Thursday, April 30, 2009

Life is Blue-tiful!

The day before yesterday, when it was 90 degrees in April, in Northern New England, I took the girls to Luna's follow-up cardiology appointment. This was her big follow-up appointment to see how well the catheter worked. Maybe because both the girls are getting older-a full year older than when I first started bringing them-(and I always have both, mostly because I don't want to pay to have our beloved babysitter take Sienna, but also, these girls are two peas in a pod; neither one likes especially to be with me alone). Now that the girls are 13 months and 3, not 3 months and 2, it's almost getting....dare I say fun?

Due to the sweltering weather, I had brought the girls to the beach in the morning and as beach time goes, I happened to look at my cellphone while they made sandcastles, and realized it was already time to leave. I quickly dragged the girls off the beach and whisked them to Dr. G's, sandy bums and all.

Luna suffers through her appointments. She has been poked and prodded her entire life, and whenever someone starts to bring a stethoscope to her chest she screams at the top of her lungs. It's interesting to me that doctors can even hear what's going on inside of her, let alone listen for a dull heart murmur.

But not this time. Luna cried a bit. But once we got her settled down on the exam bed, in the darkened room with Happy Feet on the big screen-she sucked her bottle and giggled at the silly penguins on the screen. This meant that the echo, that normally takes over an hour, only lasted about 15 minutes. And because we didn't have all the screaming, squirming and hysterical gag-crying, Cindy, the sweetest echo reader in the world, got amazing shots.

The prognosis? Luna is looking great. The balloon dilation of her left pulmonary artery worked beautifully (to quote Dr. G). The run-away blood vessels, those are all gone, and best of all her heart function is perfect.

Dr. G and I talked about the road ahead. Right now Luna's sats are back down to the low 80's. This is fine and where we want them. Once she starts getting really mobile they will drop a bit, and yes, she will get blue. As Dr. G put it, 80's blue is somewhat undetectable-especially to the untrained eye. But 70's blue is blue. Like standing in the grocery aisle-your-kid-looks-awfully-blue blue. But fear not. This is normal. And all part of the plan. For the next year, up until Luna goes in for her last repair, that of course being the Fontan, the child is going to look rather indigo.

I say blue is beautiful. I declare Blue Is The New Black. Don't hate me because I'm blue-tiful. You get my point. The worst part will be for me, having to explain to mere strangers why my child's lips are purple.

Luna's vein in her neck will also become more noticeable as she grows and her body thins out. This is because her vena cava-the big ole vein coming down from the brain-is routed straight to her pulmonary artery (thus the Glenn Operation). So if you see a blue-ish child with a bulging vein in her neck, don't fret, it's just my child, pre-Fontan. I have the same mega-vein and I didn't even have the Glenn. Luna will match Sienna, who also has the 'neck vein'. When the older child has a toddler tantrum, her jugular jiggles and bulges, displaying a mini tantrum of it's own.

The other tidbit of information we learned is when we fly, Miss Luna will need to wear oxygen. Cabin pressure is not kept to normal-sea-level oxygen levels. Basically it's kept at an altitude equivalent to standing on a really, really tall mountain. The result? All of our sats drop. So if you were to slap a monitor on every index finger on the plane, everyone would read in the 80's rather than the high 90's where most folks hang. This of course explains why one Dixie cup of wine feels like you just drained the entire bottle-by IV-in 5 minutes. So, because of the already dropped cabin pressure, Miss Lu will have to wear oxygen when we take our first family trip as a foursome to Jamaica. The Architect, along with a few friends, suggested why can't Lu simply activate the drop down oxygen masks. This struck me as really funny and immediately conjured up scenes from the classic movie Airplane where Vicky from Love Boat is sucking in her cheeks, making fish face because the cabin pressure had dropped so low. We don't want Luna to suffer fish face any time during the flight, so when we check our baggage and make sure all our shampoos fit into a zip lock, we'll also have to 'clear' the oxygen tanks with customs. THAT will be the really fun part.

But we've got plenty of time before January. The entire summer is before us, and not.a.single.cardiology.appointment.till.August. (!!)

Tuesday, April 21, 2009

Official Diagnosis... stated on Luna's discharge papers from her catheter:

13 mo F w/ dextrocardia, common atrium, DIRV, DORV, PA s/p RmBTS, BDG, LPA stenosis presents for HD cath and LPA angioplasty.

Got that?

Friday, April 17, 2009

A Brand New Day.

Am I ever glad to have the catheter behind us. The procedure itself-naturally the most important part-went as smoothly as it possibly could. The logistics end of it was a little tedious.

Luna was scheduled for the 9:30 slot, and after getting Sienna ready for her big school portraits, and then dropping her at our friend's house, then The Architect and I arguing about what vehicles to drive (he wanted to follow Luna and me in his truck, which in the end was the better idea because it avoided him having to drive the 120 round trip to the hospital to come retrieve us when we were discharged), and then battling the usual rush hour traffic; we were more than a little deflated when a nurse scurried toward us upon walking in to say there was an emergency-an infant needed an emergency cath-so could you please come back at 12.30. She gave Luna a bottle of apple juice (the last meal she had eaten was a Happy Meal at 4pm the day before, and as of this writing-Friday morning 7:15, she's eaten nothing but a few Cheerios and Pirate's Booty).

So, we gathered all our belongings and shuffled out of the waiting room and made our way down to the lobby. The lobby in Boston Children's Hospital; with its larger-than-life moving sculpture made up of pulleys and tracks where the kiddos (and adults) can spend hours following the balls' journey through the optical course; rivals the best children's museums around. Then there is a huge fish tank filled with exotic species, and of course the cushy lounge with a giant flat screen TV which airs PBS all day, until about 6pm when, depending upon the season, the janitors and service men and women change it to the Bruin's, Celtic's or Red Sox game.

We bided our time nicely during our wait. Luna watched Sesame Street, The Architect made work e-mails and phone calls, and I filled out the monster application for Katie Beckett (more to come on why it's incredibly important to enroll your special needs child-whether it be heath, emotional, or learning into this federal program).

At 12:15 we made our way back up to the cath floor-where we waited till nearly 3pm until they finally took us back. (It should be noted that Children's is a teaching hospital, and of course a major health institution mecca. Upon walking in there are signs stating that whether you speak Chinese, Cantonese, or perhaps a rural dialect of some remote country, whatever your tongue, there are trained personnel who can spring into action on a seconds notice to translate your child's medical procedure for you. Says a lot, huh?)

So, this next part I'm about to describe is a repeat of pre-Glenn, pre-cath and pre-sedated echos; and by now you're probably pretty used to hearing about Luna getting the 'happy juice'. Only this time, because she had just been napping, the happy juice wouldn't knock her out. For over a half an hour we watched Luna sway and babble, and yes, hiccup. I'm thinking if in the near future there is a roll for a drunken sailor in the pre-pre school play, Luna should try out. Remember in old cartoons when they would depict one of the character's drunk, say like Tom from Tom & Jerry? That's how Luna was acting. Luckily she's a happy drunk, and not a belligerent one, so we weren't asked to leave or anything.

Finally the nurses took her back because it was clear she wasn't going to fall asleep. Luna protested only a little, then gave Paul and me a sloppy wave, and was whisked back where the anesthesiologists would don her face with a tiny gas mask so they could then safely insert the IVs.

The entire procedure took exactly 2 hours. And it went exactly as planned. Dr. Bergersen, the cath doctor, took us back to show us the footage (which once I receive the disc, I'll post here, very amazing can see the balloon working it's magic on her left pulmonary artery-crazy!).

The best part was the results. The catheter, as I said in my last post served three purposes: enlarge the LPA, cauterize the errant blood vessels, and provide detailed information of Luna's heart, lungs and arteries. The catheter achieved all this-safely, and relatively un-invasively (is that a word?). And the results? Her heart is functioning perfectly, and her pressures, well they are just the same as someone with normal heart and lung function.

Her anatomy may look very different from yours and mine, but her body; well it's functioning just the same.

Thursday, April 16, 2009

Luna's status update:

Luna Ruth woke up from her nap with purple lips. Sats read 81-85 with heart rate of 150. Played with Sienna for 45 minutes, took Motrim and went back to sleep.

Luna's status update:

Luna Ruth woke up from her nap really cranky and agitated. Took Tylenol and went right back to sleep.

Luna's status update:

Luna Ruth is home from the hospital; a little tired but otherwise in great spirits; and is currently sitting in the bouncy seat with a bottle watching Word Girl.

Tuesday, April 14, 2009

Pre-cath, check!

It always feels so good to get through a day a Children's. The anticipation, the preparation, and just thinking about whether I'll get there on time (which today, I was 45 minutes late), is stressful.

After the 2 hour trip from NH and snaking through Boston traffic, Luna and I arrived to the hospital at 8:15 this morning.

Each step is a mini process from admitting to checking-in to Cardiac Pre-Op-and saying Luna's birth date and our home address about 14 times before I even sit down-each transaction feels like a mini-triumph.

First on our list was X-Rays. We were whisked away quickly to radiology and taken into the sterile room almost immediately. Luna was swiftly undressed and put into compromising positions so the X-Ray techs could take good pictures. I donned a lead apron and held her tiny arms above her head, while her feet dangled off the edge of the bench. The images of her were quite startling. I mean, I really don't think of her having...and I loathe this term, half a heart. And then the fact that's it's on the 'wrong side'. Well, you just can't ignore it when you looking at the X-Ray photos, when there is a big 'R' for Right, and there Luna's heart, rather apple-shaped, sits, just under the big 'R'.

Next was blood work. Another fun procedure. The wait was long so there was plenty of time for both Luna and I to study those in the waiting room (really, let's face it...but isn't so fascinating to study the others waiting in the stiff blue chairs and wonder why they are here?).

After about an 1/2 hour Luna was called, or rather 'Laura' as the nurse hollered out. I carried my bundle back and we both sat in the 'blood chair'. All in all it was quick. Luna cried, but honestly, she's so good natured (read, not like Sienna at her age), that it was all over in the blink of an eye. The best part was all her labs came back as normal as could be. Despite her croup-y cough, this kid is just healthy.

Then we were ushered back up to Cardiac Pre-op for EKG, height and weight. I guess I was wrong to think Luna would never seen the teens in weight again; she weighed in at about 19.13 and is just about 29 inches tall (or long as they say before one can walk).

After some waiting, and protesting from Luna, the cath doctor came in to explain how tomorrow would all go down. As I said in my previous post, her errant blood vessel will be cauterized. What I was wrong about is this actually happens often in kids and adults with these type of heart issues. The body thinks it's being oh-so-smart in sprouting an oxygenated vessel down to the lungs (when ones sats are low), but really it's not good for the lungs-and heart. The lungs receive this oxygenated blood and thinks, "what the heck is this? we only take blue blood, and then blow oxygen into it and send it back out to the rest of the body. why are you sending me this blood that we already worked on?". And then the body responds with elevated blood pressures, and suddenly the lungs and the heart are duplicating their very perfected and very efficient work load, and they don't like it. That's how the cath doctor explained it to me, and strangely, it made perfect sense. So, tomorrow when Luna is in la-la dream land, the doctor's will insert 2 caths-one in the groin, and one in the neck. One cath will go to work with the balloon in an attempt to enlarge her left pulmonary artery, and the other will go to work in cauterizing the blood vessel, the one that thinks it's oh-so-smart and sneaky.

Still the body feels nothing during this. Nothing. Years ago this would have been an open heart surgery (the cath will casually snake through Luna's's. still. beating. to get to it's final destination (artery going to the left lung).

The whole sha-bang should take about 4 hours-tops. After that Luna will drowsily come to, and be instructed to lay flat for 4 hours; and then stay a night for observation. Come Thursday she'll go home and play with Sienna like nothing ever happened.

So, what are your plans for tomorrow?

Monday, April 13, 2009

Luna's status update:

Luna Ruth is up at 3am with a nasty cough.

Wednesday, April 1, 2009

The renegade blood vessle.

Luna is supposed to go in for her cardiac catheter this week. I say supposed to, because as of this writing she has a pretty nasty cold and cough. Adults who have any type of catheter (angioplasty is a similar procedure) are awake for the procedure. In fact, I just had a conversation over the weekend with the husband of a good friend of mine. He underwent an exploratory cardiac catheter in his early 20's-and was awake for the procedure. I was relieved when he told me he didn't feel a thing. Strange, to think that a tiny probe with an equally tiny balloon and camera is inserted into the femoral artery (the one located at the pelvis), then snaked-up through one's veins, and in Luna's case, through her heart, yet, no sensation of pain or otherwise is felt. Amazing.

But since Luna is just 13 months, and we can hardly expect her to lay still for the hour-plus-long procedure, she will be fully anesthestisized. And of course because of the precarious-and still largely unknown-state of the body during anesthesia, she has to go in to this procedure totally healthy. Later today I'll call the doctor to see what they say about her cough.

You might be wondering why Luna is having a cardiac catheter, and really the story is quite fascinating. For the past several months, her doctors have been trying to get a good look at things Post-Glenn. Because of the nature of her anatomy-that of course being the dextrocardia, and the fact that even for a dextro kid her anatomy is still unusual (her heart is not only located on the right side of her body, and flipped mirror-image, but also sits on a different plane than most people with dextrocardia), the doctors require all the tools they can gather so they may translate what they are seeing (in one case this resulted in a 3-D imagery taken during her last sedated which Anthem declared they wouldn't contribe to the $616 procedure because it was 'too specialized' and 'not in their contract').

The 3-D imagery did produce the pictures that provided some important clues about what Luna's team thought they were seeing. It was something that I had never heard of, something I certainly never considered could ever happen. A blood vessel had sprouted off Luna's aorta.

It's thought that some time before Luna went in for her Glenn Operation, when her sats would have been at their lowest, her body decided it was going to do what it needed to get more oxygen to her lungs. If you imagine the aorta looking like a candy cane-oh, and if you need a quick refresher: the aorta is the biggest artery in the body, it carries oxygenated blood to the heart, lungs and rest of the body. Well, Luna's sprouted a small blood vessel. Basically, imagine a small stream of oxygenated blood that shoots off her candy cane and connects directly to her lungs. As Luna's card explained this to me, I felt that familiar sinking feeling. "Is this a good thing or a bad thing?" I needed to quickly reduce the news to either good or bad. It turns out that though in a way it's good, because it shows just how incredibly resilient Miss Luna's body is; her doctors don't want her sprouting all kinds of blood vessels so when she goes in for her cath, this will be cauterized.

This also explains why her sats have been hovering in the low 90's. I expressed to Luna's cardiologist how about 2 months ago I heard Luna gasping and choking in her crib. She had a cold, and was struggling to sleep with a stuffed nose and no way to relieve herself. I brought her into our bed-fully expecting that his may be the first time we have to bring Luna to the ER-because, well she just seemed like a sick kid. After a lot of protesting-aka full-out screaming-I got the oximeter strapped to her tiny finger. The results were astounding. Her sats were ranging from 88-90. I couldn't believe what I saw, so I then tested myself and got the routine 98, 99 and 100. Luna was congested, yet her sats were high. I put her back in her crib, where she slept like, well a baby, and called her card in the morning to relay the incredible story.

That had confirmed it-there was no question that her aorta had sprouted a blood vessel which is why her sats are very. close. to. normal.

So, bearing Luna recovers by this coming Wednesday, she will go in to Children's Hospital Boston for the cardiac cath. The cath will serve three purposes: cauterize the errant blood vessel, enlarge her pesky left pulmonary artery, and provide a detailed map of what is going on inside Luna's veins and heart.

The procedure requires a night stay at the hospital, and providing all goes to plan we'll be home Thursday afternoon. Until then...