Greeting the world

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

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

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

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

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

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

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

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


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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

Just smile and say nothing.

Confirmed

It's official.

Sam definitely has metaphyseal chondrodysplasia, Schmid type.

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

Dwarfism

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

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

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

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

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

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

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

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

"Metaphyseal condrodysplasia, Schmid type."

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

Dr. Lin follows these words with:

"It's related to dwarfism."

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

The Followup and Physical Therapy

It already feels like forever ago, though it's only been two weeks.

We returned to Dr. Albright, Sam's pediatric orthopedist, about three months after the initial Coxa Vara diagnosis.

I had prepared myself for either of the two possible outcomes: Bone self-correction or surgery. Looking at my son, I did not think his bones were changing, despite his tremendous physical accomplishments, and had braced myself for the recommendation for immediate surgery.

I didn't realize there was a choice C.

I'm getting ahead of myself though. I should recap what happened between those two appointments.

I was lucky enough to have heard about Early Intervention, and asked for a phone number at Sam's 2-year checkup so I could set up an assessment. It took about a month for a team of E.I. specialists, comprised of an occupational therapist, speech therapist and a physical therapist, to come to the house and test Sam. He was off the charts in almost every area, like verbal, problem solving and fine motor skills.

At 2 years-old, he tested at 16 months for gross motor skills, landing him in the Early Intervention program. A physical therapist would be in touch with us soon to set up our schedule. I was thrilled and relieved we were going to get some help, and since E.I. is largely funded federally, it wouldn't cost us much or our insurance anything.

The physical therapist, Nancy, has done amazing things for Sam. She pointed out right away that he was limiting his upper body motions, probably because he found what worked and stuck with it since movement was so challenging for him.

He started climbing half steps with his feet. He began going up small slide stairs and going down on his belly. Before, he was unable to do slides at all, and had passed the phase of wanting Mommy's help. At that point, he would knee up the slide a step or two, and then come back down. Suddenly he was going down like a penguin.

Maya would cheer him on, saying, "You can do it Sam! Great job!" His sister's praise seemed to drive him even more.

Soon after, he began using his upper body strength like a gymnast to push himself up off the slide, with only his palms making contact as he worked his feet out in front of him and hurled himself down. He had a hard time landing at first, toppling over onto his head, but he learned to jerk sideways at the bottom so he would land on his butt instead. He would shout triumphantly, "My did it, Iya!" (Iya is Maya's nickname.)

He is constantly amazing us with how hard he works to reach physical goals he must be unwittingly setting for himself. He is so strong, and so determined.

About a month after the initial diagnosis of Coxa Vara, I took Sam to the endocrinologist. The blood draw was awful, and took a little rooting to tap his vein.

I will never forget the expression on his face when he turned around, his big confused eyes brimming with tears as he asked, "Mommy?" Why would the mommy who always protected and comforted him hold him down so strangers could hurt him with needles?

I cried, but kept singing to him. He does not like the Wheels on the Bus song anymore. The tests turned up nothing, which seemed to be good news. What had looked like Rickets was not. The endocrinologist from Mass General referred us to a geneticist there. We got an appointment for the week after Sam's follow-up with Dr. Albright.

Even though I had prepared for that second batch of X-rays with the orthopedist, I was nervous and shaky, and missed my exit. My mother-in-law, Chris, came from New York's Catskills to help, and Jim got off work and we all caravaned.

Dr. Albright took more X-rays, and Sam's bones had not improved. That much I was prepared for.

But still, the unexpected happened. One thing I'm learning as we wend our way through all this is that we can not count on anything; nothing is static.

Dr. Albright, who had initially said he would do the surgery right away if there was no improvement, worried if he operated on someone so young, Sam could lose too much blood after having the bone on one side cut. That would mean two surgeries followed by two body casts. Not only that, the doctor would have to use a less favorable method to pin his bones because he is so small.

And, he said the chance of recurrence was high, more than 50 percent.

Dr. Albright had said Sam is not in pain, but watching him carefully I am not sure I agree; he seems very sore after a lot of physical exertion, which is for him much less than other children. I'm not convinced his joints are pain-free, but really can't be sure.

Dr. Albright would consult his colleagues and call us back the following week. We spent the week swallowing the odds of Sam getting multiple surgeries.

But when Dr. Albright called us back, everything had changed again. Now he was saying that there was a new infant metal bracket for placing his bones that he could use effectively.

Recurrence would be greatly reduced if he straightened the bones enough. Even though they are so curved, he said he could do it.

His colleague, a pediatric orthopedist who specializes in hips, would also attend surgery. They all agreed it should happen within a year, but in cooler months so Sam isn't miserable in that body cast, preferably in the Fall, but we could wait until after the holidays if we wanted. Immediately I thought January -- after the holidays, and Sam would be out of the body cast by his third birthday.

Dr. Albright didn't think the geneticist would find anything, but told us to let him know. Jim and I were completely exhausted, but agreed the news was better this time than a week prior.

But, the geneticist would find something, and everything was about to change ... again.