Saturday, October 4, 2008

Local Surgery Cancelled

This was the phone call I was dreading. I have cancelled surgery at Children's on October 20th. Briana did not sound upset - she was all thankful that I let her know - aparently surgery slots are always in demand.

But it makes me wonder - do people not tell her and then just not show up for surgery? Do people run away? Hide? Double book surgeries often?

The mind just boggles.

She will tell Dr Lewin who, no doubt, will call. Something else to look forward to. This feels like breaking up with someone. I hate conflict. It is so much easier to agree with everyone and make everyone happy.

Insurance coverage confirmed

My good news phonecall of the day was from 'our' Stanford insurance contracting specialist. She took all our insurance details, contacted Premera and confirmed that our insurance would pay the costs of surgery at Stanford. Even better, she said that costs would be charged at the in-network rate which would reduce the overall fee and save more of our lifetime insurance maximum.

After speaking with Premera, Karin said "you have wonderful benefits. You have things we have never heard of before down here. Although you have a lifetime maximum benefit pf $1 million, every year if you use a percentage of that they restore $5K of it so it accrues again. I have never seen that before. Anyway, I have confirmed it and everything should go smoothly. If you have any problems with billing after surgery please keep my name and number and contact me. You have wonderful benefits, its good to see."

High praise indeed.

Friday, October 3, 2008

Krabill conclusion to the affair of the many consultations

I have just spoken with Dr Krabill. She was supportive of our plan to go to Stanford although she did not say anything about not being in Seattle.

Her recommendation was:
1) Try and save the valve.
2) Kono-mechanical valve replacement if required.

Details of Conversation
Her team looked at Wren's echo and angiogram I had provided. All agreed it is a tough case to work on a plan of action. She presented the options and even after discussion they felt it may be very hard to tell best route till OR and the anatomy.

Wren's issue is defined as: Severe multi-level left ventricular outflow tract obstruction.

Most literature recommends taking least invasive option first. This would be a repair and valvotomy - go through valve to look at LVOT - resect fibromuscular tission. Also see if there is any valve fusion not obvious from Echo that can be repaired. They would open and patch ascending aorta and then re-evaluate.

If that does not work... its a toss up.

If you do a Ross Kono - how long will aortic valve and pulmonary replacement last? If you do Ross - you transect aorta and sewing ring around that and may create aneurysm at this point.

* I said we would not favor a Ross-Kono for reasons discussed earlier *

Mechanical versus tissue valve replacement.
A mechanical valve requires coumadin therapy but a tissue valve will wear faster than mechanical. In Dr Krabill's experience with tissue valves in other locations: "We find leaflets curl and leak and then don't open as freely and do leakage and backflow."

Thus, a Mechanical valve is better if Kono required. It has fewer complications and should last much longer.

"Thinking of my 20 years of being a PC and there are just a handful of children that need a Mech valve in toddler or preschool age but the Coumadin is manageable. I was thinking of a girl who has both aortic and mitral valve problems and ended up as a ... under a year of age.... I think she was 6 or 9 months of age. And those valves lasted till she was a teenager and she outgrew them and needed replacement on size. She was healthy and active and her recovery was remarkably quick."

We are going to Stanford.

On the road again

We are extremely lucky to have a neighbor whose son is a couple of years older than Frost. Not only does Elias provide hours of entertainment as a playmate, he also provides piles of outgrown clothing which Frost later inhabits. In fact, he wears Elias's pajamas to school most days (btw, I wish I knew a baby about 8 months younger than Wren who could inherit all this good stuff he is too tall for, anyone?)

These neighbors are big cyclists - their 9 year old did half of the Seattle to Portland Bike ride this year. Well, when Frost was 3, Lauren gave us a childseat which attaches behind the rider on the bicycle so Frost could ride along with me. The bicycle that we used then is now dead and Frost did not use it for long, but last weekend Joshua attached the seat on his bike for Wren.

Wren LOVES it.

When I suggest we go for a walk he says "not 'roller, BIKE! Get 'elmet!" [not stroller, bicycle, get helmet]

Since then, we have been riding all over the place - to the point that I have sore legs and bottom. We have ridden to Whole Foods, the playground, Ravenna park, Greenlake. Its been great for Frost too because he can follow behind on his bicycle and ride safely on side streets. He rode with us to Alex's house and then right around Greenlake. Alex road with us all the way home.

I know the weather is closing in and we will not be able to do this for too much longer - the hybrid bike has thinner wheels than my old mountain bike and I feel cautious on wet roads without the usual traction - but I can see us riding the trails as a family and its a wonderful exciting thing for all of us.

Frost says "this is the best thing in the whole world" and "I think this is the longest ride of my life. Its the longest ride of my childhood. I am very good at riding now."

The only problem is that my old bicycle is broken and I am not sure whether we should buy another bike now, with winter coming, or rather wait for Spring. If we get a bicycle for me and a rack that attaches on the back or roof of the minivan that will make it easier for us to do trails out of town and go to more exciting places but I know that I get all excited about things and my excitement is not always matched by my ability to sustain them. So, watch this space.

Later, I will post a picture of him sleeping in the bike seat on the way home from Whole Foods.

NOTE: DR Krabill called but I missed her because my cellphone is flat. I am charging it and she is going to call back after this patient. I hope.

Thursday, October 2, 2008

Wrenisms - updated.

Frost has been telling his friends Wren's 'joke'. It goes like this:

Wren is sitting on a spot in the kitchen near a vent. He points down the heating vent in the floor.
"Baby gone DOW-IN!" he says.

I open the vent with a fork expecting to find a toy he has dropped in the hole. I look down and say "there's nothing down here" and look at him to check what he meant.
Wren gets a big smile and points at himself "Bay-bee RIGHT HERE"
I laugh.
He laughs.

Wren repeats this joke at least 10 times and still does not tire of it.

Rhythms and routines
Wren has learned that I nurse him twice at night. This makes for really strange waking times. If he wakes at 3.30am for the first time and is nursed back to sleep, he wakes for the day at 5am sharp (90 minutes after first nursing) but if he sleeps until 5am before waking he wakes for the day at 6.30am. - a quite respectable hour.

At second waking he nurses before getting up for the day. Recently, he has begun to talk to me in the dark. Here is his morning commentary on second nursing:

Mummy not go-ing
Mummy staying nuggle wren.
Staying MILK!

A few minutes later its light on and quiet playtime as I hope to keep the others asleep slightly longer.

We are playing with the trains.

Wren says "Tain go BACKwat!
Tain go UP 'and!"

The train has driven up his hand.

While playing cars we discover a small wooden car missing three tyres. I mention that its broken because Wren bit off the tyres. The car has one tyre left.

Wren says: "Not bite that one. Wren do-not-do-it! BRO-ken."

Differentiation of things
Later, Wren is playing with his cars. He wants to know what each car is called. He asks "what this?" I tell him "Rocket car" and he tells me "see rocket go up. Big rocket." remembering the Freemont Rocket I showed him last week.

He now differentiates rocket cars, race cars, Mummy cars (blue minivans), Daddy cars (subarus), Heather car (Kia sedans), tucks (trucks) and diggers / rollers / bulldozers of various kinds.

He has also started to name di-nors (dinosaurs). This morning he asked me "what's this?" for each type of dinosaur. He can now name "T-rex", "'plod-cus" and "wooly ri-no". He has trouble with stegasaurus, triceratops and elasmosaur. We will continue to work on this vocabulary. One dream we have is of doing a road trip to Glacier next summer, maybe as far as Dakota. We would dig for fossils and look at the dinosaur museums. Wren would like this although he would not like too long in the car.

I = Wren
In another recent development, Wren has started to say "I". He says "I do it!" and "I have it" and "I go down."

The Big Snake
Today we went to Top Ten Toys on a mission to buy a very BIG elephant. When we got there Wren held the elephant affectionately for a short while and then carefully put it back in its box on the shelf. "All done" he said, taking down a very large black and red striped snake. "Take it home!" he announced and walked off with the snake.

I added a family of lemurs to our basket and Frost spent his accumulated allowance on a Playmobil shipwreck with a band of pirates. Everyone was very satisfied.

On the way home Wren kept crying out and saying "owie". At a red light I was able to turn around. He was holding his hand in the snakes mouth, making it bite him and fake crying. I told the snake to "Stop biting Wren!" which seemed to solve the problem.

Diggers gone wild
When we came home Wren walked outside and said "Garden, now." Yesterday I was weeding and cleared a large patch of garden bed that used to be full of wilting crocosmia. Wren loves the exposed earth and takes his diggers to work there while I weed in other areas. He also likes to hide his diggers in the shrubbery which is how we are now one bulldozer down. It went "hiding" and is now "all gone."

Wednesday, October 1, 2008

Where are we at with the surgery stuff?

If you're like most of my friends, you have lost track of what is going on about Wren's surgery. In the last few days I have had 3 loyal blog readers ask me to explain the current plan and also "how are you doing?" So, if you can't see the wood for the trees - here is an update to the question: When and where is Wren having his surgery for left-ventricular outflow tract obstruction and what surgery is going to be done?

We are planning to go to Stanford BUT
We have not yet cancelled our surgical date at Seattle Children's. We are waiting to hear from Dr Krabill. Dr Lewin (our primary cardiologist) has given advice which leans towards staying in Seattle. He does not think it wise to be aggressive about saving the native aortic valve. He favors a plan that assumes mechanical aortic valve replacement with a kono procedure and has made us fearful of the myectomy approach of Dr Hanley because he says that, in his view, the success of the procedure is no reoperation in <5 years. He feels this is a very real risk with a myectomy approach saving valve.

STILL we are planning to go to Stanford because:
1) We felt very comfortable with the experience, reputation, manner and plan proposed by Dr Hanley. He was willing to discuss Wren's case and differentiate between technical, lifestyle and 'unknownable' factors in the surgical plan. He was also willing to change his plan on non-technical points.
2) We were impressed that he has done 100's of kono procedures and relatively few are done in Seattle. If a valve replacement is required we feel he is a more experienced surgeon.
3) Since "surgery is largely pattern recognition" according to my friend Natasha - we want someone who has seen more variations of the pattern and can make those inexplicable judgments in times of crisis or at critical junctures IF things do not go according to plan.
4) Stanford is a high volume center. Seattle is a medium volume center. Higher volume is correlated with better outcomes in pediatric heart surgery.

No news from Dr Krabill BUT
We are still waiting to hear back from Dr Krabill. I am surprised and a bit disappointed that she has not called yet, even to say that she is waiting for more information. Right now, I have left two messages (Friday and Monday). When I spoke with Dr Lewin on Tuesday he said she was planning to call us on Tuesday as she had promised. We did not hear from her.

We want to know what she thinks of our plan to go to Stanford. In particular, whether she feels the same concern about a myectomy repair in the valve-saving option. The alternative is a hemi-kono.

On Saturday, we received a CC'ed clinic report addressed to our primary care pediatrician. This reiterated our discussion with her 2 weeks ago but also added some commentary:

"I would try to avoid a Konno or aortic valve replacement if possible. The outflow gradient would need to be assessed after this operation to determine whether or not further work needed to be done because of the size of teh aortic valve and residual gradient. I feel that there are two potential plans at that point. Both of them would be to do a type of Konno procedure. The question if the aortic valve is replaced, whether it is done with a bioprosthesis, a mechanical valve or a the pulmonary valve from the Ross procedure. Ultimately, which one is undertaken may depend on the anatomy that is seen in the OR. is difficult for us to predict whether or not it is better to do a bioprosthetic, mechanical valve or Ross-Kono should the aortic valve be problematic after the initial attempt to alleviate teh obstruction. In either plan, further operations down the line would be needed. Additionally, it is possible that at some point heart transplant might be necessary if the left ventricular relaxation remains impaired or becomes progressive."

We do not know whether this is her final advice to us or whether she is still planning to call. Josh thinks she is and we should give her till the end of the week.

I do not like seeing that "heart transplant" phrase but we know that is always an option for kids with CHD's when the defect is not reparable. In fact, some people feel that many kids with complex heart defects may require a heart transplant as adults. Still, this is a just one of many outcomes and at this point it is far more likely that the LV hypertrophy and left ventricular function is restored once the obstructions are relieved. Right now, Wren's left ventricle is like a body-builder's muscle - thick and inflexible. Heart muscles need to be lean and strong - more like the yoga or pilates version. Body-builder ventricles don't relax and fill completely with blood so they don't work as efficiently and if this continues to progress he would need a plan Z.

Mum has booked her flights BUT
we haven't booked flights or accommodation for Stanford. I have priced the options and chosen a few hotels within 10 minutes of the hospital. We are waitlisted for Ronald MacDonald House (which is free but vastly oversubscribed for the number of families hoping to stay there).

It looks like we will have to pay about $750 for flights for Wren, Josh and I and about $120 a night for accomodation which is not bad considering the price of 'inns' and Best Western's down there. I am hoping to get a room about 6 minutes from hospital at a place that has a shuttle. This will be for the first week. At that point we may fly Mum and Frost down and just pray we get into RMH or find an apartment that fits all of us. All of them have heated pools so Frost could swim all day :)

Mum is due to arrive on October 25th. We should leave for Stanford on November 5th. Pre-op appointments start at 7.30am on the 6th. We plan to have a car for the first few days so we can have a bit of R&R before surgery.

We are going to trust the surgeon to make a plan BUT
we expect that if the valve looks sufficient for the next 5 years, Dr Hanley will try to save it by performing a myectomy repair below the valve and a supra-valvar patch. If it looks like it will fail, he will perform a kono procedure with a mechanical aortic valve requiring anti-coagulation therapy for the next decade or until it is outgrown. That means coumadin.

My gut-check says we are doing the right thing BUT
I feel a lot of anxiety and 'what ifs'. What if Dr Hanley does the myectomy repair and Wren is one of the 20-30% of children in whom the obstruction regrows at the site of the repair? He will need another life-threatening open heart surgery within 2-5 years - one I could have avoided if I made the right decision.

What if we stay here and Dr Cohen does a kono valve replacement and Wren has adverse effects from Coumadin or has a complicated recovery and we keep wondering whether he could have done better, had a better outcome in Stanford? Think how much money we would save being here? Josh could work a bit more, Frost would have us around when he needs us.

We just don't know and nobody can tell us the answers for how this will or would work out.

I am doing okay BUT
I am having trouble sleeping. If Josh wakes me with a snore - even a mild rasp like a wave pulling back on a pebbled beach - I am AWAKE and READY and start to think everything through. I start to make packing lists for hospital: rescue remedy, historical romance, hand lotion, new toys to lure Wren into joy. Plus, I feel a low grade anxiety off and on during the day and a horrible guilt for what Wren is going to suffer. I just love him to bits and every time I see his joy at 'untent' or a book or playing chase I feel a quixotic terrible happy-sad for him.

As with any parent, I dread making him suffer. I dread breaking the now-solid sense he has about life. He is just at the stage of being a little king. He thinks he knows exactly when things happen, where people sit, when Daddy comes home, who is at the door. I have this nightmare when I imagine his fear and horror waking up in hospital with stuff in his arms, tied down and in pain. Its just like those X-files of alien abduction. Who took my life?

I feel awful when he runs up to me or Josh and says "owie, kiss it better!" and I kiss and he says "all better" but I know I won't be able to make it better that time. There is a tiny little pink scratch on his hand he brings to me many times a day - he points to it and says "owie!" and makes a fake whimper. I kiss and it looks suspiciously and says "all done" and looks pleased. Its a tiny pink healed scratch goddam it. How is he going to cope with the wounds of surgery?

So, I am living on the edge of a sense of horror in other ways. Sometimes I can't make everyday things matter. Its like "oh, we need to do what?" And at night I have strange dreams about things like grizzly bears lurking in puddles and cars which drive on the edge of sheets of ice. Lurking there are all the questions we're not supposed to think about or mention - the 1:20 children who dies in heart surgery. The risk associated. The risk of stroke etc. I won't stress you further. You are not supposed to personalize the statistics close up.

But its not a soap opera and I am rather fond of those at the moment. I can't read anything challenging. I can't be bothered with long conversations and the highlight of my day is lying on the couch while Joshua gives the spot beneath my big toe a good rub. A simple reflexology chart says this is the spot that correlates with my adrenal gland. Go figure!

This is all normal for the freaky world of toddlers in surgery. Really, truly. But if I respond inappropriately to things and shirk responsibility - consider it a symptom. My subconscious is engaged elsewhere. I really don't care about everything else - except painting the kitchen. I can't wait to be surrounded by avocado-lime-green-wicked-bright-pistachio.

Edited to say:
I spoke with Dr Krabill's co-ordinator and she intends to call us in about half an hour.