Of Cones and Toes

Patrick enjoyed a routine visit to the Cardiologist this afternoon {“enjoyed” may not be the right word…but he was fairly brave and tried to curb his indignation, tears, and requests to “go back to the gold car.”}

P’s troublesome ticker looks pretty much the same as it did in July. While there is no imminent danger, we will need to do something {in coming years} to reduce the amount of blood that regurgitates into the atrium–enlarging the right side of his heart. Dr Rhee feels that a surgical procedure called a cone repair will do the trick. The idea is to move the three leaflets of the tricuspid valve up where they should be and reshape them into a cone.

Before doing a cone repair, the surgeon(s) will probably want Patrick to get older/bigger. In the meantime, they will call us in soon to do a study on his heart to see if he still has electrical pathways in his heart that could cause SVT (rapid heart rhythms). If SVT is still a potential problem, then they’ll do a low-risk catheter ablation to get rid of the SVT pathways. Both the study and the cath procedure would just be overnight procedures (so that they can monitor him after he’s been under anesthesia).

In other news, we are loving our new house (will share photos in the near future) and getting excited to meet the soon-to-be fourth member of our family. My due date is May 1 and so far the baby is healthy and whole. We had a fetal echo done in January to take a close look at his heart–it looked totally normal. The only item of not was that Patrick’s baby brother has 12 toes! The better to kick me  with, of course…

IMG_6181

IMG_6182

IMG_6161

IMG_6165

IMG_6190

IMG_6192

Advertisements

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.)

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.

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.

Ultrasound & Echo – 30 Weeks Pregnant

Pedro was nice to the ultrasound tech today and he even let her  take some images of his face! I think he liked her because she said nice things about his cuteness. Braden thinks that the tech was just sneaky/stealthy (read: she didn’t push very hard) and therefore Pedro did not detect anything to attack. Whatever the reason, we are happy to see his wee little mug:

And here is his weapon of mass destruction (my ribs cringe at the sight…)

The tech calculated his weight at 3lbs. 9 oz. Apparently that is more than a large jicama, but less than a pineapple (if you like to  think in terms of produce, and who doesn’t?) A pineapple is much cuter than a jicama, but I doubt either of them would be as good-looking as Pedro is going to be in his Angels gear and various duds from the grandmas and others. (thanks ♥)

Now about his heart.  Today they could see that the right side is significantly enlarged. The regurgitation of blood from the right ventricle back into the right atrium is causing the atrium to fill up and expand. While it is no surprise that this is happening–everyone had hoped it would be later in the pregnancy (or not at all, please!)

I *think* this next image shows the problem. I could be way off because we weren’t looking at anything in particular when the doctor told me about the enlarge-ness. But I believe these measurements of his heart show a large circle to capture the circumference of the whole heart, and a smaller circle around the right side of his heart:

We’re still crossing our fingers for a full term baby. Starting after my St. Joe’s ultrasound next week, I’ll go into the OB’s office twice a week so that they can keep a close watch.

In the meantime, it’s a really good sign that Pedro is so active and it should be easy to tell if his heart starts slowing him down. I’m not so sure that this little dude would let anything deter him from his regiment of kick-boxing and aqua aerobics, but I’ll notice if he does actually get quiet.

This last image is just one that I thought was interesting–it shows the blood flow to and from Pedro via the umbilical cord:

“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.

Fetal Echo – 26 Weeks Pregnant

It has become an interesting mix of dread and excitement when I go to these appointments now. I still love, love, love to see my baby. The highlight of this particular visit was that Pedro had become quite the kicker (or elbow-er, headbutt-er, whatever he can throw at you-er). It was probably more entertaining for me than for the poor ultrasound technician when Pedro would kick the ultrasound wand. The kid’s got good aim!

Despite Pedro’s burst of activity, the technician and then Dr. Nyberg were able to see that the condition had progressed and our Pedrito was diagnosed (officially) with Ebstein’s Anomaly. By this point, his tricuspid valve was more obviously misshapen. As a result, it had become floppy and leaky. Apparently, blood is only supposed to be on a one-way track through the heart—in the right side of Pedro’s heart the blood is traveling in both directions.

The Fetal & Women’s Center has an amazing Doctor/Patient Care Coordinator, Susan, who worked with me via email to set up consultations with the cardio groups at both St. Joseph’s and the Phoenix Children’s Hospital. It will be important for us to deliver at a hospital with a NICU—even if Pedro doesn’t need surgery right away, he will definitely need to be monitored closely. Susan also sent me some information and arranged for me to transfer to the Center’s OB for my regular pregnancy visits. (My original OB does not have privileges at either of the hospitals with a NICU.)

Fetal Echo (Ultrasound) – 21 ½ Weeks Pregnant

Instead of having an ultrasound at my obstetrician’s office, the ultrasound people (aka the Fetal & Women’s Center of Arizona) had me come to their own office. We thought this would be another boring visit, so we didn’t bother scheduling it at a time that both of us could be there. Afterall, ultrasound technicians are not really allowed to tell you much, so I went solo to this appointment to let them examine Pedro’s “a little small” heart. The technician checked all the other anatomy again for good measure and she tried in vain to get a 4D image of his face for us, but Pedro was determined to thwart her plans—a scheme he must have inherited from his father. The photographer in me groaned for my future, but I couldn’t help but laugh at our funny little dude:

Blah, blah, blah she checked everything and then spent some time looking at, measuring, recording, and otherwise studying Pedro’s heart. We were right—she couldn’t tell me much. Imagine my surprise, though, when she stepped out to get “the doctor” so that he could look if he wanted. And he wanted.

Dr. David Nyberg was absolutely wonderful. He came in and did his own ultrasound to get a few more images. Pedro tried his best to move around at all the right (translation: wrong) times, but in spite of that Dr. Nyberg was able to see what he needed to. He explained as he went along that the valve on the right side of the baby’s heart seemed to be shaped a bit abnormally. Afterward, he had me come into his office to look at diagrams (from a ginormous book that he himself had co-authored).

Dr. Nyberg explained that the misshapen tricuspid valve in Pedro’s heart appeared to be similar to a congenital heart defect called Ebstein’s Anomaly.  He was quick to say that if it was indeed Ebstein’s, it was a super mild (okay, he didn’t say “super”) case of it—so much so that he was not even comfortable diagnosing it as Ebstein’s at that point. He recommended that we look at it again in few weeks to see how it would develop. He told me not to research Ebstein’s yet, because it would just scare me and would not be applicable to Pedro since his funky valve was only mildly funky.

I tried not to be scared—but they aren’t kidding about those pregnancy hormones!

We started praying more regularly for his heart to be strong, and it took me awhile not to chuckle when I would say, “…bless his heart…” in my prayers. I guess that comes from spending a lot of time in the South—which conditions a person to expect some kind of disparaging remark immediately after such a phrase. (i.e. Bless her heart…Jessica is a lot  of things, but coordinated is NOT one of them.)