The Shirt Doesn't Lie!

"How's Rory doing?" A common question I get often. The answer: Good. Rory's doing good. She continues to respond well to the dilations, and quite frequently does not require them. We await the manometry study from the Children's Hospital Orange County (you can call it CHOC, if you're in the know). 

It has been a big pain getting the referral required to go 'out of network' for this test. But CHOC is the only place in southern california to get this done for babies, so we have no choice. And while you might immediately jump to the conclusion that the insurance company is the hold up, that isn't necessarily the case here. The referral was supposed to come through the GI's office, and we're pretty sure at one time or another they 1) dropped it, 2) referred to the wrong hospital, and 3) ordered the wrong test.

But, with some help from the pediatric surgion, even calling CHOC and speaking to those folks himself, we've finally got a date for the initial consultation! Yes, initial consultation. You didn't think they were going to let us walk in off the street and get the procedure performed, did you? At least we've got our foot in the door now. Unfortunately, I doubt we'll make the schedule for the actual test being performed in this calendar year.

From the CHOC website:

Anorectal manometry
This test helps determine the strength of the muscles in the rectum and anus. These muscles normally tighten to hold in a bowel movement and relax when a bowel movement is passed. Anorectal manometry is helpful in evaluating anorectal malformations and Hirschsprung's disease, among other problems. A small tube is placed into the rectum, and the pressures inside the anus and rectum are measured.

An Ode to Insurance Companies

Well, well, well, what more can I tell?

Rory couldn't poop, so we were thrown through a loop.

And our little prize spent the summer with Rady.

They couldn't find a cause for her tiny bowel's pause,

But sent home a bill that would make most men shrill,

And now Aetna's price tag grows into a Lady.

A Halloween Tradition

Halloween 2003. Reese was just 13 months old. Christine and I had our limited Halloween supplies out of the closet, most of which consisted of a Mad Scientist cosutume I'd worn in college. Just for fun, we threw it onto Reese's head and snapped a few photos. This picture remains as one of my favorite photo's of my son's early years. The best part about the picture was how his 13-month old head almost completely filled out the adult wig.

Flash forward to Halloween 2004. We were now the proud parents of two - one toddler, and one 9-month old. Halloween box was out of the closet again, and Riley joined the 'Mad Scientist' club. Note how the 9-month-old's head almost fills the entire wig.

Halloween 2011. Seven more years have passed. The Mad Scientist wig, still clinging to life, continues to be a Halloween staple in our household. Our newest edition has some room to grow before filling out that wig, but she wasn't quite 5 months old yet, so it really isn't a fair comparison.   

The once proud Mad Scientist wig isn't doing so good these days. It's previously perky quaff is looking a little wilted and frayed. But it still makes us laugh when donned by a baby. I wonder if it will last until our first grandchild is born?

September was a Good Month for Rory

I know, its been more than three weeks since the last entry here. Summer is officially over. September has come and gone, and today marks the begining of October. School is in full force, and I'm back into the swing of things at work, up to my eyebrows in new and catchup work.

You may recall that when we left the hospital, we had the threat of an ileostomy hanging over Rory's head. The docs had pretty much told us that if Rory didn't respond to the treatment at home and ended up back in the hospital, they'd just go ahead and do an ileostomy. So there were many ups and downs those first few weeks, and each squirm, grunt, or fuss (typical behavior for any other baby) brought anguish and concern. I like to look at trends. I literally do, just about every single day. My career revolves around performance trends for water treatment systems. I recognize that you can not look at a single data point to assess a situation, you have to look at the trend of many data points. Christine was not comforted by my analogies of our daughter's health to a water treatment plant. I was not completely convinced either, because while Rory's overall trend looked good to me, I also knew she could crash in an instant.

But September turned out to be a good month for Rory. During the monthly visit to the pediatric surgeon, he reported that he was very pleased with Rory's progress and response to the dilations at home. So much so, that even if she landed back in the hospital, he would not do the ileostomy. They'll just decompress here, and send us home to keep doing what we're doing. I cannot tell you how much stress that relieved.

We also had a visit with the GI Doc. She was also pleased with how Rory is doing. So much so, that she said we could back off the liquid gold formula. She recommended a different formula. Not your standard off-the-shelf stuff yet, but another 'special' formula that's about half the cost of the previous formula. Its more like, liquid silver. We mix that with breast milk. And maybe that's the best part. I don't think I reported this previously, but we could not actually mix the liquid gold with breast milk. Rory would literally gag and spit it out when we tried that. To feed her half liquid gold and half breast milk, we were heating up two separate bottles for every feed - every three hours. Now we can use a single bottle. It's the little things...

Rory had an appointment with the physical therapist too! She was indeed a busy girl this month. Christine was too. And they said Rory looked pretty good. The mild stretching exercises, and holding her the opposite way when feeding, were doing the trick to correct her stiff neck syndrome. Now we're just working on ensuring she has a full range of motion. That means a lot of tummy time, and giving here things to look at, at specific positions so she cranes hear head many directions.

September was a good month for Rory. Let's see what October holds in store for her.

Through the Eyes of Babes

I know, that's not the exact saying, but its appropriate in this case. My son's first creative writing project is due tomorrow for his new school. He had to write a few paragraphs on what he did this summer. You remember that assignment, don't you? Here's the text from his story, aptly titled "My Summer":

"I spent most of my summer with my best friend Brody because my sister Rory was in the hospital. Brody and I did many fun things together. Some of the things we did were, went to the beach, we also visited the aquarium and zoo. My favorite thing we did together was go to Seaworld.

Sea world was so much fun! We saw a dolphin show, a clown show and the Shamu show. There were tide pools and I got to touch a starfish and feel the gross sticky skin of a sea cucumber. My favorite thing we did was to ride on Atlantis. The ride was awesome because it was scary and thats what made it fun. When it was time to leave, we got to see fireworks that the park does before they close. I had a great summer and the best part was when my sister came home from the hospital."

The second half of the project was to create diorama, collage, mobile, or some other means of illustrating the story. When Reese suggested that he wanted to do a mobile like those that Rory is so fond of, Dad joked we could even motorize it. Apparently at 9 years old, kids still think their parents can do anything. I guess I'm not ready to let go of that perception just yet, so check out the video to see how it turned out.

Video quality isn't so great, but those are pictures of all the fun things he did hanging off the mobile. And at the top are fireworks. It was a family collaboration to be sure.

Latest and not so Greatest

Not much new to report these days. The information comes in slower and slower. We have extreme ups and downs, sometimes weekly, sometimes daily. As we continue to fumble through this mess, we're not sure where the end of the road lies. But we prod along, and self correct volumes, ratios, and feeds as we see fit in order to prevent Rory from distending to such a point that we end up back in the hospital. So far, so good. But our patience runs thin, and our hopeful expectations run low.

At Rory's last pediatrician visit, they told us she is showing signs of Left Sternocleidomastoid. "Oh no, what now!?!?!" you surely cried in outrage and frustration. Left SCM simply means that she has a tight neck muscle on her left side which causes her to tilt her head to the left. In severe cases (which she is not), a helmet-like contraption is required, along with daily sessions of stretching activities. Two separate stretches, five times a day, three reps each.

Instead of all that, we need to do the stretches twice daily, and see a physical therapist. Frequency unknown at this point. Because of this preferential tilt, she can also start to develop a mis-shapen skull from keeping her head in one position while on her back.

What next?

Around the World in Rory Days

Thank you, Anonymous Irish, for your comment in the post below. I'm sorry to hear about your daughter and wish our situation offered more conclusive results for others to draw upon. Admittedly, our docs are at a bit of a loss, but one thing the surgeon has said all along is that they don't want Rory's issue to be carried into her toddler years, where she would eventually start asking us why we have to rectally dilate her to make her go poop. We are very much still in the process of trying to figure it all out, but it is clear that if this process stalls, it will turn surgical. My memory wanes the longer we're out of the hospital, but I seem to recall what seemed like two diffent surgeries that were being considered. The first was simply the pull-through procedure similar to the Hirschsprung's 'easier' corrective surgery. The second was what seemed more like cutting an o-ring and sewing it back together, but instead of a piece or rubber, the o-ring is the sphincter muscle.

At this point, all I can offer with certainty is that Anonymous Irish is right to be investigating this on their own. I just wish this blog was not near the top of the google list, because it demonstrates how little information is available out there. But this advice is for anyone with a loved one in the hospital for any reason: do not assume that your doctors are completely familiar with, or knowledgable about your particular ailment. Question them at every turn, with questions based in fact and research. Make them think about their decisions and the long term ramifications of those decisions. And if you don't like what you're hearing, speak up about it. We have been very pleased with the doctor's we've had on our case, their communication, and their forthrightness about their limited knowledge. But I suspect our continued involvement and repeated badgering didn't hurt our situation either. If you are not pleased with your doctor, then it may be time to get a new doctor.

"I thought you'd be taller"

Do you know me? Do I seem shorter to you these days? The title here is a paraphrase from a totally underappreciated movie, 'Escape from New York', where the protagonist's reputation proceeds him so much, people assume he is larger than life. That has nothing to do with my current state of mind, but it makes for a catchy title.

When I pose the rhetorical question of whether I seem shorter to you, I'm not talking about physical stature. I'm talking about patience. I have found that when information really matters, when I need to cut through the superficial BS and niceties of daily interactions, I have no time to mince words. You too would find that when your newborn daughter's long term heath and welfare is on the line, there is no room for spared feelings, miscommunications, or misinterpretations. There is no room for hesitation in the decision making process. And it would seem that once you've crossed this metaphysical barrier, once you've crossed this line between casual conversation and critical communication, there's no turning back. So maybe now is a good time to apologize for any sharp quips, or quick tempered remarks you may have observed or recieved from me in recent weeks. In some ways, I fear the Eric you once knew no longer exists. But in other ways, I embrace it.

GI Doc, a Real American Hero

Today, we met with a nurse practioner from the Gastrointestinal (GI) doctor's office. She seemed nice, and already up to speed on Rory's situation, which added to my comfort level regarding this GI group. Plus, they'll fight for freedom wherever there's trouble, so that's a bonus. Inspection of Rory revealed that all looks good so far. The past week at home with her parents alone on staff does not appear to have worsened her condition.

http://www.youtube.com/watch?v=gc8mVs2H4Vc&feature=youtube_gdata_player

We've got the direction we were looking for. GI Doc is having us mix MBM (that's mother's breast milk) with the liquid gold formula. For now, it'll be a mix of 30 ml MBM and 60 ml liquid gold. We'll increase the percentage of MBM every few days, as well as increase the total volume gradually until we eventually reach 120 ml. This is great news, not only because of the cost of that formula, but because we are up to our ears in MBM, quite literally. Needless to say, I did not own a chest freezer before the NICU visit.

The specific instructions are a little more loosey-goosey, allowing some room for our own interpretation of Rory's response and correction on the fly. GI Doc said we don't have to be pedantic about feeding her every three hours during the night. We can let her sleep until she wakes on her own and feed her then.

We'll be proceeding with the manometry study. Think 'drug mule', but instead of heroine, the balloon is filled with pressurized air. For my colleagues in the membrane industry, a better analogy would be to think about a pressure decay test for a hollow fiber membrane. If you didn't get that second reference, fret not, it probably just means you don't appreciate the Dilbert comic strip either (ie: Engi-nerd humor).

Currently awaiting referral to CHOC, Children's Hospital Orange County. They are the regional experts in applying and interpreting the manometry test for infants, but we need to get approval from the insurance company because CHOC is out of network. But the idea here is that if Rory's status goes south quickly, the docs would like to have that piece of information already in our back pocket, and not have to delay to get it. If we are forced into making a quick decision about an ileostomy, at least we can then make a more informed decision with this knowledge already in hand. And hopefully we won't even need these results.

Other than that, not much more to report. Rory continues to do well. We are less freaked out by every squirm, grunt, or wince that Rory makes. We continue to monitor her diligently, and each day pray that she is closer to outgrowing this issue all on her own.

I'm not a moron, but I play one in real life.

Did I ever mention I have a college degree? Really, I do. And my degree is in a pretty hard major from a fairly prestigous school. I'm not bragging here, but after reading some of these posts, you may have come to the conclusion that I'm a moron. We all have ridiculous stories to tell, most just never bother to write them down, let alone post them on the world wide web for all to critique. Based on the last sentence, maybe I am a moron.

Plaaaaayyyy Ball!!!!!

I've got a backlog of stuff that occurred while we were at the NICU, so here's a fun story. One Saturday, some generous person donated Padre tickets to the RMH. We took the opportunity of free tickets to get out and go see a baseball game. It was a much needed break, and gave us a chance to spend some time with our other two kids on a family expedition. Even though Rady Children's hospital is only 12 minutes from Petco Park by car, we opted to take public transit down to the game just so the kids could ride the trolley. What great parents we are, right?

Now, I'd never ridden the San Diego trolley before, but I have used electronic ticket machines elsewhere in the world, such as on the BART in Northern California, and even the City trains in both Brisbane and Sydney, Australia.  I've even purchased movie tickets from a machine, and use ATM's quite regularly with much success. In fact, I fancy myself quite computer and gadget literate.

So I'm not exactly sure what happened here, it seems easy enough to purchase four, one-day fares on the trolley. But somewhere between putting my card in the slot, and pulling out my tickets, I purchased one, four-day fare. Had I known that nowhere between the points of origin and destination would anyone actually check my tickets, I might have just left it at that. But I figured I'd better do the right thing here since my kids were staring at me asking why I was getting so angry. I went ahead and purchased three one-day fares to go with my one four-day fare. We got some free tickets to the Padres game, and the San Diego MTS made a little extra profit off of it. Oh yeah, and the Padres lost 6-10 to the Colorado Rockies.

"Hey kids, let's go bust some drunks!"

I'm no proponent of drunk driving, but should I have to explain what DWI means to my eight year old just because we stopped in to Rite-Aid for an ice cream cone?

Where's the Homicide Department's Meat Wagon so I'll be forced to explain how messed up the world really is. I don't want my kids to grow up naive, but I sure don't want them to grow up before their time either. Thanks for the ice cream and the life lessons, Rite-Aid.

A Vote of Confidence

My wife is on the phone right now. Literally, right now as I type this. When asked if she has a hard time leaving Rory to go run errands, she responds "no, Eric can take ver...[pause] Eric can take care of her". How about you finish that first part? Allow me, "Eric can take very good care of her". Thanks for the vote of confidence, honey.

[UPDATE] Christine claims her pause and rework of the thought was unintentional and purely coincidental. A natural artifact of the discourse between two human beings. Hmmm, sounds suspect.

Feisty Blog Commenters!

I applaud your passion. But let's all take a deep breath here, before the comments section on this blog becomes like those following the Yahoo! News stories. Seriously, have you read the comments at the end of those stories, especially political stories? They are downright offensive. Dont those people have anything to do all day? But admittedly, they are often more entertaining than the story itself.

So keep it clean, people. Calm down. I don't want to have to start moderating your comments now. Those that know me, know I don't like moderation in anything I do. Wait, no, that didn't come out right...

I'm Getting Desperate

I tried feigning Narcolepsy tonight but Christine wasn't having it. Something about not having it for the past 17 years. Who is she, Sherlock Holmes? Anyway, a word of advice for you younger, single guys out there. Start faking this sleeping disorder now, it will come in handy later. And not just when you have to give late night feeds to the baby. You can have an 'episode' right in the middle of your wife telling you about her day, or better yet, when she's asking you to take out the trash. The possibilities are limitless! But start early and stick with it or it will never pan out. That right there is good, solid marital advice - on the house.

Hello? Hellllllooooooooo!?!?!?!

"Are you still there? Is it over? Is this blog finished?"

C'mon people, I wouldn't leave you hanging like Jerry Seinfeld did with that sorry excuse for a series finale. I've just been busy. It turns out having a 9 week old baby at home requires a lot of time! Where's our stable of nurses with around-the-clock coverage? Where's the team of NICU doctors and pediatric surgeons, ready to invoice me diagnose Rory's every hiccup or burp at a moment's notice? Apparently, they don't come home with you!

I'm not complaining. And the invoice crack above was just a joke. It's great to be home...with Rory...intact. But it does require a lot of work.

Can I digress here for a moment and tell you what an absolute advocate I have become for breast feeding? I mean, I'm not going to get all militant about it, but I get it. I know the advantages it offers. I know the benefits of breast feeding from when our first two kids were born. You've probably seen the same studies I have documenting this fact - dads get something like 90% more sleep when moms breastfeed. So keep up the good work, ladies. It's apparently good for the baby too...wink, wink.

But none of that for us right now. We're still on the gold-standard equivalent of baby formula. Did I mention previously that we anticipated using six cans per month? We went through the first can in 4 days. By my math, $45/can x 7.5 cans/month = chapter 13. Financial ruin and I get to share in the late night feed schedule? I thought the US legal system prevented this type of double jeopardy.

Rory had an appointment with her pediatrician on Monday. She also had an appointment with the surgeon today. We were supposed to see the GI on Friday, but they bumped us until next Tuesday. The surgeon was pleased with Rory's response to the dilations, as well as the several unscheduled stools she's had since leaving the hospital. But he's only keeping an ear to the situation in the event he gets called up to the plate. The GI will be the primary investigator from this point forward, so we eagerly await our first meeting with her.

Bright Light, Brighter Future.

When we hit street level, the cloudy sunlight was too much for her eyes after 46 days under the fluorescents of the NICU. Little Rory squinted like a Chilean miner emerging from the depths of the earth after 17 days underground, sans Oakley eyewear. Where's our endorsement deal, Oakley?

Last Feed in the NICU

Mommy giving Rory her last feed in the NICU before we hit the road.

Don't Hold the Mayo...

In fact, slather it on! Everybody has heard of the Mayo Clinic, but can anyone tell me why they're famous? Anyone? From you know where:

"Mayo Clinic specializes in hard-to-treat diseases, and is known for innovative and effective treatments for diseases that had gone undiagnosed or under-treated in the same patients with other doctors. Mayo Clinic is known for being at the top of most accredited quality standard listings."

You'll recall that Rory's blood samples were sent to the Mayo Clinic earlier this week to confirm the Lymphocytic Ganglionitis reported in the pathology results. LG is a rare auto-immune problem where your own white blood cells attack the ganglion cells in your gut, thinking that they are foreign invaders. In the worst case scenario, the impacted portion of your gut is rendered useless.

Apparently, word of Rory's popularity in the medical community traveled along with her sample, because what we thought would be weeks for the results was just a few days. The Mayo Clinic has given her a clean bill of health, no presence of Lymphocytic Ganglionitis!

My, How Times Change

In my younger days, I'd carry coolers of beer. Now, the coolers have different contents.

She's Outta There!

We've been discharged today. This isn't the only good news, more on that later.

Tuesday Stoolsday

Rory stooled all on her own again today. Still on track to be home by the weekend.

I wish everything was fixed this quickly

If your car's not running right, or you just need somebody to correct your own mistakes, give Jorge at JF Performance a call. He took care of us quickly, efficiently, and honestly. Knowing an honest mechanic when you own a 12 year old car is like having a pediatric surgeon on speed-dial when your baby has Internal Anal Sphincter Achalasia. Thanks Jorge.

A Light at the End of the Tunnel

We are definitely seeing a light at the end of the tunnel. But we also know there's a bridge and perhaps another entirely new tunnel on the path ahead. Let me be clear here in order to avoid any confusion. The end of this tunnel represents an exit from the NICU and getting home. It does not represent a cure or healing of whatever it is that ails Rory, the exact identity of which is still very much up in the air. It just means we've reached a level of stability and normalcy that will allow us to continue the investigation from the comfort of our own home, with frequent visits back to the doctor.

Rory hit the 75 ml target feeds today. With the progress Rory has made, and pending no distention or other change in her status, we could be discharged by Thursday or Friday of this week. Meanwhile, the labs pulled this morning are being sent off to the Mayo Cinic for analysis. Those results will probably take quite a while. For those not in the know, 'labs' is hipster hospital speak for blood samples.

With regard to going home, we've learned not to get too excited by the wispers of good news, but we think this could be real.

Wheel You Give Me A Brake?

Continuing the saga of my own personal guffaws, here's another tragicomedy that Shakespeare himself could not have written. A few weeks back, Christine's car was making a grinding noise, so I jumped to the obvious conclusion and planned to replace the brake pads. When I pulled the tires off, the old pads still looked great. So I replaced the pads anyway, buttoned up the car, and figured that if the noise came back, I'd deal with it then. And then we went to the NICU. Since we've been at the RMH, the sound continued to worsen. One of my tasks to do while at home for the past 36 hours was to try to figure out where that sound was coming from, and what to do about it. I had a short window of time to work in, so the pressure was on.

This morning, I removed the left, front tire to get a better look, as I'm pretty sure it's probably a bearing issue. Somehow, during the entire process, I managed to break not one, but two lug nut studs. Seriously, how did that happen? I'm too old to be snapping 3/8" bolts with my own two hands! I'm left to assume that these lug nut studs were already compromised, and we are probably fortunate they didn't fall apart on the road. That's what I'm telling the wife anyway.

Any other week, this would have been a minor inconvenience. But this week it's a real headache. I have to get the car into the shop tomorrow, probably for a few days. I'll need to rent a car for the time being so I can get to work and Christine can get back to the hospital. A dung beetle could not have rolled a nicer turd-ball than this. Honestly, I cannot help but laugh.

A Very Sod Story

That's no typo. This story is about sod...grass...lawn. This summer, I finally got around to replacing the forest of weeds that had taken up residence in my backyard. I spent many hours weeding, leveling, and generally prepping the backyard for sod to be laid down. On Friday, June 24 I had the sod delivered and spent roughly 11 hours single-handedly laying the entire backyard. The following Sunday, I was working in the front yard when the sprinklers turned on. I lept up, shut the sprinklers off, and continued my task. Monday night Rory went into the ER, and subsequently the NICU. That first week in the NICU, our friends reported the newly laid sod was turning brown in patches and not doing too good, despite hand watering of the affected areas.

By Thursday, it was realized that the sprinklers were not on. I'd never turned them back on the previous Sunday, and none of us were around to notice that they weren't coming on at the appropriate times. Given my personal investment of time, energy and money into that backyard, I wasn't about to let that lawn die on my watch. Next time I was home, I cranked the sprinkler times WAY up, then didn't return home for another two or three weeks. I just got my $350 water bill in the mail.

The Laughable Loser

I like to think I'm on my game most of the time, but then again, don't we all. I now have two ridiculous stories completely unrelated, but occurring during, or perhaps because of Rory's time in the NICU. If nothing else, they offer some levity during this time, and honestly the outcomes of these stories are so absurd that given Rory's situation, if I don't laugh, I'm liable to cry.

Progression...

"Guess what daddy? I pooped a big poop on my own"

I went home on Saturday to collect up the other two kids, let them sleep in their own beds for a couple of nights, and perform some errands (well, I didn't collect them up so much as they were delivered, thanks guys). I received the above text and pic from Christine today, who stayed behind an extra day for Rory. She'll be home Sunday night, then return to Rady Children's and RMH with the kids on Monday.

So that's huge news! She pooped, a big poop, all on her own, without the dilation. This is very promising progress. Very good news at a time when we needed some good news. I've been reading up on those two issues, mostly medical journals and abstracts where the doctors refuse to use common English, probably in an attempt to fool the reader into believing that the authors know what they're talking about (I do the same thing, but don't tell my clients). You have to constantly reference a medical dictionary just to keep up, but it's manageable once you've spent 7 or 8 hours doing so. Those two issues, IASA and Lymphocytic Ganglionitis are not good news, not even close. The latter gives me more concern than the former, probably because there's not much information out there, the information that is available seems to call it by many different names, and there are some grim case studies.

Tomorrow Rory will have some blood work done in an effort to confirm the Lymphocytic Ganglionitis finding by the pathologist. I'm hopeful that the pathologist was high, and in a rush to get out of the lab because he was already late to a Nirvana-tribute concert. But the blood work means Rory won't feed for 12 hours before the test. Her unusually happy demeanor is sure to change tonight.

Ouch.....

$45 per can. 6 cans per month. Insurance doesn't cover it because it's available over the counter. I am withholding several comments that come to mind.

Seriously?!?!

Six weeks in, we realize the chair reclines.

Paging Dr. Mother, paging Dr. Mother!

Christine performing her first rectal dilation. The bond between mother and child knows no bounds.

Internal Anal Sphincter Achalasia & Lymphocytic Ganglionitis

These are not fledgling nations newly recognized by the UN, nor archeological marvels in Egypt, nor are these words written in a foreign language. Theses are two seperate and distinct medical problems that have been attributed to Rory's condition.

If a taxi driver tells you he doesn't know how to find your destination, you'd be annoyed. If a mechanic tells you he can't figure out why your car engine makes that knocking noise, you might be frustrated. If your lawyer tells you he doesn't know how to defend your case, you'd be concerned. But when the doctors tell you that the only diagnosis they have for your eight week old daughter is a term that generically represents a class of problems so rare that they've never heard of it, you can't help but be scared. We are currently 'all of the above'.

We met with Dr. Fairbanks (Surgeon) and Dr. Le (Neonatologist) today to discuss the situation and path ahead. Dr. Fairbanks has had a little more time to think about, and further research the situation (I wonder if he uses Wikipedia?). Here is a synopsis of the discussion:

*It turns out, there was in fact one hit on the biopsy results. The specialist in Seattle that was sent a portion of Rory’s upper colon found what they call lymphocytic ganglionitis. As near as I understand so far, this is when the lymphocytes (white blood cells in the immune system) attack the ganglion cells, causing an inflammation in the colon. This is probably completely wrong, but there's not much immediate or obvious literature available through google, so cut me some slack. My beloved Wikipedia has failed me and remains completely silent on the issue. This one's going to take some digging to understand. Did I mention this stuff is rare? But this biopsy sample came from the transverse colon (see pic in previous post), which is up high and pretty far from the rectum where there are clearly other issues going on.

*The docs are also looking at Internal Anal Sphincter Achalasia. This is basically the same as ultrashort Hirschsprung's disease, but with the presence of ganglion cells. The sphincter muscle just doesn't function properly. So one fairly new treatment process includes injecting Botox to relax the muscle. It's not clear, but this could have a long term corrective effect, or could offer only short lived, temporary improvement. If the former, great! If the latter, well, you can't keep doing this every few weeks or months for the patients entire life, no matter what aging socialites might suggest. So the more historical corrective action is ISM, or Internal Sphincter Myectomy. They cut the sphincter muscle. This generally corrects the problem, but can leave patients with incomplete bowel control, or 'spotting'. A very awkward condition to carry with you into your teen years.

The plan is to continue on with Rory's incrementally increasing feeds, until she hits that 70 ml target. Then continue to feed her 70 ml for each feed, all the while applying rectal dilations. If she continues to respond appropriately to the rectal dilations, without distention of the bowel, we'll go home in about two weeks with a plan for feeds and dilations at home. But we can't do that forever, and the docs are still thinking over the Botox, ISM, or ileostomy for the IASA. Simultaneously, they are talking to the GI doctors to figure out a treatment for the Lymphocytic Ganglionitis, and we'll soon be getting to know those folks very well.

So where does that leave us? We've got a baby that otherwise seems very normal and unusually happy all the time, a stressed and frustrated mother that will kick, scratch, and bite before they can drag her out of the RMH or NICU, two siblings that have spent their entire summer vacation on extended sleepovers between friends' houses and the RMH, and a dad with a blog.

Whhhheeeeeeeee!!!!!!

Strap the kids in, it's time for another ride on the emotional roller coaster that is our life in the NICU. Got a message from the wife this afternoon that indicated we were being moved to the 'intermediate care unit'. That certainly sounds promising given that 'intermediate' implies our child is in better condition than 'intensive' suggests. However, we were none too happy about it given the fact that along with the new location comes a host of new doctors. And we now know from experience, when a new doctor comes on the scene, they bring their own little black bag of tricks with them. They have their own opinions about your child's care and how to manage it. They have their own preferences for how things get done. They have the power, and control to take the treatment process an entirely different direction from what's already been established and planned. So we voiced this concern. And maybe we bought ourselves a few more days in the NICU, remaining under the watchful eyes of our previously established and somewhat consistent team for the time being.

Administration drama aside, Rory's path ahead is still very much in flux. ALL BIOPSY RESULTS CAME BACK NEGATIVE. Negative, meaning that she does not have any of the diseases they were searching for with those particular tests. So, the great results we've been getting with the rectal dilations have overshadowed everything else and have become the basis and focus of the treatment process. I was able to witness these results today - yay.

There was brief talk about sending Rory up to Children's Hospital Orange County (CHOC) because they are the only regional facility that performs anal-rectal manometry on babies. You'll recall from your high school chemistry class that a manometer measures pressure (those of you that stayed awake). This test uses a ballon-like object inserted at different depths to measure her ability to 'push' on the contents of the colon. But this won't likely happen because they've gained significant information from her response to the rectal dilation. Thank God, I don't think I could bear an invoice from a third hospital in less than two months, especially one that's Out-of-Network. A man has his limits!

Rory's response to the rectal stimulation reveals three important points:

1. She can stool (we already knew this, but it's reassuring)
2. They can feel her rectum pushing back against the probe, which confirms some level of proper function, and minimizes the information to be gained by the manometry.
3. Whatever the cause of the restriction, it must be within 0-3 cm of the anus (the length of the probe), because the stimulation causes a successful stool.

So it would seem we are at a crossroads and will have some decisions to make about the path ahead. We have a meeting scheduled with the NICU doctor and the surgeon tomorrow morning. Basically, our options boil down to the following:

1. Continue feeds as-is, eventually going home with a plan that consists of rectal dilations at home, and weekly (or more) doctor's visits to monitor progress and buy some time while continuing to investigate the issue. This may sound simple enough, but it could drag on a while, and apparently high school students have an aversion to parents trying to implement this treatment process.
2. The symptoms and responses to treatment that Rory has presented is consistent with...you guessed it, Hirschsprung's disease! (Docs love to use the word 'presented' to describe all of the evidence that they've accumulated) So, if it walks like a duck, if it quacks like a duck, and if it stools like a duck, then treat it like a duck. No, not blast it out of the sky with a 12 gauge shotgun. But rather, perform the pull-through procedure the same way they would if they had a biopsy result that confirmed Hirschsprung's. The thinking is that this would correct the issue that appears to be located within that 0-3 cm range, whatever the issue is. 
3. The ileostomy option. Don't like this one, but it's still on the table.

So there you have it. If you've read this entire post, you are as up to date as we are at 9:00 PM on Wednesday night. Oh yeah, and if you're my boss, I'll be in a little late on Thursday.

Just Because...

And here's a pic of Rory, just because.

Hershey Squirts and Chocolate Kisses!

“How dare you sir, how dare you! There’s no place for such implications in a cultured and academic setting as this! You are a gentleman, are you not?” As my wife and most of the people I know will attest, I am not. Plus, the title just rolls off the tongue, doesn't it? Ewww - too far?

Despite the potential protest from our more refined readership, the lowbrow title stays as a tribute to Rory’s past few days here in the NICU. Rory’s rectal dilations appear to working (or rectal dilatations if you are overly educated or simply pretentious). She has had several successful stools following the dilation procedure. For the less imaginative, I will describe the procedure as being similar to taking a baby’s temperature, the old fashioned way. Today, Dr. Fairbanks (aka Col. Hannibal Smith) performed the procedure himself, and the results were reported to me as being massively successful. And stink-ily successful too.

So that’s all good news, more good news in a string of goods news. In fact, every test, every result, every finding we’ve had since we arrived here 36 nights ago has shown Rory to be very normal. I’m starting to think the doctors’ and nurses’ affinity for our daughter has brought about a conspiracy to find new and inventive ways to keep her here. Not really, but wouldn’t that make for a more interesting story?

Rory is up to 38 ml every 3 hours in her feeding regime. For the metric-ly challenged, 38 milliliters equates to 1.28 US fluid ounces (or 0.08 gpd for my fellow engi-nerds). The plan is still to get her up into the range of 66-70 ml per feed, but we’ll see if the doctors can approach that goal patiently. I won’t be surprised if they get too excited and start accelerating her feeds. But right now, the goal is to develop a plan that will get us back home as a family unit. The plan will consist of intentional feeds, rectal dilations, and frequent doctors’ visits – from home. In case you missed it, the key word is ‘home’.

Optimus Primary

Day 35 in the Rory saga. Our heroine remains in the NICU, where she faces her biggest adversary yet, the bureaucratic health care system itself. Don’t misunderstand; we feel the standard of care we have received at Rady Children’s Hospital has been unquestionably excellent. But we have noticed a steady stream of new nurses over the last few weeks, sometimes for a single shift only. This weekend, we had four completely new nurses (who were all great by the way). But we have also received questions similar in nature from several sources: “Who is Rory’s primary?” So we put two and two together, and now understand that it is not uncommon for specific nurses to be assigned to specific patients for the duration of their stay.

The concept of a primary nurse sounds pretty darn simple and logical once you say it out loud. With a primary nurse, they wouldn’t have to give the entire 35 day history to a completely unfamiliar nurse at each shift (every 12 hours), just an update since the last time they were on duty. A nurse that is intimately familiar with a patient is more likely to see the signs of change in a patient, especially if that patient cannot speak. A nurse that is intimately familiar with a patient is more invested, and can work as an advocate for that patient, especially when there is also a steady stream of doctors and nurse practitioners interjecting their big ideas into the treatment process. A nurse that is intimately familiar with a patient’s history is more likely to prevent both minor missteps and major catastrophes alike. A primary nurse can act as a lighthouse; providing guidance, direction and translation to parents desperately trying to navigate their child to safety. So yeah, the concept seems like a good idea.

When we have known that a particular nurse would be on the shift, we have requested they be assigned to Rory. We have also been told by several nurses that they have requested Rory for their shifts. But since none of these requests ever get filled, we are left to wonder if the only thing a request gets you is somebody else assigned to that shift. We were a little shocked to hear about this concept of a primary nurse, and have requested a meeting with the social worker (which, ironically, they assign as soon as you step into the building) to see what can be done. More on this as it unfolds…

Sugar and Spice, and Rectal Dilations

A couple of days ago Rory started on feeds of Alimentum formula. They started at 5 milliliters every three hours, then ratcheted up by 3 ml every 12 hours. She's currently at 14 ml. She'll max out at 66 ml and hold there. Math says that's another eight and a half days of this. Another eight and a half days here, at the very least. Math is honest, that must be why nobody likes him.

Her stomach girth has been looking good, and shows no sign of increase. The last x-ray I heard about showed a hint of distention, but nothing too worrisome yet. They're performing enemas again, every 12 hours. At least twice, she had tiny stools after the enemas. This morning, they dilated her rectum and she stooled. It doesn't sound like there's much technology or science behind the dilation process, so the image your mind conjures is probably accurate.

Still no word from the pathologists, maybe they can't find anything wrong. I'm okay with that. We have pretty much run out of 'good' diseases anyway. So maybe it's just a case of a lazy bowel, you know these kids today have no work ethic.

The Quiet Times

It's quiet tonight. Lights are turned down low. Babies are all asleep. So is Rory, resting confidently in her fathers arms. His back is to the large, sliding glass door that represents all she knows about the outside world. Occasionally he hears a short-lived whimper down the hall, or the faint, but constant cluck of a monitor needing to be hushed. Nurses shuffle about behind him. His focus turns to her breathing...baby breathing. The sound brings peace as he feels the rhythmic rise and fall of her chest. Her toes flinch and curl in unison. The child has been in the NICU for 5 full weeks, with no firm answers in sight.

His mind wanders and he dares to consider the situation. He thinks about the present. He worries about her future. He wonders whether she will leave this building unscathed. He doubts that he will.

What Luck!

I can't believe my luck! The timing of this windfall is unbelievably fortunate, as I can certainly use $850,000 right now (I could use $850,000 anytime!). Don't get any ideas, I've blacked out the email address to prevent anyone from trying to steal away my opportunity. Ha Ha, too bad for you!

Modern Day Florence Nightingale's

If I say Kleenex, you'll think of 'tissue'. If I say Ajax, you'll think of 'scouring powder'. If I say Victaulic, you'll think of 'stainless steel pipe couplings' (well, maybe a small portion of you will). But if I say Florence Nightingale, you'll definitely think of Nurses! So who was this woman whose brand name has become synonymous with nurses everywhere?

Florence Nightingale was the pioneer of modern nursing. Normally I would recap what I just read on Wikipedia (I have a triple major in Wikimedicine, Wikihistory, and Wikiplagiarism), but I'm currently holding Rory with one arm and typing with my left thumb. So here's a link to this amazing lady.
http://en.wikipedia.org/wiki/Florence_Nightingale

My guess is that we've had about three dozen nurses since we've been in the NICU. And here are just a few of the modern day Florence Nightingale's we've had the distinct pleasure of meeting while in the NICU.

Nurse McLinda! You've already met her, she was Rory's day nurse on the day of her surgery.

Nurse Alex - She was Rory's night nurse the night before and after surgery.

Nurse Caitlyn - She has had many adventures with Rory (or misadventures, remember the bounce seat with Christine?) She also braided Rory a cute little head band.

Sisterhood of the Traveling Samples

I know my kids are going places in life (me and every other parent, right?). But our little Rory is already on her way! Pieces of our little starlet have apparently traveled all over the country for pathology tests by various specialists. At least one report suggests a sample has gone as far as Seattle, Washington! Can you imagine – Seattle, Washington?!?! Talk about living the cellular dream!

I’ve always wondered what the cells are like in Seattle, Washington. Do they look like ours do? Do they function like ours do? Dare I ask, do they multiply like our cells do? Maybe they wear flannel shirts and listen to grunge rock.

We’ll have no final report until our sisterhood of traveling samples return with results.

Like sands through the hourglass...

So are the Days of Our Lives. Our personal soap opera continues, with all the ups and downs, dramatic pauses, and ridiculous cliffhangers that monopolized daytime television during the summers of my youth.

Season 1: "Our heroine was admitted to the intensive care unit for a mysterious ailment. Doctors struggle to find a diagnosis, but are stymied at every turn. Nurse Caitlin is playing matchmaker between Rory and the patient next door, but Rory's overbearing father will have none of it. Rory's loving mother, consoled only by the handsomeness of her youthful husband, waits patiently for an answer. In the season finale, Rory anxiously awaits the results of multiple biopsies. The once kindly RMH threatens to toss her family out into the cold, cruel world because of policy changes. An overworked doctor rushes into the room to announce..." Dramatic pause, fade to black, and cut. That's a wrap people.

Season 2: "The overworked doctor rushes into the room to announce...[dramatic pause] 'oh, we're still waiting on those results, probably get them tomorrow, maybe the next day. Could even be by Friday. ' "

In the meantime, the docs have Rory starting feeds again, this time starting with 5ml of pediolyte, and then moving to small amounts of a special formula, the name of which eludes me right now. Babymakepoopyilac or something like that. And then monitor. Supposedly, THIS time they will proceed more slowly with the progression of the feeds, AND this approach won't impact the path forward once the biopsy results are available.

Pardon me if I appear skeptical, but I feel like I've seen this episode before, and I grow weary of the entire show.

Eric "Likes This"

If you didn't know already, or haven't figured it out by the tone and content of this blog, I'm a man. I'm an old-fashioned guy that adheres pretty strictly to Rev. 0 of the guy code. That's Rev. 0, the original version, not that touchy-feely, emotional stuff that 'men' use to define themselves today. As a result, I understand and appreciate the pat on the shoulder, followed by the awkward silence when words fail to express the obvious concern and care.

But I am amazed by what I have observed since Rory was born, and especially during this time. My family has been the recipient of an unimaginable outpouring of love and support. An outpouring so overwhelming that I literally have difficulty processing it in its entirety.

To all of you that have thought of us, prayed for us, stopped by to see us, provided meals, or otherwise supported us in our time of need: thank-you so very much. Thank you to the management at SPI for your understanding during this time. An additional thank-you is extended to the Ramaha's and the Brandon's for watching our home front while we've been camped out at the RMH all these weeks. And a very special thank-you to the Davis' and the Knudsen's, who have been caring for Reese and Riley so often and for so long, allowing us to focus on Rory and her needs. These debts can never be repaid, and we are truly appreciative. I am truly appreciative.

Nothing New

No news related to the biopsy results just yet. Christine and I are heading home to Temecula tonight to spend time with Reese and Riley, and they will all return to RMH and the NICU in the morning. Perhaps tomorrow...

Day 29

Today is the 29th day Rory will be in the NICU, or as I like to call it, the start of Week 5. This week promises to be filled with answers. Of course, each week prior has also promised answers, but everybody knows Weeks 1-4 are dirty liars.

Just waiting for the Pathologist to finalize her report, provide it to the doctors, and then have them tell us about it. Preliminary results suggested that Rory does not have ultrashort Hirschsprung's disease, but she could still perhaps have an even rarer form of Hirschsprung's disease. We're three tiers deep on the 'Rare' scale, so things aren't going so good. Hirschsprung's disease is rare. Ultrashort Hirschsprung's disease is rarER. This next one is rarEST? If I ever see Dr. Herald Hirschsprung on the street, I'm going to give him a piece of my mind (but that probably won't happen since he died in 1916).

So, we're still waiting to hear about the other things too. Meanwhile, other than all the tubes sticking out of her, Rory gives no hint that she's sick, uncomfortable, or that she has any issues at all.

The Faces of Rory

Yoda - "Use the force, you must, hmmm"

Cognitive - "Let me consider your argument"

Sneaky - "Shhhh, I have a secret."

Dramatic - "Oh my stars, I'm getting the vapors"

Baby Dreamers

Do babies dream? And if they do, what do they dream about? These are questions I've asked myself with each of my children. When you hold a baby, even a brand new baby, you'll note that they twitch, and wince, and roll their eyes under their lids. Their brains are clearly firing, but is it a dream, as you and I know dreams? Or is it the same level of limited cognitive thought they experience when they are awake - "I'm hungry"?

Maybe they dream of the future. Maybe they dream of that day when they will finally turn one year old and be able to get out from under the oppressive thumb of their parents. Maybe they dream of the day when they can rely on their own two feet to shuttle them where ever they want to go. Maybe they dream of all the foreign objects they could shove in their mouth if they were only a little bit closer and within reach.

Maybe they dream about being WWII paratroopers.

The Beaut is a Brute!

Doctors indicated it can take 24 hours for babies to wake up from their slumber after surgery. Our little beaut Rory is proving to be quite the brute, powering through anaesthesia, opening her eyes, being pretty alert and mellow, all within just a few hours. This little girl is like a freight train. Nothing's going to get in her way or slow her down as she barrels ahead on the tracks of life.

Papa & Nana visit

Papa and Nana were waiting outside during the surgery. They'll miss out on holding Rory so she can recover (nurse McLinda's orders!), but they were in the room when the docs pulled the breathing tube (with a little help from Rory). Rory's back to breathing on her own, almost awake now.

She's Out!

Rory's out of surgery, and still 'out' from the anesthesia. She's intubated for the time being, allowing technology to breathe for her until she's detoxed off the drugs.

Col. Hannibal Smith (aka Dr. Tim Fairbanks) went in rectally and pulled a 2 cm oval sliver from her rectum to biopsy for ultrashort Hirschsprung's disease.

He also performed a laparoscopic procedure consisting of three small incisions - one in the belly button, and two on either side of that. Through these holes, he was able to look around with a camera to see that everything looked very normal. He also collected two more samples for biopsy. These will be tested for one other disease specifically, as well as a grab-bag category of 'other'.

Hannibal removed Rory's appendix, because that's what docs do now if they have a reason to be inside the belly of a kid.

All in all, a very successful procedure. Rory is already starting to come around. No obvious answers were found, so we're back to our waiting game, this time waiting for biopsy results.

Two weeks ago, we were hoping it would NOT be Hirschsprung's disease. Today we are praying that it IS.

Waiting...

"Parents, party of two, your daughter is ready"

The only link we have to Rory right now.

She's In.

"If you have a problem, if no one else can help, and if you can find them, maybe you can hire the A-Team."

Rory is in the operating room. The A-team has taken our case.