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.

The dazzling whites of shining snow

I staggered out into the kitchen at 5.45am to take my medicinal licorice [1] and some tea because my throat is still croaky in the morning. Wren was yelling "come play!!!" at my departing back. The moment I came into the kitchen I knew something was up. The room was bathed in the distinctive luminosity which comes from SNOW shining through the patio doors. Yes, snow shines. Anyone who has been out at night knows how it transforms the night - even a moonless one. I called Wren to come see and he stood for a long while with his face to the glass at the back and then the front window. Finally, he had had enough. He started whining.

"Turn off that snow. I want stop it. Turn off that snow MOMMY!!! I want stop it going down on the ground. I want turn it off!!! I want to! I do no like it."

Wren throws himself on the ground fake crying. A moment later he sits up.

"I can't see snow! Booky beek. Booky beek."

And he began reading one of the new library books we picked out yesterday.

School had a delayed start due to the snow so after breakfast we rugged up and went out to make a snowman. The snow was too dry to roll so we threw snowballs, shoveled it and slid down the snowy slide. It was fun!


Potty Training
We have started potty training with Wren. Over the past 2 days he has had two successful potty visits although he doesn't seem to be able to predict when anything is likely to happen. We read a few books and sometimes we are 'lucky' with results. After we flushed a poop last night Wren was very worried about it. He left the dinner table to close the lid of the toilet in case "the poop came up again!" He had noticed the toilet refilling with water after we flushed and was concerned that it might not be clean!

We have not had any luck today but have had a few accidents as we go diaper-less sometimes hoping for results.

Here is the chicken coop
We are waiting to buy pullets after we know whether we are going to Australia. Josh has submitted his passport application [yay!] and I have submitted Frost's APP appeal [yay!] so there are only a few more Highly Significant Events to overcome before we can really plan a vacation [ok, not so Yay due to the cardiology clinic on Monday].

Doesn't it look hopeful and wonderful? Should we paint it? The second picture shows where it is located in the back yard.



[1] The licorice has been prescribed by my naturopath to help me cope with the less than desirable hours of sleep Wren requires of me. Apparently, taken on waking and at noon the licorice helps regulate cortisol production or some issues with the adrenal gland. I have noticed it has improved my sleep at night - I don't seem to wake as easily.

Various beeps in the background

Until now, arriving at Wren's bedside when he is still trying to sleep, I haven't had time to write about all those other aspects of hospital life which create the unforgettable atmosphere. Here are a few glimpses:

• Beeps. Anyone who has tried to get a kid to sleep in hospital knows about the beeps. They drive us crazy. During the critical care and recovery time you tolerate them because its reassuring to know someone knows, and could be responding to every vacillation in your child's vital signs. By the time you hit the floor you are starting to invent technology to implant the beeper in the specific nurse and have total silence. Could we have them wirelessly phoning the nurse's headset instead of alarming? I suspect that nurses are so inured to beeps that they could sleep right through them while the rest of us writhe and turn, jumping within a second to silence them. Of course, we have all learned how to silence alarms but only for 3 minutes without turning the monitor onto standby (which I do too at times when we go to telemetry). What we are supposed to do is diagnose and remedy any beep Wren has on the floor. This means retaping his toe pulsox and resticking his respiration monitors (3 wires he tosses off in bed)... without waking him. Is it worth it? Not tonight.


• Helicopters: Day and night, every few hours you hear helicopters. You don't hear them in 3W but whenever I went outside, even to the cafeteria courtyard, I have a good chance of a helicopter. The air ambulance lands on the left roof of LPCH and sometimes hovers overhead a while before swerving off away from us. In the CVICU you often get ambulance nurses [good god, I can't even remember the word for them I am so tired and brain dead - oh! Paramedics... not dead yet!] leaving with their stretcher/trolley thing piled high with bags. They must bring the patients in some way that is secret and fast but leave along with the rest of us.


• Construction: All day, you are surrounded by workmen. They carry their coolers at lunchtime, rolls of cable at 9pm, carts of tools and tape all day. As I was wheeling Wren about the floor yesterday he kept asking "what that noise?" as the roof was shuddering. I told him it was "drilling" and "tools" and he asked for "more drilling?" It was "too loud" I told him. To give the construction crew credit, they are the neatest, tidiest, most considerate workers I have ever seen. Well, you don't see them work because all work areas (however small) are encapsulated in tents before the tools are used. So all around the hospital you see these little white tents from which noises, and occasionally heads, emerge. I was told that once, when they didn't use the right tent, the roof flooded and part of the top floor. This may be a rumor. The worker who told me looked wry and amused.


• Wooshing: I don't know if its the air-conditioning, a ventilator, a noisy computer or just the oxygen mask which streams air above Wren's bed, but hospital rooms always have the hum of machines. Its a background wooshing like a white noise machine set a few notches too high.


• Hand sanitiser: Its everywhere. I know we are supposed to use soap as the first line of defence but apparently everyone has already fallen back to the secondary position. Nurses, families, kids in the playroom all get a perfunctory squirt of a goopy white stuff that smells of alcohol. It doesn't seem to be messing with my skin as much as the soap wash in the Seattle CVICU but perhaps that means it isn't working. On the plane I read Mother Jones which had an 'expose' on the soap industry. I think it mentioned the irony of our germ phobia while Australian doctors are combating the rise in asthma by feeding children dirt-tablets as medicine to build immunity. I just want to say now, we are not usually this clean. We will not be this clean again. Just give me a few months to recover, to regain my normal wallow.


• UV and Gloom: The lighting levels are strange in hospitals. The halls are that tepid level of institutional UV which is neither bright and cheerful nor gloomy. Enter a room and its a different story. The rooms are kept shaded most of the time - like little caves. However, each bed has a few light settings and none of them are mood lighting. They are just too local to feel BRIGHT unless you want BRILLIANCE (as if you are illuminated for surgery or a procedure or were growing dope in a cellar). None of it feels normal.

So far today
Discharge plans continue. Labs were done 4.45am. I think he is almost up for the day but now its Chest X-ray time.

3-West!

Wren has been prepared for transfer to 3-West. I left at 2pm to change shifts with Joshua and we received word that he will be transferred at 2.30pm. They are moving a patient from the critical-care hub of the CVICU into our spot and a new post-operative patient is coming into the unit.

Our room has already changed a lot today. The two older kids with pulmonary valve replacements were discharged. Not just to the floor, but home! Wren was third in line for the Ward but because they were discharged he got to go early.

We are very excited but now have to take a greater responsibility for his moment-to-moment care and will sleep in the room with him.

While I was there over lunch he had his central line removed and his dressing changed. The incision looks excellent. He now has only his chest tube, left arm IV and the pacer wires which stick out of his chest in an alarming way. Frost's friend Dylan would find it fascinating because they look just like wires to plug into something (which they are, kind of). They are there for "an emergency" and will be one of the last things to be removed.

Pictures
Here is Wren having his echo done yesterday. He was very good and watched a video about animals which included the fascinating fact that sloths can swim. We saw a segment of swimming sloths in a swamp. Its enough to take your mind off things.



Here is Wren this afternoon, shortly before central line removal. The central line goes into his neck and you can see the little white disc covering it. You can also see his "big bandaid" - the blue splint - which stops him bending his arm and occluding the IV. He is playing on the telphone, calling Ky-ky and saying "ello" in a singsong voice.

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.

----------------------------------
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.

The "how is his heart?" question (10 monthday)

This afternoon Wren and I got together with some babies and parents from our Listening-mothers Infant group. It was lovely to see the babies so big and social. At the group I was asked a question which has come up a lot of late. "So, how is his heart doing?" I have been asked on 2 phone calls and by 4 other people in the past 24 hours.

This question is difficult to answer. Wren's heart is not entirely normal. He still has aortic stenosis (mild), a bicuspid aortic valve, a mild coarctation of the aortic arch and the most serious concern - a left ventricular outflow tract obstruction (LVOT obstruction). His left ventricle has been mildly thickened since before he was born and remains pretty constant at a slight thickening.

The great news is that he seems to cope very well with these obstructions. If his stamina is reduced we have not seen it yet and we are unaware of any imminent problems like high blood pressure, regurgitation, problems with the valve or strain on his left ventricle.

However, we are not sure what these obstructions will mean in the long term. We have not been told that they will go away or become insignificant. We have not been told that they will require intervention. All conversations are along the lines of "if he shows the need we will do X and Y".

So far, he seems to be in the "chronic" state with his CHD - meaning he is pretty stable and will need monitoring every 6 months or so unless something changes. This is pretty hard to live with when you think about it but since Wren looks so well, is thriving developmentally and is clearly happy and 'healthy' we do not worry much day to day.

IF he did need surgery it would probably be open heart surgery to address the LVOT which can damage the aortic valve if turbulence is severe. Our cardiologist said they would be cautious about suggesting that surgery because it has a significant risk of causing scar tissue at the site of the repair which leads to further complications. We hope Wren does not need the repair.

Thank you for your concern, those who asked!

New games
Wren turned 10 months by stealth yesterday. He continues to surprise me with new games and cleverness. Today he continued his "I feed you, you feed me" routine and tried to feed me water from a cup in the bath. I had just shown him how to hold a little plastic cup and drink from it and he had taken a series of gulps while holding it himself. I showed him the sign "drink" and opened my mouth. He brought the (now empty) cup to me and pushed it to my mouth with a huge smile.

Bathing together
This is the second night we have bathed together and its fun. He likes to stand in the bath (probably because it is deep) and splashing makes him laugh. He is fascinated by the stream of water falling from a bottle and makes serious attempts to hold it. He also laughs at the gulping noise of air exiting an empty bottle pushed underwater. He squeals and laughs.

The Later Bedtime Attempt
Tonight, Wren went to bed at 8pm. This is part of the plan to get him to sleep past 5.30am but also in anticipation of daylight saving causing his current sleep habits to go awry. I really cannot go back to the days of 4.30am wakenings. It is just not going to happen. We all ate dinner together in a strange parody of a Top Chef challenge - my intention for us all to eat the same thing failed and we all ate different meals with similar ingredients:

1) Me: bunch of Kale with red pasta sauce, vegies and chicken
2) Wren: bits of Kale, vegies, chicken.
3) Frost: vegies, red pasta sauce, pasta, vegie meat balls.
4) Josh: pasta, vegies, chicken, alfredo/mushroom sauce.