Heart Repair

First things first- The surgery is over, and it went very well. Sean is recovering very nicely. We could not be more pleased with his progress. Now- how we got to this point:
We drove back up to Philadelphia Thursday

and spent the night at the Philadelphia Ronald McDonald house. What a wonderful program they run! We had called the day of arrival, as instructed, to see if they had any open rooms, and we were incredibly fortunate to get the last room available. It’s a simple room in the “Carriage House” section, with the bathroom located across the hall. Upon check-in, Sean was able to choose a book, a stuffed animal, and a toy from a huge bin of donated items; after he found a “talking” Thomas-The-Tank-Engine book, he never made it to the toys and stuffed animals… ‘peep-peep’ was all he wanted to hear. (Again, and again, and again, and over again...) We were then given a mini tour of the house. We saw where the kitchen is, with its fully stocked pantry, and where a donated dinner is provided each evening. There are also play rooms, a playground, lounges, and laundry areas. And there is a model train set up in the dining room area that Sean fell in love with....

he was so sad when we finally made him say goodnight to the train so we could all go to bed.
The house is mainly run by volunteers, and only charges $15/night for each family. You are welcome to stay as long as you need, until the day your child is discharged from the hospital. This particular Ronald McDonald House was the first ever, and is housed in a former mansion that has had several additions over the years. The mansion area is gorgeous, and if I ever get a chance, I’d love to take a more detailed tour (Michael did). But I digress... we headed to our room and pretty much went straight to bed. We needed to get up at four-thirty and we knew we would need our sleep for the next day. I’m sure I did sleep for an hour here and there, but it felt as though I simply watched the clock all night long. Sean was snuggled in between Michael and I (a very rare treat for him), and he slept very peacefully.

Since Sean couldn’t have anything to eat or drink after 11:00 pm, we let him sleep until it was time to go, leaving a few extra minutes to visit the train downstairs before leaving.

When we got to the hospital (a few blocks away), we signed in and were taken back to a room where Sean changed into a hospital gown and was given a sedative to help relax him and eventually make him sleep. It worked like a charm. Around 7:30, we met with the anesthesiologist and then followed her and Sean (asleep in his rolling bed) down the hallway to the outside of the operating rooms. We gave him a kiss, and off they went. I can honestly say at that point, I have never been more nervous in my life. I knew he was in good hands, but still, handing your child over to strangers for heart surgery is terrifying. As Sean went off to be prepped for surgery, we were taken up to Dr. Spray (the surgeon)’s office to meet with him. We had been told he is a “tall man of very few words”, and that pretty much sums it up. No idle chit-chat, there's work to be done; a heart that needs fixing. He explained the surgery and the risks (so many! becoming a paraplegic?!), asked if we had any questions (we didn’t), then off he went to get scrubbed up. We were then taken on a tour of the cardiac floor where Sean would come after surgery, and finally, we were shown to the family lounge just outside the area where Sean would be recovering (also sponsored by Ronald McDonald). The nurse promised hourly updates. By 9:30, we were told that the repair was done and they were closing him up, and by 10:00 we were told and we could go to the consult room to meet with Dr. Spray again. He told us that the surgery went very well, and after a brief visit back in the lounge, we were told we could go to Sean’s CICU (cardiac intensive care unit) room to see him. All cardiac patients go directly to the CICU following surgery. Sean was sleeping peacefully when we came in.

He was hooked up to lots of different tubes and machines (a chest drainage tube, one arterial & two vein IV lines, EKG leads, a blood pressure cuff, and oxygen). But he had good color. He would half open his eyes every now and again, but he was pretty much out of it. Until the sedative meds wore off. Then he raised hell. He was already on morphine for pain, but after he ripped out one of his IVs, the nurse (who’d been with Sean since before surgery) gave him “anti-anxiety” medicine (a sedative). That chilled him out for awhile. He went through a cycle where he would rest peacefully, but then freak-out every 2-3 hours when his pain meds wore off. Sean’s surgery required a thoracotomy, which means that instead of accessing the heart from chest, they access it from his side under his arm and around to his back. From what the doctors & nurses have told us, this is one of the more painful surgeries you can have because they have to cut through so much muscle and tissue). So he was pretty out of it the first day. By Saturday, he was off of the supplemental oxygen and was able to go a little longer between morphine doses. By afternoon, they backed off on the sedative as well. By evening, Sean was able to stand, but not really walk around. He also started to eat a little more, but was still on IV fluids because we could not get him to drink very much. He had a good night, and by Sunday morning, he was up and walking around. His full appetite was back, and he was hungry! He was (as he should be) incredibly protective of his incision- he would not use his left arm, or even lift it above waist level. Other than that, he was back to his old self. He was up out of bed, singing songs, playing with his trains,


and eating very well. Amazing. In fact, we had to settle him down a few times because he'd get excited playing with trains and his heart rate would shoot up to almost 180 beats per minute... a little time-out with a train book calmed him down, then back to playing.

At this point it was time to transfer him from the CICU to the CCU (cardiac care unit). So Sunday afternoon, we moved down the hall, and Sean continues to do well, improving almost hourly, eating and playing:


The only concern right now is that his blood pressure is still high. This is not uncommon after a coarctation repair. Basically, his body has had high blood pressure due to the narrow part of the aorta. Once that was cut out, his body is in a bit of shock and overcompensates by raising the blood pressure. This should regulate itself over time (weeks to months), but for now, he has to take blood pressure medication. They are now working on fine-tuning the dosage. Once that is figured out, I think we will get the all clear to go home! Sean has been an amazing little trooper through all of this. He has remained in good spirits through all of the poking and prodding and pain. And the nursing staff here at CHOP has been fantastic. They have all fallen in love with Sean, and work very hard to keep him happy. We could not be more pleased. God heard all of our prayers, and has taken very good care of our little man. For that, I am forever grateful.

Surgery Update

I will give more details later, but I just want to let everyone know that the surgery went very well and Sean is recovering very nicely. He is in a good bit of pain, but the pain meds are helping with that. He's in good enough spirits to flirt with the nurses now and then ;-). Thank you for your continued prayers and support!

Minor Setback

So we packed up and drove 2 hours to Philadelphia Wednesday. Sean had his pre-op appointment where they took an x-ray, took his blood pressure, and took some blood. Then they told us that due to some emergency surgeries that had come up, Sean's surgery had to be moved to Friday. So after a lot of deep breaths, (and a few choice words), we got back in the truck, and headed back home for the night. A word of advice- don't try to leave Philadelphia at 5:00. Especially in the rain. Everyone else is trying to leave, too. Over three and a half hours later, we were exhausted, but safe at home. Unless we hear otherwise, Sean is scheduled to arrive at the hospital at 6:00 am tomorrow (Friday) morning. We will drive back up tonight to spend the night so we won't have to get up so early (and sit in traffic with the same people who were trying to leave last night). I'll keep you posted if there are any changes. On a good note, we have another full day to send loving prayers Sean's way. And I send a prayer to the children who had to have the emergency surgeries, and their families, too.

A Broken Heart

**Sorry for the long post- there is a lot of important info to share**





I’m waaay behind on blog posts right now. I promise I eventually will cover everything that has happened in the past few weeks; somehow I lost the entire month of August! The last thing I remember, it was July- now here we are in mid-September. How did that happen? Actually, you are about to find out- but let me first apologize. Some of you know about this, some of you don’t. I haven’t been very good about keeping track of who I’ve talked to and what I’ve told them. So here is the whole story from the beginning. Back in the Spring (it was either April or May), Sean had croup. Twice. The second time, when Michael took him to Nighttime Pediatrics, as the doctor was listening to his chest, he off-handedly mentioned that he could hear Sean’s heart murmur. Michael was a little surprised by this, considering that we didn’t know Sean had a heart murmur. The doctor assumed we knew about it, and then suggested that we make an appointment with our regular pediatrician to have it checked out.

I took Sean to his regular pediatrician the next morning, and sure enough, he heard it, too. He said there are many “innocent” heart murmurs, but that this one sounded like it was in a different area of the heart. He referred Sean to Dr. Susan Cummings, a Pediatric Cardiologist at Children’s National Medical Center (Washington, D.C.) for further testing. As with many specialists, there was a two month wait for an appointment.

Two months later (the end of July), Sean met Dr. Cummings. She did an exam and also ordered an echo-cardiogram, which is a sonogram of the heart. This involves Sean lying still on a table for almost an hour. Luckily, they had a small TV nearby with a Thomas DVD playing, and that made life a lot easier. Based on this echo-cardiogram, the doctor diagnosed three separate heart defects. They are all congenital, meaning he was born with them. The first is what is called a Coarctation of the Aorta. Sean’s aorta narrows in the section that goes to his lower body, leaving the blood pressure in his upper part of his body about 30mm higher than in the lower part. A simple procedure to indicate this condition is to take his blood pressure in both his arm and his leg. The numbers should be similar; his are 30 mm different. So Sean’s heart has to work harder to push his blood through his body. This extra effort has resulted in some thickening of his heart walls. The heart is a muscle, and like other muscles, when you stress it, it grows bigger. While this seems like a good thing (hey, you work-out to get big muscles, right?) in the case of the heart, it’s not a good thing.

The second defect that the echo-cardiogram showed is mitral valve stenosis (or narrowing), and “regurgitation”. Normally, blood flows through the mitral valve in only one direction. In Sean, some blood is regurgitated (leaks) backwards into the left atrium with each beat of the heart. At least some of this is a result of high blood pressure caused by the coarctation; it will be re-evaluated after the coarctation is corrected. Regardless, the stenosis will remain.

Finally, the echo showed a bicuspid aortic valve. The aortic valve is supposed to have three sections, but two of Sean’s are slightly fused together. The doctor explained that Sean’s first step will be to correct the coarctation. The hope is that by fixing that, some of the problems with the mitral valve will be relieved, and the thickening will subside.

Dr. Cummings said Sean would need to have a procedure done- probably a catheterization/balloon angioplasty procedure, to open up the narrow part of the aorta. She referred us to Dr. Slack, who also works at Children’s National Hospital. We were able to get an appointment for the next week. Dr. Slack was very nice, and felt that in addition to doing the balloon angioplasty, Sean would need a stent in the aorta to keep it dilated (held open). Unfortunately, since the stent is made of metal, it will not grow with Sean’s aorta. As Sean’s heart grows, the procedure will need to be repeated at least two more times in order to continue to widen the stent to the proper size (probably at about ages 12 & 18). The idea of the stent made us a little nervous, so I spent many hours on the phone setting up appointments with Johns Hopkins (Baltimore) and Children’s Hospital of Philadelphia, PA (CHOP) to get second and third opinions.

There are two different approaches to correcting a coarctation. One approach is the catheterization I discussed above. This is performed by cardiologists called Interventionalists. They insert a catheter with a balloon attachment into the upper leg and feed it through to the heart. When the balloon reaches the narrow part of the aorta, it is dilated to open the area up. At this point, the doctor either inserts a stent to hold the aorta open, or they just remove the balloon and the tissues of the aorta stay stretched to the proper size. The other approach is corrective surgery. This is performed by cardiac surgeons. During this procedure, the surgeon accesses the aorta through an incision on the patient’s side under the arm. The surgeon cuts out the narrow part of the aorta, and then stitches the two remaining ends back together.

Two procedures, same outcome: blood-flow through the aorta is normal. One procedure is more invasive, the other needs additional procedures to be done in the future. Dr. Slack was very honest with us; he explained that he is an Interventionalist, and his specialty is catheterization. That is the procedure he recommends because that is what he does. We were eager to see what the other doctors had to say. Both Hopkins and CHOP were eager to get us in as soon as possible for evaluation. The nurse liaison at CHOP was incredibly knowledgeable and informative, and very helpful through the whole process, but due to scheduling, our next appointment was at Johns Hopkins to meet with Dr. Brenner. Dr. Brenner is a pediatric cardiologist; he does not perform either of the procedures, but, like Dr. Cummings, he examines & diagnoses heart defects. (I think at one point he may have performed procedures, but now works as a diagnostician). We had a second echo cardiogram done (before we do anything to our three-year-old’s heart, we want to be sure the first echo caught everything), and it confirmed the findings of the first. Additionally, with the new echo-cardiogram, Dr. Brenner saw a small atrial septum defect (a small hole between the right and left sides of the heart) and mild tricuspid valve regurgitation; as with the other valve defects, these do not need to be addressed at this time. The main focus is still on the coarctation. Dr. Brenner felt that we should opt to do the surgery. It is slightly more risky and much more invasive, but compared to the catheterization where Sean would need at least two more procedures, the surgery is likely to correct the problem with only one. Because of Sean’s age, the chances of having a recoarctation (meaning the corrected aorta narrows again) are very slim. So at this point, we have two different doctors, two different hospitals, two different opinions, and two different procedures. What do you do when you have a tie? You get a tie-breaker. Off to Philadelphia.

We met with an Interventionalist named Dr. Rome. Despite the fact that catheterization is his specialty, Dr. Rome also recommended surgery. Michael and I had been leaning towards that anyway, but it was nice to have some confirmation that we were making the right choice, and Dr. Rome gave us additional arguments in favor of surgery. Now we just needed to choose which hospital. Dr. Brenner from Johns Hopkins was by far one of the nicest doctors I have ever met. His bedside manner made you feel as though he was your father and he was taking care of everything; you knew that you were in the best hands. Unfortunately, as I said, he does not actually perform the procedures. Besides, CHOP has the #2 Pediatric Cardiac Unit in the nation. They literally do twice as many of these procedures each year than Hopkins does. Not too shabby. And luckily, since that is the same hospital where Sean’s lip repair was done, we are pretty familiar with the hospital. So we have chosen CHOP, and Dr. Thomas Spray will be performing the surgery. While we have not met him yet, his qualifications speak volumes. Sean is in good hands.

This Wednesday (the 16th), we will head to Philadelphia for Sean’s pre-op. appointment. Surgery is scheduled for Thursday, September 17. We have been told to expect a 5-7 day hospital stay, possibly longer if there are any complications. We have every reason to believe that surgery will go smoothly, and that Sean will recover nicely. I will do my best to keep everyone informed of his progress. I don’t know what kind of computer access will be available.

So, you can see why the month of August was pretty much a blur. We have sorted through many emotions over the past six weeks- from anger for how this has gone undetected for so long, to sadness of what Sean has to go through, to fear of what could happen… Right now we are in the “we know what is wrong, let’s get it fixed” stage. It is likely Sean will need more procedures in the future to deal with the other defects, but they are at the bottom of the checklist right now. The coarctation needs to be repaired first, and we are eager to get it fixed. Luckily, Sean is young, and doesn’t really know what is going on. He just knows that we go to the doctor’s office a lot. He has been incredibly patient for a three-year-old. Rex is old enough to have some understanding of everything, and we have been very open and honest with him. When I explained to him that parts of Sean’s heart were not working properly and we were going to have to take Sean to the hospital so they could fix it. It didn’t seem to faze him at all. He wanted to know how the parts got broken, and I explained that he was born with his heart this way. A few hours later, he came to me and said, “Mommy, why would God give Sean a broken heart? Isn’t he supposed to be good?” Seriously. It's hard to answer a five-year-old’s question when you can’t even answer it for yourself. I explained to him that everything happens for a reason. We may not always know what that reason is, but God does, and we just have to trust him. That seemed to comfort him for now. We are so blessed to have such wonderful family and friends who have reached out to us with offers to help in every way imaginable, as well as sent us much needed and very much appreciated prayers and good thoughts. Thank you all so much. Sean is a very special little boy, and he is a fighter. (Just ask his brothers…or anyone else who gets in his way...) It's somewhat humorous how the doctors keep asking us if Sean fatigues easily or sleeps a lot, and they reassure us that he’ll have more energy after surgery. HA! Sean has more energy than any six people I know combined. Between his spunk and zest for life, and lots of thoughts and prayers, I know he’s going to be just fine.