Snowboarding

This family has changed a lot in the last six months.

I didn't realize how things had been -- how not only the stress of all of the medical issues had affected us, but how the changes we made as a result of them were part of our daily lives.

I suppose you don't give that weight in the moment, because these are your children and you have to be on point and strong. If you're sitting around thinking about how hard you've got it, you really can't do that.

But yes, even this has some stress consequences. It can put strain on a marriage, on friendships and on relationships with family members. I think when people are tied to a certain situation it can become heavy. You acknowledge that and move on, you can't dwell on it.

But another nuance that I'm just coming to realize is how changing your lives and interactions with the world affects you too.

We move in a manner that seeks to protect our babies, so we tend not to think about how our own lives might be impacted in moments during that singular focus.

But then we are given, if we are lucky (like I am) these moments, a glimpse of not having those concerns, a taste of what others might take for granted. We get to feel the freedom in actual mobility. As an active family of hikers, climbers, campers, snowboarders -- in a nutshell, thrillseekers -- I can't emphasize enough how liberating it has been to feel like now our whole family can participate in these activities together. All of us.

For about a year and a half, we avoided doing anything that required too much physical activity. I didn't want Sam feeling badly that he couldn't do things. I didn't want Maya becoming frustrated that we had to move a little more slowly. I didn't want to feel myself to become frustrated by having to carry Sam on a long hike through the snow, when the poor kid just couldn't walk in even an inch or two of the stuff for any distance, even on flat terrain.

As active as I am myself, I didn't let my brain venture into how this affected us all. You just can't do that when you're adjusting your family in such a drastic way out of necessity.

So when you suddenly realize, you can now do these thrillseeking sorts of things that release all kinds of endorphins to your brain, it's pretty exciting.

Lately, we all go sledding. In a big way. What would have been impossible last year, on so many levels, was not only doable this year, we did it in a pretty extreme way.

Last year, let's keep in mind, was before Sam's first orthopedic surgery, when Coxa Vara dominated the bones in both femurs. Two major surgeries later, two body casts, and the little guy is invincible. He's only 3.5, and he soars down huge slopes, flies over big jumps and laughs the whole way down. I really should video it and put it on YouTube. It's hilarious.

Then, he would climb back up these really steep hills in a foot of powdery snow, or even when the sun made it heavy and slick. And he would do it over and over and over again, just as many times as the rest of us. A couple times I heard kids much bigger than him complaining about coming back up, but never once a peep out of Sam. The other kids gravitated to him instantly, and before I knew it some big girls were pulling his saucer back up for him, realizing he was probably too small to make it up with the saucer in tow.

He really helps remind me what life should be about.

I had told my husband several years ago that I thought I was too old to learn to snowboard. I knew I really wasn't, but I also knew it would hurt much more at age 30 than it would have at age 20. Seeing Sam take his surgeries in stride, seeing him physically dominate much older kids on that slope, I decided that I will learn to snowboard. Next winter, I hope we can go spend a week in a cozy little place and just snowboard, cook, and drink hot chocolate. We'll go camping next summer, and big hiking, and swimming too.

I was reminded of how much of my happiness relies on rigorous physical movement, but not at the gym, in life, having fun. I need endorphins, and I just can't get them going on a piece of nautilus. I need speed, excitement, maybe a little bit of the risk that comes with knowing, yes, you can get hurt.

This has been an incredible winter.

We're going to continue it until Sam gets his surgery next week, and then we'll chill for a bit, let him recoup. We're all dreading this one much less knowing we won't have to suffer through the body cast. I know he'll listen when we tell him to take it easy. Until we tell him he can GO. And I know he will, probably even more extremely since he won't have his abductor muscles rubbing up against the metal in his bones. (I'd link to an image of that metal here if I could, but would have to scan Sam's X-rays, because the technology is so new.)

And after that ..... I don't know ... surfing? Climbing? Hiking? Camping? Repelling?

Turns out, he's just like the three of us, it's just that his bones denied him access. Well, now we have access. This thrills me so much I can't even put words on it.

Here we come.

URL change

To all who have this bookmarked, please note the URL change.

It's now SamandMaya.blogspot.com.

Sam's next surgery will be Jan. 20. A new post is coming soon.

Stone fish

Sam pressed his hands against the glass, peering through intently. A little girl next to him glanced nervously back at her mother, who watched with knitted brows. Sam's concentration broke only for a minute, just long enough to send a Transformer car down the ledge he was kneeling on, complete with throaty race car sound effects. Then he glanced up through the window, making sure he didn't miss anything.

The veterinarians and assistants gathered around the tiny creature on the table.

"What's wrong with it? What's wrong with the fish?" Sam asked matter-of-factly.

"They don't know honey," I told him. "That's why they're doing surgery on her, to see what's wrong and make her all better."

"Those doctors are going to make her better?" he asked.

"They're going to try," I said. "It's different than when you had surgery. Then, the doctors knew exactly what was going on and what they wanted to fix. This is a kind of surgery where they don't know what's the matter. But they're going to try to make her all better."

The little girl next to Sam looked at him in horror.

"It's OK," he told her. "The fish will get to go home after a few days in the hospital."

This all unfolded at the New England Aquarium in the room where they treat sick animals. We've seen all kinds of creatures being treated in there -- turtles, rays, sea stars -- but this was our first surgery. I was glad Maya wasn't around. I didn't think she'd appreciate the neat square cut into the fish's flesh with a scalpel, or the tweezers being inserted inside.

Most of the kids didn't appreciate it, actually.

But Sam was fascinated.

"Why are they covering it with that plastic?"

"I really don't know. Maybe to help the fish stay wet," I guessed. (Assistants kept wetting her down with an anesthesia-laced liquid.) "Maybe to keep as many germs out as possible. Any incision has to stay clean."

The mother next to me saw the fish twitch.

"It needs more anesthesia!" she blurted out, her face pained.

"Actually, movement doesn't necessarily mean it's wearing off," I told her gently. "They have to be really careful not to over-anesthesize a creature so small. That could hurt her even more." (My way to avoid saying "kill her" since that concept might be slightly terrifying for the 3-year-old slated to have a third surgery in January.)

Her daughter told her she was ready to go, and the mother chucked her in the stroller and hurried off without a second of hesitation.

I sort of relished the fact that Sam and I were the weirdos at the aquarium that knew a creepy amount about surgeries on small creatures. If you're going to get all the experience, you might as well get a kick out of freaking out the people with none at all. I mean, we'd all prefer ignorance on this, right? And actually, I was trying to comfort her. But I think she was made nervous by these people who seemed to be showing no concern for the fish, and almost a detached voyeuristic interest. All science, no emotion.

But hell, it's a fish. I thought it was great they were performing surgery on a small stone fish. Would anyone have noticed if they'd just shuffled it off to the side, never to be seen again? At least they were trying to help her. And when you've watched your child go through much more intense surgeries, it becomes a clear where a fish ranks, even for an animal lover like me. Even with your kids, you do your best to become detached. The only way to make it through is to view surgeries as much like a scientist as possible.

And as a scientist, I would say these surgeries have been the most successful surgeries in history. Okay, maybe that's the emotional point of view, but they have really exceeded my expectations in terms of what Sam is now able to do. Medically, performing these surgeries at such a young age was exactly the right thing to do for him. How validating when you have chosen "elective surgery" not even for yourself, but for your toddler. Yes, the pediatric orthopedist recommended it, but the struggle involved in making that choice for your toddler is pretty excruciating.

For the first time since launching this blog, I hadn't gone back and looked through the whole thing until I began changing all the names. (I'm sure I've missed some identifying factors, and I'm still not sure how I'm going to handle this through Facebook. As always, I'm just winging it.)

It was amazing to me, reading about Sam almost as if he were someone else. I've almost forgotten how hard it was for him to get around just one year ago, before he had even had his first surgery. He still doesn't do everything the other kids are doing, but he is nearly indistinguishable. If you didn't know there was an mobility issue, you might not even pick up on it.

He's jogging/running without a swagger. He's not winning many races, but he's running and he's thrilled. He can now walk a balance beam, where as before his stance was far too wide to manage it. He whips right down slides and can land squarely on both feet at the bottom, whereas he used to topple over since his legs couldn't ground him at that velocity.

He no longer has coxa vara, and might never again, according to Dr. Matthew Warman, the geneticist who discovered the gene that causes metaphyseal chondrodysplasia, Schmid type. In fact, I should recount this conversation. I've given a few of you some accounts, but if I type it, I'll be more thorough. (Just ask the man who edits my 4,000-word boating industry analysis articles.) And it will help me process it all again.

Dr. Warman discovered the gene that causes Schmid, a type X collagen mutation. (Hence why his daddy calls Sam an X-Man, and being all about superheroes now, Sam loves that.)

Sam might not ever need another surgery following the one to remove his metal this winter. I'm trying not to hang too much on that, since he might need another, or several. If he does, that would likely be for his knees, his wrists, or maybe his femurs again.

Dr. Warman used to believe that if Schmid kids lived on the moon, or anywhere else without gravity, they would never get coxa vara. I had also read that it tends to affect weight-bearing bones.

But now geneticists are trying to figure out if that's the case. There is increasing evidence that a protein in the growth plates of a person with Schmid would cause the problems regardless of gravity. In Dr. Warman's words, that causes those growth plates to be "a little bit sick." It might be that those proteins would cause the short limbs and bone deformities even if those bones never had to bear an ounce of weight.

Right now some geneticists are working on trying to heal those sick growth plates so that the bones will grow and develop normally. Dr. Kathy Cheah at University of Hong Kong is working on this, and so are Dr. Mike Briggs and Professor Ray Boot-Handford at The University of Manchester.

The work hasn't progressed beyond mice at this point, but maybe in the generation of Sam's children this treatment will exist for people. That would be worthwhile for me. I wouldn't let Sam be a guinea pig, and I wouldn't consider growth hormones (if they were appropriate for Schmid, which they're not) or limb lengthening without a medical reason for doing it. However, something that would both enable Sam's child to grow typically and avoid all these surgeries would be pretty fantastic. That's assuming Sam A, has kids and B, they end up with Schmid too. They have a 50-50 chance provided Sam marries someone without a genetic form of dwarfism.

Another concern he addressed was regarding Maya. I'd mentioned Jim and I were planning to be tested just to make sure neither of us were latent carries of Schmid. We didn't care for us, but figured we should know for Maya.

This wouldn't be necessary for several reasons, Dr. Warman said. First of all, nobody would be a latent carrier. To have passed this to Sam would mean one of us had maybe 1 percent of genes affected, whereas Sam has 100 percent. That would make his probability, genetically, of getting Schmid the same as anyone who came from parents who didn't have any genes affected. Sounds crazy, but I have extensive notes on this in my effort to understand. Email if you'd like a copy.

The other, perhaps more compelling reason this is moot, is that within the next three years, Dr. Warman said we will have complete genetic sequencing fully accessible to us for a fairly nominal fee. But, just because we'll have the information doesn't mean we'll have any idea what to do with it. Which is why we're lucky it will be 20 years (at least!) before Maya's ready to have kids, because by then we should have them pretty well figured out.

In fact, people who have any sort of genetic risk (so, everyone) will get these sort of genetic maps and compare them together before having children if they're concerned.

"How romantic," I said dryly, and he quickly responded, just as dryly: "Yeah, really."

But it's pretty awesome. Science continues to astound me. I sometimes still wish I'd taken my career in that direction.

Arthritis will only be a problem for those who have endured longterm joint malalignment. Dr. Albright, Ewan's pediatric orthopedist, will be keeping his eye on that when he checks him for the rest of his growing life. (See why we can't leave Boston? Not that we were going to, but it's really out of the question.) Dr. Warman says the surgeries he's already had will keep those joints aligned the way they were meant to be. That could change, and if it did, future surgeries might be necessary.

Ewan's physical therapist has also said having the surgeries done earlier instead of later will help his overall body functionality, and minimize back problems from the swagger, and other bodily stresses from overexerting some muscles to compensate for the muscles that didn't work before the angles of the femurs were corrected.

I'm probably getting too technical and losing some people. But the very few people I've met who live with Schmid or loved ones with Schmid will likely be pretty interested. If you're not one of them and you're still reading, the technical stuff is pretty much over.

Socially, Dr. Warman said in his experience children seem to be fine until around age 12, when there is a more noticeable size difference. He said he would tell his patients to look around them though. Everyone was getting picked on for something, right?

Sam's life expectancy or life quality won't be any different from anyone, Dr. Warman said.

In essence, having Schmid doesn't make a person that much different from everyone else. I guess I knew that on some level, but it was nice to hear the guy who discovered the gene reiterating that fact. And we've been so caught up in the surgical components, and I don't want to minimize that, it's been hard to see the forest. But this sort of gave me a bird's eye view of Schmid for what feels like the first time.

My son will be small, but not as small as a person with achondroplasia, the most common form of dwarfism. If we need support it's there. Some of Dr. Warman's Schmid patients (or loved ones) have sought it, others have been more comfortable without. It's harder for him to get around than other kids, and I still bring the stroller pretty much everywhere so I won't have to carry him.

The surgical element might be almost done, but we won't know until time passes whether that's the case. And since Sam's been potty trained, he won't need a body cast this time. I'm trying not to get too excited about that one, but it's pretty hard. That cast is a pain, and it is literally painful for Sam. I'll be hypervigilant to make sure those bones are protected as they heal. One tiny sign of roughhousing, and I march the boy right in to get a brace fitted. That doesn't work, they put him in a cast that comes lower so he can still use the bathroom.

I keep forgetting to measure him so I can get back to Pass It On, based in Mashpee, Mass. The owner of this incredible nonprofit, which accepts medical equipment donations and finds people that will need them, is looking for something that will make it easier for him to go to the bathroom post-op. I've been meaning to do it for weeks and only remember when he's sleeping or in the few hours a week he's not home.

Today, I'm feeling on top of things. Sam is doing awesome, and the better he moves, the more I love his surgeons. And the more I learn from experts like Dr. Warman, the more confident I feel about what's ahead.

Anonymous

If there's one blog of mine to read as soon as it posts, it's this one.

Otherwise, you might be a little confused when you see not only has the name changed, but all the names have changed.

The people in this blog will become Maya and Sam. I will become Rachel and my husband will be Jim. Even the URL will change, if it's available, to MayaandSam.blogspot.com. My email address will stay the same so people will still be able to communicate with me. I have contemplated deleting it entirely, but selfishly, I'm just not ready to do that yet.

My son Sam (this will take some getting used to!) is almost three and a half. He's in preschool now, and Sam doesn't know that he has metaphyseal chondrodysplasia, Schmid type, a genetic disorder that is linked to dwarfism. He does know he had bilateral coxa vara, a rare bone condition that was brought on by Schmid. That he knows because he's had two major surgeries, and two osteotomies, each followed by a month in a spica cast.

Sam is starting to realize something though. He's a very smart 3.5-year-old. He sees the look of surprise people have when I tell them his age. He realizes many 2-year-olds are bigger than him. He refuses to let Maya refer to him as her little brother. Yesterday I found him a Batman cape, finally, because he'd been wanting a superhero cape for months. When I told him I needed to pin it up, he ripped the mask off and said, "I don't want my cape anymore. Take it off!" The only reason I could think of was that he was embarrassed about being what he perceives as too small, yet again.

He is also aware of his physical limitations. He knows most toddlers don't need help up and down regular-sized stairs (though he is getting so much better at this after his surgeries) and he sees them jumping off of tall things and landing on two feet. Sam doesn't even attempt this, but he is trying more and more to jump off small things to practice. He is doing so well, his jumping is getting better and better. Before his surgeries, Sam couldn't go down a slide, or if he did, he could not land on his feet. He's doing that now too post-surgery. But I think Sam just wants to be like everybody else.

It's this feeling that has led me to the sinking awareness that I should never have broadcast our real names. So as soon as I post this blog, I'm changing it all.

Sam's geneticist (all the medical names will still be true in hopes it will help others who might need expertise on these subjects) had told me it was important to protect his medical confidentiality, but I didn't listen. I don't know why I didn't listen; I think part of me just never expected Sam to become old enough to have an opinion about this. Or maybe it was just that I fully intended to raise him knowing about his genetic condition and have pride in who he is, just as he is. I still want to do this, but giving a 3-year-old this much information doesn't feel right either.

I've hit a wall again in knowing what to do, and as time passes I get more and more perplexed, to the point of nausea, about how to handle this.

I know I don't want him to hear about his situation second-hand.

I know I don't want to tell him about this too young and have him just be angry and never want to speak of it because he's embarrassed.

I definitely don't want to assume he will want this information out there.

I try to think back to 5-year-old Rachel, and how important size was then, and how everything was judged by how big you were and how fast you could run. I was the youngest in my family too. Adult Rachel often forgets how much that stung sometimes. Some of this is just knowing he is the youngest, and a normal part of that. But I am sensing that some of it is outside the realm of typical, and I always said I wouldn't just pretend all was typical when it's clearly not. That doesn't help anyone.

I know it's too late for some of you, but if the local people who do know our identities would keep this very discreet with your children, I'd really appreciate it. On the flip side, please let me know if people are very curious about his size. It's hard for me to gauge, other than the expressions I see people wear when I tell his age. At the public gardens the other day, I saw one man stare in shock as he heard Sam articulate something so brilliantly (he is verbal even for his age). I saw him nudge his spouse and ask incredulously, "Did you see that little guy talking?! That was amazing!" I don't know if Sam saw, but I'm sure he hears plenty of other comments and sees plenty of other stares.

And that makes my heart sink.

I suppose it's time to contact the LPA ... even if it's just for logistical answers, like, is there an implement that would allow him to turn the bathroom light on, for example, so we don't have to leave it on all the time.

My good friend on the phone last night, we'll call her Jill, said she just gives the talk about how people come in all shapes and sizes, and I love that talk. We have that talk often in our house. How much do you really say to a 3-year-old? Will Sam be angry with me for not telling him always? I just figured I'd raise the kids with the awareness. I go back to my adoption example. I always figured I'd always tell kids they were adopted (if I had adopted, which I didn't) so they didn't have the bomb dropped on them when they got older. But the way we're headed, there will be a bomb.

Will he be scared and shut down if I tell him this young? Will he be relieved to know why some things come harder for him than others?

Right now his favorite book (one I need to buy, we got it at the library) is Giraffes Can't Dance.

Gerald the Giraffe gets laughed at by the other animals when he attends a dance. Gerald has a hard time, he tends to trip over his own feet. Sam falls down a lot too. Gerald can't dance like the others, who all have their own special wonderful dance. They jeer at him until he gives up and leaves the dance.

He's feeling pretty bad when a cricket comes and tells him that he can dance if he finds his own song, and stops trying to dance to everyone else's. Gerald listens to the breeze in the grass, and the cricket's song, and begins to move his own way. The animals gather around him and cheer at the most beautiful dance they've ever seen.

Every time we read it, Sam lights up, and I try not to cry.

Clueless

It is finally sinking in that our lives will be different.

I've tried so hard to keep a positive attitude that I don't think I've acknowledged the reality of our situation. I'm working on doing that these days, but I'm back to the place where I don't know how much to tell Sam at age three.

He understands so much more than he did, now he is aware that he can't run as fast as the other kids (or really run at all yet) and one day, frustrated from never ever winning a race, he began bawling. "Mommy, why don't I ever winned the race?!" I picked him up, murmured that he wasn't as big as the other kids, and that I know, it's hard not to win sometimes. Right now he still wants me to scoop him up in my arms and kiss his fat cheeks, but I'm wondering about later.....

When something like this happens in your family, at least for me, you just spend all your time thinking, "OK, whatever we have to do to make things right, he'll be fine, everything will be OK....". He's not that much smaller than the kids his age, maybe we don't even have to approach the whole Schmid thing, and talk to him about dwarfism. But that doesn't feel right either. On the one hand, I don't ever want him to know he's different. But even more, I want him to be proud of who he is and understand why things are more difficult for him than for others.

He already knows something. Then there's this blog, where I've just aired his whole medical history. I'm back to wondering if that hasn't been such a good idea. Maybe I should go back, change the name. But I guess it's probably too late for that too.

Next year he's going to preschool, and I will have to bring him with a step stool. I have to put a hook in the bathroom today so he can reach the hand towels.

He wants to be independent, and still relies so heavily on adults for assistance, going up and down stairs, getting up on a swing...

He's nearly 3 and a half years old, and all these things that you take for granted, he really struggles with. Don't get me wrong, he's doing great, he has well surpassed doctor expectations with his recovery, and we keep focused on that in this house.

But reality is setting in for me. Finally. My 3-year-old boy can't climb stairs without help. He can't run. He will do these things, I'm confident, but it pays for me to acknowledge how tough this must be for him. I don't want to give it too much weight, I don't want him thinking he CAN'T do this stuff, but I also need to recognize what is real for him.

I don't want the kids to pick on him, but am unsure whether he will want the attention of me putting a flyer in everyone's cubbie, a suggestion on the Little People of America's Web site. I thought it was a good idea.... it would help the kids to understand that he's a bit smaller, but they should not pick him up and that after the winter, he'll be coming to school in a body cast, and will have to relearn to walk after that.

It's so hard because he's in between. He's not, actually, a little person. He is small, but he's not that small ... I really want him to be proud of just the way he is, because really, he's perfect. I probably should write a letter to send home with parents. But then, I want him to have the option of telling people himself if he wants. Then again, I need him to know that being small is wonderful, even if our culture places so much value on height in boys and men. But I'm sensitive to the fact that he might not necessarily feel as if he belongs in either world.

What do we do?

I have no idea, I just fluctuate all the time. It's been so much easier to deal with the day-to-day and not think about his future. But here he is, a smart, bright 3-year-old who will get that he is different. He will recognize this, and if he doesn't, another kid will surely point it out to him. Even I am just coming to terms with his differences. You want so badly as a parent to think there's "nothing wrong," but I'm finally accepting, yes, he has a genetic disorder that leads to bone abnormalities, making it more difficult for him to climb and run. This is our reality.

Just the other day, Maya told him he was a little person. I think that's the term she used, which I found interesting because I don't know that we've ever used that term in front of her. I think she was just trying to convey that he was her little brother.

He got really upset, shouting, "I'm not a little person! I'm a BIG BOY!"

I told him that was true, he was a big boy, but that he was her little brother and he would always be her little brother. I told him that I was Uncle Scott's little sister, and no matter how old I get, Scott will still be my big brother. I think he got that. I also said that people come in all shapes and sizes and colors. You know the talk. The acceptance talk in terms a 3-year-old can understand.

The thing is this, how can you help a 3-year-old be proud of who he is, when success at age 3 is measured by who is fastest and who is the biggest? Maybe it's best not to drop a big bomb on him so young .... just kind of gently fill him in as time goes on. I don't want to be deceptive. But then again, he's only three...

And, if I let him in on his real physical limitations right now, will that make him think he is limited always? Will he think that he can't play soccer, which he desperately wants to do? Do I sign him up for soccer if he insists on playing, knowing fully well he can't keep up with the other kids and it might cause him embarrassment and frustration?

I know I have to keep him in activities he can be successful at now, but what are those for a 3-year-old boy? So much of those revolve around physical capability, or at least assume that basic physical motor skills are available. I can sense from Sam that he does not want to think he is limited in any way. We do lots of art projects and have instruments.

Yesterday I mentioned as I watched World Cup soccer (replay) with the kids that I am not a fast runner. I am not a fast runner. But I have endurance, I told Maya and Sam. I can run a lot longer than many people, but I can't sprint fast.

Maya said, "I'm a fast runner!" and of course Sam said, "I'm a fast runner!"

In his little mind, he IS a fast runner, and is it so wrong for me to want to let him believe that? It might actually be wrong, since like I said, some kid will point out to him that he is not a fast runner.

But if he believes he's a fast runner, and wants to become a fast runner, maybe he will. If I tell him he has this condition, will he think he can't ever run fast? Who am I to say he isn't fast?

I don't really want to limit my little boy that way. But I also don't want to ignore what I know.

And on top of all these questions I'm asking myself, Maya had another seizure, for 15 minutes. Her cardiology tests came back fine, so we still think this is something to do with the wiring in her brain, but we're now faced with medication. After four seizures, it's time. I don't want her to have one in class, I think she'll be mortified and scared, and worse, I think the kids will be afraid.

If it changes her at all, we can just take her off of it. It's not like she'll be on it forever. We're still hoping she'll outgrow this, whatever it is. But having ruled out other possible causes, if it is a glitch in the brain wiring, medicine would probably help everything fire correctly. (I should probably note, we feel strongly that we need to make the decision involving Maya and medication ourselves since we have probably educated ourselves more than most on Maya's specific situation.)

I wish it were that easy with the questions I have about Sam's issues.

The questions I have about him are much less scientific though, and much more nebulous. If we make the wrong choice, it's not like we can just erase what we've already said or done -- just change our minds again, say oops, and move on. So I'm probably not actually asking for feedback* as much as I'm finally putting this out there: I have no idea what the hell I'm doing.

* If anyone who has gone through something similar has any ideas, please feel free so share. I can always put my email address in a comment and delete it if you want to stay anonymous on my site.

Hollow bones

“I wish I could see Mr. Sun, or, Father Sun

I wish I had hollow bones

so I could fly in the air

next to him

I want to be a bird

I want to be iridescent

so I can be a sparkle

in the sky”

• Maya, June 8, 2010

I wonder why it seems I hardly ever get my happy blog out.

Sam has been walking for weeks, beginning two weeks from when he got his spica cast off in the ER. He’s doing really well. That’s what Dr. Albright said when we went for X-rays at Mass General. He is way ahead of schedule.

“He’s got good bones,” Dr. Albright told me.

He also told me that when he goes in to remove the metal brackets in each leg -- which were added after each surgery to correct coxa vara on both sides -- he will want to put Sam in a full spica cast just to be safe. For four weeks.

We’re looking at January for Sam's third surgery, one year after the first surgery on his right leg.

Sam in his last spica cast, known as a "one and a half" since only one side went down to his ankle. The next cast will be a "full," as both sides will go down to both ankles. Maya, Sam, Dave (not pictured) and I had fun at this wheelchair-accessible playground in Sudbury.

That was quite a blow, though I have to say it was a tiny bit of a relief too. Dr. Albright had told us in the Newton-Wellesley ER when he came to personally remove Sam’s cast on a late April Friday night that we’d have to keep him off his feet for six or eight weeks.

“How do you do that with a 3-year-old?” I remember asking.

I don’t know for sure, but I think when he’s looking at the size of Sam’s bones at each X-ray, he is considering how small they are and how big the metal is. The brackets will leave some big holes in those bones, and one fall, or one kid falling on top of him, could break it and would require another surgery and more titanium.

And obviously, Sam is immune to pain and sitting still, even after two major surgeries and weeks in two body casts. He just is determined to become mobile again the second those casts comes off.

Dr. Albright wants to be safe.

So I showed Sam his bones on the screen. I showed him the before picture, where his femurs are angled severely. In the second X-ray, I showed him how much straighter his legs were, with a ‘typical’ angle, and pointed out the metal. (Unless I take a picture of our X-ray printouts, there's no way to show an example. I think the technology is that new, and the condition is that rare.)

“That’s really cool!” Sam said.

I agreed, it was cool, and reminded him how much like an X-Man he is.

I figured it was as good a time as any to tell him what was ahead, since at least he had a visual and could understand.

“Buddy, I know I told you that you wouldn’t have to be in a cast again, but Dr. Albright has to take that metal back out, and just to be safe and keep those legs strong, he’s going to go ahead and put you in another spica cast again. OK?”

“Alright,” he said, rather brightly actually. “Is that today?”

“Nope, not till winter,” I told him, making sure to match my tone to his.

“OK! .... Look! That’s my BONES!”

It didn’t even phase him.

On the way home, we listened to The Pixies (Doolittle) really loud and I cried a little behind my sunglasses, just knowing we had another one ahead, even though I think the reasons are good. (As loud as hell/ a ringing bell/ behind my smile/ it shakes my teeth...)

Then I’d turn to look at Sam, and he would give me this huge, lit-up grin, like nothing bad was in his world. Nothing bad.... He’s such an amazing kid.

Maybe I haven’t been taking this for what it’s worth. It’s been easier to just live and not acknowledge the day-to-day differences of our lives.

But grasping that this isn’t just one isolated surgery, it’s repeated surgeries, has created the dawning that there are certain things I have to address. We can't deal with the surgeries, and put off dealing with the differences in our lives later. This is just a way of life now, it’s not a fluke. I feel like Maya ... I want to have hollow bones.

But all my bones are so dense that I feel every little twinge way down in the core of them all, in each crevice of each bone in each piece of cartilage in each little appendage. I weigh about 1,000 lbs. I just think about potential pain for him, and feel the old ulcer (I think) twitch. (Whatever it is, hopefully Monday's endoscopy will bring some results.)

Until yesterday, I hadn’t broken down in a while.

My close friends in Ulster County talked to me about this, said this might be the main cause of my stomach distress, and I agree. There hasn’t been an outlet. I explained, that you really have to be on for those kids, even when there are worlds behind your eyes shattering, you have to look solidly ahead, for them, and nod calmly, take a few notes, give your kids a book to read while you listen to the doctor, talk in chipper voices when they’re screaming in terror or pain. That’s just the way it is.

I suppose, as I was told last weekend, I have to find more time for myself to break down, and thank you friends and mountains for tapping into that quiet place that knows what is best for my self ... it’s why I could allow myself cry in the car. Sam had no inkling; I felt better than if I hadn’t.

I think shoving all this down has kept me from realizing some big reality in our future too -- if Sam is going to live as a typical kid his age, we are going to have to tinker with things to see to it that he can interact with the world the way a typical 3-year-old should.

The surgeries have put him up near the first percentile for height, but until then his growth had continued to decline below the charts. It makes sense; his skeletal dysplasia (Metaphyseal Chondrodysplasia, Schmid type) is associated with dwarfism, though his projected height is between 4.5 and 5.5 feet.

We’re going to have to find creative ways to rig the slider so Sam can reach it until he’s tall enough. I have some ideas about that.

We have to put a low towel rack in the bathroom so he can dry his hands, and we have to add height to his stools so he can do things independently, like turn the water on to wash his hands or reach countertops. We need more step stools, some with two or even three steps.

I’m even thinking of reducing the heights of stairs out front so he can move up and down them without using his knees. Now he either has to get wet if it’s raining, or I have to carry him. Typically, I carry him.

He wants to do things himself, and if his world were geared just a touch smaller, he could. I want to empower him to not need me. As much as I love him trusting me to be there for him, I have to make this all work for him.

If we can give him the tools he needs to be independent as his age would dictate, he might not notice for a while that he’s smaller than many others. It’s not that I don’t want to celebrate his differences, I do, I just don’t want him to realize obstacles as a result of them. I definitely don’t want him to get hung up on any of those obstacles should they occur.

Really, he is such a happy kid. Right now he doesn’t notice that kids are taller than him, partly because he’s usually the youngest in any given situation. He and Maya are both so extraordinary. I would even say they sparkle, though it worked better in Maya’s poem than it does here. What can I say? My 5-year-old already writes better than I do.

Old pros

We’re getting a little tired.

Sam is trying so hard, and he’s been hurting so badly. We got his spica cast off two weeks ago today at the ER. Dave and I were supposed to have a date, but instead we took our little boy to the emergency room. His orthopedist met us there (I LOVE HIM!) and removed his cast. Sam screamed complete terror and pain, and I held him, and held it all together, and so did Jim.

“You’re a good boy,” I said cheerfully, hugging him as he bawled and howled. “It’s OK! It’s almost off, it won’t hurt you, you’ve done it before, you’re almost there, you’re so strong, you’re so brave, I love you so much, I’m going to give you a huge present tomorrow, and guess what? Uncle Larry and Aunt Cristy and Jack are coming tomorrow!”

It was an steady babble of anything I could think of to calm him down.

After ten minutes or so, before the air hit his body, he actually did get distracted by the idea of the Beemer visit, and by a Child Life specialist who brought in a noisy Thomas book. (I also love those Child Life specialists. Twice they’ve saved us in the Newton-Wellesley Emergency Room, once with each child. Twice the same specialist saved us at Mass General as we strolled Sam into surgery.)

We’re used to this now. We’re used to a toddler screaming in pain and terror, and I’m comfortable now doing my song and dance to make him feel as comfy as possible after his sixth round of X-rays at 10 p.m. while he’s hurting and just wants to go to bed. Now it’s not even an effort to hold the tears back, now they just don’t come because we know that being strong is the best for Sam. If we trick even ourselves into thinking this is all OK, yeah, it’s tough, but we can do this, it's bound to make him more at ease. And we can do this.

Before we went to the ER that night, Maya started to cry.

“Are you going to be gone a week again?” she wanted to know.

We hugged her, and told her no, we’d be there in the morning when she woke up. Thank God Chris was in town, or she would’ve had to accompany us on this late-night excursion. It tugged when she asked that question, but I didn't have to fight any tears.

In the ER, the X-ray attendant told me how helpful it was to have me in there, when many moms exacerbate the kids’ fears. “We’re old pros,” I told her.

I'm toughened on one hand, so it's easier to keep it all contained. But I am at the same time all raw and exposed. I’m like a crab in its shell and at the same time right after it sheds, simultaneously hardened and ready to do whatever it takes to make it easier for my kids, while also weak and vulnerable, with fleshy exposed parts that could be fatal if somebody so much as scratched them.

Just seeing Sam crawl around tonight, so frustrated, but so determined, wore me down. He’s such a great kid, and it’s getting hard to see him so frustrated by the pain and the inability to walk for a second time.

He was shouting angrily from the couch this morning, no he didn't want to go outside. He wanted to just sit on the couch. I finally gave him the option to take Tylenol himself or I'd hold him and give it. He took it, then just looked at me and said, "Mommy, I still can't walk."

"I know buddy, but you will soon. You're getting stronger, and I know you're sore from practicing so much yesterday. Soon. Just keep trying, but if it hurts, rest."

He's been so fussy the past two weeks. Of course I’ve had deadlines, and a work error that more took work to correct, and plenty of worry on my part. Dave has had a tough couple of work weeks too.

Sam wants me near him always, he wants to be held. He tries to strengthen his muscles, and last week began crying when I told him to take it easy as he tried to jump holding on to his spica chair. He thought I was chiding him, when he expected me to be so proud.

I am proud, but that cast came off really early after his second surgery, a proximal femur osteotomy on his left leg this time, to correct the coxa vara on this side. Four days made such a difference, and he was in such pain. He began crawling less than 48 hours after the removal, and cried for a week as a result, but refused medicine and refused to admit anything was wrong. He had a canker sore, and stopped eating. (But at least we figured out why his appetite had dwindled, and it's on the rebound.) He fell yesterday and hurt his hand. Still, he refused Tylenol. Today I didn't give him the option.

But at this point, we have got to be on our way to coasting. I keep thinking we'll turn that corner every day. Today has got to be the day, and it's promising thus far. Sam's playing, the medicine helped, and he continues to work his legs.

When taking the kids to drop Maya off at preschool on Tuesday, one mom said, “You guys are always smiling! You’re so happy! After all you’ve been through. You’re awesome! You’re such a great mom.”

This comment couldn't have come at a better time. Maybe she sensed that we were all wearing a little thin. Or maybe we just all genuinely seem happy. I hope that was it. Basically, I think we are happy. Just a little thin.,

Her comment was so appreciated at that moment, I had to measure myself to make sure my emotions went one way instead of another. I could've beamed with pride, or just as easily wept.

Being able to control which emotion shows itself has gotten easier, but the ability to do this has taken its toll too. I thanked her, and told her this compliment came at a crucial time.

“At least I’m faking it well!” I joked. She told me if I needed help, she'd be there.

I’m sure Sam feels me getting worn down, and I’m trying hard to stay strong so he can feel comforted by me instead of uneasy.

He’s always sending repulser blasts everybody’s way and saying he’s bad. “Sam can you pick that up please?” (Repulser blast noise and hand.) It’s funny now, but his whole attitude seems different. Maybe it’s just because he’s three. I do know they get possessed around this age. But I also think he’s finally frustrated by his situation and lack of mobility.

Sam’s cruising now, and last time, it made me so happy to see him hit this milestone again. It still makes me proud, and I’m encouraging him more now than last week. But in all honesty, seeing it a third time when he’s three years old hurts.

Knowing that he will, as Dr. Albright finally prepped us two weeks ago, be immobilized yet again when they remove the metal from his legs, has me down. He won’t be in a body cast, but it’s six weeks of little movement, no running, jumping, climbing. I’m not sure how you tell that to a 3-year-old boy. We will have to stay in the hospital overnight again.

Maya wants to go to playgrounds, but I just feel too awful bringing Sam there when he can’t even walk. I feel awful for Maya, who just wants to play as we normally would on a lovely spring day. We go outside and I'm CONSTANTLY working to find something fun they both can do.

Yesterday I pulled their play kitchen out of the garage and cleaned it, and their shopping cart filled with play food, and put it into the “cafe” part of the swing set Dave built. I opened some sparkly cider and we sipped it out of plastic cups while Sam and Maya prepared me an array of foods at their cafe. We must have done this at least for an hour.

I dropped $40 on new games at Target, money well spent just because it was something engaging we all could do together.

But still, they’re watching too much TV and I’m not engaging and playing the way that I want to. I’m trying now to stop the guilt by changing our habit of flipping on the TV after a moment of frustration from any of us. We’re reading more. My house is filthy though, and we rarely eat before 7:30.

Like I've said a million times, it could be so much worse. But right now, I fluctuate between being proactive and strong to being a sludgy stagnant puddle. More than ever before in my life, I lack motivation when I need it most, and I just want to withdraw.

But then Sam kisses my finger, holds on to me, tells me I’m such a sweet mommy. Tells me I’m a precious mommy. Maya smiles at me, kisses me, tells me I'm the best mommy in the whole world, and also figures out a new way to race so a crawling Sam can tie with her each time.

“We both won!” she’ll shout, and he laughs, so proud and thrilled.

They make it impossible to withdraw, or to stop feeling altogether.

They are so funny and awesome, it helps, and makes me feel kind of guilty even admitting to all this. Sometimes I don’t even want to write this down and share it with the world because I don’t want to feel like I’m complaining. But that’s exactly what I’m doing.

Or maybe it's not complaining, so much as venting. I know other parents have to feel this way, even if the challenges are different. I had put a status update on Facebook exalting Sam's first post-cast bath. He had been so happy. One mom commented how strong and brave we all are (I totally feel like a fraud when people say this) and how she shouldn't take something as simple as bathing her children for granted.

To which I responded, "We're not saints, we'll be taking it for granted in two weeks when Sam is kicking and flailing and doesn't want it ... it's what I tell people who think I'm doing a special or good job ... the reality shifts, and then it shifts back. Each reality comes with challenges. None is bigger than another."

Everyone goes through their parenthood challenges, and maybe it's not as obvious or overt as this one, but there's always something.

I think it comes down to this: I know I have to be strong for my family, but I get worn down seeing their frustration and pain. I try to keep things light when they’ve just had it, but sometimes I feel like I’ve had it too ... we’ve been so resilient to this point, but all are beginning to wear thin.

Knowing there’s more to come in the six months just when we thought we were all done, a whole new round of surgery, another hospital stay, more immobilization .... I’m going to try not to think about it yet. I knew the metal had to be removed, but I guess I hadn’t considered the magnitude of that surgery.

I think if Dave and I could just get out to a movie and dinner to recharge, to actually talk, it would help. Anyone know a good babysitter who doesn’t charge money?