Jeremiah

My nephew, Jeremiah, was born this morning and is in the NICU. He is a heart baby too, he has transposition of the great arteries (TGA) and a ventricular septal defect (VSD). To further complicate things, he has a congenital diaphragmatic hernia (CDH).

He was only expected to live two hours at best but surpassed the two-hour mark hours ago. We are praying for a miracle.

Jeremiah’s mom, Heidi, is recovering, but to read more information on his diagnosis pre-birth you can visit Heidi’s blog: Gibbering Madness.

My mom sent this update earlier this afternoon:

Hi All,

I just got back from University Hospital. Bryce got to Utah yesterday around 4 p.m., and by 8 p.m. Heidi was in labor. They went to the hospital around 3 a.m., and Jeremiah was born at 4:20 a.m. by C-section. He weighs 7 lb 1 oz and has quite a bit of shimmery light brown hair—that looks curly.

He is in the NICU and has already lived four times longer than they had been told to anticipate. The doctors there are so good at communicating with Bryce. They have spent most of this time trying to get the carbon dioxide out of Jeremiah. It can be as huge of a problem as too little oxygen can. His carbon dioxide numbers have been going down all morning—which is what they’ve been hoping would happen. At one point they were going to use a surfactant to try to get his lungs to “unstickify” and open up more, but when we left, they had decided not to do the surfactant.  They didn’t want to run the risk of making a hole in his lung with too much pressure if they didn’t have to do it.  They have two pulse ox machines going on Jeremiah that report his oxygenation both at the feet and at the top of his body. He is generally around 70% which is good considering his heart has the pulmony artery and the aorta connected opposite of where they should be. He has a large hole between left and right—which is usually problematic. In Jeremiah’s case, it is helping his blood to mix and making it possible for a little more oxygenated blood to get pumped out.  They have Jeremiah being cooled to help prevent brain damage—and maybe other reasons, too. His intubation took 10 minutes, so there is a possibility of brain damage. The doctors said he responded very quickly once they got the tubes in, and they thought with the cooling, his brain might be OK.

I know I was introduced to lots of doctors in the NICU, but I remember no names or titles. Bigwig doc from Primary Children’s was there observing Jeremiah. He was working with Jeremiah’s two in-the-room doctors. They would explain every move to Bryce—who didn’t leave unless they asked him to (during shift change and reports). Bigwig doc was going to call bigwig heart surgeon to discuss the possibility of doing heart surgery today and getting Jeremiah onto ECMO after the surgery. That was never offered as a remote possibility prior to today, so even that possibility is nice to cling to. He still might not make it, but there is that remote chance.  Oh, and by the way, his feet are just fine—not clubbed as they had been told they were. Miracles do happen.

Greg took a couple of pics on his new cell phone while we were at the hospital. I will get Danica to email them to me and I’ll include them here.

Heidi’s Mom has been in California helping with another grandbaby that was born. She hopped onto an earlier flight and should be back in town by 1:30 this afternoon.

As we were leaving the hospital around 11 a.m., Heidi was begging to get up and go see her baby. They had taken her there once in her hospital bed on the way to her room. Her feeling has now returned to her feet, and she thought they might let her go spent 5 minutes or so in NICU. I hope they did.

Heidi and Bryce are both exhausted, so texting or email would probably be the kindest way to communicate with them.  Heidi is in room 2305 at University Hospital. I would guess she’ll be there for 3-5 days, so that lets me know that I’m going to be running back and forth with Isaiah whenever possible.

Thank you to all of you who have been praying for our family!

Love,

Colleen

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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.

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.

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 ♥

Help me OB-one

This morning I met my new OB, Dr. Blumrick and he is great—very personable and optimistic. Also, very chatty, which is nice in some respects. He set a goal for delivering Pedro after 39 weeks (so sometime after Sept. 9th would be ideal). Dr. Blumerick was pleased that Pedro’s Right Atrium is still the normal size, and besides the Right Ventricle, the other parts of the heart are all in the 50th percentile for size. So his heart overall is just the right size—not too big and not too small.

He also told me that when my former OB did bloodwork on June 10th to check for Gestational Diabetes (which I don’t have-yay!), my platelet count was at 140—so we are getting even closer to a normal range and rising. A small win, but a win nonetheless.

And what would a doctor’s visit be without an ultrasound?—so we did that again as well. Does this image look a little even more blurry than usual?

Yep. That’s because he refused to hold still. Pedro kept kicking and wiggling when he wasn’t supposed to–and wouldn’t budge when they needed him to move into a position that would make things more visible. Oi! We are in for it with this kid!

Dr. Solomon, a Maternal Fetal Medicine Specialist, did part of the ultrasound. She explained more about hydrops: when the tricuspid valve is regurgitating blood, the leaked blood can sometimes pool up and cause fluid to be where it shouldn’t. If untreated long-term, this could cause heart failure. If untreated short-term, it could cause damage to the surrounding organs, most notably the lungs. We vote “no” on hydrops, and so far we have every reason to hope that we’ll get through the pregnancy without them developing.

“St. Joe’s” – 27 Weeks Pregnant

Early this morning we had a fetal echo and a consultation at the Scott and Laura Eller Congenital Heart Center at St. Joseph’s Hospital and Medical Center (St. Joe’s).  The ultrasound showed that Pedro’s tricuspid valve is leaking a significant amount of blood from the Right Ventricle into the Right Atrium. We sat down with a Fetal/neonatal Cardiac Nurse Practitioner and a Pediatric Cardiologist. We felt really comfortable with them and came away much more reassured. They confirmed the diagnosis of Ebstein’s Anomaly and explained the condition using diagrams and a plastic model heart. Here are some things we learned from them:

  • Congenital heart defects are the most common birth defect
  • Ebstein’s Anomaly (EA) accounts for < 1% of congenital heart defects—it’s very rare and seemingly random
  • They are not sure what causes EA, but they know that it starts forming between 4-6 weeks of gestation and that it seems to be linked to the sequencing of DNA. So they think it isn’t genetic and it isn’t environmental. It’s a fluke.
  • After birth, circulation changes and there’s a chance that the leak will not be as severe—so it’s possible that his valve and heart could function well enough to buy some time for him to mature and grow a bit before surgery.
  • Pedro’s Pulmonary Artery (which takes blood to the lungs) is developing as it should. This is significant because in cases of EA sometimes the artery is affected. Pedro’s healthy artery increases the chances of his little heart functioning adequately after birth.
  • Families who have children with congenital heart defects are usually amiable to meeting and mentoring people like us—the nurse is getting us connected with parents who have been through this.

Their approach to Pedro’s case:

  • The goal is to go full term and deliver at St. Joe’s with a team of cardiac specialists—everyone who would be involved, from the nurses to the surgeons, has a background in working with babies and children with congenital heart defects.
  • The only reason to deliver early is if hydrops form. Hydrops are moisture retention around the heart.
  • We will get to hold Pedro for a few minutes when he is born, as long as he is breathing okay. Then Pedro will go to the NICU to be monitored, Braden will go with him.
  • Pedro will most likely need heart surgery at some point in his life—after he is born we will know if he needs surgery immediately or if it can wait until he’s a little older.
  • If Pedro needs surgery immediately, they would probably keep him at St. Joe’s for a month. We would stay there with him in a family suite.
  • There are two different surgical approaches on the table:
    • If Pedro’s tricuspid valve is repairable, then it should only take one surgery to fix it up and reduce the leaking. He will never have a normal heart and there is a chance that he would eventually need a valve replacement when he’s older—but there is also a chance that it would never bother him again.
    • If Pedro’s valve is beyond repair, they can re-route the blood so that it skips the right side of his heart completely. This would take three surgeries—probably one in the first week of birth, a second surgery at 3-4 months old, and a third surgery at 2-4 years of age.
    • A valve replacement is not a good option in a newborn because the valve would not grow with him.
    • In the NICU they will nourish Pedro through an IV into his umbilical cord stub. They will also use the umbilical cord to draw blood samples so that they don’t have to stick him every time.
    • As long as the surgery/surgeries go well, Pedro should have a normal life and a normal life expectancy.
    • Dr Diab estimated Pedro’s chances of survival to be over 90%
    • Barring the development of hydrops—they do not anticipate that I will need to be induced or have a C-Section. If either of those procedures do become needed, it would be for the usual reasons and not because of Pedro’s heart.