Feeding Giraffes at the Zoo

On Friday, Wren and I spent our morning at the zoo. As always, it was a very fun visit and Woodland Park Zoo is the one membership I will gladly renew. It has become so popular recently that Wren has started to favor animals over diggers at playtime. He is still excited by any diggers that are moving (he says "digger night-night. Not MOOOving" if we pass a stationary digger) and has been enjoying an excavator working on our street but on a daily basis he interacts far more with his animals.





If I had to pick I'd say Untent [elephant] is his favorite animal to play with. These two pictures are Wren with elephant. Often he wakes up in the morning and goes to find untent and complains if its missing. Giraffe and flamingo [rarf and mingo] are others carried around in style. When we drive near the zoo he calls out "see 'mingos? Now? Mingos night night?"

Giraffe Feeding
On summer mornings at the zoo they have been offering giraffe feedings from a platform by the savannah gate. This means the giraffe are held in a small enclosure by the path and are very visible to onlookers while those who pay get to dangle leaves at them and have a picture up close. On Friday, we paid our $5 and fed the giraffe.

Wren found the giraffes very large and felt "bit scared." He was given some leaves and the brutish giraffe leaned right in to eat them. When Wren cowered away clutching the leaves to his belly the giraffe nuzzled his belly to get them. Giraffe have big teeth and black tongues like a gruffalo and I suspect Wren thought it was going to eat him.

Siamungs and Tigers
We were lucky at the tigers and the Siamungs. The apes were swinging and hollering (Wren is not sure he likes it but as soon as we leave he says "more, more." The tiger came out of his lair and walked around when the lion started roaring in the nearby enclosure. Since lion and tiger do not usually share a habitat, I am sure it was alarming. On both posts you can hear Wren commenting about the animals.




Pounce Joshua
And this one is nothing to do with the zoo except the kids are riding Josh like a horsie on the way to play POUNCE on the bed. I am not sure how long this game can continue but right now both boys want to ride on Josh and be thrown off onto the bed.

For Shones Complex - multiple OHS are better than transplant

I subscribe to some citation search tools which alert me when an article is published on Shone's Complex. This cheery little piece came through my inbox this morning.

Outcomes of Reparative and Transplantation Strategies for Multilevel Left Heart Obstructions With Mitral Stenosis
Malhotra, S. P., Lacour-Gayet, F., Campbell, D. N., Miyamoto, S., Clarke, D. R., Dines, M. L., Ivy, D. D., Mitchell, M. B.
http://ats.ctsnetjournals.org/cgi/content/abstract/86/4/1305?ct

Apparently, from a study of 43 children with Shone's Complex - some of whom received a heart transplant while others had staged heart surgeries for mitral stenosis and LVOT obstructions, they learned:

Results: There was one in-hospital death (2.5%) and six late deaths (14.2%). Actuarial 5- and 10-year survival for staged surgical and transplantation was 88% vs 61.3% and 83.1% vs 61.3% (p = 0.035). At a mean follow-up of 7.9 years, freedom from mitral reoperation was 83.3% and freedom from reoperation for subaortic stenosis was 78.0%. Wait-list mortality was 13.3% (2 of 13). Wait-list time exceeding 90 days was an incremental risk factor for death after transplantation (p = 0.005).

Conclusions: Despite the challenges of a reparative strategy for Shone's complex, favorable survival and durability outcomes can be expected. Heart transplantation, although avoiding the pitfalls of staged repair, confers increased risks from ongoing physiologic derangements due to uncorrected left heart inflow and outflow obstructions during the wait for donor heart availability.

This means that on average without differentiating for severity of the presentation 88% of kids who had the kind of surgeries Wren faces lived for 5 years and 83.1% were alive after 10 years.

The 5 year survival rate for those who received a transplant was 63.1 % and no further children died by 10 years out. So, the initial risk was quite a bit higher for the transplant but it was stable once the surgery was a success. Of course, the longer term issues with transplant are murky.

At least, in Wren's case it seems that the risk of staged surgeries is preferable and since he has low level of mitral valve problem right now, that he has at least an 83.1% chance of being alive in 10 years.

Another little nugget was that 78% of kids who had LVOT repairs did not require reoperation within 7.9 years. That is not terribly encouraging since Wren has a relatively complex form of LVOT obstruction. Still, it does not contain enough detail [presentation, type of obstruction, surgical repair done, residual gradient, age etc] to draw meaningful conclusions.

Still waiting to hear from our second opinion cardiologist, Dr Krabill. I feel her delay is encouraging - it means she doens't just agree with Dr Lewin... she is doing her own research. I need that from someone else.

Bad influence

Frost is an Offical Bad Influence. This afternoon Frost used his screen time to play Mariocart Wii. He wasn't doing well in the race and said "damn it. DAMN". Now Wren is saying "damn it!" with perfect mimicry, again and again.

He walks around stamping "damn it!" in his sing-song voice. "Damn it, damn it."

Frost is now barred from mild expletives as well as facing a screentime lockdown for language worse than this which was, thankfully, out of Wren's earshot.

Final recommendation from Children's

I spoke with our primary cardiologist at Children's who had been pondering Wren's case, data and second opinions since late last week. He has spoken with Dr Krabill who will call us later.

His final recommendation is to go into surgery anticipating a Kono procedure with mechanical valve replacement. Wren would be on Coumadin after that.

IF the surgeon sees that the valve is more robust than expected then he will revert to a valve-saving plan but that is not to be expected.

He believes that the surgery can be done successfully and well at both Seattle Children's and Stanford and that we should choose a place that has support for the family in case complications lead to a 6 week rather than 10 day hospital stay.

Reasons
The approach is based on our definition of good result. I think it is Wren NOT being back in OR for 2-5 years....

If we do a myectomy below and patch above. (myectomy - valve small and thick and not normally formed.) Our expectation is that leaving valve will lead to level of disease and obstruction that is unacceptable. Given we are NOT recommending the Ross for 2 different reasons... we feel that the kono with mechanical valve is the approach of choice.

Un-tent gone, bye-bye. MISSing. Mommy, GET un-tent. GET NOW!

Recently every day feels as if I've waded through a week of days and its Friday. Not that the weekend is particularly different except the doctors are unlikely to call on the weekend, Joshua is home to play jump and leap and chase with Wren and Frost has soccer matches. Okay, it is a bit different. We eat more icecream and I cook less.

Anyway, right now I can get my bearings pretty quickly by running my mouse across my Firefox menu bar.

• I am told it is September 23rd, Tuesday [so Dr Krabill may call?]


• I am reminded that I am in the United States of America [not Australia, or South Africa where the political machinations of Mbeki's resignation is working its way out.]


• I am within range of 7 wi-fi networks [Caliroomies, THEBIGBADPIG, cloudcitycoffee,2L8ICU,megargelville, Wendy and 2WIRE888]. My laptop has autoconnected to cloud city since it is the only one that is open.

I had my school workshift training this morning and now have a break at Cloud City Coffee. Its one of the most civilized things about Seattle that almost every coffee shop has its own culture and its own wifi network so you can get a cup, have lunch and catch up on work while enjoying some public privacy. Its so much easier than the old days when you could only hang out as long as you felt comfortable with your book and then had to get BACK to things. Now I get right back to things over a grilled vegetable wrap, among adults, out of the house. Its so just-what-I-need that I really don't want to waste babysitting time going out for dinner.

Anyway, I digress with this mildly mopey tone. I opened blogger to write about Wren's vocabulary which makes me laugh 100 times a day, that, and the smell of the top of his head. As I am thinking about toddler vocab there are a group of parents behind me in the play area conversing on the same subject. Their toddlers are a bit older than Wren and are crawling around playing with trucks.

"Its amazing how fast their sentence structure starts forming. It blows your mind!"
"Yeah, but they come to things at different paces"
"He is making really complex sentences these days!"
"Owen, don't put the truck in your mouth honey"
"You like orange? Huh? Orange is a fruit!"
"You turn it, it goes different ways"

A new toddler is dragged in the door on his car. He comes in backwards and looks bewildered.

Perhaps I should bring Wren here one day - but I am not sure its a good idea to expose him to germs. We are still a safe distance from either surgery date and I am already considering Airborne, draconian hand-washing and isolation to try and keep him safe from respiratory conditions on surgery day. If he had a cold or flu at the time he would not be able to have anesthesia and we would be delayed for some time. With things like surgery I become a bit of a control freak. It feels that if I can keep things going as expected - even if expected is awful - then I can cope. So, I am expecting bumps, delays but I am trying to avoid a virus.

Argh, another digression. What I came here to post about was Wren's speech. He has suddenly taken a leap in his communication. He was always very articulate but he now uses many words in long, sometimes unstructured, strings. For example he spoke the Title sentence while I lay in pre-6am denial on the mattress in his room.

[SHANNON IS LYING WITH HER NOSE POKING OUT FROM UNDER A BLANKET WHILE WREN DIGS AROUND IN THE TOY CUPBOARD]

WREN: Un-tent gone, bye-bye. MISSing. Mommy, GET un-tent. GET NOW!
Me: What is an untent?
Wren: UNTENT!
Me: Where is the untent?
Wren: GONE!
Me: Where are you looking? Do you want me to get the tent?
Wren: Untent gone. Anmals.
Me: Oh, the untent! Its in the bath.
Wren: Door? Open?

[SHANNON STAGGERS OVER TO THE BEDROOM DOOR AND OPENS IT. KITTY HAIKU COMES IN AS WREN RUSHES OUT TO THE BATHROOM WHERE HE SEES UNTENT IN THE BATH OUT OF REACH]

Wren: HELP! Mama, help get untent. NOW!!

[SHANNON GETS A BURST OF SPEED AND MAKES IT TO THE BATHROOM BEFORE WREN WAKES FROST IN THE NEXT ROOM. SHE RETRIEVES UNTENT [ELEPHANT] FROM THE BATH WHERE HE WAS SWIMMING LAST NIGHT]

Wren: UNTENT!!! Get more animals. More animals now.

So, I pile all the animals into Wren's arms and he carries them into his bedroom then comes back for more, opining loudly about them as he takes them back. They are wet, very wet, they are "raffes" and "horse" and "pigs" and "bear, raaah" and "BIG Animals" (dinosaurs).

Unfortunately, all this ruckus wakes Frost and its finally 6.15am and we are all on the floor with Wren, the only one wired and ready for the day, and I am certain we will play "train tacks", "block tower, big, very big. Fall down, now. HIT it." and "read book, diggers, many diggers.. WOOOOAH!" as well as "animals, out, now" at least once, before I get my first cup of decaf coffee.

Speaking of which, its time for one of those.

Harvest

Its starting to get cold at night and all those tomatoes that didn't quite make it to red are at risk of turning to mush in the first frost.

Today, Wren and I went out to harvest everything that we can eat now and this weekend we are going to bring in a portion of what remains to ripen inside.

These are a couple of pictures of Wren with some tomatoes, corn, raspberries and carrots that we just picked. He also helped me pick 3 small yellow zucchinis. We ate almost these fruit and vegetables for an afternoon snack - raw or roasted with a little olive oil, salt and pepper. I have never seen kids eat so much zucchini.

Surgery update
Both Josh and I still feel we are heading to Stanford but I had another peculiar phone call with Dr L this evening. He remains very concerned that a myectomy approach will leave Wren with a residual obstruction that could be resolved by hemi-kono. He worries that physician preference may not lead to the best course. He is going to think about it for a few days and speak with Dr Krabill to firm up his recommended course.

Once again, he said we needed to pick a surgeon / team and go with that rather than dictating surgical priorities in minutae. I completely agree but feel that clarity of intention will help when there is no path which is clearly superior and the relative benefit of various outcomes is predicated on lifestyle, perceived risk of re-operation and the actual longevity of Wren's valve.

Hanley Conference

Yesterday evening, we spoke with Dr Hanley about Wren's case. He was very reassuring and we were impressed by his experience, clarity and willingness to explore the benefits of alternative surgical paths and entertain changing what he does. After speaking with him we feel that he would take on our concerns about the Ross and still do the best for Wren. I mentioned that our second cardiologist may call him to discuss Wren's case further and said he would be happy to do so.

Here are a few of the main points which we found so reassuring:

1) Dr Hanley has performed 100's of sub-aortic resections. He has done only 12 modified kono's (a year?) because he feels that he is almost always able to get at the obstruction through the valve. "The hemi-kono is more of a destructive operation. You are making a full thickness cut of ventricular septum and there is more chance of disrupting the electical impulses to the heart". He says he expects to perform a myectomy on Wren and that you can get an effective resection either way.

2) He feels confident he could get enough of the sub-aortic obstruction via the valve. If the valve was too restrictive he would move to a modified kono (although he dislikes this terminology and calls it a hemi-kono). He describes Wren's sub-aortic obstruction as "moderate" severity - a ridge/tunnel combination.

3) Dr Hanley will be very aggressive in saving the valve. Even if Wren is left with mild to moderate obstruction after the surgery he feels that would be worth it if he believes Wren will get 5 years out of the valve. "We have looked a the pictures and my guess is that with relieving the blockage under the valve and supra valvar blockage the valve is not going to be perfect but likely it will be sufficient."

4) Regarding the Ross-Kono: "I personally don't do Ross procedure unless it is really in child's best interest. I look at it as something of a temporizing maneuver because there are no 'good' options, esp since you are putting prosthetic on right side." With a Ross you are buying lifelong procedures one way or another - surgery at age 2 and then probably conduit replacement at age 7 or cath at 7. Then at age 12 or 13 you still need a surgical conduit and then they have a 15 year lifespan.

5) If we don't want a Ross done: An alternative to the Ross is the formal- Kono. Then we would put in a mechanical valve or a bio-prosthetic. Now, you might only get a few years out of a bio-prosthetic valve - but there are some complications with the bio-prosthetic valve because these valves are more bulky than the mechanical valves. The mechanical are more streamlined and could last till puberty... but you are on Warfarin. I ask my families "are you a Bobby Fisher or Evil Knevil" (sp!) and the choice of valve is really a matter of philosophy - not a technical consideration."

After speaking with Dr Hanley we are reassured that we will be able to find a good and workable solution. We were most impressed by the way Dr Hanley was comfortable with many variables and shifting parameters.

Right now, we are leaning towards going to Stanford and have the following list of 'surgical options' in mind. The replacement valve selection is still tentative and we need more information - hopefully from the cardiologist we consulted yesterday:

1) [IF VALVE APPEARS SUFFICIENT] Save the valve with myectomy.
2) [IF VALVE ACCESS RESTRICTIVE BUT APPEARS SUFFICIENT] Retain the valve but perform aortic resection via hemi-kono.
3) [IF VALVE INADEQUATE BUT LARGER VALVE CAN BE FITTED] Perform Kono procedure with bio-prosthetic.
4) [If VALVE INADEQUATE AND SPACE SMALL] Perform Kono procedure with mechanical valve.
5) [IF NONE LOOK GOOD SOLUTIONS:] Ross-kono procedure.

We think this is the best path for Wren but we are still unsure of the valve replacement priority in options 3 --> 5.

I am now in action mode, contacting all the parties at Stanford to arrange accommodation, pre-op appointments, Mum's flights [she is doing this but we are talking dates], child-life etc.

Frost has an expander

As if one medical debate wasn't enough for our family, we have one more on the table - Frost's orthodontics.

Frost has begun orthodontic treatment for upper and lower crowding. X-rays show that his upper teeth are coming down crooked due to insufficient space. He also has a complete overbite (as does Joshua!) The debate is that Joshua is not sure that orthodontics are necessary.

He delights in exclaiming that children did not survive before modern orthodontics and that Mark has a friend on the Board of Orthodontics who claims that the treatment of young children with orthodontics is a money making scam to keep orthodontists rich.

Regardless, the advice of our orthodontist prevailed and this week Frost had a palate expander put in which I have to gradually open to stretch his palate out. He is not unusually bothered by it but has just started to complain that it hurts when he bites down and will have to live on applesauce, yoghurt and icecream.

I have paged the orthodontist to see whether I should do anything about this or just let him lose a few pounds. I don't think they said it would be sore when he chews but it may be side effect that will abate soon.

Of course, this discomfit started on the cusp of a weekend. I shall have to get him a Jamba Juice this afternoon.

Two useful conversations

Today we met with Dr Krabill at Swedish and spoke with Dr Hanley from Stanford. Both were very helpful conversations.

I shall post more detail later but the biggest breakthroughs today were:

1) Dr Krabill reviewed Wren's case and quickly absorbed and reiterated the surgical options we face. She acknowledged the conflicts and understood the various points of view. She feels we need to go to someone with experience in this particular area. She offered to consult with colleagues, do a literature search and see who has most experience with LVOT and Shone's cases and contact those people if possible. She will consult colleagues at CHOB who handle many HLHS cases with similar features. She is going to speak with Dr Cohen and Dr Hanley.

2) We were both reassured by Dr Hanley - in his manner, flexibility and obvious expertise. Most impressive, was his willingness to amend his surgical plan to account for our preferences and concerns about the Ross. He acknowledged that the Ross procedure is flawed in that it involves a healthy valve in the pathology but feels there are no good valve replacement options and he has a 10% failure rate over 100's of Ross procedures. If we prefer, he would do a mechanical or even bio-prosthetic valve with a Kono procedure instead if a VR is required. He is fairly confident that with a repair Wren's current AV will last 5 years. That is his objective in assessing the valve. He proposes the myectomy because he is able to reach the area through the valve and, unlike the modified kono, it does not put Wren at risk of Heart Block (and pacemaker for life).

I shall explain more details tomorrow. Tonight is parent meeting night at school and since Wren missed his nap and will be going to sleep early, I should make at least half of it.

As of today, we are planning on heading to Stanford for Wren's surgery. Pre-op on November 6th. Mum is hoping to arrive on October 30th to stay for almost 4 weeks.

Hanley conference delayed

Dr Hanley had to head into the OR and conference ran late so he didn't have time to speak with us this morning at 9.30. He is going to call "some time today" to "fit us in". So, except for our meeting with Dr Krabill I will have to be within reach of the phone at all times.