Quality of Life

Iggy and I received the news about our son's HLHS on Monday, and we both took the day off Tuesday to grieve and process the information, and to make a lot of phone calls to family and friends and to doctors.

Several of the people we have talked to, both family and doctors, have suggested that we terminate the fetus. We are now at 20 weeks gestation, and by law, we have 4 more weeks in which this is an option. If we knew that our child would be severely disabled, that is what I would want to do. But that's the thing...we don't know what our baby's outcome will be. There are several people in their twenties who were born with HLHS, who had these surgeries, and who had pretty normal childhoods, and went on to college, and who are doing OK. The collective wisdom is that at some point, all of these people will require heart transplants, but nobody knows for sure. And as an M.D. friend pointed out to me, cardiac surgery and transplant surgery are two of the fastest-changing fields in medicine. 20 or 30 years from now our son may have a lot more options if his heart starts to give out.

Some babies with this die during or shortly after their first surgery, in the first month of life. Some survive to adulthood. I think the hardest thing is to have a child somewhere in between there, particularly a child who has to suffer through lots of complications, who has to have tubes in his body to help him breathe and eat, a child who experiences growth restriction and developmental delays. That is my dread.

For this reason, we are trying to get all the information we can about what factors predict success in these surgeries. One huge one seems to be the site where surgeries are performed. We are currently being treated at UCSF hospital, which does a handful of these surgeries, but is not particularly considered to be a leader in the field, as far as I can tell. We're also looking into treatment at Stanford, which seems to have a slightly bigger and better-known program, but the huge kicker there is that I don't know if our insurance will cover us at Stanford because it's "out of network" and my insurance company says it won't cover out of network services that are provided in network. But I haven't yet looked into how negotiable that is.

Another factor that can predict success is the size of the baby when he's born. Obviously, we won't know that for a while, but I don't think there's any evidence of growth restriction or anything so far. We're going in for another ultrasound in 6 weeks to make sure he continues to grow on target.

Other predictors of success are the absence of other health problems. I have a bit of nagging worry about this, because when we went for our 20-week ultrasound, which picked up the "bright spot" in the left ventricle, they also said they saw a bright spot in the baby's bowel. When the Ob called to tell us about this, he said that they were both "probably nothing." Obviously, the heart thing turned out not to be nothing. They sent us for some follow-up testing on the bowel, too, but we won't have the results for a week or so.

And finally, there are some elements of the heart morphology (besides the HLHS) that can predict success with these surgeries. I'm planning to call the echocardiographer at UCSF back to talk to him about this, because I didn't know about it until yesterday. In addition, I'd sent a disk of our echocardiogram down to someone at Stanford whose name had kept coming up in my research. I had called her office, and learned that I couldn't set up a consult with her until I had insurance authorization, which, again because Stanford is out of network, would take at least a week, if I could get it at all. But I guess she took pity on me, because she told the nurse she'd look at my scan if I mailed her the disk, which I did on Tuesday, sending it overnight, with delivery confirmation. Of course then, I wondered if she would ever actually get around to looking at it, since I'm sure she's incredibly busy and she was just doing it basically as a favor to a stranger. But then, through a tremendous stroke of luck, a woman I work with, a pediatrician, told me she was good friends with one of the attendings who worked in that department at Stanford. My colleague spoke to her last night, and she agreed that she would look at the scan with the person I'd sent it to, and is going to call us tonight and talk about it, which is absolutely terrific news.

It's absolutely astonishing how much greater access you can have if you have a few friends in the medical community. My high school friend Elaine is an ER doctor in New York, and she has also been working her network for us, talking to people about where to go and what to do, which has been tremendously helpful.

Anyway, that's where we are now. Termination is off the table unless we find out something else is seriously wrong. However, if things don't go well down the line, we will also have the option of not doing the surgeries and providing comfort care only.

We feel like we need to give him a chance. But we also want to make sure that it's a real chance at a normal life, and not false hope.