Sunday, March 29, 2009

The Bioinformatics of Blaise

Erin often warns readers of this blog when she's going to talk about Blaise's output, intestine, feeding habits, or other topics that aren't safe for the squeamish. I feel I should do the same here.

I'm going to talk about math.

This all started innocently enough. A week or so ago, Blaise's milk got bumped up another cc/hour and the morning that followed saw a pretty steep increase in output. At 12 hours post-increase in milk, she was already slightly over the amount she was supposed to be outputting. Her nurse assured us that they were all keeping a close eye on her to make sure she wasn't dehydrating and told us that her output might lessen at night. "Yeah," Erin said to her, "She tends to have lower numbers at night."

Now, I see a lot of parents in the lab, and many of them want to tell me something that they just know is true about their kid. Their kid's really good at holding fixation, he's really attentive, she "gets people," he "just loves science" even though he's 9 months old (seriously...I ain't making that one up). Much of the time, they're probably right to some extent. Much of the time, it's really clear that they just couldn't know the thing they just told you. This sounds cold and callous, but VERY few parents know anything about their kids' microsaccades, for example. So what do you do as a researcher when you hear stuff like this? You take it with a grain of salt, tell them that it's very interesting, and move on.

I don't want to say that our nurse that day didn't believe us, but I've been on the other side of that interaction too often to not notice that she did all three steps: 1) Grain of salt taken. 2) "Hm. That's very interesting." 3) Moved on. My point here is not that this is a problem in any way. It's exactly what I've done many times, and I get it. What struck me however, is that Erin and I both know enough statistics to figure out if our intuition's right or not.

How? Well, first we get some data. We've been tracking Blaise's input and output numbers since early February or so and we can always ask for the numbers for each day from her records. Let's start with Erin's intuition about Blaise having lower output at it true? To do this, we need to know about output in 12-hr periods, which one of our nurses happily put together for us. Given 11 days in late February and early March, a two-sample t-test tells us that Blaise's morning numbers are significantly higher than her evening numbers by about 20mL (t(10)=3.5, p=0.006, two-tailed test). What's that mean? The short version of it that will make statistically-savvy people cringe is that Erin's basically right: Blaise poops less at night, and we're pretty sure that we didn't just see differences that look that way by chance.

Can we find anything else out? Well here's a BIG question that I think nobody knows the answer to: What makes output levels vary day to day? Over a given stretch of days, Blaise gets a constant amount of food, but her output can bounce around a good bit. What's going on? We only have access to so many variables that might explain the data, but let's start with the easy ones and see what we can see. Let's say we know her amounts of milk by bottle (PO) and by feeding tube (ENTERAL) for several weeks and we want to try and use those to guess her output. About the simplest thing we can do here is called a multiple linear basically tells us how to scale and add PO and ENTERAL so that they add up to the right output numbers for every day. If we do this, we can look at the "best fit" of that model below (blue line), plotted with her output numbers (red line):What's going on here? This mostly shows you that input is NOT the whole story by a long shot. Look how flat the blue line is compared to the red one! Her input is nice and steady while her output bounces around it all over the place. Still, we get a decent fit (Pearson's R = 0.76 for those of you that care) meaning that we can account for about 50% of the "wiggle" in the red line
by the shape of the blue line. Here's a funny detail though: When you look at the model coefficients, only the ENTERAL one comes out to be significantly different than zero. That is, statistically, we can't say for sure that the amount of milk Blaise gets by bottle plays a role in determining her output. Now there's a lot of reasons you shouldn't take that at face value: 1) PO and ENTERAL are highly correlated, which means they're fighting for a lot of the same variance in the data. 2) Her bottle feeds were a lot smaller than her enteral feeds for a lot of this data. Still, it's kind of interesting. More interesting to me is the green line on the figure above...that's the difference between our model (the best guess we can make based on input) and the real output. All the wiggling in that green line is variability we can't explain with the amount of milk she's getting. What makes it wiggle like that? I wish I knew (and so does her GI specialist, I bet), but it does look like it's got some structure to it. I'm trying to figure out if there's anything periodic going on, but we need to get some more numbers.

So now the even bigger question...why did I bore you with this? A couple of reasons, I guess. For one, this is what Erin and I do...we spend our lives trying to figure out how things work using tools like this. On some level, I don't know how else to respond to something like this except by sticking to the things I trust most. The other thing is that we were both looking forward to bringing Blaise into our laboratories and having her be part of our work. I think it's hard to articulate to others sometimes why putting an EEG cap on my baby's head or tracking her eye movements with an IR camera would mean something to me. Plenty of people find such things creepy, but for me it's a combination of "Take your daughter to work day" and finding out about her the best way I know how. I think every parent spends the remainder of their lives fascinated by the endless task of figuring out their kids...Erin and I just have a lot of training in that direction and remain excited about using it. For now, this is what we've got; instead of puppet show tasks and brain recordings we've got an Excel spreadsheet and some multiple regressions. Still, it matters.

The last (and craziest) thing is this: No one knows why that red line jiggles up and down. Nobody. There's still no real test of intestinal function, so we basically have to wait and see how Blaise's growth pans out post-op. I often find myself feeling at loose ends when I try and figure out how I can help Blaise thrive, and it's crazy to think I can add anything to the medical literature...but look, I've got a good computer and a lot of math under my belt.

Like I said, this is what we do.

Elusive baby smiles

Eventually, Blaise will smile on cue or at least predictably enough for us to get a photo of it. Until this happens, we'll just take zillions of photos of her making other facial expressions assuming that, statistically, we'll hit a smile sooner or later. See the photo link to the left for a sample of all of her facial expression except a smile. Also, there are photos of her first trip outside and new pictures in the "Visitors" album of her with some of her recent visitors.

The rest of this post will be about poop and breastfeeding, so for those of you who don't want to know (e.g., Blaise's Aunt Libby), here is a picture of a cute baby.

And now, Blaise would like to get serious for a moment.

Actually, nothing that serious. I just love that picture.

We've done two new things in the past week. One of them totally weird and the other the most normal thing I can think of. The first is "poop prep." Blaise has never pooped in the conventional way. She has a hole in her belly that stool comes out of, but nothing has ever come out of her bum except the contrast gel from her imaging study. So the skin on her bum is soft as, well, a baby's bottom. In about a week, though, the hole in her belly will be gone and she'll poop in the usual way. We've been told to expect the world's worst case of diaper rash because her skin isn't used to contact with stool. So to prepare her for regular diapers, we are supposed to take the output from her ostomy, dab it on her bum, leave it for a bit and then wipe it off. That's right. We're deliberately putting poop on her bottom. Like this whole having an ostomy thing wasn't bizarre enough to begin with.

The second thing is something I thought might be off the table completely. I had always intened to breastfeed and right after Blaise was born, I started pumping milk. We had been told that she might never eat and that even if she did, she might not be able to tolerate my milk. A nurse at the Brigham even suggested to me that pumping might not be worth the effort. As regular readers of this blog know, Blaise proved her wrong and my milk is going to excellent use at a rate of over 8 oz a day. But because they monitor her intake and output very closely, I never thought they'd let me actually nurse her and I let that go, one more thing that I would have liked to do, but that wasn't in Blaise's best interest. I figured that, for us, breastfeeding would always involve a mechanical intermediary. Then a lactation consultant at Children's casually mentioned that "when they let you put her to the breast, have them page me and I'll come help."

That bug firmly planted in my ear, I started asking occasionally if, someday, I might be able to nurse normally. I got quite the range of responses. Absolutely not. Oh, certainly, once she's over a certain amount by mouth. Maybe, but that decision is with the surgeon and surgeons are kind of anal. Two tips to other moms in my situation: 1) Be patient and 2) The right person to ask is the nurse practitioner. Most of them are moms and they totally understand the desire, but they also know the medical side of things and have some influence over care decisions. It turns out that now that Blaise is at almost half an ounce by mouth every 4 hours, they see no reason I can't nurse for one or two of those feedings a day.

We tried for the first time yesterday. We have to weigh her before and after to get an estimate of how much she ate. Nearly three months of bottles and pacifiers have made her a bit lazy and easily frustrated by food that takes a little effort to get. She doesn't know how to shape her mouth and I'm a bit awkward at holding her the right way. We'll learn. The important thing is that we get to nurse! They let me nurse my baby!

Friday, March 27, 2009

Finally, a new post!

OK, it's been a while since we've written anything here, so let's start with some exciting new numbers (courtesy of Blaise):

Direct Bilirubin: 0.3 (!) This is officially normal range.
Weight: 3.12 kg (~6.84lbs)
Total milk per day: 252ml
Blood donated on Blaise's behalf: (~2000ml)
Total amount of blood Blaise has: (~200ml, or so we're told)
Actual social smiles exhibited by Blaise: many, and they're both cute and hard to photograph.

Speaking of photographs, here are two very nice pictures of Blaise at nearly 3 months of age. These were taken by our friend Julie during her and Mathieu's visit.

It's really been a lot of fun hanging out with Blaise lately. She seems to have crossed a few developmental thresholds in terms of how social she is, and many of her nurses have remarked that she seems very interested in finding out what's happening around her. Both Erin and I have noticed how much Blaise seems to attend to conversations happening nearby these days, and the early hints we had of her orienting towards our voices have given way to full-fledged direct attention to us when we're nearby. This also means she knows when we've left, which further means that she knows turning on the waterworks is a sure-fire tactic for getting us to stay in the room. A funny detail about that is that she cries with real tears now, which makes her crabby fits seem that much more laden with grief.

The word from her care team is good all around. There've been a few small things here and there we've been a little concerned about, but nothing major. Blaise seemed like she had a small cold last week, along with an elevated white blood cell count (which could signal an infection). Both of these things resolved on their own, though, so no worries for the moment. The most recent development however, is that her lines aren't "drawing" which means her nurses can't get blood out of them to get her lab work done. This is kind of a pain, since it means they have to actually stick Blaise with a needle for the blood they need and also because it may mean there's a bit of plaque (or a clot) blocking the line. I should add that putting things into the line is fine...she's all set for getting her IV nutrition and Omegaven, but it'd be nice if we can get her lines back to being a two-way street. Today they'll try using TPA (a clot-buster) to fix 'em up, but we'll see how it goes.

Beyond that stuff though, she's really just been hanging out, charming nursing students left and right, and getting bigger every day. Of course, we're both starting to get a bit anxious about her upcoming surgery, but Blaise maintains an air of Zen-like calm. April 6th seems very close. Here's hoping I can get some of that calm to rub off on me.

PS - More pictures to follow, we promise!

Friday, March 20, 2009

Unexpected visitors

After many expected visitors over the last few weeks, Blaise was delighted by a few unexpected visitors today. First, her Gran's friend Julie came by this morning. Julie is in Boston to see her son and decided to stop in and see Blaise, too. Neither Ben nor I were at the hospital when she came, but the nurse let her hold Blaise anyway. I hope Blaise was on her best behavior and did not let loose one of the frat-boy-style burps that she is becoming known for. That would have been more appropriate for the next set of guests: the Tufts University lacrosse team.

A lot of sports teams from local schools come around Children's to play with the kids. These guys were a much bigger hit with some of the toddlers on the floor than with Blaise, who slept through their visit. Sometimes pro athletes come by, too, but not while we've been here so far. I'm hoping for a Celtics visit, not because I'm a big Celts fan, but because I think a photo of Kevin Garnett (or any other giant basketball player) holding our tiny baby would be great.

Edit: I've been asked whether the Tufts lacrosse team is somehow significant. Not as far as I know, but I don't know anything about lacrosse. I just thought the photo was funny. Maybe we'll see the BC basketball team soon, as they no longer have plans for next weekend.

Thursday, March 19, 2009

Someone finally just said it

There are three classes of people who know about Blaise: people Ben and I talk to regularly (friends, family, etc.), people who read this blog and people who have heard about Blaise from someone else. This last group of people, when we see them, often don't know what to say. They want to tell us that they know what's going on, but they don't want to just say "I hear your baby is sick." Instead, we tend to hear things like "I hear you're having a difficult time." Today, someone finally said what a lot of people must be thinking: "I hear there's some really crazy sh*t going on with your kid! Are you guys okay?!?" That's kind of refreshing to have someone say because, well, there is some really crazy sh*t going on with our kid.

Our crazy kid continues to do well, though. She now gets 14 mL (almost half an ounce) of milk in her bottle every 4 hours, in addition to the 6 mL an hour through her g-tube. The physical therapists seem very impressed with her on those occasions that she doesn't just cry through her PT. She'll do all the exercises with me, so it could just be that she doesn't like the therapists themselves. She's also starting to self-soothe and also to interact a bit more during playtime.

The founders of the Paris chapter of Blaise's fan club have returned home. They brought baby couture for Blaise, so she will be wearing the latest from Paris this summer. They were, as always, wonderful company and deserve awards for their patience when I butchered their language. (FYI: The French word for intestine is l'intestine.) We are very much looking forward to taking Blaise to meet their daughter sometime next year.

Monday, March 16, 2009

It's official

We have been at Children's too long. Some evidence:
  • The front desk security staff (for the whole hospital) recognizes our friends on sight.
  • Cafeteria workers keep trying to give me the employee discount. When I told one of them that I'm not an employee, she responded, "You might as well be."
  • Residents coming off call greet us on the street.
We were a little sad when we came in to the hospital yesterday to see that Blaise's former roommate is back. She had only been home for three days. We also saw the parents of one of her NICU suitemates in the lobby because their daughter had been readmitted. During the first month that we were here, one of the hardest things was watching other kids go home. I moved past that as I became more confident that our turn would come and I was genuinely excited for Blaise's roommate and her family when they went home last week. Now it's really hard to watch kids come back because our turn for that will come, too.

In happier thoughts, Blaise's bilirubin is down to 1.1/0.5. Oh, Omegaven, we love you!

Friday, March 13, 2009

"We're winning"

The title of this post is a direct quote from Blaise's surgeon. "We're winning." She's doing great. She tolerates feeds, both oral and enteral. Her liver is improving. Her general health is excellent and her behavior is developmentally appropriate. She's gaining weight well. AND SHE HAS A LARGE INTESTINE!

The lower GI contrast study yesterday showed a lot of large intestine, all apparently intact. They can't say for certain that it's the whole thing, although they think it might be, and they have no way of determining whether it ends in an ileocecal valve or just just a plain old dead-end. Still, she has a nice long piece of it to work with and that can only help. The plan for her surgery is officially to close her ostomy and connect her small and large intestines. Barring any kind of infection between now and then, the surgery will be April 6.

Needless to say, we're very, very happy about this news. We were braced to hear that she had almost no large intestine and hoping to hear that she had at least half. Most, if not all, is excellent. We're obviously apprehensive about her surgery in April, but knowing the plan helps a lot.

Coincidentally, Ben's fortune cookie tonight said, "Believe in miracles." Of course, mine said, "Even the greatest whale is helpless in the middle of a desert" (what??) so we're not reading too much into the fortune cookies.

Thursday, March 12, 2009

More milk and more friends

They increased Blaise's enteral feeds to 6 mL/hour yesterday, which, together with 12 mL by mouth every 4 hours, brings her daily milk intake to just over 7 ounces (216 mL). She was doing okay with it as of last night; we'll see how it went over the full 24 hours. This slow, steady progress thing is pretty awesome.

Of course, the big thing today is her lower GI contrast study. We may not have a good idea of the results and what they mean until tomorrow, but we'll be sure to post when we know.

With our family far away, our friends have been great at helping us through this, trekking over to the hospital to see Blaise, offering rides and just checking in. Our friends Kate and Andrew are regular visitors at the hospital and have been amazing about all kinds of practical and emotional support. Over the weekend, Blaise met our friends Richard and Carrie and their 10-month-old daughter Beatrice, who showed no real interest in Blaise until Carrie held her. Dave and Jarasa also came to meet Blaise and brought their daughter Siri, who is almost exactly the same age as Blaise, but a lot bigger. We're looking forward to those playdates in a few months.

And now visitors are coming from around the world to see our little one. Our friends Mathieu and Julie are arriving from Paris this afternoon. Their daughter Erin is also about the same age as Blaise, but she's staying in France for this visit. We anticipate many future visits with them both here and in Paris (once we figure out how to take PN to France).

Monday, March 9, 2009

Makes me want to hug a salmon

I just wanted to post quickly with the update that Blaise's bilirubin level is now 1.5/0.8, or just 0.8 if direct bili is what you obsess about (and it is). This means she is well within the normal range and that the Omegaven has done its thing on her liver. She also really digs the Fisher Price aquarium bouncy seat, which has me a little worried that all the fish oil has made her think that she is a fish.

Now we're just waiting not so patiently for her lower GI study on Thursday afternoon and our Friday meeting with her surgeon to discuss the plan for April. We know she has some large intestine because her surgeon saw some of it during her first surgery, but she was too fragile and her insides were too much of a mess for him to find out much more. She may have only a tiny fraction of her large intestine. She might have most of it. If we're feeling really optimistic, we hope that she has an ileocecal valve (ICV), which is the valve between the small and large intestines. The real outside shot is that she still has more small intestine than we think. The chances of that are beyond slim. We'll work with whatever she has and we're prepared to hear that she has next to nothing. We just want to know what the situation is. But the more she's got, the better.

Sunday, March 8, 2009


Yesterday during my parents’ visit to the hospital, we took Blaise for her first walk outside. Boston’s finally been getting a taste of spring (though we’re due for “wintry mix” tomorrow) and so it seemed like a perfect window of opportunity to introduce Blaise to the world beyond the walls of Children’s Hospital.

Needless to say, this isn’t your typical walk in the park. The first question was whether we could get a stroller for Blaise to ride in. She’s actually too small for a stroller right now, so once we found one we were going to have to bundle her in with some extra blankets, lay the seat flat, and snug her in with a 5-point harness. All this was made irrelevant by the absence of strollers, however…the first nice day in months means stroller demand is WAY up. We waited for a bassinet for a little while, and then asked if Erin could just carry her. This turned out to be alright with everybody (including Blaise) so off we went…Erin with the baby, me with the IV pole, and my Mum and Dad with all the cameras.

Now the IV pole…there’s a piece of equipment that’s screaming for a 21st century upgrade. It’s got six wheels on the bottom, so it’s nice and stable. The stability is further improved by the fact that at any given time about 4 of those wheels refuse to turn. If Blaise has “Short-gut Syndrome,” then the IV pole has “Shopping-cart Syndrome.” You can push the thing, but you’ve got to go very slowly and not be too concerned about what direction you want to move in. Since Erin’s got the baby and I’ve got the pole, it also means a lot of stopping and starting while you try and match pace. You know that industrial carpet they’ve got to soak up all the snow and rain from winter boots? Great absorption…in fact, the wheels of the pole were almost completely absorbed by a piece of that stuff just outside the elevators. My Dad & I eventually had to just lift the whole pole carefully over a few of the carpet edges and door runners along the way.

Still, we made it! We went down to the 1st floor of the hospital, to a small courtyard called the Prouty Garden. It’s a nice little space, with a small pathway that makes a loop around the lawn and a few patio tables and chairs for when the weather is nice. The sky had clouded over since we’d come in, but it was still about 50 degrees or so and there were lots of birds chirping, a nice breeze blowing through, and green grass to look at. What did Blaise think of all this? How did she react to her first visit to the outside world…her first breath of fresh air, the feel of wind on her face?

Yup. She slept through the whole thing. We kind of expected her to, honestly. She had been pretty excited about napping all day, so we thought the trip outside would either totally freak her out, or just be a nice change in setting while she slept. The former would have been a lot more difficult than the latter to say the least! Besides, whether she’s awake or not, it’s great that we could take her somewhere besides the playroom. And even though she was pretty sound asleep, maybe something snuck into her dreams for a bit (although we’re both convinced that she dreams exclusively of having more milk in her bottles).

After our brief adventure, Blaise settled in for a nice evening and said goodbye to her Grandma and Grandpa before they headed back to Pittsburgh. Today’s been similarly restful, to the point that we had to wait for her to get a little wound up so that her weekly blood draw didn’t take forever (crying makes the blood flow!). We described the series of events leading up to the current recession and that did the trick…instant tears and ~7cc of blood in no time. We followed up with blankets, a hug, and the assurance that limited nationalization and re-privatization of insolvent banks could indeed be a viable solution. She calmed right down.

Saturday, March 7, 2009

At least it was for a good cause

I had this plan before going on the radio yesterday that I would be totally calm and collected. I would talk intelligently about how Children's has saved Blaise's life and how grateful we are to Dr. Weldon, her surgeon, Dr. Duggan, the GI nutrition specialist, Dr. Puder, for Omegaven and all of her nurses, who are somehow as good at the end of a 12-hour shift as they were at the beginning. Listeners were going to say "Wow, what an amazing story and what a great hospital! I'll call and give money!"

Instead, I cried. On the radio. While talking to Gregg the DJ. (At least it wasn't Fast Freddy.) I made Sue the DJ cry. According to their comment on the blog yesterday, I also made the Brogan family cry. Maybe I made a whole lot of radio listeners cry, too and they said to themselves, "Oh, that poor woman and her poor little baby! I'll call and give money!" So, different means, but the same end.

Ben's parents are here this weekend, bearing more cute baby clothes. The weather is really nice, and we might take Blaise out to the garden today. She's never been outside before, so we'll see how she likes it. My guess: she'll either sleep through it or cry. Because that's what new babies do.

Thursday, March 5, 2009

Blaise: Media Darling

As though having so many internet fans wasn't enough, Blaise's story will be hitting the airwaves tomorrow afternoon. Mix 98.5 is doing a telethon to raise money for Children's and once an hour they have families come on the air to tell their story of how Children's helped them. We were asked to participate and I'll be going on at 2PM tomorrow afternoon. We'll see how much the story of one strong little baby with a short bowel can inspire people to give. If they post the interview on their website, I'll be sure to link to it.

Also in the donation vein (pun very much intended), we'd like to encourage everyone who is able to take the time to donate blood this month. Blaise was saved during her first surgery by the availabilty of donated blood products and thousands of others are helped every year by people who take the time to give blood. They always need all blood types, but Children's has a particular need right now for O-negative, which happens to be Blaise's blood type. If you are O-negative and would like to donate blood specifically for her surgery, let us know and we'll tell you how to do that. Any blood donated for her that she doesn't need will be made available for the general population. But again, they always need healthy blood of all types, so no matter where you are or what your blood type is, we'd really like to encourage you to donate.

Blaise is now pushing the 6-pound mark, weighing in at 2.7 kilograms. She's holding on at 192 mL (about 6.5 ounces) of milk a day. In less great news, her g-tube (the one that's implanted in her stomach) fell out this morning. It's not that big a deal, in the scheme of things; they can put it back in right in her room, but then we had to go to radiology to make sure it was in right and she was a very grumpy baby. We also found out that March 12 (1 week from today) will be when they do the imaging study to find out how much of her large intestine is intact and whether there are any more blockages. We're hoping for the best, but if all she has is what we're working with now, we can keep working with that.

Monday, March 2, 2009

I love fish oil!

Blaise gets blood tests every Sunday night to check her electrolyte levels, blood counts and bilirubin. The bilirubin, of course, is the one we wait for to find out how well the Omegaven is working in terms of fixing her liver. We could not have been more excited to find out this morning that her bilirubin levels are now at 1.2, down from 2.3 at their highest. This is some of the best news we've had since this whole thing started.

Blaise is now officially two months old and doing better than I think anyone expected. She has tolerated every increase in her feeds so far, she's growing well on the PN and we seem to have dodged the liver damage bullet. We know the sailing won't always be this smooth, but we're not going to complain while it is.

But I know some of you are bored with medical updates and just come to the blog for pictures of a cute baby. So here you go. (There are more up if you follow the link to the left, too.)