More Surgery updates

• I have now obtained the package of Cath CD, Surgical opinions, clinic summaries and echo images needed for our referral to Dr Krabill. I hope she can make sense of them for us or at least cast her vote in the controversy.


• Dr Hanley's scheduler called and said that he had reviewed the two dates she suggested and felt that November 7th was a better fit for Wren's surgery. October 9th was a major case before his proposed surgery so it would not have been easy but November 13th was a less large one. I worry about November 7th because it is a Friday (less staffing over weekend) AND even worse, Dr Hanley goes on vacation for 5 days the following day. Is that even safe for Wren? I shall have to ask this at our phone consult next week. PLUS what if another critical case comes along? Do we get bumped for a week, get another surgeon? I do not like it, Sam I am.


• Bri called and said Dr Cohen can see us at 11.30am on the 16th


• Stanford called AGAIN to change our consult with Dr Hanley to 9.30am on the 18th. This is great because our babysitter will get here in time to watch Wren while we talk/listen.

I also read through Dr Hanley's recommendation in more detail and was interested to see his arguments for saving the valve. He says it is not very thickened, has normal motion and no regurgitation. He feels that it may be slightly obstructed by the LVOT obstructions close to it (above, in particular).

I like to hear these explanations. If it was a head-to-head save-the-valve surgery (and no Ross, looming) I would easily pick going to Stanford. However, it is not clear that he will be doing a Kono on the area - he mentioned "extensive myomectomy" - is this the same thing? Questions, questions.

Soccer Practice at Twilight

This week at soccer practice the boys had their yellow soccer jerseys which made it easier to find the team in the sea of U8 boys on the field. Frost very much enjoyed practice and Wren loved playing with Joshua while he was doing his drills and skrimmage. Anyway, here is Frost in his gear and in practice.



Wren likes to join in soccer practice and to roll around on the articial turf until he is covered in little flecks of black rubber (which are used to give it bounce?) We are all covered in this rubbery residue after soccer practice but Wren is the only one who likes it.

In the first picture he has collapsed in the goal for a rest and in the second one he has wandered onto the field eating his dinner-peach. No-one seemed particularly concerned and he wandered off again pretty soon. Josh said he was "just another obstacle".




Near the end of practice I ordered us a Pagliacci pizza (oh gods, rejoice) but we weren't in any hurry to get home because they said they would be 45 minutes. I couldn't stand the thought of waiting for half an hour in close proximity to the fridge so we played on the fake field for a while collecting blackberries and playing "throw-Wren-very-high" and then "Joshua,throw-me-up-like-that-too" [Frost] from which Joshua learned that getting 57lbs airborne is H-A-R-D.

Heart Surgery is a Team Sport

After meeting with our cardiologist again this morning to discuss the Seattle and Stanford options we still do not know whether we are on Team Stanford or Team Seattle.
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NEXT STEPS:
Sept 18th 9am - Phone conference with Dr Hanley
Sept 18th 1pm - Second opinion with Dr Krable
Sept ?? - Early surgical consult with Dr Cohen
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Here are Dr L's responses to my questions about the Seattle Plan to save valve if possible but otherwise replace the aortic valve with a bioprosthetic:

1) If BAV is a connective tissue disorder causing dilation of the aortic root, what complications would this cause with a bio-prosthetic valve replacement?

Yes, this could become a problem. In Wren's case we don't see any dilation of the aortic root at this point although we wouldn't expect to. In fact, he has restriction in this area. But over time if this became a problem it would affect the valve in the same way as a pulmonary graft would be affected (leaking, etc) but at least his pulmonary valve would not have been damaged - it would still be in place.

2) In your experience, what is the likely lifespan of a bio-prosthetic valve from either deterioration or being outgrown?

The growth and degeneration are two processes that occur but we expect it to last about 10 years at this point. It can be less - say from 5 years but some last longer. In the Ross procedure the pulmonary valve has lasted more than 20 years in some people but it is more likely to last 20 years in adults, say from age 20 to 40 than it is to last from Wren's age to age 20.

3) What size valve is likely to fit Wren and what are the techniques and risks of inserting a larger valve in this position?
The surgeon can make pretty much any size valve fit. There are risks - you can ask the surgeon about those. The main risk is that if the valve is too big, we occasionally see this, it can obstruct the natural motion of the mitral valve because it needs something to force against but it can't be too big. So sometimes we see this post-op and we have to go and fix it.

4) What procedure will be used for the sub and supra valvular stenosis (patch? carving?)
We would use a Kono Procedure which would cut away the septum here [will insert picture] and patch that area. It will be sewn back to muscle in about 270 degrees (this is a three dimensional area) but about 90 degrees will be a patch.

5) What is an average recovery time for this procedure?
Ah, it depends. About 10 days... 12 days?

6) Could you put me in touch with someone who can give me information on the Number of cases and outcomes for this procedure?
That is something you will have to ask the surgeon. You can speak to Dina and schedule that surgical consult sooner [currently for 2 days prior to surgery].

When I asked Dr Lewin what he would do he didn't actually give us a recommendation. He emphasized that he had been part of the decision which is now "Dr Cohen's approach" - to do the valve replacement not Ross if the valve is not salvageable, implying he is party to one side of the controversy. He also gave us a lot more background on his views - how he has worked with and respects Dr Hanley a great deal, used to propose the Ross-Kono for all patients in this situation, saw many PV failures, changed his views and now is opposed in cases of BAV.

He said that if we don't reach our own decision about where to go we really have to decide "who to trust".

On one hand he feels that we have to choose a procedure because "this is a point of divergence" between Cohen and Hanley. He feels that Hanley will say that in my patients I don't see this problem [degeneration of PV in A position in patients with BAV] and perhaps "I do the surgery in a way to.." and we will have to see whether we are persuaded.

On the other hand we can consider the institution because "heart surgery is a team sport." On this count, he argued that the institutions themselves have similar levels of infrastructure and outcomes post-op but that the determination should be on the surgeon.

"They will make similar decisions with a different set of eyes"

I said that I felt popular views were that Stanford is better equipt and does higher volumes than Seattle. He felt that much of this information was "a popularity contest" although he laughed about saying this from a lower ranking on the List of Best Hospitals and questioned what volume was necessary for expertise to build up. He feels that the 12 bed CICU constantly full gives the Seattle team enough expertise to do a good job.

But we should also consider the level of family support we have here vs Stanford "in case a 2 week hospitalization turns into 2 months".

Josh was quite persuaded by this argument - he feels the advantage of being near home more keenly. I am more impressed by Hanley's reputation of excellence.

I have asked for copies of the notes and the cath tape to take to our NEXT second opinion on September 18th. We are going to see another cardiologist - Dr Kimberly Krable - who saw Wren prenatally.

We are hoping she agrees with someone so we have help with this impossible decision.

Do you want to help? We could decide by poll. Apparently there is so much doubt, projection, controversy and big UNKNOWNS that it would be as good an approach as any.

Pondering valve options

I am compiling a list of questions to ask the cardiologist on Monday and the surgeon in a few weeks. Here they are so far:

Regarding Seattle Plan A:
1) If BAV is a connective tissue disorder causing dilation of the aortic root, what complications would this cause with a bio-prosthetic valve replacement?

2) In your experience, what is the likely lifespan of a bio-prosthetic valve from either deterioration or being outgrown?

3) What size valve is likely to fit Wren and what are the techniques and risks of inserting a larger valve in this position?

4) What procedure will be used for the sub and supra valvular stenosis (patch? carving?)

5) What is an average recovery time for this procedure?

6) Could you put me in touch with someone who can give me information on the Number of cases and outcomes for this procedure?

Regarding Stanford Plan A (save valve):

1) Given the proximity of the subaortic stenosis (SAS) to the valve, how do you think you will be able to get enough of the obstruction?

2) Why would we NOT want replacement of the AV with bio-prosthetic valve if its compromised? What are risks? Future implications?

3) What would you hope be regarding the lifespan of this valve considering it has already had intervention [cath] and leaflets appear thickened?

4) What is the likely recovery time for this procedure?

5) Could you put me in touch with someone who can give me information on the Number of cases and outcomes for this procedure?

6) What procedure will be used for the sub and supra valvular stenosis (patch? carving?)

Regarding Stanford Plan B (Ross-kono):
1) How concerned are you about early failure of the pulmonary autograft in patients with with BAV?

2) What about the risks of increasing from a two-valve pathology [aortic and mitral] to a three-valve problem?

3) Number of cases and outcomes for this procedure?

4) How concerned are you about the effects of multiple passes on bypass?

5) What is the likely recovery time for this procedure?

6) What procedure will be used for the sub and supra valvular stenosis (Kono element enlarges ... how exactly)

Frost Joins the Hawks (soccer and swimming)

This is the follow-up to an earlier post for Mum (Granny). Last week, by the end of his second session of swimming lessons, Frost was able to tick all the skills on his Beginner 1 Swimming List.

The most impressive skill was "diving". He can now dive head first (almost vertical, like a frog from a log) into 9ft of water. He can also jump from a diving board (not required) and swim to the edge, cross the pool in a semblance of crawl stroke and even do a bit of back stroke in a kind of more-sinking-than-swimming way.



This shows Frost about to dive and the one below is of Frost and his friend Dylan waiting their turn to do back floats.


He was very proud and when the pediatrician asked him what sports he did he said "I don't do any sports." I reminded him of swimming but he announced "that is not a sport, that's fun!" Dr Levitt wondered aloud whether Frost had watched "that American win all those medals for swimming at the Olympics" which led to Frost conceding that swimming could be sport, sometimes.

We had so much fun swimming that we are considering joining Samena Swim Club (even though it is 22 minutes drive from our house off-peak). We figure that if we went even once a week it would be worthwhile to have some swimming on a regular basis. Usually, in winter Frost does not swim for 6 months and then we start again in summer a few steps back.

Of course, swimming may lose its popularity now that Frost has started Soccer. We have registered him for a team in the LVR Region which includes our neighborhood. The team practices on Wednesdays near our house and all the children on the team are from local schools. The first practice was last night and it was reassuring to see that most of them are at a similar level of inexperience.

I met a few parents who were friendly and interesting people so our matches on Saturdays will not be too onerous as long as Joshua is around to chase Wren while he chases the ball. Wren feels he should be involved in practice but can be distracted by a pile of orange cones and a ball of his own.

Frost immediately began playing with a boy called Max who is entering Second Grade at Wedgwood and I hope he feels comfortable with the whole team, soon.

The boys named their team The Hawks, while lead narrowly over "the Bulldogs". I believe the coach told them that "Bulldogs" was overused. You sports followers will know what that means.

Surgery Schedule

I have heard that many people had difficulty understanding the issues brought up by the second opinion yesterday. We have had difficulty making sense of it so it was reassuring to speak with Dr Lewin today and hear him say that he:

"wouldn't expect a family to be able to make this decision. It is a very difficult call even for experienced cardiologists... there are so many factors that come into it and surgeons favor different approaches. There is not one right approach in general, but you have to work out which is the best approach for each child."

Ideally, Josh and I would like him to come to agreement with Dr Hanley - either that the Ross-Kono is the preferred valve replacement option or bio-prosthetic [Debate: + side: if it works we get 15-20 years before valve replacement - tendency toward premature failure in patients with BAV] OR we cannot go that path.

Neither Josh nor I is comfortable with the fact that the Ross procedure uses the pulmonary valve as the aortic valve replacement which extends the area of pathology in Wren's heart. That is fine in cases of discrete aortic stenosis, but he has enough vague and complicated issues as it is and the risk of mitral valve issues too so we want to limit this as much as possible.

I called Stanford and scheduled a "Phone Consultation" with Dr Hanley. The first available was 9am on 9/18. On the advice of their scheduler we have also put Wren on the surgery schedule there. Dr Hanley is already pretty fully booked until the end of the year so she needs to confer with him about when to fit Wren in. The date should be confirmed today:

October 9th - after a major case
OR
November 13th - after Dr H's break

I spoke with Dr Lewin about this and he said we could also be on the Seattle schedule. Our date there is:
Pre-op: October 17th
Surgery: Monday October 20th

So, that gives us a rough timeline either way.

Shannon

Stanford Opinion & discussion "To replace or not replace AV"

Here is the summary of my conversation with Dr Lewin. He is sending me a copy of the letter.

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The team Looked at the cath and echo findings. They agreed that Wren did need 'intervention'. Surgery should be done relatively soon given the parameters.

In particular, he needs relief of subaortic narrowing and supra-valve narrowing.

Approach:
They were cognicent of the small size of the valve but felt could potentially be spared and were hopeful and inclined to take the more conservative approach of leaving in the valve.

In leaving the valve behind they know its not going to be functional long term...

[Dr Lewin's comment: Of course it is preferable to leave in the AV in a young child because the alternative is not optimal but I am very worried about the valve....]

After the surgery for the repair of the SAS and supra-valve stenosis we will test the AV valve gradient and the fallback option, should we have an unacceptable gradient will be the Ross-Kono procedure.

[Dr Lewin spoke with Dr Cohen (Seattle Surgeon) about this approach and they are concerned about the issue of multiple bypass runs and potential cardiac dysfunction as a result of this strategy. If the AV gradient is too high they would have to put Wren BACK on bypass and expose him to further risk of heart, brain damage. Overall, a much longer run on bypass increases the risk considerably. Obviously, they are tolerant of a higher degree of risk in surgery while we feel more conservative there.

[Dr Lewin continues...."In a small child I am always in favor of saving the valve...BUT in this case we have already had intervention done on the valve -so I have added concern about leaving a valve that already have intervention on.

Also, Dr Cohen said that he can potentially get in an adult size valve which would delay the need for replacement...]

HANLEY: Go ahead with Ross-kono

[Dr Lewin: "Up here we are really concerned about the Ross-Kono for patients with a bicuspid aortic valve (BAV). It used to be thought that BAV was an isolated phenomenon but current dogma is that it is more of a global tissue abnormality and we have seen a fair bit of literature to show that patients with BAV have early deterioration of the (pulmonary valve) PV in the aortic valve (AV) position because it was never meant to do that sort of work in the high pressure AV position and it has similar deficiency as the BAV in terms of tissue composition.

so people are feeling more and more uncomfortable about doing the RK procedure on these patients. If you call around you will find people willing to do it, but its not widespread anymore.

It may be that that was a more generic statement about replacing the valve.. but...

Now, the Pacific NW seems be to endemic for AV disease so we have a lot of experience seeing what happens long term when you intervene in the AV and we are pretty nervous about that whole procedure and what Dr Cohen was recommending was a bio-prosthetic valve - a metal scaffolding with a valve in the middle which is better than pure animal valve - it degenerates slower than pure animal valve.

His answers to some of my Qs:

Q: How do they decide whether the valve should be saved?
A: In making the decision on whether to keep the valve they look at:
Real valve size.
Looking at leaflets, how thick, how pliable, how much motion.]

Q: Would he be on Coumadin with the bio-prosthetic?
a: No, It tets to the point of what is the best choice - mechanical valve you get adult valve size but you require anti-coagulation but not with a bio-prosthetic.

Q: What is the timing? You said "soon" - what does that mean
A: Its not a set time. If we did nothing he may be OK in a year or he may have a rhythm disturbance which would be [long pause] catastrophic.
Q: You mean he could have a heart attack and die?
A: Well, yes... or serious damage to the heart...

Q: Dr Hanley is very highly regarded. Why would he recommend the Ross-Kono if it is falling into disrepute? Could we call and ask whether he would do another kind of valve replacement?
A: Well, he wrote ROSS-KONO on the report and he didn't just write this off the top of his head so that is what he thinks we should do. We could call and ask but... well, this is what he thinks here. I presume he thinks that the articles, the data, is flawed and that we can 'get away' with the Ross Kono in the presence of BAV disease. I don't know....

My thoughts:
I am out of my depth in making this decision. Clearly, Dr L leans toward Dr Cohen's views. He is concerned about the Ross-Kono. I don't like the idea of a Ross-Kono either but I trust Dr Hanley's reputation. I like the idea of staying here but if there is a judgment call to be made I err on the established judgment of expertise - Stanford BUT I confess I have been swayed by this information about Ross-Kono and BAV PLUS neither of us want the R-K and another valve-replacement. I like the idea of a longer term more conservative appraoch.

BUT both surgeons present their approach as more conservative. Dr H's team are more conservative because they want to save the valve. Dr Cohen will also save the valve if he looks at it and thinks it may last a while [he said that too] BUT he is more suspicious of it right now. He feels that avoiding a long period on bypass AND another re-operation soon is more conservative.

Who is right? I Dunno.

I have set up an appointment with Dr Lewin for MONDAY 5th at 9am. Josh and I will talk through the issues.

AND

A Second Opinion on this mess with Dr Krabill at Swedish. Her earliest opening is September 18th but I can phone for cancellations "as often as you need to" said the receptionist.

Wait, I need to call again immediately.

Perhaps I am too wary but I feel that while our doctor here is fabulous he cannot be the source of the dispassionate advice I need right now.

We will hear today

At 10.31am I missed a call from Dr L. I was running at Alki when the phone rang but it went to voicemail before I could fumble it out of the belt-clip I was using. Argh.

Here is the message:

"Hi its ML. Its Thursday Morning at 10.30 and I finally got the letter from Dr Hanley and have reviewed it and thought about it a bit and can have conversation with you whenever you are around. I am in a meeting from 11-12 but I shall try and give you call after that and if I don't reach you, perhaps later in the day.

You can reach me through Dina, on 9897XXXX, but again, I will be out form 11-12 but you can try me or I will call later."

So now we have some time to obsess. Obviously, Dr Hanley has suggested something different to Seattle Children's or he wouldn't have to "think about it." Right? But what? More? Less? Sooner? Later?

Anyway, check in later.

I have set my cellphone volume to HIGH and have removed it from the offending clip ;)

No news, again

Its now 10 days post the last conversation with Cardiology about our referral to Stanford (Dr Hanley). I called Dr Lewin who called Stanford but did not hear back by the end of the day. He is out of the office tomorrow but may hear back and will call from Tri-cities if he does. Otherwise Thursday.

I asked whether this kind of time frame is usual. He said it has been 2 years since he had a patient referred down there and they had a different system then where he received a call from Dr Hanley.

He didn't think it was beyond a "normal" time-frame for referral.

Sure feels too long.

Wren likes painting (and Frost does too)

This morning I brought out the paints and let Wren have a go. Last time I tried he was not keen on the mess. However, today he was very happy and I have some lovely pictures of him painting and (shock, horror) FINGER painting. Again, he used to be very opposed to the idea of finger painting because it got his hands dirty.





As Frost played with painting, I was a bit alarmed to discover that he did not know what colors were created by mixing. He had no idea that blue and yellow make green [was he only doing scrounge art?] or that red and yellow make orange, so we spent some time experimenting. This started because he told me that "blue and black never make other colors when mixed with anything". That was quickly disproved. He also seemed to expect any pair of colors to make brown and was pleasantly surprised at the range of colors beside brown which resulted from our three primaries and green.

More color theory shall be forthcoming!