Fontan Recovery: Pacemaker Surgery Over

Before surgery

The surgery is over. The pacemaker went in without problem. Colin and I were relieved to hear her determined wails down the hallway as we walked back to our room in the ICU to see her.

I will post more later about the details. For now, know that she is okay. Maddie is a brave, strong, wonderful girl. She reminds me every day what courage means.

Thank you all for your prayers and tears and words of encouragement. Your strength lifts us up.

Fontan Recovery: Pacemaker Implantation

Home at the ICU

Home at the ICU

It’s difficult for me to even write these words: Maddie is going back into surgery tomorrow morning. The cardiac team feels that her heart can no longer handle staying in the junctional (JET) rhythm. Her body just can’t seem to resolve it on its own. They’ve decided to go forward with implanting a permanent pacemaker.

Normally, this would involve placing the device in an incision below the breast, but since she has just had open heart surgery and her sternum is not yet fully healed they can re-break it and do a more thorough job of placing the leads with an open chest cavity. This process will make the connection more stable in her body. The device will be placed behind her abdominal muscles.

But the words “re-break the sternum…” They are just too harsh to accept right now. My heart hurts for this little girl who was just beginning to heal. I can’t bear the thought of her going through the nausea, the pain, the anger, the fatigue all over again as she recovers from the anesthesia and surgery.

The risk involved is lower than the last two procedures. I don’t worry as much about mortality. It’s her emotional well-being I fear will suffer. It’s the thought of her sinking into a depression again that made me cry at this news.

Again we are going forward in faith. Again we are asking for prayers and positive energy. Again we are putting our trust in God’s hands, hoping that He knows what’s best for this precious little girl.

Fontan Recovery: Day 12

Yesterday, I was completely dejected.

Maddie is still tachycardic (in a JET rhythm), she may need a permanent pacemaker, is still draining quite a bit from her chest, and has been resistant to anything improving her mood. No toys, games, shows, movies, interactions, not anything has put even a smile on that girl’s face. In short, she was depressed and has been since she woke up from her surgery twelve days ago. It was almost as if she was choosing to be unhappy. Recovery has taken it’s toll on this silly, feisty girl.

Watching your child suffer from pain is unbearable. Watching your child sink into depression is almost worse.

And then a miracle happened. I came to the hospital today to switch Colin and as I pulled into the parking lot, he sent me this picture:

laughing again

Maddie was laughing. Not just a little either.

This image. This image brings me more joy than I can express! My prayers have been that she could find peace and comfort during this process. I feel like those prayers have been answered.

Today, I am grateful. I’m grateful for a nurse that could find ways to interact with Maddie to elicit smiles and laughter. I’m grateful for family members that visit and call regularly to cheer her up. I’m grateful for friends that support us in numerous ways. I’m grateful for a little girl who fights and doesn’t give up.

She’s still tachycardic. She’s still in the ICU. She still has a long way to go. But today I am focusing on the good.

And this smile is good enough for me.

It Takes a Village…

neighbors

When we bought the Pig House, we had no idea how much of a blessing that decision would become. You can view the home’s structure and the landscaping and even the layout of the street before you close the deal, but there’s no way to really know what your neighborhood will actually feel like.

It took me most of the winter and even the early spring of last year to work through the grief of leaving our last home, but when I did, I emerged to find a set of neighbors that I now call friends.

Our children feel at home not only in our house but on the entire street.

Light Sabers on the Street

There are kids everywhere. They pour out onto the street every afternoon as soon as school is done. In the summer, they hardly go inside at all. Age makes no difference. The older ones look out for the younger ones and the little ones look up to the older kids. They are creative and active and enjoy running around exploring the world together. When squabbles arise, they work it out themselves.

I’ve never known all of my neighbors before. It’s both a foreign feeling and one so easy that I can’t believe it’s taken us this long to find such a sense of community. One neighbor opens her garage to the best playroom you can imagine. Another neighbor holds weekly Friday/Saturday night garage movies. Others let Jake and Joe follow them around in their gardens, “helping” them water and dig up worms. Conversations are frequent and easy and there always seems to be some excuse for a party.

Labor Day BBQ

When we told them all that Maddie would need this surgery, every single one of them immediately offered help. They’ve provided meals, babysat the boys, come to visit in the hospital, given numerous gifts, prayed for us, and have otherwise been an unbelievable source of support.

Warren, Jake, and Joey are feeling the stress of this situation and miss both Colin and me when we are gone but they are able to find peace in the normalcy of life on our street. Our good neighbors have looked out for them and cared for them throughout these last few days and everyday. We are so grateful to know that they are happiest when they are home.

“It takes a village to raise a child,” the old adage says. Or four, in our case. And we couldn’t have found a better one to raise ours.

Fontan Recovery: Day 8

Peaceful

NEWS FLASH: Maddie willingly took her medications today! Fervent, heart-felt prayers work, folks. How else can you explain being able to finally reason with this stubborn girl??

Her pain finally got the best of her late last night and early this morning, so not only did she willingly swallow the absolutely necessary medications but she also took the oxycodone pain reliever, another one she had refused and puked up earlier this week. Doesn’t she look more peaceful in the picture above?

Today has been so much better all around. She’s still on the pacer and still has underlying junctional ectopic tachycardia (JET) rhythms in her heartbeat, but they’re hoping those are starting to resolve now. Her potassium levels are up and she’s eating and drinking like a champ. She’s sitting up more and starting to use her right hand to feed herself.

working with dad

Our friends and awesome neighbors, plus Grandma Tuki and Aunt Melissa, came to visit today. We celebrated her victory over the oral meds. We cheered her bravery. Different neighbors stopped by last week (and brought me dinner!) as well other friends over the last few days. These visits remind her that people haven’t forgotten about her. Even though she’s not responsive when they come, I know it lifts her spirits. I know they’ve lifted mine.

She’s “blown out” all of her peripheral IVs and her arterial line which is awful but overall alright because she doesn’t need them for medications anymore. However, she may have had some infiltration, which means that the fluid leaked out of the vein and infused into the surrounding tissue. Not good. Her left arm and left leg (where former IV lines were placed) are swollen, tender, and causing her a fair amount of pain. Poor, poor peanut.

Despite this, she’s still progressing. We’re hoping that her left and right drainage tubes will come out after the weekend. Hopefully, she’ll be moved to the regular recovery floor soon after. Looks like our Friday goals are being met.

Check, check, and check!

Fontan Recovery: Day 7

Spa day

It’s been one full week since Maddie’s surgery.

So much has happened since last Thursday and at the same time we’ve barely inched along. The past two days since her heart catheterization and reopening of the fenestration have been good because her heart is beating so much better.  But they’ve also been bad because she’s becoming unreasonable about taking medications.

Once she came back to the ICU from the cath lab there was a noticeable change . You could tell immediately that she was improving. She’s been eating and drinking ever since, but she’s low in potassium because of all the pre-procedure fasting and days of barely eating from nausea while she was tachycardic. We’ve spent the last 48 hours trying to get her to drink Pedialyte, Vitamin Water, eat bananas, and anything else we could think of to increase her K levels.

She’s still in the ICU. There are two medications she’s receiving through IV that need to be taken orally before they can move her to the regular floor. She struggles taking anything that might remotely taste bad so it has been a full-time job trying to cajole her into cooperating.

But all that effort to eat seemed lost when we had to force her to take the two required meds by mouth this afternoon. She got so worked up about it that she let her gag reflex take over and she puked the entire contents of her stomach. The picture above is post puke fest. She has a no-water-necessary shampoo cap on her head to get the chunks out as best as possible. It’s just as fun as it sounds.

We’re all fed up. We’re all exhausted. We all want to go home.

Tomorrow is another day. Less drainage, higher potassium, better oral medication consumption. These are all great goals for a Friday in the ICU.

As ever, we are grateful for the many friends and family who continue to offer up words of encouragement, prayers, and hours of tireless service. Slow progress or not, we are truly blessed.

Heart Catheterization Successful

heart cath success

The surgeon just came up to tell us that Maddie’s heart cath went well. They were able to open up the fenestration and place a stent to prevent it from closing again. Only one clot was spotted in the Fontan circuit and it did not dislodge during the procedure.

This is very good news.

The pressure should now begin to decrease and the drainage should slow. But all this has set her back considerably. We are in this for the long haul. It will probably be another 3-4 weeks before she is fully recovered.

She hasn’t returned to the ICU yet. She’s getting a few more things done: her mediastinal drainage (the tube you see in the middle of her chest) slowed enough to pull it out, and we requested that they place a PICC line (peripherally inserted central catheter) in since she’s going to be here for so long and she constantly “drops” IVs because of her small veins. (That way they don’t have to continually poke her to start a new IV, a very traumatic process for little kids.)

Thank you. Thank you all so much for your heartfelt prayers, love, and concern. We are so grateful to know that you are supporting us.

More updates to come…

Fontan Recovery: Heart Catheterization

Sister and brother

Days 4 and 5 have not been very good.

Maddie’s heart rate has been consistently high over the last few days. Her tachycardia, arrhythmia, and the continual drainage from her lungs have led the cardiologists to believe that something is obstructing the Fontan system.

There are three possible reasons for this blockage: 1) A closed fenestration, 2) a blood clot, or 3) the new Fontan hardware could have been pinched during the final closure of her chest cavity.

The fenestration, or hole placed in the new tube they engineered next to the atrium, is possibly closed. The hole acts as a release if the pressure from the fluid change becomes too high. All signs point to closure as a first cause.

Another reason could be a blood clot has formed somewhere in the Fontan circuit. This, of course, is a particularly problematic situation. Clots travel through the circulatory system and can end up in the brain causing a stroke. Blood flow through the pulmonary veins show that this case is unlikely the cause right now.

The final possibility, that her tubes have been pinched, is the worst because it will require surgery to repair. The likelihood of this scenario is far less than the others.

She is absolutely miserable. She’s sweating but has cold extremities and poor profusion.  Her vascular system is dry in that the output of fluid through the lungs is greater than the amount of hydration going in or being replenished. Overall, she is not progressing.

Today we were informed that a heart catheterization would be necessary to go in and examine the Fontan and the fenestration to see if the hole needs to be reopened and widened. That is the easiest and most likely scenario and solution.

She is going in sometime in the next half hour (5:30PM Pacific) and will be in the cath lab for up to 3 hours. Of course she will be fully sedated and will come back into the ICU for recovery.

I know this post is extremely technical but I wanted to write this all down to work it through in my own mind. Hopefully, you’ll get a sense of where she is at, clinically speaking.

She has had heart caths before but this time the risk is increased because of the higher potential for dislodging a clot somewhere in the Fontan system. The 5-10% chance of stroke is weighing on my heart and mind so greatly. We’re asking, once again, for your faith, prayers, and positive energy.

We will post more once she is back in the ICU.

 

Fontan Recovery: Day 3

The lonely walk to the Forest Wing

The lonely walk to the Forest Wing

PHOTO CREDIT: Colin

Seeing your child suffer is probably the worst thing you can experience. Especially when there is nothing you can do but give it time. Maddie is in pain. She’s trapped in a hospital bed with tubes attached at every which point of her body. She desperately wants to go home but the end isn’t even in sight.

If you couldn’t wade through the technical post from yesterday, here is the gist: Maddie’s fontan restructuring has created a ton of extra fluid in her lungs caused by the increase of pressure. Messing around with her heart in surgery (and the blood transfusion she required in procedure) doesn’t help with her body’s normal electrical currents for blood flow. Subsequently, she’s dependent on an external pace maker to regulate normal heart beats and three different tubes coming out of her chest cavity to allow the extra fluid to drain.

None of these adjustments feel good. In fact, they make her quite miserable.

This poor little girl. Today was rough. This morning she screamed out in frustration, using the last of her strength. After that, it was a silent protest of listlessness, lethargy, and general sadness. She is already fed up with being at the hospital and we’re only on Day 3.

She continues to make small strides. Today she kept all her food down and used the bathroom completely. They keep trying to remove the pacer to allow her heart to beat normally on its own but it always ends up plugged back in after she becomes tachycardic again.

And as we suspected would happen, the extra fluid from her chest continues to drain and drain and drain. Pleural effusions, or the fluid buildup in the lungs, are common and expected after Fontan surgery, but because her Fontan is more complex, thanks to her dextrocardia and other abnormalities in her anatomy, the output is greater than they want to see 3 days out.

So, if you’re looking for specific items to pray for to help her, here they are:

  • Maddie’s heart will beat regularly on its own
  • The extra fluid or pleural effusions will dry up quickly
  • She will be comforted and feel peace until it’s time to go home

As soon as her heart can beat without irregularity, she can at least leave the ICU. She’s checked some good things off her recovery list. Let’s hope these next goals get met soon.

As always, we are thankful for your love, support, help, and prayers. They are what keep us going.

Geeking Out on the Fontan: A technical discussion between brothers

Signed heart pillow

A conversation started between Colin (software engineer) and his brother Jared (dental Ph.D.) when Colin described Maddie’s Fontan procedure and its findings over email. These two brilliant guys went on to discuss the benefits of one method over another using some articles* that detailed the debate. Did I mention neither one is a cardiologist?

Some of you have asked what the surgery involved this time around. Here is the dialogue that ensued between the brothers. This is more technical than you would ever need as a layperson but I love it and am always eager to learn more. Feel free to “geek out” on the fascinating medical technology with us.

Dr. Wells (the surgeon who performed her first two surgeries) left her in good condition. Dr. Chen was able to follow what he had done and had no complications due to previous surgeries. He was able to widen the pulmonary vein somewhat, but the entire system is stenotic. He was not concerned about the stenosis as the pressure differentials were equal across both lungs. Instead of passing the Fontan through the atrium, due to the location of confluence of her major veins, they needed to have the Fontan go around the heart.

Post operation, her oxygen saturation was 97%. Pre-operation, her saturation was 83%.

-Colin

===

One is a study¹, the other is a commentary² on the study from a cardiologist/electrophysiologist perspective. The study looks at the arrhythmia risks down the road of the different variations in fontan. The extracardiac conduit appears to have the best results a decade out relative to atrial arrhythmias.

-jared

===

Thanks. I read both, now I just need to find out if they did an extracardiac lateral tunnel or an extracardiac conduit.

-Colin

===

I read these two papers from both sides of the argument for and against extracardiac conduit placement. The decision was made in the moment by the surgeon, so reading these is obviously not for reaching a decision, but they do highlight the debate in the field concerning the long-term issues. I haven’t had a chance to follow up on the additional heterotaxy-based literature, which will be more pertinent to Maddie’s case.

-jared

===

A quick read. :) Maddie has a fenestrated extracardiac fontan which, according to the article, is the optimal extracardiac fontan in case they need to treat arrythmia through a catheterization procedure. The fenestration allows them access to the atrium. Now that I have a tiny background, I’m more curious about the exact anatomy. Specifically the orientation of her heart in relation to the fontan hardware as well as the relative position of the fontan hardware and glenn hardware as they enter the pulmonary artery. Also, does the pulmonary artery have to be disconnected from the “right” ventricle for this whole thing to work? It seems that there would no longer be any blood flow through the right structures and, if so, it would be oxygenated blood only except what small amount comes through the fenestration.

Now I’m hooked. I guess I just like engineering problems no matter what shape they take. Today I redesigned the bed to solve the problem of cord and tube entanglement, I helped the nurse better understand and utilize the vital statistics monitor UI, and I helped two men find their way through the hospital.

-Colin

===

Nice work, you sound like Doctor Who.

[The girls] and I sat and discussed all the tubes, needles, and bands in the picture you posted of Maddie. We tried to figure out why everything was in place. We look forward to seeing her up and moving around again, and hopefully soon enjoying some chocolate milk.

-jared

===

She’s past chocolate milk and moved on to soda. She had some sprite last night and kept it down while soaking in the wonders of original Scooby-Doo episodes. She’s off her pacer as of this morning. She is starting to feel a little homesick today though. If you guys have questions about any of the tubes, let me know. I know most of them now.

To add upon my education, I spoke with Maddie’s cardiologist last night. Some things weren’t making sense and Dr. Meg (her cardiologist) loves to geek out with me about her anatomy, so I got information from the person who knows Maddie’s anatomy second-best (assuming the surgeon knows it best). Her structures are reversed, but even for a heterotaxy kid her heart is different. The apex is not down, but to the right and the confluence of veins into the IVC occurs deep within the walls of her “right” atrium on the left side. Post Fontan, all systemic return is routed around the heart with gore-tex other than the fenestration into the “right” left atrium. I was confused because then the “right” left ventricle would seem to be a useless structure given that her pulmonary artery was severed from her heart during the Glenn procedure. I asked Dr. Meg about that and she said it would be, except Maddie’s heart has a double-exit ventricle. Her aorta is connected to both ventricles. Originally her right ventricle pumped to both arteries.

We speculated on the relative position of the Glenn entry and Fontan entry into the pulmonic artery (she didn’t know). Usually they put them right opposite each other and aim them straight at the artery so flow does not prefer one side over the other. We speculated on the fluid dynamics of that setup including churning and backflow. She agreed that there usually is some and swelling of the head after the Glenn and of the body after the Fontan is evidence of that.

Other risks of the fenestration include stroke, given that clots that are generated in the venous system have a path to the aorta through the fenestration and could get pumped to the brain. That is the main reason they often suggest to come back and close the fenestration a while after surgery. However, her liver–which is now already experiencing additional pressure–would experience even more pressure without the fenestration and would be susceptible to failure, so there is debate on which risk is greater.

I didn’t do any more Dr. Whoian deeds last night, but stay tuned…

-Colin

===

I think you should make a 3D model of Maddie’s heart. I’m getting lost in all the ‘rights’. I’m glad Maddie is awake and doing well. Is she loaded up on pain medication? I imagine most movements are painful anyway. I hope the fenestration is helpful to her liver. How much fluid exudate is coming out of her lungs now? What causes it to eventually stop?

It looks like she has a picc line in her jugular. Were they using that in the OR, or is it more for just-in-cases afterwards (or both)?

-jared

===

The IJ line is for taking pressure readings and administering drugs directly to her heart as needed. It is currently being used. She has been off most of her medications since yesterday morning. During the day yesterday they were administering morphine as needed. Today they have her on Ketoralac instead, and it is administered constantly.

I would love a 3D model, they should have one because they did an MRI a few months ago. I wonder if they would be willing to share a soft-copy of it, and I wonder what format it would be in. Her heart returned to irregular beats, so the pacer has been hooked back up.

She still has a lot of fluid coming out. The pressure change is so great with the Fontan that they expect the fluid to continue for many days to come.

-Colin

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*Extracardiac vs. Intracardiac debate: pro¹ vs. con²

See here for more “light reading” on the Fontan procedure: Intracardiac, lateral-tunnel Fontan has superior hemodynamics to the TCPC (extracardiac) and Hydrodynamic power loss of Glenn (and Hemi-Fontan) is considerably lower than that of the final completed TCPC

Reflections on our family's joys & trials

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