Excerpts from Life

Frost has been growing shiitake mushrooms in his bedroom. Once they emerged they have grown very fast and on Friday night we ate some in soup and some as tempura. Here is a quick photo diary of the mushroom cultivation:

Wren observes the mushrooms for the first time. He likes to stroke them while remembering that one fell off when he poked it too hard.


Frost observes Wren stroking the mushrooms.


We water them daily. They have grown a lot larger over the past few days. They are still soft and furry like the nose of a horse. After we water them we cover them in a plastic bag which acts as a humidity tent.


The mushrooms are ready for harvest. Frost and Alexander cut them off the block and bring them to the kitchen to be weighed.
They weigh 500g! I am going to pay Frost $5 for these mushrooms to offset some of the $10 investment I made in the mushroom log. Frost is not too interested in money at the moment because he has everything he desires and is prohibited from buying the things he desires but does not have (like a Nintendo DX).


Australian Trip Booked
We have booked to travel to Australia on March 24th for a month. I leave that afternoon with the kids while Josh will join us a few weeks later. We hope John will housesit. I realized too late that Frost's passport (the Australian one required for entry to Australia) has expired so I spent yesterday and this morning preparing and fedexing it to San Francisco to receive one in time for travel. I am also trying to get our tax return and Frost's school registration and enrollment in by that date. Joshua is also waiting for his US passport to be issued.


I spoke to the Advanced Learning Office of Seattle Public Schools and they advised us to wait to submit our enrollment until we had heard back on the status of our appeal. They would rather we not put in a list of contingency schools. She says it is likely that we will hear before March 24th but to call close to that date if we have not received notification. Talk about cutting it fine!

Potty Training
I am frying tempura when I hear the shout no mother wants. Simultaneously Wren wails "Summing on foot!!!!" and Frost shouts "Shannon, there is human poo on the deck!"

Uh oh. This is potty training crisis.

We have been doing fairly well recently. Wren is able to pee in the potty most times if I leave him without a diaper on (in pants or his new underpants) but if I put a diaper on he is unconcerned. Pooping is more tricky. He feels the need to poo a long while before he is able to do so quickly and so there is a high risk game of risk and opportunity where the parent must accompany him on long waits on the potty or a less successful outcome is achieved [see above].

I add "potty seat" to the things being brought to Australia.

Shoes
Frost is having ongoing problems with a plantars wart. The little sticky things you are supposed to stick on it fell off all the time. The doctor has frozen it off with liquid nitrogen twice and it has still not "fallen off". Tonight I put a sticky patch on again and some bandaids to hold it in place and we are going to take him back to the pediatrician next week. While we were talking about it Frost asked what shoes he would use in Australia (he wears one pair here, his Blundstone boots). He suggested "perhaps we should go shopping for shoes in Australia. I imagine the shoes they sell there are adequate for their warm climate".

Honestly, his vocabulary borders on the antiquarian sometimes.

I suggested we get some crocs since he has outgrown his.

"Yes, in case they don't have good shoes."

Wren
Here is Wren, playing washing in the kitchen. See how long his hair has grown. Wren is still very anxious about things and worries a lot. At the moment he is fearful of the bath, mummy getting sucked down the plug, washing, being splashed in the bath (without water in it), hairwashing and "That lady come" [the synagis nurse who he recalls from association with a large bandaid]. Unfortunately "that lady come" on Monday. I am not sure how to handle it as he associates the Emla with her painful arrival but if I do not apply it the shot will be more painful. Argh.

Shall we eat otters?

Wren enjoys paging through books that are not-for-kids. These are large, lavishly illustrated and heavy. It is easy for him to rip pages. Right now he is looking at the Soup Bible - which is one of my favorite recipe books these days. We are looking at the first chapter which shows and talks about Ingredients.

Wren says, "I doan know what dats is?" He is pointing at whole plucked chickens on a wooden counter. I explain they are chickens for soup.
"I buy them at store." I think he has seen them at the supermarket and I agree. We move to seafood. I ask him whether he would eat a crab and he seems confused. Next, Wren sees a basin of large fish

"I want eat dose otters!"

They do look a bit like otters, lying side by side in the tray but are in fact large fish. Wren looks carefully "salmon? BIIIG Salmon!" he suggests. Yes, I say. "Salmon."

Wren fetches his toy food and a small pan from the kitchen. He is making soup for skeleton but it is "not ready yet little keleton." There is in fact a little skeleton who is at the feet of big skeleton. Big skeleton is "daddy" and "little 'keleton is Wen Ben."

Cardiology Clinic Visit - 3 months post OHS

Wren had an echo and EKG today. The quick summary is that he looks good, 3 out of 4 of his heart obstructions / defects are stable / OK but the fourth, his aortic valve stenosis, has progressed slightly but measurably. We are going to watch that carefully next clinic visit in 4 months time. We are free to visit Australia in the interim!

Here is Wren having his echo while watching Big Bird in Japan and cuddling snuggle bunny. Laura was our echo tech.


Here is Wren having his EKG. He was very good today and after his initial anxiety about the clinic he did very well. He was very pleased and self-congratulatory when he was "all done" and free to go "supermarket."



Now the detailed version:

Wren is gaining weight fine. He now weighs 13.4 kg or 29.5 lbs. He has gained 800g since the last clinic visit 3 months ago. His physical activity and development does not show signs of any delays or problems. However, Dr Lewin mentioned that in future we should bring up any developmental concerns with our pediatrician even if they are slight. For the first time he even mentioned school, saying that some families have a complete neurological assessment prior to entering school and that it might be a good idea. Even reading delays or other apparently normal issues can be related to surgery etc. For now, we have no concerns in this area. Wren has just entered the "why" phase and is asking us "why do crows eat?" and "why does the [telephone] wire go into house?"

The Three Areas that are Fine
Wren subaortic obstruction which was resected during his last surgery is fine. There is no sign of recurrent narrowing although that is a known concern with the procedure.

His supravalvar area is open and clear as is the hypoplastic aortic arch which was repaired in his first surgery at 6 days old.

His mitral valve which was classed as having mild-moderate regurgitation last time is actually slightly better with only mild regurgitation. Yes!

His Left Ventricle is still mildly thickened but not beyond what is expected with his aortic obstruction remaining.

Aortic Valve
Wren's aortic valve annulus remains too small and has shown some progression in gradient in the past 3 months. The max velocity last echo in December was 3.9 m.s and has increased to 4.2m.s. The mean gradient has increased from 37 to 42. It is still classed as moderate stenosis but the threshold for intervention is generally 50 which is not too far off it the stenosis continues to progress like this.

Dr Lewin said he has seen cases like this where the stenosis becomes stable and that it is not clear whether it has been happening gradually or happened in the period closer to the surgery. If it is progressing the next step would be to talk about a ballooning of the valve in the cath lab.

We are going to try and be optimistic and hope for a stable aortic valve in our appointment in 4 months.

Overall, things went better than I had feared and almost as good as I had hoped (except for the AV). Plus, we can gather our emotional equanimity in Australia!!!!

Cardiology Clinic tomorrow

Wren has a cardiology appointment tomorrow at 2pm. He will have an echo, EKG and clinic consult. We are hoping he is stable post surgery without any alarming pressure gradients across the aortic valve and no further developments on the mitral side.

Calm, reassuring vibes would be appreciated.

It is 3 months since his OHS, AV repair, supra and subaortic resection.

Some overdue comments from our support group meeting...

Last month a large gathering of parents from our CHD support group met with the Directors of our local Children's Hospital. The idea was to build some bridges between the Heart Center and our group and to hear the answers to some recurrent issues discussed in our group, in particular:
1) What is the long term prognosis for our kids?
2) How do you / should we handle second opionions as families try and decide where to have a surgery performed?

I felt unhappy after the meeting which is why I haven't written about it before. It was informative, surely, but less about particulars of Wren's condition and more about the field in general and how the team works on issues and approaches to various cases. Here are a few points that were discussed.

Q: What are the long term outcomes for kids undergoing repairs for complex CHDs today?
A: We don't know.

Since the field is so new we don't have historical data going back 50 years. Developments in the field are rapid and the implications of changes in how surgeries are performed are not known. As children age the issues they face inform the surgeries that are performed today. There need not be a full study written up, surgeons meet and discuss what they are seeing and individuals come up with possible solutions.

What is the future for a child with a fontan? Its difficult to know because the children who are now teenagers had a very different fontan to that which is peformed today. There are a large number of patients but we still don't know what the future holds.

When families ask the cardiology team this questions "we have to give the parents an honest answer" and say we do not know the future. The technical advantages and impact of modifications will only be know out in the future.

With Fontan kids, we think that most of them will end up in transplant but there are possiblities for palliative steps and improving the performance of the fontan before that point.

Q: How do you feel about second opinions?
A: We encourage them and seek them ourselves on some cases.
BUT there is not enough volume in congenital heart surgery high volume centers to show significant statistical differences in mortality outcomes. Centers may show 96% survival vs 98% survival but those differences are not statistically significant. Participation in databases tracking outcomes are voluntary and many institutions do not report. This means we don't have the data to compare outcomes accurately and say which institutions does a surgery better.

Added to this is the degree of variation in surgical approach. A second opinion may well conflict with the first one because surgeons do not approach repairs in a unified way.
1) Surgeons favor procedures they do well, are comfortable with and believe are successful.
2) Surgeons use different thought processes in deciding what surgery or stages will lead to the best result.
This can lead to confusion for parents when faced with two conflicting approaches. Who can say which one is right? It is not know what is best? You have to choose what feels best.

I asked why the two surgeons / cardiology teams could not consult with each other and come to an agreed approach and plan for where and what surgery to perform. This was greeted with some laughter and the assurance that this would not happen because doctors in the field are also 'competitive' and believe in their approach.

I still do not really understand my response to their contribution. I felt really uncomfortable during the talk and afterwards I have had trouble thinking about it - almost as if I am avoiding something. Honestly, I don't know what. Perhaps it is the degree of uncertainty we all feel reflected back at us from the medical team. In some way it is easier to believe that doctors have a good idea of what the future holds for our kids but choose to keep it secret rather than believing they have some framework for assumptions but are learning while doing.

More than that though, I think it framed for me the difference between being a parent and medical professional. Even if you care for your patients there is an objectivity and generality about the work that is at odds with the specific love and advocacy that comes from being a child's parent, protector, voice in the hospital and ultimate guardian in such major life changing or even life and death decisions.

It has made me reflect on the role I play as a parent and realize again how important it is to act as an advocate and take responsibility for exploring options in the decisions that are offered to us.