Bump in the Road

It wasn't a surprise. I was the one pushing for tests. We knew something wasn't quite right. Yet, when the call came, I felt unprepared. I guess even though I knew Levi's absent pulmonary valve was beginning to cause issues, to have it confirmed, well, let's just say to hear it is a lot different than knowing it. In July, our little guy was out playing baseball. He came in complaining of chest pain, shortness of breath, and palpitations. It was 104 out so, after getting some water and resting he was fine. I checked his pulse and oxygen levels and he was good. We called the cardiologist and they ordered a heart monitor to be worn for 30 days. We made it 11 days when Levi broke out in a terrible allergic reaction to the chest pads. Even in 11 days no unusual heart activity was seen. He never experienced these symptoms again, however, he began inhaling quick deep breaths that sometimes would end in a cough. And while he continued to play hard I could tell he was holding back. It just wasn't Levi.

It's been forever since I've written a blog post, so for anyone new, Levi was born with several heart defects, one being an absent pulmonary valve. In 2014, he had a bi-ventricle repair but it was decided to hold off on the valve since he was functioning okay without it. The hope was that he would make it to 15 or so so he could have an adult valve placed which would allow fewer surgeries down the road.

In May, he was cleared as stable with no significant change. They suggested a baseline sedated MRI to help them have an idea when a valve would need to be placed. There was no rush. Just whenever his next surgery would be. In September, during his cleft lip and palate evaluation, we were told he was ready for his bone graft. We were thrilled because the MRI could be performed. God's timing is always perfect. What was to be a baseline test originally would become diagnostic test for us. It couldn't come soon enough for me as my gut told be his heart was struggling and no longer able to compensate for the absent pulmonary valve.

A week before surgery our insurance decided the MRI was not needed. They said he was stable in May and the ultrasound was inconclusive. I am so thankful for Dr. Daniels and Jenne Hicks who listened to my concerns. Jenne spent endless hours fighting for this MRI, but the day before surgery we still didn't have approval so the MRI was cancelled. I felt sick as I knew he needed this test to confirm my suspicions. The day after surgery Jenne came in and said it had finally been approved. While ecstatic to have the approval, Levi would not be sedated. His IV was still in so we decided to try it awake. Levi held still like a champ for an hour and a half.

The call came Thursday. Jenne said my instinct was spot on. Levi's heart needs a pulmonary valve placed. The right side of his heart is very enlarged and working too hard. He will need his valve placed within the next 6 months. The sooner the better.

This comes with many decisions we would like prayer for. With Levi's bi-ventricle repair we ended up in Boston for three weeks. Our plan has always been to go back there when the valve placement needed done. There is talk he may be a candidate for a transcatheter valve placement. This could probably be done locally. Some of the best heart cath doctors are at Nationwide. However, if there are complications we would feel better at Boston since his surgeon is there.

1. Pray that God opens and closes the doors so Levi is where he needs to be and with the doctor he needs to have.

2. Pray for Levi, who wasn't happy to hear about another surgery, 1 week post surgery. (Although he always amazes me and was back to his happy self 5 minutes later.

3. Pray he can have the valve placed through a catheter versus a 4th open heart surgery.

Boston Update

Yesterday was a long day. Levi had his MRI and heart cath. They also ordered a bronchoscope due to his tracheal repair. They wanted to make sure they used the right size breathing tube and cuff so damage would not be done to the trachea. The MRI took 2.5 hours and the cath 1.5 hours. The doctor was well pleased with both tests. Everything they saw was expected except his common valve leak is more severe than they thought and he developed some arrthymias. That threw them off a bit as they developed before the cath was actually begun. So he was admitted to monitor that over night. He seems to be doing fine now but there is some concern that they will return after surgery. The pulmonary artery is not going to be able to be repaired without surgical intervention, so the surgeon will be cutting that section of narrowness to open it up. There are a couple of different options after this is opened and we are unsure what route our surgeon will take at this point. Even with these three issues the doctors are extremely pleased and he has gone from being a "good" candidate to an "excellent" candidate for a BV repair. This is awesome news. <br><br>

So, our major prayer requests are:
 1. That the AV node in Levi's heart would remain undisturbed and not be damaged during surgery. This is the electrical center of the heart and controls the rhythm/beats of the heart.
2. That Levi would not need a pacemaker. That there would be no more instances if arrhythmias.
 3. That the doctors would have wisdom in dividing the common valve and in stopping it's leak.
 4. Wisdom for doctors as they determine the best route to go in opening up the pulmonary artery.
 5. For the two large holes to be patched successfully.
 6. Peace for Levi. He is doing great and everyone can not believe how well he does with a everything. Never complains, always smiling, and always cooperative. He knows this needs to be done. He wants to be able to keep up with the other 5 year old boys. He wants to make a goal this year in soccer. He doesn't want to feel tired after running a few feet. Last night while tucking him in he said he was scared, "just a little bit." Honestly, he is the bravest person I know, but pray he would feel Christ's peace and presence surround him during surgery. 7. For no additional surprises once inside the heart and a smooth and speedy recovery.

Surgery has been moved to Thursday due to an emergency case that came in yesterday. There are so many families here in need. So much hope and fear in their eyes. I am thankful that Christ is our hope. That He has conquered fear and that we can have peace. I know that whatever happens, God's got this. I pray for each family here. That they may find His gift of peace. That their hope will be placed in Him.

"For I am the Lord, your God, who takes hold of your right hand and says to you, Do not fear; I will help you." (Isaiah 41:13)

Boston

Plane tickets bought. Yawkey House reserved. Hotel reservations made. Everything is in place. Now if my heart and mind would settle. We leave for Boston in 14 days. Levi will have several tests done, a heart cath, and then open heart surgery. It will be his third one. We are praying it will be his last one. That a repair can be made. Levi is always so brave. He never asks questions or complains about his surgeries. He's had 8 in the 22 months he has been home. But heart surgery is hard. It's scary. Last week he started saying he is scared. I don't blame him. Please pray for peace for him. For the doctors to have wisdom and discernment. For a biventricular repair to be possible. For an uneventful and speedy recovery. For siblings left behind and siblings that are coming, but unable to be with me at the hospital. For peace and wisdom for Mark and I.

"Do not be afraid or discouraged, for the Lord will personally go ahead of you. He will be with you; He will neither fail you nor abandon you." (Deuteronomy 31:8)

"Do not be anxious about anything, but in every situation, by prayer and petition, with thanksgiving, present your requests to God. And the peace of God, which transcends all understanding, will guard your hearts and your minds in Christ Jesus." (Philippians 4:6-7)

Levi's First Family Day!

One year ago today, Levi became our son! I can not believe a year has passed. The time has flown.  It seems as if he has always been here.  He has blossomed so much.  He laughs, plays, teases, hugs, gets mad, and just acts so much freer now. He comes to us for help, for comfort, to cuddle.  It's not that he didn't do these things before its just the way he does it. He is comfortable.  He is at peace.  He knows we are his family and he is happy.  He has settled in! He is so much more energetic too! Now that he is breathing better and his oxygen is up this little guy is pink and on the go! He does wear out faster than his brothers and sisters, but he generally doesn't let it stop him!

It has been amazing to watch the bond form between he and Samuel.  They are finally to the point of enjoying one another as playmates.  Oh the giggling they do and the trouble they find! It is so fun to hear one of them say to the other, "you are my best friend!" or "I love you."  Of course I hear plenty of, "you are NOT my best friend anymore! Samuel has even announced once or twice, "Take Levi back to China!" But this does not last long. Within seconds they are playing and saying they are the best of friends. I love to see Samuel running off and realizing Levi is lagging behind.  He always stops, comes back, puts his arm around Levi and says, "Come on, Levi." He is becoming quite the protector.  Levi finds Samuel hilarious and looks up to his brother.  He copies everything Samuel does....not always a wise choice! ;)

I can not even imagine our family without Levi. He is pure joy. He is so incredibly brave. He never gives up and is so full of life. We were not sure what to expect with his complex heart defect. Still don't really. But I am so glad our fears did not stop us from adopting our son. God has blessed us greatly!

On September 10th,  we spoke with Dr. del Nido from Boston Children's.  He was wonderful.  Very informative and patient with our many questions.  He stated the records he received from Nationwide Children's were very good and thorough, but he still feels they can repair is heart.  He emphasized that their speciality is kids with hetertaxia (irregular placement of organs), and while Levi is a little more complex having transposition of the great arteries, heterotaxia, Complete Atrioventricular Canal defect, DORV, and pulmonary stenosis, he has repaired this before.  So, he wants us to come to Boston so he can perform his own study to determine if we can indeed move forward with a full repair.  We are beyond excited but trying to keep in check as this is what Nationwide felt too until they were actually inside Levi's chest.  But we are filled with hope and if he gets in there and says it can't be done then at least we will know we looked at all the possibilities! He did say this was a higher risk surgery short term compared to the Fontan.   However, for Levi, the long term is more stable.  

Due to Levi's upcoming cleft surgery we have put off Boston until late spring/early summer.  His oxygen saturations are holding good right now and he is thriving, so they felt it best to stay on schedule with the cleft repair.

We are still waiting to hear what they want to do with his intestinal malrotation.  We are praying they will just leave it alone since he has no symptoms and it seems to be working well for him!  

He also had his liver/spleen scan done.  As expected there is no spleen.  There is not even any splenic tissue.  His liver looks good, although is is a little more centered than normal, but that is no biggie.

It has been a wonderful first year with Levi and we are so thankful that he is our son!

Boston News

Our second opinion is in! Boston believes they can, indeed, repair Levi's heart! I have to be honest, we were a bit stunned. We sent for a second opinion in hopes of this, but  I guess deep down we were expecting confirmation of the first diagnosis.  So now we are praying, considering, praying, researching, praying, and deciding what is best for our son.  Do you choose the more safe, stable palliative surgery that has more than likely long term complications.  Or do you go for the higher risk repair, that would have few complications long term but would require other open heart surgeries down the road to replace outgrown parts? It's hard having two doctors that you like having such different opinions.  So, on September 10,  we have a phone consult with Boston Children's to ask our many questions so we can make a knowledgable decision.  Please be praying we make the right decision for our son.

On top of that Levi finally had his upper GI to determine if his intestines are positioned correctly.   They are not. :(   In fact, many things are not positioned correctly.  His stomach is on the right side, appendix on left, and intestines on left.  All this is opposite.  Oh, and his liver is midline.  Levi is definitely uniquely made!  The good news is there is no obstructions and everything is working good.  We will be talking with a GI specialist as this may need to be corrected to prevent the intestines from twisting up.  There is a good chance if he does need surgery they will remove the appendix as a preventative measure since it is not located on the right side.  We are hoping if this all needs down they can just tie it into on of his cleft lip surgeries.  What would be best is if nothing needs done! We will know for sure after we speak with the GI specialist.   

Levi celebrated his 4th birthday on the 19th!  It was so fun to watch him.  He loved everything. The balloons. The decorations. The zoo. We wanted everything elephants!  I made him an elephant cake and we are all so proud as he was able to blow out each of his candles, thanks to his tracheal repair! So proud of him! He was proud of himself!  He loved his presents and kept making sure they were his, and not Samuel's.  Haha.  So glad we were able to celebrate this birthday with him as a family!  Levi has not gained much weight since home, maybe 1.5 pounds, but he has grown 3 inches! His color is great and he is much more active know that he is breathing better. He loves his siblings and it is so fun to watch him watch them! So fun to watch them bond and become best friends.  This time last year we were waiting for LOA and TA.  It seems like forever ago.  Levi seems like he has always been here.  Can't imagine our family without him! 

Soccer season is in full swing and Levi and Samuel are playing  for the first time.  They are so cute to watch. Levi is doing much better than I expected and is quite determine to kick that ball in the goal! 

Samuel learning to dribble!

Levi learning to dribble!

Samuel makes a goal!

Levi makes a goal!

Oh, and one more thing.  As if our house is not crazy enough, we got a puppy! His name is sneakers and he may just be the best dog ever! Levi loves him and so does everyone else!

Meg and Sneakers

Tough Choices

We are between a rock and a hard place. It seems like any decision we make has pros and cons. It's frustrating to feel like your settling, when in reality you may not be settling, but you just don't know so that's what it feels like.   To make a decision that effects your son's life, in a life and death sort of way.  We are praying, but peace is not coming with either decision.
 
Many people have asked what exactly is wrong with Levi's heart.  Levi's heart diagnosis' are Double Outlet Right Ventricle (DORV), Complete Atrioventricular Canal Defect (CAVC), and Pulmonary Stenosis (PS).  
 
With DORV, the Aorta and the Pulmonary Artery are both attached to the right ventricle. This is in contrast to the normal heart, where the Aorta attaches to the left ventricle and the Pulmonary Artery attaches to the right ventricle. Blood from the left ventricle, which would normally leave the heart through the attached aorta, now must cross an abnormal hole in the wall dividing the right and left ventricles, called a Ventricular Septal Defect (VSD), in order to leave the heart and supply the head and body with blood. In this condition, the normally separated oxygen-rich arterial blood and oxygen-poor venous blood is mixed in the right ventricle prior to leaving the heart. Also, the normally low pressure right ventricle can be subjected to increased pressure from the normally high pressure left ventricle.
 
The CAVC is a second hole Levi has.  This large hole is in the center of the heart affecting all four chambers where they would normally be divided. When a heart is properly divided, the oxygen-rich blood from the lungs does not mix with the oxygen-poor blood from the body. A CAVC allows blood to mix and the chambers and valves to not properly route the blood.
 
His PS is above and below the Pulmonary Valve so not much blood can get through.
 
When we adopted Levi we were told he would need the Fontan due to an underdeveloped left ventricle that was not functioning. We were at peace with this.  But then they found his left ventricle was developed and functioning fine. While it is smaller than the right side it is functioning.  Everyone was so happy because everything lined up for Levi to have a biventricular repair.
 
But when the surgeon got inside Levi's heart he felt a biventricular repair was impossible. 
 
  "Looking in, there was noted to be a complete atrioventricular canal, right dominant with a somewhat smaller left ventricular cavity.  However, the important feature was that the ventricular septum inserted more rightward and anterior to the aorta such that any attempt to close the VSD would bring the baffle across the left ventricular outflow tract, nearly obliterating it.  It was clear that there was no way to do a 2 ventricle repair and wind up with an inadequate left ventricular outflow tract.  Therefore, the decision was made to abandon the possibility of a 2 ventricle repair and proceed with a pulsatile bidirectional Glenn." (quote from surgeon's medical report) 
 
He said his Fontan would be very good because even though his heart would work as a single ventricle, his left side is there to help the right.  This is usually not the case with the Fontan. Usually the left ventricle is not functioning and the right ventricle works alone.  With both sides working together the Fontan may last longer.  He may not ever require a heart transplant.  We just don't know.  
 
We have been told by some to get a second opinion from a doctor in Boston who specializes in biventricular repairs. We did contact him and he was more than willing to review Levi's files.  But after reading the surgeon's report we are not sure the outcome would change and we don't want to put Levi through more tests and surgeries "incase" he could have a biventricular heart.  Or what if a biventricular heart would not be as safe in the long run because of his complex defect. Sometimes a biventricular repair can be done but will require more open heart surgeries down the road to replace parts that your child out grows.  Ones heart and body can only handle that so many times.  The outcome would still be a transplant if the heart couldn't handle the surgeries.  So do you go for a Fontan that has it's own set of complications with the possibility of a transplant down the road or a biventricular  repair that could require open heart surgery every few years and still end with a heart transplant?  
 
I wish it were a cut and dry decision.  It is not.  We have decided to request a second opinion, hoping the doctors are of the same agreement as to what should be done.  We know God knows what is best for Levi.  We are glad He has gone before us. Please be praying we hear His voice. That we choose the path He has for Levi.  
 
Romans 15:13 "I pray that God, the source of hope, will fill you completely with joy and peace because you trust in him. Then you will overflow with confident hope through the power of the Holy Spirit."

Change of Plans

Sorry I neglected my blog yesterday! I gave updates, but between texting and Facebook that was all I had time for. 

Levi did great yesterday.  We got to the hospital at 6am and he gowned up and was ready to go.  They even prepared Dumbo and Josh to go to surgery, too! 

He went back to the OR about 8:10.  He was such a trooper with the hand off.   No tears and a kiss and wave goodbye.  He surgeon was expecting scar tissue under his original incision but it was way more than he anticipated.  It took from 9am, when the incision was made, until 11:20 to dissect  through all his scar tissue.  At 11:25, they placed him on the heart lung bypass and then the surgeon was going to inspect the heart. If all went well the av canal repair and TOF repair would begin at 1:00pm.  So, when the nurse came out at 1:26 and said they were done, we knew things had not gone as planned.

 The surgeon's inspection determined the above repairs were not possible.  Levi's aorta, and other great arteries are mal-rotated.  This did not show up on the 2 dimensional tests preformed previously.  The good news is his heart is strong. And his left ventricle, which was non functioning prior to January,  is functioning wonderfully! Praise God! 

While the surgery results were not what we thought,  we know God went before us and we know the results were no surprise to Him.  He made sure Levi would have a two ventricle heart, which for Levi, is very important long term. 

So, what did they do? They preformed the Glenn Procedure, which is the first step prior to the Fontan Procedure.  For those of you wondering, yes, the Fontan is generally for a single ventricle heart. However, they will be using this procedure on a two ventricle heart. Levi will continue to have his left ventricle pump the blood but the Fontan will stop the unoxygenated blood and the oxygenated blood from mixing.  With the Glenn they connected his superior vena cava to his pulmonary arteries.  His lungs are now supplying oxygenated blood to the heart. Until the Fontan is completed deoxygenated blood and oxygenated blood will continue to mix. So, he is still low on oxygen, 83-85% ( a little higher), and not completely pink. Oxygen will improve after the Fontan. When the Fontan is performed they will connect the inferior vena cava to the pulmonary artery as well. This will stop the mixing of blood. The heart will only supply blood to the body and the lungs will be supplied by the vena canvas. When will the Fontan be done? We are not sure.  They are thinking two years down the road but a lot will depend on how Levi does with the Glenn.

He was up in his room by 2:50 and woke shortly after we arrived.  He was mad because he wanted apple juice.  Generally, they like to wait 4 hours before giving liquids but he was crying so, which caused him to bleed out through his chest tube, they gave in a gave him diluted able juice.  He drank 8 ounces before he went to bed and another 8 through the night!

  He had a great night, but didn't sleep much.  He was alert and talkative and wanting to watch tv or play with his animals or firetruck.  He did have a couple of bouts with pain but the nurses have been wonderful and on top of that. He is itching like crazy as a result of a narcotic they gave him.  Nothing is touching that right now, but he is being a trooper.  He mostly is complaint about his IV's. He wants the out, now! When a nurse comes in he holds up his tiny hand and says, "out!" 

He was excited for breakfast but then changed his mind, but he ate a great lunch.  Due to his crying apple juice episode the doctors decided to give him some blood to boost him a bit before we transfer to the step down unit later this afternoon.  They  used the blood I donated  for him.  It was an awesome feeling seeing my blood become his.

Then at 11am the chest tube, arterial line, and cath were all removed! He is also off all IV fluids and meds and taking them oral. I am currently holding him and he promptly went to sleep! All is well!  At 1:30pm we moved to the step down unit. Next stop....home! Estimating a Saturday discharge. 

Psalm 111:1-4 "  Praise the Lord!  I will thank the Lord with all my heart as I meet with his godly people.  How amazing are the deeds of the Lord! All who delight in him should ponder them.  Everything he does reveals his glory and majesty.  His righteousness never fails.  He causes us to remember his wonderful works.  How gracious and merciful is our Lord!"

(I have pictures but will have to post those later as I can't do that through my iPad.)