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?

Where we are

Sam's cast came off on Friday. He is just miserable. He just wants me to hold him. He is whining and fussing. He is sad. It's worse now than right after the surgery.

I'm worried that he has an ear infection, or worse.

It's hard to comprehend how complete his misery is. He only wants me... He says daily: "I can't walk yet."

We tell him, "Soon, baby."

I don't want to eat, he tells me, after I make him his favorite dinner of quesadillas with cheddar and spinach and chicken with guacamole. His appetite has dwindled over the last few days since he's gotten his cast off. He told me today, "Mommy, I got my cast off, and it feels better." But still he won't really eat, and he's just so cranky. I'm taking him to the pediatrician tomorrow.

Hospital care pages. Catching up...

Mass General has a site called CarePages for people undergoing surgery to keep everyone up to date on progress. I thought about posting the first set of CarePage posts from Sam's first surgery -- a proximal femur osteotomy -- to correct his bilateral coxa vara, but felt it was too long. But because I've been so out of touch with my blog, I wanted to post this for those who might not have caught my hospital updates. Like the last time, it's hard to find time to blog with a 3-year-oldchild in a spica cast, and a 5-year-old child who isn't. The only links you'll find are in this introduction, but can link to information from older blogs if necessary.

Wednesday, April 7

Well, the time between Sam's cast coming off and right now has flown by. Sam got his cast off in just under four weeks, and began walking one week and four days after that. (The doctors said it would take around 3-4 weeks.)

He had a birthday on March 13, and now he's a big 3-year-old. Luckily, he's still a little young to quite get all of this.

He just went running by. He's getting faster. A few minutes ago he came jumping in. He was never able to jump at all before the first surgery.

We and the grandparents all chipped in for both kids' birthdays to buy them a playset that has a tall ladder/ rock-climbing wall and a big slide. We had a big party for the kids' birthdays (Maya turns five April 12, but we wanted Sam to be mobile for a party) and the slide and ladder were already set up. By the end of the party, Sam was scaling that thing in a second, screaming, "I'm doing it myself!" and hurling himself down the slide. His back was sore the next day since he can't really catch himself at the bottom, so the next day we arranged pillows at the bottom for him to topple onto. That worked.

He immediately began relying on his right leg the moment it would support his weight after the cast came off. This just reiterated that getting the surgery done was the right thing to do for him. He finally is starting to get the motion of a more typical walk in his right leg in the past few days, and I'm thrilled about that. It's taken this long A) due to the stiffness after being in the spica cast, and B) because he was getting used to his right leg now being about an inch longer than the left after they made that nearly right angle of his femur obtuse. His right leg used to be shorter, so he had to get used to the new lengths.

I hope that when they're both the same length, everything will be easier for him. It's just getting through the next month. Ugh. Mostly, knowing what's ahead and how to manage everything from pain to poop is a plus. But man, knowing what's ahead in terms of pain and dealing with that cast, and Sam's frustration, twists my guts in a knot.

So we get there tomorrow at 8 a.m. I have to make sure Sam's gut gets packed so full today that he still feels full when we wake him up at 6 a.m. to go to the hospital. I can't even remember where we go in the hospital. It all feels like a blur from the last time. Guess I should give them a ring...

Until tomorrow.....

What a boy

Posted Apr 8, 2010 8:31am

Sam's is the best mood.

After a few minutes of being upset this morning because he couldn't eat (we told him it would make his belly sick before the surgery) Sam snapped out of it and hasn't asked again for something to eat.

Then he told me, "OK. I'm ready for you to drive me to the hospital."

When we first got to the waiting room, he held his little arm up in sort of like a shrug, and said, "This is just a really hard day."

But he seems to be completely unafraid this time. He's obviously not looking forward to it, but I thought the fear would be even more intense for him this time because he knows what's coming. He seems to be more like us; much less afraid, just filled with a little more dread at knowing what's ahead.

Right now he's running around the waiting room, cracking up, with a huge green monster truck they have here. Daddy's playing with him too. He's doing awesome.

Doh! Spoke too soon. Dave and I are talking about coffee, and Sam wants to eat. (We're waiting on the coffee.) We told him he has to have surgery first, and he said, "Can I go that way? Can I run that way?" pointing to the staging area.

He wants to get the surgery so he can EAT.'

He's in..

Posted Apr 8, 2010 11:06am

Sam did really well, with the exception of getting him to breathe the anesthesia through the mask. I had to hold him for that, so it wasn't very pleasant, but we just kept telling him what a great job he was doing and then it kicked in, so we gave him a kiss and left.

We just got an update on the fishie beeper saying the procedure began about 10 minutes ago and everything is going well, Sam is doing fine.

Osteotomy is done, applying spica cast

Posted Apr 8, 2010 1:24pm

We just got an update saying Sam's surgery is done and they're putting the cast on. We convinced him to go with green cammo this time, so he can wear cammo like Granddad wore as a soldier. He liked that.

It will still probably be a while before we see him. I remember the cast taking quite a while last time.

Everything went well, so we're mostly out of the woods.

Some smiles are better than others

Posted Apr 8, 2010 2:10pm

I love to see Sam's surgeons faces walking in looking that happy. They said everything went smoothly, he's in the cast, and they're waking him up so we should see him soon!

YAAAAAY!

Now comes the next hard part... But I'm not going to think about that... I'm just going to ride this humongous high.

Love to you all, thanks for your support and positive energy and prayers.

In Sam's room

Posted Apr 8, 2010 6:27pm

He's sleeping peacefully. He was awake and in pretty good spirits before, and had half a small bottle of water and has had about two popsickles already.That's more than he had in two days last time. The nurses seem to be in love with him; many from post-op remembered him from last time. He was sweet and making jokes, but of course, is still in and out. He's assigned to an awesome nurse Celine, who made sure he got some valium so we don't have a repeat of the first night last time, where he was seizing and screaming in pain. They'll probably wake him up around 7 or so if he hadn't already gotten up for more morphine. I'd like to get some food in his belly! But sleep is good too.

Now I'm off to go get dinner while he's snoring.

Not the most restful night of sleep

Posted Apr 9, 2010 8:56am

Sam had a tough night last night, the kicker being a catheter and midnight because Sam's bladder was so full that he was crying out in pain. I heard his screams when she was putting it in all the way down the hall so I ran in there and helped keep him calm. Dave was already there, but I just had to run down there having never heard him scream like that.

So it was dripping out slowly, filling a huge bowl.

He was being such a good boy that Erin, the night nurse, was telling an aid to go find him a toy. "Something big," she said. About 10 minutes of dripping later, she asked him, "Does that feel better Sam?" He faintly said Mmhmm.

A couple minutes later he looks at the nurse and says weakly says, "Thank you."

"Oh my gosh! You're so sweet! You're the first to ever say thank you for a catheter! Hey Michelle can you hear me?"

*muffled* "Yeah!"

"He gets two toys. He just said thank you."

Waiting on Sam's bed were Lego Thomas the Tank Engine (Stanley) and a crazy Batman batcave that he can work with his new Batman helicopter. It was like Christmas.

So, the nurse practitioner is talking about sending him home today, and I, rousing from my 3-hour-ish nap, asked shouldn't he be eating and drinking and peeing first? She mentioned he'd had wet a big diaper (from IV fluids) had a few sips of smoothie, and I was still kind of like, but that's not really food....

So I told Dave I want his pain to be managed better -- so there's something between screaming and crying in pain and doped up daze -- and I want him at least eating and drinking.

So to me, I'd guess we're staying until tomorrow.

Sorry about all the typos, I've had no sleep and only a few sips of coffee, and I'm bed with Sam while he cuddles up with me. He doesn't want to be not touching me.

More pain

Posted Apr 9, 2010 11:05am

I wish we could make it go away.... the meds just aren't doing it. Sam is miserable. He does not want the TV on, he doesn't want to be played with, read to, spoken to, moved, eat, offered food, or anything else.

It just makes me feel so helpless when I can't help at all.

Yay!

Posted Apr 9, 2010 12:41pm

Well, after a very sound hour or so of sleep (which indicates better pain control -- they doubled his dose of Valium), Sam woke up as we were discussing the pain with the nurse practitioner and Dr. Albright just to prove me wrong. I've never been so happy to have him do that.

He asked for his drink right away, had a few bites of pizza, has had most of a smoothie since this morning, and is drinking some of Mommy's sizzie water. He wanted Thomas to be turned up (I snuck it on on while he was asleep, hoping it would just distract him when he got up and keep him calm enough to eat).

He is talking back to Thomas, and I got a little smile.

Hooray.

He seems to be sweating much less too, which leads me to believe those spasms are under control now.

He just smiled: "It's Thomas."

looks like we might be able to roll him down to the playroom in a bit and see if a change of scenery will help him further along in his progress.

Cristy rocks, and we're leaving!

Posted Apr 10, 2010 9:50am

Aunt Cristy came to see us last night. I love her so much I can't even stand it. First she made Sam smile and laugh, and brought him an Iron Man mask. Then, she took me to a pub for a few pints and some chowder and stuffed mushrooms and actually yummy quesadillas, which I tend to avoid ordering because they typically are about as pedestrian as what I do at home.

THEN, she swapped with Dave, taking him out for a pint or two and getting him the soup. It was actually good clam chowder. Not thickened w/ all that flour, and with actual fresh herbs.

This break from everything is so amazing. It's fine for Sam, he's happy as long as a Mommy or a Daddy is there. It's great for us, because you can't really sit still long while Sam's in his cast. More so in the hospital actually than at home, believe it or not, because somebody is bringing him food, taking it away, washing the dishes from the food, and emptying trash, sweeping, etc.

But, that being said, thankfully we're on our way out of here, but Sam is refusing to eat b/c his meds were an hour overdue. This is how it goes when we are just a little late. But mostly, we all slept well last night. A nurse came and woke Sam up to give him meds at 3 a.m., and we went right back to sleep .... well almost. He wanted a smoothie and we were out, so I went down the hall and found him one of his banana yogurts and mixed it with juice and water until it tasted right. he drank it all.

So about a half hour after the Codeine, and a bribe with the playroom, Sam ate a pancake, a bite or two of egg, and a bite or two of yogurt. I'm happy with that. So we're getting ready, and getting OUTTA HERE! Based on past experience it's not as quick of a process as we'd like, so we went ahead and ordered Sam lunch, but we're out. I get the sense from the staff that the sooner, the better. ;)