent, asking China if we could please make her our daughter.  Lily needed heart surgery that China would not (could not) do.  But, what Lily really needed more than surgery was the love of a family.  Prior to bringing Lily home from China Jacques and I always said that we wanted desperately to show Lily the love of a family for as long as God allows her little heart to beat.  And here we are 3 ½ years “home” and Lily does KNOW the love of a family, and she knows about Jesus and how much He loves her too!!!! 

A small percentage of children who have had the Fontan Procedure end up with a life threatening condition called Protein Losing Enteropathy (PLE).  PLE caused horrible fluid retention in Lily’s lungs and abdomen.  Lily was essentially drowning to death in her own secretions.  Try as they might, Lily’s cardiac team was unable to control her PLE symptoms with medication.  We were told that the only cure for PLE is a heart transplant.  Lily was evaluated for a heart transplant, which is when we received the devastating news that due to her complex anatomy and the fact that she only had one lung attached to her heart, Lily was denied transplant listing by three transplant centers.  Shortly after we had conversations with the hospital’s hospice team about bringing Lily home to die, her heart failure cardiologist walked into the room and asked me if she could please present Lily one more time to their transplant committee.  Twenty-four hours later Lily was listed as a 1A, top priority, for a new heart.  On June 14^th, 2014 Lily Grace received the most precious gift that could ever be given - her Hero’s heart.  Lily’s donor was a non-lung donor due to the trauma he suffered in the motor vehicle accident.  What this meant for Lily was that she also received her donor’s left pulmonary artery.  Her surgical team was able to put Lily’s new heart in its correct anatomical position in her body even though all of Lily’s connections are upside down and backwards from her heterotaxy.  Lily’s new heart is only attached to her single left lung since her right lung had previously become discontinuous from her heart due to a blood clot, and the risk of Lily being on bypass longer was not an option.   Lily Grace is said to be the most complicated pediatric heart transplant to ever be done in the USA.  A huge miracle indeed!  

After a very difficult recovery, and 508 days on supplemental oxygen, Lily Grace is absolutely thriving!  She is filled with life, extreme love, and bountiful HOPE.  It has been an honor to be Lily’s mommy.  I have learned through this adoption that even more than Lily needing a family, I needed Lily.  She has taught me more about life and love in the 3 ½ years that I have known her than I learned in the prior 34 years combined.  Loving Lily is an excruciating love.  Selfishly, Jacques and I want more than anything for Lily to grow old and outlive us.  But whether she does that or not, if I am still alive to see her take her last breath, I will know without a shadow of a doubt that adopting Lily was exactly what God meant when he told us to go and be LOVE. 

Love is risky.

But let me tell you that it is SO worth it.  

A million times over.  

Love is worth the risk.

31 Years Ago...

Cameron Edward Price 3/21/85 - 6/25/85
31 years now. Seems time has flown by. Cameron was born with Dextrocardia and RAI. He had multiple heart defects and holes between the tissue dividing both sides of his heart. It caused problems with the blood returning from the lungs to the heart. He was to undergo 3 major surgeries where the surgeons would reconfigure his heart and circulatory system. He made it through the first one, but got an infection that he just couldn't get rid of and had a hard time fighting off.
31 years ago, not much was known about heterotaxy and NO support groups even existed. Trust me when I say, I was TOTALLY alone in this fight for my son and unbearable grief. I blamed myself, my husband and I even blamed God. I screamed, yelled, begged, pleaded, but NOTHING prepares you for the grief that comes upon you.

Over the years I have explained what heterotaxy is and how it has affected my entire life, but people who have never had a really sick child, yet alone a child that died, have no idea what it's like. I've learned to live with the heartache, but I have a tiny piece of my heart named Cameron that will never heal. I'll always wonder "what could have been," but at least I got to have "what was" for a short, brief time. And for that, I'll be eternally grateful.

Juliann Price ~ Mom to Heterotaxy Hero, Cameron

Cameron has 4 siblings: Daymun (29), RiAnn (27), Jade (26), and Jena (25)
Juliann is also grandmother to Aidyn (3 1/2) & Travis (3)