Friday, August 21, 2009

Well, it's been an intensely busy couple of days, and a lot of that has been HLHS-related. I do realize that I have a tendency to go on in excessive detail about things here, so I will try to sum everything up as concisely as possible!

First of all, I finally did get to talk to Surgeon #1 yesterday, and he spoke to me for a long time. It turns out that he actually developed the Hybrid procedure, which I didn't know, and while he couldn't really give me a lot of data on outcomes of the procedure, because it doesn't really exist yet, he made some pretty strong points about why the Hybrid might be better than the Norwood.

I don't want to get bogged down in discussing all the details, but will just say that at this point, the biggest question mark in my mind about the Hybrid vs. Norwood issue is the stage 2 surgery. With a Hybrid, you have a much less invasive and less dangerous first surgery, and a more invasive and more dangerous second surgery. The idea is that by the time of the second surgery, the baby is stronger and more resilient, but it is a challenging procedure for the surgeon, so I think I want to try to meet with Dr. Azakie again and talk to him some more about it.

To be honest, one of the scary things about going the Hybrid route is that if the baby does die, I think it would be easier on us for him to die around the time of the first surgery rather than around the time of the second surgery. Obviously, either scenario would be devastating for us, but I think it would be a lot harder to lose him after we've already integrated him into our lives. So, I have to say, that is a consideration for me. But only one of many.

This morning we had an appointment with the head of Pediatric Cardiac Catheterization, who would be intimately involved in the Hybrid procedure if we were to go that route. One of the things that's kind of funny about our situation right now is that at the moment, technically I am the cardiac patient. So I had to fill out a form about the medications I take, and they had to measure my height and weight before we met with the doctor, even though we were just meeting with him to talk! The apppointment was not that eventful...basically I asked him a bunch of questions about the Hybrid, but nothing he said really made the decision any more clear, which I suppose is only appropriate since a clear answer doesn't really exist at this point.

After the doctor's appointment, we met up with Jeni, who at 24 is one of the oldest HLHS survivors and one of the first babies to undergo the Norwood surgery, and her husband Nick. They live down in Southern California, but were up visiting relatives in the Bay Area and generously took time to come and meet up with us. Jeni seems to be doing very well, although she told us that she has some limitations in her life stemming from lower-than-average energy levels which make it tough for her to find a job. And of course, like anyone in her situation would, she said that she worries about what her future holds health-wise. Oh, and perhaps most frightening of all, she told us that she doesn't have health insurance. I haven't written anything here about the current health care debate because I don't think this is really the place for that, but needless to say, I am watching events very closely, having both a husband and a son who are "uninsurable" under the current system. Anyway, Iggy and I had a good time meeting them, and really appreciate the fact that they took the time to come hang out with us!

You might have noticed that I didn't mention much about work today...that's because I pretty much got none done. But never fear, I'm going to be in the lab all day tomorrow. Someday I'll be all caught up on everything! Or so I tell myself.

Thursday, August 20, 2009

Other opinions

I know I haven't posted to the blog in nearly a week, but I've been very busy researching stage I surgical options and trying to get as many opinions as possible.

After our meeting with Dr. Azakie last week, when he suggested that we consider doing a Hybrid procedure for the stage I surgery, my first response was to try to read everything in the medical literature about Hybrid. vs. Norwood surgery. That didn't take me too long because there's not too much out there that's been published, and a lot of what has been published isn't really relevant to our case for a couple of reasons. One, the Hybrid procedure itself has been around for a while, but has only really been refined to what it is now in the last 6 years or so. And two, because a reasonably effective alternative to the Hybrid was already in place (the Norwood), most places were initially only willing to try this newer, experimental surgery on babies who had other risk factors that made them unlikely to do well with the Norwood. It's only in recent years that certain surgical centers have started doing a lot of Hybrids on babies who would also be good candidates for the Norwood.

Anyway, there are a couple of surgeons who have been clear leaders in the Hybrid field, and I was hoping to talk to both of them, but didn't particularly want to go through the insurance hassle of setting up an out-of-network consult. I had their e-mail addresses from their publications, so I asked my M.D. friend Elaine her opinion...would it be kosher to just write these guys and ask their opinion? She said she thought it was worth a shot, and advised me to 1. use flattery and 2. make myself sound important. Good advice for most things in life, I guess! Anyway, I'm not sure if it was the flattery, or if these surgeons are both nice people but they both agreed to talk to me.

Surgeon #1 has probably done more Hybrids than anyone in the world. He is the head of a surgical department that does mostly Hybrid stage I repairs, and people from all over the world fly to that hospital to have Hybrids done. Unfortunately, my attempts to actually talk to him have been marred by a series of mishaps that would be funny if I weren't so desperate to talk to him. First, he was supposed to call me on Monday, so I took off early from work so I'd be at home and able to talk to him in private. And he did call, except my cell phone didn't ring OR tell me I'd had a missed call, even though I was in an area where I got reception. In addition to being frustrating, it was also pretty embarrassing that I'd asked this surgeon to call and do me this favor and then I didn't even answer my phone. Anyway, then he asked if he could try me the next day, but I had a work commitment. And then we rescheduled yet again for this morning, but I never heard from him...apparently he'd gotten stuck on an airplane. A few hours later, he called back, and I actually did talk to him for a minute, and then he told me that he was headed into the mountains and that his reception might cut out, which sure enough it did about 30 seconds later. We're supposed to try AGAIN at around 3. God knows if it will actually happen, though!

And then I just got off the phone with Surgeon #2. My impression from reading his papers was that he was a bit more skeptical about the Hybrid procedure, and he told me that at the hospital where he used to work, he'd gone through a period of doing Hybrids on everyone, but had since determined that these had a fairly high rate of complications (although these were all things that could be fixed) between the first and second surgeries and had gone back to doing more Norwoods than Hybrids. But despite this, I felt like he didn't really push me either way...I don't want to say that he wasn't helpful, because he did answer all my questions to the best of his knowledge, but his answers were all pretty equivocal, just because nobody has really shown yet whether one surgery is clearly superior to the other.

I asked him about the learning curve for the Hybrid surgery because the UCSF team has done relatively fewer of these than Norwoods. He said yes, there is a learning curve...but that Dr. Azakie had a very good reputation as a surgeon and was likely to learn quickly. I asked him if he had any sense of whether the neurological impact of the two procedures was different, and he said he didn't know. I asked him whether he thought that blood flow to the brain was as good after either procedure, and he said he didn't know.

So, anyway, neither option seems like a clear winner at this point, but we still have a lot of people to talk to and a lot of time to decide.

In other "talking to people" news, I finally did meet with the friend-of-a-friend pediatric cardiology fellow at UCSF for a while yesterday. We only got to speak briefly before he got called to an emergency, but he seemed really nice, and it was good to meet someone else in the department. Tomorrow morning we have an appointment with the head of Pediatric Cardiac Catheterization, who would be intimately involved in the Hybrid if we chose to go that route, so I look forward to talking to him, and then we're also meeting up with a 24-year-old HLHS survivor who lives in Southern California but is visiting the area with her husband. Hopefully I'll have time to report back on all that tomorrow!

Thursday, August 13, 2009

Quick debriefing

We just got out of our meeting with Dr. Azakie, and while I should be preparing for the presentation I have to give at 9:30, I wanted to jot down a quick meeting synopsis first.

The most notable thing about our meeting is that Dr. Azakie was quite enthusiastic about doing a Hybrid procedure for our stage I surgery instead of a Norwood. From my reading, I knew that many surgical centers were doing this alternative surgery with good results, and the feeling was that it was less traumatic and less damaging than the classic Norwood procedure. This page talks about the differences between the two surgeries. Anyway, less invasive sounds good, but the drawback is that the Hybrid procedure is newer and less established, and when I asked Dr. Azakie how many he had done, he said that he had done 3 or 4 with the current method, and about 10 prior to that using an earlier method. So, not many! As I wrote before, Stanford doesn't do Hybrids because they feel that they have very good success with the Norwood, and are of the "if it ain't broke, don't fix it" school. So, that is a big decision we will have to make, but my bias is that if we can do a less invasive procedure, we probably should, even if it is less tested. But I may change my mind about that.

He said that overall, he'd had very good success with the first-stage surgery (using either method) and estimated mortality from that stage to be 5% or less, and that includes babies with low birth weight and other negative prognostic factors.

Going in to our meeting, one of my biggest worries was making sure he was going to be around to do our surgeries. We are due December 9th, although the baby could come later, and the first stage surgery is usually done about a week after birth, which puts us awfully close to Christmas. He assured me that he is not planning any big holiday vacation, and when I asked him what happened if he got the flu, he said, deadpan, "I don't get sick." "Oh, that's right," I said, "you're a surgeon!" He then mentioned that he had an "assistant"...another surgeon who is new to UCSF. I got the new guy's card but haven't had a chance to check him out yet. I expressed some doubt about being foisted off on another surgeon but Dr. Azakie assured me that HE would be our surgeon.

Some other random tidbits from our conversation:

-He estimated that the baby will be in the hospital for about 3 weeks after his first surgery regardless of whether we did the Norwood or hybrid procedure. He said the main obstacle in going home is feeding issues, so it could potentially be much less time in the hospital if he doesn't have feeding problems.

-He also estimated that about 25% of patients go home on oxygen after stage 1 surgery. This seemed pretty high to me compared to what I've read elsewhere.

-He said that he thought that the hybrid would pretty much eliminate the risk of interstage mortality between the first and second surgeries.

-I asked him his gut feeling about how long these palliated hearts could last, and he gave a rather pessimistic answer of an average of 10 years (in contrast, a more optimistic cardiologist told me she thought 30-40 years, at least). However, he didn't seem to be aware of how many people with HLHS in their 20s are out there. He told me that the oldest patients are now "about 20," and I mentioned to him that next week, I'm supposed to meet up with a 24-year-old woman with HLHS, and that I'd heard from a surgeon who had helped a couple of HLHS patients through successful pregnancies, and he was very surprised. But, since he's a surgeon and not a cardiologist, that's not really his field of expertise.

-He also said that there didn't seem to be anything particularly notable about our baby's case. The one thing that did look risky to him was that his ascending aorta seemed to be particularly small. However, he said that it still might grow more before he's born. Let's hope.

Anyway, overall, he seemed pretty nice and was willing to answer all our questions. He also said I could stop by any time to talk, although I imagine that in practice, his schedule is probably not too amenable to that. But overall, I was satisfied with our meeting.

Wednesday, August 12, 2009

Big Day Tomorrow

Tomorrow morning at 7:30 a.m. we finally meet the most important person in our lives right now: Dr. Azakie, who is slated to perform our baby's surgeries.

It's pretty strange to be finally meeting him after having spent most of the last few weeks reading so much about his career, asking other doctors about his reputation, and talking to the parents of many of his other patients. It's even more odd considering that we both work at UCSF and that his office is not too far away from my lab. In fact, a couple of weeks ago, one of my co-workers, who also reads this blog, walked into lab and told me "I think I just rode up in the elevator with your surgeon!" She read his name off his ID badge and it sounded familiar, but it took her a couple of minutes to realize where she knew the name from. She said he was tall (a piece of data that was corroborated by Jen Campodonica yesterday), and "seemed nice" (I guess he was chatting with someone in the elevator). So yeah, basically I've been stalking this guy for three weeks, and now we get to meet him! I'll let you know how it goes.

Tuesday, August 11, 2009


This week has been marked by a few notable "firsts":

-First stranger touching my pregnant belly. OK, she wasn't a total stranger, but someone who I interact with for a couple of minutes at a time maybe once a month at work. I don't know her name, so she counts as a stranger. I happened to see her yesterday, and she touched my belly and said "oh, you're pregnant!" She was just trying to be nice, and I didn't really mind, but it was a surprise!

-First Braxton-Hicks contractions. At least I think that's what they were. I was sitting in a meeting, when all of a sudden I started feeling what felt like my abdominal muscles twitching. It only happened the one time, though, so far. According to my pregnancy book, this usually starts happening "around your 22nd week of pregnancy." I am 23 weeks tomorrow, so right on target, I guess!

-First in-person meeting with another CHD family. Today I got to hang out a bit with the Campodonicas: Jennifer, Kevin, and their 4-year-old son Zakery, who has hypoplastic RIGHT heart syndrome, which as you can probably surmise, is similar to what our baby has, but involves the other side of the heart. They had come in to UCSF for a doctor's appointment for Zakery, and it was great to hear about their experiences and to meet Zakery, who certainly doesn't look or act like a kid with a serious heart condition! Hearing about and seeing kids like Zakery is a great reminder of what is possible, and that is of great comfort when I have those pesky fleeting thoughts that I cannot possibly handle this. I can, and the Campodonicas have, and we will, too.

Friday, August 7, 2009

The best people

This week I have continued to be the lucky beneficiary of assistance and advice from my various friends and the people they know.

Careful readers may remember that today I was supposed to meet up with the friend-of-a-friend UCSF pediatric cardiology fellow. That didn't come to pass because of scheduling conflicts, but I'll probably talk to him on Monday instead or some other time next week.

However, I did get a lot of helpful information from another source today. My high school friend Rachel is now a doctor at a prestigious hospital back East, and she wrote me this week to say that she'd contacted a cardiac surgeon at her institution who does these surgeries and that he'd kindly offered to meet with her and discuss HLHS. So, this morning, she took him up on that offer, and sent me a lengthy e-mail based on their discussion, which I will excerpt for you here.

A lot of it was stuff I already knew, but even hearing what I already knew was helpful, because while I've talked to a few cardiologists now, this was the first surgeon I'd heard from, so it was interesting to hear his take on things. So, here is what Rachel wrote with my own notes asterisked (by the way, I was very impressed by her note-taking skills here! I can see why she did so well in medical school!):

What you can do right now while pregnant

- stay pregnant as long as possible, and get that baby boy as big as possible. Apparently low birth weight is a poor prognostic factor, so ideally he should go to full term and be a good sized little guy if at all possible.

- He says there is nothing really prognostic to find on the ECHO right now, as much as people have tried to look.

- There is nothing that can be done in utero in terms of treatment. (I wondered if there might be growth factors or something)

- He can’t think of anything that could relate the bright spots in the bowel to HLHS. He had no idea what that might mean.

The post-natal period

- OK, so as I’m sure you are already aware, this is where things get very busy, and I imagine quite stressful for all involved. There are three stages, or surgeries. Apparently there used to be a lot of debate in the field about whether these three surgeries were preferable versus heart transplant, but as the data have matured, the staged surgeries have been shown to be clearly superior. He said the way they are done has been standardized for about 15-20 years, so any surgeon should be doing about the exact same thing. Only a couple places still do transplants.

- Once the baby is born they can look at the heart more, mostly by ECHO is sounds like. If there are other malformations (coronary arteries, etc) or the single heart valve is leaky, those are poor prognostic signs.

- Stage 1: done in the first week of life to minimize the stress on the single ventricle, thereby minimizing the chance of heart failure. The most difficult surgery with the highest mortality rate. National average is about 70% survival* of this stage. Dangers are peri-op things like bleeding, arrythmias, stroke, etc. I’m sure the surgeons will go into a lot of detail about this if they haven’t already.

- Post-stage 1: in the ICU 10-14 days, then at least another week in the hospital; all depending on the individual course your little guy takes of course.

- Then you go home, and again I’m sure others will give you more detail on this. But it sounds like oxygen monitoring at home at all times, perhaps some feeding difficulties, as the recurrent laryngeal nerve can be damaged in the initial surgery and that can affect swallowing. But he said mostly these little babies eat fine, gain weight, and do what a baby would do (sleep, poop, win you over with their adorableness)

- There is a small risk for what he called “interstage mortality” – he estimated 5% or so. This is primarily due to sudden cardiac death. But he stressed that this is unusual, and more likely between stages 1 and 2, than between 2 and 3.

- Stage 2: occurs around 4-5 months. Over time they have been trying to do this stage earlier and earlier to reduce the strain on the heart until the second procedure is done, and reduce the chance for the interstage mortality. This procedure is generally much more well-tolerated with a mortality of <5%.

- Stage 3: Doesn’t happen until the little guy is about 3 years old. Also very well tolerated, mortality <5%. After this stage the intensity of the medical visits and procedures goes down significantly for most children.

- Of course all along there will be very very close follow up with your doctors, and perhaps ancillary services and therapies depending on his exact situation.

- There may be some small cognitive deficits, but he thinks these are so minor as to be mostly un-noticeable. You can be aggressive about testing and therapy in early childhood to try to make up for anything that might be there. I guess it’s largely thought to stem from early surgery, time in the ICU, oxygen effects, less close people contact, you name it --- really, they have no idea.


- Basically, as you are probably aware, the longest term survivors are now in their 20’s, since it’s about that long since the surgery has improved.

- Overall, he feels that if they get through the first three stages, their quality of life is generally very very good. They can do many sports – probably not competitively – but for fun. They go through school normally, etc. Depending on their situation they may need other valve surgeries, etc. But so far, there are no clear boundaries or medical worries. They’ve even helped a couple of female HLHS patients through successful pregnancies, though they don’t recommend it due to the stress on the heart**.

- What is the long long-term situation? Unknown. But as he pointed out, cardiology is an advanced and advancing field. He feels that congenital heart surgery is pretty mature, but adult heart procedures are always advancing.

Overall, he said this is going to have a huge impact on your life, obviously much more than a baby already does, as I’m sure you realize. But he said parents of HLHS babies make very close bonds with, and really treasure their children. It can sometimes be a struggle later, as they grow older to not be too over-protective of them. But kids that have HLHS are wonderful children who live very normal lives, and parents he knows who have gone through this feel it was absolutely worth all the worry and work.

She then added a few more bits of info in a follow-up e-mail:

I forgot to mention in my last email that after stage 1 and even stage 2, the expected oxygen saturations are about 80%, with a partial arterial oxygen pressure of 40. These numbers perhaps won't mean much to you now, but later you'll have this to reference, and I'm sure Dr Azakie will talk to you about all of this too. These will improve to near normal after the stage 3 surgery. I can explain more about why this is, physiologically, if you want me to, though you may already know all about all this (basically the blood supplies are mixed and going in parallel until the 3rd stage is completed). I also forgot to mention that one thing long term survivors have is elevated central venous pressure (CVP). As babies and older children (likely even their whole life) HLHS survivors are usually on lasix to decrease fluid load (which stresses the heart), and vaso-dilators to bring down the CVP. Over time there is a question (no data yet) of whether elevated CVP will damage their kidneys or livers. Again, given that survivors are still quite young, the long-term sequelae of this are unknown.
Something that was interesting to me: Usually the wall of the right heart is thin, and the wall of the left heart is thicker and much more muscular, given it's job of pumping blood to the body. It made sense to me that perhaps in folks with HLHS their right ventricle might become thicker and more like a left ventricle. I asked him, and he said yes, that is what happens. And by the time the third stage is complete, unoxygenated blood never returns to the heart, but is pushed through the pulmonary system by the elevated CVP I mentioned above. The oxygenated blood then comes back to the single ventricle to be pumped out to the body. So in the end, basically, physiologically there is no right heart.

So, yet again, I am deeply indebted to my wonderful friends who have done so much to help! I know I already said thank you a bunch of times today, Rach, but again, this is really great information and I can't tell you how much I appreciate you and the surgeon taking the time to do this.

*My understanding is that survival rates are quite a bit higher for this surgery at centers where they do a lot of these. The UCSF cardiologist told me 85-90% survival and the one at Stanford said about 97% survival in the last year. But I'm not sure if these statistics take into account babies with low birth weight or other risk factors for a poor prognosis.

**I did not know this, although I'd heard of other women with related but less severe heart defects having babies.

Tuesday, August 4, 2009

A conversation with my husband

Iggy: How much bigger are you going to get?
Me: A LOT bigger.
Iggy: (looking wary) Really?
Me: Yes, of course! You've seen how big women get when they're really pregnant!
Iggy: I guess...but I don't sleep with them.

Good thing I have a healthy sense of self-esteem!

Another meeting

I haven't updated for a few days because not much has been happening. Our next round of doctor's appointments are all made, support groups are joined, e-mails have been sent, and now we mostly just wait.

One piece of news, though, is that my pediatrician friend from work actually knows one of the fellows here in pediatric cardiology and he very kindly offered to meet with me on Friday. This is great, because it's the first person who we'll be meeting (besides the cardiologist who gave us the diagnosis) who will actually probably be involved in our son's care, so I'm very excited, and, of course, grateful that he is taking time to meet with me. And thank god yet again that I have so many friends who are doctors!