Adios Amiodarone

Patrick had an appointment with Beth at the heart center today. Gary was the unlucky one who was tasked with weighing, measuring, monitoring, and echo-ing a squirmy, opinionated 7-month-old. Gary was great. Patrick was…difficult. But they were able to get enough information to tell us that Patrick is doing well. Things look the same as last time–the tricuspid valve is pretty leaky but that’s why they did the Glenn procedure. As long as the right side of his heart holds up under the pressure from the regurgitated blood, there may not be a need for more surgery in his future. This is sort of uncharted territory so we’ll have to see how it goes, but the cardiologists seem pleased with how he is doing.

In fact, he has graduated from his amiodarone–a once daily med he has been on since he was born. Amiodarone prevents the SVT (super-high heart rate) episodes that Patrick had a couple of times as a newborn while still in the PCTICU. At this point, P has outgrown the dose he’s been on and since they have been planning to take him off of the drug anyway, it is better to do it now rather than up the dose. It will take awhile to clear from his system, so in a month or so we will start a 21-day ACT monitor again (he had one for the first couple of months last Fall). I’m not that excited for the monitor, but it will be so nice to have the peace of mind knowing that the doctors (and us parents) will be alerted if his heart does something funky. AND we are happy to have him off of one more drug. Now all he will be taking is aspirin once a day for the rest of his life (to avoid any blood clots in his heart–particularly the troublesome right side).

Patrick doesn’t need to go back to the clinic until after we’re done with the ACT monitor–so not for two months! After that we may only need to take him every 4-6 months. How rad is that?

We should be hearing from someone regarding physical therapy–Patrick  is lagging a little on some of his physical development. His sternum is wired together, and you can feel the bumps under his scar. As you can imagine, this makes tummy time a bit uncomfortable. Also, because the Glenn procedure re-routed the blood from his upper-body, a natural bi-product is that his head has gotten bigger (in relation to the growth of the rest of his body). With his head so heavy, he hasn’t quite gotten sturdy in the sitting position. He’s a strong kid, though, and he’ll catch up. He just wants to skip sitting and crawling and go to walking–if he had his way we’d have him standing, jumping, or walking (assisted) ALL the time.

These photos are from his last regular pediatrician appointment–he’s getting caught up on the immunizations we had to postpone during the pre and post-surgery time period. (Tia, Patrick’s nanny, was nice enough to come with me to the appointment. Six needles…three in each leg! He was a trooper, though.)

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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!

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

Postponed

We were nervous about Patrick having surgery with the congestion and cough he has had this week, but his lungs have been clear so on Tuesday they cleared him for surgery. This morning, however, once the anesthesiologist checked him out and discussed it with the surgeon, Dr. Nigro, they recommended that we postpone. We want it to be as safe as possible, so it makes sense to wait until his cold clears up. Coughing and increased pressure on the lungs are definitely not what we want for Patrick while he recovers from having his pulmonary artery (which takes blood to the lungs) messed with, among other things.

We have an appointment to meet with Beth on Tuesday, the 11th and we’ll go from there to reschedule. It’s a bit of a roller coaster but this will be best for Patrick. It has been awesome to have such an outpouring of support–thanks everyone!

Here are some photos from Braden’s ipod–taken in ped’s pre-op and the O.R. waiting area this morning.

It’s a Date

We met with Patrick’s cardiothoracic surgeon, Dr. Nigro, and Beth on Friday to discuss surgery. We were relieved to find out that Patrick’s surgery is not an emergency and can wait until after the holidays. Patrick is scheduled for a tricuspid valve repair and possible Glenn on January 6th, 2011.

The valve repair requires open-heart surgery. Once they look at and work on the tricuspid valve they will decide if Patrick also needs the Glenn Procedure to take some pressure off of the right side of his heart. The Glenn would connect the superior vena cava (the vessel that brings blood from the upper body to the heart) directly to the pulmonary artery (which normally takes oxygen-poor blood from the heart to the lungs), skipping the right side of the heart entirely.

Once Patrick has gone under anesthesia, he will get a breathing tube and an echo tube. They will make an incision in his chest and open the bone.

He will be on a heart-lung machine–his heart will be asleep while they assess and repair the valve as well as possible. Then they will decide if we need the Glenn. They will wake up Patrick’s heart and, if the Glenn is needed, they will do that procedure. They will also patch up the ASD (hole between the right and left atriums), although they may leave a little bit of a hole to relieve pressure.

Patrick will be in the PCTICU for 7-10 days. Recovery is straight-forward after he leaves the hospital. They expect him to rebound quickly. Risks are minimal–Dr. Nigro estimated a risk factor of 1-2% for major complications. Patrick will need a blood transfusion, so Braden and I had bloodwork done to see if we are a match to donate. He’ll need 3 units of FFP & platelets. We are waiting for the hospital to contact us with the results of our bloodwork, and to let us know what Patrick’s blood type is.

We feel very blessed to not be spending the holidays at the hospital. AND we were given clearance to drive to Southern California for Christmas! We are looking forward to taking our mind off things for a bit as we vacation both in California and then with my side of the family who are visiting Arizona after Christmas. Patrick has many aunts, uncles, cousins and a grandpa to meet!

Upcoming Cardiac Catheterization

Patrick has been scheduled for cardiac catheterization on December 8th at 9:30am. We will check him in at 7:30am that morning and he’ll be kept overnight after the procedure (probably discharged by lunchtime on the 9th). Before the procedure they will give him a full check-up to clear him for the catheter. Patrick will go under general anesthesia for this event–which sounds better to me than trying to keep him calm and still. Here is a little description of the procedure:


I was relieved to learn that Patrick won’t be required to fast for an inordinate amount of time or anything. However, he is not to have any breastmilk/formula after 3am on the 8th, but can continue drinking Pedialyte, water, or diluted apple juice between 3am-5:30am. Past 5:30 he is not to eat or drink until he wakes up following catheterization.
After this they will talk to us about when surgery will happen and what they plan to do based on what they see in his messed-up little heart. If there are things that you think we should ask beforehand about the heart catheter, or about the surgery in general, let us know.

Leaky

I’ve never seen Patrick SO happy to be back home, but after all of his tests at the Heart Center yesterday afternoon Patrick looked like he could’ve kissed the living room floor upon returning to familiar surroundings. One of his favorite things to watch is the ceiling fan, and he was full of smiles for Mr. Fan. Next he was thrilled to sit in his swing again, and he sat and talked to the little lambs on the swing mobile and smiled at his own reflection in the mirror above the swing. He sure seems to like the handsome dude that looks back at him!

Although he was pretty unhappy about being held down for a chest x-ray, echo, and EKG–not to mention having blood drawn from his little arm–for the most part Patrick was smiley and friendly to the doctors and nurses. Even while the cardiologists were giving us some serious news, Patrick grinned and grinned at Doctor Rhee. Braden and I were not quite so cheerful about the doctors’ report that Patrick’s valve has gotten very, very leaky. Right now the x-ray shows that his heart has not become much more enlarged, but the echo showed that his tricuspid valve is allowing much more blood to leak back into the atrium. His heart is working SUPER hard, and it is only a matter of time before his atrium becomes so enlarged that his heart fails.

Fortunately, Patrick has been eating well and gaining weight, because it looks like the doctors will need to intervene earlier than we had planned. The bloodwork they ordered will tell us how far into heart failure he is, and in the next couple of weeks they will probably do a heart catheter to get a better look at his valve. On Tuesdays the doctors all meet to discuss certain cases and Patrick is on the docket for next Tuesday. Hopefully they’ll have a plan for us after that. Beth indicated that once they decide that he needs surgery, it will probably be about a month before the procedure happens.

For now we are watching for signs of heart failure like vomiting, appetite issues, or labored breathing. Our travel plans are on hold and we are to continue our germaphobic precautions. Airplane rides are definitely out of the question, and even road tripping may be kiboshed so that we stay close to Patrick’s medical team.

Ten Pounder

Patrick saw his cardiologist, Beth Rumack, last Thursday for a routine checkup. He weighed in at about 10 pounds!

Left: 10 Days Old, Right: 6½ Weeks Old

He has been growing steadily and according to the chest x-ray and echo done on Sept. 30th, his heart has not really become any more enlarged than it was at discharge. Also, the tests showed that his ASD (a hole between the right and left sides of his heart) has actually closed up a bit on it’s own. This might mean that the ASD could be repaired via a heart catheter rather than open heart surgery–which I think could buy us more time before the tricuspid valve repair is necessary.

Patrick had occasionally been trying to sleep longer between nighttime snacks, and Beth told us that we could start letting him do so. She told us that as long as he is getting about 20 ounces of breastmilk per day then he should be fine sleeping through the night. At the time Patrick was only eating about 15-16 ounces a day, but once I started letting him go longer between feeds at night, he really picked up his game. Over the last few days, he increased his intake from about 18 ounces to 24 ounces. So far he has still been waking up once or twice during the night to eat (most consistently at 1 or 2 am), but even that has been so nice for me (he was eating every three hours before). Yay for more sleep! I went from a total of 4-5 hours to a total of  nearly 6-7 hours of sleep each night. Amazing.

Patrick literally changes every day. He is developing so quickly, and we are having a ton of fun with him. He has become a lot more smiley and talkative with his little Patrick sounds.

At 7½ weeks old, Patrick likes to:

  • Keep his legs and arms in constant motion (often in his sleep as well)
  • Kick with his right leg more than his left
  • Have his hands balled up in fists still (although we are starting to see him keep them open occasionally)
  • Grip things in his fist (i.e. our fingers, toys, hair–unluckily for Braden’s chest hair, etc.)
  • Pee in the bathtub
  • Poop on Daddy
  • Hang out on the changing table. He has come to appreciate diaper changes more (he HATED them as a newborn) and he really likes to look around at his toys and monsters that hang by the changing pad. Some of our best conversations take place there–he likes to be sung to, talked to, and to learn new tricks while he’s laying there.
  • Hold his head up. Patrick has the head control of a 3 or 4 month-old (as per his pediatrician).
  • Put a halt to ALL other activities in order to concentrate on going #2.
  • Hold Daddy’s thumb while being bottle-fed
  • Look at himself in his crib mirror and the mirror above his swing
  • Sit in his swing, whether it is moving or not. And he loves to sleep there.
  • Watch the lights on his activity play-mat
  • Follow toys with his eyes when we move them back and forth in front of his face
  • Fight sleep with all of his might
  • Ride in his baby bjorn carrier
  • Take his heart medicine–seriously, he thinks it is super tasty and he’s sad when his daily dose is gone
  • Video chat with his grandparents
  • Sleep on Dad…

Patrick

I am finally snagging a chance to post on here. Thank you everyone for the amazing support and messages we have been receiving! While I have not been able to respond to many, I have read every single email, facebook comment, text message, and carrier pigeon correspondence. Patrick has quite the fan base and we are SO appreciative.

The main things they are watching with Patrick are his heart rate and oxygen saturation levels. There is a blood vessel called the ductus arteriosus that takes blood to the lungs while the baby is growing in the womb. After birth, the vessel closes up and the pulmonary artery takes over the task of taking blood to the lungs where they now become enriched with oxygen. Because Patrick’s pulmonary artery seemed compromised due to the problems with his heart, the doctors have been medicating Patrick with prostaglandin(s) to temporarily keep his patent ductus arteriosus (PDA) open. This allowed the PDA to help compensate for the under-developed pulmonary artery by keeping blood flow to the lungs and, in the long run, sending oxygen throughout Patrick’s little body.

This morning the cardiology team conferenced over Patrick’s case to discuss a plan of attack. From what they saw in Patrick’s echo, his pulmonary artery is working a little more than they may have initially thought it would. So this morning they took him off of the medication to see how he does without the PDA. In the morning they will do another echo to see if the PDA has closed up and check things out. So far he his oxygen saturation level has stayed in a decent range and a fun side benefit of going off the meds is that Patrick is able to be more alert. He can actually open his eyes and keep them open for awhile, which we of course think is amazing and so stinking cute. Pretty much everything he does is awesome in our book.

Depending on how things go with this PDA experiment, and with anything else that comes up in the next few days (or weeks), the doctors have a few options they are considering. I will now make a lame attempt to tell you about the procedures that are on the table. Heart Moms and Dads: please feel free to correct me where I misunderstand and misinform.

One route would be the Modified Blalock-Taussig Shunt–which would be a tube that they insert between the pulmonary artery and a branch of the aorta. This would be a temporary fix to make sure blood gets to Patrick’s lungs. Eventually Patrick would need more surgery for a longer-lasting fix.

Another possible procedure is a heart catheter. I am not entirely sure if this is just something that they would use to learn more about how his heart is working, but the impression that I got was that they can insert a balloon into Patrick’s heart and then put a little air in the balloon to open up his pulmonary artery a little more. They would go in through his leg/groin and up into his heart.

There is a third option that they’ve mentioned, but I don’t remember for certain what it was called. I think it is the Starnes Procedure. I need to find out more about that one–I’m sure it’s been explained to us but not necessarily in connection with the name of the procedure.

The doctors seem to anticipate that Patrick will need the Glenn Procedure eventually, regardless of what they do for a temporary solution right now. I *think* that the Glenn is either similar too or interchangeable/combined with the Fontan (re-routing the plumbing of the heart to skip Patrick’s faulty tricuspid valve).

There is still a chance that Patrick will not need surgery immediately, and we can take him home and grow him before the Glenn Procedure is absolutely necessary.

Hopefully I’ll be able to get on here more often now. Braden has been great about updating Facebook, I’ll try to get some photos and videos on here as well.

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.