Simon had his post-op appointment with his palate surgeon today (and the craniofacial department added a post-op for the three-months-ago ear tube surgery as well as an audiology assessment). To be fair, we originally had a more timely post-ear-tube post-op appointment scheduled, but had to cancel when the boy woke up with a fever that morning.

So basically, Simon is doing awesome. Also, he is so clever.

1. Simon’s hearing is great.
2. Simon’s ears are clear of fluid. (Also, although he hates having his ears messed with, he let the doctor look in one side without complaining. The other side…not so much, but I’ll take what I can get.)
3. Simon’s palate is healing well.

Today was a crazy day at the craniofacial clinic. I’ve never seen so many kids in the waiting room, and both surgeons were running late. The first one, by only about thirty minutes, but the second one for over an hour. Clever mother that I am, I had brought no books or toys in the diaper bag.

The waiting room at the hospital has more books and toys than most pediatric places I’ve been, and the toys are great — large cube-like things, about two feet on a side, with various non-removable things on each face for babies to grab. Simon was pulling up to kneel a lot on the cubes, and did a little crawling around the waiting area. I wanted to tell everyone to look at my baby – HE CAN CRAWL NOW. Did I mention he can crawl now?

The books in the lobby: not so much. I can only read board books to Simon in public, since he always wants to rip the pages out of paper books. The only board book I could find was about kids dressing up for a costume party, then going to the costume party and enjoying looking at everyone’s costumes while eating snacks. Seemed kind of banal but tolerable given the situation, except the boys were all dressed as cowboys, aliens, and superheroes, and the girls were butterflies and princesses, and while I’m not that kind of feminist, it made me throw up in my mouth a little. But anyway.

We ended up playing a lot with the mama’s new iphone. Simon can now take pictures of himself (he knows where to touch the screen to capture an image), and when I go to the “photos” section, he can scroll back and forth to look at the different images. He likes photos of himself the best.

The exam room where we waited (for over an hour) at the end of the day had this wonderful toy (we’ve played with it before) with buttons for all the letters and numbers, as well as some shapes, some musical notes, and some basic tunes. It has different modes so you can just press the buttons and hear the name of the thing you pushed, or you can get more advanced and put it in quiz mode, or play a tune of your choice on the numbers one through ten. So Simon likes it because it has buttons, and Todd and I like it because we can make it say funny things:

• I F 2 P (I have to pee)
• S H L O (It’s a cello)
• U 10 Square A Circle Q E D (You can square a circle! QED!)

It’s also good at proofs. QED.

Simon and I have both had more than an average number of medical appointments in the last eighteen months. Between pre-natal visits, gastroenterology, well-baby checks, and the cleft-palate team, I would say we’ve seen our fair share of medical professionals. Many times (but not all — we have some fantastic providers), I leave an appointment feeling vaguely irritated, but have had trouble putting my finger on why. It’s taken me eighteen months to figure out that it’s because one of the four following rules has been broken. (But now that I know why things bug me, I can let them go more easily, at least in theory.)

1. Introduce yourself.
It takes about five seconds, tops. It’s something our culture thinks is polite. Are you going to touch me or my child? When all is going well, only people I know do that, so it would be nice if I knew your name.

2. Read the chart.
It will save me from having to repeat myself. It will make you look like a genius with a really good memory. It says “I care.” Asking someone for information on their chart looks lazy.

3. Take two minutes to determine how smart I am.
This will keep you from wasting your time explaining things I already know or things I have no chance of understanding. A good way to do this is to ask what I do professionally. From this you can discern my general level of education and my familiarity with topics we are going to discuss. High school science teacher? Can probably read graphs and very likely knows some basic anatomy vocabulary.

4. I should leave feeling as though I’m doing something right.
Because if all I get is messages about how I need to change what I’m doing, it’s depressing, and doesn’t empower me to change anything. Likewise, leaving with a list of things I need to do (say, for my child) is overwhelming if I don’t feel like what I’ve been doing is adequate. Now, maybe it hasn’t been, but if you can find one tiny thing to affirm, I have somewhere to start.

The next step is learning how to firmly and courteously assert myself when I get irritated. Saying “I’m sorry, I didn’t catch your name…” or “Hmm, I think that’s in my chart,” in my head is one thing, but actually saying it to a person is quite another.

Apologies to the grandmas, from whose newsy e-mail I’m taking the majority of this post…but c’mon grandmas, you know you want to read it twice.

We met with Dr. Sleep today to follow up on Simon’s second sleep study. We hadn’t met him previously, and we really like him. He’s one more data point in favor of my theory that I really like health care providers (for  my child) who have kids of their own. I guess that’s not so much of a “theory,” per se, as much as…personal preference? Gut feeling? This appointment involved no poking or prodding of the boy, and certainly no looking in his ears (which he hates). In fact, the boy got to sit happily on Mama’s lap and play with his burp cloth and a little blue truck the whole time.

(I should note that in the car between picking up Todd at work and arriving at the hospital, Simon had a complete and utter screaming meltdown. He wasn’t hungry, wasn’t poopy, maybe was tired, but showed later that in fact he was just bored and lonely. As soon as I got him out of the car he was fine. He was making some real “a banshee is terrorizing me and therefore I must replicate the banshee noises” noises. It was both laughable and heart-rending, and actually bothered Todd more than it did me. Which now in retrospect makes me feel coldhearted, but whatever.)

This doctor was the first one in all the professionals we’ve seen who has asked us what we do professionally. I guess if you’re going to show some parents several pages of graphical data, it helps to know at what level they’re going to process it. But it makes me wonder why Simon’s surgeons haven’t ever asked us that – wouldn’t it be helpful to them to know whether they can use the phrase “Eustachian tube” or if they have to explain “…so there’s this ‘tube’ that connects the ear and throat…” (as two of them have done). But I digress. Dr. Sleep, not his real name for those keeping track at home, showed us printouts from the two sleep studies, and it was obvious that the most recent one was lots better. There were some freakishly low oxygen levels at the beginning of the recent one, but that was apparently an equipment malfunction, and indeed, I remember the technician coming in the room several times fiddling with the oxygen sensor. The part of the study from where the oxygen sensor was working properly looked not ideal, but pretty good. Simon had a few apneas, but his sleep and oxygen saturation was much, much improved from a few months ago. As long as nothing with his sleep-breathing changes, he doesn’t need the surgery that would lengthen his jaw and open his airway. But we need to keep monitoring him. As long as Simon is breathing without a lot of effort at night and continues to gain weight (we get another weigh-in on Monday), no surgery for now.

I asked how long we would need to be monitoring his breathing, and Dr. Sleep told us “probably until he stops growing,” which is about 19 years of age or something. I guess as doting parents, we’ll still be sneaking into his room before we go to bed to watch him breathe up until he leaves for college anyway, so no biggie. And if he goes to Rice, the Houston grandparents can take over.

However, we get to do another sleep study in six months to see how things stand. By that point, I’ll be such a sleep study expert that I ought to ask if I can do the sensor hook-up myself. Okay, no, but I will be an expert at using the waffle machine at the complimentary breakfast at the Marriott Residence Inn, the official sleep study hotel. And next time, I’m totally taking advantage of the king-sized bed for sleep and not for sitting next to on the hotel couch while I fart around on the internet.

In the meantime, this winter, we’re supposed to try not to let Simon get a cold, since that will stuff him up and inhibit his breathing. If I can figure out how to keep him from getting a cold, I will write another blog post about my secrets for all you parents out there.

Our next adventure happens on Monday, when Simon gets to undergo a CT scan ordered by his plastic surgeon (the one who will be doing his cleft repair). I originally thought she wanted the scan because they were going to go ahead and do the jaw surgery and this was their way of telling us. But she really just wants to see how his jaw is structured, which will apparently give some insight into whether it’s a small jaw, large tongue, or some other airway issue that gives him the breathing difficulties when he’s on his back.

However, doing a CT scan requires one to lie very still. So still, in fact, that no one trusts a five-month-old baby to do it on his own. So Simon and I get to undergo an adventure called Sedation Preparation — it rhymes, see — on Monday. He’s allowed formula up to four hours beforehand, and clear liquids up to two hours beforehand, and they really want me to deny him his nap so as to make him as naturally sleepy as possible for the sedation. Simon’s pediatric advice nurse said the clear liquid that will “keep him the most pleasant” in the pre-sedation period is pedialyte, so I guess that’s what we’ll do. I just have to believe that Simon won’t think I’m being mean on purpose, because he loves me or something. I’ve found, though, that when he wants to sleep, nothing I do (even singing “No Sleeping! No Sleeping! Wait for your Bed!” on the bus) will keep him from it — he’s half Melton after all. So I fully anticipate that I’ll refrain from feeding him, keep him awake through relentless poking and singing, only to have him conk out on the car ride to the hospital. I’m trying not to think of Sedation Preparation as “this terrible ordeal we have to endure,” but instead of “a typical Monday! with coffee for Mama!”

In other news, he’s now got three teeth. We’ve tried many times to capture them photographically, but Mr. Sir is pretty sly and closes his mouth just as the shutter opens. He has the two in front on the bottom and one weird poky thing off to the left where the molars are/will be. Other people have corroborated the weird poky bit. We think he’s working on another one since he’s been uncharacteristically cranky. Tylenol does seem to take the edge off, though.

We’re really thankful for the recommendation of “no surgery for now” and continue to pray that his jaw grows out on its own. This whole process has been really educational, but we’ll be glad when it’s all over and Simon doesn’t need any more special care, even if that’s when he’s a nineteen-year-old college student whose grandparents need to poke him in the ribs to make sure he’s still breathing when he’s been crashed on the couch for three hours at their house for Thanksgiving.

Todd and I have been meaning to post something like this for a while now, explaining Simon’s current and historical health situation to any and all who may be interested. The question was who should write it, and in what spare time. I guess I win? Todd promises to give his take at some point.

Let me start by saying that apart from having some medical concerns, Simon is a Truly Exemplary Child, in Practically Every Way. He is an excellent sleeper (quote from the pediatrician), he loves sitting still on my lap and watching things. He loves books and will sit through several Dr. Seuss tomes without even wiggling. He is happy to be held by anyone, family or stranger, and gives out full-face smiles with flirtatious regularity. The only reasons he cries (if you can call it that) are hunger and fatigue, which are fairly easy to recognize and ameliorate. Basically, tempermentally, I have the Best Baby Ever. Sorry if that makes you jealous. Actually, not really.

After Simon’s newborn exam in the hospital, the attending pediatrician let us know that he had been born with a cleft palate and Pierre Robin Sequence, which began a cascade of medical interventions and treatments, as well as
substantial parental education. At the time, I wasn’t sure exactly what a cleft palate was, so I will attempt to share what I’ve learned with you as well, Dear Reader.

At some point in the first trimester, the left and right sides of the hard and soft palate fuse along the midline. When this doesn’t happen, a hole is left, extending from some point behind the teeth toward the back. Simon’s cleft is bilateral (on both sides of the midline), and rather wide. We’re told it lies more to one side than the other, but not being a professional, I can’t really see it.

When we look in his mouth, instead of seeing the roof of his mouth, we see up into his sinus cavity. Now, in thirty years of life, it never occurred to me to stop and ponder: I wonder what’s inside my face and how it all fits together. Now I sort of know, but it’s really hard to describe, and you don’t really want me to. Part of the inside of your face is kind of lumpy and foldy and weird. When he was a little bitty guy, he had these strange lumps of flesh behind his upper gums in the back: his uvula (which was split in two). As he’s grown and his mouth has changed shape, these have been pressed toward each other more, so that the cleft appears a bit narrower. Ultimately, Simon will need a surgery (palatoplasty) to repair the cleft.

One challenge for me early on was that Simon’s cleft makes it impossible for him to suck since his mouth and nose are connected through an extra hole. It would be like our trying to suck through a straw that has a hole in it. Before Simon was born, I had been planning to breastfeed him, and it was kind of a shock to be told by a lactation consultant that he would never be able to nurse for nutrition. Looking back, it was the best thing someone could have told me, since it took away the pressure of trying to get my child to do something he wasn’t physically capable of doing. At the time, however, it was really difficult. I pumped milk for him for about two months, and fed it to him with the special “Haberman Adaptive Feeder.” He still uses the Haberman, but we’ve switched to formula for a variety of reasons. It hurt my feelings a little that he didn’t seem to notice the difference between milk and formula, but, well, whatever.

There can be lots of different reasons that a baby is born with a cleft palate, but missing from this list is “being a bad and negligent mother,” which took me some time to appreciate. After all, I had spent 40 weeks (and five extra days!) growing this child in my body, so it seemed natural that any deficiency in his physiology could be wholly attributed to what I had put into my body, what position I had slept in, how much stress I may have been under, had I worried too much about something, not had the right vitamin, not listened to enough Beethoven, exercised enough, or was it the two ounces of wine I had in February? No. No, no, no. Sometimes, it just happens. It took me about three months to believe that.

In Simon’s case, the cleft appears to be due to the Pierre Robin Sequence (PRS), where for some reason (genetic or as some consequence of intrauterine environment), the growth of the lower jaw is restricted, which causes the tongue to be thrust upward and backward in the mouth, which
prevents the palate from closing.

One big question that had to be addressed was whether Simon’s PRS was caused by some underlying genetic syndrome, or whether it was just a fluke. So, we saw a geneticist, who recommended this and that screening, which all came back negative (with the caveat of a frustratingly high false negative rate). As far as we can tell, he is a “normal XY male.” The screenings did not mention his status as a Truly Exemplary Child.

Simon’s small jaw caused him to have a lot of breathing difficulties early on. The same reason that he had the cleft in the first place (the tongue pushing up and back into his mouth) caused some breathing obstruction when he was lying on his back, the recommended sleeping position for babies. He often struggled to get air in, which made Todd and me freak out pretty much all the
time. He sleeps on his tummy now to allow his tongue to be pulled forward by gravity, but as he’s grown, he’s gotten better at positioning himself in a way that allows him to breathe freely. We also think (but can’t verify it quantitatively) that his jaw is growing, which is our dearest hope, since it would pull his tongue forward to where it’s supposed to be.

One concern with the breathing difficulties that we hadn’t anticipated involved weight gain. Simon was spending a lot more calories breathing than a typical baby would. Plus, genetically, he was predisposed to being really tall and skinny. So his doctors were concerned that he wasn’t getting enough calories to maintain a healthy weight (he was until very recently off the bottom of the chart in “weight for length” despite being off the top of the chart in length). On top of this, he developed acid reflux, making eating a crying, screaming challenge. Thankfully, this is all under control now thanks to infant-dose Zantac and high-calorie-density food. He’s plumping up quite nicely, something neither Todd nor I did until we were in college, if we can even say that.

Right now, we’re looking at one, maybe two, maybe three surgeries for Simon. The most obvious one is the palate repair, which is normally done around twelve months of age, but may be pushed out to fifteen to eighteen months because the surgeon wants to be sure his jaw has grown enough to allow a nice clear airway before doing the surgery.

Most children with a cleft palate also have ear tubes placed to allow fluid to drain out of the middle ear. The muscle that allows us to open and close our Eustachian tubes is connected across the palate (so Simon’s doesn’t connect to anything). As a result, fluid is collecting in his middle ear, which is harmless at the moment (but increases the chances of developing ear infections) except that it seems to be impairing his hearing a bit. The surgeons usually do that at the time of palate repair, but Simon’s ear tubes might be placed in a separate surgery if indeed his palate repair is done later. Overall, no biggie except for the general anesthesia.

The surgery that Simon’s surgeons are trying to decide if he needs, and the one that is causing his mama the most anxiety, is the one that would lengthen his lower jaw, pulling the jaw and tongue forward, fixing the breathing obstructions. We (and I really do mean “we”) have completed two sleep studies to gather data to evaluate whether this surgery is necessary at this time. The first one was inconclusive, the second one better, and we’re waiting for the follow-up appointment with the sleep doctor to give us the interpretation of the results. My guess is that they’ll tell us Simon is doing “okay” but that we need to keep an eye on him.

All this to say: Simon has some special medical needs, but mostly I don’t even think about it because he is doing so well in every other way. He’s charming and smiley and likes to read books and be thrust directly upward into the air to play Flying Boy. Since he’s our first child, I don’t have any real point of comparison, nor do I have reason to stop to think that not all babies need the special Haberman bottle, nor do they daily spit up some food through their noses, or have monthly meetings with the cleft palate team or sleep studies out the wazoo.

He just seems normal. And wonderful and snuggly and Very Bright at Math. And I’m really thankful for that.

Last night, Simon did his second sleep study. The first one (two and a half months ago) was inconclusive, which is to say that the data were really dismal (showing “moderate to severe obstructive sleep apnea”), but he’d had a terrible and atypical night, so no one knew whether he always had bad nights or if it was just that one time. So his doctors wanted to try again, which caused me no small amount of apprehension.  As Todd pointed out at some point, “If Dr. Sleep wants Simon to have another sleep study so badly, maybe he wants to take care of Simon that night!”

I should mention that the whole point of these sleep studies is to see if Simon is getting enough oxygen while he sleeps. Since he was born with such a small jaw (due to what is known as the Pierre Robin Sequence), his tongue lies farther back in his mouth and can (does?) obstruct his airway when he sleeps. As a result, he has gotten special dispensation from his doctors to sleep on his tummy, which may be one reason we usually get eleven uninterrupted hours from him at night. But are they eleven oxygenated hours?

Last night’s study (which we’ll call Number 2) was much, much better than the first one (Number 1). Let me compare:

No 1: Checked in at 8pm, and wire hook-up completed around 9:30 or 10pm — many, many hours past his bedtime, and frankly, kind of close to my own bedtime. Did not set the stage for “typical night.”
No 2: I had the foresight to ask for early check-in, which the hospital kindly accommodated. We arrived at 6:30, I fed and changed Simon, and he was hooked up by 8:00. He actually fell asleep mid-hook-up, only an hour or so after his normal bedtime.

No 1: Technician hooked up wires Very Slowly. This was likely because I asked a million questions about what everything was for and he politely answered, but this slowed him down.
No 2: Technician (different guy) paid attention when I mentioned Simon’s 6:30 bedtime, and did the speediest hook-up I could have asked for. When he had to leave the room to check the control booth, he ran — literally. He also kept apologizing to Simon for taking so long. I kept my mouth shut, although I did have questions I wanted to ask. Nothing affecting Simon, just general science-teacher curiosity that really didn’t need to be entertained.

No 1: Simon kept having problems with the toe oximeter (which measures oxygen levels by shining a bright red light on the big toe), which the technician had to keep coming in to fix.
No 2: Still had oximeter issues due to Simon’s sweaty feet, but the technician, a father of five, was an expert at baby-sock-removal and -replacement. Rather than trying to stick the sock on the foot, you have to sort of invert the sock and make it swallow the foot, as it were.

No 1: Simon woke up every thirty minutes screaming and Mama got very little sleep.
No 2: Simon had two freak-outs. One at 9pm, before I went to sleep, and one at 12:45am, which was embarrassingly soon after I had turned out the light for the night. What can I say, I was just farting around on the hotel’s free WiFi and reading Little Women (which is extremely moralizing but someone was about to die but didn’t). It took twenty minutes to calm him down, but then he was out until 5:30am.

No 1: Lots and lots of hair goop. Exceedingly much hair goop. Disgusting amounts of hair goop piled in mounds on his scalp.
No 2: Hair goop used sparingly, with Simon’s goopy hair plastered over the electrical leads to cement everything together. Much easier to wash out, and touching Simon’s head was way less disgusting.

No 1: Wore short sleeves. Simon fed twice during the night. Got hair goop in my elbow-crotch twice. Did not come off easily.
No 2: Wore sweatshirt. Simon fed once. Hair goop went on sleeve. Sweatshirt went in wash. Did not touch skin.

No 1: Mouth-nose breathing sensor frustrated baby, who kept moving his lip around trying to get it off. Maybe this was why he woke up every thirty minutes? It reminded me of when you give peanut butter to dogs.
No 2: Different kind of sensor, caused less discomfort.

No 1: Simon peed through diaper, through diaper cover, through pajamas, through swaddling blanket. Was stinky antibiotic pee.
No 2: Simon peed through diaper, through diaper cover, through pajamas (no swaddling this time). Was still stinky, but not like antibiotics.

No 1: Simon woke up hungry, but we had to take off all the leads before I could feed him. This required a lot of screaming, just so Simon could be sure I knew he was still hungry and hadn’t changed his mind. He is a good communicator.
No 2: Simon woke up hungry, but I got to feed him before detachment. This placated him so much that even the sticky face-tape removal didn’t get much of a rise out of him.

No 1: Used warm washcloth to scrub out hair goo. Baby was too small to put in big bathtub confidently, and a little too floppy for head scrubbing to proceed without my being afraid of hurting him. Lots of screaming.
No 2: Full bath. Lots of smiles. Kicking and splashing. Used his own baby wash on his head. Plus, nice plush towel at the end (he seemed to really like the towel, and tried repeatedly to eat it … Poo, I just realized I left his nice unscented baby wash at hotel).

No 1: Simon looked like a cyborg.
No 2: Simon looked like a cyborg but I was expecting it.

So now we wait a few weeks for Dr. Sleep to evaluate the data and make a recommendation. If Simon is still desaturating a lot (i.e. not getting enough oxygen) during the night, it’s possible he will need a surgery (osteogenic mandibular distraction, for you experts out there) to extend his jaw and bring his tongue forward. If he did okay, then that’s less likely.

My biggest source of anxiety with this last sleep study was that the data they collected wouldn’t be representative of Simon’s normal sleep (either better than average, which might mean the doctors were missing low oxygen levels, or worse than average, which might mean they would recommend a surgery that wasn’t really necessary). Although I wouldn’t say that Simon had the most normal night of sleep ever, I feel like it was typical enough that whatever the data show is probably reliable. And that’s comforting. So now I just get to try not to be anxious for two weeks.