Second Surgical Consult

Today Ryan, Melia and I met with our local surgeon to further discuss Melia's heart condition and the recommendations he had for surgery.  Previous to this appointment our local cardiologist had contacted three other physicians from around the country and gathered their opinions on whether or not Melia's condition required surgical intervention.  The vast majority agreed that corrective surgery is her best bet at delaying or preventing heart failure down the road.

With that in mind, I was still not convinced that surgery was the right answer.  We have a very active, joyful little girl and my thought was why fix something that isn't broken.  So, I prayed over and over that God would grant us wisdom to make wise decisions for our daughter and that he would help us know what to do.  I also prayed that we would be like-minded as we left our appointment today.

I am so thankful that God answered my prayers today.  Dr. Hammel, the pediatric cardiothoracic surgeon in Omaha explained to us that Melia very clearly needs intervention and suggested that we pursue getting started sooner rather than later.  He was confident, but not cocky.  He was calm, honest about risks and hopeful that Melia is getting the interventions she needs at an early enough age that it will make a difference for her life.

Here are the facts...
...people with this heart condition usually die from it.  They die from heart failure.  Sometimes that is early in life and other times it is later (like 40, 50 or 60) but almost all (if not all) will suffer from heart failure.
...a corrective surgery is the current, best option for patients.  It puts the workload of systemic pumping back on the left ventricle and therefore reduces the risk of right sided heart failure.
...at birth both ventricles have the same pressures, but shortly after birth the demands of the systemic pump create very different chambers.  In Melia's case the right side is getting a thicker muscle and the left side is weaker.  The early a corrective surgery is done the better chance that the left ventricle can actually take on the workload of being a systemic pump.
...in Melia's case her right side is already starting to fail.  It is a very, very mild failure, but a failure nonetheless.  Instead of pumping 2/3 of all the blood out with each pump it is only doing 1/2.  And, because she had lived this way for almost two years, the left side of her heart is very weak.
...to prepare her heart for a corrective surgery, called a Double Switch, she will have to undergo two PA Banding procedures.  The Banding of the Pulmonary Artery will make the left ventricle have to work harder and strengthen it so it is prepared to take over as the systemic pump after the Double Switch.
...The PA Banding surgery is not really a technically difficult surgery but Dr. Hammel does have three concerns:  1.  If the band is too tight the left ventricle could acutely fail (this is a life threatening situation)  2.  If the left ventricle decides not let itself be retrained it could fail down the road (teens or beyond, this would create a need for heart transplant)  3.  Not getting the PA Bands done fast enough and the RV failing before we get to the Double Switch.
...although that is scary information Dr. Hammel feels very good about her current health and believes that if we do two bands over the course of 2-3 years that Melia's heart will respond well.  If is does that a Double Switch surgery will be in her future in 3-5 years from now.
...Although it would have been better to have corrective surgery shortly after birth, the fact that she is only two is good.  Teens and certainly adults do not respond as well to banding and the double switch.

So, with all this information we have decided it is in Melia's best interest to move forward with a PA Banding in about a month.

Believe me I DON'T WANT TO DO THIS, but right now I am not scared.  God's faithfulness is steadfast and I wholeheartedly believe that He loves Melia more than I ever could.  He placed her in this family, at this time because He knew it would be best for her.  So, with confidence in God's love for us and her we willingly place our daughter's surgery, life and future into the loving hands of her Creator.

One of my friends encouraged me with words she received recently about her daughter.  She was wisely told, "God created your child FOR HIM."  So, whatever His plan is I have to submit.  She is not mine, she is His daughter and I just get the privilege of loving on her and being her mommy for whatever time He gives me.  I HOPE, HOPE, HOPE it is for a VERY LONG time, but His will, not mine be done.

So, with that information I would be so appreciative of your prayers for our family over the next few months.  Here are some specific prayer requests:
~ a successful surgery in May with no complications.  That she would not need a ventilator after surgery and that her left ventricle would not fail but respond to the banding well.  That she would not die from this surgery.
~that Melia would not be afraid and that she would not have pain (aside from what is good for her to limit her activity).
~that her siblings would not be afraid and that this would strengthen their faith in God, their prayer life and their love for each other.
~for the surgeons, nurses and anesthesiologists skill.  That their personal lives are peaceful and restful, so that they are not distracted or tired on the day of her surgery.

Thanks in advance for your prayers for Melia and our family!

Inspiring...

http://www.youtube.com/watch?feature=player_embedded&v=junwL-9SdeQ

First Surgical Consult

Today at 1:00 (it turned into 2:30...ugh) we had our first surgical consult regarding Melia's heart.  The main doctor that would do her surgery had a complication in surgery and was not able to talk with us. So his first assist talked with me about her condition, consensus opinions and gave us recommendations.  Here are the highlights of what he had to say:

~ National (if not International) Consensus is that her condition is best treated with a Full Repair.  For her that means a Pulmonary Artery (PA) Banding to strengthen her future systemic pumping ventricle (the left) followed by a Double Switch Surgery.  The Double Switch involves an Arterial Switch of the PA and Aorta and reattaching the coronary arteries and an Atrial Switch of Baffle.
~ The Arterial Switch has been done for about 30 years and the Atrial Switch a little longer.  Therefore, there is no evidence to prove that this procedure prolongs life past the 5th or 6th decade which seems to be the time when most with CCTGA have significant heart failure (presuming they have made it that long in the first place).  To put it simply, these surgeries have great short term outcomes, but the long term outcomes are theoretical at this point.
~They do know that the way the Right Ventricle pumps blood it often fails because it is exposed to high pressures if it remains the systemic pump.  In other words, the long term outlook is more favorable theoretically with surgery than the known outcomes without surgery.
~She does have some stress on her heart with more blood flow going to the lungs than the body (1.4:1 ratio), a VSD that is contributing to this disproportionate ratio and a very mild Tricuspid Regurgitation.  These are not problematic right now, but suggest that a PA Band would actually help these issues and prepare her heart for the bigger DS down the road.
~They predict a DS in 1-4 years.
~The PA Banding is a 2-3 hour surgery where they open up the chest and cut open the sternum to place a band with sutures around the Pulmonary Artery.  She would spend a night in ICU and then 1-2 nights in a regular hospital bed and then home if there are no complications.
~They would recommend this first surgery in 3-6 months.

So, our next step is to meet with the actual surgeon, ask a few more questions and then get opinions from two other physicians out of Michigan and Boston.  Once we have all that information we will make the decision as to whether or not we elect to have Melia have one or both surgeries in her future.

That is all the technical stuff, now for the real stuff.  I felt pretty overwhelmed after this meeting.  I had already lost my keys, I was late to pick the other kids up from school because the meeting was late, Melia missed her nap and was sad and mad, I had to call my sweet MIL for the zillionth time this week to help me out with the kids AND we brought all this on ourselves!  We chose this path.  We also chose to sell our house and buy a new one during all this.  So, I cried.

And, in those few tears God spoke in His still small voice.  He reminded me what He has taught me before.  Joy is NOT dependent on circumstance.  A few months ago I was feeling too settled and that made me uncomfortable.  Now, we are changing everything in our lives and I am uncomfortable.  When life is an emergency or I am frantic to figure out the future (as if I have any control over it at all) I lose joy.  But, when I GIVE THANKS for the here and now and am present in the moment I find joy!

So, fortunately, the tears shifted when I stopped to thank God for the little one sleeping in her car seat, the rain that brought out green grass in May, car keys that were found, a call with two house showings tomorrow...how quickly giving thanks can turn sad into glad.

As we gather information, if you think about it, would you mind praying for us.  Pray that we would have Holy Spirit wisdom to make the best choices for Melia.  Pray that she would not lose her joy through all of this medical stuff.  Pray that her brothers and sisters would be blessed through it all too.

Zac and Melia

Zac and Melia are becoming fast friends!

Upon arriving home with Melia, Zac was the least enthusiatic.  I think he knew he was losing his spot as baby of the family and also was having to share the attention of his parents.  For a few weeks we saw some behaviors that told us he needed affirmation that he was still loved, still special and still important to us.

Fortunately, after a few conversations about his new role as BIG brother his negative behaviors stopped and he and Melia are turning into great friends.  Zac is VERY expressive.  You always know exactly what he is feeling because he wears his heart and emotions on his sleeve.  When he loves, he loves passionately.  He is also very hands on and loves to have everyone's attention.  So, we have seen in him a growing affection for Melia.  He LOVES to touch her face and kiss her and hug and hold her.  He loves to make her laugh, and boy oh boy, does Melia laugh at him.  She thinks he is quite the comedian.  Zac will take her by the hand and gently lead her down steps or push her on the swing.  I love watching the way he protects her as well.

This brother and sister relationship is beautiful.  There have been a few times recently where Hallie and Jacob are off doing something on their own and I get to watch Zac and Melia interact as siblings.  I am so blessed when I think how God brought these two precious children from Russia and China together in Omaha, NE to be brother and sister and how he has made me their mommy.

If you have never considered adoption, just watch this video...

Isn't adoption amazing? 

I am so thankful that God blessed us with these children and is knitting their hearts together as Big Brother and Little Sister!

Getting A Better Picture

(getting ready for today's procedures)

Today was the day for Melia's Cardiac MRI and TEE at Children's Hospital so the doctors could get a better picture of her heart to make their final recommendations.

She started the day with a yummy "chicken broth in a bottle" breakfast and after 9:15 she started her fast.  I joined her in her fast inadvertently because our morning was a little chaotic (Ryan lost his car keys, Zac spilled a berry smoothie down his shirt before getting into the car, Hallie had a hair crisis and Jake was practicing spelling words).

Melia did remarkably well and was distracted fairly easily until Grammy picked us up and drove us to the hospital (remember Ryan's lost car keys).  Once we arrived we were greeted by the hospital dogs and a few friends that we happened to meet up with in the lobby.  Ryan arrived and we headed back to our room around 11:45.

At 12:30 we had confirmed her medical history and I had given consent for treatment.  She received a dose of "happy medicine" to help with the separation and she was wheeled away happily by 12:45.

The plan was that she would have the MRI then the TEE with her Cardiologist and finally she was to get her second round of immunizations while under sedation. Then she would return to us around 3:30ish.  I ran to get a bite to eat, Ryan went to get a new phone (he had dropped it and cracked the screen the night before) and then we waited.

Her Cardiologist came in to give us the report at little after 3:45.  He did not find anything surprising and suspects that after he reviews the MRI and discusses it with the group in Omaha and del Nido in Boston that they will continue to recommend a PA Banding surgery to prepare her heart for a double switch in the future.  We discussed who, where, types of surgical approaches and her condition.  Once again, we did not feel rushed while talking with him and appreciated his experienced opinion.  They will call next Monday after Grand Rounds with the final verdict.  If, indeed they do recommend surgery then we will also be discussing treatment plans with local and national surgeons to find the best place for her to have this intervention.  Her final diagnosis is:  Atrial Situs Inversus with D-looping of the heart and D transposition of the Great Vessels in Dextrocardia which is the equivalent of Ventricular Inversion with L Transposition of Congenitally Corrected Transposition with a small VSD and ASD.

We are grateful that there seems to be no hurry for us to make these decisions.  We are also thankful for anyone willing to pray for us that God would impart wisdom as we proceed.

Melia returned to me around 4:30, sobbing!  I quickly took her into my arms and they told me they had forgotten to give her the shots while she was under sedation so they decided to give them while she was in a strange recovery room with strange people all around her as she woke up from anesthesia!  I was quite upset.  My goal was to lessen her trauma and anxiety related to health care but I'm sure that experience will actually heighten her fear.  What was done was done, but I was not happy.  After about 15 minutes I was able to calm her down and comfort her.  She sat on my lap for about an hour and then it was time for us to leave.  Ryan picked us up with the big kids and we headed home.

She threw up once tonight, but then was dancing and giggling like nothing happened today.  I hope she rests well tonight.  I adore that precious child so very much and it pains me to see her experience any discomfort.  I think that is what is making the decision to proceed with surgery so difficult.  If I had an ill appearing child that was suffering, surgery would be a welcome intervention, but it is so hard to choose pain for my "healthy" child in hopes that it will prolong her life and give her a greater quality of life.  Because her diagnosis is so rare (less than .5% of all Congenital Heart Disease) the treatment plan is largely based on theoretical outcomes, not proven outcomes.  We know that without intervention she could live a "normal" life to the age of 40 or 50, but it is presumably more likely that she would suffer from some amount of heart failure before that time, maybe even in her youth.  Surgeons hope that a double switch done early in life will prevent failure, but they only got good at the surgery in the 90's, so there are not many (if any) 30-40 year olds that had the surgery and can show the world that it did improve quality and quantity of life.  The other problem is that once the heart starts to fail you can't really "get back" what you loose, so intervention before failure (ventricular or valvular) seems optimal.

These are the decisions we are faced with in the near future.  We are not feeling desperate or anxious, but we would really appreciate prayers for wise decision making, peace and positive, life-sustaining and life-enhancing outcomes for our daughter with whatever we decide to do.  One thing I know...God is good and just and loves her and loves us.  We can rest in that blessed assurance!

(A glimpse of our sweet little girl, babbling away!)

A Change of Plans

(Melia's First Echocardiogram)

Our Pediatric Cardiologist called today with his consensus opinion after discussing Melia's Congenital Heart Disease (CHD) with the Omaha Cardiologists, Cardiothoracic Surgeons and the nation's premier doctor in CHD/Double Switch operations out of Boston, MA.

I feel so strongly that God placed Melia in our home in Omaha so that she could get this kind of excellent, well-thought out, collaborative health care.  I am confident in our Cardiologists knowledge and thankful that he would go to such great lengths to get the best information regarding treatments and procedures that could enhance and prolong her life.

That being said, here is the newest recommendation (I get the feeling that dealing with CHD is like adoption...expect the unexpected and expect change).  To put it simply, Melia's heart is stable right now, but the wrong side of the heart is pumping blood to the body and might wear out in time because it was not designed to do this kind of hard work.  Our doctor recommends doing a couple more procedures so that surgeons will have the best looks at her heart before they would ever need to do surgery and then he recommends proceeding with a preparatory surgery to get her heart ready for any later, "big" surgeries that she might need.

For those reading that understand the medical terminology, here goes...

They are recommending a Cardiac MRI and TEE to complete her testing.  We will probably have all her records sent to Bove and Del Nido for second opinions and then proceed with a PA Banding surgery in Omaha, Boston or Michigan.  The PA Band will stregthen the LV muscle so that when and if a Double Switch is needed (they mentioned age 5) then the LV will be ready to pump blood systemically.  There may be a need for multiple PA Bands.  We just have to see how she responds to the banding.  They do open the chest for this surgery, but do not require use of the heart/lung bypass machine.  They would anticipate a three day hospital recovery.

So, although we have a change of plans the Unchanging, Creator God is with us.  Today, I am thankful for this good news...

~God, Immanuel (He is with us)
~God, Creator (He made Melia)
~God is Love (He works things out in pure love)
~Time.  Nothing is urgent.  We have time to build up Melia's heart.  She is not in heart failure.
~Communication.  Doctors all communicating with each other and me to make the best, wisest decisions.
~Access to Health Care.  All around the world children are suffering without access to good, safe health care but our little Melia has that right here in our own community.
~Family.  The support, love, wisdom and care of extended family.
~A Kiss.  Last night she was sitting on my lap, turned her head and puckered up for a kiss from Mommy.  Then she snuggled up in my chest with a smile.  Pure sweetness!

Jacob and Melia

It is a little harder for me to get pictures of just these two together.  Hallie and Zac are quick to hop in a picture and vie for Melia's affection and attention, but true to Jacob's peaceful and calm nature he will patiently wait for her to be unattended or in need to spend time with her.

I must admit I was nervous about this relationship.  I wondered if Melia and Jacob would have any reason or desire to have a special bond or friendship.  I am so thrilled that they have taken a liking to one another and it is a joy to watch these two together.

From the moment Melia entered our lives on Gotcha Day Jake, my observant boy, watched the way she responded and he gently introduced himself after all the rest of us had made our boisterous and excited introductions.

Jacob never forces himself on anyone but willingly gives love and affection if and when another person is ready to receive it.  I remember Jacob telling me that he hoped Melia would like him, and right from the beginning she did.  He held her in the van ride in Shijiazhuang and they became fast friends.  She was quick to give Jake a hug and his face just lit up with joy.  She liked him and he liked her!

So, in her first month home I will often see Jacob reaching out and waiting for Melia to offer him a hug.  Aside from this sweet expression of affection Jacob is also wonderful at watching out for Melia's safety.  He is quick to help her up and down steps and into her car seat.  He prides himself on fastening her belt in the car seat, but once again, never forces it.  The other kids like to help too, so Jake will only jump in if the others have not already decided they want to help.  Jacob loves to hold her on his lap (although she is getting a lot wigglier and less likely to sit still for long) and will even read to her if she stops to listen.

Ryan and I call them our, "numbers 2 and 4."  Jacob and Melia are our calm, sweetly disposed, thoughtful children and I trust that God will continue to use their similarities and differences to bind their hearts together as brother and sister.  It is a beautiful relationship in the making.