Tuesday, February 7, 2012

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.


  1. Thank you for reaching out to my Sister Carol and allowing her to share her story on your blog.

    1. Your sister is an amazing woman. My husband is a CHD survivor (now waiting for a heart transplant) but I can't even begin to imagine what Carol is going through. I wish I could have just half of her strength!


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