WOW!!! has it really been 2 months since i last posted?! CRAZY!!!!!

i can't really explain why it's been so long since the last post. not a whole lot going on, i suppose. just regular, normal life stuff, i guess, and really, who wants to read about the days passing in their swirl and whirl and swing and song... that's what My Simple Life is for (please don't click that link yet, lest you see how long i have neglected that blog, too! HAHA!) ok, on to the post!..

like i said, life has been moving along fairly normally over the last couple months, which has been delightful, to be honest. sure, there's been some blips, but nothing that had me in a tizzy or running to the blogosphere for support, so i think it's ok. ;) so i'll give you a bit of a recap:

the kids started back at school, and they're enjoying it. blithe is in grade 4, learning french now, and loving every minute of it. her goal for this year is to get A+ in every subject, and to get into a special arts school in the area for next year. :) she has been really enjoying being on the music and drama teams at our church. she's really blossoming into a beautiful person, and i'm really proud of her.

bram has started grade 2, and seems to be doing fairly well. his reading is really improving, and he's really working hard on focusing and paying attention. his goal for this school year is to "get the exact same grades as last year." his sense of humour, his gentle spirit and love of life are really endearing and inspiring. and in case you're extremely curious, yes, he still has a mohawk. he lets me shave the sides, but not the mohawk itself, which he also won't let me spike. his hair is now almost as long as blithe's at the front! just another one of his little quirks which make him so lovable.

as for asher... well... he's back at school, in senior kindergarten this year. he has the same EA (educational assistant) as last year, which is wonderful. we've all really enjoyed working with her, and asher thinks she hung the moon, so we're all really pleased about that. asher has a different teacher this year, which admittedly caused me a bit of uneasiness initially, but after speaking with the principal and after seeing how asher is doing in school, my concerns are all gone and i'm really happy with how things have gone thus far.

with one exception, which actually has nothing to do with the school itself. asher has already missed about half of the school year due to chicken pox. not that he had them, but in an effort to keep him as healthy as possible, we want to minimize his exposure to these viruses and illnesses. so, home it was for the littlest man. and while i really enjoyed getting to spend so much time with only him, it is so sweet to send him to school, to let him enjoy time with other kids his age, to study butterfly life cycles and learn to read and paint and play at the water table and so on. i am so thrilled that he has this opportunity.

he, however, isn't always so thrilled. i know that, once he gets to school, he enjoys himself and has a great time, but he's really beginning to notice that he's different than other kids. he knows they don't have pacemakers. he knows they don't have EAs. he knows they go to gym. he knows they don't ride on a special bus. he knows. what he doesn't understand is that all of this is to protect him and keep him healthy and safe and give him the best possible school experience, and that we all work as hard as we can to give him as normal a life as possible. he doesn't understand the risks. or he does, and he doesn't like them, so he wants to pretend they don't exist. either way, it's hard to explain to him so that he accepts the situation for what it is. and what it is, is a lot of people who care deeply for him doing everything they can to give him a full, beautiful, Love-filled life. one day, he'll understand, and this, too, shall pass, and we'll get through. we're all aware of his PTSD and other emotional issues.  but when he's crying in the mornings because he doesn't want to go to school, that's really tough on me, especially. but this morning's tears only lasted about 15 seconds, so i'm hopeful it's getting better. when he's been in school for a while, he does better, but since he's missed so much this year, it's rough. he'll adjust and get back into the swing of things, too, and that will help.

as for us as a family, we are now official members at the Y, and we have been enjoying this thoroughly. the four of us go swimming at least twice a week (we try for three, but it doesn't always work out), and we're loving that! we're all in better shape, we're happier, and the kids are all becoming increasingly comfortable in the water. monday afternoon, we went swimming together. blithe and bram put on life jackets and swam all over the pool. asher, who grabbed himself a floaty-belt (don't remember the real name for them LOL), clung to me most of the time. in fact, he's attached to me every second that he's in the pool. but on monday, he relaxed a little, and actually held onto my outstretched arm, put his feet out behind him, kicked, and "swam" while i walked around the shallow end. he's making so much progress in the water! i'm so proud of him!!

blithe is taking Creative Dance, bram is taking Tae Kwon Do, and they're both enrolled in swimming lessons. i had thought of putting asher in swimming lessons, too, until... we went swimming together... and he had an absense seizure on the stairs. while i'm holding him in the water, there isn't a whole lot of risk. but if no one is holding him, and he's in the water, and that happens... yeah. he's not in swimming lessons. :( but i figure, if we go often enough together, he'll learn on his own. he may never be the most fantastic swimmer, but he'll do well enough. his endurance is getting better now as a result of this activity, so who knows. the seizures don't happen very often, so maybe in a few months, once i'm a little more comfortable, i'll sign him up again. because, to be honest, it was his first seizure in several weeks, and it had been a while before that, so i'm probably being overly-careful right now. but this is a new situation for us, so i'm holding it as "caution" and not "paranoia." haha we'll just wait and see how this goes for now, and just have fun all together, like we're doing. :)

um, what else has been going on around here?... well... i had been working quite a bit over the last few weeks while my boss was away on vacation, so that was good. exhausting, but good. i also took some giant steps to pursue one of my dreams. i was the "make-up designer" (that's what it says in the program above my bio!! yes, carrie, i have an extra copy, if you're interested) for Tuesdays With Morrie, which was put on in London last week. it was a beautiful production, and every show got a standing ovation. it was an incredible experience, i can't really say enough about it, and i'm thrilled, honoured and humbled that i was given the opportunity to be a part of it.

ok, now that you're all caught up on the normal real-life stuff...

asher had two clinics yesterday: nephrology and gastroenterology. nephro was awesome! as for gastro... well... nephro was awesome!!! ok, it's not that gastro was terrible. just a bit discouraging. so i'll cover it first, and then we'll part on a renal high note, shall we? (wink)

so, we saw gastro because asher is still suffering from chronic GERD (aka reflux, aka heartburn). it is bad enough that he actually says it hurts and prays that God will "help me feel much better." yeah. he's on meds, but they are really only dealing with the heartburn itself and i'd rather address the cause of the GERD, to be honest. so, off we trekked to the clinic.

what began as "simple" GERD (in quotes because, well, it's asher we're talking about, so "simple" is relative) became GERD with chronic constipation. now, since asher has potty trained, i'll admit, i don't keep track of his BMs. but... when doc asked how often asher poops (hey, it's gastro. what do you expect?! haha!), i stopped and thought about it, and i realized... asher rarely poops. and i mean... maybe once every few weeks. and then i started thinking even more... that would explain why asher doesn't eat much... and probably why he doesn't gain weight very well... and doc reminded me that, if he's full of poop, everything gets backed up and there's nowhere for the stomach to put anything, and that causes more reflux. i reminded him of the vagus nerve damage asher had had, which i believed had healed. but he confirmed that the vagus nerve could still be a factor, since it enervates the esophagus, and if the esophagus isn't getting the signal to send things back down into the stomach, reflux will be a chronic problem.

and the longer i think about this... asher has had issues with his esophagus from the get-go. he had a swallow study done when he was 4 weeks old, just days after being discharged from SickKids the first time. they found a total lack of swallow reflex. sure, stuff went down, and he wasn't gagging very often, but we all watched as the formula (the thickest formula) just kind of spilled down his throat as opposed to being pushed and prodded by the muscles. i mentioned to him that resp had done a CT scan recently and they had found "stuff" in his lungs. this might be due to infections or something, granted, but it's also possible that asher's throat issues have not, in fact, cleared up as well as we'd thought and this is, in fact, evidence of aspiration. sigh...

he prescribed increasing asher's lansoprazole to twice daily, which should help, but again, doesn't address the cause of the GERD. he also recommended a laxative, which might help, as well. if everything is cleared out and keeps moving (the laxative would be an on-going thing), we hope to see a decrease in reflux and an increase in appetite. so, we go back in january for follow-up.

the other issue that came up was one that i have actually wondered about idly over the last couple months: celiac disease. doc seems to think it's a possibility, but he would like to investigate the other queries first before looking into a radical life-style change. which i appreciate. haha but i just might begin working some gluten-free foods into our diet over the next little while, in case this is the issue, and then the change won't be quite so dramatic.

all that being said... as we were walking to the desk to book a follow-up, doc asked, "has asher ever had a study done to see how things work in there?" i told him about the swallow study at four weeks, but there really hasn't been anything further since then. he thought for a moment, then said, "sometimes kids with congenital heart defects can also have a twist or malrotation in their bowel. when he got his G-tube, did they look for that?" i said, "not that i know of. no one said anything to me about it." he is going to be talking to Interventional Radiology (they're the ones who put in the GJ-tube and then changed it to a G) to see if they ever looked, or noticed anything. if they haven't, we're going to look into this right away.

so, what started as "simple" GERD has become something much bigger. and my heart hurts a little bit more today. :( i know it's not the end of the world, but things had been going so smoothly, and then to find out that he might have further issues just leaves me feeling sad. so prayers/vibes/crossed crossables would be very much appreciated.

and now for something completely different.

after gastro and a quick trip up to Tim Horton's (we hadn't eaten all day - stupid ultrasounds! haha), we saw nephro. asher's BP was low by all standards except nephro, because they like low BPs (81/55, anyone?). they say that's "perfect." ok... i'm going with it, since there really isn't much you can do to make his BP go up, so whatever. haha doc looked over the ultrasound from that morning and said, "wow! this is dramatically improved over last time! if you didn't know to look for anything, this would be taken as normal!" i picked myself up off the floor, resisted the urge to hug him, and said, "really?!?!" apparently, there is a small cyst in the left kidney, but it's been there all along, apparently, and hasn't changed size, so doc's not worried about it. in fact, i suspect he would have discharged us completely but for the UTI asher had a few months back, so we go back in a year. that's right, 2012!!!! i don't remember the last time i was so happy leaving an appointment!!

so, to recap, gastro was upsetting but eye-opening and at least we have a plan, and nephro was AWESOME!!!!

and now you're caught up.

have a wonderful day today! i'm so sorry about the length of this post. i'll try to post more frequently to avoid posts like this in the future. :S

you are Loved.

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.

hospital admission #24

hi, folks, heather here. thanks, wendy, for keeping everyone up-to-speed on the goings-on in asherland. :)

shortly after i got here (i went home to sleep... got about 3 hours, but i feel, um... not sore. tired, absolutely, but i'm not hurting, so i'm functional), i talked to liz (london cardiology NP). here's the deal:

the chest drain was placed last night around 10:00, and by the time i was called back in around 11, about 250 ml had drained already. that's a quarter of a liter. (for you non-metric/non-hospital-dwelling types, that's over 8 oz). by 9am today, the total that has drained is over 350 ml; approx 50 ml (almost 2 oz) since 7am. yeah. the drainage was very dark (bloody) last night, but it is slowly becoming lighter (straw-coloured). but because of the high amount of drainage, we are going to be here at least one more night, especially since the air entry in his right lung is still decreased to the base (doesn't go all the way down; basically, he's not taking in as much oxygen as he should be). but if there is a bed open on the ward today, we will be heading up there. otherwise, we're stuck in the unit for another day, but not because we need to be here. as the PCCU doc said to me this morning, "he certainly doesn't look like an ICU patient today." so we (by which i mean, i) are hoping to go upstairs today.

the tube will be staying in one more day at least. the drainage is simply too much to think that we can safely remove it yet. the problem, however, is that the tube they used is not a JP; the one they gave him is very stiff, making it very painful to move, since it gets stuck on things and tends to pull and rip (liz's words, not mine. i was busy trying not to vomit when she said that). so the tube we have now is not conducive to going home, which means that, as long as he has this tube in, we're here. but... liz is going to look into changing the tube to a JP, which is more flexible. if we can change the tube, we can go home with that. she said she could show me how to empty it; i laughed and told her i already know how to do that.

meanwhile, they're going to send some of the drained fluid to the lab today to test for a few things. i know there were three things they wanted to look for, but i only remember two:

1. culture. they want to see if anything will grow. liz thinks it's unlikely to grow anything, but hey, it's asher, and you never know with this kid, so we're trying to cover our bases.
2. re-test for chylo. the NP in toronto tested him on the very cusp of chylo-testability, so just to be sure, we're testing again.

another issue that has come up is that, this morning, asher's belly is round and hard. liz is going to consult nephro. because asher has a history of calcium in his kidneys (nephrocalcinosis, for those of us who like technical terms... which is probably just me... yes, i'm a nerd), we are limited with the diurretics we can use. lasix is the usual go-to for diuresis, but that med tends to cause calcium to build up in the kidneys. the very problem, you'll note, that asher has. yeah. so for a couple years now, asher has been on HCT, which has (generally) been doing well enough, but it's just not strong enough right now. so liz will be asking nephro if we can switch to lasix for short-term use. i'm anticipating that they will ok the switch. dr filler (asher's nephro) has told me a couple times that, if they max out the HCT and it still isn't enough, then they can switch him to lasix temporarily. here's hoping he still thinks that way.) we really need to get this fluid off; it can lead to very big problems for asher: heart issues and blood pressure, kidney issues, respiratory issues... yeah, it's not pretty. this fluid, both in his chest and in his cardiovascular system (which is where pee comes from, for those who wonder why i'd be talking about kidneys and pee but only refering to the CV system), absolutely has to go. we cannot keep it on board.

i will try to update here as often as possible, since i have found a delightful little computer in the unit's waiting room. however, if i can't do a full update, i will post on my facebook and/or text wendy, and she will update the blog for me. (yes, wen, you're forgiven for hijacking the blog last night. lol)

i'm baaaa-aaack... with mixed news

thank-you so much to wendy and mom for keeping the blogosphere up-to-speed on the goings-on in asherland over the weekend. i was much too busy visiting with blithe and bram. and don't worry, wen, you did just fine. lol so thank-you. <3

so i'll get the good news out there first, so no one has a heart attack when they see this on facebook (wendy and tanna, i'm looking at you!)...

asher is now in a much better mood, having visited with his brother and sister two days in a row. he really misses them, and i know they miss him, too, so it was lovely to see them all together. asher and bram had tons of fun playing with the train table in the playroom. the kids were even enough to inspire asher to walk down the hall... under much protest, i should add, but he did it, nonetheless, since it meant play-time with his brother and sister. so that's lovely.

and before i forget, his drainage tested negative for chylothorax. wendy did a great job describing it the other day, but for those who don't remember, a brief re-cap: during heart surgery, it is possible for a lymph node to get nicked. the lymph nodes take fat out of the blood, and when it is nicked, the fat (chyle, pronounced "kyle") leaks out into the cavity around the lungs and heart. left untreated (treatment is a fat-free diet for 6 weeks), it will slowly suffocate the patient. asher had it after his glenn and it sucked, to be honest, so when they were concerned about it on the weekend, i was worried. but it's negative, so i'm happy.

he is also eating more. last night at midnight he asked for eggs, so i got him scrambled eggs and hashbrowns for breakfast, and he ate more this morning than he has all week, which is encouraging. since he is usually a bottomless pit, it has been difficult seeing how little he wanted to eat, but he seems to be returning to normal-ish eating again, so HOORAY!!!

now for the not-so-good news.

my mother posted last night about asher's poor fluid intake. their goal is for him to get 910 ml of fluid a day. by yesterday evening, he'd had 334. hence the threat of an NG-tube. understandably, asher did not like that idea. in fact, he clamped his hands over his nose and started crying, "no! i not want tube in my nose! i not want tube!" i told him that he would have to have a tube if he didn't start drinking. over the next 2 hours, he drank 250 ml. still not enough, but much better than before. and this morning, we have had the same conversation every half hour or so. "asher, do you want something to drink?" "no." "do you want a tube?" "no, i want apple juice, please." works like a charm; he's had almost 200mls so far.

now the bad news. sigh.

asher's blood pressure has been (thanks to his special "old man cocktail" of a diurretic, ACE-inhibitor and beta blocker) perfect for several months now. 80s over 50s. beautiful. but over the last couple of days, it's been going up. and up. and up. yesterday at one point, it was 114/65. to put that into perspective, mine is 110/70. yeah. he isn't as puffy anymore, thanks to  a combination of 2 diurretics (HCT - his usual one - and metalozone. no lasix for asher because of his kidneys), but there still is some residual puffiness around his eyes, but he can open them fully, so no big deal. that being said, the only reason his fluids are even balanced at this decreased level... is because of the chest drainage. even with 2 diurretics, he isn't peeing as much as they'd like. however, last night he was refluxing pretty badly (i could hear it, and for the first time in his life - and remember he has a history of "severe GERD" - he was complaining that it hurt) and he was sweating. and by "sweating," i mean that he soaked through his sheets and jammies in 10 minutes. soaked them! you could have wrung them out! disgusting!! and in 10 minutes. yeah. the nurses kept asking if he's like this at home, because some kids sweat in their sleep. i told them that asher usually sleeps under 3 or 4 blankets and he never sweats. the nurse... the heart nurse... who deals with this sort of thing all the time... was really worried.

now, i know there are some of you out there who read this paragraph and have small coronaries of your own. yeah. that's about where i was at last night. and i'll admit, i'm still a bit concerned, since asher is still very pale. but, i'm aware that there are some of you reading this who are scratching your head and going, "why is heartburn and sweating so concerning?" so here you go... a crash course in severe CHD, ie, the list all severe heart moms memorize shortly after diagnosis:

• paleness and increased cyanosis (blue spells)
• puffiness
• increased blood pressure
• decreased urinary output
• irritability
• reflux/heartburn and vomiting
• decreased appetite
• sweating

read this list over again, then re-read my description of him above. yeah. quite similar, wouldn't you say? and for the uninitiated... that's the list of symptoms of CHF. congestive heart failure. yeah.

so please continue to pray/send good vibes/cross your crossables... whatever it is you do, please keep doing it. asher has a looooooooonnnnnnnnggggggg history of CHF (hence his old man cocktail, which he has been on for quite some time... like, forever) and believe me, it's just as scary now as it was when i first brought him home after the hybrid. what's even worse is when you're used to it... which i am. and when you expect it... which i do.

so there you go, the latest update. i'm off to grab a coffee and some lunch and head up to order asher's lunch. how awesome is that, eh?? i actually get to order him lunch, instead of going to the kitchen to grab a bottle of formula!!! i could get used to this! although, i have to say this... i've waited so long to see asher eating and drinking, swallowing with ease... but now i'm disgusted by it at the same time because honestly, he's the loudest swallower i've ever met!!!! and frankly, i don't think there's a more disgusting sound in the world than the sound of swallowing. blech!! and i'd feel guilty about saying any of this, except that wendy, another heart/tube mom has also heard asher drinking, and she agrees. yuck. LOL

because it's asher, and nothing is ever straightforward with asher

so, once again, asher is doing what asher does.

making something simple(-ish) into something far more complicated.

hence the nickname "asher crasher."

asher has had a bit of a fever all day, and has been tachypnic (breathing fast) off and on all day. he isn't working to breathe, there is no distress, but his resps tonight are 64 to 67 breaths per minute, when his baseline is mid- to high-30s. when the doc came in to check him tonight (after the nurse went to get her), she found that air entry is decreased to the bases, which means, in real english, that when he breathes, the air isn't getting down to the bottom of his lungs. his breathing is very shallow. and that's a problem, since it means that he is not taking in as much oxygen with each breath as he should/could be, which means, predictably, that his sats are lower than they should be. mid- to high-80s, when they have usually been in the low- to mid-90s.

the cause, in my own humble opinion, is that they were a bit too aggressive with asher's chest drains today. just this morning they took the drains off suction and removed one of them. this left the only remaining drain to remove all the fluid, which is fine, but it wasn't set up to suck it out anymore, which makes it more difficult for the fluid to get out. this, you can imagine, causes fluid to build up in the chest, which takes up more room, creates pressure around the lungs, and prevents them from filling properly with air which is needed to oxygenate the blood, thereby lowering sats. when they removed the left JP today, the entire length of tubing inside was clotted. basically, a foot-long blood clot. in my opinion, this made it well nigh impossible for fluid to drain through that tube, which led them to believe that the drainage was slowing enough to remove the tube.

this evening, they have put the remaining tube back on suction, and lo and behold, more drainage! more in one hour than all day, in fact! they are currently monitoring asher's breathing, and if it does not improve (slower rate + increased air entry to bases), we will be going for a chest x-ray.

asher had been doing so well up until now. it's just a bump, but a stressful one, nonetheless. and when you figure in the fact that he does not like to move, going downstairs for a chest x-ray will be unpleasant to say the least. so please pray/think positive/send good vibes... whatever it is you do, please do it now. the doc will be reassessing at midnight, and we would all like to find better air entry and continued/increased drainage. thanks so much!

poor little thing...

asher's not doing so well emotionally right now. he's become very sad; i'd almost be tempted to call it depressed. he doesn't want to play, he doesn't want to eat, he barely drinks, he doesn't want to move at all... he just wants to sleep.

that being said, i've ordered meals for him today, and breakfast and lunch weren't even touched. but when i told him i was ordering supper, he helped me to pick out some things (fyi, grilled cheese, fries, chocolate milk and chocolate pudding. poor choices nutritionally, perhaps, but remember, he may not even eat any of it, so it's moot, anyway). so we'll see how that goes.

his central line (in his jugular, right side of his neck) and pacing wires came out this afternoon, and he did really, really well. complained a bit at first, but he didn't even move while the nurses worked. he was so brave. i'm really proud of him. :) he still has two lines in his right hand, and his nurse was hoping to take them out this afternoon, too, but they have to decide what they're going to do about his diurretic, since the HCT alone isn't enough right now. he is still very puffy all over, and that is part of his problem with his sats. his lungs sound clear, however; there just seems to be a pocket of fluid floating around in there around his lungs, but moving around will take care of that... once he starts moving around, that is.

anyway, i'm gonna run for now. he's sleeping, but i don't know when he'll wake up, so i should get back to him. i'll keep you posted. :)

because it's never straightforward in asherland...

yesterday it looked like asher's drainage was slowing down, but it turns out... not so much. it's just that there were clots high up in the tubes. the nurse last night milked them out, and suddenly there was tons of drainage. and i mean, she got 60mls (2 oz) out, and 20 mins later, got out another 20mls. it's still clear red, which means (for those who know) no chylothorax. so that's a relief. but asher's right lung is crackly and his breathing is very shallow, due to the fluid and pain.

he's back to getting morphine, tylenol and katorelac (i still spelled that wrong, but i'm getting closer, right? lol). pain management and drainage are really the only issues now. so that's awesome! :)

he was also nauseous and vomiting last night, and that was yucky, not to mention painful for him. but i'm going to order him something to eat now, so we'll see how he does with that. nothing big. just something little and easy on his stomach, since he still hasn't eaten. he's been taking some ginger ale and i think that's helping.

oh, and before i go, just one more thing...

WE'RE IN OUR OWN ROOM!!!!!!!!!!

yup, it's time for the fontan...

so, yeah, asher hasn't been doing so well lately. all week, actually. he's not sick or anything... unless you count heart disease, in which case, he's one sick little boy.

i took him to see the paediatrician yesterday. all week, asher has been blue, lethargic (on wednesday, he fell asleep on the couch at 5pm and slept straight through till morning... in his bed, though), irritable, not eating as much, sweating in his sleep, puffier than usual, and his hands and feet are cold. actually, i think he's cold all over lately; he never wants to take his coat off anymore. so i took him in, and when she assessed him, she found that he's not tachypnic (breathing fast) or tachycardic (rapid heart rate), but his liver is low. and she knows, because she checked it last week when i took him in. so it has definitely lowered.

(who cares about his liver, you ask? when the liver descends, it is an indication of heart failure. the heart swells from working too hard and the influx of blood to help increase function, and this requires more room. so the liver gets pushed out of the way. it's not as low as it has been in the past, mind you, but it's working its way down, enough to be concerning.)

so, basically, the fontan is coming just in time. please pray/cross all crossables/send good vibes/whatever you do that asher remains otherwise healthy, since clearly this surgery is needed right now. if he gets sick, they will have to cancel, and that will be bad.

and it's 11 days till the fontan. click on the title of this post and scroll down till you see "The third stage of the Norwood: the Fontan operation." that tells you what you need to know, if you're curious about what is involved.

What they’re planning on doing to Asher in the near future (WARNING: don’t read if you don’t want to know, or if you have a weak stomach)

I promised a while back that i would post the details of asher's upcoming surgeries as the time approached. Well, i have some time right now, so i guess, there's no time like the present. <shrug>

On Wednesday of this week (yup, only 3 days from now), they will be doing yet another pre-fontan cath. This is a diagnostic test. they will be looking at function and pressures in his heart and aortic arch. To do this, they will insert catheters into veins and arteries in his groin, and on each side of his neck. Then they will inject a special contrast dye into his bloodstream and watch where it goes, how fast, that sort of thing, using special x-ray equipment and such.

The docs in Toronto will use the information they gather on Wednesday to determine when they will do the next two surgeries.

That's right.

Two surgeries.

The first surgery will be asher's second arch reconstruction. This is necessary because of the special (read: new) technique they used during his first reconstruction, when he was 6 months old. At the time, they used the stent and tissue in his PDA (the ductus that allows blood to by-pass the lungs in utero, which they stented when he was 17 days old) to reconstruct his aortic arch. Well, asher being asher, he complicated something that should have been very, very simple. We all know of asher's fondness for scar tissue, and how much he loves putting obscene amounts of it around anything put in there by someone other than God (the surgeon's ego notwithstanding, lol). So asher's aortic arch is now full of scar tissue, which poses a number of problems. First, scar tissue doesn't grow, which means that, as asher grows (and the amount of blood in his system increases), his arch doesn't. So now he, at 3 years and 30 lbs, has more blood trying to flow through a vessel that hasn't grown since he was 6 months old and weighed about 17 lbs. That's a problem. The pressures in the arch are mounting (dr benson was freaked out by it back in February), and this problem is affecting the pressures and bloodflow further and further back through his cardiovascular system. this needs to be corrected.

And here's how they're gonna do it (skip ahead if you don't want to know the specific details): the plan is to go through his left side, under his arm. They can't put him on by-pass because it returns the blood to the body before the arch. So you can imagine what would happen if you had blood flowing through the arch while you're operating there. Not pretty, to say the least. So they have to put him on circulatory arrest <wave of nausea>. To do this, they have to lower his body temperature to the point where all function in his body (heart, major organs, brain) ceases. Basically, they press pause on the DVD-player of his body. Then they will do the surgery, removing the stent material (wire mesh) and scar tissue from the arch (i know how they do this. Trust me, you don't want to.) once they're done that, they close everything back up, and begin to return his body temperature to normal. And they cross their fingers, say a little prayer, and hope everything starts back up again. Excuse me while i vomit.

K, i'm back.

(you can start reading again, if you skipped ahead.) A week later, they will do his fontan. The reason for the delay is this: pressures. The arch reconstruction and the fontan will each result in significant changes in the pressures in and around his heart, and if those pressures fluctuate too widely in a short period of time, bad things can happen. So we don't want that. I'll take the extra time in Toronto, thank-you very much.

So here's the details of the fontan (this one isn't as bad as the other): there are two major veins that bring the blood into the heart. The superior vena cava (SVC) brings blood from the head, neck and upper body; the inferior vena cava (IVC) brings blood from the lower body. The Glenn shunt (which asher had at 6 months) removes the SVC from the right atrium (first collecting chamber) and attaches it to the right pulmonary artery (RPA - vessel that carries blood to the right lung). This procedure creates a bi-directional blood flow (blood travels both ways through the pulmonary arteries) to the lungs. A fontan circulation requires that the IVC also be attached to the RPA. So they connect it at the top, and put a wall in between the IVC and the right atrium, which stops blood from going into the heart. Once the fontan is complete, his circulation will become passive, meaning that it skips the heart, goes straight to the lungs, and only comes to the heart once it has been oxygenated. One pump sends the blood throughout the entire body.

Now, as i mentioned earlier, the change in pressures during the fontan is big. So, in order to lower the risk associated with pressure changes, they leave holes (fenestrations) in the wall between the IVC and heart. These holes are closed in the cath lab, usually about a year later.

Oh, did i mention that this is open heart surgery? Looking back, i don't see that anywhere. So yeah, it's open heart.

Asher's cath is this coming Wednesday. And lately, while he has improved somewhat as far as energy, eating and mood, he has been grey – yes, grey – since yesterday afternoon. And if we know anything at all, we heart moms know that grey = bad. But at least he's doing it now, right before the cath, so they'll find what's going on and deal with it.

Ok, there you go. A long post, a thorough post, but i thought i'd get this out of the way while i can still stomach the details.

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

worst. choice. ever.

ok, this will be quick, because i'm nauseous thinking about it.

doc karin came in tonight to check on asher, and in the course of her assessment, she saw:

• he doesn't like turning his head
• he has a headache
• he looks terrible, even after 24 hours of two big antibiotics
• photophobia. which means that he's afraid of light. she went to look at his throat, and he freaked out, squeezing his eyes shut, covering them with his hands, and freaking out.

so here's the thought:

meningitis.

now, if it's viral, there's really nothing we can do, other than treat his symptoms and let the virus run its course. if it's bacterial, we'll need to attack it with meds.

so they're doing a lumbar puncture (spinal tap) tonight at 11. i'll post afterward with details.

plus ca change...... sigh....

ok, so remember the other day when i said that everything was going swimmingly and asher has never been healthier and more stable than he is right now? yeah. forget that. sigh.

as you may or may not know, summer is not asher's friend. he loves the sunshine, and he loves being outside, playing in the sandbox, driving around in his little tykes car, drawing with sidewalk chalk, riding his trike, you know, all that fun - and normal! - 2-year-old stuff. but, alas, summer does not return to love. heat and humidity really take a lot out of him, unfortunately. and since we live where we do, there's a lot of humidity. (as for heat, yeah, where's this global warming we're being told about? i mean, would it be too much to ask to have temps above 23C?!?! i mean, seriously!!! anyhoo...)

due to the heat wave this week, asher wasn't looking so good. very pale and sweaty, not much energy, and so on. and blue spells. yeah. he's been having blue spells all week. but again, summer is not an ideal time for my little man. so he's been looking pretty bad this week. but, the weather wasn't nice, so i didn't think much of it. just brushed it off, knowing this is what happens. (that being said, he slept at his dad's place on monday night, because his apartment is cooler than the upstairs at my place, and since ash was looking pretty rough at dinner in the cool kitchen, i sent him to ed's.) but other than that, i brushed it all of.

and then came a storm on thursday, and that broke the humidity nicely. so friday was GORGEOUS!!! asher spent most of the day outside playing, making wonderful memories (i hope) with his brother and sister in the backyard. but...

he turned blue again a few times. not too badly. i mean, i've seen him worse, and recently, at that. but still, it wasn't hot, it wasn't humid, so really, ash should not have been doing this.

at dinner on friday, he was blue. and i don't mean a pretty shade of baby blue. i mean, his mouth was navy blue, and his hands were blue-grey and COLD!!! so needless to say, i took him to emerg. if it was still hot and humid, i wouldn't have thought much of it, and moved on with my day. but it wasn't. and he was not looking good. still playful, mind you, so i wondered if i was overreacting a bit, but i can't really be too careful with ash when he does this, so i took him in.

and of course, true to form, we got there and he was great. good colour, playful, vitals were great. seriously, he played the whole time we were there.

and the resident came to see him, and i said to him, "yeah, he's looking great, he's got lots of energy, he hasn't had a blue spell the whole time we've been here, so i'm happy to just take him home now." he seemed cool with that, so he went to chat with the attending. who sent in a nurse to check his sats (can't send him home with blue spells without checking the sats). and they were... sit down... 69%!!!!!!!! and since ash usually sits in the low 80s, this was a HUGE problem. we moved the probe around, putting it on his finger, his thumb, and still 69-74%. so we went out into the hall and tried another machine, hoping it would give us a better reading. nope. still low 70s. ugh. so we had him sit down, and put the probe on a toe. and it was still low 70s. after a few minutes of resting, though, it crept back up to the low 80s. but again, that was after some rest. so....

chest x-ray, ecg, and bloodwork (just a cbc this time, so they did a finger poke). everything looked fine, although his haemoglobin was only a bit higher than cardio wanted (they said, "if it's below 130, we'll have to admit him" which means transfusion). (and why do we care about haemoglobin? haemoglobin is the fancy-schmancy name for red blood cells. these carry oxygen to the body. hypoplasts/single ventricles usually have high haemoglobin to compensate for the low oxygen levels. if it gets too low, then there won't be enough oxygen in the blood, which shows up as low sats. transfusion = higher haemoglobin = higher sats. there you go. a quick phlebotomy lesson. feel enlightened? you should. lol) ok, so cardio wanted to see something higher than 130. asher usually sits in the 170s at least. last night, he was at 140. so we got to go home! hooray!!! but wow! that was close, eh?? eep!

well, then this morning, blithe and bram got up at their usual 7:30-ish. which is usually when asher gets up. and he woke up. crying. so he came in and cuddled up with me. and fell right to sleep (with charlie. seriously, he was using the dog as a pillow. too cute!) and he stayed asleep. all morning. i got up around 11 (we didn't get home from emerg last night till after 1, so i was i tiiiiiredddd!!! and i thought ash was feeling the same, so i didn't think much of him sleeping in. though, i'll admit, that boy can run on surprisingly little sleep sometimes, and he never sleeps in. he gets up with the other kids every day, no matter what) and i thought ash would get up at the same time... which he didn't. he just stayed upstairs. so i let him stay, thinking he was playing with one of his toys or something and would be right down. nope. he fell asleep again. which he doesn't do. ever. i went up to check on him after a while, and he was feeling pretty warm, so i grabbed a thermometer and.... 38.1!!! i even checked twice, just to make sure!!! and it was under his arm, which means his "real" temp was almost 40. (for those who don't live in canadian hospitals, let me convert that for you. 38.1C is 100.5F. 40C is 104F. yeah.) so after a quick consult with his homecare nurse, we trekked off to emerg.

they did some bloodwork, and it turns out that his white cells are WAY higher than they were last night, and now his bicarb is low (which means his blood is more acidic than usual. i can't explain this part. i used up all my knowledge of phlebotomy earlier. sorry). so doc decided to admit him for a few days, at least until his blood culture comes back on monday. this way he can get some IV antibiotics to help him fight off whatever it is that bugging him right now. they're assuming it's viral, because they can't find anything. no sign of infection in his ears, throat, tube site or urine.

and earlier, when i got back from getting some clothes etc, he was on oxygen. it seems that while i was gone, his sats dropped down to the low 70s again, and the docs ordered low 80s, so they had to put him on O2 for a while. right now he's about 79 to 83%. once he falls asleep, those numbers should go back up. (hypoplasts/single ventricle kids are the opposite of the rest of us. when we sleep, our oxygen levels go down. when they rest, theirs go up.)

so now we're in his room on D7-400. he's febrile again, a little over 39 degrees under his arm (which means it's a little over 40), so now we're watching for seizures and waiting for the tylenol to kick in. and in the meantime, he's watching treehouse (go, diego, go... thrilling... why does he shout so much?!?!?!?!?!) and i'm blogging and chatting with a friend.

so, yeah. we're here for the weekend. but i'm reachable. i'll be online a lot of the time, and i have my cell phone on (i'll post the number on the sidebar, in case anyone wants to make arrangements to drop off a chai latte for me, hint hint). so, yeah, basically, we're just hanging out here for the weekend. i'll keep the blog updated as i know things (like what it means that his bicarb is low... you know me, i'm determined to figure that out!! LOL), and any results from his culture. we're crossing all our crossables that this isn't sepsis, but i'm maintaining that it's a virus, since he's done this twice before (last november, and again in may), and it was viral. so yeah, we just have to let this run its course, and then we'll be home. i don't think we'll be here long.

i'll keep you updated. i'm on FB, and i'll be keeping my status updated, and i'll update here, as well. so, i guess i'll talk to you later. :)

How have i not posted about all this yet?!

It would seem i've been somewhat remiss the last couple weeks, not posting the latest news in Asherland. So, my apologies. And now i'll attempt to make up for it.

As you may or may not recall, Asher was admitted the other week to SickKids for the weekend for some good old-fashioned observation. They saw him desatting to the 50s and 60s, high blood pressures (and by high, I mean, higher than mine!), and his heart rate jumping up at the slightest activity. and just so we're clear about this "jumping up" comment, let me give you a couple examples:

• He sat up in his crib, and his HR jumped to 140. When he sat up. Yeah.
  
• He walked around his room for 5 minutes. 150. And short of breath.
  
• He walked around the ward for 10 minutes. 170. And desat to 59%.
  
• He took 10 steps across the play room. 155. Sats in the mid-60s all afternoon.
  

Ok, i realize that those numbers don't mean much to most of my readers, but that's ok. You heart moms and docs all know what i'm talking about (oh, please, Pepy, don't read this post. LOL). For those of you unschooled in the basics of Paediatric Cardiology, i'll say this: those numbers? Yeah. Not good. Asher simply should not be doing this at this stage. Or ever, in my opinion, but what do i know? Well, other than knowing that oxygen is good, and heart attacks are bad. (ok, fine, Wendy, i know a lot. Sheesh! i can hear you protesting from here! LOL)

When we were discharged from SickKids on the Monday, we were sent home with a Holter. Gosh, i love those. <rolls eyes> i understand that they're necessary and help detect rhythm issues and that sort of thing, but let me tell, it was not invented by a heart parent. Ah, well, i guess if it helps them to figure out what's going on in Asher's heart, it's a good thing. I guess. LOL

The other thing is the cath. Asher is scheduled for a cath on Wednesday morning. Don't know what they'll do for this? Well, let me tell you: they're going to be checking pressures and that sort of thing inside asher's heart and vessels in the area around his heart and lungs. How do they do that? Well, for starters, they will be inserting a long tube through a vein in his groin/hip area (the femoral vein) and traveling up that vein and into his heart, where they will be injecting dye into his heart and using that to look around. Inside his heart. Blech. They will also at this point check his pulmonary veins, those vessels that bring red (oxygenated) blood from the lungs to the atrium (left atrium in us, common atrium in Glenn kids). Once they've checked that stuff, they will remove the tube from his groin, and put another one into a major vein (the jugular, i believe, but don't quote me) in his neck. They will then travel down that vein and into his pulmonary artery branches (the vessels that take blood from his head, neck and upper body into the lungs. Blood from his lower body doesn't go the lungs yet). They will be looking around and checking the pressures in the PAs tomorrow, as well, knowing that the pressures were high in May after his surgery, and that Asher is very fond of scar tissue, and he has some lovely patches in there thanks to Dr Caldarone.

Needless to say, Wednesday is going to be a rather freaxious day. I'm not really looking forward to hearing what they find. You all know i have my theories. But to be honest, i don't know what i'm hoping for. I think i'm hoping that they'll find something wrong, because then they can fix it and Asher will improve. And if they don't find anything, then what is causing all these symptoms?!

So that's heart stuff. I will post results of the cath as soon as i can.

In other Asher stuff... we have nephro (kidneys) clinic this morning. Ultrasound, bloodwork and doc. And how much do i love our nephrologist?! Lovely, lovely man. Very nice. I don't think we'll be getting any real news today. The HCTZ has been helping to lower asher's blood pressure and reduce the calcium build-up in the kidneys, so i don't think that will be too much of a concern today. Calcium in the kidneys is not a good thing. And asher had quite a bit of it at one point, but in a delightful, non-Asher-esque development, it has been getting better over the last year or so, and i can't imagine that anything there has changed.

Let me see... anything else to report?... ah, yes. Got a call from genetics in Toronto the other day, and we have an appointment scheduled for April. I don't know too much what to expect, although i know i will be receiving a form in the mail in the next little while which will ask all about Asher's family history. That should be interesting. I'm (almost-ish) looking forward to this appointment, i think. I would like one of two things: either we will get some answers, or they will see that HLHS and his other issues have just "happened" and then they'll leave us be. (and yes, i know... the odds of a hospital leaving us alone would be a miracle, but a mom can dream, no?)

So, there you have it. The low-down on Asher's medical stuff right now. I'll post again when i have some more info for you. Meantime, please pray for my boy, and for us all. Thanks so much. Talk to you later! J

VICTORY IS MINE...ish...

for those of you who don't know, asher was admitted to sick kids on friday after his emergency sedate echo. dr russell had ordered it after i called her, and after dr b called her, after some, um, issues with london cardio. what's happened to them there?! asher's own doc doesn't know his diagnosis and doesn't recall meeting us. and then there's the incident with a certain NP all but hanging up on asher's paediatrician with a "it's not cardiac so you're going to have to figure this out without us."

so, asher was admitted on friday for observation. he spent saturday morning sitting calmly in a stroller while i was out (have you ever known asher to sit quietly for a couple hours? no, neither have i. this would be the "lethargy" i'd been talking about). once i got back, we went for a walk on the ward. after less than 10 minutes, asher sat down in the middle of the hall, dark blue, with sats at 59%. he was short of breath, and really didn't have the energy to get back to his room on his own. after a nap (2 hours), he played in the playroom. where his sats were in the 60s. and he kept having blue spells.

the next morning, he walked around his room, and after 5 minutes, he was huffing and puffing like a 50-year-old, 300-lb chain smoker with asthma after shoveling a driveway in february in hamilton. (pretty picture, no? lol) this was mentioned on rounds, and the cardio, after reviewing the episodes from saturday, and getting that report from the nurse, described the symptoms as "very concerning."

asher's issues on sunday weren't so much blue spells or sats (though he sat in the low 70s when awake). no, it was his heart rate and blood pressure. BPs were around 110/70, and his heart rate was all over the place. he sat up after a nap, and that sent his heart rate over 140. his walk to the playroom? yeah. 170. and when he took 10 steps to the shelf and back to the table where he'd been sitting, 155. and desat.

this morning on rounds, dr d thanked me for giving them a puzzle on a monday morning. haha, very funny. but she discussed the whole thing with dr r, and came back with this: asher's not going to have a heart attack in the next couple weeks so he's safe to go home. but his symptoms were serious and concerning and require further investingation. so.....

we came home today. they're doing a cath on the 4th, and they're going to look at pressures all over the place. apparently, the pressures in the PAs, around the glenn, were somewhat high in may, so they're starting there. and given asher's love of scar tissue, they're going to check out the pressures in the PVs. we also came home with a holter.

so, drs r and d discussed asher's case today, and they decided on the cath. but they also sent us home b/c these episodes don't seem to cause him to cause him much distress when they happen, and they don't think he'll have a heart attack in the next couple weeks, so he's safe at home. but you'll notice the short date. nice, eh? ugh.

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! :)

the good, the bad and the ugly. well, i don't really know what the "ugly" would be here, but it's a catchy title, so i'm going with it.

i decided to post about asher's appointments all at once, saving us all some time. however, if you're like one of my friends, you've been going crazy (however imperceptably *wink*) waiting. well, the wait is over, my friends. here we go:

CARDIOLOGY (monday afternoon)
no echo, no ECG. they did these recently already, and they don't like to do too many tests. asher's weight is now 12.1 kg (a little over 26 lbs), though just how much of that is fluid and how much is him, i can't say. as for height, he is now at 79.5 cm (32"). that's quite a spurt: after his surgery in june, at post-op clinic, he was 75 cm. so he's gained almost 5 lbs in less than 2 weeks (can you say "fluid overload"?), but he's grown about 2 inches in the last 2 months! yikes! no wonder he can pull stuff off the bathroom counter now (yet another reason we keep the bathroom door closed. immunodeficient toddlers don't play in toilets. well, asher does, but he shouldn't. anyway...) ok, enough of the fluff, on to the meat:

liz was not impressed with asher yesterday. not that she should be. i can't imagine it's a good feeling to see a patient in congestive heart failure. and that's what this is. they can't understand why it's happening, though. yes, fluid overload, but... it came on so fast. we've been upping the volume of formula for a while now, and we've been going pretty slowly. but this came on somewhat out of the blue in the last week or so. sure, there were hints that it was coming (remember the trip to emerg when he was grey? yeah), but once it got going... well, let's just say that asher's reputation of crashing fast is well deserved. he was so short of breath during clinic. and while the colour on his forehead was better, liz said that his nose, mouth, hands and feet are blue/grey. she was also unimpressed by the puffiness around his eyes, and as she told him, "children your age shouldn't have folds around their ankles anymore." to which i replied, "what ankles?" she just kind of nodded reluctantly.

i asked if she had any ideas why this happened. she can't explain it, so we were sent for bloodwork. she wanted to check a bunch of things, but one of the main tests was to check his albumin levels. (your phlebotomy lesson o' the day: albumin is a protein in the blood. it's the largest molecule in there, and it's the part of the blood that holds water. if your albumin is low, there's not as much in your blood to hold in the water, so fluid just leaks into all the tissue of your body, causing puffiness, shortness of breath, etc.) well, it's now tuesday night, and i have yet to hear from liz, which means albumin is fine. if it were low, asher would have to be transfused, and then we'd get into that whole "CMV-negative and irradiated" issue, because of course asher can't have the regular blood products off the shelf. it has to be special. can he do nothing the easy way? i mean, seriously!!! anyway... albumin is fine, which means no transfusion. but, that also means... and, um, how can i put this? ah, this captures it nicely:

WTF???????????????

ok, so cardio was a roaring success. yeah, right. i checked out the orders for the bloodwork on our way over (you know me: hand me a piece of paper and i'm going to read it, regardless of whether it makes sense to me or not. this is how i learn too much, folks). and right at the top, it said this: REASON FOR TEST: HLHS w/ CHF & cyanosis. well, isn't that special. if you don't know what that means, thank the Good Lord. if you do, you might be vomiting with me right about now.

yeah, liz was right on friday. asher should be doing better than this.

and now moving on....

NEPHROLOGY (tuesday morning)
well, this is the "good" i refered to in the title. here's the deal:

asher's nephrocalcinosis, the calcium that has built up in his kidneys, is decreasing!!!! there's less in there! that's the miracle of HCT, my friends. this is what happens when nephro and cardio work together on a situation (back in march, asher needed a BP med, but he was still on the study drug, so they wanted to stay away from that same class of meds. so nephro decided on a diuretic, but since lasix would cause more calcium to build up, he consulted with cardio and they decided on HCT. the whole incident brought a tear to my eye and was, for so very many reasons, a dream come true. all you SN parents reading this know what i mean. sub-specialists never consult with other specialties. it's like there's no other organs but theirs. i'm not joking. so this was just beautiful.). so, yeah, less calcium means the filters are not as clogged, which means less chance of infection and better kidney function. that's right, folks, IT WAS ALL GOOD NEWS!!!!
but wait, there's more:

WE DON'T GO BACK TILL FEBRUARY!!!!!!!!!! THAT'S 6 MONTHS AWAY!!!!!!!!!!!!!!!!

honestly, what am i going to do with all that time (other than cardio and CV surgery stuff, i mean, duh!)?! do you realize that we have never been able to go 6 months between appointments with any of asher's doctors?! this has never happened before, and to be honest, i'm still a little shocked. i mean, this just doesn't happen in Asherland! not that i'm complaining, mind you. honestly, i could have hugged dr filler when he said that... if i hadn't been lying unconscious on the floor! lol so, yeah. good news from nephro. yay!!!

so there you have it. the good, the bad, and i mentioned the ugly yesterday (which would have been my "wardrobe malfunction" during my meeting with dr a). so, that's it for today. i thought i'd end with the good news. oh, crap, i forgot one more thing from cardio. so i guess we're ending on a downer (again). sorry. but here it is:

liz wants to see asher again in 4 weeks. last time it was 6 weeks. now it's 4. weeks. not months. weeks. notice, the appointments are getting closer together. they don't do that because they like to see your smiling face. nope. means they need to keep a close eye on you. and, if you've been keeping track, asher's next cardio appointment is next week in toronto, and liz knew that when she said 4 weeks. so, average that out, and we're looking at 2 weeks between appointments right now. why does this feel so familiar? oh, right. it was like this pre-Glenn. the first stage. yup, wanna vomit. anyone else? ugh.

well, i think that's it for today. for real, this time. promise. and hopefully, i'll have nothing to report for a while. talk to you later. much later. ttfn.

please pray...

we have a busy couple of days ahead of us this week. and that, of course, also means a stressful couple of days ahead of us.

today (monday) is cardiology in london. echo and liz. hopefully that will be it. i don't want to see any cardiologists, though i'm sure we will. asher's not doing so well, what with congestive heart failure (CHF) and all. i think some of the extra fluid is starting to come off, although when i went to bed last night, his head was very puffy and he vomitted after his meds. not good signs, my friends. he has about an extra kg (2.5 lbs) of fluid on board, which is not a good sign.

and just so you know the specifics of what we're facing: i took asher in to see dr b last thursday, but i already wrote some of that visit. you can check out that post later, i think the title has something to do with being desperate to understand medical terminology you have no need to understand (i can't imagine anyone being like that, can you? *wink*). well, she didn't like how he looked. bad colour, vomitting/refluxing, puffy (she made reference to his new "elephant ankles"), shortness of breath. i mentioned that his liver is enlarged and down, and she confirmed it. (FYI: the liver swells and drops because in CHF, the heart swells. this pushes the liver down out of its comfy little nook above the stomach. i'd like to know the long-term effects on the liver of repeated CHF, so if there's any hepatologists or ambitious cardiologists reading this who have the answer, please let me know. post a comment or email/facebook me. i'm serious. you may think i'm joking, but seriously, i need to know.) the liver thing, yeah, kind of a biggie symptom of CHF. ok, what else? oh, yeah. on friday, i took asher back in to see dr b, because now he's irritable. and yes, that's yet another symptom. (are you seeing any trends here?) she looked at him, and his eyes were puffy (or, puffier) and he was really working to breathe. sats are down, though not much (he had been mid- to high-80s for a while, now he's low 80s), and his breathing was wet. none of that is good, folks.

well, she called cardio and talked to liz, who talked to dr hamilton, who talked to dr russell (our toronto cardio). liz called dr b back, and said that we didn't need to come in that day (which was good, since we're going in today), but that "asher should be doing better than this." isn't that a delightful little comment? thanks, liz. ugh. but oh, wait, it gets better. dr b said to me, "has anyone talked to you about transplant yet?" well, to be honest, yes. but it was only when i asked specifically, and it was toronto cardio dr russell, and she said, "likely when he's a teenager." but that was when asher was only a couple months old, and we didn't know about his other issues, and he hadn't yet had all those infections that take a lot out of an already severely wonky heart.

so, yeah. liz isn't happy, the "T" word was spoken (albeit by paeds, not cardio, but you can bet i'll be asking about it now!). and that's our cardio prayer request.

my other request is this: after our appointment today, i'll be paging dr a, the top doc i spoke with last week (see the post called, "ok, i realize that in a perfect world...". why are my titles so long sometimes? ah, well, i'm heather, i talk. get used to it. lol). anyway, i'll be talking with dr a this afternoon about solutions to the whole "i never want to see that particular doctor again, thank-you very much. asher's too fragile, doc's too dangerous" situation. so far, i have this:

1. try to come in between 3pm and midnight. usually do-able. CHF is slow, but requires attention, so if asher looks bad in the morning, i can wait a couple hours. sometimes.
2. during the day, i can call ahead to emerg to see who the attending is. if it's this particular doc, i'll call liz and see if we can go straight to clinic instead. that should work, since they're not too keen on that doc dealing with asher, either.
3. overnight: i pray to God we never need this one, but it's the only thing i can do. call an ambulance. that will send asher to st thomas emerg. they will give him oxygen if he needs it, maybe an IV if needed. then they call london, tell them what's going on, and rush us there (again by ambulance, with full lights and sirens the whole way. how do i know? been there, done that. surprised? why?). and i just have to trust that, if that doc is the attending overnight, that he will have listened to the st thomas doc and the paramedics, since God forbid he listen to me, because what do i know? obviously, nothing.

so, there you have it. the solution i've come up with. i'm going to mention the idea about a big meeting with everyone involved in asher's care, including the suggestion of a few other emerg docs. this is actually good, because some of them aren't aware that asher has "unusual vasculature" (wonky blood vessels) in his lungs. some of them misread that as being hazy or wet, but it's not. it's just that asher's weird. or, "unusual" as liz puts it. i love the idea of this meeting. totally not do-able, but i love it. and a mom can dream, right? hey!!! unless we come up with some sort of sheet to put in asher's chart that outlines all of his conditions and quirks and how they interact (ie, heart trouble and reflux, reflux and throat, infections and t-cell deficiency, t-cell deficiency and transfusions, oh, the list goes on and on). ooh, i like this idea, and it just came to me now! delightful! i have to write this down so i don't forget later! delightful! :)

anyway, so that's my stressful day today. add to that, i have quite a few other things i need to take care of, all requiring rather urgent attention, and none of which is pleasant (like, how can i keep my house? i don't know, but i don't think it's possible for much longer.). anyhoo, that's not asher-related, so i won't burden you with the details here. maybe i'll write about it on My Simple Life later. maybe not. dunno. wait and see. anyway, please pray today, since we're in for a bumpy ride today. thanks so much! :)

for those of you who, like me, are desperate to understand medical terms that you really don't need to know...

we went to see paeds doc (dr b) today. we had a lovely visit with her. anyway, i got her all caught up on the last couple weeks in Asherland. you know the gist, congestive heart failure, trips to emerg, immunology clinic, upcoming visits to cardio in london and toronto. well...

i found out what the "muscle wad" is. so, here you go:

think of a body builder. as they lift their weights and do whatever it is bodybuilders do (wow, i so don't know anything about bodybuilding. shocking, i know. lol anyhoo...), their muscles get bigger. the more you use a muscle, the bigger it gets. makes sense, right? ok, now, asher's right ventricle. it's pumping away, lubdub lubdub lubdub (well, probably more of a lub lub lub lub, he's kinda missing the dub. and no one gets that joke except maybe a couple of doctors who are reading this, but trust me, lubdub is the technical doctor-ese term for the heartbeat). so, the ventricle's pumping, doing whatever it is a left ventricle does. oh, but wait! it's a right ventricle, so it's not built for the workload of the left. that means it's working harder than it's designed to.

so, it's working hard, and as it does, the muscle gets bigger. when you're looking at it from the outside, it still looks the same. it's the same size, because really, there's no room for it to expand out. (do you see where i'm going with this yet?) it can't grow out. it can only grow in.

so, as the muscle gets bigger and the wall gets thicker, there is less space inside the ventricle. less space to be filled with blood. this means that, while ventricular function may look good from the outside, and the blood pressure may remain the same (because the force of the pump doesn't change), there is not as much blood going out with each pump.

yeah, kind of a problem. but what i'd really like to know is: WHAT CAN THEY DO ABOUT THIS?!?!?!?!?!?????????????????????????? yikes! short of not using the muscle, how do you make it smaller? and i mean other than dr caldarone's stent/scar tissue solution of digging it out and scraping it away. i don't like that option. a little too nauseating. i'd like something a little less, um, yucky/risky/invasive/horrifying, thank-you very much. oh, and i'd rather transplantation is not an option yet, either. as it is, i'm thinking transplanty thoughts when i think about the Fontan (but that's just me freaking out. you don't need to think that; it's just that i think about all this a little too much, in case you couldn't tell already). someone, tell me there's a med we can give asher that will shrink his heart muscle, if that's what he needs.

ok, breathing, breathing, in with the good air, out with the bad air, in with the good air, out with the bad air i don't think it's working i'm getting dizzy does anyone else hear that rushing sound or am i about to pass out? yikes, i'm hyperventilating. that can't be good.

so, while i go calm down and try to stop thinking such freaxious thoughts, you have a great day. and don't worry about me, i'll be fine. serenity now, serenity now......

just so you know...

i just posted a link on the sidebar to the Children's Hospital of Philadephia, and their information on DiGeorge Syndrome. feel free to check it out; it gives some pretty good information.

in fact, i would highly recommend the Children's Hospital of Philadelphia website if you're looking for all kinds of information on children's health. it's a really good hospital, great reputation (and, coincidentally, the hybrid is their standard approach for the first stage surgery for HLHS, and they now routinely perform Dr Caldarone's arch reconstruction technique during the second stage, as well), and their website is a fantastic resource for information. so feel free to peruse at your leisure, and learn all about kids' health. enjoy!

and i'll put the link here, too, but i've included it in the "helpful links" section on the right sidebar, bottom link.
http://www.chop.edu/consumer/your_child/condition_section_index.jsp?id=-9065

oh, why can't he just pee already?!

so, we're at the tail end of our little vacation. mom and i took the kids to the ROM yesterday, which was awesome. and because asher has a disability (or several), i could get in for free because i'm his "attendant." delightful! i love doing expensive things for cheap! :)

anyway, the real reason we're in toronto today is because asher had immunology clinic this afternoon. it was a follow-up appointment from his appointment in january. i figured they'd tell us that there's nothing wrong with his immune system, he just spends too much time in hospitals, we don't need to see him again so take him home now before he gets sick again. haha, if only!

turns out, some of his t-cells are low. for those who like the technical information, his CD4 count is 718, and should be at least 1000 (the "control" they used had over 2000). and his CD8 count is at 234, and it should be at 400. this can pose some problems, but the fact is that while his t-cells are pretty low, the ones he has work well. so it's a concern, but he doesn't need to be isolated. just some hand sanitizer and such, but otherwise, treat him like a normal, healthy kid.

they are also still considering DiGeorge Syndrome as a possibility. of course, not the classic "chromosome 22q11 deletion" kind of digeorge, but some other variety. apparently, there are several different strains of the syndrome, and since asher lacks some of the more classic signs (eg, facial deformities), they're looking into a sneakier strain. because of course asher can't have the more common, classic variation of the syndrome! that just wouldn't be asher-ish, now, would it? sheesh!

i know most of you don't know anything about DiGeorge Syndrome, so i will try to find a good link about it and post it on the side bar so you can learn more. but that will have to wait till at least tonight, but probably sometime tomorrow at the earliest. in the meantime, here's a list of the usual issues that comprise DiGeorge:

1. severe heart defects, particularly involving underdevelopment
2. kidney issues, especially misshapen or underdeveloped kidneys
3. immunocompromise, especially t-cell deficiencies
4. low calcium levels
5. feeding issues
6. facial deformities

any of those sound familiar? yeah, that's why they're stuck on this digeorge query. the problem is, though, that the test immunology does for the syndrome, namely looking for a chromosome deletion (chromosome 22q11, for those who like to know) came back negative (translation: asher has that particular chromosome). there are other tests that could be done, but not by immunology. for further digeorge testing, he would require a genetics consult. so that is coming. beyond that, we have another immunology appointment in october, when they will do more bloodwork to assess his t-cell situation.

ok, i just remembered that some of you may not know what t-cells are, either. basically, they're white blood cells that fight off disease, particularly bacterial disease. which might explain why asher is so prone to weird bugs in weird places. remember that the t-cells he has work well, but still, they're low.

ok, i know i'm forgetting stuff. what else is there? man, i wish i had my notes in front of me. this blog post was kind of an impulse thing, something to do to pass the time. arg. ok, um, i'm supposed to talk to our nephrologist next month about a referal to urology regarding his frequent UTIs. we're also supposed to have nephro arrange for another VCUG, which delightful. ugh. yet another test where i have to pin asher down while he screams and fights because they're putting tubes in unholy places. oh gosh, how soon can we get in for that? i can't wait. let's see, anything else? i don't think so. i think that's it. oh, the title of this post. i should probably explain. immunology does a lot of bloodwork. and apparently, they also wanted a urine sample today. so, we're sitting here in the hospital, waiting for him to pee. you wouldn't think that would be a problem for a kid on diuretics, but you'd be wrong my friend! almost 2 hours we've been waiting! but now he has peed, so we're off to phlebotomy so they can deal with that. ok, talk to you later! :)