Wisdom Heart

I haven't posted a diagram in a while. Sophia's pre-op went good today. Now we just have to get through the weekend with no colds. We talked to the surgeon and I will do my best to recount what he said.

First, the diagram...

1. This is the homograft (human donor tissue). During Sophia's last surgery this conduit (darker blue) was placed from the right ventricle(RV) to the existing pulmonary arteries(PA). Prior to that surgery there was no direct connection from the heart to the pulmonary arteries.

2. This is the pseudoaneurysm (lighter blue) that has formed near the suture line of the conduit mentioned in 1. As you can see it is quite large. However, it should be noted, it has not grown any larger since fall. This is why the surgery was not considered to be emergent.

3. This is an area of stenosis (yellow) in the left pulmonary artery. Sophia's cardiologist will attempt to place a stent here during surgery in order to allow more blood to get to Sophia's lungs and also to relieve pressure on the heart and the new conduit.

4. This is the narrowing Sophia had corrected in the last heart cath (green). I had already posted on the success of this procedure (stretched from 2mm to 11mm). However, it should be noted, Sophia had oxygen saturations of 81 at the pre-op today. Prior to the last cath, you might remember, Sophia had saturations in the 60s.

a. This is the ventricular septal defect (VSD). This will not be repaired during this surgery. It was explained to us by the surgeon that it would create too much pressure in the heart and pulmonary arteries to close it at this time. He explained to us that the pressure in the left side of the heart is slightly higher than it is in the right. So once the left ventricle starts to force oxygenated (red) blood through the VSD into the right ventricle, Sophia's oxygen saturations will actually be too high. When this happens or if the pressures equalize to cause the blood not to mix across the VSD, they will be able to close it. The possibility of closing it when Sophia outgrows the conduit was mentioned.

So here is the plan.
Sophia will be put under using gas. She will get two I.V.s and an arterial line. She will be intubated.
She will be put on heart/lung bypass. They are going to do this via vessels in her neck this time, as they anticipate it will reduce complications caused by scar tissue just below the sternum.
Obviously, this will require the sternum be cut using the same incision line as her previous surgeries. This is called a median sternotomy.
After the scar tissue from previous surgery is resected.
The old RV-to-PA homograft along with the pseudoaneurysm will be removed. At this point, the stent will be placed in the left pulmonary artery. This will be like a cath. Only this time there will be direct access to the area to be stented.
After this, the new RV-to-PA homograft will be put in place.
After everything is put back together, Sophia will be taken off bypass and her heart will be started again.
Her chest will be closed (note:last time her chest wasn't closed until a few days after surgery). She will then go from the OR to the ICU.
I acknowledge there are some pretty big gaps in the continuity of this description. A large part of this whole process still remains magical and mysterious to me. But, I think this adequately explains the procedure in broad brush strokes. I am really not sure if I am able, emotionally, to visualize the surgery with any more detail than this. It is hard enough, as many of you know, to even imagine the incision when it comes to your child's body. Mostly though, knowledge and information helps me feel more comfortable with the whole process. I think that is why it is important for me to communicate so much of the details of all this in this forum. I hope the information is as beneficial for my audience as it is for me.
Now, we wait...

I want to thank the St. Anthony's Altar and Rosary Society (my mom's church). They had a bake sale for Sophia on June 17-18 and raised $717 for us to put toward medical bills. Thanks to everyone that contributed.
I also wanted to mention we took Sophia to see her first puppet show on Wednesday morning. It was based on stories by Rudyard Kipling and was put on by Paul Mesner Puppets.
She had a great time. She actually sat through the entire hour leaning forward in her seat and clapping. Thanks to Chrystal for the tickets. We are definitely going back.
Pre-op is tomorrow. I will try to post afterwards.
Photo Note: Sophia has not yet crawled. But, it should be noted that she now get around quite efficiently by scooting on her butt. Hey...it works for her.

Hi everyone. I know I often get pretty quiet on the blog before we go in the hospital. I will try to throw up a few photo updates this week. I just don't usually feel like writing too much prior to surgery. Thanks for your understanding.

So we have scheduled surgery.

As I explained in a previous post, Sophia has to have the right ventricle-to-pulmonary artery conduit from her last surgery replaced, as it has developed a pseudoaneurysm. This is a outpouching of blood caused by a partial rupture of the interior walls of the PA (pulmonary artery) conduit. There is no bleeding as at least one of the layers of the arterial wall is intact. In images from the cath, the pseudoaneurysm is as large as the ventricle itself. And there is an obvious risk of it rupturing.

During surgery there will also be an attempt to place a stent in the left pulmonary artery, as it is narrowed in the same way the right was prior to its stenting in the cath lab. There is also the possibility there will be a surgical option to repair this narrowing. However, the surgeon and her cardiologist will make the call in the operating room as to which is the best course of action. It should be noted, this surgery will likely leave the VSD intact. So we will still need at least one more major surgery.

Sorry for any confusion caused by my use of technical jargon. I just thought it was important to remind everyone why surgery is necessary at this time. Please, if you have questions, put them in the comments section of this post I will be glad to answer them if I can.

So here are the dates.
On June 30th, we will have Sophia's pre-op screening. This is always a long day for some reason and Sophia almost always gets ill from exposure to all the hospital germs. We are going to do our best to isolate her from any contact with possible causes of illness by keeping her in her stroller and possibly having her wear a mask. We'll see. We have postponed several procedures in the past for this reason. So this will be very important.

Next, on July 4th, we have to take Sophia in to have labs drawn. This is necessary for anesthesia to get a base line of her blood chemistry, I think.

Finally, the actual surgery will be on July 5th. As far as I know, she is the only surgery on the surgeon's schedule. So my guess...it will be a long day.

I will obviously post updates to the blog as I am able.

Here is a quick photo update. Sophia is trying some new snacks. She really likes Veggie Booty. I will post a surgery schedule update very soon.

This picture creeps me out a little bit.

Sophia wore herself out in the wading pool on our front porch. It was a beautiful summer evening in Kansas City.

Sophia loves her veggies.
We have been really conscious of the way we introduce foods to Sophia. As a consequence, Sophia has always eaten really healthy. We rarely give her anything with processed sugar. She eats all organic (with the exception of her formula, which is down to just a few ounces a day) and completely vegetarian. We went to the local organic farmer's market this evening and bought her some fresh produce. We are thinking of signing up for a CSA next year. If you are not familiar with community supported agriculture (CSA), it is basically a share holder system. Your family buys a share of a local farm's crop and you receive a share of the harvest. It seems to me like a good way to get locally grown organic food while supporting your local economy. Plus, we think it will teach Sophia to understand her food comes from a farm rather than a grocery store.
If you are interested we watched a great film on PBS last night that deals with a farmer with a very successful CSA. The film was called "The Real Dirt On Farmer John". It was quite entertaining and inspiring. I highly recommend it.
You can also check out Local Harvest to find farms in your area that offer these types of arrangements.

Sophia and I made the Kansas City Star's webpage of photos from the HeartWalk.

Sophia went to see her cardiologist this morning. She received a good report regarding the stents. Her sats are up and her color is much better.

Unfortunately, Sophia is, just now, getting over her cold/infection. Her cardiologist suspected anesthesiology would want to reschedule. We called the hospital and talked to someone regarding this problem. They confirmed our suspicions and asked we reschedule surgery two weeks after Sophia is symptom free. Our guess is this will be within the next couple of days. So we will likely be having surgery in three weeks to a month.

The reasons they are reluctant to do surgery after a cold or infection are manifold. The main reason being associated with cardiopulmonary bypass. Cardiopulmonary bypass increases the possibility of infection and can result in an inflammatory response. If a bacterial/viral infection is already present in the system, there are obvious risks of post surgical complications of this infection being bolstered by the use of cardiopulmonary bypass. This is obviously too large a risk considering this surgery is somewhat elective. And by elective, I of course do not mean she doesn't need it. I just mean it isn't necessary it be completed immediately.

As many of you know, this type of surgery requires a great deal of mental preparation. As the date grew closer, I became more and more resigned to the inevitability of surgery. And eventually dread was replaced with what could be best described as impatience to reach completion of the surgery. As a result, it became difficult to even consider waiting.
But in the larger view of things this response just points out a possible flaw in my coping mechanism. Perhaps this delay will help me work out whatever obstacles I have to coping. I am very aware of the necessity I deal with this in as healthy a way possible as it will definitely not be the last time we are in such a situation.

The high note is, of course, Sophia is doing much better after her cath. We can only hope surgery will help her progress even further.

We just got home after completing the 2.5 mile American Heart Association Heart Walk here in Kansas City. The weather was nice and many people came out to support the cause. I walked with Sophia on my back in a carrier. Patty, my mother, and many of our friends walked in honor of Sophia.
I thank everyone that supported us and this event. I think Sophia's team alone raised over $1800. Patty and I raised $500.
The larger CHD Families group we walked with raised around $10,000. At last check the walk as a whole had raised about $800,000. But I am sure all those numbers are much higher with donations collected at the walk today.
Overall, it was a great way to spend a Saturday morning. And hopefully it will help further heart research and treatment in some substantial way.
Thanks again for everyone's support.

***LAST CHANCE TO MAKE HEARTWALK DONATIONS***

Thanks to everyone who has already donated. If you would like to support us on the American Heart Association HeartWalk this Saturday, please use one of the links above. We will of course be walking in honor of Sophia. Any help would be greatly appreciated. Thanks-Michael

Note-This post is dated to remain at the top of the list until Saturday.

Thanks to Sarah at Our Little Braveheart for her HeartWalk donation. I really appreciate it.

Thanks to Erika at Snazzykat for supporting us on the heartwalk.
BTW- I have to acknowledge that Sammy's cuteness quotient has gone off the charts. If he gets any cuter he'll be dangerous.
Thanks again-Michael

Went to the pediatrician again today.
Sophia has a slight fever today. So we thought we better see her doctor.
The tube in her right ear is plugged with mucus and she's got some upper respiritory junk.
We are going to do ear drops (Floxin) and a round of antibiotics (Augmentin®).
If she can kick this stuff pretty quick, we shouldn't have to reschedule her surgery on the 15th.

On a side note-
I would like to thank everyone for all their comments. I usually do not have time to respond, but I really appreciate all the support. And a special thanks to Krystal for her HeartWalk donation.

Well, it looks like we brought a friend home from the hospital.
Sophia was up coughing all night and has a runny nose.
She doesn't have a fever, but it is still too early for a secondary infection.
We saw a pediatrician in her doctor's office today. He prescribed tylenol w/codeine. He said it should help her be a little more comfortable and suppress her cough.
We'll see. I hope this doesn't postpone her surgery. But it likely will.

We are finally home. The discharge process took forever.
Her doctor released her first thing this morning. But since he does not have the authority to write orders in this particular hospital, we have to wait for the resident assigned to Sophia to do it. Her cardiologist still directs all her care but through the resident.
It did not help, we had the most incompetent nurse we have ever met. All of our nurses until this morning were perfectly good. But the this morning was bad.
I is my thought, based on the number times we have done this, the degree of care your child requires dictates the competency of the nurse assigned to your child. The best nurses are reserved for the kids that need the most care. I guess this is understandable. But shouldn't they all at least be competent.
Let me explain.
Our nurse went to the wrong wing this morning upon reporting to work. She is called a "floater" and I can understand she may have been confused about where to report. But, not only did she report to the wrong place. She took report on all the kids in the other wing before they went looking for her where she was supposed to be, which was caring for Sophia.
Once she got where she was supposed to be she had to, of course, get Sophia's vitals. This took forever . Now, we have seen this process a multiplicity of times and it should have been a quick process.
Then when we asked for some more diapers for Sophia, she brought us size 1 diapers. Now we have had nurses bring the wrong size diapers before. But, these were newborn diapers.
She also came into our room multiple times looking for stuff she had misplaced.
I don't know what the deal was. I was just glad Sophia didn't really need a nurse this morning or we would have been screwed.
I think it took about 10 requests to get Sophia's IV removed. This, however, could have been because nurses hate to discontinue IVs. I am surprised they have not resorted to having the security desk at the exit of the hospital remove them just before you get to your car. I know it is a lot easier to keep an IV than start another one. But when someone is being discharged, I think its time to lose the IV. Luckily, we mentioned the monitors to her cardiologist this morning and he shut them off. We removed her leads after he was gone.
In the future, I may feel compelled to discontinue her monitors and take out her IVs myself. It was obvious the only reason it hadn't happened was either they hadn't got to it, or the resident hadn't got around to ordering it.
Anyway...I don't mean to paint our stay as negative. They always take good care of us. And we were glad to see the nurses we have had during previous stays. I just think we are used to a higher standard of treatment. The CHD and transplant patients always get special care. But as they get closer to discharge and get shuffled to the bottom of the priority list, I start wondering what an average child's stay must be like.
We are glad to be home.
We go back for the open-heart surgery on the 15th.
But as long as we keep making progress, we can bear the difficultly associated with all of this.
Thanks again for everyone's support.
P.S. I posted some photos on the previous hospital posts form the last couple of days.

Sophia's cardiologist has released her to go home. Now we just have to wait for the resident assigned to her to write the orders and officially release her. Hopefully we will be home in a couple of hours.
Sophia's sats have been about 10% higher since the cath. I saw them as high as 82 this morning. Which, I know doesn't sound particularly high, but she was averaging about 65 ahead of the procedure.
Thanks for everyone's support. It really makes a difference to have the support of family and friends during Sophia's hospitalizations.
It seems as though a little online "family" is emerging and it really feels good to get the support and encouragement of everyone out there who is following Sophia's progress.

Sophia just isn't feeling very well. So we are probably going to stay the night.
Hopefully she will start feeling better soon. I am still waiting to see her sats go higher. I think they will probably settle out at 80 or so.

Sophia is much improved this morning. She just got better last night with a bunch of water and a dose of tylenol. Who knew? They had decided they were going to keep her on oxygen through the night, just to remove that variable from the equation. But by the middle of the night, she had removed her nasal canulla and had sats in the 80s. So they just left it that way. Her face was beet-red when we went to sleep last night and her temperature was just barely elevated. Her chest x-ray and EKG were normal. I think she was just feeling crummy from the anesthesia, but we are not sure. We are going to talk to anesthesia about side effects this morning. We want to see if there is something they can do differently on the 15th.
Sophia looks really good this morning. Hopefully we will be going home today. She is getting the required chest x-ray right now. If it is clear I do not imagine they will keep us.
-Michael

Well, we are in our room now. Since we have been here we have seen a few changes/complications.
Sophia's heart rate has been on the high end of the spectrum since coming from recovery, not dangerously high, but consistently high. Her sats have been quite low as well. She has responded well to oxygen which is unusual for her. I think this is evidence it is respiratory rather than cardiac. Perhaps, she has some fluid in her lungs. I don't know. She just had a EKG and chest x-ray.
At this point we aren't sure what the cause could be. I will keep you posted.
It isn't looking like we are going home anytime soon. We'll see.

The cath went very well. It took most of the day. But for the most part, everything went as planned.
We got to the hospital at 7:30am, checked in at admitting and went straight to the cath lab.
After some preliminary vitals and consent forms, they gave Sophia a dose of Versed. It wasn't long before she was completely snockered. At this point, anesthesia came and got her.
We went to the waiting room and waited. It took quite a long time to get everything in place. Just the anesthesia, IVs, ventilator, etc. took 45 minutes. Then they have to get the catheters started. This time the catheters were not too difficult to get in her veins. In case you are unfamiliar with heart catheterization, they access the heart and its great vessels via the veins in the groin. So form the time they get access until they get the ends of the catheters up to the heart can also take some time. Sophia had 4 catheters, 2 in each leg, I think.
If you remember, the plan was to open up a narrow section of Sophia's right pulmonary artery using two stents placed tandomly where the artery branches toward the right lung. The narrowing was substantial. In fact, it was so narrow it was difficult to get even one of the catheters through it. In order to complete the procedure it required two catheters with balloons be placed in the vessel.
So the doctor first had to widen the vessel in order to make room for the catheters to pass through. He did this by stretching the narrow part of the artery using the balloon on the end of the first catheter. Once he stretched it enough to get both catheters through, he had to place stents over the balloons and position them just right before the balloons would be inflated, expanding the stents.
This was tricky, of course, as was the whole procedure. But here is what made the positioning difficult. The stents were parallel in the vessel. The vessel was stretched just enough to fit the stents into it. Therefore, as one of the stents needed to be shifted forward or back, it would move the other in that same direction.
Anyway, they were eventually positioned properly. This was really the most critical part, as the positioning of the stents was crucial to get the results we wanted without complications. The sizes of stents used were a 5mm and 6mm. If you remember, I had posted the size of the narrow part of the artery was about 2mm (this was confirmed). And the goal was to stretch it to 10-12mm.
The doctor expanded both stents by inflating the balloons at the same time, expanding the stents completely. At this point, one of the balloons burst. This was of course a concern. It was explained to us this morning, if the balloon bursts it can catch on the stent when the catheter is removed dislodging or moving the stent. Fortunately, this did not happen today.
The bottom line, Sophia's right pulmonary artery was stretched from a narrow 2mm to 11mm. This should substantially improve blood flow to the lungs.
There was a little blood in Sophia's breathing tube after the procedure. This was a concern but seems to have mostly resolved at this point. They have given her a large dose of diuretic (lasix) to prevent any fluid collecting in the lung.
The only other thing the doctor did was he got some images of the aorta for the surgeon doing Sophia's next surgery. He thought there was some possibility there could be some collateral pulmonary vessels that could be unifocalized during her next surgery. There did not appear to be any.
We will hopefully be moved from recovery to a regular room as soon as one becomes available.
Thanks for all your support. It means a lot.
-Michael