Six Months after Luna’s Fontan

Yesterday was one of those super-charged days; one of those days where everything seems to happen at once. Mine started with the news of Leslie Nielsen’s death. I’ve watched Airplane at least a dozen times. Learning of someone’s passing; even a celebrity who you have no personal connection to, just gives you that “feeling”. After lingering on twitter too long, I scrambled to get the girls out the door by 8am so we could make it in time for Luna’s 8:45am post-Fontan check-up. En-route to the doctors while the girls sang in unison, “My mother is a baker, a baker, a baker…” I watched a woman cross the street-in a crosswalk-where she nearly got killed by a driver who was texting.

But onto the appointment:

Luna and her favorite dolly sat very patiently and still for the entire 45 minutes echocardiogram (btw, don’t ever criticize this dolly’s hair-which consists of just a few blonde shoots stemming off the top of her head-Luna gets very upset).



After the echo, we moved to the examining room where Luna had an EGK, her blood pressure taken, and a sat check (blood saturation levels). All checked out perfectly.

Then she hopped on the scale, where the digital numbers read an even 28 lbs.

Dr. G even got to try out her snazzy new stethoscope. The ages-old medical instrument has recently been adapted to the 21st century, where now you can record the patient’s heart while you listen. This is pretty amazing stuff. The recording is then imported into the computer where you can track, map, and illustrate a patient’s heart beat. The coolest part is when Luna is twenty, her doctor’s can go back and actually listen to what her heart sounded like when she was two. Hooray for cutting edge medical technology.



We then assessed Luna’s medication, which consists of just half a baby aspirin, and determined she’ll stay where she is. Patients are advised to take the entire tablet when they weigh 35 lbs, so we still have a year or so before we increase it. Otherwise, the appointment was quick and uneventful. Her next scheduled appointment is not until (insert long and extended drum roll please) ... MAY 2011.

Upon my arrival home, I settled back into The Monday After Thanksgiving barrage of emails, and learned that a fellow CHD’er, Steve, who also had a single ventricle, died in the early hours of the morning. What’s stranger is he is thought to have passed on the 66th anniversary of the invention of the Blalock-Thomas Shunt; an operation which paved the way for the survival of all single ventricle heart patients. This of course includes Luna.

Some days it seems that the universe wants to show you how valuable and special life is; and also how fleeting it can be. Yesterday was one of those days.

Fontan Day Five (Big Sister, Big Sister)

I can’t complain. Really, I can’t. It’s Tuesday, we’ve only been here since Friday. And we were here last Thursday for pre-op, so I guess that counts for half a day. But, it’s still tedious. We’ve folded into a new month. June First today. And we missed Memorial Day weekend. It’s missing these types of landmarks that mess with you later. You feel gypped, off kilter, and find yourself later in the summer trying to recall what you did for unofficial kick-off of summer, and for the briefest of moments you actually forget, only to be disappointed when you regain your conscious footing.


On Luna:

She’s doing great. Chest tubes are out. X-Rays looking good. She does have low potassium levels, but with some regular eating we should be able to get those back up. She’s down to just one IV-in her hand. (Careless Mommy of the Year Award goes to me for accidentally pulling out Luna's IV while I was lying in bed with her. I looked down to see blood pooling in her blanket and on my shirt. Luna was sort of whimpering, and I started gasping and the nurse, thanks goodness, sprung into action and cleaned-up the mess.)

Her heart rate seems back on track too. No mysterious rhythms. And her blood-oxygen saturation levels off the vent are in the low 90’s. But really, they had been there before, but the big difference now is in her toes. Almost immediately after surgery her feet took on a healthy glow. Whereas before Luna thought she was wearing blue nail polish on her toes, now she’s sporting a nice nude look.

Perhaps the best thing to come from our extended hospital stay, is how our four-year-old is handling all this. The logistics of getting your child through three cardiac catheters and three open heart surgeries is one of the most difficult things to manage. What to do with our other child, commitments, and careers while we all hyper-focus on our youngest very noisy medical needs?

Our first born, Sienna, is a sensitive soul. No sooner had she mastered speaking when the child started spouting off a steady stream of conscious chatter; often having to do with the trees breathing in too much exhaust, the animals left out in the cold, or the flowers not getting enough, or getting too much rain. So, Luna's medical condition has always been something of a hotspot with her. Or so I had thought. In Sienna's four-year old brain, Luna’s heart is “on the outside”.

Thus, the thought of bringing Sienna into the hospital; where even the most seasoned parent can crumble at the sight of some of the patients-was simply nerve racking for me.

But Sienna did great. On the way in, The Architect prepped our precocious four-year-old; “You’re going to see little boys and girls who are sick and look different, but like Luna, they’re all here to get better”.

“I KNOW DADDY!”

And indeed, Sienna was as cool as a cucumber. I caught her wince just slightly at Luna’s tubing protruding like a plastic octopus from her chest. But otherwise Sienna was unfazed.

Later I brought her down to the food court where we hit the collective lunch break enjoyed by the staff of the several hospitals right in the area. This seemed to bother Sienna more than anything.

“Mommy, all the doctors and nurses left the babies alone in the ICU!”

Later, back in the ward, I thought I would try to capitalize on all this good-feeling toward the hospital, so I asked the four-year-old “do you think you want to be a doctor or nurse when you grow up?”

“No, Sienna responded, “I want to be a fire woman so I can put out all those forest fires and save the animals from burning.”

Fontan Day 3 (Boredom sets in)

We’re officially over the hump of the Fontan surgery. Luna is recovering nicely-actually she’s ahead of schedule. The only thing we have hanging over our head is this little pacemaker issue. During surgery, as soon as she was settled into her to anesthesiac sleep, her heart beat went into a junctional rhythm. Basically this means the heart fires its beat off from different parts of the organ, when it should come from the atrium. In people with two ventricles this is no biggie. But with one ventricle, there are no liberties; the single ventricle people get put on a pacemaker-and fast. For now, however, Luna’s heart has settled back into a normal rhythm-which is a good thing.

Oh, and did I mention her surgeon now thinks she has a left ventricle? For the last year, she was determined to have a right one, but she’s switched teams again, this time playing for the left.

Now it’s boredom. And tedium. The most exciting thing to happen around here is shift change-which happens at 7am and then again at 7pm (these nurses really deserve a special place in heaven…seriously…they’re like angels on earth).

But boredom is much better than the nightmare we experienced the first night, just after Luna’s surgery. They always tell you after everything is OK, just how bad it got for a while. “Rocky” is the word her doctor’s use for her somewhat exorcist-like behavior just after surgery. No fewer than twelve doctors were in the room-several giving a running commentary on what medicines to add, what to pull. All the while Luna cried, bucked, reached, and grabbed for me; even under all those sedatives and while attached to a net of lines; giving the impression of a small whale fighting to escape a messy trap. Witnessing her powerful spirit and will to live was nothing short of incredible.

So, I leave you with some photos. As you can see Luna is uncomfortable, and bored. And she hasn’t moved since 7:30 Friday morning. And she has IVs in her feet (her wrist wasn’t having it), so she can’t walk now even if she wanted. But next week at this time, (knock a big ole piece of wood), she’ll be home, or maybe even on the playground with her sister.

In for the Fontan and out with Ear Tubes: Part I

Last Thursday had been planned for weeks. Years, really. Luna's last of three surgeries, which we knew she would have to endure from the time she was diagnosed, at just 20 weeks gestation, had been scheduled for 6am. The Architect and I spent the entire week leading up to this All Important Date-and one that justly sends many a heart mommy and daddy to the doctor for a refill on sedatives-calling for referrals, booking hotel accommodations, arranging for our four-year-old to sleep over friends’ houses, and coordinating rides to and from school for the before-mentioned four-year-old.

The afternoon before the surgery was scheduled I called Luna's surgeon. Lu's old surgeon took a position at Columbia, and we were to have a new one. This doctor was assigned to us by Luna's esteemed team. I didn't for a minute doubt his competence, but I thought I should at least speak with him before we handed our two-year-old over for a 5 hour surgery.

I had exactly 25 minutes to talk with him before I had to jet off to pick-up the girls from school. Luckily when I placed the call, his secretary put me right through to him. The Surgeon’s voice was clear and calm. Too nervous to even think, I told him that I didn't know what questions to ask. Thankfully the kind surgeon launched into the details of the procedure. Details that have been haunting me ever since.

I had always loosely understood the Fontan. It's the last of three surgeries most single ventricle kids have in order to correct their circulatory system. It’s all about circulation and pressures in these kids. Keep the single-ventricle heart buoyant and don’t ever over-work it, and they will live long healthy lives. As a way of 'paying it forward' while also paying thanks back to the thousands of others before Luna; I had volunteered our daughter for a research study during her Fontan. The study was to determine if two medicines increased recovery time. The results to be published in the very prestigious medical journal aptly titled: Circulation.

In short, the Fontan is required to get the blood flow down to the lower extremities. As Luna stands now, her legs are getting recycled blood-mostly because of gravity. If you look at her little toes, they are indeed quite blue (and cold). So cyanotic in fact, that she thinks that she fashions blue nail polish on her toes. To which I simply keep pretences up, "yep Lu, Sienna has pink nail polish and you have blue!"

What I didn't realize about the Fontan, is that of the three operations these heart warriors have (the BT Shunt, the Glenn and the Fontan), the last is considered true open heart surgery. With the first two, the chest is opened, and the heart is exposed, but mostly the doctors are rerouting arteries around the heart. In the Fontan a 'baffle'-which is a GoreTex mesh-type piece of material-is affixed to a room of the heart. The synthetic material is then used to create a channel inside the heart, one that connects from the vena cava to the pulmonary artery.

And here's the part that nearly caused me to pass out: in Luna's case this will not be a straight forward Fontan. Because of her dextrocardia, her heart will have to be shifted and rotated to where a 'normal' heart sits in order to gain access to the room of the heart called the atrium. (Funny that word should have a double meaning; one that involves the intricate design of an architect, the other, in the case of Luna, involves a crucial piece of a heart surgeon's intricate design). Luna’s atrium, which is where the baffle will be affixed, is not assessable without moving the heart. Because her heart is rotated in her chest-a near perfect mirror image from yours or mine-this means, in clearer terms: Her Heart Will Be Moved Significantly During Surgery.

I'm weak just writing about it.

So I asked the surgeon the ridiculous: “will her heart be placed back to where it was?”

“Yes,” replied the kind surgeon, “we like for the organs to stay where they were, or where they want to be, which in Luna’s case is rotated, and on the right, and protruding slightly in the right chest wall.”

This is true, Luna’s heart can be felt quite well if you pick her up from under the arms-just under her right arm-a fact that has disorientated some of her care takers.

Then I asked another ridiculous question: “Can her heart move after surgery?"

“No,” The Surgeon replied.

Phew, check that off my list of anxieties.

The Surgeon assured me that with many single ventricles, the heart is often dextro, and has to be moved to gain access to the atrium.

The entire surgery will take up to 5 hours and is broken down into these parts:

1 1/2 hours to get lines and Luna anesthetized and stable for surgery.

1 hour to remove scar tissue from last surgeries.

1 1/2 for actual surgery, during which time Luna will be on the lung and heart by-pass machine.

1 hour wrap-up.

But none of this happened. Instead Luna underwent an hour and a half of anesthesia for a five minute procedure to implant ear tubes.

Luna’s Stage Three Open Heart Repair has been scheduled.

Luna at Panera Bread on her 2nd birthday; where she ditched her kiddie meal in favor of Mama's Broccoli Cheddar soup.

I had planned on posting about Luna’s 2nd birthday and her subsequent wellness appointment with her pediatrician when I got The Call from Children’s Hospital Boston.

If you ever wondered how these surgeries work; a liaison from the cardiac department calls and gives you the dates and logistics of your child’s upcoming appointments.

We had known all along that Luna would be going in for her sedated echocardiogram and (exploratory) cardiac catheter. What I didn’t know is that her Fontan surgery-last in the series of three surgeries to repair single ventricle anomalies-would be scheduled the Monday after Luna is discharged from her catheter.

So, this is what we’re looking at:

Wednesday, April 28th we’ll arrive at the brisk hour of 7:30 am to Boston for a day of catheter pre-tests, blood work, chest X-Rays and an EKG.

The next day Luna will undergo two exploratory procedures so her medical team can acquire two-dimensional and three-dimensional photos and live footage of her heart and arteries, both from the inside and outside of her body; these being the catheter and echocardiogram. For these procedures the young kids are fully anesthetized (there’s just no reasoning this kind of procedure to a two-year old-and adult can lay still for hours on end, not so much with a toddler).

Because she will be under general anesthesia she will stay the night for observation and be discharged the next day; Friday April 30th.

And here comes the surprise: Luna will then be admitted Monday May 3rd for her Fontan surgery.

It should be noted that the Fontan doesn’t pose any greater risk for her than either of her prior surgeries. The recovery is expected to be about a week. While the age of the Fontan kids (typically two-to-four-years-old) is better than the earlier surgeries in some respects (bigger, stronger, immune system has had time to beef-up), we’re still talking toddlers here. And just their developmental age-the stubborn phase-can keep them in the hospital a day longer. (for a good and easy-to-read-and-understand written description on the Fontan, click here.)

When I asked Luna’s cardiologist if there was any risk with having the surgery right on the heels of the catheter, she said “absolutely not.” Logistically it can be easier for the families as well, which can be summed up like this: let’s get this done and behind us-and all at once please.

There is a chance the surgery could be pushed back a week or so; and that depends upon who her medical team is made up of. Her lead surgeon and one of the most seasoned and senior of the cardiac department left to take another opportunity at Columbia Hospital. But thank goodness we’re talking Children’s Boston here, so there are a handful of other just as senior surgeons who will operate. (The rarity of her anatomy, especially her dextrocardia and ‘mirror imaged’ heart placement can and does keep even the senior surgeons on their toes-but I imagine because of her ‘uniqueness’ she does draw top talent who come across such special anatomy only a handful of times in their career).

So, for now it’s a go. Luna’s primary cardiologist here in New Hampshire will get back to us on who Luna’s dream team will be; and from there it’s on to the next leg of the journey.

Table talk (Thoughts on talking to your young children about an upcoming Fontan procedure)

Life is pretty much the same at our house as it at any other. The girls are in school Monday through Thursday (still clinging on to that last day of the week with them, but it’s becoming increasingly more difficult with each career leap).

They go to school in our tiny village, and I pick them up around 5 o’clock each day and bring them home. Several nights during the week it’s just us girls-The Architect works in various corners of New England, depending upon the day.

I’m a stickler for eating dinner at the table, with no distraction of TV, phone or other handheld devices. (I will proudly admit that both girls eat pretty well, and I attribute this to my ‘old-fashioned’ regimen of dining at the table).

Two nights ago we were enjoying The Architect’s homemade chili with chips and shredded cheese-for the second night in a row-when Sienna out of the blue said, “Mommy, does Luna need to get a point this week”. I was startled at the child’s-who will be four later this month-astuteness to the topic. I had earlier that day received the call from the pharmacy informing us it was time to ship Luna’s Syngeris shot again. I had shoved the phone conversation to the deepest corner of the brain, not wanting to think about once again where we’ll find $2800 in cash for the shot.

But before I could think much more about it, Sienna continued with, “Luna has a special heart. Her heart is different from mine. That is why she needs so many points.” (Points, if you haven’t already figured out, is Sienna’s word for shot. I love her moniker so much that I don’t bother to correct her).

I decided this would be my chance to start to explain the year ahead.

“Luna will be going to the hospital later this year.” I say as casually as possible.

“But will she be home for bedtime?!” Sienna says,-her voice lifting into a whine-gives me an instant headache.

“Well, she’s going to stay there for a few nights; the doctors have to fix her heart a little more”.

All the while, Luna, sitting across the table from Sienna; is repeating everything we’re both saying, “Nanu stay at the hospital?” only her eyes are about the widest I’d seen.

The exchange lasted for all of five minutes before the girls went on to the topic of dessert. Later that evening, in the midst of changing them into their “cozies”, Sienna threw a temper tantrum-she said over her eyes “feeling tired”-but I wondered if it had to do with what we talked about earlier.

After they went to bed and the house was quiet enough for me to think, I realized the journey through this next heart surgery will be just as much about Sienna as it is Luna. Not only are the girls truly inseparable (even at school there stands just one wall between their class rooms-and I’m told they visit each other often); but from the smell of Purell hand sanitizer, to the beeping of monitors, and even the feel of the weight of a thousand emotions swirling around us; Luna’s experiences will be just as much a part of her older sister’s memories, as they will be her own.