Our Heart Boys

Patrick had a follow-up appointment at the heart center this morning and they were pleased with how well he is doing post-op. He has gained weight (tipping the scales at 15 lbs 1 ounce) and his “zipper” is healing nicely. Patrick’s head was in the 40th percentile before surgery and despite the amount of blood that backs up in a baby’s head after the Glenn procedure, he is still only up to the 50th percentile for head size. That means he only has “Glenn head” minimally.

Patrick’s cousin, Jeremiah,  is settled at Primary Children’s Medical Center. His lungs are stronger than they had initially thought and they have been talking about ECMO (a machine that will put oxygen in his blood) and surgery today. From what I have heard, they have had some ups and downs, but Jeremiah continues to fight and to surpass expectations. Thanks everyone for your prayers for him. Be strong baby J, we love you!

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Chylothorax

Yesterday after Patrick had been drinking breastmilk again his chest tube started to drain white/milky. The nurse explained that this might be a sign of chylothorax (‘chyle’ just means ‘fat’), and this morning during rounds they confirmed that it has continued and diagnosed it. Basically, during surgery his lymphatic system was traumatized somehow and is now leaking the fats from Patrick’s breastmilk.

In response, they have Patrick eating a special formula called portagen (he’s had 1 oz so far–it is funny-smelling, but hopefully he doesn’t think so). He may need to be on a portagen diet for up to 6-8 weeks, but it will just depend on how he’s doing. From what I understand, portagen contains medium chain triglycerides instead of the long chain ones found in breastmilk and regular formula. The important thing is that the portagen does not need to be processed through Patrick’s lymphatic system and therefore the fats won’t leak out. That will be a win-win because Patrick will be getting the nourishment he needs and the drainage can eventually stop so that the chest tube can be removed. He’ll be much more comfortable without the chest tube.

Other than the chylothorax, Patrick is doing great. His BP is no longer a problem and they have been able to decrease the number of drips/meds he’s on. He’s down to oral tylenol and ibuprofin (actually he’s on a stronger version of ibuprofin, but I can’t remember what it is called) for pain.

This morning he was interested in looking at his books, although he was a little frustrated that he couldn’t hold the books and help turn the pages like he usually does (he still has an IV in his right hand so it is bandaged up and has a sock on it…) He was trying to grab his feet as usual, and wanted to be picked up. Despite his annoyance at his currant limitations he is quite a trooper and is now napping.

Patrick's personal harp performance Tuesday evening.

Yesterday he got to sit in a bouncy seat that vibrates. Having his head elevated aleviates some of the pressure that goes to his head as a result of the Glenn procedure. The vibrations kept him from sleeping too deeply (gotta keep that BP up!). And being able to bounce him was helpful for calming a baby who can't be held and rocked quite yet.

Second Opinion

It’s easy to forget that something as serious as a heart defect can be pretty subjective. Especially when observation of the problem can only be done via ultrasound. But depending on their experiences and what they see, the experts seem to come away with different assessments and expectations. Happily the surgeon, Dr. Nigro, and Beth, our fantastic Cardiac Nurse Practitioner, immediately told us this morning that they are pleased with where Pedro is at.

Even before saying hello almost, they reassured us that Pedro’s heart has only increased in size by about 10% over the last few months that they’ve been watching. While it is definitely enlarged, they estimate that it is only taking up about 52% of his chest cavity, which means his lungs have not been inhibited in their development. They anticipate that he won’t have the major breathing problems that some heart babies do at birth. AND they don’t think he will need surgery immediately. Their preference, in any case, will be to try everything else first to stabilize Pedro at birth and let him grow a little older before surgery.  Even if he is struggling, hopefully he can “eek by” until he is bigger and stronger. (Yes, the surgeon said “eek”).

In any event, we have a better idea of what to expect. Delivery should be normal, with a little extra monitoring, and just like other babies Pedro will be cleaned up and checked right there in the labor & delivery room (under a warmer). Then they’ll bundle him up for us to hold him! If he is doing okay, we should get a little bit of time with him before Braden goes with him to the NICU (which is on the same floor as L&D). He will be in the regular NICU for about 24 hours or so and then he’ll be transferred to the PCICU (Pediatric Cardiothoracic ICU). He’ll be in the PCICU about a week or week and a half. IF he does end up needing surgery right away it will be within the first week. In that case he’ll be in the PCICU for a few more weeks than he would otherwise.

Reality Check

We have been getting so excited for Pedro to arrive, and our preparations and anticipation distract us from worrying too much. Afterall, we really won’t know what will be needed until shortly after he is born. So, we are optimistic. Sometimes being optimistic makes it somewhat bitter to swallow any news other than “things look the same” or “he’s doing fine”. We find ourselves secretly hoping that one of these days the doctors will pronounce Pedro’s heart miraculously healed or something.  I know, I know, in denial much?

We made a visit to St. Joe’s this morning for a fetal echo and consult with Dr. Diab. As before, they could see that Pedro’s tricuspid valve is leaking significantly and that his heart is enlarged. Dr. Diab indicated that Pedro’s heart fills (or will soon fill) the majority of his chest cavity. Dr. Diab suspects that Pedro will need immediate surgery to put a shunt in, but it will all depend on how things function once he’s born. Apparently one of the benefits of the shunt would be that his heart would rapidly shrink down and be a closer-to-normal size. I believe the main purpose of the shunt, though, would be to make sure that enough blood can get to the lungs.

On Friday we will meet with the surgeon, Dr. Nigro, again, so we’ll have a chance to learn more about what would be entailed in the shunt procedure.

Take Heart

We had a fetal echo at St. Joe’s on Wednesday and met Dr. Alboliras, one of the Pediatric Cardiologists at the Heart Center there. He was pretty positive about everything, and got a good look at Pedro’s whacky ticker. Although the right side is enlarged, Pedro’s heart rate is still good and circulation to the lungs, etc. is still normal. Dr. Alboliras indicated that the regurgitation is only moderate at this point and he feels confident that we will be able to go full term!

Dr. Alboliras also said that Pedro’s tricuspid valve (particularly the long leaflet) looks amenable to surgical repair–meaning it’s fixable, in his opinion. As always, we will have to see how Pedro does once he makes his debut. He will probably be in the NICU for at least a week or two while they stabilize (if necessary) and monitor him.

In Good Company

It was so nice to get away for a few days and have tons of family to distract or encourage us, depending on the moment. Thanks go out to my Mom & Dad for all of their efforts for our family reunion and to our other family  members who planned activities and special occasions so that we could be there.

I mentioned a 17-month-old girl with Ebstein’s in a previous post–we have since heard from her family, the Lowry’s. Although they are from Prescott, AZ, we were actually able to meet up with them while we were in Utah this past weekend. It was awesome to meet them and to see their cute little girl running around and happy in spite of her first surgery (primarily a valve repair, I believe) on June 10th. They were hoping that additional procedures/surgeries will not be needed, but based on her follow-up visit it sounds like she might need the Glenn procedure as well. We are definitely hoping for the best for them!

The Lowry’s are pretty much great all around. They took time away from their family festivities to talk with us. It was nice to get a better idea of what to expect, particularly everyday things we will need to do for our son. Mostly, though, we appreciated the meet-up because it’s good to remember that we are not the only ones that this has happened to. It’s amazing to have such a support network, including the Lowry’s and other “heart families” who we will probably meet and/or interact with through hospital visits and the Eller Heart Family Council.

Things we have learned from the Lowry’s include:

  • Heart babies tend to have a difficult time putting on weight because their little hearts are working so hard. I will likely need to add formula to breast-milk (if we want him to have breast-milk, which we do) and bottle-feed in order to keep track of Pedro’s caloric intake.
  • When Pedro comes home with us, he will probably have a monitor that alerts us if his oxygen level drops. For the Lowry’s this usually happened during the night when the monitor got kicked off or their baby relaxed and her ox naturally dropped. We weren’t planning to get any sleep anyway, though, right? (I say “we”, but who am I kidding? We all know that Braden has slept through louder things.)
  • Some heart babies take Viagra in order to help their blood flow
  • The Mayo Clinic a youtube video about Ebstein’s Anomaly:

All Roads Lead to Nigro

Yesterday I had a consultation with Dr. Jedeikin from the Arizona Pediatric Cardiology group (affiliated with the Phoenix Children’s Hospital) for a second opinion/option kind of thing. We were tempted to cancel the appointment because we feel so good about the St. Joe’s group, but we decided that we should see what Dr. Jedeikin  had to say. You know, due diligence and everything.

Dr. Jedeikin seems really great. (And I’m not just saying that because I like his South African accent…) He is very, very passionate about hearts and cardiology. He did a fetal echo and went over Ebstein’s Anomaly with me again. I always learn new things about the heart, and I came away in awe of all of the many facets of a human heart–how do all of those parts work perfectly in most people?!

Dr. Jedeikin’s assessment and approach to Pedro’s case is pretty much the same as those we’ve heard from the other doctors involved. He hopes that immediate surgery will not be necessary, and has pretty much the same plan for monitoring our little guy’s heart–both pre and post delivery. He had good things to say about the Phoenix Children’s Hospital (PCH), but apparently he could work with us even if we choose St. Joe’s for delivery. He encouraged me to deliver at St. Joe’s since that is where my new OB delivers (rather than switch OB’s again in order to deliver at the hospital near PCH). From this I concluded that both hospitals are a good choice, and we just need to pick the one that we feel most comfortable with. They both have Level 3 NICU’s.

Probably the most interesting/encouraging part of this consultation was that Dr. Jedeikin would involve Dr. Nigro for the surgery aspect. Dr. Nigro seems to be the go-to guy for surgery in these cases, regardless of which hospital we go to.

So, since:

  1. the hospitals are comparable
  2. we get Dr. Nigro either way
  3. the Pediatric Cardiologists are excellent either way

…we are free to choose the hospital and Pediatric Cardiologist(s)  that we feel the best about. We still appreciate that at St. Joe’s I would deliver and recover at the same hospital where Pedro would be in the NICU. We also feel like the program with the St. Joe’s cardiology group is a better fit for us–much more supportive and user-friendly. Also, once you meet Beth, the nurse practitioner, it’s a no-brainer. She is off-the-hook amazing.

Although it did not change our mind, I’m really glad I went to see Dr. Jedeikin. Now we can feel even more confident about our decisions, and I appreciated learning more about Pedro’s heart. Here are a few things I now understand after meeting with him:

  • The tricuspid valve is made up of three leaflets, one of them is the ‘Septal leaflet.’ Pedro’s septal leaflet is dysplastic–which means it is gnarly, thickened and crumply. The other two leaflets are doing all of the work to open and close the valve as best they can–which is why one of the leaflets is longer and floppier, it’s trying to cover the Septal leaflet’s territory too.
  • Dr. Jedeikin did not think that Pedro’s Septal Leaflet would be fixable because it is so dysplastic.
  • If surgery does become necessary, it is possible that they would move the leaflets closer together so that the functional leaflets can adequately close the valve with each heartbeat.
  • He didn’t go into all of the details, but if it becomes necessary to surgically re-route Pedro’s heart so that it operates with a single ventricle scenario (not preferable), there are a few different procedures:

Blalock-Tuassig Shunt

Starnes Procedure

Glenn Procedure

Fontan Procedure

{Pedro could need all, none, or some combination of the procedures. We have heard a little about these procedures before, but had not learned all of the names yet. I’ll activate hyperlinks if I find good descriptions for them.}

Oh and, Pedro’s new trick of the day was rolling. The sonnographer laughed with me this time when Pedro would roll away while they were trying to record his heart. Then he would roll back and punch the ultrasound wand. He definitely has a little personality already ♥

Meet the Surgeon

Here I am getting more rotund, these were taken this morning right before we visited St. Joe’s again:

Our activity of the day was to sit down with Dr. Nigro (\nye-grow\)  in order to get to know him and play 20 Questions. I think we got really close to 20 Questions–but they didn’t give us a prize or anything…Bummer. Dr. Nigro has extensive experience handling similar cases of Ebstein’s Anomaly with successful outcomes. The only fatalaty he has seen was a result of an unrelated illness, and not from the surgery itself.

Dr. Nigro was very encouraging. He talked about the range of severity of Ebstein’s in babies like ours, a spectrum that goes from the severe end to the mild end. At the mild end the babies seem like any other baby when they are born (on the outside). Dr. Nigro feels that Pedro will probably be closer to the mild end and he is hopeful that surgery won’t be necessary right away. He compared Pedro to a little girl who was born with a similar case of Ebstein’s and she just barely had her first surgery (and they hope her only surgery) at about 17 months. Compared to surgery as a newborn, surgery after the first year sounds much better.

Dr. Nigro also feels that the Fontan procedure , where the plumbing of the heart is re-routed to skip the faulty valve, will not be needed for Pedro. He seems hopeful that Pedro’s leaky valve will be repairable. Whether or not it takes multiple surgeries will depend on how early surgery is required and on how well Pedro does with it.

All around, we enjoyed meeting Dr. Nigro–especially because in addition to being capable and professional, he has a good sense of humor. We had read his bio beforehand and knew that he had done his residencies at USC. So, of course, Braden’s first question was sports related–and Dr. Nigro just ran with it. Is humor a basis for selecting a hospital and a surgeon? Nope.com. But it’s nice to feel a connection with them anyway.