Update: Sheridan's Heart

First, I want to thank everyone who sent us comments, well wishes, and information about their experiences. My family appreciates the support.

Second, given the technical nature of some of the following info, the post is a bit lengthy and I tried to provide pics where I could...

So, I'll get to the bottom line first, then the explaining of the various items I discussed with the cardiologist (nothing is ever black and white, right?).

Bottom line: Sheridan's heart is NOT enlarged, and his heart has not changed since the last echo in February. Hurray!

We're not out of the woods with his heart, but I was so relieved to this latest news. I think I squeezed Sheridan a little too tight when I hugged him after hearing the news.

So here is what I learned:

1. The x-ray image was likely taken at the very moment Sheridan exhaled. Remember little ones are put into a contraption that defies all things decent just to keep them in place and as still as possible. One problem: you can't make babies hold their breaths on cue for any amount of predictable time. The outcome: they breath in and out when their brains tell them to and the x-ray tech is simply not privy to that information. Thus, the x-ray tech pushed the button and Sheridan exhaled and - wallah - enlarged heart on the x-ray.

2. The echo revealed the following about his various heart defects:

The PDA and PFO are still small (again, no real change since February). The cardiologist explained he has, on a few occasions, seen a PDA repair itself after a few months and even after about one year. Key words: on a few occasions. Meaning: it's rare, not likely, and realistically it will require surgical intervention. More on that in a minute.

The VSDs...

Again, not much has changed with the VSDs. It is definitely looking like 2 relatively small holes (the February echo was the first image where the doc saw two holes separated with a bit of "heart wall" between them). One is about 1.8mm, the other around 3mm, and they are separated by a part of the wall between the two ventricles that the doc said is likely thinner than a piece of paper.

To help illustrate the rest of the conversation/information, here's the picture the doc drew for me on the exam table's paperliner (you know the long strip of stuff that looks and feels like a toilette seat cover?). Very little of the paper was left unscathed by Sheridan (he loves to pull it, wrinkle it, play peek-a-boo with it). So, I was lucky to get away with this pic (which, of course, he desperately wanted to claw at):


The two areas above that I circled in blue are the VSDs in the wall separating Sheridan's right ventricle (RV) and left ventricle (LV). The arrow shows the direction the blood is flowing through the hole (blood is flowing from his LV to RV, which in a healthy heart wouldn't happen).

The spot I circled below in orange is the paper-thin wall the doc mentioned.


So, it's possible that at birth, Sheridan had two VSDs with this "paper" wall and they just didn't see it, or it's possible that his body is generating tissue to close the one big hole and now we have two holes.

Also, the "windsock tissue" I circled in green below might be tissue that Sheridan is generating to help close the bottom VSD (or the tissue might have been there at birth).


3. The cardiologist's advice: continue our wait-and-see approach. In other words, continue on our current course of careful monitoring (he now sees Sheridan roughly every 3 months), and do no intervention right now. His advice is based on two really important factors:

First, Sheridan is eating and growing well. He is not what docs call "failure to thrive." If his heart was causing him trouble, he wouldn't eat very well and he wouldn't be chubbin' up.

Second, the pressure gradient is what it should be. Sorry, folks, I tried to find a simple definition online to link to that term, but none exist without making you read a medical journal article. So, bottom line, when a hole exists between the two ventricles it causes a certain amount of pressure. If the pressure is too great, it causes big problems for the pulmonary system. Hopefully that explanation helped?

Also, the doc mentioned that if Sheridan had a leak in his aortic valve (circled in red below), he would schedule him for surgery ASAP. But Sheridan has no such leak, thank goodness.


3. The cardiologist said that if we wanted, Sheridan could have heart surgery now. He always says, "You're my boss." He indicated that if the holes were all fixed, some of the respiratory issues might very well go away, but then again, they might not. One thing would be certain, his heart would be fixed and we wouldn't have to deal with any adverse effects (way down the line when Sheridan is older) of keeping them open to wait and see if they'll fix themselves.

He offered to take Sheridan's case to the cardiac surgeons' meeting to see what they say. But, c'mon, let's be honest... they're surgeons. Of course they will say "let's operate." These are all fixable holes with open heart surgery.

The VSDs would require open heart surgery, and the other holes would be fixed at the same time. However, if the VSDs close on their own over time, the other holes can be repaired with a catheter (as minimally invasive as heart surgery will ever get).

4. As good as all this news sounds, the key concern I brought up with the doc is this: I know that, by definition, the presence of these congenital heart defects - and the longer they remain open - means that Sheridan will likely face heart issues when gets older.

So, it's a balancing act. How long do we wait to see if the holes close, knowing that the longer we wait the more likely the holes will have long-term health implications? What is the threshold at which we say, "okay, it's time to close 'em up."

The cardiologist agreed that it is a balancing act, but said that Sheridan's holes are so small right now (and, again, not causing him any problems), that we're safe waiting for now. Close monitoring will help us determine when the balance has shifted in the favor of surgery.

5. The cardiologist noted, given everything I laid out above, that all of his heart defects taken together cannot explain the respiratory issues. In other words, if we were to take the heart issues off the table, Sheridan would likely still have the same respiratory issues. Of course, he ran down a list of reasons why this might be (e.g., people with Ds have shorter airways because the center of their faces are pushed back a bit).

So, all in all, Gary and I talked it over. It was actually a very short conversation because we thought our course of action was clear... we plan to monitor everything carefully and wait and see if our little big man can work on his own do-it-yourself repairs.

Now if we could just get information about his lungs from the pulmonologist... it never ends. :)

Sheridan's Heart

A few weeks ago Sheridan had a few days where his breathing was very labored and he was wheezing. He has always been very healthy, and Gary and I were certain (as was his pediatrician) that this was due to allergies (e.g., he didn't have a fever, his nose was running clear). We treated him for asthmatic symptoms (he does not actually have asthma) to help him breathe better. At that time, Sheridan was referred to a pulmonologist.

The pulmonologist ordered a chest x-ray to make sure everything was clear, and asked us to monitor his resting (i.e., sleeping) respiratory rate every day over the next month. Also, he prescribed a breathing treatment (Xopinex) so that if Sheridan had another "episode" of wheezing, we could use it to open his airways. We never needed it.

So off to the x-ray place we go. At 7:30am to make sure we don't have to wait in some hellacious long line in the middle of the day. Besides, Sheridan is always bright and cheery in the early morning so I figured that would work in our favor.

They put my baby in what looked like a torture device (click here to see an example of the device in use), and told me to hold his arms up over his head. I thought for sure Sheridan was going to lose it. And he did, but just a little bit. Even the x-ray tech was impressed with how he handled it (and I knew Sheridan had done a great job when, on our way out, the front-desk staff asked when we were going to go in for the x-ray - they hadn't heard the typical screams that come out of that room apparently).

THEN I GET THE CALL

It is never a good sign when a doctor calls you about a test. Ever. They only call when something is "concerning" (a.k.a. potentially bad news). Otherwise they just send you a letter in the mail saying your test was normal.

Turns out the x-ray showed that Sheridan's heart is enlarged ("mildly" enlarged was the word used), and his upper-right lobe of his lung is plugged up with mucus (so it is essentially collapsed and not working well).

So, for his lungs, he was put on the breathing treatment 3 times a day for 7 days. The first set of directions were given was "4 puffs" of the breathing treatment (it's medicine placed in a nebulizer and you put a mask up to the child's face so s/he can breathe in the misted meds). The infant mask is made to look like a dinosaur. Cute, right? Not according to Sheridan. He screamed his flippin' head off. It took two people to give him the breathing treatments. One to hold him down and one to hold the mask. Then the pulmonologist clarifies that I received the wrong instructions...

Each breathing treatment should be 10-15 minutes long.

Say what now? They clearly have never had to give a breathing treatment that long to a terrified, screaming child. Ok. So, maybe they have (that is their job afterall). But I felt so bad for Sheridan - I was nearly in tears when I gave him four puffs because it freaked him out so bad. And now I have to torture him for up to 15 minutes?!

But I sucked it up, started singing, let him see the dino right before turning on the machine, and just did it. The first time he fussed for about a minute, and then he calmed down and spent the next 14 mintues looking into my eyes calmly as I sang to him. What a good boy. And he's never fussed since. When we sit to do a treatment, he just opens his mouth and leans forward slightly to help us hold the mask in place. What a sweetheart.


Also, we have to do chest percussions after each treatment. Today is the seventh day and tomorrow or Friday I will take him in for another x-ray (the hope is that the breathing treatment - which helps open his lungs - and the percussions - which should help manually breakup the mucus - will have done their job and his lung will be clear). I hope so...

For his heart, we go the cardiologist on Thursday for an echocardiogram. His heart is most concerning to me...

Sheridan was born with 3-4 small holes in his heart: a small patent foramen ovale (or PFO), a small patent ductus arteriosus (or PDA), and one or two small ventricular septal defects (or VSDs). Until now, his heart has given him no trouble. Meaning, he's eating, gaining weight, etc. Everything a cardiologist wants to see - if his heart was impacting him negatively, Sheridan wouldn't be doing those things very well. Also, as of his last echo in February, his heart showed no signs of enlargement (and enlargement can potentially lead to lots of issues down the line).

Now all of a sudden it's enlarged??? I'm hoping the x-ray is not accurate (the cardiologist said it is a good tool for diagnosing heart enlargement, but it is not nearly as precise as an echo). If it is, I'm sure we'll still do the "wait and see" game - so far each of the holes has gotten smaller since birth. Which is why I don't understand how his heart can be enlarged now.

In the meantime, what information I have is this: there is no telling what caused his lung to clog up (it is common for children with Down syndrome to have a hard time expelling anything from their lungs - low tone just makes their coughs less effective - so if Sheridan's allergies settled into his lungs, he might just not have gotten everything out). That might be what caused the wheezing. Or, if his heart is enlarged, it can cause the respiratory issues (the wheezing and working harder to breathe, not the clogged lung).

I'm just eager for Thursday's appointment.

So, for now, I hold my sweet, sweet son while he patiently does his breathing treatment and hope that his heart is ok.