Two surgeries

Suddenly it struck me that we have our pre-op appointment on Wednesday. Then we'll get an actual date for surgery, and this just makes it all more real.

I had mentioned bringing Maya to the doctor one day a couple of weeks ago to check out her cough, and Sam told me, "I not want go to the doctor Mommy. I not want get fixed."

***

I wrote this before the all-day appointment yesterday, during which we assumed we'd get a date for surgery.

We did get a date. We were also told by Dr. Albright, Sam's pediatric orthopedist, that he would prefer doing one side at a time. That means, one major surgery to correct the coxa vara in his right hip lasting six hours, followed by six weeks in a spica (body) cast, followed by a month out of the cast ... then back to the hospital, another major surgery to correct the coxa vara in the left hip, followed by another six weeks in a spica cast.

Before I go into the very logical reasoning behind this, which I agree with, I'd like to share my Liz Lemon meets Molly Shannon moment with you in the interest of levity ... and also in case any embarrassed moms find themselves in my awkward situation.

Luckily, Jim had gotten off work to come with us. I was so thankful for this because I can't tell you how many times I've done it myself, and it is not easy to take one to the potty while the other is on an exam table. Actually, I have told you this before.

We were waiting in Room 4 for Dr. Albright to come in and give us one date for surgery, and suddenly I realized I was sweating. Makes sense, they keep hospitals very warm for the naked people in there, and it was a little nerve-wracking waiting for the final date, the finalization that yes, surgery is happening.

Suddenly, I realized in addition to sweating, I was stinking. I remembered to get myself dressed, even used a curling iron (what?) on my hair. I got both kids dressed, remembered to bring two types of snack that each of them like (nobody eats the same snack, mind you) juice boxes and several books and toys. I even dug out a couple little Christmas gifts to each of them to give them in the office, a trick I learned long ago that was necessary if I was to have any sort of actual conversation about Sam's medical situation.

But I had forgotten something. What was it?

Of course, it was deoderant!

So of course I start sweating more realizing that I have forgotten this rather important step to getting ready for my day, and of course I don't have a spare in my purse, though I should for how often I forget it.

But then I have an epiphany, and I'm passing it to you other moms out there should you find yourself as scatterbrained and nervous and smelly as me.

Hand sanitizer!

I'm not saying it's the new Secret, but it's better than nothing and it works in a pinch .... so I'm squirting it in my hands and furtively rubbing it under my arms in a very Mary Katherine Gallagher kind of way, but of course Jim notices.

"What are you doing?" he asks.

So I have to tell him.

I figure since I told him, I may as well just tell everyone. In the interest of lightening the mood of the blog.

I'm glad I went ahead with that decision since the news was once again not what we were expecting. I may as well not ever say that in this blog since I say it every single time. I even wrote a whole blog about it. So from here on out, let's just all assume that nothing is ever as we thought it would be.

Dr. Albright finally came in (and I didn't stink anymore) and dropped the bomb.

"I know last time I think I told you I wanted to do both hips at once, right?" Yes, we answered. "Yeah, well now I think I should do one at a time."

I had brought up my concerns about Sam's Metaphyseal Chondrodysplasia, Schmid type, in terms of anesthesia. Because it's a type of dwarfism, I was worried about potential differences in dosage. I wanted him to be asleep for the whole 12 hours but I obviously didn't want him to have a hard time breathing. Wouldn't his genetic condition change how much anesthesia he could safely receive?

I still haven't connected with the anesthesiologist at Alfred I duPont Hospital for Children in Delaware, but want to before I talk to Sam's anesthesiologist because Dupont has so much experience with little people.

Then Dr. Albright started asking us about his size, and whether he'd dropped below the fifth percentile. He is below the fifth percentile; my understanding is that he's not on the growth chart in terms of height. He's in about the 20th for weight.

The genetic condition and anesthesia was a good point, and one that Dr. Albright seemed to already be thinking about. Because Sam has a narrower breathing passage than other people, putting him under for such a long surgery is more risky. (I have read that all people with a genetic condition causing dwarfism are prone to sleep apnea, and so this all clicked into place to me.)

The other concern is potential blood loss. Because Sam is so small, he doesn't have much blood to lose. When the bone is being cut, it bleeds. You never know how much it will bleed until you cut it.

That concern combined with the anesthesia concern was enough for Dr. Albright to want to be cautious and do one side at a time.

And of course, while inconvenient, Sam's safety and health are the only real concerns. Because this isn't emergency surgery, it's considered elective surgery. So Dr. Albright asked us what we wanted to do.

"It seems like you're uncomfortable doing both sides at a time," I said.

"Yeah, I would definitely say to do one side at a time," he answered.

"Then that's what we're doing," I told the doctor. I think we need the surgeon to be comfortable.

I like Sam's doctor a lot. He has spent so much time with us, and yesterday was no exception. He's good at gently guiding us to where we need to be, and there is no question in my mind as Sam grows that early surgery will be best for him. He really is inhibited physically, and he notices it. It's impossible not to notice. I'm glad Dr. Albright agrees with that.

At one point, when we were trying to answer Maya's questions, I told her that after it was all done, Sam would be able to better keep up when they race.

"In fact, he might even be able to beat you in a race," I told her.

I hadn't thought Sam was paying attention, but his head whipped up, and his face had this awe-struck grin on it.

I think that he suddenly got that. We'll have to focus on that when we're cheering him up as he goes through not one but two major surgeries and three months in a body cast.

I've never been looking more forward to July.

Slow down

It's not something that comes naturally to me, but I'm trying to learn.

Sam increasingly wants to walk, and I encourage this. He's doing so great, and I think for me, I'm patient with holding his hands so he can step down stairs like the other kids, instead of flopping onto his belly and sliding down feet first.

He works so hard at all of these physical challenges that most people take for granted. I do my best to slow down, slow Maya down -- which is no easy feat -- but we are one in the same. We move quickly by nature. We are always hustling from one distraction to the next, running and flitting.

Sam senses that. And as much as he wants to keep up with us, he more strongly wants to do things like us. He doesn't want to go down steps on his belly and knees at age 2 1/2, he wants to do his best to step down, one hand in mine and another on the railing, even though the lack of tension in his gluts will not actually allow him this physical movement. He will toss one straight leg out and use his upper body to move himself down to get footing.

I offer to hold his hand, and I don't rush him.

For somebody who is often in a hurry -- often late, often going, often cramming too much in -- I am learning a valuable lesson. I am slowing down. This is a shift for me. Suddenly I notice how quickly everyone around me wants to move. That was me two years ago. But now I look at them and wonder why, why are they in such a hurry?In a way, they make me sad.

I realize I have to learn patience, and understand that even if we're late, the extra five minutes it takes Sam to walk that distance isn't going to break us. There is some real beauty in this concept, particularly for someone who is used to rushing around.

Don't get me wrong, I have always rushed, but I also enjoy. You get used to living in a certain way. Although I had improved in punctuality before having children, I was and still am accustomed to constant motion. I think it's because I enjoy and am spontaneous that I find myself in a constant hurry, fighting to keep up with the social confines of things like time and schedules.

I am better at things like punctuality until things get too chaotic, and then I find myself back where I was as a teenager, being chided by my frustrated parents (only now it's me, or the director of Maya's preschool), because I'd missed yet another curfew. I can't blame them; now I'm on the other side of that and I realize the aggravation of a child who simply refuses to acknowledge any rules or authority, just like my sweet daughter, just like me.

I remember keeping up with my mother on our way to the mall, my small legs taking four steps to her one, because she walked so fast. It was fun for me. I loved to walk fast.

But something that is setting in for the very first time, something that would not have come if I'd not been forced to walk slowly, is that -- we can still be spontaneous and whimsical, even if we're not in a hurry.

If we're late to Maya's preschool, so what? We're paying for it.

The other day, we were taking a family walk. Sam opts to get pulled in the wagon for these walks often, because he sees his big sister ride in it and realizes it's age appropriate, whereas he is worried the stroller is not. He is extremely sensitive to anything that sets him apart in terms of physical capabilities. He's a smart boy. He knows he can't do what the other kids his age can do, but he works so hard, and as a result makes such amazing progress.

Maya was walking and Sam had decided to walk as well about midway through, seeing his idolized big sister flitting along, picking up scarlet leaves and tossing them into a stream.

Sam just can't go fast, and on any walk that lasts more than five minutes, he begins to waddle and swagger and even limp. At one point, he called out to us in frustration, "I can't keep up! I can't keep up Mommy!" But he didn't want me to pick him up, or put him in the wagon.

It was then that I began to work on my non-deliberate slow gait, just browsing as I walked, because I know Sam gets self-conscious if he's left behind. This is really a stretch for me; not only am I walking at about ten small steps per minute, but I have to make it seem as if I don't want to walk any faster.

What a learning experience that's been.

I was holding his hand crossing the street that Maya and Jim had crossed over probably five minutes prior, and I heard a motorcycle zooming up the road. I pointed it out to Sam, and the rider slowed down, waved and Sam and beeped. Maya and Jim were around the bend, they missed it all. Sam beamed with pride that he had been acknowledged by somebody as cool as a biker. For my 2.5-year-old boy, this was the highlight of the walk.

In the museum of Science the other day, we trekked from exhibit to exhibit.

When Sam climbs up into his stroller I know it's because he's had it. It hurts me because I see his energy level, I see his desire to run and play like all the other kids he sees, but he doesn't get frustrated. He just shouts, "Mommy, I wanna go up der!" and knees right up.

We stalled at several points to give him a break. At about 12:15, he told me he wanted to go to sleep. Instead I took them upstairs and fed them lunch, just so we could make the Harry Potter exhibit that we were going to at 1:30.

Lunch gave him some energy, and he was again determined to climb all 50 steps to the exhibit, all while I held his and Maya's hands and tried to toss my stroller up the stairs. Impossible you say?

Yes, which is why some kind grandmother taking her tween grandkids offered to hold Sam's hand up the stairs. Sometimes I hate to accept help like that from strangers, but realize I have to and like to pick the nice grandmother types.

After we reached the top of the FIFTY stairs (or what seemed like it) an employee, who had just said, "Oh, there's an elevator over there! There are no signs downstairs for it." (Thanks.) "Wow, look at that little guy! Look at his beautiful curls! And wow, he's walking really well!"

I still don't know how to react to that. I've never heard of anybody else being told that their nearly 3-year-old is walking well while struggling. My only guess is that they think he's a new walker, about 15 months old.

We were late as a result of all those stairs, and had to wait to get into the exhibit.

I began to get impatient, but realized this is another lesson in slowing down. The kids were wonderfully distracted by a nice English man they flew in to make the exhibit more realistic, who talked to us about his own child back in Britain. I felt lucky to have time to kill with my own kids. It really didn't matter that we missed our appointment; we were first in line for the next entry five minutes later.

The next day we were about to leave for errands. The kids were ignoring my requests, all ten of them, to move toward the door so we could leave, before I finally got frustrated and called out, "BYE!"

Suddenly Maya raced out after me as I went to put the reusable bags in the car. I saw Sam doing his best and most diligent run, which is admirable and heartbreaking at the same time. Then, as he often does, he tripped on his feet, but this time faceplanted into the front door.

I was there in an instant, and he was bawling. It takes a lot to get Sam to cry like that, a fact that was lost on the pediatrician we don't normally see. I made sure to tell him that when Sam falls, because of his skeletal dysplasia, his head always hits first. It's in the 80th percentile for size, and his weight is in the 0 percentile, and his weight in the 20th or so. He can't hold it up. I saw blood in his mouth, and his cheek began to swell before my eyes and turn purple. I was pretty sure he was OK, but I'm always worried about his head and neck.

So instead of starting on those errands, we rushed to the pediatrician. He was fine, and he is a trooper, so then the three of us made our way to Trader Joe's, then Whole Foods, and then ballet, before coming home to nap. (Him, not Maya and me.) It would have been a lot easier to have taken my time and not rushed, and avoided that extra errand and stress.

I'm seeing that more during our non-bodily injury days too. I've always been into nature, but I used to notice it more when I was out specifically observing it, too busy to see sometimes when buzzing from place to place.

Now we see everything just walking from the car to the front door of Maya's school -- a maple tree covered in fiery orange and red leaves with autumn sunlight streaming through, making it look as if it's on fire, ant hills, drainage pipes, low-flying airplanes, blooming flowers surprising us in November, clouds that look like dragons. Maya is slowing down with us, and we will actually stop in our tracks, even if we're late, to look at a flower or a pretty leaf.

Sometimes it's nice to slow down.

Up Down Up Down

There's a Yo Gabba Gabba episode with Jack Black, and he does a Disco Roll dancey dance (which I looked for and couldn't find) and the refrain is: "Up! Down! Up! Down! Up! Down! Up! Down! And roll! ..... And back! .... And roll! ... And back!"

I find this cadence echoing in my head frequently these days, partly because the New Friends episode is my kids' favorite and because I disco with Black like nobody's business. But it's also because one day I'm so completely up -- confident that I'm preparing myself for all of Sam's medical issues around his metaphyseal chondrodysplasia, Schmid type diagnosis and feeling utter confidence that we will all prevail and be fine -- and the next dragged-energy down, totally confused and unsure of how we'll navigate the future.

On Thursday I spoke to the pediatric orthopedist Dr. Maurice Albright about the surgery to correct Sam's bilateral coxa vara, about pain management and the spica, or body cast, for six weeks.

I felt so much better after that conversation. A lot of the answers were the best-case scenario, and that gave me hope. Dr. Albright spending 40 minutes with me on the phone in between patients in the OR also gave me confidence, as well as his steady assurance that he would take good care of our son.

He also consoled me in other ways.

A spica cast didn't seem as crazy and scary as it had before, and a good friend said he would help us build a spica chair for Sam, which is awesome.

I learned that Sam will need two sizes of diapers, one tiny swaddler size (or even a maxi pad, Dr. Albright tells me) to tuck into the cast and a big probably size 6 to go over him and the cast. We will have to try our best to keep it clean and dry ... a daunting idea for a toddler in diapers, but Dr. Albright said most people are able to keep it well enough to last the duration.

He's going to arrange for me to talk to the other surgeon (because Sam is so small and the surgery is so long and intense, there will be two of them) and to the anesthesiologist.

I am also still hoping to talk to the doctor in Delaware at the Alfred I. Dupont Hospital for Children, Dr. William Mackenzie, who specializes in pediatric orthopedics as well as skeletal dysplasia. I'd like to discuss anesthesia, and about pretty much everything, since the biological makeup of a person with a skeletal dysplasia (which is a type of dwarfism) is different from someone who does not have dysplasia.

We will give Sam Tylenol with Codeine in liquid form, so that alleviated my concerns of potentially giving him pain medication intravenously. He will have a general anesthesia for the 12-hour surgery, which will likely be followed by a spinal epidural to ease the post-op pain. He will probably go in on a Thursday (Jan. 7 or 21st) and come home Saturday morning if all goes well.

Dr. Albright sounded confident, but not eager or overconfident. It was just the right balance to me. He was exceedingly patient with my dozens and dozens of questions. Some of them were slightly more inane sounding than others, I suppose.

For instance, I asked if they had clowns.

I think Dr. Albright thought I was joking, but I referenced a story sent by a close family friend that found children were much less stressed when there was age-appropriate entertainment like clowns. Dr. Albright said he would see to it that Sam had access to child life specialists to help with anxiety.

I told him this was important -- the article (which I can't link here because the story was clipped from a paper and I don't know which one) mentioned that parents being present surprisingly did not reduce anxiety levels in kids unless the parents were receiving acupuncture or other calming techniques. (I'm wondering if Valium qualifies as a "calming technique.")

Suddenly I felt a shred of control, just a bit of confidence that I could indeed handle this, all while buying a house. This was on the eve of a follow-up with the geneticist to discuss his metaphyseal chondrodysplasia (meh-taf'-uh-SEE'-ul con'-dro-dis-PLAY'-zsa), Schmid type.

As usual, every single time I get the slightest bit comfortable, things shift.

Here we go in genetics.

It wasn't anything specifically that the geneticist Dr. Angela Lin said that I didn't already know. It was just being there this time. I couldn't get my head together.

We talked about some genetically alarming things that may or may not even pertain to Sam's future. They were pretty much things I knew, like, Sam's chances of passing this to future offspring are 50-50. That is if he partners with someone who does not have Schmid. I wonder if it's the same odds for children by two people with different types of dysplasia as it is if they have the same. They are trying to find out for me. That's about all the detail I'm prepared to go into on that subject, and at this point it is not really what we need to focus on.

We had a brief discussion about the possibility of growth hormones, a topic that makes my insides cinch up and knot. I'm almost relieved learning today that it doesn't seem they will help in Sam's case. I don't ever want him to think we're trying to fix him, that he isn't right just the way he is. If others in the world are too stupid to recognize his greatness because he is of a different stature, I mean it when I say it's their loss. Sam is so wonderful that the people who really matter, who will matter to him, will not see his size as anything but a piece of who he is.

I had an eerie feeling with other doctors in the room, as Dr. Lin explained cues for identifying Sam's condition. I didn't mind this; I'm happy for anyone to be more educated on this, particularly the doctors who will help diagnose such genetic conditions.

But I couldn't help feeling protective of him there on that table, small and agreeable, as they discussed how you could tell that his fingers were smaller proportionately because of the hand creasing.

"Are his toes also out of proportion?" one asked.

Not really, Dr. Lin said, though the big toes were broader than an average bone stature.

I asked if this is why his second toes don't touch the floor when he walks. I know this because even when he's barefoot outside and comes in with little filthy feet, those toes are clean. She said possibly, she didn't know.

It was nothing these doctors did, it was just being the center of this discussion, almost as if Sam was a subject and not my sweet little boy. All of that while running down the hall to check on Maya using the potty... it just made me feel protective, like I wanted to snatch him and say, "Don't talk about his limbs as if they are a novelty!" I wanted to kiss everyone one of his tiny perfect fingers.

I maintain that they conducted themselves with perfect professionalism and friendliness and respect for us all. They obviously like Sam as a kid, who wouldn't? But something about it made me want to take my babies and run.

We left there dazed, as I hustled the kids across the street to get some pizza. Then to Ben & Jerry's for ice cream. And then to ballet.

The kids have been acting up. Last week I believe some things sunk in and stuck that hadn't before, and that ranges from my discussion with Dr. Albright to our morning at the hospital.

Jim and I are trying our best to get them excited about buying a new house (to us, actually it was built in 1949), painting their rooms whatever color they want, and then we find ourselves talking to them or around them about the logistics of Sam's surgery, body cast, genetic predisposition.

It's a whole lot for a 4-year-old and a 2-year-old to take in.

It's a lot for a 35-year-old to take in too.

But we're bumbling along, and I'm trying so hard to do things right by them. I just restarted Being The Other One by Kate Strohm, so I can help Maya along through this. Right now, I think she needs some understanding from us. When I noticed her being a little mean to Sam, not wanting him to play with her (more so than their typical brother-sister stuff) I made sure I asked her later if she was angry at Sam. She said she was, but didn't know why. I told her that it was OK to feel angry sometimes because he is getting so much attention in this. I told her she was a good girl. This seems to have changed her attitude dramatically.

Sam is still consoled by me holding him and rocking him and telling him Mama is here. Maya is getting to the point that she needs more. I'm trying to let them both know that we're all confused sometimes, we are all scared sometimes, but I know we will all be OK. We're a family, and we'll do this, and we'll be stronger for it.

I think all in all, we're doing a fairly good job with it all.

Up! Down! Up! Down! Up! Down! Up! Down!

The world catches on

It is finally striking me how small Sam really is. Now that we're moving back into fall attire, and Sam is solidly a 24 month waist, I realize I'm rolling pants up three and four times. When they come unrolled and drag several inches below his feet, he'll sit down on the floor, crying out in frustration: "Mommy! Fix my pants!" If he has a choice, he opts out of pants altogether. I can't really blame him.

At least now he still has the 24 month option. I figured out with Maya that 2T is a longer, more slender fit, where the 18 and 24 month pants are more robust in the waist and shorter in the length.

I finally have embraced the fact that having a son with Metaphyseal Chondrodysplasia, Schmid type means I have to learn to sew. I will need a sewing machine, and soon. I don't want Sam to be picked on for having to roll his pants four times.

I'm not the only one who has noticed there is something different about Sam.

His gait is already earning him attention from the bigger boys at Maya's school. In fact, on a day that was already tough for me, two little boys looked at Sam and began mimicking his limping waddle, and when we began to leave, one meanly jeered, "Yeah, GO!" and waved his arm like he was throwing an imaginary ball toward the front door.

I stiffened, trying to refrain from going psychotic on these little 5-year-olds. Two nearby teachers were talking -- the classes were in transition and I know they were trying to work out the new system. So I said loudly, "Oh, that's not nice. You remember what it was like to be the little one."

Mrs. P, who turned out to be Maya's new, very sweet teacher, snapped to attention and said, "Who wasn't being nice?" The two boys snickered.

I just opened the door, lifted my head and walked out behind Sam, who had actually just seemed thrilled that the bigger boys were playing with him, in his mind.

It makes me insane to think of anyone teasing my kids, particularly for something out of their control. He didn't realize it that day, but the day will come that he does.

I think it was the same two boys that had also made fun of Maya's brother's "ugly face and head" one day last year. Mrs. L caught that one, and let me tell you, I wouldn't mess with her. She had those boys over at her side in an instant to reprimand them, and I loved her so much at that moment.

I feel like these kids are noticing what adults have too but can't quite place -- yet -- and that is that Sam has a form of dwarfism. Despite everyone saying Sam probably won't be different, I see that he already is.

According to the geneticist's note:

"Their (The endocrinologist's) note mentions the fact that he clearly had short stature, with a height of 76.4 cm, which had fallen from the 25th to less than the 5th percentile. In the meantime his head circumference of 49.5 cm was maintained at the 70th percentile, a relative macrocephaly. His weight 11.5 kg was holding at the 10th to the 20th percentile."

These small boys had picked up on the fact that Sam had a waddling gait due to the Coxa Vara, and that his head was not quite in proportion with his body. It astounds me that such young boys would be able to discern differences like that and hurl them out as a reasons to ostracize my sweet little boy.

Regardless of what anyone has said, he has a form of dwarfism and it is increasingly apparent. I know people mean well when they say people might not notice, or that it doesn't matter. Of course it doesn't matter, to me, I love Sam just as he is. He is awesome and resilient and hilarious. But the reactions from the rest of the world are beginning to drive home that we should raise Sam with this understanding so he can handle ignorance with grace and humor.

Now is a good time for me to put out there something I have heard and never understood. I have heard people say that they would not change their children, regardless of the situation, because that is what makes them who they are. I'm reading a book now called Expecting Adam, and author Martha Beck says she would not change a thing about her son with Down Syndrome. I didn't get it, but now I do. Sam is delicious -- he is precious and his strength is remarkable. If he falls and cries, you know it hurt, because 19 times out of 20, he pops right back up and blurts out "I OK!" before you even have a chance to ask. That's not to say that I don't knot up inside when he can't walk up average-sized stairs, or jump. I might take away the pain if I could, but I wouldn't change his stature. I would change how the world reacts to his stature.

Though I don't mind that he is different, other than how it affects him, I don't want to pretend that he is the same. I don't want to feign a reality that he can't relate to his own experience. I understand we're all different, and all the same, but some differences are more immediately apparent than others.

I also don't want to dwell on the differences, or let them define who he is. As I've written before, I just want him to be proud of who he is. I know this isn't easy while growing up, and people with far more marginal complaints beat themselves up over not being blonder/darker/prettier/skinnier/bigger/taller/cuter, or whatever, despite having physical health and capabilities that others would love to have.

I guess my biggest wish is that the world wasn't so scared of or voyueuristically fascinated with differences like dwarfism. With Metaphyseal Chondrodysplasia, the form is much slighter than other forms. That means Sam could end up being 5'6" or he could end up 4'6" on the shorter end. The geneticist had assured me that he would be short statured but in proportion -- though when I read her note, I realized, that's relative to others with different types of dwarfism. Clearly, he has different proportions than average-statured kids.

These boys have made me think about something I read on LPA's Web site in what I call the "Welcome to dwarfism" handbook.

It had mentioned that kids with dwarfism often benefit if their parents send materials in for classmates so they can understand it, and so they feel free to ask questions. That leads to knowledge and understanding and ideally, makes teasing less likely. I had thought maybe we wouldn't need to do that since Sam might end up of nearly average height... maybe we shouldn't draw unnecessary attention to him. But if people will notice anyway, maybe we should prepare everyone. And if he ends up on the smaller end of the spectrum, people will notice, he will notice. But on the other hand, I don't want to make him self-conscious about something in addition to his physical limitations if he doesn't need to be.

As usual, I go back and forth and I guess this falls under the all-encompassing heading of: Things to Figure Out As We Move Along Because There Are No Easy Answers.

Wednesday

It's only been about a week since I posted my last blog and already I feel like somebody else wrote it. I still feel lucky, and mostly good, and all that.

But I'm starting to get freaked out by the idea of the surgery, and the body cast, and my son's cognizance that well exceeds his age. The idea of him being in that kind of pain turns me inside out.

Tonight when Jim was reading Maya and Sam a bedtime story on her toddler bed, Sam wanted to get down. (I was on my never-ending mission of putting laundry away.) Then, out of nowhere, Sam began shrieking and saying his knee hurt. "It huuuuurrts!" he howled, pointing to his right knee. I told him I'd get him some special medicine, and took him to his room. I decided to give him Tylenol.

That was partly because the mom I met through this blog, whose 6-year-old daughter also has Metaphyseal Chondrodysplasia, Schmid type, and who hasn't had surgery yet for Coxa Vara, said her girl began complaining of pain in her knees around age three or four. Apparently that was the first time she could articulate it, and I guarantee, if I hadn't spoken to this mom, I wouldn't have put this together either. I would have assumed Sam bumped his knee. But I've seen him react this way before to knee pain and because of her, I realized that this stems from their form of skeletal dysplasia.

I gave him his Tylenol in a little dropper to drink down, and he took it, but then pointed to his knee again, and searched for the right words.

"No, Mommy, here, my knee hurts," he said, pointing. "I need my 'peshal' medicine."

"I know honey, this will make that feel better in a few minutes," I said.

"No," he tried again. "Want my knee medicine?"

So I told him that it would take time, but it would feel better in a few minutes -- and that this winter, he would have surgery so his knees wouldn't hurt, and so he could run and jump more easily.

"Run and jump?" he asked me.

"That's right buddy," I told him. "But it will hurt at first, and you'll be in something called a cast, and you won't be able to move much for a while. But then you will be able to run and jump, and don't worry, I'll be right there with you. The doctors will help you and I'll take care of you too."

He seemed to be thinking most about running and jumping before telling me he wanted to go back into Maya's room to keep reading. On our way there, he took my face in his hands to steer it to look in his eyes, as he only does when he really wants me to hear what he's saying.

"You be a doctor too Mommy? You be a doctor too?" he asked.

"Oh honey... I won't be a doctor, but I'll be right there the whole time... I'll be right there, and they will make you feel better and even though it will hurt at first, you will get better and I'll take care of you," I told him.

"You be a doctor too, Mommy? Pwease?"

A sweet surprise

This seems so counter-intuitive.

I felt more frantic and upset about all of this stuff regarding Sam -- all the surgery, the Coxa Vara, the spica cast, and the realization that we had something unexpected and painful to contend with -- all of this stuff, until I realized it was a symptom of a larger condition.

It's hard to explain, but knowing it is part of a bigger picture, and one that could include several surgeries as well as other unknown struggles, it just feels different. Instead of being one huge surgical thing to dread, it is something that will always be part of our lives. That so oddly makes it easier for me in a way.

Well, "easier" is the wrong word.

Of course, I'd rather this not be an issue for Sam and Maya and Jim and me. But weirdly, it felt even more devastating when it was the surgical element alone. I'm not sure if I can even articulate it, or understand it myself.

I had always hoped and prayed that my kids would never have special needs, only because I never wanted them to hurt in addition to what is just part of any healthy upbringing. I had just assumed that it would feel sort of easy, in the way I guess it did for my brother and me.

In the beginning, I think this assumption was one of the big devastations; I never expected any issues like this. Especially not so early on.

For me, part of dealing with this was redefining 'special needs,' and part of that therapy came in talking to my cousin Leigh and getting to know her son, who was diagnosed with autism at age two. I was in awe of how she handled everything, how that just all integrated into, or really became, part of her life. More therapy came from reconnecting with our friends who have two fantastic sons. One of them has had several back surgeries for a type of scoliosis that would have been fatal in another time, when this type of surgery wasn't available. Both of their boys are on the autism spectrum. They astounded me with how they lived with it all so positively, without any overt bitterness or self-pity.

But I was more surprised by how they dealt when I thought I'd have to go through this once, and then it would be over. I couldn't imagine how they could just keep dealing with medical issues on such a long-term basis, and be so positive. I can't say whether I would have eventually come to this place or not, but as much as I mourned following the geneticist meeting where Metaphyseal Chondrodysplasia Schmid type was dropped on my head, I feel like now I'm better equipped.

I suppose it's natural. You do what keeps you best positioned to not only survive, but to thrive. Really, you have to keep going and not anguish or dwell too much, or you have unnecessarily missed out on Life. Because this is life as we know it now, our reality.

It could be so much worse, and those of us who go to hospitals regularly get glimpses into other, more tragic realities.... It is terrifying, and horrible that I look around and feel relief glancing down at Sam's smart, happy, healthy face. Sadness for those families, to the point that it is difficult to make eye contact without betraying the sorrow and relief. But since I wouldn't want anybody feeling that way about me, I make sure I look the mom in the eyes and say hi, and tell her how cute her little girl is, with such pretty blue eyes, without commenting at all on her bald head or dark circles or palled skin. Maya approaches the girl, probably about three, and reiterates her cuteness, and talks about her beautiful turquoise dress.

That is not to say that I don't feel sad and scared sometimes; I do. But somehow, knowing this is not a condition that will just be 'fixed,' but a family dynamic that we will all experience together, always, changes everything.

Part of this dawning came before vacation, and part of it is because we just spent a week on the Cape with my parents. We distanced ourselves from the whole thing -- there were no doctors or even discussions of doctors. We sat on the beach. We all marveled at how well Sam walked in the sand and navigated the water, and how amazingly he has responded to the physical therapy through Early Intervention.

We knew when he was tired and needed to nap or rest. I let him go as far as he wanted. If I saw him limping and getting fatigued, I would offer once to carry him. If he said (as he usually did) "I want WALK," with a pursed-lip expression of determination on his face, I let him and told him what a great job he was doing. The rare moments he wanted to be carried, I scooped him up without a second of hesitation or a sigh to ensure he didn't feel like a burden.

Coming home, being reminded by others that this was happening, at first I didn't want to talk about it. I just feel accepting. And happy. And in a way, I still don't want to talk about the details. I don't want to think about them more than I will need to prepare us all and care for my family the best way possible. That might not always be the case, but now, this is how I feel. You could exhaust yourself silly fretting and worrying and feeling sorrow and self-pity over the course of a lifetime. I'm sure I'll have more to say to my close friends and family members, and my new friend in England whose daughter has the same thing as Sam, but largely, I don't want to dwell.

I hear people constantly telling me how great I'm doing, how well I'm handling this, how wonderful a mother and wife I am. It feels a little undeserved to me. I'm just doing what I've always done. This shift wasn't conscious, it stems from knowing this is life, and we either have fun with it and appreciate it, or it's no good. For me, that isn't an option. Is it for anyone? I remember feeling like my cousin and our friends with their sons' medical issues were so strong, so positive, and I guess now I get it when they don't quite get the praise (though they appreciate it, and so do I).

Spending a week at the beach with your family forces you to remember how amazing life is, as cliche as that is. It's beautiful and painful and delicious with opportunities and pleasures if you're not too busy or focused on the bad stuff to notice.

Greeting the world

It's amazing how different it feels to hear certain things knowing other things.

For example, when the woman sitting next to me on the airplane to St. Louis told me she believed Sam would be tall, I probably felt differently than I would have before I knew Sam has a form of skeletal dysplasia that will likely keep him between 4'6" and 5'6".

Before learning that, I probably would have laughed. Nobody in my family is very tall, really, and Sam's height percentile has steadily dropped since he was born. Not really surprising to my 5' 7" husband Jim and me; we had just assumed we would have smaller kids.

But as this lady looked over at the row of three -- Maya, Jim and Sam -- she asked me Jim's height, and sagely informed me:

"Your son will be taller than him. I can tell. Look at his hands -- they're big for his size."

I was reminded of the puppy paw theory and didn't realize it applied to humans.

I smiled at her. She was a very nice lady. I didn't say anything.

"You can always tell when you look at a toddler's hands how tall he will be," she continued.


I didn't tell her that with metaphyseal chondrodysplasia, Schmid type, (and finally a pronouncer, now that I've got it: muh-taff'- uh-SEE'-ul con'-dro-dis-PLAY'-zsa) Sam's hands would probably look larger in proportion to his arms (and legs), because his limbs are disproportionately smaller than the rest of him. Not much, but a bit. That is the case with most types of dysplasia, or at least, that's my understanding.

I am used to people commenting on his waddling gait, which is increasingly pronounced the older he gets. Not because it worsens, but because it is more obvious as he grows that it is more than just a toddler gait, and more glaringly asymmetrical. It earned him the nickname "The Mayor" from Jim early on, and that name caught on. It wasn't just his swagger, it was also his genial nature and outgoingness. I would always nod and agree enthusiastically when people would giggle and fawn over him. I still do typically.

But some days, it feels different than it did prior March 16 when we first saw the pediatric orthopedist and found out he had coxa vara. Now some days I don't grin quite as widely when they talk about his waddle, though I still agree, it is ridiculously cute. But I feel sad too because I know it will cause him pain after his major operation on both sides and six weeks in a body cast, and I know he has to work harder than other kids to move. Certain things, he just can't do, and I watch him struggle and offer gentle help.

I try my hardest not to ever let him feel my sorrow; I think that would feel terrible as a child, and he is so happy and bright. He doesn't know anything different, and he's tough. I don't let it out, except occasionally after the kids are in bed. I've heard different theories about this, from people who say not to ever get down because kids feel that, to people who say you have to mourn exactly when and how you need. I think I'm somewhere in between, but fortunate to feel positive most of the time.

The rest of the family rarely calls him the Mayor anymore, probably because they feel the same sense of sorrow that he has to struggle at times.

I am also accustomed to people gasping or trying to feign non-surprise when I tell them that Sam is nearly 2 and a half. Or they will stare open-mouthed when he starts talking. He is eloquent for his age, and he certainly is eloquent for the age people assume he is. I am guessing that's around 20 months or so, considering his gait and his size. Before I knew about his genetic condition, I just figured it was his hips combined with genes... though, looking at pictures of Maya, I realized she definitely had more of a kid stature, less toddler stature, by this age.

But the airplane lady was the first to openly comment on his height specifically. Oddly, she was there to inform me he would be tall, not to mention that he is small.

Reflecting on this later, I remembered back to one of my cousin's softball game. I was probably about 11 years-old, visiting the same family in St. Louis we were traveling to see that day, and a little girl started talking to me in the stands. She was really cute and funny, and I asked her how old she was. She said she was eight. I remember being stunned because she was so clearly short for her age, that I just started talking before I could stop myself.

"You're really tall for your age," I blurted out.

The second I said it, I thought, "WHY WHY WHY WHY? You couldn't just keep quiet?!" I don't know why I chose those words. She was obviously small, but knowing it would be rude to say "You're really SMALL for your age," instead I said "tall." It's like I couldn't stop it from coming out....

And to my humiliation, the little girl shouts incredulously up the bleachers: "Mom! HEY MOM! This girl thinks I'm TALL for my age!!!"

I turned about 100 shades of purple, I'm sure. I stammered something about how second grade was so long ago that I couldn't remember how tall people were....

It wasn't a big deal. We hung out the rest of the game. Because we only visited occasionally from whatever base my dad was stationed, I never saw her again. I had forgotten about her until right then.

I think sometimes we are surprised by things we see, such as a person who is much smaller than we expect, but the urge to comment to the contrary is very interesting to me.

The airplane lady did not comment about Sam's current size, but on her idea of his future size.

I commented that the little girl was tall though she obviously was not.

In both statements, there was the idea that height was ideal, lack of it was a shortcoming. (That's not meant to be a pun, it's an example of how even our language is wired demonstratively where height is concerned. I'm sure I'll write an entire blog around the language of tall and short one of these days.)

It is interesting to me also that people feel at liberty to comment on stature. As as my new friend Pen put it, would they comment if our children were overweight or in a wheelchair? Maybe. I don't know. But in the cases I mentioned, the comments were contrary to the truth. I think that is really based on the fact that height is valued in our culture, no matter what we say or think.

I remember when Maya had increased in growth I had reported back to the family with pride. She had always been around the 5th percentile, and shot up to the 25th. I never minded that she was small, but for some reason I felt accomplished that she had caught up, so to speak.

I remember when she was a baby, so many people would always beam when they told me their baby was in the 90th percentile for height. (It seems like too many are in the 90th... if it's that common how is it the 90th? Or maybe we just know people with really huge babies?) I never cared that she was petite, I loved it. She reminded me of a little pixie.

Sam was a big baby, and tougher to hoist. He was born pretty big, and he stayed pretty big until about five or six months, when he leveled out. This did not come as a surprise to us at all; we were surprised he stayed so big for so long. But I remember hearing myself tell people where he was on the growth charts (down to 25th, or down to 10th) and people would reassure me, "That's OK. You knew he probably wouldn't be a big kid," as if I had said it with sorrow. And I really had not. While I did report with pride that Maya had gotten bigger, I never felt remorse or shame at having a smaller child. But still, I felt sometimes people would console me.

Some of this is evolution-based... just 100 years ago size did play a much more important role in our ability to survive, particularly for men. Today there are studies that show that shorter people typically don't make as much as taller people. These are all the studies we've been reading about white men versus any other ethnic group or gender for years.

The thing is, people have expectations and preset notions of what is ideal, particularly where physical attributes are concerned. Whether fair or not, whether we ignore them or not, label our children or not, they are going to infiltrate our lives. I want to handle every comment with grace and unflappable ease. Rarely are such comments mean-spirited, but even those should be met with calm -- they are made by people who don't understand and fear the unknown.

This is crucial to helping Maya and Sam realize that it's OK to be different. This is one of the main points made in the LPA (Little People of America's) sort of introductory, welcome-to-our-World handbook.

Some days maybe I'll feel like explaining a little bit. I have already given a sort of modified version: "Yes, Sam has a type of dysplasia that causes his bones to grow differently and create that really cute waddle, though it will require surgery to correct." I have had people debate me over the need for him to have surgery, and I mean fairly random people who have little or no experience or knowledge about any of this. It gets exhausting.

Some days, I won't be in the mood. I'll be tired, I'll be stressed, I'll be cranky or overwhelmed or overextended like most moms, whether they face these or other challenges. But I'm going to do my best to react like I did to airplane lady....

Just smile and say nothing.

Confirmed

It's official.

Sam definitely has metaphyseal chondrodysplasia, Schmid type.

It's now a deciding process of how to react to this news. The typical parenting struggle of walking the line, the one you walk 1,000 times a day as a parent, from deciding what the kids will eat for lunch, to what you'll do (go to the park or take a wagon ride?) to how you react to them each tugging at the same toy bucket (never mind there are seven scattered in your yard) and both screaming that he or she had it first.

The geneticist mentioned that it is important to protect Sam's medical privacy and that we should not associate him with the word "dwarfism."

I'm feeling hesitant to post pictures of him here for that reason, but I can't help but think, if we do this right, and teach him that this is just one part of his wonderful self, maybe he could embrace it. I'm not saying we necessarily use the word "dwarfism" -- in fact, I'm all for letting it slip away from all of our consciousnesses for a while.

But I do want to raise Sam knowing that he has a form of skeletal dysplasia, and I don't want terms like "little people" and "short stature" and "dwarf" to be bad or shameful words, or to mean anything but positive things in our house. When Sam's a little older and begins asking, maybe we could tell him then that his sort of dysplasia means he might not be as tall as some other kids.

Or maybe we'll just try to raise him knowing there is power in standing apart from a group, and that he's smart and capable and adorable regardless.... and fortunately he is not living among giants. I feel grateful that we are all pretty small. I mean, we already have step stools around the house, built by my husband Jim's grandfather, who might have been around 5'2" (I'm guessing here) and we all use them daily.

Maybe we'll have some interaction with local chapter of Little People of America, per the geneticist's suggestion.

The little reading I've done about LPA demonstrates a huge amount of pride among the short-statured community. I would like for us all to be around that sometimes, to remind ourselves and teach our kids that it's important to be proud of your physical package, even if something about it varies from what is deemed normal.

And I wonder if letting him grow up aware of all these things is easier than if someone drops the bomb on him when he does start asking. It reminds me of kids I have known who grew up either A) always knowing they were adopted, even before they quite knew what that meant or B) not knowing until one day.

Even if they were very very young, my experience was that an adopted child's world was turned upside down by the realization that something they took for granted, biologically, was not so. If you don't raise a kid just knowing something, it will come as a surprise, especially something this big. I would imagine there's an element of betrayal. How could you trust these adults you thought were telling you straight all this time? I don't think it's the same as other things we tell our children (tooth fairy) because those bring magic into a child's life by them believing. Omitting a huge part of a person's Being, something they will have to know at some point, seems risky.

The geneticist mentioned limb lengthening, and assured me it is no longer controversial, but I know something about the pain involved based on an awesome Associated Press story my friend Gretchen Parker wrote while following someone going through it for two years, accompanied by the late Roberto Borea's phenomenal photos.

The procedure was developed to correct a discrepancy in children's leg lengths, but was adapted to lengthen limbs of short-statured people. To me, this would never be a decision I would make for my child. Ever. Whether his height was projected at 3 feet or 5 feet. This would be a decision only he could make, and if he did, I would be there for him, but I definitely would make sure he knew I love him just as he is. I never want him to think he isn't good enough just the way he was made.

I'm re-starting a book called Being the Other One, by Kate Strohm, since when I first began reading it, I felt like much of it wouldn't apply to Maya. Back then, I thought we would have one surgery and everything would be over; I didn't think there would be a noticeable difference between our family and others, and maybe there won't be. But maybe there will be.

The book is written by someone who grew up the sibling of a special needs child, and addresses some of the guilt and frustration fostered in her growing up. She also integrates the perspectives of many other children who have felt marginalized by their family's focus on a special-needs child.

It's so important to me, while emphasizing how wonderful Sam is despite some of these hurtles, to make sure Maya knows she is uniquely wonderful and amazing and smart and funny. She also stands out from the crowd. She is so feisty and intelligent and creative and sassy. She is sweet and empathetic and I want so badly to be sure she doesn't get lost in the shuffle, or feel less important than her brother.

As usual, I am already putting too much pressure on myself to do this exactly right, not to slip up or waver one bit from what is best for everyone...

Dwarfism

Maya, Sam and I pile in the car, the Tom-Tom we call Jane ready to navigate us into Mass General. Sometimes Boston is easy, sometimes not, and Mass Gen has given us trouble in the past. Jane tries to take us on Route 9, but I upset her by going I-90 anyway, and she has to change her whole route plan. My mother-in-law, Chris, has come from the Catskills of New York to help.

Ever since I spent all day at the pediatric orthopedist with Maya and Sam, by myself, I try to get help anywhere I can. I remember that day so well, and it seems like eons ago that we were told Sam would need surgery to correct his hip/femur deformity, but it also feels like yesterday.

My husband Jim isn't coming since we've been told this process usually takes time.

The geneticist, Dr. Lin, is awesome even though we're late due to unexpected rush hour at 9:30 a.m. Jane had navigated us through Cambridge, so I'm not sure it was the best in terms of traffic, but really, who ever heard of rush hour lasting past 9? Outside of DC and LA, that is.

Dr. Lin is tall, and she's very sweet, and genuinely interested in our journey from the office of Dr. Albright, the pediatric orthopedist, to her door. She has a similar demeanor to Professor Wiseman on Curious George, and this comforts me. We watch a lot of Curious George in our house and she's one of my favorites.

We spend about a half hour talking about how we came to be in her office on that afternoon while Chris tries to keep the kids busy. Every five minutes or so I pull another new item out of my bag (courtesy of my cousin Leigh) in an effort to divert them from this little hospital room. Sam recognizes this hospital, remembering the blood draw from the endocrinologist, and is fussy for the first doctor's visit ever. It hits me that Sam is learning about doctors and hospitals, and that it's not all good. I feel a sense of loss for him.
Dr. Lin knows a lot about us already. She tells us she has been studying Sam for three months, and knows my father-in-law has had his knee replaced and that my mother has had her hip replaced. She has studied all of Sam's medical records but wants to get the human Mom perspective from me.

I am starting to get nervous about all her knowledge, though I'm visibly impressed at the same time. I hear a panicky voice in my head say, "But Dr. Albright didn't think she would find anything!" Somehow my brain had turned that into, "Nobody will find anything here, you'll just get Sam's surgery to correct the Coxa Vara and he'll be fine from then on.."

My brain is still shouting at me when Dr. Lin eases me into what she believes Sam has. My rendition will be much more abrupt and less gentle than hers, just like Dr. Albright's, since it is truly a gift to be able to present this kind of news to parents in an easy and gentle way (and both of them really have).

"Metaphyseal condrodysplasia, Schmid type."

So, if you're like me, this means nothing to you. At least, if you are like me a few weeks ago, this means nothing to you.

Dr. Lin follows these words with:

"It's related to dwarfism."

I can't comprehend what I'm hearing. My brain won't process it

I want to know what it means for surgeries, how we find out, what it means in life.... She doesn't have all the answers, but I have many new questions for Dr. Albright.

"You're probably going to ask me about height," Dr. Lin continues.

"I wasn't even thinking of that," I say.

"You will," she assures me, and I sit blinking up at her. "His final height will be between four and a half feet and five and a half feet."

In the big scheme of things, this isn't that short, she tells me, especially given I'm sitting next to my 4-foot, 11-inch mother-in-law, who does not have any form of dwarfism.

This was not genetically passed, but was a genetic fluke at conception. I'm relieved to hear this because I don't want any family members, least of all my husband and sweet mother-in-law, to feel at fault. I again ask about genetics, because I'm in awe that nothing is linked, and Dr. Lin says, "Trust me." And I do. She has all kinds of degrees in this, and I know nothing apart from a genetics paper I did in the 6th grade on Gregor Mendel.

I ask her to spell metaphyseal chondrodysplasia, Schmid so I can go troll the Internet, but she tells me there isn't too much out there. It, of course, is very rare, only affecting about 200,000. She shows me a picture in a medical journal of a boy with the same condition, and I almost burst into tears. He looks so much like my little Sam, he could be my little Sam. He even has that magical grin that lights up the room.

It will be two months from this day until we get confirmation, but Dr. Lin assures me if he does not have Schmid type of metaphyseal chondrodysplasia, he likely has another one. Chris, a nurse, holds Sam for this blood draw and I'm immeasurably grateful. She does a much better job of keeping his eyes diverted, and I sing and dance like a fool. Maya doesn't quite know what to do, but watches with big round eyes.

Suddenly once again I find myself in uncharted territory. I know nothing about dwarfism. I don't know any little people. I come home and determine that the term midget is not appropriate or appreciated, ever. Chris tells me her mother has shrunk to 4-foot-9, and laughs that now she is a dwarf. I appreciate the humor, and the fact that we have so many small people in our family should make this easier for Sam (and society) to accept, Dr. Lin tells me. Chris is proof, along with her mother and all my other shorter friends, that great things can come in small packages.

But I'm still reeling. I am struggling with this shift. As hard as you try not to make assumptions about what your family will look like, you do. I always told myself whatever my kids wanted or were, would be great. I still think that, but never once was a little person a part of that picture. It had simply never occurred to me. Part of all of this is just shifting what I once had thought might be true into what IS.

I find myself wondering about Sam in school, and his social life. He could be 5'6" at the tall end if he has Schmid, 1/2-inch taller than me and only an inch shorter than his father, or he could wind up closer to 4'6" which I immediately quip -- 'That isn't so small for our family.' But in all actuality, that foot is a big difference in how Sam will interact logistically with the world and how he will socially connect with the world. There is a lot of discrimination out there for anybody who does not fit the status quo.

Of course, I adore him whether he never grows another inch or if he's 8 feet tall. How could anyone not adore him? He's magnetic. He's smart. He's so funny. He's sweet and affectionate and wonderful.

I wonder about Maya too. How will she cope with having a brother who looks different than the standard that has been set by society? She tells me that she wishes she had a physical therapist like Sam, and why wouldn't she? This cool lady comes once a week to play with her little brother. Maya is included, but not the focus and she's smart enough to pick up on that.

You see by this picture that Maya gets a kick out of Sam's PT too, and Nancy tries to incorporate things that will strengthen Maya's upper body since, like many girls, she is weaker in her arms than legs.

I worry Maya will grow up resenting us all for his disability that garnered him special attention. I don't tell her that she shouldn't wish that she had a PT too; instead I tell her, "That must be hard, huh, when Ms. Nancy comes to play with Sam?" She says it is, and I let her know she's done a great job playing that day too.

"I was so proud of your pirouettes over the blocks, and you are so awesome at helping your brother get stronger so he can do more things," I tell her.

This gets a smile from her, and I decide right there that she should be a part of the whole process in any way that makes her feel good. If she wants nothing to do with it, that is no problem. If she feels good helping him, and she really seems to, that's great too. No pressure, and no guilt for being jealous sometimes of the attention.

This is all in day One of hearing the word dwarfism related to my family.