CHD Warrior - Liam

First let me say I adore this guys cheeks!   Liam is another CHD Warrior that Sugar Pea and I follow on a daily basis.  Sugar Pea talks about Liam to all her friends like he's just the little boy next door.

Liam was born February 18, 2011 to Whitney and Brody Lyon in Ft. Smith, Arkansas.  It wasn't known until after Liam was born that there was a problem. Thanks to the doctor's great skills, Liam was quickly diagnosed with Hypoplastic Left Heart Syndrome, which is fatal if left untreated.  He was transferred that evening by Life Flight to The Children's Hospital at St. Francis in Tulsa, Oklahoma. The doctors spent the next week trying to decide which would be the best route to take and decided that he needed a transplant instead of the 3 normal surgeries for HLHS (Norwood, Glen, Fontan).  He was then transferred to Arkansas Children's Hospital in Little Rock, Arkansas. 

The doctors there decided that he should have surgeries to try and make his heart work as long as possible.  He had the Norwood, but it wasn't working quite right and they had to operate again. It was becoming apparent that the surgery didn't go as they expected and he was then listed for a transplant.  

On July 25th they got the cal that there was a heart for Liam.  Somewhere a family chose to give other babies a chance to live during their time of grieving.  For the 5th time in 5 months he had another surgery, his long awaited transplant.  

Liam was able to go home on 11/22/11 for the first time in his little life!


Just after Christmas Liam's family got some difficult news.  I'm coping and pasting this from Liam's Facebook page because they can explain it better than I can.

Its the last day of 2011. We have had some difficult news this week - the kind of news that makes your head swim. Liam has had the fluid collecting in his belly - he has had two seperate episodes that required a "pigtail" to drain the fluid. In investigating this, they were doing another heart cath and decided to check "south" of his heart - when the did, they discovered that the inferior vena cava (the large vein that drains the lower part of the body and returns it to the heart). They knew there was some issue with this vein before now, because they used to gain access to his heart through his groin - and thread it up through the inferior cava. At some point, they couldn't get through there anymore and they had to gain access through his neck (and I gues the superior vena cava). The inferior vena cava is just not functional any more - from the multiple heart caths - which HAD to be done. We sure are not blaming anyone for this - it HAD to be done. The problem is now, they say at Arkansas Children's, they have never seen this and there is nothing they can do about it. He has to develop what we call collateral - meaning collateral circulation to provide venous drainage and return to the blood from the body to the heart for oxygenation. We were even asked if we wanted to put him through these episodes - as nothing can be done. If you remember, before he got his transplant, he was having palliative surgeries to sustain him and he was developing collaterals so fast, they had to be coiled and recoiled to make the blood flow where it was supposed to. That is a higher pressure area - and that induces faster collateral circulation - but we know the boy is a collateral making machine.

Liam is now in Boston where hopefully doctors can help him with this problem!

If you're not already a Liam follower, check out his Facebook page for recent updates and picture of this adorable little man and his cheeks!

CHD Warrior - Baby Pierce

Baby Pierce is another little fighter that Sugar Pea and I check on every day.  Pierce was born 7 weeks premature with Heterotaxy Syndrome, TAPVR, Right atriall isomerism, common AV canal defect, 2 large VSD's, one common yet large ventricle (able to be divided into 2 functioning ventricles).  He's now 6 months old and had the open heart surgery he needed to repair his heart.

I started following Baby Pierce's story back in late November or early December.   This little guy has been through a lot in his short life.  Not to  mention everything his family has been through.

Some of you may have heard about Baby Pierce from Anderson Cooper 360.  This is the little boy that medicaid refused to transfer to a hospital that has 10 times more experience with his condition than the hospital he was at does.

After many many many CHD "Heart" Mom's got together to raise money and awareness about Baby Pierce, Elizabeth Cohen traveled to Indiana to do a story on Pierce.

After the story the hospital decided to pay for Pierce's transport to Boston Children's Hospital.

Like most CHD Warriors, Baby Pierce's story is on going.  I highly suggest following his amazing story on his Facebook page.

video from Anderson Cooper 360

CHD Warrior - Riley

I met Riley's mom, Carol, on Twitter back in July. Instantly I was hooked on this amazing little boy's journey. On August 2nd Riley got his 3rd call that they had a donor for him. Sugar Pea and I sat glued to the computer hoping for updates on Riley. Sugar Pea hoped and prayed that Riley would get his much needed transplant on August 3rd, her birthday. She was devastated when we read that Riley was sent hom to wait longer because the lungs weren't good enough to transplant.

Sugar Pea has a list of kids she wants to check on in the morning, and before she goes to bed every day.  Riley is at the top of her list.  She always tells me that even though she doesn't know Riley, she feels a connection to him because he's part of the family of people waiting for a transplant.

You can join Riley's journey at www.smile4riley.com.

Riley's journey is such a complex one, I thought it would be better coming from his mom.

I was told that Riley had Heterotaxy Syndrome and Complex Congenital Heart Disease when I was 22 wks pregnant. This was when I was sent for a third ultrasound, a Level 2. The doctor who told me the news was not very positive. He said that babies that are in Riley's situation do not fair well. He gave a survival rate of 50% to one year. I certainly wasn't expecting the news.


The following day I contacted Children's Hospital in Philadelphia for a second opinion. They set up an all day evaluation that consisted of an ultrasound, an echo and then a meeting with a team of doctors. They, too, said that Riley's case was very serious, but they would do everything they could to help him. They said he would have to have a three stage procedure to try and fix his heart, but that his biggest issue was going to be his Pulmonary veins. They were going to have to be rerouted at the first surgery, and they were very narrow. They weren’t even sure if they were going to be obstructed at birth. If they were he would need immediate surgery.


Riley also had a CCam in utero. That was discussed at the meeting, but luckily it got smaller in size, and we have not had to address it. We discussed that he may need the Ladds procedure if he had malrotation, but that couldn't be fully DX until after delivery with an upper GI.


It was also decided that it was better for me to deliver at University of Penn. next door to CHOP, in case his veins were obstructed at birth. I also was scheduled for an Amino. They wanted to rule out any deadly Trisomy's, so we knew that we could move forward with the plan of care.I transferred all my care to CHOP the next day. I went every 2 wks and had an ultrasound, and every fourth wk an echo. It was very emotional to say the least. I was preparing to have a baby that they could even tell me would live. I dreaded every echo. It was always the same bad news about his veins.


Finally they decided to induce me on April 4th. I was due April 25th.They took Riley from me immediately at birth. I did see him for about 2 minutes when they wheeled him down to my room on their way over to CHOP.Once at CHOP Riley went directly to the CICU. Luckily his pulmonary veins were not obstructed. They ran all of their lines through the umbilical cord, and set him up to the heart monitors, gave him some oxygen, and then we waited.


On the fourth day he went in for Stage one. He had a BT Shunt placed, and his veins repaired. He then spent about a week in the CICU before heading down to the CCU. While in the CCU it was determined that he did have malrotation, but that the Ladds would be discussed later. Riley first came home from the hospital after 2 weeks. He briefly had a NG Tube for feeding, but that only lasted a few days until he pulled it out.


I knew once we were home our next dreaded appt. would be an echo. Sure enough in June Riley had an echo and it showed that his veins had some obstruction. They sent him for a Cardiac Cath about 2 wks later. While in the Cath Lab it was determined that Riley's veins would need be repaired again surgically. The doctor also made sure to tell us that Riley having Heterotaxy on top TAPVR made things very difficult and hard to fix.


In July Riley went in for his "Sutureless Repair" surgery. This was now his second open heart surgery. The surgeon believed that the surgery went well, and that the blood was flowing again. At that point he believed we could head toward Stage 2 of the procedures around December, however, he could not guarantee that the repair would hold, and that Riley would not form scar tissue that would again cause obstruction. All we could do, though, is move forward. Riley spent about 2 wks in CHOP after surgery.


Then we went home, again, until the next appt. During this time Riley's meds are continuing to increase. He has antibiotics for the lack of spleen from the Heterotaxy and he has several heart and Reflux meds.


Around the end of August Riley had another echo. Would you believe that it showed obstruction again?!


Our only option now is to back to the Cath lab. There is nothing more surgically that can be done. Two weeks later Riley had another Cath. This time "cutting balloons" were used to try and open his veins. It was once again successful, but once again there was no promise that it would hold.
Now things are not looking good. Nothing can be done to Riley's heart or his abdomen unless his veins are stable. So everything is on hold.


Again we go home. All this time that Riley is having vein issues he is also having severe reflux issues. Throwing up all the time. He is below 5 percentile in weight. He is taken meds for reflux, but their is still an issue. They may surgically address the reflux, but again can’t do anything if the veins aren't stable.


The end of October Riley had another echo. The obstruction has returned! Our only hope now is to place stents in the veins. Riley went back to the cath lab mid November. During this procedure he had stents placed in three of his four pulmonary veins. The doctor told us that this was pretty much the last option. If the veins obstruct again with the stents they will balloon them, but they will not continue to do that if it’s not working.


Then in December Riley decided that he didn’t want to eat anymore. So January '09 he had a G-Tube placed to assist him in his feeding. The surgery went well we had a couple minor set backs, like the G-Tube fell out the day after surgery. Now, though, he has the tube in and receives all of his meds and feeds via gtube. In addition, to the insertion of the tube they also removed his appendix and they took out his intestines and put them back in, I would like to say the right way but they are the right way for Riley.


In March they did another Cath and used the cutting balloons on 3 veins and they put a stent in the 4th.


In April they did another Echo and two veins were obstructed so now it was off to the Cath Lab for another intervention with cutting ballons. They have spoke a little about a transplant but with his veins so unstable that still seems to be an issue to have him listed.


October '09 ~ Well we have come to that point, the place we had hoped we would never be. During a cath it showed that one of his veins was completely occluded. Riley's needs a Bilateral Lung/Heart Transplant. We had hoped it wouldn't come to this because the survival rate is Extremely Low to age five. However Transplant is Riley's only hope since his veins are failing.


Since being listed for transplant Riley has lost an additional pulmonary vein. He has a Cardiac cath performed every 8 weeks. Scheduling his 20th cath for mid Febuary. He also requires 3L of O2 24/7.



At this point Riley has been waiting for more then half his life for his Gift of Life!


Riley's official diagnosis is ~ Heterotaxy Syndrome (Asplenia Type) with CAVC (common AV canal), Pulmonary Atresia, Right Ventricle to Aorta, TAPVR (total anomolous pulmonary venous return)(Infradiaphragmatic-unobstructed) and RRA s/p LMBTS (right aortic arch status post left modified BT shunt), patch augmentation of Pulmonary Artery bifurcation and TAPVR repair at 4 days old.