Saturday, September 20, 2008


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.

Friday, September 19, 2008

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.

Thursday, September 18, 2008

There is no GOOD option

... other than saving the valve. That was the latest from yet another conversation with Dr Lewin. Every other option is really a PLAN Z. Coumadin - "we can do it but its not desirable" Bio-prosthetic - "Well, we have to defer to the surgeon on that because its a technical consideration" and Ross-Kono - well you know the drill there.

I am feeling frustrated and discouraged. I am a hairs breath away (24 hours and two more consults away?) from requesting yet another opinion. I am just not feeling satisfied that we have the right option yet.

After speaking with Dr L today I cannot see a good way forward IF the valve is not salvageable. Plus, hearing again about the difference of opinion about whether the Modified-Kono will work (rather than leading to a recurrence and re-operation) I know I will be fearful whatever outcome.

Dr L is being extremely non judgmental about the choice of where surgery should be performed. Today, I told him we were leaning towards remaining here and I did not feel any support for that plan. Instead, he started telling me that we had to balance what was good for the family with what was best for Wren and that he knew we would do what was best for Wren not just the family.


Honestly, I felt he was "wink wink" telling me to consider Stanford but I may well be losing the plot. I had very little sleep last night [combo of Joshua snoring, Wren teething and neurotic insomnia] so I am not at my most rational.

My latest plan is to pose as Solomon and suggest we have half of Wren's heart operated on in Seattle and the other half in Stanford. Perhaps that will shock the True Cardiologist into taking a stance in this sea of unknowns, controversy and speculation.

Pig Farming in South Africa or 'The Animal Box'

Mum is planning to fly over from Australia to help us while Wren is having surgery. While I was talking to her about her plans she was wondering whether there were any toys she could bring the kids, in particular, whether Wren needed any more animals for the animal box.

Now I don't know whether every family has an animal box, or if this was one of the many peccadilloes of my ancestral line, but David and I grew up with a big box of Britains animals and my children have one too. The current box contains some of these original animals and many many more. It is a much, much larger animal box collection made up of animals made by Papo and Schleis as well as a few Made in China bugs and snakes that came from party favors over the years.

Unfortunately, of the original animal box that I grew up with, few animals survived. The plastic became brittle and parts have broken off. We have the carcass of a black rhino (I think that is what it is) with only stumps remaining and the elephant has one foot missing.

The hardiest animals which remain are a family of pigs. These 6 pigs are deeply treasured because they come with an amusing anecdote of how my father's mother, Muriel Adams, went shopping at Musgrave Center one day and found that our local toy store was going out of business. It was offering the remaining toys at very low prices. Thinking of us, she purchased an entire bin of Britains pigs

giving us a sizable herd. David and I used to fence them in with our green zoo fences and play Pig Farms (we didn't have many cows). While adults tend to buy one of each animal - as a kind of vocabulary building exercise - it was very satisfying to have many pigs - so I always try and buy at least two of the same animal when we add to Wren's collection. That way, they can be a family, relate, hunt for each other or be lost and reunited.

We have so many different kinds of animals that it is possible for a bored adult with dark sensibilities to be creative. This morning I created a snake pit in which a cobra, a python and a few arbitrary wrigglers were fighting over an oversized cockroach. I also enjoyed this rearing horse being devoured by a hungry fly? Ant? I can't recall.

Anyway, when mum asked if there were any animals we needed, the word "needed" had me stalled for a while. Wren certainly doesn't NEED any more animals unless we are trying to form a complete catalog of fauna for each ecosystem BUT he absolutely loves his animals at the moment and spends more time
carrying them around in baskets and setting them up than he does with his diggers. He knows the names of all our animals - including: flamingo, lion, peacock, big-dog, wolf, octpus, panda bear, polar bear, giraffe, cicada, beetle - basically every animal we have, bar a few small ones that keep falling into the debris at the bottom.

So, I told Mum not to go and buy any more animals but to feel free to pick one out when she was visiting. It has to be guided by curiosity, by impulse, by the desire to play. The same feelings which are guiding me to buy 5 more pigs next time we go to the toy store!

Wednesday, September 17, 2008

Consult with Surgeon #1

Today we met [face to face] with the Seattle Surgeon from whom we received our first surgical opinion. I expected it to be a routine, 10 minute chat in which we asked about experience, volume and outcomes and then said "see you later".

This was not the case.

I feel a sort of insane hilarity in writing this but it seems that either the surgical plan was incorrectly communicated in prior conversations with our cardiologist OR it has changed. Regardless, there were some significantly different elements to The Plan as we learned of it today. It was a very useful and informative meeting.

The Crib Notes Version of the Consult
Plan A:
Save the Valve
Open chest. Inspect aortic valve. IF valve appears acceptable perform a modified-Kono procedure to enlarge the aortic root and sub-valvar area and remove the obstructive tissue.

IF Valve appears floppy or patently insufficient after supportive sub-aortic tissue removed -----> PLAN B (valve replacement).

Perform a myectomy through the aortic valve to remove any remaining obstruction.

IF Valve OK: Off bypass. Observe valve performance.

IF gradient is satisfactory, valve motion acceptable, minimal regurg ---> Close.

IF gradient unsatisfactory ----> PLAN B (valve replacement).

Plan B:
Aortic valve replacement with mechanical valve and aortic root enlargement
Take down the modified kono [remove patches]. Insert single large patch across valve. Insert new mechanical valve. Off bypass. Close.

Start lifelong warfarin treatment.

------------ END CRIB NOTES -------------------

Why not do a Ross-Kono procedure?

The R-K gained a lot of popularity in the 90's so we have some pretty good series data at this point. The data shows that over ten years the procedure has a 20% failure rate of the autograft in the aortic position. This is the average failure rate. We know that there is a higher failure rate in patients with risk factors of BAV and Coarctation. They are indicators of a Fibrillin disorder (a connective tissue disorder) which leads to a higher failure rate. Now, I don't know the rate offhand but even if we say that those patients are the 20% - that is too high. ..... I have three boys and if one of them had this condition I would not do a R-K procedure...

Another concern is sensitization/ development of antibodies due to the use of human donor tissue for the pulmonary graft. We try to keep options open in young children and we are having lots of problems in cases where children eventually need life saving heart transplant surgery because they have antibodies due to these grafts. This makes it very hard to find a good tissue match. In the US, bovine grafts are not FDA approved for use in initial pulmonary valve replacements but are approved for subsequent valve replacements. That means we would have to use a human tissue graft for a Ross-Kono pulmonary replacement.

Look, the R-K is a surgeon's procedure. Surgeons like it. Its about opening the whole heart up, fixing it and putting it back together. Its a great surgery - I enjoy doing it but I don't think its the right surgery in this situation.

SAM - Systolic Anterior Motion
A risk in cases of complex LVOT is SAM p where one of the mitral valve leaflets gets pulled into the valve due to the venturi effect. This leads to a significant obstruction. A modified Kono would open up the area and overcomes risk of this complication.

Heart Block
All the Kono procedures are high-risk for heart block. We see about 15% of patients develop heart block. Interestingly we have had a couple develop late onset (2 weeks out) heart block. In most cases it occurs post-op. Children then need to be fitted with a pacemaker. Its risky because the place where we make the incision is the electrical center of the heart but it is impossible to know exactly where it is so it is sometimes cut or damaged by scar tissue. We do our best to follow indications about position but its just a high risk in this procedure.

What about the bio-prosthetic valve we were expecting?
These Bio-p valves do not last long in young children with their fast metabolisms. Its not about outgrowing the valve in size but it degenerates. You would be lucky not to be back inside 5 years. We see it all the time. I would hope to get in a small-adult sized valve but he would need Coumadin therapy with it. I would have to see how big the valve could be but if he grows to his father's size (5 7") he may not need another valve for size - if we can get in a 21mm valve. I will just do my best but you can't be sure till you see it. Sometimes a large valve will impede the motion of the pulmonary valve and some kids can tolerate that and do very well but we just have to see.

Kono-procedure complicates subsequent valve replacements
A Kono procedure also makes it difficult to do valve replacements in future. Many surgeons can't/won't do it. Its a tricky surgery. You have to take down the Kono and resize and reshape the area with new patches [I didn't really follow the detailed explanation of the steps in the take-down and redo. Frankly, anything with Take-Down in it freaks me].

In usual Modified-Kono a L-shaped incision is made but in order to enable a translation to a Kono and Valve replacement in future he would use a straight incision that is appropriate to both surgeries. Also, he would use some techniques that would allow a Ross to be done in future [I may have this wrong].

Surgical experience/outcomes
The facility should do over 400 OHS this year. They are classed as a moderate sized institution. I have never lost a patient doing a Kono procedure but I can't promise that in writing. I do about 3 a year - not because of the complexity but because of the rarity of this condition in children. I have done about 40 of this procedure and related Ross procedures.

Next Steps
I have placed a call to Dr Lewin to talk about a lifetime on Coumadin/Warfarin. I am also interested in the issues of serial valve replacements with the Kono-take-down issues.

Meeting Dr K, 1pm Thursday.

Phone consult with Hanley, 9.30am Thursday.

Tuesday, September 16, 2008

The Bears

This morning Wren and I went to the zoo and saw the bears. It was kind of by accident that we ended up at the bears instead of the flamingos. Wren likes flamingos and we visit them every time but for once we were driving to the zoo so I parked at the North entrance which is close to the wallabies, northern trail.... and bears. It was early, and the enclosures were being cleaned and food put out so it was an exciting time.

Just as we arrived at the bear enclosure the keeper finished her inspection. Wren was watching the salmon in the bear pond which is about 5ft deep and up against a heavy glass window so you can see the salmon in the pond, just like in an aquarium. The bears lumbered out into the sunlight and came straight down to the pond where one settled on a large rock while the other waded into the water and had a swim and tried to catch fish.

People pulled out cell-phones and tiny cameras and clicked away. An old man in camouflage gear poked his huge telephoto lens at the window. He would have an image of a single tooth - the bear was so close that when it pressed its paws against the glass I could put my hand against it.

Wren said "All done bear. All done claws. GO. NOW"

I let him run over to play with the copper otters near the otter pond. The otters were still sleeping. I respect his concern about bears. When the keeper was in the cage looking around, my first thought was "cr*p... she doesn't know where the bears are, they have escaped!"

The strange thing is that I used to watch the bear cam when I was living in Australia and Josh lived here. It is just dark right now but at times you can see the bears in their bear day and it can be quite entertaining.

Now I am right here - or I was.

We also saw the emu having a shower, fed a budgie from a seed stick, watched the snow leopard walk around with its extraordinarily long tail, ate choc chip cookies and stared a komodo dragon in the eye.

It was a very entertaining visit and even though I had seen the bears in their enclosure, I was still ghosted by the thought that they had escaped and imagined meeting them on a path without that 2" of glass. How different that would be.

Monday, September 15, 2008

"Throw the vacuum cleaner downstairs"

Frost has been punished. I overheard him telling Wren to push the vacuum cleaner downstairs. It was out on the deck being used by the contractor for cleaning up drywall dust. Just yesterday, it mysteriously 'tipped over' [I don't know who did it, says Frost] spilling piles of the fine white dust all over the deck. It took a fair while to clean up so I was pretty invested in the vacuum cleaning not being thrown downstairs.

He was sent to his room and will be denied desert this evening. He is disconsolate but doesn't seem to realize the magnitude of his naughtiness.

Frost: "But Wren didn't DO IT! He said 'no throw down'"

At least one of them has some sense.

Sunday, September 14, 2008

Fierce Pistachio

In Seattle, its one of those sublimely bright fall mornings where the shadows actually are violet. The trees have dark shadowed hearts and the houses and trees up the ridge are blurred like a scenic postcard. In Canberra, on Saturdays like this I would head out to Manuka to eat pastries and read the weekend papers with the other bureaucrats. I can still remember the taste of lime tarts.

Today, we are going to be within range of many lime tarts (aka key lime tarts in the US) but I doubt I will be eating one because its less pleasant to eat a tart rapidly while fighting Wren to sit down and keeping Frost from shooting bits of paper napkin and saliva from his straw.

Still, we have a number of fun and feasible things on the radar. First, we have been up for 4 hours and I have built a block castle, hidden Playmobil horses, eaten veggie sausage and a good decaf coffee and generally enjoyed the cool sunlight streaming off the back deck.

At 3pm, Frost has his first soccer game with the Hawks (Wren will have to be kept off the field for that one) and then we need to pick up our new deep freezer from the store. We have a plan to store more food and spend less time shopping. Grocery prices have gone up astronomically in the past year or two and it seems a good time to have a 5 gallon tub of something. At times I think fondly of a cabin and a rainwater tank and those old cast iron tools that peel apples and grind meat (from the goat we slaughter?) and poke the eyes out of people who come too close, but I think the freezer is a realistic compromise between dream and reality.

We are also thinking of getting some test pots of the various greens we are considering for the kitchen. If all goes to plan we should be able to paint next weekend which will be a big deal after a few years of DIY rennovation. Selecting paint is so much fun because of the possibility and the delicious names they give the colors. We are considering Adobe Sun and Southwestern-Sun for the kitchen walls but what I would really call my favorite color is Fierce Pistachio.

Finally, this evening we are going out with friends to dinner and Josh is heading out to a late show of Damien Jurado at the Triple Door. I would go, but then I'd only qualify for the budget sleep plan (U4 hours with one interruption) and because Wren is growing the upper molars that could be reduced unexpectedly. He is very short tempered and intolerant and we have given him Tylenol to restore his good humor.