66 years ago today...

on 29 November, 1944, Dr Alfred Blalock, Dr Helen Taussig (yes, a woman) and Vivien Thomas (a black man) performed the first Blalock-Taussig shunt operation at Johns Hopkins in Baltimore.

the shunt was designed to help "blue babies," wee ones with a CHD known as Tetralogy of Fallot. in ToF, there are several issues:

1. pulmonary stenosis. the pulmonary artery carries de-oxygenated (blue) blood from the right side of the heart to the lungs. with PS, the opening to that artery is too small and restricts blood flow to the lungs.
2. VSD, a hole between the two pumping chambers. this allows blue blood to pass over to the left side of the heart and mix with the red blood and travel out to the body.
3. right ventricular hypertrophy. the right ventricle has to work harder than usual to overcompensate for the PS and VSD.
4. overriding aorta. this exacerbates the VSD and allows more blue and red blood to mix and travel to the body.

at this point in history, in 1944, there was no open heart surgery for these babies yet... that would come much later. and actually, up until this day, there was nothing at all that could be done for babies with severely wonky hearts. nothing. this procedure was the first of its kind, anywhere, ever. 

if you're interested in learning more about the surgery, including how to do it, click here. this will take you to a site which will teach you about ToF, and you can do the surgery yourself, in a cyberish kind of way. it's amazing, it really is!

this procedure is not what Asher had. Asher had a modified (or reverse) BT shunt, which went from his MPA (main pulmonary artery, right before it branches off to the lungs) to his proximal innominate artery, allowing most of the blood to by-pass his lungs and go out to his body.

but without this first procedure 66 years ago, Asher would never have survived beyond his diagnosis. there would have been no hope for him at all. and yet...

here he is.

almost 4.

(and for the record, i know of many people with ToF. you can click on Anniek's blog on the sidebar to read her story. and in addition to Anniek, there are two people at my church with tetralogy: a father and son. yes, the father has it, too. i've met him. he's an adult, and if you met him, you'd never know he has a wonky heart. simply amazing. and when you consider that these people would not be here today without this first surgery so very long ago... awesome. just awesome.)

look what my baby can do!!!!!!!

that's right, asher fans.

HE CAN WRITE HIS OWN NAME!!!!!!!!!

and he's not even 4.

yup. he rocks.

and those faces... those perfectly (for a kid) drawn faces... you guessed it, asher drew those, too. he's awesome.

happy hallowe'en!!!

yes, yes, i know. it's already november and i still haven't posted about hallowe'en. bad asher's mom, bad bad asher's mom. and so here we go: the long-awaited post.

asher got his costume a couple weeks ago, and so he wore it. a lot. everywhere. all the time. yup, he LURVS his costume.

alright, fine, twist my arm. here's a pic:

that's right, folks. he was ELMO!!!!!!

(funny story: so, you may or may not remember how i got through his diagnosis and first admission. when i learned that he would be blue, i thought, "well, that makes hallowe'en easy. he'll be grover." so, now that he's pink, he doesn't need to be anyone blue. nope. asher almost blends in with the costume now!!! WOOHOO!!!!)

so, i'll tell you about hallowe'en now. i got all the kids dressed. you've already seen asher.

bram was Sheerluck Holmes, from VeggieTales. Sheerluck is their version of Sherlock, and is played by Larry the Cucumber. hence the green face and buck tooth. how cute is he, eh??

blithe was Mina Harker, from Dracula. look at her working that costume. i'm actually quite proud of this one.

here's the close-up of the make-up. now, i don't usually allow the kids to be anything evil or scary, and blithe wasn't supposed to be. this started as a medieval princess, which blithe understood to be an evil princess, and well, it went from there. and then i was having so much fun doing her make-up, and i guess i got a little carried away. but she looks fang-tastic, wouldn't you say?

anyway, the kids got all costumed, and out we went.

with bram's bestest buddy nicholas. nicholas' mom lori took us all around her usual "haunts." we left their place at 6:00, and didn't get back till almost 8. and you know what?

asher walked.

the whole way.

the whole time.

sure, he was a little slower than the other boys, but that's because they are two years older and have longer legs.

not because asher was tired.

yes, you read that right.

asher walked.

and ran.

and jumped.

and carried a pail full of candy etc.

the

whole

long

way.

for two hours.

and he wasn't even winded.

just a normal kid doing normal kid stuff at a normal 3 year old pace.

extraordinary, no?

i love it.

(and you're gonna laugh... i told him he's allergic to chocolate. and i got blithe and bram in on it. so he handed me every chocolate bar he got. mwahaha)

(and yes, i have some ideas about future costumes. next year: a robot. {wink})

would it surprise you to know i spent last night reading?

no? ah, you know me well. ;)

well, here is what i found: epilepsy adds yet another layer of complexity to asher's already complex health and treatment. take some of these statements from an article i read:

Isolated seizures can transiently influence cardiac function, and epilepsy, if not managed appropriately, can lead to serious cardiac dysfunction.

gosh, isn't that fantastic. basically, it means this: seizures can cause a heart to fail. this is rare in patients with a healthy heart. but the problem is that asher's heart is not in the greatest shape, he lives with congestive heart failure, and one cardiologist, when asked just how much seizure activity asher's heart can tolerate, answered "not much." too much seizure activity is not good for asher's heart.

but, you'll note, having epilepsy means that asher is prone to seizures.

On the other hand, cardiovascular disease and treatments can precipitate new seizures or alter the management of pre-existing epilepsy.

heart disease and treatments can cause seizures.

asher has heart disease. and he is being treated for it.

so, you see the problem here. seizures can cause the heart to fail. and a failing heart can cause more seizures.  which can lead to more failure. which can lead to more seizures. which can lead to more failure. which can lead to more seizures. and so on, and so on, and so on, and so on.

isn't neurocardiology fantastic? (the article i just linked to is where i got some of the information for this post. it's actually quite interesting.)

check this out:

Brain ischemia [...] can cause seizures.

what's "brain ischemia," you ask? it's a sudden decrease in blood flow to the brain. you'll note, this is also what causes fainting, as per the neurologist. you'll also note, asher fainted the other week. twice. (is anyone else nauseous right now, or is it just me?)

but, what would cause brain ischemia, you ask? well, how's this for an answer, again from the article:

Structural cardiac disease and arrhythmias cause impaired or turbulent cardiac output. The result may be poor cerebral perfusion.

translation: when there are problems with how the heart is built (and/or rebuilt), there are problems with how the blood leaves the heart and goes to the brain. arrhythmias also cause this.

asher has structural problems with his heart, and he has arrhythmia. and these issues, because of the issues with blood flow to the brain, cause seizures. which diminishes heart function. which, you'll remember, leads to more seizures.

the article also lists cardiac conditions and treatments that can "lower the seizure threshold" (the minimal conditions necessary to produce a seizure). four of these are congestive heart failure, congenital heart defects, "cardiac surgeries and other interventions," and heart meds. there are others, but i listed the four that apply specifically to asher.

ok, now, i realize that this all sounds pretty terrifying. and i'll admit, right now, i'm more than a little worried. give me a couple days, once i've sorted this all out in my head, and i won't feel so bad. but it's still very new and fresh, and i just need to get all this out before it really starts messing with me. but you know that i will be calling cardiology today, in london, and probably in toronto, too. i just need some reassurance right now. thanks for humouring me today.

We Heart Recipes

another heart mom (wendy, chris' mom) and i have started a blog together (yes, i know, i have a lot of blogs. LOL). it is a recipe blog called We Heart Recipes, and we post heart-friendly recipes (most of the time; there will be the occasional comfort-food recipe, as well). we'll be posting fairly regularly, not only the recipes but also any conditions it may help (i just posted the fat-free creamy pasta sauce i made for asher's chylothorax). there are some great links to check out, and our boys' stories. check it out, and check back regularly.

and for the record, the cappuccino at the top is decaf. ;)

you've gotta check out this blog! (click the title of this post to take you there)

i have a link to this blog on the side bar (under "heart journeys") but i wanted to point it out to you tonight specifically. the blog is called Adventures of a Funky Heart! and it is written by a man in his 40s who is a CHD survivor... he was born with tricuspid atresia (the valve between the right atrium and right ventricle is closed... similar to asher's mitral atresia, but on the other side of the heart, and creating similar, but distinct, issues).

he's quite incredible, actually, working constantly to raise CHD awareness and to keep his readers abrest of the latest developments in the world of congenital heart disease and surgery... as well as a look back at some significant developments in the treatment of CHD. he also reaches out to his readers; in a recent post, he asked is there was anyone who had, or whose child had undergone, a one-and-a-half ventricular repair.

Funky Heart is a great blog. he updates regularly, his information is very interesting (especially if you're a heart mom, but i'm sure someone else out there might find it interesting, too). he included a link the other day to Dr John M, who is a (adult) cardiologist and electrophysiologist who writes about heart health, cardiology, and healthy living. also a great blog, also included in the sidebar here.

i highly recommend you check out these blogs, if you are at all interested in learning anything about wonky hearts. steve at funky heart is especially inspiring, given that he has CHD himself, and that he works so hard for the cause.

look what i got!!!!

that's right, folks, i'm going to spend the next few days learning how to read and interpret ECGs. :D

i <3 justina. 'nuff said.

oh, wow, isn't this fantastic? (the problem with blogs is that you can't hear the sarcasm in my voice)

so, this should be interesting. they're opening a new $100million research centre in hamilton, ontario. they will be researching heart and stroke prevention, diagnosis and treatment. isn't that fantastic??

except for one minor problem.

they aren't researching congenital heart disease. only acquired heart disease.

you'll understand if i'm not thrilled about this. don't get me wrong, i appreciate the need to research acquired heart disease. it affects millions of people around the world every year. but, um... what about the people who have heart disease who did nothing other than be born?? do they not count? or is researching the #1 birth defect not important. in canada, 1 in 70 babies is born with heart disease of one type or another (and there are over 100 kinds, of varying degrees of severity). i guess they don't count.

you can read the article about the reseach centre here. and fyi... i'm planning on writing a few letters about this. to the research centre, the spec and other local news media, the local MPP, the minister of health... because this just isn't right. these kids are overlooked too often. and that's gotta change. grrrr.

this is wonderful!!!

my wonderful friend wendy sent me the link to this blog about a girl with HLHS... and there is a story about a fontan/HLHS survivor living a completely normal life... at 30 years old!!!! amazing!!! thanks, wen, for sharing.

oh, this feels good!!!

i just had to post about this right now. i'm about to edit asher's story on the sidebar. and remove a couple details. in the first paragraph. go ahead, look. by the time you read this, you'll see that asher has issues with his heart, kidneys and immune system. gone will be the reference to throat and GI problems and a feeding tube.

DO YOU HAVE ANY IDEA HOW AMAZING THAT IS?!?!?!?!!?!?!?!?!

i know my friend wendy does. her son chris is also (relatively) tube-free. eating by mouth for the first time in two and a half years! GO CHRIS GO!!!!!

and the countdown continues... surgery is two weeks from tomorrow... 15 days. i don't know if i want to smile, cry or vomit. maybe some bizarre combination of the three. but right now asher is strong and healthy. the cold he's been fighting for over 2 weeks is almost gone, and he's doing well. this is the first time he's ever gone into a surgery strong and healthy. this doesn't happen!!! :)

please pray for him that he stays healthy, and that i can get everything taken care of and organized before we go. the next couple weeks are going to be pretty busy and hectic, so if you don't hear from me, assume we're doing well. and don't feel dissed if i don't contact you or hang out in the next little bit. sooooo much to do, and sooooooooo little time to do it all in. but yeah, 15 days. yikes!!!!

click this title or the text of this post to check out a VERY interesting article

well, this is interesting... they've identified a gene which leads to CHD... including (potentially) HLHS. it seems that researchers at the University of California, San Diego Medical Center may have stumbled upon something here. you can bet i'll be asking about this at our genetics appointment... once we get one (the referral is in; we're just waiting for the date).

vote for me! :)

i don't know how many of you realize this, but i'm part of a huge network of health bloggers at http://www.wellsphere.com/ - communities of people blogging about a wide range of health issues (and not just paeds cardio - believe it or not, there ARE other health issues out there! who knew?! LOL). i'm part of the Children's Health Community. feel free to check out the site. it's pretty cool. :) there's an icon on the left sidebar, and you can click there to go to my profile, or just click the link above to go to Wellsphere and browse on your own.

anyhoo... right now there's the People's Health Blogger Awards 2009, and i'm in the running for Best Blogger in the Children's Health Community. there's a button on the right sidebar on this blog. click it and vote for me. :)

and then, feel free to add the "vote for heather" badge to your blog, too. the code is on my profile at wellsphere.

thanks so much!! :)

Heartchitecture

now, normally i wouldn't review a book on asher's blog (especially a book i'm not finished reading yet), but as soon as i read this passage tonight i knew it had to go on his blog. in fact, if it weren't such a long quote, i'd put it on the sidebar so everyone could read it whenever. but alas, it's lengthy.

it comes from an amazing book called The Wet Engine by Brian Doyle, who is the father of a remarkable single ventricle boy named Liam. the book is all about the heart, from a physiological point of view, but also from a more metaphorical/spiritual/poetic perspective. i'm only on page 44, and i'm already hooked. (oh, and the name of this post - heartchitecture - is the title of the chapter.) so here you go:

consider the astounding journey your blood embarks upon as it enters the pumping station of your heart. in a healthy heart, a heart that works as it has been designed to work over many millions of years by its creative and curious and tireless and nameless holy wild silent engineer, blood that has been plucked and shucked of its oxygen by the body straggles back into the right atrium, the capacious gleaming lobby of the heart.

this tired blood, dusty veteran of an immense and exhausting journey, shuffles forward to and through a small circular door in the wall, a door with three symmetrical flaps: the tricuspid valve.

this circular door opens into another big room, the right ventricle; but at the very instant the right ventricle is filled to capacity with tired blood the entire ventricle contracts! slamming in on itself, and our tired heroes are sent flying through the pulmonary valve and thence into the pulmonary artery, which immediately branches, carrying blood to the right and left lungs, and there, in the joyous airy countries of the blood vessels of the lungs, your blood is given fresh clean joyous oxygen! gobs and slathers of it! o sweet and delicious air! as much as those heroic blood cells can hoist aboard their tiny cellular ships, and now they resume their endless journey, heading into the marshlands and swamps of the lungs, the capillary beds, which open in the small streams and creeks called venules, which are tributaries of the pulmonary veins. there are four of these magic pulmonary rivers carrying your necessary elixir back to the looming holy castle of the heart, which they will enter this time through the left atrium, whose job is to send it on its quest and voyage and journey to the vast and mysterious wilderness that is You, and to tell that tale of the journeys of your blood cells through the universe of you, would take a billion books, each alike, each utterly different.

- Brian Doyle, in The Wet Engine: Exploring the Mad Wild Miracle of the Heart, pp 16-17



here's the link, for ordering this book on amazon.ca: http://www.amazon.ca/Wet-Engine-Exploring-Miracle-Heart/dp/1557254052/ref=sr_1_1?ie=UTF8&s=books&qid=1257856172&sr=1-1

because it's not enough with asher's stuff...

so much has happened recently around here...

asher's tube is still (or again) infected, quite badly. doc put him back on clyndamycin for another week. i'm hoping this will clear it up, because he spent wednesday, thursday and part of friday crying because his tube hurt so badly. he hasn't been crying this weekend, so i think it's on the mend.

i'm going to try something new with him once this course of antibiotics is done. i'm going to try giving him some probiotics. he's been on antibiotics since early august, with only a few days here and there without them. and given that, after the staph infection earlier this month, he ended up with a fungal infection in the tube site, i really think this has the potential to help him immensely. (for those who don't live in paeds clinics: antibiotics kill bacteria. that's what they're for. and they do a good job. but the problem is that they kill all the bacteria. our bodies still need some bacteria to keep everything balanced. but when the good bacteria is gone, it disrupts the balance, and things like yeast and fungus can begin to cause problems, which explains why some people struggle with yeast infections while on antibiotics. probiotics help restore the balance to the body, by promoting the growth of "good" bacteria.)

on another non-asher note... blithe was diagnosed earlier this week with ADHD. and, i know this will shock you, so make sure you're sitting before continuing to read this sentence... sitting?... good. i've started a blog. it's called My Girlie & Me, and in it, i'm going to write about how blithe is doing, medical and non-medical ways of dealing with this condition and how blithe is doing with it all, and resources i've found on the subject. feel free to check it out: http://adhdmomandkid.blogspot.com/ i just set it up today, but rest assured there will be more posts coming. (as if there was any doubt about that! LOL)

If You Only Had One Wish...

so, i have an app on facebook called "circle of moms" and every once in a while (every week or so, i don't know, i don't keep track) i get a link to their blog. this week, there is a poll on there, asking what you would wish for your child. the choices are:

• health
• true love
• wealth
• influence
• professional success

so i'm posting the link here for all you moms... please participate, and read the comments that people have left (including my own, if you feel so inclined).

http://fansofbeingamom.com/?p=73

well, we're here....

that's about all i have to report so far! LOL

we got here safely, traffic was fine. we registered at the desk on 4D and asher ran straight into the playroom to play "hippos" (hungry hungry hippos). and......

for the first time ever......

i ordered lunch for Asher!!!!

and he ate it!!!!!

without problems!!!!!!

mac & cheese.

ok, i know, not the healthiest food there is, but hey, it's really just bonus calories right now anyway, and as long as he doesn't drown on it, i'm not being picky! lol

and next time you're on 4D, look around for a gorgeous pic of my gorgeous boy!!! :)

anyhoo... that's all for now. ttyl! :)

"Glife! Bam! teezza!" or, A Boy and His Temper, or Not NPO Anymore (albeit unofficially - wink wink)

... which, translated into actual-speak (as opposed to toddler-speak), is "Blithe! Bram! Pizza!"

so, in case you hadn't yet figured it out, we had pizza for supper tonight. asher's choice. seriously! mom and i were discussing our options (basically just "pizza? or what else is there?"). asher jumped on the "teezza" idea. he even helped nana get it ready! (ok, it was frozen, so he cheered her on). mind you, i don't think he has yet grasped to concept of cooking time. he screamed and wailed the whole time it was in the oven, as though we were depriving him of food. nice, eh?

and let's count the number of things wrong with the previous paragraph. first, we're letting a toddler dictate the menu. next, we're letting a kid with terrible circulation help take food from the freezer. i don't like taking him past the freezer section at the grocery store without a winter coat, mitts and a hat! but there he was tonight. finally, he spent 15 minutes or so screaming (you all remember my dischipline dilemma, right?). that's some great parenting there, let me tell you. lol
anyhoo... eventually, and none too soon for asher, it was time to eat. we put asher in his high chair while i sliced up the pizza. he passed the time calling his sister and brother to the table, and yelling at me to hurry up. yeah, he's a delight today.

i gave him a piece. it's on his plate. now, of course, he's ticked because he has food in front of him and we haven't prayed yet, so he can't eat. (it's almost pavlovian, really.) you can't tell from the pic, but he's the only one at the table.

by now the kids have said grace, and we're digging in. (btw, asher during grace is adorable! he closes his eyes and sort of mumbles along and says "ahmeh" at the end. mom and i are godless heathens, however, because we inevitably just watch him and giggle quietly.)

"don't bother me. i'm eating." 'nuff said.

EUREKA!!!! Oh, dr bertoldi, you’re gonna be impressed, i think! Lol (the rest of you don’t have to read this if you don’t want to. It’s about poop.)

Ok, for those of you who've been privy to my "i was so lost in immunology" rants over the last week and a half (honestly, i have never been more lost in an appointment as i was then! I mean, seriously! Does anyone understand immunology?! Because i'd like to talk to you if if you do! I have questions!), you may remember my confusion over the gastro issue that was discussed. The question of whether Asher is constipated because of nerve damage, as we (esp I) suspect, or if it's something congenital. Immuno's guess was anal atresia that's patent. I think i wrote about it last week. how can you have anal atresia that's patent, i asked. (for those who aren't versed in medi-speak, "atresia" means closed and "patent" means open. So now you understand my confusion!)

well, in my cyber-wanderings tonight, i decided to look up VACTERL (not VECTOR, as i had called it previously. Doc had an accent so i misunderstood). I went to Children's Hospital of Philadelphia (wonderful website, very good information), because that's where i learned about DiGeorge, so i thought i'd at least start there. So i searched "VACTERL." A few things came up, so i went through them. The first link just talked about causes of CHD, and included some info about a few syndromes and genetic abnormalities that contribute to CHD. DiGeorge is one of them. But they also said that some "associations" are linked to CHD. Like VACTERL, which includes Vertebral, Anal, Cardiac, Tracheal, Esophageal, Renal and Limb abnormalities. You'll notice that Asher's limbs and spine are fine, but the rest... yeah. I'm not saying he's got this VACTERL thing, but maybe it's worth pursuing, even if just to rule it out...

ok, so here's the "eureka" part of this story...

http://www.chop.edu/consumer/your_child/condition_section_index.jsp?id=-8782

here's the gist: there are several kinds of anal abnormalities. the first is anal narrowing, which basically means that it's technically intact, but it's narrow, so BM's are difficult, but possible. But every so often, they have to dilate it, to help things go a little easier. Yup, a balloon in the bum. Hello! L another abnormality is a membrane over the anus, so nothing can get out. Eep! and then, there's the whole anal atresia thing. Basically, the rectum and the anus are not connected. This can present one of two ways. First, they could be unattached, but there's a fistula, or abnormal channel going from the rectum to the outside, so poop can get out. Phew! Yucky, but lucky. Or, they're completely disconnected, and there's no fistula. Still yucky, but not so lucky. On the other hand, according to my medical dictionary, when it comes to fistulas, "surgical repair is not always possible." The above link explains the repairs in detail.

What does any of this have to do with Asher, you ask? Well, gentle (and by now, grossed out) reader, allow me to explain further. (and feel free to stop reading now. It's all about poop and bacteria and so forth, and my own opinion, albeit humble and uneducated, on Asher's gastro condition.) ok, here we go.

so, in the case of Anal Atresia, which, for the sake of this blog post, we'll assume asher has, there is sometimes a fistula present. Now, this fistula may not necessarily be a straight tube going straight out. Remember, this is an abnormality in an already wonky physiology, so why would it be so straightforward as that? And remember, too, that the gastro and urinary tracts are smooshed together pretty snugly in there. Not much for wiggle room. Sometimes these fistulae (that's the plural. Don't i look smart?! Lol) kind of connect with the urinary tract. Poop still gets out, and mostly through the bum, but not through the anus, and having taken a slight detour first.

Are you getting this? Alright, poop still gets out, but may sort of collide first with the urinary tract.

In which Asher has had numerous infections.

But not from any reflux in the urinary tract.

Nope. They've done tests, and that's not it. His pee goes the right way, just as it should. It doesn't go shooting back up toward the kidneys.

But what if, and think about this, what if bacteria are getting in there from another tract altogether?

"but, heather," you might ask, "wouldn't that mean he'd have poop bacteria in his urinary tract?"

My answer?

Yes. Quite possibly. It would.

One example of a poop bug: e. Coli.

Can you imagine having e. Coli in your urinary tract? It's possible for girls. Just wipe the wrong way, and voila! But for boys... not so easy.

Or common.

And yet...

Asher has had e. Coli in his urinary tract.

At least once.

I'm not saying, i'm just saying.

Ok, now, i know paeds reads this. So, just think about it. that's all i ask. oh, and then let me know what you think. K? Thanks! :)

i have great friends

yesterday i had coffee with another heart mom... actually, another HLHS mom, to be specific. it was great. the kids were happy to see each other, too... well, they would have been, i'm sure, if they weren't suffering from heart failure and a double ear infection/tube infection, respectively. but we moms had a good time. :) anyway, i was telling her (and i've recently written on My Simple Life) about how horrible i feel with the whole food situation with asher, and the choice i have to make every mealtime. he wants food, but it could kill him, but so would a heart attack from crying if i withhold it from him. well, my friend wrote on my wall on Facebook today. here's what she wrote:

I had a thought yesterday when walking home... about how you feel a little bit of guilt when giving into Asher's wanting to eat... I was thinking there IS going to be a point when he will no longer need the tube right? and sure its dangerous for him to drink and eat...but how is he ever going to learn if you don't give him the experience.... You're a great mom!

you know, this little wall post really helped. what an encouragement on an otherwise horrid day. so, thank-you, T, for being such a great friend. and if you need anything, just let me know. i'm not far if you need me...

Yale update

check out his blog for details. keep him and tanna in your prayers. thanks!

http://babyboyseaton.blogspot.com/2008/10/still-at-sick-kids.html